Textbook of Oral Pathology-Anil G. Ghom, S. Mhaske (2013)

S E C O N D l D I T I O N
Textbook of
ORAL
PATHOLOGY
ft '
m * Editors
3ivSv \ m *A ANIL GOVINDRAO GIIOM
SHUBHANGI MHASKE ( lEDHE)
JAYPEE
Textbook of
ORAL PATHOLOGY
Textbook of
ORAL PATHOLOGY
Second Edition
Editors
Anil Govindrao Ghom Shubhangi Mhaske ( Jedhe )

MDS ( Oral Medicine and Radiology ) MDS (Oral Pathology )
Professor and Head Professor and Head
Department of Oral Medicine and Radiology Department of Oral Pathology
Chhattisgarh Dental College and Research Center People’s Dental Academy
Sundra, Rajnandgaon, Chhattisgarh , India Bhopal, Madhya Pradesh, India
Forewords
Manisha Sanjay Tijare
Jagdish V Tupkari
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Textbook of Oral Pathology
First Edition: 2009
Second Edition: 2013
ISBN 978-93-5090-171-7
Printed at
Contributors
Amol Gadbail Monal Yuwanati

MDS (Oral Pathology) MDS (Oral Pathology)
Lecturer Assistant Professor
Department of Oral Pathology and Department of Oral Pathology and
Microbiology Microbiology
Sharad Pawar Dental College People’s College of Dental Academy
Sawangi, Wardha, Maharashtra, India Bhopal, Madhya Pradesh, India
Anil Govindrao Ghom Pradnya Lele

MDS (Oral Medicine and Radiology) MDS (Oral Pathology)
Professor and Head Lecturer
Department of Oral Medicine and Department of Oral Pathology and
Radiology Microbiology
Chhattisgarh Dental College and Government Dental College and
Research Institute Hospital, Mumbai, Maharashtra, India
Sundra, Rajnandgaon, Chhattisgarh, India
Pranoti Pradhan
Aparna Thombre MDS (Oral Medicine and Radiology)
MDS (Oral Pathology) Professor
Reader Department of Oral Medicine and
Department of Oral Pathology and Radiology
Microbiology Maitri Dental College and Research
VSPM Dental College and Research Centre, Durg, Chhattisgarh, India
Institute, Nagpur, Maharashtra, India
Rashmi Ekka
MDS (Oral Medicine and Radiology)
Ashok Mhaske
Lecturer
MS (General Surgery)
Department of Oral Medicine and
Vice Dean, Professor and Head
Radiology
Department of Surgery
Chhattisgarh Dental College and
People’s College of Medical Sciences
Research Institute
and Research Centre
Bhopal, Madhya Pradesh, India
Sangamesh Halawar
Avadhoot Avadhani MDS (Oral Pathology)
MDS (Oral Pathology) Reader
Faculty of Dentistry Department of Oral Pathology
Sir John Walsh Research Institute Vasantdada Patil Dental College and
University of Otago Hospital
Dunedin, New Zealand Kavalapur, Sangli, Maharashtra, India
Satish Chhugani Shubhangi Mhaske (Jedhe)

MDS (Oral Medicine and Radiology) MDS (Oral Pathology)
Postgraduate Student Professor and Head
vi Department of Oral Medicine and Department of Oral Pathology
Radiology People’s Dental Academy, Bhopal,
Chhattisgarh Dental College and Madhya Pradesh, India
Research Institute
Smruti Nanda BDS

Savita Ghom Lecturer
MDS (Oral Medicine and Radiology)
Department of Oral Medicine and
Lecturer Radiology
Department of Oral Medicine and Chhattisgarh Dental College and
Radiology Research Institute
Chhattisgarh Dental College and Sundra, Rajnandgaon, Chhattisgarh, India
Research Institute
Seema Vaidya Vivek Thombre

MDS (Oral Medicine and Radiology) MDS (Periodontology)
Lecturer Reader
Department of Oral Medicine and Department of Periodontology
Radiology Chhattisgarh Dental College and
Chhattisgarh Dental College and Research Institute
Research Institute Sundra, Rajnandgaon, Chhattisgarh, India
Foreword to the Second Edition
It is at once a great privilege and pleasure to write a foreword for the invaluable compilation
of 2nd edition of Textbook of Oral Pathology by Dr Anil Govindrao Ghom and Dr Shubhangi
Mhaske (Jedhe). The authors have taken extraordinary pains and care in putting together
meticulously the data collected over many years. This has resulted in production of superb new
edition. The excellent features of this edition are that the authors covered all the topics according
to new syllabus of almost all universities. Special attraction, which I found in the book, is the
multiple choice questions at the end of each chapter and the points to remember.
An attempt has been made in the book, to simplify and make it easy to remember the subject.
The book will help the undergraduate and postgraduate students to get a bird’s eye view of the
entire topics.
I would like to congratulate the efforts of editors for designing 2nd edition of Textbook of Oral Pathology. I am sure
that the book will rapidly find a place in most progressive libraries the world over.
Manisha Sanjay Tijare MDS (Oral Pathology)

Professor and Head
Department of Oral Pathology and Microbiology
People’s College of Dental Sciences and
Research Centre
Dean, Faculty of Dentistry
Barkatullah University, Bhopal, Madhya Pradesh, India
PhD Guide
Barkatullah University, Bhopal, Madhya Pradesh, India
Executive Committee Member
Indian Association of Oral and Maxillofacial Pathologists (IAOMP)
Foreword to the First Edition
It gives me immense pleasure to write a few words about the Textbook of Oral Pathology.
This book is the outcome of combined efforts of Dr Anil Govindrao Ghom, Professor of Oral Medicine and Radiology
and Dr (Mrs) Shubhangi Mhaske (Jedhe), Associate Professor of Oral Pathology and Microbiology. I know Dr Shubhangi
as undergraduate as well as postgraduate student. I feel her sincerity coupled with hard work and humanly approach
toward the patients during her postgraduate studies contributed in making the textbook.
My heartiest congratulations to Professor (Dr) Anil Govindrao Ghom and Dr (Mrs) Shubhangi Mhaske (Jedhe) for
their great endeavor in bringing out the book. The book contains six sections which are divided into 37 chapters related
to oral lesions/diseases inclusive of basic topics of oral pathology. The topics like microscopy, stains and routine as well
as special investigations are noteworthy. The lots of updated information in the book will be helpful to undergraduates,
postgraduates and also for practising dental fraternity.
The total of over 600 clinical photographs, microphotographs and line diagrams incorporated in the book are definitely
useful for in-depth understanding of the subject. The textbooks available on these subjects are many, but only a few ones
cover both the subjects: oral medicine and radiology as well as oral pathology. Contribution of Indian authors toward
the books in dentistry is less as compared to the foreign authors. Therefore, Mrs Mhaske (Jedhe) deserves a word of
appreciation for her sincere and painstaking efforts.
The book is an excellent contribution to a scientific literature in Indian scenario and thereby facilitating our students
to understand various diseases.
With regards and best wishes
Jagdish V Tupkari
Professor and Head
Department of Oral Pathology and Microbiology
Government Dental College and Hospital
Mumbai, Maharashtra, India
Preface to the Second Edition
I may not have gone where I intended to go, but I think I have ended up where I needed to be.
― Douglas Adams
In the study of oral and dental sciences, oral pathology is the subject that concentrates on the mechanisms of the disease
process and the morphologic changes in tissue that it causes. Everyday, there are new additions to the knowledge of the
subject and we have to keep pace with it to update our students about it. That is the reason, we are here with the 2nd
edition of Textbook of Oral Pathology.
In our first edition, there is a lot of feedback which has come to improve the quality of the book. We tried to incorporate
all the feedback in the book. As due to new Dental Council of India (DCI) guidelines, multiple choice questions (MCQs)
are part and parcel of examination pattern of the Bachelor of Dental Surgery (BDS) curriculum. We have incorporated
MCQs at the end of every chapter so that students can practice it and have some insight into what type MCQs can be there
in the examination. Also, we have included ‘points to remember’ in every disease so that with respect to time, students
can revise it fast. There are also many new additions of photographs—clinical, histopathological as well as radiological
in this edition.
In spite of an essential sincere efforts, elements of human error or shortcomings are likely; the readers are welcome to
point out all such mistakes and render valuable suggestions for further improvements and shall be greatly acknowledged.
Anil Govindrao Ghom

Shubhangi Mhaske (Jedhe)
Preface to the First Edition
In the study of oral and dental sciences, oral pathology is the subject that concentrates on the morphologic changes in oral
tissues which cause diseases and the mechanisms of the disease process. Most recently published works on the subject
are the product of eminent authorities with multiple authorship with current research advances. ‘Why another book in oral
pathology’? This question can probably be answered by the phrase,
Nothing can be changed by changing the face

But many things can be changed by facing the change.
The purpose of the book is mainly to provide the undergraduate and postgraduate students, an easy way of reference
for covering a broad-spectrum of oral pathology in lucid and simple language. In support of all the composite works, it
can be stated that the progress of oral and maxillofacial pathology in its many varied specialties coming up has made the
subject too vast to be covered adequately by a single author. Also, a balance between the advances and the basic essentials
is need of the hour. With this balanced perspective and from the viewpoint of the graduate and postgraduate students under
training, the authors have endeavored to compile the oral and maxillofacial pathology with respect to the related essential
clinical oral medicine and radiology. The book gives an extensive coverage and emphasizes on detailed description,
adequate well-labeled illustrations, flow charts, recent developments and molecular aspects. Aside from oral pathology
in general, the initial phase of the book includes the basics of the embryology, anatomy and pathology. The study of
microscope, tissue processing, diagnostic tests and advanced techniques are also included. The photomicrographs and the
clinical photographs in the book signify the adage: “A picture is worth a thousand words”; as the reader is encouraged to
study the details and to clarify the confusion of the topics.
In spite of sincere efforts, elements of human error or shortcomings are likely; the readers are welcome to point out
all such mistakes and render valuable suggestions for further improvements and shall be greatly acknowledged.
Anil Govindrao Ghom

Acknowledgments
This book could not have been written without the encouragement and motivation of my teachers and students. I would
also like to express my gratitude for my contributors Drs Sangamesh Halawar, Pranoti Pradhan, Monal Yuwanati,
Pradnya Lele, Aparna Thombre, Avadhoot Avadhani, Vivek Thombre, Amol Gadbail, Rashmi Ekka, Seema Vaidya,
Smruti Nanda, Savita Ghom, Satish Chhugani, without whose help and cooperation writing the book would have been
an uphill task.
I would like to acknowledge the efforts of my Postgraduate students Satish, Manjari, Mansi and Bharani. I am also
thankful to Drs Swati Arora and Varun Rastogi, Senior Lecturers, Department of Oral and Maxillofacial Pathology, Kalka
Dental College, Meerut, Uttar Pradesh, India, for their contribution of different classification systems of odontogenic
tumors.
Last but not least, my dear wife, “As fish is without water, so is me without my wife Savita.” Her contribution to my
life is beyond what I can express in words. I am also thankful to my daughter Milini and son Sanvil for their love and
affection.
Finally, I am indebted to the almighty for presenting me with such wonderful opportunities and people in this life.
Anil Govindrao Ghom
I dedicate my work to my caring devoted mother, Late Mrs Sulochana Jedhe to whom I owe special idiosyncratic
gratitude throughout my life. I express my profound thankfulness to my enthusiastic, dynamic and empathetic husband
Dr Ashok Mhaske (Vice Dean, Professor and Head, Department of Surgery, People’s College of Medical Sciences and
Research Centre); my compassionate son Sumedh; benevolent father Shri Shrikant Jedhe (Retd), Additional Registrar,
Cooperative Societies, Maharashtra Government; munificent parents-in-laws Anna and Aai (Shri NS Mhaske and
Mrs Rukhmini Mhaske); knit family ties Niket, Samir (Engineer), Sharayu Tayade (Engineer), Shirin, Dr Mandakini,
Dr Maya and Advocate Dharmanand.
I extend my indebted thankfulness to all who contributed especially my colleagues, relatives, friends, and dear
students. Each one provided extremely and distinctly valuable support for the completion of this work. My special thanks
to Honorable Suresh Vijaywargiya, Chairman, People’s Group, Bhopal; Ms Megha Vijaywargiya, Director (Human
Resource), People’s Group, Bhopal, Madhya Pradesh, India, for the inestimable support and my colossal inspiration;
Dr Jagdish V Tupkari, Professor and Head, Department of Oral Pathology and Microbiology, Government Dental College
and Hospital, Mumbai, Maharashtra, India; Dr Suresh Barpande, Dean, Government Dental College and Hospital,
Aurangabad; Dr Vinay Hazare, Dean, Government Dental College and Hospital, Nagpur, Maharashtra, India; Dr MK
Gupta, Dean, People’s Dental Academy, Bhopal, Madhya Pradesh, India; Dr Alka Kale, Dean, KLE Institute of Dental
Sciences, Belgaum, Karnataka, India; Dr PV Wanjari and Dr Sangeeta Wanjari, Professor and Head, Department of Oral
Pathology, Modern Dental College, Indore, Madhya Pradesh, India.

Contents
1. MICROSCOPE 1
Shubhangi Mhaske (Jedhe )
Definition of Microscope 1 ; History of Microscope 1 ; Simple Microscope 3;
Compound Microscope 3; Parts of Microscope 3; Image Formation in
Microscope 10 ; Specialized Microscopy Techniques 11 ; Maintenance of Laboratory
Microscope 19
2. TISSUE PROCESSING METHODS 22

Shubhangi Mhaske ( Jedhe ) , Avadhoot Avadhani
Introduction and Terminology 22 ; Gross Examination 22; Preparation of Tissue Specimen
for Histological Staining 23; Routine Method for Histological Study 25; Study ofHard
Tissues 30 ; Frozen Sections 32; Staining of Cut Sections 32; Mounting 33; Artifacts
in Histological Sections 33
3. HISTOLOGICAL STAINING METHODS 36

Shubhangi Mhaske ( Jedhe ) , Amol Gadbail
Chemistry of Stains 36 ; Classification of Stains 37 ; Theories of Staining 38 ; Vital
Staining 39 ; Factors Affecting Staining 39 ; Staining Procedure 40 ; Hematoxylin and
Eosin Stains 40 ; Special Stains 42 ; Gordon and Sweets ' Methodfor Reticulin Fibers 43
4. DIAGNOSTIC PATHOLOGY 47
Shubhangi Mhaske ( Jedhe ) , Pradnya Lele
Biopsy 47; Types of Biopsy Procedures 48 ; Exfoliative Cytology 51 ; Oral
Mucosal Brush Biopsy 54 ; Liquid Based Cytology 55 ; Fine Needle Aspiration
Cytology 55 ; Frozen Section Biopsy 56
5. ADVANCED DIAGNOSTIC TECHNIQUES 57

Shubhangi Mhaske (Jedhe ) , Monal Yuwanati
Hi stoche mi cal Techniques 57 ; Fixation in Histochemistry 57 ; Enzyme Histochemistry 58 ;
Immunohistochemical Methods 58 ; Immunofluorescent Techniques 59 ;
Flow Cytometry 61 ; Polymerase Chain Reaction 61 ; Hybridization Methods 62;
Laser Captures Microdissection 63; Proteomics 63; Cytogenetics 64
6. HEALING OF WOUND 68
Shubhangi Mhaske ( Jedhe )
Factors Affecting the Wound Healing 68; Cascade of Wound Healing 72;
Healing of Biopsy Wounds 74 ; Healing of Extraction Wounds 74; Healing of
Fractures 75; Healing of Osseointegrated Implants 76 ; Healing of Pulp 76 ;

Cementum 77 ; Dentin 77; Enamel 77; Skin Healing and Oral Mucosal Wound
Healing 77 ; A Clinical Approach to Optimizing Wound Healing 79
7. HYPERPLASIA , HAMARTOMA AND NEOPLASM 82

Dysplasia 82; Metaplasia 82; Hyperplasia 82; Hamartoma 83; Choriostoma 84 ;
Neoplasm 85 ; Carcinogenesis 87; Chemical Carcinogenesis 87 ; Physical
Carcinogenesis 88 ; Hormonal Carcinogenesis 89 ; Biologic Carcinogenesis 89 ;
Metastasis 92 ; Grading and Staging of Tumors 93
8. TEETH ANOMALIES 97
Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe ) , Savita Ghom
Disorders of Development of Teeth 97 ; Scale of Human Tooth Development 98 ; Disorders
of Size of Teeth 98 ; Disturbances in Shape of Teeth 99 ; Disorders of Number of
Teeth 109 ; Structure of Teeth 111
9. CRANIOFACIAL ANOMALIES 111

Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe )
Developmental Anomalies of Jaws 128 ; Developmental Disorders of Oral Mucosa 138
10. DENTAL CARIES 144

Theories of Cariogenesis 145 ; Secondary Contributing Factors in Dental Caries 150 ;
Classification 152; Smooth Surface Caries 153 ; Pit and Fissure Caries 156 ; Root
Caries 158 ; Recurrent Caries 160 ; Chemical Measures of Caries Control 164
11. BENIGN TUMORS 167

Characteristics of Benign Tumor 168 ; Classification of Benign Tumor 168 ; Epithelial
Origin 168 ; Fibrous Connective Tissue 178 ; Cartilage 184 ; Adipose Tissue 186 ;
Bone 189; Vascular Tissue 196 ; Neural Tissue 203; Muscle 211 ; Giant Cell
Lesion 213
12. PREMALIGNANT LESIONS AND CONDITIONS 219

Concept of Precancer 219 ; Terminology and Definitions 219 ; Leukoplakia 220 ;
Erythroplakia 229 ; Carcinoma in situ 231 ; Oral Lesion Associated with use of
Tobacco 233; Lichen Planus 235; Oral Submucous Fibrosis 243 ; Dyskeratosis
Congenita 249 ; Lupus Erythematosus 250
Contents
13. MALIGNANT TUMORS 255

Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe ) , Ashok Mhaske
Classification 256 ; Etiology and Risk Factors for Oral Cancer 256 ; Risk Factors 258 ;
Epithelial Tumors 258 ; Metastatic Carcinoma 266 ; Basal Cell Carcinoma 267;
Adenosquamous Carcinoma 269 ; Basaloid Squamous Carcinoma 269 ; Sinonasal
Undifferentiated Carcinoma 270 ; Verrucous Carcinoma 271 ; Transitional Cell
Carcinoma 273; Malignant Melanoma 273; Spindle Cell Carcinoma 277;
Adenoid Squamous Cell Carcinoma 277 ; Nasopharyngeal Carcinoma 278 ; Merkel Cell
Carcinoma 279 ; Fibrous Connective Tissue 279 ; Malignant Fibrous Histiocytoma 282;
Synovial Sarcoma 282; Adipose Tissue 283; Cartilage 284 ; Mesenchymal
Chondrosarcoma 287; Bone 287; Ewing 's Sarcoma 289 ; Vascular 291 ; Neural
Tissue 292; Muscle 294
14. ODONTOGENIC TUMORS 299

Classification of Odontogenic Tumors 299 ; Development of Tooth 301 ; Stages of Tooth
Development 301 ; Ameloblastoma 303; Variant of Ameloblastoma 313;
Squamous Odontogenic Tumor 317 ; Calcifying Epithelial Odontogenic Tumor 318 ;
Adenomatoid Odontogenic Tumor or Cyst 321 ; Mixed Odontogenic Tumors 325 ;
Continuum Concept (Cahn and Blum) 325; Ameloblastic Fibroma 326 ;
Ameloblastic Fibrodentinoma 328 ; Ameloblastic Fibro-odontoma 328 ; Odontoma 329 ;
Odontoameloblastoma 331 ; Odontogenic Fibroma 332; Granular Cell Odontogenic
Tumor 333; Odontogenic Myxoma 333; Malignant Tumors 335
15. CYST OF OROFACIAL REGION 342

Classification 343; Theories of Cyst Enlargement 343; Dentigerous Cyst 345;
Eruption Cyst 349 ; Odontogenic Keratocyst 350 ; Primordial Cyst 355 ; Gingival Cyst
of Newborn 355 ; Gingival Cyst of Adult 356 ; Lateral Periodontal Cyst 357; Glandular
Odontogenic Cyst 358 ; Calcifying Epithelial Odontogenic Cyst 359 ; Inflammatory
Radicular Cyst 361 ; Residual Cyst 364 ; Inflammatory Collateral Cyst 365 ; Paradental
Cyst 365; Mandibular Buccal Infected Cyst 366 ; Suppurating Cyst 366 ; Healing
Cyst 366 ; Nonodontogenic Cysts 366 ; Median Palatine Cyst 368 ; Nasoalveolar
Cyst 369 ; Median Mandibular Cyst 370 ; Globulomaxillary Cyst 370 ; Nonepithelial
Cysts 370 ; Aneurysmal Bone Cyst 371 ; Cysts of the Maxillary Sinus 372; Antral
Pseudocyst 374 ; Retention Cyst 374 ; Soft Tissue Cyst 374 ; Branchial Cleft
Cyst 376 ; Oral Lymphoepithelial Cyst 376 ; Thyroglossal Duct Cyst 377; Anterior
Median Lingual Cyst 377 ; Oral Cyst with Gastric or Intestinal Epithelium 377 ; Cystic
Hygroma 377; Follicular Cysts of the Skin 378 ; Nasopharyngeal Cyst 378 ; Thymic
Cyst 378 ; Cysts of Salivary Glands 378 ; Parasitic Cyst 378 ; Cysticercosis
Cellulose 379 ; Syndromes Associated with Odontogenic Cysts 380 ; Treatment of
Cysts 381
16. PERIODONTAL PATHOLOGY 386

Vivek Thombre , Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe )
Fibromatosis Gingiva 386 ; Retrocuspid Papilla 388 ; Gingival Inflammation or
Gingivitis 388 ; Necrotizing Ulcerative Gingivitis 391 ; Desquamative Gingivitis 392 ;
Plasma Cell Gingivitis 393; Granulomatous Gingivitis 394 ; Gingival Abscess 394 ;
Pericoronal Abscess 395; Chronic Inflammatory Enlargement 396 ; Gingival Enlargement
due to Drugs 397 ; Pregnancy Tumor 398 ; Granuloma Pyogenicum 399 ;
Periodontal Pockets 401 ; Adult Periodontitis 402; Rapidly Progressive Periodontitis 402 ;
Aggressive Periodontitis/Juvenile Periodontitis 403; Papillon-Lefevre Syndrome 405 ;
Haim-Munk Syndrome 406
17. SALIVARY GLAND PATHOLOGY 409

Classification of Salivary Gland Disorders 410 ; Development of Salivary
Gland 410 ; Major Salivary Glands 411 ; Aberrancy 412 ; Aplasia and
Hypoplasia 412; Hyperplasia of Salivary Gland 413 ; Atresia 413; Accessory
Duct 414 ; Diverticuli 414 ; Sialorrhea 414 ; Xerostomia 414 ; Sialolithiasis 415;
Strictures and Stenosis 417 ; Mucocele (Mucous Extravasation Phenomenon) 417; Salivary
Duct Cyst or Mucus Retention Cyst 419 ; Ranula 419 ; Sialosis (Sialadenosis) 420 ;
Allergic Sialadenitis 421 ; Mumps 421 ; Cytomegalovirus Inclusion Disease 422 ;
Bacterial Sialadenitis 422; Sjogren s Syndrome 424 ; Mikulicz s Disease or
Benign Lymphoepithelial Lesion 427; Uveoparotid Fever 428 ; Tumors of
Salivary Glands 428 ; Histogenesis 428 ; Theories of Salivary Gland Tumor
Histogenesis 429; General Features of Salivary Gland Tumors 429 ;
Clinical Staging of Salivary Gland Tumors 429 ; Pleomorphic Adenoma 430 ;
Basal Cell Adenoma 433; Canalicular Adenoma 434 ; Warthin 's Tumor 435 ;
Oncocytoma 437; Myoepithelioma 438 ; Ductal Papillomas 438 ; Mucoepidermoid
Carcinoma 440 ; Central Mucoepidermoid Carcinoma 444 ; Acinic Cell
Adenocarcinoma 444 ; Adenoid Cystic Carcinoma 445 ; Polymorphous Low -
Grade Adenocarcinoma 447 ; Malignant Mixed Tumor 448 ; Connective Tissue
Tumors 449 ; Necrotizing Sialometaplasia 450
18. BACTERIAL INFECTION 453

Impetigo 453; Erysipelas 454 ; Syphilis 455 ; Gonorrhea 460 ; Leprosy (Hansen
Disease ) 462; Tuberculosis 465; Actinomycosis 468 ; Noma 471 ; Scarlet Fever 472;
Diphtheria 473 ; Tularemia 474 ; Rhinoscleroma 474 ; Granuloma Inguinale 475; Oral
Manifestations 475 ; Streptococcal Tonsillitis and Pharyngitis 476 ; Tonsillar Concretion
and Tonsillolithiasis 476 ; Lymphogranuloma Venereum 476 ; Myiasis 477 ; Cat Scratch
Disease 478 ; Pyostomatitis Vegetans 479 ; Sinusitis 479
19. FUNGAL OR MYOCOTIC INFECTION 484

Candidiasis 484 ; Oral Candidiasis 486 ; Chronic Mucocutaneous Candidiasis 491 ;
Forms ofCandidiasis 491 ; Histoplasmosis 492; Blastomycosis 493; Mucormycosis 495;
Contents
Cryptococcosis 496 ; Coccidioidomycosis 497; Geotrichosis 498 ; Sporotrichosis 498 ;

Rhinosporidiosis 499 ; Aspergillosis 500 ; Paracoccidioidomycosis 501 ;
Toxoplasmosis 502; Leishmaniasis 502; Trichinosis 503
20. VIRAL INFECTION 505

Human Herpes Virus 505 ; Herpes Simplex Infection 505 ; Measles 509 ; Varicella Zoster
Infection 510 ; Herpes Zoster 512; James Ramsey Hunt Syndrome 514 ; Rubella 514 ;
Enteroviruses 514 ; Foot and Mouth Disease 516 ; Condyloma Acuminatum 517 ;
Verruca Vulgaris 518 ; Focal Epithelial Hyperplasia 518 ; Molluscum Contagiosum
Infection 519 ; Cytomegalovirus Infection 520 ; Infectious Mononucleosis 521
21. ACQUIRED IMMUNODEFICIENCY SYNDROME 524

Classification 525 ; AIDS Related Complex 526 ; Prevalence 526 ; Characteristic of
HIV Virus 527; Clinical Features 527 ; Oral Manifestations 528 ; Uncommon Oral
Manifestation of HIV 534 ; Diagnostic Tests 536 ; Screening Test for AIDS 536 ;
Management 537 ; Prevention 538
22. ODONTOGENIC INFECTION AND PULP PATHOLOGY 540

Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe ) , Seema Vaidya
Effect of Infection on Host 541 ; Pathophysiology of Infection 541 ; Pulp 542;
Classification of Pulpitis 542; Pulpitis 542; Pulp Degeneration 545 ; Pulp
Calcifications 546 ; Necrosis of Pulp 548 ; Cracked Tooth Syndrome 549 ; Periapical
Abscess 549 ; Periodontal Abscess 551 ; Acute Exacerbation of a Chronic Lesion 552;
Periapical Granuloma 553; Osteomyelitis 555; Acute Suppurative Osteomyelitis 557 ;
Chronic Suppurative Osteomyelitis 559 ; Infantile Osteomyelitis 560 ; Synovitis, Acne ,
Pustulosis, Hyperostosis and Osteomyelitis Syndrome 562; Chronic Recurrent Multifocal
Osteomyelitis 563; Cellulitis 565; Ludwig 's Angina 566 ; Fatal Complications of Oral
Infection 568 ; Oral Foci of Infections 571 ; Dry Socket 573
23. BONE DISEASE MANIFESTED IN JAW 576

Anil Govindrao Ghom , Shubhangi Mhaske ( Jedhe ) , Pranoti Pradhan
Fibro-osseous Lesions 576 ; Classification 576 ; Fibrous Dysplasia 577 ; Cherubism 580 ;
Central Giant Cell Granuloma 582; Paget 's Disease 584 ; Familial Gigantiform
Cementoma 587 ; Ossifying Fibroma, Cementifying Fibroma and Cemento-ossifying
Fibroma 588 ; Juvenile Ossifying Fibroma 590 ; Osteoporosis 591 ; Infantile Cortical
Hyperostosis 593; Osteopetrosis 594 ; Osteogenesis Imperfecta 596; Pierre Robin
Syndrome 597 ; Marfan 's Syndrome 597 ; Down 's Syndrome 598 ; Achondroplasia 599;
Osteosclerosis 600 ; Massive Osteolysis 600 ; Gardner ' s Syndrome 601
24. DISEASES OF LIP 604

Classification of Lip Disorders 604 ; Anatomy 604 ; Developmental Disturbance of
Lip 605 ; Cheilitis 610 ; Etiology 610 ; Miscellaneous 617
25. TONGUE DISORDERS 619

Embryology of Tongue 619 ; Anatomy of Tongue 620 ; Papillae 620 ; Muscle 621 ;
Arterial Supply 621 ; Venous Drainage 621 ; Nerve Supply 621 ;
Lymphatic Drainage 622; Functions of Tongue 622 ; Classification of Tongue
Disorders 623; Aglossia and Microglossia 623; Macroglossia 624 ;
Ankyloglossia 625 ; Cleft Tongue 626 ; Ankyloglossum Superius Syndrome 626 ; Lingual
Varicosities 627; Lingual Thyroid Nodule 627; Variations in Tongue Movement 628 ;
Patent Thyroglossal Duct Cyst 628 ; Lingual Polyp 629 ; Lingual Cyst 629 ; Fissured
Tongue 629 ; Median Rhomboid Glossitis 630 ; Benign Migratory Glossitis 631 ; Hairy
Tongue 633; Crenated Tongue 634 ; Foliate Papillitis 634 ; Leukokeratosis Nicotina
Glossi 634 ; Depapillation of the Tongue 635; Dysgeusia and Hypogeusia 637;
Dyskinesia 638 ; Paralysis of Tongue 638 ; Squamous Cell Carcinoma 639 ;
Pigmentation of Tongue 640
26. TEMPOROMANDIBULAR JOINT PATHOLOGY 643

Coronoid Hyperplasia 643; Condylar Hyperplasia 643; Condylar Hypoplasia 644 ;
Bifid Condyle 645; Osteoarthritis 645 ; Rheumatoid Arthritis 646 ;
Ankylosis 648 ; Subluxation (Hypermobility) 650 ; Gout 651 ; Synovial
Chondromatosis 652; Temporomandibular Joint Dysfunction 652
27. CHEMICAL AND PHYSICAL INJURIES 655

LineaAlba 655 ; Habitual Cheek or Lip Biting 656 ; Traumatic Ulcer 657 ; Electrical
and Thermal Burns 658 ; Anesthetic Necrosis 659 ; Chemical Burns 659 ; Smoker
Melanosis 661 ; Drug Induced Discoloration of Oral Mucosa 661 ; Outright
Lesion 662; Traumatic Sequestration 662; Methamphetamine Abuse
Lesion 663; Submucosal Hemorrhage 663; Oral Lesion as Complication to Anti-
Neoplastic Therapy (Non-Infectious) 664 ; Cervicofacial Emphysema 665;
Myospherulosis 666 ; Attrition 666 ; Abrasion 668 ; Erosion 669 ; Abfraction 670 ;
Dentinal Sclerosis 671 ; Secondary and Tertiary Dentin 671 ; Resorption of Teeth 672;
Hypercementosis 675; Cementicles 676 ; Bruxism 676 ; Traumatic Lesion
Due Sexual Habit 678 ; Oral Piercing and other Body Modification 679 ;
Fracture of Teeth 680 ; Amalgam Tattoo 681 ; Bismuthism 681 ; Plumbism 682;
Mercurialism 683; Argyria 684 ; Arsenism 685 Auric Stomatitis 685 ; Inflammatory
Fibrous Hyperplasia 685; Inflammatory Papillary Hyperplasia 686 ; Epulis
Granulomatosum 687 ; Nodular Fasciitis 688 ; Uremic Stomatitis 688 ; Traumatic
Keratosis 689 ; Bisphosphonates Associated Osteonecrosis 689
28. BLOOD PATHOLOGY 693

Disease of Lymph Tissue 693 ; Disease of Red Blood Cells 694 ; White
Blood Cell Disorders 705; Disease of Platelet 708 ; Disease due to Clotting
Defect 710 ; Dysfibrinogenemia 713 ; Macroglobulinemia 714 ; Malignancy Involving
Contents
Blood Tissue 714 ; Primary Reticular Cell Sarcoma 717 ; Mycosis Fungoides 718 ;
Burkitt ’s Lymphoma 718 ; Chronic Myeloid Leukemia 723; Chronic Lymphatic
Leukemia 724 ; Multiple Myeloma 725 ; Plasmacytoma 727 ; Extranodal NK/T-Cell
Lymphoma 728
29. SKIN DISORDERS 731

Erythema Multiforme 731 ; Pemphigus 734 ; Paraneoplastic Pemphigus 737 ;
Bullous Pemphigoid 737 ; Benign Mucous Membrane Pemphigoid 738 ; Familial
Benign Chronic Pemphigus 740 ; Dermatitis Herpetiformis 742; Pityriasis
Rosea 743; Incontinentia Pigmenti 743; Acanthosis Nigricans 744 ; Ehlers Danlos
Syndrome 745 ; Psoriasis 746 ; Pachyonychia Congenita 747 ; Porokeratosis 748 ;
Keratosis Follicularis 749 ; Warty Dyskeratoma 750 ; Seborrheic Keratosis 750 ;
Hereditary Mucoepithelial Dysplasia 751 ; Pseudoxanthoma Elasticum 751 ; Hyalinosis
Cutis Et Mucosa Oris 752; White Sponge Nevus 753; Hereditary Benign Intraepithelial
Dyskeratosis 754 ; Hereditary Hemorrhagic Telangiectasia 754 ; Peutz-Jeghers
Syndrome 755; Ephelis 755 ; Actinic Lentigo 756 ; Lentigo Simplex 756 ; Sebaceous
Hyperplasia 756 ; Xeroderma Pigmentosum 757 ; Tuberous Sclerosis 757 ; Ectodermal
Dysplasia 758 ; Cow den Syndrome 760 ; Graft versus Host Resistance 760 ; Crest
Syndrome 761 ; Scleroderma 761 ; Kawasaki Disease 763
30. ALLERGIC AND IMMUNOLOGIC DISEASES OF ORAL CAVITY 766

Introduction/Overview 766 ; Hypersensitivity Reaction 766 ; Wegner ’ s
Granulomatosis 767; Sarcoidosis 769 ; Drug Allergy 770 ; Allergic Contact
Stomatitis 771 ; Secondary Vaccinia 772; Angioedema 773; Aphthous Stomatitis
(Recurrent Aphthous Ulcers (RAUs) or Canker Sores) 774 ; Behqet ’s Syndrome 776 ;
Transient Lingual Papillitis 777 ; Perioral Dermatitis 778 ; Reiter ’ s Syndrome 778 ;
Lichenoid Contact Stomatitis/Lichenoid Tissue Reaction 779 ; Chronic Ulcerative
Stomatitis 781 ; Crohn ’s Disease 782
31. ENDOCRINE DISORDERS 784

Anatomy and Physiology 784 ; Diseases of Pituitary Gland 785; Progeria 788 ;
Hyperthyroidism 788 ; Hypothyroidism 790 ; Hyperparathyroidism 791 ;
Hypoparathyroidism 793 ; Pseudohypoparathyroidism 793; Diabetes Mellitus 794 ;
Addison 's Disease 796 ; Adrenogenital Syndrome 797 ; Melasma 797 ; Cushing ’s
Syndrome 797
32. NUTRITION AND ORAL CAVITY 800

Disturbances in Protein Metabolism 800 ; Disturbances in Lipid
Metabolism 804 ; Disturbances in Carbohydrate Metabolism 807 ; Disturbances in
Mineral Metabolism 809 ; Miscellaneous Disorders 811 ; Fat Soluble Vitamins 821 ;
Disorders of Bilirubin 825
33. NEUROMUSCULAR DISORDERS AND OROFACIAL PAIN 830

Muscle Disorders 830 ; Neuromuscular Disorders 834 ; Facial Pain 839
34. FORENSIC ODONTOLOGY 849

Anil Govindrao Ghom , Savita Ghom
Record Management 849 ; Identification 850 ; Dental Evaluation 850 ; Personal
Recognition 853; Fingerprinting 853 ; Physical Anthropologic Examination of Bones and
Teeth 853 ; Postmortem Serology and DNA Profiling 854 ; Bite Marks 854 ; Human
Abuse 858 ; Dentist as Expert Witness 859
35. SYNDROMES OF THE OROFACIAL REGION 860

Syndromes Associated with Craniofacial Anomalies of Genetic Origin 862; Syndromes
Associated with Skin and Pigmentation 868 ; Broad Groups of Pigmentary Disorders 871 ;
Syndromes Associated with Salivary and Lacrimal Glands 872; Syndromes Affecting
Teeth 873 ; Syndromes Associated with Lips and Cheek 875; Syndromes Associated
with Tongue 876 ; Syndromes Associated with Gingiva 877 ; Syndromes Associated with
Nerves 878 ; Syndromes Associated with Blood 880 ; Syndromes Associated with Vascular
Malformations 882; Syndromes Associated with Immunodeficiency 882 ; Syndromes
Associated with Hormonal Disturbances 883; Syndromes with Benign Oral Neoplastic or
Hamartomatous Components 884
APPENDICES 889
Appendix I: Differential Diagnosis of Most Common Lesions of Oral Cavity 890
Aparna Thombre
Appendix II: Glossary 938
Rashmi Ekka
Appendix III: Normal Values of Various Laboratory Parameters 951
Smruti Nanda
Appendix IV: Classification Systems of Odontogenic Tumor 953
Satish Chhugani
Appendix V: Histology Diagrams of Oral Tissues 978
Sangamesh Halawar
ANSWERS KEY OF MCQS 1003
Index 1005
Microscope
A Chapter Outline
3 Definition • Mechanical tube

3 History of microscopy • Eyepieces
3 Simple microscope 3 Micrometry
3 Compound microscope 3 Image formation in microscope
3 Parts of microscope 3 Specialized microscopy techniques
• Light source • Stereomicroscope
•Dark field microscope
• Lens
•Phase contrast microscope
• Illumination in microscope •Polarized microscopy
• Condensers •Fluorescence microscopy
• Object mechanical stage •Confocal microscope
• Objectives •Electron microscope
• Nosepiece 3 Maintenance of microscope
The light microscope, now 400 years old , is the standard HISTORY OF MICROSCOPE
instrument for the examination of histological preparations .
( FIGS 1.1 AND 1.2 )
The word microscope is derived from two Greek words
micro meaning small and scope meaning to view . Thus, it The early pioneers in the history of the microscope are
is an instrument which enables us to view small objects . Digges of England and Hans and Zachcharias Janssen
It magnifies (enlarges) the image of that small object and (1590 ) of Holland , Robert Hooke (1665 ), John Marshal
thus makes it possible to be seen by the viewer. (1700 ), Martin Frobenius Ledermuller (1768 ), Louis
Jablot (1755 ), Meyen (1747). But it was Antony van
DEFINITION OF MICROSCOPE Leeuwenhoek who was the first to make and use a real
An optical instrument that uses a lens or a combination microscope (Table 1.1).
of lenses to produce magnified images of small objects, Early microscopes were simple microscopes but with
especially of objects too small to be seen by the unaided eye . advance of science compound microscopes were built .
Figure 1.1 Antony van Leeuwenhoek microscope Figure 1.2 Robert Hooke microscope
Table 1.1 Contributors in history of microscope

Year Inventor/Scientist Contribution
Circa 1000AD Unknown Reading stone, A glass sphere that magnified when laid on top of
reading material
Circa 1284 Salvino D’Armate (Italy) Inventing the first wearable eye glasses
1590 Watchmakers Multiple lenses placed in a tube magnified object kept in front
Hans and Zachcharias Janssen (Holland)
1665 Physicist Looked at a sliver of cork through a microscope lens and noticed
Robert Hooke (England) some “pores” or “cells” in it
1674 Antony van Leeuwenhoek Built a simple microscope with only one lens to examine blood, yeast,
(Father of Microscopy) insects and many other tiny objects
1830 Joseph Jackson Lister Reduction of spherical aberration or the “chromatic effect” by lens
combinations without blurring the image for compound microscope
1872 Ernst Abbe “Abbe Sine Condition” formula for the maximum resolution in
microscopes
1903 Richard Zsigmondy Developed the ultramicroscope
1932 Frits Zernike Phase-contrast microscope
1931 Ernst Ruska Electron microscope
1981 Gerd Binnig and Heinrich Rohrer Scanning tunneling microscope
Microscope
SIMPLE MICROSCOPE
A simple microscope consists of a single lens or a
magnifying glass. 3
Principle: A lens of short focal length is used to produce an
enlarged image of an illuminated object at a short distance;
the lens fixed in a frame is adjustable to view the object.
The shorter the focal length, the larger the magnified image.
COMPOUND MICROSCOPE
(FIGS 1.3A AND B)
A compound microscope consists of two or more lenses.
Principle: If a lens of short focal length is used to produce
an enlarged image of an illuminated object at a short
distance, then another lens can be so fixed that it would
produce a further enlargement of that image. A B
Figures 1.3A and B The first compound microscope
PARTS OF MICROSCOPE
Fluorescent microscope: Uses ultraviolet light with a
Microscope Part: shorter wavelength below 400 mm which a light microscope
cannot. It can demonstrate, with the help of fluorochrome
• Light source
dyes. High pressure mercury lamp, halogen lamps are used
• Lens
generate ultraviolet light. Light source used in fluorescent
• Illumination in microscope
microscope is different, i.e. in modern microscope high
• Condensers
intensity illumination systems are used. They should to be
• Object mechanical stage
used with specialized filters for protection of eyes.
• Objectives
• Nosepiece Electron microscope: This technique of microscopy
• Mechanical tube is different from light microscopy as it uses a stream of
• Eyepieces. electrons in a magnetic field. This stream of electrons has
a very short wavelength (a 50 KV electron beam produces
Light Source light of 0.0055 nm) This is one hundred thousandth that of
the visible light.
The light microscope uses natural daylight or artificial
visible light. (The resolution of light microscope is limited Illumination
by wavelength of its light source). A progression of light
In light microscope two different types of illuminations are
sources has developed from oil lamps to the low voltage
used (Figs 1.4A and B)
electric lights of today.
In histopathology laboratories microscopes with three Critical illumination: When the object and light source
different types of light sources are found, the conventional from the substage condenser is in the same plane it is called
light microscope using natural or artificial visible light, as the critical illumination, as commonly used in simple
the fluorescence microscope using ultraviolet light, and equipments, but this produces uneven illumination of the
electron microscope using a beam of electrons. object though modern filament lamps are used.
Light microscope: Uses a visible light of 400 to 800 nm Kohler illumination: This is used for specialized type of
wavelengths to illuminate an object up to 0.2 mm made microscopy where an image of the light source is focused
visible with perfect optics given in a microscope. In light by the lamp collector or field lens in the focal plane of the
microscope two different types of illuminations are used. condenser. The image of the field or lamp diaphragm is
∙ Negative: It is concave shaped and diverge rays and

forms virtual image.
4 Condensers
Light from the lamp is directed into the first major optical
component—the sub stage condenser-either directly or
from a mirror or prism.
The main purpose of the condenser is to focus or
concentrate the available light into the plane of the
object, i.e. the condenser collects the maximum possible
light reflected by the mirror or the inbuilt light source
and condenses or converges it to a very small area at the
position of the specimen.
A B Condensers used for routine microscopy should have
Figures 1.4A and B Critical and Kohler illumination the same numerical aperture. The ideal condenser should
form a true image of the light source. It is practically useful
to have a condenser with a top lens that can be swung out
of the path of light, thus filling the whole field with light
when very low power objective are used (Fig. 1.6). Three
types of condensers are used.
Abbe condenser: Named after Ernst Abbe. It is simplest
and least expensive type. Because of its simplicity and good
light gathering capacity, it is used with most microscopes
unless specified otherwise; it has an NA of 0.25. It consists
of two lens elements (Figs 1.7A to C). Abbe condenser is
not corrected for spherical and chromatic aberration but
serves well for general observation. Some types of Abbe
condensers are “variable focus condensers” in which the
upper lens element is fixed and lower lens is focusable.
When the lower element is raised to it is to position it is
Figure 1.5 Types of lenses used in optical components of

microscope
focused in the object plane and the aperture diaphragm is

in turn focused at the back focal plane of the objective and
can be examined with the eyepiece removed.
Lens
It is named lenses because shaped like the seeds of lentil.
Piece of glass or other transparent material, usually circular,
having two surface ground or polished in a specific form in
order that light ray passing through it either converges or
diverges. There are two types of lens which are used. They
are (Fig. 1.5):
∙ Positive: It can be convex shaped and converges rays
of light forming real image Figure 1.6 Parts of condenser
Microscope
A B C
Figures 1.7A to C Types of condensers used in modern microscopes. (A) Aplantic condenser; (B) Achromatic condenser;
(C) Achromat/aplanat condenser
similar to the above condenser. But when its position is

lowered, light is focused in between the elements, thus the
light can emerge as a large diameter parallel bundle. For
10x the field area is larger. To illuminate this large area the
top lens element is removed to achieve the illumination of
entire field. For medium and high magnification the top lens
element of the variable focus condenser remains in place.
Aplanatic condensers: These types of condensers are
optically corrected for spherical aberration. These are not
available form all microscope manufacturers, but are of
better quality than Abbe condensers (Figs 1.7A to C).
Figure 1.8 Parts of mechanical stage
The achromatic condensers: These are corrected for both
spherical and chromatic aberrations. It has NA of 1.40.
Because of its high degree of correction, it is recommended
for research microscopy and for color photomicrography Objectives
where the highest degree of perfection in the image is Performance of a microscope is dependent wholly on the
desired (Figs 1.7A to C). quality of the optics—the objectives. The main task of
objective is to collect maximum light possible from the
Object Stage object, unite it and form a high quality magnified image
A rigid platform above the condenser which supports the some distance above.
glass slide is object stage. This object stage has an aperture Every objective has a fixed working distance, focal
in the center through which the light can pass to illuminate length, magnification and numerical aperture (NA) (Fig.
the specimen on the glass slide (Fig. 1.8). 1.9).
The stage holds the slide firmly and allows the slide The working distance is the distance between an object
movements with a mechanical vertical and horizontal in focus and the front of the lens system.
adjustment screws. The mechanical stage is graduated The focal length is the distance from the center of
with Vernier scales and the x and y movements assist a simple lens to the point at which parallel rays of light
the operator to return to an exact desired location in the are brought to a sharp focus; in the compound lens it
specimen. Traveling range in most of the microscopes is is the distance between an object in focus and a point
76 mm(X) 30 mm(Y). approximately halfway between the component lenses.
Table 1.2 Color codes used for objectives

Objective color codes
6 Magnification Color code
4x Red
10x Yellow
40x Light blue
100x White
Numerical aperture depends primarily on the extreme

range of the divergent rays that can be made to admit
into the lens (angular aperture) and secondarily on the
refractive index of the medium between the object and
the objective. The relation between numerical aperture,
Figure 1.9 Specifications mentioned on objectives
angular aperture and refractive index is
NA = Refractive index × sine angular aperture

Magnification
It is product of magnification values of eyepiece and The numerical aperture for any objective is always
objective in a standard microscope. imprinted on its mount. A 10x achromatic objective usually
Magnification in a standard microscope with tube has a numerical aperture of 0.25 and a 20x achromat will
length of 160 mm is calculated using the formula: usually have a numerical aperture of 0.50; apochromatic
objectives have higher numerical apertures than achromats
Tube length (Fig. 1.9 ).
Magnification =
Focal length of objective Resolution
For microscopes with tube length other than 160 mm: It is the smallest distance between two dots or lines that can
be seen as separate entities. It depends on the wavelength
Tubelength × eyepiece magnification of light and the NA of the lens. As the NA of the objective
Magnification = increases, the resolving power increases. It is calculated
Focal length of the objective
as:
Magnification for low power objective with focal ∙ R = λ/2NA
length 16 mm and standard tube length of 160 mm is: ∙ R = 0.61λ/NA
Magnification = 160/16 = 10 ∙ R = 1.22λ/[NA (obj) + NA (cond)]
λ = Wave length
NA = Numerical aperture
Color Codes
Microscope manufacturers label their objectives with color Types of Objectives (Figs 1.10A to C)
codes to help in rapid identification of the magnification. In
In most modern microscopes objectives are usually made
addition to color coding other information is also embossed
up more than one lens. This series of lenses is used to
on the objective (Table 1.2).
overcome certain limitations in the lenses, i.e.
Numerical Aperture Optical aberrations: Aberration is the failure of a lens to
The ability of the lens to distinguish fine structural adjacent produce exact point to point correspondence between an
details in a specimen is known as the resolving power. This object and its image. Every lens system has an aberration
ability is expressed in terms of numerical aperture, as NA, to a greater or lesser extent. To improve the image quality,
as it is usually called. the lenses are designed by combining different lens shapes
Microscope
A
A B C
Figures 1.10A to C Design and arrangement of lens system
in objectives
and glass materials. It is possible to construct compound

lenses of different glass elements to correct this fault. An
achromat lens is corrected for two colors, blue and red, B
producing a secondary spectrum of yellow/green. This
Figures 1.11A and B Chromatic aberrations and its
secondary spectrum can be reduced by adding fluorite to correction
objective. Such a lens is called as fluorite lens. Fluorite
lenses need to be corrected for yellow green, which is done
by adding more lens components. Such type of lens is
apochromat which is most expensive.
Chromatic aberration: White light is composed of all
the spectral colors on passing through a simple lens, each
wavelength will be refracted to a different extent, with blue
being brought to a shorter focus than red. This defect of
lens is ‘chromatic aberration’ and results in an unsharp
image with colored fringes (Figs 1.11A and B). Figure 1.12 Spherical aberration
The objective can be both ‘apochromat’ and ‘achromat’
types to correct these optical aberrations.
Plan-achromat: Although histological sections are flat the
Spherical aberration: It is caused when light rays entering image produced by the microscope is not flat. It is saucer
a curved lens at its periphery which are refracted more shaped; it is not possible to focus the whole of the field
than those rays entering the center of the lens and are not sharply at any one time. This aberration is corrected using
brought to a common focus (Fig. 1.12). flat-field objectives also called plan-achromat lenses.
Different types of objectives are as followes:
Nosepiece
Achromatic: Corrected for two colors red and blue. It is
the most widely used for routine purposes. In most modern microscopes up to six objectives are
mounted on resolving nosepiece. For rapid change of all
Fluorite: Green light is brought to a shorter focus and objectives they should be at focus and they should focus
violet light to a longer focus. the same central area of the section when brought into the
Apochromat: All colors are brought into same focus. It is position. Such nosepieces are known as ‘par-focal’ and
fully corrected for three colors. By the design of the lens ‘par-central’.
and use of fluorite, the formation of a secondary spectrum
is almost completely eliminated and all colors are brought Mechanical Tube
to the same focus. These lenses are used especially for Light from the objective is received into the bottom of the
photomicrography and for screening cytological smears. microscope body-tube. From there it travels to the eyepiece
in a tube called mechanical tube. The distance from

objective to eyepiece is called “mechanical tube length”
and it is defined as “the distance from the nosepiece
8 opening, where the objective is mounted, to the top edge
of the observation tubes where the eyepieces (oculars) are
inserted”. In standard microscopes this is 160 mm, while in
few special purpose microscopes it is 170 mm.
In most microscopes tube length cannot be altered. Such
microscopes are called as finite length microscope. In these
microscopes if additional filters such as polarizer, analyzer
fluorescent filters are used, tube length becomes more than
160 mm and aberrations will be introduced in the image
formed. To overcome this limitation, most of the modern A B
microscopes use infinity corrected optics where image is
projected to the infinity. In this system, tube length can be Figures 1.13A and B Position of field lens and eye lens in
altered without affecting the quality of the image. Infinity- Ramsden and Huygenian eyepieces
corrected systems have the advantage of being easier to
design and also make possible the insertion of less costly
accessories in the “parallel” light path. This advanced new
optical system allows microscopes to support complex
optical component clusters in the optical pathway between
the objective and the lens tube. This is especially useful
for techniques such as confocal, polarized, DIC, and
fluorescence microscopy where specialized lens systems
must be employed for optimum results.
Eyepieces
The purpose of an eyepiece in a compound microscope is
to enlarge the primary image formed by the objective, and
to render it visible as a virtual image in the microscope and
also to correct some of the defects of the objective.
Huygenian eyepieces are the simplest form of
eyepiece in common use; they are cheap, but they are Figure 1.14 Compensating eyepieces are required in
binocular microscopes
not corrected for chromatic difference of magnification.
Although, Huygenian eyepieces can be used with low-
power achromats, they give under-corrected curvature of are essential for use with apochromatic objectives,
field and lateral colors with intermediate and higher power but they also improve the performance of most high-
objectives. The other main kind of eyepiece is the positive power achromatic objectives. Eyepieces for binocular
eyepiece with a diaphragm below its lenses, commonly microscopes must be accurately paired, with equal
known as the Ramsden eyepiece. These eyepieces are centration, magnification, and field in order to reduce
corrected for chromatic aberration of magnification (Figs eye strain. Interocular distance should be accurately
1.13A and B). adjusted, and the microscopist should sit at the correct
height for the eyepieces to come to the exact height of
Different Types of Eyepieces the observer’s eyes (Fig. 1.14). Eyepieces, generally, are
Compensating eyepieces are compound lenses with produced with different magnifying powers, ranging from
a chromatic difference of magnification which is equal about 4x to 25x. The most common in use are those with
and opposite to that of high-power objectives. They a magnifying power of 10x or 15x.
Microscope
Pointer eyepieces: A fine pointer could be incorporated

in the eyepiece in order to enable us to point out a certain
portion of the specimen. Such types of eyepieces are called
as pointer eyepieces (Figs 1.15A and B). 9
Multihead demonstration eyepieces: Some types of

eyepieces are such that 4 to 5 people can view the same
portion of an object at a time. This type is called ‘double
demonstration eyepieces’ (Fig. 1.16).
Micrometry
The most common method of making such measurements
is the use of ocular micrometer and stage micrometer. We
can make measurements in compound microscopes only in
the range of 0.2 to 25 mm. We cannot measure dimensions
smaller than 0.2 mm because it is less than the resolving Figure 1.16 Multihead viewing microscope
power of a compound microscope. Likewise, measurement
A Figure 1.17 Ocular micrometer/Graduated reticle
above 25 mm is also not practical because it will be above

the average field diameter of a wide field eyepiece. Larger
objects can, however, be measured with a stereomicroscope.
The ocular micrometer (OM) (Fig. 1.17) is a glass disk
with a diameter of 1 cm. It is engraved with an arbitrary
scale of 100 divisions or less. It is also referred to as a reticle,
reticule or graticule. Since it is fitted into the eyepiece of
the compound microscope it is more appropriate to call
it an ocular micrometer. This is the scale that is used for
all measurements. Since the scale is arbitrary, is to be
calibrated (standardize) using a known standard scale, the
stage micrometer (SM).
B A stage micrometer is a standard microscope slide
Figures 1.15A and B Pointer eyepieces are helpful in having a scale of defined length. Usually, the scale is
locating area of interest 1 mm (1000 mm) divided into 100 divisions, so that one
stage micrometer division = 10 mm. Such a microlevel

scale is made by methods such as photographic process,
physical engraving or electro-deposition of a metallic film
10 directly onto the glass surface. A protective cover glass slip
is usually mounted on the scale. The scale may be encircled
by a black line during use for easy location and focusing
under the microscope.
The calibration of the ocular micrometer refers to
determination of the distance of one division in terms of the
absolute distance of a stage micrometer. A simple Vernier
principle is used for this purpose. How many of OM
divisions are equal to how many of the SM divisions under
a particular microscope–eyepiece–objective combination
is found out. Suppose, it is found that 2 OM divisions are
equal to 1 SM division which means that 2 OM divisions
have a value equivalent to the absolute distance of 1 SM
division, i.e. 10 mm. This is given by 1 OM division = 10
mm/2 = 5 mm.
This value is often known as micrometer value or
calibration factor. Once this value has been determined, the
dimension of any specimen can be calculated by multiplying Figure 1.18 OM (Ocular micrometer) (eyepiece scale) is
the number of OM divisions spanned by the specimen coincided with the SM (Stage micrometer)
with the calibration factor. It must be remembered that a
calibration factor only applies to a specific microscope–
eyepiece–objective combination (Fig. 1.18). surface. The reflection of visible light is a property of the
behavior of light that is fundamental in the function of
IMAGE FORMATION IN MICROSCOPE majority of today microscopes. Light is often reflected by
one or more plane or flat mirrors within the microscope
Real image: The real image in a microscope is formed
to direct the light path through lenses that form the virtual
by the objective lens. The image is formed at a greater
images which is visible in the eyepieces. Other optical
magnification, and is inverted. This is when the object is
components in the microscope, such as prisms, filters, and
moved nearer the lens.
lens coatings, also carry out their functions in forming the
Virtual image: If the object is placed still nearer the lens image with a crucial dependence on the phenomenon of
within the principal focus, the image is formed on the same light reflection.
side as the object, is enlarged, the right way up, and cannot A transmitted light microscope will typically be of little
be projected onto the screen. This is the virtual image. The use to anyone wanting to examine the structure of biological
eyepiece in a microscope forms the virtual image of the specimen. As a result, the reflected light microscope has been
real image projected by the objective. developed for these purposes. Reflected light microscopy
The microscopy utilizes transmitted and reflected is often referred to as incident light, epi-illumination, or
light for image formation. Transmitted light is light which metallurgical microscopy (Mostly used in Metallurgy
passes through the object or specimen from source below studies), and is the method of choice for fluorescence and
and image is create in eyepiece after passing through the for imaging specimens that remain opaque (Fig. 1.19).
objective. Whereas when light reflects from a smooth In reflected light microscopy, the pathway for reflected
surface, the incoming light is referred to as an incident light begins with illuminating rays originating in the lamp
light and the light that is bounced away from the surface housing for reflected light. This light next passes through
is termed the reflected light. Reflection of light occurs the collector lens and into the vertical illuminator where
when the light come upon an object surface that does it is controlled by the aperture and field diaphragms.
not absorb the light and bounces the light away from the After passing through the vertical illuminator, the light is
Microscope
Specialized Microscopy Techniques:

• Stereomicroscope
• Dark field microscope 11
• Phase contrast microscope
• Polarized microscopy
• Fluorescence microscopy
• Confocal microscope
• Electron microscope.
SPECIALIZED MICROSCOPY
TECHNIQUES
Stereomicroscopy (Figs 1.21A and B)
Figure 1.19 Image formation in reflected light microscope It is optical microscope designed for low magnification
observation or a sample using incident light illumination
rather than transillumination. It uses two separate optical
paths with two objectives and two eyepieces to provide
slightly different viewing angles to the left and right eyes.
Stereomicroscopy overlaps macro photography for
recording and examining solid samples with complex
surface topography where a three-dimensional view is
essential for analyzing the detail. The stereo microscope
is often used to study the surfaces of solid specimens or to
carry out close work such as grossing of specimen, etc.
Dark Field Microscopy

Dark field microscopy is an optical microscopy illumination
technique used to enhance the contrast in unstained
samples. It works on the principle of illuminating the
sample with light that will not be collected by the objective
lens, so not form part of the image. This produces the
classic appearance of a dark, almost black, background
with bright objects on it.
Figure 1.20 Image formation in light microscopes For the dark field method, the cone of light normally
illuminating the specimen should not enter the microscope
objective, only light that is scattered or reflected by the
then reflected by a beamsplitter through the objective to specimen is seen by the objective. This is achieved in the
illuminate the specimen. Light reflected from the surface conventional microscope by use of dark field diaphragm
of the specimen re-enters the objective and passes into the stops or a special dark field substage condenser like Abbe,
binocular head where it is directed either to the eyepieces paraboloid or cardioid condensers. A dark field stop is
or to a port for photomicrography (Fig. 1.20). inserting the stop below the condenser. The light rays from the
The principal focus or focal point is the single point condenser pass outside the objective and thus form a hollow
where the parallel rays of light entering the lens are cone. Now any object with a refractive index different from
brought together by refraction. The focal point is the point the surrounding medium, placed within this hollow cone, will
where the clear image of an object is formed. Focal length only reflect light into the objective and thus the object will
is the distance between the optical center of the lens and the appear ‘bright’ against the dark background. The condensers
principal focus. used for this type of microscopy are (Figs 1.22A to C).
12
A B
Figures 1.21A and B Stereomicroscope with two objectives and binocular eyepieces. Two light sources reflect light from above
the specimen and beneath the object stage for surface topography and viewing low magnification of sections respectively
A B C
Figures 1.22A to C Condenser used for dark field microscopy. (A) Cardioid condenser; (B) Abbe condenser;
(C) Paraboloid condenser
which forms the true image of the light source at the

Types of Condenser Used in Dark Field Microscopy specimen plane. The illuminated specimen is magnified
• Abbec ondenser: Used within low power objective by the objective to produce a real, inverted image. A
• Paraboloid condenser: High power oil immersion magnified upright virtual image of this real, inverted image
objective is produced by the eyepiece which will be seen by the
• Cardioid condenser: More refined and corrected for observer’s eyes.
aberrations than the paraboloid condenser. It is used Dark field microscope is used to demonstrate spirochetes
with oil immersion objective. trypanosomes, parasites, and other microorganisms in body
fluids and cell suspensions, also in flow cell techniques
Two lenses, the objective and eyepiece, are responsible and autoradiographic grain counting. When observed
for the formation of the image. The object or specimen under dark field microscope, these organism appear bright
is illuminated by the light passing through the condenser in dark background.
Microscope
Phase Contrast Microscopy processes can be observed and recorded in high contrast
(Figs 1.23 and 1.24) with sharp clarity of minute specimen detail.
Phase contrast microscopy is widely employed in
Phase contrast microscopy, first described in 1934 by 13
diagnosis of tumor cells and the growth, dynamics, and
Dutch physicist Frits Zernike, is a contrast-enhancing
behavior of a wide variety of living cells in culture.
optical technique which is utilized to produce high-
Brightfield microscope can be converted into phase
contrast images of transparent specimens, such as living
contrast by two specialized accessories. A specially
cells (usually in culture), microorganisms, thin tissue
designed annular diaphragm, which is matched in diameter
slices, lithographic patterns, fibers, latex dispersions, glass
and optically conjugates to an internal phase plate residing
fragments, and subcellular particles (including nuclei and
in the objective rear focal plane, is placed in the condenser
other organelles).
front focal plane.
One of the major advantages of phase contrast
The phase contrast microscope is probably the most
microscopy is that living cells can be examined in their
outstanding contribution to microscopy in recent years. It
natural state without previously being killed, fixed, and
can be used to produce excellent contrast effects, with a
stained. As a result, the dynamics of ongoing biological
wide variety of otherwise transparent specimens. Since it
permits visualization of interior details in cell structures,
it has a definite advantage over the dark field microscope.
Probably its widest application is in the field of tissue
culture, where it permits one to examine and photograph
living, growing cell.
Phase contrast microscope is standard biological
microscope and is equiped with modified objective and
condensers.
∙ Condensers: Condenser has a series of annular
diaphragm made of opaque glass with a clear narrow
ring, to produce a controlled, hollow cone of light.
∙ Objective: It requires a different size of annulus an
image of which is formed by the condenser in the basic
focal plane of the objective as a bright ring of light. The
Figure 1.23 Phase contrast objective Objective has a phase shifting plate or positive phase
plate which is a clear glass disk with a circular trough
etched in it to half the depth of disk. The trough also
contains a neutral density light absorbing material to
reduce the brightness of the direct rays which could
otherwise obscure the contrast obtained.
The light passing through the trough has a phase
difference of 1/4 of wavelength (Figs 1.25A and B).
Principles of Phase Contrast Microscope

The basis of phase contrast microscope is the exaggeration
of minute differences in the refractive indices by
advancing or retarding light waves, thus converting them
into difference of amplitude, which are seen as variation
in brightness. Thus if two unstained structures of almost
the same refractive index are examined by ordinary
Figure 1.24 Phase plate of phase contrast microscope illumination it will be found that they are indistinguishable
showing different objectives from each other.
14
Figure 1.26 Different types of polarizing filters for polarizing

A microscope
structure of living cell due to difference in the refractive

index of the components of the cell and is of great value in
cytology, hematology and microbiology (Figs 1.25A and B).
Polarized Light Microscopy (Fig. 1.26)

B
Birefringence is a property, which is shown by crystalline
Figures 1.25A and B Image formation and photomicrograph structures, amyloid deposits, proteins, pigments and lipids.
in phase contrast microscope When such substances are viewed in a microscope with
polarized filters, they may appear bright or even colored
For each transparent or translucent particle in the against a dark background.
object, two rays result from an incident light, The direct, In this type of microscope is used two ‘polarizers’
or undiffracted ray comes through the angular diaphragm, made up of Nicole Prisms are used. One is placed
passes through the object and is focused on the phase beneath the substage condenser and is held in a rotatable
shifting ring which either retards or advances the ray 1/4th graduated mount, and can be removed from the light path
wavelength with respect to the secondary. when not required. The other called ‘analyzer’ is placed
The second ray of the incident beam is modified by between objective and eyepiece and is also graduated for
being scattered and diffracted in passing around the margin measurements to be taken.
of the object. This ray does not pass through the phase When a birefringent substance is rotated between
shifting ring but traverses the other areas of the transparent two polarizers, which are crossed, the image appears and
disk, and the wavelength is neither advanced nor retarded. disappears alternately at each 45° of rotation. In a complete
Thus there is an optical difference of 1/4th wavelength, revolution of 360° the image appears four times.
which causes a phase difference with the asynchronous Only, the polarizer is used, and if no rotating stage
waves producing reinforced darkness or brightness at is available, the polarizer itself can be rotated. Changes
certain points. Thus ‘phase contrast’ is made visible to in intensity and color are seen during rotation. The color
the observer’s eye with the help of ‘phase shifting plate’, changes in a rotation of 90°, and back to its original color
which enhances the optical effect of the difference. in the next 90°. This is due to differential absorption of
light, depending upon the vibration direction of two rays in
Applications a birefringent substance.
Phase contrast microscope is valuable in examination of wet Principle of polarized microscopy: A ray of light consists
mounts and hanging drop preparations. It can reveal cellular of electromagnetic waves vibrating in all directions at right
Microscope
angles to the path of the ray of light itself. In polarized light

the waves are made to vibrate in one plane only. This is
achieved by the rotating Nicole prisms, i.e. polarizer and
analyzers, which is interrupted in the beam of light. 15
The specimen are labeled into two categories isotropic
or anisotropic.
Isotropic (Singly refractive): The substance which are not
illuminated by the change in direction of the beam or 90°
rotation of the analyzer, the rays transmitted by the lower
prism will not pass through the upper, the field is now dark
and the position is called crossed Nicole
Anisotropic (Birefringent or doubly refractive): These
substances are seen as positive after the changes in
direction of the beam of light, i.e. 90° rotation of analyzer,
Figure 1.27 Polarized microscopy can be applied to see
the objects are seen as bright against a dark background.
enamel hypoplasia and dental caries
Collagen fibers, bone matrix, striated muscle, cholesterol,
Zenker fixed RBC, pigments such as formalin pigment,
crystal such as talc and vegetable, fibers like cotton and
linen are anisotropic”.
Applications: The polarizing microscope can be a
useful means of identification of tissue components and
of exogenous an endogenous crystal specially when
combined with special staining techniques and with
histochemistry” With this—“ The polarizing microscope
can be a useful in identification of exogenous an
endogenous tissue components and crystal. It is more
effective when combined with special staining techniques
and histochemistry” (Fig. 1.27).
Fluorescence Microscopy
(Figs 1.28 to 1.30)
Objects invisible by ultraviolet light may become
brilliantly luminous if coated with a fluorescent substance, Figure 1.28 Fluorescent microscope principle showing filters,
like fluorchromes. Fluorchromes are the dyes which absorb arc lamp and the image formed
radiation (e.g. Ultraviolet light) and become excited; these
excited molecules are then capable of emitting radiation background. A completely dark room is desirable. Brilliant
of longer wavelength and which disappear almost fluorescence depends upon maximum contrast and is
immediately after withdrawal of the exciting radiation. reduced if there is background light in the room.
This is called ‘fluorescence’. Thus fluorescence is the
The equipment consists of:
property of some substances, which illuminated by light of
Light source: Halogen lamps which give off sufficient
certain wavelength causes them to emit the rays of different
blue light (9400–500 nm). Ultraviolet lamps need to be
and longer one. In fluorescence microscopy a fluorescent
warmed up before use and have short life.
specimen is illuminated with invisible ‘ultraviolet light’
(UV light has wavelength below 400), the light rays of Filters: Two filters are place, each in between condenser
longer wavelength within the spectrum of visible light are and source (Exciter filter); other is placed between the
given off and those are seen as various colors of on dark objective and eyepiece (Barrier filter).
16
Figure 1.29 Fluorescent microscope path of light
– Exciter filter: The exciter filter is made up of heat filter

(heat absorbing filter), red stop filter (this filter removes
red light) and wavelength selection (this is main exciter
filter that allows only the desired wavelength to pass).
– Barrier filter: Light on passing through these filters
illuminates the specimen, the objective collects both
exciting and fluorescent wavelengths. The former is
removed by barrier filter to prevent short wavelength
light from damaging the retina of the eye.
Condensers: Dark ground condensers which do not
allow direct light into the objective, and in addition to a
dark contrasting background to the fluorescence are used.
Routine bright field condensers are able to illuminate the
object using all the available energy but they also direct the
rays beyond the object into the objective. This is hazardous Figure 1.30 Epithelial cells in fluorescent microscopy
to the eyes of the observer. exhibiting tonofilaments
Objectives: For fluorescence microscopy objectives with

high numerical apertures are preferred. The intensity
Applications: Fluorescent techniques have become
increases with increase in the numerical apertures. Hence
recently widely used in research and fluorchrome dye
apochromat are generally preferred.
methods are routinely employed for the demonstration of
Eyepiece: The eyepiece with lower magnification is tissue components, bacteria, fungi, heavy metal in sections
desirable as fluorescence is inversely proportional to the and for the identification of malignant cell in exfoliative
square of the eyepiece magnification. cytology (Fig. 1.29).
Microscope
Fluorescent microscopy is the bases for immuno- serial optical sections from thick specimens and create 3-D
fluorescence techniques for the demonstration of antigens image of the the specimen using microcomputers.
and antibodies in tissues and sera. In conventional fluorescence entire specimen is
Fluorochrome dyes which are used routinely are illuminated by the light from xenon or mercury bulbs, and 17
Thioflavin T (amyloid) acridine orange (Malignant cells, light from all areas of the specimen enter the objective
mucin and fungi) Auramine-Rhodamine (Acid fast bacilli). and image is obtained. In confocal microscope only a
pinpoint area is illuminated and light from this area enters
Confocal Microscope (Figs 1.31A and B) objective and passes through a pinhole filter to eliminate
Confocal (“having the same focus”) microscopy is one of out of focus light. As only a small area is focused very
the most significant advances in microscopy. The principle bright light is needed. This is provided by the laser
of confocal imaging was patented by Marvin Minsky. system. Coherent light emitted by the laser system passes
With this technique it is possible to control depth of field, through a pinhole aperture that is situated in a conjugate
elimination, reduction of background illumination, to collect plane (confocal) with a scanning point on the specimen
and a second pinhole aperture positioned in front of the
detector (a photomultiplier tube). As the laser is reflected
by a dichromatic mirror and scanned across the specimen
in a defined focal plane, secondary fluorescence emitted
from points on the specimen (in the same focal plane)
pass back through the dichromatic mirror and are focused
as a confocal point at the detector pinhole aperture. The
significant amount of fluorescence emission that occurs
at points above and below the objective focal plane is not
confocal with the pinhole termed.
Out-of-focus light rays and is eliminated. Refocusing
the objective in a confocal microscope shifts the excitation
and emission points on a specimen to a new plane that
becomes confocal with the pinhole apertures of the light
source and detector. In this way entire specimen is covered
A point by point using a scanner, images of individual points
are acquired, processed, analyzed and image is displayed.
Applications: The broad range of applications available
to laser scanning confocal microscopy includes a wide
variety of studies in neuroanatomy and neurophysiology,
stem cell research as well as morphological studies of a
wide spectrum of cells and tissues. In addition, the growing
use of new fluorescent proteins is rapidly expanding the
number of original research reports coupling these useful
tools to modern microscopic investigations.
Electron Microscope
The electron microscope has gained its fundamental
superiority over the light microscope is because of its high
resolving power to produce extreme fine details. In light
microscopy highest resolution that is possible theoretically
B is half of the wavelength of light. Thus limit of resolution
Figures 1.31A and B Confocal microscope and its image of light microscopy is 0.2 microns (Fig. 1.32). With use of
formation ultraviolet rays this can be improved to 0.1 microns. But
18
A
Figure 1.32 Image formation in electron microscopes and
light microscopes
intracellular components, certain bacteria and most of the

viruses are smaller than this and cannot be visualized. So
attempts were made to use other types of radiation.
In 1933 Ernst Ruska and Max Knoll succeeded in this
by building electron microscope. In electron microscope
it is possible to enlarge the image 250,000 times or more.
This image can be photographed for permanent record and
it enlarged 4 to 6 time without undue loss of details, thus
giving pictures in the range of two million times as large
as the object. Greater resolving power (0.2 nm) makes it
possible to obtain images of protein molecular viruses, B
unstained flagella, internal structure of cell, etc. Two types
Figures 1.33A and B Transmission electron microscope
of electron microscope are used:
1. The transmission electron microscope (TEM)
2. The scanning electron microscope (SEM).
Electron gun: It generates beam of electrons. It is made of
Transmission Electron Microscope anode and tungsten filament, housed in a wehnelt shield.
Filament generates electrons by thermionic emission.
The transmission electron microscope is similar to the light
Between tungsten filament and anode high voltage
microscope in that it uses lenses to form magnified image.
difference is maintained so that electrons that are emitted
Both have condenser lenses to concentrate the incident
from filament are accelerated towards object.
beam upon the specimen. This beam passes through the
specimen to the objective (magnetic) then to the projector Electron lenses: These electrons pass through a set of
lens and forms an enlarged image onto a fluorescent screen. electron lenses. These are made up of electromagnetic coils
Difference lies in the radiation used and type of lenses. In or solenoids. When energized these generate a magnetic
electron microscope beam of electron is used instead of field that forces electrons to a focus. Current and voltage
visible light, and electromagnetic lenses are used in place in these coils can be varied to change the focus of electron
of glass lenses (Figs 1.32 to 1.34). beam.
Microscope
19
Figure 1.34 TEM of cartilage cells Figure 1.36 SEM image of blood filled artery
MAINTENANCE OF LABORATORY
MICROSCOPE
Like every precision mechanical instrument, microscopes
will last longer and provide better performance if cleaned
and lubricated at regular intervals. The actual work
involved is simple and not time-consuming. After long use
of the instrument, overhauling, cleaning and lubricating
are required. Major defects come from forced movement
especially when dried grease or fixed dirt on the movable
parts causes wearing out the teeth of the gears. This occurs
commonly between fine adjustment and coarse adjustment
gears.
Optical Maintenance
Figure 1.35 Tooth enamel crack (SEM)
After long use of microscope, lenses become covered by
fixed dust, dirt and film. Under the worst conditions, such
Image formation: Focused electrons pass through object. as high humidity, fungi may grow on the inner lens surfaces.
These electrons are directed to the viewing screen or image This microbial growth may erode the lens surfaces. Such
recording unit. lenses cannot be cleaned routinely and should be returned
to the manufacturer.
Scanning Electron Microscope Optical glasses are generally softer than window glasses
The scanning electron microscope provides a topographic so gentle touch is required while cleaning such glasses.
view of the surface contours of the specimen. For this it Lenses are cemented with adhesive materials. The lenses
uses an incident electron beam and the reflected electrons may become loose, if there is prolonged use of solvent
produce the image which is three dimensional. As it materials are used for cleaning as these dissolve the adhesive
mimics our own natural perception, the image is instantly cement. So xylene should not be used. Petroleum spirit is
appreciated (Figs 1.35 and 1.36). recommended by some manufacturers. The recommended
agent to clean the lenses is xylol. Commercially available though narrow hole, only skilled repairman or the
detergent based glass cleaning agents may be used. Alcohol manufacturer can clean it. Usually, lens surfaces of the
and acetone should be avoided as they may seep into the objectives are smaller than eyepieces, for checking dirt or
20 mount and dissolve the cements. crack on the surface is recommended to use a magnifying
glass.
Cleaning of Eyepiece
If eyepieces are observed under good light, dirt and film Condenser
that may be present on the outer surfaces of lens can be For the Abbe condenser, take apart iris diaphragm unit
seen readily. Dirt on the inner lens surface may be seen by from condenser, clean the top lens from surface and back
looking through the field lens. Following steps should be surface of the field lens.
followed for cleaning:
∙ Loosen dirt with camel-hair brush, and blow it off with BIBLIOGRAPHY
blower.
1. Clyde Walter Mason, Émile Monnin Chamotl. Handbook of
∙ If oil or other grease film remains, distilled water should
chemical microscopy, Wiley. 1983;4(1).
be sprayed and wiped off with lens paper or clean lint- 2. From cells to proteins. Imaging Nature across dimensions;
free cloth. Valtere Evangelista (Ed). Springer; 2004.
∙ If the film persists lens cleaning solution should be 3. http://www.leica-microsystems.com/products/light-
applied and wiped off promptly. microscopes/
∙ Circular motion should be applied for cleaning and 4. http://www.microscopesmanufacturer.com/
polishing. 5. http://www.olympusmicro.com/primer/techniques/
∙ The necessity of cleaning inner surfaces may be fluorescence/fluorhome.html
determined by focusing the microscope on a specimen 6. http://www.olympusmicro.com/primer/techniques/index.
and rotating the eyepiece, if dirt spots rotate, cleaning is html
required. Unscrew lower and upper lens elements and 7. http://www.sciencephoto.com/media/467645/view
8. http://www.sciencephoto.com/media/85586/view
clean as described for outer surfaces.
9. John Bankroft, Marilyn Gamble; Theory and Practice of
Objectives histological techniques; Churchil Livingstone; 2008.
10. Molecular biology of the cell: Reference edition, Bruce
Objectives should be taken apart from nosepiece for Alberts, (Eds) Garland Science Publishers. 2008:5(1).
cleaning. Exposed front surfaces of all objectives cleaned. 11. Simon Henry Gage. The Microscope and Histology.
Because the back lens usually located in the deep position BiblioBazaar; 2010.
MULTIPLE CHOICE QUESTIONS
1. Who discovered microscope: 3. Range of wavelength used in fluorescent microscope is:

a. Galileo a. 600–800 nm
b. Ruska b. Below 400 nm
c. Leeuwenhoek c. Below 600 nm
d. Janssen d. 400–800 nm
2. Range of wavelength used in light microscope is: 4. The numerical aperture (NA) of Abbe condenser is:
a. 400–800 nm a. 2.5
b. 200–400 nm b. 0.25
c. 800–1000 nm c. 25
d. 40–80 nm d. 0.10
Microscope
5. Traveling range in most of the microscope is: 8. Length of a mechanical tube in a standard microscope
a. 26 mm (X) 30 mm (Y) is:
b. 30 mm (X) 76 mm (Y) a. 100 mm b. 120 mm
c. 96 mm (X) 30 mm (Y) c. 160 mm d. 200 mm 21
d. 76 mm (X) 30 mm (Y) 9. Most simple and common form of eyepiece is:
6. Most expensive lens is: a. Huygenian
a. Apochromat b. Compensating
b. Achromat c. Pointer
c. Fluorite d. None
d. Plan-achromat 10. Widely used method for diagnosis of tumor cells is:
7. The numerical aperture (NA) of achromatic condenser a. Polarized light microscopy
is: b. Fluorescence microscopy
a. 2.5 b. 0.25 c. Phase contrast microscopy
c. 25 d. 1.40 d. Confocal microscopy
Tissue Processing Methods
Shubhangi Mhaske (Jedhe ), Avadhoot Avadhani
A
Chapter Outline
O Introduction and terminology O Microwave tissue processing/ microwave stimulate tissue

O Gross examination processing
O Tissue preparation O Study of hard tissues
O Tissue processing • Ground section
• Fixation • Hard tissue microtome/Saw microtome
• Dehydration • Decalcification methods for hard tissue
• Clearing O Frozen sections
• Paraffin infiltration O Staining of cut sections
• Embedding O Mounting
• Sectioning with microtome 3 Artifacts in histological sections
• Picking the section
INTRODUCTION AND details with a brief clinical history and provisional diagnosis
(Fig . 2.1) . It also describes the surgical details with date and
TERMINOLOGY time of procedure performed . The specimens are placed in
Histology: This is the microscopic examination or study fixative containers or jars immediately after biopsy.
of tissues . The specimens are accessioned by giving them a unique
number that will identify each specimen for each patient.
Histopathologv: This refers to the microscopic Each laboratory has its own way of biopsy specimen
examination of tissue to study the manifestations of the labeling giving the tissue the unique accession number.
disease . Specifically, it refers to the examination of a Bar codes and Quick response codes (QRC) are also
biopsy of surgical specimen or autopsy by a pathologist, being practiced in some modem pathology laboratories
after the specimen has been processed and histological ( Fig . 2.2 ) . The Bar code on the request form is read by the
sections are made onto glass slides . department computer generating the complete information
Histotechnique: This refers to the procedure of preparing of the patient.
the tissue for histological study.
GROSS EXAMINATION
Specimen accessioning: This tissue specimens received
in the surgical pathology laboratory^ are accompanied by Tissues removed from the body for diagnosis arrive in the
a request form or a requisition form that lists the patient’s Pathology department and are examined and grossed by
https: //t.me / LibraryE

23
Figure 2.2 Bar codes and quick response code add ease of
access of data in computer records
Figure 2.1 Biopsy request form
a pathologist, resident pathologist, pathology assistant or

technician. Large variety of specimens are received in the
Oral and Maxillofacial Pathology laboratory ranging from
Jaw resections, large and small tumor masses, cysts, or
small incisional biopsies of large lesional tissues. Smaller
biopsies are difficult to describe from their anatomical
source or site unless described thoroughly in request card.
Gross examination consists of describing the specimen for
size, color, shape, number, etc. and the time at which tissue
Figure 2.3 The biopsy specimen received in the laboratory
was received (Fig. 2.3).
with details of Registration number, date and time of biopsy
After grossing, the tissue is placed in tissue cassettes removal, nature of biopsy and other specimen details
for further treatment by serial processing steps (Figs 2.4
and 2.5).
render it firm and soft enough to be able to cut into thin
PREPARATION OF TISSUE SPECIMEN sections by special knives.
The tissue has to be fixed, following which it must be
FOR HISTOLOGICAL STAINING
processed into a form in which it can be made into thin
Tissues from the body whether biopsies or tissues from microscopic sections. The usual way this is done is with
autopsy are processed in a series of chemical reagents to paraffin. Tissues embedded in paraffin, which is similar
24
B
Figures 2.4A and B Tissue processing/carrying cassettes
A B
Figures 2.5A and B The tissue is placed with a small label of biopsy number in this tissue cassette after careful desired grossing
in density to tissue, can be sectioned at anywhere from Aim of fixation: The aim of fixation is to preserve the
3 to 10 microns, usually 6 to 8 microns routinely. The tissues permanently in are life-like a state as possible.
technique of getting fixed tissue into paraffin is called Fixation should be carried out as soon as possible after
tissue processing. Though the term tissue processing is removal of the tissues (in the case of surgical pathology) 25
used after the tissue fixation, but the entire procedure of or soon after death (with autopsy) to prevent autolysis.
histotechnique can be enlisted as the main steps in this Purpose of fixation: Its main aim is to prevent or arrest
process are (Flow chart 2.1): autolysis, and bacterial decomposition and putrefaction.
Another role of fixation is to coagulate the tissue so as to avoid
Steps in Processing
or prevent loss of diffusible substances, to make the tissue
• Obtaining the specimen
strong enough to withstand the tissue processing treatment
• Fixation
with reagents and wax embedding and to prepare the tissue
• Dehydration
for differential staining methods with reagents and dyes.
• Clearing
• Embedding Effects of fixation: There is coagulation of proteins and
• Cutting. other coagulable constituents. The loss of the cellular
components is prevented. The refractive index of tissues is
altered in varying degree. Fixation has a marked effect on
ROUTINE METHOD FOR staining and it facilitates the action of dyes.
HISTOLOGICAL STUDY Removal of fixative: The fixative should be removed by
Fixation overnight washing. The tissue cassette is placed in a trough
and is kept under running water overnight with a small
Fixation in simple terms is strengthening and prevention
stream of water directed onto the specimen cassette.
of tissue decomposition. Fresh tissue is placed in a
preservative. The agent used for fixation of tissue is called Requisites of Fixation
as fixative.
• T he tissue ideally be grossed or cut into 0.5 cm
thickness for better penetration of the fixative.
Flow chart 2.1 Steps in processing • The volume of the fixative used must be 20 times
that of the specimen.
• The time or length of fixation depends on the size of
the specimen.
Factors Affecting Fixation

Size of tissue: Smaller tissue samples are fixed in short
duration but larger specimens require minimum 24 hours.
Type of tissue: Soft tissues are penetrated faster by the
fixative solutions than hard tissue.
Duration: Most fixatives penetrate the depth of 1mm in
one hour. So it also relates with size of specimen.
Temperature: The diffusion of molecules of fixatives
increases with increase in temperature.
Concentration of fixative: Concentrations above 10
percent of the neutral buffered formalin (NBF) increases
the chances of hardening and shrinkage.
Buffer: Osmolality of buffers also affects the fixation.
Hypertonic and hypotonic solutions may lead to shrinkage
of tissue.
Ionic composition: Various ions (Na+, K+, Ca2+, and Mg+) Therefore, it is essential to use an intermediate solvent
can affect the cellular structure. that is fully miscible with both ethanol and paraffin wax.
This solvent will displace the ethanol in the tissue, and then
26 Common Fixatives this in turn will be displaced by molten paraffin wax. This
• 1 0 percent neutral buffered formalin (NBF) stage in the process is called clearing and the reagent used
• Zenker’s fluid (mercuric chloride, and potassium is called a clearing agent.
dichromate solution) It is called “clearing” because this procedure imparts
• Lugol’s solution (potassium iodide and iodine an optical clarity or transparency to the tissue due to their
solution) relatively high refractive index. Another important role
• Bouin’s fluid (picric acid and formaldehyde solution) of the clearing agent is to remove a substantial amount
• Carnoy’s solution (absolute alcohol and chloroform). of fat from the tissue, which otherwise presents a barrier
to wax infiltration. A popular clearing agent is xylene
Dehydration and multiple changes are required to completely displace
ethanol.
Since paraffin wax is immiscible with water, the water in
the specimen must be removed before the infiltration with Wax Infiltration
wax. This process is usually carried out by submerging
specimens in an ethanol of increasing concentration. In this procedure the tissue is infiltrated with a suitable
Ascending grades of alcohol starting from 70 percent, histological wax preferably the paraffin wax-based
80 percent, 90 percent, 95 percent and then absolute histological waxes due to its physical characteristics. A
alcohol (two changes) are used to dehydrate the specimen. typical wax is liquid at 60°C and can be infiltrated into
The tissue cassette is placed in each of the solutions for tissue at this temperature then allowed to cool to 20°C
minimum 1 hour. Ethanol is miscible with water in all where it solidifies to a consistency that allows sections to
proportions so that the water in the specimen is replaced be consistently cut.
by the alcohol. Increasing concentrations of alcohol are to These waxes are mixtures of purified paraffin wax and
avoid excessive distortion of the tissue (Fig. 2.6). various additives that may include resins such as styrene or
polyethylene.
Clearing These waxes have very particular physical properties
which allow tissues infiltrated with the wax to be sectioned
After the tissue fixation is now essentially water-free, still
at a thickness down to at least 3 to 4 mm, to form ribbons
it is not possible to infiltrate it with wax because wax and
as the sections are cut on the microtome, and to retain
ethanol are largely immiscible.
sufficient elasticity to flatten fully during flotation on a
warm water bath (Fig 2.7).
Embedding
Once the specimen, is thoroughly infiltrated with wax, it
should be embedded into block which can be clamped into
a microtome for section cutting. This can be carried out
either manually or using embedding machines.
In embedding machine the embedding procedure is
carried out using an embedding center where a mould is
filled with molten wax and the specimen placed into it. The
specimen orientation is very important at this step as it will
determine the “plane of section”.
A cassette is placed on top of the mould, topped up with
more wax and the whole thing is placed on a cold plate to
Figure 2.6 Manual processing of tissue involves dehydration of solidify. When this is completed the block with its attached
the tissue with increasing grades of alcohol and clearing with two cassette can be removed from the mould and is ready for
changes of xylene. The tissue is kept for almost one hour in each jar microtomy (Figs 2.8A to D).
Sectioning with Microtome

When the tissues have been embedded, they must be cut
into sections that can be placed on a slide. This is done with 27
a microtome.
The microtome is a equipment that holds a knife with
a mechanism for advancing a paraffin block. The most
important necessities for proper sectioning are a very sharp
knife.
Knives are either of the standard thick metal variety or
thin disposable variety (like a disposable razor blade).
Microtome has a mechanism for advancing the block
across the knife. Usually this distance can be set, for most par-
affin embedded tissues at 6 to 8 microns (Figs 2.9 and 2.10).
Figure 2.7 Tissue is kept in melted paraffin for 2 hours for Picking the Sections
complete infiltration of wax. This equipment is called paraffin Once sections are cut, they are floated on a warm water
wax bath bath that helps to remove wrinkles. Then they are picked
up on a glass microscopic slide.
A B
C D
Figures 2.8A to D Preparation of the tissue paraffin wax blocks requires two L molds and tissue embedding blocks. Care
should be taken that tissue infiltrated in paraffin is placed at the base properly oriented for section cutting
The glass slides are then placed in a warm oven for

about 15 minutes to help the section adhere to the egg
albumin coated slide (Fig. 2.11).
28
MICROWAVE TISSUE PROCESSING/
MICROWAVE-STIMULATED
PROCESSING
Rapid manual microwave-stimulated paraffin wax
processing of small batches of tissues gives excellent
results, which are comparable to tissues processed by
longer automated non-microwave methods.
Processing is undertaken in a dedicated microwave
oven, which is fitted with precise temperature control
Figure 2.9 Section cutting or tissue sectioning is done on the and timer, and an interlocked fume extraction system to
microtome preclude accidental solvent vapor ignition (Fig. 2.12).
Agitation is provided by an air-nitrogen system.
Domestic microwave ovens with a temperature probe and
timer accurate in seconds are suitable for tissue processing.
Toxic and flammable solvent vapors generated during
processing cannot always be adequately vented from
these ovens and may present an ignition hazard if the
Figure 2.10 The desired thickness in microns approx 5 μ Figure 2.11 After cutting the sections are spread on a hot
(arrow) is adjusted and the sections are cut by rotating the water bath and picked up on glue coated slides
wheel
containers of about 200 mL capacity are ideal for processing

batches of up to 14 cassettes per container.
Fixation 29
For rapid processing, tissues are fixed by microwave
irradiation, or in 95 percent ethanol (600 mL)-polyethylene
glycol PEG 400 (45 mL) 102 from which specimens can be
transferred directly to dehydrant.
Formaldehyde-fixed tissues must be rinsed in running
tap water for 5 minutes before microwave processing and
an extra dehydration change incorporated into the schedule.
Processing times for formaldehyde-fixed tissues need to be
increased above those provided for coagulant-fixed tissues.
Picric acid fixed tissues should not be microwave
Figure 2.12 Microwave tissue processing
processed as there is an explosion risk even in well washed
tissues.
electrical system is unprotected. Ovens should therefore Principle

be used within a fume cupboard to minimize this problem.
Microwave exposure is used to dehydrate, clear and
Calibration of domestic ovens is essential for optimum
impregnate tissue samples.
results and the accuracy of the temperature probe,
Fixation of tissue samples is achieved prior to processing
duration of cycle time, and net power levels at various settings
in the microwave oven. Processing schedules are developed
must be determined before the oven is used to process tissues.
using tissue thickness as a determining factor.
Dehydration is accomplished using ethyl alcohol.
Hints for Microwave Processing Isopropanol is substituted for xylene as a clearing agent.
• issue blocks should be as thin as possible
T The isopropanol is boiled out of the tissue during the
• Process blocks of similar thickness together impregnation process by heating paraffin above the boiling
• Length and width are not important point of isopropanol.
• Reagent volumes should be at least 50 times that of
specimen volume Equipment and Reagent Used
• The temperature probe should be placed centrally in Equipment
processing baths • Rotator
• Use a dummy load to check heat generation should • Commercial microwave operating at approximately
reagents boil on minimum settings—an equal volume 600 Watt
of reagent irradiated together with the primary load • Paraffin pot. Temperature set at 84°C. This instrument
effectively halves the energy received by the primary is used to recycle paraffin
load. • Paraffin pot. Temperature set at 60°C.
• Pre-heat paraffin wax baths in a conventional oven. Reagent
• An increase in the number of cassettes or fluid • Ethyl alcohol
volumes will require a concomitant increase in • Isopropanol
power and or time to achieve the correct processing • Paraffin.
temperature.
Procedure
Equipment Microwave Biopsy (1 mm Thick) Procedure
Tissues are processed in conventional plastic cassettes, (Process Time 45 Minutes Including Fixation)
including those with (provided the metal lids lie below Tissue samples are placed in plastic prelabeled cassettes and
the fluid). Transparent glass or solvent-resistant plastic allowed a fixation time of 30 minutes for fresh tissue (Most
samples are fixed adequately upon receipt). Fresh samples into a paraffin pot that is at a temperature of 84°C. Refill
should be placed on a rotomixer agitator to enhance fixation. the container with fresh paraffin that is 60°C.
Rinse cassettes with water to eliminate the possibility Place the container in the microwave. Add the
30 of a salt precipitation when the cassettes are placed in the temperature probe centrally into the bath and microwave at
ethyl alcohol. a temperature of 65°C for 2 minutes.
Place cassettes into the teflon processing rack. Around Open the microwave door and agitate the rack several
20 cassettes will fit in 1 rack leaving the top level empty to times to ensure temperature consistency. Adjust the tempe-
allow for evaporation. Around 80 cassettes can be processed rature setting to 80°C for 5 minutes.
at a time using 4 racks and 4 plastic processing containers. Remove the container from the microwave and dump
If you are processing less than 8 cassettes you should place paraffin into the paraffin pot that is set at a temperature of
at least 8 empty cassettes in the bottom of the rack. This will 84°C.
regulate the temperature and ensure proper processing. Remove the processing rack from the plastic container
Place processing rack in plastic container and fill with and place cassettes at embedding center.
100 percent ethyl alcohol to rinse off water. Discard alcohol Place processing rack into xylene to remove excess
and fill with 400 mL of fresh 100 percent ethyl alcohol. paraffin. Both plastic containers can be reused for the next
Place the plastic container in the microwave oven and run.
place the temperature probe centrally into the container.
Make sure the probe does not touch the cassettes.
When starting the microwave follow these steps:
STUDY OF HARD TISSUES
∙ Depress the power switch and observe that after a brief Hard mineralized tissues are extremely firm and very
period, during which the vent system comes up to difficult to prepare histological sections. The teeth and
speed, the stop switch indicator extinguishes. bone are studied histologically by following methods:
∙ Depress the lamp switch and observe that the chamber
light is illuminated. Types of Hard
∙ The turntable is not used therefore the switch should • G round section
not be depressed. • Hard tissue microtomy
∙ The airflow bubblier is not used therefore the switch • Decalcification with chemical agents and thereafter
should not be depressed. regular tissue processing and staining.
∙ Set the timer select pushbutton to the minute’s selection.
∙ Set the time-at temperature mode using the timer mode
In procedure of decalcification inorganic or mineral
select switch.
composition is lost and organic structure remains. For
∙ Set the time pause position (out) of a timer/door button.
example, enamel is completely lost in decalcification and
Microwave at a Temperature of what remain is dentin, pulp and cementum in tooth.
67˚C for 5 Minutes Ground Section
Take the plastic container and processing rack from Calcified tissue like teeth and bone may be ground to a thin
the microwave and remove the rack from the container section.
draining the rack on a paper towel. Dump the ethyl alcohol.
Place the processing rack back into the plastic container
Equipment for Making Ground Section
and fill with isopropyl alcohol.
Place the plastic container in the microwave. Add the • L aboratory lathe
temperature probe centrally into the bath and microwave at • Coarse and fine abrasive lathe wheel with water
a temperature of 74°C for 3 minutes. directed onto wheel
Remove the plastic container and rack from the • Wooden block 1 × 1 inches in size
microwave. Place the processing rack into an empty • Adhesive tape
container and fill with paraffin that is at a temperature • Camel hair brush
setting of 60°C. Agitate the rack so that the excess • Mounting medium
Isopropanol will mix with the paraffin. Pour this paraffin • Microscope slide and cover glass.
Procedure for Ground Section Hard Tissue Microtome/Saw

The specimen is cut in mesiodistal or buccolingual plane Microtome (Fig. 2.15)
into half by means of carborundum wheel if a longitudinal Slices of very hard materials such as resin embedded 31
section is to be prepared. For the cross, section the tooth is undecalcified bone or teeth can be prepared without
cut half in horizontal direction. destroying the morphology of the specimens for the use in
The wooden block is wrapped with adhesive tape, light microscopy section thicknesses of approximately 30
sticky side directed outwards towards the tooth specimen. microns can be achieved under optimal conditions.
First coarse abrasive wheel of the lathe is used and then With the saw microtome, slices of about 100 to 500
a fine abrasive wheel up to 1 mm thickness of the tooth or microns thickness are prepared and finished to a thickness of
bone. The hard tissue or tooth is ground on an abrasive about 20-30 microns for the transmission light microscope.
stone manually on an Arkansas stone (Fig. 2.13).
When the desired thickness is achieved, the section is Decalcification Methods for
carefully lifted by camel hair brush and mounted on the Hard Tissue (Fig. 2.16)
slide (Fig. 2.14). The ground section can demonstrate the inorganic or
calcified portion. For the study of organic component and
structure, it is required that the hard tissue be made soft
enough to render it to be cut by knife into thin sections and
then stain it.
The steps for decalcification and staining of the hard
tissue includes.
Decalcification
The decalcification is done by immersing the tooth or
bone in acid of specific concentration until the specimen
becomes soft.
The decalcifying agents are nitric acid 5 percent,
formic acid 10 percent, EDTA. The nitric acid decalcifies
the tooth fast than any other reagent, i.e. approximately
within a week. The disadvantage of HNO3 method is that
Figure 2.13 The tooth is slowly abraded in one direction keeping the tissue integrity may not be preserved intact.
the tooth parallel to the arkansas stone immersed in water
Figure 2.14 Ground sections mounted on slides Figure 2.15 Saw microtome-Specimens are embedded in
resin after fixation (Courtesy: Lieca)
32
Figure 2.16 The decalcifying solution is changed till the teeth

become soft enough for further processing and cutting
Figure 2.17 Cryostat for frozen sections

Formic acid and EDTA are slow decalcifies but
give better results. The tissue is checked by piercing
with needle or a X-ray is taken for remains of calcified
material. The tissue is washed in running water for 24
hours to remove the acid completely.
For fixation, it is then immersed in neutralized and 10
percent buffered formalin to which excess CaCO3 is added.
The procedure after fixation is same as for other soft tissue
specimens.
FROZEN SECTIONS
At times during performance of surgical procedures, it is
necessary to get a rapid diagnosis of a pathologic process. Figure 2.18 The tissue section on the slide needs to be
The surgeon may want to know if the margins of his deparaffinized in xylene before the actual staining procedure starts
resection for a malignant neoplasm are clear before closing,
or an unexpected disease process may be found and require
cryostat is about –20 to –30° Celsius. The tissue sections
diagnosis to decide what to do next, or it may be necessary
are cut and picked up on a glass slide. The sections are then
to determine if the appropriate tissue has been obtained for
ready for staining.
further workup of a disease process.
The embedding process must be reversed in order
This is accomplished through use of a frozen section.
to get the paraffin wax out of the tissue and allow water
The piece(s) of tissue to be studied are snap frozen in a
soluble dyes to penetrate the sections. Therefore, before
cold liquid or cold environment (–20 to –70° Celsius) (Fig.
any staining to be done, the slides are “deparaffinized” by
2.17). Freezing makes the tissue solid enough to section
running them through xylenes (or substitutes) to alcohols
with a microtome.
to water (Fig. 2.18). There are no stains that can be done on
tissues containing paraffin.
STAINING OF CUT SECTIONS The staining process makes use of a variety of dyes
Frozen sections are performed with an instrument called that have been chosen for their ability to stain various
a cryostat (Fig. 2.17). The cryostat is just a refrigerated cellular components of tissue. The routine stain is that
box containing a microtome. The temperature inside the of hematoxylin and eosin (H and E) (Fig. 2.19). Other
to remove the water, then through clearing agents to a point

at which a permanent resinous substance beneath the glass
cover slip, or a plastic film, can be placed over the section.
33
ARTIFACTS IN HISTOLOGICAL
SECTIONS
A number of artifacts that appear in stained slides may
result from improper fixation, from the type of fixative,
from poor dehydration, incomplete paraffin infiltration,
improper reagents, and poor microtome sectioning.
Figure 2.19 Hematoxylin and eosin stained slides Tissues that are insufficiently dehydrated prior to
clearing and infiltration with paraffin wax will be hard to
section on the microtome, with tearing artifacts and holes
in the sections.
In humid climates, tissue processing cycles should
allow sufficient time for dehydration and final ethanol
dehydrant solution should be at 100 percent concentration.
Though alcohols such as ethanol make excellent
fixatives for cytologic smears, they tend to make tissue
sections brittle, resulting in microtome sectioning artifacts
with chattering and a “venetian blind” appearance.
Bubbles under the cover slip may form when the
mounting media is too thin, and as it dries air is sucked in
under the cover slip.
Contamination of clearing agents or cover slipping
media may also produce a bubbled appearance under the
microscope.
BIBLIOGRAPHY
1. Artifacts in Histological and Cytological Preparations. http://
Figure 2.20 Slide storing cabinet with slide trays that can www.leica-microsystems.com/pathologyleaders/artifacts-
hold atleast 100 slides in-histological-and-cytological-preparations/. Accessed on
30-06-12.
stains are referred to as “special stains” because they are 2. Baird IL, Willian B, Bockman OT. Technique of
decalcification suited to electron microscopy of tissues
employed in specific situations according to the diagnostic
closely associated with bonnet. Anat Rec. 1067;159:281-90.
need.
3. Boon ME, Kok LP. Microwave Cookbook of Pathology:
The art of microscopic visualization, 2nd Edn. Rev Leiden.
MOUNTING (FIG. 2.20) Leyden: Coulomb Press; 1998.
4. Clayden EC. A discussion on the preparation of bone
The stained section on the slide must be covered with a
sections by the paraffin wax-method with special reference
thin piece plastic or glass to protect the tissue from being
to the control of decalcification. J Med Lab Technol. 70:103-
scratched, to provide better optical quality for viewing 23.
under the microscope, and to preserve the tissue section for 5. Clearly SF. Survey of microwave and radiofrequency
years to come. biological effects and mechanisms. DWE publication. FDA.
The stained slide must go through the reverse process 1978;75:1-33.
that it went through from paraffin section to water. The 6. Comanescu M, Annaratone L, D’Armento G, Cardos G,
stained slide is taken through a series of alcohol solutions Sapino A, Bussolati G. Critical steps in tissue processing
in histopathology. Recent Pat DNA Gene Seq. 2012;6(1): 17. Ng K PL, Ng, L. Microwave-stimulated decalcification of
22-32. compact bones. Eur J Morphol. 1992;50:150-5.
7. Cooke, Colour Atlas of Anatomical Pathology, 3rd Ed. J. 18. Quick Reference Handbook for Surgical Pathologists, 1st
34 8. Cunningham CD, Schulte BA, Bianchi LM, Weber PC, Edn. Springer; 2011.p.201.
Schmiedt BN. Microwave decalcification of human temporal 19. Rode SM, Faria MR, Monteiro MP. Using microwaves goes
bones. Laryngoscope. 2001;777:278-82. the decalcification of mineralized tissues of rat mandibles.
9. DC Allen, RI Cameron. Histopathology. Specimens: Rev Odontol Univ Sao Paulo. 1996;70:15-8.
Clinical, Pathological and Laboratory Aspects, Springer; 20. Roncaroli E, Mussa B, Bussolati G. Microwave oven for
2004.p.518. improved tissue fixation and decalcification. Pathologica.
10. Diana Weedman Molavi. The Practice of Surgical Pathology: 1991;55:307-10.
A Beginner’s Guide to the Diagnostic Process, 1st Edn. 21. Susan C, Lester MD. Manual of Surgical Pathology 2nd
Springer; 2008;344. Edn.). 2005.p.384.
11. Engelbreth-Holm J, Plum CM. A rapid and easy method of 22. The Washington Manual of Surgical Pathology. Peter A
decalcification. J Pathol Bacteriol. 1951;65:751-3. Humphrey, Louis P Dehner, John D Pfeifer L W and W
12. Geoffrey Rolls. An Introduction to Specimen Processing. 2008.p.816.
Leica Biosystems, Wetzlar, Germany 26. May 2011. 23. Vongsavan N, Matthews B, Harrison GK. Decalcification
http://www.leica-microsystems.com/pathologyleaders/an-
of teeth in the microwave oven. Histochem J. 1990;22:311-
introduction-to-specimen-processing/. Accessed on 30-06-12.
80.
13. Hornbeck C, Emmanual J, Bloebaum RD. A comparative
24. Werner Martin, Chott Andreas, Fabiano Alfredo, Battifora
study of three paraffin media for preparing large decalcified
Hector. Effect of Formalin Tissue Fixation and Processing
bone sections. J Histotechnol. 1986:9:227-9.
on Immunohistochemistry American Journal of Surgical
14. http://www.leica-microsystems.com/biosystems/products/
Pathology. 2000;24(7):pp.1016-9.
total-histology/tissue-processing/. Accessed on 30-06-2012.
25. Westra. Surgical Pathology Dissection: An Illustrated
15. Iza KB. Dimenstein, Grossing Technology in Surgical
Guide. WH Westra, RH Hruban, TH Phelps, C Isacson, 2nd
Pathology http://www.grossing-technology.com. Accessed
on 30-06-2012. Edn. 2003.p.280.
16. Lillie RD, Laskey A, Greco J, Jacquier Burtner H, Jones P. 26. Winsor L. Tissue processing. In Woods A and Ellis R
Decalcification of bone in relation to staining and phosphatases eds. Laboratory histopathology. New York: Churchill
techniques. Am J Clin Pathol. 1951;21:711-22. Livingstone, 1994;4.2-1–4.2-39.
1. Which one of the following is NOT a decalcifying 5. Glass microscope slide is coated with:
agent: a. Alcohols b. Nitric acid
a. Nitric acid 5% b. EDTA c. Paraffin d. Egg albumin
c. Ethyl alcohol d. Formic acid 10%
6. Lugol’s solution is:
2. Most common fixative agent is: a. Potassium iodide, iodine solution
a. Xylene b. 10% Neutral formalin b. Mercuric chloride, potassium dichromate
c. Chloroform d. EDTA c. Absolute alcohol, chloroform
3. Carnoy solution contains: d. Picric acid, formaldehyde
a. Mercuric chloride, potassium dichromate
7. Embedding is usually done with:
b. Absolute alcohol, chloroform
a. Paraffin b. Chloroform
c. Potassium iodide, iodine solution
c. Both d. None
d. Picric acid, formaldehyde
4. Dehydration of specimen is done by: 8. Paraffin wax bath is used for:
a. Alcohols b. Chloroform a. Infiltration b. Dehydration
c. Acids d. Distill water c. Clearing d. Sectioning
9. Potassium chloride and potassium dichromate solution 15. Poly-L-lysine coating is:
is: a. 0.05% PLL aqueous b. 0.03% PLL aqueous
a. Lugol’s solution b. Zenker’s solution c. 0.01% PLL aqueous d. 0.02% PLL aqueous
c. Carnoy’s solution d. Bouin’s fluid 16. The minimum thickness up to which a tissue should be 35
10. Melting point of paraffin is: dissected:
a. 20 degree centigrade b. 30 degree centigrade a. 3–4 mm b. 1–2 mm
c. 100 degree centigrade d. 60 degree centigrade c. 7–8 mm d. 5–6 mm
11. Disposable blades used in microtomy are coated with: 17. Cryostates were introduced in:
a. Acetone b. Polytetrafluoroethylene a. 1959 b. 1954
c. Stainless steel d. Carbon c. 1969 d. 1988
18. Which gas is given of while chloroform is heated:
12. Universal size of slides used in microtomy:
a. Laughing gas b. Ethylene gas
a. 35 × 25 mm b. 76 × 25 mm
c. Phosgene gas d. Hydrogen sulfide
c. 30 × 40 mm d. 40 × 50 mm
19. Stages of tissue processing are
13. Temperature required to prevent splitting and a. Embedding, clearing, infiltrating, dehydration
cracking of the section: b. Clearing, dehydration, embedding, infiltrating
a. 100°C for 1 hour b. 150°C for 30 minutes c. Infiltrating, clearing, dehydration, embedding
c. 37°C for 24 hours d. 200°C for 8 hours d. Dehydration, clearing, infiltrating, embedding
14. Cryostates were introduced in: 20. Dehydration reagents are mainly:
a. 1959 b. 1954 a. Hydrophobic b. Hydrophilic
c. 1969 d. 1988 c. Both d. None.
Histological Staining Methods
Shubhangi Mhaske (Jedhe ), Amol Gadbail
A Chapter Outline
O Chemistry of stains Masson trichrome

O Classification of stains van Gieson 's method
O Theories of staining Gordon and sweets ' method for reticulin fibers
• Chemical theory Mallory' s phosphotungstic acid hematoxylin (PTAH) for
• Physical theory muscle striations
O Vital staining Oil red O stain
O Factors affecting staining Congo red
O Staining procedure Sudan black B
O Hematoxylin and eosin stain Prussian blue (Perl 's Prussian blue reaction)
O Special stains Safranin O
• PAS (Periodic acid Schiff method) Toluidine stains
• Trichrome stains Giemsa stain
INTRODUCTION can be altered by the use of silver or gold impregnation

techniques also .
Antoniy van Leeuwenhoek discovered microscope (1719)
and started a new era in biological sciences . Robert Hook Alteration of color: Alteration of color is achieved
demonstrated that cell is basic unit of life . In following by using some chemical that binds to some structural
years description of cell and its constituents was given. component of a cell and imparting a color to it, which can
All these observations were made without any aid except be easily identified . This is very cost effective, specific and
use of microscope . Under microscope, tissues and their sensitive technique that can be used in day today practice
constituents are usually transparent and colorless and of histology .
cannot be easily distinguished from each other. In order to
make different structures easily identifiable two different CHEMISTRY OF STAINS
techniques were developed .
Stain is any substance which when added to living cells or
Altering contrast and resolution : Contrast and resolu - to fixed structures or structural components makes them
tion can be altered using microscopes such as phase clearly visible or detectable . A staining agent is composed
contrast, polarizing and electron microscope. Contrast of two components, chromogen and auxochrome .
Chromogen: Property of color to a substance comes from CLASSIFICATION OF STAINS (TABLES

the chromogen group. Most important chromogen groups
are azo, nitro, nitroso, quinoid and ethylene groups act as
3.1 TO 3.4)
chromogen. These groups do not have the ability to bind to 37
Table 3.1 First—based on the source
the tissues.
Natural • Xanthene
Auxochrome: This ability of binding to the tissues comes
• Hematoxylin – Eosin
from auxochrome group, which contains ionizable groups
• Carmine – Rose Bengal
that bind to tissues. Sulfates, carboxyls and hydroxyls Synthetic or artificial – Rhodamine B
groups form auxochrome, e.g. picric acid. It contains nitro • Nitroso – Erythrosin
and hydroxy groups. Nitro group is chromogen as it imparts – Naphthol green – Phenolphthalein
yellow color. Hydroxy group helps picric acid to bind to • Nitro – Pyronin Y
the tissues thus acts as auxochrome. If hydroxy radicals – Picric acid – Anthra quinone
are removed although the resulting compound has yellow • Azo – Alizarin red
color it cannot bind to the tissues, so it is not a true staining – Congo red • Thiazole
agent. Similarly if nitro groups are removed from picric – Orange G – Tital yellow
acid it loses its color although it can bind to the tissues, – Sudan III and IV • Quinolin
again not a true staining agent. Thus both chromogen and – Bismark brown – Pinacyanol
– Methyl orange • Phthalocyanine
auxochrome are necessary for calling a compound staining
– Alcian blue
agent.
Table 3.2 Second—based on tissue component of stains
Protein Ninhydrin Schiff’s method, Millon reaction, diazotization-coupling method for Tyrosine, performic acid-alcian
blue method for sulfide and disulfide bonds, DMBnitrite method modified Sakaguchi method for arginine.
Nucleic acid DNA Feulgen reaction, naphthoic acid hydrazineFeulgen method
RNA Methyl green pyronine method
DNA and RNA Gallocyaninchrome alum, acridine orange
Carbohydrate Glycogen PAS, Best’s Carmine, Hexamine silver method
Mucins PAS, alcian blue, alcian bluePAS technique, Dialyzed IronPrussian blue technique,
Hale’s Technique, Azure A, Toluidine blue, Southgate’s mucicarmine
Lipids Routine Oil red O, Sudan black B, bromine-acetone Sudan black, Nile blue sulfate
Free fatty acid Copper rubeanic acid method
Cholesterol Perchloric acid-naphthoquinone (PAN)
Proteoglycerides Gold hexamine method, Filipino method, UVSchiff method osmium tetraoxide
method
Triglycerides Calcium lipase method
Connective tissue Masson trichrome, van Gieson’s method.
Bone Trichrome stains, PAS, Schmorl’s picrothionin method, silver staining, von Kossa method, solochrome
cyanine method
Neural Silver impregnation, Bielschowsky’s silver stain, PTAH method, Cajal’s method
Amyloid Highman’s congored, Sirius red, Toluidine blue, crystal violet, Thioflavin T
Mast cell granules Toluidine blue, azure A, PAS, alcian bluesafranine
Keratine, keratohyalin Performic acid alcian blue method, Lendrum’s Phloxin-tartazine method, Ayub-Schlar method
Table 3.3 Based on pH
Acidic Basic
38
Eosin, erythrosine, fluorescein, picric acid, alizarin, acid Hematoxylin, acridine red, aniline blue, azure, basic fuchsin,
fuchsin, bismarck brown crystal violet, malachite green, safranine
Table 3.4 Example of different stain
Type of stain Examples

Reaction of stain: Acidic—Eosin stain
Basic—Hematoxylin stain
Neutral—Leishman stain
Physical stain Stain dissolve in tissue without any chemical reaction such as: SUDAN III
for fatty tissues
Vital stain Staining living tissue inside the body—Trypan blue stain
Supra-vital stain: Staining living tissues outside the body—Brilliant cresyl blue
Metachromatic stain Staining the tissues with a color different from the original color of stain
Toluidine blue staining for mast cells
Polychromatic stain Staining the tissues with multiple colors in spite of using a single stain
Giemsa stain for blood
Orthochromatic stain: Staining the tissues with the same color of the stain, such as H & E
Histochemical stain Staining the different chemical components of the cell
Immuno-histo-chemical stain: Localization and staining specific proteins by the antigen antibody reaction
THEORIES OF STAINING new products, which is one of properties of chemical

reactions. Also a chemical reaction is continuous until
Much of the discussion pertaining to the theory of staining one of the reactants is exhausted. This is rarely observed
took place during 1920 to 1930. It was related to the doubt in staining.
whether staining is chemical or physical. Thus there are
two theories, chemical and physical. Physical Theory
According to physical theory one of the three physical
Chemical Theory
factors are responsible for staining.
Certain parts of cell are acidic and others basic and few
Physical penetration of dyes: Most of the tissue
are neutral. Similarly staining agents are also acidic, basic
components are more or less porous. In these pores staining
or neutral. Thus it is natural to expect that acidic stains
agents penetrate because of simple physical forces such as
form chemical bonds with basic tissue components and
capillarity and osmosis.
basic stains combine with acidic tissue components, e.g.
cytoplasm of most of cells is acidic so it combines with Adsorption of stains: Staining agents are adsorbed onto
basic stain eosin. Nucleus is acidic as it contains DNA and the surface of tissue by physical forces such as van Der
RNA stains with basic stain hematoxylin. Waal’s forces.
Chemical theory explains the specificity of staining. Absorption of stains: Absorption is a process by which
But it cannot explain certain facts such as absence of one substance takes another substance into it. Tissues take
up staining agent in solution form and retain it in the same Progressive staining: Different component of tissues
form even it dried completely. are stained in sequence, so that at the end of correct time
Any or all of these factors may be responsible for differential staining is achieved, e.g. eosin.
staining. Physical theory does not explain the specificity of 39
Regressive staining: In this technique tissue is first over
staining and differential staining.
stained so that all parts of tissue take up the stain and then
Now it is believed that reactions involved in staining
excess stain is removed from unwanted parts of tissue
lies in the borderland between the chemistry and physics,
by a process known as differentiation. Hematoxylin is
where it is impossible to say that a given product is purely
differentiated using acid alcohol or prolonged water wash.
physical or purely chemical. Various mechanisms such as
electrostatic bonding, hydrogen bonding, van Der Waal’s Staining by selective solubility: Certain substances have
forces, covalent bonding, hydrophobic bonding and dye ability to dissolve in particular tissue components, such as
aggregation are involved in staining. lipids. Such substances are known as lysochromes. Usually
lysochromes are alcoholic solutions that preferentially
VITAL STAINING dissolve in lipids than in alcohol. This results in staining of
lipids, e.g. Sudan Dyes.
It is the method of demonstrating living cells. It is of two
types: supravital staining and infravital staining. Staining by chemical production of colored substance:
Supravital staining is done for live tissue, where cells Some staining procedures use pale or colorless solution,
retain vitality after staining. which react with tissue components to produce colored
Infravital staining is done to cells that are removed substances, e.g. Feulgen reaction. In feulgen reaction straw
from the body and cells loose their vitality after application colored or colorless solution of leuco basic fuchsin is used.
of the stain, e.g. alizarin red is supravital staining used to It is converted into purple colored substance in presence of
demonstrate developing bone. Toluidine blue is supravital aldehyde group in tissues.
staining that is used to demonstrate precancerous lesions in Metallic impregnation: Some metallic substances can
the oral cavity. India ink preparation is infravital staining be reduced by tissues into opaque, usually black deposits.
method used to demonstrate macrophages. Certain intestinal cells contain melanin and phenolic
Direct staining: Dyes such as eosin stain tissues perfectly substances which reduce ammoniacal silver nitrate into
when in alcoholic or aqueous solutions. This is known as silver and appear black such cells are called argentaffin
direct staining (Fig. 3.1). cells. Certain cells do not reduce ammoniacal silver nitrate
directly but do so when extraneous reducer is added. Such
Indirect staining: Stains such as hematoxylin require cells are known as argyrophil cells. Metallic impregnation
additional substance known as mordant before satisfactorily techniques are used to demonstrate reticulin, nerve fibers,
binding to the tissue. This is known as indirect staining spirochetes and fungi.
(Fig. 3.2).
Metachromatic staining: Certain tissue substances
combine with stains to produce a color that is different
from the original color produced in the rest of the
tissue. This is known as metachromasia and substance
that produces metachromatic staining is known as
chromotrope, e.g. Toluidine blue: original color of this
Figure 3.1 Direct staining dye is blue. But in tissue components such as cartilage,
mucins, mast cell granules and amyloid it takes red color.
Other metachromatic stains are azure A and B, methyl
violet and safranine.
FACTORS AFFECTING STAINING

Fixation: Fixation has profound effects of staining.
Figure 3.2 Indirect staining Different fixative retains different tissue components to
differencing degrees. Wellretained tissue substances are Deparaffinization: Hematoxylin and eosin stains are
stained well. For example, aldehyde retain proteins well usually aqueous solutions, not miscible with paraffin. To
but do not retain lipids. Alcohols are poor fixatives but stain tissues, paraffin must be removed from the tissues
40 retain lipids well. first. This is usually done by two changes of xylene.
Temperature: Usually staining is done at room Hydration: Xylene is also hydrophobic. This is removed
temperature. But temperature of staining solution can be by an agent that is both miscible with water and xylene.
changed. It has two effects: The ideal candidate for this is alcohol. Xylene from tissues
1. It increases diffusion of the dye molecules. is removed by increasing decreasing concentrations of
2. It allows greater reactivity between dye and tissue. alcohol in water.
pH: Staining depends on pH as it controls the ionization Nuclear staining: It is done by dipping the slide in
of both tissues and stains which is prerequisite for staining. hematoxylin solution.
Presence of impurities: Impurities present in any stain Differentiation: In regressive staining excess hematoxylin
sample have influence on dye solubility and they have real in tissues is removed usually by one percent acid alcohol
effect on the intensity of staining. An impurity may alter (1% HCl in 70% alcohol). Differentiation is also brought
the pH, it may alter the dissociation of dye or tissue or it by prolonged water wash. In progressive staining this step
may act as mordant. In some cases impurities are necessary is omitted.
to assure proper staining, pure stain may be detrimental to
Bluing: Nuclear staining results in red colored nucleus.
staining. For example, if Rose Bengal is used with distilled
This is converted into bluish black color by weak alkali
water poor results are obtained. If tap water is used instead
solutions such as lithium carbonate, ammonia water or tap
of distilled water excellent results are obtained.
water.
Ripening of staining solution: Some staining solutions
are effective only after exposure to air, light or warmth Counterstaining: This is done by keeping slides in eosin
for weeks or months or exposure to chemical oxidizers. solution.
Hematoxylin when freshly prepared is useless as a nuclear Dehydration: After staining slide is mounted using DPX.
stain. It becomes active after some weeks of storage. DPX is hydrophobic. So water from the tissues should be
This is because natural hematoxylene is not a dye but removed first. This is done by increasing concentration of
when oxidized it forms hematin which is a good stain. alcohol. Later alcohol is removed by the changes of xylene.
Hematoxylene can be oxidized naturally by sunlight which Any remaining water is removed by warming on the slide
takes months or by chemical oxidizers such potassium warmer.
permanganate or mercuric oxide instantaneously.
HEMATOXYLIN AND EOSIN STAINS
Steps of Staining Procedure
(FIG. 3.3)
• Deparaffinization
• Hydration The most popular, time tested routinely used stain in histology
• Nuclear staining and histopathology methods is hematoxylin and eosin,
• Differentiation commonly called as HE. Their popularity is because of the
• Bluing simplicity of staining method and stain preparation, good
• Counterstaining contrast, long lasting staining which is beneficial for preserving
• Dehydration. slides. It mainly stains nucleus with good intranuclear detail.
Similarly, eosin stains cytoplasm and most of connective
tissue fibers in varying degrees of pink color.
STAINING PROCEDURE
After making paraffin sections of the tissue block routine Hematoxylin
staining of hematoxylin and eosin and if required, special It is most successfully used natural staining agent. It is
staining is done. Most of the principals of staining hold extracted from Haematoxylin campechianum, which is
good for most of the stains with minor modifications. A cultivated in West India. It is extracted from the wood using
section usually goes through seven steps during staining. hot water and precipitated by urea from aqueous solution.
Classification of Hematoxylin Based on Mordants

• Alum: Ehrlich’s, Delafield’s, Mayer’s, Harris,
Cole’s, Carazzi’s and Gill’s 41
• Iron: Weigert’s, Heidenhain’s, Loyez’s, Verhoeff’s
• Tungsten: Phosphotungstic acid hematoxylin
(PTAH)
• Molybdenum: Phosphomolybdic acid hematoxylin
• L ead: For demonstration of endocrine cells of GIT
• C hromate: Weigert-pal’s technique
• H ematoxylin without mordant: For demonstration of
minerals like iron and copper.
Alum hematoxylin: Alum hematoxylin are most com

monly used hematoxylin solutions. Mordant used is either
Figure 3.3 Hematoxylin and eosin stain potash alum (aluminum potassium sulfate) or ammonium
alum (aluminum ammonium sulfate). These stains can be
Natural hematoxylin is not a stain as it does bind to the used either progressively or regressively. These initially
tissues. On oxidation it forms hematin. This hematin binds give red color to nucleus. This color is converted into blue
to the tissue and gives color. Hematoxylin can be oxidized black color when section is washed in a weak alkali solution.
to hematin by two methods. This is called as bluing. Alkaline solutions such as saturated
1. Natural oxidation solution of lithium carbonate, 0.5 percent ammonia in distilled
2. Chemical oxidation. water, Scott’s tap water substitute or tap water can be used
for bluing. Alum hematoxylin are sensitive to acidic solutions
Natural oxidation: Exposure to sunlight, heat or prolonged used in few staining techniques such as trichrome staining. So
storage in light can oxidize hematoxylin to hematin. This in connective tissue stains alum hematoxylin cannot be used.
process takes a very long time as long as 3 to 4 months. But
Iron hematoxylin: Ferric chloride and ferric ammonium
once the solution is ripened in this way it can be stored for
sulfate are used as mordants in iron hematoxylin. These
longer time. And it becomes better with passage of time.
salts are strong oxidizers. So they are not mixed with
Ehrlich and Delafield’s hematoxylin solutions are prepared
hematoxylin; they are either used before or after application
in this manner.
of hematoxylin solution, to prevent over oxidation. In
Chemical oxidation: Oxidation of hematoxylin to hematin Weigert’s hematoxylin, mordant is applied after keeping
can be achieved rapidly by the use of chemical oxidizers slide in hematoxylin solution (postmordanting). In
such as sodium idodate (Mayer’s Harris, Gill’s), potassium Heidenhain’s hematoxylin it is applied before putting the
idodate (Carazzi’s) mercuric oxide (Harris) iodine solution slide in hematoxylin solution (premordanting). Mordant
(Cole’s hematoxylin) or potassium permanganate (PTAH). solution is also used for differentiation. Iron hematoxylin
Chemically oxidized has a very short life as chemicals can be used to demonstrate wide variety of tissue in addition
continue is to oxidize hematoxylin continuously. Once all to nucleus. Using Heidenhain’s hematoxylin mitochondria,
is converted to hematin further oxidation of hematin into a muscle fibers and myelin can be demonstrated. Verhoeff’s
colorless compound occurs which is unsuitable for staining. hematoxylin is used to demonstrate elastic fibers. Iron
hematoxylins are technique sensitive, time consuming
Use of mordant: Hematoxylin is a anionic substance
and have short lifespan. Differentiation stage requires
which does not have good affinity to tissue. To increase
microscopic control for accuracy.
affinity various mordants are used. Mordants form a
complex with hematoxylin forming a positive charge Phosphotungstic acid hematoxylin: It is used to
which binds to negatively charged tissue components like demonstrate myelin sheath. Here phosphotungstic acid
nuclear chromatin. Based on mordants hematoxylin can be acts as a mordant. If Phosphomolybdic acid is used in
classified into various types. Only major three like alum, place of phosphotungstic acid, as a mordant, it can be
iron and tungsten described below. used to demonstrate collagen, coarse reticulin fibers and
argentaffin cell granules. Lead salts are used as mordants cartilage, fungi and Russell bodies. All these structures can
in demonstration of granules of endocrine cells of GIT. be demonstrated by PAS reaction (Fig. 3.4).
Chromate is used as mordant in Weigertpal technique
42 to demonstrate myelin. In this technique tissues are Trichrome Stains (Fig. 3.5)
treated with dichromate solution before embedding. Later Popularly called as connective tissue stains, trichrome stains
sections are dipped in hematoxylin to get brilliant staining are used in differential demonstration of connective tissue
of myelin. In few techniques minerals present in tissues components such as collagen, muscle, fibrin, etc. In this
act as mordants. In this way iron and copper present in technique two or more acidic dyes of different molecular
tissues can be demonstrated with hematoxylin without any weight and contrasting color are used in the staining.
mordant. How exactly differential staining observed in trichrome
stains is not understood. But various tissue factors and
Eosin molecular weight of the dyes are thought to be responsible
Eosin is a Xanthene dye. It is available in various forms for this. Due to fixation tissues form networks. These
such as eosin Y: eosin yellow, water soluble eosin s: ethyl
eosin, alcohol soluble eosin B: bluish eosin.
Of this eosin Y is more popular and is widely used. Eosin
is most suitable and popular stain used with hematoxylin.
This is because it gives good contrast with hematoxylin.
It stains cytoplasm of different cells differently in varying
shades of pink and red. Similarly it can stain different types
of connective tissue fibers and matrices differently.
In addition to routine staining it is used in pap stain
along with azure. It is usually used in concentration of 0.5
to 1 percent aqueous solution. To increase staining capacity
and to get sharp and crisp staining little acetic acid is added
to this solution.
SPECIAL STAINS
When there is need to identify stain special structures use
Figure 3.4 PAS stain showing positive magenta color for
of H & E stain is limited. In such cases staining agents mucin
that can identify structure of interest are employed. There
are numerous special stains that are available, but only a
handful are employed regularly. PAS and trichromes are
most commonly employed in identifying glycogen and
collagen fibers respectively.
PAS (Periodic Acid Schiff Method)

This is a technique for the demonstration of carbohydrates
in tissue sections. When basic fuschin is reduced using HCl
it form Schiff’s base. This is a colorless compound which
regains its color once it comes in contact with oxidizing
groups in tissue sections it again gains its color.
In this technique periodic acid applied to expose
oxidizing groups of glycogen molecule. Later tissue is
flooded with Schiff’s base. Glycogen molecules are stained
into a bright pink or magenta color. Glycogen is present
in many substances such as basement membrane, mucins, Figure 3.5 Trichrome stain showing more than one color
networks create pores of different sizes in different

structures. In RBC’s very dense network with small pores
is produced. In muscles pores of intermediates size are
produced. As collagen is loose tissue it produces least 43
dense network and pores or large size. Dyes of different
molecular weight occupy these pores. Thus dye of least
molecular weight occupies smallest pore and larger pores
are occupied by large dye molecules.
Masson Trichrome (Fig. 3.6)

In this method with a small molecule size dye acid fuchsin/
ponceau is used first and this stains all tissue elements in the
section. This stain is selectively removed from unwanted
areas by differentiating with phosphomolybdic acid, which
also acts as a mordant for the next step of the procedure. A Figure 3.7 van Gieson’s stain showing collagen stained red
dye of large molecular size, light green is then applied to
the section. Nucleus is stained by hematoxylin. Thus nuclei
saturated solution of picric acid and acid fuschin. The
and elastic fibers are stained blue to black, cytoplasm,
mixture of the two acid dyes in acid solution competes for
muscle and acidophilic granules are stained red; collagen,
available linkages.
reticulin, basement membranes, osteoid and basophilic
Acid fuchsin being a larger molecular size is restricted
granules are stained green.
to more permeable collagen fibers whereas the diffusible
van Gieson’s Method (Fig. 3.7) picric acid penetrates the compact muscle, cytoplasm and
red cells. These results in blue/black nuclei, red collagen
The van Gieson Technique is a trichrome stain for the
and other tissues such as muscle, elastin, reticulin, basement
demonstration of connective tissue. It is suitable for
membrane, and fibrin take yellow color.
showing coarse collagen fibers, but not recommended for
fine collagen fibers or muscle collagen comparisons. In
this technique nucleus is stained first using hematoxylin.
GORDON AND SWEETS’ METHOD
Then slides are kept in van Gieson solution which contains FOR RETICULIN FIBERS (FIG. 3.8)
This method is used for reticulin fibers. Reticulin fibers are
fine delicate fibers, which are normally found connected to
stronger and coarser collagen fibers.
They make up the bulk of the supporting framework
of the liver, spleen and lymph nodes. The basic principle
in this stain is silver from silver oxides is selectively
deposited on the reticulin fibers, which appear black
after conversion to reduced silver, by the reducing agent
(formalin).
Gold chloride is used as a toner to give a clearer
background and unreduced silver is removed by treatment
with sodium thiosulphate.
Mallory’s Phosphotungstic Acid Hematoxylin

(PTAH) for Muscle Striations (Fig. 3.9)
The PTAH stain demonstrates many tissue structures,
Figure 3.6 Masson’s trichrome stain showing green collagen particularly fibrin, muscle striations, cilia and glial fibers,
fibers and red muscle fibers plus many CNS structures.
44
Figure 3.8 Gordon and Sweet stain for reticulin fiber Figure 3.10 Oil red O staining
Figure 3.9 PTAH stain Figure 3.11 Congo red stain of amyloid
The mechanism by which twocolor staining is Congo Red

achieved from a mixture of hematin and phosphotungstic
Amyloid is homogeneous and eosinophilic; the deposits
acid is obscure. The blue color was due to metachromatic
are extracellular and may become sufficiently large
like staining effect.
enough to cause damage to surrounding tissues. When
stained with the Congo red stain the amyloid will
Oil Red O Stain (Fig. 3.10) birefringe an apple green color under the polarizing
The purpose of oil red stain is to demonstrate fat or lipids microscope (Fig. 3.11).
in fresh tissue sections. Fat occurring in an abnormal place,
such as fatty emboli that may develop after either a bone Sudan Black B (Fig. 3.12)
fracture or an injury that crushes a fatty body area. This stain is used for lipofuscin pigments. Lipofuscins,
Tumors arising from fat cells (liposarcomas) can be the wear-and-tear pigment, and is the accumulation of
differentiated from other types of tumors. Staining with liposome’s, which have absorbed the worn-out, indigestible
oilsoluble dyes is based on the greater solubility of the dye parts of the cell and known as residual bodies. It is a yellow
in the lipoid substances than in the usual hydroalcoholic brown pigment, and will stain after being processed in
dye solvents. paraffin.
Prussian Blue (Perl’s Prussian Blue

Reaction)
Prussian blue histology stain is used to stain iron (ferric 45
iron and ferritin). This method is considered by many to
be the first classical histochemical reaction. The reaction
occurs with the treatment of sections in acid solutions of
ferrocyanides.
Any ferric ion (+3) in the tissue is combined with the
ferrocyanides and results in the formation of a bright blue
pigment called Prussian blue or ferric ferrocyanides (Fig.
3.13). It demonstrates ferric iron in tissue sections.
Figure 3.14 Safranin O stain showing orange color
Figure 3.12 Sudan black B stain for triglyceride
Figure 3.15 Toluidine blue stain
Safranin O
This histology stain will stain mucin, cartilage and mast
cells granules on formalin-fixed, paraffin-embedded tissue
sections, and may be used for frozen sections as well.
It stains them orange/red (Fig. 3.14). Safranin O is
sometimes used as a counterstain. The cartilage and mucin
will be stained orange to red, and the nuclei will be stained
black. The background is stained green.
Toluidine Stains (Fig. 3.15)

Mast cells are found in the connective tissue and their
cytoplasm contains granules (metachromatic) composed of
Figure 3.13 Persian stain for iron detection heparin and histamine.
Giemsa Stain (Fig. 3.16)

This is a histology stain for peripheral blood smears and
46 bone marrow. It is also used to visualize parasites and
malaria. This is a Romanowski type stain. Methylene blue
and eosin are used. Erythrocytes stain pink/red.
Platelets and leukocytes stain blue. Giemsa’s stain is
a member of the Romanowski group of stains, which are
defined as being the black precipitate formed from the
addition of aqueous solutions of methylene blue and eosin,
dissolved in methanol.
BIBLIOGRAPHY
1. Bancroft JD, Gamble M. Theory and Practice of
Histological Techniques, 6th edn. London: Churchill
Figure 3.16 Giemsa stain showing parasites
Livingstone, 2008.
2. Brown RW. Histologic Preparations: Common Problems
and Their Solutions. Northfield, IL: College of American
Pathologists, 2009.
Toluidine blue should stain mast cells redpurple and
3. Horobin RW, Bancroft JD. Troubleshooting Histology
the background blue. Metachromasia, tissue elements Stains. New York, Edinburgh: Churchill Livingstone, 1998.
staining a different color from the dye solution, is due to 4. Kiernan JA. Histological and Histochemical Methods:
the pH, dye concentration and temperature of the basic dye. Theory and Practice, 4th edn. Bloxham, UK: scion, 2008.
Blue or violet dyes will show a red color shift, and red dyes 5. Michael H Ross, Wojciech Pawlina. Histology: A Text and
will show a yellow color shift with metachromatic tissue Atlas. Hagerstwon, MD: Lippincott Williams and Wilkins,
elements. 2006. ISBN 0-7817-5056-3.
1. Staining agent composed of: 6. Stains with acidic pH is:

a. Chromogen group b. Auxochrome group a. Picric acid b. Erythrosine
c. Both d. None of the above c. Hematoxylin d. Alizarin
2. Which one belongs to the chromogen group: 7. Mordant used in which staining technique:
a. Sulfates b. Carboxyl a. Indirect staining b. Direct staining
c. Hydroxyl d. Azo c. Progressive staining d. Regressive staining
3. Which one belongs to the auxochrome group: 8. Hematoxylin mainly stains:
a. Azo b. Carboxyl a. Cytoplasm b. Nucleus
c. Nitro d. Nitroso c. Muscle fiber d. Connective tissue
4. Which one is the natural source of stains: 9. Carbohydrate in tissue can be demonstrated by:
a. Hematoxylin b. Picric acid a. Trichrome stains b. Iron hematoxylin
c. Eosin d. Quinolin c. PAS d. Eosin
5. Stains with acidic pH is: 10. Popularly used eosin is:
a. Hematoxylin b. Eosin a. Eosin Y b. Eosin S
c. Safranine d. Basic fuchsin c. Eosin B d. Eosin Z.
Diagnostic Pathology
Shubhangi Mhaske (Jedhe), Pradnya Lele
A Chapter Outline
3 Biopsy 3 Exfoliative cytology

3 Types of biopsy 3 Oral mucosal brush biopsy
3 Incisional biopsy 3 Liquid based cytology
3 Excisional biopsy 3 Fine needle aspiration cytology
3 Punch biopsy 3 Frozen section biopsy
INTRODUCTION Indications
There are various important investigations which are Alteration from normal : When after careful clinical
required for the diagnosis and treatment plan of various examination, any alteration from normal is seen and
disorders related to the oral cavity. Laboratory studies are it is not possible to identify the condition clinically, a
histopathological investigation is necessary.
an extension of physical examination in which tissue; blood,
urine or other specimens are obtained from patients and are Evaluation of histological nature: It is also indicated to
subjected to histological, bio-examination, microbiological or evaluate the exact histological nature of any soft tissue or
immunological examination. Information obtained from these intra-osseous lesion .
investigations help in identifying the nature of the disease .
Screening of abnormal tissue : To screen abnormal tissues
Autopsy: It is the histopathological study of the tissues
removed from oral cavity including granuloma and cyst.
removed after the death of an individual .
Biopsy: It is the study of tissues removed from a living being Confirmation of diagnosis : It is also done to confirm the
to confirm the diagnosis through histopathological study. existence and nature of directly apparent malignancy so
that the treatment can be undertaken immediately .
BIOPSY Evaluation of nonneoplastic lesion : It is also done for
It is a process of surgically removing tissue from a patient diagnostic tests for evaluation of nonneoplastic lesions
for histopathological examination. It provides valuable such as mucosal nodules, papilloma, erosive lichen planus,
information in determining the prognosis and type of the erythema multiforme, lupus erythematous pemphigus,
treatment required . pemphigoid and desquamative gingivitis .
Contraindication Normal adjacent tissue: To compare and strengthen the

diagnosis normal adjacent tissue should be included.
Inflammatory lesion: Biopsy is not usually indicated in
48 acute infalmmatory lesion. Handling Biopsy Tissue
Site near the vital structure: One should be very careful Biopsy should reach to histopathologist without any damage.
while performing biopsy of the lesion adjacent to vital During biopsy the grasping area of forceps should be
structure. away from the site to be removed.
Angiomatous lesion: Unless it is needed you shouldn’t go
for the biopsy of angiomatous lesion. Submission of Specimen
Biopsy should not be delayed when following features
Information: The submission of specimen should be
are present:
accompanied by the date of biopsy, name, age and sex
Rapid increase in size of the lesion that cannot be
of the patient, the area from where biopsy specimen is
explained by inflammation, edema and opening of new
taken and brief description of clinical appearance of lesion
vascular channels.
and associated symptoms, along with tentative clinical
Absence of any recognized irritant, particularly when
diagnosis.
the lesion is chronically ulcerated or bleeds spontaneously.
Iodine containing surface antiseptics should be avoided
Presence of firm regional lymph nodes, especially when
since they have a tendency to stain certain tissue cells
they seem to be fixed to surrounding tissues.
permanently.
Destruction of roots and loosening of teeth with
evidence of rapid expansion of the jaw Includes normal tissue: The biopsy specimen should
History of malignancy elsewhere in the body, previous not only include some of the lesion but also the adjacent
history of oral cancer and radiation therapy. clinically normal tissue.
The portion of the biopsy specimen to be used for
Application of Biopsy in Dentistry routine histological study should be placed at once in
• Diagnosis of pathologic lesions a suitable fixing solution—usually 10 percent neutral
• Determining neoplastic and non-neoplastic lesions buffered formalin and sent to the pathology laboratory.
• Therapeutic assessment Teeth specimen: For the histologic examination of teeth,
• Grading of tumor the apex of tooth should be clipped with a pair of pliers or
• Diagnosis of metastatic lesions a small hole should be drilled into the radicular pulp with
• Evaluation of recurrence. dental bur to allow penetration of the fixative.
The excellent preservation of cellular detail required is
Complication of Biopsy obtained by following methods:
• Hemorrhage ∙ Cutting the specimen into tiny blocks before fixation.
• Infection ∙ Use of special fixatives that preserve cellular detail
• Poor biopsy wound healing with minimum disruption from rapid dehydration or
• Spread to adjacent organs and reaction to local osmotic shock.
anesthesia. ∙ Post fixation and processing of tissues in the laboratory
after the initial period of prefixation. As soon as
Ideal Requirement of Biopsy Tissue possible after the surgical procedure.
Less traumatized: The tissue taken for biopsy should have
minimal trauma. TYPES OF BIOPSY PROCEDURES
(TABLE 4.1)
Adequate representative tissue: It must include the most
suitable representative pathologic region of a lesion for a Incisional Biopsy
pathologist to interpret. Incisional biopsy can be performed by removing a wedge
To facilitate treatment: Biopsy sample should help to shaped specimen of the pathological tissue along with
facilitate to prescribed treatment and assess its efficacy. surrounding normal zone.
available in market. The different sizes are color coded to

Table 4.1 Types of biopsy
help quickly identify the correct size (Figs 4.1A and B).
Commonly used Less commonly used
• Aspiration • Bite Procedure 49
• Curettage • Brush In this technique, a sharpened hollow tube; several
• Excisional • Cone millimeters in diameter is rotated until underlying bone or
• Incisional • Core
• Fine needle • Endoscopic
• Punch • Irrigation
• Scrape • Pressure
• Trephine • Shave
• Sponge
Indications
Large lesion: If the lesion is large and diffuse and extends

deeply into the surrounding tissue so that total removal
cannot be obtained easily with local anesthesia, an
incisional biopsy is indicated.
Management point of view: Lesions in which diagnosis
will determine whether the treatment should be conservative
or radical.
Procedure
Site selection: Carefully observe and palpate the lesion so

that a decision can be made regarding the appropriate site
that will produce a more representative specimen. It is best
to select the site away from an area of necrosis, and areas
of intense inflammation which may make interpretation A
difficult.
Infiltration of local anesthesia: The tissue around the
specimen is infiltrate with 2 percent local anesthetic solution.
Make a incision: With a scalpel, make an elliptical
incision encompassing the selected area of the lesion. The
incisional lines must be deep enough to include underlying
connective tissue to the level of muscle or bone.
Suturing: Suture is inserted through the end; upward
tension is applied while tissue sample is dissected out.
Punch Biopsy
With this technique the surgical defect that is produced is
small and does not require suturing. B
Nowadays disposable, punch tools with a sharp Figures 4.1A and B Disposable punch biopsy kit with different
seamless blade covered with a safety cap for protection are sizes and color codes (Courtesy: Healthlink Biopsy company tools)
muscle is reached. The tissue is then removed in the same It is the preferred treatment if, the size of lesion is such
manner as in incisional or excisional biopsy. that it may be removed along with the margins of normal
tissue and wound can be closed primarily.
50 Excisional Biopsy
Total excision of a small lesion for microscopic examination Contraindication
is called as ‘excisional biopsy’. It is a therapeutic as well Larger lesions than 2 to 4 cm—more cases are to be
as a diagnostic procedure. Normal tissue on the margins of operated with proper surgical planning and anesthesia
the lesion should be included. Vascular lesions—e.g. hemangioma
Tumors adherent to important vital structures or major
Indications blood vessels.
It is indicated when the lesion is relatively small and less
than 1 cm in diameter, sessile or pedunculated and well Procedure
circumscribed (Figs 4.2A and B). Anesthetize the lesion with 2 percent local anesthetic
Tissues which are freely movable and located above the containing vasoconstrictor. Care is taken not to inject
mucosa or just beneath the surface. directly into the lesion that is to be removed.
With the scalpel make an elliptical incision on either
side of the base of the lesion so that incision line intersected.
The blade should be at an angle of 45° towards the
center of the lesion.
Outward tension is placed on the lesion by means of
suture or with the help of tissue forceps attached at the edge
of specimen. Care must be taken not to crush the specimen.
The specimen is now gently dissected out with either
a scalpel or a pair of surgical scissors. The tissue must
immediately be submerged in 10 percent formalin solution.
Surgical site is closed with either silk or absorbable
sutures placed approximately 5 mm apart.
Care of sending large excisional tumor mass/cyst
biopsy specimens for histopathology.
A The specimen should be immediately immersed in 10
percent neutral buffered formalin for fixation (Figs 4.3A
and B). The volume of the fixative should be enough to
fix the specimen. It is said that the volume of the fixative
solution should be 20 times more than the size of the biopsy
mass.
Mounting of the Specimen

Making of a good museum specimen involves meticulous
planning at the time of biopsy, grossing and careful
handling thereafter. Oral lesions vary widely in their gross
presentations, color, texture, pattern of growth and are
relatively smaller and more fragile.
The specimen is tied or mounted onto a glass plate or
B glass or with the help of suture thread or fine nylon wires
Figures 4.2A and B Smaller lesions of approximately 2 to (Figs 4.3A and B). Then it is carefully placed or immersed
4 cm can be excised under local anesthesia with normal into a transparent jar of acrylic or preferably glass filled
adjacent skin or mucosa with 10 to 20 percent formalin (Figs 4.4A and B).
51
A B
Figures 4.3A and B Biopsy specimen should be immerzed in 10 percent neutral buffered formalin immediately after
removal to fix or preserve in a lifelike state
Exfoliative cytology is an attractive option for early

diagnosis of oral cancer including atypias and squamous
cell carcinomas.
It is a useful tool for detection, monitoring of initial
alterations and establishment of adequate treatment.
Recent advances in exfoliative cytology such as
development of cytomorphometric method, DNA content
determination, detection of tumor markers has contributed
to renewed interest in this field.
In this, the surface of the lesion is either wiped with
some sponge material or scraped to make a smear. The
appreciation of the fact that some cancer cells are so typical
that they can be recognized individually has allowed
A B the development of this diagnostic technique, which is
Figures 4.4A and B Nowadays PET bottles acrylic is also developed by Dr George Papanicolaou who, is also known
used for ease of mounting the specimen as the ‘father of cytology’ and the technique is called as
PAP smear. Use of cytology in orofacial area was done in
The size of the jar is selected as per the suitable display the year 1949 by Morrison.
of the specimen. The jar is sealed airtight with adhesive.
Labeling should be properly done so as to demonstrate the Principle of PAP smear
structures and pathology. Individual cells can often be diagnosed as such
microscopically by their large size, their pleomorphism,
EXFOLIATIVE CYTOLOGY increased nucleo-cytoplasmic ratio, hyperchromatism and
Exfoliative cytology is a technique in which exfoliated prominence of nuclei and their abnormal mitosis. Cancer
cells assessed for pathological change. The cells examined cells exfoliate more easily than normal cells most likely
are either manually scraped (mechanical exfoliation) or because their cohesiveness is lowered as a result of either
they are the cells which are spontaneously exfoliated. decrease in number of tight junctions.
Indication, Advantage and Disadvantage of malignant and for which, the dentist is unable to obtain
Exfoliative Cytology permission for a biopsy.
52 Indication Patient who received radiotherapy: For sequential

• Patient refusal for biopsy laboratory evaluation of an area of the mucosa that has
• Follow up previously been treated by radiation or by excisional
• Debilitated patient biopsies to remove malignancy.
• Aid in the diagnosis Vesicular lesion: For evaluation of vesicular lesions
• Rapid evaluation where facilities for rapid evaluation of Tzanck smears are
• Patient who received radiotherapy not available.
• Vesicular lesion
Advantages Advantages
• Blood less procedure
Blood less procedure: It is quick, simple, painless,
• Minimum discomfort
bloodless and an inexpensive procedure.
• No anesthesia
• Check against false negative biopsy Minimum discomfort: It causes minimum discomfort to
• Recurrent carcinoma the patient and is easily performed.
• Screening test No anesthesia: No anesthesia is required.
• No complication
• Rapid diagnosis Check against false negative biopsy: It helps to check
• No delayed wound against false negative biopsy.
• Healing Recurrent carcinoma: It is especially helpful in a follow-
• Cost effective up detection of recurrent carcinoma.
Disadvantages
• Firm tumors Screening test: It is valuable for screening lesions whose
• Inadequate sampling gross appearance is such that biopsy is not warranted.
• Poor cellularity. No complication: It is a safe procedure as complications
are rare.
Indications
Rapid diagnosis: It enables a rapid diagnosis and is
Compromize situation: It is done as a compromize, when economical.
the patient refuses for biopsy.
No delayed wound healing: Less risk of delayed wound
Follow up: As a means of follow-up for recurrence in healing and infection.
patients who had radiation therapy for the lesion that was
superficial or adjacent to bone, periodic recall of high-risk Cost effective: Cost of cytological investigation is less as
patient. compared to others.
Debilitated patient: In place of biopsy, when dealing Others advantage: 100 percent accuracy in lymph
with extremely debilitated patients posing problems to node aspiration from metastatic carcinoma, melanoma,
determine a suitable biopsy site. Hodgkin’s and Non-Hodgkin’s lymphoma.
Aid in the diagnosis: As an aid to the diagnosis of some Disadvantages

dermatological diseases such as Pemphigus, white sponge ∙ Firm tumors: Firm tumors may prevent a proper cyto-
nevus, oral malignant and premalignant lesions. diagnosis due to paucity of cells in the aspirate.
Rapid evaluation: For rapid evaluation of an oral lesion ∙ Inadequate sampling: Oral cytology can give false
that on clinical grounds, is thought to be malignant or pre- negative findings due to inadequate sampling.
∙ Poor cellularity: Some specimens cannot be assessed ∙ Class III (indeterminate): This is a stage in between
due to poor cellularity. that of class II and IV and separates noncancer cells
from cancer cells displaying wider atypia that may be
Instruments Used suggestive of cancer but they are not clear-cut and may 53
• G lass microscopic slide, lead pencil, cement spatula represent precancerous lesion or carcinoma in situ and
or wax carver. a biopsy is recommended in such cases.
• Wooden tongue depressor, tooth pick, canister of ∙ Class IV (suggestive of cancer): Few cells with
cytospray. malignant characteristic or many cells with borderline
• 95 percent isopropyl alcohol or ethyl alcohol. features. Biopsy is mandatory in such cases.
∙ Class V (positive of cancer): Cells that are obviously
Procedure malignant. Biopsy is mandatory in such cases (Figs 4.5
and 4.6).
You should always use two slides for each site to be
sampled.
Patient data: With lead pencil print the patient’s name,
date when the slide is prepared and the site of the lesion on
frosted end of glass microscopic slide.
The instrument selected to remove the superficial cell
must have a square edge with a contour sufficient to scrape
off the superficial layer of cells. When the lesion is very
small, the edge of tooth pick is effective.
Clearing the surface: Clear the surface of oral lesions of
debris and mucus.
While the tissue is stretched, the squared edge of the
collection instrument is positioned at the back of the lesion
and is firmly held and brought forward and pressure applied
until visible material is collected.
Vigorous scraping of the entire surface of the lesion Figure 4.5 Class V cytology showing cellular and nuclear
several times is done with a metal cement spatula or a pleomorphism with increased mitosis. PAP stain (x 400)
moistened tongue blade.
Collected material is then quickly spread evenly over
the microscopic slide.
Fix it in commercial preparation such as spraycyte,
95 percent alcohol or equal part of alcohol and ether,
immediately before it dries.
Then allow it to stand for thirty minutes so that it air
dried. Repeat the procedure and prepare a second smear.
Reporting/Interpretation
It is reported by a cytologist as follows into one of the
following five classes:
∙ Class I (normal): It indicates that only normal cells
are observed
∙ Class II (atypical): Presence of minor atypia but no
evidence of malignant changes Figure 4.6 Keratinizing and nonkeratinizing epithelial cells
ORAL MUCOSAL BRUSH BIOPSY

The most recent development in oral biopsy technique is
54 the oral mucosal brush biopsy. It was introduced in 1999.
This technique utilizes a disposable brush to collect a
transepithelial sampling of cells. This brush has got 2
cutting surfaces i.e. flat end and circular border (Figs 4.7
and 4.8).
Specimen obtained brushing on the site and smeared on
clean labelled glass slide (Figs 4.9 and 4.10).
The sample is screened by an neurally networked
computer that is programmed to detect cytologic changes
associated with premalignancy and squamous cell
carcinoma.
Figure 4.7 Oral CDx brush for mucosal brush biopsy Figure 4.9 Technique of CDx brush biopsy
Figure 4.8 Circular brush can generate deeper cells for Figure 4.10 Brush is rolled onto the slide to shed
analysis or the plating of the cells
The specimen is reviewed by a pathologist for final

diagnosis. This technique is ideal for determining the
need for scalpel biopsy in benign-appearing oral mucosal
leukoplakias. 55
LIQUID BASED CYTOLOGY

Correspond to a sampling where cells are put in a
suspension in a conservative liquid. Sample taken in the
same manner with the help of cytobrush and immediately
rinsed in bottle containing a fixative and is transported. Figure 4.13 Aspirate expressed carefully onto slide
After centrifugation, sediments that are obtained spread and spread evenly before fixation
onto the slide followed by staining with PAP staining (Figs
4.11 and 4.12).
Advantages of Liquid Cytology
• Produces homogenous smears
• Decreases debris
• Has a long storage life
• Samples used for immunohistochemistry, HPV
testing, DNA ploidy analysis, micronuclei count, etc.
FINE NEEDLE ASPIRATION

CYTOLOGY
It is the microscopic examination of an aspirate obtained
by inserting a fine needle into the lesion. It is a painless and
a safe procedure for rapid diagnosis. First discovered by
Kun in 1847 and reintroduced in 1930 by Martin and Ellis.
Figure 4.11 Cells are put in suspension before placing
onto slide in steps 1, 2 and 3
Indications
FNAC of salivary glands is a useful procedure for
evaluation of salivary gland tumors.
It is indicated in lesions in which open biopsy require
extensive procedures.
It is also used for examination of enlarged clinically
suspicious lymph nodes.
It is used to check against recurrence or local extension.
Detection of metastatic squamous cell carcinoma
within cervical nodes can also be done by FNAC.
Procedure
Insert needle into lesion: Position the needle within the
target tissue.
Apply full suction: Plunger is pulled to apply negative
pressure. Redirect needle within target, Apply suction until
Figure 4.12 Liquid cytology and PAP stain small amount of aspirate appears in hub of the needle.
Obtain greater field: Needle is moved back and forth FROZEN SECTION BIOPSY
within the target tissue to obtain a greater field.
It is performed in order to get an immediate histological
56 Release negative pressure: Negative pressure is then report of a lesion. It is done to determine whether a lesion
released while the needle remains within the target tissue. is malignant or not. It is also used to evaluate the margins
Blowing of aspirate on slide: Needle is withdrawn and of an excised cancer, to ascertain that the entire lesion is
then the defumed air drawn in the syringe and the aspirate removed at the time of surgery. The tissue is obtained from
is blown onto the slide (Fig. 4.13). lesion and it is kept in deep freeze and then frozen tissue
is sectioned and stained to get a prompt diagnosis. In this
Fixing: Fixing is done in 95 percent alcohol for 1 hour for
type of biopsy, the slides cannot be preserved for future
PAP stain and a little prolonged for HE stain.
reference.
Steps in FNAC
BIBLIOGRAPHY
• Insert needle into lesion
• Apply full suction 1. John Bankroft. Theory and practice of histological
• Obtain greater field techniques; Churchill Livingstone; 6th edn.
• Release negative pressure 2. Natarajan S, Ranjan J, Boaz K. Museum mounting
techniques: Revisited econo-mode. Indian J Pathol
• Blowing of aspirate on slide
Microbiol. 2012;55:260-1.
• Fixing.
1. Biopsy is contraindicated in: 6. To determine the nature of fluid the procedure is:
a. Pappiloma b. Erythema multiforme a. Excisional biopsy b. Incisional biopsy
c. Angiomatous lesions d. Erosive LP c. Aspiration biopsy d. Intraosseous biopsy
2. Biopsy is used for: 7. Which of the following procedure is rarely performed
a. Diagnosis of pathological lesions in oral cavity:
b. Grading of tumor for diagnosis a. Punch biopsy b. Excisional biopsy
c. Diagnosis of metastatic lesions c. Incisional biopsy d. Aspiration biopsy
d. All of the above
8. Slides cannot be preserved for future in:
3. Karnovsky’s fixative is:
a. Oral mucosal brush biopsy
a. 4% Formaldehyde b. 10% Formalin
b. Punch biopsy
c. 10% Acetic acid d. None
c. Frozen section biopsy
4. Excisional biopsy is indicated when a lesion is: d. Incisional biopsy
a. Less than 2 cm in diameter
b. Less than 1 cm in diameter 9. Father of cytology is:
c. More than 2 cm in diameter a. C. Gram
d. More than 5 cm in diameter b. Edward Jenner
c. Morrison
5. Incisional biopsy is indicated when the lesion is: d. Dr George Papanicolaou
a. Small and diffuse
b. Small and localized 10. Test which determines the number of colonies of
c. Large bacteria is:
d. Large and diffuse extends deeply into surrounding a. Snyder test b. Lactobacillus count test
tissues c. Albans test d. Dewar test
Advanced Diagnostic
Techniques
Shubhangi Mhaske (Jedhe), Monal Yuwanati
A Chapter Outline
O Histochemical techniques O Proteomics

O Fixation in histochemistry O Cytogenetics
O Enzyme histochemistry • Karyotyping
O Immunohistochemical methods • Chromosomal banding
O Immunofluorescent techniques • Karyotype analysis
O Flow cytometry • Chromosome ideograms/karyogram
O Polymerase chain reaction ( PCR) • Alterations in chromosome number
O Hybridization methods • Number and size of bands
O Laser captures microdissection
As knowledge of pathology expanded, it was found that HISTOCHEMICAL TECHNIQUES

hematoxylin and eosin staining alone is not adequate to
identify various structures present within tissues . A need Histochemistry is study of the chemical composition of
for procedures that can identify special structures was tissues by means of specific staining reactions. It involves
felt . This led to identification of various special dyes and identification of various enzymes and other chemical
various techniques that can identify one or few components substances in relation to tissues or organelles within a cell .
that are difficult to identify by routine H&E staining . Such To study the distribution of these chemical substances
techniques involved identifying definite chemical groups, it is essential to preserve these carefully. Certain fixatives
so they were called as histochemical techniques . Advances cause alteration in chemical structure, making identification
in immunology led to replacement of these chemicals and of these substances difficult . Thus fixation is one of
dyes with antibodies . This improvement led to increased important aspect during histochemistry .
sensitivity and specificity in identifying various tissue
components .
FIXATION IN HISTOCHEMISTRY
Advances in genetics brought about a revolution in For identification and localization of chemical substances
field of pathology and microbiology . Now because of use tissue must be preserved in a such way that it causes
of various DNA /RNA based techniques, it is now possible minimal changes the reactivity of the cytoplasmic and
to identify pathology at gene level . Also very rapid extracellular macromolecules, for example enzymes,
identification of microorganisms is possible . proteins, carbohydrates, lipids and nucleic acids . This is
accomplished by using optimum osmotic conditions, cold

Table 5.1 Techniques in enzyme histochemistry
temperatures, controlled pH of the fixing solutions, and the
minimum possible exposure to the fixative. Enzymes Technique
58 Formaldehyde is widely used fixative. It is ideal for Alkaline phosphatases Gomori Calcium method, Azo
fixing proteins and enzymes. It causes cross linking of dye coupling method, Naphthol
proteins without affecting their reactivity. But it is a poor AS-BI method
preservation of lipids. But it is used to fix lipids especially Acid phosphatases Gomori lead method, Azo dye
phospholipids by adding calcium which prevents coupling method, Naphthol
dissolution of phospholipids. For histochemical techniques AS-BI method
neutral buffered formaldehyde is used as cold solution. ATP Metal precipitation method
Formal calcium is also used. Other substances used are Esterase a Naphthyl acetate method,
gluteraldehyde and acrolein. Indoxyl acetate method
For study of glycogen, glycoprotein, proteoglycans Cytochrome oxidase Seligman’s technique
and nucleic acids mixtures of many chemicals are used β-glucuronidase Naphthol AS-BI method
as fixatives. For example Rossmans fluid is used for G6PD Lead method
visualization of glycogen, glycoprotein, and proteoglycans,
Conroy’s solution is used as fixative for nucleic acids.
Certain tissue groups are highly labile and sensitive. autoradiography. Since immuno-histochemistry involves
Such chemicals cannot be fixed by use of fixative. They specific antigen-antibody reaction, it has apparent
are visualized from fresh frozen sections, where tissue is advantage over traditionally used special and enzyme
rapidly frozen by used of liquid nitrogen immediately after staining techniques that identify only a limited number
removal from the body. Other techniques are freeze drying of proteins, enzymes and tissue structures. Therefore,
and freeze substitution. immunohistochemistry has become a crucial technique and
Various techniques involved in identifying different widely used in many medical research laboratories as well
chemicals and structures are given in the chapter Theory as clinical diagnostics.
and practice of staining.
Principle
ENZYME HISTOCHEMISTRY The basic principle of this technique is isolation of tissue
antigen that is needed to be localized in tissue. Using this
It involves identifying and locating enzymes within the
pure protein, antibodies are formed. Such antibodies are
cell. The technique involves series of chemicals to get
labeled with enzymes. These labeled antibodies are used
insoluble colored reaction product. By this technique
to cleave a suitable substrate that produces a insoluble
various enzymes have been identified (Table 5.1).
colored precipitate in the tissues.
Animals such as mouse, guinea pig, sheep or rabbit
IMMUNOHISTOCHEMICAL METHODS are used to develop antibodies. Such antibodies contain
Immunohistochemistry is the localization of antigens different types of antibodies derived from different clones
in tissue sections by the use of antibodies labeled with of plasma cell. Such antibodies are called polyclonal
fluorescent dye, enzyme or radioactive isotope or colloidal antibodies. They are highly sensitive but specificity is
gold. Albert H Coons 1941 was the first to label antibodies low. To increase specificity monoclonal antibodies are
with a fluorescent dye, and use it to identify antigens in used. They are manufactured by a special process known
tissue sections. With the expansion and development of as hybridoma technique. Here plasma cells producing
immunohistochemistry technique, enzyme labels have been specific antibody are fused with malignant cells obtained
introduced such as peroxidase and alkaline phosphatases. from myeloma, a neoplasm of B-lymphocytes. Monoclonal
Colloidal gold label has also been discovered and used antibodies are highly specific.
to identify immunohistochemical reactions at both light and Various enzymes are used for labeling of the anti-
electron microscopy level. Other labels include radioactive bodies. More commonly used are alkaline phosphatases
elements, and the immunoreactions can be visualized by and horse radish peroxidase. These enzymes produce color
Advanced Diagnostic Techniques
when substrate is applied. Different enzymes use different adjunct to H & E diagnosis in a majority of equivocal tumor
substrates. Alkaline phosphatase enzyme uses Fast Red and cases, through the establishment of a definitive diagnosis
New Fuchsin. On the other hand horse radish peroxidase or through confirmation of H & E section impression. It is
uses Diamino Benzidine and AEC as substrate. used to establish origin of a tumor. For this various markers 59
are used.
Types
Tumor markers of patient course and outcome: Certain
• Direct method proteins and enzymes are indicators of prognosis and
• Indirect method. response to therapy. Such molecules are used to determine
the course and outcome of the therapy (Table 5.2).
Types (Fig. 5.1)
Other applications: Immunohistochemistry is used to
There are numerous immunohistochemistry methods that
identify bacteria and viruses in odontogenic tumors and
may be used to localize antigens.
other lesions. It is used to identify human papilloma virus
Direct method: It is also called as single step method. Here in ameloblastoma. Similarly it has been used to identify
labeled antibody is directly applied to tissues. Later addition various genes that contribute to tumorigenesis, growth and
of substrate causes formation of colored precipitate. spread of tumors.
Indirect method: This is two step methods where two
antibodies are applied. First, primary antibody is applied IMMUNOFLUORESCENT
which reacts with protein of interest. This is followed by a TECHNIQUES
second antibody which is “antibody of primary antibody”. Immunofluorescent techniques are similar to immuno-
This secondary antibody is labeled with enzyme, which on histochemistry. Here instead of enzymes antibodies are
application of substrate produces colored precipitate. labeled with a fluorescent dye-fluorescence. There are two
There are numerous other methods available for
immunohistochemistry which vary in specificity and
sensitivity. They are labeled antibody method, enzyme Table 5.2 Various markers used in oral pathology
bridge method, PAP method, APAAP method, immune
Tissue Markers
complex method, avidine biotine Method, streptavidine
Epithelium Keratins
biotine method, and avidin-biotine-peroxidase complex
method, LSAB method, polymeric methods and CSA General mesenchymal marker Vimentin, desmin, GFAP
method. Muscle markers Desmin, actins, myoglobin,
myogenin
Applications of Immunohistochemistry Neural markers S-100, GFAP,
Diagnostically challenging oral malignant neoplasm: neurofilaments, and CD57
Immunohistochemistry has been shown to be an effective Endothelial markers CD31, CD34, and factor
VIII–related antigen
Melanocytic markers HMB45, MART-1
(Melan-A), and S-100 protein
Lymphoid markers κ and l, CD3, CD15, CD20,
CD30, CD45, CD68, CD79a,
ALK-1, and TdT
Neuroendocrine markers Synaptophysin and
chromogranin
Ewing’s tumor marker CD99
Metastatic tumor markers CK7, CK20, villin
Salivary gland tumor markers S-100 protein and actins
Odontogenic tumor markers Shethilin, enamelin,
Figure 5.1 Direct and indirect immunohistochemistry ameloblastin
techniques in immunofluorescence, i.e. direct and indirect

technique.
60 Direct immunofluorescence technique: In this technique

antigens are detected in the tissues of patients. Here labeled
antibodies are directly applied to the tissues. If antigen of
interest is present in the tissues it will show fluorescence
when viewed under fluorescent microscope (Fig. 5.2).
Indirect immunofluorescence technique: This is a method
of detecting antibodies in plasma. In this technique (Fig.
5.2) direct immunofluorescence method plasma containing
primary antibodies is applied to suitable substrate. Over
this secondary antibody labeled with fluorescein is applied
and slide is observed under fluorescent microscope for
fluorescence (Fig. 5.3).
Applications: Direct immunofluorescence has more
application in dentistry than indirect technique. Direct
technique has application in diagnosis of various
vesiculobullous lesions (Table 5.3). It is used in diagnosis
of lichen planus, lupus erythematosus, pemphigus vulgaris,
and mucous membrane pemphigoid. Anti-human IgG,
IgA, IgM, complement component (C3), and fibrinogen
are used to diagnose these lesions. These are used on fresh Figure 5.3 Indirect immunofluorescence technique
frozen sections or tissues fixed with Michael’s solution.
Based on location and appearance of immunofluorescence
these lesions are diagnosed (Fig. 5.3).
Table 5.3 Immunofluorescence appearance of various
Pemphigus vulgaris: It shows interepithelial distribution vesiculobullous lesions
of anti-IgG immunofluorescence (some C3 deposition is Lesion Serum component Appearance
also noted). It takes a net like distribution pattern. Pemphigus IgG Net like ditribution
Mucous membrane pemphigoid: It shows a linear pattern vulgaris
of immunofluorescence at the basement membrane. All Mucous IgG, IgA, C3 Linear distribution
three antibodies, i.e. anti-IgG, anti-IgA, and anti-C3 membrane along basement
antibodies are deposited at the basement membrane. pemphigoid membrane
Lichen planus IgG, IgA, C3 Shaggy or fibrillar
pattern
Lupus C3, IgM, IgA, and Coarse granular
erythematosus IgG deposition
Lichen planus: Lichen planus (LP) shows a characteristic

pattern of fibrinogen deposition outlining the basement
zone and extending irregularly into the superficial lamina
propria, described as a “shaggy” or “fibrillar” pattern.
Another finding typical of but less frequently observed in
lichen planus is the presence of IgM-, IgA-, IgG-, or C3-
positive “cytoid,” “colloid,” or “apoptotic” bodies, located
Figure 5.2 Direct immunofluorescence technique in the epithelium and superficial connective tissue.
Lupus erythematosus: DIF reactions in specimens of oral The main limitation of flow cytometry is need for single
mucosal lupus erythematosus (LE) exhibit coarse granular cell suspension. This is not a problem for samples such as
deposits of C3, IgM, IgA, and IgG in the basement zone. blood and other body fluids. It is difficult to employ this
technique for solid tumors. But now various techniques 61
FLOW CYTOMETRY have been developed to overcome this difficulty.
Flow cytometry is the process in which measurements are Application of Flow Cytometry in Pathology
made while cells in a liquid suspension are forced to flow
• It is used to support a diagnosis of malignancy
one at a time through a measuring device.
when the morphological changes are equivocal.
It is a technique for counting, examining and sorting
• It can be used to classify tumors of borderline
cells suspended in a stream of fluid. It allows simultaneous
malignancy.
analysis of the physical and/or chemical characteristics of
• It provides prognostic information independent of
single cells flowing through an optical and/or electronic
stage and grade of the tumor.
detection apparatus (Fig. 5.4).
• It helps to identify tumor relapse.
A beam of laser light is directed onto a stream of fluid
• It helps to detect tissue of region of a tumor.
containing suspension of cells. A number of detectors are
aimed at the point where the stream passes through the Tissue from paraffin embedded tissue can also be used for
light beam; one in line with the light beam and several flow cytometry. The section is dewaxed hydrated by passing
perpendicular to it. through xylene and graded alcohols. Then tissue is minced
Each cell passing through the beam scatters the light in with sharp scissors in a watch glass. Following this tissue is
some way. This scattered light is picked up by the detectors, digested using 0.5 percent pepsin and a liquid suspension
and analyzed by using computers. By detecting variations is prepared. This releases the DNA which is stained using
in the brightness at each detector it is then possible to appropriate agent and then fed into the flow cytometer.
extrapolate various types of information about the physical
and chemical structure of each individual cell. To aid in
POLYMERASE CHAIN REACTION
detection cells are usually stained immunocytochemically
or by immunofluorescent stains. Polymerase chain reaction (PCR) is the enzymatic
amplification of a specific DNA sequence in vitro to obtain
large number of DNA copies. This technique amplifies
DNA without involving any live organism, so it can be
repeated any number of times. As it is a in vitro procedure
it can be subjected to different modifications.
Polymerase chain reaction (PCR) is used to amplify
specific regions of a DNA strand. Following steps are
followed in PCR (Fig. 5.5).
Denaturation: The DNA which needs to be amplified is
isolated. It is added to reaction mixture which contains
nucleotides, primer, which closely resembles gene
of interest, and polymerase enzyme in a micropipette
and heated to high temperature of 90 to 95°C. At this
temperature two strands of DNA separate.
Application: Once primary attaches to the DNA
polymerase starts synthesizing the DNA for nucleotides
present in the reaction mixture.
Annealing: Temperature lowered to 50 to 60°C. At this
temperature primer attaches to gene of interest. In this way
Figure 5.4 Parts of a flow cytometer two DNA chains are formed at the end of one cycle. In next
RT-PCR: The RT-PCR (Reverse transcription-PCR)

is a method used to amplify, isolate or identify a known
sequence from RNA. In this technique DNA is synthesized
62 first from RNA using the enzyme reverse transcriptase.
This DNA acts as an template for further amplification.
Application of PCR in Dentistry

Microbial identification: The use of PCR has revolu-
tionized the diagnosis and study of infectious diseases
and malignancies associated with microorganisms. PCR is
used to identify bacteria and viruses in infections. It has
been used to study microorganisms causing periodontal,
endodontic infections and to identify microorganisms of
dental caries.
Human genetics: PCR plays an important role in the
identification of chromosomal disorders and hereditary
diseases, including cystic fibrosis, Gaucher’s disease,
alpha-1-antitrypsin deficiency, hemophilia, and sickle
cell anemia. PCR can also be used to analyze fetal DNA
for aneuploidy the presence of extra chromosomes or the
absence of chromosomes, trisomy 21, Turner’s syndrome,
Klinefelter’s syndrome, and for sex determination.
Forensic odontology: PCR is used for identification
of mutilated or decomposed human tissues, for sex
determination, and for disputed paternity cases.
Figure 5.5 Steps involved in PCR Tumor biology: PCR is used to identify various cancer
associated genes.
step these two DNA chains act as template for next cycle.
HYBRIDIZATION METHODS
By the end of next cycle 2X2 DNA are formed. In next
step 4X2 DNA chains are formed. This process continuous Hybridization refers to pairing of complimentary copies
exponentially. This whole step is repeated many number of of DNA. As pairing between two DNA fragments is very
times to amplify the DNA. The reaction is terminated after specific, one copy can be used to detect other copy. This
sufficient number of DNA fragments is formed. The DNA detector copy is known as a probe. It is labeled with either
amplified is separated by electrophoresis and analyzed. fluorescent material or radioactive isotopes.
Southern blot: This is a detection method for DNA.
Modifications of PCR
The DNA under question is digested using enzymes to
Hot-start PCR: This is a technique that reduces nonspecific produce small fragments. These fragments are separated
amplification during the initial set-up stages of the PCR. by electrophoresis on agar gel. Once these fragments are
Multiplex-PCR: The use of multiple, unique primer sets separated they are blotted on to a paper by placing the paper
within a single PCR reaction. on agar gel. Once the DAN fragments are separated and
blotted probe is applied. After washing it may be observed
Nested PCR: Nested PCR increases the specificity of under UV light. If it shows positive reaction it means that it
DNA amplification, by reducing background due to non- contains DNA under investigation.
specific amplification of DNA.
Northern blotting: It is similar to southern blotting. The
Quantitative PCR: This is used to measure the quantity of only difference is in this technique instead of DNA, RNA
a PCR product. is detected. The procedure is similar to Southern blotting.
Western blotting: Western blotting is not a DNA/RNA precise, and adaptable to a wide range of tissues and
blotting method. It a method used to separated proteins. It molecules to be studied. Both the tissue left behind and the
is also similar to southern blotting. tissue retrieved can be identified, and the morphology of
both is excellent. 63
In situ hybridization: It is a method of identifying DNA
Large numbers of well-characterized cells can be
and RNA in tissue samples. It is similar to southern blotting
obtained within a few minutes. The applications of
method. Here a probe containing complementary copy of
microdissection in pathology increasing. It is used to
DNA/RNA under investigation is prepared. The probe is
obtaining pure cell populations from fresh, frozen, or fixed
either labeled with radioactive isotope or fluorescent dyes.
tissues and cytology samples for DNA molecular genetic
In latter case it is called as fluorescent in situ hybridization
analysis; gene expression studies involving mRNA such as
(FISH). The labeled probe is placed on tissue and allowed
RT-PCR (Fig. 5.7).
to hybridis. This is detected by observation under
fluorescent microscope or by exposing X-ray film in case
of radioactive labeled probes. ISH has wide applications in PROTEOMICS
dentistry. It is used to detect various viruses in the tissues. It is also called as microarray technology. Here it is
Similarly it is used to identify various genes involved in possible to simultaneously analyze tens of thousands of
tumor biology (Fig. 5.6). genes in single step.
The main applications of this technology are study
LASER CAPTURES MICRODISSECTION of gene expression analysis, genotyping for detection of
point mutations, single nucleotide pleomorphism, etc.
Tissue obtained by biopsy contains mixture of different
Conceptually, DNA microarray technology is similar to the
cell populations. In such case it becomes difficult to
obtain homogeneous population of cells for further study.
In a new technique it is possible to isolate and obtain a
homogeneous population of cells using infrared laser light.
In this technique a plastic sheet is placed on the slide.
The area of interest is identified and it is radiated with
laser light. This caused plastic film in that area to melt
and adhere to tissue. The adherent tissue can be lifted and
further processed.
Thus in this technique it is possible to obtain specific
area of tissue and homogeneous population of cell without
distorting the structure and contents. This method is fast,
Figure 5.6 In situ hybridization Figure 5.7 Laser capture microdissection

underlying principles of Northern and Southern blotting. There are 22 pairs of chromosomes which match up
Here first step is preparation of probes. exactly. The karyotyping procedure is time consuming and
Probes are labeled genomic DNA, cDNA and technically demanding, when done manually. Nowadays
64 oligonucleotides produced from tumor mRNA by a process karyotyping has become fully automatic due to software
of reverse transcription. These probes are attached or (e.g. LeicaTM, Metasystems TM and CytovisionTM) are
“printed” on a solid phase or a “chip”. The tumor to be tested utilized.
is applied on this chip. Complementary DNA hybridizes Cytogenetic analyzes are almost always based on
with the corresponding probes. This hybridization results examination of chromosomes fixed during mitotic
in varying green/red and yellow fluorescent emissions. metaphase. Centromere position and arm ratios can assist
These emissions are then scanned by a argon laser reader in identifying specific pairs of chromosomes, but inevitably
and a scanning confocal microscope. This is analyzed by several or many pairs of chromosomes appear identical by
computer for quantification of intensity of thousands of these criteria. The ability to identify specific chromosomes
different genes on the array. This is compared with normal with certainty was revolutionized by the discovery that
tissues. certain dyes would produce reproducible patterns of bands
Thus in recent times diagnostic pathology has witnessed when used to stain chromosomes.
a range new technologies to help in diagnosis, prognosis,
and treatment of the diseases. Some of these methods are Chromosomal Banding
based on antigen and antibody reactions, some are based Chromosome banding has since become a standard and
on DNA hybridization, and some are based on laser indispensable tool for cytogenetic analysis and several
technology. banding techniques have been developed:
Q banding: Chromosomes are stained with a fluorescent
CYTOGENETICS dye such as quinacrine.
Cytogenetics is the study of normal and abnormal
G banding: Produced by staining with Giemsa after
chromosomes. This includes examination of chromosome
digesting the chromosomes with trypsin.
structure, learning and describing the relationships between
chromosome structure and phenotype, and in quest of the C banding: Chromosomes are treated with acid and base,
causes of chromosomal abnormalities. In the simplest case, then stained with Giemsa stain.
examination of chromosomes and characterization of an Each of these techniques produces a pattern of dark
individual’s karyotype for detection of acquired or genetic and light (or fluorescent versus non-fluorescent) bands
chromosomal abnormalities, such as translocations, along the length of the chromosomes. Importantly, each
deletions, monosomies or trisomies, etc. chromosome displays a unique banding pattern, analogous
The karyotyping procedure is one of the most important to a bar code, which allows it to be reliably differentiated
steps in conventional cytogenetic analysis. The karyogram from other chromosomes of the same size and centromeric
is an image representation of stained human chromosomes position.
with the widely used Giemsa Stain metaphase spread
(G-banding) in which homologous chromosomes are Number and Size of Bands
paired in 23 classes, arranged in order of decreasing size. Idealized diagrams (ideograms) of G-banded chromosomes
are published as standard reference points for chromosome
Karyotyping banding. The G-bands are usually portrayed in black and
The karyotyping is a set of procedures, in the scope of the the R-bands in white. Bands are numbered consecutively
cytogenetics, which produces a visual representation of the away from the centromere on both the short (p) and long
46 chromosomes, paired and arranged in decreasing order (q) arms. The total number of bands or ‘resolution’ in the
of size, observed during the metaphase step of the cellular human karyotype depends on state of chromosomes and
division (meiosis). These chromosomes are then arranged stage of mitosis.
systematically for comparison. The chromosomes pairs are Karyotypes are arranged with the short arm of the
numbered from largest to smallest. chromosome on top, and the long arm on the bottom. In
addition, the differently stained regions and sub-regions the International System for Cytogenetic Nomenclature
are given numerical designations from proximal to distal (ISCN) was established.
on the chromosome arms. The rules of the ISCN numbering system
∙ Numbering of a chromosome begins at its centromere. 65
Chromosomes can be Classified by the Position of ∙ Chromosomes are assigned a long arm and a short arm,
Their Centromere based on the position of their centromere. The shorter
• Metacentric: If its two arms are equal in length. arm of the chromosome is known as the p, or peptite
• Submetacentric: If arms’ lengths are unequal. arm, from the French word for small. The longer arm is
• Acrocentric: If the p arm is so short that is hard to known as the q. Chromosomal regions that are present
observe, but still present. on the short arm will begin with the designation p,
while regions on the long arm will begin with q.
Alterations in Chromosome Number ∙ By convention, the p arm of the chromosome is always
shown at the top in a karyotype.
Aneuploidy: Abnormal chromosome number.
∙ Each arm of the chromosome is divided into regions.
Trisomy: Cell has one extra chromosome. The numbers assigned to each region gets larger as the
distance from the centromere to the telomere increases.
Monosomic: Cell has one missing chromosome.
Regions are identified by specific morphological
Nondisjunction: It occurs when either homologue fail to features that are consistently found on a chromosome,
separate during anaphase I of meiosis, or sister chromatids such as the presence of a prominent Giemsa-staining
fail to separate during anaphase II. band. The regions are named p1, p2, etc. on the short
arm and q1, q2, etc. on the long arm.
Translocation: It is the result of chromosomal breakage
∙ Depending on the resolution of the staining procedure,
but the broken segment transfers itself to a broken segment
it may also be possible to detect additional bands within
of another chromosome. There are both balanced and
each region, which are designated by adding a digit to
unbalanced translocations.
the number of the region, increasing in value as the
Deletion: Deletion occurs when a chromosome breaks and distance from the centromere increases.
a portion of the chromosome is lost.
Groups of Chromosomes
Inversion: A section of the chromosome is inverted
(reversed) on the same chromosome. • G roup A: Chromosomes 1–3 are largest with
median centromere
Karyotype Analysis • G roup B: Chromosomes 4–5 are large with
Obtain chromosome: Obtain a set of chromosomes. submedian centromere
• G roup C: Chromosomes 6–12 are medium sized
Matching: Match the chromosomes with their homologous with submedian centromere
mate. It should be very systematic. The number one • G roup D: Chromosomes 13–15 are medium sized
chromosome is the largest. Its corresponding mate should with acrocentric centromere
be of the same size, with the same banding pattern, and • Group E: Chromosomes 16–18 are short with
have the same centromere location. median or submedian centromere
Determining the karyotype abnormalities: Determine • Group F: Chromosomes 19–20 are short with
the karyotype abnormality using the chromosome analysis median centromere
key that is below (Fig. 1). • Group G: Chromosomes 21–22 are very short with
acrocentric centromere
Identify abnormality: Identify abnormality using as standard • Chromosome X is similar to group C
reference points for chromosome banding for the disorder. • Chromosome Y is similar to group G.
Chromosome Ideograms/Karyogram Chromosomes are arranged into seven groups based
A consistent numbering system is essential for mapping on size and centromere location. The centromeres can be
chromosomes. In Paris 1971 a mapping system known as found in the middle of the chromosome (median), near one
66
Figure 5.8 Classification of chromosomes for karyotyping
end (acrocentric), or in between these first two (submedian) Fletcher M, Maxwell P (Eds), John Wiley & Sons, Ltd,
(Fig. 5.8). Chichester, UK, 2009. doi: 10.1002/9780470745069.ch2
Cytogenetic approaches enable researchers to precisely 5. Jordan RC, Daniels TE, Greenspan JS, Regezi JA. Advanced
identify the chromosomal location of any gene and examine diagnostic methods in oral and maxillofacial pathology.
cells from any type of tissue, especially tumor cells. Part I: molecular methods. Oral Surg Oral Med Oral Pathol
Identify cells that have lost or gained a specific chro- Oral Radiol Endod. 2001;92(6):650-69.
6. Jordan RC, Daniels TE, Greenspan JS, Regezi JA. Advanced
mosome, undergone a translocation event involving a
diagnostic methods in oral and maxillofacial pathology.
specific set of chromosomes, or lost or gained a copy of a
Part II: immunohistochemical and immunofluorescent
given gene or genes.
methods. Oral Surg Oral Med Oral Pathol Oral Radiol
Determine whether specific regions of chromosomes
Endod. 2002;93(1):56-74.
have been lost or gained without ever looking at the 7. Nederlof PM, van der Flier S, Wiegant J, Raap AK, Tanke
chromosomes under a microscope. HJ, Ploem JS, van der Ploeg M. Multiple fluorescence in situ
hybridization. Cytometry, 1990;11:126-31. doi: 10.1002/
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4. Hannon-Fletcher M. Cytopathology, in Advanced Tech- 10. Strachan T, Read AP. Human molecular genetics. Bios
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1. Who was the first to label antibodies with a fluorescent 6. Immunofluorescence appearance of lichen planus is: 67
dye: a. Net like b. Shaggy or fibrillar type
a. Albert Coons b. Morrison c. Coarse granular d. Linear
c. Alban d. Flemming 7. Southern blot method is used to detect:
2. Which one of the following is a marker for epithelium: a. RNA b. DNA
a. Desmin b. Keratin c. Both RNA and DNA d. Proteins
c. CD99 d. CK7 8. Method employed for identifying ATP enzymes are:
3. Which one of the following is NOT a muscle marker: a. Gomori calcium method
a. Desmin b. Actin b. Seligman’s technique
c. Myoglobin d. Enamelin c. Metal precipitation method
4. CD3, CD15, CD20 are the examples of: d. All
a. Epithelium markers b. Neural markers 9. Odontogenic tumor marker is:
c. Lymphoid markers d. Endothelial markers a. Shethilin b. CK20
5. S-100 protein is: c. Villin d. ALK-1
a. Salivary gland marker 10. Coarse granular deposition immunofluorescence
b. Melanocytic marker appearance seen in:
c. Ewing’s tumor marker a. Pemphigus b. Lichen planus
d. None c. Candidiasis d. Lupus erythematous
Healing of Wound
A \
Chapter Outline
O General factors affecting healing O Healing cementum

O Cascade of wound healing O Healing of dentin
O Healing of biopsy wound O Healing of enamel
O Healing of tooth extraction wound O Difference between skin healing and mucosal wound
O Healing of bone fracture healing
O Healing of pulp O Clinical approach to optimizing wound healing
INTRODUCTION events occur in varying degrees . The same basic molecular

mechanisms governing growth and differentiation are
Oral wounds are relatively common mucosal wounds; active to a different extent . Without these being properly
healing in these tissues can reflect the susceptibility of in place, even old wounds may be become subject to “re-
other mucosal tissue to repair and infection . Wound repair opening ”.
is a well orchestrated and highly coordinated process that
includes a series of overlapping phases : inflammation, cell FACTORS AFFECTING THE
proliferation, matrix deposition, and tissue remodeling .
This involves a complex, dynamic series of events
WOUND HEALING
including : clotting, inflammation, granulation tissue for- There are many factors that can affect wound healing which
mation , epithelialization, neovascularization, collagen interfere with one or more phases in this process, thus causing
synthesis, and wound contraction. The knowledge of the improper or impaired tissue repair. These multiple factors can
physiology of the normal wound healing process through lead to impaired wound healing . Factors may be considered
the phases of hemostasis, inflammation, granulation and in one of two categories depending on their source .
maturation provides a framework for an understanding of
Extrinsic factors: Impinge on the patient from the external
the basic principles of wound healing .
environment.
Wound healing is the physiologic response to tissue
trauma. It is related to tissue reconstitution which is the Intrinsic factors : Directly affect the performance of
process by which the body replenishes cells that are being bodily functions through the patient 's own physiology or
lost by normal physiologic events . In both processes similar condition .
Healing of Wound
In general terms, the factors that influence repair can be regeneration of capillaries and restoration of nutrient
categorized into local and systemic. Local factors are those delivery. Thus, other factors collaterally play a role in
that directly influence the characteristics of the wound situations where oxygen delivery is impaired and chronic
itself, while systemic factors are the overall health or nonhealing wounds may develop. 69
disease state of the individual that affect his or her ability
to heal (Table 6.1). Many of these factors are related, and Dressings and Local Infection
the systemic factors act through the local effects affecting Wound infection delays collagen synthesis and causes
wound healing. granulation tissue to become more fragile and prone
to bleeding. Wound Infection also delays healing as
Local Factors microorganisms compete for oxygen and nutrients with
Location of Wound macrophages and fibroblasts. The use of dressings, which
Wound in an area which has a good vascular bed heals more adhere to the wound bed, and the inappropriate usage of
rapidly than wounds in an area which is relatively avascular. antiseptics can all lead to the hindrance of wound healing.
Immobilization is important in healing of a fracture. If the Foreign Bodies
wound is in an area that is subjected to constant movement
and so the formation of new connective tissue is disrupted Foreign bodies in the wound may be due to the presence
(in the corner of mouth) thus delaying the healing process. of grit, parts of old dressings, suture material, staples,
etc. These setup an inflammatory response, which may
Poor Circulation and Oxygenation increase the length of the inflammatory phase. Presence
It has been shown in numerous clinical studies that typical of foreign body also hampers the process of response of
wound partial pressures of oxygen are markedly reduced and cellular events, mesh formation and collagen integrity
may be the rate limiting process in wound repair. Oxygen subsequently leading to delayed healing.
is essential for maintaining cellular integrity, function, Wound Temperature
and repair when tissues are injured. Oxygen not only
plays an important role in energy metabolism, but also This will inevitably slow down the healing process. The
is very important in polymorphonuclear cell function, optimum temperature for cellular activity and division is
neovascularization, fibroblast proliferation, and collagen 37°C and with a drop of 1°C it will take up to three hours for
deposition. Though in cases of acute hypoxia, healing mitotic cell division to restart. Frequent dressing changes,
will occur as long as other factors such as nutrients, blood application of cold solution and leaving the wound exposed
flow, and immune function remain adequate, to allow can decrease the local temperature.
Table 6.1 Local and systemic factor affecting the wound healing
Local factors Systemic factors Social factors
• Location of wound • Nutritional status • Poverty
• Poor circulation and oxygenation • Age and gender • Lifestyle
• Dressings and local infection • Smoking and alcohol drinking • Housing
• Foreign bodies • Drugs • Cultural beliefs
• Wound temperature • Vascular and oxygen supply
• Saliva (in case of oral wounds) • Surgical techniques
• Mechanical stress • Stress
• Desiccation or dryness of wound • Obesity
• Infection
• Diseases
• Sex hormones
• Immunocompromised conditions,
cancer, radiation therapy, AIDS
Saliva (in Case of Oral Wounds) Desiccation or Dryness of Wound

Besides prevention of wound infections through the above Dry wounds do not heal well due to the formation of eschar
70 antimicrobial effects, saliva plays other roles in the healing which slows down the migration of cells in the epidermis.
of oral wounds as well. Salivary EGF speeds up the healing This is because migrating cells are forced deep into
process by its angiogenetic and cell proliferating effects. the dermis beneath the scab and healthy tissue becomes
EGF promotes re-epithelialization of oral mucosa. devitalized.
In addition to EGF in saliva, many other growth factors In turn healing is delayed due to the enlargement of
including insulin-like growth factor (IGF), transforming the wound. This is more in case of skin/cutaneous wound.
growth factor (TGF), platelet-derived growth factor Therefore the patient must not become dehydrated.
(PDGF), fibroblast growth factor (FGF), insulin like
growth factors and nerve growth factor (NGF) produced at Systemic Factors
the wound also contribute to the healing process. Nutritional Status
Insulin-like growth factor (IGF-1), an isoform of IGF, Wound healing requires an adequate supply of macro and
stimulates chemotaxis of endothelial cells and proliferation micronutrients. Deficiencies can result in poor wound
of keratinocytes and fibroblasts, which promote re healing, reduced tensile strength, wound dehiscence, and
epithelialization and extension of wound. increased vulnerability to infection and poor quality scars.
Transforming growth factor -beta1, an isoform of TGF-
beta, promotes chemotaxis of monocytes, macrophages, Age
neutrophils, lymphocytes, keratinocytes and fibroblasts, In advancing age, many processes slow down. The
and production of growth factors from those cells. inflammatory response is reduced; therefore the risk of
These accelerate vascularization, deposition of extra infection is increased. Collagen metabolism is reduced
cellular matrices and inhibition of degradation of extra with the resulting scar being more fragile and there is less
cellular matrices. support for blood vessels thus making them more prone
Furthermore, saliva contains several blood clotting to damage. It is more likely that other medical problem,
factors (IXa, VIII, XI) at a level comparable to plasma, and which are common in the elderly, slow down the healing
saliva can replace platelets in the thrombin generation. process more than age itself.
This property of saliva is highly important in the oral
wound healing because, although saliva dilutes blood Smoking and Alcohol Drinking
clotting factors of blood origin, bloodclotting can be Smoking and alcohol abuse which leads to damage of
initiated. the Liver and digestive system which can indirectly
A relatively high amount of salivary kallikrein is lead to delayed wound healing. Smoking can also affect
suggested to play a role in vasodilatation around mucosal epithelialization rates and cause problems with scarring.
injuries to facilitate healing and defense of the injured area. The pathophysiological effects are multidimensional,
Hence, any condition which affect the salivary secretion including arteriolar vasoconstriction, cellular hypoxia,
any delay the wound healing. demineralisation of bone, and delayed revascularization.
Mechanical Stress Drugs
Mechanical stress can disturb the growth of granulation There are a variety of drugs that can impair the healing
tissue which is an essential part of wound healing process. Medication can have a delaying effect on healing
particularly in the process of healing by secondary as well as an accelerating effect depending on the nature
intension. This is because mechanical stress interrupts the of the drug. Steroids are used as anti-inflammatory
proliferation of new capillary growth. and immunosuppressant which will reduce the body’s
These new capillaries carry the oxygen that is needed response to damage, delaying the healing process. For
for epithelialization and without the capillaries the wound example, Corticosteroids and nonsteroidal inflammatory
will be unable to heal up due to the hypoxic conditions. drugs reduce the normal inflammatory response and by
Healing of Wound
suppressing the synthesis of fibroblasts and collagen and Infection

slowing down epithelialization.
Healing is delayed as bacteria compete with macrophages
Antibiotics can enhance wound healing as they kill
and fibroblasts for oxygen within the wound. There can 71
infective agents allowing healing to proceed. For example
be further tissue damage from this inflammatory response
Aspirin and anticoagulants may cause excessive bleeding
and abscesses may be formed. As the wound returns to the
with the potential of a hematoma if not given in the correct
inflammatory phase, healing is slowed down.
dosage. Immunosuppressive drugs reduce leukocyte activity
which reduces the inflammatory response and increases Diseases
the risk of infection. Cytotoxic drugs interfere with cell Various diseases have an influence on the healing process
proliferation including cells needed for wound healing. such as:
∙ Diabetes: There is a highrisk of infection in diabetic
Vascular and Oxygen Supply
patients. High blood glucose levels will encourage
A good blood supply is needed for wounds to heal. Taking invading microorganisms to multiply and hyperglycemia
excessive quantities of caffeine (coffee, cola drinks, or has a damaging effect on phagocytosis. Both of these
chocolate) or a high nicotine intake (smoking) can lead to will increase the risk of infection.
vasoconstriction and lead to reduced tissue perfusion of the ∙ Malignancy: Patients may have chemotherapy or radio
wound area. therapy. Radiotherapy can produce local skin damage
Shock, hypoxia, diseases such as anemia and chronic and slows down healing. It has a fibrosing effect on
obstructive airways disease, or an impaired arterial blood local blood vessels as well as reducing the amount of
supply may cause a reduced supply of oxygen getting to fibroblasts and endothelial cells (Cutting, 1994).
the wound. ∙ Respiratory disease (e.g. chronic obstructive airways
Although angiogenesis is stimulated by hypoxia, an disease): The amount of oxygen to the wound bed is
adequate oxygen supply is required by the wound. Without diminished so causing a hypoxic state.
it collagen synthesis and epithelialization are impaired.
Social Factors
Surgical Techniques
Poverty
If tissue is handled roughly during surgery, it may become
The black report found that people who were living in
devitalized and provide a focus for infection. A hematoma
poverty were more likely to become ill than those that were
may form if hemostasis is not achieved which can cause
fairly affluent. Poverty can lead to a poor nutritional intake,
tissue damage by exerting pressure at the wound edges
which is important for wound healing. It can also affect the
and is also a perfect environment for bacteria to grow.
patient’s ability to afford to have sufficient heating during
If sutures or staples are put in too tightly then tissue will
cold weather, so causing peripheral vasoconstriction and a
become damaged with a poor cosmetic result.
decreased blood supply to the wound.
Stress Lifestyle and Habits
Stress can be caused by a variety of things such as hypoxia, The lifestyle of the patient can influence wound healing,
hypothermia, pain, and psychological issues. Irrespective especially if the person smokes, drinks excessive amounts
of the cause, the overall effect is the stimulation of the of alcohol or abuses drugs.
sympathetic nervous system which leads to increase in
levels of noradrenaline causing vasoconstriction and a Housekeeping
diminished perfusion to the wound. Glucocorticoid has Poor housekeeping can mean a lack of cleanliness, thereby
inhibitory effect on fibroblast activity, collagen synthesis increasing the risk of wound infection.
and granulation tissue formation which results in delay in
wound healing. Cultural Beliefs
These may have an influence on diet, e.g. fasting or which
Obesity dressings can be used as some contain porcine or bovine
Wound dehiscence and wound infection is increased in an within them. Some patients may also have objections to
obese person due to a decrease in perfusion to the wound. being ‘cared for’ by members of the opposite sex.
Table 6.2 General and cellular events in wound healing

General events Factors or cellular events
72 • Rapid hemostasis • Coordinated cell activation
• Inflammation • Cell division
• Mesenchymal cell differentiation, proliferation, • Chemotaxis
and migration to the wound site • Migration
• Appropriate angiogenesis • Differentiation of many cell types
• Re-epithelialization
• Proper synthesis and desired alteration of collagen
to provide strength to the healing tissue
CASCADE OF WOUND HEALING vasoconstrictive substances to aid in this process but their
prime role is to form a stable clot sealing the damaged
Wound healing can be described as a complex and vessel.
dynamic cascade of events initiated by injury. The initial The injured blood vessel vasoconstricts, and the
or primitive response to injury is essential occurs in phases endothelium and nearby platelets activate the intrinsic part
and can be called as an innate host immune response of the coagulation cascade. Under the influence of ADP
for the restoration of tissue integrity. The events of each (adenosine diphosphate) leaking from damaged tissues the
phase should happen in a precise and regulated manner. platelets aggregate and adhere to the exposed collagen. They
Delayed wound healing occurs if there are interruptions, also secrete factors which interact with and stimulate the
aberrancies, or prolongation in the process. These phases intrinsic clotting cascade through the production of thrombin,
and their biophysiological functions must occur in the which in turn initiates the formation of fibrin from fibrinogen.
proper sequence, at a specific time, and continue for a The fibrin mesh strengthens the platelet aggregate into
specific duration at an optimal intensity (Table 6.2). a stable hemostatic plug. The clot that forms is made of
All the cellular events are mediated by locally released collagen, platelets, thrombin, and fibronectin, and these
growth factors and cytokines, which may act in an autocrine factors release cytokines and growth factors that initiate
or paracrine manner. Research work on acute wounds in an the inflammatory response. Finally platelets also secrete
animal model shows that wounds heal in four phases. It cytokines such as platelet-derived growth factor (PDGF),
is believed that chronic wounds must also go through the which is recognized as one of the first factors secreted in
same basic phases. initiating subsequent steps. The fibrin clot also serves as a
scaffold for invading cells, such as neutrophils, monocytes,
Phases of Wound Healing
fibroblasts and endothelial cells. The clot also serves to
• Hemostasis
concentrate the elaborated cytokines and growth factors in
• Inflammation
wound healing.
• Proliferation or granulation
Hemostasis occurs within minutes of the initial injury
• Remodeling or maturation.
unless there are underlying clotting disorders such as
deficiency of Factor XIII (the fibrinstabilizing factor)
Hemostasis is associated with impaired wound healing secondary to
The first action the body takes immediately after wounding decreased chemotaxis or decreased adhesion of cells in the
is to control bleeding as damaged blood vessels must inflammatory area.
be sealed. Hemostasis serves as the initiating step and
foundation for the healing process. Inflammation results in Hemostasis → Blood vessels constrict → Platelets →
vasodilatation and increased vascular permeability. Secrete vasoconstrictive substance → ADP influence
The blood vessels themselves constrict in response to aggregation of platelets → Intrinsic cascade → Thrombin
injury but this spasm ultimately relaxes. The platelet is secretion → Fibrinogen stimulates → Fibrin formation
the cell which acts as the important utility worker sealing → Fibrin mesh entangles platelets to form hemostatic
off the damaged blood vessels. The platelets secrete plug.
Healing of Wound
Inflammation Epithelial cells located on the skin edge begin

proliferating and sending out projections to reestablish a
Clinically inflammation is the second stage of wound
protective barrier against fluid losses and further bacterial
healing add marked as erythema, swelling and warmth 73
invasion.
often associated with pain. This stage usually lasts up to 4
The stimulus for epithelial proliferation and chemotaxis
days post injury.
is EGF and TGF-beta produced by activated platelets and
In wound healing, it is important to clean up the debris.
macrophages (fibroblasts do not appear to synthesize TGF-
Neutrophils arrive via the blood and migrate into the
beta). Epithelialization begins shortly after wounding and is
tissue spaces of the wound within the first 24 hours. The
first stimulated by inflammatory cytokines (IL-1 and TNF-
neutrophils phagocytize debris and microorganisms and
beta) up regulate KGF gene expression in fibroblasts).
provide the first line of defense against infection. They are
In turn, fibroblasts synthesize and secrete keratinocyte
aided by local mast cells.
growth factor (KGF)-1, KGF-2, and IL-6, which simulate
As fibrin is broken down as part of this clean-up
neighbouring keratinocytes to migrate in the wound
the degradation products attract the next cell involved.
area, proliferate, and differentiate in the epidermis. Also
Monocytes also migrate into the wound after about 24
there is marked increase in new blood vessel formation
hours. Once in the tissues, these cells also phagocytose
called angiogenesis. This serves purpose of providing the
bacteria and dead tissue, this causes them to grow and they
nutrition to the cells which help in wound healing.
become large cells called macrophages.
Fibroblasts and endothelial cells are the predominant
Macrophages provide a second line of defense. They
cells proliferating during this phase. Endothelial cells located
also secrete a variety of chemotactic and growth factors
at intact small capillaries are attracted by VEGF (secreted
such as fibroblast growth factor (FGF), epidermal growth
predominantly by keratinocytes on the wound edge, but also
factor (EGF), transforming growth factor beta (TGF-beta)
by macrophages, fibroblasts, platelets, and other endothelial
and interleukin-1 (IL-1) there by coordinating much of the
cells) to begin forming new capillary tubes.
healing process.
Fibroblasts travel into the wound site from the
An activated macrophage is important for the transition
surrounding tissue, become activated and commence
into the proliferative phase. These locally acting chemicals
producing collagen, and proliferate. PDGF and EGF are the
stimulate the regrowth of epithelium, new capillaries and
main signals to fibroblasts and are derived from platelets
the migration of fibroblasts.
and macrophages. PDGF expression by fibroblasts is
At least 20 different growth factors are involved in
amplified by autocrine and paracrine signaling.
normal wound healing. In the absence of monocytes, there
Fibroblasts already located in the wound site (termed
are no growth factors to stimulate mitosis in adjacent
“wound fibroblasts”) begin synthesizing collagen and
healthy tissues. This means that regeneration of damaged
transform into myofibroblasts for wound contraction
tissues cannot occur.
(induced by macrophage- secreted TGF-beta 1).
Proliferation or Granulation
To replenish the damage part is prime importance for
Remodeling or Maturation
successful healing of wound. This is achieved by the The main feature of this phase is the deposition of collagen
cell induction, proliferation and migration. This leads to in an organized and wellmannered network.
granulation tissue formation as well as epithelialization. Once the basic structure of the house is completed
The granulation stage starts approximately four days after interior finishing may begin. So too in wound repair the
wounding and usually lasts until day 21 in acute wounds healing process involves remodeling the dermal tissues to
depending on the size of the wound. produce greater tensile strength. The principle cell involved
It is characterized clinically by the presence of pebbled in this process is the fibroblast.
red tissue in the wound base and involves replacement of Early in wound healing, the matrix is thin and allows
dermal tissues and sometimes subdermal tissues in deeper fibroblasts, neutrophils, lymphocytes, and macrophages to
wounds as well as contraction of the wound. easily maneuver through it.
Epithelialization begins shortly after wounding and is Initially, the matrix is composed mainly of fibrin and
first stimulated by inflammatory cytokines (IL-1 and TNF- fibronectin (arising from the efforts for hemostasis and by
alpha up regulate KGF gene expression in fibroblasts). macrophages).
Glycosaminoglycans, proteo-glycans, and other proteins When the edges of the wound are brought together into
(such as secreted protein acidic rich in cysteine, or SPARC) contact and held in place by sutures, the blood clots, and
are synthesized next by the fibroblasts. As the matrix in a matter of hours, numerous leukocytes are mobilized in
74 becomes denser with thicker, stronger collagen fibrils, it the area.
becomes stiff and less resistant. The fibroblasts are capable Connective tissue cells in immediate vicinity undergo
of “adaptive response” to the changing mechanical loading transformation into fibroblasts which in turn undergo
on the matrix as it matures. mitotic division. New fibroblasts begin to migrate into and
The remodeling of the accommodating matrix depends across the line of incision. These cells form thin, delicate
on the cell migration throughout the matrix and proteolysis collagen fibrils which intertwine and coalesce in a general
of the matrix proteins. direction parallel to the surface of the wound.
Endothelial cells of capillaries begin to proliferate and
Keloid: In case of defect in extracellular matrix formation
small capillary buds grow out and across the wound. These
(from diet or disease), then the wound’s strength is greatly
buds eventually form new capillaries which fill with blood
compromised. In contrast, presence of excessive collagen
and a rich network of young capillaries and capillary loops
synthesis, a hypertrophic scar or keloid can result.
are formed.
The final phase of wound healing involves wound
contraction and the remodeling of the ECM produced by Secondary Healing
fibroblasts during the proliferative phase. The fibronectin
produced in the formation of granulation tissue diminishes It occurs when there is a loss of tissue and the edges of
over time as the matrix is remodelled. This process involves the wound cannot be approximated like in the palate or on
the induction of MMPs 13, enzymes which are each the alveolar mucosa. The material which fills the defect in
involved in the catabolism of different ECM components. secondary healing is called granulation tissue.
These enzymes are controlled by natural tissue After the removal of the lesion, blood clots and the
inhibitors of matrix metalloproteinase (TIMPS), and the repair process begins. It is basically identical with the
balance between MMP and TIMP expression is crucial for healing by primary intention except that the fibroblasts
normal matrix remodeling. Remodeling can take up to 2 and capillaries have a greater distance to migrate, more
years after wounding. granulation tissue must form and healing is slower.
During wound contraction phenotypic changes to the Cellular proliferation begins around the periphery
fibroblasts populating the wound occur, with a switch for of the wound and fibroblasts and endothelial cells
a profibrotic myofibroblasts phenotype, characterized by grow into the clot along the fibrin strands. In addition,
an increase in expression of asmooth muscle actin. The polymorphonuclear leukocytes, mononuclear phagocytes
attachment of these cells to each other and the surrounding and later the lymphocytes migrate into the granulation
matrix then facilitates wound contraction, with the tissue from the adjacent vessels and tissues.
myofibroblasts contracting pseudopodia attached to the Large numbers of leukocytes also accumulate on the
ECM. This remodeling of the ECM in the skin leads to surface of the wound. As granulation tissue matures, it
scar formation. However, some marked differences in becomes more fibrous through condensation of collagen
the oral mucosa wound healing may be due to intrinsic bundles and the surface of the lesion becomes epithelialized.
characteristics of the tissue. Lesions become somewhat avascular.
HEALING OF BIOPSY WOUNDS HEALING OF EXTRACTION WOUNDS

Biopsy: It is the removal of tissue for the purpose of The healing of extraction wound does not differ from the
microscopic examination and diagnosis. healing of other wounds of the body except as it is modified
by the peculiar anatomic situation which exists after the
Primary Healing removal of tooth.
Healing by primary intention or healing by first intention
is that type of healing which occurs after excision of a Immediate Reaction Following an Extraction
piece of tissue with close apposition of the edges of the After the removal of a tooth, blood which fills the socket
wound. coagulates, red blood cells get entrapped in the fibrin
Healing of Wound
meshwork and the ends of blood vessels in the periodontal Third Week Wound
ligament are sealed off.
Original clot appears almost completely organized by
Hours after the tooth extraction, if blood clot is
maturation of granulation tissue. Very young trabeculae of 75
dislodged, healing may be greatly affected and may be
osteoid or uncalcified bone are forming around the entire
extremely painful. However, if the healing is normal, within
periphery of the wound from the socket wall.
24 to 48 hours, there is vasodilation and engorgement of
Early bone is formed by osteoblasts derived from
blood vessels in the remnants of periodontal ligament and
pluripotential cells of the original periodontal ligament
there is mobilization of leukocytes to the immediate area
which assume osteogenic function.
around the clot.
Original cortical bone of alveolar socket undergoes
Surface of the blood clot is covered by a thick layer
remodeling so that it no longer consists of such a dense
of fibrin. It is important to recognize that the collapse
layer. Crests of alveolar bone have been rounded off by
of unsupported gingival tissue into the opening of fresh
osteoclastic resorption. By this time, surface of the wound
extraction wound is of great aid in maintaining the clot in
may have become completely epithelialized.
position.
Fourth Week Wound
First Week Wound
There is continuous deposition and remodeling, resorption
Proliferation of fibroblasts from connective tissue into
of the bone filling the alveolar socket.
the remnants of periodontal ligament is evident and these
Due to this, crest of the alveolar bone undergoes
fibroblasts begin to grow into the clot around the periphery.
considerable amount of osteoclastic resorption during the
The clot is gradually replaced by granulation tissue.
healing process and because of this, bone filling the socket
Epithelium at the periphery of the wound exhibits evidence
does not extend beyond alveolar bone crest. It is obvious
of proliferation seen in the form of mild mitotic activity.
that crest of the healing socket does not extend above the
Crest of the alveolar bone, which marks up the margin
alveolar crest.
or neck of the socket exhibits beginning of osteoclastic
activity. Endothelial proliferation signals the beginning Radiographic Changes in Healing Sockets
of capillary growth. During this period, blood clot begins
to undergo organization by ingrowth of fibroblasts A tooth having been removed and after variable and
and occasionally by small capillaries from the residual unspecified length of time, there is gradual loss of density
periodontal ligament. of the lamina dura and at the same time bone develops at
An extremely thick layer of leukocytes gathers over the the base and sides of the socket. By the time that the socket
surface of the clot and the edges of the wound continue to is filled with bone, all traces of lamina dura is gone.
exhibit epithelial proliferation. Later the new bone consolidates and comes to
resemble the adjacent bone. Most healed socket reveal
Second Week Wound slight or marked cortical bone formation at the surface of
the alveolar process. Following the removal of teeth, the
During the second week, after extraction of the tooth, the
alveolar margins undergo some resorption. The surface
blood clot is becoming organized by fibroblasts growing
usually becomes flat or slightly curved but smooth.
into the clot and forming fibrin meshwork.
At this stage, new delicate capillaries have penetrated
to the center of the clot. Remnants of periodontal ligament HEALING OF FRACTURES
have been gradually undergoing degeneration. Walls of
the bony socket appear slightly frayed. In some cases, Immediate Effect of a Fracture
trabeculae of osteoid can be seen extending outward from After fracture, Haversian vessels of the bone are torn at
the walls of the alveolus. the fracture site so are the vessels of periosteum and
Epithelial proliferation over the surface of the wound marrow cavity that happen to cross the fracture line. Due
is extensive. Margins of the alveolar socket exhibits to disruption of vessels, there is considerable extravasation
prominent osteoclastic resorption and fragments of necrotic of blood in that area, but at the same time, there is loss of
bone are seen in the process of resorption or sequestration. circulation and lack of local blood supply. The bone cells
or osteocytes of Haversian system die due to tearing of HEALING OF OSSEOINTEGRATED

vessels at the fractured site.
Concomitant with the disruption of blood supply and the
IMPLANTS
76 tearing of the blood vessels, there is death of bone marrow Implants are of three types, i.e. endodontic, endoosseous
adjacent to the fracture line. The blood clot formation, play and subperiosteal.
an important role in healing of the fracture, which later on Osseointegration is the term used for healing of bone
is replaced by granulation tissue followed by its subsequent around an endoosseous implant. This results in an intimate
replacement by bone. interface between the bone and the implant. The healing
process in the initial phases is same as of the healing of
Callus Formation the extraction wound. Osteogenesis and remodeling are the
prime features seen during the process.
It is a structure which unites the fractured ends of bone and
For successful osseointegration, it is required that the
is composed of varying amounts of fibrous tissue, cartilage
implant should be totally free of any small amount of load
and bone.
or movement.
There are two types of callus:
Healing process consist initially of deposition of the
1. External callus: It consists of new tissue which forms
granulation tissue and woven bone. Complete healing
around the outside of the two fragments of bone.
requires a period of several months together. The process
2. Internal callus: It consists of new tissue arising from
is completed with the deposition of cancellous or compact
the marrow cavity.
bone around the implant. Currently the implants used for
Periosteum is an important structure in callus formation
support the restorative or rehabilitative oral procedures are
and ultimate healing of fracture. Cells of periosteum
biocompatible.
torn at the fracture line usually die, but peripheral to the
Biocompatible implants serve as a normal interface
area is found a flurry of cellular activity within hours of
of the connective tissue and the implant as is with the
injury. Outer or fibrous layer of periosteum is relatively
dentogingival junction. Periimplant disease is the term
inert and actually lifted away from the surface of bone
used for the pathologic changes seen around the implant.
by proliferation of cells in osteogenic or inner layer of
It involves periimplant mucositis periimplantitis. The
periosteum which assumes features of osteoblasts which in
inflammation is seemed more at the coronal aspect; the
turn begins the formation of small amount of new bone at
apical osseointegration is maintained under favorable
some distances from the fractured site. There is continuous
conditions.
proliferation of these osteogenic cells forming a collar of
callus around or over the surface of fracture.
New bone which begins to form in the external callus
HEALING OF PULP
usually consists of irregular trabeculae laid down at right Dental pulp regeneration is difficult task as it is enclosed in
angles to the surface. This differentiation of cells into dentin without the collateral blood supply except through
osteoblasts and subsequent formation of bone occurs in the apical foramen. With the advent of the concept of tissue
the deepest part of the callus collar. In the rapidly growing engineering and discovery of stem cells regeneration of
area of collar, varying number cells of the osteogenic layer dental pulp have been a long quest. Moony and Rutherford
differentiate into chondroblasts rather than osteoblasts and were the pioneers in the regeneration of the pulp.
actually form cartilage. The regeneration can be cell based pulp/dentin tissue
This cartilage fuses with bone and then begins to regeneration or noncellbased regeneration. Dental pulp
calcify by endochondroal bone formation. The calcified stem cells (DPSCs), Stem cells from human exfoliated
cartilage is gradually resorbed and replaced by bone. deciduous teeth (SHED), Stem cells for apical papilla
Internal callus forms from endosteum of Haversian canals (SCAP) are potentially suitable cell source for dentin –
and undifferentiated cells of bone marrow. Shortly after pulp regeneration.
the fracture, endosteum begins to proliferate within a week These stem cells can differentiate into specific tissue
to form new bone, which gradually unite and establish the cells and can be used further for the other tissue defects
continuity of bone. After this, both external and internal after in vitro culture and characterization. Healing in pulp
callus remodel to form indistinguishable bone. consists of initial response in form of acute pulpitis or pulpal
Healing of Wound
inflammation consisting of vasodilatation and capillary dentin. The dentin has less and irregularly arranged tubules.
proliferation. This response is reversible or irreversible There can be a demarcation in the normal dentin. Also the
based on the nature of the stimulus. (Refer chapter Pulp continuity is lost between the normal and tertiary dentin.
and periapical diseases). The degree of inflammation, This reparative dentin is formed by odontoblasts and sub 77
the time of irritation and infection, and the location of odontoblasts.
the exposure must be regarded as decisive factors for
the healing of the inflamed pulp. The origins of newly Types of Tertiary/Reparative Dentin
differentiated odontoblasts and ability of precursor cells in • Reactionary dentin:
the pulp to differentiate, particularly under the influence – Formed by damaged existing odontoblasts
of bone morphogenic protein, a cytokine responsible for • Reparative dentin:
the differentiation of osteoblasts is main factor which will – Formed by odontoblast-like cells
determine the healing or repair of pulp. In majority of case, • Sclerotic dentin:
the pulp is not uniformly affected by deep carious lesions. – Transparent dentin
After a week new collagen is formed against the – Due to advancing caries and attrition
necrotic zone and the beginnings of a calcifying front. – Octocalcium phosphate crystals and some
Odontoblastlike cells orientate against the calcifying front derived from saliva.
and develop cellular extension around which bonelike
tissue is deposited after 4 weeks. After 12 weeks, a hard
tissue barrier with tubules is formed.
ENAMEL
Although it was said earlier that repair or regeneration of
CEMENTUM enamel is not possible as the cell responsible for enamel
formation during the tooth development, ameloblasts
Cementum is considered to be avascular dental tissue. Then are not present lifelong. They are lost once the complete
too it has the capacity to repair to a limited extent by the enamel formation occurs. But, nowadays, various studies
formation of cellular intrinsic fiber cementum. Cementum regarding the regeneration of enamel are being carried
resorption may be by local or systemic factors. It is not out. There can be remineralization of the subsurface
considered to be a continuous process. There is alteration of the enamel depending on the supply of calcium and
of the resorption and the repair process. Cementum repair phosphate ions from saliva. The use of fluoride is also
requires presence of viable connective tissue. Cementum done in remineralization of enamel. The enamel treated
repair can occur in both vital and nonvital teeth. The cells with fluoride becomes more resistant than the normal to
for cemental repair are gained from the undifferentiated further demineralization. Studies have been going on the
mesenchymal cells present in the periodontal ligament. use of nano hydroxyapatite crystals (20–40 nm) for repair
The recruitment of the cementoblasts occurs after the of enamel.
stimulation by the various growth factors like the BMP2,
BMP-3, PDGF, IGF-1, TGF-beta, etc. The proteins like the SKIN HEALING AND ORAL MUCOSAL
bone sialoprotein (BSP), osteopontin (OPN), etc. are also WOUND HEALING
involved in the differentiation of cementoblast progenitor
cells to cementoblast. Repair and the regeneration of Although cutaneous and mucosal wound healing proceed
cementum is important as the principal fibers of the through the same stages of hemostasis, inflammation,
periodontal ligament are embedded into the cementum at proliferation, and remodeling, mucosal wounds demonst
one end. This prevents the tooth from being extruded and rate accelerated healing compared to cutaneous wounds.
thus increases its stay in the oral cavity. Mucosal wounds also generally heal with minimal scar
formation, and hypertrophic scars are rare in the oral cavity.
DENTIN Healing Response in Skin
Reparative dentin is the tertiary dentin which is the dentin The damaged epidermis is regenerated by two mechanisms:
formed due to caries, attrition, cavity preparation and 1. Involves the activation of epidermal keratinocytes in
microleakage. This can be in the form of tubular or atubular the wound margin.
2. Involves the proliferation, simultaneously with this Notably, the mucosal epithelium differs from skin
keratinocyte activation, of hair follicle cells, their in that it lacks a stratum corneum and does not need to
migration into the skin, and their transformation to serve functionally as a barrier to water loss. Furthermore,
78 epidermal keratinocytes. mucosal healing occurs in a fully hydrated environment. In
Phospholipids of the cellular membrane are essential for the skin, migrating keratinocytes at the wound edge express
both the cellular function and morphological maintenance. high levels of VEGF, suggesting that keratinocyte derived
Fatty acids, the major component of these phospholipids, VEGF stimulates angiogenesis during wound healing.
are key factors in the regulation of cell proliferation and Keratinocytes are also capable of modulating fibroblast
differentiation. Among the fatty acids known to constitute behavior, including collagen synthesis, through the
the cell membrane, palmitic acid (16:0) is a basic unit in production and release of soluble factors. Keratinocytes
the membranes of all human cells. Once the epidermis is from skin and oral mucosa respond differently to equivalent
damaged, then rapid cell proliferation becomes necessary, IL1b stimulation. Keratinocytes from skin and mucosa
and the abundant palmitic acid (16:0) stored in hair follicle maintain differential regulatory pathways that lead to
cells is used for wound epithelialization, i.e. wound healing differential responsiveness at sites of injury.
in a dynamic environment. This fundamental difference in intrinsic genetic
response to wounding between skin and mucosa,
Healing Response in Oral Mucosa which makes mucosal wounds heal faster and with less
inflammation and scar formation.
The oral mucosa shows earlier wound healing than does
Excessive scarring such as hypertrophic scars and
the skin, because “early wound healing” of the oral mucosa
keloid scars have not been reported in the oral mucosa.
has been studied in terms of the “moisture environment in
Scalds to the oral mucosa do not result in contractures
the oral cavity” but not from other aspects.
unlike scalding to the skin.
The oral mucosal epithelium (both the basal and
It was hypothesized that secretory leukocyte protease
suprabasal layers) demonstrated a significantly higher
inhibitor (SLPI; a cationic serine protease inhibitor with
percent composition of palmitic acid (16:0) than did the
antimicrobial and anti-inflammatory properties) found in
epidermis, but with no difference in its distribution between
large quantities in the saliva may have a role in mediating
the two layers. There is a much higher energy metabolism
scarless healing in the oral mucosa.
in the oral mucosa than in the skin.
Various reasons have been suggested for minimal
The skin is significantly more dependent on essential
scarring in the oral cavity, including distinct fibroblast
fatty acids than the oral mucosa, thus suggesting that the
phenotype, the presence of bacteria that stimulate wound
skin is more susceptible than the oral mucosa to wound
healing and the moist environment and growth factors
healing failure attributed to malnutrition.
present in saliva.
Lower neutrophil, macrophage, and Tcell infiltrations
Oral mucosal wounds also demonstrate a more highly
were consistently observed in the intraoral injury site. One
regulated angiogenic response and have a differential
possibility is that within the oral cavity, saliva provides
expression of key profibrotic growth factors compared
many necessary growth factors, making macrophage
with skin, resulting in less scarring than in skin wounds.
function less critical.
Great differences have been observed in wound healing
Saliva containing abundant amounts of cytokines, growth between the oral mucosa and the skin. Oral mucosa wound
factors, and protease inhibitors—is the primary factor that repair is characterized by rapid reepithelialization as well
accounts for rapid oral wound healing. At sites of injury, as enhanced wound repopulation and matrix reorganization
the epithelium is a rich source of both proinflammatory in vitro.
mediators, such as IL-1, and TNF-a, as well as growth Oral mucosal wounds were shown to contain
factors, such as vascular endothelial growth factor (VEGF). significantly lower levels of macrophages, neutrophils and
The rapid healing and the absence of scars in oral Tcells when compared with dermal wounds
mucosa are most directly related to intrinsic characteristics Research had demonstrated that oral fibroblasts are
of the tissue and not to environmental factors such as phenotypically distinct, and are capable of achieving a
temperature, salivary flow, the absence of a hemostatic higher number of cumulative population doublings in vitro
plug, or microflora. when compared to patient matched skin fibroblasts.
Healing of Wound
Table 6.3 Systemic and local aspects

Systemic aspects Local aspects
• Optimize nutritional status • Optimize wound environment by 79
Consult with dietician Thorough wound debridement
- Ensure adequate protein and caloric intake, trace - Eliminate foreign bodies, dead space and necrotic
metals, vitamins tissue
• Examine psychosocial status - Elimination of infection systemic/topical
Depression inhibits compliance and wound healing antimicrobials Ph active agents
Pain control is extremely important Decreased edema
• Optimize biochemical status Offload pressure
- Acid base balance Provide moist wound environment
- Endocrinologic status, blood sugars, hypo-
thyroidism
Correct renal failure, liver failure
• Optimize perfusion and oxygenation
Cardiac status
Pulmonary status
- Anemia
- Vascular bypass surgery if required
• Correct spasticity and other physical factors
contributing to abnormal mobility
• Examine and reduce all medications
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Healing of Wound
1. Salivary EGF speeds up healing process by: 8. Radiographic changes of a healing socket involves: 81
a. Angiogenetic effect a. Loss of lamina dura
b. Cell proliferation b. Sharper image of lamina dura
c. Reepithelialization c. Both a and b
d. All of the above d. None of the above
2. Following are the phases of wound healing except: 9. Primary factor that accounts for rapid oral wound
a. Hemostasis b. Inflammation healing:
c. Loss of function d. Granulation a. Saliva b. Blood
3. Deficiency of following factor is associated with c. Fibrin d. All of the above
impaired wound healing: 10. Factor which delay healing is:
a. Factor II b. Factor IV a. Proteins b. Anemia
c. Factor XIII d. Factor XII c. Low radiation dose d. Vitamins
4. Initially, during maturation the matrix is composed of: 11. Hormones which delay wound healing is:
a. Fibrin b. Fibronectin a. Growth hormone b. Thyroid hormones
c. Both a and b d. None of the above c. ACTH d. None
5. After wounding, remodeling can take up to: 12. In healing of extraction of wounds, osteoid or
a. 5 years b. 2 years uncalcified bones are form during:
c. 6 months d. 8 weeks a. 1st week b. 2nd week
6. The material which fills the defect in secondary healing c. 3rd week d. 4th week
is called as: 13. In healing of extraction of wounds, complete surface
a. Scar tissue b. Eschar epithelialization occurs during:
c. Granulation tissue d. Fibronectin a. 1st week b. 2nd week
7. The following, are correct, except: c. 3rd week d. 4th week
a. I week wound–proliferation of fibroblasts 14. Wound contraction occurs in healing wound of skin
b. II week wound–fibrin meshwork due to presence of:
c. III week wound–maturation of granulation tissue a. Actin and myosin b. Fibroblasts
d. IV week wound–maturation of granulation tissue c. Cytokine d. Glycosaminoglycans
Hyperplasia, Hamartoma
and Neoplasm
A Chapter Outline
O Dysplasia O Type of carcinogenesis

O Metaplasia O Chemical carcinogens
O Hyperplasia O Physical carcinogens ( radiation)
O Hamartoma O Hormonal carcinogens
O Choriostoma O Biologic carcinogens ( virus)
O Neoplasm O Metastasis
O Carcinogenesis O Tumor grading
DYSPLASIA in response to abnormal stimuli, and often reverts back to

normal after removal of the stimulus .
This word is originated from ancient Greek word dys -,
difficulty + plasia, formation. HYPERPLASIA
It is a loss in the uniformity of the cells, as well as a (From ancient Greek huper, “ over ” plasis, ‘ [ formation” )
loss in the architectural orientation. Dysplasia is usually
Hyperplasia represents overgrowth of normal mature
referred to change seen in the epithelium , e . g . epithelial
tissues with a limited cellular proliferation or it is an
dysplasia in premalignant and malignant lesions .
increase in the number of parenchymal cells resulting in
enlargement of the organ or tissue . Hyperplasia can be :
METAPLASIA Physiologic hyperplasia: It occurs due to hormonal
(Greek: change in form) changes as in pregnancy.
Metaplasia is defined as a reversible change of one Pathologic hyperplasia: It may occur as inflammatory,
type of mature epithelial or mesenchymal cells, usually non-inflammatory growth .
Hyperplasia, Hamartoma and Neoplasm
Characteristic Features of Hyperplasia

• It does not have autonomous growth.
• It ceases growth at some point like hamartoma. 83
• Clinically hyperplasia may be seen as an overgrowth,
or mass, or tumorous swelling.
• Histologically, it is increased cellularity by virtue of
their proliferation. It makes them resemble neoplastic
proliferations, difference being absence of dysplastic
features.
• Hyperplasias are usually reactive lesions.
• They are initiated by an identifiable stimulus, e.g.
irritation of mucosa—fibrous hyperplasia (Fig. 7.1).
• The process or growth stops when stimulus is
removed.
• Inflammatory lesions may be initiated and controlled Figure 7.1 Localized fibrous hyperplasia due to irritation
by same stimulus repeatedly, e.g. peripheral giant of removable prosthesis
cell granuloma, pulp polyp (chronic hyperplastic
pulpitis).
They are dysmorphic proliferation of tissue often with one
• The process or growth stops but the size of the lesion
element predominating.
does not usually regress on its own.
These tissues do not have inherent capacity for
continuous growth but they nearly parallel the growth
of host. Because of this property the distinction between
HAMARTOMA
hamartoma and benign tumors is often subjective as most
Hamartoma is an overgrowth or abnormal proliferation of benign tumors of infancy and childhood are actually
mature cells and tissues or structures native to that part. developmental hamartoma (Table 7.1).
Table 7.1 Classification of hamartoma
Odontogenic hamartoma Nonodontogenic hamartoma Unknown or doubtful origin

Those involving teeth: Epithelial origin • Granular cell myoblastoma
• Dens invaginatus • Epstein pearls and Bohn’s • Congenital epulis of the newborn
• Dens evaginatus nodules • Melanotic neuroectodermal tumor
• Talon’s cusp • Oral and labial melanotic of infancy
• Those not involving teeth (as these lesions do not macule • Fibromatosis gingivae
have uncoordinated uncontrolled growth they • Pigmented cellular nevus
can be called hamartoma than neoplasm) Vascular origin
• Enameloma • Hemangioma
• Odontoma (complex and compound) • Lymphangioma
• Gigantiform cementoma • Glomus tumor
• Dental lamina cyst of the newborn Osseous origin
• Torus palatinus
• Torus mandibularis
Adipose tissue
• Lipoblastomatosis
Neural tissue
• Neurofibromatosis
Characteristic Features of Hamartoma Grading of Teratoma (Gonzalez-Crussi)

• G rowth of hamartoma ceases with general body • 0 or mature (benign)
84 growth. • 1 or immature: Probably benign
• They do not infiltrate into surrounding tissue. • 2 or immature, possible malignant
• In bone they are treated by enucleation. If they are • 3 or frankly malignant—additional cancer staging
present in soft tissue they are removed by pericapsular applies.
incision.
Cystic teratomas usually are grade 0 and, conversely,
CHORIOSTOMA grade 0 teratomas usually are cystic. Grade 0, 1 and 2 pure
teratomas have the potential to become malignant (grade
Choriostoma are tumor like proliferation of tissue not native 3), and malignant grade 3 pure teratomas have the potential
to the site. Histologically it is normal tissue found at abnormal to metastasize. A teratoma can be pure and not malignant
site. They are similar to hamartoma with basic difference is yet highly aggressive.
that proliferating tissue is not usual or native to the site. Growing teratoma syndrome, is referred when chemo-
Heterotrophic gastrointestinal cyst: It may occur therapy the malignant elements are eliminated of a mixed
in tongue or floor of the mouth of infants and contains tumor, leaving pure teratoma which has potential to grow
gastrointestinal glandular structures. Rarely bone and very rapidly.
cartilage may be found in the tongue, known as osseous Malignant transformation: A teratoma with malignant
choriostoma and cartilagnous choriostoma respectively. transformation (TMT) is a very rare form of teratoma that
Lingual thyroid nodule: Presence of thyroid tissue in the may contain elements of somatic (nongerm cell) malignant
posterior part of tongue. tumors such as carcinoma, sarcoma or leukemia.
Fordyce’s granules: It is ectopic sebaceous glands in the Teratomas of Tongue
oral cavity. Presence of salivary gland within lymph nodes.
Teratoma in a few cases, has been found within the
Teratoma body of the tongue, but occasionally has been attached
to the base of the tongue by a pedicle or to the side of
Teratomas are thought to be present at birth (congenital),
the tongue. They may be so large as to fill the mouth
but small ones are often not discovered until much later
completely.
in life. A teratoma is an encapsulated tumor with tissue
There does not appear to be any sex predilection.
or organ components resembling normal derivatives of
Although teratomas are thought to arise in the
all three germ layers. The tissues of a teratoma, although
developing blastomere, some cells being displaced or
normal in themselves, may be quite different from
separated; it is possible that normal muscle-forming
surrounding tissues and may be highly disparate.
cells migrating from the occipital myotomes have been
Teratomas have been reported to contain hair, teeth,
accompanied by neuroectodermal and other cells. In a
bone and very rarely, more complex organs such as eyes,
few examples, a second teratoma has been found in the
torso, and hands, feet or other limbs.
proximate area.
Classification of Teratoma Histopathology
• S olid teratoma contains only tissue (perhaps
Most often immature neuroectodermal tissue present;
including more complex structure).
however, cartilage, bone, skin, or cystlike structures lined
• Cystic teratoma contains only pockets of fluid or
by stratified squamous epithelium, pseudostratified ciliated
semifluid such as cerebrospinal fluid, sebum or fat.
columnar epithelium, intestinal epithelium, muscle, and
• Mixed teratoma contain both solid and cystic parts.
nerve have been found.
Lab Findings Normal Cell Cycle (Fig. 7.2)

Elevated carcinoembryonic antigen and alpha-fetoprotein All renewing cells go through a series of events known as
levels have been found. In some cases urinary vanillyl- cell cycle. After mitosis (M phase), cells spend a variable 85
mandelic acid and dopamine levels have also been period of resting (G1 phase).
increased.
Etiology of Oral Cancer
Management • Hereditary predisposition
There is excellent prognosis following surgical excision. • Racial and geographic factors
In spite of the rule (regarding ovarian teratomas) that the • Environmental and cultural factors
greater the immaturity and amount of neuroepithelium, the • Age
poorer the prognosis, there is evidence reported that lingual • Sex
teratomas are not malignant. • Acquired preneoplastic conditions
• Tobacco and related products/smoking
NEOPLASM • Diet
• Chronic inflammation
Neoplasm is an abnormal mass of tissue as a result of
• Hormonal factors.
neoplasia. Neoplasia (new growth in Greek) is the abnormal
proliferation of cells. Before the progression to neoplasia,
cells often show an abnormal pattern of growth, such as
Etiology of Oral Cancer
metaplasia or dysplasia. However, they do not always The etiology of oral cancer in man is unknown. However,
progress to neoplasia. several pre-existing conditions/factors have been found
The growth of neoplastic cells exceeds and is not with such frequency in patients with cancer that they may
coordinated with that of the normal tissues around it. The be considered. There are various risk factors associated
growth persists in the same excessive manner even after with oral cancer which are described in Table 7.2.
cessation of the stimuli.
Hereditary Predisposition
Definition The risk of developing cancer in relatives of a known
“It is an abnormal mass of tissue, growth of which exceeds cancer patient is 3 times higher than control population.
and is uncoordinated with that of normal tissue and persists Genetic cancers comprise not greater than 5 percent of all
in the same excessive manner after cessation of the stimuli
which evoked the change”.
Nomenclature
Neoplasms may be benign, pre-malignant (carcinoma in
situ) or malignant (cancer). Benign tumors are designated
by attaching oma to cell of the origin. Tumor from fibrous
tissue is called as fibroma.
Malignant tumors arising from mesenchymal tissues
are known as sarcomas, like osteosarcoma.
Malignant tumors of epithelial origin are called
carcinoma, like adenocarcinoma and squamous cell
carcinoma.
Metastatic cancer occurs when cancerous cells spread
into other surrounding tissues, or enter the circulatory
system and travel to other parts of the body, producing new
tumors. Figure 7.2 Normal cell cycle
Table 7.2 Risk factors of oral cancer

Age
Generally it occurs in older individual past fifth decade of
• Tobacco use
86 • Alcohol use life but there is variation in age groups. For example, acute
• Dietary factors, including insufficient fruit and vegetable leukemia occurs in children, neuroblastoma in infancy.
intake
• Overweight and obesity Sex
• Physical inactivity Certain malignancies are more common in men such as basal
• Chronic infections from Helicobacter pylori, hepatitis B cell carcinoma. Similarly, female are also prone to specific
virus (HBV), hepatitis C virus (HCV) and some types of malignancies such as breast cancer or cervical cancers.
human papilloma virus (HPV)
• Environment and occupational risks including ionizing and Acquired Preneoplastic Conditions
non-ionizing radiation.
These may be inflammatory, hyperplastic conditions
cancers, e.g. retinoblastoma, familial polyposis coli, cancer or may be certain benign tumors. For example, chronic
of breast, etc. atrophic glossitis, leukoplakia of oral cavity, vulva and
penis, cirrhosis of liver, chronic irritation and multiple
Racial and Geographic Factors neurofibromas.
Cancers are largely due to the influence of environment Tobacco and Related Products/Smoking
and geographic differences affecting the whole population
such as climate, water, diet, habit. For example: Tobacco consumption and smoking contribute to cancer
Black Africans commonly have cancers of skin, penis, (cancer, heart disease, and cerebrovascular disease).
cervix, and liver. Tobacco is a cause of cancer of the lung, mouth, pharynx,
Europeans and Americans commonly develop larynx, esophagus, bladder, pancreas, stomach, kidney,
malignancies of lung, breast, and colon. uterine cervix, and myeloid leukemia. Tobacco and smoke
Carcinoma of stomach is five times higher in Japanese contain a wide variety of mutagens and substances that are
than in Americans. carcinogenic to human.
Nasopharyngeal cancer is common in South East
Diet
Asians.
Excessive consumption of alcoholic beverages is
Environmental and Cultural Factors associated with cancers of the breast, oral cavity (primarily
The environment contains number of carcinogens which in smokers), and liver.
produce its effect directly or indirectly.
For example: Hormonal Factors
Cigarette smoking is the etiology of cancer of oral Endogenous reproductive hormones play a large role in
cavity, pharynx, larynx, esophagus, lung, pancreas and cancer, including that of the breast, prostate, ovary, and
urinary bladder. endometrium.
Alcohol causes cancer of esophagus and liver.
Alcohol and tobacco together accelerate the risk of Chronic Inflammation
developing cancer of upper aerodigestive tract. Chronic inflammation results in the release of oxidative
Betel nut chewing causes cancer of cheek and tongue. mutagens from white cells and other sentinel cells of
Industrial and environmental materials are carcino- the immune system, which combat bacteria, parasites,
genic. This includes exposure to substances like arsenic, and viruses by destroying them with potent, mutagenic
asbestos, benzene, and naphthylamine. oxidizing agents. These oxidants protect humans from
Overweight individuals, deficiency of vitamin A, immediate death from infection but they also cause
people consuming foods rich in animal fats and low in fiber oxidative damage to DNA, chronic killing of cells with
content have more risk of developing cancers like colon compensatory cell division, and mutation; thus, they
cancer. contribute to cancer.
CARCINOGENESIS ∙ Polycyclic aromatic hydrocarbons (in tobacco,

smoke, animal foods, industrial oil, and atmospheric
Carcinogenesis or oncogenesis or tumorigenesis means pollutants). Important chemical compounds included
induction of tumors; agents which can induce tumors are are benzanthracene, benzpyrene, methylcholan threne. 87
called carcinogens. They may cause lung cancer, skin cancer, cancer of oral
The Hallmarks of Cancer cavity, and sarcoma.
∙ Aromatic amines and azo dyes: These are β-
The hallmarks of cancer encompass six biological
naphthylamine, benzidine, azo dyes used for coloring
capabilities acquired during the multistep development of
foods, and acetyl aminofluorene. They may cause
human tumors. The hallmarks represent an organizing code
bladder cancer and hepatocellular carcinoma.
for rationalizing the complexities of neoplastic disease.
∙ Naturally occurring products: Aflatoxin, acti-
They include sustaining proliferative signaling, evading
nomycin-D, mitomycin-C, safrole, and betel nut. They
growth suppressors, resisting cell death, enabling replicative
can cause hepatocellular carcinoma.
immortality, inducing angiogenesis, and activating invasion
∙ Miscellaneous: Nitroso compounds, vinyl chloride
and metastasis. Underlying these hallmarks is genome
monomer, asbestos, arsenical compounds, metals like
instability, which generates the genetic diversity that
nickel, lead, chromium, and insecticides, fungicides
expedites their acquisition, and inflammation, which fosters
multiple hallmark functions. can cause gastric carcinoma, hemangiosarcoma of
liver, bronchogenic carcinoma, epidermal hyperplasia,
Types of Carcinogens basal cell carcinoma, and lung cancer.
• Chemical carcinogens
Carcinogenesis
• Physical carcinogens (radiation)
• Hormonal carcinogens Chemical carcinogenesis
• Biologic carcinogens (virus). • Initiators of carcinogenesis
– Direct-acting carcinogens
- Alkylating agents
CHEMICAL CARCINOGENESIS - Acylating agents
Chemical carcinogens are divided into two broad groups: – Indirect-acting carcinogens or procarcinogens
- Polycyclic aromatic hydrocarbons
Initiators of Carcinogenesis - Aromatic amines and azo dyes
Direct-acting carcinogens: These require no metabolic - Naturally occurring products
conversion to become carcinogens. - Nitroso compounds
∙ Alkylating agents: It includes various chemo- • Promoters of carcinogenesis
therapeutic drugs that have successfully cured, – Phorbol, phenols, drugs like phenobarbital
controlled or delayed recurrence of certain types Physical carcinogenesis
of cancers only to later evoke a second form of • Radiation carcinogenesis
cancer usually leukemia. Various agents used are • Nonradiation physical carcinogenesis like mechani cal
cyclophosphamide, chlorambucil, busulfan, melphalan, injury or implants of inert materials
nitrosourea, β-propiolactone and epoxides. This tragic Hormonal carcinogenesis
consequence is called as “Pyrrhic victory” which • Estrogen
becomes less of a victory when their initial use has been • Contraceptive hormones
converted to cause later on second form of cancer. • Anabolic steroids
∙ Acylating agents: Substances like acetyl imidazole. • Hormone dependent tumors
Indirect-acting carcinogens or procarcinogens: These Biologic carcinogenesis
are chemical substances requiring metabolic activation for • RNA oncogenic viruses
becoming potent initial carcinogens. • DNA oncogenic viruses.
Promoters of Carcinogenesis further clonal proliferation of the initiated cell. Sometimes

two or more initiators are the chemical, oncogenic virus
Certain chemical substances lacking the intrinsic
or radiant energy may act in concert to induce malignant
88 carcinogenic potential but helping the initiated cell to
transformation referred to as co-carcinogens.
proliferate further are called promoter of carcinogenesis.
For example, phorbol, phenols, drugs like phenobarbital,
and artificial sweeteners likes saccharine. PHYSICAL CARCINOGENESIS
It is divided into two groups:
Mechanism of Action and Stage
of Chemical Carcinogen Radiation Carcinogenesis
The great majority of chemical carcinogens are mutagens. Radiation whenever in the form of UV light from sunlight,
They bind directly to DNA and RNA or cytoplasmic UV lamp, welder’s arc, or ionizing radiation like X-ray, α,
proteins to specific sites within molecule inducing β and γ ray, radioactive isotopes, protons and neutrons are
miscoding error during transcription and replication. The established carcinogens. Most frequent radiation induced
carcinogenicity of chemical agents is dose dependent and cancers are leukemia, cancer of thyroid, skin, breast, lung,
multiple traditional doses have same oncogenecity as a and salivary gland. Therapeutic irradiation can also induce
single comparative dose. carcinogenesis.
The carcinogenicity of chemical agents can be Radiant energy has potential of producing mutation and
significantly enhanced by the subsequent administration even killing the cells. It can affect carcinogenesis by the
of promotors. To be effective the promotor must follow following facts:
the initiator. The phenomenon of cellular transformation Few tumors appear only after long latent period during
by chemical carcinogenesis is a progressive process which successive generation of clones are developed.
involving two different stages. They are initiation and The radiation initiation is generally irreversible, but at
promotion. a low dosage level is amenable to repair.
The effect of radiation depends upon a number of factors
Initiation such as type of radiation, dose, length of interval between
In this initiator, interacts with DNA of target cell to induce the doses, capability of cells to repair in intervals and
mutation that is more or less irreversible to transform it various host factors such as age, individual susceptibility,
into initiated cell: immune competence, hormonal influence and type of cell
irradiated.
Metabolic activation: Only indirect acting carcinogen or
The ultimate mechanism of radiation may directly alter
pro-carcinogens require metabolic activation, chiefly by
the cellular DNA and it may dislodge ions from water and
mixed oxidizes of cytochrome P 450 system located in
other molecules of cell and result in the formation of highly
microsomal compounds of the endoplasmic reticulum or
reactive free radicals that may bring about the damage.
in the nucleus.
Radiation mutation may render cell vulnerable to other
Reactive electrophiles: They are electron deficient carcinogenic influence, i.e. acting as cocarcinogen, inhibition
protons, which bind to electron rich portions of other of cell division and inactivation of enzymes, and radiation
molecules of cell such as DNA, RNA or other proteins. might cause cell killing; permitting survivors to proliferate
Target molecules: The primary target is DNA, producing and thereby become vulnerable to oncogenic influence.
mutagenesis. Nonradiation Carcinogenesis
Initiated cell: The unrepaired damage produced in the Mechanical injury to tissues such as from stones in the
DNA of the cell becomes permanent only if the altered cell gallbladder, stones in the urinary tract, and healed scars
undergoes at least one cycle of proliferation. following burns or trauma have been suggested as causes
of increased risk of the carcinoma.
Promotion
Implants of inert materials such as plastic, glass, etc. in
It does not damage the DNA but enhances the effect of direct- prostheses and foreign bodies like metal foils observed to
acting carcinogen or procarcinogens. The ultimate effect cause tumor development in experimental animals.
HORMONAL CARCINOGENESIS and in certain types of cancers in humans. The association

of oncogenic virus with neoplasia was observed by an
Carcinoma is most likely to develop in organs and Italian physician Sanarelli in 1889 who noted association
tissues which undergo proliferation under influence of between myxomatosis of rabbit with poxvirus. Oncogenic 89
excessive hormonal stimulation. Hormone sensitive tissues viruses fall into two broad groups, i.e. those containing
developing tumors are breast, endometrium, myometrium, ribonucleic acid are termed as RNA oncogenic viruses and
vagina, thyroid, liver, prostate, and testis. those containing deoxyribonucleic acid are termed as DNA
oncogenic viruses.
Estrogen
In experimental animals: Induction of breast cancer in Types of Virus Causing Cancer
mice by administration of high doses of estrogen. Other
RNA oncogenic viruses
cancers which can be induced in mice by estrogens are
• Acute transforming viruses (Rous sarcoma virus)
squamous cell carcinoma of cervix, connective tissue
• Slow transforming tumor viruses (mammary tumor
tumor of myometrium, tumor of kidney in hamsters, and
virus MMTV)
benign and malignant tumors of liver in rats.
• Human T-cell lymphotropic viruses (HTLV)
In humans: Women receiving estrogen therapy and DNA oncogenic viruses
women with estrogen secreting granulosa cell tumor of • Papovavirus group
the ovary have increased risk of developing endometrial • Adenoviruses
carcinoma. Adenocarcinoma of the vagina is seen with • Poxvirus
increased frequency in adolescent daughter of mother who • Hepadnaviruses
had received estrogen therapy during pregnancy. • Herpes virus.
Contraceptive Hormones RNA Oncogenic Viruses

Increased risk of developing breast cancer, benign tumors These are retroviruses, i.e. they contain the enzyme reverse
of the liver and few patients have developed hepatocellular transcriptase, which is required for reverse transcription of
carcinoma. viral RNA to synthesize viral DNA strands. Based on their
Anabolic Steroids activity to transplant target cells into neoplastic cells, they
all are divided into three subgroups:
Consumption of anabolic steroids by athletes to increase 1. Acute transforming viruses: It includes Rous sarcoma
the muscle mass also increases the risk of developing virus in chickens, leukemia-sarcoma viruses of avian,
benign and malignant tumors of the liver. feline, bovine and primate.
Hormone Dependent Tumors 2. Slow transforming tumor viruses: Mouse mammary
tumor virus (MMTV) that causes breast cancer in
It has been shown in experimental animals that induction daughter mice.
of hyperfunction of adenohypophysis is associated with
3. Human T-cell lymphotropic viruses (HTLV): It can
increased risk of developing neoplasia of the target organs
cause adult T-cell leukemia-lymphoma syndrome and
following preceding functional hyperplasia.
AIDS.
BIOLOGIC CARCINOGENESIS Mechanism of RNA Viral Oncogenesis

The epidemiological studies on different types of cancer Reverse transcriptase acts as a template to synthesize a
indicate the involvement of transmissible biologic agents single strand of matching viral DNA. Single strand of viral
in their development, chiefly viruses. It has been estimated DNA is then copied by DNA dependent DNA synthetase
that about 20 percent of all cancers worldwide are virus to form another strand of complementary DNA resulting
associated cancers. Therefore biological carcinogenesis is in double stranded viral DNA or provirus. The provirus is
largely viral oncogenesis. A large number of viruses have then integrated into the DNA of the host cell genome and
been proved to be oncogenic in wide variety of animals may transform the cell into a neoplastic cell.
Virus replication begins after integration of provirus development in humans. Free radicals scavenging vitamins
into host cell genome. Integration results in transcription C and E have been shown to protect against cancer
of proviral genes or progenes into messenger RNA which development in animal models.
90 then forms components of the virus particle, i.e. virion core
proteins from gag gene, enveloped glycoprotein from env Biology of Tumor Growth
gene and reverse transcriptase from pol gene. The life cycle of malignant tumors can be divided into four
The three components of virus particles are then phases.
assembled at the plasma membrane of host cells and virus
particles released by budding off from plasma membrane, Induction of Malignant Changes in the Target Cell
thus completing the process of replication. (Transformation)
Large number of carcinogen agents induces neoplastic
DNA Oncogenic Viruses
transformation of cells in vivo and in experimental animals.
They are divided into four groups: All etiologic factors ultimately affect the function of two
1. Papovavirus group: Human papilloma virus, sets of genes, one is proto-oncogenes or oncogenes and
polyoma virus, SV-40 (simian vacuolating) virus. another one is anti-oncogenes or cancer suppressor genes.
Herpes virus: Epstein-Barr virus, human herpesvirus, The majority of carcinogens are mutagenes which bind
cytomegalovirus, lucke’s frog virus, Marek’s disease the DNA directly or indirectly by undergoing enzymatic
virus. activation, inducing miscoding errors during transcription
2. Adenoviruses: It can cause upper respiratory infections and replication. Oncogenes may code for growth promoting
and pharyngitis. In man, they are not known to be factors and as a result the tumor cells produce large amount of
involved in tumors but in hamsters they may induce growth factors to which, only they can respond. Oncogenes
sarcomas. may encode a defective receptor that sends stimulating
3. Poxvirus: In rabbits it can cause myxomatosis and in signals to the cells, even in the absence of growth factors.
humans it can cause molluscum contagiosum and may Thus cancer is a genetic disease that results when multiple
induce squamous cell papilloma. mutations accumulate in the DNA of a cell and specific
4. Hepadnaviruses: Hepatitis B virus is a member of this chromosomal abnormalities predispose to cancer.
family and it can cause acute hepatitis and is responsible
for carrier state which can result in some cases to Growth of Transformed Cells (Kinetics of
chronic hepatitis progressing to hepatic cirrhosis and Tumor Cell Growth)
onto hepatocellular carcinoma.
The monoclonal cancer cell (10 mm in diameter) has to
Mechanism of DNA Viral Oncogenesis undergo about 30 population doublings to produce 109
cells weighing approximately 1 gm, which is the smallest
Replication: The virus may replicate in the host cell with
clinical detectable mass. To produce a tumor of 1012
consequent lysis of infected cell and release of virions.
cells, weighing 1 kg approximately, which is usually the
Integration: The viral DNA may integrate into the host maximum size compatible with life, the tumor cells have
cell DNA. This results in neoplastic transformation of the to undergo 10 further population doublings. So by the time
host cell. the tumor is clinically detectable, it has already completed
a major portion of its life cycle. In tumor cells, there is an
Oxidative Mechanism of Carcinogenesis imbalance between cell production and cell loss, therefore
Active oxygen species and other free radicals are known the tumor grows progressively. The rate of tumor growth
to be mutagenic. Further these agents have emerged as depends upon the growth fraction and the degree of
mediators of the other phenotypic and genotypic changes imbalance between cell production and cell loss.
that lead to form mutation to neoplasia.
Free radical production is ubiquitous in all respiring Mechanism of Local Invasion and
organism and is enhanced by many disease states, by Distant Metastases
carcinogen exposure and under conditions of stress. There are three routes through which metastases of tumor
Free radicals may, therefore, contribute widely to cancer cells occur, i.e. local invasion, via blood vessels and via
lymphatics. The local invasion takes the path of least perform the function of suppressing cell proliferation, thus
resistance and the tumor cells invade the surrounding tissue allowing them to proliferate.
spaces. In case of oral malignancies distant metastasis is According to genetic regulatory mechanism theory,
mainly via lymphatics, either by lymphatic permeation or primary change in the cell consists of a modification of 91
by lymphatic embolism. It spreads through blood vessels repressor molecule which controls the functions of the
and if this occurs, the tumor cells invade the lumen of blood gene. The repressor molecules are either RNA or protein.
vessels, the tumor emboli form, which are fragmented and The modification of repressor molecules removes their
the tumor cells are lodged into distant tissues. orderly inhibitory control, which is responsible for normal
morphogenesis and differentiation, and unearths the cell
Theories of Carcinogenesis genetic potentiality for unrestricted growth. This concept of
• Epigenetic theory loss of growth control is described as ‘feedback deletion’.
• Genetic theory
• Virus theory Virus Theory
• Immune surveillance theory Viruses participate at some stage in the development of
• Monoclonal hypothesis cancer. The concept of mode of action of virus has taken
• Multistep theory. many forms.
Virus is present as a parasite in all tumor cells and
Theories of Carcinogenesis it is transmitted from cell to cell and stimulates extreme
hyperplasia without affecting the genome cell. It acts as a
Epigenetic Theory biologic carcinogen on some cellular constituents to release
According to this theory, the carcinogenic agents act on or activate neoplastic potentialities normally present in cells.
the activators or suppressors of genes and not on the genes Carcinogens of all kinds ultimately act by creating
themselves and result in the abnormal expression of genes. some new auto-synthesizing cytoplasmic constituents,
probably an autocatalytic protein, which can excite the cell
Genetic Theory to unlimited growth.
This is the most popular theory which suggests that cells
become neoeplastic because of alteration in the DNA. Immune Surveillance Theory
It is suggested that the secret of cancer lies within the It suggests that an immune-competent host mounts an
normal cells themselves in the form of proto-oncogenes attack on developing tumor cells so as to destroy them
(c-oncs). The mutated cells transmit their characters to while an immune incompetent host fails to do so.
the next progeny of cells. Expression of mutated gene or According to original immunological theory, normal
point mutation leads production of various growth factors cells contain specific self-marker (identity proteins) which
or they disrupt underlying normal regulatory control. The is recognized by the normal growth regulating mechanism.
qualitative and quantitative changes in the expression of
These proteins serve as receptor for chemical carcinogens
genome may be brought about by carcinogenic influence, (hapten) and the resulting complex is self-replicating.
i.e. chemicals, viruses, radiation or spontaneous random The complex (complex antigen) triggers off an immune
mutations. response and the antibody (free or cell bound) combines
Oncogenes: Oncogenes are the transforming genes with the self-marker carcinogen complex and eliminates it.
present in many tumor cells. Closely related genes are The new race of cells produced is with self-markers deleted
detected on normal animal and human cells and are called and goes unrecognized by growth regulatory mechanism.
‘proto-oncogenes’ or ‘cellular oncogenes’, abbreviated as The high incidence of cancer in AIDS patients is in support
c-oncs. of this theory.
Cellular oncogenes of the host cells can transcribe its
copies in the viral genome of acute transforming oncogenic Monoclonal Hypothesis
retroviruses called as viral oncogenes or v-oncs. Currently, there is strong evidence on studies of human
An alternate mechanism is by anti-oncogenes in which and experimental animals that most cancers arise from
there is inactivation or deletion of genes that normally single clone of transformed cell. The best documentation
of monoclonal origin of cancer cells comes from the study the basement membrane and invasion of connective tissue
of G6PD in women who are heterozygous for its two (carcinoma) → Entering the wall of blood and lymphatic
isoenzymes A, and B. It is observed that all tumor cells in vessels → Survival of malignant cells in the blood stream
92 benign uterine tumor (leiomyoma) contain either A or B → Emergence of the malignant cells from the blood vessels
genotypes of G6PD, i.e. the tumor cells are derived from a in the form of the emboli and lodgment in other tissues →
single progenitor cell. Survival in the compatible tissue environment and induction
of growth factor to stimulate new vessel formation to obtain
Multistep Theory nutrition → Multiplication of neoplastic cells and growth
According to this theory, carcinogenesis is a multistep to form secondary neoplasm at the new site.
process which is substantiated by in vitro changes in Each of these steps is probably controlled by different
experimental animals as well as in vivo changes in human molecular mechanism and this may explain the differences
cancers. in the behavior with reference to tumor metastasis.
In chemical carcinogenesis, there are two essential Neoplastic cells within a single tumor might differ in their
features, i.e. initiation and promotion. Many tumors arise ability to metastasize. A subpopulation of cells pre-exists
from combination of activation or growth promoting within the heterogeneous primary tumor. The relative size
oncogenes and inactivation of growth suppressing anti- of this subpopulation in the primary tumor may vary with
oncogenes. In some cancers, there is initial dysplastic time between the neoplasms.
change that may progress into carcinoma in situ and then
into invasive carcinoma. Routes of Metastasis
Lymphatics: Particularly for carcinoma and lymphosar-
METASTASIS coma. For example, mouth to neck nodes and breast to ax-
illary nodes.
Metastasis is defined as spread of tumor by invasion in such
a way that discontinuous secondary tumor mass/masses Blood stream: Particularly veins from gut via portal
are formed at the site of lodgment. This metastasis is the circulation to liver, from systemic sites through right heart
transfer of the disease from one organ or part to another not to lung, from left heart to any systemic sites.
directly connected with it. Cavities: Along epithelium lined cavities, for example,
If malignant cells do not metastasize, the surgical respiratory tract, gut, urogenital tract, etc.
removal of primary neoplasm would completely cure the Others: Transcelomic spread, cerebrospinal fluid, tissue
patient. Metastasis is fundamentally an embolic process. planes and through nerve sheath.
The invasiveness of malignant cells involves motility,
which requires changes of shape and adhesiveness and Pattern of Metastatic Spread
ability to degrade the matrix in order to penetrate it. Thus,
Mechanistic theory: The capillary bed of the first organ
a definition of the behavior of the metastatic tumor cells
which encounters viable neoplastic cells is the preferred
is the tendency to cross the tissue compartment/boundary
site of metastasis.
and intermix with other cell types. The metastatic process
can be divided into several sequential steps although these Seed and soil hypothesis: It suggests that availability of
steps are interconnected. fertile environment (the soil) in which compatible tumor
Factors which control metastasis are proteolysis, cell cells (the seed) can grow is important. Ewing suggested
adhesion, tumor angiogenesis, cell mediated immunity and that varying pattern of metastasis is due to fact that different
genetic factor. tumor cells thrive in certain biological sites (soils) but not
in the other sites.
Steps of Metastasis Cell interaction: Interaction between cell surface
The breaking of loose neoplastic cells from the parent protein of malignant cells and organ specific protein, e.g.
tumor → Invasion of the matrix (sarcoma), penetration of fibronectin receptor.
GRADING AND STAGING OF CIN Grading Depending on Thickness of Squamous

TUMORS Epithelium Involved by Dysplastic Cells
• Mild 93
Grading • Moderate
The grading features are those indicative of proliferation • Severe.
and differentiation. It is defined as macroscopic and
microscopic degree of differentiation of tumor. Grading Staging
depends mainly on two histologic features, the degree of It is the extent of spread of tumor within patients. It is
anaplasia and the rate of growth. Different types of grading assessed by clinical examination (size and extent of primary
systems are as follows: lesion), investigations, pathological examination, degree
of infiltration of primary lesion, presence or absence of
Broder’s Classification System metastasis to regional lymph nodes, presence and absence
• G rade I (Well differentiated, i.e. less than 25 percent of distant metastasis, involvement of contralateral or
anaplastic cells): It is characterized by the presence ipsilateral node and whether nodes are fixed or not.
of relatively mature cell with little nuclear aberration
Objectives
and with the presence of keratin pearls and individual
cell keratinization. ∙ To aid clinician in the planning of treatment.
• Grade II (Moderately differentiated, i.e. 25 to 50 ∙ To give some indication of prognosis.
percent anaplastic cells): It is characterized by the ∙ To assist in evaluation of the result of treatment.
presence of tumor cell exhibiting a wide range of ∙ To facilitate the exchange of information between
differentiation, keratinization is occasionally present, treatment centers.
and nuclear aberrations are moderately abundant. ∙ To contribute to the continuing investigations of human
Usually the invasion is poorly delineated from the cancer.
stroma. TNM Staging (Table 7.3)
• Grade III (Moderately differentiated, i.e. 50 to
75 percent anaplastic cells): It is characterized by It is universally accepted system which is developed by
disorderly and poorly differentiated cells with no UICC (Union International Control of Cancer).
tendency towards keratinization, nuclear aberrations
AJC (American Joint Committee)
are abundant.
It divides all cancers into stage 0 to 4, and takes into
• Grade IV (Poorly differentiated, i.e. more than 75
account all three previous TNM systems.
percent anaplastic cells): In it cells are so poorly
• Stage 0: Tis N0 M0
differentiated that they cannot be identified as
• Stage 1: T1 N0 M0
epithelial origin on the basis of histology alone, nuclear
• Stage 2: T2 N0 M0
aberrations are abundant and no keratinization is found.
• Stage 3: T3 N0 M0, T1 N1 M0, T2 N1 M0 and T3 N1 M0
• Stage 4A: T4 N0 M0, T4 N1 M0, any T N2 M0
CIN Grading • Stage 4B: Any T N3 M0
Alternative classification for grading of dysplasia and • Stage 4C: Any T, any N, M1
carcinoma in situ together is cervical intraepithelial
neoplasia (CIN). Dukes ABC Staging
It is used in cancers of bowel:
CIN Grading • Stage A: When tumor is confined to submucosa and
• CIN I: It represent less than one-third involvement of muscle and cure rate is 100 percent.
the thickness of the epithelium. • Stage B: Tumor penetrates the entire thickness of
• CIN II: In it there is one-third to two-thirds bowel wall into pericolic or perirectal tissues and
involvement. cure rate is 70 percent.
• CIN III: It is full thickness or equivalent to carcinoma • Stage C: It is characterized by lymph node metastasis
in situ. and reduces the cure rate to 30 percent.
Table 7.3 TNM staging
Primary tumor (T) Regional lymph nodes (N) Distant metastasis (M)
94
Local extent is major factor contributing • Nx: Regional lymph node cannot be • Mx: Distant metastasis cannot be
to prognosis. assessed. assessed.
• Tx: Primary tumor cannot be assessed. • N0: No regional lymph node metasta- • M0: No distant metastasis.
• T0: No evidence of primary tumor. sis. • M1: Distant metastasis. Category M1
• Tis: Carcinoma in situ. • N1: Metastasis in single ipsilateral may be further specified according to
• T1: Tumor 2 cm or less in diameter. lymph node less than 3 cm in diam- the notation.
• T2: Tumor 2-4 cm in diameter. eter. – Pulmonary—PUL
• T3: Tumor more than 4 cm in greatest – N1a: Nodes considered not to – Osseous—OSS
diameter. contain tumor growth. – Hepatic—HEP
• T4: Tumor of any size in which – N1b: Nodes considered to contain – Brain—BRA
tumor invades adjacent structure (e.g. growth. – Lymph nodes—LYM
cortical bone, inferior alveolar nerve, • N2: Single lymph node, no more than 6 – Bone marrow—MAR
floor of mouth, skin of face, etc.). cm in greatest dimension, of bilateral/ – Pleura—PLE
contra-lateral lymph node, none more – Peritoneum—PER
than 6 cm. – Skin—SKI
– N2a: Single ipsilateral lymph node – Other—OTH
more than 3 cm but less than 6 cm.
– N2b: Multiple ipsilateral lymph
nodes less than 6 cm.
– N2c: Bilateral or contralateral
lymph node less than 6 cm in
greatest dimension.
• N3: Metastasis in lymph node more
than 6 cm and it is fixed.
– N3a: Ipsilateral nodes at least one
greater than 6 cm.
– N3b: Bilateral nodes greater than 6
cm.
– N3c: Contralateral nodes at least
one greater than 6 cm.
Table 7.4 STNMP staging
S—site of primary Size of tumor—it is Regional nodes were Metastasis Pathology of lesion
tumor denoted by T grouped as 95
• S1 - Lip and skin. • T1 - Less than 2 cm • N0 - No palpable • M0 - No distant • P0 - Hyperkeratotic

• S2 - Lip mucosa. in diameter. nodes. metastasis lesion showing
• S3 - Tongue. • T2 - Between 2 • N1 - Equifocal node • M1 - Clinical atypia.
• S4 - Cheek. cm and 4 cm in enlargement. evidence of distant • P1 - Carcinoma in
• S5 - Palate. diameter. • N2 - Clinically metastasis without situ.
• S6 - Floor of mouth. • T3 - Between palpable definite histological • P2 - Basal cell
• S7 - Alveolar 4 cm and 6 cm homolateral or radiographic carcinoma.
process. in diameter and regional nodes, not conformation. • P3 - Verrucous
• S8 - Antrum. extending beyond fixed • M2 - Proven carcinoma.
• S9 - Central the primary region • N3 - Same as N2 but evidence of • Well differentiated
carcinoma of bone. and extending fixed metastases beyond squamous cell
through adjacent • N4 - Clinically regional nodes carcinoma.
periosteum. palpable • Moderately
• T4 - Greater than contralateral or differentiated
6 cm in diameter bilateral nodes, not squamous cell
and extending to fixed. carcinoma.
involve adjacent • N5 - Same as N4 but • Poorly differentiated
structures fixed squamous cell
carcinoma.
STNMP Staging System (Table 7.4) 6. Mahour GH, Landing BH, Wooley MM. Teratomas in
children: clinicopathologic studies in 133 children. Z
Nowaday in the TNM staging site and pathology of lesion
Kinderchir. 1978;23:365.
is added. This is described in Table 7.4.
7. Rodin AE, Singula P. Teratoma of the tongue at birth.
Pediatr Pathol. 1985;3:291.
BIBLIOGRAPHY
8. Shafer, Levy H. A Textbook of Oral Pathology, 4th edn.
1. Ashley JV, Shafer AD. Teratoma of the tongue in a newborn. WB Saunder’ s company; Philadelphia.
Cleve Clin Q. 1983;50:34. 9. Sperber G. Craniofacial development; BC Decker Inc;
2. Grier EA, MacNerland RH. Benign teratoma of the tongue. Canada; 2001.
Ill Med J. 1967;132:43.
10. Stevenson R, Hall J. Human malformations and elated
3. Kjeld P. Focal epithelial hyperplasia, 1st edn. Chrono Press;
anomalies, 2nd edn. Oxford University Press; 2006.
2012.
4. Kumar V, Abbas A, Mitchel R. Robbins and Cotran Pathologic 11. Toll A. Intravascular papillary endothelial hyperplasia;
Basis of Disease, 8th edn. Elesevier India Pvt. Ltd; 2009. Ceed publishing; 2012.
5. Lalwani AK, Engel TL. Teratoma of the tongue: a case report 12. Uchida K, Urata H, Suzuki H. Teratoma of the tongue
and review of the literature. Int J Pediatr Otorhinolaryngol. in neonates: report of a case and review of the literature.
1992;24:261. Pediatr Surg Int. 1998;14:79.
96 1. Loss in uniformity and architectural orientation is 6. Malignant tumors of epithelial origin are called as:
called as: a. Fibromas
a. Dysplasia b. Metaplasia b. Sarcomas
c. Hyperplasia d. Hamartoma c. Carcinomas
2. Pulp polyp is an example of: d. Odontogenic hamartoma
a. Hamartoma b. Dysplasia 7. Following are the DNA oncogenic virus except:
c. Hyperplasia d. Metaplasia a. HTLV b b. Hepadna virus c
3. Fordyces granules comes under: c. Herpes d d. Adeno virus
a. Choriostoma
8. All are the theories of carcinogenesis except:
b. Odontogenic hamartoma
a. Genetic theory b. Epigenetic theory
c. Nonodontogenic hamartoma
c. Virus theory d. Mechanistic theory
d. Teratoma
4. DNA synthesis is absent in which phase: 9. Grading of the tumors mainly depends on two
a. M phase b. G 2 phase histologic features:
c. S phase d. G 1 phase a. The degree of anaplasia
b. The rate or growth
5. Chlorambucil, cyclophosphamide, nitrosourea are the
c. Both
examples of:
d. None of the above
a. Physical carcinogens
b. Chemical carcinogens 10. Universally accepted grading system for tumors is:
c. Biologic carcinogens a. TNM staging b. STNMP staging
d. Hormonal carcinogens c. Dukes ABC system d. AJC system
Teeth Anomalies
Anil Govindrao Ghom , Shubhangi Mhaske (Jedhe), Savita Ghom
A Chapter Outline
O Scale of human tooth development • Supernumerary teeth

• Disorders of size of teeth • Structure of teeth
• Microdontia • Environmental enamel hypoplasia
o Macrodontia • Mottled enamel or dental fluorosis
• Disturbances in shape of teeth • Molar incisor hypominerlization
• Gemination • Amelogenesis imperfecta
• Twining • Dentinogenesis imperfecta
• Fusion • Dentin dysplasia
• Concrescence • Regional odontodysplasia
• Talon' s cusp • Fibrous dysplasia of dentin
• Di laceration • Dentin hypocalcification
• Dens in dente O Anomalies associated with eruption of the teeth
• Dens evaginatus • Eruption of teeth
• Taurodontism • Theories of tooth eruption
• Supernumerary roots • Pre- deciduous dentition
• Ectopic enamel • Delayed eruption
• Globodontia • Embedded and impacted teeth
• Shovel shaped incisors • Ankylosis or submerged teeth
• Moon' s molar • Transposition
• Hutchinson' s incisor • Eruption sequestrum
• Carabelli' s cusp • Ectopic eruption
• Mulberry molar • Premature exfoliation
o Disorders of number of teeth • Postpermanent dentition
• Anodontia or hypodontia
DISORDERS OF DEVELOPMENT of the normal chronology of the human dentition and of

the normal development and structure of the teeth. These
OF TEETH disorders may be due to abnormalities in the differentiation
Developmental disorders or anomalies are either prenatal or of the dental lamina and the tooth germs, causing anomalies
postnatal in origin, which can be inherited or acquired . Their in the number, size , and form of teeth (abnormalities of
recognition and evaluation require a thorough knowledge morphodifferentiation) or to abnormalities in the formation
https: //t.me / LibraryEDent

of the dental hard tissues resulting in disturbances in

Table 8.1 Classification of anomalies of teeth
tooth structure (abnormalities of histodifferentiation).
Abnormality in morphodifferentiation occur in early stage S. Disorder Subtypes
98 while in histodifferentiation it occur in later stage; in some No. affecting teeth
disorders both stages of differentiation are abnormal. 1. Size • Microdontia
• Macrodontia
SCALE OF HUMAN TOOTH 2. Shape • Gemination
DEVELOPMENT • Twining
• Fusion
∙ 6 weeks (40–42 days): Rupture of buccopharyngeal
• Concrescence
membrane.
• Talon’s cusp
∙ 42–48 days: Dental lamina formation. • Dilaceration
∙ 55–56 days: Bud stagedeciduous incisor, canine and • Dens evaginatus
molar. • Dens invaginatus
∙ 14th week: Bell stage for deciduous bud for permanent. • Taurodontism
∙ 18th week: Dentin and functional ameloblast. • Supernumerary roots
∙ 32nd week: Dentin and functional ameloblasts of • Miscellaneous
permanent 1st molar. 3. Number • Anodontia
The developmental disturbances of teeth can be • Supernumerary teeth
classified under broad category as (Table 8.1) 4. Structure A. Enamel
∙ Disorders of size of teeth i. Amelogenesis imperfecta
∙ Disorders of shape of teeth ii. Enamel hypoplasia
∙ Disorders of number of teeth B. Dentin
∙ Disorders of structure of teeth i. Dentinogenesis imperfect
ii. Dentin dysplasia
∙ Disorders of eruption of teeth.
C. Both enamel and dentin
i. Regional odontodysplasia
DISORDERS OF SIZE OF TEETH D. Cementum
The size of both the teeth as well as the jaws is influenced 5. Eruption • Premature eruption
by genetic and environmental factors. Studies of twins • Delayed eruption
have shown that for the teeth, at least, genetic factors plays • Embedded and impacted teeth
a major role this variation. The anomalies of teeth due to • Ankylosis or submerged teeth
• Transposition
disturbance in size are microdontia and macrodontia.
• Eruption sequestrum
Microdontia • Ectopic eruption
• Premature exfoliation
It refers to teeth that are smaller than normal. There are
three types of microdontia.
∙ True generalized: All the teeth are smaller than normal. Clinical Features
It occurs in pituitary dwarfism, Down’s syndrome and Localized microdontia
congenital heart disease. Most commonly affected teeth are maxillary lateral
∙ Relative generalized: The teeth are or slightly smaller incisors and 3rd molars (Fig. 8.1). Supernumerary teeth are
than normal teeth; but the jaws are larger than the frequently smaller than normal. One of the common form
normal which simulate the microdontia. It is hereditary. of localized microdontia is peg shaped laterals in which
It often exhibits spacing between the teeth. the mesial and distal sides converges or taper incisally,
∙ Localized: It involves only single tooth. It occurs forming peg shaped or cone shaped crown.
with congenital heart diseases, Down’s syndrome and In case of molar, they may also undergo a change in
progeria (Hutchison’s–Gilford syndrome characterized shape from five to four cusps in case of mandibular molar
by dwarfism and premature senility). and from four to three cusps in upper molars.
Teeth Anomalies
99
Figure 8.1 Microdontia of 3rd molar showing small size Figure 8.2 Macrodontia of 2nd molar showing more cusp of
the tooth
Management In many cases tooth with macrodontia show more cusps

Crown and bridge work is required for esthetic rehabilitation (Fig. 8.2).
of teeth.
Management
Points to Remember If necessary orthodontic treatment is done. If impacted,
Teeth smaller than normal, occurs with Down’s extraction is indicated.
syndrome, dwarfism, congenital heart disease, true
Points to Remember
generalized, relative generalized, localized, peg shaped
laterals. Teeth are larger than normal, associated with pituitary
gigantism, facial hemihypertrophy, angioma of face,
true generalized, localized, relative generalized.
Macrodontia
It is also called megadontia. These are the teeth which are
larger than normal. It is of three types: DISTURBANCES IN SHAPE OF TEETH
1. True generalized: All the teeth are larger than normal. Disturbances in tooth form may involve the crown, the
It is commonly associated with pituitary gigantism. root, or both. The most frequent variations of the crowns
2. Relative generalized: Teeth are normal or slightly of teeth affect maxillary permanent lateral incisors, which
larger than normal, but present in a smaller jaw. may be peg-shaped or show an accentuated cingulum-either
3. Localized: One or more large teeth exist in relation to variation sometimes being associated with an invagination.
an otherwise normal dentition and body size. Premolars or molars with an increased or decreased
number of cusps are also frequently seen. Variations in
Causes
the number, course, form, and size of roots are particularly
It is occasionally seen in facial hemihypertrophy, in which common.
half of the teeth in unilateral distribution are affected.
Angioma of face, pituitary gigantism and genetic component. Gemination
It refers to the process whereby, single tooth germ
Clinical Features invaginates resulting in incomplete formation of two teeth
Teeth are larger than normal. There is crowding, which that may appear as bifid crown on single root.
may result in malocclusion. Due to lack of space impaction It occurs during the proliferation stage of the growth
of teeth is common. It should not be confused with fusion. cycle of tooth.
Etiology Management
Hereditary and familial tendency is present. Affected tooth structure should be removed and crown may
100 It results from the splitting of a tooth germ during be restored and reshaped.
development or from the fusion of a normal tooth bud with Reduction of mesiodistal width with periodic disking.
a developing supernumerary tooth (Fig. 8.3). Final jacket crown preparation.
Clinical Features Points to Remember

Males and females are equally affected. Hereditary and familial tendency, bifid crown or single
root, results from splitting of tooth germ, commonly
Location: The commonly affected teeth are deciduous
affected permanent maxillary incisors, deciduous
mandibular incisors and permanent maxillary incisors.
mandibular incisors, pulpal infection, malocclusion, and
Bifid crown: It appears clinically as bifid crown on single periodontal pathosis.
root. It does not increase or decrease the number of teeth
present. Twining
There are common pulp canals and either single or
It indicates cleavage of tooth germ which results in
partially divided pulp chambers.
formation of supernumerary teeth that is mirror image or
Crown is wider than normal with shallow groove near image of tooth from which it has developed.
extending from incisal edge to cervical region.
Enamel or dentin of crown of geminated teeth may be Fusion
hypoplastic or hypocalcified. Invagination of crown occurs It is also called ‘synodontia’. It represents the embryonic
with complete and incomplete division. union of normally separated tooth germs. It represents
Complication: Areas of hypoplasia and invagination lines junction at the level of dentin between juxtaposed normal
or areas of coronal separation represent caries susceptible tooth germs.
area, which may lead to pulpal infection. It may also cause
Etiology
malocclusion and periodontal pathosis.
It is transmitted as autosomal dominant trait with reduced
penetration. Physical force or pressure generated during
development causes contact of tooth germs.
Two separate developing tooth germs being initially
close together; as they grow and expand; they contact with
each other and the germs fuse to varying degrees (Fig. 8.4).
Classification of Fusion
• C omplete: If fusion takes place before calcification
begins, the two teeth may be completely united to
form a single large tooth.
• Incomplete: If contact of teeth occurs later, i.e. when
the portion of crown has completed its formation;
then there is union of root only.
Clinical Features
Location: It is seen more commonly in anterior teeth. It is
more common in deciduous dentition than in permanent
dentition.
Figure 8.3 Gemination is a macrodont formed when there is It may occur between a normal tooth and a
partial division of tooth germ supernumerary tooth such as mesiodens or distomolar.
Teeth Anomalies
101
Figure 8.6 Fusion of molar teeth
Management
Morphology of teeth should be determined radiographically
Figure 8.4 Fusion is a process in which two adjacent tooth for endodontic treatment. After endodontic treatment,
germ fuse to form a macrodont tooth may be reshaped with a restoration that will mimic
independent crown.
Points to Remember
Synodontia, tooth is almost twice in size, spacing,
periodontal conditions, dental caries, endodontic
treatment.
Concrescence
It is a form of fusion that occurs after the root and other
major parts involved in teeth are formed or when the roots
of two or more teeth are united by cementum, below the
cementoenamel junction. It is also called ‘false gemination.
Etiology
It may occur due to traumatic injury, overcrowding of
Figure 8.5 Fusion of crown (cusps) the teeth with resorption and interdental bone loss, distal
inclination of crown of molar, space restriction during
development, excessive occlusal trauma and local infection
Tooth is almost twice in size than normal, with or
after development.
without bifid crown. Tooth may have separate or fused root
canals (Figs 8.5 and 8.6).
Classification Concrescence
Sign: Clinically there may be spacing and periodontal
problems (Fig. 8.3). • T rue concrescence: If roots are bound during
Dental caries is common in fused teeth. It may result in development.
reduced number of teeth in the jaws. When deciduous teeth • Acquired concrescence: If the condition occurs after
fuse, the corresponding permanent teeth may be absent. development.
Clinical Features Talon’s Cusp

Location: It is common in maxillary 2nd and 3rd molar It projects lingually from cingulum area of maxillary
102 area. and mandibular teeth or it is an anomalous hyperplasia
Either primary or secondary teeth are affected. Usually of cingulum on the lingual of maxillary and mandibular
involved two teeth, roots are fused by cementum (Figs 8.7 incisors, resulting in the formation of supernumerary cusp.
and 8.8).
Teeth may fail to erupt or incompletely erupt. There Pathogenesis
may be malocclusion or the teeth may be impacted. A focal proliferation of tissue during development and
exuberant development of the fourth lobe (cingulum) leads
Management to development of talon cusp.
Dentist must be careful while doing extraction.
Clinical Features (Fig. 8.5)
Points to Remember It may be found in both sexes and common in both
Traumatic injury, roots are fused by cementum, careful dentitions.
while extraction. It resembles like an eagle’s talon. It blends smoothly with
the erupted tooth, except that there is deep developmental
groove where the cusp blends with sloping lingual tooth
surface (Figs 8.9 and 8.10).
It is composed of normal enamel, dentin and contains
a form of pulp tissue. Cusp may or may not contain pulp
horn and is usually T shaped. Patients face problems with
esthetic and there is high incidence of caries. In some cases
occlusal interference may be there. It may be associated
with Rubinstein-Taybi syndrome.
Management
Removal of cusp followed by endodontic therapy should
be carried out.
Points to Remember
Figure 8.7 Concrescence is fusion between two teeth by means Focal proliferation of tissue during development of forth
of cementum (Courtesy: Dr Alka Kale, Dean and Prof Head
lobe, eagle’s talon, Rubinstein Taybi syndrome.
Oral Pathology, KLES’s Institute of Dental Sciences, Belgaum)
Dilaceration
It refers to angulations or sharp bends or curve in the roots
or crowns of the teeth.
Etiology
Mechanical trauma to calcified portion of partially formed
teeth results in dilaceration. The portion formed after
trauma is in different direction causing the dilaceration.
Developmental defect and obstacle to the normal direction
of growth can cause dilacerations.
Clinical Features
Location: It is most commonly found in maxillary
Figure 8.8 Fused roots due to concrescence incisors.
Teeth Anomalies
103
Figure 8.9 Talon’s cusp (Courtesy: Dr Alka Kale, Prof and Figure 8.11 Dilacerations seen in incisors and molars
Head, Oral Pathology, KLES’s Institute of Dental Sciences,
Belgaum)
Figure 8.10 Talon’s cusp is seen on permanent maxillary Figure 8.12 Dilacerations at the crown of the teeth
lateral
Curve or bending occurs anywhere along the length of Points to Remember

tooth, sometimes at cervical portion or midway along the
Angulations or sharp bends, mechanical trauma, curve
root or even just at the apex of root. Sometimes, angles are
or bending, angular distortion, dilacerated crown.
so acute that a tooth does not erupt.
If the defect is in the crown of an erupted tooth, the
angular distortion will be recognized (Figs 8.11 to 8.13). Dens in Dente
It is also called dens invaginatus or dilated composite
Management odontome or gestant odontome.
There is difficulty at the time of extraction. Infolding of the outer surface of the tooth into its
Dilacerated crown has to be restored with crown to interior surface occurs. It is a developmental variation
improve esthetics and function and to preclude dental which is thought to arise as a result of an invagination in
caries and periodontal disease. the surface of crown before calcification.
104
A B
Figures 8.13A and B Curved root seen in dilacerations
Etiology Figure 8.14 Pathogenesis of dense invaginatus

There is relative retardation in growth of a portion of
the enamel organ, with the result that this part remains Radicular dens invaginatus: It is a result of invagination of
stationary and the remainder grows around it. Hertwig’s epithelial root sheath resulting in accentuation of
Another view is that invagination is due to active normal longitudinal root grooves. It is lined by cementum.
proliferation of an area of enamel organ which then grows Root sheath may bud off and form a invagination, that
into dental papilla, as a sort of adenoma. results in a circumscribed cementum defect in root.
Study shows that there is presence of extravascular
fluid in the soft tissue that fills the potential invagination Clinical Features
cavity of the tooth, before it erupts, suggesting that there is Coronal dens invaginatus
increased venous pressure within the invagination.
Location: Commonly affected tooth is permanent maxillary
It could be due to pressure on the blood vessels as they
lateral and central incisor. The mandibular incisor or cuspid
pass through the entrance channel of the invagination cavity
is the next most commonly affected tooth.
where enamel is forming concentrically and centripetally,
thus tending to progressively narrow the entrance. Age and sex predilection: It is diagnosed in children or in
Expansion of the invagination could then result from adolescents. It is more common in females. This condition
the increased venous pressure and transudation (Fig. 8.14). is frequently bilateral.
It can occur due to infection of the deciduous In mild type form there is a deep pit in cingulum
predecessor or trauma. Pressure on the growing teeth can (Fig. 8.15).
also cause invagination. In moderate type pocket of enamel is formed within
tooth, with dentin at periphery. Opening to the surface
Types of Dens in Dente is constricted or remains open. Food debris may become
• Coronal dens invaginatus packed in this area with resultant caries and infection of
• Radicular dens invaginatus. pulp.
In severe type it may exhibit an invagination extending
nearly to the apex of the root.
Classification The crown may or may not be enlarged in size. The
Coronal dens invaginatus: It is anomalous infolding of shape of crown may be conical or it may be of irregular
enamel organ into dental papilla. It results in fold of hard shape. In some cases there appears to be a grossly
tissue within the tooth, characterized by enamel lining the magnified cingulum rising to the level of the incisive edge
fold and covering the dentin peripheral to it (Fig. 8.9). of the tooth, but lacking the normal contour of a cingulum
Teeth Anomalies
105
Figure 8.15 Severity of dens invaginatus Figure 8.17 Radicular dens invaginatus
chamber by a thin wall and opens in oral environment

through very narrow constriction (Fig. 8.17).
Radiological Features
The affected tooth demonstrates an enlargement of root.
Opening is situated along the lateral aspect of the root.
Histopathological Features
The lining consists of enamel and at the opening of the
between the two cusps this is continuous with the enamel
that covers the exterior of the tooth.
In invagination of severe type, pulp cavity is grossly
encroached upon and may be represented by a mere slit in
the dentine on each side of the invagination cavity. Enamel
lining is defective owing to poor mineralization and may
Figure 8.16 Coronal dens invaginatus (Courtesy: Dr Alka
be totally absent in the area.
Kale, Prof and Head, Oral Pathology, KLES’s Institute of Dental
Sciences, Belgaum, Karnataka, India)
Management
Tooth should be restored prophylactically.
(Fig. 8.16). The labial face of the tooth is often bulbous.
Some teeth with these abnormalities are so misshapen as to Points to Remember
defy verbal description. Mild type, moderate type, severe type, constricted
Radicular dens invaginatus (Fig. 8.17) opening to the surface, conical crown, grossly magnified
cingulum, crown is small, short, prominent lingual
Location: It is more is common in 1st mandibular premolar, marginal ridge, enlargement of root, lining consists of
upper lateral incisor and second molar. Abnormality is enamel, mere slit in the dentine.
usually unilateral. It occurs most frequently at the site of
an anatomical defect of the root. It is also said to be an
incomplete attempt of bifurcation of roots. Dens Evaginatus
∙ Crown is small, short and conical with a small orifice. It is also called Leong’s premolar, evaginated odontome or
∙ Lingual marginal ridge is prominent. Invagination occlusal enamel pearl. Dens evaginatus is a developmental
presents as cavity that is separated from the pulp condition that appears clinically as an accessory cusp or
globules of enamel on occlusal surface, between buccal

Points to Remember
and lingual cusps of premolar.
Leong’s premolar, evagination of an area of inner enamel
106 Pathogenesis epithelium, tubercle of enamel, polyp like protuberance,
incomplete eruption radiologically tuberculated appear-
It is caused by proliferation and evagination of an area
ance.
of inner enamel epithelium during tooth development
process. After this there is also proliferation of odontogenic
mesenchyme into dental organ also occurs. Taurodontism
It is described in 1913 by Sir Arthur Keith. In this, crown
Clinical and Radiological Features of tooth is enlarged at the expense of root.
Location: It occurs on premolar and molar teeth and usually The shape of taurodont teeth resembles that of molar
occurs unilaterally or bilaterally. It develops in persons of teeth of cud chewing animal, i.e. tauro-bull dont-tooth.
Mongoloid ancestry. It consists of all three dental tissues, It is characterized by anatomical crown of normal shape
i.e. enamel, dentine and cementum. and size, an elongated and short roots, it exhibits enlarged
It appears as a tubercle of enamel on occlusal surface of pulp chambers.
the affected tooth.
Polyp like protuberance in central groove, on lingual Etiology
ridge of buccal cusp is seen. It is hereditary in origin and usually caused by genetically
determined trait. Failure of Hertwig’s epithelial root
Other features: There may be incomplete eruption. Pulp
sheath to invaginate at proper horizontal level and
exposure and subsequent infection may occur, following
mutation resulting from odontoblastic deficiency during
occlusal wear or fracture. Pulpal extension is seen in the
dentinogenesis of the root can leads to taurodontism.
pulp of patient.
Radiological features: Occlusal surface exhibits Shaw’s Types of Taurodontism (Fig. 8.19)
tuberculated appearance (Fig. 8.18). ∙ Hypotaurodont—in this condition bi or trifurcation
extents near the cervical area of the root. This is mild
Management form.
If tubercle is a cause of occlusal interference, it should be ∙ Mesotaurodont—in this bi or trifurcation occur at the
removed under aseptic conditions. middle area of the root.
∙ Hypertaurodont—in this condition bi or trifurcation
occur at the apices of the root.
Figure 8.18 Dens evaginatus showing tuberculated Figure 8.19 Types of taurodontism (redraw image)
appearance
Teeth Anomalies
Clinical and Radiological Features (Fig. 8.20) Radiological features: There is increase apico-occlusal
height with bifurcation close to apex of tooth.
Age: It is common in early aged men but has gradually
decreased in incidence over last 3 million years. Management 107
Location: It may affect either deciduous or permanent No specific treatment is necessary.
dentition and teeth involved are invariably molars. It may
be unilateral or bilateral, or may exhibit any combination Points to Remember
of quadrant involvement. Hereditary, failure of Hertwig’s epithelial root sheath to
Involved teeth tend to be of rectangular shape rather invaginate, Klinefelter’s syndrome, Trichodento-osseous
than the normal tapering towards root. syndrome, rectangular shape teeth.
It may associate with certain dermatological condition
like epidermolysis bullosa, otodental dysplasia and Supernumerary Roots
dyskeratosis congenita.
Syndromes which are associated with this disease are It is the development of increase number of root on the
Klinefelter syndrome and Trichodento-osseous syndrome tooth.
(Table 8.2).
Clinical Features
Location: Teeth that are normally single rooted exhibit two
roots. Both, maxillary and mandibular molars particularly
3rd molars are affected showing supernumerary roots (Fig.
8.21).
They develop as slender outgrowths at the center of
furcation area of molar teeth.
Management
It assumes significance only during exodontia as these
roots may be broken off during extraction.
Points to Remember
3rd molars, slender outgrowths, center of furcation area.
Figure 8.20 Taurodontism showing enlarged root trunk.

Radiograph showing enlarged pulp chamber (Courtesy: Dr
Alka Kale, Prof and Head, Oral Pathology, KLES’s Institute of
Dental Sciences, Belgaum)
Table 8.2 Syndrome associated with taurodontism

Autosomal dominant Chromosomal and other
conditions conditions
• Apert syndrome • Klinefleter syndrome
• Axenfled-Rieger • Down syndrome
• Basal cell nevus • Dyschomdrosteosis
• Ecodermal dysplasia X-linked conditions
• Otodental dysplasia • Dyskeratosis congenital
• Trichodento-osseous • Fragile X
Autosomal recessive • Orofaciodigital , type I
conditions
• Ackerman Figure 8.21 Supernumerary root in the teeth
Ectopic Enamel
These are presence of enamel at location which is not
108 normal.
Types
• Enamel pearls
• Cervical enamel extension.
Types
Enamel pearls: Pearls or droplets are described as small
buttons or nodules of enamel, usually about 1 mm or 2
mm in diameter, that form on the root, or at bifurcation or
trifurcation of multirooted teeth. It arises from local activity
of remnants of Hertwig’s epithelium before it reduces to Figure 8.22 Enamel pearl in the cervical area
rests of Malassez.
Cervical enamel extension: Represent the dipping of
enamel from the cementoenamel junction towards the
bifurcation of molar teeth. This is triangular extension of
coronal enamel with apex directed towards the bifurcation
of the tooth.
Clinical and Radiological Features

Location: It is common in trifurcation of maxillary molars,
followed by bifurcation of mandibular molars. Those found
on maxillary molars are on, usually, mesial or distal aspect
in contrast to those on mandibular molars, which are most
often on buccal or lingual aspect.
Rarely, small pulpal extensions may reach into center
of nodule and thereby constitute additional hazard.
Rarely, tiny nodule may be sufficiently close to gingival
margin to become involved in the periodontal problems. Figure 8.23 Mulberry molar with spherical aggregate
Radiographic features: It appear as well-defined

radiopaque nodules along the root’s surface (Fig. 8.22). Globodontia
Histopathological Features It is common in crown of premolar and molar teeth. Teeth
have a round globular clover leaf appearance.
Overgrowth may be simply a cap of enamel over dentin
It is characteristic of otodental syndrome in which
and replacing cementum in that area.
there is also deafness. Other anomalies associated with it
Management are fusion of molar and premolar teeth and double pulp
chamber (Fig. 8.3).
If it is causing periodontal problems, mass can be removed.
Mulberry Molar
Points to Remember
It is a characteristic syphilitic lesion of posterior teeth
Enamel pearls, cervical enamel extension, small pulpal in which hyperplastic enamel develops with spherical
extensions, radiopaque nodules, periodontal problems. aggregates or globules on the surface of dentin (Fig. 8.23).
Teeth Anomalies
Moon’s Molar Classification

First molar is commonly involved. It is syphilitic lesion of • True
posterior teeth. Cusp of teeth shows exaggerated rounded – Total (oligodontia) 109
or nodular shapes. – Partial (hypodontia)
It is often grossly distorted, usually narrower • False
mesiodistally with loss of angulation of occlusal edge, • Pseudo
which may give cusp a squarish appearance.
Hutchinson’s Incisor Clinical Features

It commonly affected the anterior teeth. Center of the It is higher in women and most probably in Mongoloid,
incisal edge shows typical notching. than whites. Absence may be unilateral or bilateral.
An affected tooth is screwdriver shaped, with tapering In true total anodontia all teeth are missing and it
marginal ridges converging towards the incisor edge. Teeth involves both deciduous and permanent dentition.
may show barrel shaped outline. In true partial anodontia there is absence of one or
more teeth. Commonly missing are 3rd molar, maxillary
Carabelli’s Cusp lateral incisor, maxillary or mandibular 2nd premolar (Fig.
8.24).
It is accessory lingual cusp located on the mesiopalatal
False anodontia results due to extraction of teeth.
cusp of maxillary second deciduous molars and 1st, 2nd
Pseudoanodontia refers to multiple unerupted teeth.
and 3rd permanent molars. It may be unilateral or bilateral,
Hypodontia is usually associated with microdontia,
with marked deviation in size.
reduce alveolar development, increases freeway space and
Shovel Shaped Incisors retained primary teeth.
It is morphologically an anomaly of the crowns of incisor Management
teeth. It is more common in the maxillary arch.
Patient can be managed by orthodontic treatment and by
The shovel shape is manifested by the prominence
restoration or prosthesis.
of the mesial and distal marginal ridges which enclose a
central fossa on the lingual surface of incisor teeth. It has
frequently a short root.
DISORDERS OF NUMBER OF TEETH

It includes, anodontia, ectodermal dysplasia and super-
numerary teeth.
Anodontia or Hypodontia
It is congenital absence of teeth.
Etiology
Hereditary ectodermal dysplasia, cleidocranial dysplasia,
craniofacial dysostosis, cleft lip, and cleft palate can cause
anodontia.
Genetic factors, evolutionary trend towards few teeth
also lead to anodontia. In some cases X-ray radiation can Figure 8.24 Patient showing missing teeth in partial
be causative factors. anodontia
Points to Remember
Hypodontia
110 • More common in permanent than primary dentition
• May be associated with mutations in developmental
control genes
• Absence of primary teeth associated with absence of
permanent successors
• May be associated with other developmental abnor-
malities.
Severe hypodontia/anodontia
• Rare
• Associated most frequently with hypohidrotic
ectodermal dysplasia (HED)
• HED usually X-linked recessive. Figure 8.25 Extracted mesiodens
Distomolar: It is found in molar region frequently located

Supernumerary Teeth distal to 3rd molar. Generally, these teeth are smaller than
It is also called hyperdontia. normal 2nd and 3rd molar. General crown morphology is
highly abnormal.
Etiology
Paramolar: It is supernumerary molar, usually small and
A supernumerary tooth develops from 3rd tooth bud rudimentary and is situated buccally or lingually to one of
arising from dental lamina near the permanent tooth bud or the maxillary molars or inter-proximally between 1st, 2nd
possibly from splitting of the permanent bud itself. and 3rd maxillary molars.
It is inherited as an autosomal dominant trait, if
associated with syndromes. It is inherited as an autosomal Peridens: Supernumerary teeth that erupt ectopically,
recessive trait when associated with only supernumerary either buccally or lingually to the normal arch are referred
teeth. as peridens.
Types of Supernumerary Teeth Clinical Features

• Supplemental supernumerary teeth: These teeth Sex predilection: Males are affected more than females.
duplicate the typical anatomy of posterior and Male to female ratio is 2:1.
anterior teeth. Location: It may occur in both dentitions, but frequently
• Rudimentary supernumerary teeth: These are found in permanent dentition and more often in mandible.
dysmorphic and can assume conical forms. It may be erupted or impacted and occurs in 1 percent of
– Conical – small peg shaped the population.
– Tuberculate – barrel shaped anterior with more
than one cusp Supernumerary teeth occurs most often with cleft palate
– Molariform – small premolar like or molar like. Supernumerary teeth which occur in the bicuspid region,
most frequently in the lower jaw, are commonly well
Most common supernumerary teeth are as follows: formed and resemble the normal teeth.
Mesiodens: It is located at or near the midline in the incisal Shape: Their form is normal or conical or can be just
region of maxilla between central incisors. It may occur arranged as masses of dental tissue. Supernumerary teeth
singly or paired, erupted or impacted or even inverted. It frequently prevent permanent teeth from erupting or cause
is a small tooth with cone shaped crown and short root. them to erupt in an abnormal direction or site, even without
It may cause retarded eruption, displacement or resorption being any contact between them (Fig. 8.26).
of adjacent root. It frequently causes improper alignment In some cases fusion of supernumerary teeth can occur
(Fig. 8.25). with normal teeth (Fig. 8.27).
Teeth Anomalies
Table 8.3 Syndrome with globodontia or supernumerary

cusps as component
Syndrome Prominent features 111
Otodental dysplasia Sensorineural hearing loss, dental
anamolies, missing premolars
Cartilage hair – Short-limbed dwarfism due to skeletal
hypoplasia dysplasia, sparse hair, dental anomalies
(supernumerary cusps), lymphopenia,
anemia, neutropenia
Rothmund-thomson Atrophy, pigmentation, telangiectasia,
juvenile cataract, saddle nose, congenital
bone defect, disturbances of hair growth,
hypogonadism, dental anomalies, soft
tissue contractures, proportionate short
Figure 8.26 Supernumerary teeth in upper anterior region stature, anemia, osteogenic sarcoma
STRUCTURE OF TEETH
The disturbances during odontogenesis or development of
tooth germ cause defects in the hard tissues of teeth. These
can be discussed under following headings, enamel, dentin
and both enamel and dentin.
Environmental Enamel Hypoplasia

It is an incomplete or defective formation of organic enamel
matrix. Local and systemic factors that interfere with the
normal matrix formation can cause enamel surface defects
and irregularities.
Classification of Enamel Hypoplasia

Figure 8.27 Fusion of supernumerary tooth with permanent
molar tooth • M ild: There may be only few small grooves, pits and
fissures on enamel surface.
• Moderate: Enamel exhibits rows of deep pits
Syndrome: It is associated with cleidocranial dysplasia, arranged horizontally across the surface.
orofacial digital syndrome and Gardner’s syndrome • Severe: Considerable portion of enamel may be
(Table 8.3). absent.
Management Causes of Environmental Hypoplasia

It depends on potential effect on normal dentition, their • Hypoplasia due to nutritional deficiency
position, number and complications that may result from • Hypoplasia due to exanthematous disease
surgical removal. If required, they should be extracted. • Hypoplasia due to congenital syphilis
• Hypoplasia due to hypocalcemia
Points to Remember • Hypoplasia due to birth injury
More common in maxilla than mandible, occasionally • Hypoplasia due to local infection or trauma
associated with other developmental defects more • Hypoplasia due to tetracycline
common in females than males. • Hypoplasia due to chronic lead poisoning.
Clinical Features occlusal surface and occlusal third of the tooth appears to be
arranged in agglomerate mass of globule, rather than in well
Hypoplasia due to nutritional deficiency: It occur due
formed cusp. The crown is narrower on occlusal surface,
to deficiency of vitamin A, C, D, calcium and phosphorus.
112 than at the cervical margin (Figs 8.29A and B).
Two-thirds of this occurs during infancy period or early
childhood. Frequently involved are those teeth which Hypoplasia due to hypocalcemia: Tetany induced by
are formed within the first year of after birth. Vitamin D decreased level of calcium in the blood, which is as low
deficiency causes rickettsial phenomenon, resulting from as 6 to 8 mg/mL. As calcium is required for normal tooth
lack of proper calcification of enamel matrix. Horizontal formation, there is defective formation of the enamel.
pitting occurs in rows, on the teeth undergoing matrix Enamel hypoplasia in it is usually of ‘pitting’ variety.
formation at the time of dietary deficiency or during course
Hypoplasia due to birth injury–in prenatal type marked
of febrile episode. Pitting characteristically picks up stain
enamel hypoplasia affects incisal 2/3rds of enamel on
and discoloration occurs (Fig. 8.28).
maxillary primary incisors. It is due to gastrointestinal
Hypoplasia due to exanthematous disease: It include tract disturbances or metabolic disorders in the fetal life,
measles, chickenpox and scarlet fever. There is temporary probably during 2nd and 3rd trimester of pregnancy. In
elevation of body temperature. Temperature may remain neonatal type a wide band or line of enamel affects the
elevated for prolonged period of time and under these
circumstances, ameloblasts may be adversely affected.
Hypoplasia due to congenital syphilis: It involves
maxillary and mandibular permanent incisors and 1st
molars. Incisors affected are called Hutchinson incisors and
molar are called mulberry molar’s (Moon molar, Fournier’s
molar). Hutchinson’s incisors are upper central incisor is
screw driver shaped. Mesial and distal surfaces of crown are
tapering and converging towards incisal edge of the tooth,
rather than towards cervix. In addition, incisal edge is also
notched. The cause behind this is the absence of the central
tubercle or calcification center. In mulberry molars crown of
1st molar in congenital syphilis is irregular. Enamel of the
B
Figure 8.28 Hypoplasia occur due to nutritional deficiency Figures 8.29A and B Hypoplasia occur affecting the anterior
presented as horizontal pitting teeth
Teeth Anomalies
primary teeth of children associated with premature birth called mottled enamel. It is due to disturbance in tooth
or low birth weight. In traumatic birth, it may affect the formation caused by excessive intake of fluoride, during
process of amelogenesis. the formative period of dentition.
113
Hypoplasia due to local infection or trauma: Most Pathogenesis
commonly affected teeth are permanent maxillary incisor
Formative stage: Disturbance of ameloblasts during the
or maxillary or mandibular premolar. Localized type of
formative stage of tooth development and higher level of
hypoplasia caused by local infection or trauma is called
fluorides interfere with the calcification process of matrix.
turner’s hypoplasia and that tooth is called as turner’s
tooth. There may be any degree of hypoplasia, ranging from Matrix formation stage: There is diminished matrix
mild brownish discoloration of enamel to severe pitting and production, change of matrix composition and change in
irregularity of the crown. If deciduous teeth become carious ion transport mechanism.
during the period when the crown of succeeding permanent
Maturation stage: In maturation phase, there is diminished
tooth is formed, bacterial infection involving periapical
withdrawal of protein and water.
tissues may occur and this may disturb the ameloblastic
layer of permanent tooth bud, resulting in hypoplastic Clinical Features
crown. When deciduous teeth have been driven into
Dental fluorosis in primary dentition is less severe as
alveolus and have disturbed the permanent tooth bud and if
compared to permanent dentition. It frequently becomes
this permanent tooth bud is still being formed, the resulting
stained as unsightly yellow to brown color, which is
injury may be manifested as yellowish or brownish stains or
pigmentation of enamel, usually on labial surface or as true caused by coloring agents from food, medicine and
hypoplastic pitting defect. Hypoplastic defect may contain by disintegration of the increase protein contain in the
cementum, which may be stained yellowish brown. hypomineralized parts of the enamel. Sometimes, white
patches in enamel may become striated, pitted and mottled.
Hypoplasia associated with tetracycline ingestion: It may
The range of severity and appearances changes:
be incorporated in calcifying enamel matrix by formation
∙ Questionable change: It is characterized by occasional
of a tetracycline calcium orthophosphate complex. After
white flecking or spotting of enamel.
teeth eruption and exposure to sunlight, discoloration may
∙ Mild changes: It is manifested by white opaque areas
result, ranging from light yellow to brown. Varying degree
involving more of the tooth surface.
of hypocalcification may also exist. Tetracycline should
∙ Moderate and severe: Changes showing pitting and
not be administered during pregnancy and until the child
brownish staining of the surface and sometimes even
become 8-year-old.
corroded appearance. Teeth which are moderately or
Hypoplasia associated with chronic lead poisoning: It severely affected may show tendency for wear or even
is more common in children with low economic status. fracture of enamel (Fig. 8.30).
Fetus of lead poisoned mother can be affected because lead
readily crosses the placenta during pregnancy. Pitting type Clinical Classification for Fluorosis (TF)
of hypoplasia is more common in cases of lead poisoning. ∙ Score 0: The normal translucency of the glossy creamy-
white enamel remains after wiping and drying of the
Management
surface.
The hypoplastic teeth are more susceptible to dental caries ∙ Score 1: Thin white opaque lines are seen running
than the normal teeth. The restoration is usually confined across the tooth surface. The lines correspond to the
to area of involvement. Chrome steel crown is given in position of perikymata. In some cases snow-capping of
case of severe hypoplasia. Eight percent stannous fluoride cusps may also be seen.
has been found to decrease the sensitivity of teeth which ∙ Score 2: The opaque whites flecks are more pronounced
may be due to exposed dentin. and frequently merge to form small cloudy areas
scattered over the whole surface.
Mottled Enamel or Dental Fluorosis ∙ Score 3: Merging of the white lines occurs and cloudy
Drinking water that contains in excess of 1 PPM (part areas of opacity occur to spread into many parts of the
per million) fluoride can affect the ameloblasts during surface. In between the cloudy area, white lines can
the tooth formation stage and can cause the clinical entity also be seen.
placed on the affected teeth. The patient was instructed not

to rinse or eat for 30 minutes.
114 Points to Remember

Questionable changes, mild changes, moderate, severe
changes, clinical classification for fluorosis, hypo-
mineralized with an irregular prism pattern, bleaching
with 30 percent H2O2, calcium sucrose phosphate gel.
Molar Incisor Hypomineralization

There is enamel defect of one ore more permanent molar
and incisor.
The enamel may be white, yellow, brown with sharp
Figure 8.30 Hypoplasia occur due to fluoride toxicity showing
demarcation between defective and surrounding normal
corroded appearance
enamel.
Enamel is soft and porous like discolored chalk or Old
∙ Score 4: The entire surface exhibits a marked opacity
Dutch cheese (cheese molar).
or appears chalky white.
The enamel of affected molar is fragile, can chip easily.
∙ Score 5: The entire surface is opaque and there are
Enamel molar sensitive to cold, warm, mechanical trauma.
round pits that are less than 2 mm in diameter.
∙ Score 6: The small pits may frequently be seem Amelogenesis Imperfecta
merging in the opaque enamel to form bands that are It is also called hereditary enamel dysplasia, hereditary
less than 2 mm in vertical height. brown enamel and hereditary brown opalescent teeth.
∙ Score 7: There is loss of the outermost enamel It represents group of hereditary defects of enamel asso-
in irregular areas and less than half of surface is ciated with any other generalized defect. It is an ectodermal
involved. disease in which mesodermal component is normal.
∙ Score 8: The loss of the outermost enamel involves
more than half of the enamel. The remaining intact Classification
enamel is opaque. Hypoplastic type: There is defective formation of enamel
∙ Score 9: The loss of the major part of outer enamel matrix.
results in change of the anatomic shape of the surface. • Autosomal dominant
A cervical rim of opaque enamel is often noted. – Pitted
– Local
– Smooth
Enamel of these teeth has been described as hypomin- – Rough
eralized with an irregular prism pattern and with scalloped • Autosomal recessive
or arcading pattern of enamel. DE junction is more – Local
pronounced than in normal teeth. – Enamel agenesis
• X-linked dominant
Management
Hypocalcification type: There is defective mineraliz-
Bleaching with 30 percent H2O2 (hydrogen peroxide): ation of formed matrix.
This technique is enhanced by micro-abrasion or grinding • Autosomal dominant
of the surface layer. • Autosomal recessive
Calcium sucrose phosphate gel: Treatment involves Hypomaturation type: In this enamel crystal lattice
cleaning the affected teeth with pumice and glycerin, remains immature.
rinsing with water and applying 37 percent phosphoric • X-linked recessive
acid for 1 or 2 minutes. The treatment is repeated followed • Autosomal recessive – pigmented
by application of 2 percent sodium fluoride for 4 minute. • Snow-capped teeth autosomal dominant
Finally, a thick layer of 40 percent calcium sucrose gel is Hypomaturation/hypoplastic with taurodontism.
Teeth Anomalies
Classification vary from opaque white to translucent brown. Some of the

enamel may be missing on newly erupted teeth, especially
Classification depends upon the stage of at which the
on the incisal and occlusal surface and may be chalky
disease occurs. This classification was given by Witkop. 115
in interproximal areas. Delay in eruption occurs with
According to this classification, there are mainly four
resorption of teeth in the alveolus.
type of amelogenesis imperfecta exists.
Rough: Both primary and secondary teeth are affected.
Clinical Features Enamel is hard with rough granular surface that may be
Hypoplastic type (Fig. 8.31) chipped from underlying dentine, rather than abrade away as
It includes localized portions of enamel that do not reach seen with smooth type. Enamel is 1/4th to 1/8th in thickness.
normal thickness during development. Teeth are white to yellow-white when newly erupted. Teeth
do not meet at contact points but retain normal tooth outline
Autosomal dominant
than the smooth type of imperfecta. May have thicker enamel
Pitted: Both the dentitions are affected. It appears as at cervical areas. There is also anterior open bite present.
thin enamel on teeth that do not contact with each other
Autosomal recessive type
mesiodistally. Pinpoint to pinhead pits randomly distributed
Teeth which are erupted have distinct yellow color like of
over the surface. Enamel on newly erupted teeth is hard
normal dentition. Surface is rough and granular, resembling
with normal yellow white color. Staining of teeth occurs
ground glass.
after exposure to oral environment, giving teeth a black
appearance. Enamel agenesis: Nearly complete lack of enamel
formation. Teeth are widely spaced and do not meet each
Local: Teeth in both the dentitions are affected. Horizontal
other at contact point. Patient may have anterior open bite.
rows of depressions or one large hypoplastic area with
Numerous teeth are missing in the dentition and represent
hypocalcification adjacent to and below the hypoplastic
radiographically as unerupted teeth undergoing resorption.
area is found. Defects are most prominent on buccal
surfaces of the teeth, involving middle 3rd of enamel. Hypocalcified (Fig. 8.32)
Incisal or occlusal surfaces of the teeth are usually not The enamel is so soft that it can be removed by a prophylaxis
involved. instrument.
Smooth: Enamel is thin, hard and glossy with smooth Autosomal dominant
surface. Enamel is 1/4th to 1/8th of its normal thickness. Enamel is of normal thickness, although occasional areas
On newly erupted teeth they have yellow color, but may of hypoplasia are seen on middle 3rd of labial surface.
Figure 8.31 Amelogenesis imperfecta showing exposed Figure 8.32 Amelogenesis imperfecta (hypocalcification type)
dentin and yellow enamel
The enamel is so soft that it may be lost soon after Teeth are of normal thickness and tend to chip away,
eruption, leaving crown composed of only dentin. especially around restoration. Patient tends to form large
Enamel has cheesy consistency and can be scraped amount of calculus which may contain pigment forming
116 from dentin with an instrument or penetrated easily by agents. Teeth may be seen undergoing resorption within
dental explorer. Newly erupted teeth are covered with dull alveolus.
lusterless opaque, white, and honey colored or yellowish
Snow capped teeth
orange or brown enamel.
Location: Maxillary teeth are affected more commonly
Exposed dentin may be hypersensitive. Anterior open
than mandibular one. Both primary and secondary
bite may be present. Patients with this condition are prone
dentitions are affected.
to form calculus rapidly.
Appearance: In this condition varying amount of enamel
Hypomaturation type (Fig. 8.33)
on incisal or occlusal aspect of crown is present and has
The enamel can be pierced by an explorer point under
opaque white appearances. Opacity may be solid or
firm pressure and can be lost by chipping away from the
flecked and may involve enamel surface. Junctional line of
underlying, normal appearing dentin.
opaque white and translucent enamel is sharp.
Autosomal dominant Pattern of defect on teeth anterior to the posterior teeth
It is more commonly found in males both primary and resemble that which would be obtained when dipped into
permanent dentitions are affected permanent teeth are white paints.
mottled yellow white in color, but gradually may be
Hypomaturation type/hypoplastic with taurodontism/
darkened with absorption of stains. Primary teeth of
amelogenesis imperfecta with taurodontism
affected males have ground glass opaque white appearance.
In this type there is enamel hypoplasia with hypomaturation
Patient occasionally shows slight yellow cast to enamel
is seen. Both deciduous and permanent dentition is
surface. Teeth meet at contact points and have normal
involved.
contour. Enamel approaches normal thickness, but it may
be thinner. Point of explorer can be forced into enamel. Hypomaturation-hypoplastic pattern: In this predo-
minant defect is hypomaturation. Enamel appears as
Autosomal recessive pigmented
yellow-white to yellow brown color. Radiologically enamel
Both primary and permanent dentitions are affected.
and dentin have same density.
Enamel has milky to shiny, agar brown color on newly
erupted teeth. It may become more deeply stained on Hypoplastic-hypomaturation type: Primary defect is
contact with exogenous agents. enamel hypoplasia with thin enamel.
Amelogenesis imperfecta with taurodontism: In this
taurodontism is associated with amelogenesis imperfecta.
This is seen in Tricho-Dento-Osseous syndrome.
Hypoplastic type: There are disturbances in differentiation
or viability of ameloblasts in hypoplastic type.
Hypocalcification type: In hypocalcification, there is
defect in matrix structure and mineral deposition.
Hypomaturation type: In hypomaturation type there is
alteration in enamel rods and rod sheath structure.
Management
Figure 8.33 Brownish discoloration of teeth in amelogenesis Cosmetic improvement should be done with the help of
imperfecta (hypomaturation type) crown and veneer placement.
Teeth Anomalies
Points to Remember
• Hypoplastic type: Pitted, local, smooth, rough,
enamel agenesis disturbances in differentiation or 117
viability of ameloblasts.
• Hypocalcified: Enamel is so soft, cheesy consis-
tency hypersensitive exposed dentin defect in
matrix.
• Hypomaturation type: Affected permanent teeth
are mottled yellow white in color, pigmented, snow
capped teeth, dipped into white paints alteration in
enamel rods.
Dentinogenesis Imperfecta
There are various names for dentinogenesis imperfecta like Figure 8.34 Bluish type of discoloration seen in case of
hereditary opalescent dentin and odontogenesis imperfecta dentinogenesis imperfecta
or capdepont’s teeth.
Affected teeth are of tulip shape (broad crown with
constriction are the cervical area). suddenly narrows down. The appearance of crowns may
be described as ‘dumpy’.
Classification
Shield type II: It is inherited as an autosomal dominant
• Shield type I: Dentinogenesis imperfecta always trait. Both dentitions are affected. Other clinical features
occurs with osteogenesis imperfecta. are same, except they are somewhat of severe form and it
• Shield type II: It is also called hereditary is not associated with osteogenesis imperfecta.
opalescent dentin. It does not occur in association
with osteogenesis imperfecta. Shield type III: It is also inherited as an autosomal
• Shield type III: It is also called ‘Brandywine type’. dominant trait. Both the dentitions are affected. Opalescent
It has got shell teeth appearances and multiple pulp color, bell shaped crown and multiple pulp exposure. It has
exposure. got shell teeth appearance, i.e. normal thickness of enamel
with thin dentin in association with enlarged pulp.
Clinical Features (Fig. 8.34) Radiographic features: Teeth have bullous crown, cer-
Shield type I: It segregates as an autosomal dominant vical constriction, thin roots and early obliteration of root
trait with variable expressivity. Features of this condition canals and pulp chambers.
are multiple bone fractures, hyperextensible joints, blue
sclera and progressive deafness. Deciduous teeth are more
Histopathology Features
severely affected than permanent teeth. Color of teeth may Enamel is normal and dentin is composed of irregular
vary from brownish violet to yellowish brown. Amber dentinal tubules, with large areas of uncalcified matrix.
translucency of both primary and permanent dentition Cellular inclusions like odontoblasts are common with
may be seen. Enamel may be lost and dentin undergoes obliteration of pulp chamber. Odontoblasts degenerate
rapid attrition. Usual scalloping of dentinoenamel rapidly, becoming entrapped in the matrix.
junction is absent. The teeth are shorter than normal Tubules are larger in diameter and less numerous than
often markedly, in respect to the roots and crowns. In normal, in a given volume of dentin.
the incisor region the crowns tend to more nearly square, Atypical odontoblast cells occur at the surface of pulp.
but the mesial and distal borders are sometimes curve.
The bicuspids and molars are flatter than normal and the Management
normal circumferential curves are accentuated so that Cast metal crown in posteriors and jacket crown in anterior
the teeth shows bulbous appearance. The neck of teeth can be given.
Points to Remember
Capdepont’s teeth, tulip shape teeth, multiple bone
118 fractures, hyperextensible joints, blue sclera, brownish
violet to yellowish brown teeth, dumpy appearance of
crowns, opalescent color, bell shaped crown shell teeth
appearance, radiographically bullous crown, cervical
constriction, thin roots, irregular dentinal tubules, with
large areas of uncalcified matrix, cellular inclusions like
odontoblasts, atypical odontoblast.
Dentin Dysplasia
It is a rare disturbance of dentin formation, characterized
by normal but atypical dentin formation, with abnormal
pulp morphology. Hereditary and autosomal dominant trait Figure 8.35 Pulp is absent in case of dentin dysplasia
can lead to dentin dysplasia.
dentinal tubule formation appears to be blocked so that new
Classification dentin forms around obstacle and takes the characteristic
According to shield (clinical): appearance described as lava flowing around boulder.
• Shield type I—dentin dysplasia Electron studies (Sauk) shows this pattern results due from
• Shield type II—anomalous dysplasia. repetitive attempts to form root structure.
According to witkop (radiological): Shield type II: Deciduous teeth exhibit defective dentin
• Radicular dentin dysplasia and permanent teeth show relatively normal dentin. In
• Coronal dentin dysplasia. radicular portion dentin is amorphous and atubular and in
coronal portion dentin is relatively normal. In permanent
teeth pulp contain multiple pulp stones or denticles.
Shield type I: It is also called rootless teeth, non-opalescent Management
and opalescent dentin and radicular dentin dysplasia. Prosthetic replacement should be done for esthetic point
Permanent and primary teeth are of normal size, shape and of view.
consistency. Affected teeth are occasionally slightly amber Endodontic therapy: It is difficult in type I dentin dysplasia
and translucent. There is malalignment and malpositioning as there is no pulp canal is seen. In case of periapical
due to extreme mobility. Minor trauma may result in radiolucency it can be treated with retrograde filling.
exfoliation.
Shield type II: Significant differences in color in both the Points to Remember
dentitions. Primary teeth with yellow, brown, bluish, gray- Rootless teeth, malalignment, malpositioning, yellow
amber translucent appearances. Permanent teeth of normal brown, bluish, gray-amber primary teeth and normal
color. Obliteration of pulp chamber does not occur before color permanent teeth, no pulp, thistle tube shaped or
eruption. flame shaped, lava flowing around boulder, dentin is
Radiological features: There is no pulp or very little pulp amorphous atubular, multiple pulp stones or denticles.
is present in the deciduous teeth. There is also presence
of periapical radiolucency without any cause. In case of Regional Odontodysplasia
dentin dysplasia type there is thistle tube shaped or flame It is also called odontogenic dysplasia or ghost teeth. It is a
shaped pulp is seen. localized arrest in tooth development.
Histopathological Features Clinical Features
Shield type I: Coronal dentin is normal apically there are Location: Deciduous and permanent teeth are involved.
areas of tubular dentin which obliterates the pulp. Normal Maxillary arch involved more than mandibular. Most
Teeth Anomalies
frequently affected are permanent central incisor, lateral

incisor and cuspid. Single tooth or several teeth in one
quadrant are affected.
119
Appearance: Affected teeth are small and mottled brown
(Fig. 8.36).
Shape is irregular with evidence of defective mineralization.
Affected teeth are susceptible to caries, local infection and
prone to fracture. There is either delay or total failure of
eruption. All the elements of tooth are hypocalcified and
hypoplastic.
Radiological features: There is thin enamel and dentin
which surround the large radiolucent pulp. This will results
in pale wispy image of tooth and, hence, it is called ghost
teeth (Fig. 8.37). Figure 8.37 Ghost teeth appearance seen in regional
odontodysplasia
There is marked reduction in amount of dentin with
widening of predentin layer. Large areas of interglobular In some cases endodontic treatment on nonvital teeth
dentin and irregular tubular pattern of dentin is also yield good results.
evident.
Reduced enamel epithelium of unerupted teeth shows Points to Remember
irregular calcified bodies which is called enameloid Odontogenic dysplasia, ghost teeth, small and mottled
conglomerates. brown teeth, irregular shape, local infection, thin enamel
Scattered area of odontogenic epithelium with patterns and dentin, interglobular dentin, irregular tubular pattern
of intramural calcification is present. of dentin, enameloid conglomerates, scattered area of
odontogenic epithelium.
Management
Extraction of teeth followed by prosthetic appliances Fibrous Dysplasia of Dentin
should be done. This is autosomal dominant disease in which teeth are
normal clinically.
Radiologically there are radiodense products occupying
pulp chamber and canals. There is also presence of small
foci of radiolucency in the pulp. There is no decrease in
length of root in contrast to dentin dysplasia.
Dentin Hypocalcification
In this condition normal dentin is calcified by deposition of
calcium salts in the organic matrix in the form of globules,
which increases in size by further peripheral deposition
of salts, until all the globules are finally united into a
homogenous structure.
Etiological factors responsible for are same as that of
environmental hypocalcification of enamel.
In dentinal hypocalcification, there is failure union
Figure 8.36 Mottled brown discoloration of tooth in regional of many of these globules, leaving interglobular areas of
odontodysplasia uncalcified matrix.
There is no alteration in their clinical appearance. It divergent roots of deciduous molars. The permanent
is easily detected in both, ground section and decalcified molars which do not have predecessors also move from
histological matrix. the site of their initial differentiation.
120 2. Eruptive: There is axial or occlusal movement of
Points to Remember tooth from its developmental position within the jaw
Deposition of calcium salts, interglobular area of to its final functional position in the occlusal plane. It
uncalcified matrix. is important to recognize that jaw growth is normally
occurring while most teeth are erupting, so that
movement in plane other than axial is superimposed on
ANOMALIES ASSOCIATED eruptive movement.
WITH ERUPTION OF THE TEETH 3. Posteruptive: These movements are those that maintain
(TABLE 8.4) the position of the erupted tooth while the jaw continued
to grow and compensates for proximal and occlusal wear.
Eruption of Teeth
The axial or occlusal movement of tooth from its Theories of Tooth Eruption
developmental position within the jaw to its functional Bone Remodeling
position in the occlusal plane is known as eruption of teeth.
It is supposes that selective deposition and resorption of
There are three types of movements:
bone brings eruption. In experiments, where tooth germ
1. Pre-eruptive: When deciduous tooth germ first
is removed and the follicle is left in position the eruptive
differentiates, there is good deal of space between
pathway still forms in bone. Thus this indicates the dental
them. But due to their rapid growth, this available
follicle and not bone as major determinant in tooth eruption.
space is utilized and developing teeth become crowded
together, especially in incisor and canine region. This Root Growth
crowding is relieved by growth in length of infant jaws,
Root formation is also unlikely to be the cause of tooth
which provides room for second deciduous molars to
eruption; as the onset of root formation is not synchronous
drift backward and anterior teeth to drift forward. At
with onset of axial tooth movement. It causes overall
the same time, the tooth germ also moves outward as
increase in length of tooth that must be accompanied
jaw increases in width and height. Permanent teeth
by root growing in the bone of jaw by an increase in
with deciduous predecessors also undergo complete
jaw length or by crown moving occlusally. But it is not
movement before they reach the position from which
accepted. For example, if erupting tooth is prevented from
they will erupt.
erupting by pinning it to the bone, root growth continues
As their deciduous predecessors erupt, they move
and is surrounded by resorption of bone at base of socket.
to a more apical position and occupy their own bony
Thus although it can produce force, root growth cannot
crypt. Premolars begin their development lingual to
be translated into eruptive tooth movement unless there is
their predecessors at the level of occlusal surface and
some structure at the base of tooth, capable of withstanding
in same bony crypt. They are situated beneath the
this force. But no such structure is exists.
Advocates of the root growth theory postulated the
Table 8.4 Eruption disturbances existence of a ligament, the cushion–hammock ligament,
Anomalies affecting eruption of teeth straddling the base of the socket from one bony wall to the
• Premature eruption/predecidious dentition other like a sling. But the structure described as the cushion-
• Delayed eruption hammock ligament is the pulp delineating membrane that
• Embedded and impacted teeth runs across the apex of the tooth and has no bony insertion.
• Ankylosis or submerged teeth So it cannot act as a fixed base.
• Transposition
• Eruption sequestrum Vascular Pressure
• Ectopic eruption It states that there is a higher pressure system either
• Premature exfoliation within or around the base of tooth. It is known that
• Postpermanent dentition
teeth move in synchrony with arterial pulsation, so local
Teeth Anomalies
volume changes can produce limited tooth movement.

Whether such pressure is prime for movement of teeth
is debatable because surgical excision of the root and,
therefore, the local vasculature does not prevent tooth 121
eruption.
Periodontal Ligament Traction

There is good deal of evidence that eruptive force resides in
the dental follicle-periodontal ligament complex. As long
as periodontal tissue is available tooth movement occurs.
Tissue culture experiments have shown that ligament
fibroblast is able to contract a collagen-gel which in turn
brings about movement of a disk of root tissue attached to
that gel.
Thus there is no doubt that periodontal ligament Figure 8.38 Neonatal teeth in lowers anterior
fibroblasts have the ability to contract and transmit a
contractile force to the extracellular environment and
in particular to the collagen fiber bundles in vitro. The Classification
entire morphological features exist in vivo to permit • Mature: They are fully developed in shape and
similar movement. In summary, eruptive movement is comparable in morphology to the primary teeth.
brought about by a combination of events involving a Prognosis is relatively good.
force initiated by the fibroblasts. This force is transmitted • Immature: Their structure and development is
to the extracellular compartment via fibronexuses and to incomplete. Poor prognosis of teeth.
collagen fiber bundles; which aligned in an appropriate
inclination brought about by root formation, bring about
Clinical Features
tooth movement. These fiber bundles must have the ability
to remodel for eruption to continue and interference with Location: Usually only one or two teeth erupt early and
this ability affects the eruptive process. The removal of most often deciduous and mandibular central incisors.
bone to create the eruptive pathway is also dictated by the Appearance: They are often well-formed and normal in all
tissue surrounding the tooth. aspects, except they may be mobile.
Predeciduous Dentition Natal teeth: There is premature eruption of teeth or teeth
like structures that are present at birth. They are hyper
It is also called congenital teeth, fetal deciduous teeth,
mobile because of their limited root development. Within
dentition proceox and natal and neonatal teeth.
relatively short time, premature erupted tooth will become
The teeth erupted in the oral cavity at the time of
stabilized and other teeth of the arch are erupted. Teeth may
birth are called natal teeth and those teeth erupting
be conical or may be normal in size and shape and opaque
prematurely in 1st 30 days of life are called neonatal
yellow-brownish in color. Teeth appear to be attached to a
teeth (Fig. 8.38).
small mass of soft tissue. Some teeth are so much mobile
Etiology that there is danger of displacement and possible aspiration
and in this case, removal is indicated. Sharp incisal edges of
There is familial pattern is seen. Secretion of several
tooth may cause laceration of lingual surface of tongue or
endocrine organs like thyroid, adrenals and gonads may
may interfere with nursing. It may associate with syndromes
alter the eruption rate of teeth. Endocrinal disturbances
like Ellis-Van Creveld syndrome and cleft palate.
and adrenogenital syndrome can also cause premature
eruption. Neonatal teeth: They are teeth or teeth like structures that
Eruption accelerated by febrile incident or hormonal erupt prematurely during neonatal period, from birth to 30
stimulation. days.
Riga Fede disease: It is a complication from natal and Embedded and Impacted Teeth
neonatal teeth. There is ulceration of the ventral surface
Embedded teeth are those which are unerupted, usually
of the tongue caused by the sharp incisal edges. It leads to
122 because of lack of eruptive force.
interference with proper suckling and feeding and thus the
neonate is at risk of nutritional deficiency. Impacted teeth are those prevented from erupting by some
physical barrier in eruption path.
Most of the crowns of natal and neonatal teeth are covered Etiology
with hypoplastic enamel with varying degrees of severity. Lack of space due to crowding of dental arch and premature
Absence of root formation, ample vascularized pulp, loss of deciduous teeth with subsequent partial closure of
irregular dentin formation and lack of cementum formation the spaces they occupied.
are characteristic features. Rotation of tooth bud results in teeth which are aimed at
wrong direction because their axis is not parallel to normal
Management eruption path.
Extraction of the teeth can be done if it is causing Some systemic disease like osteopetrosis, ectodermal
inconvenience during suckling, interference with breast dysplasia, cleidocranial dysostosis, rickets and cretinism
feeding and causing traumatic injury. Extraction should be can be associated with impactions.
done after 10 days of life.
The other option that may be used is rounding of the Clinical Features
sharp angle of incisal edges of teeth. If not necessary, tooth Location: Most commonly affected teeth are maxillary and
should not be removed. mandibular third molars and maxillary cuspids, followed
by the premolars and supernumerary teeth. Teeth may be
Points to Remember
impacted distally, mesially, horizontally, etc. (Figs 8.39
Congenital teeth, dentition proceox, hyper mobile teeth, and 8.40).
teeth may be conical, possible aspiration, Ellis-Van Dentigerous cyst may be associated with impacted
Creveld syndrome, Riga Fede disease, ulceration of teeth and may cause displacement and destruction of bone.
the ventral surface of the tongue, hypoplastic enamel, Periodontal pocket formation and subsequent infections
absence of root formation, irregular dentin formation. may occur.
Because of location, impacted tooth may cause
Delayed Eruption resorption of roots of adjacent teeth. There may be periodic
Etiology pain and trismus when infection occurs around the partially
impacted teeth.
Systemic disease like rickets, cretinism and cleidocranial
Referred pain from impacted teeth is also been
dysplasia and local factors like fibromatosis gingiva in
described.
which dense connective tissue does not permit the eruption
of teeth. Management
Clinical Features It depends upon the tooth involved. In some cases, like
in maxillary cuspids, orthodontic treatment with surgical
Individual permanent teeth are observed to be delayed
exposure can be done to bring the tooth in normal occlusion.
in eruption. There may be partially impacted permanent
Surgical removal can be done.
teeth. There is deviation in the eruption path of teeth.
Patients may suffer from pseudoanodontia.
Points to Remember
Management Commonly affected maxillary mandibular third molars,
Extract the primary teeth and use space maintainers until dentigerous cyst, periodontal pocket formation, resorption
the permanent tooth erupts. of roots of adjacent teeth, referred pain.
Teeth Anomalies
abnormal pressure from the tongue can lead to ankylosis

of tooth.
Absence of periodontal ligament may also lead to
ankylosis of teeth. 123
It occurs when partial root resorption is followed by
repair; with either cementum or bone that unite the tooth
root with alveolar bone.
Ankylosis does occur after traumatic injury particularly
occlusal trauma. It may follow root canal therapy, if
the apical periodontal ligament is irritated or seriously
damaged.
Clinical Features
Location: Most commonly affected are mandibular
Figure 8.39 Inverted impacted canine deciduous second molars, followed by anterior teeth.
Exfoliation and subsequent replacement by permanent
teeth is prevented due to ankylosis. Patient who has one
or two ankylosed teeth is more likely to have other teeth
ankylosed.
Gradual loss of occlusal plane as the tooth is submerged
below the level of occlusion. Teeth affected lack mobility
even after root resorption.
On percussion it produces characteristic solid muffled
sound in contrast to dull, cushioned sound of normal teeth
on percussion.
There may be development of malocclusion, local
periodontal disturbances and dental caries occurs.
There is considerable difficulty while doing extraction
of the teeth, sometimes necessitating surgical removal.
Radiological features: There is absence of periodontal
ligament space (Fig. 8.41).
Figure 8.40 Mesioangular impacted of mandibular right and
left third molar (Courtesy: Dr Parate) Histopathological Features
There is an area of root resorption which has been
Ankylosis or Submerged Teeth
repaired by a calcified material, bone or cementum which
Submerged teeth are deciduous teeth that have undergone is continuous with the alveolar bone. The periodontal
variable degree of root resorption and then have become ligament is completely obliterated in the area of ankylosis.
ankylosed to bone.
Management
Ankylosis of the teeth should be considered as interruption
in rhythm of eruption. Unerupted permanent teeth may Keep tooth under observation. If required, surgical excision
become ankylosed by enostosis of enamel. is carried out.
Many terms like infraocclusion, secondary retention,
reimpaction and reinclusion are also used for this. Points to Remember
Disturbance in local metabolism, absence of periodontal
Etiology ligament, gradual loss of occlusal plane, solid muffled
There is disturbance in local metabolism, injury, chemical sound, malocclusion, local periodontal disturbances,
and thermal irritation, local failure of bone growth and calcified material, bone or cementum.
Management
These teeth can be prosthetically altered to improve
124 function and esthetics.
Eruption Sequestrum
Etiology
As the molar teeth erupt through bone, they will
occasionally separate small osseous fragments of bone
like corkscrew. If bony spicule is large or eruption is fast,
complete resorption cannot occur.
Clinical Features
Appearance: It is a tiny irregular spicule of nonviable
bone overlying the crown of an erupting permanent molar.
Figure 8.41 Ankylosis showing absence of periodontal
ligament space Spicule directly overlies the central occlusal fossa, but is
contained within the soft tissues.
Sign: As the tooth continues to erupt, the cusps emerge
and the fragments of bone completely sequestrate through
mucosa and are lost.
Symptoms: Child may complain of slight soreness
produced by compression of soft tissues over the spicule, by
the movement of the spicule in the soft tissue crypt during
mastication and following eruption through mucosa. For
few days, fragment of bone may be seen lying on crest of
ridge in a tiny depression which can be easily removed.
Management
Removal of spicule should be done, if it is needed.
Points to Remember
Figure 8.42 Transposition of canine with premolar Separate small osseous fragments of bone like
corkscrew, spicule of nonviable bone, fragments of bone
Transposition sequestrate, slight soreness.
Tooth may be found occupying an unusual position in
relation to other teeth, in the dental arch, i.e. two teeth Ectopic Eruption
apparently exchanging their position. If the tooth is larger than the normal mean size of all
maxillary primary and permanent teeth and smaller maxilla
Clinical Features (Fig. 8.42) or posterior positioning of maxilla in relation to the cranial
Teeth often exchange their positions. Permanent canine is base can lead to ectopic eruption.
most often involved, with its position interchanged with Some local factors like abnormal angulation and
lateral incisor. delayed calcification also lead to ectopic eruption.
Second premolar is infrequently found between first
and second molar. Transposition of central and lateral Clinical Features
incisor is rare. Transposition does not occur in primary It occurs most frequently in boys than girls. Eruption of
dentition. permanent 1st molar into roots of primary 2nd molar may
Teeth Anomalies
cause destruction of distal root of maxillary 2nd molar. 2. Antunes NL, Gorlick R, Callaja E, Lis E. Numb chin
It may become hopelessly locked and produce premature syndrome in Ewing sarcoma. J Pediatr Hematol Oncol.
exfoliation. 2000;22:521-3.
3. August M, Magennis P, Dewitt D. Osteogenic sarcoma of 125
the jaws: factors influencing prognosis. Int J Oral Maxillofac
Management
Surg. 1997;26:198-204.
You should looped brass wires in contact area. 4. Ayers KM, Colquhoun AN. Leukaemia in children. Part I:
Orofacial complications and side-effects of treatment. N Z
Premature Exfoliation Dent J. 2000;96:60-5.
5. Bartlett DW, Evans DF, Smith BG. The relationship
It is seen in Papillon lefevre syndrome, familial juvenile between gastroesophageal reflux disease and dental erosion.
periodontitis, familial fibrous dysplasia, hypophosphatasia, J Oral Rehabil. 1996;23:289-97.
cyclic neutropenia, histiocytosis and acrodynia. 6. Bennett JH, Thomas G, Evans AW, Speight PM. Osteosarcoma
There is widespread loss of supporting alveolar bone. of the jaws: a 30-year retrospective review. Oral Surg Oral
There is also loosening, migration of the teeth. In some Med Oral Pathol Oral Radiol Endod. 2000;90:323-32.
cases there is also spontaneous loss of teeth. 7. Brenneise CV, Conway KR. Dentin dysplasia, type II: report
There is no treatment, except to correct the etiological of 2 new families and review of the literature. Oral Surg
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8. Cunha RF, Boer FA, Torriani DD, Frossard WT. Natal
and neonatal teeth: review of the literature. Pediatr Dent.
Postpermanent Dentition 2001;23:158-62.
Person who have all the permanent teeth extracted and yet 9. de Alava E, Pardo J. Ewing tumor: tumor biology and
have subsequently erupted teeth, particularly after insertion clinical applications. Int J Surg Pathol. 2001;9:7-17.
of complete denture come in this category. They possibly 10. Gorsky M, Epstein JB. Craniofacial osseous and
chondromatous sarcomas in British Columbia—a review of
develop from bud of dental lamina beyond the permanent
34 cases. Oral Oncol. 2000;36:27-31.
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1. In microdontia, most commonly affected teeth are: 4. Dilacerations most commonly found in:
a. Mandibular central incisior a. Mandibular incisiors
b. Maxillary lateral incisior b. Mandibular canine
c. Mandibular first molar c. Maxillary incisiors
d. Mandibular lateral incisior d. Maxillary canine
2. ‘Syndontia’ refers to: 5. Taurodontism is associated with:
a. Fusion b. Gemination a. Klinefelter’s syndrome
c. Twining d. Concrescence b. Rubinstein-Taybi syndrome
3. Talon’s cusp is associated with: c. Down’s syndrome
a. Down’s syndrome d. Turner’s syndrome
b. Hunter’s syndrome 6. Mulberry molar commonly involves:
c. Eagle’s syndrome a. First molars b. Second molars
d. Rubinstein-Taybi syndrome c. Canines d. First premolars
7. Screwdriver shape, typical notching is the feature of: 10. Drinking water that contains in excess of 1 PPM
a. Mulberry molar fluoride results in:
b. Hutchinson’s molar a. Enamel dysplasia
126 c. Dens evaginatus b. Dentin dysplasia
d. Fournier’s molar c. Dental fluorosis
8. Tooth located between central incisors of maxilla is d. Dentin hypocalcification
called:
11. Which one of the following is also refers as “stress
a. Paramolar b. Distomolar
lesion”:
c. Mesiodens d. Peridens
a. Attrition b. Abfraction
9. Multiple unerupted supernumerary teeth seen in: c. Abrasion d. Erosion
a. Eagle’s syndrome
b. First arch syndrome 12. ‘Exostosis of roots’ refers to:
c. Gorham’s syndrome a. Hypercementosis b. Internal resorption
d. Gardner’s syndrome c. Cementicles d. Dentinal sclerosis
Craniofacial Anomalies
Anil Govindrao Ghom , Shubhangi Mhaske (Jedhe)
A Chapter Outline
3 Agnathia 3 Focal osteoporosis bone marrow defect

3 Agenesis 3 Chondroectodermal dysplasia
3 Micrognathia 3 Arhinencephaly
3 Macrognathia 3 Apert's syndrome
3 Facial hemihypertrophy 3 Stafne defect
3 Facial hemiatrophy 3 Labial and oral melanotic macule
3 Segmental odontomaxillary dysplasia 3 Fordyce granule
3 Cleidocranial dysplasia 3 Leukoedema
3 Craniofacial dysostosis 3 Caliber persistent artery
3 Mandibulofacial dysostosis 3 Lateral soft tissue fistulas
Congenital : Present at or before birth but not necessarily If both the allele at a given locus are identical to pair, it
inherited, i . e. transmitted through the genes . is called as homozygous and if the allele are different it is
Hereditary: They are apparent at birth but some may not
called as heterozygous . A gene showing trait in heterozygous
is considered as dominant . Genes are transmitted from one
become evident for years .
generation to other in a generally predictive way .
The cell consists of cytoplasm and nucleus. Nucleus of
each somatic cell contains 23 pairs of chromosome out of Autosomal dominant inheritance: Every affected person
which one is the pair of sex chromosome; rest 22 pairs of has at least one affected parent . Males and females are
chromosomes are called as autosomes . both affected likely . There is no skipping of generation.
Chromosome is a nuclear structure composed of Affected person typically transmit trait to their offsprings.
DNA, which contains units of hereditary gene. Gene is a
Autosomal recessive inheritance: Appear only in the
portion of DNA coded for the synthesis of specific protein
siblings . One-fourth siblings are affected . Males and
or polypeptide chain. Gene is determinants of hereditary
females are equally likely to be affected .
characteristics . Locus is the site occupied by the gene on
chromosome . An allele is number of alternative form that X -linked dominant : Since females have twice as many
gene may take. X-chromosomes, they exhibit a higher frequency of trait .
Affected female will transmit the gene to one-half of her

Types
offsprings.
• Apparent micrognathia: This is not due to
128 X-linked recessive: Incidence of the trait is much higher abnormality of small jaw, in terms of size but rather
in males than in females. Affected man can transmit it to to an abnormal positioning or abnormal relation
his daughters who are carriers. Trait cannot be transmitted of one jaw to another, which produces illusion of
from father to son. micrognathia.
• True micrognathia: It is due to small jaw. It is again
DEVELOPMENTAL ANOMALIES classified as:
OF JAWS – Congenital: It is present since birth
– Acquired: It is acquired later in the life.
Agnathia
It is also called hypognathous. It is a extremely rare Etiology
congenital defect characterized by absence of maxilla
Congenital: In congenital type, etiology is usually
or mandible. If mandible is absent, the upper part of the
unknown. In many instances it is associated with
face may be normal and the skin of the lower part will be
other congenital abnormalities, particularly congenital
continuous with the suprasternal integument.
heart disease and Pierre robin syndrome (cleft palate,
The hyoid bone is sometimes absent, despite the
micrognathia, glossoptosis).
presence of a rudimentary tongue. In the place of buccal
orifice, there may be a vertical slit. In case of unilateral Acquired: Acquired type is postnatal type and result from
absence of mandibular ramus, it is not unusual for ear to be disturbances in the area of TM joint (ankylosis). Mouth
deformed or absent. breathing can be a predisposing factor for maxillary
There may be absence of ears, absent or hypoplastic micrognathia. Agenesis of condyle also results in true
tongue, cleft palate, dysplastic ears, hypertelorism, mandibular micrognathia which may occur due to ankylosis
microstomia, narrow auditory canal with palpebral fissures of TMJ. Posterior positioning of mandible with regard to
slanting down. skull or to a steep mandibular angle result in an apparent
retrusion of the jaw.
Agenesis
Clinical Features
The failure of development of some part of mandible or
maxilla is termed as agenesis. Micrognathia of maxilla is due to deficiency of premaxillary
Mandible is most commonly affected than maxilla the area and patient with this deformity appears to have the
condyle, entire mandible on one side and ramus reveal the middle third of face retracted.
abnormality. In maxilla, one maxillary process or even pre- True mandibular micrognathia is uncommon and
maxilla are usually imperfect. patient appears clinically to have severe retrusion of chin,
The most common developmental defect in mandible steep mandibular angle and deficient chin button.
is the absence of condyle, in which there is no articular Micrognathia is one cause of abnormal alignment of
fossa and the eminentia articularis is either absent or teeth. This can be seen by observing the occlusion of teeth.
rudimentary. Often, there will not be enough room for the teeth to grow. If
Majority of the patients have their chin deviating the upper jaw is short, then, occlusion may also be abnormal.
towards the affected side. The ramus is small and usually In true micrognathia, the jaw is small enough to
coronoid process is absent, with the result that the interfere with feeding of the infant and may require special
anteroposterior diameter of ramus is reduced. nipples in order to feed adequately. There may be difficulty
in respiration. Due to the small size of the arch, the jaw is
Micrognathia not able to accommodate the tongue, which is forced back
into the oropharynx, blocking the air passage.
It means small jaws as compared to normal size. In this
case either maxilla or mandible may be affected. There are Syndromes associated with micrognathia: Pierre Robin
two types of micrognathia. syndrome, Hallerman-Streiff syndrome, trisomy 13, trisomy
18, Turner’s syndrome, Treacher-Collins syndrome and

Marfan’s syndrome.
Management 129
Surgery or orthodontic appliances may be recommended.
If upper jaw is short, then it can be corrected with surgical
orthodontic treatment by properly aligning the teeth and
then moving surgically and elongating the short maxilla,
in order for three to four millimeters of the upper central
incisors to show when an individual is smiling.
Macrognathia
It refers to the condition in which jaw size is larger than the
normal. It is also called as megagnathia.
Figure 9.1 Macrognathia
Etiology
It is cause by Pituitary gigantism, Paget’s disease of bone,
acromegaly and in some form of fibrous dysplasia. Points to Remember
Clinical Features Megagnathia, mandibular protrusion, size of ramus is
large, gummy smile, resection of portion of mandible.
Mandibular protrusion or proganthism is common
occurrence, which is due to disparity in the size of maxilla
to mandible and posterior positioning of maxilla in relation Facial Hemihypertrophy
to the cranium. Mandible is larger than normal which It is also called as Friedreich’s disease, hemihyperplasia.
results in increased mandibular body length (Fig. 9.1). It may involve entire half of the body, one or both limbs,
face, head and associated structures.
Points to Remember
Deficiency of premaxillary area, steep mandibular
Etiology
angle, deficient chin button, abnormal alignment of Hormonal imbalance, incomplete twinning, chromo-
teeth, blocking the air passage, Pierre Robin syndrome, somal abnormalities, localized alteration of intrauterine
Hallerman-Streiff syndrome, surgery or orthodontic development, lymphatic abnormalities, vascular abnor-
appliances. malities and neurogenic abnormalities can leads to facial
hemihypertrophy.
Gummy smile: In certain patients with congenital
abnormalities, there may be elongation of maxilla. There Types
is much “show” when the patient smiles, so that there is
a so-called “gummy” smile. This is due to the upper jaw • Complex hemihyperplasia: One entire side of body
being too long. is enlarged
Size of ramus is large, which forms less steep angle • Simple hemihyperplasia: Enlargement is limited to
with body of mandible. There is excessive condylar growth single limb
and anterior positioning of the glenoid fossa, which may • Hemifacial hyperplasia: It is confined to one side
also result in mandibular prognathism. There is prominent of face.
chin button.
Clinical Features
Management Age and sex distribution: Females are affected more than
Resection of portion of mandible should be done to decrease males. It has vague onset, usually in childhood, adolescence
the length, followed by orthodontic treatment. or early adult life.
Buccal mucosa frequently appears velvety and may hang

in soft pendulous folds on affected side.
130 Histopathological Features

Epithelium is increase in size. Connective tissue also show
hyperplasia.
Management
After patient growth is ceased, cosmetic repair by soft tissue
debulking, face lifts can be performed.
Points to Remember
Friedreich’s disease, poorly localized, vague, painful
sensation in muscles, mental deficiency, pigmentation,
Figure 9.2 Facial hemihypertrophy showing enlargement of hemangioma, Proteus syndrome, macroglossia of tongue,
one side of face epithelium is increase in size, soft tissue debulking, face
lifts.
Location: It may occur alone or in generalized hemi-
hypertrophy. It involves the eyelids, cheeks, lips, facial Facial Hemiatrophy
bones, tongue, ears and tonsils (Fig 9.2).
It is also called as Parry-Romberg syndrome, Romberg
Symptoms: Occasionally, poorly localized, vague, painful hemifacial atrophy, hemifacial microstomia and progressive
sensation in muscles affected. Enlargement of one half of facial hemiatrophy.
the head present since birth. Enlarged side grows at a rate Parry-Romberg syndrome is a rare disorder characterized
proportional to uninvolved side. by slowly progressive wasting (atrophy) of the soft tissues of
It is associated with other abnormalities like half of the face (hemifacial atrophy).
mental deficiency, skin abnormalities and compensatory
scoliosis. Hemifacial hypertrophy may be accompanied by Etiology
hemimegalencephaly, which is characterized by hypertrophy Atrophic malfunction of cervical sympathetic nervous
of one cerebral hemisphere with ipsilateral ventricular system, trauma, infection, peripheral trigeminal neuralgia,
dilatation. localized scleroderma and hereditary factors also cause
Pigmentation and hemangioma may occur on skin. Parry-Romberg syndrome.
There is progressive asymmetry due to enlargement of soft Recently, it has been observed Borrelia species.
tissues, including the lips, ear and tongue. Infection (lyme disease) can also cause facial hemiatrophy.
Syndrome associated: Syndromes associated with facial In most cases, Parry-Romberg syndrome appears to
hemihypertrophy are Proteus syndrome, Maffucci’s occur randomly for unknown reasons (sporadically).
syndrome, Ollier syndrome and Klippel-Trenaunay-Weber
Clinical Features
syndrome.
Onset: Onset noted in 1st or 2nd decades of life as a white
Oral Manifestations line furrow or mark on one side of face or eyebrow near
Size of crown, root may enlarge. Rate of development of midline. In rare cases, the disorder is apparent at birth.
permanent teeth on the affected side is more rapid and erupt Location: In most cases, progressive tissue wasting is
before their counterparts on the uninvolved side. Primary teeth limited to one-half of the face, usually the left side.
shed early.
Bone of maxilla or mandible is also enlarged. Progress: In most affected individuals, hemifacial atrophy
Unilateral macroglossia of tongue: Tongue is commonly typically progresses over approximately three to five years
involved and bizarre patterns of enlargement of papilla, in and then ceases. Affected areas may demonstrate shrinkage
addition to unilateral enlargement of tongue. and atrophy of tissues beneath the skin (subcutaneous
tissue), in the layer of fat under the skin (subcutaneous fat) Malocclusion: There is reduced growth of jaws and
and in underlying cartilage, muscle and bone. eruption of teeth is retarded. There is also malocclusion on
the affected side.
en coup de sabre (strike of sword): Sharp line of 131
demarcation resembling a scar between normal and Histopathological Features
abnormal near midline of forehead known as linear
scleroderma. There is atrophy of epidermis with perivascular infiltrate
of lymphocytes and monocytes. Degenerative changes in
Skin: In addition, the skin overlying the affected areas may vascular endothelium.
become darkly pigmented (hyperpigmentation) with, in
some cases, certain areas of white (depigmented) patches Management
(vitiligo).There may be hollowing of cheek and eyes may Orthodontic treatment, plastic surgery and hearing aids are
appear depressed in the orbits. recommended.
Neurological manifestation: This may include severe
headache that lasts for extended periods of time and Points to Remember
may be accompanied by visual abnormalities, nausea Parry-Romberg syndrome, lyme disease, tissue wasting
and vomiting (migraine). There is also facial pain due to one-half of the face, shrinkage and atrophy of tissues
trigeminal neuralgia. There are periods of uncontrolled beneath the skin, en coup de sabre (strike of sword),
electrical disturbances in brain (seizures) that usually hyperpigmentation, vitiligo, seizures, contralateral
are characterized by rapid spasms of a muscle group that Jacksonian epilepsy, loss of eyelashes, soft tissue of face
spread to adjacent muscles (contralateral Jacksonian are small, aplasia or hypoplasia of external ear, atrophy
epilepsy). of half of the upper lip, delayed eruption, malocclusion,
atrophy of epidermis with perivascular infiltrate of
Hair: There is graying (blanching) of hair as well as lymphocytes.
abnormal bald patches on the scalp with loss of eyelashes
and the middle (median) portion of eyebrows (alopecia).
Segmental Odontomaxillary Dysplasia
Base of skull: There is underdevelopment of the base of It is also called hemimaxillofacial dysplasia. It can be
skull in some cases whose face is affected. confused with craniofacial fibrous dysplasia or hemifacial
Malar bones: When the malar bone is small, the side of the hyperplasia.
face is flat but an absent malar bone produces a depression
inferior to the orbit. Clinical and Radiological Features
It is discovered in childhood.
Face: The soft tissue of face are small and thinner than
normal. Sign: There is painless unilateral enlargement of the
maxillary bone.
Ear: Aplasia or hypoplasia of external ear. The ear canal
is missing. There may be fibrous hyperplasia of overlying gingival
soft tissue.
Oral Manifestations
Primary teeth in the affected area may be hypoplastic
Lip and tongue: Many individuals also experience atrophy showing enamel defect.
of half of the upper lip and tongue.
Radiographic features: There is vertically oriented
Teeth: Delayed eruption or wasting of the roots of certain thickened trabecular which results in granular appearance.
teeth on the affected side. The maxillary sinus is smaller on affected side.
Jaws: In individuals with the disorder, initial facial changes
usually involve the tissues above the upper jaw (maxilla) or Histopathological Features
between the nose and the upper corner of the lip (nasolabial Gingival soft tissue may show fibrosis. Affected bone
fold) and progress to involve the angle of mouth, the areas consists of irregular trabeculae with woven appearance.
around the eye, brow, ear and/or the neck. There are also presences of resting and reversal lines.
Management Lacrimal and zygomatic bone are also underdeveloped.

Sagittal suture is characteristically sunken, giving skull a
Orthodontic therapy and orthognathic surgery can be
flat appearance.
132 performed in this case.
Oral Manifestations
Points to Remember
Maxilla and paranasal sinus are underdeveloped, resulting
Hemimaxillofacial dysplasia, fibrous hyperplasia, hypo-
in maxillary micrognathia. Maxilla is underdeveloped and
plastic primary teeth, vertically oriented thickened
smaller than normal, in relation to mandible.
trabecular, irregular trabeculae with woven appearance.
Unerupted teeth: Prolonged retention of primary dentition
Cleidocranial Dysplasia and delayed eruption of permanent dentition. Numerous
unerupted teeth are found which are most prevalent in the
It is also called as cleidocranial dysostosis, Marie and
mandibular premolar and incisor area (Figs 9.4 and 9.5).
sainton disease, craniocleido-dysostosis. Hereditary and
when inherited, it appear as a true dominant Mendelian
characteristic incomplete penetration of genetic trait.
Clinical Features
The disease affects men and women with equal frequency.
Location: It primarily affects skull, clavicle and dentition.
There may be complete absence of clavicle and patients
have unusual mobility. They are being able to bring their
shoulders forward until they meet in midline (Fig. 9.3).
The head is brachycephalic (reduced anterior-posterior
dimension but increased skull width) or wide and short.
Nasal bridge is depressed with a broad base. In skull
the fontanels often remain open or at least exhibit delayed
closing and for this reason, tend to be rather large. Open
skull suture and multiple wormian bones are present and
there is occasional stunting of long bone. Figure 9.4 Patient showing unerupted teeth
Figure 9.3 Patient showing unusual mobility of shoulder in Figure 9.5 Over retained deciduous teeth in cleidocranial
cleidocranial dysplasia dysplasia
There is paucity and complete absence of cementum, Mandible show coarse trabeculation with areas of
crowding and disorganization of developing permanent increased density.
dentin and presence of supernumerary teeth usually in
anterior region. Histopathological Features 133
High and narrow arched palate and cleft palate may be Microscopic study of unerupted teeth shows that is lack
common. Roots of teeth are often some what short and thinner secondary cementum.
than the normal. Maxilla is small and mandible is usually Some author stated that insufficient alveolar bone
normal in size, which gives the appearance of prognathism. resorption also lead to impaired tooth eruption.
The crown may be pitted as a result of enamel hypoplasia.
Management
Radiological Features Full mouth extraction with denture construction,
PA chest view will show absence of clavicle (Fig. 9.6). autotransplantation of selected impacted teeth followed by
Radiograph shows suture and fontanels having prosthetic restoration should be done.
delayed closure or sometime they remain open throughout
the life. Wormian bone can also be seen (Fig. 9.7). Points to Remember
Marie and sainton disease, absence of clavicle,
brachycephalic, nasal bridge is depressed, flat appearance
of skull, maxillary micrognathia, unerupted teeth,
paucity and complete absence of cementum, high narrow
arched palate, enamel hypoplasia, mandible show coarse
trabeculation, and lack secondary cementum.
Craniofacial Dysostosis
It is also called as Crouzon’s disease or syndrome. In some
instances, Crouzon syndrome is inherited as an autosomal
dominant trait. In other cases, affected individuals have
no family history of disease. The disorder is characterized
by distinctive malformations of the skull and facial
(craniofacial) region.
Figure 9.6 Cleidocranial dysplasia showing clavicle Clinical Features

deficiency appreciated in radiograph Age: Crouzon’s syndrome is a rare genetic disorder that
may be evident at birth (congenital) or during infancy.
In most infants with Crouzon syndrome, the fibrous
joints between certain bones of the skull (cranial sutures)
close prematurely (craniosynostosis). The premature closing
results in brachycephaly (short head), scaphocephaly (boat
shaped head) and trigonocephaly (triangle shaped head).
In addition, facial abnormalities typically include
unusual bulging or protrusion of the eyeballs (proptosis)
due to shallow eye cavities.
Outward deviation of one of the eyes (divergent
strabismus or exotropia); widely spaced eyes (ocular
hypertelorism). Exophthalmos (protrusion of eyes) with
divergent strabismus and optic neuritis and choked disks,
resulting frequently in blindness are also present.
Bulging of frontal bone in midline, over the nose and
Figure 9.7 Open sutures of skull in cleidocranial dysplasia downward sloping of back of head (Fig. 9.8).
Unilateral or bilateral cross-bite is evident with open

bite and crowding in mandibular teeth.
Shovel shaped maxillary incisors, cleft lip and palate
134 are also evident. The antrum is small and underdeveloped.
The mandible is large as compared to maxilla, so there is
prognathism.
Management
Maxillofacial surgery for correction of facial deformities
should be done.
Points to Remember
Craniosynostosis, brachycephaly (short head), scapho-
cephaly (boat shaped head) and trigono cephaly
Figure 9.8 Patient is having Crouzon’s syndrome. Note (triangle shaped head), proptosis, divergent strabismus
parrot beak and bulging of frontal bone in midline or exotropia, ocular hypertelorism, protrusion of eyes,
bulging of frontal bone in midline, triangular frontal
defect, maxillary hypoplasia, parrot beak, highly arch
palate, unilateral or bilateral cross-bite, shovel shaped
maxillary incisors cleft lip.
Mandibulofacial Dysostosis
It is also called Treacher Collin syndrome and ‘Franceschetti
syndrome. It is often inherited as autosomal dominant trait.
It results from retardation or failure of differentiation of
maxillary mesoderm at and after the 50 mm stage of the
embryo.
Clinical Features
There is underdevelopment of zygomatic bone, resulting in
midfacial deformities.
Craniofacial malformations associated with Treacher
Figure 9.9 High arch with cross bite in patient with Crouzon’s Collins syndrome include underdeveloped (hypoplastic) or
syndrome absent cheek (malar) bone. There is downward inclination
of palpebral fissure. There is deficiency of eyelashes. In
some cases, eyes assume corresponding slant.
There is protuberant frontal region with an anterior- A notching (colobomas) from the outer third of lower
posterior ridge overhanging the frontal eminence and often eyelids, There is varying degree of visual impairment in
passing to the root of nose (triangular frontal defect). some cases.
Affected infants may also have underdeveloped
Oral Manifestations (hypoplastic) and/or malformed (dysplastic) ears (pinnae)
Maxillary hypoplasia with shortened anteroposterior with blind ending or absent external ear canals (microtia),
dimension of maxillary arch is present. Dental arch width resulting in hearing impairment (conductive hearing
is reduced and this gives an appearance of highly arch loss). There is absence of external auditory canal resulting
palate (Fig. 9.9). in partial or complete deafness of patients (Fig. 9.10).
In some cases, facial angle is exaggerated and the The normal prominence of cheek is either missing or
patient nose is prominent and pointed, resembling parrot reduced depending upon the presence or absence of malar
beak. bone. There is usually hypoplasia of malar bone.
An incompletely developed, abnormally small lower

jaw and macrostomia (an unusually large mouth) can also
occur. There is presence of high arch palate with cleft
palate, abnormal position and malocclusion of teeth with 135
anterior open bite.
Radiographic Features
It demonstrated hypoplasia of condyle and coronoid
process with prominent antegonial notching.
Management
Cosmetic improvement and surgical interventions to
improve osseous and ear defect is done.
Figure 9.10 Ear deformity seen in case of Treacher Collins

Points to Remember
syndrome Treacher Collin syndrome, underdevelopment of
zygomatic bone, hypoplastic or absent cheek bone,
deficiency of eyelashes, colobomas, malformed ears,
hypoplasia of malar bone, lower anterior teeth stand
away, bird face appearance, macrostomia, hypoplasia of
condyle.
Focal Osteoporosis Bone Marrow Defect

They are derived from bone marrow hyperplasia of
persisting embryonic marrow remnants and site of
abnormal healing following extraction, trauma and local
inflammation.
Sex predilection: It is more common in females than

males.
Figure 9.11 Bird fish appearance seen in Treacher Collins Location: It is common in molar, premolar region in
syndrome mandible.
Radiographic features: It is incidentally found on
Other features are secondary mental deficiency, blind radiograph. It appear as radiolucent, circumscribed area
fistulae between the angle of ears and the angle of mouth. from several millimeter to centimeter in diameter (Fig.
Associated features: Anal atresia/stenosis, congenital 9.12).
cardiac anomaly, rectovaginal fistula and tracheo-
esophageal fistula. Histopathological Features
Bone marrow contain cellular hemopoietic component
Oral Manifestations which may show variable fatty components. In some cases
There is underdevelopment of mandible with steep lymphoid aggregates may be present.
mandibular angle. Due to this, lower anterior teeth stand
away from upper teeth, when the mouth is closed. Management
Bird face appearance: Facial appearance sometimes There is no specific treatment for it after diagnosis is
resembles fish or bird (Fig. 9.11). confirmed.
136
Figure 9.12 Focal osteoporotic bone defect showing Figure 9.13 Chondroectodermal dysplasia showing extra
radiolucent circumscribed area finger
irregular in position. The teeth are affected, with eruption

Points to Remember
occurring at birth or shortly thereafter. The deciduous
Persisting embryonic marrow remnants, radiolucent, molars present a crenate occlusal surface. The permanent
circumscribed area, cellular hemopoietic component, dentition is more likely to be defective than the deciduous
lymphoid aggregates. one. Eruption is delayed.
The lip deformity often referred to as a partial hairlip
Chondroectodermal Dysplasia results from an abnormally short upper lip, which may also
It is also called as Ellis-van-Creveld disease. be sunken secondary to hypoplasia of maxilla.
Clinical Features Management

It is congenital and the patient present evidence of No treatment is necessary. Dental care of patient should
chondrodysplasia, ectodermal dysplasia, polydactylism be done.
and congenital morbus cordis.
Post-axial polydactyly in the hands, i.e. an extra finger Points to Remember
lateral to the normal fifth finger, is a consistent finding Ellis-van-Creveld disease, chondrodysplasia, ectodermal
(Fig. 9.13). Polydactyly in the feet is a rare finding. dysplasia, polydactylism, congenital morbus cordis,
Bone dysplasia is characterized by acromesomelia, i.e. post-axial polydactyly, bone dysplasia, congenital heart
relative shortening of the distal (acromelic) and middle defects, dwarfism, teeth is deficient, partial hairlip.
(mesomelic) segments. It may interfere with the ability to
make a tight fist. There may be a deformity of the knees that
frequently progress and causes a significant malalignment. Arhinencephaly
The nails of the fingers and toes are dysplastic. The hairs It is a developmental abnormality of the skull and face
tend to be fine and sparse. in which there is absence or deficiency of the olfactory
Congenital heart defects may include hypoplasia of aorta, portion of brain.
atrial and ventricular septal defects and a single atrium. Absence of the vertical and cribriform plates of ethmoid
There is dwarfism, i.e. the long bones being short and and of crista galli result in the orbits being more closely set,
the trunk of normal length. a condition known as hypotelorism.
There is depression of nose and absence of the bridge
Oral Manifestations because of lack of nasal bones and nasal septum.
Teeth: Teeth are deficient in number; those which do There is wide cleft in the central portion of the lip, where
develop are small, rudimentary, conical, spaced and the philtrum is absent and with failure of development of
any part of the premaxilla, the cleft is continuous through Management

the palate.
Surgical management: Cosmetic and functional defect
In some cases an associated deformity of the frontal
is treated by interdisciplinary approach using multiple 137
portion of skull presents an angular appearance resembling
surgical procedures.
the prow of a boat presumably due to premature fusion of
metopic sutures. Craniotomy: It is performed in first year of life to treat
craniosynostosis.
Points to Remember
Points to Remember
Hypotelorism, wide cleft, depression of nose, failure of
development of premaxilla, prow of a boat’ appearance. Acrocephalosyndactyly, craniosynostosis, ocular proptosis,
hypertelorism, syndactylism, cutaneous fusion of fingers,
high palatal vault, uvular clefts, dental malocclusion,
Apert’s Syndrome trapezoid lip, craniotomy.
It is also called as acrocephalosyndactly. Acrocephalosyn-
dactyly is believed to be transmitted by an autosomal dom- Stafne Defect
inant gene occurring sporadically in about 1–20,00,000 of
It is also called as Stafne bone cyst, lingual mandibular
the general population. It is characterized by craniosynos-
salivary gland depression, latent bone cyst, static bone cyst
tosis.
and lingual cortical mandibular defect.
It is discovered in 1942 by Stafne. This represents focal
Clinical Features
concavity of the cortical bone on the lingual surface of the
Craniosynostosis: It can produce acrobrachycephaly mandible.
(tower skull), kleeblattschadel deformity (cloverleaf
skull). Skull often presents a horizontal supraorbital Clinical Features
grove. Premature closure of the sutures occur and the Age: It is commonly noticed in middle aged and older
often and anterior fontanelle is open due to late closure. adults.
The occiput is flattened and tall appearance to the
forehead is seen. Location: It is most commonly seen in posterior mandible
in the location of salivary gland. In some cases this can be
Ocular lesion: There is ocular proptosis, hypertelorism seen in anterior region where sublingual gland is present.
and downward slanting of palpebral fissure. Patient may be Rarely parotid gland can cause depression in the area of
having visual loss which can occur due to chronic exposure ramus of the mandible.
of the unprotected eye, increased intracranial pressure and
Sign and symptoms: It is asymptomatic and remain stable
compression of optic nerve.
in size so bears a name static bone cavity.
Limb defect: Features of hand and feet include Radiological features: There is radiolucent defect below
syndactylism. Osseous or cutaneous fusion of fingers is the mandibular canal in the posterior mandible between
usually present. molar teeth and angle of the mandible. Lesion is well-
defined with sclerotic border (Fig. 9.14).
Oral Manifestations
Oral features include a high palatal vault and the presence Histopathological Features
of posterior palatal and uvular clefts. In the defect normal submandibular gland tissue can be
Dental malocclusion is consistent. Other oral features seen. In some cases no tissue can be found assuming that
include mandibular proganthism, malocclusion and gland has displaced at the time of biopsy.
retarded eruption.
Trapezoid lip: This appearance occur when lips are Management
relaxed and result due to midface hypoplasia and mouth No treatment is necessary as it is asymptomatic and does
breathing. not increase in size.
138
Figure 9.14 Stafne’s bone cyst presented as radiolucency Figure 9.15 Multiple melanotic macule present on lower lip
below the mandibular canal and surrounded by well corticated
border
Points to Remember Increase amount of melanin in basal cell layer. Also there
Stafne bone cyst, posterior mandible, asymptomatic, is melanin in lamina propria.
radiolucent defect below the mandibular canal, normal Increase number of clear cells and dendritic cells are
submandibular gland tissue. also found. Melanophagocytosis can also be seen. Normal
stratified squamous epithelium is seen in this case.
There is presence of melanin incontinence (deposits in
DEVELOPMENTAL DISORDERS OF subepithelial stroma) in macrophages or melanophages.
ORAL MUCOSA The melanin can be distinguished from iron deposits by the
Labial and Oral Melanotic Macule loss of brown color after bleaching.
It is also called as focal melanosis or solitary labial Management

Lentigo. It represents an increase in synthesis of melanin Excision biopsy, electrocautery, laser ablation or
pigments by basal cell layer melanocytes without increase cryosurgery can be done.
in the number of melanocytes. It is the most common
pigmentation to occur in oral cavity of light skinned Points to Remember
individuals. It is not depended on sun exposure.
Focal melanosis, actinic exposure, small, flat macule,
Clinical Features lesion is less than 1 cm, increase melanin pigmentation
in basal cell layer, clear cells, dendritic, melanin
Age and sex distribution: Mean age is 41 to 45 years. incontinence.
Equal occurrence in both sexes.
Location: It is attributed to actinic exposure and therefore Fordyce Granule
occurs on vermilion border of the lower lip. Sometimes it
A Fordyce granule is a developmental anomaly
can also occur on gingiva, palate and buccal mucosa.
characterized by heterotrophic collection of sebaceous
Appearance: It present as small, flat macule which may glands at various sites in oral cavity which is covered with
be single or multiple. Color of pigmentation is brown or intact mucosa.
brown black.
Sign: Solitary lesion is less than 1 cm in diameter and Pathogenesis
constant in size. Lesions are oval or irregular in outline. It It has been postulated that the occurrence of sebaceous
is an asymptomatic condition (Fig. 9.15). glands in the mouth may result from the inclusion in the
It consists of submucosal cluster of sebaceous acini and
communicated with the oral epithelium by the way of duct 139
(Figs 9.17 and 9.18).
In some cases all are grouped around one or more duct
which opens on the surface of mucosa. These ducts may
show keratin plugging.
They are identical with those seen with normal
sebaceous gland on the skin except for the absence of hair
follicles.
Figure 9.16 Fordyce granules seen on buccal mucosa
oral cavity of the ectoderm having some of the potentialities

of the skin in the course of development of the maxillary
and mandibular processes during embryonic life.
Clinical Features
Age and sex distribution: It is seen in any age group
with somewhat more prevalent in males as compared to
females.
Location: It is most commonly found bilaterally in
symmetrical pattern on mucosa of the cheek, opposite to Figure 9.17 Sebaceous gland showing pilosebaceous tract
the molar teeth. It is also found on inner surface of lips, (Courtesy: Dr Sangamesh Halawar, Reader, Deptt of Oral
in retromolar area lateral to anterior faucial pillar and Pathology, VPDC and H. Kavalapur, Sangli, Maharashtra)
occasionally on the tongue, gingiva, frenum and palate.
Appearance: They appear as small yellow spots, either
discretely separated or forming relatively large plaques
often projecting slightly above the surface of tissue (Fig.
9.16). The granules may be isolated or they may occur in
confluent sheets. Sometime they may occur in clusters and
may form plaque like lesions.
On the tongue it appears as dome shaped nodules
varying in size from a few millimeters to 2 cm in diameter
on the midline dorsum of the tongue.
They are more yellow than white. It increases rapidly
in number at puberty and continues to increase through
the adult life. They are neither ectopic nor adenomas and
usually are submucosal. They are sharply delineated and
with smooth surface which is not ulcerated. They have got
slightly cheesy consistency. Figure 9.18 Fordyce’s granule
Management
If it causes disfigurement then surgical removal can be
140 done.
Points to Remember
Heterotrophic collection of sebaceous glands, bilaterally
in symmetrical pattern on mucosa of the cheek, small
yellow spots, on tongue dome shaped nodules, more
yellow than white, cheesy consistency, submucosal
cluster of sebaceous acini, keratin plugging.
Leukoedema
It is an abnormality of the buccal mucosa, which clinically
resembles early leukoplakia.
Figure 9.19 Leukoedema affecting buccal mucosa
It has got prevalence in blacks due to presence of
background pigmentation that makes edematous changes
noticeable. It is seen more commonly in a person who is Management
useing tobacco. It may be related to poor oral hygiene.
No treatment is necessary as it has not got any pre-
Clinical Features malignant potential.
Age and sex distribution: It is common in age group of Points to Remember
15 to 35 year with prevalence in black. Male predilection
Common sites are buccal mucosa and lip, velvetlike
in the ratio of 2:1.
veil appearance, Mother of pearl appearance, eliminated
Location: The most common sites of involvement are by the stretching and scraping of mucosa, thickness
buccal mucosa and lip. The lesion is bilateral. of epithelium, broad rete pegs, intracellular edema of
spinous layer, pyknotic nuclei.
Appearance: Buccal mucosa retains the normal softness
and flexibility but exhibits grayish white, slightly folded
opalescent appearance that is described as epithelium Caliber Persistent Artery
covered with diffuse edematous film or velvetlike veil It is vascular anomalies where main arterial branch reached
(Fig. 9.19). the superficial surface without reduction in size.
Mother of pearl appearance: In some cases, lesion is Clinical Features
diffuse and shows a filmy, mother of pearl appearance,
often with delicate overlapping curtain like mucosal folds. Age and sex distribution: It is more commonly seen in
In this disease desquamation may occur which may older individuals without any sex predilection.
leave surface of the lesion eroded. It can be eliminated by Location: It is most commonly seen on lip mucosa with
the stretching and scraping of mucosa but re-establishes some lesions have bilateral or involving both lip.
itself almost immediately.
Appearance: It appears as linear, arcuate or papular
Histopathological Features elevation. This can be of black to bluish in color.
There is increase in thickness of epithelium, broad rete Sign: Stretching of lip can cause artery inconspicuous. There
pegs and intracellular edema of spinous layer. is presence of vertical pulsation or pulsatile lip nodules. In
Cells at the surface are flattened and may retain pyknotic some cases there is ulceration of overlying mucosa.
nuclei that contain glycogen. The characteristic edematous
cells appear extremely large and pale, and they present a Histopathological Features
reticular pattern. There is thick wall artery located close to surface.
Management (Eds): Dermatology, 2nd edn. New York, Springer-Verlag

Berlin Heidilberg; 2000.pp.773-74.
No treatment is necessary for this. Sometime if biopsy is 6. Bufalino A, Paranaíba LM, Gouvêa AF, Gueiros LA.
performed for mistaken diagnosis, there is brisk bleeding Cleidocranial dysplasia: oral features and genetic analysis 141
from the lesion. of 11 patients Oral Dis. 2012;18(2):184-90. doi: 10.1111/
j.1601-0825. 2011.01862.x. Epub 2011 Oct 24.
Points to Remember 7. Burglen L, Soupre V, Diner PA, Gonzales M, Vazquez MP.
Lip mucosa, linear, arcuate or papular elevation, Oto-mandibular dysplasias: genetics and nomenclature of
stretching of lip can cause artery inconspicuous, thick syndromes. Ann Chir Plast Esthet. 2001;46:400-9.
wall artery. 8. Cleidocranial Dysplasia. Mendoza-Londono R, Lee B.
Editors In: Pagon RA, Bird TD, Dolan CR, Stephens K,
Adam MP, (Eds). SourceGeneReviews™ [Internet]. Seattle
Lateral Soft Tissue Fistulas
(WA): University of Washington, Seattle; 1993-. 2006
It can occur due to defect in development of second [updated 2009 Jun 25].
branchial arch. It is very rare anomaly. 9. CN, Shakuntala BS, Mathew S, Krishnamurthy NH,
Yumkham R. Cleidocranial dysplasia presenting with
Clinical Features retained deciduous teeth in a 15-year-old girl: a case report.
Location: They are bilaterally seen on anterior tonsillar J Med Case Rep. 2012;6(1):25.
10. Demendi C, Németh M, Langmár Z. Orv Hetil. Congenital
pillar. Sometime they can also be seen on posterior pillar.
disorders. Holoprosencephalia. 2011;152(52):2105-8.
Appearance: There is perforation ranging from few mm to 11. Danforth RA, Melrose RJ, Abrams AM, Handlers JP.
1 cm in diameter. Segmental odontomaxillary dysplasia: Report of eight cases
and comparison with hemimaxillofacial dysplasia. Oral Surg
Sign and symptoms: It is asymptomatic and some cases Oral Med Oral Pathol. 1990;70:81-5.
may be associated with absence or hypoplasia of palatine 12. Golan I, Baumert U, Hrala BP, Müssig D. Dentomaxillofacial
tonsil, hearing loss and preauricular fistulas. variability of cleidocranial dysplasia: clinicoradiological
presentation and systematic review.
Management 13. Dervis E, Dervis E. Progressive facial hemiatrophy with
No treatment is necessary for this as the lesion is linear scleroderma. Ped Dermatol. 2005;22(5):436-9.
asymptomatic. 14. Diego Mauricio Bravo-Calderón, Denise Tostes Oliveira,
Wagner Humberto Martins dos Santos. Bilateral osteoporotic
Points to Remember bone marrow defects of the mandible: a case report Head
and Face Medicine. 2012;8:22.
Anterior tonsillar pillar, perforation, hearing loss and
15. Drake DL. Segmental odontomaxillary dysplasia: an unusual
preauricular fistulas. orthodontic challenge. Am J Orthod Dentofacial Orthop.
2003;123:84-6.
16. De Felice C, Parrini S, Chitano G, Gentile M, Dipaola L,
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1. ‘Friedreich’s disease’ is: 6. ‘Parrot beak’ appearance of nose seen in:

a. Facial hemihypertrophy a. Franceschetti syndrome
b. Macrognathia b. Parry-Romberg syndrome
c. Genesis c. Marie Sainton disease
d. Micrognathia d. Crouzon’s disease
2. Following are associated with micrognathia except: 7. Chondroectodermal dysplasia is also known as:
a. Pierre-Robin syndrome a. Ellis-van Crevald disease
b. Turner’s syndrome b. Treacher Collins syndrome
c. Marfan’s syndrome c. Crouzon’s disease
d. Costen’s syndrome d. None
3. ‘Hypognathus’ means: 8. In oral melanotic macule there is:
a. Micrognathia a. Decrease in synthesis of melanin pigments
b. Macrognathia b. An increase in synthesis of melanin pigments
c. Agnathia c. An increase in the number of melanocyte
d. Facial hemiatrophy d. Decrease in the number of melanocyte
4. ‘Parry Romberg’ syndrome refers to: 9. Deficiency of eyelashes seen in the following except:
a. Macrognathia a. Crouzon’s disease
b. Facial hemiatrophy b. Treacher Collins syndrome
c. Micrognathia c. Mandibulofacial dysostosis
d. Agnathia d. Franceschetti syndrome
5. Complete absence of clavicle seen in: 10. Keratin plugging seen in:
a. Mandibulofacial dysostosis a. Focal epithelial hyperplasia
b. Arhinencephaly b. Fordyce’s granule
c. Craniofacial dysostosis c. Oral melanotic macule
d. Cleidocranial dysplasia d. Crouzon’s disease
Dental Caries
Anil Govindrao Ghom , Shubhangi Mhaske (Jedhe)
A Chapter Outline
3 Definition 3 Nursing bottle caries

3 Theories of cariogenesis 3 Rampant caries
• The legend of worm 3 Arrested caries
• Chemical theory 3 Pre- eruptive caries
• Humoral theory 3 Caries activity tests
• Vital theory • Lactobacillus count test
• Miller' s acidogenic/ chemoparasitic theory • Snyder test
• Proteolysis theory • Alban' s test
• Proteolysis chelation theory • Streptococcus mutans level in saliva
• Sucrose chelation theory • Buffer capacity test
• Autoimmune theory • Fosdick calcium dissolution test
3 Secondary contributing factors in dental caries • Dewar test
3 Classification • Swab test
3 Smooth surface caries • Reductase test
3 Pit and fissure caries 3 Control of dental caries
3 Root caries 3 Chemical measures of caries control
3 Recurrent caries
INTRODUCTION DEFINITION
Dental caries is a complex, continuous, dynamic biologi- Dental caries is defined as a progressive irreversible
cal process of tooth decay with multifactorial etiology. The microbial disease affecting the hard parts of tooth exposed
process of dental decay comprises of periods of progres - to the oral environment, resulting in demineralization of
sion alternating with periods of arrest and repair of affected the inorganic constituents and dissolution of the organic
dental tissues . The word caries derived from Latin meaning constituent, thereby leading to a cavity formation .
4
rot’ or decay .
Dental Caries
THEORIES OF CARIOGENESIS Chemical Theory

These are the current concepts of caries pathogenesis. Parmly (1819) proposed that an unidentified chemical agent
These are based on sound experimental evidences. Among was responsible for caries. He stated that caries began on 145
these Miller’s acidogenic theory is most accepted one. enamel surface where food putrefied and acquired sufficient
dissolving power to produce the disease chemically.
History/Old Theories
Humoral Theory
Exogenous theories
• The legend of worm According to this ancient theory the four humors of the
• Chemical theory body are blood, phlegm, black bile and yellow bile and any
• Parasitic or septic theory change in the relative proportion of these elements causes
Endogenous theories disease. Similarly, dental caries is caused by the change in
• Humoral theory these humors.
• Vital theory. According to Greek physician and philosopher Gallen,
Dental caries is produced by the internal action of acid and
corroding humors. Hippocrates, the father of medicine,
Theories of Etiopathogenesis while favoring the concept of humoral pathology also
• Miller’s acidogenic theory referred to the accumulated debris around teeth and to their
• Proteolytic theory corroding action. He also stated that the stagnation of food
• Proteolysis-chelation theory and juices was the cause of the decay.
• Sucrose chelation theory.
Vital Theory
The Legend of Worm The theory stated in 18th century says that tooth decay
originated like bone gangrene, from within the tooth itself.
In ancient Sumerian text, it is stated that dental caries is
caused by a worm that drank the blood of the teeth and Miller’s Acidogenic/Chemoparasitic Theory
fed on the roots of jaws. This is known as the legend of
WD Miller proposed this theory in 1890. He based his
worms.
theory on observations of following investigators:
It was obtained from the Mesopotamian area which
∙ Pasteur discovered that microorganisms can transform
dates to about 5000 BC. This theory is supported in the
sugars into lactic acid.
ancient literature of India, China, Finland, Scotland and the
∙ Emil Magitot demonstrated that fermentation of sugars
writing of Homer (Fig. 10.1).
can cause dissolution of tooth mineral in vitro.
∙ Underwod and Miles stated that caries is absolutely
dependent on the presence of organisms that create an
acid which removes the lime salt.
WD Miller’s chemoparasitic theory states, “Dental
decay is a chemoparasitic process consisting of two stages,
the decalcification of enamel, which results in its total
destruction and the decalcification of dentin as a preliminary
stage, followed by dissolution of the softened residue.”
The chemoparasitic theory can be better described with
the role of the following factors together causing decay:
Factors Responsible for Chemoparasitic Theory

• Role of carbohydrates
• Role of microorganisms
• Role of acids
• Role of dental plaque.
Figure 10.1 Legend of worm theory
Role of Carbohydrate ∙ Other polysaccharides synthesized by the cariogenic

bacteria are glucan (from glucose) and levan (from
Various food substances can directly or indirectly affect
fructose). Glucan and levan are weakly adhering
146 dental caries. Food substances act as substrates for
substances so are less cariogenic.
microorganisms of dental plaque, which form acids,
thereby causing dental caries. Other substances may affect Route of administration: Glucose or sucrose adminis-
microflora in negative way thus protecting the tooth from tered intravenously or by gastric tube do not contribute to
caries. decay as they are unavailable for microbial breakdown,
Among these carbohydrates is main cause of dental thus oral intake of sticky carbohydrates are found to be the
caries. There is considerable increase in caries incidence cause behind the dental decay.
after exposure of modern civilization to refined foods. This
Presence of other food constituents: Refined pure
is mainly because these substances contain more amounts
carbohydrates are more caries producing than crude
of sticky carbohydrates.
carbohydrates complexes with other food elements capable
Cariogenicity of Carbohydrate Varies with of reducing enamel solubility.
• Frequency of ingestion Role of Microorganisms (Fig. 10.2)
• Physical form
It is generally agreed that dental caries is caused by acid
• Chemical composition
resulting from action of microorganisms on carbohydrates.
• Route of administration
Microbiology of Dental Caries (Table 10.1).
• Presence of other food constituents.
Animal studies with sophisticated experiments and
Frequency of ingestion: More is the frequency of sterile conditions have confirmed the essential role of
ingestion; more is the chance of dental caries. The risk microorganisms in the initiation and progression of dental
of caries incidence increases greatly if carbohydrates are caries. The absolute requirement of the microorganisms
taken repeatedly in between two major meals. It provides which are said to be inducing dental caries, specifically
an almost constant supply of carbohydrate to the plaque bacteria that can produce acids, particularly lactic acid,
bacteria for fermentation and subsequent production of from the substrate of host’s diet and can tolerate very
acids. low pH (<5) are suspected of initiating caries. The most
important organism [according to Clarke (1924) and
Physical form: Sticky, solid carbohydrates are more Orland and co-workers (1954)] is Streptococcus mutans.
cariogenic than in any other forms. Sugars that are easily
cleared are less damaging than soft retentive ones.
Chemical composition: Cariogenicity among carbo-
hydrates also varies.
∙ The carbohydrates in the form of glucose, sucrose and
fructose, etc. rapidly diffuse into the plaque due to their
low molecular weight and therefore make themselves
easily available for fermentation by plaque bacteria.
∙ Polysaccharides are less fermented by plaque bacteria
than the monosaccharides and disaccharides.
∙ Sucrose is utilized by Streptococcus mutans to
synthesize an extracellular insoluble polysaccharide
(dextran) with the help of glucosyl transferase enzyme.
Dextran helps the plaque to adhere firmly onto the
tooth surface and helps a direct contact between the
acids liberated by microorganisms and the tooth, thus
causing demineralization. Thus, sucrose is most potent
cariogenic substance. Figure 10.2 Pioneer bacteria responsible for dental caries
Dental Caries
Table 10.1 Localization of oral flora in dental caries counts with the increase in caries activity shows that these
organisms play an important role in the initiation as well
Pit and fissure Streptococcus mutans as progression of caries. The lactobacilli strains which
Lactobacillus species 147
are responsible for formation of carious lesions include:
Smooth surface Streptococcus mutans L. casei and L. acidophilus.
caries ∙ Acidogenic: They produce acids which mainly cause
Root caries Actinomycosis viscosus the initial demineralization of the enamel surface.
Actinomycosis naeslundii ∙ Aciduric: They particularly grow well in an
Other filamentous rod
environment of low pH. Though the role as a organism
Streptococcus mutans
for initiating or inducing carious lesion is not specific
Streptococcus salivarius
Streptococcus sanguis
as of the S. mutans strain but it is definitively a co-
cariogenic.
Deep dentinal caries Lactobacillus species
Actinomycosis viscosus Actinomycoses: The Actinomycoses form a major
Actinomycosis naeslundii proportion of the plaque flora at most sites on the teeth.
Other filamentous rod
The species which are responsible for the initiation of
Streptococcus mutans
carious lesions on the basis of acidogenic properties
and they also produce extracellular polysaccharides.
Streptococcus mutans: The role of S. mutans has been The strains in initiation of caries are: A. viscosus and A.
proved for the initiation of caries on the following basis. naeslundii.
It increases the amount of sticky plaque as it is capable of Progression of dental caries: The organisms responsible
producing extracellular polysaccharides. It also generates for progression of the lesion are usually present in the
acid rapidly from sucrose and helps in initial establishment advancing front of the dentinal caries. These are mostly
of the lesion. It multiply in abundance in low pH plaque facultative and strict anaerobes in advanced dentinal caries.
thereby colonizing and increasing the plaque bulk. It also They are streptococcal species in deep dentinal caries and
provides a favorable substrate for other cariogenic micro- root caries and Lactobacillus casei and acidophilus in
organisms. dentin.
S. salivarius: This is present in saliva, and epithelial
surfaces mostly on tongue, throat and establishes itself in Role of Acids
dental plaque. Acids play most important role in the pathogenesis of
dental caries. It has been noted that pH of plaque decreases
S. sanguis: Present in dental plaque and is capable of
within 2-4 minutes of rinsing oral cavity with glucose.
producing adhesive extracellular polymers which aids in
Stephan curve Robert M Stephan conducted experiments
colonization of cariogenic organisms.
to determine the plaque pH in relation to dental caries
S. mitior: Most commonly isolated in plaque, this is and carbohydrate intake. Plaque pH was measured using
present in smooth surface and pit and fissure caries along antimony touch electrodes.
with other strains. The Stephan curve describes the change in pH at
S. milleri: This is also present in dental plaque and produces interface of dental plaque and tooth surface in response
enzymes which help in sucrose degradation. to various dietary components. The Stephan curve reveals
Other streptococcal strains which are present in the a rapid drop in plaque pH, followed by a slower rise until
carious lesions may aid in its initial establishment. These the resting pH is attained. The initial drop is usually rapid
are S. oralis, S. lactis, S. faecalis and S. bovis. with the lowest pH being attained within 2-4 minutes.
However, pH recovery takes about 40 minutes. This
Lactobacilli: Lactobacilli form a small proportion of recovery is depended upon saliva’s ability to neutralize
the oral flora at any part of the mouth. These are gram acids (Fig. 10.3).
positive rods that may be straight or curved and usually The rapid fall in pH is mainly due to microorganisms
have blunt ends. Most of them are facultative anerobes metabolizing food substances and producing acids. This
but some are strict anerobes. Increased Lactobacillus fall in pH depends upon amount of diffusible carbohydrates,
Flow chart 10.1 Formation of acid
148
Figure 10.3 Stephan curve
nature of microorganisms and rate of diffusion of Role of Dental Plaque

microorganism. The rise in pH is slower and it depends
Dental plaque is a general term for the diverse microbial
upon ability of saliva to neutralize acids and diffusibility
community (predominantly bacteria) found on the tooth
of acids out of plaque. The pH 5.5 is called critical pH
surface, embedded in a matrix of polymers of bacterial and
because below this pH demineralization of tooth substance
salivary origin.
begins.
GV Black (1899) defined cariogenic plaque as
The rapid fall in pH is mainly due to microorganisms
follows—”The gelatinous plaque of the caries fungus is
metabolizing food substances and producing acids. This fall
a thin, transparent film that usually escapes observation,
in pH depends upon amount of diffusible carbohydrates,
and which is revealed only by careful search. It is not the
nature of microorganisms and rate of diffusion of
thick mass of materia alba found on the teeth, nor is it the
microorganism. The rise in pH is slower and it depends
whitish gummy material known as sordes, which is often
upon ability of saliva to neutralize acids and diffusibility of
prominent in fevers and often present in the mouth in
acids out of plaque.
smaller quantities in the absence of fever.”
The pH 5.5 is called critical pH because below this
The dental plaque is variable in both chemical and
pH demineralization of tooth substance begins. Acids are
physical composition, but usually consists of salivary
produced due to enzymatic breakdown of the sugar and
components such as mucin and desquamated epithelial
the acids formed are chiefly lactic acid and butyric acid
cells and microorganisms.
(Flow chart 10.1). Other acids which are produced are
Plaque is found on uncleaned tooth surfaces and appears
acetic acid, propionic acid, glutamic acid, aspartic acid.
as tenacious, thin film which may accumulate within 24 to
These acids cause demineralization of inorganic portion
48 hours.
(initially enamel and later dentin) and eventually cause
tooth decay. The Development of Dental Plaque
These acids result in highly localized drop in the pH at • Pellicle formation
the tooth surface and plaque interface. pH below 5.5 causes • Attachment of single bacterial cells (0–4 hours)
demineralization. The tooth minerals act as buffers which • Growth of bacteria leading to formation of
initially help to maintain the local pH at 5.5. Further drop microcolonies (4–24 hours)
in the pH causes subsurface demineralization, i.e. surface • Microbial succession and co-aggregation leading
enamel remains intact and demineralization is below the to increased species diversity concomitant with
surface. When the pH is decreased further, i.e. more acidic continued growth of microcolonies (1–14 days)
pH of about 3.0 to 4.0 causes etching of enamel surface and • Mature plaque (2 weeks or older).
resorption, this leads to cavitations.
Dental Caries
Acquired pellicle: This form is a glycoprotein that is There are hypothesis as follows:
derived from the saliva and is adsorbed on tooth surface. ∙ The specific plaque hypothesis: According to this
It is on this component of dental plaque the bacterial hypothesis, only a few organisms outside the different
colonization takes place. Thus, the pellicle serves as a 149
group in the plaque flora were actively involved in the
nutrient for plaque microorganisms. disease.
Microbial homeostasis: Plaque develops naturally ∙ The nonspecific plaque hypothesis: This state that the
on teeth, and gives benefit to the host by providing overall activity of the plaque microflora is responsible
colonization resistance. Once established at a site, the for the carious process. A consequence of this approach
plaque flora remains relatively stable with time despite is that all plaques should be disturbed by mechanical
regular environmental challenges. This stability (microbial plaque control (tooth brushing).
homeostasis) is not due to any metabolic indifference by the ∙ The ecological plaque hypothesis: Usually, resident
resident microflora but is due to a dynamic balance being microbial flora contains the organisms associated with
established among the resident microbial colony. Breaks disease may be present at sites without development
down in the homeostasis and imbalances in the microflora of lesion. Any shift in the balance of resident micro-
can occur which predispose a site to disease. floras by a change in the local environment lead to
The repeated intake of fermentable sugar in the diet demineralization. Frequent sugar intake (or decreased
produces frequent conditions of low pH in plaque which sugar clearance if salivary secretion is low) encourages
inhibits the growth of many of the species associated the growth of acidogenic and aciduric species, thus
with highly acidogenic (acid-producing) and aciduric predisposing a site to caries. The consequence of this
(acid-loving) species, such as mutans streptococci and hypothesis is that both mechanical cleaning and some
lactobacilli, associated with dental caries. As already stated, restriction of sugar intake are important in controlling
pH of 5.5 is critical threshold for the demineralization. caries progression.
Cariogenic plaque contains 2 × 108 bacteria per
Proteolysis Theory
milligram weight. The filamentous organisms grow in long
interlacing threads and have the property of adhering to This theory suggests the role of the proteolysis of the
smooth enamel surfaces. Smaller bacilli and cocci become organic components of the tooth as an initial process than
entrapped in this reticular meshwork. The adhering property the actual demineralization and dissolution of inorganic
and acidogenic property of streptococci and aciduric substances.
property of lactobacilli help in further development of The organic degradation is said to be the initial pathway of
cariogenicity of the dental plaque. invasion of the microorganisms. It has also been proposed that
Early colonizers (pioneers) of the tooth surface are mainly the enamel lamellae or rod sheath (proteins) may be lysed which
Neisseria spp. and streptococci. The growth and metabolism means proteolysis as the first event in the further progression
of these pioneer species changes local environmental of the bacterial invasion and demineralization carious
conditions (e.g. pH, coaggregation, substrate availability). lesions. Some authors suggest that the Nasmyth’s membrane
Thereby enabling more fastidious organisms to colonize, and enamel proteins produce sulfuric acids on hydrolysis.
e.g. obligate anaerobes tend to be late colonizers in plaque. This formed calcium sulfate compounds when combined
According to some authors; the pioneering organisms are with sulfatase enzymes liberated by microorganisms. This
S. sanguis, A. viscosus and Peptostreptococcus. subsequently leads to formation of the carious enamel. The
S. mutans further colonize in this noncariogenic plaque possibility of the events may be as follows:
and grows in an acidic environment. Enzyme glucosyl ∙ The enamel may be intact or clinically produce chalky
transferase helps in the synthesis of the extracellular area only by alteration in structure and underlying
matrix and aids in adhesion with the tooth surface. Thus, dentin may be involved through this pathway of
dental plaque provides environment for the cariogenic degraded proteins though in very minor quantities.
microorganisms, a medium for acid produced and their ∙ Minor variation in the organic and inorganic structure
adhesion to the tooth surface for longer period. Subsurface of the tooth determines the pattern of the rate and
demineralization cannot be described by this acidogenic progression of the carious lesions. Thus, caries
chemicoparasitic theory. Acute caries progression also is penetrates through enamel rods or along inter rod areas
not explained by this concept. available for degradation.
This theory could not be accepted for the fact that the in tooth decay. This theory is unlikely to be a significant
induction of the caries in experimental animals was seen in because once the sucrose is in the oral cavity, it readily gets
the absence of proteolytic organisms. metabolized to form acids, and there is hardly any scope
150 for the formation of calcium saccharates, a very high level
Proteolysis Chelation Theory of pH is required, the range of which is never achieved in
Chelation is a process of combining a metallic ion to a the oral cavity.
complex substance with the help of coordinate covalent
bond which results in a highly stable, weakly ionized and Autoimmune Theory
poorly dissociated compound. Jackson and Bunch suggest that zones or regions of
odontoblasts in specific sites with the pulp of specific
For example
teeth are damaged by an autoimmune process so that
Hem (iron) + 4 pyrrole globin = hemoglobin
the defense capacity of the overlying dentin and enamel
The four pyrrole nuclei are attached to iron by covalent is compromized and concluded that caries should be
bond. The proteolytic chelation theory suggests that the regarded as a degenerative process. Initially disease
caries is caused by simultaneous events of proteolysis and event corresponds to a form of somatic gene mutation in
chelation. Proteolysis is destruction of the organic portion central growth control stem cells. Descendent mutant cells
of the tooth by the proteolytic microorganisms. Chelation synthesize autoantibody which damage specific groups
is removal of calcium by forming soluble chelates by of odontoblasts and thus determine the sites of caries
biologic chelators such as certain citrates, amino acids, susceptibility.
phosphatases, tartrates, oxalates and enzymes, etc.
It is postulated that oral bacteria attack organic SECONDARY CONTRIBUTING
component of enamel (proteolysis) and that the breakdown FACTORS IN DENTAL CARIES
products have chelating ability and this dissolves the tooth
minerals. This results in formation of soluble chelates with
Saliva
the minerals of the enamel and thereby decalcifies it at a
• Salivary flow rate
neutral or even at an alkaline pH (Chelation).
• pH and buffering capacity
Some authors suggest that the dental caries is not merely
• Viscosity
an event due to proteolysis or chelation but it occurs due to
• Antibacterial substances
sequential events.
Teeth
∙ Simultaneous degradation of organic substances and • Structural composition
demineralization of the mineralized tissues. • Morphology
∙ This leads to low pH which leads to promotion of the • Arrangement in the arch
growth of the lactobacilli. • Presence of dental appliance
∙ The combined effects of the organic degradation Diet
mineralized substances decalcification and increased • Physical nature
microbial population lead to the formation of carious • Composition
lesion. Hereditary
Sucrose Chelation Theory
Saliva
This theory states that if there is a very excessive
concentration of sucrose in the mouth of a caries active Salivary Flow Rate
individual, there can be formation of complex substances Decrease or lack of salivary secretion results in increased
like calcium saccharates and calcium complexing rate of dental caries and rapid destruction of tooth as the
intermediaries, etc. by the action of phosphorylating cleaning or flushing of the bacterial deposits is hampered.
enzymes. Xerostomia (Greek: xeros—dry, stoma—mouth)
These complexes cause release of calcium and was first described by Bartley in 1968. Synonyms for
phosphorus ions from the enamel and thereby resulting xerostomia include oligosalia, asalia, and stomatitis sicca.
Dental Caries
Conditions leading to xerostomia are discussed in diseases Teeth which have high percentage of fluoride are more
of salivary glands. resistant to caries process. Accumulation of elements like
In patients with xerostomia the caries is atypical. fluoride, chloride, zinc, lead, and iron in the surface enamel
Decay often attacks the cervical area, involves cementum with aging takes place. Thus; this enamel is more resistant 151
and dentin, and progresses inwardly until the crown is to dissolution to acids.
destructed. It involves those teeth that are less commonly
affected by caries such as incisors and canines. Morphology
Occasionally, a rapid wearing of the incisal and the Pits and narrow fissures allow retention of food debris
occlusal surfaces will be seen. Alterations in the amount and thus are prone to development of decay. These areas
and bacteriological composition of the plaque are also are also not easily approachable to routine oral hygiene
reported. practices. The formation of plaque at the base of the defect
Streptococcus mutans, lactobacilli, yeast; actinomyces further aids in the progression of the caries in these areas.
and staphylococci increase in number while Veillonella, The most susceptible permanent teeth are the
Streptococcus sanguis, Neisseria, bacteroids and mandibular first molars followed by the maxillary first
Fusobacterium have been observed to decrease in number. molars and the mandibular and maxillary molars. The
Postradiation therapy there is changes in the main second premolars, maxillary incisors and first premolars
salivary glands with altered composition and flow of saliva. are the teeth next in sequence for occurrence of caries. The
mandibular incisors and canines are the least likely teeth
pH and Buffering Capacity for caries incidence.
A buffer is a solution that tends to maintain a constant pH.
In saliva, the chief buffer systems are the bicarbonate ions Arrangement in the Arch
and phosphate ions. High concentrations of bicarbonate ions Crowded, misaligned, rotated and irregular teeth are not
neutralize the acids produced by the cariogenic bacteria. readily cleansed during the natural masticatory process.
Urea secreted in saliva helps in the formation of ammonia Such teeth favor the accumulation of food and debris and
by action of the plaque microorganisms. Ammonia acts as may be susceptible for caries.
buffer in maintaining the salivary pH.
Presence of Dental Appliance
Viscosity Partial dentures, space maintainers and orthodontic
Thick and gluey consistency tends to attach more number appliances often encourage the retention of food debris
of the microorganisms and more plaque also. and plaque material and have been shown to result in an
increase in the bacterial population.
Antibacterial Substances
Lysozyme along with sodium lauryl sulfate, a detergent, Diet
can lyse many cariogenic streptococci. Lysozyme cleaves Physical Nature
the linkage between N-acetyl glucosamine and N-acetyl
muramic acid, which are responsible for the repeating units Fibrous foods help to keep the tooth surfaces clean and
of the bacterial cell wall peptidoglycans. stimulate the salivary flow which reduces the incidence
of caries. Soft and sticky foods tend to be retained on the
Teeth tooth surface and thereby predispose for more bacterial
accumulation and decay.
Structural Composition
Teeth are usually susceptible to caries during first 2 Composition
years after eruption as additional 2 years are required for Carbohydrate content plays an important role in the causation
completion of calcification after eruption. of caries as stated in the Miller’s acidogenic theory.
Concentration of higher number minerals in the surface Presence of phosphates in the diet can reduce the caries
enamel renders it more resistant to decay. Hypomineralized by increasing the remineralization. Among other components
and hypoplastic enamel has more incidences of caries. proteins are less cariogenic and lipids are least cariogenic.
Increased permeability of the enamel surface increases the Lipids help in preventing retention of food substances.
risk of the caries activity. Medium chain fatty acids have some antibacterial properties
at acidic pH. Although vitamin A deficiency results in

Anatomical Classification of Caries
defects of developing teeth, but it has no definitive role in
formation of dental caries. According to anatomical depth of the defect:
152 Similarly vitamin D, vitamin C deficiency result in • Enamel caries
tooth defects but their association with dental caries is • Dentin caries
not definitive one. Traces of molybdenum, strontium, • Cementum caries
and vanadium in diet are found to reduce the incidence of According to location of the lesion:
dental caries. • Pit and fissure caries
Selenium in diet and drinking water is found to increase – Occlusal
incidence of dental caries. Fluoride in drinking water – Buccal or lingual pit
is proven to be cariostatic. But dietary fluoride is less • Smooth surface caries
important because of metabolic unavailability. – Proximal
– Buccal or lingual surface
Hereditary • Root caries
It has been suggested that genetic factors contribute the According to intensively of caries within the dentition:
causation of dental caries. Another possibility suggested • Single lesion
is that inheritance of tooth form or structure can also be • Multiple lesions
a factor which predisposes to caries susceptibility or • Systemic destruction.
immunity. As dietary habits, cooking habits and tooth
brushing frequency are passed on generation to generation Depending on Nature of Attack
from parents to offspring, the exact role of inheritance
• P
rimary caries (Incipient, initial): First attack on
cannot be assessed.
tooth surface
• Secondary caries (Recurrent): Caries occurring at
CLASSIFICATION the margins or walls of existing restorations.
Clinical Classification of Caries Depending on the Progression of the Caries

According to the stage of lesion progression: • Acute: It rapidly invading process that involves
• Noncavitated lesion several teeth. Lesions are soft and light colored.
• Cavity. Usually pulp is involved at the early stage.
According to the severity of the disease: – Rampant caries
• Acute caries (active) – Nursing bottle caries
• Chronic caries (slowly progression) – Radiation caries.
• Stabilized caries (arrested). • Chronic: These lesions are long standing and fewer
According to clinical manifestation: in number.
• White spot lesion macula caroisa
• Superficial caries: Caries superficialis Based on Tissue Involved
• Medium caries: Caries media • Enamel caries
• Deep caries: Caries profunda • Dentinal caries
• Secondary caries: Caries secundaria. • Cemental caries.
Dental Caries
Based on Direction of Caries Attack SMOOTH SURFACE CARIES

• F orward caries: It is type of caries that proceeds Interproximal Caries
from enamel to dentin. The lesion is triangle shaped 153
with base of the triangle at the enamel surface and Clinical Features
apex towards dentin. In pits and fissures base is at It takes 3 to 4 years to manifest clinically as loss of enamel
dentinoenamel junction and the apex is in the pit. transparency resulting in opaque chalky region (white
• Backward caries: Caries that proceeds from denti- spot). In some cases, it appears as a yellow or brown
noenamel junction towards enamel surface. This is pigmented area but it is usually well demarcated. Spots are
also triangle shaped with base at the dentinoenamel generally located on the outer surface of enamel between
junction and apex towards enamel surface. contact point and height of free gingival margin. The early
white chalky spot becomes slightly roughened owing to
Based on Number of Surfaces Involved superficial decalcification of the enamel.
• Simple: Only one surface is involved by caries As the caries penetrates the enamel, the enamel
• Compound: Two surfaces are involved. surrounding the lesion assumes bluish white appearance
• Complex: More than three surfaces involved. which is usually apparent as laterally spreading caries at
the dentinoenamel junction. Caries does not initiate below
free gingival margin. It is common for proximal caries to
GV Black’s Classification Based on Treatment and
extend both buccally and lingually (Fig. 10.4).
Restoration Design
• C lass I: These begin in pits, fissures and defective Cervical, Buccal, Lingual or
grooves. These are seen in occlusal surface, occlusal Palatal Caries (Fig. 10.5)
two-thirds of molars and lingual pits of incisors.
• Class II: These are lesions seen on proximal aspects
Clinical Features
of molars and premolars. It usually extends from the area opposite the gingival crest
• Class III: These are lesions involving proximal occlusally to the convexity of the tooth surface. It extends
aspects of incisors that do not involve or necessitate laterally towards the proximal surfaces and on occasion
removal of incisal edge. extends beneath the free margin of the gingiva.
• Class IV: These are lesions involving proximal
aspects of incisors that involve or require removal of
incisal edge.
• Class V: These are lesions present on gingival third
of all the teeth.
• Class VI: Lesions found on incisal edges and cusp tips.
Miscellaneous Types
• S enile caries: It is caries associated with aging
process. These are almost exclusively seen on root
surface.
• Residual caries: It is caries that is not removed
during restorative procedure.
• Arrested caries: Sometimes progress of caries is
halted because of treatment or change in conditions.
Such lesions are mineralized but retain brown color. Figure 10.4 Proximal caries seen on premolar
154
Figure 10.5 Labial smooth surface caries Figure 10.7 Deep proximal carious lesion of first molar
Histopathological Features of Smooth

Surface Caries
Enamel Changes
The earliest changes are loss of the inter-prismatic or inter-
rod substance of enamel with increased prominence of
the rods. In some cases the changes are roughening of the
ends of the enamel rods, suggesting that the prisms may be
more susceptible to early attack. Another change in early
enamel caries is the accentuation of the incremental striae
of Retzius (Fig. 10.8).
As the process advances and involves deeper layers of
enamel, it will form triangular or actually a cone-shaped
lesion with apex towards the dentinoenamel junction and
Figure 10.6 Initial proximal surface caries presenting as the base towards the surface of the tooth. Enamel feels
radiolucent area in premolar rough to hand in advanced caries which may be due to
disintegration of the enamel prisms after decalcification
It usually occurs in cervical area and the typical of the inter-prismatic substance and the accumulation of
cervical lesion is a crescent shaped cavity beginning as debris and microorganism over the enamel rods (Figs 10.9
slightly roughened chalky area which gradually becomes to 10.15).
excavated. Several zones can be identified in the enamel which is
as follows (Figs 10.9 and 10.10):
Radiographic Features ∙ Zone 1: The translucent zone lies at the advancing
The initial lesion appears as opaque white or brown spot front of the enamel lesion. By use of polarized light it is
beneath the plaque layer. As the caries process results in learnt that this zone is slightly more porous than sound
demineralization, the affected area of the tooth appears enamel.
more radiolucent than unaffected area. Carious area ∙ Zone 2: The dark zone lies adjacent and superficial to
attenuates less radiation than intact tooth substance so the translucent zone. It has been referred to as positive
that the area of the film on which remnant beam from the zone because it is usually present. This zone is present
deminerlized area falls receives higher exposure and thus as a result of demineralization.
appears darker on the processed radiograph (Figs 10.6 ∙ Zone 3: The body of the lesion lies between the
and 10.7). relatively and the accumulation of debris and micro-
Dental Caries
155
Figure 10.8 Smooth surface caries with intact enamel surface Figure 10.11 Smooth surface caries
Figure 10.9 Various zones of dental caries the intact surface zone Figure 10.12 Smooth surface caries is triangular shaped
(4) covers the body of lesion (3) at the advancing front of caries lesion with apex towards dentinoenamel junction and base
translucent zone (1) separated dark zone from normal enamel (2) towards surface
Figure 10.10 Smooth surface caries showing different zones Figure 10.13 Smooth surface caries showing prominent
dark zone
156
Figure 10.14 Caries may develop in the enamel crack and Figure 10.16 Caries in dentin demonstrating microorganisms
lamellae; smooth surface caries showing intact surface zone in dental tubules (decalcified section, low power view)
and subsurface demineralization
become packed with masses of microorganisms. The

decalcification of the walls of the individual tubules leads
to their confluence.
A thickening and swelling of the sheath of Neumann
may sometimes be noted at irregular intervals along the
course of the involved dentinal tubules, in addition to
increased diameter of the dentinal tubules due to packing
of the tubules by microorganisms.
Tiny Liquefaction foci are formed by focal coalescence
and breakdown of a few dentinal tubules. This ‘focus’ is
an ovoid area of destruction parallel to the course of the
tubules and filled with necrotic debris which tends to
increase in size by expansion. This produces compression
and distortion of adjacent dentinal tubules so that their
Figure 10.15 Enamel smooth surface lesion under course is bent around the liquefaction focuses (Figs 10.17
polarized microscope to 10.21).
As the carious lesion progresses various zones beginning
pulpally at the advancing edge of the lesion adjacent to the
organism over the enamel rods. Several zones can be normal dentin are seen which are as follows:
identified in the enamel which is as follows.
∙ Zone 1: Zone of fatty degeneration of Tomes fibers.
∙ Zone 4: The surface zone, when examined by polarizing
∙ Zone 2: Zone of dentinal sclerosis characterized by
light appears relatively unaffected.
deposition of calcium salts in dentinal tubules.
Dentin Changes ∙ Zone 3: Decalcification of dentin, a narrow zone
preceding bacterial invasion.
The initial penetration of the dentin by caries may result ∙ Zone 4: Zone of bacterial invasion of decalcified but
in alteration in the dentin called as ‘dentinal sclerosis’. intact dentin.
Dentinal sclerosis is a reaction of vital dentinal tubules ∙ Zone 5: Zone of decomposed dentin.
and a vital pulp in which there results a calcification of the
dentinal tubules which finally seals them against further
penetration by microorganisms (Fig. 10.16).
PIT AND FISSURE CARIES
The initial decalcification involves the walls of It is also called occlusal caries. It is primary type and
the tubules allowing them to distend slightly as they develops in the occlusal surface of molars and premolars.
Dental Caries
157
Figure 10.17 Liquefaction necrosis of dentinal tubules Figure 10.19 Advanced dental caries in a decalcified section
showing cleft (CFT), coagulation necrosis of dentinal tubules
(CN), isolated Miller’s liquefaction foci (M) and pioneer
bacteria (pb)
Figure 10.18 Decalcified and H and E stained section dentinal

caries showing Miller liquefaction foci in an area of coagulation
necrosis and coalescence of dentinal tubules parallel to the
dentinal tubules Figure 10.20 Miller’s liquefaction foci
Deep narrow pits and fissures favor the retention of food The lateral spread of caries at the dentinoenamel
debris and microorganisms and caries may result due to junction as well as penetration into the dentin along the
dentinal tubules may be extensive without fracturing away
fermentation of this food and the formation of acids.
the overhanging enamel. Thus, there may be large carious
lesion with only a tiny point of opening.
Clinical Features
Radiologically it appear as radiolucent area in the
It usually occurs in pits and fissures with high steep enamel and dentin (Fig. 10.23)
walls and narrow bases. It appears brown or black and
will feel slightly soft and catch a fine explorer point. Histopathological Features
The enamel directly bordering the pit and fissure may (Figs 10.24 to 10.28)
appear opaque, bluish white as it becomes undermined Enamel changes: They are more or less same as smooth
(Fig. 10.22). surface caries. Enamel at the bottom of the pit or fissure
158
Figure 10.21 Pioneer bacteria in dentinal tubules called Figure 10.23 Deep occlusal caries with first molar
beaded appearance and Miller’s foci (low power)
Figure 10.22 Pit and fissure caries Figure 10.24 Pit caries
may be very thin so that early dentin involvement can retention of teeth into the later decades of life and increase
occur. in the number of people exhibiting gingival recession with
Enamel rods flare laterally at the bottom of the pits and clinical exposure of cemental surface. Freshly exposed root
fissures. Lesion forms a triangular or cone-shaped lesion are more vulnerable to an acid attack because of higher
with its apex at the outer surface and its base toward the porosity and smaller crystal.
dentinoenamel junction.
Clinical Features
ROOT CARIES It appears as slowly progressing chronic lesion. It is
usually found in mandibular molar and premolar region.
It is also called cemental caries and involves both dentin Tooth surface involved in decreasing order of frequency
and cementum. Nowadays there is greater prevalence of are buccal, lingual, interproximal. Gingival recession is
root caries due to longer life span of persons, with the associated with root surface caries (Fig. 10.29).
Dental Caries
159
Figure 10.25 Pit and fissure carious lesion demonstrating Figure 10.28 Pit and fissure lesion under polarized
translucent zone and body of lesion microscope shows positive birefringence in deminerlized areas
Figure 10.26 Pit and fissure caries Figure 10.29 Root caries is seen in gingival recession
It is a soft progressive lesion that is found anywhere on the
root surface that has lost connective tissue attachment and
is exposed to the oral environment.
Microorganisms appear to invade the cementum either
along Sharpe’s fibers or between bundles of fibers, in a
manner comparable to the invasion along dentinal tubules.
As cementum is formed in concentric layers and presents a
lamellated appearance, the microorganisms tend to spread
laterally between the various layers.
The carious lesion assumes the shape of a saucer (Fig.
10.30). After decalcification of cementum destruction
of the remaining matrix occurs similar to the process in
Figure 10.27 High power view of pit and fissure caries dentin.
160
Figure 10.31 Recurrent caries seen below the restoration

Figure 10.30 Cemental caries showing a saucer-shaped
lesion
RECURRENT CARIES
Dental caries that occurs immediately adjacent to the
restoration is referred to as recurrent caries. It may be
caused by inadequate extension of restoration and if there
has not been careful and complete excavation of original
carious lesion.
Clinical Features
Sixteen percent of restored teeth have recurrent caries.
Restoration will show poor margins which permitted
leakage and the entrance of both bacteria and substrate
(Fig. 10.31). Figure 10.32 Nursing bottle caries showing root stumps in
upper anterior region
Nursing Bottle Caries
Etiology of human milk, as well as that of bovine milk, can be
It occurs due to nursing bottle containing milk or milk cariogenic if the milk is allowed to stagnate on the teeth.
formulae, fruit juice or sweetened water. Sometimes it
occurs due to sugar or honey–sweetened pacifier. Clinical Features
Prolonged bottle feeding beyond the usual time when the
Pathogenesis child is waned from the bottle and introduced to solid
The reasons for this are that the child is put on bed at food may result in early and rampant caries. There is
afternoon nap time or at night with nursing bottle containing early carious involvement of the maxillary anterior teeth,
milk or a sugar containing beverage. The child falls asleep the maxillary and mandibular first permanent molars, the
and the milk or sweetened liquid becomes pooled around mandibular canines. The carious process in the teeth is so
the maxillary anterior teeth. The carbohydrate containing severe that only the root stumps remain (Fig. 10.32).
liquid provides an excellent culture medium for acidogenic
microorganisms. Prevention
Salivary flow is decreased during sleep and clearance The infant should be held while feeding. The child who
of the liquid from the oral cavity is slowed. Lactose content falls asleep while nursing should be burped and then placed
Dental Caries
in bed. Parent should start brushing the child teeth as soon Management
as they erupt in the oral cavity. Discontinue bottle feeding Extensive dental care and parent education.
as soon as child can drink from a cup at approximately 12
to 15 months of age. Arrested Caries 161
It has been described as caries which becomes static or

Rampant Caries
stationary and does not show any tendency for further
It is defined as a suddenly appearing, widespread, rapidly progression.
burrowing type of caries, resulting in early involvement
of the pulp and affecting those teeth usually regarded as Clinical Features
immune to ordinary decay. Some believe that the term Both deciduous and permanent dentitions are affected by this
rampant caries should be applied to those carious lesions condition. It occurs exclusively in caries of occlusal surfaces.
with 10 or more new lesions per year. It is characterized by large open cavities in which there
is lack of food retention and in which the superficially
Etiology softened and decalcified dentin is gradually burnished
Under laboratory conditions sucrose is more likely to cause and has taken a brown-stained polished appearance and is
rampant multisurface cavitations than glucose, fructose, hard. This has been referred to as eburnation of dentin. In
etc. It may occur due to nutritional deficiency, malnutrition, some cases there is brown stained area at or just below the
emotional disturbances. contact of the affected tooth (Fig. 10.34).
Various forms of stress in both children and adults,
as well as various medication (such a tranquilizers and Pre-eruptive Caries
sedatives) commonly taken to help persons cope with stress, Occasionally defect on the crowns of developing permanent
are associated with decreased salivary flow and decreased teeth are evident radiographically, even though no infection
caries resistance caused by impaired remineralization. of the primary tooth or surrounding area is apparent. It is
called pre- eruptive caries. Such lesions do resemble caries
Clinical Features when it is observed clinically and the destructive nature of
It usually occurs in children with poor dietary habits. It lesion progresses if it is not restored.
demonstrates extensive interproximal and smooth surface
Treatment
caries (Fig. 10.33). Rampant caries can occur suddenly
in teeth that were for many years relatively immune to As soon as the lesion is reasonably accessible, the tooth
decay. should be uncovered by removal of the overlying primary
Figure 10.33 Rampant caries of children involves all the teeth Figure 10.34 Arrested caries
rapidly with cervical lesions; lower incisors are intact
tooth or by surgical exposure and restoration should be Snyder Test

done.
This test measures the ability of microorganisms in saliva
162 Caries Activity Tests responsible for formation of acids from carbohydrate
media.
Caries activity can be defined as the occurrence and rate at The saliva sample is collected in a manner similar to
which teeth are destroyed by the acid produced by plaque the Lactobacillus colony count test. Then 0.2 cc of saliva
bacteria or it can also be defined as the sum total of new is pipette into the media which is incubated at 37�C up to a
carious lesions and the enlargement of existing carious period of 72 hours.
cavities during the given time. The various caries activity The media contains bactopeptone (20 g), dextrose (20
tests are as follows: g), sodium chloride (5 g), agar (16 g) and bromocresol
green (0.02 g). Bromocresol green, being an indicator,
Uses of Caries Activity Test
changes the color from blue green to yellow in the range of
• To determine the need and extent of preventive pH of 5.4 to 3.8. The color change is then correlated with
measures. the caries activity.
• To determine the success of therapeutic measures.
• To motivate and monitor the effect of education Interpretation
programs related to diet counseling and oral hygiene • High: If color changes in 24 hours, caries activity are
procedures. high.
• To identify high-risk groups and individuals. • Medium: If the color changes in 48 hours, it is
medium.
Lactobacillus Count Test • S light: If color changes in 72 hours, it is slight.
It was introduced by Hadley in 1933. It estimates the • Immune: If there are no color change patient is
number of bacteria in the patient’s saliva by counting the immune to caries.
number of colonies appearing on tomato Peptone Agar or
LBB Agar. Alban’s Test
In this stimulated saliva is collected before breakfast
by chewing paraffin. This is shaken and 1:10 and 1:100 It is a modification of Snyder’s test. It uses less quantity
dilutions are spread on the surface of agar plate. These are of agar i.e. 5 mL per tube. Because of its simplicity and its
incubated at 37� for a period of 3 to 4 days. The number low cost it is recommended for all patients prone to caries.
of colonies is then counted in a Quebec counter. The count The main feature of Alban’s test is the use of a softer
expressed as the average number of colonies per milliliter medium that permits the diffusion of saliva and acids
of the original saliva sample. without the necessity of melting the medium and use
of a simpler sampling procedure in which the patient
Disadvantages: Although it is quick and easy, the results expectorates directly into the tubes that contain the medium.
are not available for several days and counting colonies is Sixty grams of Snyder test agar is placed in 1 liter of water
a tedious process and is complex. Equipment and personals and the suspension is brought to boil over a low flame or
are required. a hot plate at medium heat (excessive heating should be
avoided to prevent scorching of medium).
Interpretation When thoroughly melted, agar is distributed, using
• I mmune: If the count is less than 103 then the patient about 5 mL per tube. The tube should be autoclaved for
is immune. 15 minutes, allowed to cool and stored in a refrigerator.
• Slight: If the count is between 103 and 5000, caries Two tubes of Alban medium are taken from the refrigerator
activity is slight. and saliva is drooled directly in to the tubes and tubes are
• Medium: If the count is between 5000 and 104, caries incubated for 4 days at 37�C. The tubes are observed daily
activity is medium. for change in color. The color change is noted from bluish
• High: If it is more than 104 then caries activity is high. green to yellow and the depth to which change has occurred
is noted.
Dental Caries
increases as the caries activity increases which is indicated

Interpretation
by an increase in the calcium content of saliva.
• Negative: No color changes.
• + - beginning of color change (from top of the Dewar Test 163
medium towards bottom).
Plaque samples are collected from the gingival third of
• ++ - one-half color changes.
buccal tooth surfaces and placed in Ringer’s solution. The
• +++ - three-fourths color changes.
sample is shaken until homogenized.
• ++++ - total color change.
The plaque suspension is streaked across a Mitis-
Salivarius agar plate. After aerobic incubation at 37�C
Streptococcus Mutans Level in Saliva for 72 hours, the culture is examined under a low
Saliva samples are obtained by using tongue blades (after power microscope and the total colonies in 10 fields are
air drying the tooth for plaque samples). These are then recorded.
incubated on MSB agar (Mitis Salivarius Bacitracin Agar). This test is an attempt to semiquantitatively screens
The numbers of colonies are then used to estimate the the dental plaque for a specific group of caries causative
caries activity and more than 105 colonies per mL of saliva organisms including streptococci.
is indicative of high caries activity. Swab Test
Buffer Capacity Test The swab test involves sampling of the oral flora by
Ten milliliter of stimulated saliva is collected Under oil at swabbing the buccal surface of teeth and placing it in
least one hour after eating; 4 mL of this is measured into a Snyder media. This is incubated for 48 hours and the pH
beaker. After collecting the pH meter, the pH of saliva is changes are read and correlated with caries activity.
adjusted to 7.0 by addition of lactic acid or base at room Reductase Test
temperature.
It measures the activity of salivary enzyme reductase.
The level of lactic acid in the graduated cylinder is re-
Saliva is collected and the sample is mixed with a
recorded. Lactic acid is then added to the sample until a pH
diazoresorcinol, which colors the saliva blue. The
of 6.0 is reached. The number of millimeter of lactic acid
change in color from blue to red is measured after 30
needed to reduce pH from 7.0 to 6.0 is a measure of buffer
seconds and 15 minutes and this is taken as measure of
capacity of saliva.
caries activity.
Interpretation
Interpretation
• Low buffer capacity: Saliva sample requiring less
• N onconductive: If it remains blue after 15 minutes it
than 0.45 ml of standard hydrochloride acid to reduce
is nonconductive.
the pH to 5 has low buffer capacity.
• Slightly conductive: If it changes to orchid after
• High buffering capacity: Saliva sample requiring
15 minutes, it is slightly conductive.
0.45 ml or more has high buffering capacity.
• Moderately conductive: If it changes to red after
15 minutes, moderately conductive.
Fosdick Calcium Dissolution Test • Highly conductive: If it changes immediately to red,
Twenty five milliliter of gum stimulated saliva is collected. it is highly conductive.
Part of this is analyzed for calcium content and the rest is • Extremely conductive: If it changes to pink or white
placed in an eight inch sterile test tube with about 0.1 g of immediately, it is extremely conductive.
powdered human enamel.
The tube is sealed and shaken for four hours at body As soon as the lesion is reasonably accessible, the tooth
temperature after which, it is again analyzed for calcium should be uncovered by removal of the overlying primary
content. If paraffin is used, a concentration of about 5 tooth or by surgical exposure. Restoration of the teeth
percent glucose is added. The amount of enamel dissolution should be carried out.
Control of Dental Caries Detergent: Some workers have related the high caries
incidence among modern civilized races to the unrestrained
Control of all active lesions:
use of soft, sticky, refined foods, which tend to adhere
164 • Nutritional measures for caries control
to the teeth. It has been stated that fibrous food prevents
• Mechanical measures for caries control
lodging of food in pits and fissures of teeth and in addition
– Tooth brushing
acts as detergent.
– Mouth rinsing
– Dental floss Pit and fissure sealants: Pits and fissures of occlusal
– Detergent surface are among the most difficult areas on teeth to
– Pit and fissure sealants keep clean and from which to remove plaque. The pit and
• Chemical measures of caries control: fissure sealants generally used in conjunction with an acid
– Fluorine pretreatment to enhance their retention, contain either
– Bis-biguanides cyanoacrylate, polyurethane or the product of bisphenol A
– Silver nitrate and glycidyl methacrylate.
– Zinc chloride and potassium ferrocyanide
– Vitamin K CHEMICAL MEASURES OF
– Sarcoside
CARIES CONTROL
– Urea and ammonium compounds
– Chlorophyll Substances Which Alter the Tooth Surface or
– Nitrofurans Tooth Structure
– Penicillin.
Fluorine
The cariostatic activity of fluoride involves several
Control of Dental Caries
different mechanisms. The ingestion of fluoride results in
Control of All Active Lesions its incorporation into the dentin and enamel of unerupted
Initial treatment of all active lesion involves gross teeth. This makes the teeth more resistant to acid attack
excavation of all carious lesions followed by systematic after eruption into oral cavity. In addition, ingested
manner of restoring a tooth to normal contour. fluoride is secreted into saliva; although present in low
concentration in saliva; the fluoride is accumulated in
Nutritional Measures for Caries Control plaque where it decreases microbial acid production and
Group of patients whose diet is high in fat, low in enhances the remineralization of the underlying enamel.
carbohydrate and practically free from sugar have low Fluoride from saliva is also incorporated into the enamel
caries activity. In a study, when refined sugar was added of newly erupted teeth, thereby enhancing the enamel
to the diet in the form of a mealtime supplement there calcification.
was little or no caries activity. Phosphates diet causes Fluoridation of the communal water supply is the most
significant reduction in incidence of caries. effective method of reducing the dental caries problems in
Mechanical Measures for Caries Control the general population.
Tooth brushing: Tooth brushing reduces the number of ∙ Fluoride containing dentifrices: It contains stannous
oral microorganisms, particularly if the teeth are brushed fluoride in combination with calcium pyrophosphate as
after each meal. Tooth brush also removes gross amounts the cleaning and polishing system and was accepted as
of food debris and plaque material. the first therapeutic dentifrice.
∙ Fluoride mouth rinses: It should be given cautiously
Mouth rinsing: The use of mouthwash for the benefit of
in children under 4 years of age as they may not have
its action in loosening food debris from the teeth has been
full control over the swallowing reflex.
suggested as a measure of caries control.
∙ Dietary fluoride supplement: The administration of
Dental floss: Dental flossing has been shown to remove fluoride supplement commences shortly after birth and
plaque from an area gingival to the contact areas on proximal should continue through the time of eruption of the
surfaces of teeth, an area impossible to reach with toothbrush. second permanent molars.
Dental Caries
Bis-biguanides and salivary pH generally increased to value over 8 and

remained high for approximately an hour.
Chlorhexidine and alexidine are potential anticaries agents
The evidence indicated that urea upon degradation by
as they are antiplaque agents. It has been shown that 165
urease; releases ammonia which acts to neutralize acids
chlorhexidine is adsorbed onto tooth surface and salivary
formed through carbohydrate digestion and also interferes
mucins then it is released slowly in an active form. But
with bacterial growth. Although there are some studies
disadvantage of chlorhexidine is that it has bitter taste,
to indicate that ammoniated dentifrices are capable of
produces brownish discoloration of hard and soft tissues
producing some reduction in dental caries incidence, the
and may produce painful desquamation of mucosa.
magnitude of this reduction, particularly in persons whose
Silver Nitrate tooth-brushing habits are not controlled or supervised, is not
so great as to justify recommending them for widespread
Silver nitrate impregnation of teeth was used for many
use as an anti-cariogenic agent.
years to prevent or arrest caries. Silver plugs the enamel
by either the organic invasion pathways such as the enamel Chlorophyll
lamellae or the inorganic portion of enamel to form a less
It is a green pigment of plants and has been proposed as
soluble combination.
an anticariogenic agent on the basis of a number of in
Zinc Chloride and Potassium Ferrocyanide vitro studies and animal studies. Water soluble form of
chlorophyll, sodium copper chlorophyllin, was capable of
Use of solution of zinc chloride and potassium ferrocyanide
preventing or reducing the pH fall in carbohydrate-saliva
would effectively impregnate the enamel and seal off caries
mixtures in vitro.
invasion pathways. But study shows that it is of little value
in reduction of caries. Nitrofurans
Substances Which Interfere with Carbohy- They are derivatives of furfural which itself is derived from
drate Degradation through Enzymatic Altera- pentoses. They have been found to exert bacteriostatic
tion and bactericidal action on many gram-positive and gram-
negative bacteria and they can also inhibit acid formation.
Vitamin K Studies show that nitrofurans compounds like furadroxyl
Synthetic vitamin K (2-methyl 1, 4 naphthoquinone) (5-nitro-2-furaldehyde-2-hydroxyethyal semicarbazone)
prevents acid formation in incubated mixture of glucose reduce dental caries.
and saliva. In study also it has shown to decrease incidence
of caries formation in persons given chewing gums Penicillin
containing vitamin K. It has got ability to inhibit the normal biologic processes
of lactobacilli which is one of the etiological factors in the
Sarcoside dental caries. Penicillin is given in dentifrices.
Persons who brushed teeth with dentifrices containing
sodium N-lauroyl sarcosinate have been shown to have BIBLIOGRAPHY
decreased incidence of caries. It has been stated that it has
1. Folayan MO, Sowole CA, Owotade FJ, et al. impact of
got ability to penetrate the dental plaque and prevent pH to infant feeding practices on caries experience of preschool
fall below 5.5 after carbohydrate rinse. children. J Clin Pediatr Dent. 2010;34(4):297-301.
2. Gao XL, Hsu CY, Loh T, et al: dental caries prevalence and
Substances which Interfere with Bacterial distribution among preschoolers in Singapore. Community
Growth and Metabolism Dent Health. 2009;26(1):12-7.
Urea and Ammonium Compounds 3. Grewal H, Verma M, Kumar A. Prevalence of dental caries
and treatment needs amongst the school children of three
Reports suggest that a quinine urea mouthwash prevents educational zones of urban Delhi, India. Indian J Dent Res.
acid formation in test in vitro on carbohydrate saliva 2011;22(4):517-9.
mixtures. It may also be noted that oral bacteria count 4. Kaste LM, Marianos D, Chang R, et al. The assessment
was decreased after the use of quinine urea mouthwash of nursing caries and its relationship to high caries in the
permanent dentition. 1992. J Indiana Dent Assoc. 2010; with composite resin. J Prosthet Dent. 1985;53(4):521-
89(2):20-4. 5.
5. Kodama Y, Matsuura M, Sano T, et al. Diabetes enhances 10. Su N, Marek CL, Ching V, et al: caries prevention for
166 dental caries and apical periodontitis in caries-susceptible patients with dry mouth. J Can Dent Assoc. 2011;77:b85.
WBN/KobSlc rats: Nakahara Y, Ozaki K, Narama I, 11. Trachtenberg F, Maserejian NN, Soncini JA, et al: does
Matsuura T. Comp Med. 2011;61(1):53-9. fluoride in compomers prevent future caries in children? J
6. Lewis DW, Hargreaves JA. Epidemiology of dental caries Dent Res. 2009;88(3):276-9.
in relation to pits and fissures. Br Dent J. 1975;138(9): 12. Utreja D, Tewari A, Chawla HS. A study of influence of
345-8. sugars on the modulations of dental plaque pH in children
7. Misra S, Tahmassebi JF, Brosnan M. Early childhood with rampant caries, moderate caries and no caries. J Indian
caries-a review. Dent Update. 2007;34(9):556-8, 561-2, Soc Pedod Prev Dent. 2010;28(4):278-81.
564. 13. Wang X, Willing MC, Marazita ML, et al: genetic and
8. Mozaffari MS, Abdelsayed R, Zakhary I, et al: submandibular environmental factors associated with dental caries in
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9. Staninec M, Mochizuki A, Tanizaki K, et al: effect of 14. Winter GB. Clinical procedures in the prevention of dental
etching and bonding on recurrent caries in teeth restored caries. Int Dent J. 1973;23(2):372-7.
1. Most accepted theory of cariogenesis is: 6. The microorganism related to smooth surface caries is:
a. Phosphatase theory a. A. viscosus b. S. mitior
b. Chemical theory c. S. mutans d. S. salivarius
c. Miller’s acidogenic theory 7. The critical pH below which demineralization of tooth
d. Vital theory substance begins is:
2. The most potent cariogenic substance is: a. 4.5 b. 3.5
a. Glucose b. Sucrose c. 6.0 d. 5.5
c. Fructose d. Lipids 8. In enamel caries, the area of greatest demineralization
3. Most cariogenic polysaccharide synthesize by micro- is:
organism is: a. Zone–1 b. Zone–2
a. Levan b. Glucan c. Zone–3 d. Zone–4
c. Dextran d. All of the above 9. Most commonly affected teeth in nursing bottle caries
4. Following are the microorganism requires for the are:
progression of dental caries except: a. Maxillary incisors b. Maxillary molars
a. L. acidophilus b. L. casei c. Mandibular molars d. Mandibular incisors
c. A. Israelii d. A. viscosus 10. The disadvantage of chlorhexidine is:
5. Most commonly isolated microorganism in dental a. Costly
plaque is: b. Produces brownish discoloration
a. S. mitior b. S. milleri c. Acidic in taste
c. S. salivarius d. S. sanguis d. All
11 Benign Tumors
Anil Govindrao Ghom, Shubhangi Mhaske (Jedhe)
Chapter Outline
Â Characteristics of benign tumor Â Osteochondroma

Â Terminology Â Torus palatinus
Â Squamous papilloma Â Torus mandibularis
Â Sinonasal papilloma Â Exostosis hemangioma
Â Verruciform xanthoma Â Vascular malformation
Â Keratoacanthoma Â Struge-Weber syndrome
Â Squamous acanthoma Â Lymphangioma
Â Oral melanoacanthoma Â Glomus tumor
Â Melanocytic nevus Â Hemangiopericytoma
Â Fibroma Â Nasopharyngeal angiofibroma
Â Fibrous histiocytoma Â Neuroma
Â Fibromatosis Â Neurilemmoma
Â Giant cell fibroma Â Multiple endocrine neoplasia syndromes (MEN syndrome)
Â Oral focal mucinosis Â Paraganglioma
Â Peripheral ossifying fibroma Â Neurofibroma or neurofibromatosis
Â Myxoma Â Ganglioneuroma
Â Chondroma Â Congenital granular cell tumor
Â Myxofibroma Â Melanotic neuroectodermal tumor of infancy
Â Chondroblastoma Â Leiomyoma
Â Chondromyxoid fibroma Â Rhabdomyoma
Â Lipoma Â Granular cell tumor
Â Osteoma Â Giant cell lesion
Â Osteoblastoma Â Peripheral giant cell granuloma
Â Osteoid osteoma
INTRODUCTION with the clinical features, diagnosis, and management of

localized nonmalignant growths of the oral cavity. Tissue
An oral tumor comprises of a plethora of lesions with varied enlargements attributable to irritation or injury represent
miscellaneous etiologies some of which are true neoplasms a hyperplastic reaction and are collectively grouped as
and some are reactive lesions. This chapter is concerned reactive proliferations.
Tumor is a new growth representing the tissue of EPITHELIAL ORIGIN

origin. If these are left untreated, some of the oral lesions
or neoplasms may lead to extensive tissue destruction and Squamous Papilloma
168 deformity whereas others will interfere with mastication It is a relatively common, benign neoplasm of unknown
and will become secondarily infected following masticatory origin, which arises from the surface of epithelium. It may
trauma. be caused by papilloma virus.
Regardless, the major clinical consideration in the
management of all of these tumors is to identify their Types
benign nature and to distinguish them from potentially • Congenital: It is usually present since birth.
life-threatening malignant lesions. Since this decision • Infective: It arises from viral infection.
usually can be made with certainty only by microscopic • Soft papilloma: It is often seen in eyelids of elderly
examination of excised tissue, biopsy is generally an people.
essential mandatory step. • Keratin horns: It is due to excess keratin formation
and seen in older people.
CHARACTERISTICS OF • Basal cell papilloma: It is also called as ‘seborrheic
BENIGN TUMOR or senile wart’. It occurs on the trunk, face, arms and
arm pits.
Benign neoplasms has got insidious onset and the rate of
growth is generally gradual and is for a longer period of
time. Clinical Features
Soft tissue benign tumors present as well defined mass Age: This is more commonly seen in the age in the 3rd and
of regular smooth outline and it possesses a fibrous capsule. 4th decade of life, only 20 percent of cases are found below
Benign tumors usually produce symptoms due to the 20 years of age.
swelling and pressure effect on the surrounding structures
Location: It is most commonly seen on tongue, palate,
with no actual tissue invasion unlike malignant tumors.
buccal mucosa, gingiva, lip, mandibular ridge and floor of
Benign tumors are smaller, as compared to malignant
mouth (Fig. 11.1).
tumors. There is displacement of adjacent normal tissues
and neurovascular bundles. They are usually painless and
they never metastasize.
Terminology
Hamartomas: It is an abnormal proliferation of normal
tissue at its usual location. For example, hemangioma.
Neoplasm: Tumors that continue to grow indefinitely are
called as neoplasm.
Hypertrophy: Enlargement caused by an increase in the
size of cells.
Hyperplasia: Enlargement caused by an increase in the
number of the cells.
CLASSIFICATION OF BENIGN TUMOR

Figure 11.1 Papilloma showing exophytic papillary growth on
Benign tumors are classified as shown in Table 11.1. cheek
Benign Tumors
Table 11.1 Classification of benign tumor

Epithelial tissue Vascular tissue
Papilloma Hemangioma 169
Keratoacanthoma Hereditary hemorrhagic telangiectasia
Squamous acanthoma Lymphangioma
Nevus Arteriovenous fistula
Fibrous connective tissue Glomus tumor
Fibroma Neural tissue
Fibrous hyperplasia Neurofibroma
Giant cell fibroma Neurolemmoma
Fibrous histiocytomas Schwanoma
Desmoplastic fibroma Ganglioneuroma
Myxoma Traumatic neuroma
Myxofibroma Melanotic neuro-ectodermal tumor of infancy
Cartilage tissue Muscles
Chondroma Leiomyoma
Chondroblastoma Rhabdomyoma
Chondromyxoid fibroma Granular cell myoblastoma
Adipose tissue Giant cell tumor
Lipoma Central and peripheral giant cell tumor
Angiolipoma Giant cell granuloma
Bone Giant cell tumor of hyperthyroidism
Osteoma Teratoma
Osteoid osteoma Adenoma
Osteoblastoma Oxycytoma
Exostosis-tori-torus palatinus-torus mandibularis Oxyphilic adenoma
Osteomatosis Warthin’s tumor
Pleomorphic adenoma
Appearance: It is a typically an exophytic lesion with a Syndrome associated: The other syndromes which may
cauliflower-like surface or with finger-like projection. This be associated with multiple papillomas are Cowden’s
appearance is caused by presence of deep clefts that extend syndrome, Down’s syndrome and nevus unius lateris.
well into lesion from the surface.
Base: It is generally arising from a pedunculated base.
Sometime base may be broad rather than pedunculated It consists of many long, thin, finger-like projections
(Figs 11.2A and B). extending from the epithelium and containing a thin,
Size: The size of tumor may vary from 2 millimeters but it central connective tissue core, which supports the nutrient
is seldom larger than 2 centimeters. Tumor in which there blood vessels (Fig. 11.3).
is much keratinization is white and lesion without much Keratin covering the epithelium follows.
keratinization are grayish pink in color. Tumor is firm, Superficial vacuolated cells are present in papilloma.
when keratinized and soft when it is nonkeratinized. Virus antibody is found. A supporting fibrous connective
The term focal dermal hypoplasia syndrome is used tissue stroma often contains prominent number of small
when there are multiple oral papillomas, dermal hypoplasia blood vessels and inflammatory cell infiltrate.
and syndactyly with fatty herniation, coloboma and Koilocytes virus altered epithelial clear cells with dark
strabismus. pyknotic nuclei are sometimes seen.
170
A Figure 11.3 Papilloma shows many fingers like projections (P)

of epithelial cells (E). Each projection is supported by connective
tissue papilla (CT). Keratin (K) is seen at the surface
Sinonasal Papilloma
These are localized proliferation of respiratory mucosa of
sinonasal tract. Half of the sinonasal papilloma arises from
mucosa of lateral nasal wall and other from maxillary and
ethmoid sinuses.
It can be neoplasm or reactive hyperplasia secondary to
chronic bacterial infection, tobacco smoking.
Types of Sinonasal Papilloma

B
• Fungiform (septal squamous exophytic) papilloma
Figures 11.2A and B Pedunculated papilloma on tongue
• Inverted papilloma (inverted schneiderian papilloma)
• Cylindrical cell papilloma (oncocytic schneiderian
Management papilloma).
Elliptic incision on the tissue underlying the lesion should
be done. Incision should be from the base of mucosa, into Clinical Features
which the pedicle or stalk inserted. Fungiform papilloma
Application of formaldehyde at night on the wart may
Age and sex distribution: It is seen in age group of 20 to
cure the condition. Sometimes, silver nitrate application
50 years age. It is twice is common in male as compared
also cures the condition. If the tumor is properly excised,
to female.
recurrence is rare.
Location: It is seen in exclusively in nasal septum.
Points to Remember
Appearance: It appears as pink or tan, broad base nodule
Papilloma virus, cauliflower-like surface or with finger- with papillary or warty surface projection.
like projection, focal dermal hypoplasia syndrome,
Cowden’s syndrome, Down’s syndrome, long, thin, Inverted papilloma
finger-like projections extending from the epithelium, Age and sex distribution: The mean age of 55 years with
keratin covering, superficial vacuolated cells, koilocytes, strong male predilection.
elliptic incision, application of formaldehyde.
Location: It is lateral nasal cavity wall or paranasal sinus.
Benign Tumors
Appearance: It appears as soft, pink or tan polypoid or

nodular growth.
Sign and symptoms: There is unilateral nasal obstruction, 171
pain, epistaxis, purulent discharge and local deformity.
Radiological features: Pressure erosion of underlying
bone presents as irregular radiolucency. There is also
mucosal thickening of radiograph.
Cylindrical cell papilloma
Age and sex distribution: It is more common in males
than female. It occurs in age group of 20 to 50 years of age.
Location: It is seen in maxillary antrum, lateral nasal
cavity wall, ethmoid sinus.
Appearance: It appears as beefy red or brown mass with Figure 11.4 Sinonasal papilloma showing clefting
multinodular surface.
Inverted papilloma: Aggressive surgical therapy with
Fungiform papilloma
medial maxilloectomy via lateral rhinotomy or midfacial
It is similar to oral squamous papilloma. There is fingerlike
degloving approach.
projection. Respiratory epithelium may be seen in some
cases. Goblet cells intraepithelial microcyst which contains Cylindrical cell papilloma: It is same as that of inverted
mucus is often present. papilloma.
The underlying connective tissue shows delicate
fibrous tissue with minimal inflammatory component. Points to Remember
• F ungiform papilloma: Pink or tan, broad base nodule
Inverted papilloma
fingerlike projection, goblet cells, delicate fibrous
There is squamous epithelial proliferation into submucosal tissue
stroma. The basement membrane shows pushing into • Inverted papilloma: Lateral nasal cavity, tan
underlying connective tissue. polypoid or nodular growth, nasal obstruction, pain,
Goblet cells and mucin filled microcyst are seen in epistaxis, irregular radiolucency squamous epithelial
epithelium. Papillary surface projection with deep clefts is proliferation, goblet, papillary surface projection
seen between projections (Fig. 11.4). with deep cleft
Cylindrical cell papilloma • Cylindrical cell papilloma: Maxillary antrum, beefy
It shows endophytic or exophytic growth. Papillary red or brown mass endophytic or exophytic growth,
projection shows fibrovascular connective tissue core and multilayered epithelium of tall columnar cells with
covered by multilayered epithelium of tall columnar cells small dark nuclei.
with small dark nuclei.
Cytoplasm is eosinophilic and granular. Cilia may Verruciform Xanthoma
be seen on surface and there is numerous intraepithelial It is also called as ‘histiocytosis Y’. It is a papillomatous
microcyst filed with mucin. lesion of oral cavity in which the foam cells fill the
connective tissue papillae between the epithelial pegs.
Management Etiology is unknown and it is thought to be unusual
Fungiform papilloma: Complete surgical excision is the immune response to localized epithelial trauma or
treatment of choice for tumor. damage.
Clinical Features
Age and sex distribution: There is slight male predilection
172 and is usually seen in middle age.
Location: It can occur at any site and is most frequently
found on the gingiva or alveolar ridge, followed by the
buccal mucosa, palate, floor of the mouth, lip and lower
mucobuccal fold. It occurs as a solitary lesion.
Appearance: It has got verruciform surface, i.e. papillary
or roughened surface. In some cases, crateriform surface
have also been reported. It has got either normal or red in
color, but sometimes pale or hyperkeratotic lesion with a
rough pebbly surface may be seen.
Base: It is either sessile or has a pedunculated base with
size as small as 2 mm to as large as 1.5 cm.
Figure 11.6 Finger-like projections showing
Histopathological Features hyperkeratinization
The lesion has a verrucous, hyperkeratotic surface with

severe parakeratin plugging.
Surface parakeratin is often shaggy with superimposed
bacterial colonies. The rete pegs are extremely elongated
but in a uniformly fashion.
There is also papillary or finger like projection seen
(Figs 11.5 and 11.6).
No increase in mitosis or pseudoepitheliomatous
hyperplasia is found.
There is presence of large swollen foam cells or
xanthoma cells (Fig. 11.7), which are presumably
Figure 11.5 Papillary projections (P) of verrucous xanthoma. Figure 11.7 Foam cells are seen in the connective tissue
Connective tissue cores below epithelium (E) show presence of papilla in verruciform xanthoma
foam cells (F). Inflammatory cells (I) are seen
Benign Tumors
histiocytes, which fill the connective tissue papillae Keratin pit is frequently discolored, being yellowish
between the epithelial rete pegs. brown in color. It grows to maximum size of 1 to 2 cm in
diameter.
Management The lesion is often painful and regional lymphadenopathy 173
Simple surgical excision shows good prognosis with no may be present. Lesion appear fixed to the surrounding
recurrence usually. tissues.
Muir-Torre syndrome: Keratoacanthoma associated with
Points to Remember sebaceous neoplasm and gastrointestinal carcinoma is
Histiocytosis Y, verruciform surface, crateriform surface, called as Muir-Torre syndrome.
hyperkeratotic lesion, verrucous, hyperkeratotic surface There may be unsightly scar formation.
with severe parakeratin plugging, foam cells or xanthoma
cells. Histopathological Features (Fig. 11.8)
The lesion consists of hyperplastic squamous epithelium
Keratoacanthoma growing into the underlying connective tissue.
It is also called as ‘self-healing carcinoma’, ‘pseudocarci- The surface is covered by a thickened layer of
noma’, keratocarcinoma’, ‘molluscum sebaceum’. It, clini- parakeratin or orthokeratin with central plugging. At the
cally and histologically, resembles epidermoid carcinoma deep margins of the tumor, islands of epithelium often
and it is frequently mistaken as cancer. It is believed to appear to be invading. Epithelial tissue adjacent to the
arise from hair follicles. lesion is sharply demarcated from that of the lesion, which
Genetic and viral etiological factors have been appears to lie in a cup-shaped depression.
postulated. Exposure to sun could be responsible for the The connective tissue in the area shows chronic
case occurring in lip. In some cases trauma and chemicals, inflammatory cell infiltration. The most characteristic
such as coal tar and mineral oil can be responsible for feature of the lesion is found at the margins, where the
keratoacanthoma. Some author thought HPV virus can be normal adjacent epithelium is elevated towards the center.
causative organism. An abrupt change in the normal epithelium occurs as the
hyperplastic acanthotic epithelium is reached.
Clinical Features
Management
Age and sex distribution: It is more common in male as
compare to female (2:1). Majority of cases occur between It often resolves spontaneously without treatment. The
the ages of 50 to 70 years. lesion may be treated by surgical excision as the scar
remaining from excision will be more cosmetic than that
Location: Exposed skin including cheeks, nose and dorsum resulting from spontaneous regression.
of the hands are the most common site of occurrence.
Intraoral lesion is uncommon; if found, is more common
on lips.
Appearance: The lesion appears as an elevated umblicated
or crateriform with depressed central core. It appears as
dome shaped. On the lower lip the lesion shows smooth,
raised, rolled borders with a central plug of hard keratin.
It begins as small, firm nodules that develop to full size
over a period of four to eight weeks (growth phase) and
persist as static lesions for another 4 to 8 weeks (stationary
phase). After that it undergoes spontaneous regression
over the next six to eight weeks period by expulsion of the
keratin core with resorption of the mass (involution phase).
Signs: The lesion is round with rolled margins. Margins
are sharply delineated. There may be elevation of the rolled
margins. Figure 11.8 Keratoacanthoma
Points to Remember Histopathological Features

Self-healing carcinoma, exposure to sun, elevated There are numerous benign dendritic melanocytes scattered
174 umbilicated or crateriform, dome shaped, growth phase, in the epithelium.
stationary phase, involution phase, rolled margins, Other features which are present are spongiosis, mild
keratin pit, regional lymphadenopathy, Muir-Torre acanthosis, eosinophils, and mild to moderate chronic
syndrome, hyperplastic squamous epithelium, thickened inflammatory cell infiltrate in connective tissue.
layer of parakeratin, cup-shaped depression, chronic
Management
inflammatory cell infiltration.
Incisional biopsy is performed to rule out melanoma.
Squamous Acanthoma
Points to Remember
It is an uncommon lesion which probably represents a
Pain, burning, pruritis, smooth, flat, dark brown to black
reactive phenomenon, rather than a true neoplasm. It is
color, benign dendritic melanocytes, spongiosis, mild
caused by trauma.
acanthosis, eosinophils.
Clinical Features
Melanocytic Nevus
It can occur at any site and at any age. It is generally
described as a small, flat or elevated, white, sessile or It is also called as ‘pigmented mole’ or ‘benign melanocytic
pedunculated lesion on the mucosa. nevi. Pigmented nevus is a superficial lesion composed of
so called nevus cells; hence the term ‘cellular nevus’.
Histopathological Features Nevus is defined as a congenital, developmental tumor
It consists of well demarcated elevated and/or umbilicated like malformation of the skin or mucous membrane.
epithelial proliferation with a markedly thickened layer
of orthokeratin and underlying spinous layer. There Types
are epithelial alterations beginning with a localized Congenital
pseudoepitheliomatous hyperplasia. • Small: They are greater than 1 cm in diameter and are
usually 3 to 5 cm.
• Garment: They are greater than 10 cm in diameter
Points to Remember
and can cover larger areas of the skin.
Caused by trauma, flat or elevated, white, sessile or pe- Acquired
dunculated lesion, pseudoepitheliomatous hyperplasia, • Intra-dermal nevus (common mole)
umbilicated epithelial proliferation. • Junctional nevus
• Compound nevus
Oral Melanoacanthoma • Halo nevus
• Blue (Jadassohn-Tieche) nevus
It is benign acquired pigmentation cause of dentritic
• Spitz nevus.
melanocytes in epithelium.
Clinical Features Clinical Features

Age and sex distribution: It is seen in black people with Congenital nevi: The congenital nevi, with passage of time,
female prevalence. It is most commonly seen in 3rd and 4th may change from flat, pale tanned macules to elevated,
decade of life. verrucous lesion. There is presence of hypertrichosis
(excess hair) in the lesion which becomes prominent with
Location: It is located on buccal mucosa, lip, palate,
the age (giant hairy nevus). Very large nevus is called as
gingiva, and alveolar mucosa.
bathing trunk nevus or garment nevus because it given
Sign and symptoms: There may be pain, burning, pruritis. patient appearance of wearing of article of clothing.
The lesion can reach to size of several centimeters.
Acquired nevi: Acquired nevi are extremely common and
Appearance: It is smooth, flat, dark brown to black color. appear at 8th month of life and increase in number with
Benign Tumors
age, apparently reaching their peak numerically in the late other areas. The majority of blue nevi are present at birth
third decade of life. and early childhood. They persist unchanged throughout the
life. The lesions are smooth and exhibit hair growing from
Junctional nevus: This is the earliest presentation of nevus. 175
the surface. The color of blue nevi occurs as melanocytes
It may appear clinically similar to intradermal nevus with
reside deep in the connective tissue and the overlying
chiefly being histological. Early in the life, it arises from
vessels dampen the brown coloration of melanin and thus
the basal cell layer of melanocytes. Junctional type derived
yield a blue tint. It is composed of dermal melanocytes,
name from its location. They are situated in basal layer just
which only rarely undergo malignant transformation.
above junction of epidermis and dermis. They are flat and
brown and have regular, oval and round outline. It appears Spitz nevus: It is also called as juvenile melanoma,
as brown black macules affecting, both skin, as well as the spindle cell or epitheloid cell nevus and it shares many
oral mucosal surface. histopathological features with melanoma. It occurs during
childhood on face or extremities. It appears solitary, dome
Compound nevus: This is usually 2nd stage in the
shaped, pink to reddish brown papule. Size is smaller than
formation of nevus. It is more common on skin, as compared
6 mm.
to oral mucosa. It is firm, raised nodule or polypoid mass
with smooth surface. The lesion is composed of two parts Halo nevus: It is melanocytic nevus with pale
intradermal and junctional nevus. hypopigmented border or halo of the surrounding
epithelium due to nevus cell destruction by immune
Intradermal nevi: It is most common and many patient
system. It is common on skin of trunk. It is appeared as
persons exhibit several nevi scattered over the body. It is
central pigmented macule or papule surrounded by zone of
more common in children and it is referred as common mole.
hypopigmentation.
It may be smooth flat lesion or may be elevated above the
surface. It may or may not exhibit brown pigmentation and Oral Manifestations
it often shows strands of hair growing from the surface. It
is firm on palpation and rise above the surface (Fig. 11.9). Location: Intraorally nevus can occur at any site but most
commonly occur on hard palate, buccal mucosa, and lips
Blue nevus: It is also called as dermal melanocytoma and gingiva.
or Jadassohn-Tieche nevus. It occurs chiefly on buttock,
dorsum of the feet and hands, face and occasionally on Appearance: Most nevi present as raised, macular lesions,
but some are flat and macular. They are slow growing and
their size is usually less than 1 cm in diameter.
Compound nevi appears as pigmented papules or
macules over the hard palate. Blue nevi may be macular or
nodular in appearance (Fig. 11.10).
The nevus cells are large discrete cells with an ovoid,
vesicular nucleus and pale cytoplasm. They tend to group
in sheets or cords and may contain granules of melanin
pigment in their cytoplasm. The arrangement of these cells
in an alveolar pattern is referred to as theques.
Congenital nevus: As such appearance is similar to that
of acquired nevus. In contrast to acquired type it may show
extension of nevus cells into deeper level of the dermis
with infiltration between collagen bundles.
Junctional nevus: Zone of demarcation is absent and
the nevus cells contact and seem to blend into the surface
Figure 11.9 Common mole showing hair epithelium. This overlying epithelium is usually thin and
176
Figure 11.10 Compound nevi seen on face Figure 11.12 Intradermal nevus showing presence of
melanocytes in the epithelial portion only. Connective tissue
does not contain any melanocytes
Intradermal nevi (Fig. 11.12): It is composed of bulk of

cells packed within dense collagenous connective tissue
stroma. The nevus cells are situated within the connective
tissue and are separated from the overlying epithelium
by a well defined band of connective tissue. Thus in it,
nevus cells are not in contact with the surface epithelium.
Multiple multinucleated giant cells may be seen. Few cells
are spindle shaped.
Spindle cell and epithelioid nevus: It is commonly
composed of pleomorphic cells of three basic types;
spindle cell, oval/epithelioid cells, mononuclear and
multinuclear giant cells. These are arranged in well
circumscribed sheets and there is generally, considerable
junctional activity.
Blue nevus: It is of two types, i.e. the common blue
Figure 11.11 Junctional nevus showing nevus cells at the nevus and cellular blue nevus. In the common blue nevus,
junction of epithelium and connective tissue elongated melanocytes with long branching dendritic
processes lie in bundles, usually oriented parallel to the
epidermis, in the middle and lower thirds of dermis. There
irregular and shows cells apparently crossing the junction
is no junctional activity. The melanocytes are typically
and growing down into the connective tissue–the so called
packed with melanin granules, sometimes obscuring the
abtropfung or “dropping off” effect. This junctional
nucleus. In cellular blue nevus an additional cell type is
activity has serious implication as it has been known to
present which is a large, round or spindle cell with pale
undergo malignant transformation (Fig. 11.11).
vacuolated cytoplasm. These cells are commonly arranged
Compound nevus: It shows features of both, the junctional in alveolar pattern. Blue type has stellate and fusiform cells
nevus and intradermal nevus. Nests of nevus cells are seen that contain melanin and are located deep in lamina propria.
dropping off from the epidermis while large nests of nevus Few pigmented macrophages may be present among the
cells are also present in the dermis. dendritic nevus cells (Figs 11.13 and 11.14).
Benign Tumors
177
Figure 11.13 Blue nevus showing nevus cells (Nv) arranged Figure 11.15 Spitz nevus showing multinucleated epithelioid
in small nests and theques in connective tissue. Overlying cells
epithelium is normal
begin to increase in size, deepen in color or become
ulcerated.
Points to Remember
• C ongenital nevi: Flat, pale tanned macules to
elevated, verrucous, hypertrichosis, giant hairy
nevus, bathing trunk nevus or garment nevus.
• Junctional nevus: It arises from the basal cell layer of
melanocyte, situated in basal layer just above junction
of epidermis and dermis, flat and brown regular, oval
and round outline. Extension of nevus cells into
deeper level of the dermis zone of demarcation is
absent, abtropfung” or “dropping off” effect.
• Compound nevus: Firm, raised nodule or polypoid
mass with smooth surface features of both, the
Figure 11.14 Blue nevus showing nevus (Nv) cells in sheets
Junctional nevus and intradermal nevus.
and melanin pigmentation (M) • Intradermal nevi: Common mole, smooth flat lesion
or elevated lesion, shows strands of hair bulk of cells
packed within dense collagenous connective tissue
Halo nevus: In this there is presence of intense chronic stroma, multiple multinucleated giant cells.
inflammatory cell infiltrate. • Blue nevus: Dermal melanocytoma, smooth and
Spitz nevus: Cells are spindle shaped (epithelioid) or exhibit hair growing from the surface, blue color
plump. Epithelioid cells are multinucleated arranged in elongated melanocytes with long branching dendritic
bizarre pattern. There is lack of cell cohesiveness (Fig. processes large, round or spindle cell with pale
11.15). vacuolated cytoplasm.
• Spitz nevus: Spindle cell or epitheloid cell nevus-
Management solitary, dome shaped, pink to reddish brown spindle
It has been customary to recommend the removal of cell, oval/epithelioid cells lack of cell cohesiveness.
pigmented mole if it occurs in areas where they are irritated • Halo nevus: Pale hypopigmented border or halo
by clothing, such as belt of collar line or if they suddenly chronic inflammatory cell infiltrate.
FIBROUS CONNECTIVE TISSUE

Fibroma
178
It is also called as irritation fibroma, traumatic fibroma,
focal fibrous hyperplasia, and fibrous nodule.
It is a benign soft tissue tumor found in the oral cavity.
True benign neoplasm of the fibrous tissue is relatively
an infrequent lesion. Most of these lesions are in fact,
hyperplasia or reactive proliferation of fibrous tissue.
Clinical Features
Age and sex distribution: It can occur at any age but
is common in 3rd, 4th and 5th decades. There is no sex
predilection. Figure 11.17 Fibroma presents as a smooth surfaced firm
soft tissue growth (Courtesy: Dr Swapnil Moghe)
Location: It occurs on the gingiva, tongue, buccal mucosa
and palate. Irritation fibroma occurs at bite line on buccal
mucosa.
Symptoms: They are usually painless, but if they are in a
position where they can be bitten or injured, there may be
pain and discomfort.
Sign: It is most often sessile, dome shaped or slightly
pedunculated with smooth contour. The lesions on lips
and tongue present as circumscribed nodules (Figs 11.16
to 11.18).
Tumor sometimes becomes irritated and inflamed and
may show superficial ulceration.
Size and consistency: Tumor may be very small or in rare
instances may range up to several centimeters in diameter.
The consistency can range from soft and myxomatous to
firm and elastic (Fig. 11.19). Figure 11.18 Excised fibroma with pedicle
Figure 11.16 Fibroma occurring on tongue which is dome Figure 11.19 Fibrous hyperplastic growth due to chronic
shaped irritation caused by sharp teeth
Benign Tumors
Frenal tag: It is commonly seen at maxillary labial frenum Table 11.2 Differences between fibroma and inflamma-
which appear as exophytic growth attached to it. tory fibrous hyperplasia
According to the consistency the tumor is termed as
Characters Fibroma Inflammatory 179
‘hard fibroma’ and ‘soft fibroma’. Most of fibroma is
fibrous hyperplasia
peripheral, central variety is rare in oral cavity.
Nature Neoplasia of Inflammatory process
Histopathological Features connective tissue
origin
Fibroma consists of dense bundles of collagen fibers which
Etiology Constant irritation Trauma
interlace with each other. These bundles are associated
with fibroblasts with are plump cells which actively secret Reversibility Does not regress Promptly resolves
even after removal of once irritant is
collagen fibers.
cause removed
There is also blunt rete pegs or no retepegs with
parakeratinized stratified epithelium (Fig. 11.20). Epithelium Stretched and Proliferative with
atrophic pseudoepithelium
In addition to these inactive fibrocytes are also seen.
atous hyperplasia
As collagen accumulation increase it creates tension on the
surrounding epithelium. This causes epithelium to stretch Inflammation It is seen only if Inflammation is
resulting in epithelial atrophy. lesion is traumatized integral part of the
As fibroma is growth seen on the mucosa it is lesion.
frequently subjected to trauma. If trauma to tissue occurs,
vasodilation, edema and inflammatory cell infiltration will
be seen. Points to Remember
Sometimes diagnosing fibroma from inflammatory Irritation fibroma, on the gingiva, usually painless,
fibrous hyperplasia becomes difficult. It is inflammatory sessile, dome shaped, superficial ulceration, frenal tag,
lesion caused by trauma to the tissue that heals by formation hard fibroma, soft fibroma, dense bundles of collagen
of fibrous tissue. When healed completely these resemble fibers, fibroblasts, plump cells, inactive fibrocytes,
fibroma. But still there are some differences between both vasodilation, edema, inflammatory cell infiltration.
the lesions (Table 11.2).
Management
It is treated by conservative surgical excision.
Fibrous Histiocytoma
It is also called as ‘fibroxanthoma’, ‘dermatofibroma’,
sclerosing hemangioma’, ‘nodular subepidermal fibrosis’.
It may occur in dermis and is rare in oral cavity. It may
arise from tissue histiocytes which undergo fibroblastic
properties.
Clinical Features
Age and sex distribution: It is common in young adults,
with male predominance.
Location: It is common on extremities where it is called as
dermatofibroma. Intraorally it is common on lips, tongue,
buccal mucosa and palate.
Figure 11.20 Parakeratinized stratified epithelium with short Sign and symptoms: There is soft, nontender, firm
and blunt retepegs (Courtesy: Dr Aparna Thombre, Reader, swelling of varying size. There is displacement of regional
Department of Oral Pathology, VSPM Dental College and teeth. It is a locally aggressive tumor.
Hospital, Nagpur)
Histopathological Features Appearance: Clinically, it is present with swelling of the

jaw and occasionally with associated pain and tenderness.
A benign richly vascular growth, made up of histiocytes
180 and collagen producing fibroblast like cells, which are Sign: Buccal cortex is enlarged and more advanced lesions
arranged in a whorl or cartwheel pattern due to resembles exhibit facial asymmetry. Lesions are aggressive and
to the irregular whorled appearance of straw mat. proliferate rapidly.
Fibroblasts are elongated spindle shaped cells, which
Radiographic features: It is multilocular or unilocular
synthesize collagen. Histiocytes are large cells with oval
nuclei and very thin cytoplasm. radiolucent area with ill or well defined margin. Expansion
of bone with thin cortex is also present. Displacement of
Management adjacent teeth and root resorption is also common.
Surgical excision can be done and recurrence is uncommon.
Points to Remember Juvenile fibromatosis
Fibroxanthoma, dermatofibroma, soft, nontender, firm There is cellular proliferation of spindle shaped cells
swelling, histiocytes, collagen producing fibroblast which are arranged in streaming fascicles. It is associated
like cells, whorl or cartwheel pattern, elongated spindle with variable amount of collagen.
shaped cells.
Desmoplastic fibroma
Fibromatosis It is composed of cells that may be either small or uniform,
plump, or both. The fibrous product is generally mature
It is group of fibrous proliferation which is intermediate
with thick, wavy collagen fibers arranged in fascicle.
between benign proliferation and fibrosarcoma.
The collagen fibers are usually thin and delicate with
Types fasciculation produces a ‘herring bone’ or ‘chevron’ or
∙ Juvenile aggressive fibromatosis or extra-abdominal ‘storiform’ pattern.
desmoids: This is soft tissue lesion. Bone spicule may be present between tumor and
∙ Desmoplastic fibroma: It is intraosseous counterpart adjacent bone.
of fibroma. It arises from the mesenchyme of bone.
Management
Wide local excision is the treatment of choice in these
Clinical Features cases. Involved teeth should be extracted.
Juvenile fibromatosis Curettage: It should be done for localize lesion without
Age and sex distribution: It is most commonly seen in cortical perforation.
children and young adults with no sex predilection.
Points to Remember
Location: Most common intraoral site is paramandibular
• J uvenile fibromatosis: Paramandibular soft tissue
soft tissue region.
region, facial disfigurement, Gardener’s syndrome.
Sign: Facial disfigurement due to large lesion is present. cellular proliferation of spindle shaped cell, collagen
Destruction of adjacent bone is present on radiograph. • Desmoplastic fibroma: Mandible, swelling of jaw,
It may be associated with Gardener’s syndrome. enlarged buccal cortex, multilocular or unilocular
radiolucent area small or uniform, plump, thick,
Desmoplastic fibroma
wavy collagen fibers, herring bone or ‘chevron’ or
Age and sex distribution: It is most commonly occurs in storiform, bone spicule.
2nd decade of life. There is no sex predilection.
Location: In oral cavity it involves mandible more Giant Cell Fibroma
commonly than maxilla, usually at molar-ramus-angle It is a well described, benign hyperplastic lesion of oral
area. mucosa. Although name seems to indicate presence of
Benign Tumors
giant cells in this lesion no multinucleated giant cells are

found. Instead large stellate fibroblast which single dark
nucleus are seen. Difference between fibroma and giant
cell fibroma are described in Table 11.3. 181
Clinical Features
Age and sex distribution: It occurs at young age as
compared to irritation fibroma. It is slightly more prevalent
in women.
Location: It is commonly seen in gingiva followed by
tongue, palate, buccal mucosa and lips. Mandibular gingiva
affected more commonly than maxillary gingiva.
Appearance: It is usually small, raised, pedunculated,
papillary lesion, less than 1 cm in diameter. Figure 11.21 Giant cell fibroma showing many stellate
shaped fibroblasts
Symptoms: It is asymptomatic and may be present for
several years.
There are few which are similar to giant cell fibroma.
These are retrocuspid papilla, fibrous papule of nose, acral
fibrokeratoma, fibroblastoma, pearly penile papule of the
glans penis.
This tumor is also similar to fibroma. But there is presence
of characteristic giant cells. These are large stellate giant
fibroblasts in the connective tissue (Figs 11.21 and 11.22).
The nuclei with giant cells are large, hyperchromatic
and vesicular. Stellate cells have large well demarcated
cytoplasm and processes which give star shape to the cells.
These giant cells present at the surface in more numbers.
Some cells may occasionally contain melanin pigment.
The surface of the lesion is covered by mucosal Figure 11.22 High power view of giant cell fibroma showing
squamous epithelium, which may exhibit areas of stellate fibroblasts dendritic-like
thickening and acanthosis in the form of deeply extending

Table 11.3 Differences between fibroma and giant cell
but narrow and tapering rete pegs.
fibroma
Characters Fibroma Giant cell fibroma Management
Etiology Chronic irritation Unknown Complete surgical excision is treatment of choice.
Age Commonly after age Frequently during Recurrence is rare.
of 30 years third decade.
Size Larger (1-2 cm) Smaller than fibroma Points to Remember
(5 mm) No multinucleated giant cells are found, small, raised,
Site More commonly on More commonly on pedunculated, papillary lesion, asymptomatic, large
cheek and lips gingival stellate giant fibroblasts, hyperchromatic nuclei, mucosal
Surface Smooth Lobulated or papillary squamous epithelium, thickening and acanthosis.
Oral Focal Mucinosis Location: It is seen in maxillary arch and exclusively seen
on gingiva in incisor-cuspid region.
This results from overproduction of hyaluronic acid by
182 fibroblasts. It represents oral counterpart of cutaneous Appearance: It appears as nodular mass which is sessile
focal mucinosis. and pedunculated. This emanates from interdental papillae
(Fig. 11.23).
Clinical Features
Sign and symptoms: Color of lesion ranges from red to
Age and sex distribution: It is more common in female
pink. Surface is ulcerated in many cases.
and young adults.
Location: It is most common on gingiva followed by Histopathological Features (Figs 11.24A and B)
palate. There is fibrous proliferation with mineralization.
Mineralized component may be bone, cementum like
Appearance: It presented as sessile or pedunculated,
material or dystrophic calcification. In some cases
painless nodular mass with smooth surface. In some cases
multinucleated giant cell are seen.
lobulated appearance can be seen.
Sign: Size of lesion varies from few millimeters to less Management
than 2 cm in diameter. Local surgical excision with deep incision in the periosteum
should be done.
There is nonencapsulated area of loose myxomatous Points to Remember
connective tissue surrounded by dense collagenous Ossifying fibrous epulis, nodular mass which is sessile
connective tissue. and pedunculated, color red to pink, fibrous proliferation
The fibroblast is ovoid, fusiform or stellate which with mineralization.
demonstrate fibrillar process. Few capillaries are also seen
in the lesion. Myxoma
Management It is composed of stellate cells arranged in a loose myxoid
stroma, which also contains delicate reticulin fibers. Soft
It is treated by surgical removal.
tissue myxoma is very rare in the oral cavity.
Points to Remember
Overproduction of hyaluronic acid by fibroblasts, sessile
or pedunculated, painless nodular mass, nonencapsolated
area of loose myxomatous connective tissue, fibroblast
is ovoid, fusiform or stellate.
Peripheral Ossifying Fibroma

It is also called as ‘ossifying fibrous epulis’, ‘peripheral
fibroma with calcification’, calcifying fibroblastic granu-
loma’.
Long standing chronic peripheral granulomas may also
transform into ossifying fibroma as a result of subsequent
calcification in response to chronic inflammation and
reparative process.
Clinical Features
Figure 11.23 Clinical photograph of peripheral ossifying
Age and sex distribution: It is seen in young adults in 2nd fibroma (Courtesy: Dr Aparna Thombre, Reader, Department of
decade of life with more commonly seen in females. Oral pathology, VSPM Dental College and Hospital, Nagpur)
Benign Tumors
∙ Sign and symptoms: It is slow growing, expansive,

insidious infiltration with period of accelerated growth.
True myxoma does not metastasize but is considered to
be destructive. It is usually asymptomatic. 183
The soft tissue myxoma present characteristically with
a loose textured tissue containing moderate number of
delicate reticulin fibers and myxoid material. It consist
of abundant ground substance with wide intercellular
spaces. The tumor is not encapsulated and may invade the
surrounding tissues (Fig. 11.25).
A Management
It is essentially surgical, recurrence is common.
Points to Remember
Stellate cells, delicate reticulin fibers, nerve sheath myxoma,
arise from perineural cells, slow growing, expansive,
insidious infiltration, loose textured tissue containing
moderate number of delicate reticulin fibers myxoid tissue.
Myxofibroma
Some areas of fibroma undergo myxomatous degeneration.
This is called as myxofibroma.
Clinical Features
It occurs anywhere in the oral cavity, most commonly on
palate, lip and gingiva. It feels softer than fibroma and is
B less pale.
Figures 11.24A and B Peripheral ossifying fibroma (Courtesy:
Dr Aparna Thombre, Reader, Department of Oral Pathology,
VSPM Dental College and Hospital, Nagpur, Maharashtra, India)
Clinical Features
Age and sex distribution: It can occur at any age and there
is no definite sex predilection.
Location: They are deeply situated lesions, occurring in
the skin of the subcutaneous tissues, genitourinary tract and
gastrointestinal tract or in organs such as liver, spleen, or
even in parotid gland. Intraorally, it occurs on the tongue,
buccal mucosa and retromolar area.
The nerve sheath myxoma is a benign tumor thought
to arise from perineural cells of peripheral nerves and is
characterized by occurrence of stellate cells in prominent Figure 11.25 Myxoma showing loose connective tissue stroma
myxoid matrix. composed of delicate reticulin fibers with myxoid background
Histopathological Features Signs and symptoms: It is painless, slowly growing

and is locally invasive. Teeth become loose and may be
There are regions of dense fibroblastic tissue which are
exfoliated. The overlying mucosa is seldom ulcerated (Fig.
184 interspersed with pale myxomatous appearing tissue.
11.26).
Management It is associated with Ollier’s syndrome, in which there
are multiple enchondromatosis.
Complete excision of lesion should be carried out.
Radiological features: They appear as radiolucency with
CARTILAGE central area of radiopacity (Fig. 11.27).
Chondroma Histopathological Features

It is a benign cartilaginous tumor. In spite of the fact Chondroma is cartilage producing tumor. It is made of
that mandible and maxilla are membranous bones, they mass of hyaline cartilage, which may exhibit areas of
sometimes contain vestigeal rests of cartilage. It often calcification or necrosis.
develops after adolescence (in contrast to osteochondroma)
Types
∙ Central chondroma (Enchondroma): It develops
deep into the bone (within the medullary cavity). It is
more commonly seen.
∙ Periosteal chondroma (Ecchondroma): It develops
on the surface.
∙ Soft tissue chondroma in the soft tissue.
Origin
Meckel’s cartilage is present in the mandibular arch, prior
to the appearance of the bone. It usually disappears with
the occurrence of ossification in the mandibular arch, but it
is possible that the remnants still persist.
In some cases secondary cartilage like fibrocartilage Figure 11.26 Chondroma usually presents as a hard bony
of mandibular symphysis may persist in the jaw bone expansile growth
and can give rise to chondroma. They often develop after
adolescence (in contrast to osteochondroma).
The maxilla develops in close association with
chondrocranium. The maxillary sinus develops as an
outgrowth from the lateral walls of the nasal capsule. As it
grows into the maxilla, it may take with it remnants of the
cartilage from the capsule. In some cases, remnants of the
paraseptal cartilage might persist within the maxilla.
Clinical Features
Age and sex distribution: It occurs in the 5th and 6th
decades and males are slightly favored.
Location: It usually occurs in the phalanges and
metacarpals. Intraorally, maxilla is slightly favored and it
occurs in the anterior region while in mandible, it occurs
in premolar-molar region and at symphysis; it may also be Figure 11.27 Chondroma showing buccolingual cortical
found in condyle and coronoid process. expansion with change in radiodensity
Benign Tumors
Tumor shows hypocellularity and it is avascular.

Points to Remember
The cartilage cells or chondrocytes appear small, contain
only single nuclei and do not exhibit great variation in size, Origin from Meckel’s cartilage, painless, slowly
shape or staining reaction. These are present in sharp edged growing and is locally invasive, Ollier’s syndrome, 185
lacunae. These are surrounded by large amount of ground radiolucency with central area of radiopacity, mass of
substance. Ground substance stains with blue color. The hyaline cartilage, cartilage cells or chondrocytes appear
cytoplasm of the cells is slightly eosinophilic, granular and small, sharp edged lacunae, excised along with the lining
often shows presence of vacuoles (Figs 11.28 and 11.29). capsule.
Management Chondroblastoma
It should be excised along with the lining capsule. It should It is also called as ‘Codman’s’ tumor. It usually involves
be covered by chip graft. Recurrence is common. long bone.
Clinical Features
Age and sex distribution: It occurs in young persons,
under the age of 25 years, with male predominance over
the female, usually in the ratio of 2:1.
Location: It occurs usually in epiphysis region of long
bones. The usual site is femur and tibia. There are reports
that it can occur in condyle of mandible.
Signs and symptoms: It is slow growing, painless mass.
The tumor is composed of relatively uniform, closely
packed, polyhedral cells, with occasional foci of chondroid
matrix (Fig. 11.30).
Figure 11.28 Chondroma showing small chondroblasts A scattering of multinucleated giant cells may be found,
enclosed in lacunae and producing large amount of ground usually associated with areas of hemorrhage, necrosis or
substance calcification of the chondroid material. Such giant cells
resemble osteoclasts. Formation of bone and osteoid also
occurs. The calcification is present around the cells and
follows chicken wire arrangement.
Management
Conservative surgical excision is carried out. Recurrence is
possible after excision.
Points to Remember
Codman’s’ tumor, epiphysis region of long bones,
slow growing, painless mass, uniform, closely, packed,
polyhedral cells, occasional foci of chondroid matrix,
multinucleated giant cells, hemorrhage, necrosis, calci-
fication of the chondroid material.
Chondromyxoid Fibroma
Figure 11.29 Chondroma showing chondroblast under high
power It is an uncommon benign tumor of cartilaginous derivation.
Types of Lipoma
∙ Encapsulated lipoma: It is the most common tumor
186 ∙ Diffuse lipoma: It does not possess typical features of
lipoma. It is also called as ‘pseudolipoma’
∙ Lipomatosis: It has multiple lipomas. It refers to the
symmetrical masses of fat, which sometimes occurs
around the neck in middle aged man and occurs as
painful deposits of fat in women in Dercum’s disease
Clinical Features (Figs 11.31 to 11.35)

Age and sex distribution: It occurs age after 40 years with
peak at 50 years, male to female ratio is 1:1.
Figure 11.30 Chondroblastoma showing giant cells and
polyhedral cells
Clinical Features
Age and sex distribution: It occurs in young persons
under the age of 25 years, with no definite sex predilection.
Location: It is extremely rare in jaws, but there are some
cases reported in mandible.
Symptoms: Pain is outstanding feature of this disease and
sometimes, swelling can be seen.
Radiological features: It is circumscribed radiolucent
defect with sclerotic or scalloped margins.
Figure 11.31 Lipoma presented as round and lobulated mass
It exhibits lobulated myxomatous and fibrous areas and has
a chondroid appearance. Chondrocytes lie in lacunae in a
chondroid matrix. Foci of calcification are with also seen.
There is also presence of osteoblast in form of
multinucleated giant cells.
Management
Conservative surgical excision can be done.
Points to Remember
Pain, circumscribed radiolucent defect, myxomatous
fibrous areas and has a chondroid appearance, multi-
nucleated giant cells.
ADIPOSE TISSUE
Lipoma
Figure 11.32 Lipoma presents clinically as a dome shaped
It seldom occurs in oral cavity. It is a benign, slow growing, compressible pedunculated/sessile growth of pale pink color
tumor composed of mature fat cells. with a smooth shiny surface
Benign Tumors
187
Figure 11.33 Lipoma showing translucent swelling on the Figure 11.35 Gross specimen of lipoma showing yellowish
cheek mucosa jelly like content of adipose tissue. This specimen floats in the
formalin fixative solution due to low density
broadly based or have narrow pedicle. Due to thinness of

the overlying epithelium, yellow coloration of the fat can
be seen.
Most of the lesions are fluctuant and are not freely
movable. Surface is smooth, nontender, soft and cheesy
in consistency. The epithelium is usually thin and the
superficial blood vessels are readily visible over the
surface. Some lesions of lipoma are deep and feel fluid on
palpation; may be mistaken as cysts.
Slip signs, i.e. the edge of lipoma is soft, compressible
and often slips away from the examining fingers is positive
in this case.
Transillumination test is also positive.
Lipoblastoma/Lipoblastomatosis: It is a variant of lipoma,
Figure 11.34 Lipoma showing ovoid mass (Courtesy: Dr Aparna but is not a true neoplasm. The term “lipoblastoma” first
Thombre, Reader, Department of Oral Pathology, VSPM Dental used by Jaffe in 1926. It is the continuation of the normal
College and Hospital, Nagpur, Maharashtra, India) process of fetal fat development carried into the postnatal
life. It is characterized clinically by the occurrence in
infants of solitary or multiple soft tissue masses, developing
Location: It usually occurs in upper parts of the trunk, neck at various sites such as the buttocks, chest, axilla or neck. It
and arms. In oral cavity, it occurs on buccal mucosa and comprises of less than 1 percent of head and neck cancers.
mucobuccal fold followed by tongue, floor of mouth and These neoplasms occur almost exclusively in infants and
lip. It appears as a solitary lesion with sessile, pedunculated children younger than 3 years of age. The histological type
or submerged base. includes well differentiated, myxoid, pleomorphic, and
Size of lesion is approximately of 1 cm in diameter. round cell.
Sign: Margins are well contoured, well defined, round Hibernoma: This is developed as multi-vacuolated fat
to large ill defined lobulated mass. It grows as round or that is analogous to the brown fat of hibernating animals;
ovoid mass in oral cavity. It may be lobulated or may be however lesions in oral cavity are not reported.
Histopathological Features (Figs 11.36 to 11.39)

It is made of circumscribed mass of mature fat cells. These
188 cells are arranged in lobular pattern which is formed by
connective tissue septae coursing through the tumor. These
septae carry blood vessels and nerve fibers.
Fat cells are large polygonal cells. They contain large
amount of fat. This fat pushes the cytoplasm and nucleus to
the periphery. Nucleus is flattened against the cell wall.
Histological Types of Lipoma

Angiolipomas: Painful and usually arise shortly after
puberty.
Figure 11.38 Lipoma high magnification
Figure 11.36 Lipoma showing collection of adipocytes (A)

laden with fat. Connective tissue septa (CS) is seen
A
B
Figures 11.39A and B Lipoma (Courtesy: Dr Aparna Thombre,
Figure 11.37 Lipoma showing eccentric and pathognomonic Reader, Department of Oral Pathology, VSPM Dental College
signet ring appearance of adipocytes (high power view) and Hospital, Nagpur)
Benign Tumors
Pleomorphic lipomas: Bizarre, multinucleated giant cells

Types
are admixed with normal adipocytes.
∙ Compact osteoma (Ivory osteoma): It consists of
Spindle cell lipomas: Slender spindle cells admixed in a
compact bone, which has dense lamellae of bone. 189
localized portion of regular-appearing adipocytes
∙ Cancellous osteoma: Consisting of trabeculae of
Superficial lipoma, adenolipoma: Characterized by the bone.
presence of eccrine sweat glands in the fatty tumor (often ∙ Periosteal, peripheral or exophytic osteoma: Arise
located on the proximal parts of the limbs). on surface of bone as sessile mass.
Intramuscular lipoma (infiltrating): They are deeply ∙ Endosteal or central osteoma: It is located in
situated and infiltrative growth pattern. medullary bone.
∙ Osteoma cutis: It is extraskeletal lesion of soft tissue
Fibrolipoma: Significant fibrous component intermixed
seen in dermis of skin.
with fats cells.
Management Etiology
Surgical excision and recurrence is uncommon. The exact etiology and pathogenesis of osteoma is
unknown. Both hamartomatous and neoplastic factors have
Points to Remember been suggested.
Margin are well contoured, lobulated, lesions are Role of neoplastic factors was considered due to
fluctuant, deep and feel fluid on palpation, slip presence of infiltration of interdental bone and abnormal
signs, positive transillumination test, Lipoblastoma/ histological bone structures. Developmental, neoplastic
lipoblastomatosis, hibernoma, circumscribed mass of and reactive causes have been attributed in past.
mature fat cells, lobular pattern, large polygonal cells,
nucleus is flattened. Clinical Features
Compact osteoma
BONE Age and sex distribution: It occurs in individuals older
than 40 years. It is more common in males, as compared
Osteoma to females.
Osteoma was described as a specific entity by Jaffe in 1935. Location: It occurs exclusively on skull and facial bone.
It is a benign often asymptomatic neoplasm characterized It may occur in more than one bone. Mandible is more
by proliferation of either compact or cancellous bone affected on the lingual side of ramus and inferior border,
consisting of well-differentiated matured bone. It usually below the molars.
occurs in endosteal or periosteal location.
Sign and symptoms: There is asymmetry caused by
Origin bony hard swelling of jaw. It is usually painless. Mucosa
It may arise from cartilage or embryonic periosteum. It can is normal in color and freely movable. Mandibular lesion
be central, peripheral, or of an extraskeletal type. may be exophytic extending outwards in soft tissues.
The central osteoma arises from the endosteum, Condylar lesion will cause shift in the patient occlusion
the peripheral osteoma from the periosteum, and the with midline of chin towards affected side.
extraskeletal soft tissue osteoma usually develops within Cancellous osteoma
soft tissue. Age and sex distribution: It more commonly occurs in
It can be either periosteal types arise on the surface females with age same as for ivory osteoma.
of bone as a pedunculated mass or endosteal is located
in the medullary bone. Histologically, it can compact or Location: There is predilection to occur in the alveolar
cancellous osteoma. process.
Appearance: It is usually pedunculated, although it might

have a broad base. The surface may be smooth or slightly
irregular.
190
Osteomatosis: Multiple osteomas may occur as a feature
of Gardner’s syndrome. Multiple osteomas can also occur
in absence of other abnormalities. They have been reported
in the mandible, frontal and maxillary sinus. Multiple
osteomas rarely occur in soft tissue.
Radiological features: It appears as circumscribed
sclerotic mass (Fig. 11.40).
Histopathological Features (Figs 11.41 and 11.42)

The tumor is composed of either extremely dense compact
bone or of coarse cancellous bone.
Figure 11.41 Cancellous osteoma showing bony trabeculae
Cancellous osteomas are composed of trabeculae of enclosing marrow spaces (MS)
cancellous bone and the marrow tissue is fatty. Prominent
osteoblastic activity can be seen.
Compact osteoma consists of a dense mass of lamellar
bone with very few marrow spaces. It is most often well
circumscribed and in some cases, foci of cartilage can be seen.
Management
Resection of osteoma is generally successful.
Points to Remember
Proliferation of either compact or cancellous bone,
asymmetry caused by bonya hard swelling, condylar lesion
will cause shift in the patient occlusion, osteomatosis,
circumscribed sclerotic mass, dense compact bone or of
coarse cancellous bone, prominent osteoblastic activity,
dense mass of lamellar bone, resection.
Figure 11.42 Cancellous osteoma showing bony trabaculae
with overlying oral epithelium and containing fibro-fatty
marrow (Courtesy: Dr Alka Kale, Dean, KLES’s Institute of
Dental Sciences, Belgaum)
Osteoblastoma
It is also called as ‘giant osteoid osteoma’. Osteoblastoma
is a rare benign bone forming neoplasm which produces
woven bone spicules, which are bordered by prominent
osteoblasts.

Figure 11.40 Circumscribed sclerotic mass seen on Age and sex distribution: Male to female ratio is 2:1.
radiograph Most lesions occur in 2nd and 3rd decades of life.
Benign Tumors
Location: It more commonly occurs in vertebral column

and sacrum. It is rare in jaws and if occurs, is found more
commonly in tooth bearing areas of mandible.
191
Signs and symptoms: It is characterized by pain and
swelling of the affected region, which may be of few weeks
to a year duration. There is local expansion of the bone.
Aggressive osteoblastoma: This tumor have locally
aggressive behavior. These tumor are larger than
conventional osteoblastoma.
Radiological features: It appears as well defined or
ill defined radiolucent lesion with patchy distribution
mineralization.
Histopathological Features Figure 11.43 Benign osteoblastoma showing irregular bony

Histopathologically, it resembles osteoid osteoma. It is trabeculae
made up of spicules of woven bone. These spicules are
arranged haphazardly (Fig. 11.43). Cancellous
Many giant cells are seen in the tumor. Numerous Intramedullary lesion; mild reactive sclerosis and difficult
osteoblasts line these bony trabeculae. These tumors are to identify, significantly delaying the diagnosis
quite vascular. Common sites: Femoral neck, posterior spine, hands
Many dilated capillaries are seen scattered throughout and feet.
the tissue. The actively proliferating osteoblasts pave way
for the irregular trabeculae of new bone. Intra-articular
Joint effusion or synovitis.
Management
Subperiosteal
Osteomas are diagnosed and treated by local excision.
Round mass adjacent to cortex
Points to Remember Absent periosteal reaction
Very rare.
Giant osteoid osteoma, pain and swelling of the affected
region, aggressive osteoblastoma, well defined or ill Clinical Features (Table 11.4)
defined radiolucent lesion, spicules of woven bone,
numerous osteoblasts many dilated capillari. Age: It occurs in males between 10 to 15 years ages.
Location: Any part of the skeleton is involved, including
Osteoid Osteoma the small bone of the hands, feet and vertebrae. The skull
It is small variant of osteoblastoma. It present as a small, and jaws are rarely involved, with slight predilection for
ovoid hard tumor. It has a characteristic central calcified the mandible.
nidus around which deposition of osseous tissue takes place. Symptoms: The main feature of this neoplasm is severe
pain, in spite of the small size of the lesion. Pain usually
Classification of Osteoid Osteomas occurs at night.
Cortical Sign: Soft tissues over the involved bone area may be
∙ Most common location (80%) swollen and tender. It is an oval or round tumor like lesion.
∙ Radiolucent nidus seen within the bone cortex and It has a core of about 1 cm in diameter. There tends to be
surrounded by fusiform cortical thickening/laminated a marked reaction, which may extend for a considerable
periosteal formation. distance from the tumor itself.
Table 11.4 Differences between osteoid osteoma and

composed of osteoid and newly formed trabeculae within
osteoblastoma highly vascularized osteogenic connective tissue.
From this core osteoblasts differentiate towards surface.
192 Osteoid osteoma Osteoblastoma
Formation of definite trabeculae, with rimming of active
Usually < 2 cm diameter Usually > 2 cm diameter osteoblasts occurs. Osteoclasts and foci of bone resorption
Presents with intense pain, Lack of intense pain are also usually evident.
often sharply localized and Aggressive behavior
worse at night Typically in the vertebrae Management
Pain characteristically or major bones of the lower
Medical management: NSAIDs like salicylates relives
relieved by aspirin/NSAIDs extremity
Non-aggressive behavior Often affects the spongiosa of the pain.
Variable locations: Femur, the bone Surgical management: Open surgical curettage and CT
tibia, fibula, humerus, hands/ Absence of neural axons upon guided percutaneous radio‐frequency (RF) ablation, laser,
feet, vertebrae. staining ethanol, or thermocoagulation therapy.
Neural staining reveals axons
throughout the tumor (may Points to Remember
explain bone pain)
Unknown etiology, elevated prostaglandin E2 levels
in nidus responsible for bone pain and vasodilatation,
tumor infarction may be involved during cases of
spontaneous regression, cortical osteoid osteomas are
often associated with nonaggressive periosteal reactions,
OOs and osteoblastomas are histologically similar but
differ in size, pain intensity, location, aggressiveness,
and neural staining patterns. OOs are classified by their
relative position to the bone: cortical, cancellous, intra-
articular, and subperiosteal.
Osteochondroma
Osteochondroma or solitary osteocartilaginous exostosis
is characterized by a cartilage-capped, osseous projection
protruding from the surface of affected bone.
It is considered the most common tumor of skeletal
Figure 11.44 Osteoid osteoma showing central nidus around bones. These tumors are considered to be developmental
which bony trabeculae enclosing marrow spaces are formed: lesions rather than true neoplasms and are often referred to
CN–Central nidus; T–Trabeculae; MS–Marrow space as an osteocartilaginous exostosis (or simply exostosis).
It is most likely to represent a choristoma, rather than a
neoplasm. There is intermingling of two lesions resulting
Radiographic Features in the term osteochondroma. They are very common and
There is well-defined radiolucent nidus with surrounding account for 10 to 15 percent of all bone tumors and 20 to
zone of sclerosis. Central nidus is typically < 1.5 cm in 50 percent of all benign bone tumors. It may be of central
diameter. and peripheral types or solitary and multiple types.
Angiography shows highly vascular central nidus,
intense circumscribed blush that develops during the early Etiology
arterial phase and persists into venous phase is diagnostic. Several theories have been suggested to explain the
pathogenesis of osteochondroma. Few of them are
Histopathological Features explained as follows:
Osteoid osteoma consists of bony trabeculae that are Osteochondromas are most likely caused by either a
formed around a central core (Fig. 11.44). The core is congenital defect or trauma of the perichondrium.
Benign Tumors
According to Keith et al, this tumor resulted from defects Hereditary multiple exostoses (HME); (Familial osteo-
in the periosteal cuff and herniation of the epiphyseal plate chondromatosis diaphyseal aclasis): It is an autosomal
cartilage during fetal growth and development. dominant condition that can lead to both sessile and
Lichtenstein theorized the periosteum has the potential pedunculated lesions. The lesions may occur on different 193
to develop osteoblasts and chondroblasts. bones or on the same bone, and symptoms present in the
According to Virchow’s physeal theory, a portion of first decade of life. The risk of malignant transformation to
the physeal cartilage becomes separated from the parent chondrosarcoma in hereditary multiple osteochondromatosis
tissue then rotates 90� and grows in a direction transverse is unknown, but may be 25 to 30 percent compared to
to the long axis of the bone. approximately 1 percent for a solitary osteochondroma.
Clinical Features Complications: It includes fracture, vascular compromise,

neurologic sequelae, bursa formation, and malignant
Age and sex distribution: The mean age at diagnosis is transformation.
usually 40 years. Lesions grow even after patient attains
skeletal maturity. Females are more prone for the lesion. Radiographic Features
Location: It is relatively uncommon in the jaws. When It usually appears smooth, regular surface and projection of
jaws are affected, it occurs in coronoid process followed the cortex in continuity with the underlying bone.
by the condyle, symphysis and other sites occasionally. Irregular calcifications are often seen. CT scan or MRI
The lesion is usually discovered incidentally on images demonstrate continuity of marrow space into lesion.
radiographic examination or on palpation of a protruding The diagnosis of osteochondroma is based on clinical and
mass in the affected area. radiographic findings.
In case of condylar osteochondroma, malocclusion in
the form of a lateral open bite on the contralateral side and Histopathological Features (Fig. 11.45)
progressive facial asymmetry is seen. Pain may precede or This tumor consists of three layers, i.e. outer perichondrium,
accompany facial asymmetry. cartilage and innermost layer of bone.
Signs and symptoms: Clinically, osteochondroma present Perichondrium is continuous with the periosteal layer
with pain due to mechanical irritation or a painless mass. of bone in which this tumor develops. Beneath this layer
Other signs and symptoms of TM joint dysfunction which chondrocytes are clustered. This arrangement gives
may be present are swelling, pain, clicking, malocclusion, appearance of a growth plate.
facial asymmetry and posterior apertognathia. Chondrocytes form cartilage that undergoes ossifica-
On gross examination, an osteochondroma is an tion. Bone may contain normal bone marrow.
irregular bony mass with a bluish gray cap of cartilage.
Opaque yellow cartilage has calcification within the
matrix. The base of the lesion has a rim of cortical bone and
central cancellous bone. The risk of malignant degeneration
increases as the number and size of the osteochondroma
increases. In general, a sessile lesion is more likely to
degenerate into sarcoma than an exostosis.
Peripheral osteochondroma: Tongue is the most common
site of involvement in the oral cavity. On the tongue, it
appears as a pedunculated swelling of about 1 to 2 cms in
the posterior part of the dorsum of tongue, near the foramen
cecum. It has broad base.
Variants of osteochondroma include subungual exostosis,
dysplasia epiphyseal is hemimelica, turret exostosis,
traction exostosis, bizarre parosteal osteochondromatous
proliferation, and florid reactive periostitis. They are usually Figure 11.45 Osteochondroma showing outer perichondrium,
sporadic, but can be part of: middle cartilage layer and innermost bone layer
The criteria of osteochondroma includes the presence of Torus Palatinus

clusters of chondrocytes in the cartilaginous cap, arranged
It is also called as ‘palatine torus’. It is a slowly growing
in parallel, oblong, lacunar spaces, similar to those of
194 flat based bony protuberance or excrescence which occurs
normal epiphyseal cartilage.
in the midline of the hard palate.
The histological distinction between osteochondroma
It has been stated that functional stress and genetic
and chondrosarcoma can be difficult, however, lack of high
factors are important for its origin.
cellularity, pleomorphism, and plump cells with large or
double nuclei favor a benign lesion. Types
∙ Flat torus: It has broad base and extend symmetrically
Histopathology on both side of raphe
Outer layer: Fibrous perichondrium that is continuous with ∙ Spindle torus: It is midline ridge along the palatine
periosteum of underlying bone raphe
Middle layer: Cartilaginous cap < 2 cm thick. Superficial ∙ Nodular torus: Multiple protuberance with indi-
chondrocytes are clustered, deeper chondrocytes vidual base
resemble a growth plate ∙ Lobular torus: Lobulated mass from single base.
Deeper layer: Endochondral ossification and transition to
cancellous bone with inter trabecular red or Clinical Features
fatty marrow
Age and sex distribution: It occurs in about 20 percent of
the population. Females are affected twice more commonly
Hyperplasias Osteochondroma than males. Development is initiated in young adults,
• B
one shape preserved • E
xophytic bulbous or before 30 years.
•
Smooth, regular outline globular dome shaped Location: It occurs in the midline of the palate and may
•
Symmetrical sessile or pedunculated extend to involve the palatal process of the palatine bone.
proportionate out growth over the
enlargement cortical surface Appearance: The growth may be slow or there may be
•
Increased opacification •
May have irregular a period of rather rapid growth which ceases altogether
•
Normal marrow outline before the patient has advanced into adult life. It may be
trabeculation •
Cortex and marrow variable in size and shape and be described as flat, spindle
•
Enlargement of ipsilateral continue into lesion shaped, nodular or mushroom like (Fig. 11.46).
mandible •
Rest of jaw bone normal
Sign and symptoms: It is covered with normal mucosa,
which appears pale and occasionally ulcerated, when
Management traumatized. There is a nodular irregularity around some of
the masses of new bone, formed on one side of the midline.
It consists of surgical removal.
Points to Remember
It is usually composed of compact bone or cancellous bone
Choristoma, periosteal cuff, herniation of the epiphy-
surrounded by a capsule of compact bone. An inner core
seal plate cartilage, physeal theory, condylar osteochon-
of cancellous bone may be present in the larger specimens.
droma, pain due to mechanical irritation, peripheral os-
teochondroma, hereditary multiple exostoses (HME); Management
Familial osteochondromatosis diaphyseal aclasis, ir-
regular calcifications, outer perichondrium, cartilage As it is benign it is usually not treated, except in some
and innermost layer of bone layer, chondrocytes are cases where patient requires a complete denture and tori is
clustered. causing more undercuts and problems for fitting of complete
denture. In these cases surgical removal of tori can be done.
Benign Tumors
195
Figure 11.46 Torus palatinus presenting as a hard bony Figure 11.47 Mandibular tori present on lingual side in the
lobulated growth with a smooth overlying mucosal surface region of molar
Points to Remember Points to Remember

Palatine torus, flat, spindle shaped, nodular or mushroom Mandibular tori growth on the lingual surface of the
like, composed of compact bone or cancellous bone. mandible, above the mylohyoid line.
Torus Mandibularis Exostosis

It is also called as ‘mandibular tori’. It is an exostosis It is also called as ‘hyperostoses’. They are small regions
or outgrowth of bone found on the lingual surface of the of osseous hyperplasia of cortical bone and occasionally,
mandible. They consist primarily of compact bone. cancellous bone, on the surface of the alveolar process.
Causes
Clinical Features
Genetic and environmental factors are responsible for its
formation. Masticatory stress is reported as an essential Location: They are less common than mandibular and
factor underlying the formation. palatal tori. It may develop on the palatal surface of
maxillary alveolar process, at the border between the
Clinical Features attached gingiva and vestibular mucosa, in canine or molar
area.
Age: It occurs in 8 percent of the population. It is usually
They seldom attain large size and may be solitary or
discovered in middle aged adults.
multiple. Their shape may be nodular, pedunculated or flat
Location: It may occur singly, multiply; unilaterally, but is protuberance on the surface of bone. They are bony hard on
usually bilateral in premolar region. palpation.
Sign: There is growth on the lingual surface of the Buccal exostosis: It is bilateral row of bony hard nodule
mandible, above the mylohyoid line, usually opposite to along the facial aspect of maxillary or mandibular alveolar
the bicuspid teeth. Their sizes are variable ranging from an ridge.
outgrowth that is just palpable to one that contacts a torus
Palatal tubercle: It is bony protuberance seen at lingual
on the opposite side (Fig. 11.47).
aspect of maxillary tuberosity.
Management Reactive subpontic exostosis: It is also called as subpontic
It does not require any treatment. Removal may be osseous proliferation, subpontic osseous hyperplasia—
necessary, if a mandibular denture is planned. these develop beneath pontic of posterior bridge.
Management Location: Mandible to maxilla ratio is 2:1. Fifty percent

cases are found in mandible, mostly in the body and ramus
They do not required any treatment.
area.
196
Points to Remember Sign: There is nontender expansion of jaws and the
Hyperostosis, palatal surface of maxillary alveolar swelling is bony hard in consistency.
process, nodular, pedunculated or flat protuberance,
Symptoms: Pain is present in many cases and is probably
buccal exostosis, palatal tubercle, reactive subpontic
throbbing in nature.
exostosis.
Compressible swelling, which may pulsate and bruit
may be detected on auscultation.
VASCULAR TISSUE There is anesthesia of skin supplied by mental nerve.
Bleeding from gingiva around the neck of affected teeth
Hemangioma can occur.
It is also called as ‘vascular nevi. It is a benign tumor Pumping tooth syndrome is present, i.e. it demonstrates
which occurs most commonly in vertebrae and skull. It is pumping action, i.e. if the tooth is depressed into the
characterized by endothelial proliferation of blood vessels. socket, it will rebound into its original position within few
It is often congenital in origin. minutes. Teeth in affected area may be loosened and may
It is composed of seemingly disorganized vessels that migrate.
are filled with blood and is connected to the main vascular Aspiration of the lesion produces blood.
system.
Radiological features: Lesion shows honeycomb
Initially hemangioma has been thought to be
appearance (small loculations) or soap bubble appearance
developmental vascular malformation. Nowadays heman-
(large loculations). Large lesion can cause cortical
gioma is the term used for tumors with endothelial cell
expansion and in some cases ‘sunburst appearance’, can
proliferation. For structural anomalies of blood vessel
be seen.
without endothelial proliferation is term as vascular
malformation. Hemangioma of infancy (Figs 11.48 and 11.49)
Vascular malformations are described later in this Age and sex distribution: It is more commonly seen in
chapter. Under the heading of hemangioma, we will children in first decade of life. More seen in female than in
describe hemangioma of infancy and central hemangioma. males in the ratio of 3:1.
Types
∙ Superficial hemangioma: Usually consists of raised,
reddish to purple tumor with a distinct margin.
∙ Deep subcutaneous hemangioma: Often have a
deep bluish hue with normal overlying skin, making
diagnosis more difficult.
∙ Central: It occurs in the bone.

Central
The lesion originates either from the periosteum and
resorbs the underlying bone or it occurs within the bone as
an anomaly of blood vessels in the marrow spaces.
Age and sex distribution: It is rarely discovered in the

jaws. Most cases are found at birth or arise at early age. Figure 11.48 Vascular malformation seen along the
Female to male ratio is 2:1. distribution of trigeminal nerve
Benign Tumors
dome-shaped with normal or blue surface coloration, they

seldom blanch.
After 6 to 10 months tumors slows down and color
changes from red to dull-purple hue. Tumor resolves by 197
the age of 9 years.
In some individual permanent change like atrophy,
scarring, wrinkling or telangiectasia can occur.
Compressibility test is positive continued pressure and
squeezing will drive the blood out of the lesion and the
swelling crumbles. As soon as the pressure removed, the
swelling reappears with refilling. In cases of hemangioma
affecting the tongue, there may be loss of mobility of the
tongue. It may affect a part of tongue or the entire tongue.
PHACE(S) syndrome: Many cases of hemangioma
are associated with PHACE (S) syndrome which consist
Figure 11.49 Hemangioma of tongue
of posterior fossa brain anomalies, hemangioma, arterial
anomalies, cardiac defect and coarctation of aorta, eye
anomalies and sternal cleft or supraumbilical raphe.

In it, many small capillaries lined by a single layer of
endothelial cells supported by connective tissue stroma are
seen. It is comprised of numerous interwining capillary-
sized vessels, lined by endothelium with relatively flat or
plump nuclei; depending on the duration of the lesion.
Those with plump endothelial nuclei are younger and
often demonstrate mitotic activity. There is endothelial
proliferation occurs in this lesion.
Figure 11.50 Strawberry angioma of lip showing irregular

margin and ulceration
Location: It is most commonly seen in head and neck

region. The most common site of occurrence are the lips,
tongue, buccal mucosa and palate.
Strawberry hemangioma (Fig. 11.50): Superficial
tumors of the skin appear raised and bosselated with bright
red color lesion. They are firm on palpation. Subcutaneous
tissues as well as skin are often involved. Swelling is
compressible. It slightly protrudes from the skin surface as
a sessile hemisphere. It is irregular and there may be small
areas of ulceration with scabs. Figure 11.51 Blood vessels (BV) of capillary hemangioma tend
The more superficial ones are often lobulated and will to be small and of irregular shape. They are lined by plump
blanch under finger pressure. Deeper lesions tend to be endothelial cells (EC)
Clinical Features
Location: They are more common on face along the
198 distribution of trigeminal nerve (Figs 11.48 and 11.49).
Port-wine stain: It is capillary, rather than cavernous
variety which is usually more bluish. These reddish
macular vascular malformations are called as port- wine
stain. It generally starts at birth and darkens as the child
grows, but it does not really grow. It is common on the
face and at the shoulders, neck and buttock. The port wine
stain is generally smooth, but could be slightly raised. It
is seldom over 5 mm in diameter. It is deep purple-red in
color, which may become paler in later life. Color blanches
readily on pressure (Fig. 11.53).
Figure 11.52 Large dilated cavernous blood spaces Salmon patch: It is present since birth and usually
containing blood disappears before the first birthday. It is seen over the
forehead, occiput or anywhere in the midline of the body.
As the lesion resolves vascular spaces become less Venous malformation: They are blue and easily
prominent and replaced by fibrous connective tissue. compressible. They are presented as small isolated
ecstasies to complex growth which involve multiple
Management
tissues. Secondary thrombosis and phlebolith can occur.
As most of the lesion undergo spontaneous remission, they It appears as a flat or raised lesion of mucosa. Mass may
do not required any treatment for the disease. vary in size and may become larger on physical activity
Systemic corticosteroid: It will reduce the size of lesion. or standing, but may reduce in size once the patient is flat
Other drug which can be used are IV vincristine, on the examining table. Sometimes lesion may increase in
interferon. size, which can burry the teeth and cause serious deformity
and disfigurement. The texture of mucosa may be more
Points to Remember or less unchanged, showing an increased vascularity on
Central: Pain is present, compressible swelling,
anesthesia of skin supplied by mental nerve, pumping
tooth syndrome, aspiration of the lesion produces blood,
radiologically honeycomb and soap bubble appearance.
Hemangioma of infancy: Strawberry hemangioma,
atrophy, scarring, wrinkling or telangiectasia, compres-
sibility test is positive, PHACE(S) syndrome, bosselated
with bright red color lesion.
Histopathologically single layer of endothelial cells
flat or plump nuclei, numerous intertwining capillary-
sized vessels, plump endothelial nuclei, systemic
corticosteroid, vincristine, interferon.
Vascular Malformation
In contrast to hemangioma vascular malformation are
present at birth and persist throughout the life. Figure 11.53 Port-wine stain is deep red in color
Benign Tumors
the surface; but in some cases, appearance in pebbly. The When lesion vascular channels are considerably
mucosal hemangioma is typically a soft, moderately well enlarged, the term cavernous hemangioma has traditionally
circumscribed lesion. if associated with large vessels. been applied. This differs from capillary hemangioma in
that it is less well circumscribed, is larger and is usually 199
Arteriovenous malformation: It also called as
deeper in submucosal tissues.
‘arteriovenous shunt’ or ‘arteriovenous malformation’.
These are high flow lesion result from arterial and venous Sclerosing hemangiomas: Sluggish blood flow may result
communication. Palpable thrill or bruit is noticeable. in organized or dystrophically calcified thrombi within the
Overlying skin warm to touch. The tumor more often dilated vessels. Such hemangiomas are called as sclerosing
is traumatized and bleeds profusely. It also undergoes hemangiomas. The vessels may be arranged in a haphazard
ulceration with secondary infection. or a somewhat lobular pattern and there may be areas with
fibrosis of the background stroma.
Types of Arteriovenous Malformation Chronic inflammatory cells may be scattered in multiple
∙ Cirsoid aneurysm: It is a tortuous mass of small foci. Walls are occasionally thickened as a result of
arteries and veins linking a larger artery and vein. adventitial fibrosis and inflammatory cells may be scattered
∙ Varicose aneurysm: It consists of endothelium lined throughout the stroma. In long standing cases thrombus
sac connecting an artery and a vein. formation may take place, followed by calcification. Such
∙ Aneurysm varix: It is a direct connection between hemangiomas are called as phleboliths.
artery and vein.
Management
Histopathological Features It usually regresses by itself during adolescent period.
Vascular malformation does not show endothelial Laser surgery, cryosurgery by dry ice can also be effective.
proliferation. Sclerosing technique: Intralesional injections of sclerosing
In capillary malformation there is central feeder vessel chemicals, such as 3 percent sodium morrhuate are effective.
with radiating, lobular extensions. The lumina are typically Injection of boiling water or hypertonic saline may also be
small, perhaps to the point of masking the vascular nature given.
of the lesion (Fig. 11.54).
In cavernous type there are large dilated blood sinuses Flashlamp pulsed dye laser: It is effective in treatment of
which have thin walls each showing endothelial lining. The port-wine stain.
sinusoidal spaces are usually filled with blood.
Points to Remember
Port-wine stain, Salmon patch, arteriovenous malforma-
tion, arteriovenous shunt or arteriovenous malformation,
central feeder vessel with radiating, lobular extensions,
sclerosing hemangioma, dystrophically calcified throm-
bi, chronic inflammatory cells, phleboliths, adenventitial
fibrosis and inflammatory, sclerosing technique, flash-
lamp pulsed dye laser.
Struge-Weber Syndrome
It is also called as Struge-Weber angiomatosis or
encephalotrigeminal angiomatosis. It is characteristic by
hamartomatous vascular proliferation involving tissue and
brain. It is causes by persistence vascular plexus around the
cephalic portion of the neural tube.
It is postulated to be a developmental defect of certain
Figure 11.54 Capillary hemangioma ectodermal and mesodermal elements closely approximated
in the brain and meninges at 4 to 8 weeks of gestational Lymphangioma

age.
It is a benign hamartomatous proliferation of lymphatic
vascular tissue. It is a hamartoma, rather than a neoplasm.
200 Clinical and Radiological Features
In it abnormal vessels are filled with clear protein rich
Port-wine stain or nevus flammeus: A facial port-wine fluid containing lymph rather than blood.
stain in the distribution of the first branch of the trigeminal
nerve is present. The port-wine stain is present at birth and Types
is unilateral, but may cross the midline and also involve
mucosae. Some patients also have bilateral facial lesions, ∙ Superficial: It presents as a circumscribed lesion,
involvement of the trunk or extremities. which appears as small blisters and slightly elevated
skin patches.
Leptomeningeal angiomatosis: There is homolateral ∙ Lymphangioma simplex (capillary lymphangioma):
leptomeningeal angiomatosis, usually over the posterior It consists of small capillary sized vessels.
parietal and occipital lobes of the cerebral cortex. This ∙ Cavernous lymphangioma: It composed of larger
angiomatosis can cause convulsive disorder of grand mal dilated lymphatic vessels.
type. Characteristic S-shaped intracranial calcifications are ∙ Cystic lymphangioma: They are large, cystic, and
found in the leptomeninges within the first few months of translucent and may be seen in the neck, mediastinum
life, on CT scans and on X-rays after the age of 2 years. or axilla. These are called as cystic hygroma.
They increase in density until the end of second decade
when 50 to 60 percent of patients show this on radiography.
Clinical Features
Ocular manifestation: Patients with additional
Age: They may be present at birth with majority becoming
involvement of in the distribution of the second branch of the
clinically evident early in life, but with a small number not
trigeminal nerve have a high-risk of ocular complications
being manifested for a number of years.
like glaucoma, episclera, choroid and retina.
Location: It may occur alone or in association with
Oral features: The gingiva exhibits slight vascular
hemangioma or other anomalous blood vessels. It occurs
hyperplasia.
in dorsal and lateral borders of tongue, lips, gingiva and
Histopathological Features buccal mucosa.
There is excessive number of dilated blood vessels in Appearance: Usually the disfigurement is noticed by the
middle and deep dermis. There is vascular dilatation in child parents. Occasionally, the vesicles may be rubbed
gingival lesion. with clothes, get infected and become painful.
Management Signs: They are soft masses that dissect along the tissue
planes and turn out to be more extensive than anticipated.
Port-wine stain is managed by newer Flashlamp pulsed The surface of the lesion may be smooth or nodular. Color
dye lasers. Care should be taken while performing surgical ranging from normal mucosal pink to bluish and may be
procedure in affected area. quite translucent. They are liable to trauma. Due to this,
lesions are subjected to periodic attacks of inflammation
Points to Remember
which cause the swelling to become larger and tender for
Struge-Weber angiomatosis, port-wine stain or nevus the time being. Aspiration yields lymph that is high in lipid.
flammeus, leptomeningeal angiomatosis, gingiva exhibits If the tongue is affected, enlargement may occur and
slight vascular hyperplasia, high-risk of ocular complica- the term ‘macroglossia’ is applied. On the tongue, it is
tions like glaucoma, episclera, choroid and retina, exces- characterized by irregular nodularity of the surface of the
sive number of dilated blood vessels, newer flashlamp tongue with gray and pink, grapelike projection. They are
pulsed dye lasers. often elevated and nodular in appearance and may have the
Benign Tumors
same color as the surrounding mucosa. Lip involvement Cavernous type is characterized by presence of dilated
and its deformity is called as macrocheilia. sinusoidal endothelium lined vascular channels, devoid of
Cystic hygroma is the term used for large lymphangiomas erythrocytes. Occasional channels may be filled with blood
spreading into and distending the neck, are called as cystic and it is called as hemangio-lymphangioma. 201
hygroma.
Management
Histopathological Features (Figs 11.55 and 11.56) Treatment is generally not indicated for small lesions.
Capillary types are composed of proliferation of thin Surgical removal of the bulk of the lesion can be done.
walled endothelium-lined channels, primarily devoid of Partial or complete spontaneous involution is occasionally
erythrocytes. noted.
Points to Remember
Benign hamartomatous proliferation of lymphatic vascu-
lar tissue, disfigurement, surface smooth or nodular, mac-
roglossia, irregular nodularity, cystic hygroma, macrocheil-
ia, proliferation of thin walled endothelium-lined channels,
dilated sinusoidal endothelium, hemangio-lymphangioma.
Glomus Tumor
It is also called as ‘glomangioma’. It is a rare neoplasm
derived from glomus cells. They are thought to be closely
related to hemangiopericytoma.
The glomus is arteriovenous anastomosis that controls
the blood supply and temperature of the skin and certain
deeper tissues. These functions appear to be mediated in
some way by the rich nerve supply and by certain epithelioid
cells that ensheath the arteriole of the glomus. The epithelial
cells are thought to be comparable to pericytes.
Figure 11.55 Lymphangioma showing lymph channels with
this wall consisting of thin endothelial lining. Channels contain Clinical Features
lymph
Location: The tumor probably arises from these specialized
glomus cells and occurs most frequently under the nails and
on the body surface especially in head and neck area. In the
oral cavity the lesion is usually located on the dorsum of
the tongue, lip, palate, buccal mucosa and tongue.
Age: The tumor usually occurs in the 5th decade.
Size: They are small lesions, rarely exceeding a cm in
diameter.
Symptoms: They often give rise to attacks of very severe
pain and are exquisitely tender. Pain is stabbing in nature.
Signs: The color varies from deep red to purple or blue.
It consists of glomus cells and these may reproduce to some
Figure 11.56 Lymphangiomas high power extent, the structure of the normal glomus (Fig. 11.57).
It shows tightly packed cells which surrounds endothelial
202 line vascular channels. Arrangement of cells is haphazard
with round to ovoid nuclei.
The blood vessels show irregular branching which
results in ‘staghorn’ and ‘anterlike’ appearance. Dense
reticulin network also surrounds the vessels (Fig. 11.58).
Points to Remember
Derived from pericytes cells, slow growing, painless
mass, nasal obstruction and epistaxis, tightly packed
cells, round to ovoid nuclei, staghorn’ and ‘anterlike’
appearance.
Figure 11.57 Glomus cells (G) of glomus tumor
In some cases, glomus cell may be arranged around the

blood vessels in a manner suggesting hemangiopericytoma
or the blood vessels may be so prominent as to resemble
cavernous hemangioma.
Management
It is a benign tumor and removal effects cure.
Points to Remember
Glomangioma, arteriovenous anastomosis, pericytes,
attacks of very severe pain, color varies from deep red to
purple or blue, glomus cells arranged around the blood
vessels.
Hemangiopericytoma
It is rare tumor derived from pericytes cells with processes
which encircles endothelial cells of capillaries.
Clinical Features
Age: Adults are more commonly affected.
Location: It is most common seen in lower extremities
with some cases also occurs in head and neck region.
Intraorally it is seen in buccal mucosa.
Appearance: It is slow growing, painless mass. In
some cases of superficial region there may be vascular
pigmentation.
Symptoms: In nasal cavity it may occur result in symptoms
of nasal obstruction and epistaxis. Figure 11.58 Hemangiopericytoma showing staghorn pattern
Benign Tumors
Nasopharyngeal Angiofibroma NEURAL TISSUE

It is rare vascular and fibrous tumor like lesion that occurs
Neuroma
only in nasopharynx. It is locally destructive behavior. This 203
tumor expands medially via sphenopalatine foramen. It can It is also called as amputation neuroma or traumatic
also extent to maxillary sinus, middle cranial fossa and oral neuroma. It is not a true neoplasm, but an exuberant attempt
cavity. at repair of a damaged nerve trunk.
Clinical Features Pathogenesis

Age and sex distribution: It occurs exclusively in males Nerve damage may result from fracture, dissection, removal
in age group of 2nd decade. of cyst, nerve avulsion for neuralgia or even extraction of
teeth.
Symptoms: Nasal obstruction and epistaxis are common It is an overgrowth of severed nerve, attempting to
symptoms. regenerate when the scar tissue or misalignment of a
Radiological features: Anterior bowing of posterior wall fractured nutrient canal blocks the distal end.
of maxillary sinus is characteristic features of this disease. Proliferating nerve forms unorganized collection of
nerve fibers, composed of varying proportion of axons,
Histopathological Features perineural connective tissue and Schwann cells.
It consist of dense fibrous connective tissue that contain
numerous dilates, thin walled blood vessels (Fig. 11.59). Clinical Features
Vascular components are more prominence at periphery Location: It is typically occurs near the mental foramen,
than at the center. on the alveolar ridge in edentulous areas or on the lips and
tongue.
Management
Age and sex distribution: It is seen middle age adults and
Surgical excision should be done. more commonly seen in the women.
Points to Remember Appearance: It appears as a small nodule or swelling of
Nasal obstruction, epistaxis anterior bowing of posterior the mucosa.
wall of maxillary sinus, numerous dilates, thin walled Symptoms: Due to the pressure applied by enlargement
blood vessels. of the tangled mass in its bony cavity, severe pain may be
experience.
It may have reflex neuralgia with pain referred to the eye,
face and head. It is a slow growing reactive hyperplasia that
seldom becomes large, rarely in excess of 1 cm in diameter.
It shows mass of irregular and interlacing neurofibrils
and Schwann cells, situated in connective tissue stroma of
either scanty or plentiful proportion (Fig. 11.60).
The proliferating nerve fibers themselves may occur
either in small discrete bundles or spread diffusely
throughout the tissue.
Mild chronic inflammatory cell infiltrate can also be
present.
Management
Figure 11.59 Dilated blood vessels present in fibrous stroma Simple excision of nodule along with proximal portion of
in case of nasopharyngeal angiofibroma the involved nerve.
Signs: It usually occurs singly and jaw expansion (Fig.

11.61) may lead to perforation. The mass is firm on
palpation. It is nonproductive to aspiration.
204
Schwannoma is proliferation of Schwann cells. The tumor
is well encapsulated with epineurium making the capsule.
It is composed of two types of tissues: Antoni type A and
Antoni type B.
Antoni type A: This pattern is distinctive and well

organized. It is made up of cells with elongated or spindle
Figure 11.60 Traumatic neuroma showing neurofibrils and

Schwann cells
Points to Remember
Amputation neuroma, unorganized collection of nerve
fibers, small nodule or swelling, severe pain, reflex
neuralgia, irregular and interlacing neurofibrils and
Schwann cells, mild chronic inflammatory cell infiltrate.
Neurilemmoma
It is also called as Schwannoma, perineural fibroblastoma,
neurinoma and lemmoma. It is of neuroectodermal origin,
arising from Schwann cells that make the inner layer cov- Figure 11.61 Jaw expansion seen in neurilemmoma
ering the peripheral nerves.
Clinical Features
Age and sex distribution: It occurs at any age, from very
young to very old, with equal frequency in both the sexes.
Location: The tumor usually occurs in the subcutaneous
tissue, but internal organs such as stomach may be affected.
Intraorally, mandible is the most commonly affected site
for central lesion. Other sites which can be involved in
these tumors are palate, floor of mouth and buccal mucosa.
It is a slowly growing lesion and is usually of long
duration at the time of presentation.
Symptoms: Usual complain is lump in jaw, in case of
central tumor and single circumscribed nodule, in case of
soft tissue lesions. Paresthesia may be associated, which
occurs anterior to the tumor. Pain is localized to the tumor Figure 11.62 Neurilemmoma showing Antoni type A cells
site. surrounding verocay body
Benign Tumors
of view multiple endocrine neoplasia syndromes (MEN)

2b type is important which is discussed below.
Types 205
∙ MEN 1: Benign tumor of pancreatic islet, adrenal
cortex, parathyroid gland and pituitary gland
∙ MEN 2A (Sipple syndrome): There is development
of adrenal pheochromocytomas and medullary thyroid
carcinoma
∙ MEN 2B: It have mucosal neuroma which involve oral
mucous membrane.
Clinical Features
Oral neuroma: The neuroma is most common on the lips,
Figure 11.63 Antoni A area is composed of spindle shaped tongue and buccal mucosa. They produce Bumpy Lips since
Schwann cells arranged in interlacing fascicles. There is the neuroma present at birth or may develop later appear as
characteristic nuclear palisading in between two compact rows small elevated sessile nodules on the vermilion producing
of well aligned nuclei; the cell processes form eosinophilic puffy lips. On the tongue they are commonly present on the
acellular areas verocay bodies. Mitotic figures also may be anterior third. Bilateral neuroma can occur commissural
present mucosa.
Pheochromocytomas of adrenal gland: These are bilateral
shaped nuclei, which are aligned to form a characteristic and multifocal. The tumor cells secrete catecholamine which
palisading pattern, while intercellular fibers are arranged results in profuse sweating, intractable diarrhea, headache,
in parallel fashion between rows of nuclei. When such flushing, heart palpitation and severe hypertension.
clusters of palisaded cells occur around eosinophilic Medullary carcinoma of thyroid gland also occur which
substance they create verocay body. The eosinophilic arise from the parafollicular cells.
material substance is made up of cytoplasmic processes of
Schwann cells and duplicated basement membrane. Histopathological and Laboratory Features
Antoni type B: It does not exhibit this characteristic Neuroma is characterized by marked hyperplasia of nerve
palisading, but rather a disorderedly arrangement of cells bundles with prominent thickening of perineurium is also
and fibers, with vacuolated areas. seen.
Laboratory finding: Serum or urinary level of calcitonin
Management
are elevated in patient who is having thyroid gland
Surgical excision is the treatment of choice. medullary carcinoma.
Points to Remember Management

Schwannoma, slowly growing lesion, lump in jaw, Prophylactic removal of thyroid gland should be done as
paresthesia, jaw expansion, perforation, Antoni type there are chances of medullary carcinoma.
A, Antoni type B, intercellular fibers are arranged in
parallel, verocay body. Points to Remember
Oral neuroma, bumpy lips, puffy lips, pheochr-
Multiple Endocrine Neoplasia Syndromes omocytomas of adrenal gland, medullary carcinoma
(MEN Syndrome) of thyroid gland, marked hyperplasia of nerve bundles
This is a group of syndromes characterized by tumors of with prominent thickening of perineurium, level of
various endocrine organs occurring in association with a calcitonin are elevated, prophylactic removal of thyroid
variety of other pathologic features. In oral physician point glands.
Paraganglioma It is inherited as a simple autosomal dominant trait with

variable penetrance. It arises from the connective tissue
It is also called as carotid body tumor, chemodectoma,
sheath of Schwann cells and axons. There are three sites of
206 glomus jugulare tumor, glomus tympanicum tumor.
origin: within the inferior dental canal, in the substance of
Paraganglia are specialized tissue of neural crest origin
the bone and beneath the periosteum.
which are associated with autonomic nerves and ganglia
Solitary lesions are termed as neurofibroma. Neuro-
throughout the body.
fibroma can be part of neurofibromatosis which is having
Types of Paraganglioma multiple lesions.
Carotid body tumor: These are located in carotid body. Clinical Features
Jugulotympanic paraganglioma: These are present in Age and sex distribution: It occurs at any age but is found
temporal bone and middle ear. more in younger group with no sex predilection.
Clinical and Radiographic Features Location: Most commonly affected sites are trunk, face
and extremities.
Age and sex distribution: It is seen in middle age adults
with no sex predilection. Appearance: It may appear as numerous sessile or
pedunculated, elevated smooth surfaced nodules of variable
Location: The head and neck area is common site for this
size, which are scattered over the skin surface (Fig. 11.64).
tumor.
Solitary lesion: They are soft, painless lesion varying in
Carotid body tumor: It is seen at bifurcation of internal
size from small nodules to larger masses.
and external carotid artery. It is slow growing painless
mass below the angle of jaw in neck region. Angiography Elephantiasis neuromatosa: In other forms, there are
will demonstrate characteristic vascular lesion. deeper, more diffuse lesions which are often of greater
proportion than superficial nodules and are sometimes
Jugulotympanic paraganglioma: In these symptoms
referred to as elephantiasis neuromatosa.
includes dizziness, tinnitus, hearing loss and cranial nerve
In some cases, loose overgrowth of thickened,
palsies.
pigmented skin may hang in folds.
It is characterized by round or polygonal epitheloid cells
that are organized in nest or Zellballen. The Zellballen are
large and irregular in shaped. The tumor is vascular and
surrounded by thin fibrous capsule.
Management
It includes surgery, radiation therapy depending on extent
of tumor.
Points to Remember
Carotid body tumor, seen at bifurcation of internal and
external carotid artery, Jugulotympanic paraganglioma,
dizziness, tinnitus, polygonal epitheloid, nest or
Zellballen.
Neurofibroma or Neurofibromatosis
It is also called, von Recklinghausen’s disease of skin or Figure 11.64 Neurofibromatosis of left side of face involving
fibroma Molluscum. orbital region
Benign Tumors
The plexiform variant of neurofibromatosis feels like a

bag of worms.
Café au lait spots: In addition majority of the patients 207

exhibit asymmetric areas of cutaneous pigmentation, often
described as cafe au lait spots.
Crowe’s sign: Axillary freckling present in neuro-
fibromatosis is called Crowe’s sign.
Lisch bodies: These are translucent brown pigmented
spots on the iris.
Oral Manifestations
Location: It may occur in mandibular canal, buccal mucosa
and alveolar ridge, and below the periosteum.
The central lesion may have multiple lesions, occurring Figure 11.65 Herringbone (H) pattern seen in neurofibromas
in both jaws simultaneously, expanding and filling the
maxillary sinus. Solitary central lesion may infrequently
be associated with brown spots on skin.
Appearance: There are discrete, nonulcerated nodules,
which tend to be of the same color as the normal mucosa.
Symptoms: It may produce pain or paresthesia, if
associated with mandibular nerve.
Sign: It may expand and perforate the cortex, producing
a swelling that is either hard or firm on palpation.
Macroglossia may be there due to diffuse involvement of
the tongue.
In some cases it is well demarcated or poorly demarcated
unilocular or multilocular radiolucency.
There is also enlargement of mandibular foramina, Figure 11.66 Neurofibroma (high power)
mandibular canal and increase in dimension of coronoid
notch.
Cellular and myxoid patterns predominate. Melano-
Histopathological Features (Figs 11.65 and 11.66) cytes are sometimes found in addition to mast cells.
Neurofibroma is proliferation of fibroblasts surrounding Plexiform neuromas: Some of the lesions may consist of
the neurites as well as of neurites themselves. masses of convoluted nerves the individual axons of which
It is composed of proliferation of delicate spindle are surrounded by thickened perineurium. Lesion of this
cells with thin wavy or serpentine nuclei intermingled type is called as plexiform neuromas.
with neurites in an irregular pattern as well as delicate
interwining connective tissue fibrils. Management
Fibers give rise to herringbone or storiform pattern. It Solitary lesion may be surgically excised.
consists of collagenous tissue and nerve fibers, the later Facial lesion of neurofibromatosis should be removed
running throughout the lesion. Mast cells are typically with the help of carbon dioxide laser and dermabrasion.
found in this lesion. It has got high potential for malignant change.
Location: The CGCT shows very peculiar site

Points to Remember
predilection for the maxillary anterior alveolar ridge
von Recklinghausen’s disease of skin, numerous sessile though cases have been reported in mandibular anterior
208 or pedunculated, elevated smooth surfaced nodules, region. The ratio of occurrence of CGCT in maxillary/
elephantiasis neuromatosa, bag of worms, Café au mandibular jaw is 3:1. Canine-incisor region is most
lait spots, Crowe’s sign, Lisch bodies, unilocular or frequently affected.
multilocular radiolucency, enlargement of mandibular
foramina, mandibular canal, proliferation of fibroblasts, Size: The size of the mass varies from a few millimeters to
thin wavy or serpentine nuclei intermingled with nine centimeter in diameter.
neuritis, herring bone or storiform pattern, mast cells, Appearance: Clinically congenital granular cell tumor
melanocyte, plexiform neuromas, carbon dioxide laser may mimic oral teratoma-epignathus and melanotic
and dermabrasion. neuroectodermal tumor of infancy, as well as other
possible diagnoses, such as fibroma, lipoma, leiomyoma,
Ganglioneuroma rhabdomyoma, rhabdomyosarcoma, peripheral giant cell
It is same like neuroblastoma, but in it, differentiated cells granuloma, pyogenic granuloma, neurofibroma, myxoma,
are numerous. It grows less rapidly than the neuroblastoma hemangioma, lymphangioma, and alveolar lymphangioma.
and when fully differentiated, it is depicts benign The large lesions are of therapeutic concern as they
characteristic. interfere with feeding and respiration of the infant.
The fully differentiated ganglioneuroma is composed Signs and symptoms: Patient complains of smooth
of cells that are very much like the normal cells. Numerous swelling. The swelling appears as a pink-to-red polypoid
nerve fibers and well differentiated nerve bundles are mass on the alveolar ridge which is usually round or oval,
present. showing irregular lobulation. Base is usually pedunculated,
but it may be sessile. Usually size is 2 cm or less, although
Congenital Granular Cell Tumor
lesions as large as 7.5 cm have been reported. Larger lesion
It is also celled as congenital epulis’, congenital epulis of may be large enough to protrude from the mouth (Figs
newborn’. 11.67 and 11.68).
Congenital granular cell tumor (CGCT) is an uncommon
congenital tumor. It is a benign gingival soft tissue lesion
of unknown etiology. The original first description of the
lesion was dated in 1871 by Neumann. It is also termed as
congenital granular cell epulis of newborn.
Histogenesis
The histogenesis of the Neumann’s tumor/congenital
granular cell tumor has long been debatable as various
authors suggested different source of origin of the tumor.
The proposed source of origin includes undifferenti-
ated mesenchymal cell, odontogenic epithelial, pericytic,
and fibroblastic, histiocytes, nerve-related, smooth muscle,
and primitive mesenchymal cells.
Clinical Features
Age and sex distribution: It presents at birth as a congenital
epulis, i.e. fibrous mass arising from the gingival mucosa of
the maxilla or mandible. There is a female preponderance
of 8:1 perhaps indicating a hormonal component in its Figure 11.67 A fibrous mass arising from the anterior
development. alveolar/gingival mucosa of the maxilla
Benign Tumors
209
Figure 11.68 Specimen of congenital granular cell tumor Figure 11.69 Congenital granular cell tumor showing uniform
distribution of polygonal granular cells arranged in sheets

Histologically, CGCT shows uniform distribution of large
ovoid cells with fine granular eosinophilic cytoplasm
and this cell may show arrangement as ribbons nest or as
pattern less diffuse sheets as seen in our case.
The cells have ill defined cell outlines and intercellular
bridges. Electron microscopic examination of the granular
cells of CGCT shows heterogeneous electron-dense
granules, lysosomes, and cytoplasmic lipid droplets.
The CGCT and granular cell/myoblastoma tumor have
similar microscopic features. However, the latter is less
vascular and is typically covered by hyperplastic squamous
cell epithelium.
In older tumors, these cells may become elongated
and separated by fibrous connective tissue. Immuno-
Figure 11.70 Congenital granular cell tumor showing granular
histochemical analysis shows the tumor cells to be negative cells with indistinct cell outlines and eosinophilic granular
for S–100 proteins. cytoplasm
Management
Melanotic Neuroectodermal Tumor of Infancy
The large lesions are of therapeutic concern as they
interfere with feeding and respiration of the infant. The The tumor is of neural crest origin. In the past, it is been
recommended treatment is surgical excision under local thought to be arise from odontogenic epithelium and retina.
or general anesthesia, although spontaneous regression. Due to this fact these tumors are called as pigmented
Recurrence or malignant change is rare. ameloblastoma, melanoameloblastoma, melanotic amelo-
blastoma, retinal anlage tumor, and melanotic progonoma.
Points to Remember But this origin is now proven that is not present so all
Congenital epulis, mimic oral teratoma-epignathus, interfere above term should be discarded and it should be called as
with feeding, pink-to-red polypoid mass, pedunculated, melanotic neuroectodermal tumor of infancy.
sessile, uniform distribution of large ovoid cells, fine Clinical and Radiological Features
granular eosinophilic cytoplasm, heterogeneous electron-
dense granules, lysosomes, cytoplasmic lipid droplets. Age and sex distribution: It occurs in infants under the
age of six months, with equal sex distribution.
210
A
NSE - Positive Figure 11.72 Congenital epulis of newborn showing
fibroblasts
B
CD 68 -Negative
Figures 11.71A and B Immunohistochemical study of CGCT
shows positivity for neuron specific enolase (NSE) and negative Figure 11.73 Melanotic tumor of infancy
for CD 68 marker
Location: Maxilla is more commonly affected than Pigmented cells are cubical or rather flattened and have
mandible. It is more common in anterior region. large, pale nuclei. The cytoplasm contains melanin in the
form of minute rod shaped particles, often aggregated into
Sign: The tumor forms a mass that expands the bone without
large masses that obscure all the internal cellular details.
pain and tenderness. It has rapidly growing, nonulcerated,
These pigmented cells are arranged in solid groups or form
darkly pigmented lesions. The color of lesion can be blue
a lining of the small cleft like spaces.
or black.
Unpigmented cells are small, round well stained nucleus
Radiological features: The tumor destroyed underlying that nearly fills the cell body. They occur in groups, often
bone causing displacement of developing teeth. within the spaces lined by the pigmented cells. The central
portion of the alveolar spaces contain many neuroblast
Histopathological Features (Fig. 11.73) like cells which show little cytoplasm and exhibit a round
It consists of both pigmented and nonpigmented cells. deeply staining nucleus.
Benign Tumors
Laboratory finding: There is high urinary level of

vanillylmandelic acid (VMA).
Management 211
Conservative surgical excision or simple curettage can be
done.
Points to Remember
Pigmented ameloblastoma, expands the bone without
pain, destroyed underlying bone, pigmented cells are
cubical, unpigmented cells are small, round, high urinary
level of vanillylmandelic acid.
MUSCLE
Figure 11.74 Leiomyoma
Leiomyoma
It is a benign tumor derived from smooth muscle and
is found in a variety of anatomic sites like skin and
subcutaneous tissues.
Types
∙ Solid leiomyomas
∙ Vascular leiomyomas (angiomyomas)
∙ Epitheloid leiomyomas.
Clinical Features
Age and sex distribution: It occurs in middle decades of
life. Males are affected more commonly than females.
Location: It usually occurs in uterus. It is uncommon
in oral cavity due to general absence of smooth muscles
except in blood vessel walls and circumvallate papillae of
Figure 11.75 Leiomyoma showing fiber bundles with blunt
tongue. The smooth muscle of the arrectores pilorum may
ended nucleus (cigar-shaped nucleus)
be a source of cheek tumor. In oral cavity, it can occur in
lip, tongue, palate and cheek.
Appearance: It is a slow growing, painless lesion, which The muscle nuclei are typically spindle shaped with
is superficial and often pedunculated. blunt ends.
The bundles of fibers appear to form whorls because of
Symptoms: The patient may complain of sore throat or
their fascicular arrangement in varying planes. There may
tumor in the throat. In some cases, there may be pain.
be palisading arrangement of the nuclei.
Signs: In most of the cases, the lesion is small. It does not Angioleiomyoma: In some cases, origin from the
ulcerate and resembles the normal mucosa in color and blood vessels is obvious since the vessels are enlarged
texture. with thick muscular walls and around the tumor muscle
fibers are dispersed in a circular manner. It is called as
Histopathological Features (Figs 11.74 and 11.75) angioleiomyoma or angiomyoma. Rarely, angiomyoma may
It is composed of interlacing bundles of smooth muscle contain adipose tissue, then it is called as angiomyolipoma.
fibers, interspersed by varying amounts of fibrous Epitheloid leiomyoma composed of epitheloid cells
connective tissue. rather than spindle cells.
Management
It is treated by conservative surgical excision of the tumor.
212
Points to Remember
Slow growing, painless lesion, sore throat, interlacing
bundles of smooth muscle fibers, muscle nuclei, spindle
shaped, fascicular arrangement in varying planes,
angioleiomyoma, epitheloid leiomyoma.
Rhabdomyoma
It is a benign tumor of striated muscle origin. Term was
introduced by Zenker. Rare but have predilection for head
and neck region.
Types
∙ Adults rhabdomyomas
∙ Fetal rhabdomyomas. Figure 11.76 Rhabdomyoma showing deeply eosinophilic
polygonal cells with small peripherally placed nuclei and few
Clinical Features intracellular vacuoles
Age and sex distribution: Adult type most commonly
occurs in 5th decade of life with male to female ratio of Management
2:1. Fetal types usually occur in young children.
It is excised conservatively usually enucleating with ease.
Location: It mostly occurs in the head and neck regions.
The most common sites of occurrence, intraorally, are the Points to Remember
floor of the mouth, tongue, soft palate, buccal mucosa and Striated muscle origin, painless, well circumscribed
lower lip. tumor mass nucleus is vesicular, spider web appearance.
Symptoms: It is painless and slowly growing. Laryngeal
and pharyngeal lesion may lead to airway obstruction. Granular Cell Tumor
Appearance: It presents as a well circumscribed tumor It is also called myoblastic myoma, granular cell
mass which may have a known duration of months or even myoblastoma and granular cell schwannoma.
several years. Initially it is thought to be derived from striated muscles.
So this is called as granular cell myoblastoma. But, these
Histopathological Features (Fig. 11.76) tumors may be found in areas like breast and skin, where
The nucleus is vesicular and cells with several nuclei are the striated muscles are absent.
sometimes seen. Based on histological features, there are In neural theory it is proposed that these tumors are
two types seen, i.e. adults and fetal. derived from the connective tissue of nerves and hence was
In adult type, the tumor is composed of large, round called as granular cell neural fibroma.
cells that have granular, eosinophilic cytoplasm and show It is also believed to be originated from Schwann cells
irregular cross striations. The cytoplasm is rich in glycogen so it is called as granular cell schwannoma.
and glycoprotein. This type also demonstrates peripheral Some authors state that it is derived from stem cells
vacuolization which results in spider web appearance. with a leiomyofibrillogenic capacity, which may be
In fetal type is characterized by immature skeletal some type of specialized smooth muscle cells peculiar to
muscles in varying stages of development and undifferen- certain tissue, that are found in characteristic sites of the
tiated mesenchymal cells. tumor.
Benign Tumors

Age and sex distribution: It can occur at any age and Conservative surgical excision is recommended.
females are affected more commonly than males. 213
Points to Remember
Location: Most common site of occurrence is dorsum of
tongue followed by skin, lips, breast, subcutaneous tissue, Myoblastic myoma, firm, submucosal nodule, granular
vocal cord and floor of mouth. cells, satellite cells, granular eosinophilic cytoplasm,
Abrikossoff myocytes, consesvative surgical excision.
Appearance: Lesion which is found on tongue is usually
single firm, submucosal nodule within the substance of the
tongue itself. GIANT CELL LESION
Signs: The size of the tumor varies from a few millimeters It is applied for the lesions, containing giant cells. Giant
to centimeters. Lesion is not ulcerated and may have cells are large multinucleated cells of different origins. It is
normal covering or may exhibit some clinical leukoplakia. chiefly a tumor of long bones, occurring at the epiphyseal
end involving the adjacent metaphysis. It is an extremely
Histopathological Features (Fig. 11.77) uncommon tumor of head and neck and creates a lot of
confusion with respect to diagnosis. It exhibits variable
The lesion is composed of two types of cells granular cells
clinical and histopathological features ranging from benign
and satellite cells.
to malignant. Foreign body giant cells, Langhan’s giant
It is made up of strands and fascicles of cells which are
cells, Touton giant cells, tumor giant cells, are various types
large, 20 to 40 microns in diameter and show an extremely
of giant cells apart from the miscellaneous types of giant
granular eosinophilic cytoplasm, interspersed with a
cells such as Reed Sternberg cells of Hodgkin’s lymphoma.
collagenous stroma and covered with hyperplastic lingual
mucosa. Origin of Giant Cells
The large granular cells are called as Abrikossoff
myocytes. These granules may be fine or in some instance Different theories are given to describe the origin of giant
may be very coarse. cells in these lesions:
In some cases, the tumor cells have been found to be Giant cells might be derived from the proliferating giant
arranged in concentric whorls around myelinated nerve cells associated with the resorption of deciduous tooth
fibers. roots. But for this association of the lesion in transition
period, i.e. from of deciduous to permanent tooth should
be there: but such association is found in only few cases.
Another theory states that it originated from the
endothelial cells of capillaries. To support this theory:
there is a common occurrence of giant cells in vascular
channels, suggesting that they arise from endothelial cells.
Sapp found that giant cells, ultrastructurally, contain a
sufficient number of features in common with osteoclasts.
This concludes that they represent a slightly modified form
of the cells.
Giant Cell Tumors

It is debatable that true giant cell tumor occur in the jaw.
Giant cell tumor usually found in long tubular bone.
Clinical Features
Figure 11.77 Granular cell myoblastoma showing abundant Age: It is most frequently seen in 3rd and 4th decades, it is
cytoplasm unusual in patients less than 20 years.
Location: The commonest sites for giant cell tumor are the Common Giant Cell Lesions of the Oral Cavity
lower end of the femur, upper end of the tibia and lower (Ipe Varghese et al)
end of the radius. In the oral cavity, it is rare and if found
214 Microbial lesions: Tuberculosis, leprosy, Actinomycosis,
it is in jaw.
sarcoidosis.
Signs and symptoms: The principle symptoms are
Tumor and tumor-like lesions: Central giant cell granuloma,
swelling of the bone accompanied in some cases by pain.
peripheral giant cell granuloma, giant cell fibroma, giant
The swelling may be tender and egg shell crackling can be
cell tumor, osteosarcoma, rhabdomyosarcoma, Hodgkin’s
elicited in large tumors.
lymphoma.
Histopathological Features Cystic lesions: Traumatic bone cyst, aneurysmal bone
The tumor forms a maroon or reddish brown fleshy mass cyst.
that replaces the spongiosa of the bone. Metabolic lesions: Hyperparathyroidism.
It consists of numerous giant cells lying in cellular
matrix composed of spindle-shaped cells and scanty Osteodystrophic lesions: Noonan-like multiple giant cell
collagen. The giant cells are large with numerous vesicular lesion syndrome.
nuclei situated towards the center of the cells leaving a Miscellaneous lesions: Cherubism, Paget’s disease,
clear area of cytoplasm around the periphery (Fig. 11.78). fibrous dysplasia.
The cytoplasm is granular and vacuoles are often present.
There is even distribution of giant cells throughout the Peripheral Giant Cell Granuloma
lesion. It is also called as peripheral giant cell reparative
granuloma, giant cell epulis, osteoclastoma and peripheral
Management
giant cell tumor. It is five times more common as compared
The usual method of treatment is curettage, but this is to central giant cell granuloma. It seems to originate from
followed by a high recurrence rate. either periodontal ligament or mucoperiosteum.
Points to Remember Etiology
Egg shell crackling, a maroon or reddish brown fleshy It is an unusual response of tissue to injury. The trauma
mass, numerous giant cells. may be caused by tooth extraction and dental irritation. It
can occur in chronic infections.
It may appear under the stimulus of increased
circulating parathormone, i.e. primary and secondary
hyperparathyroidism.

Age and sex distribution: It is most often seen over 20
years of age, with an average of 45 years. Females are
affected twice as common as males. It is predominant in
white persons.
Location: It occurs on gingiva and alveolar mucosa, most
frequently anterior to molars. It is common in mandible
than maxilla.
Appearance: In early stage, it appears as discoloration and
slight swelling of the buccal aspect of the gingiva. Later the
Figure 11.78 Giant cell tumor showing many giant cell lesion increase in size and becomes rounded and very often
dispersed in cellular background pedunculated.
Benign Tumors
215
Figure 11.79 Peripheral giant cell granuloma showing growth Figure 11.81 Peripheral giant cell (Courtesy: Dr Aparna
in hour-glass manner Thombre, Reader, Department of Oral Pathology, VSPM Dental
College and Hospital, Nagpur, Maharashtra, India)
Lesions with much fibrous tissue are paler. It may feel

from soft to hard. The lesions vary in size from 0.5 cm to
1.5 cm in diameter.
It is vascular or hemorrhagic and sometimes ulceration
is also present. There may be tenderness on palpation. In
edentulous patients, it may present as a vascular, ovoid or
fusiform swelling of the crest of the ridge, seldom over 1 to
2 cm in diameter or there may be granular mass of tissue
which seems to be growing from the tissue covering the
slope of the ridge.
Radiographic features: There is cupping resorption of
bone seen on the radiograph.

Figure 11.80 Peripheral giant cell granuloma showing a Multinucleated giant cells and young connective tissue
dumb-bell/hour-glass shaped growth joining buccal and spindle shaped cells are scattered throughout the granulation
lingual interdental papillae of maxillary molars
tissue in delicate reticular and fibrillar connective tissue
stroma. Giant cells are usually more numerous and may
be large.
Sometimes, it grows in an hour-glass manner, with
The matrix consists of spindle shaped cells with oval
the waist of the lesion between two teeth and the globular
nuclei, similar to those if the giant cells.
extremities presenting buccally and lingually.
Capillaries are numerous around the periphery of the
Signs: The color of the lesion is usually dark red or maroon lesions and giant cells, sometimes, may be found in it.
color. If sufficient amount of hemosiderin exists near the Extravagated erythrocytes and varying amounts of
periphery, the lesion is bluish; otherwise it is red and or pink. hemosiderin are also present. The overlying epithelium
is normal, but sometimes it may shows acanthosis and

ulceration.
216 Management
Excision with borders of normal tissue with entire base
of the lesion and elimination of chronic irritant should be
done to avoid recurrence.
Points to Remember
Hour-glass manner, dark red or maroon color, vascular,
ovoid or fusiform swelling, cupping resorption of bone,
multinucleated giant cells and young connective tissue
spindle shaped cells, capillaries are numerous, extrava-
gated erythrocytes, varying amounts of hemosiderin, ex-
Figure 11.82 Peripheral giant cell granuloma showing
covering epithelium, a subepithelial normal connective tissue cision with borders of normal tissue.
zone and lesional tissue showing giant cells
BIBLIOGRAPHY
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3. Ethunandan M, Mellor TK. Hemangiomas and vascular
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A osteoblastoma in children and adolescents. Orthop Clin
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8. Golding JSR. The natural history of osteoid osteoma: A
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10. Makley JT, Dunn MJ. Prostaglandin synthesis by osteoid
osteoma. Lancet. 1982;2:42, Letter.
B 11. Mayur Chaudhary, Meena Kulkarni: Osteoid osteoma of
Figures 11.83A and B Peripheral giant cell granuloma (Courtesy: mandible, JOMFP. 2007:11(2);52-5.
Dr Aparna Thombre, Reader, Department of Oral Pathology, 12. Orzincolo C, Ceruti S, Cardona P et al. Diagnostic imaging
VSPM Dental College and Hospital, Nagpur, Maharashtra, India) of osteoid osteoma. Radiol Med. 1995;92:351-7.
Benign Tumors
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2007; 8(4):52-9.
1. Which one of the following tumor is of epithelial origin: 11. Antoni type A and Antoni type B tissue seen in:
a. Fibroma b. Lipoma a. Neuroma b. Glomus tumor
c. Papilloma d. Osteoma c. Neurilemmoma d. Neurofibroma
2. Mark the odd one from the following: 12. “ Café-au-lait” spots seen in:
a. Papilloma b. Keratoacanthoma a. Ganglioneuroma b. Fibroma
c. Nevus d. Myxoma c. Rhabdomyoma d. Neurofibroma
3. ‘Self healing carcinoma’ refers to: 13. Multinucleated giant cells and young spindle shaped
a. Verruciform xanthoma cells seen in:
b. Keratoacanthoma a. Peripheral giant cell granuloma
c. Nevus b. Pyogenic granuloma
d. Fibroma c. Teratoma
4. Multiple papillomas are associated with: d. Pregnancy tumor
a. Cowden’s syndrome b. Ramsay hunt syndrome 14. Staphylococcus or streptococci involved in:
c. Proteus syndrome d. Nager’s syndrome a. Pyogenic granuloma b. Teratoma
5. Foam cells are seen in: c. Both d. None
a. Keratoacanthoma b. Verruciform xanthoma 15. Dysphagia seen in:
c. Adenoma d. Both a. Torus palatines b. Osteochondroma
6. ‘Self healing carcinoma’ refers to: c. Torus mandibularis d. None
a. Verruciform xanthoma 16. Reed Sternberg cells are characteristically seen in:
b. Myxoma a. Alpha thalassemia b. Glandular fever
c. Keratoacanthoma c. Hansans disease d. Hodgkins disease
d. Nevus 17. Rhabdomyoma is a tumor originating from:
7. ‘Whorl and cartwheel’ pattern of fibroblasts seen in: a. Nerve tissue b. Smooth muscle
a. Giant cell fibroma b. Fibrous epulis c. Striated muscle d. Vascular endothelium
c. Chondroma d. Fibrous histiocytoma 18. Abtropfing affect is seen in:
8. ‘Slip sign’ seen in: a. Junctional nevus b. Pemphigus
a. Lipoma b. Osteoid osteoma c. Apthous ulcer d. Erythema multiforme
c. Fibroma d. Nevus 19. Satellite lesion with locally invasive property is seen in:
9. ‘Pumping tooth syndrome’ is present is: a. Chronic hypertrophic candidiasis
a. Osteochondroma b. Hemangioma b. Leukoplakia
c. Lymphangioma d. Neuroma c. Dental ulcers
10. Strawberry angioma and Salmon’s patch are types of: d. Hemangioma
a. Cavernous hemangioma 20. Oral hairy leukoplakia is seen in which of the following
b. Arterial hemangioma conditions:
c. Central hemangioma a. AIDS b. Hepatitis B
d. Capillary hemangioma c. Smokers keratitis d. Candidiasis
21. Acanthosis with intraepithelial vacuolation and hyper- 27. Storiform pattern of fibrous tissue is seen in:
keratosis is seen in: a. Fibrosarcoma
a. Hairy tongue b. Malignant fibrous histiocytoma
218 b. Hyperplastic candidiasis c. Neurofibroma
c. Speckeled leukoplakia d. Ameloblastic Fibroma
d. Desquamative gingivitis 28. Which of the following shows pseudo epitheliomatous
22. Which of the following is not a features of torus hyperplasia:
mandibularis: a. Sq cell carcinoma
a. Common in mongoloid b. Basal cell carcinoma
b. Present on the lingual surface of mandible below the c. Verrucous carcinoma
mylohyoid line d. Granular cell myoblastoma
c. Usually bilateral 29. White rough pedunculated lesion on palate is most
d. May or may not associated with torus palatinus likely:
23. Hemangiopericytoma resembles: a. Pleomorphic adenoma
a. Hemangioma b. Glomous tumor b. Papilloma
c. Ewings tumor d. Plasmacytoma c. Nevus
24. Which of the following epithelial changes commonly d. Fibroma
signify precancerous condition: 30. Which of the following is a connective tissue tumor:
a. Dyskeratosis a. Lipoma b. Melanoma
b. Hyperkeratosis c. Carcinoma d. Papilloma
c. Parakeratosis 31. Etiology of neurofibromatosis is:
d. Acanthosis a. Genetic b. Viral
25. A patient has filmy white opalescence bilaterally on c. Injury d. Endocrine
buccal mucosa, the lesion fades on stretching. The most 32. Hodgkin disease is considered to be:
likely diagnosis is: a. Follicular reticulosis
a. White sponge nevus b. Inflammatory disease
b. Leukoplakia c. Chronic granulomatous disease
c. Lichen planus d. A malignant neoplasm
d. Leukoedema
33. Presence of verocay bodies and having predilection for
26. Papillomatous tongue is observed in: occurrence in the tongue are seen in:
a. Lymphangioma a. Granular cell myoblastoma
b. Hyalinia cutus et mucosa syndrome b. Neurilemmoma
c. Fetal face syndrome c. Neurofibroma
d. Tuberous sclerosis d. Metaplasia
12 Premalignant Lesions and
Conditions
Chapter Outline
Â Concept of precancer Â Snuff dipper lesion

Â Terminology and Definitions Â Cigarette smoker’s lip lesion
Â Field cancerization Â Lichen planus
Â Leukoplakia Â Erosive lichen planus
Â Erythroplakia Â Oral submucous fibrosis
Â Carcinoma in situ Â Dyskeratosis congenita
Â Bowen’s disease Â Lupus erythematosus
Â Smoker’s palate or stomatitis nicotina
CONCEPT OF PRECANCER fact, do so only in a small proportion of cases, forcing the

clinician to make some very real choices relative to the
The concept of a two-step process of cancer development management of such lesions. Individuals with oral precancer
in the oral mucosa, i.e. the initial presence of a precursor run 69 times greater risk of developing oral cancer as
(premalignant, precancerous) lesion subsequently deve- compared to tobacco users who do not have precancer.
loping into cancer, is well-established.
It is applied to that altered state of tissue which often,
TERMINOLOGY AND DEFINITIONS
but not always, has high potential to undergo malignant
transformation. Terminology and definitions currently used in the field
Some benign lesions or conditions, for varying length of oral precancer are still debated. The use of the terms
of time, also generally precede oral cancer. Interestingly, ‘oral precancer’ and ‘oral premalignancy’ is bit confusing
these lesions or conditions share the same etiological and poses few difficulties, since this terminology signifies
factors with oral cancer, particularly the use of tobacco an invariable development of carcinoma from precursor
and exhibit same site and habit relationship. Many of them diseases.
show high potential to become cancer and are therefore Several researchers have tried to come to consensus
termed as precancerous. over internationally accepted terminologies and definitions
It is especially important to remember that a premali- since many decades.
gnancy is not guaranteed to eventually transform into cancer, The World Health Organization (WHO) has accepted the
as is often but erroneously believed. Many precancers, in latest international proposed terminology and definitions,
subdividing oral precancer into precancerous lesions and since attempted to use recent molecular techniques
precancerous conditions. to elucidate the mechanism that underlies the clinical
phenomenon of field cancerization.
220 Oral Precancer is Distinguished by WHO
Precancerous lesion: It is defined as a morphologically LEUKOPLAKIA
altered tissue in which cancer is more likely to occur,
than its apparently normal counter parts. For example, The term leukoplakia originates from two Greek words-
Leukoplakia, erythroplakia, mucosal changes associated leuko, i.e. white and plakia, i.e. patch.
with smoking habits, carcinoma in situ, Bowen disease, It is defined as any white patch on mucosa, which
and actinic keratosis, cheilitis and elastosis. cannot be rubbed or scraped off and which cannot be
attributed to any other diagnosable disease. It may also
Precancerous condition: It is defined as a generalized state be defined as a keratotic white lesion of oral mucosa that
or condition associated with significantly increased risk for cannot be characterized clinically or histopathologically as
cancer development. For example, oral submucous fibrosis any other disease entity. Different definition of leukoplakia
(OSMF), syphilis, sideropenic dysplasia, oral lichen is described in Table 12.2.
planus, dyskeratosis congenita and lupus erythematosus
(Table 12.1). Classification
It is desirable to record separately the various forms of
Field Cancerization
leukoplakia and for this purpose, the following subdivisions
The concept of field cancerization also should be understood are recommended.
with precancerous lesions and conditions. Slaughter et al in
1953 postulated the concept of field cancerization. It was
hypothesized that the entire epithelial surface of the upper
Table 12.2 Definition of leukoplakia
aerodigestive tract has an increased risk for the development
of (pre)malignant lesions because of multiple genetic A white patch or plaque that WHO 1978
abnormalities in the whole tissue region. It is described cannot be characterized clinically
as the histologically altered epithelium surrounding tumor or pathologically as any other
disease
samples taken from the upper aerodigestive tract.
It is considered to be the result of exposure to Whitish patch or plaque that can- First International
carcinogens that caused multiple genetic abnormalities in not be characterized, clinically Conference on oral
or pathologically, as any leukoplakia. Malmo,
the whole tissue region and due to widespread migration of
other disease and which is not Sweden, 1984
transformed cells through the whole aerodigestive tract.
associated with any other physical
Two types of migration might be involved in the or chemical causative agent
concept of this last theory: except the use of tobacco
∙ Migration of tumor cells by, for example, saliva A predominantly white lesion International Symposium,
(micrometastases); or of the oral mucosa that cannot Uppsala, Sweden,1996
∙ Intraepithelial migration of the progeny of the initially be characterized as any other
transformed cells. However, many researchers have definable disease
A predominantly white lesion International Symposium,
of the oral mucosa that cannot Uppsala, Sweden
Table 12.1 Premalignant lesions and conditions be characterized as any other
(WHO 1978) definable disease
A predominantly white lesion WHO (1997)
Precancerous lesions Precancerous conditions
of the oral mucosa that cannot
Leukoplakia Submucous fibrosis be characterized as any other
Erythroplakia Actinic keratosis definable lesion
Palatal lesions in reverse Lichen planus Not defined-no distinction is WHO (2005)
smokers Discoid lupus erythromatosus made from other white patches
Premalignant Lesions and Conditions
Clinical Classification (WHO 1980) Flow chart 12.1 Etiopathogenesis of leukoplakia
∙ Homogeneous: Lesions that are uniformly white

– Smooth 221
– Furrowed( fissured)
– Ulcerated
∙ Nonhomogeneous: Lesions in which part of the lesion
is white and rest appears reddened.
– Nodular
– speckled.
According to Banoczy
∙ Leukoplakia simplex: A uniform raised plaque formation,
varying in size, with regular edges. It corresponds to
homogeneous type of leukoplakia.
∙ Leukoplakia erosiva: A lesion with slightly raised,
rounded, red and/or whitish excrescence, that may be
described as granules or nodules
∙ Leukoplakia verrucosa: It is characterized by verrucous
proliferation raised above the mucosal surface.
Points to Remember
WHO (1998) described subdivisions of leukoplakia as:
• Thin, smooth leukoplakia (preleukoplakia older
terminology): Translucent thin gray soft flat plaques
usually with sharply demarcated borders. smokeless tobacco like chewable tobacco and oral use of
• Thick, fissured leukoplakia: Two-thirds of white snuff or it can be used as smoking tobacco such as cigar,
plaques has distinctly white appearance (from cigarette, bidi and pipe. When tobacco is chewed, various
thickening of keratin layer), fissured and are leathery materials leach out of it, such as tobacco tars and resins.
to palpation. These are the extracts of tobacco, containing various
• Granular, verruciform leukoplakia: Lesions have chemical constituents such as Nitroso-no-rnicotine,
surface irregularities of nodular or granular nature nicotine, pyridine, picoline and collidin. All these chemical
with verrucous appearance. constituents as well as the alkaline pH of snuff (8.2 to
• Erythroleukoplakia: Lesion showing intermixed red 9.3) act as local irritants and are related to the alterations
and white areas, because the epithelial cells are so of mucosa. Smokeless tobacco is believed to result in
immature that they no longer are able to produce chemical damage that produces sublethal cell injury within
keratin. the deeper layers of oral epithelium. This in turn, induces
concomitant epithelial hyperplasia. Smoking tobacco is
Etiology harmful as this smoke contains polycyclic hydrocarbons,
Tobacco, alcohol, chronic irritation, candidiasis, electro- beta-naphthylamine, nitrosamines, carbon monoxide,
magnetic reaction or galvanism, syphilis, nutritional nicotine, etc. which act as source of irritation. The heat
deficiency, condition causing xerostomia, hormones, produced by smoking tobacco also plays a major role.
drugs, virus, idiopathic or cryptogenic leukoplakia. Exposure to heat results in alteration of tissue. The initial
signs of heat-induced alteration of tissue are increased
Etiopathogenesis (Flow chart 12.1) reddening and stippling of mucosal surface. As the use of
irritant continues with the exposure to heat, minute white
Local Factors and red striations are formed and the tissue surface may
Tobacco: It refers to dried leaves of nicotina tobaccum. appear slightly swollen. The striations may be caused by
Tobacco is used widely in two forms. It can be used in increased capillary proliferation and keratin formation.
With continued irritation, the lesion precipitates with Regional and Systemic Factors
circumscribed configuration.
Syphilis: It is regarded as a predisposing factor for the
222 Alcohol: The prevalence of leukoplakia is higher among development of leukoplakia. White patches are often seen
the regular and occasional drinkers than the non-drinkers. It on tongue in tertiary syphilis. Spirochete, the causative
causes irritation and burning sensation of oral mucosa, when agent for syphilis has predilection for the actively mobile
applied locally. Alcohol facilitates the entry of carcinogen tissues of tongue. These tissues are heavily involved during
into exposed cells and thus alters the oral epithelium and secondary stage of syphilis which leads to diffuse vasculitis
its metabolism. But most alcohol consumers use tobacco in and progresses to obliterative endarteritis, eventually
some form or the other. resulting in a circulatory deficiency to the lingual papillae.
This causes atrophy of filiform and fungiform papillae and
Sanguinarine: Persons who used toothpaste or mouth
results in bald, smooth lingual surface. Shrinkage of the
rinse containing herbal extract sanguinarine, develop
lingual musculature may also occur resulting in a wrinkled
leukoplakia. This type of leukoplakia sanguinaria
surface. With the protective papillae missing, the dorsum
associated with keratosis.
of tongue is left extremely susceptible to oral irritation
Chronic irritation: Continuous trauma or local irritation and leukoplakia frequently develops on it. Leukoplakic
in the oral cavity is suspected as a causative agent for involvement may be minor or severe and it may be diffuse
leukoplakia. The source of irritation or trauma may be or localized. In many cases, leukoplakia is of dysplastic
any, viz. malocclusion, ill-fitting dentures, sharp broken variety. Carcinoma of tongue frequently develops in such
teeth, hot spicy food, root piece, etc. The usual site to cases of leutic glossitis.
such irritation is the buccal mucosa and less often the
Nutritional deficiency: Deficiency of vitamin A is
alveolar ridge. Chronic irritation must be intense enough
known to produce metaplasia and keratinization of certain
to induce surface epithelium to produce and retain keratin.
epithelial structures. Hence, it may be causative factor for
Leukoplakia is a protective reaction of the traumatized
leukoplakia. Patients with leukoplakia show lower serum
mucosa against chronic irritation. Mechanical factor in
levels of vitamin A. Vitamin B complex deficiency has also
combination with other thermal and chemical factors,
been suggested as a predisposing factor. It might be related
account for the etiology of more than 40 percent cases of
to alteration in the oxidation pattern of the epithelium,
leukoplakia.
making it more susceptible to irritation. In some cases of
Candidiasis: The presence of Candida albicans has sideropenic anemia leukoplakia can occur.
been reported very frequently in association with
leukoplakia, more commonly with nodular type. Candidal Condition causing xerostomia: Salivary gland diseases,
leukoplakia may be associated with other local factors, anticholinergic drugs and radiation can cause some cases
such as tobacco smoking, denture wearing or occlusal of leukoplakia.
friction. Tobacco smoking may result in candidal Hormones: It is difficult to demonstrate significance of
colonization because of increased keratinization, reduced male and female sex hormone deficiency and endocrine
salivary immunoglobulin-A concentration or depressed dysfunction in the etiology of leukoplakia.
polymorphonuclear leukocyte function.
Drugs: Some drugs like anticholinergic, antimetabolic and
Electromagnetic reaction or galvanism: Galvanism is systemically administered alcohol may predispose for the
the generation of current due to difference in the electrical leukoplakia.
potential of two dissimilar metals. Galvanic current may
arise in mouth between dissimilar, opposing or adjacent Virus: Two types of viruses have been linked with
metallic restorations. Patient’s complaint may range from leukoplakia, viz. herpes simplex virus (HSV) and human
a mere metallic taste to persistent pain, due to chronic papilloma virus (HPV). A specific increase in cell-mediated
inflammation of adjacent oral mucosa to even neuralgic immunity to HSV was observed in dysplastic leukoplakia.
pain. These mucosal changes may promote malignant HPV associated antigen has also been demonstrated in
transformation of leukoplakia. cases of leukoplakia. These viruses are believed to induce
mucosal changes by altering the DNA and chromosomal Appearance: The extent of involvement may vary
structure of the cells and by inducing proliferation of such from small, well-localized, irregular patches to diffused
altered cells. lesions involving considerable portion of oral mucosa.
Multiple patches of leukoplakia may vary from non- 223
Idiopathic or cryptogenic leukoplakia: In a small
palpable, faintly translucent, white area to thick fissured
proportion of cases, no underlying cause has been found.
papillomatous indurated lesion areas of involvement
Such lesions are termed as idiopathic (cryptogenic)
are not uncommon (Fig. 12.2). The surface of the lesion
leukoplakia. These lesions have higher potential for
is often finely wrinkled or shriveled in appearance and
malignant transformation.
may feel rough on palpation. Lesion may be white or
Clinical Features yellowish white, but with heavy use of tobacco may
assume brownish color.
Sex and age distribution: It occurs more commonly
in older age group, i.e. 35 to 45 years and above. Males Ebbing tide type: Some leukoplakias that occur in the floor
are affected more frequently than females, due to direct of the mouth are referred to as ebbing tide type, since they
consequence of tobacco habit. Prevalence in India is 0.2 appear similar to the undulations left on the sand by the
to 4.9 percent. ebbing tide. It occurs due to loose binding and consequent
movement of the mucosa in the floor of mouth.
Location: It can occur anywhere on the oral mucosa.
Buccal mucosa and commissures are more commonly Symptoms: Some patients may report a feeling of
involved. The lesion is regarded as commissural, if it increased thickness of mucosa. Those with ulcerated and
extends posteriorly from labial commissure over a distance nodular type may complain of burning sensation. Enlarged
of about 2 cm, in triangular shape and as buccal, if it cervical lymph nodes may signal occurrence of metastasis.
involves the central zone of buccal mucosa in the molar Sometimes the leukoplakic changes may be reversed by
region and along the occlusal line. In males commissural merely removing the source of irritation.
involvement occurs more frequently than buccal one with
the latter’s being the commoner site in females (Fig. 12.1). Pre-leukoplakia: A different entity termed as pre-
Lip lesions are more common in men and tongue lesions leukoplakia has been distinguished. It is a low grade or
are more common in women. The involvement of various very mild reaction of the mucosa appearing as grayish
sites depends upon the type of tobacco habit. Sublingual white but not completely white lesion with slight lobular
keratosis refers to leukoplakia occurring in floor of mouth pattern and indistinct borders blending with the adjacent
and ventral surface of tongue. normal mucosa.
Figure 12.1 Commissural leukoplakia Figure 12.2 Leukoplakia on right side of buccal mucosa
showing homogeneous pattern
Sharp’s Staging
• S tage I: Earliest lesion—nonpalpable, faintly trans-
224 lucent, white discoloration.
• Stage II: Localized or diffuse, slightly elevated
plaque of irregular outline. It is opaque white and
may have fine granular texture.
• Stage III: Thickened white lesion showing induration
and fissuring.
Clinical Types
Homogeneous: It is also called leukoplakia simplex. It
accounts for 84 percent of cases. It is usually, localized
lesions of extensive white patches present a relatively
consistent pattern throughout. However, sometimes the Figure 12.4 Homogeneous leukoplakia (Courtesy: Dr Aparna
surface lesion may be described as corrugated, with pattern Thombre, Reader, Department of Oral Pathology, VSPM Dental
of fine lines, or wrinkled or papillomatous surface. It is College and Hospital, Nagpur, Maharashtra, India)
characterized by raised plaque formation consisting of
single or group of plaques varying in size with irregular with minimum keratinization. Sometimes, it is associated
edges. They are usually white in color but may be with pigmentation of varying intensity, usually on the
yellowish white or yellow. Leukoplakia seen amongst periphery of the lesion. Heat produced during smoking also
clay pipe smokers and betel quid chewers are generally contributes to the occurrence of pigmentation.
of homogeneous type. They have no red component (Figs Nodular leukoplakia: It is also called ‘leukoplakia
12.3 and 12.4). erosiva’ or ‘speckled leukoplakia’. A mixed red white
Ulcerated leukoplakia: It occurs in 13 percent of cases. It is lesion is seen in which small keratotic nodules are scattered
characterized by red area, which at times exhibit yellowish over an atrophic patch of oral mucosa. Nodules may be
areas of fibrin, giving the appearance of ulceration. pinhead sized or even larger. It has got a high malignant
White patches are present at the periphery of the lesion. potential.
It is seen exclusively at commissures and anterior part Verrucous leukoplakia: It is also called leukoplakia
of buccal mucosa. Sometimes, it manifests as ulceration verrucosa. It is characterized by verrucous proliferation
above the mucosal surface. The most common sites are
buccal mucosa, alveolar mucosa, and tongue, floor of
mouth, gingiva and lips. The disease is most commonly
seen in elderly women and is usually detected several years
after its occurrence due to its slow growing nature. It is
seen in 6th and 7th decades of life. It is seen on alveolar
mucosa and cheek. It is a white lesion with a broken up
surface due to multiple papillary projections that may
be heavily keratinized. They may be accompanied by
homogeneous leukoplakia on other oral mucosal surfaces.
It begins as a simple solitary hyperkeratosis, but tends to
become multifocal over varying period of time. It is slow
growing, persistent and irreversible. In the course of time
erythematous component may develop in the lesion. Later,
it becomes exophytic and wart-like and transforms into a
Figure 12.3 Homogeneous leukoplakia seen on right buccal lesion that is clinically and microscopically identical to
mucosa verrucous carcinoma or squamous cell carcinoma.
Oral florid papillomatosis: Extensive lesions of verrucous

leukoplakia are called ‘oral florid papillomatosis’.
Points to Remember 225

Leuko (white) and plakia (patch), commissural type
is triangular in shape, sublingual keratosis, non-
palpable, faintly translucent, white area to thick fissured
papillomatous indurated lesion, ebbing tide type, feeling
of increased thickness of mucosa, pre-leukoplakia,
homogeneous has localized lesions of extensive white
patches present a relatively consistent pattern throughout,
ulcerated leukoplakia, nodular leukoplakia, verrucous
leukoplakia, verrucous proliferation above the mucosal
surface, oral florid papillomatosis.
Figure 12.5 Frictional keratosis (Courtesy: Dr Sangamesh
Staging of Leukoplakia (OLP Staging) Halawar, Reader, Department of Oral Pathology, CDCRI, Ra-
jnandgao, Chhattisgarh, India)
A clinical staging system for oral leukoplakia (OL
system) on the lines of TNM staging was recommended
by WHO (2005) taking into account the size (L) and the
histopathological features (P) of the lesion.
Staging of Leukoplakia
• Lx: Size not specified
• L1: Single or multiple lesions together <2 cm
• L2: Single or multiple lesions together 2–4 cm
• L3: Single or multiple lesions together >4 cm
– Px: Epithelial dysplasia not specified
– P0: No epithelial dysplasia
– P1: Mild-to-moderate dysplasia
– P3: Severe epithelial dysplasia
• Stage I: L1P0
• Stage II: L2P0
• Stage III: L3P0 or L1/L2 P1 Figure 12.6 Leukoplakia showing acanthosis of stratum
• Stage IV: L3P1 or any LP2. spinosum
Histopathological Features (Figs 12.5 to 12.8) Surface Changes

It should be understood that leukoplakia is purely a clinical Keratosis: There occurs keratinization of mucosal
terminology and histopathologically it is reported as epithelium that is normally non-keratinized or there is an
epithelial dysplasia. increase in the keratin layer in the areas that are normally
WHO in 2005 proposed four grades of epithelial keratinized.
dysplasia based on architectural disturbances and
Hyperorthokeratinization/hyperkeratinization: There
cytological atypia.
is abnormal increase in thickness of orthokeratin layer or
∙ Squamous hyperplasia – benign lesion
stratum corneum over the surface.
∙ Mild dysplasia – better prognosis
∙ Moderate dysplasia Hyperparakeratinization: Superficial epithelial cells are
∙ Severe dysplasia. flat and acidophilic with pyknotic nuclei. There is increased
226
A
Figure 12.7 Leukoplakia showing 1-hyperorthokeratosis,
2-acanthosis, 3-juxta epithelial inflammatory cells, 4-broad
blunt rete pegs (Courtesy: Reader, Department of Oral Pathol-
ogy, VPDC and H, Karalapur, Sangli, Maharashtra, India)
thickness of parakeratin layer, exceeding the normal

thickness.
Epithelial Changes
Vacuolar degeneration: Cytoplasmic structure is
indistinct and the nuclei are pushed to the cell periphery.
Vacuolar degeneration may occur in the stratum spinosum
or stratum germinatum.
B
Acanthosis: An increase in the number of cells of stratum
Figures 12.8A and B Leukoplakia (Courtesy: Dr Aparna
spinosum (spinous layer or prickle cell layer) leading to the Thombre, Reader, Department of Oral Pathology, VSPM
abnormal thickening of the layer. Dental College and Hospital, Nagpur, Maharashtra, India)
Basal cell hyperplasia: An increase in the number of cells
in the basal layer of epithelium.
probability of leukoplakia progressing to malignancy
Intraepithelial edema: There can be intracellular edema which are called as dysplastic features and lesions which
(hydropic degeneration) which consists of swollen, show such features are said to be having epithelial
rounded epithelial cells with a honeycomb like cytoplasm, dysplasia.
but with the same staining affinity for the nucleus or Epithelial dysplasia is reported in 3 percent of snuff
intercellular edema (spongiosis) which consists of dilation induced leukoplakias and 16 percent of smoking habit-
of intercellular spaces and disruption of intercellular related to leukoplakia. Nodular leukoplakia shows
junctions due to accumulation of fluid between the higher frequency of epithelial dysplasia as compared
epithelial cells. to others. It is usually graded into mild, moderate and
severe categories, depending upon the degree and extent
Dysplastic Changes of involvement of the epithelium. Histological diagnosis
All leukoplakias do not lead to malignancy. Only a few is established, if two or more of the following signs are
of them do so. There are various features which indicate present.
Microscopic Features Associated with Oral maturation. In dysplasia this process is hampered. Cells fail
Epithelial Dysplasia to mature and retain their basal cell appearance leading to
immature morphology sometimes.
Architectural (tissue) changes: 227
• Loss of polarity Abnormal stratification of the epithelium: As maturation
• Abnormal orientation of epithelial cells is affected, different layers of the epithelium are not clearly
• Basal cell hyperplasia demarcated. The cells are not defined in strata and appear
• Increased cellular density almost similar to each other.
• Bulbous drop-shaped rete pegs Dyskeratosis: Keratinization is a process where epithelial
• Disordered maturation from basal to squamous cells cells mature to accumulate keratin proteins. This process
• Abnormal stratification of the epithelium is always prominent in the stratum corneum or uppermost
• Dyskeratosis layer of the epithelium. In dysplasia, epithelium shows
Cellular changes: dyskeratosis where this process is abnormal and it may start
• Abnormal variation in nuclear size (anisonucleosis) from the lower level itself. So in such lesion keratinized
• Abnormal variation in cell size (anisocytosis) epithelial cells may be found in prickle cell layer where
• Increased nuclear/cytoplasmic ratio they are usually not present. This is called as individual cell
• Enlarged nuclei and cells keratinization. Sometimes, a group of flattened epithelial
• Hyperchromatic nuclei cells form tight concentric rings leading to formation of
• Increased mitotic figures keratin pearl may be seen.
• Abnormal mitotic figures (abnormal in shape or
location) Cellular Changes
• Nuclear and cellular pleomorphism Abnormal variation in nuclear size (anisonucleosis):
• Increased number and size of nucleoli. In dysplasia, there is profound changes in the nucleus.
These include variation in nuclear size and shape. Nucleus
Cytological Features of Dysplasia becomes enlarged with prominent nucleoli. All these
changes indicate that nucleus is very active.
Loss of polarity and abnormal orientation of epithelial
cells: Basal epithelial cells are usually cuboidal or short Abnormal variation in cell size (anisocytosis): Cytoplasm
columnar. They are arranged perpendicular to basement also shows great variation is size and shape. Few cells
membrane. In dysplastic lesions this arrangement is become very big gaining giant proportions.
changed. Cells become haphazardly arranged on the Increased nuclear/cytoplasmic ratio: Normal nuclear/
basement membrane. cytoplasmic ratio is in the range of 1:4 to 1:6. In malignancy
Basal cell hyperplasia: Basal cells are most active cells and premalignant lesions, this ratio is altered as there is
that have capacity to divide. As in dysplasia there is increase in size of nucleus and cytoplasm. Nucleus and
increased mitosis the basal cells divide to form a large cytoplasm become almost equal in volume.
number of basaloid cells. Abnormal mitotic figures (abnormal in shape or
Increased cellular density: Because of increased mitosis location): In addition to increased mitosis, there is
there is increase in the cell density in the epithelium formation of abnormal mitosis leading to formation enlarged
tripolar or tetrapolar mitotic figures. Sometimes nucleus
Bulbous drop-shaped rete pegs: As basal cell proliferation divides without division of cytoplasm (poikilocarynosis).
induces the change in basal part of rete pegs and they Normally, mitosis is limited to basal cells. But in dysplasia
appear very broad. This gives rete peg bulbous shape of even upper layers show mitotic figures.
drop shape.
Disordered maturation from basal to squamous cells: Connective Tissue Changes
Epithelial cells gradually mature as they move towards the Chronic inflammatory cell infiltration is seen in connective
surface. As they move they differentiate or mature to start tissue of leukoplakia in 50 percent cases. Sub mucosal,
forming keratin and depositing it. This process is called as homogeneous, eosinophilic material is usually seen in
connective tissue. Also, there is replacement of degenerated hyperplastic cells without significant atypical nuclear
elastic fibers by a hyaline fibrous tissue. This is known as changes. Nuclei in the cells of the augmented basal and
hyaline degeneration. It is seen in 10 percent of cases. parabasal layers may be moderately enlarged but still
228 maintain a uniform distribution of nuclear chromatin.
Grading of Dysplasia Occasional typical mitosis may be found in or near the
There are various schemes for grading dysplasia. These basal layer. Small numbers of epithelial cells, less than 5
are WHO system, Ljubljana system Smith and Pindborg percent, are dyskeratotic.
system of photographic standards, etc. Atypical hyperplasia (risky epithelium): Epithelium
demonstrates a recognizable alteration of epithelial cells
WHO Dysplasia System towards malignancy, but not to such a degree as is seen in
• Hyperplasia with increased number of cells: This carcinomatous cells. Stratification is still preserved in the
may be in the spinous layer (acanthosis) or in the basal general epithelial structure. The nuclei are enlarged and the
and parabasal cell layer (basal cell hyperplasia). The nuclear contour may be irregular with marked variations
architecture of the epithelium is preserved, and there is in staining intensity. The nuclear/cytoplasmic ratio is
no cellular atypia. increased. Mitotic figures are increased but not numerous,
• Dysplasia with three grades: and they are found within the two-thirds of the epithelium
1. Mild dysplasia: Architectural disturbance is limited above the basement membrane. They are rarely, if ever,
to the lower-third of the epithelium, accompanied abnormal. Dyskeratotic cells are frequent. Civatte bodies
by cytological atypia. (apoptotic cells) may be present.
2. Moderate dysplasia: Architectural disturbance Carcinoma in situ: This shows the features of carcinoma
extends into the middle-third of the epithelium, without invasion. Stratification of the epithelium as a
accompanied by an upgraded degree of cytological whole is lost. Marked cellular alterations of the type
atypia. found in atypical hyperplasia are present to a considerably
3. Severe dysplasia: Architectural disturbance is greater degree. Many mitotic figures present throughout
greater than two-thirds of the epithelium with the epithelium, including its upper one-third and abnormal
associated cytological atypia or architectural mitoses are frequently found.
disturbance in the middle-third of the epithelium
with sufficient cytological atypia to upgrade from Malignant Potential
moderate-to-severe dysplasia. The term malignant transformation is used to denote
• Carcinoma in situ: Full or almost full thickness development of oral cancer from pre-existing leukoplakia
of the epithelium shows architectural disturbance, (Table 12.3). Malignant transformation occurs in 0.3 percent
accompanied by pronounced cytological atypia. to 10 percent of cases. It is higher in women (6%) than
Atypical mitotic figures and abnormal superficial men (3.9%), due to involvement of endogenous factors.
mitoses are present. Leukoplakia associated with chewing habit of tobacco shows
higher rate of transformation as compared to others. In buccal
Ljubljana Classification System
mucosa and commissural region 1.8 percent malignant
Simple hyperplasia: A benign hyperplastic process with transformation occurs. In lip and tongue region 16 percent
retention of the normal pattern of the epithelium which is to 38.8 percent malignant transformation occurs. Nodular
thickened because of an increased prickle cell layer. The dysplasia has got higher risk of malignant transformation
cellular components of the basal and parabasal region of than other clinical types. Idiopathic leukoplakia and candida-
the epithelium (one to three layers) remain unchanged. associated leukoplakia also come under high risk (Table 12.4).
There is no cellular atypia. If the following features are present, there is a high risk
Abnormal hyperplasia: Basal and parabasal cells are of malignant transformation:
augmented to a degree which constitutes up to one- ∙ Persistence of lesion for several years
half of the total epithelial thickness. It is important that ∙ Female patient
stratification is fully retained. Occasionally, more than ∙ Lesion situated on the margins, base of tongue and
this proportion of the epithelium may be involved by the floor of mouth
Table 12.3 WHO (2005) classification for prognosis

weight for 3 months. If relapse occurs then low doses of
13-cis-retinoic acid 0.5 mg/kg body weight are given for
Phases of leukoplakic lesions based on malignant risk and 9 months. It may be given with β carotene (this drug may
prognosis 229
produce a variety of toxic effects which include facial
• Phase I: Thin, smooth leukoplakia – better prognosis erythema, dryness, peeling of skin, conjunctivitis and
• Phase II: Thick, fissured leukoplakia hypertriglyceridemia).
• Phase III: Proliferative verrucous leukoplakia (PVL) –
higher malignant transformation rate Antioxidant therapy: Considerable data shows that
• Phase IV: Erythroleukoplakia – poor prognosis β-carotene supplementation can be beneficial for treatment
of oral leukoplakia.
Vitamin A palmitate: Short-term treatment with vitamin
Table 12.4 Malignant transformation potential of various
A palmitate along with aromatic retinoid of all trans -B-A
lesions
vitamin acid plus B-cis-B-A vitamin acid may show
Disease Malignant healing and improvement.
potential
Proliferative verrucous leukoplakia ++++++
Nystatin therapy: It is given in candidal leukoplakia.
500000 IU twice daily plus 20 percent borax glycerol or
Erythroplakia ++++++
1 percent gentian violet or mouth rinses with chlorogen
Nicotine palatinus in reverse smokers +++++
solution.
Oral submucous fibrosis +++
Vitamin B complex: It is given as supplement in cases of
Speckled, granular (non-homogeneous) +++
leukoplakia commissural and lingual lesion.
Actinic cheilitis +++ Antimycotic preparation: The antimycotic preparation
Smooth, thick (homogeneous) leukoplakia ++ canesten and pimafucin have also been effective.
Smokeless tobacco keratosis + Panthenol: Panthenol lingual tablet and oral spray may be
Lichen planus + used against glossitis and glossodynia, in case of tongue
lesion.
∙ Erosive lesions Estrogen: In some cases, administration of estrogen can
∙ Combination of above factors. be helpful.
Management Surgical management: To give proper treatment
Elimination of etiological factor: Various etiological microscopic examination is necessary for which biopsy is
factors like smoking, sharp, broken down teeth should taken. The most meaningful sites for taking biopsy specimen
be stopped or removed. You should also exchange faulty are the areas that display greater surface irregularities
metal restorations and metal bridge and elimination of such as cracks and fissures and those associated with
other etiological factors like syphilis, alcohol, etc. erythematous areas. We can go for conventional surgery or
cryosurgery or fulguration or LASER.
Vitamin therapy: It has a protective effect on the
epithelium. Daily requirement is 4000 IU. It is given orally,
ERYTHROPLAKIA
parentally or topically in a therapeutic dose of 75000-
300000 IU for 3 months. Vitamin A may be used topically It is also called erythroplasia of Queyrat. Erythroplakia is
after painting the lesion with podophyllin solution (it a persistent velvety red patch. Reddish color results from
inhibit mitosis). absence of surface keratin layer and due to presence of
connective tissue papillae containing enlarged capillaries
Vitamin A + Vitamin E: This therapy is given to inhibit
projected close to the surface.
metabolic degradation.
The first mention of a non-hemorrhagic red patch of
13-cis-retinoic acid: It is a synthetic analog of vitamin an upper aerodigestive tract site was in a vocal cord lesion
A; usually given in high doses of 1.5 to 2 mg/kg body reported in 1852.
The first truly descriptive paper of erythroplakia,

however, was a 1911 report by Queyrat, of a red macule
on the glans penis of a syphilitic patient. It was Queyrat
230 who coined the term erythroplasia. About the same time,
Rubin used the term incipient carcinoma to describe the
microscopic features of erythroplakia, most common
histopathological diagnosis as carcinoma in situ, based on
his experience with lesions of uterine cervix.
Definition
It is term used for chronic red mucosal macule which cannot
be given any other specific diagnostic name and cannot be
attributed to traumatic, vascular or inflammatory causes.
Classification
Figure 12.9 Erythroplakia on right side showing red lesion
∙ Homogeneous
∙ Erythroplakia interspersed with patches of leuko-
plakia the number of underlying blood vessels through which the
∙ Speckled or granular. blood flows, which in turn may be secondary to localized
inflammatory or immunological responses caused by the
dysplastic, i.e. ‘foreign,’ epithelial cells. In some cases, the
Etiology
color may result from a lack of surface keratin or extreme
It can be idiopathic. Alcohol and smoking can be causative thinness of the epithelium.
factor for erythroplakia.
A secondary infection or superinfection with candi- Homogeneous form: Commonly found on buccal mucosa
diasis may be associated with dysplastic oral mucosal and soft palate and rarely on tongue and floor of mouth.
cells. Candida albicans has often been demonstrated in Homogeneous form appears as a bright red, soft, velvety
erythroleukoplakia lesions and the red component of these lesion with straight or scalloped, well-demarcated margins.
lesions (often the white component as well) diminishes or It is often quite extensive in size. Regardless of the cause
disappears after antifungal therapy, in at least some cases. of the color change, the typical lesion is less than 1.5 cm.
in greatest diameter and half are less than 1.0 cm, but
Clinical Features examples larger than 4 cm have also been seen. It usually
Age and sex predilection: Erythroplakia has been quite sharply demarcated from the surrounding pink
observed to show male predilection and most common in mucosa and its surface is typically smooth and regular in
6th and 7th decade of life. coloration.
Location: It occurs on all mucosal surfaces of the head Granular or speckled form: These are soft, red lesions
and neck area. Half of all cases, however, are found on the that are slightly elevated with irregular outlines and
vermilion or intraoral surfaces, with the rest being evenly granular or fine nodular surface speckled with tiny white
divided between the larynx and the pharynx. Vermilion plaques.
lesions are relatively common and are most often seen on Smooth erythroplakia: This is soft to palpation and has
the lower lip. Intraorally, the lateral and ventral tongue, often been described as having a velvety feel. The pebbled
oral floor and soft palate are most frequently involved. lesions tend to be somewhat firm, but erythroplakia never
Erythroplakia, as the name obviously implies, is actually becomes hard or indurated, until an invasive
asymptomatic. carcinoma develops within it.
Appearance: It is non-elevated, red macule or patch on Erythroleukoplakia: It is quite common to see
an epithelial surface (Fig. 12.9). The exact cause of the red erythroplakia admixed with or adjacent to leukoplakia in
appearance is unknown, but may be related to an increase in mouth. In such lesions, the red areas are the sites most
likely to contain or to develop dysplastic cells and should The spinous layer contains cells displaying atypia,
therefore be the sites most readily biopsied and most hyperchromatism, pleomorphism and increase in number
carefully examined clinically. Erythroplakia interspersed of mitotic figures.
with patches of leukoplakia in which erythematous areas 231
are irregular and often not as bright as homogeneous form, Diagnosis
are most frequently seen on tongue and floor of mouth. The Differentiation of erythroplakia with malignant changes
borders may be well circumscribed or blend impercibly and early squamous cell carcinoma, from the benign
with surrounding oral mucosa. inflammatory lesions of oral mucosa is enhanced by use of
Unlike leukoplakia, erythroplakia is seldom multiple 1 percent toludine blue test. The solution is applied locally
and rarely covers extensive areas of mouth. Also unlike by swab or oral rinse. Malignant type retains it, owing to
leukoplakia, erythroplakia seems seldom to expand increased nuclear DNA content of tumor cells.
laterally after initial diagnosis, although this may be an
artifactual feature because most lesions are completely Management
removed or destroyed immediately after formal diagnosis. Incisional biopsy is always the preferred method for
establishing a microscopic diagnosis of suspicious intraoral
Histopathological Features lesions. Since erythroplakia is so closely correlated with
It exhibits epithelial changes ranging from mild dysplasia severe dysplasia, carcinoma in situ and invasive carcinoma,
to carcinoma in situ and even invasive carcinoma (Fig. incisional biopsy is especially indicated. Excisional biopsy
12.10). Epidermoid carcinoma may show any degree of of a potential malignancy may result in undertreatment and
differentiation from poorly to well differentiated. It appears violation of surgical oncologic principles.
multicentric in origin. The definitive treatment of erythroplastic lesions
The carcinoma in situ shows epithelial dysplasia remains controversial. A conservative surgical procedure
throughout the entire thickness of the epithelium, without such as mucosal stripping is often performed, with
invasion into the underlying connective tissue. minimal damage to the deeper connective tissues. This has
Connective tissue rete pegs extend very high into the the distinct advantage of preserving tissues for microscopic
epithelium and the epithelium over the tips of these rete pegs evaluation of potential regions of invasion.
is often very thin, capillaries in these superficial pegs are Destructive techniques such as laser ablation,
frequently dilated and the absence of any significant amount electrocoagulation and cryotherapy have also proved to be
of surface orthokeratin or parakeratin or at the most only thin effective. The key to therapy in this disease is extended
layer, also contributes to the red color of the lesion. clinical follow-up. Patients should be examined every 3
months for the first postoperative year and every 6 months
for an additional 4 years. After that, annual re-evaluation
with a thorough head and neck examination is advisable.
Elimination of a suspected irritant.
Points to Remember
Erythroplasia of Queyrat, absence of surface keratin
layer, nonelevated, red macule or patch on an epithelial
surface, homogeneous form, granular or speckled form,
smooth erythroplakia, erythroleukoplakia, mild dyspla-
sia to carcinoma in situ, atypia, hyperchromatism, pleo-
morphism, toludine blue test, laser ablation, electroco-
agulation, cryotherapy.
CARCINOMA IN SITU
Figure 12.10 Erythroplakia showing carcinoma in situ with It is also called intraepithelial carcinoma. Severe
hyperchromatism dysplastic changes in a white lesion indicate considerable
risk of development of cancer. The more severe grade of to find multiple areas of carcinoma in situ interspersed by
dysplasia merges with the condition known as carcinoma essentially normal appearing epithelium producing multi-
in situ. It is more common on skin but can also occur on focal carcinoma in situ.
232 mucous membrane.
Management
Clinical Features No uniformly accepted treatment. Lesion may be surgical
Age and sex distribution: Male predilection and occurs excise, cauterized and even exposed to solid carbon dioxide.
more commonly in elderly persons.
Points to Remember
Location: It common sites are floor of mouth, tongue and
lips. Intraepithelial carcinoma, combination of leukoplakia
and erythroplakia, parakeratin, loss of orientation of
Appearance: Lesion appearance may be like leukoplakia, cells, blending of the epithelia, hyperplasia of the
like erythroplakia. It may be a combination of leukoplakia altered epithelium, nuclear/cytoplasmic ratio, nuclear
and erythroplakia, ulcerated lesion, ulcerated and white hyperchromatism.
lesion, red and ulcerated lesion or may be nonspecific.
Bowen’s Disease
It is localized intraepidermoid carcinoma that may progress to
Keratin may or may not be present in/on the surface of
invasive carcinoma over many years, which is characterized
lesion but if present is more likely to be parakeratin, rather
by progressive scaly or crusted plaque like lesion.
than orthokeratin (Fig. 12.11).
It can be caused by sun exposure and arsenic ingestion.
There is loss of orientation of cells and their polarity.
There is sharp line of division between normal and altered Clinical Features
epithelium extending from the surface, down to the
connective tissue rather than blending of the epithelia. It occurs on male and female genital mucosa and in oral
In some cases, there appears to be hyperplasia of the mucosa as erythroplakia, leukoplakia or erythematous lesion.
altered epithelium, while in others there may be atrophy. It appears as a slowly enlarging erythematous patches
An increase in nuclear/cytoplasmic ratio and nuclear with little to suggest the malignant process.
hyperchromatism are sometimes seen. It is also unusual There is a red and slightly scaly area on the skin, which
eventually enlarges and turns into white or yellowish
lesion. When these scales are removed it produced a
granular surface without bleeding.
There are intra-epithelial features of malignancy. The
epithelial cells of the lesion lie in complete disarray with
highly atypical hyperchromatic nuclei and multiple nuclear
forms.
Management
Use of freezing technique, diathermy, cauterization,
radiotherapy or application of cytotoxic drugs.
Points to Remember
Intraepidermoid carcinoma, caused by sun exposure,
slowing enlarging erythematous patches, red and slightly
Figure 12.11 Carcinoma in situ showing nuclear hyperchromatism
scaly area, intraepithelial features of malignancy, freezing
(Courtesy: Dr Sangamesh Halawar, Reader, Department of Oral
Pathology, CDCRI, Rajnandgao, Chhattisgarh, India)
technique, diathermy, cauterization, radiotherapy.
ORAL LESION ASSOCIATED WITH

USE OF TOBACCO
233
Smoker’s Palate or Stomatitis Nicotina
It is also called nicotine palatinus. It is seen in persons who
are heavy pipe and cigar smokers. As such it does not have
premalignant potential as this occur in response to heat of
rather than chemical in tobacco smoke.
In some region smoking is done reversely with the lit
end held in mouth. This will produce palatal keratosis and
is called reverse smoker’s palate.
Clinical Types Figure 12.12 Stomatitis nicotina showing red spot

representing palatal salivary gland duct
∙ Mild: Consisting of red, dot like opening on blanched
area.
∙ Moderate: Characterized by well define elevation
with central umbilication.
∙ Severe: Marked by papules of 5 mm or more with
umbilication of 2 to 3 mm.
Clinical Features
Age and sex distribution: It is usually seen in men who
are pipe smokers. It is common in middle age and elderly
adults.
Location: Most commonly affected site is palate. The
lesion is well developed and prominent on keratinized hard
palate. It is restricted to the area which is exposed to heavy Figure 12.13 White plaques seen on palate in patient with
cigarette smoke. stomatitis nicotina
Sign: There is redness and inflammation of the palate. Dried mud appearance: In some cases palatal keratin
The palate develops diffuse, grayish-white, thickened, may become so thickened and fissured that it gives dried
multinodular papular appearance. There is small red spot mud appearance.
in the center of each tiny nodule, representing a dilated
and sometimes partially occluded orifice of an accessory Palatal Changes in Reverse Smoking
palatal salivary gland duct around which inflammatory cell • K eratosis: Diffuse whitening of the entire palatal
infiltration is prominent. The epithelium around the duct mucosa.
shows excessive thickening and keratinization (Figs 12.12 • Excrescences: 1–3 mm elevated nodules, often with
and 12.13). central red dots corresponding to the opening of
It represent focal thickening surrounding the orifice palatal mucous glands.
of the salivary gland which appears as white umblicated • Patches: Well defined, elevated white plaques,
nodule with red center that may be stain brown by deposits which could qualify for the clinical term leukoplakia.
of tar. There is discrete elevated striae with an appearance • Red areas: Well defined reddening of the palatal
somewhat similar to pumice. mucosa.
Tonsillar pillars are usually erythematous. Disco- • Ulcerated area: Crater like areas covered by fibrin.
loration is homogeneous with the exceptions of numerous • Nonpigmented areas: Area of palatal mucosa which
erythematous spot. is devoid of pigmentation.

There is hyperkeratosis and acanthosis of palatal epithe- Location: It occurs in mucosal surface, where snuff is
234 lium. There is also chronic inflammation of subepithelial habitually held.
connective tissue and mucous gland.
Sign: There is painless loss of gingival tissue in the area of
There is also squamous metaplasia of excretory duct
tobacco contact. This can be accompanied by destruction
and hyperplastic ductal epithelium may be seen.
of alveolar bone.
Epithelium lining of minor salivary gland often shows
squamous cell metaplasia and hyperplasia. Obstruction of Tooth wear: There can be localized or generalized wear
duct may lead to formation of retention cyst. of tooth surface. Brown black extrinsic stains are found on
Periductal inflammatory cell infiltrate composed of enamel.
lymphocytes and plasma cells accompanied capillary
Smokeless tobacco keratosis: It is thin, gray, or grapy
proliferation and dilation in zone.
white plaque which is translucent with margin blends
Management gradually into the surrounding mucosa.
It is completely reversible once the habit is discontinued. Snuff pouch: The mucosa has soft velvety feel and if you
The lesions usually resolve within 2 weeks of cessation of stretch the mucosa it will reveal distinct pouch which is
smoking. Biopsy of nicotine stomatitis is rarely indicated. caused by flaccidity in chronically stretched tissue of area
But biopsy should be performed on any white lesion of the of placement of tobacco (Fig. 12.14).
palatal mucosa that persists after 1 month of discontinuation Mucosa usually appears fissured and rippled when not
of smoking habit. stretched. It resembled a sand on a beach after an ebbing
tide.
Points to Remember
Stomatitis nicotina, redness and inflammation of the
palate, diffuse, grayish-white, thickened, multinodular The squamous epithelium is hyperkeratinized and acan-
papular appearance, pumice appearance, tonsillar thotic with or without intracellular vacuolization.
pillars are usually erythematosus, discoloration is Chevrons: These are pointed projection of parakeratin
homogeneous, dried mud appearance, hyperkeratosis, above the superficial epithelial layer.
acanthosis of palatal epithelium, squamous metaplasia Increase subepithelial vascularity and vessels engorge-
of excretory duct, periductal inflammatory cell infiltrate. ment are often seen.
Snuff Dipper Lesion

It is also called smokeless tobacco keratosis, snuff pouch;,
tobacco pouch keratosis and spit tobacco keratosis.
The compound N-nitroso-nor-nicotine (NNN), which
is derived partly from bacterial action on nicotine during
the curing process, is contributed by the action of salivary
nitrites, when tobacco is held in the mouth; occurs in
greater concentration in snuff tobacco.
Clinical Lesion of Smokeless Tobacco

• H yperkeratosis or erythematous lesion of oral
mucosa
• Gingiva and periodontal inflammation
• Combination of above
• Cervical erosion of teeth.
Figure 12.14 Snuff dipper lesion showing hyperkeratosis
Management and pruritic papules. The disease may also affect the
mucosa, hair and nails.
If habit is eliminated, majority of the lesion disappear
Relatively common dermatological disorder occurring
in about 2 weeks. Long exposure to snuff may results in 235
on skin and oral mucous membrane and refers to lace-like
malignant changes in the lesion.
pattern produced by symbolic algae and fungal colonies on
Points to Remember the surface of rocks in nature (lichens). The term planus is
Latin word meaning flat.
Smokeless tobacco keratosis, snuff pouch, painless
loss of gingival tissue, tooth wear, smokeless tobacco Etiology
keratosis, if you stretch the mucosa it will reveal distinct
• Cell-mediated immune response
pouch, sand on a beach after an edding tide, chevrons,
• Haptens
increase subepithelial vascularity.
• Immunodeficiency
• Genetic factors
Cigarette Smoker’s Lip Lesion • Infections
They are generally flat or slightly elevated nodular white • Drugs and chemicals
lesion on one or both lips, corresponding to the site at • Psychogenic factor
which the cigarette is held and apparently smoked down to • Habit
an extremely short length. There is increased redness and • Deficiency of vitamin B1, B6 and C
stippling of lip in localized area. • Electric.
It has elliptical, circular or irregular borders. Pale to
white and were slightly elevated with nodular or papillary Etiology
shape (Fig. 12.15).
Cell-mediated immune response: It is a cell mediated
immune response associated with lymphocyte-epidermal
LICHEN PLANUS interactions, resulting in degeneration of basal cell layer.
Erasmus Wilson described it in 1869. Various mucosal Possible hypothesis are alternation of keratinocytes as a
surfaces may be involved, either independently or result of unknown events resulting in antigenic alternation
concurrently, with cutaneous involvement or serially. of these cells thereby stimulating immunological reaction
Oral mucosa is frequently involved. It is a probable pre- or a primary immunologic reaction causing alternation
cancerous condition. and degeneration of keratinocytes. Cell-mediated immune
Lichen planus is a common inflammatory disease of the response may be caused by various mononuclear cells,
skin presenting with characteristic violaceous, polygonal, i.e. Langerhans cell, macrophages predominantly T
lymphocytes, lymphoblast cells, B lymphocytes and mast
cells. These cells infiltrate the upper part of lamina propria
of sub mucosa. In this cell-mediated response Langerhans
cells are potent antigen presenting cells, lymphocytes
are effective cells and keratinocytes are target cells. The
macrophages are mostly mature, which probably have
functional role with mononuclear cells is suggesting of
cell-to-cell cooperation.
Recent hypothesis of pathogenesis of lichen planus: In
a genetically predisposed individual, haptens (certain drug
or dental material), conventional antigen or super-antigen
of oral microbial origin can induce cell-mediated immune
response resulting in subepithelial T cell infiltration of the
site in oral mucosa with cytokine generation HSP-60 and C
1/10 expression by basal keratinocytes. If individual is not
Figure 12.15 Cigarette smoker lip lesion predisposed to react to HSP-60, then non specific mucositis
occur. If the individual has genetic predisposition, it results followed by reduction in clinical severity. It is suggested
in autoimmune reaction → activation of cytotoxic T cell that drugs that are known to induce lichenoid response, act
→ destruction of basal keratinocytes → oral lichen planus. as agents which amplify the disorder, rather than induce it.
236 To implicate a drug responsible for a lichenoid reaction can
Autoimmunity: The activated T lymphocytes also secrete
be difficult as there is no specific test for it. Association
gamma interferons which induce keratinocytes to produce
between dental filling material and lichen planus has also
HLA-DR and increase their rate of differentiation with
been suggested.
formation of thickened surface. Antigenic information is
transferred from Langerhans cells to lymphocytes, when Psychogenic factor: A relationship of lichen planus
there is mutual expression of HLA-DR. Lymphocytes with stress is quoted and neurogenic basis is suggested.
normally are attracted towards HLA-DR expressing Observation mostly in nervous and highly stressed persons
keratinocytes and may contact the epithelial cell. During this is associated with emotional upset, over work and some
contact, inappropriate epithelial antigenic information may form of mental strain.
be passed to lymphocytes due to HLA-DR linkage. With
Habit: Oral lichen planus has shown association with
this mechanism, self antigen may be recognized as foreign
tobacco habit. Chewers of tobacco and betel have increased
bodies, leading to destruction of basal cells, resulting in an
prevalence of oral lichen planus. Smoking may play a role
autoimmune response. Autoimmune disorders classically
in initiating oral lichen planus of plaque type.
have female predilection and are associated with serum
antibodies with hypergamma-globulinemia. There are Miscellaneous: Occurrence of lichen planus is also
numerous studies which show immune deposits in lichen suggested in association with deficiency of vitamin B1, B6
planus affected tissues but it is not specific. and C, electric potential difference, anemia and patients
with secondary syphilis. It can also occur in some cases
Immunodeficiency: There has been report of decreased
due to trauma and malnutrition. Exacerbation of lichen
serum levels of IgG, IgA, or IgM in lichen planus and the
planus also correspond to periods of emotional upset,
possibility of it as a manifestation of immunodeficiency
overwork, anxiety, hysteria attack, depression and some
has been raised. But at the same time, reports of normal
form of mental strain.
concentrations of IgA and IgM are found; therefore the role
of immunodeficiency is questionable.
Types of Lichen Planus
Genetic factors: Cases of lichen planus are reported in • Reticular
families, twins and husband and wife. Clinically, familial • Papular
lichen planus is somewhat unusual as it appears to affect • Plaque
young patients, is severe, often extensive, involves skin • Atrophic
nails and mucous membrane and is persistent. It has also • Classical
been suggested that familial cause might be environmental • Erythematous
and related to infection, rather than to genetics. • Ulcerative
Infections: A bacterial etiology may be there but results are • Hypertrophic erosive
not confirmed. Spirochetes and rod-like bodies resembling • Bullous
bacteria have also been detected. • Hypertrophied
• Annular
Drugs and chemicals: It is also called lichenoid reaction.
• Actinic
Although the clinical disease of lichen planus has an
• Follicular
immunological basis, some persons with lichen planus
• Linear
have a diathesis for the disorder. The tissues of a person
with diathesis react in a special way to certain extrinsic
Clinical Features
stimuli, making it more susceptible to certain diseases.
Drugs act to increase temporarily the specific antigenic Age and sex distribution: It occurs in adulthood with age
stimulus and hence increase the reaction. If the drug is range for males as 35 to 44 years and for females 45 to 54
withdrawn at a later time, the antigenic stimulus is reduced, years. It has more predilections for females.
Incidence: Prevalence of lichen planus in general Oral Lichen Planus

population is about 0.9 to 1.2 percent and prevalence of
Location: Common sites are buccal mucosa (84%) and to
oral lichen planus is reported between 0.1 and 2.2 percent.
lesser extent tongue, lips, gingiva, floor of mouth and palate. 237
Location: It may involve skin, oral and other mucous Patient may report with burning sensation of oral mucosa.
membranes. About 50 percent of the patients with skin
Appearance: Oral lesion is characterized by radiating
lesions have oral lesions whereas 25 percent of all lichen
white and gray velvety thread-like papules in a linear,
planus have only oral lesions. Oral lesions coexisting
angular or retiform arrangement forming typical lacy,
with genital mucosal lesions are known as vaginogingival
reticular patterns, rings and streaks over the buccal mucosa
syndrome. Other mucosal surfaces including larynx,
and to a lesser extent on the lip, tongue and palate.
glans penis, esophagus, stomach, nasal and vulva may get
involved. Wickham’s striae: Tiny white elevated dots are present at
the intersection of white lines, called as Wickham’s striae
Symptoms: The chief complaint is usually of intense
(Fig. 12.16).
pruritus. The itching associated with LP usually provokes
rubbing of the lesions, rather than scratching. Reticular type: It is most common form and it is mostly
bilateral. It consists of slightly elevated fine whitish lines
Signs: The lesions have a characteristic violet hue. They
that produce lace-like pattern of fine radiating lines, called
are flat-topped, shiny, polygonal papules and plaques. The
as Wickham’s striae. The lesion may present radiating
surface is dry with thin, adherent scales.
white thread-like papules in a linear, annular or retiform
Six P of lichen planus: Six ‘P’s characterize the lesions of arrangement. A tiny white dot is frequently present at the
lichen planus intersection of white lines (Figs 12.17 and 12.18).
They are planar, polygonal, purple, pruritic, papules
Papular: Whitish elevated lesions of 0.5 to 1 mm in size,
and plaques.
well seen on keratinized areas of oral mucosa. Papules are
Cutaneous: Common sites are flexor surface of wrist and spaced apart; still close enough to give pebbled white or
forearm, inner surface of knees and thigh and the trunk, gray color. Sometimes they coalesce. Most often, papules
usually the lumbar and sacral areas. The skin lesions are seen at the periphery of reticular pattern (Fig. 12.19).
appear as small, angular, flat topped papules, only a
Plaque: It is seen on dorsum of tongue and buccal mucosa.
few millimeter in diameter, which may be discrete or
In case of plaque of tongue, disappearance of the tongue
gradually coalesce into larger plaque and is covered by fine
papillae is seen. It spreads in concentric peripheral growth.
glistening scales. Primary symptom is severe pruritis that
may be intolerable. Papules are sharply demarcated from
surrounding skin, which appears red but soon takes reddish
purple or violaceous blue color. Later, dirty brown color
develops. Center of papule may be slightly umblicated.
Wickham’s striae: These are very fine grayish lines which
cover the papules.
Graham-little syndrome: Alopecia occurs in some
patients which may be termed lichen planus planopilaris
(Graham-Little syndrome). Nail changes, particularly
longitudinal ridging and grooving may be seen.
Variant: Various variants of lichen planus like
hypertrophied, actinic, ulcerative, linear forms, etc. are
present, but in dental point of they are not found intraorally
so they are not discuss here.
Figure 12.16 Lichen planus showing Wickham’s Striae (Cour
Koebner phenomenon: Fresh lesions may appear on tesy: Dr Aparna Thombre, Reader, Department of Oral Pathology,
scratch marks or at sites of other nonspecific trauma. VSPM Dental College and Hospital, Nagpur, Maharashtra, India)
238
Figure 12.17 Reticular types of lichen planus showing Figure 12.19 Papular type of lichen planus showing whitish
annular arrangement elevated lesion
common site is buccal mucosa, especially into posterior

and lateral margins of tongue. It is often associated with
striated or keratotic component.
Hypertrophic form: It appears as well circumscribed,
elevated and white lesion resembling leukoplakia.
Annular form: Appears as round or ovoid, white outline
with either pink or reddish pink center. In about 11 percent
of cases, oral lichen planus may be associated with
pigmentation (Fig. 12.20).
Points to Remember
Vaginogingival syndrome, intense pruritus, flat-topped,
Figure 12.18 Reticular types of lichen planus shiny, polygonal papules and plaque, Six ‘P’ of lichen
planus—planar, polygonal, purple, pruritic, papules,
plaques.
It consists of either pearly white or grayish white plaque. • Cutaneous: Violaceous blue color, Wickham’s striae,
Such plaques generally range from slightly elevated and Graham-Little syndrome, Koebner phenomenon
smooth to slightly irregular form. • Reticular type: Slightly elevated fine whitish
Atrophic form: Appears as smooth, red, poorly defined lines, Wickham’s striae, linear, annular or retiform
area, often but not always, with peripheral striae evident. arrangement, a tiny white dot
The attached gingiva is frequently involved in this form of • Papular: Whitish elevated lesions pebbled white or
lichen planus in a so-called desquamative gingivitis pattern. gray color
At the margins of atrophic zones, whitish keratotic striae • Plaque: It is seen on dorsum of concentric peripheral
are usually evident, radiating peripherally and blending growth, pearly white or grayish white plaque
into surrounding mucosa. The gingiva tends to show • Atrophic form: Smooth, red, poorly defined area,
patchy distribution overall the four quadrants in a relatively desquamative gingivitis
symmetrical pattern. It is always symptomatic with • Bullous form: Vesicles and bullae, leave an ulcerated
complain of pain and burning in the areas of involvement. lession
• Hypertrophic form: Well circumscribed, elevated
Bullous form: It consists of vesicles and bullae and
white lesion
there which are short lived. These upon rupturing, leave
• Annular form: Appears as round or ovoid, white outline.
an ulcerated extremely uncomfortable surface. The most
basal cell degeneration induced by the inflammatory

process.
Ulcerative lichen planus: Chronic ulcers occur on the feet. 239
It may accompany oral ulcer and loss of nails.
Lichen planus pigmentosus: Described mainly from
India and Middle East, these lesions present with deeply
pigmented macules on the face and extremities.
Variations in shape:
Linear lichen planus: This may follow marks of injury by
koebnerization, or may occur spontaneously in long linear
arrays or rarely in a dermatome in a zoster-like fashion.
Annular lichen planus: These lesions have central depressed
areas with raised margins. Typically occurs on penis.
Figure 12.20 Annular types of lichen planus of labial mucosa
showing round appearance Guttate lichen planus: Large number of drop-shaped
lesions.
Clinical Variation of Lichen Planus Variation by site:
In many cases, the clinical picture may differ from the Oral: Oral lesions frequently occur in lichen planus.
classical one, the variations being in morphology and Genital: Annular plaques often occur on glans penis,
configuration or there may be modifications of clinical vulval or vaginal lesions, often presenting with chronic
features by the site of involvement. ulcers, may rarely occur.
Variations in morphology: Nails: Nail dystrophies may occur in lichen planus. The
Hypertrophic lichen planus: This may occur as a sole various changes that may occur are—thinning of nail plate,
manifestation or as a part of a more generalized subacute longitudinal ridging, onycholysis, subungual hyperkeratosis
disease. The lesions are elevated, warty, pigmented plaques and pterygium formation (fusion of proximal nail fold with
typically occurring on the shin or around the ankle. These the nail plate).
lesions tend to be persistent.
Palms and soles: Lichen planus of these locations lack
Atrophic lichen planus: The lesions are few in number. the typical color; they are yellowish rather that violaceous
There are depressed and pigmented lesions. papules and plaques.
Follicular lichen planus: It also known as lichen Scalp: Follicular lesions with scarring alopecia are seen at
planopilaris, this may accompany typical lesions or may this site.
be the sole morphologic type. There are small lesions
Special variant:
centered around hair follicles. Lesions on scalp may result
Drug-induced lichenoid reactions: A number of drugs
in alopecia. The combination of follicular lichen planos
may induce development of lesions that clinically
with scarring alopecia of scalp and non-scarring alopecia
and histologically resemble idiopathic lichen planus.
of axilla and pubis or other areas is known as Graham-
The principal offenders are: gold, penicillamine, beta-
Little syndrome.
blockers, antimalarials, captopril, lithium, carbamazepine,
Actinic lichen planus: Described mainly from the Middle chlorpromazine, thiazides, methyldopa, INH, PAS and
East and India, these lesions are common on the face and NSAIDs.
present as hyperpigmented patches with a surrounding
Lichen planus pemphigoides: Bullous lesions occur on
zone of hypopigmentation.
lesions as well as normal skin. This appears to be a variant
Bullous lichen planus: Vesicles and bullae may occur on with combined features of lichen planus and bullous
typical lesions of lichen planus in this variant, due to severe pemphigoid.
Lichenoid contact dermatitis: Contact with color film

developer containing para-phenyldiamine may give rise to
lichen planus like lesions on hands. Amalgams in dental
240 filling material may also give rise to lichenoid oral lesions.
Lichen planus-lupus erythematosus overlap: Lesions with
atrophic center violaceous border occur mainly on hands
and feet. Immunohistologic evidence of both lichen planus
and lupus erythematous, are present.

Hyperorthokeratosis and hyperparakeratosis, acanthosis
with intercellular edema of spinous cells occurs in some
instances.
Civatte bodies are often seen in the epithelium mostly
in the spinous and basal cell layers and lamina propria. Figure 12.22 Lichen planus showing 1-saw tooth rete pegs
They appear as round or ovoid, well defined eosinophilic and hydropic degeneration of basal cells, 2-orthokeratosis,
juxtaepithelial band of epithelial cells
globule with a maximum size of about 20 µm, probably
representing fibrillar transformation degeneration and
premature cell death of basal keratinocytes.
Saw tooth appearance of the rete pegs is seen.
Necrosis or liquefaction degeneration of basal cell
layer with appearance of a band of eosinophilic coagulum
in place of the basal layer.
There is also thickening of granular cell layer with
band like dense inflammatory cellular infiltrate, containing
lymphocytes and occasional plasma cells, in the upper
lamina propria.
Sometimes, the necrotic material is exuded into lamina
propria (apoptosis). The material may be taken up by
Figure 12.23 Lichen planus showing parakeratinized stratified

squamous epithelium connective and tissue shows a juxta-
epithelial band of chronic inflammatory cells (Courtesy:
phagocytes or provide a matrix for the deposition of serum

protein including immune component, resulting in colloid
bodies formation.
Cells from the lamina propria may also contribute to
the formation of colloid bodies. Cellular infiltrate consists
of lymphocytes but plasma cell and histiocytes are also
present sometimes.
Figure 12.21 Lichen planus showing subepithelial Mast cells are observed sometimes below the sub-
inflammation and saw tooth appearance epithelial infiltrate. In deeper layers they contain granules
but undergo degranulation. It is suggested that mast cells Bullous: It shows hydropic degeneration of basal cell layer.
participate in recruitment of lymphocytes to subepithelial Due to this, there is collection of edema at the epithelial
infiltrate. connective tissue interface, resulting in the formation of
Ryan suggested that the pattern of striae of Wickham’s subepithelial bulla. The bulla or vesicle contains clear fluid 241
is due to absence of capillaries in the center with growing and occasionally hemorrhage. A broad band of lymphocytic
vessels at the periphery. cells is seen in the upper corium, prior to rupture of vesicle
Immunofluorescent study: It is positive for direct or bulla.
immunofluorescence reaction with IgA, IgM, IgG antisera.
Most constant feature is presence of sub-epithelial deposits Points to Remember
of fibrinogen and antigenically related substance, which • R eticular: Hyperparakeratosis or hyperorthokeratosis,
can be stain by anti-fibrinogen antisera. granular cell layer, epithelial hyperplasia, saw-tooth
configuration
Histopathological Features of Different Types of • Papular: Keratosis, hyperkeratosis or parakeratosis,
Oral Lichen Planus broad band of lymphocytic infiltration
Reticular: This shows hyperparakeratosis or hyperor- • Plaque: Hyperorthokeratosis, hyperparakeratosis,
thokeratosis. In some cases, both types of keratinization stratum granulosum, lymphocytic juxtaepithelial
may be seen. Granular cell layer is also seen. It also infiltration
features epithelial hyperplasia, although atrophy is present • Atrophic: Epithelium is thin, stratum corneum blend
in some cases. Accentuation of rete pegs in the typical saw- into stratum spinosum
tooth configuration is uncommon. Basal cell layer shows • Bullous: Hydropic degeneration of basal cell
liquefaction degeneration. Cellular infiltrate is primarily of layer, vesicle contains clear fluid, A broad band of
lymphocytes, plasma cells may be seen. lymphocytic cells.
Papular: Keratosis, usually hyperkeratosis or parakera-
Malignant Potential
tosis, may be extensive and stratum corneum may show
considerable increase in width. Parakeratosis is found more The incidence of malignant transformation ranges form
commonly than hyperkeratosis, although alternate areas of 0.4 to 12.3 percent. In India, the incidence of malignant
both types may be present. Acanthosis is not usually seen. transformation is 0.4 percent. Carcinoma development is
Epithelium shows moderate hyperplasia and saw tooth more common in women than in men. Atrophic, erosive
configuration of rete pegs is rarely observed. Basal cell layer and ulcerative lesions showing erythroplakic component
shows liquefaction degeneration and it consists of inter and and tobacco chewers are indicated to be more cancer prone.
intra-cellular vacuoles. Juxtaepithelial connective tissue Management
consists of broad band of lymphocytic infiltration. Occasional
plasma cell and histiocytes may be seen. Inflammatory cells Removal of cause: The causative factor is removed and
usually penetrate into lower layers of epithelium and are this may lead to resolution of lesion subsequently. This can
seen between degenerating epithelial cells. be particularly applicable to lichenoid drug eruption.
Corticosteroid: In most patients with erosive and ulcerative
Plaque: Marked hyperorthokeratosis and hyperparakera-
lesion steroids are commonly used. The rationale behind
tosis is seen in connection with stratum granulosum.
their use is their ability to modulate inflammation and
Epithelial hyperplasia as well as atrophy is seen. A
immune response. Small and moderately sized painful
narrow zone, free of inflammatory cells is seen in relation
lesions can be treated with beclomethasone dipropionate
with basement membrane. Lymphocytic juxtaepithelial
spray, triamcinolone acetonide in gel or cream base. Topical
infiltration is seen in connective tissue stroma.
and intralesional routes are used when systemic steroids are
Atrophic: The epithelium is thin. There is little contraindicated. These routes are useful when the patient
keratinization at the surface and stratum corneum tends refuses needle injection or when treating painful gingival sites,
to blend into stratum spinosum. The basal cells present where injection delivery is impossible. The topical, injectable
hydropic degeneration. Rete pegs are absent. Pattern is and systemic routes are used when there is no systemic
similar to desquamative gingivitis. contraindication and a full steroid dose is required. In case of
some painful gingival lesions topical steroids may be applied mg/kg/day. Oral lesions can be treated using cyclosporine
using soft custom trays by coating steroids on the undersurface as a rinse and expectorant. It is used as 5 mL rinse, TID,
of the tray. This tray anchors to the dental arch while for 8 weeks.
242 covering the painful gingival lesion. Earlier regime consists
Surgical therapy: It is indicated when conventional
of triamcinolone acetonide topically, methylprednisolone 40
methods fail in ulcerative lesion and small solitary lesions.
mg/ml as injectable and prednisolone 5 mg tablet as systemic
In some cases, cryosurgery and cauterization have also
steroid therapy. Injection triamcinolone acetonide 10 mg/ml
been tried.
was also used in patients with serious complain, in a dose of
0.1 mL/cm2. This earlier regime called for topical delivery Psychotherapy: Emotional status of the patient is
QID, for 3 or more weeks, once a week intralesional injection, important in the development of this disease. In some cases,
for 3 weeks (usually 0.5–1.5 mL) and systemic steroid the lesion may regress when the patient is made aware of
(prednisolone 5 mg tab) in tapering doses of 30 mg/day for psychogenic implication of the condition and the nature
the first of 3 weeks, 15 mg/day for the second week and 5 of emotional stress is understood. When the lesions are
mg/day for the third and final week. Topical application of asymptomatic and there is no source of emotional distress
fluocinolone acetonide for 4 weeks is also effective in curing it is often advisable to refrain from therapy as failure to
the disease. Another regime consisting of prednisolone and eradicate the lesion by medication may trigger the patient
levamisole has also been tried successfully recently. This into becoming fearful of cancer. Tranquilizers have been
systemic regime calls for prednisolone 5 mg and levamisole also been tried to reduce anxiety.
50 mg tablets for first three days of rest and this schedule to be Dapsone therapy: Dapsone diamino-diphenylsulfone is
followed for two to three more weeks. an antibacterial sulfone. It is postulated that this particular
Topical application of antifungal agent: Steroid therapy agent may help to control the lymphocyte-mediated
is routinely accompanied by antifungal treatment as progress of lichen planus by modulating the release of
steroid therapy tends to generate an oral fungal infection. inflammatory or chemotactic factor for mast cells or
The prophylactic antifungal therapy usually consists of neutrophils. It is used in severe form of erosive lesions.
clotrimazole oral torches. Nystatin and ketoconazole can
PUVA therapy: In this form of therapy, psoralens and high
also be used.
intensity long wave ultraviolet (PUVA) light is used as a
Vitamin A (Retinoid) analog: Retinoids are useful usually therapeutic agent. The lesion shows improvement during
in conjunction with topical corticosteroids as adjunctive and immediately after the treatment.
therapy either topically or systemically. This is because
of their anti-keratinizing and immuno-modulating effects. Points to Remember
They are particularly effective against keratinized reticular Removal of cause, corticosteroid, beclomethasone
and plaque variants. Topical vitamin A acid cream (0.1%) dipropionate spray, triamcinolone acetonide in gel or
Tretinoin, beta altransretinoic acid, systemic etretinate and cream, topical application of antifungal agent, Vitamin
systemic and topical isotretinoin are also useful in resolution A (Retinoid) analog, cyclosporine, surgical therapy,
of the lesions, but withdrawal of the medication leads to psychotherapy, dapsone therapy, PUVA therapy.
rapid recurrence to the lesion very oftenly. The side effects
of retinoids are more and it includes foci of erythema during
Erosive Lichen Planus
and after the topical treatment. For systemic retinoids, it
includes liver dysfunction, cheilitis and dryness of mucous It presents as chronic multiple oral mucosal ulcers, which
membrane. A new systemic retinoid; temarotene, has been occur when there is extensive degeneration of basal cell
reported effective and free of side effects, other than a layer of epithelium.
slight increase in liver enzymes.
Etiology
Cyclosporine: It is a selective inhibitor of CD4 helper
Drug therapy like NSAIDs, hydrochlorothiazide, penicil-
T lymphocytes that is used systemically to achieve
lamine, angiotensin converting enzyme inhibitors.
immunosuppression. It can be used both, topically and
systemically. The lesion shows complete healing with no Chronic hepatitis: Underlying medical disorders like
recurrence following 8 weeks of systemic cyclosporine 8 chronic hepatitis.
Dental restoration: Reaction to dental restorations. of 10 days to 2 weeks. They may change the pattern of
presentation and involvement from time to time.
Graft versus host disease: Graft versus host disease due
to bone marrow transplantation can cause lichen planus. Histopathological Features 243
Stress: Emotional stress can lead to erosive lichen planus. In the erosive form, the epithelium is completely missing or
only remnants of epithelial tissues are seen. Erosive lesion
Clinical Features appears as ulceration, epithelial thinning and eventual
Age and sex distribution: Female to male ratio is 2:1. destruction.
Average age is 50 years. It is primarily a disease of whites, Hemorrhage may be noted. It shows classical feature of
but it may be seen in blacks. lichen planus, i.e. hydropic degeneration of basal cell layer
Location: Common site is buccal mucosa and lingual with juxtraepithelial inflammatory cell infiltrate.
mucosa. The basal cells are gradually destroyed and overlying
epithelium becomes thin and atrophic. Eventually, epithelium
Symptoms: There is complain of burning sensation and undergoes necrosis and an area of ulceration appears.
pain. Ulcerated area, if infected may show altered population
Appearance: After rupture of vesicles, eroded or frankly of inflammatory infiltrate with polymorphonuclear
ulcerated lesion are seen which appears as a raw painful leukocytes predominating at the ulcerated surface, which is
areas (Fig. 12.24). covered by fibrin.
Lacy white pattern may be present. Eroded and frankly Points to Remember
ulcerated lesions are irregular in size and shape and
Burning sensation and pain, eroded or frankly ulcerated
appear as raw and painful areas. The surface is generally
lesion, lacy white pattern, brightly erythematosus, erosive
granular and brightly erythematous and may bleed upon
component is severe, malignant potential is 1 to 25%,
slight provocation or manipulation. A fibrinous plaque or
epithelium is completely missing, ulceration, epithelial
pseudomembrane may be seen over erosion, while later is
thinning, hemorrhage, juxtraepithelial inflammatory cell
significant.
infiltrate, polymorphonuclear leukocytes.
Bullous type: In case when erosive component is severe and
epithelial separation is from underlying connective tissue
occur which result in rare form called bullous lichen planus. ORAL SUBMUCOUS FIBROSIS
Malignant potential: Malignant potential is 1 to 25 It is a chronic and high-risk precancerous condition. The
percent. Present for a week to month and heal in periods condition was prevalent in the days of Sushruta (600 B.C.), a
great practitioner of ancient medicine where he labeled this
condition as ‘Vidhari’. After lapse of many years, Schwartz
(1952) was the first person to bring this condition again
into limelight. He described the condition as ‘atrophica
idiopathic mucosae oris’. After that the condition has
also been described as idiopathic scleroderma of mouth,
idiopathic palatal fibrosis and sclerosing stomatitis.
Definition
It is an insidious, chronic disease affecting any part of the
oral cavity and sometimes pharynx, although occasionally
preceded by and/or associated with vesicle formation, it
is always associated with juxtaepithelial inflammatory
reaction followed by fibroelastic changes of lamina propria,
with epithelial atrophy leading to stiffness of oral mucosa
Figure 12.24 Erosive lichen planus showing raw painful area and causing trismus and inability to eat.
which adducts with o’methyl guanine in DNA. Prolonged

Etiology
exposure to this irritant leads to malignant transformation.
• Chilies Recently suggested pathogenesis of oral submucous
244 • Tobacco fibrosis is by dual action of areca nut. It is suggested
• Lime that arecoline stimulates fibroblastic proliferation and
• Betel nut
collagen synthesis. The flavanoid catachin and tannins
• Nutritional deficiency
are also components of areca nut and they stabilize the
• Defective iron metabolism
collagen fibrils rendering them resistance to degradation
• Bacterial infections
by collagenase. The attendant trismus is the result of
• Collagen disorders
juxtaepithelial hyalinization and secondary muscle
• Immunological disorders
involvement. Glycogen consumption is physiologically
• Genetic susceptibility
related to cellular activity. Overactivity of muscle results
• Altered salivary composition.
in excessive glycogen consumption, leading to glycogen
depletion. The increased muscle activity and diminished
Etiology blood supply, following connective tissue changes; owing
Chilies: The use of chilies (Capsicum annum and Capsicum to extensive oral submucous fibrosis leads to muscle
frutescence) have been thought to play an etiological role in degeneration and fibrosis.
oral submucous fibrosis. Capsaicin is the active ingredient
Nutritional deficiency: The disease is characterized
of chilies. It is the vanillylamide of 8-methyl-6-nonenic
by repeated vesiculations and ulcerations of oral cavity.
acid, which is the active irritant of the chilies.
A subclinical vitamin B complex deficiency has been
Tobacco: It is a known irritant and causative factor in oral suspected in such cases. The deficiency could be
malignancy. It may act as a local irritant. precipitated by the effect of defective nutrition due to
Lime: Lime is used with betel nut for chewing. It causes local impaired food intake in advanced cases and may be the
irritation and damage to the mucosa with vesicle and ulcer effect, rather than the cause of the disease.
formation in susceptible individuals. It acts as a local irritant. Defective iron metabolism: Microcytic hypo chromic
anemia with high serum iron have been reported in
Betel nut: The term areca nut is used to denote the
submucous fibrosis but as such, there is no definite proof
unhusked whole fruit of the areca nut tree and term betel
available to support this cause effect relation.
nut is used exclusively to refer to the inner karnel or
seed which is obtained after removing husk. The betel Bacterial infections: Streptococcal toxicity is also a factor
nut has psychotropic and antihelmintic property due to in etiology of oral submucous fibrosis, as in some other
presence of areca alkaloids, predominantly arecoline. collagen disorder such as rheumatic disease. Klebsiella
These alkaloids have powerful parasympathetic properties rhinoscleromatis may be a factor in cause of submucous
which produce euphoria and counteract fatigue. Areca fibrosis.
nut is found to contain different types of alkaloids like Collagen disorders: Oral submucous fibrosis is thought to
arecoline, arecadine, arecalidine, guvacoline, guvacine be localized collagen disease of oral cavity. It is linked to
and isoguvacine. In a habitual betel nut chewer, oral scleroderma,rheumatoid arthritis, Duputreyen’s contracture
submucous fibrosis may be caused by the amount of and intestinal fibrosis. A link between scleroderma and oral
tannic acid contained in the betel nut, the influence of submucous fibrosis has also been suspected on the basis of
mixed calcium powder and the conditional action of similarity of histological characteristics.
arecoline content in betel nut, affecting the vascular Immunological disorders: Raised ESR and globulin
supply of oral mucosa and causing neurotropic disorder. levels are indicative of immunodeficiency disorder. Serum
Nitrosation of arecoline leads to the formation of areca immunoglobulin levels of IgA, IgG and IgM are raised
nut specific nitrosamine namely nitrosoguvacoline, significantly in oral submucous fibrosis. These raised
nitrosoguvacine and 3-methyl nitrosomino pripionitrile, levels suggest an antigenic stimulus in the absence of any
which they alkylate DNA. Metabolism of this areca nut infection. Circulating auto antibodies are also present in
specific nitrosamine will lead to formation of cyanoethyl, some cases of oral submucous fibrosis.
Genetic susceptibility: The familial occurrence of oral Another component of betel nut that aids this cross-
submucous fibrosis has also been reported. linking is copper. Copper is present in betel nut in high
amounts. It is a constituent of enzyme lysyl oxidase. This
Altered salivary composition: The study of saliva in 245
enzyme causes cross-linking and makes collagen resistant
cases of oral submucous fibrosis has shown increased
to degradation.
pH, increase in salivary amylase, low levels of calcium,
increase in alkaline phosphatase and potassium and normal Decreased Collagen Breakdown
levels of salivary immunoglobulin. The fibrin precipitating
factor in saliva has been attributed to the increased plasma Due to action of tannin and copper collagen that is produced
fibrinogen. This is likely due to increased dietary content in OSMF is highly resistant to remodeling and phagocytes.
of fibrin. It is fibroblasts that bring about remodeling and phagocytes
of collagen. As in OSMF these fibroblasts are affected they
Pathogenesis cannot degrade collagen.
Thus in oral submucous fibrosis there is increased
• Increased collagen production
production and decreased degradation of collagen.
• Stabilization of collagen
This leads to accumulation of collagen in oral mucosa.
• Decreased collagen breakdown.
Pathogenesis of oral submucous fibrosis is shown in Flow
chart 12.2.
Pathogenesis
Oral submucous fibrosis results from increased production Clinical Features
of collagen by fibroblasts. In addition to this there is Age and sex distribution: It affects both sexes. The age
decreased breakdown leading to accumulation of excessive group varies, although majority of patients are between 20
amounts of collagen. and 40 years of age.
There are various mechanisms by which this occurs:
∙ Increased collagen production Location: The most frequent location of oral submucous
∙ Stabilization of collagen fibrosis is the buccal mucosa and the retro molar areas. It
∙ Decreased collagen breakdown. also commonly involves soft palate, palatal fauces, uvula,
tongue and labial mucosa. Sometimes, it involves the floor
Increased Collagen Production of mouth and gingiva.
Under the influence of areca nut fibroblasts differentiated Onset: The onset of the condition is insidious and is often
into phenotypes that produce more collagen. The alkaloids of 2 to 5 years of duration.
present in areca nut arecadine and arecoline are responsible
Symptoms: The most common initial symptom is burning
for this. Arecadine is more important. Arecoline gets
sensation of oral mucosa (stomatopyrisis), aggravated by
converted in arecadine which is the active metabolite.
spicy food, followed by either hyper salivation or dryness
There is dose dependent increase in production of collagen
of mouth. Vesiculation, ulceration, pigmentation, recurrent
by fibroblasts under influence these factors.
stomatitis and defective gustatory sensation have also been
Various cytokines are increased in oral sub mucous
indicated as early symptoms. Referred pain in the ears and
fibrosis. These are TGF-β, PDGF, bFGF. These are
deafness, due to occlusion of Eustachian tube and a typical
fibrogenic growth factors that stimulate collagen
nasal voice has been reported.
production. Another cytokine that has anti-collagen effect
is IFN-a. This is decreased in OSMF. Thus overall there is Signs (Fig. 12.25): Gradual stiffening of the oral mucosa
stimulation of collagen synthesis. occurs in few years after the initial symptoms appear.
This leads to inability to open the mouth. Later on patients
Stabilization of Collagen Structure experience difficulty in protruding the tongue. When the
Betel nut contains tannin. Tannin has ability to stabilize fibrosis extends to pharynx and esophagus, the patient may
collagen by cross-linking it. This cross-linked collagen is experience difficulty in swallowing the food. The most
more resistant to degradation. common and earliest sign is blanching of mucosa, caused
Flow chart 12.2 Pathogenesis of oral submucous fibrosis
246
Table 12.5 Changes in oral submucous fibrosis

Early changes
• Burning sensation excerbated by spicy food
• Vesiculation
• Blanching of mucosa
• Leathery mucosa
Late changes
• Fibrous bands within mucosa
• Limitation of mouth opening
• Narrowing of oropharyngeal orifice with distortion of uvula
• Woody changes to mucosa and tongue.
Affected Sites and Their Signs

Figure 12.25 Oral submucous fibrosis showing fibrous band Lips (36%): Mucosa is blanched, becomes rubbery and is
and decreased mouth opening characterized by the presence of circular bands around the
rima oris like a thin band. In severe labial involvement, the
opening of mouth is altered to an elliptical shape (elliptical
by impairment of local vascularity. The blanched mucosa rima oris), lips become leathery and it become difficult to
becomes slightly opaque and white. The whitening often evert them.
takes place in spots so that the mucosa acquires a marble
Buccal mucosa (98%): The affected mucosa becomes
like appearance. Blanching may be localized or diffuse,
coarse, blanched and inelastic. In advanced cases, the
involving greater part of the oral mucosa or reticular, in
mucosa becomes tough and leathery with numerous
which blanching consists of blanched area with intervening
vertical fibrous bands.
clinically normal mucosa, giving it a lace-like appearance.
As disease progresses the mucosa becomes stiff and vertical Soft palate (49%) and uvula: Involvement of soft palate
fibrous bands appear there (Table 12.5). is marked by fibrotic changes and a clear delineation of the
soft palate from hard palate. The mobility of soft palate

is restricted. Uvula, when involved, is shrunken and in
extreme cases, it becomes bud-like.
247
Palatal fauces: In the soft palate the bands radiate from
pterygomandibular raphe to the anterior faucial pillars. The
faucial pillars become thick and short and tonsils may get
pressed in between fibrosed pillars.
Tongue (37%): The initial change is depapillation, usually
in the lateral margins. Tongue becomes smooth; its mobility,
especially in protrusion, becomes impaired. Patient cannot
protrude the tongue beyond the incisal edges.
Floor of mouth (29%): When floor of mouth is affected,
it becomes inelastic.
Figure 12.26 Submucous fibrosis showing atrophic epithelium
Gingiva: When affected, it becomes fibrotic, blanched and (Courtesy: Dr Sangmesh Halawar, Reader, Department of Oral
inelastic. Pathology, CDCRI, Rajnandgao, Chhattisgarh, India)
Associated Features
Pigmentation: Hyperpigmentation or occasional loss
of pigmentation is very common in association with oral
submucous fibrosis. Many a times pigmentation changes
in vermilion border are so striking that this disease can be
suspected even before examining the patient.
Vesicle (32%): It is usually found in areas of redness in
the soft palate, the anterior faucial pillar, buccal mucosa or
the mucosal surface of lip, particularly the lower lip. The
vesicles are painful and they soon rupture leaving behind
superficial ulceration. Often there is history of vesiculation
following the intake of spicy food, suggesting an allergic
reaction to spicy food.
Ulceration (43%): Ulceration often develops in the course
of disease, particularly in advanced cases. In advanced
Figure 12.27 Oral submucous fibrosis Oral submucous
cases, epithelium becomes atrophic, which render it fragile
fibrosis showing (1) abundant deposit of collagen fibers; (2)
and vulnerable to ulceration.
flattening of rete pegs; (3) juxtaepithelial inflammatory cells
Petechiae: These are small raised reddish blue spots which
sometimes occur in oral submucous fibrosis. It may be few
or many. They occur most commonly on tongue and the atypia (7%). Sometimes, the atrophic epithelium is
labial and buccal mucosa. The petechiae are transient in associated with hyperorthokeratosis and pyknotic changes
nature and do not require any specific treatment. in the nuclei of basal cell layer. There is liquefaction
degeneration of the basal layer of cells. Rete pegs are
Histopathological Features completely lost.
(Figs 12.26 to 12.28) Connective tissue: It shows vesicles, which are caused
Epithelium: In most of the cases, the oral epithelium by subepithelial accumulation of fluid. The inflammatory
is markedly atrophic. The atrophic epithelium exhibits cells are mostly mononuclear; eosinophils and occasional
intercellular edema (18%), signet cells (13%) and epithelial plasma cell may be seen.
the juxtaepithelial basement membrane. Occasionally,

thickened collagen bundles are still seen separated
by slight residual edema. The fibroblastic response
248 is less marked; the cells present being mostly adult
fibrocytes with elongated, spindle-shaped nuclei and
scanty cytoplasm. Blood vessels are either normal
or constricted, as a result of increased surrounding
fibrous tissue. The inflammatory exudate consists of
lymphocytes and plasma cells, although occasional
eosinophils may be present.
4. Advanced stage: Collagen is completely hyalinized
and is seen as a smooth sheath with no separate
bundles. Edema is absent. The hyalinized area is devoid
of fibroblasts, although a thin elongated cell or vestigial
Figure 12.28 Oral submucous fibrosis (Courtesy: Dr Aparna nucleus is seen in a rare interval along the fiber bundle.
Thombre, Reader, Department of Oral Pathology, VSPM Dental Blood vessels are completely obliterated or narrowed.
College and Hospital, Nagpur, Maharashtra, India) The inflammatory cells are lymphocytes and plasma
cells.
Epithelial Atypia in Oral Submucous Fibrosis Staging of Oral Submucous Fibrosis

The features most often associated are irregular epithelial There are various schemes for staging of oral submucous
stratification, increased number of mitotic figures, nuclear fibrosis. These schemes help clinicians in determining
pleomorphism, hyperchromatism and loss of polarity of the severity of the disease and to modify the treatment
cell. Another striking feature is spongiosis, especially in accordingly (Haider et al).
basal cell layer. A considerable number of signet cells are
also seen, mostly located in basal layer. Marked reduction Clinical Staging (Table 12.6)
in melanin pigmentation in basal cell layer. It is based on bands seen in the oral cavity.
Grading of Oral Submucous Fibrosis Functional Staging (Table 12.7)
Pindborg has described the connective tissue changes in Functional staging is based on amount of mouth opening.
four consecutive stages as follows It is measured between the central incisors of maxilla and
1. Very early stage: It is characterized by finely fibrillar mandible.
collagen dispersed with marked edema. The fibroblastic
response is strong with plump young cells containing
abundant cytoplasm. The blood vessels are sometimes Table 12.6 Clinical staging of oral submucous fibrosis
normal, but more often they are dilated and congested. Clinical stage Clinical features
The inflammatory cells mainly polymorphs with 1 Faucial bands only
occasional eosinophils are present. 2 Faucial and buccal bands
2. Early stage: The juxtaepithelial area shows early
3 Faucial, buccal, and labial bands
hyalinization. The collagen is still seen as separated
bundles which are thickened. Plump young fibroblasts
are present in moderate numbers. The blood vessels are Table 12.7 Functional staging of oral submucous fibrosis
often dilated and congested. The inflammatory cells
Functional staging Amount of mouth opening
are mostly mononuclear lymphocytes eosinophils and
A 20 mm
occasional plasma cell.
3. Moderately advanced stage: The collagen is mode- B 11–19 mm
rately hyalinized and amorphous changes start from C 10 mm
Malignant Potential Steroids: Hydrocortisone injection along with procaine

hydrochloride injection locally in the area of fibrosis.
Oral cancer originates in submucous fibrosis from diverse
Injections are given fortnightly. The early cases show good
intraoral location, without any noticeable predilection 249
improvement with this therapy. Systemic steroid can also
for any specific site. Atrophic epithelium first becomes
be given in severe cases.
hyperkeratotic and later, intracellular edema and basal cell
hyperplasia develop eventually, following epithelial atypia Placental extract: Injection of placentrix can also be
with moderate epithelial hyperplasia and then, carcinoma given. Hyaluronidase can be given.
can develop at any time. To substantiate the precancerous
Oral physiotherapy: Oral exercises are advised in early
nature of the condition, following points are noted:
and moderately advanced cases. This includes mouth
∙ High occurrence of submucous fibrosis in oral cancer opening and ballooning of mouth. This is thought to put
patient. pressure on fibrous bands. Forceful mouth opening have
∙ Higher incidence of oral cancer in patient with oral been tried with mouth gag and acrylic surgical screw.
submucous fibrosis.
∙ Histological diagnosis of carcinoma, without the Points to Remember
clinical suspicion of it. Burning sensation of oral mucosa, referred pain in the
∙ Higher prevalence of leukoplakia among oral ears, inability to open the mouth, difficulty in protruding
submucous fibrosis patients. the tongue, difficulty in swallowing the food, blanching
∙ Higher frequency of epithelial dysplasia. of mucosa, bud-like uvula, pigmentation, vesicle,
The WHO Collaborating Center for Oral Precancerous ulceration, petechiae, oral epithelium is markedly
Lesions has concluded that although oral submucous atrophic, connective tissue shows vesicles, inflammatory
fibrosis predisposes to cancer, it is not absolutely cells are mostly mononuclear, eosinophils, epithelial
conclusive. It is highly probable that such relationship does atypia, pleomorphism, hyperchromatism, loss of polarity
exist. Following facts support this hypothesis: of cell, restriction of habit/behavioral therapy, vitamin
The frequency of oral leukoplakia in oral submucous rich diet, hydrocortisone injection, placental extract, oral
fibrosis patient is 6 to 8 times higher than in control group. physiotherapy.
Carcinoma patients exhibiting oral submucous fibrosis
have a frequency with exceed the 1.2 percent of submucous
fibrosis in general population.
DYSKERATOSIS CONGENITA
Immunological alternations observed in oral submucous It is also called Zinssner-Engman-Cole syndrome. It is a
fibrosis are almost similar to that observed in oral cancer. well recognized but rare genokeratosis, which is probably
inherited as X linked recessive characteristic.
Management Disease manifested has three typical signs: oral
leukoplakia, dystrophy of nails and pigmentation of skin.
Restriction of habit/behavioral therapy: The preventive
measure should be in the form of stoppage of habit, which Clinical Features
can be encouraged through public education. Affected
Age and sex distributions are almost exclusively seen in
patients should be explained about the disease and its
males during first 10 years of life.
possible malignant potential. Improvement in clinical
Nail changes are the first manifestation, becoming
features like gradual increase in inter-incisal opening has
dystrophic and shedding some time after the age of 5
been observed in most of the patients who discontinue the
years.
habit.
Grayish brown pigmentations appear in some time
Medicinal therapy: A vitamin rich diet along with which is seen usually on trunk, neck and thigh.
iron preparation is helpful to some extent but has little The skin may become atrophic, telangiectatic and face
therapeutic value in relieving trismus. Iodine-B-complex appears red.
preparation (Injection Ranodine) is a combination of iodine Other minor manifestations are frail skeleton, mental
preparation with synthetic vitamin B complex. retardation, small sella turcica, dysphagia, transparent
tympanic membrane, deafness, epiphora, eyelid infection, Management

urethral anomalies, small testes and hyperhydrosis of the
Symptomatic management of oral mucosal lesion should
palms and soles.
250 be done.
Development of aplastic anemia and thrombocytopenia
occur usually in second decade of life. Points to Remember
Zinssner-Engman-Cole syndrome, leukoplakia, dystrophy
Oral Manifestations
of nails, pigmentation of skin, grayish brown pigmen-
Age: The oral lesions have onset between the age of 5 to tations on trunk, mental retardation, development of
14 years. aplastic anemia, tongue diffusely distributed vesicles and
ulcerations, repeated recurrent ulceration, development
Location: Most common sites are tongue and buccal
of erythroplasia, increased number of melanin containing
mucosa.
chromatophores.
Appearance: It appears as diffusely distributed vesicles
and ulcerations, followed by accumulation of white
patches of necrotic epithelium (Fig. 12.29) and sometimes, LUPUS ERYTHEMATOSUS
superimposed monolial infection. It is characterized by presence of abnormal antibodies and
After sometime, in the age group of 14 to 20 years, immune complex.
there are repeated recurrent ulceration and development of
erythroplasia or red mucosal lesion. Types
Finally between the age of 20 and 30 years, there is • S ystemic lupus erythematosus: If multiorgan involve-
development of erosive leukoplakia and carcinoma. ment occurs.
• Discoid lupus erythematosus: It is also called chronic
Histopathological Features cutaneous lupus erythematosus. If it is confined to
The skin lesion shows increased number of melanin skin and mucosa.
containing chromatophores and increased vascularity. • Subacute cutaneous erythematosus: It present
Depending upon the stage of disease epithelium may show features of systemic as well as discoid type.
dysplasia.
Etiology
Genetic predisposition: Relative of patients have higher
incidences autoantibodies, immune deficiency and connec-
tive tissue disease. This tendency is greatest among
identical twins
Immunological abnormality possibly mediated by viral
infection: Immune complex consisting chiefly of nucleic acid
and antibody account for majority of the tissue change seen.
Autoimmune disease: As these patients develop antibodies
to many of their own cells.
Deposition of antigen: Antibody complexes.
Endocrine (high incidence in females in pregnancy):
Finding of increased estrogen level.
Biochemical increase in excretion of metabolic
products, particularly tyrosine and phenylalanine, in certain
Figure 12.29 Dyskeratosis congenita lesion on tongue SLE patient.
Discoid Lupus Erythematosus Symptoms: It is manifested by symptoms of fever and

pain in the muscle and joints. It may present as itching or
Clinical Features
burning sensation as well as area of hyperpigmentation.
Age and sex distribution: It occurs in 3rd and 4th decades, Severely intensifies after exposure to sunlight. 251
female predilection in the ratio of 5:1.
Cutaneous lesion: The cutaneous lesion consists of
Location: Most common sites are face, oral mucosa, chest, erythematosus patches on the face, which coalesce to form
back and extremities. roughly symmetrical pattern over the cheeks and across
Appearance: It is a circumscribed, slightly elevated, white the bridge of the nose, in a so called butterfly distribution.
patch that may be surrounded by red telangiectatic halo. Skin lesions are widespread, bilateral with signs of acute
inflammation. This finding helps to differentiate between
Cutaneous lesions: These are slightly elevated, red or skin lesions of DLE and SLE.
purple macules; that are often covered by gray or yellow
adherent scales. Other manifestation: There is involvement of various
organs including kidneys and heart. In kidney, fibrinoid
Carpet track extension: Forceful removal of scale results thickening of glomerular capillaries produce the
in ‘carpet track extension’, which has dipped into enlarged characteristic ‘wire loop’ which may be sufficient to result
pilosebaceous canals. in renal insufficiency.
The lesion increases in size by peripheral growth. Heart may suffer a Libman-Sacks endocarditis involving
Periphery of the lesion appears pink or red, while the center valves as well as fibrinoid degeneration of epicardium and
exhibits an atrophic scarred appearance. myocardium.
Butterfly distribution on macular region and across the
bridge of the nose is present. Oral Manifestation
Oral Manifestations Location: The most common sites are buccal mucosa,
Location: Most common sites are buccal mucosa, tongue, lip and palate. Patient complains of burning sensation,
palate and vermilion border of lip. xerostomia or soreness of mouth.
Appearance: It begins as erythematous area, sometimes Appearance: The intraoral lesion is composed of a central
slightly elevated, but more often depressed, usually with depressed red atrophic area surrounded by 2 to 4 mm
induration and typically with white spots. elevated keratotic zone that dissolves into small white lines
(Fig. 12.30).
Signs: Occasionally, superficial painful ulceration may occur
with crusting or bleeding, but no actual scale formation.
Symptoms: There may be burning and tenderness which
may be intermittent or disappear if the lesion becomes
inactive.
The margins of the lesion are not sharply demarcated. Fine
white striae radiate out from the margins. Central healing
may result in depression. Erythematous, atrophic plaque,
surrounded by keratotic border may involve the entire lip.
Systemic Lupus Erythematosus

Clinical Features
Age and sex distribution: It occurs in 3rd (female) and
4th (male) decades and has female predilection (8:1).
Location: It is characterized by repeated remissions and
exacerbations with common sites being face, neck, upper Figure 12.30 Lesion seen on palate of patient with systemic
arm, shoulders and fingers. lupus erythematosus
Lesions similar to DLE, except that hyperemia, edema

and extension of lesion is more pronounced.
252 Signs: There is greater tendency to bleed and petechiae,

suspected ulcerations surrounded by red halo.
Lupus cheilitis: The lip lesions appear with central atrophic
area with small white dots surrounded by keratinized
border, which is composed of small radiating white striae.
There is occasional ulceration of central area.
Laboratory Findings
LE cell inclusion phenomenon with surrounding pale
nuclear mass apparently devoid of lymphocytes is present.
It is characterized by presence of abnormal serum
antibodies and immune complexes. Figure 12.31 (1) The hair follicles cut in cross section; (2)
There is also anemia, leukopenia and thrombocytope- Degeneration of basal cell layer; (3) Dense aggregate of
chronic inflammatory cells; (4) Keratin plugging. (Courtesy: Dr
nia, with sedimentation rate increased.
Sangamesh Halawar, Reader, Department of Oral Pathology,
Serum gamma globulin increased and Coomb’s test is VPDC and H Kavalapur Sangli, Maharashtra, India)
positive.
(Figs 12.31 and 12.32)
Hyperorthokeratinization, hyperparakeratinization with
keratotic plugging, atrophy of the rete pegs and liquefac-
tion degeneration of basal layer is preset.
There is perivascular infiltration of lymphocytes and
their collection about dermal appendages, basophilic
degeneration of collagen and elastic fibers with hyali-
nization, edema and fibrinoid change.
Skin lesions show hyperkeratosis, with keratin packed
into the openings of the hair follicles, called follicular
plugging. Degeneration of the basal cell layers is seen
common in both skin and oral lesions. The underlying
connective tissue stroma shows patchy to dense aggregates Figure 12.32 SLE with keratin plugging (KP)
of chronic inflammatory infiltrate.
Differential diagnosis with lichen planus: LE shows Management
perivascular infiltrate, PAS positive material in the It is treated by systemic corticosteroids therapy and should
basement membrane zone and subepithelial edema which be managed by physician.
may form vesicle. Anti-malarial drugs can be used in some cases.
and oropharynx; natural history—cellular and molecular

Points to Remember
markers of risk. Eur J Cancer Prev. 1993;2:31-51.
• D iscoid lupus erythematosus: Circumscribed, slightly 9. Meghji S, Warnakulasuriya S. Oral submucous fibrosis: an
elevated, white patch red telangiectatic halo, elevated expert symposium. Oral Dis. 1997;3:276-91. 253
cutaneous lesions, carpet track extension, Butterfly 10. Miller AB, Hoogstraten B, Staquet M, Winkler A. Reporting
distribution of macular region, erythematous area on results of cancer treatment. Cancer. 1981;47:207-14.
tongue, fine white striae radiate out from the margins. 11. Pindborg JJ, Reichart PA, Smith CJ, van der Waal I. World
• Systemic lupus erythematosus: Fever and pain in Health Organization International Histological Classification
muscle and joints, cutaneous lesion consists of of Tumours. Histological typing of cancer and precancer of
the oral mucosa. Berlin: Springer; 1997.
erythematosus patches, wire loop, Libman-Sacks
12. Reibel J. Prognosis of oral pre-malignant lesions: signifi-
endocarditis, central depressed red atrophic area
cance of clinical, histopathological and molecular biological
surrounded by 2 to 4 mm elevated keratotic zone in characteristics. Crit Rev Oral Biol Med. 2003;14:47-62.
palate, lupus cheilitis 13. Reichart PA, Philipsen HP. Oral erythroplakia – a review.
• Histopathological: LE cell inclusion phenomenon, Oral Oncol. 2005;41:551-61.
hyperorthokeratinization, hyperparakeratinization 14. Silverman S. Oral lichen planus: a potentially premalignant
with keratotic plugging, perivascular infiltration of lesion. J Oral Maxillofac Surg. 2000;58:1286-8.
lymphocytes, follicular plugging. 15. Thompson PJ. Field change and oral cancer: new evidence
for widespread carcinogenesis? Int J Oral Maxillofac Surg.
2002;31:262-6.
BIBLIOGRAPHY 16. Thornhill MH, Sankar V, Xu XJ, et al. The role of
1. Axe, ll T, Holmstrup P, Kramer IRH, Pindborg JJ, Shear histopathological characteristics in distinguishing amalgam-
M. International seminar on oral leukoplakia and associated associated oral lichenoid reactions and oral lichen planus. J
lesions related to tobacco habits. Community Dent Oral Oral Pathol Med. 2006;35:233-40.
Epidemiol. 1984;12:145-54. 17. van der Meij EH, Schepman K-P, van der Wall I. The
2. Axe’ ll T, Pindborg JJ, Smith CJ, van der Waal I and an possible malignant character of oral lichen planus and oral
International Collaborative Group on Oral White Lesions. lichenoid lesions: a prospective study. Oral Surg Oral Med
Oral white lesions with special reference to precancerous Oral Pathol. 2003;96:164-71.
and tobacco-related lesions: conclusions of an international 18. Waal van der I, Axe, ll T. Oral leukoplakia: a proposal for
symposium held in Uppsala, Sweden, May 18–21, 1994. J uniform reporting. Oral Oncol. 2002;38:521-6.
Oral Pathol Med. 1996;25:49-54. 19. Warnakulasuriya S. Histological grading of oral epithelial
3. Bremmer JF, Braakhuis BJM, Ruijter-Schippers HJ, et dysplasia: revisited. J Pathol. 200;194:294-7.
al. A non invasive genetic screening test to detect oral 20. World Health Organization. Report of a meeting of
preneoplastic lesions. Lab Invest. 2005;85:1481-8. investigators on the histological definition of precancerous
4. Epstein JB, Wan LS, Gorsky M, Zhang L. Oral lichen lesions. Geneva: World Health Organization; 1973, Can 731.
planus: progress in understanding its malignant potential 21. World Health Organization. World Health Organization
and implications for clinical management. Oral Surg Oral Classification of Tumours. In: Barnes L, Eveson JW,
Med Oral Pathol. 2003;96:32-7. Reichart P, Sidransky D (Eds). Pathology and Genetics.
5. Gupta PC, Mehta FS, Daftary DK, et al. Incidence rates of Head and Neck Tumours. Lyon: International Agency for
oral cancer and natural history of oral precancerous lesions Research on Cancer (IARC) IARC Press, 2005.pp.177-9.
in a 10-year follow-up study of Indian villagers. Community 22. World Health Organization Collaborating Centre for Oral
Dent Oral Epidemiol. 1980;8:287-333. Precancerous lesions. Definition of leukoplakia and related
6. Handley TP, McCaul JA, Ogden GR. Dyskeratosis lesions: an aid to studies on oral precancer. Oral Surg Oral
congenita. Oral Oncol. 2006;42:331-6. Med Oral Pathol. 1978;46:518-39.
7. Hansen LS, Olson JA, Silverman S. Proliferative verrucous 23. Zain RB, Ikeda N, Gupta PC, et al. Oral mucosal lesions
leukoplakia. A longterm study of thirty patients. Oral Surg associated with betel quid, areca nut and tobacco chewing
Oral Med Oral Pathol. 1985;60:285-9. habits: consensus from a workshop held in Kuala Lumpur,
8. Johnson NW, Ranasinghe AW, Warnakulasuriya KAAS. Malaysia, November 25–27, 1996. J Oral Pathol Med.
Potentially malignant lesions and conditions of the mouth 1999;28:1-4.
254 1. Following are the examples of precancerous lesion 6. Syndrome associated with lichen planus is:
except: a. Hanhart’s syndrome
a. OSMF b. Leukoplakia b. Graham Little syndrome
c. Erythroplakia d. Cheilitis c. Grinspan syndrome
2. Following are the examples of precancerous condition d. Both b and c
except: 7. Wickham’s striae is characteristic feature of:
a. OSMF b. Lupus erythematosus a. Leukoplakia b. Lichen planus
c. Oral lichen planus d. Carcinoma in situ c. Erythroplakia d. Both a and b
3. Mother of pearl appearance seen in:
8. Most common type of oral lichen planus is:
a. Leukoplakia b. OSMF
a. Reticular type b. Bullous type
c. Leukoedema d. Bowen’s disease
c. Plaque type d. Atrophic form
4. Leukoplakia seen amongst clay pipe smokers and betel
quid chewers are generally: 9. Civatte bodies are seen in:
a. Nodular type b. Verrcous type a. Bowen’s disease b. Leukoplakia
c. Homogeneous type d. Ulcerated type c. Lichen planus d. OSMF
5. ‘Oral florid papillomatosis’ is the extensive lesions of: 10. The most common and earliest sign seen in OSMF:
a. Homogeneous leukoplakia a. Blanching of mucosa
b. Nodular leukoplakia b. Wickham’s striae
c. Verrucous leukoplakia c. Bleeding of mucosa
d. None d. None
13 Malignant Tumors
Anil Govindrao Ghom, Shubhangi Mhaske (Jedhe), Ashok Mhaske
Chapter Outline
ÂÂ Classification ÂÂ Nasopharyngeal carcinoma

ÂÂ Etiology and risk factors for oral cancer ÂÂ Merkel cell carcinoma
ÂÂ Oral squamous cell carcinoma ÂÂ Fibrosarcoma
• Carcinoma of floor of mouth ÂÂ Malignant fibrous histiocytoma
• Carcinoma of alveolar ridge ÂÂ Synovial sarcoma
• Carcinoma of buccal mucosa ÂÂ Liposarcoma
• Carcinoma of labial mucosa ÂÂ Chondrosarcoma
• Carcinoma of palate ÂÂ Mesenchymal chondrosarcoma
• Carcinoma of maxillary sinus ÂÂ Osteosarcoma
• Multiple carcinomas ÂÂ Ewing’s sarcoma
ÂÂ Metastatic carcinoma ÂÂ Malignant hemangioendothelioma
ÂÂ Basal cell carcinoma ÂÂ Malignant hemangiopericytoma
ÂÂ Adenosquamous carcinoma ÂÂ Neuroblastoma
ÂÂ Basaloid squamous carcinoma ÂÂ Angiosarcoma
ÂÂ Sinonasal undifferentiated carcinoma ÂÂ Olfactory neuroblastoma
ÂÂ Verrucous carcinoma ÂÂ Neurofibrosarcoma
ÂÂ Transitional cell carcinoma ÂÂ Leiomyosarcoma
ÂÂ Malignant melanoma ÂÂ Rhabdomyosarcoma
ÂÂ Spindle cell carcinoma ÂÂ Malignant granular cell myoblastoma
ÂÂ Adenoid squamous cell carcinoma ÂÂ Alveolar soft part sarcoma
INTRODUCTION Cancer is Latinized from Greek word Karkinos,

meaning crab denoting how carcinoma extends its claws
Oral cancer although prevalent worldwide, but is very like a crab into the adjacent tissues.
common in some countries such as India, Pakistan and Oral cancer can be divided into three categories:
Taiwan, and in some areas of France. Oral cancer most carcinomas of the oral cavity proper, carcinomas of the
commonly occurs in middle-aged and older individuals, lip vermilion, and carcinomas arising in the oropharynx,
although number cases of these malignancies are also from an epidemiological and clinicopathological pers
being reported in younger adults in recent years. pective.
Common Sign and Symptoms Table 13.1 WHO classification of malignant

tumor of oral cavity
• Sore in the mouth that bleeds easily and does not heal
256 • Pain in the mouth that does not go away Malignant epithelial tumor
• A lump or thickening in the cheek Squamous cell carcinoma
• A white or red patch on the gums, tongue, tonsil, or • Verrucous carcinoma
lining of the mouth • Basaloid squamous cell carcinoma
• A sore throat or a feeling that something is caught in • Papillary squamous cell carcinoma
the throat • Spindle cell carcinoma
• Acantholytic squamous cell carcinoma
• Difficulties in chewing, swallowing, or moving the
• Adenosquamous carcinoma
tongue or jaw.
• Carcinoma cuniculatum
Lymphoepithelial carcinoma
Etiology of Oral Cancer Epithelial precursor lesions
• Actinic radiation Soft tissue tumors
• Familial and genetic Kaposi sarcoma
• Atmospheric pollution Lymphangioma
• Orodental factors Ectomesenchymal chondromyxoid tumor
• Immunity Focal oral mucinosis
• Smokeless tobacco Congenital granular cell epulis
• Smoking Hematolymphoid tumors
• Alcohol Diffuse large B-cell lymphoma (DLBCL)
• Syphilis Mantle cell lymphoma
• Diet deficiency and deficiency status Follicular lymphoma
• Ionizing radiation Extranodal marginal zone B-cell lymphoma of MALT type
• Trauma Burkitt lymphoma
• Virus T-cell lymphoma (including anaplastic large cell lymphoma)
• Intraoral lesions like chronic ulceration and fissure, Extramedullary plasmacytoma
lichen planus, candidosis, leukoplakia, median Langerhans cell histiocytosis
rhomboid glossitis, Plummer-Vinson syndrome, Extramedullary myeloid sarcoma
Follicular dendritic cell sarcoma/tumor
submucus fibrosis, oral melanosis, discoid lupus
erythematosus and epidermolysis bullosa. Mucosal malignant melanoma
Secondary tumors
CLASSIFICATION Familial and genetic: There is little evidence that there

It is discussed in Table 13.1. is familial and genetic predisposition for the development
of oral cancer. Lip cancer is amongst the sites which show
strongest cancer clustering within families. But it also
ETIOLOGY AND RISK FACTORS FOR
reflects the fact that families tend to have same occupation,
ORAL CANCER i.e. fishing and farming, which is related with ultraviolet
The etiology of SCC of the oral cavity has been studied exposure. Oral cancers are more prevalent in black as
comprehensively last few decades. Various risk factors compared to white.
have been proposed as etiologic agents for the development Atmospheric pollution: Parts of the urban/rural difference
of OSCC. in incidence of head and neck cancers have been related
Actinic radiation: It is a minor etiologic factor in cases to atmospheric pollution. Sulphur dioxide and smoke
of lip cancer. Lip cancer occurs more commonly in fair concentration in the atmosphere are positively correlated
skinned people who are generally engaged in outdoor with squamous cancer of larynx and pharynx. The impact on
occupation, such as farming and fishing. cancer of the mouth is likely to be less, but merits careful
Malignant Tumors
study. Blue collar workers exposed to dust or inhalation of Alcohol: All forms of alcohol; including hard liquor, wine and
organic and inorganic agents are at increased risk of cancers beer have been implicated in the etiology of oral cancer. The
of mouth. mechanism by which alcohol affects includes the dehydrating
effect of alcohol on the mucosa which increases mucosal 257
Orodental factors: It is more prevalent in patients with
permeability and the effects of carcinogens on the mucosa.
poor oral hygiene, faulty restorations, sharp teeth, ill fitting
Beverage congeners include nitrosamines and impurities
dentures and those with syphilitic glossitis.
which can act as carcinogens. Most heavy alcohol consumer
Immunity: The increasing incidence of oral cancers is also uses tobacco so it is difficult to separate the ill effects
clearly age-related, which may reflect declining immune individually.
surveillance with age. It may occur in immuno-suppressed
Syphilis: It is traditionally associated with oral cancer.
patients following organ and bone marrow transplantation.
HIV/AIDS patient are at increased risk of oral cancer. Diet deficiency and deficiency status: Nutritional
deficiency and liver dysfunction can also play a role in it.
Smokeless tobacco: Taken together; the effect of tobacco
The relationship between sideropenic dysphagia and oral
use, heavy alcohol consumption and poor diet can probably
cancer is well-established.
explain over 90 percent of cases of oral cancer. Much of
tobacco in the world is consumed without combustion, by Ionizing radiation: Carcinoma of buccal mucosa may
being placed into contact with mucous membrane, through occur as a complication of long-term radiotherapy.
which nicotine is absorbed. It contains potent carcinogens
Trauma: Trauma in combination with other factors like
like nitrosamine, polycyclic hydrocarbons and polonium
chronic cheek biting, denture use and irregular teeth may
and metabolites of these constituents, which have been the
act as a co-carcinogen and may promote transformation of
suggested etiologic factors in oral cancer (Table 13.2).
epithelial cells.
Smoking: Tobacco smoke contains carbon monoxide. It is
Phenolic agents: There is increase risk for workers who
an important factor in the development of oral cancer. Study are working in wood products industry which are exposed
shows that cigar and pipe smoking increase the risk of cancer to chemical such as phenoxyacetic acids.
than cigarette smoking. It has been stated that the pooling
of carcinogens in saliva gives rise to cancer in the floor of Virus: The possibility of type I herpes simplex virus being
mouth and ventral and lateral tongue. Smoking is strongly associated with oral cancer has been suggested. Other
associated with soft palate cancer than anterior sites. viruses which can lead to oral cancer are human papilloma
virus and HIV virus.
Table 13.2 Forms of oral smokeless tobacco

Intraoral lesions: Some intraoral lesion can be responsible
for the oral malignancy. They are as follows:
Some common forms of oral smokeless tobacco are as – Chronic ulceration and fissure: It can cause oral cancer
follows: particularly of lip.
Pan/paan/betal quid—it contains areca nut, betel leaf, slaked – Lichen planus: Reports of carcinoma supervening on
lime, catechu, condiments with or without tobacco. lichen planus have been made in some cases.
Khaini—it contains tobacco and lime. – Candidiasis: It is possible precursor of carcinoma.
Mishri—it is burned tobacco. Candidal infection is often associated with acanthosis
Zarda—boiled tobacco. and parakeratosis.
Gudakhu—it contains tobacco and molasses. – Leukoplakia: It is common premalignant condition
Mawa—tobacco, lime and areca. seen in oral cavity. Nodular leukoplakia show higher
rates of epithelial dysplasia.
Nass—tobacco, ash, cotton or sesame oil.
– Median rhomboid glossitis: In some cases, it has
Naswar/niswar—tobacco, lime, indigo, cardamom, oil and
followed by cancer.
menthol.
– Plummer-Vinson syndrome: The atrophic changes in
Shammah—tobacco, ash and lime.
the mucous membrane of the upper alimentary tract
Toombak—tobacco and sodium bicarbonate that occur in this syndrome predispose to malignancy.
– Submucous fibrosis: It is precancerous condition occur sulcus, floor of mouth, tongue throat may occur at any
in oral cavity. intraoral site.
– Oral melanosis: It appears to be associated with oral
258 cancers in India. Location
– Discoid lupus erythematosus: A number of cases of SCC can occur either in: (1) the nasal cavity and paranasal
carcinoma of lip developing in the lesion of lupus sinuses, (2) the nasopharynx, (3) the hypopharynx,
erythematosus have been reported. larynx, and trachea, or (4) the oral cavity and oropharynx.
– Epidermolysis bullosa: It is occasionally followed by Commonly involved areas are the posterior/lateral borders
carcinoma. of the tongue and lower lip and less frequently the floor of
mouth, alveolar mucosa, palate and buccal mucosa. It may
RISK FACTORS be solitary and multifocal.
It is discussed in Table 13.3.
The 2005 World Health Organization (WHO) classi
EPITHELIAL TUMORS fication of Head and Neck Tumors (Barnes et al., 2005)
distinguishes different types of SCC:
Oral Squamous Cell Carcinoma • Conventional
Squamous cell carcinoma (SCC) is the most frequent of • Verrucous
oral carcinomas. Majority of oral carcinomas are squamous • Basaloid
cell carcinomas. It represents 90 percent of all malignant • Papillary
tumors occurring in the mouth and jaws. It is defined as • Spindle cell (Sarcomatoid)
“A malignant epithelial neoplasm exhibiting squamous • Acantholytic
differentiation as characterized by the formation of keratin • Adenosquamous
and/or the presence of intercellular bridges”. The oral • Cuniculatum
lesion often invades the jaw. It frequently involves buccal Each variant can arise in any one of the 4 above mentioned
head and neck regions, except for the cuniculatum type
Table 13.3 Risk factors for oral cancers (adapted from which only develops from the oral mucosa.
JCDA April 2008, Vol. 74, No. 3, page no 269-272)
Very strong risk factors (> 10-fold increased risk) Clinical Features
• Increased age Age and sex distribution: It predominately, it occurs in
• Using tobacco and alcohol, especially combined use (risks males with ratio of 2:1, older than 50 years with an average
for heavy smokers and drinkers are increased more than 30- age of approximately 60 years.
fold)
• Using smokeless tobacco, including snuff and chewing Appearance: Clinically, majority of oral cancers are
tobacco characterized by ulceration and indurated margins with
• Chewing betel quid, areca nut and paan certain variations depends upon site of occurrence.
• Being immunologically compromised (e.g. after bone-
Sign and symptoms: Patient may present with awareness
marrow transplantation)
of a mass or lump in the head and neck region or complain
Strong risk factors (4- to 10-fold increased risk) of long duration, non-healing ulcer in the oral cavity (Figs
• Smoking cigarettes 13.1 and 13.2). Lesion may be asymptomatic or may
• Drinking alcohol become symptomatic due to involvement of vital structure
• Having a human papilloma virus infection, especial type or superadded infection. In certain case paresthesia can be
Moderate risk factors (≤ 4-fold increased risk) evident in patients. Function of organ is impaired.
• Being male The clinical appearance of a carcinomatous ulcer is that
• Smoking pipes and cigars one of irregular shape, induration and raised everted edges.
• Smoking marijuana Usually have broad base and are dome like or nodular.
• Being exposed to environmental tobacco smoke Surface may range from granular to pebbly to deeply
• Having low fruit and vegetable intake creviced. In some cases, surface may be entirely necrotic
Malignant Tumors
tumor to adjacent tissues, i.e. overlying bone suggests

involvement of periosteum and possible spread to bone.
Histopathology Features 259

The histopathological features are graded into three
types depending upon the degree of differentiation of the
neoplastic proliferating cell type:
Well differentiated or highly differentiated squamous
cell carcinoma
Moderately differentiated squamous cell carcinoma
Poorly differentiated squamous cell carcinoma.
Common Features of all Carcinomas

• Epithelial dysplasia
Figure 13.1 Squamous cell carcinoma of mandibular buccal • Keratinization varies with degree of differentiation.
mucosa showing extraoral growth • Local invasion by break in the basement membrane
and invasion and proliferation into the underlying
connective tissue—this is clinically manifested as
fixation and induration of the lesion.
• Metastasis by blood or lymphatic channels
histopathologically seen as invasion of tumor cells
into the capillaries and lymphatic ducts permeation.
• Perinueral invasion—some of the tumor cells may
have a typical invasion pattern along the nerve
sheath.
Well Differentiated Squamous Cell

Carcinoma (Figs 13.3 to 13.5)
It consists of sheets and nest of cells with characteristic
appearing of squamous origin. These cells are generally
large and show distinct cell membrane. They resemble
the cells of squamous epithelium, both structurally and
Figure 13.2 Carcinoma palate spreading on the alveolar
mucosa and vestibule functionally.
The intercellular bridges are prominent. The nuclei are
distinctly dark staining and hyperchromatic due to increase
and have ragged whitish gray appearance. It may be
in condensed chromatin. Increased number of mitotic
completely red or red surface may be sprinkled with white
figures, less as compared to moderately differentiated
necrotic or keratin area. Base and borders are firm to
carcinoma is evident.
palpate and the lesion may get fixed after infiltration into
Other prominent feature includes multiple nucleoli
underlying tissues.
and increased nucleo-cytosplasmic ratio. Most prominent
Lymph nodes: Superficial and deep cervical nodes are feature is presence of individual cell keratinization and
commonly affected. They become enlarged and are firm formation of numerous keratin pearls of varying size. Each
to hard on palpation. The nodes are non-tender unless keratin pearl consists of a central area of keratin surrounded
associated with secondary infection or an inflammatory by whorls of prickle cells.
response. It may be nodular or polypoid and pink to red and Pleomorphism of cells, keratinization, and keratin
have at least one ulcerated patch on their surface. Fixation pearls (Figs 13.6 and 13.7) deep to epithelial surface and
of nodes to adjacent tissues occurs later. Fixation of primary loss of intercellular bridges or cohesiveness may be seen.
260
Figure 13.3 Well differentiated squamous cell carcinoma Figure 13.5 Well differentiated squamous cell carcinoma
(low power) showing keratin pearl
Figure 13.4 Well differentiated squamous cell carcinoma Figure 13.6 Keratin pearls in well differentiated squamous
showing hyperchromatism cell carcinoma
Moderately Differentiated/Less Well The keratinization is absent as the cells cannot in size
Differentiated Squamous Cell Carcinoma and shape. Keratin pearls may not be present function to
(Figs 13.8 to 13.13) the differentiation point of keratin formation.
Numerous epithelial islands of prickle cells with
The tumor cells are less differentiated and have less
peripheral basal cells may be seen.
resemblance to squamous epithelium. The characteristic
shape of an epithelial cell may not be evident.
Poorly Differentiated Squamous
The cell to cell contacts and relation and arrangement
Cell Carcinoma
are altered. The greater number of mitotic figures shows
that the growth rate is more rapid. This may be varied size This is the tumor with proliferating anaplastic cells,
and shape. highly invasive with poor prognosis. The tumor cells bear
Malignant Tumors
261
Figure 13.7 Formation of keratin pearl in concentric rings Figure 13.10 Moderately differentiated squamous cell carcinoma
showing less differentiated epithelial islands in the stroma
Figure 13.8 Moderately differentiated squamous cell Figure 13.11 Moderately differentiated carcinoma with high
carcinoma with greater number of mitotic figures mitosis and epithelial pearl formations
Figure 13.9 Moderately differentiated squamous cell Figure 13.12 Moderately differentiated carcinoma (high
carcinoma showing pleomorphism of cells power)
262
Figure 13.13 Poorly differentiated squamous cell carcinoma Figure 13.14 Radiograph showing destruction of bone in
showing lack of cohesiveness mandible due to malignancy
little resemblance to their cells of origin and often will

present diagnostic difficulties because of the primitive and
uncharacteristic histological appearance.
These cells show lack of cohesiveness and are extremely
vagarious. The mitotic figures are extremely high.
On the radiographic there is destruction of bone. The
appearance which is seen as moth eaten with ill-defined
ragged margin (Figs 13.14 and 13.15).
Points to Remember
• Mass or lump, non-healing ulcer in the oral cavity,
irregular shape, induration and raised everted edges,
broad base, surface may range from granular to Figure 13.15 Moth eaten appearance of squamous cell
pebbly to deeply creviced, lymph nodes become carcinoma
enlarged firm to hard on palpation, moth eaten with
ill defined ragged margin
Different Types of Carcinoma According to Site
• Well differentiated squamous cell carcinoma—
resemble the cells of squamous epithelium, increased Below we have described different types of carcinoma
number of mitotic figures, multiple nucleoli and according to their location. Carcinoma of tongue and
increased nucleo-cytosplasmic ratio, individual cell lip are described in chapter of disease of tongue and lip
keratinization, keratin pearls, pleomorphism of cells, respectively.
keratinization, and keratin pearls
• Moderately differentiated/less well differentiated squa-
Carcinoma of Floor of Mouth
mous cell carcinoma—cell to cell contacts, greater Sex distribution: It is seen more commonly in men.
number of mitotic figures, keratinization is absent Appearance: It is seen most frequently in the anterior
• Poorly differentiated squamous cell carcinoma— portion of floor. The typical carcinoma of the floor of
proliferating anaplastic cells, highly invasive, lack of mouth is an indurated ulcer (Fig. 13.16) of varying size, on
cohesiveness, extremely vagarious. one side of the midline.
Malignant Tumors
263
Figure 13.16 Carcinoma of floor of mouth showing Figure 13.17 Malignancy of lower alveolar ridge
ulcerative growth showing growth
Sign and symptoms: It may take form of wart like growth,

which tend to spread superficially rather than in depth.
Carcinoma in close relation to teeth may cause loosening
or exfoliation and root resorption. It may or may not be
painful. In some cases there may be referred pain in the
ears. There may be excessive salivation.
The proximity of this tumor to the tongue produces
some limitation of motion of these organs, often induces
peculiar thickening or slurring of the speech.
Metastatic: Carcinoma of floor of mouth may invade the
deeper tissues and may even extend into the sub-maxillary
and sublingual glands. Metastasis from the floor of the
mouth are found most commonly in the sub-maxillary
group of lymph nodes and since the primary lesion
frequently occurs near the midline where a lymphatic cross Figure 13.18 Carcinoma of buccal mucosa showing
drainage exists, contra-lateral metastasis is often present. extending extraorally
Carcinoma of Alveolar Ridge (Fig. 13.17)

It is cause by ill fitting dentures, smoking, and alcohol. It is
presented as ulcer or growth on alveolar ridge.
Symptoms: Numbness, mobility teeth, pain, bleeding,
dysphagia.
Sign: There is invasion to adjacent structures—buccal
mucosa, floor of mouth. Metastasis occurs into upper deep
jugular nodes.
Carcinoma of Buccal Mucosa

(Figs 13.18 to 13.21)
Location: The lesions develop most frequently along or
inferior to a line opposite the plane of occlusion. It usually Figure 13.19 Carcinoma of buccal mucosa showing intraoral
occurs opposite to the 3rd molar. ulceration
Carcinoma of Labial Mucosa (Fig. 13.22)

It is frequently encountered in person who habitually keeps
264 a mixture of tobacco lime in the labial groove.
Location: The lower labial mucosa is more commonly
involved than the upper.
Signs and symptoms: The most common initial signs
and symptoms are growth or swelling, soreness and
ulceration. Advanced lesion may be ulcerative-infiltrative
type.
Lymph node involvement may occur, which may be
unilateral or bilateral. Most common lymph nodes involve
is submandibular lymph nodes.
Figure 13.20 Carcinoma of buccal mucosa showing Carcinoma of Palate
ulcerative growth on cheek
Sex distribution: It is common in area where reverse
smoking is practiced. It is seen more amongst women as
compared to men, in case of reverse smoking.
Appearance: Palatal cancer usually manifest as a poorly
defined ulcerated painful lesion on one side of the midline.
Most of the lesions are exophytic and with broad base and
nodular surface.
It frequently crosses the midline and may extend
laterally to include tonsillar pillars or even the uvula.
The tumor of hard palate may invade the bone or
occasionally the nasal cavity while infiltrating lesions of
the soft palate may extend into the nasopharynx.
Figure 13.21 Carcinoma seen as an ulcerative fungating

growth in buccal vestibule
Appearance: The tumor begins as small nodules and

enlarges to form a wart like growth which ultimately
ulcerates. The lesion is often painful. There is induration
and infiltration of deeper tissues. Extension into the muscle
of neck, alveolar mucosa and ultimately into bone may
occur.
Some cases appear to be growing outward from
the surface rather than invading the tissues is called as
exophytic or verrucous growth.
Metastasis: The most common site of metastasis is the Figure 13.22 Carcinoma of labial mucosa showing
submaxillary lymph nodes. ulceration
Malignant Tumors
Carcinoma of Maxillary Sinus

(Figs 13.23 to 13.25)
It is most common primary tumor of paranasal sinuses 265
comprising 80 to 90 percent of cancers in this site.
Cause: It is caused by sinusitis, snuff and smoke and
occupational hazards like in boot and shoe manufacturing
and nickel worker.
Age and sex distribution: Mean age of occurrence is 60
years. Males are commonly affected more than females in
the ratio of 2:1.
Figure 13.25 CT scan showing increase radiopacity in right

maxillary antrum with destruction of borders
Symptoms: There is facial pain, swelling, nasal obstruction

and lymphadenopathy.
Medial wall involvement leads to nasal obstruction,
discharge, bleeding and pain. Epiphora will result if the
lacrimal sac or naso-lacrimal duct is obstructed.
Involvement of the floor of the sinus leads to expansion
of the alveolus, unexplained pain, and numbness of teeth,
loose teeth and swelling of the palate or alveolar ridge and
ill-fitting dentures. It may erode the floor and penetrate the
oral cavity.
Figure 13.23 Maxillary sinus malignancy showing intraoral Lateral wall involvement leads to facial and vestibular
ulceration swelling, pain and hyperesthesia of maxillary teeth.
Roof involvement leads to diplopia, proptosis and pain
over the cheek and upper teeth.
Posterior wall involvement leads to painful trismus,
obstruction of Eustachian tube causing stuffy ear, referred
pain and hyperesthesia over the distribution of second
and third division of trigeminal nerve. It may involved
infraorbital nerve and produces paresthesia of the cheek or
erodes blood vessels giving rise to epistaxis.
Paresthesia of mandibular nerve can also occur if the
tumor invades the cranium.
Multiple Carcinomas
Patient who is having one carcinoma of mouth or throat
are increase risk of additional malignancy of upper
aerodigestive tract, esophagus, stomach, lungs or other
sites.
Figure 13.24 Extraoral swelling seen in maxillary sinus This is common in patients who take alcohol and smoke
malignancy after the therapy.
The tendency to develop multiple mucosal cancers METASTATIC CARCINOMA

which sometime called as field cancerization reflects
diffuse exposure to local carcinogen. The reason for this It is also called as secondary carcinoma. It is the most
266 is that the whole mucosa is often in an abnormal state for common malignant tumor in the skeleton.
long periods, prior to the development of over cancer. The This tumor is transported to an area distant from its
most common finding is that there is area of atrophy, either origin and establishes a new foot holds and are said to have
adjacent to a carcinoma or randomly distributed. metastasized.
Although the metastatic carcinoma of jaw is uncommon,
Management its reorganization is important because the jaw tumors
Average time delay between onset and examination is 4 may be the first indication that the patient has a malignant
to 9 months and detection and Accurate diagnosis is 5 to disease.
6 months. Metastasis carcinoma occurs to soft tissue as well as
Removal of any local irritants (smoking, spices, spirit, bone. For soft tissue metastasis is usually blood borne
sepsis and syphilis) is very important as part of treatment. metastasis usually by Batson’s plexus (valveless vertebral
Biopsy is mandatory in every case of carcinoma. venous plexus which allow retrograde spread of tumors
Surgery: Cryosurgery, laser surgery and radical cells, bypassing filtration through lungs). For bone
surgery. metastasis it occurs through hematogenous route.
Radiation and chemotherapy can also be performed. Oral diagnostician can make an invaluable contribution
Commonly used chemotherapeutic agents are cisplatin, of pathologic process of bone. Most common sites of ori-
carboplatin, 5-fluorouracil and taxanes are used. gin are breast, lung, kidney, thyroid, prostate and colon.
Clinical Features
Points to Remember
Location: For jaw metastatic mandible is involved much
• Carcinoma of floor of mouth: Anterior portion of
more frequently than maxilla, especially in the region near
floor, indurated ulcer, wart like growth, loosening
premolars and molars as tumor metastasizes to those bones
of teeth, root resorption, slurring of the speech,
which are rich in hemopoietic marrow. It is said that blood
Metastatic to sub-maxillary and sublingual glands.
flow rate is decreased in areas of hemopoietic marrow and
• Carcinoma of alveolar ridge: Ill fitting dentures,
this predisposes tumor emboli to settle and grow in these
numbness, mobility teeth, dysphagia, metastasis to
areas. This hypothesis is consistent with jaw metastases as
upper deep jugular nodes.
hemopoietic marrow is most routinely found at this site.
• Carcinoma of buccal mucosa: Small nodules and
The other sites involved are maxillary sinus, anterior hard
enlarges to form a wart like growth, extension into
palate and mandibular condyle. For soft tissue metastatic
the muscle of neck, metastasis to submaxillary lymph
gingiva followed by tongue is involved.
nodes.
• Carcinoma of labial mucosa: Swelling, soreness, Age: It is found in patients between 40 and 60 years of age
lymph node involvement, submandibular lymph nodes. and there may be history of primary tumor.
• Carcinoma of palate: Reverse smoking, poorly Appearance: Early lesion is nodule or dome with shaped
defined ulcerated painful, crosses the midline, smooth surface and due to trauma may get ulcerated.
infiltrating lesions of the soft palate.
• Carcinoma of maxillary sinus: Sinusitis, snuff, facial Symptoms: There may be pain followed by paresthesia or
pain, nasal obstruction, medial wall involvement, anesthesia of lip or chin.
involvement of the floor of the sinus, lateral wall Sign: Teeth in this region may become loose or exfoliate
involvement, roof involvement, posterior wall and root resorption may occur. On occasion, tumor may
involvement, paresthesia of mandibular nerve. breach the outer cortical palate of jaws and extend into
• Multiple carcinomas: One carcinoma of mouth surrounding soft tissue or presents as an intraoral mass.
with malignancy of upper aerodigestive tract, field Metastatic tumors are diagnosed only when the sockets
cancerization. of extracted teeth do not heal because of periodontal
• Management: Surgery, radiation, chemotherapy. disease. If invasion occurs in muscle then their function is
impaired.
Malignant Tumors
Numb chin syndrome: When there is involvement

inferior alveolar nerve in mandible due to metastatic
when there is unexplained loss of sensation of lip and
chin. 267
All the soft tissues curetted from an extraction socket,
even in absence of clinical suspicion should be submitted
for histological examination.
Radiological features: Loss of bony support, involving
one or several adjacent teeth, in the absence of generalized
periodontitis is an important symptom. There may be
pathological fracture of the jaw or hemorrhage from the
tumor site. Extraction socket fails to heal or enlarged.
Patient may have moth eaten appearance (Fig. 13.26).
Histopathological Features Figure 13.27 Metastatic carcinoma showing well differentiated

It is well differentiated tumor or poorly differentiated carcinoma (Courtesy: Dr Aparna Thombre, Reader, Department
which resembles features of primary tumor (Fig. 13.27). of Oral Pathology, VSPM Dental College and Hospital, Nagpur,
Maharashtra, India)
Management
Prognosis is poor and death occurs within a short time. It
can be treated by chemotherapy, radiation therapy, surgery, BASAL CELL CARCINOMA
and immunotherapy and hormone therapy.
It is also called as basal cell epithelioma or Rodent ulcer.
Points to Remember It arises from basal layer of epidermis or from the hair
follicle.
Root resorption, mandible is involved, lesion is nod-
ule or dome with shaped smooth surface, paresthesia Etiology
or anesthesia of lip teeth, loose or exfoliate, numb chin
The specific factor in sunlight responsible for skin
syndrome, pathological fracture of the jaw, moth eaten
carcinogenesis appears to be ultraviolet radiation.
appearance, well differentiated tumor or poorly differ-
Other factors are also responsible like burn scars and
entiated.
ionizing radiation. General atrophy associated with aging
process, at least, predispose to development of skin cancer.
Clinical Features
Age and sex distribution: It occurs in middle aged or
elderly person usually in 4th decade of life. Blond people
with fair complexion who have spent much of their lives
outdoors are often victim of these lesions. It is much more
common in men than women because men are exposed to
the environmental elements more than women.
Location: It develops most frequently on exposed surface
of skin (Figs 13.28 and 13.29), middle thirds of face and
the scalp. There is also involvement of lip. The upper lip is
involved more commonly than the lower lip.
Signs: It begins as a small, slightly elevated papule
which ulcerates, heals over and then breaks down again
Figure 13.26 Metastatic tumor showing destruction to form crusted ulcer. It develops a smooth, rolled border
Clinical Variant of Basal Cell Carcinoma

• Noduloulcertive: Firm painless papule that enlarge
268 and develop into central depression and umblicated
appearance.
• Pigmented basal cell carcinoma: Melanin production
gives brown, black, or bluish color.
• Sclerosing (morpheaform) basal cell carcinoma: It
mimic scar tissue. Slight elevation at the edges of
tumor.
• Superficial basal cell carcinoma: Multiple lesion
appear as well demarcated scaly patches with
threadlike border.
• Nevoid basal cell carcinoma: Basal cell carcinoma
associated with syndrome.
Figure 13.28 Basal cell carcinoma showing extensive
destruction of face
It is characterized by appearance of nests, islands or sheets
of cells showing indistinct cell membranes with large
deeply staining nuclei and variable number of mitotic
figures.
The periphery of cell nests is composed of a layer of
cells, usually well polarized, that are strongly suggestive
of cell of the basal cell layer of skin. The basal cell is a
pluripotential cell which may develop in several directions.
It may form hair, sebaceous glands, sweat glands or
squamous epithelium and eventually keratin.
Histological Types of Basal Cell Carcinoma

• Adenoid basal cell carcinoma: It represent neoplasm
which mimics glandular formation.
• Cystic basal cell carcinoma: Presence of many cyst
Figure 13.29 Melanoma showing diffuse border in the lesion.
• Keratotic basal cell carcinoma: Formation of
parakeratotic cells and horn cysts and attempted
representing tumor cells spreading laterally beneath the
formation of hair structures to the trichoepithe
skin. Untreated lesion continues to enlarge and infiltrate
lioma.
the adjacent and deeper tissues and it may even erode
• Solid or primordial basal ell carcinoma: Cells have
deeply into the cartilage or bone.
little tendency to differentiate.
Due to its invading and destructive infiltration into
adjoining tissues, it gradually increases in size and accounts
for its synonym rodent ulcer. It is never seen in oral cavity Management
unless it arrives there by invasion and infiltration from a Surgical excision or X-ray radiation can be given.
skin surface. Occasionally, it may metastasize to lymph Mohs micrognathic surgery: This technique used frozen
nodes. section evaluation of specially mapped and marked
Malignant Tumors
surgical specimen to determine whether tumor tissue left

behind. If it is left behind surgeon can immediately return
and remove more tissue.
Photodynamic therapy is useful for superficial variety 269
of basal cell carcinoma.
Points to Remember
Basal cell epithelioma, ultraviolet radiation, exposed
surface of skin, smooth, rolled border, invading and
destructive infiltration, rodent ulcer, indistinct cell
membranes, basal cell is a pluripotential cell, Mohs
micrognathic surgery, photodynamic therapy.
ADENOSQUAMOUS CARCINOMA
Figure 13.30 Basal cell carcinoma showing sheets and nest
of hyperchromatic epithelial cells It is combination of adenocarcinoma and squamous cell
carcinoma.
Clinical Features
Age and sex distribution: It is seen in older individual
with more commonly seen in male.
Location: It is seen on tongue, oral floor and other mucosal
surface.
Appearance: It appear as nodular broad based painful
mass with or without surface ulceration.
Sign: There is metastasis deposit in the lymph nodes of
neck.
Figure 13.31 Basal cell carcinoma (low power)
There is presence of features of squamous cell carcinoma
and ductal adenocarcinoma. Glandular portion seen in
deeper portion of the tumor.
Management
Radical surgical excision with radiation therapy is the
treatment of choice.
Points to Remember
Nodular broad based painful mass, metastasis deposit in
lymph nodes of neck, ductal adenocarcinoma, features
of squamous cell carcinoma, radical surgical excision.
BASALOID SQUAMOUS CARCINOMA

It is also called as basaloid squamous cell carcinoma. It
Figure 13.32 Basal cell carcinoma (high power) occur in alcohol user and person who smoked tobacco.
Clinical Features SINONASAL UNDIFFERENTIATED

Age and sex distribution: It is seen in 4th to 7th decade of CARCINOMA
270 life with male predilection.
It is highly aggressive neoplasm of nasal cavity and
Location: It is seen in larynx, pyriform sinus, tongue base paranasal sinuses. The cells of origin are from schneiderian
but any region of aerodigestive tract can be affected. membrane or olfactory epithelium.
Appearance: It appears as a fungating mass or ulcer and
interferes with swallowing.
Age and sex distribution: There is tendency to older
Sign: There is cervical metastasis. patient with more prevalence in males.
Histopathological Features Location: It involve sinonasal tract including nasal cavity,
There is superficial well differentiated or moderately maxillary sinus, ethmoid sinuses. Lesion may extend
differentiated squamous cell carcinoma. The deeper nasopharynx, orbit and cranial cavity.
component shows an invasive basaloid epithelium arranged Sign and symptoms: There is nasal obstruction or discharge,
in islands, cords and glandlike lobules (Fig. 13.33). There epistaxis, swelling and pain, orbital involvement lead to
is also palisading peripheral cells, necrosis of central proptosis, periorbital swelling, diplopia, and vision loss.
region. Basaloid cells and islands of cells are surrounded
Radiographic features: Large expansile sinonasal mass
by mucoid stroma.
with bony destruction and invasion of adjacent structure.
Management
Surgery followed by radiotherapy is recommended
It is characterized by trabecular, ribbons, sheets, and nest
treatment for the patients.
of polygonal cells with minimal cytoplasm (Fig. 13.34).
Points to Remember There are numerous mitotic figures. There is also tumor
necrosis, apoptosis, and lymphovascular invasion.
Basaloid squamous cell carcinoma, fungating mass or
ulcer, interferes with swallowing, cervical metastasis, Management
superficial well differentiated or moderately differentiated
Complete surgical therapy with adjunct radiation or
squamous cell carcinoma, cords and glandlike lobules.
chemotherapy.
Figure 13.33 Basaloid squamous cell carcinoma showing Figure 13.34 Sinonasal carcinoma showing nest of
gland like structure polygonal cells
Malignant Tumors
Symptoms: Pain and difficulty in mastication are common

Points to Remember
complain. Regional lymph nodes are often tender and
Nasal obstruction or discharge, epistaxis, expansile enlarged simulating metastatic tumor, but the node
sinonasal mass, trabecular, ribbons, sheets, nest of involvement is usually inflammatory. 271
polygonal cells with minimal cytoplasm, numerous
mitotic figures apoptosis, lymphovascular invasion. Signs: They appear papillary in nature with pebbly surface
which is sometimes covered by a white leukoplakic film.
They have rugae-like folds with deep cleft between them.
VERRUCOUS CARCINOMA In some cases, there may be warty fungating mass. Large
It is also called as Snuff Dipper’s cancer, Ackerman’s broad lesion with minimum to extensive elevation above
tumor. The verrucous carcinoma (VC), a low-grade variant surface of the mucosa. Margins are well-defined and show
of squamous cell carcinoma, has been reported in the head rim of slightly elevated normal mucosa (Figs 13.35 to 13.37).
and neck area, with predilection by oral cavity and larynx.
The clinical presentation of verrucous carcinoma
often shows a local invasive pattern without any distant
metastases and excellent prognosis.
The establishment of clinical or histopathological
diagnosis of verrucous carcinoma in the oral cavity may be
difficult to interpret. This complexity has been attributed to
the indolence of the tumor’s grow, its benign histological
features, and the lack of awareness by the pathologist of the
tumor’s gross appearance.
Etiology
The etiological factor of verrucous carcinoma in the oral
cavity is not completely established, although the lesion had
been associated with tobacco use and human papilloma virus
(HPV). There is co-relation between tobacco chewing habit
and occurrence of high percentage of VC. It occurs usually in Figure 13.35 Verrucous carcinoma showing papillary growth
a person habitual to hold the quid in the buccal sulcus. (Courtesy: Dr Aparna Thombre, Reader, Department of Oral
Path
ology, VSPM Dental College and Hospital, Nagpur,
Clinical Features Maharashtra, India)
Age and sex distribution: It is generally seen in elder

population with mean age of occurrences range from 50 to
80 years with a male predominance and the median age is
67 years.
Location: Verrucous carcinoma (VC) may occur in several
locations in the head and neck and in the genitalia. The
oral cavity is the most common site of this tumor, with
more affinity for buccal mucosa, gingiva or alveolar ridge.
Other extra oral sites involved are larynx, external auditory
meatus, lacrimal duct, skin, scrotum, penis, vulva, vagina,
uterine cervix, perineum, and leg and odontogenic cyst
linings.
Onset: Oral lesion is slow growing, chiefly exophytic and
only superficial invasive, at least until late in the course of
the disease and has a low metastatic potential. Figure 13.36 Verrucous carcinoma showing papillary growth
272
Figure 13.37 Verrucous carcinoma on lower alveolar ridge Figure 13.38 Verrucous carcinoma showing downgrowth of
epithelium into connective tissue
Lesion on the mandibular mucosa grows into overlying

soft tissue and rapidly becomes fixed to the periosteum,
gradually invading and destroying the mandible.
There is marked epithelial proliferation with downgrowth
(Figs 13.38 and 13.39) of epithelium into connective tissue
but usually without the pattern of true invasion.
The epithelium is well differentiated and shows little
mitotic activity, pleomorphism or hyperchromatism.
Cleft like spaces lined by a thick layer of parakeratin
extend from the surface deep into the lesion is seen.
Parakeratin plugging (Fig. 13.40) also occurs the
epithelium. Parakeratin lining of clefts with parakeratin
plugging is the hallmark of verrucous carcinoma. Even Figure 13.39 Verrucous carcinoma showing parakeratin
though the lesion may be very extensive, basement plugging
membrane will be intact. When the lesion becomes
infected, focal intraepithelial abscesses are often seen.
Chronic inflammatory cell infiltration in the underlying
connective tissue may or may not be present.
All bulbous rete pegs of the epithelium tend to project
into the underlying connective tissue, at more or less the
same level and this is called as pushing margin.
Downgrowth of epithelium in connective tissue show
pushing borders which are rather small that invasive
extension. Abundance of keratin is seen on the surface
and with invaginating epithelium as keratin plugging (Figs
13.41A and B).
Management
Prognosis in verrucous carcinoma is very good because of Figure 13.40 Verrucous carcinoma showing parakeratin
absence or late appearance of metastases. plugging and blunt rete pegs
Malignant Tumors
273
A B
Figures 13.41A and B Histopathological picture of verrucous carcinoma (Courtesy: Dr Aparna Thombre, Reader,
Department of Oral Pathology, VSPM Dental College and Hospital, Nagpur, Maharashtra, India)
Excisional Surgery is considered as the treatment of exophytic or fungating growth. Swelling of regional lymph
choice. The extent of surgical margin and the adjuvant node is most common occurrence.
radiotherapy are still controversial.
Points to Remember The transitional cell is a moderately large, round or
Snuff Dipper’s cancer, Ackerman’s tumor, tobacco polyhedral and exhibits a lightly basophilic cytoplasm and
use, human papilloma virus, slow growing, pain and has indistinct cell outlines.
difficulty mastication, papillary pebbly surface, deep The tumor consists of cells arranged and growing in
cleft between warty fungating mass, invading and solid sheets or in cords and nests. The nuclei are large
destroying the mandible, marked epithelial proliferation and round and they exhibit varying degree of mitotic
with downgrowth, epithelium is well differentiated, activity. Keratinization and pearl formation is not
cleft like spaces lined by a thick layer of parakeratin, evident.
parakeratin plugging, pushing margin, downgrowth of
epithelium, excisional surgery. Management
X-ray radiation is most commonly the accepted treatment.
TRANSITIONAL CELL CARCINOMA Points to Remember
These lesions arise chiefly from the tonsil, base of the Primary lesions, slightly elevated, frankly ulcerated,
tongue and nasopharynx. It is extremely malignant, running granular eroded surface, moderately large, round or
a rapid clinical course, metastasizing widely and causing polyhedral and exhibits a lightly basophilic cytoplasm,
very early death. solid sheets or in cords.
Clinical Features
MALIGNANT MELANOMA
Age: Mean age of occurrence is 44 years.
Melanoma is the third most common skin cancer and
Symptoms: There may be sore throat, nasal obstruction,
accounts for 5 percent of the total. It is a neoplasm of
defective hearing or ear pain, headache, dysphagia,
epidermal melanocytes. It is one of the biologically
epistaxis and ocular symptoms.
unpredictable and deadly of all human neoplasms.
Signs: The primary lesions are very small often completely Sunlight is very important etiological factor in
hidden, usually slightly elevated and either frankly cutaneous melanoma. People with a sunburn tendency are
ulcerated or presenting a granular eroded surface. The at high-risk. The fair skinned people are more prone for
tumor is indurated and in some instances appears as an development of the tumor.
There is rapid infiltration in nodular type of melanoma.

Types
It may be focal or diffuse. Many times it may ulcerate and
• Superficial spreading melanoma hemorrhage may be seen. In later stages, it becomes more
274 • Nodular melanoma diffuse, nodular and tumefactive with foci of hypo and
• Lentigo maligna melanoma hyperpigmentation.
• Acral lentiginous melanoma. It presents as a sharply delineated nodule with some
degree of pigmentation.
Clinical Features
Melanoma tends to have two growth phases, i.e. radial Lentigo Maligna Melanoma
growth phase and vertical growth phase. Radial growth Location and sex: It has got predilection for exposed parts.
phase is common in superficial spreading melanoma, It occurs more often in women than in men.
Lentigo maligna melanoma and acral lentiginous melanoma.
Hutchinson’s freckles: This is procurer lesion of Lentigo
In this lesion melanocytes spread horizontally through
maligna melanoma.
basal layer of epidermis. Later on it invade underlying
tissue casing vertical growth phase. Vertical growth phase Appearance: It is pigment macule with ill-defined
is seen in nodular melanoma from the beginning. margins. It occurs characteristically as a macular lesion of
the malar skin.
Superficial Spreading Melanoma It grows slowly in radial phase, with around the central
It is the most common cutaneous melanoma. axis and in a superficial manner. As the disease advances,
Age and sex distribution: The majority of case diagnosed invasion and metastasis are frequent.
in the 5th to 7th decade of life. It is more common in males
as compared to females in the ratio of 2:1.
Acral Lentiginous Melanoma
Location: It is the most common melanoma in blacks and
Location: It also occurs on skin of face, head and neck,
also most common form in oral cavity. It typically on the
chest, abdomen and extremities. In male, it is common in
soles of hands and mucous membranes.
interscapular areas of males and back of legs in females.
Appearance: It begins as a dark pigmented, irregularly
Appearance: The lesion presents as a tan brown, black
margined macule which further develops into a nodular
or admixed lesion on sun exposed skin, in especially in
growth.
blacks. It begins as pigmented macule in superficial
radial growth pattern, restricted mostly to epithelium and Oral Manifestations
junction.
Age and sex distribution: It is an uncommon neoplasm
In advanced cases, melanoma present as an ulcerated,
of the oral mucosa and it is more common in men than in
fungating growth which is associated with bleeding.
women with the overall age of occurrence being 55 years,
The radical growth phase may last for several months
with most cases occurring between 40 to 70 years.
to several years. The vertical growth phase is characterized
by increase in size, change in color, nodularity and at times, Location: It has got definite predilection for palate and
ulceration. maxillary gingiva/alveolar ridge followed by buccal
Some lesions are devoid of pigmentation which is mucosa, mandibular mucosa, tongue, lips and floor of
called as amelanotic melanoma. mouth.
Sign: Focal pigmentation (Fig. 13.42) preceding the
Nodular Melanoma development of actual neoplasm frequently occurs, several
Location: They have a predilection for occurrence on head months to years before clinical symptoms appear.
and neck in male.
Symptoms: The lesions usually appear as deeply pigmented
Appearance: Color varies from mucosal pink through areas, at times ulcerated and hemorrhagic, which tend
brown and blue to black. It is firm on palpation. It has got to increase progressively in size. Oral melanomas are
erythematous borders which surround the tumor. generally painless. The lesion presents as a soft, darkish
Malignant Tumors
275
Figure 13.42 Melanoma in lower mandibular region showing Figure 13.43 Vertical growth phase of the malignant epitheloid
pigmentation cells invading the connective tissue malignant melanoma
brown or black mass. It may have a nodular or a papillary

surface. There may be ulcerations and bleeding of the
oral mucosa. There is minimum induration and no rolled
margins are seen. There is also loosening of teeth may be a
concomitant clinical finding.
Radiological features: The tumor causes extensive
destruction of the underlying bone which is seen
radiologically as radiolucency with ill-defined border.
Clinical Features (ABCDE Criteria) to differentiate

between Melanoma and Melanocytic Nevus
• A – Asymmetry
• B – Border irregularity with notching Figure 13.44 Pagetoid arrangement of tumor cells in
• C – Color variegation (brown to black, white, red and melanoma
blue)
• D – Diameter greater than 6 mm
• E – Evolving lesion with change in size, shape and
color.

The cutaneous lesions are seen initial at the junctional
zone of epithelium and connective tissue. Later the tumor
cell proliferation is seen throughout the thickness of the
epithelium and spread laterally along the basal cell layer and
downward into the connective tissue. Atypical melanocytes
are seen which are usually larger than normal melanocytes
and have varying degrees of nuclear pleomorphism and
hyperchromatism. Figure 13.45 High power view of melanoma
Table 13.4 Grading of malignant melanoma

Various grades of malignant melanoma (Clarks)
276 Grade I: Malignant cells are confined within the epithelium.
Grade II: Malignant cells have invaded into the papillary
dermis.
Grade III: Malignant cells have invaded into the level of
reticular dermis.
Grade IV: Malignant cells have completely invaded the
reticular dermis.
Grade V: Malignant cells have extended into the subcutaneous
fat.
Figure 13.46 Malignant melanoma showing TNM Staging of Cutaneous Melanoma (by Greene
spindle shaped cells Fl, Page DL, Fleming ID at AJCC Cancer Staging
Manual 2002)
Size
Superficial spreading melanoma: The melanocytes are
distributed in a so-called pagetoid manner or sheets. When T1: ≤ 1 mm
melanocytes penetrate basement membrane, a florid host- T2: 1.01–2 mm
cell response of inflammatory cell chiefly lymphocytes T3: 2.01–4 mm
develops. Macrophages and melanophages may be present. T4: > 4 mm
The melanocytes are limited to the epithelium only. (a-without ulceration b-with ulceration)
Lymph nodes
Nodular melanoma: The connective tissue invasion or
deeper lesins are nodular melanomas. It is characterized N0: No lymph nodes involved
by large, epitheloid melanocytes within the connective N1: One lymph nodes
tissue. Small ovoid and spindle shaped cells may be N2: Two or three lymph nodes involved without nodal
present. The tumor cell may invade and ulcerate the metastasis
overlying epithelium and penetrate the deep soft tissue. N3: Four or more matted nodes with metastatic nodes
This is the vertical growth phase in nodular type of (a-microscopic, b-macroscopic, c-in transit metastasis
melanoma. without metastatic nodes)
Metastasis
Lentigo melanoma: It is characterized by increased
number of atypical melanocytes with the basal epithelial M0: No distant metastasis
layer. The epithelium becomes atrophic and dermal M1a: Distant skin, subcutaneous or nodal metastasis
collagen shows the effect of sun damage. If skin M1b: Lung metastasis
appendages are present they are often involved with M1c: All other visceral metastasis or any distant
atypical melanocytes as well. metastasis with elevated serum lactate dehydrogenase.
Acral lentiginous melanoma: This is more commonly

Staging of Melanoma
seen on palate and demonstrated numerous atypical
melanocytes along the epithelium with invasion into the • Stage IA: T1a N0 M0
superficial lamina propria. • Stage IB: T1b N0 M0/ T2a N0 M0
• Stage IIA: T2a N0 M0/T3b N0 M0
Histological Grading and Staging of Malignant • Stage IIB: T3b N0 M0/T4a N0 M0
Melanoma (Table 13.4) • Stage IIC: T4b, N0 M0
It depends upon the depth upto which malignant cells have • Stage III: any T N1 M0, any T N2, M0, any T N3 M0
invaded or infiltrated into the connective tissue. • Any M1.
Malignant Tumors
Management Sign and symptoms: There is swelling, and presence

of a non-healing ulcer. The initial lesions appear either
It is treated by surgical irradiation, immunotherapy and
with a polypoid, exophytic or endophytic configuration.
by chemotherapy or, by combination of these methods. 277
The lesion is fleshy. Pain and paresthesia are prominent
Survival rate is very poor and are worse with metastases.
features.
Points to Remember
Radial growth phase, vertical growth phase
It will show foci of surface epidermoid carcinoma or
• Superficial spreading melanoma: Cutaneous epithelial dysplasia of surface mucosa, usually just at the
melanoma, tan brown, black or admixed lesion, periphery and often quite limited.
pigmented macule, advanced cases ulcerated, Proliferation and dropping off of basal cell to spindle
fungating growth, amelanotic melanoma, pagetoid cell. The tumor cells are arranged in different patterns such
manner or sheets, melanocytes penetrate basement as fasciculated, myxomatosis or streaming.
membrane, macrophages and melanophages. The Spindle cells are characteristic elongated with
• Nodular melanoma: Color varies blue to black, elliptical nuclei, although pleomorphic cell are also
rapid infiltration in nodular, diffuse, nodular and common. Spindle cells are atypical mesenchymal cells.
tumefactive, sharply delineated nodule, invasion Giant cells and inflammatory cell infiltrates are often
or deeper lesions are nodular melanoma, epitheloid present.
melanocytes, ovoid and spindle shaped cells.
• Lentigo maligna melanoma: Hutchinson’s freckles, Management
pigment macule with ill-defined margins, invasion
Surgical removal of tumor with or without radical neck
and metastasis, increased number of atypical
dissection and radiation therapy is used.
melanocytes epithelium becomes atrophic.
• Acral lentiginous melanoma: Soles of hands and
Points to Remember
mucous membranes, dark pigmented, irregularly
margined macule, numerous atypical melanocytes Lane tumor, swelling, non-healing ulcer, polypoid,
along the epithelium. exophytic or endophytic configuration, epithelial dysp
• Oral manifestations: Palate and maxillary gingiva/ lasia, mucosa, dropping off, spindle cells are characte
alveolar ridge, focal pigmentation, soft, darkish ristic elongated with elliptical nuclei, surgical removal
brown or black mass. of tumor.
• Radiological features: Extensive destruction of the
underlying bone.
ADENOID SQUAMOUS
CELL CARCINOMA
SPINDLE CELL CARCINOMA
It is also called as adenoacanthoma. It arises from pilose-
It is also known as Lane tumor, polypoid squamous cell baceous structures or senile keratosis with acantholysis.
carcinoma or carcinosarcoma.
It occurs chiefly in respiratory and alimentary tracts. Clinical Features
It is a variant of squamous cell carcinoma. There is
proliferation of spindle cells believed to be arising from Age and sex distribution: Females are affected more with
the surface epithelium. Spindle cells are anaplastic type of age ranging from 20 to 50 years and older.
carcinoma cells. Location: It more commonly occurs on lip and also in head
and neck region. The lower lip is affected more commonly
Clinical Features than upper lip and it is also common on the vermilion
Age and sex distribution: It is more common in male with border of lip.
mean age of occurrence of 57 years.
Sign and symptoms: It appears as simply elevated nodules
Location: Lower lip, tongue and alveolar ridge or gingiva that may be slow crusting, scaling or ulcerated. Sometimes,
with remainder scattered at other site. there are elevated or rolled borders of the lesion.
Histopathological Features Histopathological Features

This is an adenoid variant of conventional squamous cell It shows three patterns:
278 carcinoma. It shows proliferation of surface dysplastic Keratinizing squamous cell carcinoma: It is same as that
epithelium into the connective tissue as in typical of other squamous cell carcinoma.
epidermoid carcinoma.
The lateral and deep extension of this epithelium show Differentiated nonkeratinizing carcinoma: Broad
the characteristic solid and tubular ductal structures which interconnecting bands of oval or round cells are organized
are lined by a layer of cuboidal cells and often contain or in plexiform and papillary patterns. They are mature
enclose acantholytic or dyskeratotic cells. without keratin formation.
There is heavy chronic inflammatory infiltration in the Undifferentiated nonkeratinizing carcinoma: It consist
corneum, which always show basophilic degeneration: of sheets of lesion cells with less distinct margin which
typical of solar damage. show no differentiation in most cases. Tumor cell also
mixed with lymphoid cells normally found at anatomic site
Management (Fig. 13.47).
Prognosis is good and metastasis is rare. It is treated by
surgical excision. Management
Radiotherapy to nasopharynx and neck is treatment of
Points to Remember choice.
Adenoacanthoma, head and neck region, elevated nodules,
crusting, scaling or ulcerated, proliferation of surface Points to Remember
dysplastic epithelium, solid and tubular ductal layer of Waldeyer’s tonsillar tissue, enlarge, firm to hard cervical
cuboidal cells, heavy chronic inflammatory infiltration. lymph node, otitis media, otalgia, CNS involvement,
Keratinizing squamous cell carcinoma, differentiated
nonkeratinizing carcinoma, undifferentiated nonkerati
NASOPHARYNGEAL CARCINOMA nizing carcinoma.
These are tumors arise from lining epithelium of lymphoid
rich nasopharynx, palatine tonsil and base of tongue.
These three sites are called Waldeyer’s tonsillar tissue or
Waldeyer’s ring.
It is thought to be caused by Epstein-Barr virus, diets
deficient in vitamin C, consumption of salt fish which
contain carcinogen N-Nitrosamine.
Clinical Features
Age and sex distribution: It is seen in 4th to 6th decade of
life. Male to female ratio is 3:1.
Sign and symptoms: There is enlarge, firm to hard
cervical lymph nodes due to metastasis. Primary lesion is
very difficult detect clinically. If it arises at eustachian tube
then otitis media, otalgia or hearing loss can occur. There
may be nasal obstruction and pharyngeal pain.
CNS involvement: Tumor may invade through foramina Figure 13.47 Undifferentiated type of nasopharyngeal
lacerum into brain produce CNS symptoms. carcinoma
Malignant Tumors
MERKEL CELL CARCINOMA It can occur in any location being the bone extremities
the main affected site. It arises in the periosteal tissue or
It is also called as Merkel cell tumor, neuroendocrine endosteally or can arise from pre-existing lesion such as
carcinoma of skin, small cell carcinoma of skin, trabecular fibrous dysplasia, chronic osteomyelitis, Paget’s disease, 279
carcinoma of skin. post radiation cases.
It is more common associated with ultraviolet light In case of fibrosarcoma of oral cavity, the tissue of
exposure. It can be associated with immunosuppressed origin seems to be the periosteum rather than mucosal
individual like HIV infection, patient with chronic connective tissue.
lymphocytic leukemia. This tumor cells contain cytoplasmic
granules which resembles neurosecretary granules found Type of Fibrosarcoma
within the epidermal Merkel cells of touch receptor region. According to Location
Clinical Features • Central fibrosarcoma
• Periosteum type fibrosarcoma
Age and sex distribution: It is more commonly seen in
older individual with slight male predilection. Grade
• Low-grade
Location: It is seen on skin of the face, lower lip, • High-grade
pharyngeal, laryngeal and vaginal mucosa.
Differentiation
Sign and symptoms: It appear as slowly enlarging dome • Well differentiated
shaped nodules with smooth surface and prominent surface • Less differentiated.
vessels. It is red and violaceous with size less than 5 cm.
Histopathological Features Clinical and Radiological Features

It consists of infiltrating sheets with strands of moderately Age and sex distribution: It can occur at any age with mean
sized uniform undifferentiated basophilic cells in the age of occurrence of 50 years with male predominance
dermis and subcutaneous fat. with duration of lesion from few weeks to 20 years (Figs
Pseudoglandular trabecular Cribriform (Swiss Chesse) 13.48 to 13.50).
and sheetlike patterns can be seen. Tumor cells have Location: Fibrosarcomas of the head and neck region with
prominent nuclei with abundant mitotic figure and scant the neck being the most common site (25%), followed by
cytoplasm. face (20%), scalp (16%), and maxillary sinus (12%). Only
12 percent of the fibrosarcoma were located intraorally,
Management
Wide local excision can be done.
Points to Remember
Neuroendocrine carcinoma of skin, Merkel cells,
enlarging dome shaped nodules, infiltrating sheets with
strands of moderately sized uniform undifferentiated
basophilic cells, Swiss Cheese, abundant mitotic figure.
FIBROUS CONNECTIVE TISSUE

Fibrosarcoma
Fibrosarcoma is a malignant neoplasm of the fibroblastic
origin. Fibrosarcoma accounts for approximately 15
percent of all soft tissue sarcomas which represent only
1 percent of all malignant tumors of the head and neck
region. Figure 13.48 Extraoral swelling in fibrosarcoma
Signs: It produces solitary, soft, firm, fleshy, rounded to

lobulated mass that is grossly white to tan yellow. Small
tumors may be well circumscribed, partly or completely
280 encapsulated.
Involvement of TMJ and para-mandibular musculature
results in trismus. Sensory neural abnormalities may occur
if it involves peripheral nerves.
Initially they resemble benign fibrous outgrowth, but
they grow rapidly to produce large tumor. Large tumors are
prone to ulceration and hemorrhage. Secondary infections
are seen in some cases. Pathological fracture may occur
if the lesion in intraosseous. Maxillary lesions are quite
destructive and invade the antrum. They tend to penetrate
the cortex and spread along the periosteum.
Figure 13.49 Intraoral growth seen in fibrosarcoma (Courtesy:
The clinical behavior of the fibrosarcomas is
Dr Aparna Thombre, Reader, Department of Oral Pathology, characterized by a high local recurrence rate and a
VSPM Dental College and Hospital, Nagpur, Maharashtra, India) low incidence of regional lymph node and/or distant
hematogenous metostasis.
However, hematogenous metastasis may involve the
lungs, mediastinum, abdominal cavity and bone.
Local recurrence poses a serious and complex problem
with occurrence of infiltration, local destruction, airway
compression, esophageal compression and extension into
the mediastinum.
Radiographically, an osteolytic lesion is usually present,
with ill-defined borders; however, fibrosarcoma of the
jaws cannot be distinguished from other destructive lesions
of the bone.

It is characterized by high degree of rapid proliferation of
Figure 13.50 Irregular radiolucency seen in posterior area of fibroblasts. It is associated with formation of collagen and
bone (Courtesy: Dr Aparna Thombre, Reader, Department of reticular fibers.
Oral Pathology, VSPM Dental College and Hospital, Nagpur, Cells are spindle shaped and show elongated nuclei.
Maharashtra, India) Associated fibers are generally arranged in interlacing
bands or fascicles. In well differentiated tumors, fibroblasts
are regular in size and shape. The prominent collagen
approximately half of them in the lower jaw. Intraorally,
bundles tend to be arranged in a typical herring bone
it is commonly associated with cheek, maxillary sinus,
pattern.
pharynx, palate, lip and periosteum of maxilla and
Mitotic figures are prominent in small group of poorly
mandible.
differentiated tumors. Fibrosarcoma can be graded in low
Symptoms: Clinically, in the oral cavity the major and high grade of malignancy. Its histological grading
symptoms are pain, swelling, and sometimes associated is based on the degree of cellularity, degree of cellular
with loosening of the teeth, paresthesia and occasionally differentiation, mitotic activity, the amount of collagen
ulceration of the overlying mucosa. produced by the tumor cells and the extent of necrosis.
Malignant Tumors
281
Figure 13.53 Fibrosarcoma (high power)
B
Figures 13.51A and B Fibrosarcoma (Courtesy: Dr Aparna
Thombre, Reader, Department of Oral Pathology, VSPM Dental
Figure 13.54 High power view of fibrosarcoma showing high

mitosis (arrows) in the fibroblast cells. The cells are arranged
in whorled pattern
Low grade fibrosarcoma show spindle cells arranged

in fascicles with low to moderate cellularity with a herring
bone appearance. There is a mild degree of nuclear
pleomorphism and rare mitosis, with a collagenous stroma.
High-grade lesion showed an intense nuclear
pleomorphism, greater cellularity, and atypical mitosis.
The nuclei can be spindle shaped oval or round nuclei. The
histological appearance of high-grade fibrosarcoma may be
similar to other tumors, as malignant fibrous histiocytoma,
liposarcoma or synovial sarcoma (Table 13.5).
Figure 13.52 Fibrosarcoma showing whorled arrangement of Histologically, it is often difficult to distinguish
malignant fibroblasts, (Whr) collagen fibers arranged in herring fibrosarcomas from other soft tissue sarcomas and
bone pattern (Herr), abnormal mitosis (Mito) diagnosis is often achieved by exclusion.
Types
• Giant cell
282 • Inflammatory
• Myxoid
• Storiform
• Pleomorphic
• Angiomatoid.
Clinical Features
Sex distribution: There is slight male predilection and
malignant variety is common in adults.
Location: In the head and neck area, more commonly
encountered in the paranasal sinuses or centrally, within
Figure 13.55 High power view of fibrosarcoma showing high
the jaw.
mitosis (arrows) in the fibroblast cells. The cells are arranged
in whorled pattern Appearance: Occasionally it arises in the oral cavity and
in the lateral neck, it appears as indurated swelling.
Metastasis in nearly one-fourth of cases has been
Variants of Fibrosarcoma reported.
• Sclerosing epitheloid type
• Myxoid type Histopathological Features
• Fibromyxoid type.
It presents with variety of patterns. The spindle cells are
arranged in fascicles with a pinwheel or storiform pattern
Radical surgical excision is most commonly used treatment The deep seated tumors contain both epithelioid
modality. histiocytes and spindle shaped fibroblast. Both cell
component display marked hyperchromatism, pleomor
Points to Remember phism and atypical mitosis with many multinucleated cells
Pain, swelling, solitary, soft, firm, fleshy, rounded exhibiting angulated cytoplasmic borders.
to lobulated mass, involvement of TMJ, prone to
ulceration and hemorrhage, high local recurrence rate, Management
hematogenous metastasis, radiographically, an osteolytic It is treated by surgical excision or radiation with 5 year
lesion ill-defined borders, formation of collagen, reticular survival rate in 20 to 60 percent of cases.
fibers, herring bone, mitotic figures are prominent, show
spindle cells arranged in fascicles, an intense nuclear Points to Remember
pleomorphism, greater cellularity, and atypical mitosis. Malignant fibroxanthoma, indurated swelling, pinwheel
or storiform pattern, epithelioid histiocytes, spindle
shaped fibroblast, hyperchromatism, pleomorphism,
MALIGNANT FIBROUS
mitosis.
HISTIOCYTOMA
It is also called as malignant fibroxanthoman. Neoplasm
SYNOVIAL SARCOMA
that exhibits both histiocytic and fibrocytic features are
referred to as histiocytomas. It is one of the more common It is uncommon malignancy which occur near and large
soft tissue sarcoma of soft tissues of body. joints and bursae.
Malignant Tumors
Table 13.5 Difference between high grade, low grade and fibromatosis
Fibromatosis Low grade fibrosarcoma High grade fibrosarcoma
Cellularity Low to mod Low to mod Mod to severe 283
Nuclear overlap Absent Present Absent
Hyperchromasia Absent Present Present
Nucleoli Inconspicuous Prominent More prominent and
pleomorphic
Mitotic activity 1+ 1+ to 3 + More than 3+
Necrosis Absent Rare Present (Hemorrhage)
Vessel wall infiltration Absent Rare Present
Herring bone pattern Absent Present Present (less distinct)
Collagen fibers bundles Abundant Abundant Less collagen fibers
Management
Early radical resection is the best method of treatment.
Points to Remember
Painless deep seated swelling, biphasic cellular pattern
of cleft like slit like spaces lined by cuboidal epithelial
like cells.
ADIPOSE TISSUE
Liposarcoma
Liposarcoma is a malignant mesenchymal neoplasm
that arises from adipose tissue, most commonly in the
Figure 13.56 Fibrous histiocytoma showing pinwheel pattern retroperitoneum and lower extremities. It is extremely
uncommon malignant tumor of head and neck region.
Clinical Features There is morphological diversity of liposarcoma reflects
Age and sex distribution: It is predominately disease of the great variation in biological behavior of the tumor. It
young adults mean age being 19 years. ranges from tumors with low metastatic potential, that is,
Location: Intraoral sites are cheek, tongue, floor of mouth WDLPS, to tumors with high propensity to metastasize,
and soft palate. It is seen at para-articular sites like bursae that is, the round cell (RC) variant of MLPS or PLPS. In
or tendon sheath. addition to histological distinctiveness, anatomical location
impacts upon prognosis, given that local control is a prime
Signs and symptoms: There is painless deep seated
concern for curative intent.
swelling which may produce difficulties in breathing or
swallowing. Clinical Features
Histopathological Features Age and sex distribution: It most frequently occurs in
adults over the age of 40 years with predilection in males
It is characterized by biphasic cellular pattern of cleft like
in ratio of 2:1.
or slit like spaces lined by cuboidal epithelial like cells.
The space may contain PAS positive mucoid material. Appearance: It has a slow, silent growth, submucosal
There may be fibrosarcoma like proliferation of cells, with or deep in location, producing firm, resilient lesions,
associated collagen or reticulum. sometimes lobulated and often suggestive of cyst.
On gross inspection, as less yellow and less lobulated.
Histological Types of Liposarcoma

284 • Well differentiated liposarcoma
• Myxoid cell liposarcoma
• Round cell liposarcoma
• Pleomorphic liposarcoma
• Dedifferentiated liposarcomas.
Histologically, it is classified into well differentiated,
myxoid/round cell, and pleomorphic types.
In general, it consists of fat cells (Figs 13.57 and 13.58)
and lipoblast in varying degrees of differentiation and
anaplasia with variable stromal component. Figure 13.57 Liposarcoma showing large fat cells (L)—
In well differentiated type this demonstrates scattered lipoblast cells, surrounded by collagen fibers (CL) (Courtesy: Dr
lipoblast and atypical hyperchromatic stromal cells.
CDCRI, Rajnandgaon, Chhattisgarh, India)
Myxoid type demonstrated proliferating lipoblast within
myxoid stroma which contain rich capillary network.
Round cell demonstrates less differentiated rounded
cells.
Pleomorphic type exhibits extreme cellular pleomor
phism and bizarre giant ells.
Dedifferentiated type shows poorly differentiated,
nonlipogenic sarcomatous changes.
Management
It is treated by surgical excision with or without radiation
therapy.
Points to Remember
Slow, silent growth, submucosal, lobulated, well
differentiated, myxoid/round cell, pleomorphic types,
lipoblast, fat cells, atypical hyperchromatic stromal
Figure 13.58 Liposarcoma showing large fat cell-lipoblast
cells, myxoid type, round cell, pleomorphic type, cells, surrounded by collagen fibers
dedifferentiated type.
Chondrosarcomas of the maxillofacial region, accoun

CARTILAGE ting for 1 to 3 percent of all chondrosarcomas of the
Chondrosarcoma entire body, arise predominately in the maxilla with a
predilection for the anterior maxillary region, and occur at
Chondrosarcomas are rare malignant tumors characterized
lower incidence in the mandible.
by the formation of cartilage, but not bone, by tumors cells.
They are also called as chondrogenic sarcoma. Types
It develops from natural cartilage or a benign
Primary: They directly arise from the cartilage.
cartilaginous tumor. Most of them develop from cartilage
Secondary: It develops in a pre-existing benign
located in bone either centrally in bone (medullary cavity) or
cartilaginous tumor.
peripherally, from the cartilage cup of an osteo-chondroma.
Malignant Tumors
Clinical Features
Age and sex distribution: It develops during 3rd through
6th decades and male to female ratio is 2:1. Secondary 285
chondrosarcoma occur at early age than the primary type
of chondrosarcoma.
Location: It is rare in jaws and may occur in maxilla or
mandible. Often found in anterior alveolar process of
maxilla and in mandible it is found at angle and alveolar
ridge of premolar-molar region. The preferred site in the
mandible is the molar region. Other infrequently site in
mandible are the ramus, condyle, coronoid process, or
symphysis. It is a slow growing and less malignant.
Symptoms: Chondrosarcomas of jaws are most commonly
present as a painless swelling or mass of long duration, and Figure 13.59 Chondrosarcoma of condyle showing swelling
pain, paresthesia, trismus, and loosening of the teeth are in condylar region
associated with the evolution of the disease. Teeth adjacent
to the lesion are resorbed, loosened and get exfoliated.
Sign: In some cases, there may be hemorrhage from the
neck of teeth. There may be sensory nerve deficit, proptosis
and visual disturbances. If swelling erodes through the
cortical plate, it tends to be tender, smoothly contoured firm
mass due to presence of cartilage. Mucosal covering appears
normal in early stage but later it ulcerates and develops
necrotic surface, if chronically traumatized. If it occurs in/
or near the temporomandibular joint region, trismus and
abnormal joint function may result (Figs 13.59 and 13.60).
Maxillary lesion may cause nasal obstruction,
congestion, epistaxis, photophobia or visual loss.
Metastatic spread by vascular channel. Malignant cells Figure 13.60 Chondrosarcoma of palate showing smooth
may erode through wall or venules and extend along inside contoured mass
the venules without adhering to vessels wall but still altered
at their site of entry. Lung is common region of metastasis.
Radiological features: Radiographically, the appearance
of the lesion varies from ill-defined radiolucency to obvious
radiopacity, but these findings are not pathognomonic. CT
and MRI can provide important insight in determining the
nature and extent of the lesion, but a definitive diagnosis
has to be made by histologically examination (Fig. 13.61).
(Figs 13.62 and 13.63)
Histological appearance of chondrosarcoma is more or less
same as that of chondroma.
It is composed of hyaline cartilage. Cells are of variable Figure 13.61 CT scan of chondrosarcoma showing
size and binucleated cells are present. radiopaque lesion
Grade III chondrosarcoma: They are highly cellular with

prominent spindle cell proliferation. Mitosis is prominent.
Chondroma and chondroblastic osteosarcoma tumors are
286 difficult to differentiate with chondrosarcoma. Chondroma
predominantly occurs in small bone and is extremely rare
in the jaw and facial bones. It is important to differentiate
chondrosarcoma from chondroblastic osteosarcoma in the
jaw, because the reported prognosis for the former is more
favorable than for the later. The absence of osteoid and
neoplastic bone rules out chondroblastic osteosarcoma.
Histological Variant of Chondrosarcoma

• C
lear cell chondrosarcoma: Cell with abundant
clear cytoplasm. It is low grade lesion
Figure 13.62 Chondrosarcoma showing variable form of • Dedifferentiated chondrosarcoma: It high grade lesion
cartilage cells—normal appearing cartilage cells (NC), clear with mixture of well differentiated chondrosarcoma
cells of cartilage (CL), binucleate cells in other area (BC), and malignant mesenchymal fibrosarcoma
normal hyaline cartilage (HY) • Myxoid chondrosarcoma: It is mainly soft tissue
tumor with proliferation of cells with clear,
vacuolated or eosinophilic cytoplasm
• Mesenchymal: It is separated variety and discuss
separately in this chapter.
Management
Radical resection is the most effective primary modality
for the treatment of chondrosarcoma, because the tumors
are commonly considered to be radio resistant.
Radiotherapy or chemotherapy is usually reserved
for locally recurrent or residual tumors and surgically
unresectable tumors. The five-year survival rate for
chondrosarcomas of the jaws and facial bones was reported
to be 67.6 percent.
Chondrosarcoma of the mandibular symphysis have a
Figure 13.63 Chondrosarcoma showing binucleated cells more favorable prognosis than those of the other mandibular
sites as well as maxilla. The prognosis of chondrosarcomas
The signs of malignancy in tumor are increased depends on the size, location, grade and surgical respectability
cellularity with an increased number of cells with plump of the tumors.
nuclei. The clear cell chondrosarcoma consist of single or
Points to Remember
clustered benign cells and tumor cells with clear cytoplasm.
Chondrogenic sarcoma, a painless swelling or mass
Grade I chondrosarcoma: It resembles to chondroma. of long duration, hemorrhage from the neck of teeth,
It contain chondroid matrix, chondroblast, plump temporomandibular joint region, trismus, nasal obstruction,
chondroblast, binucleated chondrocytes. congestion, epistaxis, photophobia, metastatic spread
by vascular channel, ill-defined radiolucency to hyaline
Grade II chondrosarcoma: It has got low mitotic rate,
cartilage, plump nuclei, grade I chondrosarcoma, grade
increase cellularity at periphery of lobules. Cartilaginous
II chondrosarcoma, grade III chondrosarcoma, radical
tissue tend to be more myxoid with less prominent hyaline
resection.
matrix.
Malignant Tumors
MESENCHYMAL CHONDROSARCOMA In jaws occur in somewhat older person then in other

skeletal site. Mandible is most common primary site (due
Mesenchymal chondrosarcoma (MCS) is one of the most to primary growth center). In maxilla, the alveolar ridge
unusual neoplasms. It was first described by Bernstein and is frequent site of origin. It is derived from osteoblasts in 287
Lichtenstein in 1959 as a distinct variant of chondrosarcoma which tumor cells contain high level of alkaline phosphates.
(CS).
It is more commonly occur second and third decades of Types of Osteosarcoma
life. It contains mesenchymal cells.
• Parosteal (Juxtacortical) osteosarcoma
It tends to metastasize to unusual locations and that too
• Periosteal osteosarcoma
after long period of time. Most of the tumors arise in bone
• Extra-osseous
but an appreciable number occur in soft tissues.
• Intraosseous (well differentiated)
Clinical and Radiological Features • Intracortical
• Multifocal synchronous
Location: It is most commonly seen in maxilla, skull
• Asynchronous
bone, mandible and ribs. It also occurs in tubular bones
• Postradiation osteosarcoma
and pelvis.
Histological
Age: It is seen in younger age group than usual type of
• Fibroblastic
chondrosarcoma. It is usually seen between the ages of 10
• Osteoblastic
to 30 years.
• Chondroblastic
Sign and symptoms: There may be pain due to compression • Telangiectatic
of nerve. In some cases, there may be swelling. Pathological • Small cell
fracture may also occur. • High grade
Radiological features: Radiolucency with infiltrative
margin with stippled calcification present in some cases. Classification
Location
Parosteal (Juxtacortical) osteosarcoma: It is uncommon,
The mesenchymal chondrosarcoma consist of sheets of extremely rare in the jaws and is characterized by slow
small, round or ovoid, undifferentiated cells interspersed growth. It has good prognosis due to low tendency of
by small islands of well differentiated cartilage which often tumor for metastasis. The tumor grows from external
show calcifications and metaplastic bone formation. surface of the bone. It is more common in femur; as well
as in the jaw.
Management
The prognosis is poor and most patients die due to Periosteal osteosarcoma: It is an aggressive variant of
metastasis. Surgical excision with wide margin is most parosteal type of osteosarcoma but with better prognosis
recommended treatment of this malignancy. than conventional intra-medullary osteosarcoma.
Extraosseous: Osteosarcoma of soft tissue in absence
Points to Remember of primary skeletal tumor and occur in breast, liver and
Mesenchymal cells, pain due to compression of nerve, kidney. It is highly aggressive type of tumor.
radiolucency with infiltrative margin, small, round or ovoid,
undifferentiated cells, surgical excision with wide margin. Etiology
Postradiation osteosarcoma: This can develop after 3
BONE years of radiation. Frequency is related to radiation dose.
Traumatic irritation may be causative factor for
Osteosarcoma osteosarcoma. Osteogenic sarcoma may be seen in fibrous
It is also called as osteogenic sarcoma. It is the most dysplasia and Paget’s disease.
common primary malignant tumor of bone and relatively Other causes which can cause osteosarcoma are genetic
rare in oral and facial region. mutation and some viral causes.

Age and sex distribution: It has got bimodal age
288 distribution first in 10 to 20 years and second is in older
than 50 years. It is more common in males.
Location: It is more common in long bones like femur and
tibia and in jaw bone. It is equal in maxilla and mandible.
In the mandible, lesion is seen in body and in maxilla it
occurs in antrum or alveolar ridge.
It grows rapidly with a doubling time of 32 days and
shows recurrence and early metastasis via blood stream to
lungs.
Symptoms: Swelling of a short history and is usually
always accompanied by pain. Affected tooth may become
displaced or loose. Numbness of lip and chin in majority of Figure 13.64 Sun ray appearance seen in osteosarcoma
case is due to involvement of inferior alveolar nerve. There
may be trismus and hemorrhage.
Signs: There may be history of tooth extraction with
nodular or polypoid reddish granulomatous appearing
mass growing from tooth sockets.
Expansion is very firm due to dense fibrous tissue which
is produced. Initially, swelling is smoothly contoured and
covered by normal mucosa. When expansion becomes
chronically traumatized, mucositis develops on surface.
Later, the surface gets ulcerated and looks like whitish
gray in color. From maxillofacial point of view, it may
mimic the periapical inflammation (misdiagnosed or
underdiagnosed).
Gross specimen usually appears white tan, yellow
in color and can be firm in consistency. Exophthalmos,
blindness, nasal obstruction and epistaxis can be seen later Figure 13.65 Osteosarcoma showing bone formation
stage of tumor progression.
Radiological features: Sun ray appearance (Fig. 13.64) Osteoid and bone formation occur in irregular pattern
can be seen on periapical radiograph. There is symmetrical and sometimes, in solid sheets, rather than in trabeculae.
widening of periodontal ligament which can be evident Vascular channels can be present in the tumor and may be
panoramic radiograph. There is spiking resorption of roots prominent and this is called as telangiectatic type of tumor.
of teeth involved by tumors. In some cases Codman’s Infiltrative stroma is formed by spindle, oval or
triangle can be seen. polyhedral cells with hyperchromatic nuclei. A highly
variegated and anaplastic morphology may be presented.
Histopathological Features Sarcomatous connective tissue stroma is best seen in
(Figs 13.65 and 13.66) peripheral zone, central portion is tends to be richer in
A tumor is characterized by proliferation of both, atypical osteoid and osseous matrix.
osteoblasts and their less differentiated precursors.
Pleomorphism occurs in size and shape of cells which Laboratory Investigation
show large, deep staining nuclei and are arranged in Serum alkaline phosphates level is increase in osteosar
disorderly fashion about the trabeculae. coma.
Malignant Tumors
systemic chemotherapy is critical for the treatment of

patients with osteosarcoma.
Points to Remember 289

Osteogenic sarcoma, parosteal (Juxtracortical) osteo
sarcoma, periosteal osteosarcoma, extra-osseous, dou-
bling time of 32 days, pain, affected tooth may become
displaced, granulomatous appearing mass, expansion
is very firm, sun ray appearance, symmetrical widen-
ing of periodontal ligament, Codman’s triangle, atypi-
cal osteoblasts, pleomorphism, vascular channels,
spindle, oval or polyhedral cells with hyperchromatic
nuclei.
Figure 13.66 Osteosarcoma showing abnormal bone or

osteoid (abB), large osteoblasts cells (lo), blood elements (bl)
EWING’S SARCOMA
(Courtesy: Dr Sangamesh Halawar, Reader, Department of It was first described in 1921 by James Ewing. It is also
Oral Pathology, CDCRI, Rajnandgaon, Chhattisgarh, India) called as round cell sarcoma or endothelial myeloma. It is
a distinctive tumor of bone of uncertain histogenesis. Some
Peripheral (Ojuxtacortical) Osteosarcoma studies suggest its neuroectodermal origin.
This osteosarcoma does not involve medullary cavity. As it is having an origin from the mesenchymal
Peripheral osteosarcoma originates adjacent to cortex of connective tissue of bone marrow it is placed here
bone. These occur in long bone but some cases are reported in the category of bone tumors. It represents as third
in jaw bone. There are two types of it, i.e. parosteal and most common bone tumor after osteosarcoma and
periosteal. chondrosarcoma.
It may arise from endothelial elements in the marrow.
Parosteal type: It is lobulated nodules attached to
It may be a secondary deposit of neuroblastoma. It may
cortex by short stalk. It is low grade sarcoma with better
arise from reticulum cell lining of the marrow spaces. So
prognosis. Histologically it shows spindle cell fibroblast
consider it to be a metastatic tumor, the primary of which
like proliferation which contains well-developed trabecular
is located in different sites, including bronchus.
of bone.
Periosteal types: Is it sessile lesion arise within cortex Clinical Features
and elevates overlying periosteum. It produces significant Age and sex distribution: It occurs between the ages of 5
peripheral periosteal new bone formation. It may perforate to 25 years with a male to female ratio of 2:1.
periosteum and invades soft tissue. Histologically it
Location: The bones affected are the long bone of
shows primitive sarcomatous cell within tumor with
extremities although the skull, clavicle, ribs, shoulder and
chondroblastic differentiation. There is also presence of
pelvic girdles are involved. 10 to 15 percent occur in jaw,
osteoid and immature bone formation.
usually in mandible.
Management It is a very rapidly growing highly invasive tumor with
early and widespread metastasis.
Osteosarcoma was treated primarily with surgical resection
with clear margins. Symptoms: Initially, patient present himself with complaint

Still more than 80 percent of patients subsequently of intermittent pain eventually becomes continuous
developed recurrent disease that typically presented as and associated rapid growth of the tumor causing the
pulmonary metastases. This high recurrence rate could be enlargement of bone. During the attacks of pain the tumor
attributed to the fact that most patients have micrometastatic enlarges visibly. It is associated with febrile attacks and
disease at the time of diagnosis. Hence, the use of adjuvant leukocytosis. It may metastasize to other bones.
Signs: The swelling is hard but occasionally it may be

soft and fluctuant. In the early lesion, when the tumor
is intraosseous, swelling is firm. When the tumor
290 breaks through the cortex, it spreads extensively in the
soft tissues and forms a soft mass which may ulcerate.
Swelling is warm and tender. There may be hyperemia
of the overlying tissues, suggesting inflammatory
condition.
The patient may have low grade fever, facial neuralgias,
lip paresthesia. Teeth may become mobile and paresthesia
may develop. There may be and sinusitis.
There is large destructive lesion in the diaphysis or
metaphysis with a moth-eaten appearance. Lesion may Figure 13.68 Ewing’s sarcoma (low power)
be purely lytic or have variable amounts of reactive new
bone formation. Periosteal reaction may give onion skin or
sunburst appearance.
(Figs 13.67 to 13.69)
It is a cellular neoplasm which is composed of solid sheets
or masses of small round cells with very little stroma,
although few connective tissue septa can be seen.
The tumor comprises of small, characteristically round,
neoplastic cells with large oval hyperchromatic nuclei; the
tumor cells have vague indistinct (scanty) cytoplasm and
cytoplasmic membrane.
Figure 13.69 Ewing sarcoma
The tumor cells are usually spread out or arranged

in large sheets. Mitoses are common, and pseudorosette
patterns are typical.
Occasionally, the cells are arranged around blood
vessels, giving rise to rosette formation. It presents high
degree of cellularity, and very little intracellular stroma.
Large cell (atypical) Ewing’s sarcoma: Some lesion
may contain foci of larger cells.
Management
Surgery, X-ray radiation can be used but survival period is
Figure 13.67 Ewing’s sarcoma showing round cells (ro), very less. Prognosis is poor, death occurs within a year of
arranged in sheets separated by connective tissue septae (ct) diagnosis.
Malignant Tumors
Each vessel in turn is surrounded by a connective tissue

Points to Remember
sheath, outside of which are found masses of tumor cells.
Round cell sarcoma, endothelial elements in the The tumor cells may appear large or small, round or spindle
marrow, intermittent pain, swelling is hard, soft shaped and show tendency for concentric layering about 291
and fluctuant, moth-eaten appearance, onion skin or the capillaries.
sunburst appearance, masses of small round cells, Cellular pleomorphism can also occur.
round, neoplastic cells, pseudorosette patterns, rosette
formation, large cell (atypical). Management
Surgery and X-ray radiation can be given.
VASCULAR
Points to Remember
Malignant Hemangioendothelioma
Localized swelling, dark red or bluish red, purely lytic
The term malignant hemangioendothelioma was suggested lesion that erodes and expands the cortex, neoplasm of
originally by Lichtenstein. vascular origin, irregular vascular channels, atypical
It is a neoplasm of mesenchymal origin which is endothelial cells, pericytes, concentric layering, cellular
angiomatous in origin and derived from the endothelial pleomorphism.
cells. It is also called hemangioendothelial sarcoma.
Clinical Features Malignant Hemangiopericytoma

Age and sex distribution: It has slight predilection for Hemangiopericytoma was initially described by Stout
females. It can arise at any age and has been found at birth and Murray in 1942. It is a soft tissue tumor derived from
also. mesenchymal cells with pericytic differentiation.
Location: It may occur anywhere in the body but most
Clinical Features
commonly found in skin and subcutaneous tissues. In oral
cavity, it can occur on lips, palate, gingiva, tongue and Age and sex distribution: There is no sex predilection
centrally within the maxilla and mandible. with age ranging from birth to old age.
Symptoms: Localized swelling with pain may be the Location: It can occur at any site in the oral cavity. Two
feature of lesion. types have been described, infantile and adult.
Infantile lesions manifestation are usually congenital or
Signs: It appears as flat or slightly raised lesion of varying in early age till 1 year. Pediatric lesions present as skin
size, dark red or bluish red, sometimes ulcerated and show or oral soft tissue multinodular mass which respond well
a tendency to bleed even after slight trauma. Bone may be to chemotherapy and some spontaneous regressions are
involved by tumor producing a destructive process. reported.
Adult lesions behave aggressively and have poor
prognosis.
There are no distinctive features of this lesion. It produces
a purely lytic lesion that erodes and expands the cortex; Appearance: Clinical presentation is non-specific and
frequently, it is associated with a mild periosteal reaction. pain is a late feature. It presents as a soft tissue mass
slowly growing. Occasional cases have been reported with
Histopathological Features association of hypoglycemia due to the secretion of insulin
Malignant hemangioendothelioma is a neoplasm of vascular like growth factor.
origin characterized by irregular vascular channels lined Signs and symptoms: It is usually painless. The lesions
with atypical endothelial cells. are firm, apparently circumscribed and often nodular.
The tumor reproduces the normal arrangement of It may or may not exhibit redness indicative of vascular
capillary endothelial channels surrounded by pericytes. nature. Majority of tumors grow rapidly and are therefore
There is profuse proliferation of occult capillaries. of short duration.
Radiologic findings: They are not specific, they consist of Angiosarcoma

a well circumscribed, faintly radiopaque soft tissue mass
This is rare and arises from blood and lymphatic vessels.
that often displaces adjacent structures. Cystic changes are
292 also seen. Clinical Features
Histopathological Features Location: It is more commonly seen in head and neck
Microscopically it is characterized by the so-called region, scalp and forehead. Intraorally it is seen on tongue
pericytoma pattern with tightly packed cells around the and mandible.
thin walled capillaries. Age: It is common in older individual.
It shows proliferation of capillaries surrounded by
Appearance: It start as simple bruise to nodular or
masses of round or spindle shaped cells which are called
ulcerated lesion.
as pericytes. It resembles to Glomus tumor but lack its
organoid pattern, encapsulation and clinical manifestation Histopathological Features
of pain.
Factors such as mitotic activity, cellularity, hemorrhage, It is characterized by infiltrative proliferation of endothelium
and necrosis help in grading the tumor. It mimics fibrous line blood vessels which form as anastomosing network.
histiocytoma, synovial sarcoma histologically (Fig. 13.70). There is hyperchromatic and atypical endothelial cells.
Increase mitotic activity is also seen.
Management
Management
It is treated by surgical removal of the lesion with utmost
care to control hemorrhage. Radical surgical excision is the treatment of choice.
Infantile lesions, multinodular mass, pain, lesions are Simple bruise to nodular or ulcerated lesion, infiltrative
firm, circumscribed often nodular, well circumscribed, proliferation of endothelium, hyperchromatic and atypical
faintly radiopaque soft tissue mass, pericytoma pattern endothelial cells, radical surgical excision.
round or spindle shaped cells, mitotic activity, cellularity,
hemorrhage, and necrosis. NEURAL TISSUE
Neuroblastoma
Peripheral neuroblastic tumors (PNTs including neuro-
blastoma, anglioneuroblastoma, and ganglioneuroma) are
common solid tumors in infancy and childhood. Because
of their diverse clinical behaviors: such as involution/spon-
taneous regression, maturation, and aggressive progres-
sion, they were often described as enigmatic in the past.
The unpredictable nature and variable clinical behaviors
of PNTs have been recognized for decades, and there
currently are concerted efforts to identify reproducible and
robust prognostic factors that allow treatment to be tailored
to the individual cases.
Neuroblastomas begin in the abdomen in the
adrenal gland or next to the spinal cord, or in the chest.
Neuroblastoma can spread to the bones (face, skull, pelvis,
Figure 13.70 Hemangiopericytoma showing proliferation of shoulders, arms, and legs), bone marrow, liver, lymph
pericytes surrounding the blood vessels nodes, skin, and around the orbits.
Malignant Tumors
The cause of the tumor is unknown. Neuroblastoma is Clinical Features

most commonly diagnosed in children before age 5. The
Age and sex distribution: It is more common in adults
disorder occurs in approximately 1 out of 100,000 children
with no sex predilection. 293
and is slightly more common in boys. In most patients, the
neuroblastoma has already spread when it is first diagnosed. Location: It is seen in nasal cavity close to the cribriform
After leukemia it is the most common malignancy of plate. It can extend into paranasal sinus.
children. Sign and symptoms: Patient may complaint of nasal
obstruction, anosmia, epistaxis and pain.
Clinical Features
Age: It usually occurs in children under the age of 5 years. Histopathological Features
Location: It sometimes arises in the nerves or ganglia of the It consists of small, round to ovoid basophilic cells which
sympathetic system in the neck, thorax or abdomen. Oral are arranged in sheets and lobules.
cavity and nasal sinuses are involved. Oral involvement Rosette and pseudorosette formation can be seen.
is usually due to metastasis and it occurs in mandible and
maxilla. Management
Symptoms and sign: The first symptoms are usually Surgical excision with adjuvant radiation therapy.
fever, malaise, and pain. There may also be loss of
Points to Remember
appetite, weight loss, and diarrhea. Other symptoms
depend on the site of the tumor, and may include bone Esthesioneuroblastoma, nasal obstruction, anosmia,
pain or tenderness. Difficulty breathing or a chronic epistaxis and pain, small, round to ovoid basophilic
cough. Enlarged abdomen, flushed, red skin, pale skin cells, Rosette and pseudorosette formation.
and bluish color around the eyes, profuse sweating and
tachycardia. Neurofibrosarcoma
Histopathology They arise from Schwann cells or perineuronal fibroblasts
they are also called as malignant peripheral nerve sheath
The tumor varies very much in its degree of differentiation.
tumors, malignant Schwannoma and neurosarcomas or
The least well differentiated tumor consists of small round
neurogenic sarcoma, account for about 10 percent of
cells arranged merely in diffuse masses.
soft-tissue sarcomas, and approximately half of these
In some cases, differentiating cells begin to form
lesions occur in the setting of neurofibromatosis type 1
groupings; generally referred to as rosettes and the cell
(NF1).
themselves develop neurofibrillary processes. These
processes become young nerve fibers and form tangled Clinical Features
masses in the centers of rosettes.
Age and sex distribution: Neurofibrosarcomas occur only
Management rarely in the head and neck region and are most common
in young adults (mean age 40 to 46 years). The mean age
Radiotherapy and chemotherapy can be given.
of occurrence in patients with neurofibromatosis is 29 to
Points to Remember 36 years, although such lesions have been described in
younger patients.
Peripheral neuroblastic tumors, difficulty breathing or a
chronic cough, enlarged abdomen, flushed, red skin, pale Location: In the oral regions, the mandible, lips and buccal
skin, rosettes, neurofibrillary processes, tangled masses. mucosa are most commonly involved.
Appearance: The tumors present as rapidly enlarging
Olfactory Neuroblastoma masses, sometimes with associated pain.
It is also called as esthesioneuroblastoma. It is tumor of Malignant transformation usually occurs in association
upper nasal vault and arises from olfactory epithelium. with a large, diffuse neurofibroma in cases of NF1.
Radiographic Features if present, it is seen commonly on cheeks and floor of

mouth.
It may show widening of the inferior alveolar canal, with or
294 without destruction of surrounding bone and involvement Signs and symptoms: The lesion appears as painful
of the inferior alveolar nerve. swelling. Secondary ulceration of mucosal surface can
occur.
Histopathological Features They tend to metastasize through hematogenous route.
Microscopically the tumor shows fascicles of atypical
spindle-shaped cells, which may resemble those of Histopathological Features
fibrosarcomas but which are usually more irregular, with There is presence of mitosis, nuclear pleomorphism,
wavy or comma-shaped nuclei. hyperchromatism and bizarre cell forms.
The plumps spindle shaped cells are arranged in streams There is fascicles of spindle shaped cells with abundant
and cords with random nuclei. Some less cellular myxoid eosinophilic cytoplasm and blunt ended, cigar shaped
areas may also be seen. nuclei (Fig. 13.71).
Positive immunostaining for S100 protein is often Proliferating smooth muscles cells are arranged as
helpful, but a definitive diagnosis of neural origin is often interlacing bands or cords.
difficult. It exhibits variation in degree of malignancy, i.e.
tumors that are relatively acellular and show little cellular Management
pleomorphism to tumors that are highly cellular with Patient should undergo radical surgical excision with
pleomorphism and bizarre mitotic activity. adjunctive chemotherapy or radiation therapy.
Management Points to Remember

Surgery and radiation can be given and it has got marked Painful swelling, hematogenous route, mitosis, nuclear
tendency for local recurrence. pleomorphism, hyperchromatism, cigar shaped nuclei,
The prognosis for patients with neurofibrosarcomas proliferating smooth muscles cells.
arising de novo is about 50 percent for 5-year survival,
whereas it is 15 percent for neurofibrosarcoma arising Rhabdomyosarcoma
in cases of neurofibromatosis. Wide excision is done.
It is a malignant neoplasm of skeletal muscle origin and it
The affected nerve should be resected over 5 cm from its
is uncommon in oral cavity.
encasement by the tumor.
Points to Remember
Malignant peripheral nerve sheath tumors, rapidly
enlarging masses, widening of the inferior alveolar
canal, fascicles of atypical spindle-shaped cells, plumps
spindle shaped cells, little cellular pleomorphism.
MUSCLE
Leiomyosarcoma
It is a malignant tumor of smooth muscle origin.
Clinical Features
Age and sex distribution: It can occur at any age with no
sex predilection.
Location: It is usually seen in uterine wall and Figure 13.71 Leiomyosarcoma showing highly cellular tumor,
gastrointestinal tract. It is very rare in oral cavity and cellular atypia, cigar shaped nuclei with increased mitosis
Malignant Tumors
Clinical Features
Age: It is most common soft tissue sarcoma in children and
adolescents. 295
Location: It is a mass occurring in any region of the
head and neck where striated muscle or its mesenchymal
progenitor cells exist. Intraorally, the tonsils and soft palate
are most frequently involved.
Appearance: Typically, it is a rapidly growing soft tissue
mass. It forms polypoid fleshy growth beneath the mucous
membrane, with club like extensions at periphery.
Spread: It may spread by either lymphatic or hematogenous
routes.
Signs and symptoms: Depending upon of the size Figure 13.72 Rhabdomyosarcoma (low power)
of lesion, there may be divergence of eyes, abnormal
phonation, dysphagia, cough, aural discharge or deviation
of the jaw. The overlying skin is usually erythematous
or telangiectatic. The lesions are occasionally ulcerated
and may invade the underlying bone and develop distant
metastasis.
Types (Histological)
• Pleomorphic: It occurs most common in extremities
and in older individuals.
• Alveolar: It is found in head and neck region and in
extremities, with early age of occurrence.
• Embryonal: It is found in the genitourinary tract and
in nasopharynx, with cases reported in oral cavity in
the upper and lower labial folds.
• Botryoid: Is a malignant tumor of vagina, prostate
Figure 13.73 Rhabdomyosarcoma (high power)
and base of bladder in young children.
Alveolar rhabdomyosarcoma: It is characterized by
Histopathological Features spaces lined by epithelium like cells which appear to be
(Figs 13.72 and 13.73) dropping off from collagen. The cells are often small,
Pleomorphic rhabdomyosarcoma: It is composed monomorphic, round or ovoid with dark staining nuclei.
chiefly of spindle cells in haphazard arrangement. These Cells floating in the alveolar spaces are common.
cells are generally large and show considerable variation in Embryonal rhabdomyosarcoma: Here four types of cells
appearance. The nuclei are ovoid or elongated with packed are present—eosinophilic spindle cells, usually arranged
chromatin. The nuclei are situated often in an expanded in interlacing fascicles. Round eosinophilic cells, large and
end of cells. The racquet cell. Strap-like and ribbon cells intermediate in size, with small nucleus and interspersed
typically show process of long streaming cytoplasm. among other cell types. Broad elongated eosinophilic cells
The cytoplasm is eosinophilic and intra-cytoplasmic occasionally with cross striations. Small, round and spindle
longitudinal fibrils as well as transverse cross striations cells with dark staining nuclei and little cytoplasm is present.
may be seen. Cytoplasmic vacuoles are present as a result
of large amount of glycogen in the cells. Multinucleated Botryoid type: It is sparsely cellular and has a pronounced
giant cells are often seen. myxoid stroma. Cambium layer (increase cellularity).

It is treated by chemotherapeutic agents and radiation. It Alveolar soft-part sarcoma, muscles of extremities, slow
296 has got poor prognosis. growing, well circumscribed masses, finely granular
cytoplasm, uniform pseudo-alveolar or organoid pattern.
Points to Remember
• Growing soft tissue mass, club like extensions, Alveolar Soft Part Sarcoma
divergence of eyes, abnormal phonation, dysphagia, It is rare neoplasm of uncertain histogenesis.
cough, aural discharge
• Pleomorphic rhabdomyosarcoma: Spindle cells, Clinical Features
racquet cell. Strap-like, ribbon cells, nuclei are ovoid, Age and sex distribution: It is most common in young
cytoplasmic vacuoles adults and children with female predilection in the ratio of
• Alveolar rhabdomyosarcoma: Dropping off cells, 2:1 in younger patient and male in the adults.
monomorphic, round or ovoid with dark staining
nuclei Location: It is seen on lower extremities, head and neck
• Embryonal rhabdomyosarcoma: Eosinophilic spin- region, orbit and tongue.
dle cells, round eosinophilic cells, broad elongated Appearance: It is slow growing painless mass.
eosinophilic cells, round and spindle cells
• Botryoid type: Sparsely cellular, myxoid stroma. Histopathological Features
It composed of groups of large, polygonal cells which
Malignant Granular Cell Myoblastoma are arranged in central alveolar spaces. These cells have
It is also called as alveolar soft-part sarcoma. It is thought abundant granular, eosinophilic cytoplasm and vesicular
to be of striated muscle origin. nuclei.
PAS stains reveal diastase resistant crystal which under
Clinical Features electron microscope rhomboid, polygonal or rod shaped
Age and sex distribution: It is predominantly a tumor structure with regular lattice work pattern.
of females, occurring usually in teens or early twenties.
Management
Occasional cases also occur in older patients.
Radical surgical excision in conjunction with radiation
Location: The lesion occurs with greatest predilection in therapy and chemotherapy.
muscles of extremities.
Sign and symptoms: They are usually slow growing, well Points to Remember
circumscribed masses. Slow growing painless mass, polygonal cells which are
arranged in central alveolar spaces, rhomboid, polygonal
Histopathological Features or rod shaped structure.
The tumor is composed of large cells with finely granular
cytoplasm that is not as eosinophilic as the cells of BIBLIOGRAPHY
rhabdomyosarcoma.
These cells have a uniform pseudo-alveolar or 1. Billings SD, Folpe AL. Cutaneous and subcutaneous
organoid pattern arranged in relation to numerous delicates fibrohistiocytic tumors of intermediate malignancy: an
update. Am J Dermatopathol. 2004;26(2):141.
endothelial lined vascular channels and septa.
2. Fletcher CDM, Unni KK, Mertens F. Pathology and
The pattern is reminiscent of that seen in the non-
Genetics of Tumours of Soft Tissue and Bone, World
chromaffin paraganglioma. Health Organization Classification of Tumours, 2002.
3. Folpe AL, Morris RJ, Weiss SW. Soft tissue giant cell
Management tumor of low malignant potential: a proposal for the
Due to the high recurrence rate, radical surgical excision is reclassification of malignant giant cell tumor of soft parts.
the treatment of choice. Mod Pathol. 1999;12(9):894-902.
Malignant Tumors
4. Guccion JG, Enzinger FM. Malignant giant cell tumor of soft 7. O’Connell JX, Wehrli BM, Nielsen GP, Rosenberg AE.
parts: An analysis of 32 cases. Cancer. 1972;29(6):1518-29. Giant cell tumors of soft tissue: a clinicopathologic study
5. Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, of 18 benign and malignant tumors. Am J Surg Pathol.
Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor 2000;24(3):386-95. 297
Pathology, AFIP Third Series, Fascicle 30, 2001. 8. Salm R, Sissons HA. Giant-cell tumours of soft tissues.J
6. Oliveira AM, Dei Tos AP, Fletcher CD, Nascimento AG. Pathol. 1972;107(1):27-39.
Primary giant cell tumor of soft tissues: a study of 22 cases. 9. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue
Am J Surg Pathol. 2000;24(2):248-56. Tumors, 5th edn, 2008.
1. Individual cell keratinization and keratin pearls seen in: 9. Most common malignant tumor of bone is:
a. Verrucous carcinoma a. Osteosarcoma
b. Squamous cell carcinoma b. Ewing’s sarcoma
c. Basal cell carcinoma c. Round cell carcinoma
d. Both a. and b. d. None
2. ‘Rodent ulcer’ refers to: 10. ‘Racquet cell’, ‘strap- like’ and ‘ribbon’ cells typically
a. Squamous cell carcinoma seen in:
b. Verrucous carcinoma a. Neuroblastoma b. Rhabdomyosarcoma
c. Basal cell carcinoma c. Leiomyosarcoma d. Ewing’s sarcoma
d. Metastatic carcinoma 11. Pel-Ebstein fever is the characteristic feature of:
3. Histopathologic appearance of nests, islands or sheets a. Malignant lymphoma
of cells seen in: b. Non-Hodgkin’s lymphoma
a. Basal cell carcinoma b. Rodent ulcer c. Hodgkin’s lymphoma
c. Metastatic carcinoma d. Both a. and b. d. None
4. Carcinoma arises chiefly from the tonsil,base of the 12. Reed–Sternberg cells is the histologic feature seen in:
tongue and nasopharynx is: a. Hodgkin’s lymphoma
a. Squamous cell carcinoma b. Non-Hodgkin’s lymphoma
b. Malignant melanoma c. Burkitt’s lymphoma
c. Transitional cell carcinoma d. Both a. and b.
d. Rodent ulcer 13. A characteristic ‘starry-sky’ appearance seen in:
5. Sunlight is an important etiological factor in: a. Mycosis fungoides b. Leukemia
a. Squamous cell carcinoma c. Rodent ulcer d. Burkitt’s lymphoma
b. Basal cell carcinoma 14. Oral amyloidosis is the complication of:
c. Malignant melanoma a. Hairy leukemia b. Plasmacytoma
d. Both b. and c. c. Multiple myeloma d. Both a. and b.
6. Parakeratin plugging seen in: 15. Egg shell crackling on palpation present in:
a. Verrucous carcinoma b. Liposarcoma a. Multiple myeloma b. Chronic leukemia
c. Osteosarcoma d. Rodent ulcer c. Burkitt’s lymphoma d. None
7. A characteristic ‘pushing margin’ histological feature 16. Most common primary malignant bone tumor:
seen in: a. Osteosarcoma b. Osteochondroma
a. Malignant melanoma b. Verrucous carcinoma c. Ewing’s sarcoma d. Kaposi’s sarcoma
c. Basal cell carcinoma d. Both a. and b. 17. Most commonly metastatising tumor in children:
8. ‘Lane tumor’ refers to: a. Osteosarcoma b. Neuroblastoma
a. Fibrosarcoma c. Osteochondroma d. Kaposi’s sarcoma
b. Multicentric oral carcinoma 18. Most common malignancy in AIDS:
c. Osteosarcoma a. Osteosarcoma b. Neuroblastoma
d. Spindle cell carcinoma c. Ewing sarcoma d. Kaposi’s sarcoma
19. Most common salivary gland tumor in bone: 22. The most common site of metastasis from the mandi
a. Mucoepidermoid carcinoma bular sarcoma:
b. Fibroma a. Lung b. Liver
298 c. Papilloma c. Spleen d. Heart
d. Squamous cell carcinoma 23. A malignant tumor of the striated muscle:
20. Which of the following tumor is most aggressive: a. Rhabdomyoma
a. Myxoma b. Rhabdomyosarcoma
b. Cementoblastoma c. Leiomyoma
c. Ameloblastic fibroma d. Leiomyosarcoma
d. Ameloblastic fibro-odontoma 24. A rhabdomyoma is a tumor originating from:
21. Which of the following does not have a viral etiology: a. Nerve tissue b. Smooth muscle
a. Burkitt’s lymphoma c. Striated muscle d. Vascular endothelium
b. Nasopharyngeal carcinoma 25. Sarcoma of soft tissue spreads by:
c. Hodgkins lymphoma a. Blood vessels b. Lymphatics
d. Hepatocellular carcinoma c. Direct extension d. Local invasion
14 Odontogenic Tumors
Chapter Outline
ÂÂ Classification of odontogenic tumors ÂÂ Philipsen and Reichart theory

ÂÂ Development of tooth ÂÂ Ameloblastic fibroma
ÂÂ Stages of tooth development ÂÂ Ameloblastic fibrodentinoma
ÂÂ Molecular Pathology of odontogenic tumors ÂÂ Ameloblastic fibro-odontoma
ÂÂ Ameloblastoma ÂÂ Odontoma
ÂÂ Variant of ameloblastoma ÂÂ Odontoameloblastoma Odontogenic fibroma
• Desmoplastic ameloblastoma ÂÂ Granular cell odontogenic tumor
• Extraosseous/Peripheral ameloblastoma ÂÂ Odontogenic myxoma
• Pituitary ameloblastoma ÂÂ Cementoma
• Adamantinoma of long bones ÂÂ Malignant ameloblastoma and malignant carcinoma
• Unicystic ameloblastoma ÂÂ Primary intraosseous carcinoma
ÂÂ Squamous odontogenic tumor ÂÂ Clear cell odontogenic tumor or carcinoma
ÂÂ Calcifying epithelial odontogenic tumor ÂÂ Malignant changes in odontogenic cyst
ÂÂ Adenomatoid odontogenic tumor or cyst ÂÂ Ameloblastic fibrosarcoma
ÂÂ Continuum concept: (Cahn and Blum) ÂÂ Odontogenic Fibrosarcoma
Odontogenic tumors are lesions derived from epithelial, Though, the term tumor is used for all lesions or growths
ectomesenchymal and/or mesenchymal elements that originating from the odontogenic tissue, they are considered
still are, or have been, part of the tooth forming apparatus. as a heterogeneous group ranging from hamartomatous or
These tumors, therefore, are found exclusively within non-neoplastic proliferations to malignant tumors with
the maxillofacial skeleton (intraosseous or centrally metastatic capacities.
located), or in the soft tissue (gingiva) overlying tooth-
bearing areas or alveolar mucosa in edentulous regions
CLASSIFICATION OF ODONTOGENIC
(extraosseous or peripherally located). The tumors may
be generated at any stage in the life of an individual. TUMORS (TABLE 14.1)
Knowledge of basic clinical features such as age, gender, Odontogenic tumors are classified mostly according to the
and location can be extremely valuable in developing tissue of origin they belong or resemble (Fig. 14.1). There
differential diagnoses of odontogenic tumors. are various schemes of classifying odontogenic tumors.
Table 14.1 Histological classification of odontogenic tumors (World Health Organization classification, 1992)
Benign odontogenic tumors
300 Odontogenic epithelium without Odontogenic epithelium with Odontogenic ectomesenchyme with
odontogenic ectomesenchyme odontogenic ectomesenchyme, with or or without included odontogenic
without dental hard tissue epithelium
Ameloblastoma Ameloblastic fibroma Odontogenic fibroma
Squamous odontogenic tumor Ameloblastic fibrodentinoma Myxoma (Odontogenic myxoma,
Calcifying epithelial odontogenic tumor Ameloblastic fibro-odontoma myxofibroma)
(Pindborg tumor) Odontoameloblastoma Benign cementoblastoma
Clear cell odontogenic tumor Adenomatoid odontogenic tumor
Calcifying odontogenic cyst
Complex odontoma
Compound odontoma
Malignant tumors
Odontogenic carcinomas Odontogenic sarcoma
Malignant ameloblastoma Ameloblastic fibrosarcoma Odontogenic carcinosarcoma
Primary intraosseous carcinoma Ameloblastic fibrodentinosarcoma and
Malignant variants of other odontogenic Ameloblastic fibro-odontosarcoma
epithelial tumors
Malignant changes in odontogenic cysts
Figure 14.1 Probable sources of epithelium for development of odontogenic tumors

Odontogenic Tumors
DEVELOPMENT OF TOOTH Cap stage: Cap shape of enamel organ due to invagination
or pushing of ectomesenchyme into the tooth bud. Tooth
To understand the histogenesis of the odontogenic bud develops concavity which becomes occupied by
tumors, the knowledge of process of tooth development is mesenchymal cells called as dental papilla. In this stage 301
mandatory. morphogenesis of tooth begins and continuous till hard
tissue formation begins. Histodifferentiation also begins at
Odontogenesis this stage with tooth germ composed of four distinct cell
It is a highly coordinated and complex process which relies layers.
upon cell to cell interactions that result in the initiation and ∙∙ Outer enamel epithelium: Layer of cuboidal cells
generation of the tooth. Tooth is derived from complex covering outer surface of tooth bud.
interactions between ectoderm of the oral cavity and ∙∙ Inner enamel epithelium: Single layer of low columnar
ectomesenchyme. cells at the concavity of cap.
Ectomesenchyme of oral cavity comes from neural ∙∙ Stratum intermedium: Several layers of polygonal cells
crest cells. These are cells derived from neural fold which present over inner enamel epithelium.
is formed from ectoderm. ∙∙ Stratum reticulum: Many layers of polygonal cells
Certain cells at the tip of this fold are loose and mobile occupy space between stratum intermedium and outer
and have an ability to migrate. The ectoderm along enamel epithelium.
pathways and enter the developing facial region.
Dental follicle or sac: A layer of mesenchymal cells,
After reaching or facial region neural crest cells initiate
which surrounds the tooth bud and dental papilla. This
proliferation of oral epithelium at certain areas forming a
layer gives rise to periodontal ligament.
thick epithelial band.
This band forms horse-shoe shaped epithelial sheet in Bell stage: Morphodifferentiation of enamel organ gets
both the arches the free end of the arch gives two extensions completed in this stage. The concavity of cap further
in mesiolateral direction. The sheet present laterally is deepens and assumes bell shape. The shape of inner enamel
known as vestibular lamina and mesial sheet is known as epithelium roughly outlines the shape of the crown.
dental lamina.
Stratum intermedium: 2 to 3 layers of flatter cells
Growth of dental lamina proceeds and results in
between stellate reticulum and inner enamel epithelium.
formation of rounded localized growth of epithelial cells
These cells which contain high amount of enzymes
called as tooth buds. Initially 20 tooth buds corresponding
phosphatase enzyme are highly active cells concerned with
to deciduous tooth germs develop.
protein synthesis and together with cells of inner enamel
Dental lamina gives a lingual extension lingual to
epithelium, act as single functional unit responsible for
developing deciduous teeth except molar develops from
formation of enamel. These cells divide even after cells of
these extensions. Molars develop from a direct posterior
inner enamel epithelium cease to divide. At secretory stage
extension of dental lamina.
the inner enamel epithelium cells are called as ameloblasts.
They are tall columnar cells with nucleus placed away
STAGES OF TOOTH DEVELOPMENT from the basement membrane.
Tooth development is continuous process. During
Cell Rest of Malassez and Serrae
development of tooth germ takes various shapes, based
on these shapes tooth development is classified into three The cells rests of Malassez are the remnants of the Hertwig’s
stages. epithelial root sheath. These are the only odontogenic
epithelial cells that remain in the periodontium after the
Bud stage (round-shaped epithelial condensation): eruption of teeth.
At this stage there is no histodifferentiation or morpho Cell rests of Serrae are remnants of dental lamina. They
differentiation and cells are highly proliferative. There are usually present in the gingiva, but few may be present
is condensation of ectomesenchyme cells which also in the periodontal ligament. These cells are believed to
proliferate without differentiation. Peripheral epithelial behave in a manner similar to cell rests of Malassez. They
cells are low columnar and central cells are polygonal. possess an inherent potential to diversely transform towards
various tumor lines by exerting inductive influence on the Tooth Germ

adjacent connective tissue. They are believed to give rise to
The similarity between tooth germ and odontogenic tumors
most of the peripheral odontogenic tumors.
302 has led to the belief that odontogenic tumor develop from
Few epithelial rests, which are derivatives of distal
tooth germ. This may be true in case of tumors that develop
extension of dental lamina are present in the molar region.
in the first and second decade of life.
These also give rise to odontogenic tumors, especially
adenomatoid odontogenic tumor and odontogenic keratocyst. Heterotopic Epithelium
Pericoronal Dental Follicle Ameloblastoma like tumors are seen in sites other than
jaws. Craniopharyngioma or pituitary ameloblastoma
Dental follicle is a soft delicate tissue, which covers the
arises from caniopharyngial duct, a derivative of Rathke’s
crowns of unerupted teeth. This dental follicle helps in
pouch a derivative of oral epithelium.
the protection and eruption of newly formed tooth. Dental
In long bones, a tumor similar to ameloblastoma called
follicles are comprised of nests of odontogenic epithelium
adamantinoma develops. But other than resemblance to
probably derived form the dental lamina, and the reduced
ameloblastoma, it is not related to it. Pilomatrixoma is
enamel epithelium supported by ectomesenchyme of
another tumor that shares few features with CEOT. It is
developed tooth. It was observed that nests have a greater
thought to originate from hair follicles.
proliferative capacity than the cells of the reduced enamel
epithelium and thus they are believed to be responsible for Basal Cells of Oral Epithelium
development of odontogenic tumors.
As age advances chances of alteration in pericoronal Tooth germs are developed from oral ectoderm as a
tissue increases. In advance age of over 40 years, it is down growth into the connective tissue. A few basal
common to find odontogenic carcinomas in pericoronal cells of oral epithelium retain their pluripotentiality and
tissue. Dental follicle of impacted tooth remains in in some cases may give rise to tumors in adults. Few
the oral cavity for a very long time. So, it is exposed to peripheral odontogenic tumors appear as down growth
various pathological processes for a long time. This may of basal cells.
be one of the explanations why many odontogenic tumors In few cases, epithelial proliferation known as
are common in molar area of mandible. The commonly odontogenic epithelial hamartomas (OEH: Also known as
occurring lesions are ameloblastoma, carcinomas, calcifying odontogenic gingival epithelial hamartias OGEH) believed
odontogenic cysts, calcifying epithelial odontogenic tumors to be an intermediate stage in development of tumors.
and odontogenic myxoma.
Gubernacular Dentis
Cyst Lining Gubernacular dentis is fibrous tissue that forms connection
It is thought that epithelial lining of odontogenic cyst is a between developing permanent teeth and lamina propria
source of epithelium for a variety of odontogenic tumors. of gingiva. These occupy Gubernacular canals found in
This contention is supported by the fact that both cyst and dried skull. Gubernacular dentis contains central strand of
tumors develop from same odontogenic epithelium, so epithelial cells derived from the dental lamina, surrounded
transition from cyst to tumor is possible. by the connective tissue.
Dentigerous cysts are believed to give origin to This connective tissue is organized in two layers. In
ameloblastoma. Calcifying epithelial odontogenic cyst inner layer, collagen fibers show greater organization and
exists in spectrum where tumor is one extreme end and run mainly parallel to long axis of the chord. Outer layer is
simple cyst forms the other end. Few AOT’s are reported made up of fewer collagen fibers which are less organized.
in CEOC lining. It is more vascular than the inner layer.
Adenomatoid odontogenic tumor has a dentigerous It is thought to play a role in eruption of the permanent
relation with the tooth, so it was also believed to originate teeth. Epithelial cells are occurring more numerously in the
from dentigerous cyst. Unicystic ameloblastoma is inner layer like pearls on string.
believed to be a cystic counterpart of ameloblastoma. It hypothesized that these epithelial cells undergo
OKC is now classified as a tumor rather than as a cyst proliferation to form odontogenic tumors and hamarto
by WHO. matous lesions. But what initiates these cells is not
Odontogenic Tumors
known. It is thought that different variants of adenomatoid which is not found in this tumor, Churchil in 1934 suggested
odontogenic tumor are derived from gubernacular dentis. alternative name of ameloblastoma which became popular
and well accepted.
MOLECULAR PATHOLOGY OF There are different names given to this tumor. These 303
ODONTOGENIC TUMORS are adamantine epithelioma, adamantinoma, adamantino-
blastoma, epithelial odontome and multilocular cyst.
(TABLES 14.2 AND 14.3)
Exact etiology and pathogenesis of odontogenic tumors is Definitions
unknown. With evolution of immunohistochemistry and It has been defined in various ways ameloblastoma has
other advanced diagnostic techniques it has been found that been defined and described by various authors in the
various molecules and genes are altered in odontogenic literature in the past few decades. The few most commonly
tumors. A series of genetic and molecular alterations referred are:
appear to promote the development and progression of
WHO definition: Solid multicystic ameloblastoma
tumors via multiple steps.
is polymorphic neoplasm consisting of proliferating
The odontogenic tumors discussed in this text are
odontogenic epithelium, which usually has a follicular or
according to the most commonly followed 1992 WHO
plexiform pattern, lying in a fibrous stroma.
classification.
Robinson: Ameloblastoma is a tumor usually unicentric,
AMELOBLASTOMA nonfunctional, intermittent in growth, anatomically benign
and clinically persistent.
Ameloblastoma is a benign, locally invasive, polymorphic
neoplasm, presumably derived from intraosseous remnants Shafer, Hine and Levy: The ameloblastoma is a true
of odontogenic epithelium. It is primarily located centrally neoplasm of enamel organ-type tissue which does not
in the jaw bones. Ameloblastoma presents as a solid, undergo differentiation to the point enamel formation.
unicystic, or mixed solid and multicystic neoplasm. It Reichart and Slootweg: A polymorphous neoplasm
is the second most common tumor of the odontogenic consisting of proliferating odontogenic epithelium, usually
tissues after odontomas. It is more common than all other occurring in two main patterns. In the follicular type of
odontogenic tumors except odontomas are combined. growth, the tumor consists of enamel organ-like islands
Broca in 1868 was the first to report to ameloblastoma. or follicles of epithelial cells, while in the plexiform type,
First detailed description of ameloblastoma was given the epithelium forms continuous anatomizing strands. In
by Falkson in 1879. In 1885 Malassez coined the term both types, the epithelial tumor components are embedded
Adamantinoma. As this term suggests formation enamel in mature, connective tissue stroma. Generally, a tumor
Table 14.2 Cytokeratin expression and odontogenic tumors

Tissue CK7 CK8 CK10 CK13 CK14 CK18 CK19 VIM
Dental germ +c - - +d +a - +b -
Reduced enamel epithelium - - - +e + - - -
Ameloblastoma - - - +f + - +f -
Adenomatoid odontogenic tumor - - - - + - - +g
Cacifying epithelial odontogenic tumor +h - - ±i + - ±i +
Ameloblastic fibroma +h - - +j + - - -
Odontoma + - - - +a - - -
. +, positive; –, negative; ±, weak positive; a–(except)–secretory ameloblasts; b–preameloblasts, ameloblasts, external enamel
epithelium; c–Hertwig root sheath, stellate reticulum; d–dental lamina; e–some cells; f–metaplastic scamous cells, inner stellate
cells, cystic structures; g–fusiform and ovoid cells near calcified bodies and darker eosinophilic materials; h–one tumor; i–two
tumors; j–stellate reticulum or cords (Crivelini et al, (2003).
Table 14.3 Molecules possibly associated with tumorigenesis and/or tumor cell differentiation
(Modified from H Kumamoto)
Molecules involved in tumor genesis and differentiation
304
Oncogenes Normal cellular genes which promote Contribute to neoplastic transformation
oncogenesis if they become overactivated
Ras Required for functioning of growth factors Expressed in odontogenic epithelial cells
Myc Regulates cell proliferation Expressed in parabasal epithelial cells of
ameloblastoma
Fos Regulates cell proliferation and Gene altered in ameloblastoma-tumor genesis via
differentiation dysregulation of cell proliferation
Tumor suppressor These inhibit cell proliferation. Loss of genes leads to loss of control of cell proliferation leading to
genes tumorigenesis
p53 It is called as guardian of genome. It and Increased expression in ameloblastomas, malignant
its products control DNA damage, help ameloblastomas, primary intraosseous carcinomas, and
in repair of damaged DNA, control cell ameloblastic fibro sarcomas
proliferation and cell death Regulators of p53 like MDM, p14ARF are also
expressed in odontogenic tumors
APC This normal inhibits cell proliferation. Loss Decreased APC expression is possibly involved in
of function leads to tumorigenesis oncogenesis or cytodifferentiation of odontogenic
epithelium
Retinoblastoma Controls cell cycle progression Transgenic mice develop ameloblastoma
DNA-repair genes Abnormality in these genes leads to genetic Development and progression of these tumors do not
(hMSH2 and instability. depend on a defect in the DNA repair system
hMLH1)
Growth factors Key regulators of cell growth differentiation TGF-a and EGFR are involved in odontogenic
and proliferation tumorigenesis
TGF-a, -b, FGF-1, TGF-b, HGF regulate epithelial– TGF-b, HGF regulate or dysregulate epithelial–
-2, mesenchymal interactions mesenchymal interactions
HGF HGF essential for morphogenesis of tooth HGF is associated with the malignant potential of
germs epithelial odontogenic tumors
Telomerase Anti-aging enzyme. It is responsible for cell Telomerase activation is associated with tumorigenesis
immortalization of odontogenic epithelium
Cell cycle regulators Consist of genes and proteins and involved Proliferation of odontogenic epithelial cells is strictly
Cyclin D1, in regulation of progression in the cell controlled by these cell cycle regulators
p16INK4a, cycle. Cyclins, cyclin dependent kinases
p21WAF1/Cip1, and (CDK) and CDK inhibitors (CDKI) are these
p27Kip1 regulators.
Cell proliferation Reflect proliferation activity and malignant potential

markers Indicators of cell proliferation
PCNA, H3, DNA
topoisomerase IIa
Contd...
Odontogenic Tumors
Contd...

Apoptosis-related These factors control physiologic cell death. 305
factors These help in killing of damaged cells and
check-in proliferation of cells.
Pro-apoptotic Expressed in odontogenic tumors but the Apoptosis is decreased in odontogenic tumors even
signals mediator of these signals known as caspases 8 is though the signals are present
Fas, TNFR I, not fully expressed
TRAILR I and II There is increased survival of tumor cell
Expressed in various types of tumors
Anti-apoptotic
signals
Bcl-2, Bcl-x,
survivin
Regulators of tooth Involved in positioning and morphogenesis of Play a role in epithelial–mesenchymal
development tooth germs interactions and cell proliferation during the
Msx-1, Msx-2, Dlx- growth of odontogenic tumors as well as during tooth
2, Barx-1, and Pax-9 development
SHH, PTCH, SMO, Involved in morphogenesis and

GLI 1 cytodifferentiation of tooth germs
BMP, Wnt, HGF,
and
FGF
Hard tissue-related Expressed in epithelial components of tumors Aberrations of enamel-related proteins are involved in
proteins oncogenesis of odontogenic epithelium
Enamel related
proteins-
amelogenin, Mutation are detected in ameloblastoma
enamelin, tuftelin,
enamel sin
ameloblastin (amelin
or sheathlin)
These proteins play a role in pathologic mineralization
Dentin, bone and and/or tumor formation
cementum related
proteins
BSP, osteonectin,
osteocalcin,
osteopontin, DSP,
DPP
Matrix-degrading Help in tumor remodeling Help in tumor progression
proteinases
MMPs-1, -2, and -9 Expressed in ameloblastoma, myxoma and other Responsible for invasiveness of odontogenic tumors
MSP TIMP, odontogenic tumors
heparanase
Contd...
Contd...
306 Angiogenic factors Necessary for development of vascularity Help in tumor angiogenesis and may be involved
during inflammation and repair tumorigenesis or malignant transformation
VEGF, FGF, Necessary for tumor angiogenesis
HGF, TGF-b
Osteolytic cytokines Maintain bone homeostasis Responsible for invasion by local resorption of bone
IL-1, IL-6, and
TNF-a, PTHrP,
RANKL
shows one or the other pattern throughout. However, not be polyoma viruses. Recently various studies indicate that
infrequently both patterns are present in the same tumor. human papilloma virus also plays a role in development of
ameloblastoma. HPV type 16 or 18 and type 6 have been
Classification detected. Few studies have shown that even Epstein-Bar
• Central or intraosseous variant: virus genes are present in few ameloblastoma.
– Follicular or dentigerous type: It is AOT associated Chemical carcinogens: Injection of nitrosoureas have
with the crown of unerupted tooth. It is usually been shown to produce ameloblastoma like lesion in
misdiagnosed as dentigerous cyst. Seventy-one animals.
percent of AOTs belong to this group.
– Extra-follicular type: There is no association Pathogenesis
with tooth. Twenty-one percent AOTs are of this
The resemblance of the tumor epithelium to the normal
type.
enamel organ indicates that ameloblastoma arises from
• Peripheral or extraosseous type: This type is
dental epithelium. The possibilities for its development are
presented in gingiva. Only 4 percent are peripherally
as follows:
located.
Enamel organ: Due to histological similarities such as in
Etiology origin, it has been thought the tumor growth starts at an early
Irritation: Ameloblastoma is common in mandibular angle age, i.e. during the period of existence of enamel organ but
region. Here, there is constant pooling of saliva containing most of the patients are middle aged, i.e. in a period which
various irritating factors and microorganis. This irritation is long after regression of the enamel organ. However, the
is thought to cause ameloblastoma. occasional occurrence of the tumor as a unilocular cystic
lesion surrounding the crown of an unerupted tooth also
Oral sepsis: In blacks ameloblastoma more common. In suggests that in some cases, the enamel organ may give
such cases, oral hygiene was found to be poor, so it was rise to it.
thought to be a factor.
Cell rests: Cell rests of enamel organ either remnants
Trauma: A few patients gave history of either trauma or of dental lamina or remnants of Hertwig’s sheath, i.e.
extraction prior to the ameloblastoma. So trauma can be a epithelial cell rests of Malassez have the potential of
causative factor for ameloblastoma. transforming into ameloblastoma.
Dietary deficiency: Dietary deficiency has been considered Epithelium of odontogenic cysts, i.e. from dentigerous
to be a possible factor. For example, pronounced defect in cyst and odontoma: This be possible as the epithelium in
development of tooth germ as seen in rickets may lead to the wall of the cyst and that of ameloblastoma are derived
irregularity in the ameloblastic layer. from the same embryonic source.
Virus: A few ultra structure studies fo amelobasltoma Oral mucosa: Basal cells layer of the oral epithelium
showed presence of viral particles which were thought to of jaws can be the origin. The reason behind this is that
Odontogenic Tumors
there is communication between the tumors and overlying Table 14.4 Incidence of different types of ameloblastoma
mucosal epithelium as seen in peripheral ameloblastoma.
Relative frequency of different types
Heterotrophic epithelium: Heterotrophic epithelium in Histologic variants Frequency 307
other parts of the body especially of pituitary gland can
Follicular 33.9
serve as source of origin. Plexiform 30.2
Acanthomatous 11.3
Classification Granular cell 3.5
• Solid/Multicystic ameloblastoma Basal cell 1.4
– Follicular ameloblastoma Desmoplastic 1.4
– Plexiform ameloblastoma Keratoameloblastoma 0.1
– Acanthomatous ameloblastoma
– Granular cell ameloblastoma type of ameloblastoma is more common in the 2nd and 3rd
– Basal cell ameloblastoma decade and the extraosseous form is more common in the
– Clear cell ameloblastoma older age group.
– Keratoameloblastoma
Location: It develops in the molar ramus area
– Desmoplastic ameloblastoma
(approximately 3/4 of cases) in the mandible and also
• Unicystic ameloblastoma
occurs in maxilla in third molar area, followed by the
• Peripheral ameloblastoma
maxillary sinus and floor of the nose. The right side of the
• Extra oral ameloblastoma
mandible is affected slightly more as compared to the left
– Craniopharyngioma
side.
– Adamantinoma of long bones
It begins as a central lesion of the bone which is slowly
destructive but tends to expand bone rather than perforating
Classification
it.
Ameloblastoma shows different clinical features and
radiographic appearances. These clinical-radiographic Symptoms: Patient notices a gradually increasing facial
types are further subdivided on the basis of clincopathology asymmetry. Teeth in involved region are displaced and
of the tumor. become mobile. Pain and paresthesia may occur, if the
Among the histologic types of ameloblastoma, follicu- lesion is pressing upon a nerve or is secondarily infected.
lar and plexiform patterns are the most common; less fre- Signs: In later stages, the lesion may show ovoid and
quent variants are acanthomatous and granular cell types. fusiform enlargement that is hard but nontender (Fig.
Less common cellular variants are the desmoplastic amelo- 14.2). As tumor enlarges palpation may elicit a hard
blastoma, basal cell ameloblastoma, keratoameloblastoma, sensation or crepitus. Surrounding bone may become thin
papilliferous keratoameloblastoma, clear cell ameloblasto- so that fluctuation and egg shell crackling may be elicited.
ma and unicystic ameloblastoma. Except for the unicystic If it is left untreated for many years, the expansion may
type, which has a low recurrence rate, no significant differ- be extremely disfiguring, fungating and ulcerative type of
ences in the behavior of these variants have been observed growth characteristic of that of carcinoma can be seen.
(Table 14.4). Few “hybrid forms” having combinations of
Maxillary ameloblastoma: Maxillary lesions are more
histologic variants are also reported.
dangerous than mandibular lesions due to tendency for
Clinical Features the former lesion to spread more extensively in the more
porous maxillary bone and possibility of the involvement
Ameloblastoma accounts for approximately 1 percent of
of the cranial base. It may extend into the paranasal air
all oral tumors and 11 percent of all odontogenic tumors.
sinuses, orbit or the nasopharynx.
Age and sex distribution: It has slight predilection for
males and often seen in blacks. Most patients are between Radiographic Features (Figs 14.3 and 14.4)
20 to 50 years of age with mean age of discovery being 40 In early stages, there is an area of bone destruction which
years. The tumor can occur in young children. Unicystic is well defined and is indicative of slow growth with
308
Figure 14.2 Ameloblastoma showing huge swelling and Figure 14.4 CT scan of ameloblastoma
disfigurement
Figure 14.3 Ameloblastoma showing multilocular Figure 14.5 Gross specimen of ameloblastoma
radiolucency
hyperostotic borders. Margin is smooth, scalloped, well belong to any one of these patterns. Most of the varieties
defined and well corticated. show follicular pattern.
There is presence of septa in the lesion. In some cases,
number and arrangement of septa may give the area a Follicular Ameloblastoma
honeycomb appearance (numerous small compartments) (Figs 14.6 to 14.10)
or a soap bubble appearance (larger compartments). In This is the most commonly encountered type of
advanced stages, perforated cortical plate. ameloblastoma. It grows mainly in form of islands, but to
Extensive root resorption may occur. Expansion and the smaller extent cords and strands are also present. The
thinning of cortical plate occurs leaving thin eggshell of peripheral cells are tall columnar. They show palisading
bone. arrangement, where cells are arranged perpendicular to the
basement membrane.
Histopathological Features These cells show reverse polarity of the nucleus, i.e.
Macroscopically it shows hard swelling (Fig. 14.5). nucleus is placed away from the basement membrane.
Histologically ameloblastoma exists in two main forms, Nucleus is hyperchromatic. At the basal area of these cells
i.e. follicular and plexiform patterns. Other variants are vacuoles are present.
Odontogenic Tumors
309
Figure 14.6 Follicular ameloblastoma showing follicles (F) Figure 14.8 Follicular ameloblastoma showing palisading,
of varying size and shape. They are lined by tall columnar reverse polarity and basal vacuolation of ameloblast like cells
ameloblast like cells (A). Centrally stellate reticulum like cells surrounding stellate reticulum like cells (High power)
(S) are seen. Sometimes cystic degeneration (CS) may be seen
(Courtesy: Dr Raju Ragavendra T)
Figure 14.9 High power view of follicular ameloblastoma

showing palisading, reverse polarity and basal vacuolation of
Figure 14.7 Follicular ameloblastoma showing follicular ameloblast like cells surrounding stellate reticulum like cells
spaces (Courtesy: Dr Raju Ragavendra T. Department of Oral Pathology,
People Dental Academy, Bhopal, Madhya Pradesh, India)
Thus, the peripheral cells resemble preameloblasts and

they are called as ameloblast like cells. Plexiform Ameloblastoma
Cells that are present at the center of islands are (Figs 14.11 and 14.12)
star shaped, with interconnecting delicate cytoplasmic In this variant epithelium proliferates in the form of
processes. These are called as stellate reticulum like cells. interconnected cords of epithelial cells. These chords
Sometimes, there is degeneration in central cells resulting create a network like structure. These chords are lined by
microcystic spaces. ameloblast like cells.
These cystic spaces may enlarge exerting pressure on Stellate reticulum like cells are present in the center.
the peripheral cells and making the cubical to flat cells. Stellate reticulum like cells are not as prominent as follicular
310
Figure 14.10 Follicular ameloblastoma Figure 14.12 Plexiform ameloblastoma (Courtesy: Dr Aparna
Thombre, Reader, Department of Oral Pathology, VSPM Dental
Acanthomatous Ameloblastoma
(Figs 14.13 and 14.14)
This variant closely resembles the follicular ameloblastoma.
Here, stallate reticulum like cells shows squamous meta-
plasia. They loose there star shape and become polygonal.
They produce parakeratin at the central portions of island.
Sometimes, it is present in the form of keratin pearls.
Granular Cell Ameloblastoma (Fig 14.15)

This is also shows follicular arrangement of cells. This
shows transformation of stellate reticulum like cells into
granular cells.
Sometimes, a layer of stellate reticulum may be present
Figure 14.11 Plexiform ameloblastoma showing cords (C) of at the periphery of granular cells. These granular cells
ameloblastic cells creating network like structure in a loose may be cuboidal, columnar or rounded. The granular cells
stroma (L). Cells at the center resemble stallate reticulum (S) contain no cell organelle except the presence of coarse
eosinophilic granules and nucleus is displaced to periphery
ameloblastoma. Sometimes ameloblast like cells are lined of cells.
back to back with less or no stroma in-between. Supporting The granules pack the cytoplasm and distend it. The
stroma tends to be loosely arranged and myxoid. It shows nature of granules is not fully understood. Ultrastructural
tendency towards cystic degeneration, which is unlike studies indicate that these are lysosomal aggregates. Other
follicular type where cystic degeneration is seen mainly in studies indicate that the granularity is caused by increased
the epithelium. apoptotic cell death and associated phagocytes by
The growth pattern of plexiform ameloblastoma neighboring neoplastic cells. Previously it was thought that
resembles that of dental lamina stage of tooth development granular cell ameloblastoma are very aggressive tumors
before enamel organ mophodifferentiation. Differentiation but this notion is not accepted now.
towards bud stage of tooth development may be evidenced There are a few reported cases of plexiform granular
by rounded nodules of epithelium. cell ameloblastoma.
Odontogenic Tumors
311
Figure 14.13 Acanthomatous ameloblastoma showing squ Figure 14.15 Ameloblastoma showing foci of granular cells
amous metaplasia (SM) of stellate reticulum (SR) like cells in (G) in place of stellate reticulum like cells (SR). (Courtesy:
the connective tissue (CT) Dr Sangamesh Halawar)
Figure 14.14 Acanthomatous ameloblastoma showing Figure 14.16 Basal cells ameloblastoma
squamous metaplasia of central stellate reticulum cells
Basal Cell Ameloblastoma Clear Cell Ameloblastoma

(Figs 14.16 and 14.17) Some ameloblastoma may contain cells without eosinophilic
cytoplasm and appearing clear. Clear cells are usually present
This type of ameloblastoma shows a close similarity to
in place of stellate reticulum like cells. These cells contain
basal cell carcinoma. The cells are arranged in sheet like high amount of glycogen, which results in clear appearance.
pattern rather than islands. All cells including stellate Clear cell ameloblastoma are very aggressive tumors.
reticulum like cells have a basaloid appearance.
This type of ameloblastoma has highest proliferative Keratoameloblastoma
rate than any other type of ameloblastoma, leading to It consists of keratin filled cystic spaces and solid
formation of immature cells which have basal cell like proliferation of epithelial cells. Cysts are lined by
appearance. The typical cellular morphology is often parakeratotic epithelium.
altered, with ameloblast like cells appearing low columnar The basal layer consists of cuboidal cells, and the rest
to cuboidal. There is a loss of reverse polarity and of the layers of the epithelial lining show loosely arranged
subnuclear vacuolation. But cells retain palisading. epithelial cells. Cysts resemble odontogenic keratocyst.
Marginal resection (Block resection): It is surgical

removal of an intact tumor, with a rim of uninvolved bone.
This procedure usually implies maintaining the continuity
312 of the inferior or posterior borders of the mandible. Small
solid or multicystic ameloblastomas of the body of the
mandible are treated in this way.
Segmental resection: Surgical removal of a segment of the
mandible or maxillary without maintaining the continuity
of the bone.
Peripheral osteotomy: It is a procedure which allows
complete excision of the tumor but at the same time, a part
of bone is retained which preserves the continuity of the
jaw.
Figure 14.17 Basal cell ameloblastoma showing presence of Peripheral ameloblastoma: Peripheral ameloblastomas
basaloid cells (B) are relatively innocuous lesions that should be excised with
a small margin of normal tissue, and the surgical site re-
The stroma of the tumor consists of dense collagenous examined periodically.
fibrous connective tissue septa. Solid component of tumor
consists of large solid masses of parakeratin and keratin, Points to Remember
surrounded by multilayered squamous epithelium with Molar ramus area, teeth in involved region are displaced,
peripheral, palisaded, cuboidal basal cells. gradually increasing facial asymmetry, eggshell
Epithelium of solid areas differs from cystic areas. crackling, non-tender, fusiform enlargement, maxillary
It is not of uniform thickness, and there is separation lesion are more dangerous, area of bone destruction
and edema between the basal cells and the rest of the which is well defined, honeycomb appearance, soap
epithelium. Although the basal cells are palisaded and bubble appearance, eggshell of bone:
demonstrate nuclear polarization, they are cuboidal rather • Follicular ameloblastoma: Form of islands,
than columnar. palisading arrangement, reverse polarity, ameloblast-
Keratoameloblastoma is believed to be a link be- like cells, delicate cytoplasmic processes
tween a cystic neoplasm-odontogenic keratocyst and a • Plexiform ameloblastoma: Interconnected chords of
solid neoplasm having same origin. In few cases, epithe- epithelial cells, stellate reticulum-like cells, cystic
lial cells surrounding the cystic spaces show papillary degeneration
projections. Such Keratoameloblastoma are called as • Acanthomatous ameloblastoma: Stellate reticulum
papilliferous keratoameloblastoma. In few cases, papil- like cells shows squamous metaplasia, parakeratin at
liferous ameloblastoma exist independent of Keratoa- the central portions of island
meloblastoma. • Granular cell ameloblastoma: Transformation
of stellate reticulum-like cells into granular cells,
Management lysosomal aggregates
Surgical enucleation and curettage with a rim of • Basal cell ameloblastoma: Sheet like pattern rather
peripheral normal tissue is usually carried out. Marx and than islands, immature cells
others 16 reported unpublished data from an analysis of • Clear cell ameloblastoma: Cells without eosinophil-
34 mandibular ameloblastoma, which showed that the ic cytoplasm appearing clear
tumor extended 2.3 to 8.0 mm beyond the radiographic • Keratoameloblastoma: Keratin filled cystic spaces,
margin. As a result, they have recommended resection of dense collagenous fibrous connective tissue septa,
1 cm of normal appearing bone beyond the radiographic separation and edema between the basal cells and the
margin which could be the reason for the ill-defined rest of the epithelium
radiographic borders and the high recurrence rate after • Management: Surgical enucleation, curettage,
curettage. marginal resection (Block resection).
Odontogenic Tumors
VARIANT OF AMELOBLASTOMA pattern and indistinct borders. Displacement of teeth can

be seen.
Desmoplastic Ameloblastoma
Desmoplastic ameloblastoma is different from other types
Histopathological Features 313
of solid multicystic ameloblastoma, so it is thought to be a Histologically, desmoplastic ameloblastoma are non-
separate clinico-pathological entity of ameloblastoma and encapsulated tumors. They show presence of large amount
not just histologic subtype. of collagen fibers (Desmoplasia) (Figs 14.18 and 14.19).
Eversole and others (1984) described 3 cases of a variant This extensive collagenous stroma contains small
of ameloblastoma. The characteristic feature was extensive islands and nests of odontogenic cells. These islands are
stromal desmoplasia with small nests, cords and strands of compressed by the collagenous dense stroma. The follicles
odontogenic epithelium. This variant was included later in tend to be morphologically irregular due to compression.
the World Health Organization’s Histopathological Typing Peripheral cells are mostly cuboidal, columnar cells are
of Odontogenic Tumors (1992). rarely seen.
It differs clinically, radiologically and histologically
from classic ameloblastoma.
Clinical Features
The incidence of desmoplastic ameloblastoma is low; rates
of 0.9 to 12.1 percent of all ameloblastoma have been
reported. It has been found more in Chinese, Malaysian
and Japanese population:
Age and sex distribution: It is seen in patients aged 18
to 70 years, with a mean of 41.2 years. It occurs in older
patients than conventional tumor. No difference between
sexes has been reported.
Location: Desmoplastic ameloblastoma is characteris
tically seen in anterior maxilla.
Sign and symptoms: It presents as a gradual tumor mass Figure 14.18 Desmoplastic ameloblastoma showing com-
pressed follicles (F) due to deposition of large amount of col-
in the initial stages which soon acquires an aggressive
lagen fibers in the connective tissue (Desmoplasia, D)
rapidly growing form the maxilla is expanded anteriorly
and obvious facial asymmetry is noted. Ameloblastoma
of the posterior maxilla is dangerous because of its close
proximity to the orbit, pterygomaxillary fossa and cranium
and due to the difficulty in achieving an adequate surgical
margin. Intracranial extension can be lethal. The entire
maxilla possesses a thin cortical plate that offers little
resistance to the tumor, thereby enhancing its rapid spread
into the adjacent vital structures.
Radiologic Features
The radiological appearance is also different than
conventional ameloblastoma. Radiologically it appears as
a fibro-osseous lesion.
It occurs mainly in the anterior region of the jaws and
appears radiographically as a diffuse, mixed radiolucent–
radiopaque lesion with a honeycomb or soap bubble Figure 14.19 Desmoplastic ameloblastoma
Desmoplastic ameloblastoma must be histologically

differentiated from ameloblastic fibroma, odontogenic
fibroma and squamous odontogenic tumor. As the tumor
314 is non-encapsulated, the cells infiltrate between the
trabeculae of the cancellus bone leaving them intact for
some time. Thus, the tumor actually extends beyond the
radiographic margin, It has been suggested that an altered
intracellular distribution of collagen XVII and consequent
loss of critical cellular attachments may contribute to
the infiltrative and progressive growth characteristics of
ameloblastoma.
Points to Remember
Gradual tumor mass, expanded anteriorly, entire maxilla
possesses a thin cortical plate, mixed radiolucent– Figure 14.20 Peripheral ameloblastoma of follicular type
radiopaque lesion with a honeycomb or soap bubble showing stellate reticulum (SR) and ameloblasts like cells (AC)
pattern, Desmoplasia, non-encapsulated tumors, (Courtesy: Dr Sangamesh Halawar)
cuboidal, columnar cells, cells infiltrate, trabeculae of
the cancellous bone.
Management
Extraosseous/Peripheral Ameloblastoma Local surgical excision: Patient respond well to this
It is a tumor which occurs in the soft tissue outside and Recurrence: Peripheral lesion is relatively innocuous,
overlying the alveolar bone. It originates from either the lacks persistent invasiveness and has a limited tendency of
surface epithelium or the remnants of dental lamina. recurrence.
Clinical and Radiological Features Points to Remember

Location: It is seen on the mandibular gingiva in the molar- Appears nodular on gingiva, evidence of pressure
premolar region, buccal mucosa and maxillary tuberosity resorption, histologically, it exhibits the same pattern
area. as seen in intraosseous ameloblastoma, local surgical
excision.
Symptoms: The patient complains of a mass which is
growing in size and interfering with speech, mastication
and esthetics. Pituitary Ameloblastoma
It is also called as craniopharyngioma or Rathke’s pouch
Sign: Mass is mildly tender on palpation at times ulcerated
tumor.
and bleeding may occur on slight touch.
Origin: It occur in anterior lobe, which is of ectodermal
Appearance: The lesion appears nodular on gingiva
origin. The lobe is derived from Rathke’s pouch, an
varying in size from 3 to 2 cm in diameter.
outgrowth of oral ectoderm. The pouch give rise to
Radiological features: Sometimes underlying bone may craniopharyngeal duct, which in due course degenerates
exhibit evidence of pressure resorption in the form of itself leaving residues of squamous epithelial cells. From
saucer-shaped depression beneath the tumor.This is called these squamous epithelial cells ameloblastoma like tumor
as cupping resorption. develops.
Histopathological Features Age: It is most common in childhood and adolescents

before the age of 25 years.
Tumor may exhibit in one or more areas, continuity with
the surface epithelium. Histologically, it exhibits the same Location: It may be located within the sella tursica
pattern as seen in intraosseous ameloblastoma (Fig. 14.20). or commonly in suprasellar space. It grows as a
Odontogenic Tumors
pseudoencapsulated mass causing pressure effect and often Pathogenesis of Unicystic Ameloblastoma
destroys the pituitary gland.
Three hypothesis are put forward.
Symptoms: It is related due to destruction of pituitary In dentigerous cyst lining basal cells undergoes 315
gland (diabetes insipidus) or due to compression of nearby transformation into ameloblast like cells, which proliferate
cranial nerve. either into the lumen or connective tissue forming nodules
of ameloblastoma.
Histopathological features: Columnar cells in
The follicles of ameloblastoma undergo degeneration
craniopharyngioma have central nuclei, longitudinal
leading to formation of a cavity or microcystic space,
cytoplasmic fibrils. The central areas of the trabeculae of
which enlarges forming large cystic space.
cells that compose the tumor consist of squamous cells
Unicystic ameloblastoma arises de novo.
showing intercellular bridges, but degeneration and edema
are common and produces an appearance superficially Clinical Features
similar to stellate reticulum. Frequent presence of calcified
Unicystic ameloblastoma accounts for 10 to 15 percent of
material and keratinization in pituitary ameloblastoma.
all extraosseous ameloblastoma. This variant is believed to
be less aggressive.
Points to Remember
Craniopharyngioma, destruction of pituitary gland, Age: Unicystic ameloblastomas are most often seen in
central nuclei, longitudinal cytoplasmic fibril, squamous young patients, with about 50 percent of such tumors
cells showing intercellular bridges. diagnosed during the second decade of life.
Location: More than 90 percent of unicystic
Adamantinoma of Long Bones ameloblastomas are found in the mandible, usually in the
Origin: It is derived from epithelial rests misplaced during posterior region.
the course of development. Trauma causing implantation of Symptoms: The lesion is often asymptomatic, although a
epithelium with subsequent tumor formation may cause it. large lesion may cause painless swelling of the jaws.
Site: It occurs in tibia, ulna, femur and occasionally in
others bone. Histological Subtypes Unicystic Ameloblastoma
• Luminal
Clinical course: Some lesions behave aggressively and • Intraluminal
sometimes are metastasizing. • Intramural.
Histopathological features: It bears a superficial
microscopic resemblance to ameloblastoma of jaws. Histopathological Features (Figs 14.21 to 14.24)
Based on the character and extent of tumor cell proliferation
Points to Remember within the cyst wall, several histologic subtypes of unicystic
Trauma causing implantation of epithelium, aggressive, ameloblastoma are recognized.
superficial microscopic resemblance. Three histopathological variants of unicystic amelo
blastoma have been described, i.e. luminal unicystic
Unicystic Ameloblastoma ameloblastoma, intraluminal ameloblastoma and mural
It is cystic variant of ameloblastoma. It is thought to unicystic ameloblastoma.
develop from dentigerous cyst. Unicystic ameloblastoma, Luminal type: In the first type, the tumor is confined to
is a variant of ameloblastoma first described by Robinson luminal surface of cyst; while the lesion consists of fibrous
and Martinez in 1977, though refers to those cystic lesions cyst wall, with a lining that consists partially or totally
that show clinical and radiologic characteristics of an of ameloblastic epithelium. This demonstrates a basal
odontogenic cyst but in histologic examination show a layer of columnar or cuboidal cells with hyperchromatic
typical ameloblastomatous epithelium lining part of the nuclei that show reverse polarity and basilar cytoplasmic
cyst cavity, with or without luminal and/or mural tumor vacuolization. The overlying epithelial cells are loosely
proliferation. cohesive and resemble stellate reticulum.
316
Figure 14.21 Mural ameloblastoma showing cystic lining Figure 14.24 Unicystic ameloblastoma (Intraluminal)
In the second microscopic variant (intraluminal

unicystic ameloblastoma), one or more nodules of
ameloblastoma project from cystic lining into lumen
of cyst. These nodules may be relatively small or they
largely fill the cystic lumen. In some cases, nodules of
tumor that project into lumen demonstrate an edematous
plexiform pattern that resembles plexiform pattern seen in
conventional ameloblastoma. These lesions are referred to
as plexiform unicystic ameloblastoma.
In the third variant (mural unicystic ameloblastoma),
the cystic fibrous wall is infiltrated by of typical follicular
or plexiform ameloblastoma islands. The extent and depth
of ameloblastic proliferation may vary considerably.
Management
Figure 14.22 Unicystic ameloblastoma showing proliferative
While the first two groups of lesions may be treated
epithelial lining
successfully by enucleation or curettage, it has been
suggested that recurrence following conservative surgery
is more likely to occur in the third group and that these
lesions should therefore be treated in the same manner as
solid ameloblastoma.
Its response to enucleation or curettage is more favora-
ble than the classic solid or multicystic ameloblastoma.
Points to Remember
Ameloblastomatous epithelium, asymptomatic, luminal
unicystic ameloblastoma, intraluminal amelo blastoma
and mural unicystic ameloblastoma, in luminal type
basal layer of columnar or cuboidal cells, in intraluminal
unicystic ameloblastoma more nodules of ameloblastoma
project from cystic lining into lumen of cyst, in mural
unicystic ameloblastoma typical follicular or plexiform
Figure 14.23 Unicystic ameloblastoma (Luminal) ameloblastoma islands.
Odontogenic Tumors
SQUAMOUS ODONTOGENIC TUMOR

It is well differentiated odontogenic tumor composed
of islands or sheets of squamous epithelium that lack 317
recognizable features of enamel organ differentiation. It is
locally infiltrative odontogenic neoplasm.
Peter and Reichart Definition

It is a benign but locally infiltrative neoplasm consisting
of islands of well differentiated squamous epithelium in a
fibrous stroma. The epithelial islands occasionally show
foci of central cystic degeneration.
Origin
The origin of squamous odontogenic tumor is not clear. It is Figure 14.25 Squamous odontogenic tumor showing islands
believed to originate from the cell rests of Malassez located containing keratinized squamous cells (S) arranged in islands
in periodontal ligament. Peripheral variety may originate in loose fibrillar stroma background (ST)
from gingival surface epithelium or from the remnants of
dental lamina.

Age and sex: There is a wide age distribution in the range
of 11 to 67 years with mean age 40 years and females are
more commonly affected than the males.
Site: It occurs with equal frequency in maxilla and mandible.
In maxilla, it occurs in incisor-cuspid area and in the mandible,
it has got a predilection for the bicuspid-molar area.
Symptoms: It is usually asymptomatic but there may
be mobility of the involved teeth, pain, tenderness to
percussion and occasionally abnormal sensation.
Radiological features: It is well circumscribed radiolucent
area which is presented as semicircular or roughly triangular
Figure 14.26 Squamous odontogenic tumor
area. Border may or may not be sclerotic.
Microcystic degeneration may be seen few islands.
(Figs 14.25 and 14.26) Intraepithelial calcification may be seen in few islands.
It is composed entirely of round to oval islands of squamous Calcifications tend to be laminar.
epithelium. Sometimes islands have irregular or cord like Globular, hyaline eosinophilic structure with the
shape without peripheral palisaded or polarized columnar islands which are not amyloid are sometime found in
cells. this tumor. The fibrous stroma of tumor contains mature
Islands contain peripheral flat to cuboidal epithelial bundles of collagen fibers, devoid of any inductive
cells. Central cells show squamous differentiation. effect.
They are of very uniform size and shape. They exhibit
no pleomorphism, nuclear hyperchromatism or mitotic Management
activity. Sometimes individual cell keratinization may be Conservative enucleation and curettage is usually curative
present. But epithelial pearl formation is not seen. with a low recurrence rate.
Points to Remember
Cell rests of Malassez, usually asymptomatic, well
318 circumscribed radiolucent area, round to oval islands
of squamous epithelium, peripheral flat to cuboidal
epithelial cells, microcystic degeneration, and globular,
hyaline eosinophilic structure.
CALCIFYING EPITHELIAL
ODONTOGENIC TUMOR
It is also called as Pindborg tumor. The calcifying epithelial
odontogenic tumor (CEOT) has been reported prior to Figure 14.27 Pathogenesis of CEOT arising from reduced
1955 under different names, such as ameloblastoma of enamel epithelium
unusual type with calcification, calcifying ameloblastoma,
malignant odontoma, adenoid adamantoblastoma, cystic
complex odontoma and as a variant of the simple amelo
blastoma.
The term calcifying epithelial odontogenic tumor
(CEOT)’ has been generally accepted and adopted by
the WHO in the first edition of Histological Typing of
Odontogenic Tumors, Jaw Cysts, and Allied Lesions,
where it was recognized as a distinct entity.
For more than 30 years the CEOT has been known
eponymously as the Pindborg tumor, named after JJ
Pindborg who described it as calcifying epithelial
odontogenic tumor in 1955. Pindborg reported few cases
of intraosseous CEOT.
The CEOT is a benign neoplasm located either
intraosseously or extraosseously. When occurring intra
osseously, which is by far the most common location; it Figure 14.28 Cell rest of malassez and cell rest of serrae can
may occasionally show local invasiveness. The tumor proliferate either into jaw bone or towards gingiva
resembles ameloblastoma for the site and the age. It forms
1 percent of all odontogenic tumors.
Clinical Features
Origin Age and sex: It is more common in men with an age range
Two hypotheses have been suggested for the pathogenesis of of 8 to 92 years with a mean age of 42 years.
the tumor. It develops from the reduced enamel epithelium Location: Both the tumors occur commonly in the
of the embedded tooth or from stratum intermedium. mandible than maxilla by a ratio of 2:1. They occur three
times in molar region than the premolar.
Pathogenesis of CEOT
Symptoms: It is asymptomatic and only presenting
The peripheral locations strongly suggested of an origin
symptom is a painless swelling. In rare cases, there is
from the cell rest of dental lamina. Another source might
associated mild paresthesia. When occurs in maxilla, it
be from the basal cells of the oral epithelium. Most
may show epistaxis, nasal congestion and head ache.
widely accepted origin in current literature is from the
disintegration of the dental lamina cell rest (Figs 14.27 and Signs: Cortical expansion occurs. Palpation will show hard
14.28). tumor with well defined or diffuse border.
Odontogenic Tumors
Peripheral CEOT: It occurs as a painless firm gingival

mass clinically diagnosed as fibroma, peripheral giant cell
granuloma. The diagnosis is confirmed histopathologically.
319
The characteristic radiographic appearance is of an
irregular, uni or multilocular radiolucent area, containing
radiopaque masses of varying size and opacity. In many
cases, especially in tumors of relative short duration,
the calcified concernments are very minute and may be
undetectable on radiographs.
Driven snow appearance: Later, it reveals a unilocular
or a multilocular cystic lesion with numerous scattered
radiopaque foci of varying sizes and density which gives it
an appearance as that of ‘driven snow (Fig. 14.29). Figure 14.30 Pindborg tumor showing epithelial cells with
prominent intercellular bridges (ICB) forming eosinophilic
Histopathological Features (Figs 14.30 to 14.36) material (EM). Few cells show dysplastic features (D)
The tumor consists of sheets, or islands of polygonal cells.

The cells have eosinophilic cytoplasm, distinct cell borders
and very conspicuous intercellular bridges.
Nuclei are pleomorphic with prominent nucleoli; cells
with giant nuclei and multiple nuclei are also present. The
pleomorphism of nuclei mimics a malignant tumor, but
as mitotic figures are rare and clinical behavior is slow
growing this possibility is ruled.
The connective tissue stroma is scanty and fibrous.
Occasionally, cells are arranged in cords and rows
mimicking adenocarcinoma.
Amyloid like material in CEOT: There is presence of
homogeneous eosinophilic substance. This protienaceous
material which has been interpreted as amyloid has also
been suggested to be comparable glycoprotein, basal
lamina, and keratin or enamel matrix.
Figure 14.31 Pindborg tumor showing presence of

Liesengang rings (LR)
Liesegang rings: There are few deposits of calcified

material which is said to be cementum like material. There
is presence of calcification in large amounts and often in
the form of Liesegang rings.
Clear cell variant: A well recognized form of this
neoplasm is the clear cell variant which exhibits a
clear vacuolated cytoplasm rather than an eosinophilic
Figure 14.29 Driven snow appearance seen in CEOT cytoplasm. The nucleus may remain round or oval in
320
Figure 14.32 Calcification seen in CEOT Figure 14.34 CEOT showing nest and islands of tumor cells
Figure 14.33 CEOT showing Liesegang ring Figure 14.35 CEOT showing sheets of odontogenic cells
with distinct cell membranes and darkly staining nuclei and
eosinophilic material
the center of the cells or be flattened against the cell

Points to Remember
membrane. In some tumors, clear cells comprise the bulk
of the tumor cells while in others, it consist of only a few Pindborg tumor, painless swelling, mild paresthesia,
scattered foci. maxillary lesion show epistaxis, nasal congestion,
cortical expansion, peripheral CEOT, uni or multilocular
Management radiolucent area, driven snow appearance, sheets, or
islands of polygonal cells, nuclei are pleomorphic with
It has limited invasive potential therefore local excision
prominent nucleoli, amyloid like material, Liesegang
with limited margins is indicated. Simple enucleation or
rings, clear cell variant.
radical resection can be done.
Odontogenic Tumors
Its clinico-pathological profile is unique among the

odontogenic tumors. It has sometimes been referred to as
the two-thirds tumor because about two-thirds occur in the
maxilla, two-thirds occur in young women (preteen and 321
teenage years), two-thirds are associated with an unerupted
tooth, and two-thirds of those teeth are canine teeth.
Definition
WHO definition: A tumor of odontogenic epithelium with
duct like structures and with varying degrees of inductive
change in the connective tissue. The tumor may be partly
cystic and in some cases the solid lesion may be present
only as masses in the wall of a large cyst. It is generally
believed that the lesion is not a neoplasm.
Figure 14.36 Calcification seen in CEOT
Types/Classification
According to Philipson and Riechart the AOT appears in
ADENOMATOID ODONTOGENIC three different clinicotopographic variants. Terminology
TUMOR OR CYST suggested for the two central tumor variants serves
single purpose of making a distinction between tumors
Adenomatoid odontogenci tumor (AOT) is composed of
having and those lacking an association with crown of an
odontogenic epithelium in a variety of histoarchitechtural
embedded tooth. These terms do not indicate or suggest
patterns, embedded in mature connective tissue stroma and
any pathologic principle.
characterized by slow but progressive growth.
The adenomatoid odontogenic tumors (AOT) or cyst Pathogenesis
(AOC) represents 3.7 percent of all odontogenic tumors
Origin of AOT is a debated issue. Initially it was thought
and was considered as one of the variant of ameloblas
that it is a variant of ameloblastoma as tall columnar cells
toma. It is much more common than ameloblastoma or the
similar to that found in ameloblastoma are also found here.
calcifying epithelial odontogenic tumor.
Because of presence of duct-like structures dual
It shows a glandular pattern of arrangement of
salivary and odontogenic origin was suggested. AOT
the pre-ameloblasts like tumor cells and was called
occurs exclusively within tooth bearing areas of the jaws.
adenoameloblastoma or adenoid ameloblastoma. Many
This indicates that this tumor is mainly odontogenic.
other different names have been used like adenoid
Transmission electron microscopy has shown that cells
adamantinoma, ameloblastic odontogenic tumor, epithe
of AOT resemble enamel organ. Immunohistochemical
lioma adamantinum or teratomatous odontoma.
staining ruled out salivary gland origin and supported
In contrast to ameloblastoma adenomatoid odonto
odontogenic nature.
genic tumor is a circumscribed lesion with slow growth.
The epithelium that probably gives rise to AOT is cell
Seventy-five percent of the cases have been reported to
rests of dental lamina. Disintegration of dental lamina
be associated with unerupted or impacted tooth, the most
gives rise to numerous epithelial remnants persisting in
common being maxillary canine.
the jaws. These rests are distributed in gingiva and few are
Adenomatoid odontogenci tumor (AOT) was first
present in Gubernacular dentis which is connective tissue
described by Steensland in 1905. The term AOT was coined
that occupies Gubernacular canal. Based on this origin
by Philipsen and Birn in 1969. AOT is histomorphologically
pathogenesis of various clinical forms of AOT can be
similar to dental organ. It is believed to be an attempt at
explained (Fig. 14.37).
enamel formation by the tumor cells. It is termed recently
as a hamartoma and not a tumor. Also recent incidences Follicular AOT: Cell rests of Gubernacular dentis start
are reported in association with dentigerous cyst linings proliferating prior to eruption of permanent tooth. As tumor
as mural nodules thus representing it as an adenomatoid growth continuous and as tooth begins to erupt contact
odontogenic cyst due to its cystic presentation. occurs between the two. This leads to the fusion between
322
Figure 14.38 Schematic diagram showing different types of

AOT. Follicular (F), extrafollicular (EXF) and peripheral (P)
Figure 14.37 Schematic diagram showing origin of AOT
from Gubernacular dentis (GD) which connect gingiva and
permanent tooth germ (PTG) and contains many and follicular Age and sex distribution: It is found in individuals
space (FS) ranging from 3 to 82 years with 70 percent occurrence in
2nd decade with an average age of 16 years. Females are
affected more than males in a ratio of 2:1.
tumor and reduced enamel epithelium of the erupting
Site: It occurs more commonly in the maxilla than in the
tooth. Sooner or later tumor completely embraces the tooth
mandible, usually in the anterior region and especially
leading to follicular radiological appearance.
in the cuspid area. It is commonly associated with an
Extrafollicular AOT: If tumor develops from cell rests unerupted tooth.
situated at the periphery of the Gubernacular dentis or if
Symptoms: Most are asymptomatic. Few present as an
eruption is not impeded by the tumor tooth may bypass
area of swelling over an unerupted tooth. Some-times it
it and erupt into the oral cavity. Thus tumor and tooth are
may expand cortical bone but is not invasive.
not in contact leading to extrafollicular AOT. It assumes
that shape of residual, globulomaxillary, lateral periodontal Signs: AOT is frequently associated with an unerupted
or radicular cyst on radiograph based on its relation to tooth in which the epithelial proliferation is confined within
tooth. AOT occurring in molars likely to originate from a connective tissue capsule that is attached to the tooth in
distal extension of dental lamina as molars do not have manner similar to the attachment of a dentigerous cyst.
Gubernacular dentis. When the tumor occurs independently of unerupted teeth
it is often encapsulated. It may cause delayed eruption of
Peripheral AOT: It is likely to originate from cell rests
tooth. The tumor causes expansion of bone and fluctuation
of Serrae. It is also possible that it develops from cell rest
may be elicited (Figs 14.39 to 14.41).
located at the peripheral most part of the Gubernacular
dentis (Fig. 14.38). Extraosseous tumor: This uncommon type usually occurs
as pink colored mass in gingiva.
Clinical Features
Some studies have reported incidence in 1.2 percent in Radiographic Features
Caucasian and 9 percent in African black patients. It It is well-demarcated mixed radiolucent or opaque lesion.
represents 3 to 7 percent of all odontotogenic tumors and is It may or may not be well circumscribed. Borders are
a developmental outgrowth of odontogenic tissue. sclerotic. Unilocular radiolucency but may contain faint to
Odontogenic Tumors
dense radiopaque foci which may be seen peripherally as

the lesion matures. Dense cluster of radiopacities appear
as small pebbles. Separation of roots or displacement of
a adjacent tooth occurs frequently (Figs 14.42 and 14.43). 323

Macroscopic (Fig. 14.44): Most AOTs are small spherical
up to 1 to 3 cm in diameter, the tumor may be or may not
be encapsulated. Cut surface shows white to yellow firm
surface with or without cystic areas containing yellowish
to brown central gelatinous material. The cut section
sometimes may be gritty which may represent poorly
formed tooth structures or an aberrant type of dystrophic
calcification.
Figure 14.39 AOT showing swelling and expansion
Figure 14.40 AOT showing extraoral swelling Figure 14.42 AOT showing radiolucency
Figure 14.41 AOT appears as a swelling associated with an Figure 14.43 AOT radiograph showing inverted pear shaped
impacted canine. Over retained deciduous canine can be radiolucency associated with impacted canine. (Courtesy: Dr
seen. (Courtesy: Dr Avadhoot Avadhani) Avadhoot Avadhani)
324
Figure 14.44 AOT gross specimen showing soft tissue mass Figure 14.46 AOT showing many rosette pattern and duct-
around impacted canine (Courtesy: Dr Avadhoot Avadhani) like structure
Figure 14.45 AOT shows many duct like spaces (D) lined by Figure 14.47 Different types of cells and their location in a
single cells and rosette patterns (R) duct-like pattern
Tumor shows fibrous capsule of variable thickness. the tumor cells arranged in concentric circles each having
The histologic appearance show remarkably identical eosinophilic material sandwiched between the epithelial
histologic features. The tumor may be partly cystic but layers. The space between the duct-like spaces and rosette
mostly presents as a solid lesion. patterns is filled by spindle shaped cells.
The tumor shows highly cellular nodules arranged in The tumor cells are characteristically preameloblasts
small nest or island like pattern. There is prominence of like cells and the eosinophilic material is believed to
duct-like spaces or duct-like arrangement of the tumor cells be enamel matrix. The stromal component exhibits
lined by single or double row of low columnar cells which fibrocellular tissue characteristically composed of focal
are reversely polarized (the nuclei are placed away from areas of eosinophilic matrix material similar to dentinoid.
the duct like spaces or lumen). These eosinophilic uncalcified amorphous materials
The lumens of the duct may be empty or contain eosin- are referred to as Tumor droplets.
ophilic material. Focal areas show whorled appearance These eosinophilic tumor droplets are suggested by
forming Rosette pattern. Rosette is a nodular appearance of histochemical studies similar to amyloid. The tumor nests
Odontogenic Tumors
prominent nucleoli. These are present in solid nodules,

rosette patterns and duct-like structures.
Cell type II: These cell surround the epithelial nodules. 325
These are spindle shaped cells with dark eosinophilic
cytoplasm. Nucleus is oval and hyperchromatic. Long axis
of these cell are perpendicular to type I cells.
Cell type III: These are islands or nodules of polygonal
cells having squamous appearance. They contain prominent
intercellular bridges. These cells surround the type II cells.
Management
Adenomatoid odontogenic tumor (AOT) is a small
circumscribed tumor and can be successfully managed by
Figure 14.48 AOT showing duct-like spaces conservative surgical enucleation and curettage in contrast
to the aggressive treatment regime for ameloblastoma.
Prognosis is very good and is and the recurrence rate is
are supported by scanty connective tissue stroma and some
minimal up to 0.2 percent.
spindle cells can be identified surrounding these ducts like
spaces. Calcified material is common throughout the tumor Points to Remember
which usually is seen at junctions between aggregates of
epithelial cells. These calcified material have been shown Cuspids area in maxilla, area of swelling over an
to resemble enamel, pre-enamel or dentin. unerupted tooth, delayed eruption of tooth, extraosseous
tumor, well-demarcated mixed radiolucent, dense
Immunohistochemical studies: Classical AOT has shown cluster of radiopacities appear as ‘small pebbles’,
positivity for CK 5, CK17 and CK19. The classical AOT fibrous capsule of variable thickness, highly cellular
is negative for CK4, CK10, CK13 and CK 18. Crivelini et nodules arranged in small nest or island like pattern,
al (2003) has detected expression of CK14 indicating its single or double row of low columnar cells, reversely
origin from reduced enamel epithelium. Few studies have polarized, whorled appearance forming Rosette pattern,
reported positive reaction for Amelogenin in limited areas preameloblasts like cells, tumor droplets, conservative
in AOT. surgical enucleation.
Takahashi et al observed positive staining for ion
binding proteins (Transferrin, Ferritin) and Gao et al studied
MIXED ODONTOGENIC TUMORS
bone morphogenic protein (BMP) in AOT. BMP showed
positive reaction in compound odontome dentinoma and Mixed odontogenic tumors consist of tumors where both
cementifying fibroma whereas it was a negative reaction epithelium and connective tissue proliferate. The epithelial
with AOT, ameloblastoma and CEOT. component resembles ameloblast like cells. Connective
tissue resembles dental papilla. The clinical features,
Unusual Variants of AOT radiological features and to some extent histopathological
Sometimes AOT and CEOT like may co-exist. In some features overlap. These tumors are seen more commonly
cases AOT is found in the wall of CEOC and few cases second decade of life. Radiologically produce radiolucency
of AOT with melanin pigmentation are reported. AOT can containing radio opacities. The treatment and prognosis is
be present along with odontoma (Adenomatoid odontoma). almost same for these. Thus these tumors are thought to be
interrelated. Two hypothesis are proposed in this regard.
Cells of AOT
Cells of AOT are divided into three types based on CONTINUUM CONCEPT
morphology. (CAHN AND BLUM)
Cell type I: These are cuboidal or columnar cells with pale According to this concept ameloblastic fibroma is the tumor
cytoplasm. They contain vesicular nucleus with one or two that develops during active odontogenic and it develops
similar tooth, i.e. over the time it matures and finally forms Key Points of Continuum Concept
odontoma. During maturation there is gradual formation ∙∙ Proposed by Cahn and Blum in 1952
of dental hard strucutures. Initially there is formation of ∙∙ Stated that Ameloblastic Fibroma will, overtime mature
326 dentin like structure. This is called as ameloblastic fibro- and finally result in the formation of odontoma
dentinoma. Later enamel also forms leading ameloblastic ∙∙ Drawbacks:
fibro-odontoma. As maturation progresses fibrous compo ∙∙ Residual and recurrent ameloblastic fibroma never
nent reduces and calcified structures increase. This stage is show further steps of differentiation and maturation
called complex odontoma. This matures to form compound into dental hard tissue formation
odontoma. ∙∙ Ameloblastic fibroma known to occur at ages beyond
But this theory is not accepted completely. There are completion of odontogenesis (beyond 20 years).
various reasons for this.
When residual tumor of ameloblastic fibroma is left in
AMELOBLASTIC FIBROMA
the jaw for long time it is observed that it does not develop
into other type of odontogenic lesion. Ameloblastic fibroma is mixed odontogenic tumors in
Few odontoma develop well after tooth formation. which both epithelial components as well as mesenchy-
mal components proliferate. These cells resemble early
Philipsen and Reichart Theory functional ameloblasts and primitive mesenchymal com-
But it is clear that odontoma and other lesions cannot exist ponents of the dental papilla. It is believed to be precursor
de novo. They must pass through an initial stage where of other mixed odontogenic tumors. If this tumor is left
there is absence of any calcification. This led Philipsen undisturbed, it will ultimately differentiate into other le-
and Reichart put forth another theory where they propose sions terminating in development of compound odontoma.
existence of two lines (Table 14.5). There are several synonyms for this such as fibrous
adamantinoma, soft odontoma, soft mixed odontoma and
Line I: This is neoplastic line, consisting of ameloblastic
fibroadamanblastoma.
fibroma occurring after tooth formation. This does not
undergo any maturation. Pathogenesis
Line II: It is also called as hamartomatous line. This Ameloblastic fibroma has odontogenic origin. It resembles
includes the ameloblastic fibroma that occurs during tooth normal tooth germ just before formation of hard structures.
formation. This matures over the time to form complex There is failure or alteration in epithelial mesenchymal
odontoma. But this line excludes compound odontoma. It interactions leading to failure of hard tissue formation.
is believed that this lesion has a different pathogenesis than Ameloblastic fibroma exists in two forms. One is
complex odontoma. neoplastic variety that develops in adults after formation of
Thus it is believed that few mixed odontogenic tumors tooth is over. Other is hamartomatous variety that develops
are interrelated. They start their develop from ameloblastic during tooth formation. This is more common.
fibroma follow stages similar to development of tooth.
Complex odontoma is terminal stage after which there is Clinical Features
no further growth. Growth of these mixed tumors stop after Age and sex distribution: Most frequently observed
some time and does not progress further. during first two decades of life with 40 percent of patients
under the age of 10 years. Average age of occurrence is
14.8 years. Few cases are seen in adults as old as 60 years.
There is slight predilection for occurrence in males. Male
Table 14.5 Two lines of mixed odontogenic tumors
to female ratio is 1.4:1.
Hamartomatous or Neoplasitc line
Line I Line II Site: Developed in premolar molar area of the mandible.
Ameloblastic fibroma Ameloblastic fibroma Three times more common is mandible than in maxilla.
Ameloblastic fibrodentinoma Symptoms: It is painless and expands slowly. There is
Ameloblastic fibro-odontoma bulging of the cortical plates rather than erosion through
Complex odontoma them. There is also migration of involved teeth.
Odontogenic Tumors
Signs: It enlarges by gradual expansion so that the

periphery of bone often remains smooth. It is associated
with unerupted teeth. It reaches up to an approximate size
of 1 to 8.5 cm. It has got a slower clinical growth than 327
ameloblastoma and does not tend to infiltrate between
trabeculae of bone.
It may present with cyst like area of bone destruction or
there may wide area of bone destruction. As it is usually
associated with an impacted tooth, it appears as pericoronal
radiolucency. It may be either unilocular or multilocular
and is associated with unerupted or missing tooth. Margins
are well defined often with sclerotic borders (Fig. 14.49).
Figure 14.50 Ameloblastic fibroma showing islands and
Histopathological Features Cords (C ) of odontogenic epithelium made up of ameloblasts
(Figs 14.50 and 14.51) like cells (A) and stellate reticulum like cells (S). Connective
This tumor is made up of epithelial and mesenchymal tissue resembles dental papilla (EM). A cell free zone is seen
around the islands (CFZ)
components
Epithelial components: These are arranged in form islands
cords and stands. Islands show presence of tall columnar
cells with reverse polarity at the periphery thus resembling
the ameloblasts (Ameloblast-like cells). The central area
of islands contains star shaped cells resembling stellate
reticulum. In cords and small islands, stellate reticulum
like tissue is absent so ameloblasts like cells are present in
two layers. Cords resemble dental lamina.
Mesenchymal components: Mesenchymal compo nents
resemble dental papilla. These contain rounded or angular
Figure 14.51 Ameloblastic fibroma showing ameloblasts like

cells (Courtesy: Dr Sangamesh Halawar)
cells. There is presence of few delicate collagen fibers. One

important feature is presence of cell free zone around the
epithelial islands. This zone is believed to be due to in-
ductive effect of epithelium and contains excess basement
membrane like material.
Few ameloblastic fibroma show presence of melanin
pigmentation in lesions. Sometimes granular cells similar
to those found in granular cell ameloblastoma are found in
Figure 14.49 CT scan of ameloblastic fibroma showing mesenchymal portion. Such lesions are called granular cell
radiolucent lesion ameloblastic fibroma.
Management zone. The hard tissue is similar to dentin so it is called

as dentinoid. Rarely hard structure show dentinal tubules
Curettage can be done which has successful results in many
similar to true dentin.
328 cases. Surgical excision of lesion is carried out.
Management
Points to Remember
Surgical excision of the lesion should be done.
•
Fibrous adamantinoma, premolar molar area,
painless and expands slowly, cyst like area of bone
Points to Remember
destruction, unilocular or multilocular
•
Epithelial components: Islands cords and stands, tall Premolar molar area, painless, gradual expansion, well
columnar cells with reverse polarity, cords resemble delineated radiolucency, epithelial and mesenchymal
dental lamina portions are similar to ameloblastic fibroma, cell free
Mesenchymal components: Resemble dental papilla,
• zone.
rounded or angular cells, few delicate collagen fibers.
AMELOBLASTIC FIBRO-ODONTOMA
AMELOBLASTIC FIBRODENTINOMA It is very rare neoplasm similar to ameloblastic fibroma
It is very rare neoplasm composed of odontogenic epithelium and ameloblastic fibrodentinoma except the formation of
and immature connective tissue and characterized by the enamel like material.
formation of dysplastic dentin. It is similar to ameloblastic
Pathogenesis
fibroma showing formation of dentinoid. It is also called
as dentinoma. Continued maturation of ameloblastic fibrodentinoma
leading to formation of enamel gives rise to this tumor.
Pathogenesis
It is hamartomatous lesion of odontogenic origin. It is Clinical and Radiographic Features
considered to an intermediate stage between ameloblastic Age and sex distribution: It is observed during first two
fibroma and ameloblastic fibro-odontoma. decades of life with 99 percent of cases are seen before 20
years. Average age of occurrence is 9.0 years. It is more
Clinical and Radiological Features common in males. Male to female ratio is 1.4:1.
Age and sex distribution: It is observed during first two Site: Developed in premolar molar area of the mandible.
decades of life with 75 percent of cases are seen before 20
years. Average age of occurrence is 13.6 years. Few cases Symptoms: The most common presenting complaint is
are seen in adults as old as 60 years. It is more common in swelling and failure of tooth eruption. The maxillary tumor
males. Male to female ratio is 3:1. if large interferes with nasal respiration, eating and speech.
Site: Developed in premolar molar area of the mandible. Ameloblastic fibro-odontoma consists of elements of
Symptoms and signs: It is painless and expands slowly. It ameloblastic odontoma and is more aggressive than the
enlarges by gradual expansion so that the periphery of bone common odontoma.
often remains smooth. Radiographic features: It shows well delineated
Radiological features: It shows well delineated unilocular of multilocular radiolucency. Central portion of
radiolucency. It contains varying degrees of radio opacity, it contains radio-opaque, masses. These masses irregular in
depending on amount of hard tissue formed. It it associated shape and size. They are round and homogeneous.
with unerupted tooth both are closely associated.
Epithelial and mesenchymal portions are similar to It contains hard and soft structures. Hard tissues are present
ameloblastic fibroma. There is presence of hard tissue at the center and they are covered by the peripheral soft
around the epithelial cells. This is preceded by cell free tissue.
Odontogenic Tumors
329
Figure 14.52 Ameloblastic fibro-odontoma (Ground section- Figure 14.54 Ameloblastic fibro-odontoma (high power)
dentinoid material)
Figure 14.53 Ameloblastic fibro-odontoma (Ground section- Figure 14.55 Ameloblastic fibro-odontoma showing ameloblastic
enamel like material) follicles (A) surrounded by dentin like calcifications (D)
Hard tissue: It consists of dentin like material and enamel

Points to Remember
like material. Dentin like material is usually present at the
central portion. It is covered by enamel like material called Swelling and failure of tooth eruption, unilocular of
enamel oid. Sometimes cementum like material may be multilocular radiolucency, central portion of it contains
found. radio paque masses, dentin like material and enamel like
material, soft tissue is similar to ameloblastic fibroma.
Soft tissue: It is similar to ameloblastic fibroma. Thus
it contains epithelial islands in dental papilla like
mesenchymal tissue. ODONTOMA
It is a hamartoma of odontogenic origin in which both epithelial
Management and mesenchymal cells exhibit complete differentiation with
Surgical excision of the lesion should be carried out. enamel and dentin laid down in abnormal position.
The term odontoma was applied to wide variety of Clinical Features

lesions showing defects in hard tissue formation. But now
Age and sex distribution: Mean age of detection is
they are applied only for mixed odontogenic tumor.
330 14.8 years. Most begin to form while normal dentition is
Definition developing. There is slight predilection for occurrence in
males.
A malformation in which all dental tissues are represented, Compound type of odontome is twice as frequently
individual tissues being mainly well formed but occurring seen complex variety of odontome.
in a more or less disorderly pattern.
Site: Compound occurs in incisor, canine area of maxilla
Origin and complex occurs in mandibular 1st and 2nd molar area.
They result from extraneous buds of odontogenic Unusual situation include the maxillary sinus, inferior
epithelial cells from the dental lamina. Odontoma may border of the mandible, ramus and condylar region.
arise from any of three dental tissues, i.e. enamel, dentin Signs: It is common for a tooth or teeth to be absent from
and cementum. the arch in the presence of an odontome.
Classification Symptoms: Occasionally, it may produce expansion of

bone with consequent facial asymmetry. There may be
• Complex composite odontoma: Nondiscrete masses
evidence of swelling and infection.
of dental tissue.
• Compound composite odontoma: Multiple well Radiographic Features
formed teeth.
It appears as an irregular mass of calcified material
surrounded by narrow radiolucent bands with a small outer
Pathogenesis
periphery.
Odontoma are hamartomatous proliferation of odon
togenic origin. It is thought that local trauma, infection and Compound variety: It shows number of teeth like
genetic mutations cause this proliferation of odontogenic structures in the region of the canine. There is cluster of
epithelium. These result in unsuccessful or altered small shapeless dense masses of solid tissue having equal
ectomesenchyme interaction during early or later phases or more density, depending on the size of the mass. There
of tooth development leading to haphazard formation of may be many masses each of which has own dark line
enamel, dentin and cementum. surrounding it. If a large number of teeth are present, the
Both the epithelial and mesenchymal cells exhibit radiopaque mass is surrounded by a radiolucent line that
complete differentiation with the result that functional represents the pericoronal space of the unerupted teeth.
ameloblasts and odontoblasts form enamel and dentin. It is Borders are well defined in both the cases but vary from
laid down in an abnormal pattern because of failure of cells smooth to irregular and may have hyperostotic borders.
to reach the morphodifferentiation stage. Complex: Complex composite odontoma appears as a
Lesion is composed of more than one type of tissue, for dense radiopaque object sometimes lying in clear space.
this reason it is called as composite odontome. Density is greater than that of bone and to greater than or
In some composite odontomes, the enamel and dentin equal to the teeth (Fig. 14.56).
are laid down in such a fashion that the structure bears
a considerable anatomical resemblance to that of normal Histopathological Features (Fig. 14.57)
teeth except they are often smaller than the typical Normal appearing enamel or enamel matrix, dentin,
teeth, which have been termed as compound composite pulp tissue and cementum like tissue which may or may
odontome. not exhibit normal relation to one another. The lesion
When calcified dental tissue are simply arranged in a is surrounded by fibrous capsule which is partially
irregular mass bearing no morphological similarity even separated by the fluid; the resultant cyst is usually lined
to rudimentary tooth then that form is called as complex by squamous epithelium. There is presence of ghost cells
composite odontome. in odontoma.
Odontogenic Tumors
ODONTOAMELOBLASTOMA
It is also called as ameloblastic odontomas. It is a very
rare mixed odontogenic neoplasm characterized by the 331
simultaneous occurrence of an ameloblastoma and a
compound or complex odontoma in the same tumor mass.
Pathogenesis
The pathogenesis of odonto-ameloblastoma is unknown.
One possible explanation is that the mineralized dental
tissues are formed as a hamartomatous proliferation in
response to inductive stimuli produced by the proliferating
epithelium over the mesenchymal tissue.
Other is two tumors developing separately and coming
Figure 14.56 Radiographic appearance of odontoma
closer to collide each other (Collision tumor).

Age and location: It is seen in younger individual and
either jaw can be affected.
Sign and symptoms: There is pain, delayed eruption,
expansion of affected jaw.
Radiological features: It is radiolucent process consisting
of calcified material.
Histopathological Features (Fig. 14.58)

The epithelial proliferation form islands or intermingled
cords that produce the follicular or plexiform patterns
typical of ameloblastoma.
Unlike conventional ameloblastoma, these induce the
production of mineralized dental tissues on the adjacent
Figure 14.57 Odontoma showing well formed dentin (D) and
enamel (Enamel space, ES)
Management
Mass has to be removed if it is causing periodontal diseases.
Points to Remember
Epithelial cells from the dental lamina, hamartomatous
proliferation of odontogenic origin, compound occurs
in incisor, canine area of maxilla, complex occurs in
mandibular 1st and 2nd molar area, tooth is absent,
expansion of bone, teeth like structures in compound
variety, borders are well defined, dense radiopaque
object sometimes lying in clear space in complex variety,
normal appearing enamel or enamel matrix, dentin, pulp
Figure 14.58 Odontoameloblastoma showing dentinoid (D)
tissue cementum like tissue, ghost cells.
and ameloblastic follicle (AF)
mesenchymal cells and may respond to this change with are well defined and sclerotic. Larger lesions cause root
the production of enamel. divergence and resorption.
332 Management Histopathological Features (Fig. 14.59)

It should be treated in same manner as that of ameloblastoma. Odontogenic fibroma are composed of fibrous tissue
of variable cellularity and density; variable amount of
Points to Remember inactive appearing odontogenic epithelium; and variable
Collision tumor, pain, delayed eruption, expansion of presence of calcifications resembling dysplastic dentin,
affected jaw radiolucent process consisting of calcified cementum-like tissue or bone. Mesenchyme also varies in
materiae, follicular or plexiform patterns, mineralized fibrosis. Few lesions are highly fibrous while few contain
dental tissues on the adjacent mesenchymal cells. less collagen fibers. Few lesions contain myxoid tissue.
Cellularity is sparse to moderate. Sometimes eosinophilic
amorphous globules representing enamel matrix protein are
ODONTOGENIC FIBROMA seen. Many calcifications are seen. These are not specific.
It is fibroblastic neoplasm containing varying amounts of Epithelial islands are few to numerous. They are
apparently inactive odontogenic epithelium. It is found inactive looking and does not play any role in the growth
around the crown of unerupted tooth resembling a small of the tumor.
dentigerous cyst. But some say that it is a hyperplastic
Simple odontogenic fibroma: This lesion resembles
dental follicle and not an odontogenic tumor. It may occur
dental follicle. It contains mature fibrous tissue with
centrally or in the periphery. Peripheral variant clinically
sparsely scattered inactive odontogenic epithelial rests. It is
mimics fibroma.
composed of stellate fibroblasts with fine collagen fibrils
in which rests of odontogenic epithelium and dystrophic
Clinical types
calcification may or may not be present.
• Central odontogenic fibroma
• Peripheral odontogenic fibroma WHO type: It is more cellular fibrous tissue with collagen
fibers arranged in interlacing bundles. Odontogenic
Histological types
epithelium in form of long strands or isolated nests is
• Simple odontogenic fibroma present. In some areas, calcified material resembling
• WHO type. dysplastic dentin or cementum like material may be present.
Pathogenesis
It is said that WHO type of odontogenic fibroma originates
from periodontal ligament and simple type is originating
from dental follicle.

Age and sex distribution: It occurs more frequently
in older individual with mean age of 40 years. There is
marked female predilection.
Site: It is more common in maxilla and in anterior region.
Symptoms: It is generally asymptomatic except for the
swelling of the jaws.
Signs: It may cause localized bony expansion or loosening
of teeth.
Figure 14.59 Odontogenic fibroma showing stellate fibroblasts
Radiological features: It produces an expansile and immature collagen fibers thus resembling dental papilla
radiolucency similar to that of ameloblastoma. Margins (Courtesy: Dr Sangamesh Halawar)
Odontogenic Tumors
Management and angular cells lying in an abundant mucoid stroma. It

accounts for 3 to 6 percent of odontogenic tumors.
Treated with enucleation and curettage. Surgical excision
and usually it does not recur. Pathogenesis 333
Points to Remember Origin is not clear. Various tissues are thought to be a
Asymptomatic, localized bony expansion, expansile source this tumor. This includes dental papilla, dental
radiolucency, root divergence, fibrous tissue of variable follicle, and periodontal ligament. Other possible sources
cellularity, dysplastic dentin, simple odontogenic are fibroblastic or fibroblastic-histolytic cells.
fibroma, WHO type. The myxomatous tissue arises as a direct outgrowth of
dental papilla of tooth or as an indirect effect of odontogenic
epithelium on mesenchymal tissue. Thought to occur due
GRANULAR CELL ODONTOGENIC to degeneration of odontogenic fibroma.
TUMOR
Clinical Features
It is also called as granular cell odontogenic fibroma. It is
Age and sex: It is slightly more common in females with
very rare tumor.
an age range of 10 to 30 years.
Clinical and Radiological Features Site: Mandible is more commonly affected than maxilla
Age and sex distribution: It is more commonly seen older by a ratio of 3:1. Premolar-molar area in mandible and
than 40 years of age. It is more commonly seen in female zygoma in maxilla. Rarely tumor may appear in the
as compared to male. condylar region.
Location: It is more commonly located in premolar molar Symptoms: It is associated with congenitally missing
area of mandible. teeth. The growth rate is slow and pain is variable. There
is a hard swelling which may be sometime large enough to
Symptoms: It is usually asymptomatic and painless produce facial asymmetry.
expansion of jaw is seen
Signs: Sometimes it perforates the cortical plate producing
Radiological features: It is presented as well defined a bosselated surface (several small nodules on the surface).
radiolucency which can be unilocular or multilocular with It can invade maxillary sinus and cause exophthalmos.
some calcification.
Appearance: It may appear as fusiform swelling that may
Histopathological Features be hard and or may be covered by a layer of bone of only
eggshell.
It is composed of large eosinophilic granular cells. There
are also cords or small island of odontogenic epithelium Radiographic Features
see among the granular cells.
It may be either unilocular or multilocular. It may be mixed
Cementum like calcification is seen in this tumor.
radiopaque-radiolucent lesion. Margin are usually well
Management defined but sometimes it may be poorly defined. It may be
scalloped between the roots of adjacent teeth.
This tumor responds well to curettage
Tennis racket or honey comb appearance: Locules are
Points to Remember small and uniform with typical honeycomb appearance or
Granular cell odontogenic fibroma, painless expansion, strings of tennis racket (Fig. 14.60).
well defined radiolucency, large eosinophilic granular Soap bubble appearance: Exceptionally, fine septa cross
cells, cementum like calcification. the radiolucent area producing a soap bubble appearance.
ODONTOGENIC MYXOMA
It is made up of loosely arranged, spindle shaped and
It is also called as odontogenic fibro-myxoma, myxofibroma. stellate cells, many of which have long fibrillar processes
It is a rare locally invasive neoplasm consisting of rounded that tend to intermesh.
they are surrounded by a zone of hyalinization or cell free

zone. The intercellular substance is mucoid. The tumor
is usually interspersed with a variable number of tiny
334 capillaries and occasionally strands of collagen.
Management
Tumors may be difficult to enucleate due to their loose
consistency, therefore surgical excision is indicated.
Resection with generous amount of surrounding bone.
Points to Remember
Myxofibroma, premolar-molar area bosselated surface,
congenitally missing teeth, fusiform swelling, mixed
radiopaque-radiolucent lesion, tennis racket or honey
comb appearance, soap bubble appearance, loosely
arranged, spindle shaped and stellate cells, mucoid
Figure 14.60 Tennis racket pattern seen in odontogenic
myxoma material, zone of hyalinization.
Cementoma
It is also called as cementoblastoma or true cementoma.
It is a true neoplasm of functional cementoblasts, which
forms large masses of cementum or cementum-like tissue
on the tooth root.
Pathogenesis
It is derived from the mesenchymal cells of periodontal
ligament and cementoblasts. It evoles in three stages:
Osteolytic stage: There is periapical bone resoption. This

stage radiographically appears as radiolucent lesion.
Osteolytic-osteoblastic stage (Cementoblastic stage): It
is characterized by both breakdown of bone and formation
of cementum. Appears mixed radiolucency.
Maturation stage: This is inactive stage where calcification
increases.
Figure 14.61 Odontogenic myxoma showing
loose mucoid tissue Clinical and Radiological Features
Age and sex: It occurs most frequently under the age of 25
Collagen fibers are sparse and delicate. Some have a years and with no significant sex predilection.
tendency to form large amount of collagen fibers and have
Site: Mandible is affected three times more frequently than
been designated as fibromyxomas. The space between the
the maxilla. Mandibular first molar is the most frequently
cells and fibers is filled by large amount of mucoid material
affected tooth; other involved teeth are the mandibular
(Fig. 14.61).
second and third molars.
Some may exhibit marked cellularity and atypia and
have a more aggressive course. Few odontogenic epithelial Symptoms: Associated tooth is vital unless coincidentally
cells in form of cords and nests are present. In some tumors involved. In some cases, pain may be there.
Odontogenic Tumors
Signs: Lesion is slow growing and may cause expansion of Trabeculae are separated by marrow spaces which
cortical plates of bone. contain dialated blood vessels and few cells. At the
periphery of the lesion a soft tissue rimming consisting of
Radiographic features: There is an area of increased 335
numerous cementoblasts and multinucleated cementoblasts
density surrounded by the dark line of the fibrous capsule
is seen. This mass is usually attached to the root of the
and with a thin white line of the adjacent cortical layer of
tooth.
the bone.
Management
It consists of surgical extraction of tooth with attached
Gross specimen shows troth attached to the mass of
calcified mass.
cementum (Fig. 14.62).
Cementoma contains trabeculae of cementum which Points to Remember
are lined by plump active cemetocytes (Fig. 14.63). These
Cementoblastoma or true cementoma, tooth is vital,
trabeculae contain several basophilic lines called reversal
slow growing, increased density surrounded by the dark
lines.
line of the fibrous capsule, plump active cemetocytes,
reversal lines, multinucleated cementoblasts.
MALIGNANT TUMORS
Malignant Ameloblastoma and
Malignant Carcinoma
In some cases ameloblastoma can undergo malignant
transformation. It is occur in less than 1 percent of all
ameloblastoma. It is a rare type of tumor and diagnosis
depends upon presence of metastases, which is in some
cases is seen in lymph nodes and lungs.
Terminology
Malignant ameloblastoma are those ameloblastomas that
Figure 14.62 Gross specimen of cementoma metastasize but in which the metastatic lesion do not show
any histological difference from the primary tumor, i.e.
it show histopathological features of ameloblastoma in
primary as well as metastatic deposit.
Ameloblastic carcinoma: It is lesion similar to
ameloblastoma but that shows obvious histological
malignant transformation in the primary, in recurrence as
well in metastatic deposit.
Pathogenesis
Malignant ameloblastomas originate from solid
ameloblastoma which have been treated surgically but
recur after sometime. Because of repeated surgeries tumor
cells gain entry into blood vessels and lymph channels and
get disseminated to distant organs mainly lung where they
proliferate forming new tumor. Other sites include bones
Figure 14.63 Cementoblastoma showing excessive such as skull, vertebrae and femur, cervical lymph nodes,
cementum formation (CT) around root (R) liver, brain spleen and kidney.
Some ameloblastic carcinomas originate from pre-

existing solid or multicystic ameloblastoma. Cells of such
pre-existing tumors dedifferentiated over time forming
336 carcinoma. Some tumors originate de novo as ameloblastic
carcinoma.

Age and sex: Male are affected more commonly than
female. It occurs in 1st to 6th decade with mean age of
diagnosis 28 to 32 years.
Site: It is almost exclusively in the mandible.
Symptoms: Swelling followed by pain and/or rapid
growth.
Sign: Teeth may be displaced and loosened. Tenderness of Figure 14.64 Ameloblastic carcinoma mild pleomorphism
overlying soft tissue is present. There may be paresthesia of
nerves depending upon site, such mental nerve, infraorbital
nerve paresthesia. Points to Remember
Sites for metastasis: Lungs, spleen, kidney, lymph nodes Mandible, swelling followed by pain, displaced teeth,
and ileum are commonly site where metastasis can occur. paresthesia of nerves, well defined border with cortication,
Radiological features: It has well defined border with honey comb or soap bubble pattern, lamina dura may be
cortication, presence of crenations or scalloping in the lost, malignant ameloblastoma resemble ameloblastomas
perimeter. There may be loss of cortical boundary and of jaw, in ameloblastic carcinoma, nuclear enlargement,
breaching of the cortical boundary with soft tissue spread. hyperchromatic, mild pleomorphism, increased nucleous
It is either unilocular or multilocular giving the appearance to cytoplasmic ratio.
of honey comb or soap bubble pattern. Most of the septa are
robust and thick. Teeth may be displaced and may exhibits Primary Intraosseous Carcinoma
root resorption. Lamina dura may be lost. Bony borders It is also called as central mandibular carcinoma, primary
may be effaced or breached. The mandibular neurovascular intraosseous carcinoma, primary epithelial tumor of the
canal may be displaced or eroded. jaw, primary intra-alveolar epidermoid carcinoma and
central squamous cell carcinoma. It develops within the
Histopathological Features depth of the jaw. It is rare and may remain silent until they
Malignant ameloblastoma resemble ameloblastomas of have reached a fairly large size.
jaw from which they have metastasized. Most of the lesions
are of mixed variety or of plexiform type. Other type may Origin
also produce metastasis. Malignant transformation of epithelial lining of odonto
In ameloblastic carcinoma cells at the center become genic or non-odontogenic cyst can occur. Malig nant
condensed and hypercellular. Tumor shows cellular transformation of ameloblastoma by metastases from
features of malignancy such as nuclear enlargement, different sites and in case of maxilla may arise from
hyperchromatic, mild pleomorphism, increased nucleous primary tumor of the maxillary sinus.
to cytoplasmic ratio increased mitotic activity and The tumor arises from the cell rests of the odontogenic
abnormal mitosis. In some cases keratin pearl formation epithelium or from the epithelial remnants at the site
and individual cell keratinization may be seen (Fig. 14.64). of fusion between two embryonic processes. In normal
situation for some of the original cells of the dental lamina
Management or enamel organ to remain in the jaw long after the function
It is treated with en bloc resection. Radiation therapy and of these cells are completed. Malignant tumor may develop
chemotherapy for pulmonary metastasis. from these cells.
Odontogenic Tumors
Types
• Those arising from odontogenic cysts.
• Those arising from ameloblastoma either well 337
differentiated (malignant ameloblastoma) or poorly
differentiated (ameloblastic carcinoma).
• Those arising de novo from odontogenic epithelium
residues, either keratinizing or non-keratinizing.
Clinical Features
Age and sex distribution: There is a wide range of age
distribution with majority of cases occurring in 6th and 7th
decade of life. It is more common in males than in females
with a ratio of 2:1 and more common in mandible than in
maxilla. Figure 14.65 Primary intraosseous carcinoma
Symptoms: The early symptom is swelling of the jaw
with pain and mobility of the teeth before ulceration has
Excessive proliferation of neoplastic epithelium cells
occurred. Pathological fracture and lip paresthesia also
either in form of diffuse sheets or epithelial islands.
occurs.
Areas of acanthotic changes with epithelial pearl
Signs: There is rapid expansion and destruction of jaw bones. formation are often seen. There is presence of rim of
Tumor invades the periodontal ligament and the alveolar ameloblast like cells in the position which is usually
bone, destroying it. There may be lymphadenopathy. assumed by the basal cell in conventional squamous cell
Surface epithelium is normal. Occasionally the pulp of the carcinoma.
teeth may be invaded by neoplasm. Perforation of cortical
plate may occur. Points to Remember
Extraction of teeth result in nonhealing socket and Central mandibular carcinoma, swelling of the jaw with
sometimes tumor may protrude from the nonhealed socket. pain, rapid expansion and destruction of jaw bones, non-
healing socket, diffuse radiolucency, expansion and
Radiological Features distortion of the cortical plates, alveolar or plexiform
It presents as a diffuse radiolucency similar to other central pattern, nuclear pleomorphism and hyperchromatic,
malignant neoplasms of the jaws. Its borders become mitotic changes activity, areas of acanthotic changes.
ragged and there is no evidence of bone formation within
the tumor. Clear Cell Odontogenic Tumor or Carcinoma
There is expansion and distortion of the cortical plates
It is rare odontogenic tumor showing highly aggressive
of the jaw bone. They may cause destruction of the antral
behavior.
or nasal flows, loss of cortical outline of the mandibular
neurovascular canal and effacement of the lamina dura. Clinical and Radiological Features
Histopathological Features (Fig. 14.65) Age and sex: Most cases reported in women over 60 years
of age.
It has an alveolar or plexiform pattern with peripheral
cells of the tumor masses showing palisading arrangement, Site: Both maxilla and mandible have been involved.
thereby resembling odontogenic epithelium. It is usually
Symptoms: It may present as asymptomatic or painful
of basal cell type although on occasion, spinous cells may
bony swelling.
be found.
The tumor cells themselves generally exhibit nuclear Radiological features: Unilocular or multilocular
pleomorphism and hyperchromatic, mitotic activity. radiolucency with ill defined margins.
Histopathological Features Histopathological Features

Biphasic pattern: There are nests of epithelial cells with It usually shows well differentiated squamous cell
338 clear or faintly eosinophilic cytoplasm admixed with carcinoma.
eosinophilic polygonal cells.
Management
Monophasic pattern: There is only clear cell arranged in
nest and cords and is separated by thin strands of hyalanized It is same as with primary intraosseous carcinoma.
material.
Points to Remember
Palisading pattern: Peripheral cells may demonstrate Carcinoma ex odontogenic cyst, fistula formation,
palisading. smooth border of cyst is lost, ill defined, well differen
Mitoses are sparse and clear cells contain small amount tiated squamous cell carcinoma.
of glycogen.
Management Ameloblastic Fibrosarcoma

It is the malignant counterpart of the ameloblastic fibroma
Tumors demonstrate aggressive local behavior and potential
in which mesenchymal elements have become malignant.
lymphatic and pulmonary metastases and therefore should
Odontogenic epithelium remains benign. It is also called as
be treated with extensive resection.
ameloblastic sarcoma.
Points to Remember
Clinical Features
Asymptomatic or painful bony swelling, unilocular or
Age and sex: It is very rare and most frequently occurs in
multilocular radiolucency ill defined margins, biphasic
young adults with an average age of occurrence being 27
pattern, monophasic pattern, palisading pattern, treated
years. There is more common in males.
with extensive resection.
Site: It more frequently occurs in mandible than in maxilla.
Malignant Changes in Odontogenic Cyst Symptoms: It is uniformly painful, generally grows readily
It is also called as carcinoma ex odontogenic cyst. It is and causes destruction of bone with loosening of teeth.
uncommon but in some cases there may be chance that, Sign: There may be ulceration and bleeding of the overlying
there are carcinomatous changes found in odontogenic cyst. mucosa. Swelling is usually soft in consistency.
In some cases adjacent carcinoma may involve otherwise
unrelated cyst. Radiological Features
Clinical and Radiological Features Radiographically, radiolucencies with irregular and
indistinct margins are characteristic. Large radiolucencies
Site: It can affect any cyst in the jaw. But keratocyst is with a multilocular appearance and gross expansion and
more likely to undergo malignant changes than other cysts. thinning of the cortical bone may be seen (Fig. 14.66).
Symptoms: The most common complain is pain, which
is dull and of several months duration. If upper jaw is Histopathological Features
involved sinus pain and swelling may be present. There are no apparent remarkable changes in the
odontogenic epithelium. It remains similar to ameloblastic
Signs: Pathological fracture, fistula formation and regional
fibroma. In some lesions, it is diminished in quantity,
lymphadenopathy may occur.
apparently as a result of overgrowth of the malignant
Radiological features: Smooth border of cyst is lost mesenchymal portions of the lesion.
or becomes ill defined. In advanced lesion has an ill It is mesenchyme that exhibits malignant features.
defined, infiltrative periphery that lacks any cortication. Mesenchymal tissue exhibits a remarkable increase in
There is thinning and destruction of lamina dura of cellularity; the malignant fibroblast being polygonal to
adjacent teeth. fusiform, bizarre and pleomorphism, with hyperchromatic
Odontogenic Tumors
Clinical Features
It is a destructive lesion which produces a fleshy, bulky
growth. It as such is asymptomatic but pain may be present 339
in many cases.
It is identical with that of fibrosarcoma of non-odontogenic
origin. The cellular element may or may not be prominent
than the fibrillar component. The cells often exhibit
considerable mitotic activity.
They resemble immature fibroblasts and appear as
elongated cells containing ovoid nuclei with varying degree
of pleomorphism and are situated in a fibrous meshwork
which may or may not exhibit foci of odontogenic
Figure 14.66 Ameloblastic fibrosarcoma showing irregular epithelium.
radiolucency
Management
Radical surgical removal with resection of the jaw.
nuclei and numerous atypical mitotic figures. Stroma is
Prognosis is poor.
myxoid with less collagen fibers.
In some cases dysplastic dentin or small amount of Points to Remember
enamel can be seen. Some have called this as ameloblastic
dentinosarcoma or ameloblastic fibro-odontosarcoma. Asymptomatic, cellular element may or may not be
prominent, mitotic activity, immature fibroblasts, elong
Ameloblastic carcinosarcoma: In rare cases there is ated cells.
malignant transformation of epithelial and mesenchymal
elements in ameloblastic fibroma. This is called as
ameloblastic carcinosarcoma. BIBLIOGRAPHY
1. Casaroto AR, Toledo GL, Filho JL, et al. Ameloblastic
Management carcinoma, primary type: case report, immunohistochemical
Radical resection such as hemimandibulectomy or analysis and literature review. [Case Reports, Journal
hemimaxillectomy can be done. Recurrence is common Article, Review] Anticancer Res. 2012;32(4):1515-25.
and prognosis is poor. 2. Chen Y, Li TJ, Gao Y, Yu SF. Ameloblastic fibroma and
related lesions: a clinicopathologic study with reference to
Points to Remember their nature and interrelationship. J Oral Pathol Med. 2005.
3. Darlington CG, Lefkowitz LL. A pathologic study of “so-
Ameloblastic sarcoma, painful destruction of bone, called” dental tumors. Am J Clin Path. 1936,6:330-48.
ulceration and bleeding of the overlying mucosa, radio 4. Dayi E, Gurbuz G, Bilge OM, Ciftcioglu MA. Adenomatoid
lucencies with irregular indistinct margins, mesenchymal odontogenic tumour (adenoameloblastoma). Case report and
tissue exhibits increase in cellularity, stroma is myxoid, review of the literature. Aust Dent J. 1997.
ameloblastic dentinosarcoma or ameloblastic fibro- 5. Etit D, Uyaroglu MA, Erdogan N . Mixed odontogenic
odontosarcoma, ameloblastic carcinosarcoma, radical tumor: ameloblastoma and calcifying epithelial odontogenic
resection. tumor. Indian J Pathol Microbiol. 2010;53(1):122-4.
6. Fitzgerald GM. Multiple composite odontomas coincidental
with other timorous conditions; report of a case. J Am Dent
Odontogenic Fibrosarcoma A, I943;30:I408-I7.
It is the malignant counterpart of odontogenic fibroma. 7. Friedrich RE, Scheuer HA, Fuhrmann A, et al. Radiographic
It originates from same mesenchymal tissue, as same in findings of odontogenic myxomas on conventional radio
central fibroma. graphs. [Journal Article] Anticancer Res. 2012;32(5): 2173-7.
8. Gardner DG, Corio RL. The relationship of plexiform 18. Maria A, Sharma Y, Malik M. Calcifying epithelial
unicystic ameloblastoma to conventional ameloblastoma. odontogenic tumor: a case report: J Oral Maxillofac Oral
Oral Surg Oral Med Oral Pathol. 1983,56(1):54-60. Surg. 2010;9(3):302-6.
340 9. Gunhan O, Erseven G, Ruacan S, Celasun B, Aydintug Y, 19. Philipsen HP, Reichart PA, Zhang KH, Nikai H, Yu QX.
Ergun E, Demiriz M. Odontogenic tumours. A series of 409 Adenomatoid odontogenic tumor: biologic profile based on
cases. Aust Dent J. 1990. 499 cases. J Oral Pathol Med. 1991.
10. Gupta N, Saxena S, Rathod VC, Aggarwal P. Unicystic 20. Philipsen HP, Reichart PA. Classification of odontogenic
ameloblastoma of the mandible. J Oral Maxillofac Pathol. tumours. A historical review. J Oral Pathol Med. 2006.
2011;15(2):228-231. 21. Piloni MJ, Keszler A, Itoiz ME. “Agnor as a marker of
11. Hansen LS, Eversole LR, Green TL, Powell NB. Clear malignant transformation in odontogenic keratocysts”. Acta
cell odontogenic tumor—a new histologic variant with Odontol Latinoam. 2005;8(1):37-42.
aggressive potential. Head Neck Surg. 1985. 22. Ramesh RS, Manjunath S, Ustad TH, Pais S, Shivakumar
12. Ide F, Mishima K, Saito I, Kusama K. Rare peripheral K. Unicystic ameloblastoma of the mandible—an unusual
odontogenic tumors: report of 5 cases and comprehensive case report and review of literature. Head Neck Oncol
review of the literature. Oral Surg Oral Med Oral Pathol 2010;2:1.
Oral Radiol Endod. 2008. 23. Reichart PA, Philipsen HP, Sonner S. Ameloblastoma:
13. Kumamoto H, Ohki K, Ooya K. Association between vascular biological profile of 3677 cases. Eur J Cancer, B, Oral
endothelial growth factor (VEGF) expression and tumor Oncol. [1995]
angiogenesis in ameloblastomas. J Oral Pathol Med. 2002. 24. Thoma KH. Cementoblastoma. Internat J Orthodont, I937,
14. Kumamoto H. Molecular pathology of odontogenic tumors. 23,I127-37.
J Oral Pathol Med. 2006. 25. T Thoma KH. Oral Pathology. The CV Mosby Co, St. Louis,
15. Lawal AO, Adisa AO, Olusanya AA, et al. “Hybrid” 1941;pp.9I4-69.
ameloblastoma: a report of two cases. [Case Reports, Journal 26. Zanakis S, Maria F, Dicoglou C, Dendrinos C. Calcifying
Article] Afr J Med Med Sci. 2011;40(4):413-5. epithelial odontogenic tumor: a case report. Oral Surg Oral
16. Madras J, Lapointe H. “Keratocystic odontogenic tumour: Med Oral Pathol Oral Radiol Endod. 2011;112(6):e117-
reclassification of the odontogenic keratocyst from cyst to 20.
tumour”. J Can Dent Assoc. 2008;74(2):165-5. 27. Zhong Y, Wang L, Lit T, et al. Calcifying epithelial
17. Mahadesh J, Rayapati DK, Maligi PM, Ramachandra P. odontogenic tumor showing malignant transformation: a
Unicystic ameloblastoma with diverse mural proliferation - case report and review of the literature: Chin J Dent Res.
a hybrid lesion. Imaging Sci Dent. 2011;41(1):29-33. 2010;13(2):157-62.
1. At which stage there is no histo or morphodifferentia 4. Liesegang rings is the histopathological feature seen
tion seen: in:
a. Bud stage b. Cap stage a. Ameloblastoma b. CEOT
c. Bell stage d. Advance bell c. Cementoblastoma d. All of the above
2. Ameloblastoma which shows palisading, reverse polarity 5. Duct like spaces lined by single cells and rosette pattern
seen in:
and basal vacuolation of ameloblast like cells is:
a. AOT
a. Plexiform type b. Follicular type
b. Ameloblastic fibroma
c. Basal cell type d. Both a. and b.
c. Odontogenic fibroma
3. ‘Rathke’s Pouch tumor’ refers to: d. Myxoma
a. Squamous odontogenic tumor 6. Genes which are considered as ‘guardian of genome’
b. Ameloblastic fibroma is:
c. Pituitary ameloblastoma a. APC b. Retinoblastoma
d. Odontoma c. Ras d. p53
Odontogenic Tumors
7. Antiaging enzyme refers to: 16. Adenomatoid odontogenic tumor is most commonly
a. Telomerase b. Kinase found in:
c. Lipase d. None a. Anterior mandible b. Posterior maxilla
8. Ameloblastic follicles surrounded by dentin like c. Anterior maxilla d. Ramus of mandible 341
calcifications is the histopathological feature of: 17. Which of the following is a true neoplasm of functional
a. Compound odontoma cementoblasts:
b. Ameloblastic fibro-odontoma a. Periapical cemental dysplasia
c. Complex odontoma b. Familial cemental dysplasia
d. CEOT c. Benign cementoblastoma
9. Stellate fibroblasts and immature collagen fibers d. Hyercementosis
resembling dental papilla is the histopathological 18. Which of the following is odontogenic tumor?
feature of: a. Astrocytoma b. Arrhenoblastoma
a. Odontoameloblastoma c. Ameloblastoma d. Granular cell tumor
b. Myxoma
19. Adamantinoma is:
a. A tumor from embryonal cells of developing teeth
d. Complex odontoma
b. Also known as Ameloblastoma
10. Excessive cementum formation seen in: c. Is a complication of dentigerous cyst
a. Benign cementoblastoma d. All of these
b. Malignant ameloblastoma
20. The most common odontogenic tumor which occurs in
relation to an unerupted tooth in anterior maxilla is:
d. Complex odontoma
a. Odontogenic adenomatoid tumor
11. All of the following lesions may be classified as b. Odontoma
odontogenic tumors EXCEPT: c. Myxoma
a. Acanthomatous amelobastoma d. Cementifying fibroma
b. Myxoma
c. Branchial cleft cyst 21. Dentigerous cyst is likely to cause which neoplasm?
d. Simple ameloblastoma a. Ameloblastoma b. Adeno carcinoma
c. Fibrosarcoma d. All of these
12. Ameloblastoma most frequently occurs in:
a. Mandibular molar region 22. Radiographic finding in Pindborg tumor is:
b. Maxillary molar region a. Sun burst appearance
c. Mandibular premolar region b. Onion-peel appearance
d. Maxillary premolar region c. Driven-snow appearance
d. Cherry-blossom appearance
13. Compound odontoma shows on a radiograph as:
a. Supernumerary teeth 23. Resorption of teeth is caused by:
b. Radiolucent and radiopaque areas a. Cysts b. Benign tumors
c. Masses of calcified areas c. Malignant tumors d. All of these
d. Distinguishable tooth – like structures 24. Which of the following tumor occurs in minor salivary
14. Which of the following is the most common lesion of gland?
the mandible? a. Pleomorphic adenoma
a. Adamantinoma b. Adenocarcinoma
b. Osteogenic sarcoma c. Mucoepidemoid carcinoma
c. Squamous cell carcinoma d. Warthin’s tumor
d. Osteoclastoma 25. Ghost cells are seen in:
15. Robinson’s classification of ameloblastoma does not a. Ameloblastic fibrodontoma
include: b. Calcifying odontogenic tumor
a. Multicentric b. Nonfunctional c. Compound odontoma
c. Anatomically benign d. Clinically persistent d. All of these.
15 Cyst of Orofacial Region
Chapter Outline
Â Definitions Â Nasoalveolar cyst

Â Theories of cyst enlargement Â Median mandibular cyst
Â Dentigerous cyst Â Globulomaxillary cyst
Â Eruption cyst Â Traumatic bone cyst
Â Odontogenic keratocyst (OKC) Â Aneurysmal bone cyst
Â Primordial cyst Â Sinus mucocele
Â Gingival cyst of newborn Â Antral pseudocyst
Â Gingival cyst of adult Â Dermoid and epidermoid cyst
Â Lateral periodontal cyst Â Retention cyst
Â Glandular odontogenic cyst Â Branchial cleft cyst
Â Palatal cyst of newborn (Epstein’s pearls, Bohn’s Â Oral lymphoepithelial cyst
nodules) Â Anterior median lingual cyst
Â Calcifying epithelial odontogenic cyst Â Oral cyst with gastric or intestinal epithelium
Â Inflammatory radicular cyst Â Cystic hygroma
Â Residual cyst Â Follicular cysts of the skin
Â Inflammatory collateral cyst Â Nasopharyngeal cyst
Â Paradental cyst Â Thymic cyst
Â Mandibular buccal infected cyst Â Parasitic cyst
Â Suppurating cyst Â Hydatid cyst
Â Healingcyst Â Cysticercosis cellulose
Â Nasoplalatine cyst Â Jaw cyst-basal cell nevus-bifid rib syndrome
Â Median palatine cyst Â Treatment of cysts
INTRODUCTION which can be found from the mildest form to a greatly

disfiguring form.
Cyst has been known to arise in man (ever since he has teeth) Cysts of jaws are of great clinical importance, not
and in certain animals. All it takes is some odontogenic only because they often attain a large size and therefore
epithelium plus some unknown initiating factor which produce facial asymmetry, disturbance of dentition,
stimulates it to proliferate to cause this destructive lesion, neurological symptoms and predispose the jaws to fracture
Cyst of Orofacial Region
but particularly because they have a very high frequency of (D) Cysts of the soft tissue of the mouth, face and
occurrence. neck
∙ Dermoid and epidermoid
DEFINITIONS ∙ Branchial cleft cyst (lympho-epithelial)
343
By killey and key 1966: This entity constituted an ∙ Thyroglossal duct cyst
epithelium-lined sac filled with fluid or semifluid material. ∙ Anterior medial lingual cyst
∙ Oral cyst with gastric or intestinal epithelium
By some unknown author 1966: A cyst is an abnormal ∙ Cystic hygroma
cavity in hard and soft tissue which contains fluid, ∙ Cysts of salivary glands
semifluid, or gas and is often encapsulated and lined by ∙ Parasitic cyst, hydatid cyst, cysticercosis cellulosae.
epithelium.
By Kramer in 1974: Pathologic cavity having fluid, THEORIES OF CYST ENLARGEMENT
semifluid, or gaseous content but not always is lined by
The exact mechanism is not known, but it could be that the
epithelium.
mechanism-governing enlargement of cysts of the jaws
is the same irrespective of the type of cysts. The various
CLASSIFICATION steps involved in the formation of a cyst seem to be as
follows:
By Shear
∙ The attraction of fluid into the cystic cavity.
(A) Epithelial ∙ The retention of fluid into the cavity.
Odontogenic ∙ The production of raised internal hydrostatic pressure.
Developmental ∙ The resorption of surrounding bone with an increase in
∙ Dentigerous cyst (follicular) cyst the size of bone cavity.
∙ Eruption cyst
∙ Primordial cyst Harries Classification of Theories of Cyst
∙ Gingival cyst of adults Enlargement
∙ Lateral periodontal cyst Mural growth
∙ Calcifying odontogenic cyst. • Peripheral cell division
Inflammatory Cyst • Accumulation of cellular content
∙ Radicular cyst
Hydrostatic enlargement
∙ Residual cyst
• Secretion
∙ Inflammatory collateral cyst
∙ Paradental cyst. • Transudation and exudation
• Dialysis
Nonodontogenic
∙ Nasopalatine duct (incisive canal) cyst Bone resorbing factor
∙ Median palatine median alveolar and median man-
dibular cysts Mural Growth (Fig. 15.1)
∙ Globulomaxillary cyst
∙ Naso-labial cyst. Peripheral Cell Division
(B) Nonepithelial Peripheral enlargement is attributed to active cell division
∙ Simple bone cyst (traumatic solitary hemorrhagic of the lining epithelium in response to an irritant stimulus.
bone cyst) Cyst regression occurs following the removal of such
∙ Aneurysmal bone cyst. stimulus. The theory has been criticized on the basis that
such regression would lead to an irregularly thickened
(C) Cysts associated with maxillary antrum
inner surface because of the resistance of the surrounding
∙ Benign mucosal cyst of the maxillary antrum bone. However, this ignores the possibility that the cyst
∙ Surgical ciliated cyst of maxilla. wall is not only well supported by its fluid content but
Dialysis: The mean osmolality of the cystic fluid is 10

miliosmoles higher than that of serum. This gradient is
attributed to the accumulation of the low molecular weight
344 cells shed from the lining epithelium and maintained
by inadequate lymphatic access to the cyst lumen, the
consequence is net entry of fluid from the capsule capillaries
into the cystic lumen.
Bone Resorbing Factor (Fig. 15.3)

Vital cyst tissue in culture has been shown to release a
potent bone resorbing factor, which is predominately a
mixture of prostaglandin E2 (pgE2) and prostaglandin E3
(pgE3). The source of this resorbing factor appears to be
the capsule and leukocyte content. Prostaglandin release
is reduced in some cysts when the epithelium is removed
Figure 15.1 Cyst enlargement by active proliferation
of the cyst wall
can also actively resorb bone sufficiently and rapidly to

accommodate the expanding perimeter.
Accumulation of Cellular Content

Kramer has suggested that keratocyst enlarges by the
increasing accumulation of mural squames as they are
cast off from the living epithelium. The characteristic
finger like projections of growth represents local areas
of increased cell division. An alternative explanation for
this elongation is that keratocyst although persistent in
their growth are poor bone resorbers and simply extend
preferentially along the less dense cancellous bone with
little resorption and expansion of dense cortex. Figure 15.2 Enlargement of cyst by hydrostatic pressure
Hydrostatic Enlargement (Fig. 15.2)

Growth is attributed to the distension of the cystic wall by
fluid that has accumulated by one or more of the following
processes.
Secretion: Apart from the occasional goblet cells usually
found in follicular cyst, there is little morphological
evidence of intracystic secretion.
Transudation and exudation: These have been proposed
mainly for the enlargement of the follicular and periodontal
cyst respectively. This conclusion was derived from an
examination of the protein content and specific gravity of
the cystic fluid. The presence of fibrin and cholesterol in
periodontal and follicular cysts suggests that hemorrhage
also contributed to the cystic fluid. Figure 15.3 Cyst enlargement by bone resorption
before culture but it is not clear whether the reduction the degeneration of stellate reticulum at an early stage of
results from the removal of an epithelial inductive effect tooth development resulting into cyst formation associated
or whether it merely reflects the loss of prostaglandin with enamel hypoplasia. The fluid is pressure incites a
produced within the epithelium. proliferation of the outer enamel epithelium, which remains 345
The mechanism of prostaglandin production is attached to the tooth at the cementoenamel junction; the inner
not known. One possibility is that production takes enamel epithelium is then pressed onto the crown surface.
place in the capsule under the influence of epithelial
Extrafollicular theories/causes: This theory suggest that
proliferation, lysosomal phospholipase from fibroblasts
the cyst forms by accumulation of fluid in between the
and polymorphonuclear leukocytes; breaking down of
unerupted tooth and the reduced enamel epithelium. Other
phospholipid cell membrane to produce arachidonic acid
theory says that the crown of a permanent tooth may erupt
which is converted by the ubiquitous enzyme prostaglandin
into a radicular cyst of a deciduous tooth. As the incidence
synthetase to prostaglandin.
of radicular cyst is uncommon with the deciduous teeth,
the possibilities are rare of these type of cysts. The cyst
DENTIGEROUS CYST may occur if a impacted tooth erupts into an existing
Dentigerous cyst is the developmental odontogenic cyst odontogenic keratocyst. This incidence is uncommon; the
of epithelial origin. It is also called as ‘follicular cyst’ features are more of OKC than the dentigerous cyst (Fig.
or ‘pericoronal cyst’. It is the most common type of Schematic diagram).
odontogenic cyst which encloses the crown of an unerupted Main’s theory (1970): Impacted tooth exerts pressure on
tooth by expansion of its follicle and is attached to the neck. its follicle due to the eruptive force. This obstructs the
venous outflow and thereby induces rapid transudation of
Pathogenesis
serum across the capillary walls. The increased hydrostatic
Though a number of pathogenesis theories have put pressure exerted by this pooling of fluid causes separation
forth, the cyst has been demonstrated as originating from of the crown from the follicle, with or without reduced
the dental follicle surrounding the tooth after crown enamel epithelium. Further the osmolality of the cyst
completion. Thus it can be intrafollicular or extrafollicular. fluid is modified by various factors such as increased
Intrafollicular theories/causes (Fig. 15.4): It occurs due permeability to passage of greater quantities of proteins,
to fluid accumulation between the layer of reduced enamel increased amount of glycosaminoglycans, predominantly
epithelium, i.e. inner and outer enamel epithelium after hyaluronic acid and heparin and chondroitin sulfate in the
formation of crown. Within the enamel organ itself by cyst wall causes expansile growth rapidly.
Thus in each theory, the fluid generates the cystic
proliferation by its hyperosmolar content created by
cellular breakdown and cell products, causing an osmotic
gradient to pump fluid into the cyst lumen.
Clinical Features
Some of the asymptomatic cysts are discovered by
radiographs, when the X-rays are taken for a missing tooth
or for malaligned teeth.
Age and sex distribution: As it arises from the follicle
of an unerupted tooth, it is usually found in children and
adolescents with a higher incidence in 2nd and 3rd decades.
It is equal in both sexes.
Site: It is most commonly associated with mandibular 3rd
molars and maxillary canines which are most commonly
Figure 15.4 The cyst forms by accumulation of fluid in between
the unerupted tooth and the reduced enamel epithelium impacted. It may also be found enclosing a complex
(extrafollicular origin) compound odontome or involving a supernumerary tooth.
Size: They vary in size from a little more than the diameter
of the involved crown to an expansion that causes
progressive but painless enlargement of jaws and facial
346 asymmetry.
Teeth: Teeth adjacent to the developing cyst and involved
teeth may get severely displaced and resorbed. There may
be displacement of third molars to such an extent that it
sometimes comes to lie compressed against the inferior
border of the mandible.
Symptoms: Generally, it is painless but may be painful
if it gets infected. When dentigerous cyst expands rapidly
to compress sensory nerve it produces pain which may be
referred to other sites and described as headache.
Signs: Dentigerous cyst has a tendency to grow or expand Figure 15.6 Intraoral swelling seen in anterior region
laterally so it causes obvious buccal expansion of cortical due to dentigerous cyst
plates (Figs 15.5 and 15.6). In some cases pathological
fracture can occur. Cystic involvement of an unerupted Radiographic Features
third molar may result in hollowing out of the entire It is well defined radiolucency usually associated with
ramus extending up to the coronoid process and condyle hyperostotic borders unless they are secondarily infected
as well as the body causing expansion of cortical plates. and is seen around an unerupted tooth. Usually, it is
In the case of a cyst associated with maxillary cuspids, unilocular but some times it may appear multilocular, this
expansion of the anterior maxilla often occurs and may image is caused by ridges in the bony wall and not by the
superficially resemble acute sinusitis or cellulitis. presence of bony septa. The bony margins are well defined
and sharp (Figs 15.7 to 15.9).
Associated disease: Bilateral cysts are found in association
Associated tooth may be displaced in any direction. It is
with basal cell nevus syndrome, cleidocranial dysplasia
usual for those unerupted teeth which become surrounded
and a rare form of amelogenesis imperfecta.
by the growing cyst to retain their follicle for a time at least,
Blue domed cyst: When it contains blood, then it is called which serves to indicate that the tooth is actually outside
as ‘blue domed cyst’. the cyst.
Radiological types: It is mainly three type, i.e. central
variety (in it crown is enveloped symmetrically), lateral
type (cyst on one aspect of the crown), circumferential type
(in it the entire tooth appears to be enveloped by the cyst)
(Figs 15.10A to C).
(Figs 15.11 to 15.14)
It is composed of thin cystic wall. The lining is a thin layer
of nonkeratinized stratified squamous epithelium. In very
few instances the lining may be keratinized and it may be
mistaken as keratocyst or keratin may be produced rarely
as due to metaplastic changes. As the lining is derived
from reduced enamel epithelium it is 2 to 4 cell layer thick
primitive type of epithelium.
Figure 15.5 Dentigerous cyst associated with impacted canine The cells are cuboidal or low columnar. Retepegs
clinically showing swelling on left maxillary region formation is absent except in cases that are secondarily
347
A B
Figure 15.7 Dentigerous cyst showing radiolucency which

is well defined (Courtesy: Dr Aparna Thombre, Reader,
Department of Oral Pathology, VSPM Dental College and
Hospital, Nagpur, Maharashtra, India)
C
Figures 15.10A to C (A) Central variety; (B) Lateral variety;
(C) Circumferential variety
Figure 15.8 Bilateral dentigerous cyst
Figure 15.9 Dentigerous cyst showing well defined Figure 15.11 Dentigerous cyst with thin lining resembling
radiolucency reduced enamel epithelium with no rete pegs
348
A
Figure 15.12 Inflammation in dentigerous cyst with lining of
variable thickness. The stroma of connective tissue is organized,
fibrous and shows vascular proliferation and juxta epithelial
inflammatory infiltrate
B
Figures 15.14A and B Dentigerous cyst with secondary infec-
tion. (Courtesy: Dr Aparna Thombre, Reader, Department of
Oral Pathology, VSPM Dental College and Hospital, Nagpur,
Maharashtra, India)
Occasional islands of odontogenic epithelium are obse-

Figure 15.13 Dentigerous cyst with thin lining resembling rved. The connective tissue wall is frequently quite thick-
reduced enamel epithelium. The connective tissue is delicate as ened and composed of organized fibrous connective tissue.
of primitive type (high power)
Rushton bodies within the lining epithelium which are
peculiarly seen as linear or hairpin shaped curved bodies
infected. As the connective tissue wall is derived from the have been reported as a result of inflammation. Cholesterol
dental follicle of developing enamel organ, it is a loose clefts also may be noted.
connective tissue stroma. Young fibroblasts are present in Metaplasia in dentigerous cyst: The cell lining may show
the stroma which is rich in acid mucopolysaccharide. metaplastic changes in the form of mucous producing cells
Inflammation in dentigerous cyst: The epithelial lining or secretory cells such as goblet cells. Pseudostratified
shows variable thickness in response to inflammation. ciliated columnar epithelium also has been reported. Rarely
Budding or proliferation of the epithelium into the sebaceous glands in the walls are observed. The content of
connective tissue wall may be seen. Inflammatory cells the cystic lumen is usually thin watery yellow fluid and is
infiltrate the connective tissue mostly juxtaepithelial. occasionally blood tinged.
Management An eruption cyst is in fact a dentigerous cyst occurring

when a tooth is impeded in its eruption within the soft
Treatment of dentigerous cyst depends upon the size of the
tissues overlying the bone, whereas the dentigerous cyst
cyst. 349
develops around the crown of an unerupted tooth which is
Surgical: Smaller lesions can be surgically removed with lying in the bone. This cyst has often been termed ‘eruption
little difficulty. The larger cyst involves surgical drainage cyst’ or ‘eruption hematoma’.
and marsupialization. This procedure results in relief It is essentially a dilation of the normal tooth caused
of pressure and gradual shrinking of the cystic lesion by by accumulation of tissue fluid or blood. Cyst usually
peripheral opposition of new bone. develop due to collagen deposition in the gingival
Decompression: Small acrylic button or short section of connective tissue which results in thicker less penetrable
rubber–is placed in preformed surgical opening in cyst pericoronal roof.
which keeps opening open and permits drainage. Clinical and Radiological Features
Recurrence is relatively uncommon unless there has
been fragmentation of the cystic lining with remnants Location: In 11 percent of cases it occurs during the
allowed to remain. eruption of incisors and in 30 percent of cases it occurs
during eruption of canines and molars.
Potential Complication Appearance: Clinically the lesion appears as a
There are several potential complications besides the circumscribed, fluctuant, often translucent swelling of the
possibility of recurrence following incomplete surgical alveolar ridge over the site of eruption of the tooth.
removal. These include:
Eruption hematoma: When the circumscribed cystic
Ameloblastoma: The development of an ameloblastoma cavity contains blood the swelling appears purple or, deep
either from the lining epithelium of the cyst or rests of blue, hence it is termed ‘eruption hematoma’.
odontogenic epithelium in the wall of the cyst which is also The lesion is called ‘eruption cyst’ because of their
called as mural ameloblastoma. association with erupting teeth. The cause for development
Mucoepidermoid carcinoma: The development of of this form of dentigerous cyst is not known.
mucoepidermoid carcinoma, which is a malignancy of Radiographic features: Radiographically expansion
salivary glands, associated with the lining epithelium or of the normal follicular space of erupting tooth crown.
dentigerous cyst which contain mucous secreting cell as a In some cases there is saucer shaped excavation of bone
result of mucous metaplasia. projecting very slightly into the cavity (Fig. 15.15).
Points to Remember
Mandibular 3rd molars, teeth severely displaced and re-
sorbed, painless, expand laterally, pathological fracture,
basal cell nevus syndrome, cleidocranial dysplasia, Blue
domed cyst, unilocular, margins are well defined, non-
keratinized stratified squamous epithelium, cells are
cuboidal or low columnar, retepegs formation is absent,
loose connective tissue stroma, young fibroblasts, Inflam-
mation in dentigerous cyst, Rushton bodies, metaplasia in
dentigerous cyst, marsupialization, decompression, com-
plication like ameloblastoma mucoepidermoid carcinoma.
ERUPTION CYST
A specific type of cyst, which must be classified as a
form of dentigerous cyst, is frequently associated with the
erupting deciduous or permanent teeth in children. Figure 15.15 Radiographic appearance of eruption cyst
Histopathological Features and residual cysts are of keratocyst variety. According

to browne, odontogenic keratocyst is a histopathological
The underlying lamina propria of surface oral epithelial of
term and should be restricted to its original sense and to
350 cyst shows inflammatory cell infiltrate. The deep portion
describe the nature of the cyst. To avoid confusion between
of the cyst shows nonkeratinizing squamous epithelium.
primordial cyst and keratocyst, Browne suggested that the
Management term primordial cyst should be restricted to describe the
origin and odontogenic keratocyst should be restricted to
Simple excision of roof of cyst will allow eruption of
describe the nature of the cyst.
associated teeth.
Odontogenic Keratocyst as a
Points to Remember
Benign Neoplasm
Eruption hematoma, cyst usually develop due to
Odontogenic keratocyst is considered in newer
collagen deposition in the gingival connective tissue.
classifications of the odontogenic neoplasms (see chapter
Eruption hematoma expansion of the normal follicular
of odontogenic tumors) as a neoplasm than a cyst for the
inflammatory cell infiltrate. T nonkeratinizing squamous
following reasons.
epithelium.
Aggressive clinical behavior than any other cyst,
asymptomatic till it reaches a large size and it tends to extend
ODONTOGENIC KERATOCYST through the medullary cavities rapidly as in some reported
Odontogenic keratocyst (OKC) is a developmental cases complete hollowing of the ramus of mandible,
odontogenic cyst of epithelial origin. including the condylar and coronoid process has been
The OKC was previously termed primoidial cyst by recorded before any symptoms. In maxilla, if it occurs, it
Robinson (1945). According to Pindborg and Hansen the may involve the entire maxillary sinus in a similar fashion.
designation keratocyst was used to described any jaw cyst Highest turnover rate of the epithelium occur in OKC
exhibiting keratinization in their lining which may occur in than any other cyst.
follicular, residual and very rarely in a radicular cyst. High mitotic count of basal and para basal layers is
Recently in World Health Organization (WHO) present. The studies for mean mitotic counts- shows the
classification of odontogenic tumor this cyst has given a proliferative index OKC (mean = 8), nonodontogenic
name of keratocystic odontogenic tumor. cyst (mean = 2.3) and radicular cyst (mean = 4.5). The
11 percent of all jaw cysts are OKC. OKC is not a proliferative index is more similar to those seen in
clinical diagnosis but a designation for a group of cysts ameloblastoma and dental lamina. (Richard Jordan, oral
of possibly diverse origins which have a number of highly and maxillofacial surgery clinics of north America - 2003).
characteristic microscopic and clinical features in common Active collagenase aids to rapid growth and extension
with highest recurrence rate than any of the odontogenic of the cyst. In view of the established association of OKC
cyst. and Nevoid basal cell carcinoma syndrome it was strongly
In general, thin connective tissue wall and thin squamous hypothesized that the keratocyst might express proteins not
type epithelial lining (4 to 8 cell thick) that contains keratin commonly expressed in normal epithelia.
which is either para (87%) or ortho (13%) and without rete Other ultra structural and histochemical studies have
pegs. proved that altered gene expression of the epithelial cells
The basal layer is either columnar or cuboidal epithelium as suggested by gp38 positivity (epithelial-specific cell
and its prickle cells are present and they are vacuolated. surface glycoprotein) which is seen in cells with neoplastic
In some cases, bud-like proliferation from the basal layer potential.
into adjacent connective tissue wall or proliferation of p53 protein: A mutant product of the tumor suppressor
islands of odontogenic epithelium that may be present in gene p53 was shown to have an increased expression. This
the wall giving rise to satellite microcysts which support is seen in malignant cells and not in normal cell cycle.
the fact that OKC’s have a high recurrence rate. 5 percent Ki-67 is an antigen or cell specific marker for
of dentigerous and radicular cysts are keratocysts and a proliferating cells; it is also shown to be in higher reactivity
variety of many primordial, gingival, lateral periodontal for parakeratinized OKC.
PCNA: Proliferating cell nuclear antigen, is widely

regarded as a marker for replication and is associated with
DNA repair processes and stimulation growth factors.
This immunohistochemical expression is also raised in the 351
lining cells.
AgNOR: Argyrophillic nucleolar organizing regions
are shown to be more. The mean number of AgNORS
per nucleus was significantly more in the inflammed
odontogenic keratocyst than in any other inflamed cyst.
Origin of Cyst
The OKC is originating from the odontogenic epithelium;
Dental lamina or it is remnants which possesses marked
growth potential or alternatively from proliferation of basal
cells as ‘basal cell hamartomas’ which are offshoots of the Figure 15.16 Odontogenic keratocyst showing extraoral
basal cells. swelling in the ramus area
Clinical Features Recurrence

Age and sex distribution: Odontogenic keratocyst occurs Rate of recurrence of odontogenic keratocyst is very high.
over a wide age range and cases have been recorded as There are number of reasons for it.
early as the first decade and as late as the ninth with age Occurrence of satellite cyst, which is a bud-like
group 4 to 84 years. It is initiated early in life, during the projection of basal cell layer into the connective tissue
period of tooth development with a peak incidence in 2nd which is retained during the enucleation procedure. Some
and 3rd decades. It is found more frequently in males than instances of recurrence are likely because of new cyst
in females and this sex predilection is more pronounced in formation rather than true recurrence.
blacks than in whites. Secondarily, its lining is very thin and fragile
Site: It is more common in mandible with a greater particularly when the cyst is large and therefore more
incidence at the angle and extending for varying distance difficult to enucleate than a cyst with thick wall. Portion
into the ascending ramus and forward into the body. of the lining may be left behind and constitute the origin of
recurrence.
Symptoms: It is asymptomatic unless they become Enucleation in one piece may be more difficult with
secondarily infected, in which case patient complains of cysts which have a scalloped margins and this may explain
pain, soft tissue swelling and drainage. The maxillary sinus the higher recurrence rate than those with smoother contour.
gets infected in the initial phase of cyst enlargement, so these Toller suggested that there may be an intrinsic growth
OKC may manifest earlier than the duration taken by the potential in the epithelial lining which may be responsible
mandibular cyst to manifest. Occasionally, paresthesia is for a higher recurrence rate.
experienced of the lower lip or teeth with mandibular cases. It may arise from proliferation of the basal cells of
Signs: The lesion can lead to pathologic fracture. Those that the oral mucosa particularly in the third molar area and
occur in the maxilla causes buccal expansion (Fig. 15.16). ascending ramus of the mandible. It is referred that there
On aspiration there is odorless creamy or caseous content. is often a firm adhesion of the cyst to overlying mucosa
Multiple odontogenic keratocyst are found in Gorlin and it is recommended that mucosa should be excised with
Goltz syndrome, Marfan syndrome, Ehler’s Danlos them in an attempt to prevent possible recurrence from the
syndrome and Noonan’s syndrome. residual basal cell proliferation.
Radiographic Features Radiological Types of Keratocyst (Fig. 15.19)

Majority of lesions are unilocular with smooth borders but • Envelopment type: It is referred to a variety of
352 some unilocular lesions are large with irregular borders. keratocyst which embraces an adjacent unerupted
Radiolucency is usually hazy due to keratin filled cavity tooth.
and it is surrounded by thin sclerotic rim due to reactive • Replacement: Those which forms in the place of
osteocytes. normal teeth.
Bone can expand in anterior posterior direction (Figs • Extraneous: Those in the ascending ramus away
15.17 and 15.18) and perforate the buccal and lingual from the teeth.
cortical plates of bone and involve the adjacent soft tissue. • Collateral: Those adjacent to the root of teeth which
Downward displacement of the inferior alveolar canal are indistinguishable radiologically from the lateral
and resorption of the lower cortical plate of the mandible periodontal cyst.
may be seen as well as perforation of bone and a pathologic
fracture may occasionally occurs. Histopathological Features
(Figs 15.20 to 15.28)
Macroscopic features: The odontogenic keratocyst
gross pathological features are characteristic as the cysts
are hardly received intact in the laboratory. The small
cysts may have a well defined cystic wall, but the large
cyst almost always show thin walled, collapsed folded or
detached linings. The lumen of keratocyst may be filled
with thin straw colored fluid or with thick creamy material.
The odontogenic keratocyst shows two types of linings,
i.e. parakeratinized stratified squamous epithelium. The par-
akeratinized epithelium is more common, (80-90%) cases.
The orthokeratinized OKC shows less common occurrence.
The characteristic feature of the lining of is
pathognomic corrugated, with a regular thickness of the
epithelium between 5-8 cell layers. The lining is without
Figure 15.17 OKC showing large radiolucency
in mandibular ramus area rete ridges. The basal cell layer is columnar with palisaded
arrangement of the nuclei. The nuclei tend to be placed
Figure 15.18 OKC showing anteroposterior extension Figure 15.19 Types of OKC: 1. Replacemental,
2. Envelopmental, 3. Extraneous, 4. Collateral
353
Figure 15.20 Cystic epithelium shows folding and detachment Figure 15.23 Inflamed OKC with epithelial discontinuity
at places and inflammatory infiltrate. Note the basal cells with palisading
nuclei
Figure 15.21 Inflamed OKC with epithelial discontinuity Figure 15.24 Higher magnification view showing severe
and inflammatory infiltrate epithelial dysplasia and basal cell proliferation
Figure 15.22 OKC lined by corrugated parakeratinized Figure 15.25 OKC lined by orthokeratin
stratified epithelium. The epithelium is collapsed and folded
away from the basement membrane The nuclei of the basal

cells are darkly staining, show basal cell hyperplasia, this
is not present in other keratocyst. Highly aggressive OKC
354 shows moderate to severe epithelial dysplasia.
The fibrous capsule is thin with relatively few cells
widely separated by a stroma which is often in mucopoly-
saccharide and resembles mesenchymal connective tissue.
In the inflamed cases the epithelium might lose the kerati-
nization and develop rete ridges. Connective tissue shows
islands of odontogenic epithelium forming small duplicate
daughter cysts or small satellite cysts.
The satellite cysts are more common in patients with
multiple cysts and nevoid basal cell carcinoma syndrome.
Figure 15.26 Odontogenic keratocyst showing parakeratinized Another most important feature of this cyst is that there is
stratified squamous epithelium with dark hyperchromatic basal a weak epithelial–connective tissue attachment. This causes
cell layer (low power) and organized collagenous connective the detachment of the epithelium and further recurrences as
tissue stroma it becomes difficult during removal of the cyst.
Other less common findings are mucous metaplasia,
Rushton bodies and cholesterol clefts in inflammed cysts.
Sometimes lumen contains a great deal of keratin, while
at other times it has little. Cholesterol as well as hyaline
bodies at the site inflammation, may also be present.
Orthokeratinized stratified squamous epithelium:
Nowaday this cyst is called as separated entity. Cyst lining
is composed of stratified squamous epithelium lined by
orthokeratotic surface of varying thickness. Keratohyaline
granules are prominent in superficial epithelial layer.
There is thin epithelial lining.
Figure 15.27 Odontogenic keratocyst—parakeratinized strati- Management

fied squamous epithelium with dark hyperchromatic basal cell Enucleation of entire cyst with vigorous curettage of the
layer (high power)
cystic wall should be done.
Periodic post-treatment examination should also be
carried out to check recurrence.
Points to Remember
Common in mandible, asymptomatic, pain, soft tissue
swelling, pathologic fracture, on aspiration there is odor-
less creamy or caseous, Gorlin Goltz syndrome, Marfan
syndrome, Ehler’s Danlos syndrome, Noonan’s syndrome,
satellite cyst, bud-like projection of basal cell layer, lining
is very thin, enucleation in one piece may be more difficult,
proliferation of the basal cells of the oral mucosa, unilocular
with smooth borders, hazy radiolucency, bone can expand
in anterior posterior direction, parakeratinized stratified
squamous epithelium, lining is without rete ridges, muco-
Figure 15.28 Odontogenic keratocyst show high proliferative
index (PCNA) the basal cells shows typical arrangement of polysaccharide, satellite cysts, weak epithelial – connective
palisading or parallel orientation of nuclei called ‘tomb stone’ tissue attachment, cholesterol, Orthokeratinized stratified
or picket fence arrangement squamous epithelium, keratohyaline granules, enucleation.
PRIMORDIAL CYST In other cases, the epithelium may exhibit a surface

layer of orthokeratin, while the spinous layer may be
It is one of the less common types of odontogenic cysts. relatively thin or be of moderate thickness. The basal
As described earlier, it was named by Robinson in 1945. cell layer is not prominent and is sometimes with an 355
He defined a primoidial cyst as “a cyst which arises by atypical corrugated appearance, while the remainder of the
breakdown of the stellate reticulum of the enamel organ epithelium is exceptionally uniform in thickness, usually 6
before any hard tissue is formed and hence which may be to 10 cells thick with extremely prominent basal layer. The
one of the normal series or a supernumerary”. cells are arranged in a ‘picket fence’ or ‘tombstone pattern’
It originates when cystic changes take place in the and showing no rete peg formation.
stellate reticulum of the tooth germ before any calcified
enamel or dentin has been formed. So it is found in place Management
of a tooth rather than directly associated with it. It may
Surgical enucleation should be done. Regular check up due
originate from the enamel organ of supernumerary tooth or
to a high recurrence rate.
from the remnants of dental lamina.
There is lot of controversies and confusion regarding the Points to Remember
primordial cyst and odontogenic keratocyst. As the origin
Cystic changes take place in the stellate reticulum,
is same this cyst is term as odontogenic keratocyst and
ascending ramus of the mandible, painlessly enlarge,
primordial cyst are used synonymously. In 1992 WHO listed
intact or interrupted layer of stratified squamous
odontogenic keratocyst as term used for this type of cyst.
epithelium, ‘picket fence’ or ‘tombstone pattern’ and
Primordial cyst described below is for the sake of
showing no rete peg formation.
completion and for academic point of view so that student
should know about it.
GINGIVAL CYST OF NEWBORN
Clinical Features
It is also called an alveolar cyst of newborn. It is seen on
Age and sex distribution: It is found in children and alveolar mucosa of infant. It is derived from the remnants
young adult between 10 to 30 years of age, although it may of the dental lamina. It is found on the crest of the maxillary
persist in older age group and occurs with equal frequency and mandibular dental ridges. The cyst apparently
in both the sexes. originated form remnants of dental lamina.
Site: It can arise in any portion of the jaw, but most often
Clinical Features
seen in the ascending ramus of the mandible and in from
third molar area. It is occasionally associated with an over Appearance: They appear as small, multiple whitish
retained erupted deciduous tooth. papules on the alveolar mucosa (Fig. 15.29). They
spontaneously rupture in oral cavity.
Symptoms: It has a tendency to painlessly enlarge and
slowly replace large portions of cancellous bone before Size: It is not more than 2 to 3 mm.
expansion of the cortical plate by way of which it reveals
its presence. Pain which is associated with a large cyst is
caused by infection that may follow the perforation of the There is thin flattened epithelial lining with para-keratotic
expanded cortical plate. luminal surface. Lumen contains keratin aceous debris.
Histopathological Features Management

This is similar to that of other odontogenic cysts. It is No treatment is necessary as they rupture easily in oral
lined on the inner surface facing the lumen by an intact or cavity.
interrupted layer of stratified squamous epithelium.
Points to Remember
In some cases, the epithelium is non-keratinized and
exhibits a very prominent spinous layer with elongated and Alveolar cyst of newborn, remnants of the dental lamina,
some times confluent retepegs. Basal cell layer is present small, multiple whitish papules, thin flattened epithelial
but is not prominent. lining, keratin aceous debris.
the radiolucency is dark and sharply demarcated and a

communication with the periodontium is indicated then the
lesion is more likely to be lateral periodontal cyst that has
356 eroded outward. These assumptions based on radiological
features and can be confirmed by surgical exploration
when the lesion is being removed.
Etiology and Pathogenesis

There are degenerative changes in proliferating epithelial
tissue. Remnants of dental lamina, enamel organ or
epithelial islands of periodontal membrane can cause
gingival cyst of adults.
Traumatic implantation of epithelium can also lead to
gingival cyst. It also arises from post functional rests of
Figure 15.29 Small nodule seen on upper gingiva dental lamina.
Clinical Features
GINGIVAL CYST OF ADULT Age and sex distribution: The gingival cyst may occur at
any age, but it is most common in adults in the 5th and 6th
Gingival cyst appears as a dome shaped swelling in the decade of life with predilection for males.
attached gingiva. It is soft tissue counterpart of lateral
periodontal cyst. It is an uncommon cyst occurring either Site: The location of the lesion closely follows that of
on free or attached gingiva. lateral periodontal cyst. It is more common in mandible in
Although, it has been customary to consider these cysts premolar and canine region. Maxillary gingival cyst is seen
as a distinctly different entity, their clinical appearance and in incisor, canine and premolar area.
behavior, morphologic and histochemical features and site Appearance: The surface may be smooth and the color
of occurrence are so similar in appearance that they are in may appear as that of normal gingiva or bluish and may
reality the intraosseous and extraosseous manifestations of appear red when it is blood filled as a result of recent
the same pathoses. trauma. It is dome like swelling.
Bhasker grouped the gingival and lateral periodontal Symptoms: It is slowly enlarging, painless swelling,
cysts together as gingival cyst and considered that they usually less than 1 cm in diameter and may occur in
both arise from extraosseous odontogenic epithelium. attached gingiva or the interdental papilla.
Some cases have shown a circumscribed radiolucency
Signs: The lesions are soft and fluctuant and adjacent
indicative of lateral periodontal cyst, which he believed
teeth are usually vital during surgical exploration. Slight
were because of cup shaped depressions on the periosteal
erosion on the surface of the bone may be observed without
surface of cortical plates produced by enlargement of the
extension into the periodontium.
gingival cysts.
Buchner and Hansen also suggested them to be distinct Radiological features: There may be superficial cupping
entities based on its pathological origin. The gingival out of bone (Fig. 15.30).
cyst may certainly occur without bone involvement and
may produce a gingival swelling. Most of the times, the Histopathological Features
swelling goes unnoticed. It is improbable though not The lining epithelium is generally identical to that found in
impossible that a cyst originating in the gingival soft lateral periodontal cyst. In cases of traumatic implantation
tissue could enlarge sufficiently to produce radiolucency type of gingival cyst, calcification or ectopic ossification,
by obvious bone erosion without producing any gingival on rare occasions, it may be associated with cystic lesion.
swelling. In the case of a lesion, which has produced both The epithelium ranges in thickness from simply one
gingival swelling and radiolucency, faint shadow (which is layer to several layers of cells. The layer consists of flat or
due to surface depression) indicates gingival cyst. Where cuboidal cells with thin stratified squamous epithelium.
assumes a position in approximation to the lateral surface

of the root.
Origin from proliferation of cell rests of Malassez in
the periodontal ligament although the stimulus for this 357
proliferation is unknown.
Origin simply as a primordial cyst of a supernumerary
tooth germ, since the predilection for occurrence of
the lateral periodontal cyst in the mandibular bicuspid
area corresponds well with the known high incidence of
supernumerary teeth in the same region.
Origin from proliferation and cystic transformation
of rests of dental lamina (which is in post functional state
and therefore has only limited growth potential), that is in
accordance with the usual small size of the cyst.
Figure 15.30 Gingival cyst showing superficial cupping Recent theory suggests that the lateral periodontal cyst
in the bone and gingival cyst of adult share the common histogenesis
from post functional dental lamina rests. These two
cysts represent basically the central or intraosseous and
Small nests of glycogen rich clear cells can be seen in peripheral or extra-osseous manifestations of the same
connective tissue. lesion.
Management Types
Surgical excision of the lesion in adults is usually • Inflammatory: It occurs near alveolar crest. Pocket
recommended and the lesion does not tend to recur. A content may irritate and stimulate rest of Malassez.
neoplastic potential has never been reported. • Developmental: It is associated with developing
tooth germ.
Points to Remember
Dome shaped swelling, remnants of dental lamina, Clinical and Radiological Features
enamel organ or epithelial islands of periodontal
membrane, smooth surface, slowly enlarging, painless Age and sex distribution: The lateral periodontal cyst
swelling, lesions are soft, small nests of glycogen rich occurs chiefly in adults with an age range from 22 to
clear cells, calcification or ectopic ossification. 85 years with a mean age of 50 years. It shows a male
predilection for occurrence.
LATERAL PERIODONTAL CYST Site: The most frequent location of lateral periodontal
cyst reported on lateral surface of the roots of vital teeth in
They are named so, due to its location. Lateral periodontal mandibular canine and premolar region and is followed by
cyst arises in the peridontium and located in the the anterior region of the maxilla.
interproximal bone between the apex and the alveolar crest.
The lateral periodontal cyst is uncommon but a well Symptoms: Gingival swelling may occur on the facial
recognized type of developmental odontogenic cyst. The aspect and in these types of cases, it must be differentiated
designation lateral periodontal cyst is confined to that cyst, from the gingival cyst. In gingival cyst the overlying
which occurs as a result of inflammatory etiology and the mucosa is blue but in lateral periodontal cyst the overlying
diagnosis of collateral keratocyst has been excluded on mucosa appears normal.
clinical and histological ground. Sign: When the cyst is located on the labial surface of
the root, it appears as a slight obvious mass, overlying
Pathogenesis and Etiology the mucosa. The associated tooth is vital. If the cyst
Origin initially as a dentigerious cyst developing along the becomes infected, it may resemble a lateral periodontal
lateral surface of the crown and as the tooth erupt the cyst abscess.
Radiographic features: The intra-bony lateral periodontal

cyst is seen as a round or ovoid well defined radiolucency
with hyperostotic borders (Fig. 15.31). Most of them are
358 less than 1 cm in diameter except the botryoid variety
which may larger ad multilocular.
Botryoid odontogenic cyst: This term was proposed by
Waldron, which refers to a multilocular periodontal cyst.
It resembles a cluster of lateral periodontal cysts exhibiting
some difference. The lesion is multilocular with thin
fibrous connective tissue septa. It is clear from numerous
reports of recurrence that the botryoid odontogenic cyst
requires careful excision.

It is lined by a layer of stratified squamous epithelium with Figure 15.32 Lateral periodontal cyst show thin nonkeratinized
connective tissue wall. Cuboidal and columnar cell may be stratified squamous epithelium
found compassing the lining.
The epithelium consists of flattened squamous cells.
Many of the lining cells have clear vacuolated glycogen Points to Remember
rich cytoplasm. The lumen cyst show focal thickened Gingival swelling a round or ovoid well defined radio-
plaque of proliferating lining cell. These are especially lucency with hyperostotic borders, Botryoid odontogenic
prominent in botryoid cyst. cyst, layer of stratified squamous epithelium, cuboidal
columnar cell, flattened squamous cells, focal thickened
Management plaque of proliferating lining cell. These are especially
The lateral periodontal cyst must be surgically removed if prominent in botryoid cyst.
possible without extracting the associated tooth. If this can
not be accomplished, the tooth must be sacrificed. There is
GLANDULAR ODONTOGENIC CYST
a tendency for recurrence for this type of cysts following
its surgical excision. It is also called as ‘Sialo odontogenic cyst’, or ‘mucoepi-
dermoid cyst’. The term most descriptive of the lesion is
probably mucoepidermoid odontogenic cyst because of the
presence of both secretory elements and stratified squa-
mous epithelium.
The mucous and cylindrical cells form an integral
part of the epithelial component with mucinous material
within the cystic space. Gardner, who favors the name
glandular, suggests that the features and biological
behavior are sufficiently distinct for it to be regarded as a
distinct entity.
It also shows glandular or salivary features.
The typical feature of the cyst is that it is intrabony
radiologically and which can recur if not adequately
excised.

Figure 15.31 Lateral periodontal cyst showing radiolucency Age and sex distribution: It occurs over a wide age range
on the lateral surface of teeth with mean age of occurrence 48 years.
Location: It has got strong predilection for anterior region lingual aspect of the dental ridge and on the palate away
of jaw with many lesions crossing the midline. forms the raphe. The nodules are considered remnants
of mucous gland and are histologically different from
Sign and symptoms: They are small lesion less than 1 cm 359
Epstein’s pearls.
in diameter to large destructive lesion that may involve
most of the jaw. Large lesion can cause expansion which Clinical Features
can cause pain or paresthesia.
Radiologically, it is a unilocular or multilocular Age: These cysts are rarely seen after 3 months of age.
radiolucency with either smooth or scalloped margins. Site: They tend to cluster along the junction of the hard
and soft palate in a linear fashion or are scattered over the
Histopathological Features hard palate.
Histologically, shows cystic space lined by non-keratinized Symptoms: Occasionally, they become large to be
epithelium. The fibrous cyst wall is usually devoid of any clinically obvious as small discrete white swellings of the
inflammatory cell infiltrate. alveolar ridge.
Superficial epithelial are cuboidal to columnar resulting
Appearance: Clinically it appears as small whitish
in an uneven hobnail with papillary surface.
projection of the alveolar ridge of the jaws of infants giving
Cilia and mucicarminopphilic material are also present.
rise mistaken appearance of a tooth. Sometimes appearing
Mucous cells may or may not be present.
blanched as though from internal pressure.
Management Signs: It is raised nodules, usually multiple, measuring
It should be treated by enucleation or curettage can be a fraction of a millimeter to 2-3 mm in diameter. These
done. probably correspond to those structures described as ‘pre-
deciduous dentition’ in older literature.
Points to Remember
Sialo odontogenic cyst, secretory elements and stratified
squamous epithelium, less than 1 cm in diameter, pain These are true cysts with thin epithelial lining and show
or paresthesia, unilocular or multilocular radiolucency, lumen filled with desquamated keratin, occasionally
nonkeratinized epithelium, uneven hobnail with papillary containing inflammatory cells.
surface, cilia and mucicarminopphilic. Dystrophic calcification and hyaline bodies of Rushton,
commonly seen in dentigerous cyst, are also sometimes
seen her.
PALATAL CYST OF NEWBORN
(EPSTEIN’S PEARLS, BOHN’S Management
NODULES) No treatment is required as these lesions almost invariably
disappear by opening onto the surface of the mucosa or
Small development cysts are common finding in the palate through disruption by erupting teeth.
of newborn. These are inclusion cyst occur due to epithelial
entrapment in the fusion of palate. Points to Remember
Epstein’s pearls, Bohn’s nodules, small discrete white
Pathogenesis
swellings of the alveolar ridge, lumen filled with
Epstein's pearls: These are cystic keratin filled nodules desquamated keratin, dyystrophic calcification and
found along the midpalatine raphe probably derived hyaline bodies of Rushton.
from entrapped epithelial remnants along the line of
fusion.
CALCIFYING EPITHELIAL
Bohn’s nodules: These are keratin filled cyst scattered ODONTOGENIC CYST
over the palate most numerous along the junction of
hard and soft palate and apparently derived from palatal It is also called as ‘keratinizing and calcifying odontogenic
salivary gland structure. It is formed along the buccal and cyst’, ‘dentinogenic ghost cell tumor’, calcifying cystic
odontogenic tumor’, ‘calcifying ghost cell odontogenic

cyst’ and ‘Gorlin’s cyst’ as it was first described by Gorlin
in 1962. The lesion is unusual in that it has some features
360 suggestive of cyst but also has many characteristic of a
solid neoplasm.
Most cases of calcifying odontogenic cyst arise
centrally within the bone, but it may occur peripherally
in the soft tissue overlying the tooth bearing area. Central
calcifying odontogenic cyst is also known as intraosseous
odontogenic cyst.
Types (Praetorius)
• Simple unicystic type
• Unicystic odontome producing type
• Unicystic ameloblastomatous proliferating type.
Figure 15.33 CEOC showing calcification in the anterior
region
Clinical Features
Age and sex: Age distribution from 10 to19 years with a Histopathological Features
mean age of 36 years. However, there may be another peak (Figs 15.34 to 15.37)
incidence through the seventh decade. It is slightly more
prevalent in women. The epithelium of the cystic lining is chiefly low cuboidal
or squamous type and is two or three layers thick. Focal
Site: 3/4th of the lesions occur centrally, with about equal areas of stellate reticulum and ghost cell may be present.
in both jaws and 75 percent occuring anterior to the first Ghost cells according to some authors are abnormal
molar. keratin; they are pale eosinophilic swollen epithelial
Symptoms: It is slow growing, painless, nontender cells, which have lost the nucleus and nuclear membrane.
swelling of the jaws. Occasionally some patients may They have affinity for calcification. More accepted is the
complain of pain. concept that they are product of coagulative necrosis of the
epithelial cells as some amount of calcified material may
Signs: In some cases, cortical plate over the expanding be seen.
lesion may be destroyed and cystic mass may be palpable Masses of ghost cells may fuse to form large sheets of
with patients reporting of discharge. Aspiration yields amorphous, acellular material. Multiple daughter cyst can
viscous granular yellow fluid. be seen in fibrous wall and foreign body reaction to ghost
Teeth: Adjacent teeth may be displaced. cell can be seen.
Unicystic odontome producing type: About 20 percent
of COC are associated with odontoma. It show features of
The central lesion may appear as a cyst like radiolucency. complex or compound odontoma plus features of COC.
Margin may be well defined outline or irregular in shape
with poorly defined borders. Unicystic ameloblastomatous proliferating type:
It may contain small foci of calcified material that Epithelial proliferation resembling ameloblastoma can
are only microscopically apparent (Fig. 15.33). In some intermixed with ghost cell.
cases, it is completely radiolucent while in other cases an Odontogenic ghost cell carcinoma: There lesion have
increasing amount of calcified material may be seen as cellular pleomorphism and mitotic activity with invasion
white flecks. It may be unilocular or multilocular. of surrounding tissue can occur.
361
Figure 15.34 Unicystic CEOC showing ameloblastomatous Figure 15.36 Basophilic dystrophic calcifications in ghost cells
proliferation of the epithelial lining
Figure 15.35 Ghost cells are pale eosinophilic swollen epi- Figure 15.37 Dentinoid material induced by the adjacent
thelial cells that have lost its nucleus and nuclear membrane odontogenic epithelial islands
Management INFLAMMATORY RADICULAR CYST

Enucleation and curettage should be done. Malignant
It is also called as ‘apical periodontal cyst’, ‘periapical
transformation has been recorded.
cyst’, or ‘dental root end cyst’. It is a common sequel in
Points to Remember progressive changes associated with bacterial invasion and
Keratinizing and calcifying odontogenic cyst, slow death of the dental pulp. It most commonly occurs at the
growing, painless, nontender swelling, expanding apices of teeth.
lesion, margin may be well defined outline or irregular, The radicular cyst is classified as an inflammatory cyst
small foci of calcified material, epithelium low cuboidal because this process is thought to initiate the growth of the
or squamous type, ghost cells, coagulative necrosis of epithelial component. It may be classified as odontogenic
the epithelial cells, multiple daughter cyst, Unicystic cyst because of its origin in cell rests of Malassez which are
odontome producing type, unicystic ameloblastomatous remnants of Hertwig’s root sheath and is a product of the
proliferating type, odontogenic ghost cell carcinoma. odontogenic epithelial layer.
The epithelium may be derived in some cases from

respiratory epithelium of the maxillary sinus, when the
periapical lesion communicates with the sinus wall, oral
362 epithelium proliferates apically from the periodontal
pocket and oral epithelium forms a fistulous tract.
Pathogenesis
Periradicular inflammatory changes cause the epithelium
to proliferate. As the epithelium grows into a mass of cells,
the center losses the source of nutrition from the periapical
tissue. These changes produce necrosis in the center and a
cavity is formed and cyst is created. Another theory is that
an abscess cavity is formed in the connective tissue and is
lined with proliferating epithelial tissue.
Figure 15.38 Swelling seen in palate due to radicular cyst
Types (Courtesy: Dr Aparna Thombre, Reader, Department of Oral
• Periapical pocket cyst: There is incomplete epithelial Pathology, VSPM Dental College and Hospital, Nagpur,
lining due to extension of apical portion into cyst Maharashtra, India)
lumen
• Periapical true cyst: There is complete epithelium percussion. Swelling may be bony hard or crepitations
baglike structure which are separated from tooth may be present as bone is thinned or it may be rubbery or
apex fluctuate, if the bone is completely destroyed (Fig. 15.38).
• Lateral radicular cyst: It appear along lateral aspect
of root. Complication: Ameloblastoma, epidermoid carcinoma
and mucoepidermoid carcinoma may arise in the epithelial
lining of periapical cyst.
Clinical Features
Age and sex distribution: Peak frequency occurs in the 3rd Radiographic Features
decade and there are large numbers of cases in the 4th and It appears as a rounded or pear shaped radiolucency at
5th decades after which there is a gradual decline. Although the apex of non sensitive tooth or with non vital tooth.
dental caries is more common in deciduous teeth in the first Radiolucency is more than 1.5 cms in diameter but usually
decade but radicular cysts are not often found in deciduous less than 3 cms in diameter. It has got well defined outline
teeth may be because teeth tend to drain more readily than with thin hyperostotic borders (Figs 15.39A and B).
the permanent teeth and the antigenic stimuli which evoke Adjacent tooth are usually displaced and rarely
the changes leading to the formation of radicular cyst may resorbed. There is also buccal expansion and if it involves
be different. There is male predominance, as females are maxillary area then displacement of antrum can occur.
less likely to neglect their maxillary anterior teeth and In case of lateral radicular cyst discrete radiolucency
males are more likely to sustain trauma to their maxillary along the lateral surface of tooth can be seen.
teeth. It shows a higher frequency of occurrence in whites.
Site: Maxillary anterior are more commonly affected. Also
in addition to caries, maxillary teeth are more prone to (Figs 15.40 to 15.44)
traumatic injuries which lead to pulp death. Macroscopic features: The contents of radicular cysts are
usually a soft brown material, often with glistening, oily,
Symptoms: It represents an asymptomatic phase in
yellowish flecks. Nodules of opaque yellow crystalline
periapical inflammatory process following death of the
material, representing cholesterol, maybe seen protruding
dental pulp. It is associated with non vital tooth.
into the lumen or within the wall is seen.
Sigs: It rarely causes nontender expansion of the overlying The apical periodontal cyst is usually lined by
cortical bone. The associated tooth is not sensitive to stratified squamous epithelium. Occasionally, it is lined
363
Figure 15.41 Radicular cyst showing arcading growth pattern

of epithelium
B
Figures 15.39A and B Well-defined radiolucency in radicular
cyst (Courtesy: Dr Aparna Thombre, Reader, Department of
Maharashtra, India)
Figure 15.42 Radicular cyst histopathological picture (Courtesy:

by pseudostratified, columnar or respiratory type of

epithelium, when it occurs in the teeth that involve the
maxillary sinus. The lining shows variation in thickness
with rare keratin formation.
Rushton bodies often found in great numbers in the
epithelium of apical periodontal cyst is seen. These are
linear arch shaped calcification.
In some cases hyaline bodies (pulse granuloma, giant
cell hyaline angiopathy) can seen scattered at the wall of
cysts. These are small circumscribed pools of eosinophilic
Figure 15.40 Linear, straight or curved, hairpin shaped material which exhibits corrugated periphery of condense
Rushton bodies. Some show concentric lamellations collagen.
Management
Root canal treatment: It is the treatment of choice as in
364 many cases, radicular cyst resolve after root canal treatment.
The reason behind it is that as drainage is established, the
inflammatory process subsides and the fibroblast start
producing collagen. The pressure of proliferating-collagen
reduces the blood supply to the epithelium lining and causes
it to degenerate. Macrophages remove the degenerating
epithelial tissue.
Extraction: If the lesion fails to resolve, extraction of
associated tooth is carried out.
Enucleation and marsupialization: Enucleation or
marsupialization of a larger lesion is done.
Figure 15.43 Lining of radicular cyst showing presence
of Rushton bodies Points to Remember
Apical periodontal cyst, maxillary anterior, nonvital
tooth, bony hard, crepitation, complication like amelo-
blastoma, epidermoid carcinoma, mucoepidermoid car-
cinoma, rounded or pear shaped radiolucency, well de-
fined outline, stratified squamous epithelium, Rushton
bodies, hyaline bodies, parallel bundles of collagen fib-
ers, collection of cholesterol cleft.
RESIDUAL CYST
It is a cyst that either remained as such in the jaw when it
is associated tooth was removed or was formed in residual
epithelium of cell rests from a periodontal ligament of the
lost tooth. Low grade inflammation of parent cyst might
predispose formation of residual cyst.
Figure 15.44 Cholesterol clefts in radicular cyst
Age and sex: Cyst is common in patients older than 20
Connective tissue that makes up the wall of apical years with an average age of about 52 years. It is twice
periodontal cyst is composed of parallel bundles of more common in male than female.
collagen fibers with variable number of fibroblasts and Site: Higher incidence in the maxilla. Mostly found on
small blood vessels. alveolar process or body of the tooth bearing area with
The universal occurrence of an inflammatory infiltrate some cysts described in the lower ramus of the mandible.
in the connective tissue immediately adjacent to the
epithelium is characteristic feature. Symptoms: It is asymptomatic with a previous history of
Collection of cholesterol cleft with associated pain in the tooth.
multinucleated giant cells is found in the wall of the lesion. Size: It is seldom more than 5 to 10 mms in diameter.
The lumen of the cyst usually contains fluid with a low
concentration of protein. Lumen may contain cholesterol Radiological features: Pre-extraction radiographs
or keratin. show tooth with an evidence of deep caries or fracture
more readily from the lateral periodontium which is closely

associated with gingival crevice.
PARADENTAL CYST 365
It was first described by Craig in 1976. It is a cyst of

inflammatory origin occurring on the lateral aspect of the
root of partially erupted mandibular third molar with an
associated history of pericornitis.
Origin
Cell rests of Malassez can be the origin, but argument is
that if rests of Malassez were responsible then the lesion
should be equally distributed around the root surface.
Second theory is that it originates from the reduced
Figure 15.45 Residual cyst showing radiolucency enamel epithelium as the presence of reduced enamel
epithelium over the enamel projection might be the source
adequate for pulp involvement and/or an associated cyst. and could explain the common location of the cyst.
Thin radioopaque margins are common with unilocular
appearance although the infected cyst will not have such
well defined margins (Fig. 15.45). Age and sex: It occurs between 10 to 39 years of age.
It is most commonly in the third decade of life. It shows
Management predilection for males.
Enucleation: If the cyst is not large and patient’s age Site: Usually associated with the third molar on the buccal
and health will tolerate the insult, the cyst wall should surface and covers the bifurcation. It may occur bilaterally.
be completely enucleated. The extent of repair of the
defect will depend on the size of the cyst and health of Signs: The involved tooth is vital.
the patient. Radiographic features: There is well demarcated
Marsupialization: In the cases where the surgical proce- radiolucency occurring distal to the partially erupted
dure must be as atraumatic as possible, marsupialization or tooth but there was often buccal superimposition. The
decompression may be used. radiolucency sometimes extends apically but an intact
periodontal ligament space provided the evidence that the
Excision: Complete excision and replacement with auto-
lesion did not originates at the apex.
genously bone graft or single segment of bone fixed in it.
Points to Remember
It is lined by proliferating, nonkeratinized squamous epi-
Asymptomatic with a previous history of pain, evidence
thelium of varying thickness. The fibrous capsule is the seat
of deep caries, pre-extraction radiographs, thin radio-
of an intense chronic or mixed inflammatory cell infiltrate.
paque margins.
Management
INFLAMMATORY COLLATERAL CYST The lesion is treated by surgical enucleation.
It is cyst which arises in the periodontium on the lateral
Points to Remember
aspect of an erupted tooth as a result of inflammatory
process in the periodontal pocket. Cell rests of Malassez, vital tooth, well demarcated
They arise by proliferation of cell rests of Malassez in radiolucency, distal to the partially erupted tooth
the lateral periodontium. They are rare as drainage occurs proliferating, nonkeratinized squamous epithelium.
MANDIBULAR BUCCAL Points to Remember

INFECTED CYST Buccal bifurcation cyst, enamel extension into the
366 It is also called as ‘buccal bifurcation cyst’. It is an bifurcation, discomfort, pain, tenderness, inflamed
inflammatory cyst occurring on the buccal surface of swelling, vital pulp, associated tooth tilted, new bone
mandibular molars usually first molar in young children. may be laid down linear band or laminated, non-
keratinizing stratified squamous epithelium, chronic
Pathogenesis inflammatory cell infiltrate.
It has been thought to be occur due to buccal enamel
extension into the bifurcation area. This extension SUPPURATING CYST
predispose for pocket formation in teeth which could
enlarge to form a cyst in response to pericoronitis. When pyogenic organisms are present in sufficient number
to produce changes in the cyst, suppuration will result.
Clinical and Radiological Features The thin white line which represents the cortex of the
Age: A younger age group than the paradental cyst. cyst becomes less dense and thinner and may be entirely
lost.
Site: It affects the mandibular first and second molars more In rare cases there is slight irregularity of the walls of
commonly rather than the wisdom teeth. The cyst always the cyst, with some decalcification extending into the bone
is situated on the buccal surface of the mandibular molar to a short distance.
most frequently the first permanent molar after their partial In the place of thin white cortex, there is sometimes a
or complete eruption. broad band of sclerosed bone which is less dense but wider
Symptoms: There may be discomfort, pain, tenderness and than a normal cortex and presents a granular appearance.
rarely suppuration. Swelling particularly, if inflamed, is
the clinical feature most likely to induce the patient to seek HEALING CYST
advice. Facial swelling may follow and this may be inflamed.
A cyst that is cause by an infected tooth often heals after
Signs: Usually, a vital pulp and intact lamina dura. removal of that tooth. One of earliest changes is the gradual
loss of the cortical layer of bone which lines most of the
Teeth: The associated tooth is usually tilted, so that the
cysts.
apices are adjacent to the lingual cortex, a feature which is
At the same time, if the cyst becomes infected, the bone
demonstrable in occlusal radiographs.
in the immediate vicinity of the walls becomes radiolucent
Radiological features: Due to involvement of the as a result of hyperemia. After the period of time, there is
periosteum new bone may be laid down either as a single gradual lessening of radiolucency of the cavity and a return
linear band or laminated, if there two or more layers. of the surrounding bone to a normal density.
Sometimes, the new bone may be homogeneous. The The dark shadow of the cavity is replaced by a grey
inferior margins of the cyst are concave and rarely, the cyst one and it is apparent that the cavity is becoming smaller.
may extend to the inferior border of the mandible but not A large cyst may fail to heal completely, resulting in a
leading to any external deformity. smaller cyst. This is due to the retention of some part of the
epithelial wall of the cyst, which has continued its activity.
It is not specific and show nonkeratinizing stratified NONODONTOGENIC CYSTS
squamous epithelium with areas of hyperplasia. There is
also chronic inflammatory cell infiltrate present. Nasopalatine cyst
It is also called as ‘nasopalatine duct cyst’, ‘incisive canal
Management cyst’, ‘median anterior maxillary cyst’ or ‘vestigial cyst’. It
It is generally agreed that enucleation of the cyst without is the most common nonodontogenic cyst in the oral cavity.
removal of the associated tooth is the treatment of choice. It is the most common nonodontogenic cyst.
Origin covered by normal mucosa, unless it is ulcerated. If cyst

expands, it may penetrate the labial plate and produce a
It is developmental in origin and arises in the incisive canal
swelling below the maxillary labial frenum.
when embryonic epithelial remnants of the nasopalatine 367
duct undergo proliferation and cystic transformation. Teeth: Roots of central incisors diverge. It may bulge into
They are found to form in the canal or at the oral the nasal cavity and distort nasal septum.
terminus of the canal. It is generally agreed that the
Cyst of palatine papillae: Sometimes cyst formed in pala-
nasopalatine duct cyst is an entity. It may occur with in the
tine papilla will be evident as an elevation or a soft round
nasopalatine canal or in the soft tissue at the opening of the
swelling of palatine papilla which extends posteriorly along
canal where it is called as cyst of the palatine papilla.
the midline of the palate. It occurs anterior to the incisive
Etiological Factors foramen below the periosteum and does not enter invades.
Trauma: In the form of direct blow to the incisive canal Radiological Features
or indirectly from mastication, particularly when ill-fitting
dentures are involved, have been suggested. But if this is Image of radiopaque anterior nasal spine may superimpose
true, the cyst would likely be found more often and there over the dark cystic cavity giving it a heart shape or shape
would not be a male predilection. of inverted tear drop (Fig. 15.46). Two separated cysts
may develop in two canals and cause paired cysts like
Bacterial infection: Either from the nasal cavity or from radiolucency. Cyst in canal cavity also erodes the bone
the oral cavity, stimulates to the epithelial remnants to posterior to the canal and creates impression of midpalatal
proliferate has been suggested as a cause. However, since cyst.
an open connection with the oral cavity or the nasal cavity
is extremely rare, an evidence for the bacteria is lacking. Cyst of palatine papillae: The cyst in the palatine papillae
is not usually apparent because it does not cause enough
Retention phenomenon: Blocked duct of mucus glands pressure on the bone to cause discernible bone resorption
and an accumulation of secretion would be responsible for but it can produce some bony erosion.
the cystic expansion.
Epithelium: Some workers believe that it is derived from
the epithelium included in the lines of fusion of embryonic
(Figs 15.47 to 15.49)
facial processes. The cystic epithelial lining depends upon the proximity of
the lesion to the nasal cavity. The most superficially located
Clinical Features cysts are lined with respiratory epithelium while those in
Age and sex distribution: Most cases discovered in the
4th and 6th decades and it is also frequently found in
edentulous patients. It is three times higher in males than in
females and found equally in blacks and whites.
Symptoms: There is a small well defined swelling just
posterior to the palatine papilla. Sometime it may become
infected, producing pain. Patients complain of salty taste in
mouth produced by small sinus or remnant of nasopalatine
duct that permits cystic fluid to drain into oral cavity.
Burning sensation and numbness may be experience due to
pressure on the nasopalatine nerve. Sometimes cystic fluid
may drain and patient reports a salty taste.
Signs: Swelling is fluctuant and bluish if it is near the
surface. It opens by a tiny fistula on or near the palatine
papilla. In such cases a tiny drop of watery fluid or pus Figure 15.46 Nasopalatine canal cyst showing
may be elicited by pressure in this area. Deeper cysts are radiolucency in canal region
368
Figure 15.47 Nasopalatine cyst lined by respiratory epithelium Figure 15.49 Stratified squamous epithelium lining
and connective tissue capsule with prominent neurovascular nasopalatine duct cyst
bundle
before dentures are introduced. You can also go for

enucleation and if it is large, then marsupialization.
Points to Remember
Incisive canal cyst, cyst of the palatine papilla, small well
defined swelling just posterior to the palatine papilla,
salty taste, fluctuant bluish swelling, tiny fistula, diverge
roots, heart shape or shape of inverted tear drop, paired
cysts like radiolucency, respiratory epithelium, stratified
squamous variety, simple columnar epithelium, simple
cuboidal epithelial, collections of mucus glands.
MEDIAN PALATINE CYST

It is very difficult to distinguish from the nasopalatine cyst.
Figure 15.48 High power view shows pseudostratified ciliated Normally there is septopalatal contact, fusion and lamination
columnar epithelium
of adult epithelial surfaces followed by breakdown (of
opposing epithelial surface) into epithelial remnants and
the inferior position close to the oral cavity are composed actual fusion by inter-shelf mesenchymal bridge.
of epithelium of stratified squamous variety. In some cases In cases, due to continued differentiation of these
simple columnar epithelium and simple cuboidal epithelial epithelial remnants pathological median palatine cyst is
can also be seen. The connective tissue wall of this cyst formed. It may be the only true fissural cyst in oral region.
frequently shows inflammatory infiltration. It is now felt that it represent posterior extension of incisive
Collections of mucus glands are also often present as canal cyst.
well as several large blood vessels and nerves. In the cystic
fluid, erythrocytes, leukocytes, desquamated epithelial Clinical and Radiological Features
cells, tissue debris and bacteria can be found.
Site: It is very rare and develops in midline of the hard
Management palate posterior to pre-maxilla.
Its removal is not indicated unless there are clinical Symptoms: If it becomes larger, then it bulges into the oral
symptoms. Removal is indicated in edentulous patients cavity and produces a swelling in the roof of mouth.
Signs: It is fluctuant and nontender. Overlying mucosa Clinical Features

is normal. Corticated plate is rapidly perforated as the
Age and sex distribution: It ranges from 12 to 75 years
cyst grows. If floor of nasal fossa is eroded, cyst may be
with a mean age of 41 with peak in the 3rd decade. Women 369
superiorly displaced.
are more commonly affected.
Teeth: Maxillary teeth are vital and aspiration produces
amber colored fluid. Symptoms: It may cause pain and difficulty in breathing
through the nose. There is swelling of the nasolabial fold
Radiological features: Radiolucent lesion is behind the
and nose.
incisive canal in premolar-molar area. Well defined borders
which are hyperostotic. Nasal septum image crosses the Signs: Swelling is fluctuant. There is flaring of ala and
septum and appears on occlusal radiographs. distortion of nostril and fullness of upper lip below the
nasal vestibule. It may bulge into the nasal cavity and
Histopathological Features cause some obstruction. Infection may drain into the nasal
The lining of such cyst usually consists of stratified cavity.
squamous epithelium overlying relatively dense fibrous Superficial erosion of the outer surface of the maxilla
connective tissue band which may show chronic may be produced by pressure of the nasoalveolar cyst.
inflammatory cell infiltration.
In some cases, it may be lined by pseudo-stratified
ciliated columnar epithelium or even a ‘modified’ There is increased radiolucency of alveolar bone beneath
squamous epithelium. the cyst and above the apices of incisors. Cyst causes
erosion of the underlying bone by virtue of its presence
Management and pressure.
Surgical excision is done. Care should be taken not be Usual outline of inferior border of nasal fossa is
perforate the nasal mucosa. Healing defect is protected by distorted resulting in posterior convergence of the
a acrylic stent prefabricated. margins.
The actual shape and position of the cyst can be
Points to Remember demonstrated by aspirating the typical cyst fluid and
Only true fissural cyst in oral region, swelling in the roof replacing it with radio-contrast material. A tangential view
of mouth, fluctuant, nontender, nasal fossa is eroded, then demonstrates the kidney shaped lesion below the nasal
vital teeth, aspiration amber colored fluid, radiolucent fossa and above the apices of the incisors.
lesion in premolar molar area, stratified squamous
epithelium.
It may be lined by pseudostratified columnar epithelium
which is sometimes ciliated often with goblet cells or by
NASOALVEOLAR CYST stratified squamous epithelium.
It is also called as ‘nasolabial cyst’ or ‘Klestadt’s cyst’. It Inflammation may be seen in the lesion if it is secondary
is a soft tissue cyst, which involves the bone secondarily. infected.
Pathogenesis Management
Some state that it is a fissural cyst arising from epithelial It should be excised using an intraoral approach. There is
rests in the fusion lines of globular process, lateral nasal no tendency to recur.
process and maxillary process.
Others state that it is actually a merging of mesenchymal Points to Remember
processes and not a fusion per se, so there is no epithelial Nasolabial cyst, Klestadt’s cyst, pain, difficulty in
entrapment in the nasooptic fissure. They state that the breathing, swelling is fluctuant, superficial erosion of the
location of nasoalveolar cyst strongly argues in favor of its outer surface maxilla, increased radiolucency of alveolar
development from the embryonic nasolacrimal duct that bone apices of incisors, pseudo-stratified columnar
initially lies on the surface. epithelium, inflammation.
MEDIAN MANDIBULAR CYST medullary space of bone after trauma heals in most cases
by organization of the clot and eventual formation of
This cyst has got question existence. Theoretically it connective tissue and formation of new bone.
370 represent fissural cyst in the anterior midline of the According to the traumatic injury theory, however it is
mandible due epithelium entrapment during the fusion of suggested that after traumatic injury to an area of spongy
halves of the mandible during embryonic life. bone containing hemopoietic marrow enclosed by layer
But as mandibles develop as a single bilobed of dense cortical bone, there is failure of organization of
proliferation of mesenchymal with central isthmus in the blood clot and for some unexplained reasons subsequent
midline, which after growth is eliminated. So there is no degeneration of the clot that eventually produce an empty
fusion occur and so no entrapment. So the term median cavity within the bone.
mandibular cyst should be discarded. In the development of the lesion, the trabeculae of bone
Cyst occurring in this region are of odontogenic origin, in the involved area become necrotic after degeneration of
i.e. either radicular cyst, OKC or lateral periodontal cyst. the clot and bone marrow, although some viable marrow
tissue must persist to initiate resorption of the involved
GLOBULOMAXILLARY CYST trabeculae.
It is also called as ‘intra-alveolar cyst’. It occurs in The lesion then appears to increase in size by steady
globulomaxillary area. It was considered to be an inclusion expansion produced by a progressive infiltrating edema on
or developmental cyst that arises from entrapped non- the basis of restriction of venous drainage. This expansion
odontogenic epithelium in globulomaxillary suture which tends to cease when the cyst like lesion reaches the cortical
occurs at the junction of globular portion of the medial layer of the bone, so that expansion of the involved bone is
nasal process and maxillary process. not a common finding in the solitary bone cyst.
Development of anterior maxilla occurs by merging of It also origin from bone tumors that have undergone
growth center and not by fusion of facial processes so no cystic degeneration, as a result of faulty calcium metabolism
entrapment occur so it can not exist. such as that induced by parathyroid disease.
Cysts which are present in this region are either It also has a origin from necrosis of faulty marrow due
periapical cyst, odontogenic keratocyst, dentigerous cyst to ischemia. The end result of low grade chronic infection.
or lateral periodontal cyst. A result of osteoclastic activity resulting from disturbed
So as fissural cyst does not exist in this region the use circulation caused by trauma thereby creating an unequal
of term globulomaxillary cyst should be discarded. balance of osteoclastic activity and repair of bone.
Clinical Features
NONEPITHELIAL CYSTS
Age and sex: The traumatic bone cyst occurs most fre-
Traumatic Bone Cyst quently in young persons at an age of 6 to 20 years with a
male predominance as they are exposed to traumatic injury
It is also known as ‘solitary bone cyst’, ‘hemorrhagic bone
most frequently than females with the ratio being 3:2.
cyst’, ‘extravasation cyst’, ‘simple bone cyst’, ‘unicameral
cyst’ and ‘idiopathic bone cavity’. Site: It is usually found in mandible anywhere from the
It is an unusual lesion which occurs with considerable symphysis to the ramus, but about one third are found in
frequency in the jaws as well as in other bones of the the maxilla, usually in the anterior region.
skeleton. The traumatic bone cyst is a misnomer, since Symptoms: It is asymptomatic in most cases but
these intrabony cavities are not lined by epithelium. It occasionally, there may be evidence of pain and tenderness.
results from trauma induced intramedullary hematoma
with subsequently result in bone resorption and cavitations Signs: Cortical swelling or slight tooth movement are not
during hematoma resolution. the usual finding and the teeth are vital.
Aspiration: Needle aspiration is actually unproductive and
Pathogenesis if it is productive it contains either a small amount of straw
It originates from intramedullary hemorrhage following colored fluid shed off necrotic blood clot and fragment of
traumatic injury. Hemorrhage occurring within the fibrous connective tissue.
Radiographic Features Management

It appears as a radiolucent lesion with a spectrum of well This lesion must be surgically exposed to rule out the
defined to moderately defined borders (Fig. 15.50). Most possibility that the lesions of serious nature are not being 371
cases are unilocular with a fairly regular border. There is confronted.
evidence of hyperostotic borders around the entire lesion When the correct diagnosis is determined, enucleation
but occasionally such border is lacking. Most characteristic and curettage are carried out.
radiographic feature of this cyst is scalloped superior or Now a day intralesional injection of steroids is also one
occlusal margins where it extends between the roots of the of the treatment modalities.
teeth.
Teeth may be superimposed with the root or ‘scallop’ Points to Remember
superiorly between the roots. Occasionally lamina dura of Solitary bone cyst, simple bone cyst, intramedullary
the tooth may be absent and even less frequently the root hemorrhage, symphysis to the ramus, asymptomatic,
may show resorption. cortical swelling, unproductive needle aspiration,
It rarely causes cortical expansion but if it occurs it is radiolucent lesion well defined to moderately defined
mostly buccal. Surface is smooth, the cortical plates are borders, scalloped superior or occlusal margins, vascular
not disrupted and pathological fracture does not result. The fibrous tissue, fragments of fibrin with enmeshed red
cavity occupies the position in the body of the jaw, but it cells, stringy lacelike dystrophic calcification.
may extend to involve the alveolar process.
Histopathological Features ANEURYSMAL BONE CYST

The simple bone cyst consists of loose vascular fibrous tissue It is an uncommon hemorrhagic lesion of the bone
membrane of variable thickness with no epithelial lining. which is rarely seen in the jaw. It was first described as a
Fragments of fibrin with enmeshed red cells may be clinicopathological entity by Jaffe and Lichtenstein in 1942.
seen. Hemorrhage and hemosiderin fragments are usually It is most often categorized as to be a tumor like reactive
present and scattered small cells are often found. lesion of bone. The name of this entity is misleading, in
Stringy lacelike dystrophic calcification is also seen in that, it does not contain vascular aneurysms and it is not a
some cases. true bony cyst.
It represents an exaggerated localized proliferative
response of the vascular tissue. It may be similar to
peripheral and central giant cell granuloma to which it
resembles in some ways including the histological presence
of giant cells.
Pathogenesis
Persistent local alteration in hemodynamics leads to
increased venous pressure and development of dilated and
engorged vessels in transformed bone area. Resorption of
bone occurs, to which giant cells are related and this is
replaced by connective tissue, osteoid and new bone.
It has exuberant attempt at repair of hematoma of
bone, similar to that of central giant cell granuloma. But,
in the case of aneurysmal bone cyst, it is postulated that
hematoma maintains a circulating connection with the
Figure 15.50 Traumatic bone cyst showing well defined border damaged vessel.
This would lead to a slower flow of blood through the Simple tilting and bodily displacement of erupted teeth
lesion and account for a clinical “welling” of blood. This is seen. Some degree of external root resorption may be
is the only difference between the aneurysmal cyst and the seen though it will not devitalize the tooth. There is also
372 giant cell granuloma that is in later lesion, the blood vessels buccal and lingual expansion of the cortex often marked
fail to retain circulating connection with lesion. and described as ballooning or blowing out.
Biesecker and his associates have proposed a new
hypothesis for etiology and pathogenesis of this lesion Histopathological Features
that a primary lesion of the bone initiates an arteriovenous (Figs 15.51 and 15.52)
fistula and thereby creates, via its hemodynamic forces, a The lesion consists of mainly capillaries and blood filled
secondary reactive lesion of the bone. Thus occurrence of spaces of varying sizes, lined by flat spindle shaped cells
this cyst is secondary in association with osseous lesions and separated by delicate loose textured fibrous tissue. It
like nonosteogenic fibroma, benign osteoblastoma and contains many cavernous sinusoidal blood filled spaces
hemangioma of bone. which may or may not show thrombosis.
Young fibroblasts are numerous in the connective tissue
Clinical Features stroma. Most of the cysts contain small multinucleated
Age and sex distribution: Although, it may be seen giant cells with a patchy distribution. Scattered trabeculae
in adults, it is more commonly seen as abnormalities of osteoid and woven bone are seen.
of older children and adolescents with more than 90
percent of lesions occuring in individuals younger than Management
the age of 30 years. It is more common in females than Surgical curettage or partial resections are the primary
in males. forms of treatment for aneurysmal bone cyst. Cryosurgery
and immediate packing with bone chips is the treatment
Site: It is most commonly seen in long bone or in the
of choice. The recurrence rate is fairly high, ranging form
vertebral column. Intraorally it usually involves the
5 to 19 percent after curettage. Thus indicate the need for
mandibular molar region as compared to anterior region.
careful follow-up.
Symptoms: Aneurysmal cyst of the jaw produces a firm
swelling which may be painful and tender on motion. Points to Remember
Swelling becomes progressively worse and the rate of Persistent local alteration in hemodynamic, welling” of
development is often described as rapid. Sometimes blood, mandibular molar region, firm swelling, painful,
patient may complain of difficulty in opening the mouth, tender on motion tilting or bodily displacement of teeth,
i.e. if there is impingement of the lesion on the capsule of springiness or egg shell crackling, expansile osteolytic,
temporomandibular joint (TMJ). soap bubble, flat spindle shaped cells, young fibroblasts,
Signs: Usually there is tilting or bodily displacement of scattered trabeculae of osteoid and woven bone.
teeth in the affected areas though it does not devitalize the
affected tooth. Excessive bleeding may occur. When the CYSTS OF THE MAXILLARY SINUS
lesion perforates the cortex and is covered by periosteum
or only a thin shell of bone, it may exhibit springiness or Sinus Mucocele
egg shell crackling, but it is not pulsatile. Bruit is not heard. The Mucocele of the maxillary antrum is a true cyst
filled with mucus and lined by the mucoperiosteum of the
Radiographic Features involved maxillary sinus.
The aneurysmal bone cyst is an expansile osteolytic process A true antral mucocele sometimes completely fills the
within the affected bone and is projected as a definite sinus and is caused by blockage of the ostium, which may
radiolucency. Invariably, fine septa are seen crossing be secondary to inflammatory changes associated with
through the lesion in a random pattern. chronic rhinosinusitis.
The term ‘soap bubble’ may be applied to describe an
occasional multilocular radiographic appearance. Margin Pathogenesis
are somewhat less regular and distinct than odontogenic Blockage of sinus ostium: The ostium block results in
cyst but more discrete than a central malignancy. mucus retention which results in cyst initiation. This is

Sigs: Mucocele may cause expansile ballooning resulting
into destruction and perforation of the surrounding bone 373
and displacement of adjacent structures. Sometimes it
presents severe signs to warrant as another aggressive
disease. There may be nasal blockage, or proptosis.
Site: Mucocele are more common in frontal and ethmoid
sinus than the maxillary sinus. The incidence accounts only
for 10 percent.
Age: Age affected is middle age group with average being
48.2 years.
It usually occupies the entire sinus. Cystic structure is
filled with mucus and lined by antral epithelium. Associated
Figure 15.51 Aneurysmal bone cyst with cavernous with blockage of the ostium and may be secondary to
sinusoidal blood filled spaces chronic sinusitis.
Radiographic features: There can be cloudy radiopacity
which may be noticeable but in the early lesion the bony
walls of the antrum appear normal. As the lesion develops
there may be a well-defined radiolucency with expansion
and perforation of the bone margins (Fig. 15.53). The
lesion is often quite spherical in outline. There is always
medial expansion .The lesion may also raise the orbital
floor or the anterior wall of the antrum. Expansion of the
thicker lateral wall is rare.
Figure 15.52 Multinucleated giant cells seen
further added by the increase in the protein content and

osmolarity of the cyst resulting in expansion by hydrostatic
pressure. This is accompanied by inflammation and
activation of bone resorbing factors and osteoclasts which
allow bony expansion and destruction of adjacent tissues.
Trauma or surgery to sinus: This type of cyst called
as ‘surgical ciliated cyst’, traumatic ciliated cyst’, or
postoperative maxillary cyst’. Most frequently seen after
Caldwell Luc operation. In his sinus lining becomes
separated from main body of sinus and forms epithelium Figure 15.53 Mucocele in left maxillary sinus showing
line cavity in which mucin is secreted. perforation of border of maxillary sinus
Histopathological Features Radiological features: The cysts appear as spherical,

ovoid or dome-shaped radiopacities which have a smooth
The maxillary mucocele is lined by normal antral mucosa,
and uniform outline. They may have a narrow or broad
374 covered by ciliated respiratory type epithelium or flattened
base. They vary in size from minute to very large and may
simple cuboidal or squamous epithelium. There may
occasionally occupy the entire maxillary sinus.
be chronic inflammation in the wall and if there is an
associated allergic sinusitis. Histopathological Features
Management It has a smooth blue surface, is thin-walled and contains
As these cysts are expansile and destructive surgical mucinous material. Microscopically, a pseudocyst shows
removal should be done. pools of mucoid material lined by inflamed fibrous connective
tissue which in places may be the raised periosteum.
Points to Remember Collection of cholesterol cleft and scattered small
dystrophic calcification can be seen.
Blockage of sinus ostium, trauma or surgery to sinus,
Antral mucosa may be seen on one aspect. Ducts may
expansile ballooning destruction and perforation of
be seen. A calculus or mucus plug may be evident.
the surrounding bone occupies the entire sinus, cloudy
radiopacity, expansion and perforation of the bone Management
margins, ciliated respiratory type epithelium flattened
simple cuboidal or squamous epithelium. Retention cysts and pseudocyst are not destructive
and usually remain static, many appear to regress
spontaneously, and as they usually cause little discomfort
ANTRAL PSEUDOCYST surgical intervention may not be necessary.
These are dome shaped cyst seen on sinus floor.
Points to Remember
Pathogenesis Focal accumulations of inflammatory exudate asymp-
These are the result of focal accumulations of inflammatory tomatic, localized dull pain in the antral region, spherical,
exudate that lift the antral mucosa away from the underlying ovoid or dome-shaped radiopacities, mucinous material,
bone. This will cause sessile elevation of sinus floor. The inflamed fibrous connective tissue, cholesterol cleft,
exudate is surrounded by connective tissue and epithelial calculus, mucus plug.
lining is superior to the fluid.
There is increase prevalence of this lesion in winter
RETENTION CYST
months. So many authors believed that upper respiratory
infection can lead to this cyst. They arise from partial blockage of duct of seromucous
glands. They may arise from invagination of respiratory
Clinical Features epithelium. They are located around the ostium or in antral
Age and sex distribution: These cysts are seen in the age polyps.
group 21 to 30 years with equal incidences in both sexes. Histopathologically sinus retention cyst show focal
dilatation of duct associated with seromucous glands of
Site: Usually the cyst occurs singly, but in a few instances sinus lining.
they may be multiple and sometimes bilateral.
Symptoms: These may be asymptomatic, the lesions SOFT TISSUE CYST
being discovered only in the course of routine radiological
examination. Patients may complain of a wide range of Dermoid and Epidermoid Cyst
symptoms such as a localized dull pain in the antral region, It may occur as developmental anomalies and about 1 to
or fullness or numbness of the cheek, nasal obstruction, 2 percent occurs in oral cavity. The lumen of simple cyst
postnasal drip and a copious discharge of yellow fluid from filled with cyst fluid or keratin and no other specialized
the nostrils. The symptoms may be associated with sinusitis. structure, is called as ‘epidermoid cyst’.
If lumen contains sebaceous material as well as keratin

then it is called as ‘dermoid cyst’. If lumen contains
elements such as bone, muscle or teeth from various
germinal layer is called as ‘teratoma’. 375
Dermoid cyst is lined by epidermis and skin appendages
are present in the fibrous wall. Epidermoid cyst is lined by
epidermis, but contains no appendages.
Pathogenesis
It is most likely site for their origin is anteriorly, between
the contributions from the mandibular arches to the tongue.
Implantation keratinizing epidermoid cyst may occur in
other parts of the mouth as a result of trauma.
Types Figure 15.54 Dermoid cyst seen on forehead of the patient

• Median variety
– Supramylohyoid variety
– Inframylohyoid variety
• Lateral variety
– Supramylohyoid variety
– Inframylohyoid variety.
Clinical Features
Age and sex: It occurs at any time from birth to adolescence
and it is small in infancy and large in adolescence. Mainly
it is apparent between 12 to 25 years of age and occurrence
is equal in both sexes.
Site: Midline of the floor of mouth is the commonest
location of these cysts which may cause a swelling in the
midline of the neck or some times, it may be lateral.
Symptoms: Swelling is slow and painless. It may interfere
Figure 15.55 Dermoid cyst lined by keratinized epidermis
with breathing, speaking, closing the mouth and eating.
and shows dermal appendages, the lumen is filled with
Sigs: The size may vary up to several centimeters keratin
in diameter. Fluctuation test is generally positive. If
superficial, it is yellow to white and surface is smooth and
non ulcerated until traumatized. Soft to firm, fluctuant, Histopathological Features (Fig. 15.55)
rubbery or cheesy sharply delineated with straw colored
They are lined by orthokeratinized epidermis. Cysts of
fluid (Fig. 15.54). It may be fluctuant and doughy
the floor of the mouth lined predominately by secretory
depending on the content. It does not move with protrusion
epithelium are probably of salivary duct origin.
of the tongue or deglutition.
Dermoid cyst is characterized by presence of one or
Double chin variety: It occur in inframylohyoid variety more dermal appendages such as hair follicles, sweat
when it causes swelling in the sub-mental area and it give glands or sebaceous glands in the wall with the lumen
rise to a ‘double chin’ appearance. usually filled with keratin.
Management
Surgical excision is the treatment of choice. It should
376 be excised through the floor of mouth by retracting the
posterior border of the mylohyoid muscle.
Points to Remember
Midline of the floor of mouth, painless swelling,
fluctuation test positive, soft to firm, rubbery or cheesy,
double chin appearance, orthokeratinized epidermis, one
or more dermal appendages.
BRANCHIAL CLEFT CYST

It is also called as cervical lymphoepithelial cyst.
Figure 15.56 Branchial cyst lined by stratified squamous
Pathogenesis epithelium. The cystic wall typically contains lymphoid tissue
It develop from remnant of branchial cleft as these cyst
occur in embryonic gill arch apparatus. ORAL LYMPHOEPITHELIAL CYST
Another version is that it arises from parotid epithelium
that becomes entrapped in upper cervical lymph nodes It originates from cystic transformation of glandular
during embryonic life. epithelium entrapped within the oral lymphoid aggregates
They usually develop from 2nd branchial arch. during embryogenesis. Epithelium in lymphoepithelial cyst
might be derived from the ductal epithelium of salivary
Clinical Features glands.
Age: It occurs in the age group of 20 to 40 years. Lymphoid tissue consists of Waldeyer’s ring which
includes palatine tonsil, lingual tonsil and pharyngeal
Site: Location is superficially in the neck, close to the adenoids. This tissue has a close relationship overlying
angle of the mandible, anterior to the sternocleidomastoid mucosal epithelium. This epithelium invaginates into
muscle. Two third appear on left side and one third appear tonsillar tissue resulting in pouches or tonsillar crypts.
on right side. These crypts filled with keratin debris producing keratin
Appearance: The neck lesions vary in size from small to filled cyst below the mucosal surface.
very large (about 10 cms in diameter) soft fluctuant mass.
Clinical Features
Symptoms: There is swelling, which may be progressive
or intermittent and pain may also be a feature. Age: The age ranges from 16 to 69 years with a there was
male to female ratio of 3:1.
Histopathological Features (Fig. 15.56) Site: It affects mainly the floor of mouth and the tongue.
Cyst is lined by stratified squamous epithelium which may These cyst may develop in the area of palatine tonsil.
or may not be keratinized.
Appearance: The cyst appears as a nonulcerated freely
Wall of cyst contain lymphoid tissue, germinal center
movable mass which has been present for a period ranging
formation.
from 1 month to a year.
Management Symptoms: Patients may complain of swelling and
Surgical removal should be done and this cyst never recurs. discharge.
Sigs: Size may vary from few mm to 2 cm fairly mobile,
Points to Remember
superficial soft fluctuant, sharply delineated swelling. The
Anterior to the sternocleidomastoid muscle, soft fluc- usual observation was of round or oval swelling of the oral
tuant mass, pain, stratified squamous epithelium, mucosa of normal color but when large swelling they were
lymphoid tissue, germinal center formation. yellow or white in color.
Histopathological Features Pathogenesis

Cyst lined with thin stratified squamous epithelium, usually Gorlin pointed out that in the 3 to 4 mm embryo, the
parakeratinized devoid of rete pegs. The lining epithelial is undifferentiated primitive stomach lies in the mid-neck 377
quite thin; lacking rete pegs and is usually parakeratinized region, not far from anlage of the tongue. Gastric mucosa
and occasionally orthokeratinized. has been shown to occur in the esophagus of 7.8% of infant
The lumen of the cyst often contains sloughed epithelial and in 51 percent of these, heterotrophic tissues were
cells, lymphocytes and eosinophilic amorphous coagulum. located in the upper third.
Occasionally, lining epithelium is of columnar type with or They suggested that in the sublingual region of the oral
without goblet cells. cavity and in the region of apex and dorsum of tongue, the
ectodermal and endodermal epithelia fuse and this will explain
Management presence of heterotrophic gastric or intestinal mucosa.
Local surgical excision should be carried out.
Clinical Features
Points to Remember Age and sex: Most cases occur in infants and young
Nonulcerated freely movable mass, swelling, parakerati- children between the ages of 2 to 11 years. The male to
nized devoid of rete pegs, sloughed epithelial cells, lym- female ratio is 3:1.
phocytes. Site: Common location in the anterior part of the tongue,
floor of mouth and submandibular gland. The cyst may be
THYROGLOSSAL DUCT CYST enclosed entirely within the tongue or floor of mouth or
may communicate with the surface.
It is described in chapter of tongue.
ANTERIOR MEDIAN LINGUAL CYST The cyst may be lined partly by stratified squamous
It is also called as intralingual cyst of foregut origin. epithelium and partly by gastric or intestinal mucosa.
Management
Clinical Features
Surgical excision. Recurrence is rare but has been reported.
Age: It is a rare lesion and is commonly found in children.
It can be present since birth and recurrence is common. Points to Remember
Site: It is a fluctuant swelling in anterior half of the tongue. Oral alimentary tract cyst, anterior part of the tongue,
Symptoms: There may be difficulty in feeding. stratified squamous epithelium, gastric or intestinal
mucosa.
It is lined by pseudo-stratified ciliated columnar epithelium CYSTIC HYGROMA
and by cuboidal epithelium.
It is a developmental abnormality in which there is
Points to Remember progressive dilatation of lymphatic channels.
Intralingual cyst of foregut origin, fluctuant swelling, Clinical Features
difficulty in feeding, pseudo-stratified ciliated columnar
Site: It frequently involves the neck and face, although it
epithelium.
can occur anywhere in the body.
Age: It is often present at birth and most cases are diagnosed
ORAL CYST WITH GASTRIC OR before the age of 2 years.
INTESTINAL EPITHELIUM Symptoms: Those that involve facial tissue produce a
It is also called as ‘oral alimentary tract cyst’. swelling often painless and usually compressible.
Signs: The overlying skin may be blue and the swelling Management
transilluminates. There may be a history of gradual or
Conservative surgical excision can be carried out.
sudden enlargement.
378
Points to Remember
Head, face, nodular fluctuant subcutaneous lesion,
Histologically, the cystic hygroma consists of dilated cystic
stratified squamous epithelium resembling epidermis,
spaces lined by endothelial cells.
Management
Complete surgical removal of the mass should be done. NASOPHARYNGEAL CYST
They are rare clinical entities. They may be classified as
Points to Remember congenital or acquired and in midline or lateral.
Swelling often painless, blue overlying skin, dilated They are lined by ciliated or non ciliated columnar
cystic spaces lined by endothelial cells. epithelium with areas of squamous metaplasia in response
to inflammatory stimuli.
Lymphoid follicles are present in the wall. Congenital
FOLLICULAR CYSTS OF THE SKIN midline cyst arises either from the pharyngeal bursa or
These are keratin filled lesion which arises from hair from Rathke’s pouch. Cysts arising from Rathke’s pouch
follicle. These cyst arise after localized inflammation of are exceedingly rare. They have a median base attached to
hair follicles. the nasopharyngeal vault and lie anterior to the usual site
of origin of retention and pharyngeal bursa cyst. They are
Types lined by stratified squamous epithelium, in keeping with
• Infundibular cyst or epidermoid cyst: It is derived their ectodermal origin.
from follicular infundibulum
• Pilar, tricholemmal or isthmuscatagen cyst: These THYMIC CYST
are located on scalp They are rare clinical entities, which arise, in persistent thymic
• Epidermal inclusion (implantation) cyst: These tissue, which may occur in any location between the angle
occur after traumatic implantation of epithelium. of the mandible and midline of the upper neck to the sternal
notch. Histologically, the cyst is lined by squamous and
Clinical Features cuboidal epithelium and thymic tissue is present in the wall.
Age and sex distribution: Young patient have it on face CYSTS OF SALIVARY GLANDS
and older patient have it on back. Males are commonly
affected than female. In case of pilar cyst female is It is described in chapter of salivary gland disorders.
commonly affected than male.
Location: It is seen in head, face, neck, and back of the PARASITIC CYST
patient.
Hydatid Cyst
Appearance: It appear as nodular fluctuant subcutaneous
It is also called hydatid disease or echinococcosis. It is
lesion which can be associated with inflammation.
caused by the larvae E. granulosus, the dog tapeworm and
Sign: Lesion is movable and shells out easily. E. multilocularis.
Histopathological Features Pathogenesis
Cavity is lined by stratified squamous epithelium resem- This tapeworm lives in the intestinal tract of the dog. Its ova
bling epidermis. Granular cell layer is also seen. are excreted in the faeces of the dog and may be ingested
In some case prominent granulomatous inflammatory by the intermediate host like cattle sheep and pigs.
reaction like multinucleated giant cells can be seen in Man is also susceptible as an intermediate host as dogs
lesion. Pilar cyst granular cell layer is absent. are common household pets, so may accidentally ingest the
ova. The ingested ova hatch in the upper gastrointestinal Management

tract, from where the small embryo permeate the intestinal
The ideal treatment for hydatid cyst is surgical. Limiting
mucosa and are distributed through the blood vessels and
contact between dogs and humans and deworming dogs at 379
lymphatics to all parts of the body.
regular intervals are useful steps towards prevention.
Site: It is more common in liver, but others are found in Echinococcosis, larvae E. granulosus, asymptomatic,
lung, bones and brain. hepatomegaly, jaundice, intraorally tongue, painless,
Symptoms: Usually they are asymptomatic but they progressively increasing, soft, elastic, speech and
enlarge progressively to cause pressure symptoms. mastication difficulties, outer layer fibrous tissue
chronic inflammatory cells, intermediate layer white,
Signs: Liver involvement causes hepatomegaly and nonnucleated and consists of numerous delicate
jaundice. Pulmonary hydatid cyst may cause shortness of laminations, innermost layer nucleated germinal layer,
breath and hemoptysis. hydatid sand.
Complications: Rare complication of hydatid cyst includes
rupture, suppuration and calcification of the cyst. CYSTICERCOSIS CELLULOSE
Oral Manifestations Pathogenesis
Site: Intraorally, found in tongue and angle of mandible. Man develops cysticercosis through the larval form,
Appearance: Oral hydatid cyst presents as painless, pro- cysticercus cellulose, of the pork tapeworm Taenia solium.
gressively increasing, soft, elastic and well circumscribed He can act as both the intermediate and the definitive host.
fluctuant swelling. The adult worm may be ingested from inadequately heated
or frozen pork. Alternatively, man may ingest the cysticerci
Symptoms: They may pose speech and mastication themselves from infested pork and these develop into adult
difficulties. worms.
These lives attach to the wall of the small intestine
where they fully grow and at times reach a length of 7 mm.
The mature cyst consists of three layers, one of host and Gravid proglottids or eggs begin to drop off and are passed
two of parasitic origin. in the feces.
The outer layer which is derived from the host is In this way, man through contaminated food or from
made up of fibrous tissue and is infiltrated by chronic their own dirty hands may ingest them or they may be
inflammatory cells, eosinophils, lymphocytes and giant regurgitated into the stomach. In the stomach, their covering
cells. is digested off and the larval forms are hatched. These
The intermediate layer is white, non-nucleated and penetrate the intestinal mucosa and are then distributed
consists of numerous delicate laminations. It usually through the blood vessels and lymphatics to all the parts of
shrinks away from the outer fibrous layer when the tension the body where they develop into cysticerci.
within the cyst is relieved.
Innermost layer is a nucleated germinal layer. Clinical Features
The cystic fluid is usually clear, albumin free and It depends upon the site and the number of the cysticerci
contains the so called hydatid sand consisting of brood in the body. During the stage of invasion there are no
capsule and scolices. The brood capsules or daughter cysts symptoms or slight muscular pain and mild fever may be
develop originally as minute projections of germinative present. CNS involvement produces serious effects.
layer which develops a central vesicle and become a minute
cyst. Oral Manifestations
Scolices of the head of the worm develop in the inner There is very little report about cysticercosis of oral
aspect of the brood capsule. It is when they separate from region. In oral cavity, most common site involved is the
the germinative layer that they form the hydatid sand. tongue. Other sites involved are cheek and lips. There is
a firm mass and contains watery fluid and coiled white

structure apparently attached to the inner aspect of cyst
(Fig. 15.57A). It is a painless, well circumscribed, elastic
380 and fluctuant swelling.
It shows dense fibrous outer capsule which is derived
from host tissue (Fig. 15.57B). This contains a fairly dense
inflammatory cell infiltrate consisting predominantly of
lymphocytes, plasma cells and histiocytes.
Few foci of dystrophic calcification are present in this
capsule and some of these are concentrically laminated.
Within the capsule is a delicate double layered membrane
Figure 15.58 Lesion show delicate double layered membrane

consisting of the outer acellular hyaline, eosinophilic layer and
inner cellular layer (High power view)
consisting of an outer acellular hyaline, eosinophilic layer

and an inner sparsely cellular layer (Fig. 15.58). This layer
has a loose attachment to the fibrous capsule and is readily
torn away from it. The cyst lies within this membrane and
contains larval form of T. solium.
Management
Cutaneous cyst should be surgically removed. Mebendazole
has some value in the treatment. Pork should be properly
cooked as preventive measures.
Figure 15.57A Gross specimen hydatid cyst

Points to Remember
Taenia solium, no symptoms, tongue common site,
fairly dense inflammatory cell infiltrate consisting, foci
of dystrophic calcification, larval form of T. solium,
mebendazole.
SYNDROMES ASSOCIATED WITH

ODONTOGENIC CYSTS
Jaw Cyst-Basal Cell Nevus-bifid Rib Syndrome
It is also called as ‘nevoid basal cell carcinoma’, ‘Gorlin’s
and Goltz’s syndrome’. It is transmitted as autosomal
dominant trait with poor degree of penetrance and variable
expressivity.
Clinical Features
Figure 15.57B Cysticercosis cellulose the outer fibrous Age: It appears early in life after 5 years and before 30
capsule and inner T. solium (Low power view) years.
Cutaneous anomalies: Basal cell carcinoma, dermal cyst

Points to Remember
and tumors, palmar pitting, palmar and plantar keratosis
Cutaneous anomalies, skeletal abnormalities, ophthal-
and dermal calcinosis. Nevoid basal cell carcinoma is
mologic abnormalities, neurological anomalies, sexual 381
brownish colored papules predominant on skin, neck and
abnormalities, multiple odontogenic keratocysts, radio-
trunk. Skin lesions are small, flattened, flesh colored or
lucency of variable size, foci of calcification, calcified
brownish papules occurring anywhere in the body, but are
falx cerebri.
prominent on the face and trunk. Basal cell carcinoma is
less aggressive in this syndrome than in solitary basal cell
carcinoma. TREATMENT OF CYSTS
Skeletal abnormalities: Rib anomalies and brachymeta- Regression of Cysts without
carpalism, bifid rib, agenesis, deformity and synostosis Surgical Treatment
of rib; kyphoscoliosis, vertebral fusion, polydactyly and
shortening of metacarpals. There is good evidence that some small radicular cyst
will regress if the necrotic pulp remnant and bacteria are
Ophthalmologic abnormalities: Hypertelorism with wide removed from the root canal of the causative tooth and the
nasal bridge, dystopia canthorum, congenital blindness and canal is effectively filled. Circumscribed rounded zone
internal strabismus. of periapical bone destruction perhaps up to 1.5 cm in
Neurological anomalies: Mental retardation, calcification diameter will be seen in some patients to reduce in size and
of falx cerebri and other parts of dura, agenesis of corpus resolve over a period of month.
callosum, congenital hydrocephalus and occurrence of me-
Marsupialization of Dental Cysts
dulloblastomas.
It is known as ‘Partsch’s operation’ as it is discovered
Sexual abnormalities: Hypogonadism in males and by Partsch. It involves making an opening into the cyst
ovarian tumors. as large as practical and packing the cavity. It is a more
effective way of emptying a cyst.
Oral Manifestations
Jaw lesions appear as multiple odontogenic keratocysts Indications
usually appearing in multiple quadrants. Mild mandibular Age: In a young child, with developing tooth germs.
proganthism is seen.
Proximity to vital structure: When proximity of the
Radiographic Features cyst to vital structure can create an oronasal or oroantral
fistula or injure the neurovascular structure, this treatment
Jaw cysts appear as a multiple cyst like radiolucency of modality should be considered.
variable size varying from few millimeters to several
centimeters. They occur most frequently in premolar- Eruption of teeth: In a young patient with a dentigerious
molar region. Radiopaque lines of calcified falx cerebri are cyst, marsupialization will permit the eruption of the
prominent on PA projection. unerupted tooth or any other developing tooth which has
been displaced.
Histopathological Features Vitality of teeth: When the apices of many adjacent
They are odontogenic keratocyst. They have more satellite erupted teeth are involved within a large cyst, enucleation
cyst, solid island of epithelial proliferation. Foci of can prejudice the vitality of these teeth.
calcification is also seen.
Advantages
Management ∙ The procedure at least for large cysts, is technically
Complete enucleation of the lesion of OKC should be done. simple.
Periodic examination and genetic counseling should be ∙ Even quite large cysts can be dealt with under local
done. anesthesia. As anesthesia of deeper recesses is not
essential and this is particularly an advantage in the Disadvantages

maxilla.
∙ After primary closure, it is not possible directly observe
∙ Because the deeper part of the lining is not disturbed,
382 the healing of the cavity as with marsupialization.
adjacent important structures are not put at risk, i.e. the
∙ In young persons, the unerupted teeth in a dentigerious
blood vessels to the apices of adjacent vital teeth, the
cyst will be removed with the lesion in this mode of
inferior dental neurovascular bundle and the integrity
treatment.
of lining of the antrum or nose are well preserved.
∙ Removal of large cysts will weaken the mandible
∙ Marsupialization may be the best way to conserve the
making it prone to jaw fracture.
tooth of origin of a dentigerous cyst and to permit its
∙ Damage to adjacent vital structures.
eruption.
∙ Pulpal necrosis.
∙ It is the simplest way to treat a fracture complicating a
large cyst of the mandible.
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dontogenic cyst epithelium keratinization in odontogenic Dentistryfor Children. 1988;55:288-90.
384 8. Arcading pattern of ret pegs and Rushton bodies

1. Which one of the following is an example of non- present in:
odontogenic cyst: a. Radicular cyst
a. Eruption cyst b. Gorlin’s cyst
b. Nasolabial cyst c. Paradental cyst
c. Primordial cyst d. Nasopalatine cyst
d. Glandular odontogenic cyst
9. Cholesterol clefts is present in:
2. Following are the cysts of soft tissues of mouth, face a. Median mandibular cyst
and neck except: b. Radicular cyst
a. Dermoid cyst c. Nasopharyngeal cyst
b. Thyroglossal duct cyst d. Traumatic bone cyst
c. Paradental cyst
d. Cystic hygroma 10. Connective tissue capsule with prominent neuro-
vascular bundle and respiratory epithelium present in:
3. A thin layer of nonkeratinized stratified squamous a. Nasopalatine cyst
epithelium with absence of rete pegs is histopathological b. Radicular cyst
feature of: c. Paradental cyst
a. OKC d. Dentigerous cyst
b. Eruption cyst
c. Primordial cyst 11. Dentigerous cyst associated with:
d. Dentigerous cyst a. Gardner syndrome
b. Ectodermial dysplasia
4. Multiple OKC are found in the following syndromes, c. Maroteaux-Lamy syndrome
except: d. Gorlin-Goltz syndrome
a. Gorlin-Goltz syndrome
b. Marfan syndrome 12. Percoronal cyst is:
c. Costen syndrome a. OKC
d. Noonan’s syndrome b. Gorlin cyst
c. Dentigerous cyst
5. A corrugated parakeratinized stratified epithelium d. Gingival cyst of newborn
without rete ridges seen in:
a. OKC 13. Gingival cyst of newborn originates from:a:
b. Dentigerous cyst a. Remnants of dental lamina
c. Gorlin’s syndrome b. Epithelial rest of Malassez
d. Glandular odontogenic cyst c. Oral epithelium
6. ‘Picket fence’ or ‘tombstone pattern’ seen in:
a. Dentigerous cyst 14. Bohn’s modules present on:
b. Primordial cyst a. Hard Palate
c. Radicular cyst b. Soft Palate
d. Dental lamina cyst c. Junction of hard and soft palate
7. Focal areas of stellate reticulum and ghost cells present
in: 15. Features of basal cell nevus syndrome are:
a. Gingival cyst of adults a. Palmar and plantar keratosis
b. Lateral periodontal cyst b. Bifid rib
c. Gorlin’s cyst c. Multiple odontogenic keratocyst
d. Radicular cyst d. All of the above
16. The radiological variant of the dentigerous cyst may 19. Histologic features which are considered as mani-
be: festations of developing neoplasia in a dentigerous cyst
a. Lateral type b. Circumferential type is/area:
c. Central type d. All the above a. Hyperchromatism of basal cell nuclei 385
17. The treatment of choice of eruption cyst is: b. Palisade arrangement with polarization of basal cells
a. Surgical newborn c. Cytoplasmic vacuolization with inter cellular spacing
b. Curettage of lining epithelium
c. Marsupilization d. All of the above
d. No treatment 20. ‘Envelopment’ radiological type of keratocyst is
18. Nodules filled keratin are found along the midpalatine referred as:
raphe are: a. Those which forms in place of normal teeth
a. Dental lamina cyst b. Those in the ascending ramus away from the teeth
b. Bohn’s modules c. It is referred to a varity of keratocyst which embraces
c. Epstein’s pearls an adjacent unerupted tooth
d. None of the above d. None of the above
16 Periodontal Pathology
Vivek Thombre, Anil Govindrao Ghom, Shubhangi Mhaske (Jedhe)
Chapter Outline
Â Fibromatosis gingiva Â Gingival enlargement due to drugs

Â Retrocuspid papilla Â Pregnancy tumor
Â Gingival inflammation or gingivitis Â Granuloma pyogenicum
Â Necrotizing ulcerative gingivitis Â Periodontal pockets
Â Desquamative gingivitis Â Adult periodontitis
Â Plasma cell gingivitis Â Rapidly progressive periodontitis
Â Granulomatous gingivitis Â Aggressive periodontitis/Juvenile periodontitis
Â Gingival abscess Â Papillion-Lefevre syndrome
Â Pericoronal abscess Â Haim-Munk syndrome
Â Chronic inflammatory enlargement
Diseases of the periodontium are commonly encountered in soft tissue and bone. It may be familial, i.e. hereditary
in daily practice. It is one of the implicated causes having autosomal dominant as well as recessive type or
of loss of teeth in adults. In nearly every case, the idiopathic.
condition begins as a minor localized disturbance which
unless adequately treated, may gradually progress to Clinical Features
the resorption of alveolar bone and exfoliation of tooth. Age: It may be present at birth or may become apparent
Periodontal diseases are classified mainly into two groups, with eruption of deciduous or permanent teeth. Mostly, the
on the basis of pathological process, i.e. inflammation enlargement begins before age of 20.
(gingivitis, periodontitis) and dystrophy (gingivosis and
Syndrome associated: The familial variations may occur
periodontosis).
as an isolated finding or in association with one of the
hereditary syndrome like ZimmermannLaband, Murray
FIBROMATOSIS GINGIVA PureticDrescher, Rutherford, Multiple Hamartoma, and
It is also called elephantiasis gingivae, congenital Cross Syndrome.
macrogingivae. It is a slowly progressive diffuse over Other findings such as hypertrichosis, epilepsy,
growth of gingival fibrous connective tissue. Inconsistent mental retardation, sensinural deafness, hypothyroidism,
with the name, the disorder bears no relationship with the chondrodystrophia, and growth hormone deficiency are
hypercellular and neoplastic fibromatosis that can occur associated with gingival fibromatosis.
Periodontal Pathology
Appearance: It is manifested as dense smooth diffuse or

nodular overgrowth of gingival tissue of one or both arches
that usually occurs at the time of eruption of teeth. It has a
characteristic pebbled and finely stippled surface. In some 387
cases, surface has a nodular appearance and may develop
numerous papillary projections. Tissue is of normal or pale
color. In some cases, it may appear pink. It is often so firm,
leathery and dense that it prevents normal eruption of teeth.
Sign and symptoms: It is not painful and shows no tendency
for hemorrhage. Extent may be such that the crown of fully
erupted teeth may be hidden. Typically, extension past the
alveolar mucosal junction into the mucobuccal fold is not
seen, but palatal extension can cause significant distortion
of the contour of the palate and, many of the times almost A
can meet in the midline (Figs 16.1 and 16.2).
The dense firm gingival swelling results in varying
spacing between the teeth and change in profile and facial
appearance. The frenular attachments may appear to divide
the gingival tissues of the alveolar ridge into lobules.

Epithelium is thickened with elongation of rete pegs that
extend deeply into the underlying fibrous connective tissue.
The bulk of the tissue is composed of dense fibrous
connective tissue which is hypocellular and hypovascular.
Bundles of collagen fibers are coarse and show few
interspersed fibroblasts or blood vessels.
B
Figures 16.2A and B Fibromatosis gingivae preventing
normal eruption of teeth
Mucoid changes in fibrous tissue are frequent and

occasional presence of giant cells has been noted.
Inflammation is mostly absent or mild, and dystrophic
calcification is sometimes seen.
The covering epithelium is usually mildly acanthotic and
some hyperkeratosis may be present. Electron microscopic
study shows both fibroblasts and myofibroblast like cells.
Management
Surgical correction (gingivectomy) should be done along
with instructions for oral hygiene and followup, as there is
tendency for recurrence within a few years. In severe cases,
Figure 16.1 Dense nodular overgrowth seen in patient selective extraction of teeth is required to achieve normal
of fibromatosis gingivae morphology of gingiva.
The covering epithelium often is thin and atrophic,

although rete ridges may appear narrow and elongated.
The underlying connective tissue is sometimes highly
388 vascularized and may exhibit large stellate fibroblasts as
well as occasional epithelial rests. The stellate fibroblasts
may contain several nuclei.
Management
It disappears with the age so no treatment is necessary for
this disease.
Points to Remember
Lingual to the mandibular cuspids, soft, wellcircum
scribed, sessile, mucosal nodule, mild hyperorthokerato
Figure 16.3 Histopathological picture of fibromatosis
gingivae sis or hyperparakeratosis, rete ridges may appear narrow
and elongated, stellate fibroblasts.
Points to Remember
GINGIVAL INFLAMMATION
Elephantiasis gingivae, ZimmermannLaband, hypertri
OR GINGIVITIS
chosis, epilepsy, mental retardation, diffuse or nodular
overgrowth of gingival tissue, pebbled and finely stip It may occur in an acute, subacute, or chronic form.
pled surface, teeth may be hidden, elongation of rete
pegs, collagen fibers are coarse, surgical correction (gin Etiology
givectomy). Local factors
• Microorganisms
RETROCUSPID PAPILLA • Calculus
• Food impaction
It is a small elevated nodule located on the lingual mucosa
• Faulty or irritating restorations or appliances
of the mandibular cuspids.
• Mouth-breathing
Clinical Features • Tooth malposition
• Chemical and drug application
Age and sex distribution: It is extremely common in
children usually between the age of 8 to 16 years. The Systemic factors
prevalence in adults drops to 6 to 19 percent, suggesting it • Nutritional disturbances
as an anatomic variation that disappears with age. There is • Pregnancy
no sex predilection. • Diabetes mellitus
• Psychiatric phenomena
Location: It is located lingual to the mandibular cuspids,
• Hormonal changes
between the free gingival margin and the mucogingival
• Allergy and hereditary factor.
junction.
Appearance: It is a soft, wellcircumscribed, sessile, Etiology
mucosal nodule, commonly bilateral. Typically appears as a
small, pink papule that measures less than 5 mm in diameter. Local Factors
Microorganisms: The plaque associated with gingivitis is
Histopathological Features complex and heterogeneous. In early stages of gingivitis,
The structure appears as an elevated mucosal tag, often the Actinomyces group of organisms are the dominant genus
showing mild hyperorthokeratosis or hyperparakeratosis, in the supragingival plaque. In children, flora associated
with or without acanthosis. with gingivitis is different as compared to adults. In
children, it is associated with L. eptotrichia, Selenomonas Hormonal changes: Puberty, menstruation may also result
and some Bacteroides. In patient with persistent gingivitis, in gingival inflammation.
there is appearance of gram negative rods and filamentous
Others: Allergy and hereditary factor can also play 389
microorganism which are followed by spirochetes. There is
important role in gingivitis.
an increase of P. intermedia in pregnancy gingivitis.
Calculus: Either supragingival or subgingivae or both cause Types of Gingivitis (According to Course and
irritation of the contacting gingival tissue. This irritation Duration)
is produced by the byproducts of the microorganisms or • Acute gingivitis: It is a painful condition that comes
by the mechanical friction resulting from the hard, rough on suddenly and is of short duration.
surface of the calculus. • Subacute gingivitis: It is a less severe phase of the
acute condition.
Food impaction: The impaction of food and accumulation
• Recurrent gingivitis: It reappears after having been
of debris on the teeth due to oral negligence results in
eliminated by treatment or disappears spontaneously
gingivitis. The degradation products of food debris may
and then reappears.
also prove irritating to the gingival tissues.
• Chronic gingivitis: It comes slowly, is of long dura
Faulty or irritating restorations or appliances: Faulty tion and is painless unless complicated by acute and
restoration may act as an irritant to gingival tissues subacute exacerbations.
and thereby induce gingivitis. Overhanging margins of
proximal restorations may directly irritate the gingiva and in According to Distribution
addition allow the collection of food debris and organisms
• Localized gingivitis: It is confined to the gingiva in
which add further insult to these tissues. Prosthetic and
relation to a single tooth or a group teeth.
orthodontic appliances impinging on the gingival tissues
• Generalized gingivitis: It involves the entire mouth.
produce gingivitis as a result of pressure due to and of the
• Marginal gingivitis: It involves gingival margins
trapping of food and microorganisms.
but may include a portion of the contiguous attached
Mouth-breathing: Drying of the oral mucous membrane, gingiva.
because of mouthbreathing, due to environment of • Papillary gingivitis: It involves the interdental
excessive heat or from excessive smoking, will result in papillae and often extends into the adjacent portion
gingival irritation with accompanying inflammation. of the gingival margin.
Tooth malposition: Teeth which have erupted or which • Diffuse gingivitis: It affects the gingival margins,
have been moved out of physiologic occlusion, due to attached gingiva and interdental papillae.
repeated subjected to abnormal forces during mastication.
Types of Gingivitis
Chemical and drug application: Many drugs like phenol,
silver nitrates, volatile oils or aspirin, if applied to gingiva • Plaque-related gingivitis
will provoke an inflammatory reaction. • Necrotizing ulcerative gingivitis
• Medication influence gingivitis
Systemic Factors • Allergic gingivitis
• Specific infection-related gingivitis
Nutritional disturbances: Nutritional imbalance is fre
• Dermatosis-related gingivitis.
quently manifested as changes in the gingiva and deeper
underlying periodontium.
Clinical Features
Pregnancy: Gingiva undergoes changes during pregnacy Symptoms: The earliest symptoms of gingival infla
which are termed as ‘pregnancy gingivitis’. mmation are increased gingival fluid production and
Diabetes mellitus: It is reported in association with severe bleeding from the gingival sulcus on gentle probing and
periodontal diseases and gingival inflammation. loss of stippling.
Psychiatric phenomena: It appears to have a definite Signs: Gingiva become light red. With progression, the
influence upon the severity of periodontal disease. area becomes reddened and edematous. Subsequently, the
gingiva becomes brighter red or magenta. The color may Chronic hyperplastic gingivitis: When the chronic
be sometimes reddish blue or deep blue, if venous stasis inflammation causes enlargement of due edema and
has occurred (Figs 16.4 and 16.5). fibrosis, is called chronic hyperplastic gingivitis.
390 The color change starts in the interdental papillae,
gingival margins and spreads to the attached gingiva. The Histopathological Features
consistency of gingiva may be spongy that pits on pressure It will reveal infiltration of the connective tissue by varying
and there may be marked softness of the gingiva. numbers of lymphocytes, monocytes and plasma cells.
Sometimes in inflammation, there may be gingival Polymorphonuclear leukocytes are occasionally noted,
enlargement because of edema or fibrosis and it may lead particularly beneath the crevicular epithelium which is non
to changes in the contour of gingiva. The margins often keratinized and irregular. It is infiltrated by inflammatory
appears blunted, receded, or hyperplastic. cells and is frequently ulcerated.
Puberty gingivitis: At the time of puberty, there is the The capillaries of the connective tissue may be engorged
increase chances of gingivitis. This is called puberty and sometimes may increase in number. Areas of fibrosis,
gingivitis. hyperemia, edema and hemorrhage may be present.
Histopathological Types
Early lesion: It follows when an infiltrate of lymphocytes,
plasma cells, neutrophils, mast cells and macrophages
appear in addition to the polymorphonuclear leukocytes.
There is destruction of collagen. Initially, there is
developmental of rete pegs with increases number of
neutrophils in junctional epithelium.
Established lesion: In this stage, gingival pocket is formed
and the infiltrate contains a predominance of plasma cells
and lymphocytes; polymorphs continued to predominate in
the junctional epithelium. The junctional epithelium shows
elongated rete pegs in connective tissue with destruction
of basement membrane. There is also cellular debris seen
within the widened intercellular spaces of junctional
Figure 16.4 Gingival inflammation presented epithelium.
as redness of gingiva
Advanced lesion: In this stage, there is marking of the
initiation of periodontitis.
Management
The local irritants should be removed at this stage.
Thorough plaque control should be done with scaling and
polishing.
Use of chlorhexidine, on a shortterm basis.
Points to Remember
Loss of stippling, bleeding from the gingival sulcus on
gentle probing, light red gingiva, gingiva may be spongy,
puberty gingivitis, chronic hyperplastic gingivitis, lym
phocytes, monocytes, plasma cells, polymorphonuclear
leukocytes, infiltrated by inflammatory cell, fibrosis, hy
Figure 16.5 Localized gingivitis seen in lower anterior region peremia, edema and hemorrhage (chlorhexidine).
NECROTIZING ULCERATIVE prevalence in the normal population is less than 0.1 percent;
however, in stressed populations, the frequency increases
GINGIVITIS up to 7 percent. In developing countries, ANUG typically
It is an endogenous oral infection that is characterized by occurs in very young children suffering from malnutrition. 391
necrosis of gingiva. It is also called trench mouth due to its
Symptoms: Onset is sudden with pain, tenderness, profuse
prevalence in combat trenches. Other synonyms for this are
salivation and peculiar metallic taste. There is spontaneous
Vincent’s infection, acute ulceromembranous gingivitis,
bleeding from gingival tissue. There is also a loss of sense
fusospirochetal gingivitis and acute ulcerative gingivitis.
of taste and diminished pleasure from smoking. The typical
Tissue destruction is caused by endogenous organisms
fetid odor ultimately develops, which may be extremely
that act either on the tissue or, indirectly by triggering an
unpleasant. Teeth seem slightly to be extruded and are
inflammatory reaction. Recently, several investigators
sensitive to pressure or have a woody sensation. They are
have discontinued the use of the word “acute” because,
slightly movable and the patient is unable to eat properly.
there is no chronic form of the disease.
Gingiva may become superficially stained with brown
Etiology color. There is blunting of interdental papillae.
Role of bacteria: It is caused by fusiform bacilli and Signs: A typical lesion consists of necrotic punched out,
spirochetes. In addition to this, species of Treponema, crater like ulcerations developing most commonly on the
Selenomonas, and Fusobacterium, besides Prevotella interdental papillae and marginal gingiva. Removal of the
intermedia are also responsible for acute necrotizing lesion leaves raw surface. The surface of gingival crater is
ulcerative gingivitis (ANUG). covered by a gray, pseudomembranous slough, demarcated
from the reminder of the gingival mucosa by pronounced
Local predisposing factors: Local factors like poor oral linear erythema (Fig. 16.6).
hygiene, preexisting marginal gingivitis and faulty dental If untreated, then it may result in progressive destruction
restorations, deep periodontal pockets offered favorable of the periodontium, loss of attachment and denudation
environment for occurrence of the disease. It is also seen of the roots (necrotizing ulcerative periodontitis)
in area of gingiva traumatized by opposing in malocclude accompanied by increase in the severity of complications.
teeth (Local trauma). As tobacco smoke has a direct toxic Regional lymph nodes are enlarged. There may be a
effect on the gingiva, smoking and emotional stress can slight elevation of temperature. In some cases, process
predispose for ANUG. may spread to adjacent soft tissue such as cheek, lip,
Nutritional deficiency: Nutritional deficiency like vita tongue, palate and pharyngeal area (necrotizing ulcerative
min C, vitamin B2 accentuate the severity of the pathologic mucositis; necrotizing stomatitis).
changes induced by the fusospirochetal bacterial complex.
Debilitating disease: Chronic diseases like leukemia,
aplastic anemia, syphilis, severe gastrointestinal distur
bances and AIDS can act as predisposing factors.
Psychosomatic factors: The disease often occurs in
association with a stress situation as well as with increase
in adrenocortical secretion.
Other systemic factors like inadequate sleep, marked
malnutrition, immunosuppression and recent illness.
Clinical Features
Age: It is most commonly seen in the age group of 16 to
30 years.
Incidence: It can be seen in children from a low
socioeconomic group, in underdeveloped countries. Several Figure 16.6 Necrotizing ulcerative gingivitis showing necrotic
investigators have reported a higher frequency in whites. The tissue in lower anterior region
Histopathological Features Treatment should include instructions on oral hygiene

and patient motivation; identification and resolution of
It appears as a nonspecific acute necrotizing inflammation
any predisposing factors. Followup appointments are
392 of the gingival margin involving, both, stratified squamous
necessary to reinforce the home care instructions and to
epithelium and the underlying connective tissue.
rule out recurrence.
The surface epithelium is destroyed and is replaced by a
pseudomembranous meshwork of fibrin, necrotic epithelial
cells, polymorphonuclear neutrophils and various types of DESQUAMATIVE GINGIVITIS
microorganisms. It is described as gingival epithelium that spontaneously
The underlying connective tissue markedly appears sloughs or can be removed with minor manipulation. It is a
with intense acute or mixed inflammatory infiltrate and clinical manifestation of several different vesiculoerosive
extensive hyperemia with numerous engorged capillaries diseases.
and a dense infiltration of PMN. Numerous plasma
cells may appear in the periphery of infiltrate. Necrotic Etiology
material and extensive bacterial colonization often are seen Dermatological diseases: Like lichen planus, mucous
microscopically. membrane pemphigoid, bullous pemphigoid or pemphigus.
Management Infections: Tuberculosis, chronic candidiasis, histoplas
The involved areas are isolated with cotton rolls and dried. mosis.
A topical anesthetic is applied and after 2 to 3 minutes, the Other diseases like endocrine imbalance, chronic infec
areas are gently swabbed with a cotton pellet to remove tion and drug reaction, abnormal response to irritation and
the pseudomembrane and nonattached surface. After the idiopathic causes. It can be caused by chemical burns.
area is cleansed with warm water, the superficial calculus Clinical Features
is removed. The patient is told to rinse the mouth every 2
hours, with a glassful of an equal mixture of warm water Age and sex distribution: Both sexes are affected but it is
and 3 percent hydrogen peroxide. Twice daily rinse with more prevalent in women and occurs after age of 40.
0.12 percent chlorhexidine is also effective. Appearance: Gingiva becomes bright red, edematous and
Patients with severe ANUG and lymphadenopathy desquamation of the surface epithelium of the attached
are treated with antibiotics Penicillin V250 or 500 mg, gingiva is also seen. The facial surface is involved more
6 hourly or erythromycin 250 or 500 mg, 6 hourly with frequently than the lingual gingiva (Fig. 16.7).
metronidazole 400 mg, 8 hourly, for 7 days are the drugs
of choice. Scaling is performed, if sensitivity permits.
After the disease process is diminished, complete gingival
curettage and root planning is done. Supportive treatment
consists of copious fluid consumption and administration
of nutritional supplements.
Points to Remember
Trench mouth, Vincent’s infection, low socioeconomic
group, profuse salivation, metallic taste, fetid odor,
woody sensation, crater like ulcerations, gray, pseu
domembranous slough, necrotizing ulcerative peri
odontitis, necrotizing ulcerative mucositis, necrotizing
stomatitis, nonspecific acute necrotizing inflammation,
pseudomembranous meshwork of fibrin, necrotic epi
thelial cells, polymorphonuclear neutrophils, extensive
hyperemia, topical anesthetic, Penicillin V, metronida Figure 16.7 Denudation of gingiva seen
zol, erythromycin. in desquamative gingivitis
It is usually manifested in three forms, i.e. mild, Topical corticosteroid ointment and creams such as
moderate and severe. triamcinolone 0.1 percent fluocinolone 0.05 percent is
applied and gently rubbed into the gingiva several times
Mild: It may be diffuse and extends throughout the gingiva. 393
daily.
It is painless.
Patients whose conditions are resistant to corticoster
Moderate: There is a patchy distribution of bright red and oids often respond to dapsone or sulfapyridine. In cases that
gray areas involving the marginal and attached gingiva. demonstrate negative immunofluorescent findings, therapy
The surface is smooth, shiny and normally resilient. Patient with estrogens have been attempted with equal results.
may complain of a burning sensation and sensitivity to
thermal changes. There is slight pitting on pressure and Points to Remember
epithelium is not firmly adherent to the underlying tissues. Red, edematous and desquamation of the surface epithe
Severe form: It is characterized by scattered irregularly lium of the attached gingiva, mild forms, moderate form,
shaped areas, in which gingiva is denuded and strikingly severe form, burning sensation, sensitivity to thermal
red in appearance. The overall appearance of gingiva changes, speckled gingiva, spontaneous desquamation,
is speckled. There is blister formation, spontaneous formation of bubbles, blast of air, epithelium may be thin
desquamation, or zones of erosion. If blisters are present, atrophic, tetracycline therapy, doxycycline monohydrate,
then they are filled with clear fluid or can be contaminated topical corticosteroid ointment, dapsone or sulfapyridine.
with blood. Epithelium is friable and can be easily
removed from the underlying connective tissue leaving PLASMA CELL GINGIVITIS
a red surface that bleeds readily to minimum trauma. A
blast of air directed at the gingiva causes elevation of This is also called atypical gingivostomatitis.
the epithelium and consequent formation of bubbles.
Yellowish fibrinopurulent membranes cover areas of frank
Cause
erosion, and significant pain is usually present. It usually caused by allergic reaction to chewing gum,
herbal toothpaste, mint candy and peppers used for cooking.
Histopathological features may mimic either bullous
Clinical Features
lesions of mucous membrane pemphigoid or lichenoid Onset: There is rapid onset of sore mouth, which is
reaction. aggravated by dentifrice or spicy food.
Sometimes, epithelium may be thin and atrophic Appearance: Gingiva appears diffusely enlarge with
with little or no keratin at the surface and dense diffuse bright erythema and loss of stippling (Fig. 16.8).
inflammatory cells in the underlying connective tissue.
Rete pegs are blunted and there may be clefting below Spread: In some cases, spread of this disease is reported
the basement membrane with edema and a mild chronic form lip and tongue.
inflammatory infiltrate.
Management There is psoriasiform hyperplasia and spongiosis of
A complete history should be taken to uncover a possible surface epithelium present. There is also intense exocytosis
coexistent extraoral cause. Before definitive treatment, all and neutrophilic microabscess.
possible local irritants should be removed. Lamina propria contains numerous dilated vascular
The patient must be carefully instructed in plaque channels with chronic inflammatory infiltrates which is
control and instructed for using a soft toothbrush, oxidizing composed of plasma cells.
mouthwash (hydrogen peroxide 3% diluted to one part
peroxide and two parts of warm water) should be use twice
Management
daily. Elimination of possible allergen should be carried out.
Improvement has been noticed with tetracycline Topical and systemic immunosuppressive agents like
therapy. Doxycycline monohydrate 100 g daily for 4 to 11 betamethasone rinse, fluocinonide gel, topical triamci-
weeks. nolone and topical fusidic acid should be given.
Foreign body gingivitis: It can cause area of erythematous

and atrophic mucositis which can resembles gingival lichen
planus.
394
There is the focal collection of histiocytes mixed with intense
lymphocytes infiltrates. In some cases, multinucleated
giant cell can be seen.
Foreign bodies appear as black or brownblack granules.
Management
Surgical excision of affected tissue is the therapy of choice.
Points to Remember
Figure 16.8 Plasma cell gingivitis with bright erythema Red or redwhite macules, pain and sensitivity,
foreign body gingivitis, histiocytes mixed with intense
lymphocytes infiltrates.
Points to Remember
Atypical gingivostomatitis, allergic reaction, gingiva ap GINGIVAL ABSCESS
pear diffusely enlarge, bright erythema, psoriasiform hy It is localized, painful, rapidly expanding lesion that is
perplasia, spongiosis of surface epithelium, neutrophilic usually of sudden onset.
microabscess, plasma cells, betamethasone rinse, fluo
cinonide gel, topical triamcinolone, topical fusidic acid. Etiology
It results from bacteria carried deep into the tissue, when
GRANULOMATOUS GINGIVITIS a foreign substance such as a toothbrush bristle, a piece
of applecore or a lobster shell fragment is forcefully
In some cases, patient have localized area of granulomatous
embedded into the gingiva.
inflammation of gingiva without signs and symptoms of
granulomatous disease which is termed granulomatous Clinical Features
gingivitis.
Location: It is limited to the marginal gingiva or the
Cause interdental papilla.
It is thought to be cause by introduction of dental material Signs: In early stages, it appears as a red swelling with
into the connective tissue deep to sulcular epithelium. The a smooth and shiny surface (Fig. 16.9). Within 24 to 48
most common material which can cause are silver, copper, hours, the lesion usually becomes fluctuant and pointed
calcium, phosphorus and iron. This is also called foreign with a surface orifice, from which an exudate may be
body gingivitis. expressed. The adjacent teeth are sensitive to percussion.
Clinical Features Histopathological Features

Age: It can occur at any age but more commonly seen in It consists of a purulent focus in the connective tissue,
adults. surrounded by a diffuse infiltration of polymorphonuclear
leukocytes, edematous tissue and vascular engorgement.
Appearance: It appears as red or redwhite macules involving
The surface epithelium has a varying degree of intra
interdental papillae but can occurs on marginal gingiva.
and extracellular edema, with invasion of leukocytes and
Symptoms: Pain and sensitivity are common findings. ulceration.
this fluctuance is digitally ascertained, incision and

drainage should be carried out, followed by warm saline
rinses at frequent intervals.
395
Symptoms: Pain, malaise, leukocytosis and swelling of
peritonsillar region. The patient complaints of foul taste
and inability to close the jaws. The pain may radiate to the
throat, ear, or floor of the mouth.
Signs: It may result in cellulitis and muscular trismus.
There is also regional lymphadenopathy, submaxillary and
pharyngeal abscess. Operculum may get traumatized by
opposing teeth during mastication. Edema, visible in both
submandibular area and peritonsillar region occurs. There
is an extreme tenderness on palpation of the abscess.
Figure 16.9 Gingival abscess presented as red swelling Histopathological Features

Gingivitis is present and the pocket lining is hyperplastic,
Management with extensive exocytosis of acute inflammatory cells.
Connective tissue stroma is hyperemic which shows
After topical anesthesia is applied, the fluctuant area
acute inflammatory cells consisting predominantly of
of the lesion is incised with a blade and the incision is
lymphocytes and plasma cells, but with a variable number
gently widened to permit drainage. The area is cleansed
of polymorphonuclear leukocytes. Mostly, large microbial
with warm water and covered with a gauze pad. After the
colonies representing plaque and calculus are found.
bleeding stops, patient is instructed to rinse every 2 hours
with a glassful of water. Complications
Points to Remember The involvement may become localized in the form of
a pericoronal abscess. It may spread posteriorly into the
Marginal gingiva or the interdental papilla a red swelling
oropharyngeal area and medially, to the base of the tongue.
with a smooth and shiny surface, fluctuant surface
Peritonsillar abscess, cellulitis and Ludwig’s angina are
orifice, adjacent teeth are sensitive polymorphonuclear
common.
leukocytes, vascular engorgement, intra and extra
cellular edema. Management
Antibiotics: Immediately, antibiotics should be started.
PERICORONAL ABSCESS Most commonly used antibiotics are phenoxylmethyl
penicillin 250 mg four times daily. In pericoronitis due to
It is also called pericoronitis. It is the infection of soft
ulceromembranous gingivitis metronidazole 200 g three
tissues surrounding the crown of an impacted or partially
times daily for 7 days is given.
erupted tooth when food debris and bacteria are present
beneath the gingival flap overlying the crown. Drainage: Careful probing should be done around the 3rd
molar, which permits entry into the expanded follicle and
Clinical Features allows evacuation of pus and other septic material. The
The most common type of pericoronal infection is found patient is instructed to use warm salt water rinses and to
around the mandibular 3rd molar. return in 24 hours.
Pericoronal infection of infancy: Pericoronal infection Extraction: When the symptoms become subacute, then
of infancy is often associated with the supradental tissues, the impacted 3rd molar should be extracted.
involving the superior portion of the follicle and the Operculectomy: Sometimes when the retention of 3rd
overlying mucoperiosteum, which may become inflamed. molar is necessary, the inflamed tissue surrounding the
It ultimately develops into small fluctuant abscess. When occlusal portion of the tooth should be excised.
Extraction of maxillary 3rd molar: Maxillary 3rd molar

can be a contributing factor to the pericoronal infection of
the mandibular 3rd molar. In such cases, especially when
396 the mandibular 3rd molar is fully erupted in proper place,
the maxillary 3rd molar should be extracted prior to the
retention of the mandibular 3rd molar, considering the
recurrent nature of the inflammatory episode.
Points to Remember
Pericoronal infection of infancy, fluctuance is digitally
ascertained, pain, malaise, leukocytosis, muscular
trismus, cellulitis, hyperplastic gingiva, exocytosis of
acute inflammatory cells, acute inflammatory cells,
antibiotics, drainage, extraction, operculectomy.
Figure 16.10 Inflammatory enlargement of gingiva showing
CHRONIC INFLAMMATORY ballooning of the interdental papilla (Courtesy: Dr Aparna
Thombre, Reader, Department of Oral Pathology, VSPM
ENLARGEMENT Dental College and Hospital, Nagpur, Maharashtra, India)
Etiology
It can be caused by prolonged exposure to dental plaque,
which may occur due to poor oral hygiene, abnormal
relationship of adjacent and opposing teeth, lack of tooth
function, overhanging margins of dental restoration and
improperly contoured dental restoration or pontics.
It can also caused by food impaction, irritation from
clasps or saddle areas of removable prosthesis and nasal
obstruction.
Habits: Such as mouthbreathing can cause gingival en
largement, which is more common in the anterior region.
Clinical Features
The enlargement is generally papillary or marginal and
localized or generalized. It originates as a slight ballooning
of the interdental papilla or the marginal gingiva. Figure 16.11 Inflammatory hyperplasia on gingiva
Signs: In early stages, it produces a life preserver-like

bulge around the involved teeth. It increases in size, until ulceration, sometimes, occurs between the mass and the
it covers a part of the crown. Lesions may be deep red or adjacent gingiva.
bluish red (Figs 16.10 and 16.11).
They are soft and friable, with a smooth and shiny Histopathological Features
surface and tendency to bleed. It progresses slowly and It shows exudative and proliferative features of chronic
painlessly, unless complicated by acute infection or trauma. inflammation. Lesions may contain preponderance of
Sometimes, enlargement can occur as a discrete, inflammatory cells and fluid with vascular engorgement,
sessile or pedunculated mass, resembling a tumor. They new capillary formation, associated with degenerative
may undergo spontaneous reduction in size, followed changes. Areas of fibrosis, hyperemia, edema, and
by exacerbation and continued enlargement. Painful hemorrhage may be present (Fig. 16.12).
397
Figure 16.12 Inflammatory cells seen in gingival enlargement Figure 16.13 Drug-induced gingival enlargement showing
lobulated appearance
Points to Remember
Prolonged exposure to dental plaque, papillary enlarge
ment, preserverlike bulge around teeth, smooth and
shiny surface, tendency to bleed, sessile or pedunculated
mass shows exudative and proliferative features of
chronic inflammation, inflammatory cells, vascular
engorgement, new capillary formation.
GINGIVAL ENLARGEMENT
DUE TO DRUGS
It refers to an abnormal growth of the gingival tissues
secondary to use of systemic medication. The increased
gingival size is due to the production of an increased Figure 16.14 Drug-induced enlargement showing
amount of extracellular matrix, mainly collagen. bead-like enlargement
It can be caused by Dilantin sodium, cyclosporine or
nifedipine. As the enlargement increases, the gingival tissue
becomes lobulated and clefts are seen between each
Clinical Features enlarged gingiva. Palpation reveals that the tissue is dense,
Onset: Gingival hyperplasia may begin as early as two resilient and insensitive. It shows little tendency to bleed
weeks after Dilantin therapy. (Fig. 16.15).
They may develop massively, covering a considerable
Location: The hyperplasia is generalized throughout the portion of the crown. They may interfere with occlusion.
mouth, but it is most severe in maxillary and mandibular The presence of an enlargement makes plaque control
anterior region. difficult, resulting in a secondary inflammatory process
Signs: The first change noted is a painless bead-like that complicates the gingival hyperplasia.
enlargement in the size of the gingiva, starting with one
or two interdental papillae. The surface of gingiva shows Histopathological Features
an increase in stippling and finally, a cauliflower, warty or The stratified squamous epithelium covering the tissue is
pebbled surface (Figs 16.13 and 16.14). thick and has a thin keratinized layer. The rete pegs are
398
Figure 16.15 Drug-induced gingival enlargement (Courtesy: Figure 16.16 H&E stained section shows proliferative stratified
Aparna Thombre, Reader, VSPM Dental College and Hospital, squamous epithelium with elongated rete pegs with underlying
Nagpur, Maharashtra, India) connective tissue shows densely arranged collagen fiber bundle
with few fibroblasts, inflammatory cells and blood vessels
extremely long and thin, sometimes called ‘test tube’ pegs,
with considerable confluence. Mitotic figures are seldom
seen. PREGNANCY TUMOR
The bulk of tissue is made up of large bundles of fibers, It is also known as granuloma gravidarum. It is an
interspersed with fibroblasts and fibrocytes. There may be inflammatory reaction to the local irritants. It is pyogenic
a pronounced hyperplasia of the connective tissue and the granuloma occurring in pregnancy.
epithelium.
There is acanthosis of epithelium and the elongated rete Clinical Features
pegs extend deep into the connective tissue (Fig. 16.16). In Age: Such lesions may begin to develop during the first
patients with secondary inflammation, there is increased trimester, and their incidence increases up through the 7th
vascularity and a chronic inflammatory cellular infiltrate month of pregnancy. It usually appears after 3rd month of
that mostly consists of lymphocytes and plasma cells. pregnancy.
The gradual rise in development of these lesions
Management throughout pregnancy may be related to the increasing
Discontinuation of medication often results in cessation of levels of estrogen and progesterone.
lesion. Substitution of drugs which are causing it should
Appearance: The lesions appear as discrete, mushroom
be done.
like, flattened spherical masses, that protrude from the
Systemic or topical folic acid has been effective in
gingival margins or more frequently from the interproximal
some cases of hyperplasia.
space and are attached by sessile or pedunculated base
In some cases, metronidiazole or azithromycin is also
(Figs 16.17 and 16.18).
useful in cyclosporine induce enlargement.
It tends to expand laterally. The pressure from the
Points to Remember tongue and the cheek perpetuates its flattened appearance.
Dilantin sodium, cyclosporine or nifedipine, hyperplasia Sign and symptoms: It is generally dusky red or magenta;
is generalized, painless bead like enlargement, test tube it has a smooth glistening surface that frequently exhibits
rete pegs, fibroblasts and fibrocytes, hyperplasia of the numerous deep red, pinpoint markings. The consistency
connective tissue and the epithelium, elongated rete varies from semifirm, but may have a varying degree of
pegs, discontinuation of medication, topical folic acid, softness and friability. It is usually painless, unless its
metronidiazole or azithromycin. size and shape foster the accumulation of debris under its
399
Figure 16.17 Pregnancy tumor showing mushroom Figure 16.19 Pregnancy tumor showing edema
like enlargement and engorged capillaries
The surface is usually ulcerated and replaced by a thick

fibrinopurulent membrane. Between the capillaries, there
is moderately fibrous stroma, with a varying degree of
edema and leukocytic infiltration.
Management
Most gingival enlargement during pregnancy can be
prevented by the removal of local irritants and institution
of a fastidious oral hygiene. Usually, treatment should be
avoided unless significant functional or esthetic problems
develop. The lesion mostly resolves spontaneously after
parturition.
Points to Remember
Figure 16.18 Pregnancy tumor presented as pinpoint marking Granuloma gravidarum, first trimester, discrete,
mushroom like, flattened spherical masses, expand
laterally, flattened appearance, numerous deep
margin or interfere with occlusion, in which cases painful red, pinpoint markings, semifirm, thick stratified
ulceration may occur. squamous epithelium, lined by cuboids endothelial
cell, prominent intercellular bridges and leukocytic
Histopathological Features infiltration, edema and leukocytic infiltration, removal
It consists of a central mass of connective tissue, the of local irritants.
periphery of which is outlined by thick stratified squamous
epithelium with prominent rete pegs.
GRANULOMA PYOGENICUM
The connective tissue consists of numerous, diffusely
arranged, newly formed and engorged capillaries, lined by It is also called pyogenic granuloma. It is a non-specific,
cuboids endothelial cells (Fig. 16.19). The epithelium exhibits tumor-like, conditional enlargement of the gingiva that is
some degree of intracellular or extracellular edema with considered as an exaggerated conditional response to minor
prominent intercellular bridges and leukocytic infiltration. trauma. Inspite of its name, it is not a true granuloma.
Clinical Features
Location: The lesions are slightly more common on the
400 maxillary gingiva than the mandibular gingiva; anterior
areas are more frequently affected than the posterior areas.
The lesions are more common on the facial aspect than the
lingual aspect. Some extends between the teeth and involve
both the facial and the lingual gingiva. In some cases, this
can also occur on lip, tongue and buccal mucosa.
Appearance: It varies from a discrete spherical, tumor
like mass with a pedunculated attachment to a flattened,
keloid like enlargement with a broadbase. Some lesions
although are sessile.
Size: They vary from small growths to larger lesions that
may measure several centimeters in diameter. Figure 16.21 Pyogenic granuloma showing capillary
proliferation (c) and inflammatory infiltrates
Sign and symptoms: The mass is painless and the surface
is characteristically ulcerated and the color appears bright
red or purple color and either friable or firm, depending
on its duration. In majority of the cases, it presents with Microscopic examination shows a highly vascular
surface ulceration and purulent exudation. Young pyogenic proliferation that resembles granulation tissue.
granuloma are highly vascular in appearance whereas older Endothelial proliferation and formation of numerous
lesions tend to become more collagenized and pink (Fig. vascular spaces are the prominent features. The
16.20). blood vessels are engorged. The surface epithelium is
usually ulcerated and replaced by thick fibrinopurulent
Histopathological Features membrane.
It appears as a mass of granulation tissue with chronic A mixed infiltration of neutrophils, plasma cells, and
inflammatory cell infiltration (Fig. 16.21). lymphocytes is evident. Neutrophils are mostly evident
near the ulcerated surface; chronic inflammatory cells are
found deeper in the specimen. The surface is atrophic in
some areas and hyperplastic in others. Older lesions may
have areas with a more fibrous appearance.
Management
Removal of lesion, along with elimination of irritating
factors. Occasionally, recurrence is seen and reexcision is
necessary.
Points to Remember
Pyogenic granuloma, nonspecific, tumorlike, condi
tional enlargement of the gingiva, keloid like enlarge
ment, painless surface, ulcerated bright red color, fri
able or firm, chronic inflammatory cell infiltration,
endothelial proliferation, infiltration of neutrophils,
Figure 16.20 Granuloma pyogenicum presented
as discrete spherical tumor like mass
plasma cells.
PERIODONTAL POCKETS
Classification 401
• Gingival pocket (relative or false): It is formed by
gingival enlargement, without destruction of the un
derlying periodontal tissues. The sulcus is deepened
because of the increased bulk of the gingiva.
• Periodontal pocket (absolute or true): There is
destruction of the supporting periodontal tissue;
progressive pocket deepening leads to destruction of
the supporting periodontal tissues and loosening and
exfoliation of the teeth.
• Suprabony pocket (supracrestal or supra-alveolar):
In it, bottom of the pocket is coronal to the underlying
alveolar bone. Figure 16.22 Periodontal pocket is examined
• Infrabony (intrabony, subcrestal or intra-alveolar): with the help of periodontal probe
In it, bottom of the pocket is apical to the level of the
adjacent alveolar bone.
Signs: In some cases, pus may be expressed by applying
Pathogenesis digital pressure. When explored with a probe, the inner
aspect of the periodontal pocket is generally painful. There
Periodontal pockets are caused by microorganisms and
may be bluish red, thickened marginal gingiva and a bluish
their products, which produce pathologic tissue changes
red vertical zone from the gingival margin to the alveolar
that lead to the deepening of the gingival sulcus.
mucosa (Fig. 16.22).
Pocket formation starts as an inflammatory change in
the connective tissue wall of the gingival sulcus. The cellu Histopathological Features
lar and fluid inflammatory exudates cause degeneration of
the surrounding connective tissues, including the gingival There may be circulatory stagnation, destruction of the
fibers. Just apical to the junctional epithelium, an area of gingival fibers and surrounding tissues, which results in
destroyed collagen fibers develops and becomes occupied discoloration.
by inflammatory cells and edema. There is an atrophy of the epithelium and edema, which
Collagen loss may occur due to enzymes, like colla results in a shiny surface of lesion. There is an increased
genase and other lysosomal enzymes from polymorphonu vascularity, thinning and degeneration of the epithelium
clear leukocytes and macrophages, which become extracel with close proximity to the engorged vessels.
lular and destroy the collagen. As a consequence of loss of
collagen, the apical portion of the junctional epithelium pro- Management
liferates along the root, extending in finger like projections. Pocket irrigation: Devices like squeeze bottles and blunt
As the apical portion migrates the coronal portion of hypodermic needles can be use to irrigate the pocket
the junctional epithelium detaches from the root. with chemotherapeutic agents. Flap surgery to eliminate
As a result of inflammation, polymorphonuclear neu-
pockets.
trophils invade the coronal end of junctional epithelium.
An increase in number of these cells, results in loss of tis- Points to Remember
sue cohesiveness and tissue detachment from the tooth sur-
face. Thus, the bottom of the sulcus shifts apically, result Deepening of the gingival sulcus, gingival bleeding,
ing in deepening of the periodontal pocket. suppuration, explored with a probe painful inner aspect
periodontal pocket, circulatory stagnation, destruction
Clinical Features of the gingival fibers, an atrophy of the epithelium, an
Symptoms: Gingival bleeding or/and suppuration, tooth increased vascularity, thinning and degeneration, pocket
mobility and diastema formation may be present. irrigation.
ADULT PERIODONTITIS
It is also called slowly progressive periodontitis, chronic
402 adult periodontitis and chronic inflammatory periodontitis.
Etiology
Local factors: It occurs in association with plaque, calculus
and poor oral hygiene.
Systemic disease: Development of systemic disease like
diabetes mellitus, hormonal alteration or an immunologic
defect can accelerate periodontal destruction.
Types of Adults Periodontitis

• Mild periodontitis: It is characterized by an attach
ment loss of 1 to 2 mm, minimum furcation invasion Figure 16.23 Gingival recession of lower anterior region
and little tooth mobility. in periodontal disease
• Moderate periodontitis: It exhibits 3 to 4 mm of
probing attachment loss, early to moderate furcation Histopathological Features
invasion and slight to moderate tooth mobility. The enlarged free marginal gingiva is densely infiltrated
• Severe periodontitis: Probing attachment loss of 5 with lymphocytes and plasma cells. The apical borders of
mm or more, with significant furcation invasion often the inflamed area approach the crest of the alveolar bone
through and through, with excessive tooth mobility. and the crestal fibers of the periodontal ligament.
The crevicular epithelium shows various areas
Clinical Features of proliferation and often, tiny ulcerations. There is
It generally affects both the sexes equally and is seen in appearance of giant cells and osteoclasts on the surface
older age groups more frequently. It is usually generalized, of the bony crest. Principal periodontal ligament fibers
although some areas may be involved more deeply than become disorganized and are detached from the tooth.
others.
Management
Symptoms: It is usually painless, but sometimes exposed Scaling, curettage and periodontal surgery can be done
root may be sensitive to heat and cold, in absence of caries. depending upon the depth, type of pocket and the type of
Areas of localized deep pain, sometimes radiating deep the bone loss associated.
into the jaws are established.
Signs: The characteristic finding in it is gingival Points to Remember
inflammation, which results from accumulation of plaque Slowly progressive periodontitis, painless or localized
and loss of periodontal attachment. The gingiva is slightly deep pain, gingival inflammation, gingiva swollen with
or moderately swollen and exhibits alteration in color from color from pale to magenta, tooth mobility, gingival
pale to magenta. There is also loss of stippling and gingival recession, infiltrated with lymphocytes, giant cells and
bleeding, which may be either spontaneous or easily osteoclasts on the surface.
provoked. There is presence of pocket with variable pocket
depth. Both, horizontal and angular bone loss can be found.
RAPIDLY PROGRESSIVE
Tooth mobility is found in advanced cases, where
bone loss has been considerable. Teeth give off a rather PERIODONTITIS
dull sound when tapped with a metal instrument. The Rapidly progressive periodontitis or generalized aggressive
embrasures may be open because the interdental papillae periodontitis may not represent a distinct disease entity but,
are deficient. Gingival recession is a common phenomenon rather, may be a collection of young adults with advanced
in later stages of disease, which may expose the cementum periodontal disease. There is poor serum antibody response
(Fig. 16.23). to infecting agents.
Etiology
Microorganisms responsible are Actinobacillus actinomy-
cetemcomitans, P. intermedia, P. gingivalis, B. forsythus, 403
F. nucleatum, Camphylobacter rectus, Spirochetes, etc.
Altered chemotactic response to neutrophils has been re
ported in some cases.
Clinical Features
Rate of destruction is rapid over a period of time, as
compared to slowly progressive periodontitis. The lesions
are more generalized and all or most of the teeth are
affected, without any definite pattern of distribution.
Age: The age of onset of the disease ranges from the middle
to late teens and till 30 years of age. Figure 16.24B Rapidly progressing periodontitis presented as
There is marked episodic destruction of periodontal destruction of periodontal ligament
attachment and alveolar bone.
Signs: Gingiva is acutely inflamed, often proliferated, radiographic, histopathological, and microbiologic findings
ulcerated and fiery red. Bleeding may occur spontaneously, together with family history and leukocyte function tests.
or on slight provocation. In some cases, gingiva appears
pink and free of inflammation; but in spite of this, deep Management
pockets can be revealed on probing (Figs 16.24A and B). If microflora contains grampositive microorganisms, then
In contrast to the localized variant, heavy plaque, it should be treated with 250 mg amoxicillin and 125 mg
calculus, and marked gingival inflammation may be potassium clavulanate three times daily, for 14 days, along
present. with scaling and root planning. If flora is gram negative,
Some patients may have systemic manifestations like then clindamycin should be given with dose of 150 mg,
weight loss, mental depression and general malaise. four times a day, for 7 days, along with scaling and root
planning.
Histopathological Features If surgery is required, it is performed 2 days after the
It is as not different from chronic periodontitis. Definitive initiation of the antimicrobial therapy. Chlorhexidine rinses
diagnosis should be made on the basis of clinical, are used for 2 weeks postsurgery. Revaluation is done and
oral prophylaxis is carried out once a month for 6 months
and then every 3 months thereafter.
Points to Remember
Actinobacillus actinomycetemcomitans, marked epi
sodic destruction of periodontal attachment, gingiva is
acutely inflamed, spontaneous bleeding, systemic mani-
festations like weight loss, amoxicillin, potassium clavu-
lanate, clindamycin.
AGGRESSIVE PERIODONTITIS/
JUVENILE PERIODONTITIS
It is also called periodontosis. It is an autosomal recessive
and Xlinked character. It is an aggressive, but uncommon
Figure 16.24A Periodontitis showing severe bone destruction form of periodontitis found in children and young adults.
It is a disease of the periodontium, occurring in an Location: It can be localized or generalized and maxillary
otherwise healthy adolescent and is characterized by a teeth are more frequently affected than mandibular, with
rapid loss of alveolar bone about one or more teeth of the strong leftright symmetry. Teeth more commonly affected
404 permanent dentition. are 1st molars and incisors. There is an increase in the
number of affected teeth with advancing age, which leads
Causes to widely accepted assumption that the disease starts with
Inherited defect in neutrophilic chemotactic function, a localized lesion and at later stages becomes generalized.
which affects the ability of polymorphonuclear leukocytes Signs: The most striking feature is the lack of clinical
to phagocytose and degranulate, thus impairing the host inflammation, despite the presence of deep periodontal
resistance is responsible for juvenile periodontitis. pocket. Presence of deep pockets with secondary
Several authors describe it as a hereditary and familial inflammation may occur. Deep, dull radiating pain may
pattern. Microorganisms responsible are usually gram occur with mastication, due to irritation of the supporting
negative anaerobic rods, along with minimum amount of structures by mobile teeth and impacted food.
attached plaque.
Symptoms: Initial complain is mobility and pathological
Mechanism of Bone Loss drifting of first molars and incisors. Classically, the clinician
Actinobacillus actinomycetemcomitans can produce sees a distolabial migration of the maxillary incisors, with
substances that can kill PMNs and monocytes, thereby a diastema formation. Subgingival calculus is uncommon.
compromising the patient’s ability to fight the invading As the disease process continues, denuded root surfaces
bacteria or their products. These leukotoxins may be become sensitive to thermal and tactile stimuli.
counteracted by the development of serum antibodies. Periodontal abscess may form at this stage and regional
Inhibition of PMNs may be induced by some gram lymph node enlargement may occur. The rate of bone loss
negative bacteria. Endotoxins from Actinobacillus is three to four times faster than in typical periodontitis.
actinomycetemcomitans can induce Schwartzman reaction, Rapid and typically angular loss of alveolar bone occurs,
macrophage toxicity, platelet aggregation, complement which may progress to tooth loss. In classic cases an arc
activation and bone resorption. shaped zone of bone loss extends from the distal aspect
Actinobacillus actinomycetemcomitans, Capnocyto- of the second bicuspid to the mesial aspect of the second
phaga and Bacteroides can produce proteolytic enzymes molar. One of the striking features is a robust serum
that can destroy collagen, activate complement system or antibody response to infecting agents.
degrade immunoglobulins. Actinobacillus actinomycet- Radiological features: There is vertical bone resorption
emcomitans and Capnocytophaga can produce fibroblast which is bilateral and symmetrical.
inhibiting factors, which impair the defense mechanism.
Polyclonal B-lymphocyte activation by periodontal bacte Histopathological Features
ria may result in production of antibodies which are unre A thin, frequently ulcerated pocket epithelium, infiltrated
lated to the activating agent. by numerous leukocytes covers large area of inflammatory
cell accumulation, composed mainly of plasma cells and
Types of Aggressive
blast cells with lymphocytes and macrophages present in
• Localized aggressive periodontitis or juvenile small numbers. There is a proliferation of the epithelial
periodontitis attachment along the root surface. There is also slight
• Generalized aggressive periodontitis or juvenile cellular infiltration in the connective tissue.
periodontitis.
Management
Clinical Features Standard periodontal therapy: It includes scaling and
Age and sex distribution: Juvenile periodontitis affects root planning, curettage, flap surgery with and without bone
both, males and females and is seen most frequently in the grafts, root amputation, hemisection, occlusal adjustment
periods between puberty and 20 years of age. and strict plaque control.
Antibiotic therapy: Tetracycline 250 mg, four times a day

7 days is given or doxycycline 200 mg stat followed by 100
mg once a day for 14 days.
405
Points to Remember
Periodontosis, neutrophilic chemotactic function, Actino-
bacillus actinomycetemcomitans, lack of clinical infla
mmation, deep periodontal pocket, diastema formation,
periodontal abscess, bilateral vertical bone resorption,
numerous leukocytes, inflammatory cell accumulation,
lymphocytes and macrophages, epithelial attachment
along the root surface, antibiotic therapy.
PAPILLON-LEFEVRE SYNDROME A
It is an autosomal recessive and inherited disorder. It is a

triad of hyperkeratosis of palms of the hand and soles of
feet; extensive prepubertal destruction of the periodontal
bone supporting the dentition, usually extensive genera
lized horizontal bone loss and calcification of dura is seen.
Clinical Features (Figs 16.25A and B)

There is reddened, scaly, rough palms and soles, inflamed
gingivae and dramatic bone destruction seen in both
deciduous as well as permanent dentitions.
Onset: It usually begins after eruption of the primary
second molars. There is loss of entire primary dentition by
the age of 5 years and loss of secondary dentition by the B
age of 20 years. Figures 16.25A and B Papillion-Lefevre syndrome
Sign and symptoms: Mobility and migration of the teeth

are observed consistently, and mastication often is painful
because of the lack of support. When the teeth are absent, The crevicular epithelium is hyperplastic with
the alveolar mucosa is normal in appearance. Gingival exocytosis. An extremely thin cementum is present.
swelling and severe halitosis can also occur. The underlying connective tissue exhibits increased
Although other pathogenic bacteria have been isolated vascularity and mixed inflammatory cellular infiltrates,
from sites of active disease, A. actinomycetemcomitans mixed infiltrates consist of polymorphonuclear leukocytes,
has been directly related to the periodontal destruction. lymphocytes, Histiocytes and plasma cells.
There are also indications that leukocyte dysfunction may
be induced by infection with A. actinomycetemcomitans Management
(secondary to generated leukotoxins). Management should be carried out with the help of
antibiotics, mechanical plaque control and management of
Histopathological Features skin lesion.
It includes marked chronic inflammation of the lateral walls With the use of mechanical plaque control and appro
of the pocket, with predominantly plasma cell infiltrate, priate antibiotics (amoxicillin and potassium clavulanate
considerable osteoclastic activity and apparent lack of or ofloxacin) directed towards A. actinomycetemcomitans,
osteoblastic activity. the course of the disease may be altered.
The progression of the attachment loss is drastically 10. Bhaskar SN, Jacoway JR. Pyogenic granuloma-clinical
slowed down and the teeth that erupt after the initiation features, incidence, histology, and result of treatment:
of therapy do not develop periodontal destruction. Long report of 242 cases. J Oral Surg. 1966;24:3918.
406 term maintenance consists of meticulous oral hygiene, 11. Buchner A, Merrell PW, Hansen LS, et al. The retrocuspid
chlorhexidine mouth rinses, frequent oral prophylaxis by papilla of the mandibular lingual gingiva, J Periodontol.
1990;61:58690.
professional, and periodic appropriate antibiotic therapy is
12. Butler RT, Kalkwarf KL, Kaldahl WB. Druginduced
necessary.
gingival hyperplasia: phenytoin, cyclosporine, and
nifedipine. J Am Dent Assoc. 1987;114:56-60.
Points to Remember 13. Ciancio SG. Agents for the management of plaque and
Hyperkeratosis of palms of the hand and soles of feet, gingivitis, J Dent Res. 1992;71:14504.
destruction of the periodontal bone, calcification of 14. Daley TD, Nartey NO, Wysocki GP. Pregnancy tumour. An
dura, plasma cell infiltrate, osteoclastic activity, lack analysis. Oral Surg Oral Med Oral Pathol. 1991;72:1969.
of osteoblastic activity, hyperplastic with exocytosis 15. Damm DD, Cibull ML, Geissler RH, et al. Investigation
in crevicular epithelium, increased vascularity and into the histogenesis of congenital epulis of the newborn.
mixed inflammatory cellular infiltrates, leukocytes, Oral Surg Oral Med Oral Pathol. 1993;76:20512.
16. Dongari A, McDonnell HT, Langlais RP. Drug induced
lymphocytes, Histiocytes and plasma cells, amoxicillin
gingival overgrowth, Oral Surg Oral Med Oral Pathol.
and potassium clavulanate or ofloxacin.
1993;76:5438.
17. Flemmig TF. Periodontitis. Ann Periodontol. 1999;4:32-7.
HAIM-MUNK SYNDROME 18. Hartnett AC, Shiloah J. The treatment of acute necrotizing
ulcerative gingivitis, Quintessence Int. 1991;22:95100.
In this, there is plamoplanter keratosis, progressive 19. Hattab FN, Rawashdeh MA, Yassin OM, et al. Papillon-
periodontal disease, recurrent skin infection and skeletal Lefevre syndrome: a review of the literature and report of 4
manifestation is seen. Periodontal disease is of milder as cases. J Periodontol. 1995;66:41320.
compared to PapillonLefevre syndrome. 20. Hirschfeld I. Hypertrophic gingivitis; its clinical aspect. J
Am Dent Assoc. 1932;19:799.
BIBLIOGRAPHY 21. Ishikawa I, Umeda M, Laosrisin N. Clinical, bacteriological,
and immunological examinations and the treatment process
1. American Academy of Peridontology. Parameter on plaque- of two PapillonLefevre syndrome patients. J Periodontol.
induced gingivitis. J Peridontol. 2000;71 (Suppl):8512. 1994;65:36471.
2. American Academy of Peridontology. Position paper. 22. Jacobs MH. Pericoronal and Vincent’s infections:
Treatment of gingivitis and periodontitis. J Peridontol. bacteriology and treatment. J Am Dent Asso. 1953;30:392.
1997;68:124653. 23. Johnson BD, Engel D. Acute nectrotising ulcerative
3. American Academy of Peridontology: Position paper, gingivitis: a review of diagnosis, etiology and treatment, J
periodontal diseases of children and adolescents. J Peridontol. 1986;57:14150.
Periodontol. 1996;67:5762. 24. Jorgenson RJ, Cocker ME. Variation in the inheritance
4. American Academy of Periodontology: Concensus report: and expression of gingival fibromatosis, J Periodontol.
aggressive periodontitis, Ann Periodontol. 1999;4:53. 1974;45:4727.
5. American Academy of Periodontology: Informational paper. 25. Kerr DA. Granuloma pyogenicum. Oral Surg Oral Med
The pathogenesis of periodontal diseases. J Periodontol. Oral Pathol. 1951;4:15876.
1999;70:45770. 26. Liakoni H, Barber P, Newman HN. Bacterial penetration of
6. American Academy of Periodontology: Parameter on acute pocket soft tissues in chronic adult and juvenile periodontitis
periodontal diseases. J Peridontol. 2000;71(Suppl):8636. cases. An ultrastructural study. J Clin Periodontol.
7. Bakaeen G, Scully C. Hereditary gingival fibromatosis in a 1987;14:22.
family with ZimmermanLaband syndrome. J Oral Pathol 27. Lindhe J, Liljenberg B. Treatment of localized juvenile
Med. 1991;20:4579. periodontitis: results after 5 years. J Clin Periodontol.
8. Barker DS, Lucas RB. Localized fibrous overgrowth of the 1984;11:399410.
oral mucosa. Br J Oral Surg. 1967;5:8692. 28. Listgarten MA, Hellden L. Relative distributions of bacteria
9. Becks H. Normal and pathologic pocket formation. J Am at clinically healthy and periodontally diseased sites in
Dent Assoc. 1929;16:2167. humans. J Clin Periodontol. 1978;5:665.
29. Low SB, Clancio SG. Reviewing nonsurgical periodontal 36. Preus HR. Treatment of rapidly destructive periodontitis
therapy, J Am Dent Assoc. 1990;121:467-70. in PapillonLefevre syndrome: laboratory and clinical
30. Loyola AM, Gatti AF, Santos Pinto D Jr, et al. Alveolar and observations. J Clin Periodontol. 1988;15:61339.
extraalveolar granular cell lesions of the newborn: report 37. Ronbeck BA, Lind PO, Thrane PS. Desquamative gingivitis: 407
of case and review of literature. Oral Surg Oral Med Oral preliminary observations with tetracycline treatment. Oral
Pathol Oral Radiol Endod. 1997;84:66871. Surg Oral Med Oral Pathol. 1990;69:6947.
31. Moskow BS, Polson AM. Histologic studies on the extension 38. Seymour RA, Jacobs DJ. Cyclosporin and the gingival
of the inflammatory infiltrate in human periodontitis. J Clin tissues, J Clin Periodontol. 1992;19:111.
Periodontol. 1991;18:53442. 39. Sheiham A. Is the chemical prevention of gingivitis
32. Newman MG, Carranza FA Jr, Takei HH. Clinical necessary to prevent severe periodontitis? Periodontol
periodontology. Philadelphia: WB Saunders; 2001.p.9. 2000. 1997;15:1524.
33. Oikarinen K, Salo T, Kaar ML, et al. Hereditary gingival 40. Tinanoff N, Tanzer JM, Kornman KS, et al. Treatment of
fibromatosis associated with growth hormone deficiency. periodontal component of PapillonLefevre syndrome. J
Br J Oral Maxillofac Surg. 1990;28:3359. Clin Periodontol. 1986;13:610.
34. PenarrochaDiago M, BaganSebastian JV, VeraSempere F. 41. Yih W-Y, Maier T, Kratochvil FJ, et al. Analysis of
Diphenylhydantoininduced gingival overgrowths in man; a desquamative gingivitis using direct immunofluorescence
clinicpathological study. J Periodontol. 1990;61:5714. in conjunction with histology, J Periodontol. 1998;69:678
35. Perkins AE. Acute infections around erupting mandibular 85.
third molar. Br Dent J. 1944;76:199.
1. Trench mouth refers to: 6. Which of the following indices is used to measure
a. ANUG periodontal destruction:
b. Desquamative gingivitis a. Russell’s peridontal index
c. Fibromatosis gingival b. Extent and severity index by carlos and coworkers
d. Congenital epulis c. Both a and b
2. A typical necrotic punched out, crater like ulceration d. None of the above
seen on the interdental papaillae in: 7. The red complex associated with bleeding on probing
a. PapillonLefevre syndrome is comprised of:
b. Epulis a. E.corrodens, A.actinomycetem, Capnocytophaga
c. ANUG b. A.naeslundii, A.viscous, A.odontolyticus
d. None
c. P.gingivalis, T.forsythia, T.denticola
3. Elephantiasis gingivae refers to: d. Streptococus, Fusobacterium, Camphylobacter
a. ANUG
b. Granuloma pyogenicum 8. Plaque differs from materia alba:
c. Epulis a. Presence of bacteria
d. Fibromatosis gingiva b. Presence of glycoprotein
c. Presence of salvia
4. Following are the drug responsible for gingival
d. Absence of glycoprotein
enlargement, except:
a. Dilantin sodium b. Ciprofloxacin 9. Electronic instrument used to measure gingival
c. Cyclosporine d. Nifedipine crevicular fluid:
5. Reddened, scaly, rough palms and soles, inflamed a. Pericheck b. Periotemp
gingivae and horizontal bone destruction seen in: c. Periscan d. Periotron
a. Nager’s syndrome 10. Which of the following is not implicated in the etiology
b. PapillionLefevre syndrome of periodontal disease:
c. Patau’s syndrome a. Bacteriodes b. Veionella
d. ANUG c. Neisseria d. Eikenella
11. Breakdown of periodontal fibers in periodontitis is due 16. The organism least likely to be found in normal
to bacterial enzyme: gingival crevice:
a. Collagenase b. Hyaluronidase a. Fusobacterium
408 c. Coagulase d. None of the above b. Actinomyces
12. Corncob appearance seen in: c. Diptheroides
a. Supragingival calculus d. Streptococci species
b. Subgingival calculus 17. The number of bacteria in the oral cavity is greater:
c. Supragingival plaque a. In morning b. After meals
d. Subgingival plaque c. At night d. After brushing
13. Red complex includes: 18. Bacterial communication with each other in a biofilm
a. P.Gingivalis is known as:
b. A.actinomycetem comitans a. Corncob formation
c. Terenella forsythia b. Coaggregation
d. All of the above c. Quarum sensing
14. Suprabony pocket is: d. Translocation
a. Base of the pocket is apical to crest of the alveolar 19. Plaque differs from materia alba:
bone a. Presence of bacteria
b. Base of the pocket is coronal to crest of the alveolar b. Presence of glycoprotien
bone c. Presence of salvia
c. Pocket is at the level of CEJ d. Absence of glycoprotien
d. None of the above 20. Which of the following is not implicated in the etiology
15. Radiographic feature of peridontitis is: of periodontal disease:
a. Widening of peridontal ligament space a. Bacteriodes
b. Alvealor bone destruction b. Veionella
c. Loss of lamina dura c. Neisseria
d. All of the above d. Eikenella
17 Salivary Gland Pathology
Chapter Outline
Â Classification of salivary gland Â Uveoparotid fever

Â Development of salivary gland Â Tumors of salivary glands
Â Parotid salivary gland Â Histogenesis
Â Submandibular gland Â Theories of salivary gland tumor histogenesis
Â Sublingual gland Â General features of salivary gland tumors-
Â Aberrancy Â Clinical staging of salivary gland tumors
Â Aplasia and hypoplasia Â Pleomorphic adenoma
Â Hyperplasia of salivary gland Â Basal cell adenoma
Â Atresia Â Canalicular adenoma
Â Accessory duct Â Warthin’s tumor
Â Diverticuli Â Oncocytoma
Â Sialorrhea Â Myoepithelioma
Â Xerostomia Â Ductal papillomas
Â Sialolithiasis Â Mucoepidermoid carcinoma
Â Strictures and stenosis Â Central mucoepidermoid carcinoma
Â Mucocele (mucous extravasation phenomenon) Â Acinic cell adenocarcinoma
Â Salivary duct cyst or mucus retention cyst Â Adenoid cystic carcinoma
Â Ranula Â Polymorphous low grade adenocarcinoma
Â Sialosis (sialadenosis) Â Malignant mixed tumor
Â Allergic sialadenitis Â Carcinoma ex-pleomorphic adenoma
Â Mumps Â Carcinosarcoma
Â Cytomegalovirus inclusion disease Â Metastasizing mixed tumor
Â Bacterial sialadenitis Â Connective tissue tumors
Â Sjögren’s syndrome Â Necrotizing sialometaplasia
Â Mikulicz’s disease or benign lympho-epithelial lesion
CLASSIFICATION OF SALIVARY DEVELOPMENT OF SALIVARY GLAND

GLAND DISORDERS (FIGS 17.1A TO E)
410 (A) Developmental Disorders The three major sets of salivary gland- the parotid,
• Aberrancy submandibular and sublingual originate in a uniform
• Aplasia and Hypoplasia manner by oral ectodermal epithelium buds invading the
• Hyperplasia underlying mesenchyme.
• Atresia The parotid gland buds are the first to appear at the 6th
• Accessory Ducts week of intrauterine life, on the inner cheek, near the angles
• Diverticuli of mouth and grow back towards the ear. In the parotid and
• Congenital Fistula
ear region, the epithelial cord of cells branches and canalizes
(B) Functional Disorders to provide the acini and ducts of the gland. The duct and
• Sialorrhea acinar system are embedded in a mesenchymal stroma,
• Xerostomia
get organized into lobules and become encapsulated. The
• Allergic
parotid gland duct, although repositioned, traces the path of
• Associated with Malnutrition and Alcoholism
embryonic epithelial cord in the adult.
(C) Obstructive Disorders
The submandibular gland buds also appear in the 6th
• Sialolithiasis
week of intrauterine life as a grouped series, forming
• Mucus Plug
• Stricture and Stenosis epithelial ridges on the either sides of the midline, in the
• Foreign Bodies floor of the mouth. An epithelial cord proliferates back into
• Extra-ductal Causes the mesenchyme beneath the developing mandible to branch
(D) Cyst and canalize, forming the acini and ducts of submandibular
• Mucocele gland. The mesenchymal stroma is separated off the
• Ranula parenchyma lobules and provides a capsule for the gland.
(E) Asymptomatic Enlargement The sublingual glands arise in the 8th week of
• Sialosis (cont on next page) intrauterine life, as a series of about ten epithelial buds, just
• Oncocytoma lateral to the submandibular gland anlage. These branch and
• Monomorphic Salivary Adenomas canalize to provide a number of ducts opening independently
Benign but often recurrent beneath the tongue. The minor salivary glands begin their
• Pleomorphic Adenoma development during the third month of life. These glands
• Mucoepidermoid Tumor (Low Grade) arise from the oral ectodermal and endodermal epithelium.
• Acinic Cell Tumor (Some) Labial glands arise during the 9th week of intrauterine life
Malignant and are morphologically mature by 25th week.
• Carcinoma in Pleomorphic Adenoma
• Adenoid cystic carcinoma Classification of Salivary Glands
• Mucoepidermoid tumor(high grade)
• Exocrine glands: These are the glands whose products
• Acinic cell tumor (some)
are carried away by the ducts leading from the gland.
• Squamous carcinoma
• Adenocarcinoma • Mesocrine glands: The secretory product pass
• Undifferentiated carcinoma through the cell walls losing the cytoplasm.
• Serous salivary gland: The salivary glands which
(F) Infection
• Viral Infection produce a thin watery secretion are called serous.
• Bacterial Infection • Mucous salivary gland: These produce a thick,
• Mycotic Infection viscous substance called mucous.
(G) Autoimmune Disorders • Seromucous salivary gland: Salivary glands which
• Sjögren’s Syndrome produce serous and mucous in varying quantities.
• Mickulicz’s Disease • Major salivary gland: These are large salivary glands
• Uveoparotid Fever which are located outside the oral cavity and convey
• Recurrent Non-specific Parotitis their secretions through their ducts.
(H) Neoplasms • Minor salivary gland: These are smaller salivary
Benign but seldom recurrent glands confined to the mucous and submucous coat
• Warthin’s Tumor of the oral cavity.
Salivary Gland Pathology
411
B C
A D E
Figures 17.1A to E Development of salivary gland systematic approach
MAJOR SALIVARY GLANDS It then runs for a short distance obliquely forward, between
the buccinator and mucous membrane of the oral cavity
Major salivary glands are parotid, submandibular and and opens on the oral surface of the cheek, opposite the
sublingual. upper second molar.
Parotid Gland Blood supply: Parotid gland is supplied by the external
carotid artery and its branches near the gland.
It comes from the word para- around and otic-ear. It is like
an inverted flattened pyramid. It is the largest of the salivary Lymphatic drainage: Drains first to the parotid nodes and
glands weighing about 15 grams each. It lies between the from there to the upper deep cervical nodes.
mastoid process and vertical ramus of the mandible. The
Nerve supply: It is supplied by auriculotemporal nerve,
bulk of the parotid gland is situated in the retromandibular
plexus around the external carotid artery and greater
fossa. It is wedge shaped, with the broad edge of the wedge
auricular nerve.
lying subcutaneously and the apex lying deep between the
parotid fascia. It is divided into superficial and deep lobes
Submandibular Gland
by the facial nerve and its branches. It forms an irregular
lobulated yellowish mass, lying below the external acoustic It is a round biconvex salivary gland situated in the
meatus, between the mandible and the sternocleidomastoid. anterior part of the digastric triangle. It is irregular in
A small part of it, more or less detached lies between the form and about the size of a walnut. It is enclosed by
zygomatic arch superiorly and the parotid duct inferiorly is two layers of deep cervical fascia. The inner surface of
named accessory part of the gland. the submandibular gland is in contact with stylohyoid,
digastric and styloglossus muscle, posteriorly and with the
Stensen’s duct: The parotid duct which is called ‘Stensen’s’ hyoglossus and posterior border of the mylohyoid muscle,
duct is about 5 cm long and has thick walls. It emerges anteriorly.
from the substance of the gland to course anteriorly until
it reaches the anterior border of the masseter muscle Wharton’s duct: The submandibular duct which is called
at the point of upper and middle thirds. When it crosses ‘Wharton’s duct’, is about 5 cms long and its wall is much
the masseter muscle it receives the duct of the accessory thinner than that of parotid duct. It emerges from the middle
lobe. Around the border of the masseter muscle, the duct of the deep surface, of the superficial part, of the gland. It
turns sharply medially, often embedded in a furrow of the runs forward, beneath the deep part of the gland, between
protruding buccal fat pad. In its medial course, the duct the mylohyoid and hyoglossus muscle. It runs further
reaches the outer surface of the buccinator muscle, where it forward between the medial surface of the sublingual gland
perforates in an oblique direction anteriorly and medially. and the genioglossus muscle and finally ends by opening
into the summit of the sublingual papilla, situated in the the mandible is found posterior to the first molar and often
floor of the mouth, on the side of the frenulum. The last has a small communication with a major salivary gland.
few millimeters of the duct are often slightly widened. Developmental lingual salivary gland depression:
412
Arterial supply: The arteries supplying the submandibular The aberrancy of the salivary gland tissue represents
gland are derived from the lingual and facial branches of only an extreme example of the condition known as
external carotid artery. the developmental lingual mandibular salivary gland
depression. It is the developmental inclusion of the
Venous drainage: It drains into facial and lingual vein.
glandular tissue within or more commonly, adjacent to the
Nerve supply: Its nerve supply is from the branches of lingual surface of the body of the mandible, in a deep well
submandibular ganglion through which it receives fibers circumscribed depression. Mandible develops around the
from chorda tympani. lobe during development. It was first described by Stafne
in 1942 and hence referred to as Stafne’s cyst. It is rare
Lymphatic drainage: It passes to the submandibular
with incidence of 4 in every adults. Males are affected
lymph node.
more commonly than females. It is asymptomatic and only
Sublingual Gland diagnosed on radiographical examination. Microscopically
salivary gland tissue may be seen.
It lies above the mylohyoid and below the mucosa of the
floor of mouth. It is medial to the sublingual fossa of the Gingival salivary gland choristoma: Salivary tissue is
mandible, on either side of the symphysis menti and lateral regularly found in lymph nodes within the neck and can
to genioglossus muscle. It has about 15 ducts which open be mistaken for metastatic disease, if found in a neck
directly into the floor of mouth. dissection specimen. Ectopic salivary gland tissue has been
reported to occur in the gingiva, where it may be described
Bartholin’s duct: The duct of sublingual gland is called as ‘gingival salivary gland choristoma’.
‘Bartholin’s duct’. They are eight to twenty in number.
Some of the smaller sublingual ducts open into the Clinical significance: They may become site for develop-
sublingual fold, in the floor of the mouth, on either side ment of a retention cyst or neoplasm.
of lingual frenum. Some open into the submandibular duct
and others unite to form the “principle sublingual duct” Points to Remember
which opens in the floor. Ectopic salivary gland, developmental lingual salivary
gland depression, inclusion of the glandular tissue,
Blood supply: It is supplied by sublingual and submental Stafne’s cyst’, gingival salivary gland choristoma site
arteries. for development of a retention cyst.
Nerve supply: It is by lingual and chorda tympani nerve.
Lymphatic drainage: It passes to the submandibular APLASIA AND HYPOPLASIA
lymph nodes.
Aplasia or agenesis is the congenital absence of the salivary
gland. It was first described by Gruber in 1885.
ABERRANCY Aplasia may occur in association with other develop-
It is defined as that situation in which the salivary gland mental abnormalities such as atresia of lacrimal puncta and
congenital malformation of temporomandibular component.
tissue develops at a site where it is not normally found. It is
also called ectopic salivary gland.
Causes
Ectopic salivary tissue can develop anywhere within
the territory of the first and second branchial arches, in the It results due to regional action of some disturbing influ-
lateral neck, pharynx or middle ear. ence in early fetal development. Macdonald suggested ec-
todermal origin for this anomaly.
Clinical Features
Location: True aberrant salivary glands are most fre- Clinical Features
quently reported in the cervical region, near the parotid Any one of the glands or group of glands is missing, either
gland or body of the mandible. The salivary gland tissue in unilaterally or bilaterally (Fig. 17.2).
HYPERPLASIA OF SALIVARY GLAND

Sometime it is also called Adenomatoid hyperplasia of
minor salivary gland. 413
Causes
Hormonal disorders: Endocrine disorders and meno-
pause.
Metabolic disorders: Gout, diabetes mellitus.
Autoimmune: Sjögren’s syndrome, Waldenstrom macro-
globulinemia.
Syndrome: Aglossia-adactylia syndrome, Heerfordt’s
syndrome and Felty’s syndrome.
Figure 17.2 Hypoplasia of the unilateral parotid gland (Plain Miscellaneous: Hepatic disease, starvation, alcoholism,
CT). Size of the left parotid gland (yellow arrow) is remarkably inflammation, benign lympho-epithelial lesion, adiposity,
smaller compared with normal size of the right parotid gland hyperthermia, oligomenorrhea and certain drugs.
(red arrow) (Courtesy: M Shimizu)
Clinical Features
Symptoms: Patient complains of xerostomia, which may Location: It is more common in minor salivary glands of
be so severe as to necessitate the constant sipping of water the palate. It is seen on the hard palate or at junction of hard
throughout the day and particularly, during meal times. and soft palate
The lack of saliva results in rampant dental caries and early
Signs: It is usually asymptomatic palatal gland hyperplasia
loss of deciduous and permanent teeth.
appears as small localized swelling of varying size,
Signs: The oral mucosa appears dry, smooth, or sometimes measuring from several millimeters to 1 cm. The lesion has
pebbly and shows a tendency for accumulation of debris. an intact surface and is firm, sessile and normal in color.
Patients exhibit characteristic cracking of lips and fissuring
of the corners of mouth. Hypoplasia of salivary glands is Histopathological Features
rare but hypoplasia of parotid gland has been reported to be The mass appears microscopically as a closely packed
present with Melkerson Rosenthal syndrome. collection of normal appearing mucous acini, with the
usual intermingling of normal ducts.
LADD: Lacrimo-auriculo-dento-digital syndrome. In
this, hypoplasia of lacrimal and salivary gland can occur Management
in association with cup shaped ears, dental and digital
anomalies. As it cannot be differentiated from minor salivary gland
tumors, it becomes essential to excise it for microscopic
Management examination.
Institution of scrupulous oral hygiene in an attempt to Points to Remember
decrease dental caries and preserve the teeth as long as
Adenomatoid hyperplasia of minor salivary gland, minor
possible.
salivary glands of the palate, small localized swelling of
varying size, normal appearing mucous acini.
Points to Remember
Artesia of lacrimal puncta, xerostomia, oral mucosa
appears dry, cracking of lips, Melkerson Rosenthal ATRESIA
syndrome, lacrimo-auriculo-dento-digital syndrome, It is the congenital occlusion or absence of one or two
scrupulous oral hygiene. major salivary gland ducts.
Usually the submandibular duct in the floor of the Idiopathic paroxysmal sialorrhea: There is excessive
mouth fails to canulate during embryological development. salivation lasting for 2 hr 5 minutes which is associated
The newborn infant presents, within 2 or 3 days of life, with nausea and epigastric pain.
414 with submandibular swelling on the affected side due to
the presence of a retention cyst. It may produce a relatively Management
severe xerostomia. Speech therapy: This can be used to control neuromuscu-
lar control.
ACCESSORY DUCT Anti-cholinergic medication: Transdermal scopolamine
An accessory parotid lobe is the most common developmen- is used.
tal anomaly. It occurs in as many as 20 percent of subjects. Intraglandular injection of botulinum toxins as also
Its position is constant, arising from the horizontal been successful.
component of the parotid duct as it crosses the masseter Surgical technique like relocation and ligation of ducts
muscle. of gland can be done.
Its importance lies in the fact that any of the diseases that
can affect the salivary glands, may involve the accessory Points to Remember
lobe and lead to diagnostic confusion, as the possibility is Aphthous ulcer, ill fitting denture maceration around
not considered. This is because the symptoms and signs are mouth, idiopathic paroxysmal sialorrhea, speech therapy,
not within the normal anatomical territory of the parotid. anti-cholinergic medication, intraglandular injection of
Presence of additional duct in some salivary glands has botulinum toxins.
been reported.
XEROSTOMIA
DIVERTICULI It is dry mouth in which there is reduced secretion of saliva.
They are small pouches or out pocketing of the ductal
system of one of the major salivary glands. Their presence Causes
leads to recurrent episodes of acute parotitis. It can be caused by systemic disease salivary gland aplasia,
water/metabolic loss, radiation therapy, chemotherapy,
SIALORRHEA Sjögren's syndrome, diabetes, HIV infection, sarcoidosis
and graft versus host resistance.
Excess saliva production is called ‘Sialorrhea’. Local factors like decrease mastication, smoking and
Causes mouth breathing can also cause xerostomia.
Medication like antihistamine (diphenhydramine),
It may results from local irritation, aphthous ulcer, ill fitting decongestant, antidepressant, and sedative and anti-
denture, rabies, heavy metal poisoning, medication such as cholinergic agent can also cause xerostomia.
antipsychotic agent, cholinergic agonist.
Protective buffering system – episodic hypersecretion Clinical Features
to neutralized stomach acid in gastroesophageal reflux Symptoms: There is reduction of salivary secretion and
disease also can cause Sialorrhea. residual saliva appears as foamy or thick and ropey. Patient
Various disorders like mental retardation, cerebral pal- complaint of difficulty in mastication and swallowing
sy, Parkinson’s disease and amyotrophic lateral sclerosis
can also cause Sialorrhea. Sign: Mucosa is dry and gloves can stick to it. There is also
atrophy of filiform papillae.
Clinical Features There is increase prevalence of candidiasis, dental
Symptoms: There is social embarrassment due to drooling caries in the patient of xerostomia.
of saliva. Management
Sign: There is maceration around mouth, chin, and neck Artificial saliva can be given. Sugarless candy should be
which can get secondary infected. used to stimulate saliva.
Systemic pilocarpine can be used as sialagogue. Another Metabolic mechanism: In the presence of coexisting
sialagogue which can be used is cevimeline hydrochloride inflammation, a metabolic mechanism favors precipitation
an acetyl choline derivative. of salivary salts into the matrix.
415
Submandibular gland calculi more common due to Clinical and Radiological Features
following reasons: Age and sex distribution: They are usually encountered
Anatomic factors in middle-aged patients with slight predilection for
occurrence in men.
• The length and irregular course of Wharton’s duct
It usually occurs as a solitary concretion varying in size
• The submandibular gland and ductal system lies in a
from a few millimeters up to several centimeters.
dependent position
• The greater size and position of the orifice Symptoms: The symptoms of sialolithiasis vary but intra-
• The orifice is much smaller than duct lumen glandular stones seem to cause less severe symptoms than
Physiochemical factors the extra-glandular or intra-ductal types. On occasions,
there may be complete absence of subjective symptoms.
• High mucin content of saliva
Many patients complain of moderately severe pain and
• Great degree of alkalinity with high percentage of
intermittent transient swelling during meals, which
organic matter
resolves after meals. As the calculus itself rarely blocks a
• Greater concentration of calcium and phosphate salts
duct completely, the swelling subsides as salivary demand
• Low content of carbon dioxide
diminishes and as saliva seeps past the partial obstruction.
• Richness in phosphatase enzyme.
The occlusion of the duct prevents the free flow of saliva
and this stagnation or accumulation of saliva, when under
Points to Remember pressure, produces pain. If no treatment is instituted, it
Reduction of salivary secretion, dry mucosa, candidiasis, appears as a pronounced exacerbation characterized by
dental caries, artificial saliva, systemic pilocarpine. an acute suppurative process with attendant systemic
manifestations such as fever and malaise.
SIALOLITHIASIS Signs: Pus may exude from the duct orifice. The soft tissues
surrounding the duct show a severe inflammatory reaction,
It is the also called salivary gland stone or salivary gland
which may appear as swelling, redness and tenderness.
calculus. These are stones within major and minor salivary
Stones in the more peripheral portion of the duct may often
glands. These are the most common calcifications found in
be palpated, if they are of sufficient size. Sometimes, the
soft tissues of oro-orbital region.
overlying mucosa may ulcerate over the stone allowing the
It is the formation of calcific concretions within the
calculus extends into the oral floor. No saliva is seen to
parenchyma or ductal system of the major or minor salivary
be coming out through the duct orifice. If stone is present
glands.
in one duct only then saliva will not come out from that
Composition duct. It can be tested by placing two dry swabs one on each
orifice and some lemon juice is dropped on the dorsum
The calculus consists of laminated layers of organic material,
of the tongue. A minute later patient is asked to move the
covered with concentric shells of calcified material.
tongue up. The swab on the orifice of the duct where the
The crystalline structure is chiefly hydroxyapatite and
stone is impacted will remain dry, whereas the other swab
contains octacalcium phosphate. The chemical composi-
will be wet.
tion is principally calcium phosphate and carbon with trac-
es of magnesium, potassium, chloride and ammonium. Stones in minor salivary glands: Sialolithiasis of minor
salivary gland is a rare occurrence. The most common site is
Etiology and Pathogenesis buccal mucosa either near the commissure or in proximity
Neurohumoral mechanism: A neurohumoral condition, to the mandibular mucobuccal fold. It is more common
leading to salivary stagnation, results in a nidus and matrix after the age of 39 years. The lesions appear as firm, freely
formation. movable masses, deeply situated into the mucosal surface.
Radiological features: It appear as radiopaque mass on

radiograph (Fig. 17.3).

Macroscopic features: It appears as hard mass which is
round, oval or cylindrical. They are yellow or white or
yellow-brown in color (Fig. 17.4).
It is seen as calcific concretions arranged in concentric
laminations around the amorphous debris (Fig. 17.5).
There is also metaplasia of lining cells with
inflammatory infiltrates surrounding it. Sialoliths are
Figure 17.5 Concentric laminations seen in sialolithiasis
also irregular, hexagonal and needle like plate shaped

crystals.
Diagnosis
Palpation is an indispensable tool in the diagnosis of
sialoliths. Palpation of the suspected gland frequently
reveals it to be larger or firmer than the normal gland of
the opposite side. Digital manipulation will produce a flow
of saliva through the duct orifice and will allow visual
inspection of the salivary fluid.
Figure 17.3 Sialolith in Parotid Stenson’s duct (arrow). During examination, the soft tissues overlying the duct
(Courtesy: Dr Swapnil Moghe, Senior Lecturer, Department should be manually stretched. Often, the physical distortion
of Oral and Maxillofacial Surgery, People Dental Academy,
caused by the presence of calculus will become apparent.
In addition yellowish color of the calcific deposits may be
seen through the distended and thinned mucous membrane.
A metallic duct probe can also be of value. Careful
probing of the duct with a metallic probe will indicate the
existence as well as the location of calculus.
Radiographic examination usually reveals the presence
of calcific deposits.
Sialography is an invaluable aid in isolating the
sialoliths which had not been identified on the standard
intraoral and extraoral radiography.
Management
Manual manipulation of stone within the duct should be
carried out.
Surgical approach should be taken for large stone
Figure 17.4 Parotid duct stone removed. (Courtesy: Dr Swapnil Shock wave lithotripsy, salivary gland endoscopy and
Moghe, Senior Lecturer, Department of Oral and Maxillofacial radiologically guided basket retrieval are newer technique
Surgery, People Dental Academy, Bhopal, Madhya Pradesh, India that can be carried out.
It is not a true cyst as it lack epithelial lining. Retention

Points to Remember
mucocele also known as ‘salivary duct cyst’ is separated
Salivary gland stone or salivary gland calculus, entity so this is described under separate heading.
laminated layers of organic material, hydroxyapatite 417
octacalcium phosphate, moderately severe pain, Pathogenesis
intermittent transient swelling, severe inflammatory
Obstruction of salivary gland duct leads to its dilatation
reaction surrounding the duct, stones in minor salivary
proximal to the obstruction, with the formation of epithelial
glands, radiopaque mass on radiograph, stone is of
lining retention cyst.
yellow or white or yellow-brown, calcific concretions,
Cut or traumatic defect of a salivary gland is
metaplasia of lining cells.
responsible for the production of mucus extravasation
mucocele. There is accumulation of mucus in the
STRICTURES AND STENOSIS connective tissue and with the continuous pooling of
saliva a clearly demarcated cavity develops which has no
It is cause by irritation from prosthetic appliances, maloc- epithelial lining.
cluded or malpositioned teeth, acute trauma with resultant
edema and/ or scarring and intraductal tumor formation. Clinical Features
Age and sex distribution: Age of the patient varies with
Types peak frequency in the 3rd decade but most of the cases
• Papillary obstruction occur before the age of 50. Retention cysts occur most
• Duct obstruction often in older patients, where as mucus extravasation cysts
occur in younger patients. Equal in sex frequency, with
Types most cases are reported in white.
Papillary obstruction: It may be either acute ulcerative Location: They are very common and occur most
obstruction or chronic fibrotic stenosis. Acute ulcerative frequently on the inner aspect of lower lip; but may also
obstruction is usually caused by acute trauma to the papilla occur on the palate, cheek, tongue and floor of mouth.
and is treated conservatively with saline rinses and salivary
Symptoms: The lesion may lie fairly deep in the tissues
gland massage. The ulcer generally heals without scarring
or be exceptionally superficial. Patient may complain
and the symptoms will subside in such cases. In chronic
of painless swelling which is frequently recurrent. The
fibrotic papillary obstruction, irritations to papilla has been
swelling may suddenly develop at meal time and may drain
recurrent and scarring exists.
simultaneously at intervals.
Duct obstruction: It may be due to a variety of factors.
Signs: The mucocele may be only 1 to 2 mm in diameter,
In cases of ductal obstruction secondary to acute trauma
but is usually larger; majority of them being between 5
treatment is directed towards providing the duct patency
to 10 mm in diameter. Superficial cyst appears as bluish
until the edema is resolved. When the ductal obstruction
mass, as the thin overlying mucosa permits the pool of
occur secondary to irritation or scar contracture, sialo-
mucus fluid to absorb most of visible wavelength of light.
grams are helpful in localizing the status of gland. If the
If inflamed, it is fluctuant, soft, nodular and dome shaped
gland is healthy, progressive and frequent dilatation of
elevation. Deeper lesions have the color of normal mucosa
involved duct with lacrimal probes is generally successful
and are firmer. The swelling is round or oval and smooth.
in relieving the symptoms and signs. If this does not prove
It is either soft or hard depending upon the tension in
beneficial, ductoplasty is indicated.
the fluid. It cannot be emptied by digital pressure. On
aspiration, it yields sticky viscous clear fluid (Figs 17.6
MUCOCELE (MUCOUS and 17.7).
EXTRAVASATION PHENOMENON)
Superficial mucocele: It can be seen in soft palate,
It is a term used to describe the swelling caused by pooling retromolar and along the posterior border of buccal mucosa.
of saliva at the site of injured minor salivary gland. It is They presented as single or multiple tense vesicle with less
also know mucus escape phenomenon. than 4 mm in diameter. This lesion burst leaving painful
418
Figure 17.6 Mucocele presented as dome shaped elevation Figure 17.8 Mucocele showing cyst irregularly shaped area
on lower lip of mucous pool surrounded by inflammatory cell reaction
Figure 17.7 Mucocele presented as bluish swelling on lower Figure 17.9 Mucocele showing circumscribed appearance
lip (Courtesy: Dr Aparna Thombre, Reader, Department of (Courtesy: Dr Aparna Thombre, Reader, Department of Oral
Oral Pathology, VSPM Dental College and Hospital, Nagpur, Pathology, VSPM Dental College and Hospital, Nagpur,
Maharashtra, India) Maharashtra)
ulcer which heal in few days. Vesicular appearance occur In well defined cysts the periphery consists of
due to superficial spilling of mucin causing separation of granulation tissue or condensed fibrous tissue or both and
epithelium from connective tissue. is infiltrated by vacuolated macrophages, lymphocytes and
polymorphonuclear leukocytes, including eosinophils. One
Histopathological Features or more dilated ducts are present and sometimes a breach
(Figs 17.8 to 17.10) may be seen in the duct.
Poorly defined cysts consist of irregularly shaped, poorly
defined pools containing faintly eosinophilic mucinous Management
material and numerous vacuolated macrophages which are Complete excision of the mucocele under local anesthesia.
sometimes called muciphage. To prevent recurrence adjacent minor salivary gland should
Some of these cysts are smaller and others extend be removed.
widely into the connective tissue. Injection of steroid and cryosurgery can be tried.
The lumen of cyst like cavity is filled with an eosinophilic
coagulum, containing variable numbers of cells chiefly 419
leukocytes and mononuclear phagocytes.
The second group of well-defined cysts may be
partially or completely lined by epithelium. The
epithelium varies, it may consist of one or two layer of
cuboidal cells or a thicker pseudo stratified columnar
epithelium.
Management
Conservative surgical excision of isolated cyst should be
carried out.
Figure 17.10 Mucocele seen under high power showing Points to Remember
muciphages Mucus duct cyst, sialocyst, parotid gland, bluish, firm
on palpation, mucous retentions cysts, eosinophilic
Points to Remember coagulum, one or two layer of cuboidal cells.
Mucus escape phenomenon, obstruction of salivary
gland, inner aspect of lower lip, painless swelling, blu-
ish mass, dome shaped elevation, superficial mucocele, RANULA
sticky viscous clear fluid, muciphage, vacuolated mac- It is derived from Latin word Rana tigerina, i.e. frog belly.
rophages, lymphocytes, polymorphonuclear leukocytes,
complete excision. Definition
The term ranula is used for the mucocele occurring
SALIVARY DUCT CYST OR MUCUS in the floor of the mouth, in association with ducts of
submandibular or sublingual glands.
RETENTION CYST
It is caused by obstruction of minor salivary gland duct Types of Ranula
which causes the backup of saliva. This continuous pressure • Superficial: The superficial variety may develop as
dilates the duct and forms a cyst like lesion. It is lined by a retention or extravasation phenomenon associated
epithelium. It is also called mucus duct cyst, sialocyst. with trauma to one or more of the numerous excretory
ducts of the sublingual salivary gland.
Clinical Features
• Plunging or cervical: It ramifies deeply into the neck.
Age: It is more commonly seen in the adult patient.
Location: It is seen in parotid gland, intraorally it is floor Clinical Features
of mouth, buccal mucosa and lips. Age and sex distribution: They are usually unilateral.
Appearance: They appear bluish depending on the depths It is usually in children and young adults with no sex
of cyst below the surface. Cyst adjacent to submandibular predilection.
gland duct may have amber color. Location: The typical position is on the floor of the mouth,
Sign: They are firm on palpation. below the tongue and on the side of frenum.
Mucous retentions cysts: Some patient develop promi- Appearance: They produce blue swelling like a frog’s
nent ectasia of excretory ducts of salivary gland resulting belly; hence it was given the term ‘ranula’ (‘ranula’ in
in mucus retention cyst. Lesion present is painful nodules Greek mean frog’s belly). The overlying mucosa is normal
from which mucus or pus can be expressed. in appearance.
Symptoms: It develops as slowly enlarging painless mass Bidigital palpation: To inspect plunging ranula, bidigital
on one side of the floor of mouth. When the swelling palpation should be performed. On finger is placed inside
suddenly grows it may be painful. Big ranula may cause the mouth on the ranula and the other finger is place on the
420 difficulty in speech or eating. swelling in the submandibular region. If pressure on the
first finger causes sense of fluctuation on 2nd finger or vice
Signs: It is spherical or dome shaped with only top half is
versa, then it is plunging ranula.
visible. It is smaller in early morning and largest just before
meals, due to increased secretory activity in periods of Histopathological Feature
gustatory stimulation and water absorption from the pooled
mucus during inactive period. It is soft and tends to fluctuant Histopathological appearance is same as that seen in
(Fig. 17.11). It cannot empty by pressure and is non-pulsatile. mucocele.
Fluctuation and transillumination: Both tests are posi- Management

tive. The ranula is typically known as brilliant translucent Surgical excision: They are best treated by surgical
swelling. Aspiration yields sticky clear fluid. excision including a portion of the surrounding tissues.
Points to Remember Partial excision with marsupialization: The major

Rana tigerina, blue swelling like a frog’s belly, painless part of the cyst wall together with its overlying mucous
mass, spherical or dome shaped, non-pulsatile, fluctua- membrane is excised.
tion and transillumination tests are positive.
Plunging ranula: Bidigital palpation, cervical pro- SIALOSIS (SIALADENOSIS)
longation, partial excision with marsupialization. It is characterized by non-neoplastic non-inflammatory
enlargement of the salivary gland.
Plunging Ranula
Cause
When intrabuccal ranula has a cervical prolongation,
it is called deep or plunging ranula. It is derived from The condition is found in association with systemic
cervical sinus. It lies along the posterior border of the diseases especially cirrhosis, diabetes, ovarian and thyroid
mylohyoid muscle and appears in the submandibular insufficiency, alcoholism and malnutrition.
region. Sometimes, plunging ranula herniated through the There is dysregulation of autonomic innervations of the
mylohyoid muscle and cause a swelling in suprahyoid or salivary acini, causing an aberrant intracellular secretory
infrahyoid region. cycle. This lead to excessive accumulation of secretory
granules with marked enlargement of aciner cells.
Clinical Features
Sex distribution: More commonly affects the females.
Location: The enlargement is usually bilateral and may
present a course of recurrent painless enlargement of gland.
The parotid gland is more frequently affected.
Symptoms: Swelling of the preauricular portion of the pa-
rotid gland is the most common symptom, but retroman-
dibular portion of the gland may also be affected.
Sialographic features: It will show leafless tree appear-
ance.
Histopathological and Laboratory Features

Figure 17.11 Ranula seen as bluish swelling in the floor of Laboratory finding: A characteristic alteration in the
mouth chemical constituents of saliva is a distinguishing feature
of sialosis. Significant elevation of salivary potassium and MUMPS

concomitant decrease in salivary sodium is observed.
Microscopically there is hypertrophy of acinar cells. It is an acute contagious viral infection, characterized
The nuclei are displaced to cell base, and cytoplasm is chiefly by unilateral or bilateral swelling of the salivary 421
engorged with zymogen granules. glands. It mainly affects major salivary glands, but also
affects the testis, meninges, pancreas, heart and mammary
Management glands. It is also called endemic parotitis.
The underlying cause should be treated, which will help in
Etiology
reducing the swelling.
It is endemic in most urban population. It is caused by
Points to Remember paramyxovirus family with genus Rubella virus. It usually
spreads from human reservoir, by airborne infection of
Non-neoplastic non-inflammatory enlargement of
infected saliva and possibly urine.
the salivary gland, recurrent painless enlargement of
gland, leafless tree appearance, significant elevation of Clinical Features
salivary potassium, hypertrophy of acinar cells, zymogen
Age, sex and incubation period: It has got incubation
granules.
period of 2 to 3 weeks. It is more common in boys than in
girls and most often seen between the ages of 5 to 15 years.
ALLERGIC SIALADENITIS
Prodromal symptoms: It is preceded by onset of headache,
In some cases, it may not appear as a true hypersensitivity chills’ moderate fever, vomiting and pain below the ear
reaction but rather as a toxic or idiosyncratic reaction to which lasts for about one week.
drugs that cause a decreased salivary flow, resulting in
Symptoms: The parotid gland is most commonly involved
secondary infection.
and it is usually bilateral. Submandibular gland may also
Various drugs which have been reported to cause al-
be involved, although this is less noticeable and cause less
lergic sialadenitis include sulfisoxazole, phenothiazines,
pain. Both the parotid glands may involve simultaneously,
iodine containing compounds, mercury, thiouracil and
but more commonly one parotid gland swells 24 to 48
phenylbutazone.
hours after the other. It is then followed by sudden onset
Mechanism: The exact mechanism of salivary gland of salivary gland swelling which is firm somewhat rubbery
enlargement and loss of function following administration or elastic and without purulent discharge from the salivary
of these drugs is not known. Most of the drugs cause gland duct.
decrease in capillary permeability, where as others cause
Signs: The enlargement of parotid gland causes elevation of
sodium and chloride retention, which subsequently leads
ear lobule and it produces pain upon mastication especially
to edema.
while eating sour food (Fig. 17.12). Papilla on the opening
Symptoms: The clinical appearance of allergic sialadenitis of parotid duct is often puffy and reddened.
varies, but in most of the cases there is bilateral parotid
Epididymo-orchitis: This is common finding seen in
gland enlargement following the administration of the drug.
mumps. Affected testicle exhibits rapid swelling with pain
The enlargement may be painful and is usually associated
and tenderness. After resolution atrophy occur in testicle.
with conjunctivitis and skin rashes.
Other systemic disease like oophoritis, mastitis,
It is a self limiting disease and needs no treatment. But
menigoencephalitis, cerebellar ataxia, hearing loss, pan-
in some cases, secondary bacterial infection may develop
creatitis, arthritis, carditis and decrease renal function can
and need treatment.
occur.
Points to Remember Diagnosis
Sulfisoxazole, phenothiazines, iodine containing com- The presence of parotitis and accompanying systemic signs
pounds, mercury, thiouracil and phenylbutazone, bilat- of viral infections. Salivary amylase level is increased. A
eral parotid gland enlargement. paramyxovirus may be isolated from saliva for as long as
Site: In newborns, infection is generalized and is usually

fatal with involvement of liver, lungs and central nervous
system.
422
Signs: Infants who survive the infection may have per-
manent central nervous system involvement, including
metal retardation and seizures. There may be hepatosple-
nomegaly, hemolytic anemia and hemorrhagic tendency.
It may cause clinical disease of salivary gland, causing
enlargement of the gland.
Laboratory Investigations
Intranuclear and cytoplasmic inclusions in the cells of
Figure 17.12 Mumps showing swelling and elevation of
salivary glands are constant features of the disease.
ear lobules (Courtesy: Dr Swapnil Moghe, Senior Lecturer
Department of Oral and Maxillofacial Surgery, People Dental Points to Remember
Academy, Bhopal, Madhya Pradesh, India) Salivary gland virus disease, infection is generalized,
central nervous system involvement, heapatospleno-
6 days before and up to 99 days after the appearance of megaly, enlargement of the gland.
salivary gland swelling.
Management
BACTERIAL SIALADENITIS
Most of the cases are self limiting, with salivary gland It is also called suppurative parotitis.
enlargement subsiding within a week.
Etiology
Prevention with live attenuated vaccine is the best
method of controlling the disease. Symptomatic treatment Microorganisms: It is most commonly caused by penicil-
is given to control pain and swelling. lin resistant Staphylococcus aureus or streptococci virid-
ians.
Points to Remember Predisposing factors: It can occur in conditions such as
Paramyxovirus family with genus Rubella virus, prodor- dehydration, malnutrition, cancer and surgical infections.
mal symptoms, bilateral parotid gland swelling, rubbery
or elastic, elevation of ear lobule, epididym-oorchitis, Oral hygiene: Poor oral hygiene is an important contribu-
ophoritis, mastitis, menigoencephalitis, cerebellar ataxia, tory factor.
hearing loss, pancreatitis, arthritis, carditis, salivary Drugs: Drugs like anti-Parkinson’s, diuretics and antihis-
amylase level increased, live attenuated vaccine. taminic have been reported to be a contributory factor for
acute bacterial sialadenitis.
CYTOMEGALOVIRUS INCLUSION Types of Bacterial Sialadenitis
DISEASE • Acute bacterial sialadenitis
It is also called salivary gland virus disease. It is caused • Chronic bacterial sialadenitis
by cytomegalovirus, a herpes virus. It is common in • Subacute necrotizing sialadenitis
immunosuppressed adult. Although it is congenital in • Chronic sclerosing sialadenitis
nature, it is usually secondary to concurrent disease which
has caused debilitation. Clinical Features
Clinical Features Acute Bacterial Sialadenitis
Age: It affects primarily in newborn infants and children, Age: Most of the cases occur in adults but neonates and
but adults are also affected. childhood form of the disease may occur.
Location: Unilateral involvement of parotid gland is

common.
Symptoms: It begins with the elevation of body tempera- 423
ture and sudden onset of pain at the angle of the jaw which
is intense when the extensive infection is contained within
the confines of the parotid capsule. The localized symp-
toms are accompanied by fever, leukocytosis and other
generalized signs and symptoms of acute bacterial infec-
tion.
Signs (Fig. 17.13): Parotid gland is tender, enlarged and
the overlying skin is warm and red. The swelling usually
causes elevation of the ear lobule and the overlying skin
is characteristically warm and red. Early in the disease, Figure 17.14 Parotid infection showing purulent discharge
flecks of purulent material can be expressed from the from the duct (Courtesy: Dr Swapnil Moghe, Senior Lecturer
salivary duct orifice. Intraorally the parotid papilla may be Department of Oral and Maxillofacial Surgery, People Dental
inflamed and pus may exude or be milked from the duct of Academy, Bhopal, Madhya Pradesh, India)
the affected gland (Fig. 17.14). Cervical lymphadenopathy
usually develops.
Chronic Bacterial Sialadenitis

It is usually caused by Streptococcus viridans, E. coli or
proteus. As compared to acute parotitis, it can be seen in
normal children or in as in adults.
Age: The childhood form commonly begins between the
ages of 3 to 5 years and is usually unilateral.
Signs: It appears as a unilateral swelling at the angle of
the jaw in a patient with the history of similar occurrence.
Salivary flow is accompanied by flecks of purulent
Figure 17.15 Chronic bacterial sialadenitis presenting as
swelling at angle of jaw (Courtesy: Dr Chole, Modern Dental
College, Indore, Madhya Pradesh, India)
material. After several recurrences, fibrosis of the glandular

parenchyma occurs, which leads to decreased salivary
flow. The pain is usually minimal and antibiotic therapy
resolves the infection within a week.
Recurrent form is most often due to duct obstruction,
congenital stenosis, Sjögren’s syndrome, or previous viral
infection or allergy.
Chronic Sclerosing Sialadenitis

It is also called Kuttner’s disease.
Location: It is common in submandibular gland.
Figure 17.13 Bacterial sialadenitis showing enlargement of It is cause by salivary ductal calculi causing subsequent
parotid gland pyogenic bacterial infections (Fig. 17.15).
Appearance: It is a chronic inflammatory disease of the

Points to Remember
major salivary glands characterized by enlargement of the
glands, resulting in fibrous tumor like masses. Acute type: Sudden onset of pain, overlying skin is
424 warm and red, flecks of purulent material, accumulation
Subacute Necrotizing Sialadenitis of neutrophils in ductal system.
It is most commonly seen in teenagers and young adults. Chronic type: Streptococcus viridans, E. coli or proteus,
It involves minor salivary gland or hard and soft palate. unilateral swelling at the angle of the jaw, recurrent form
There is a painful nodule covered by intact erythematous scattered or patchy infiltration of salivary parenchyma
mucosa. chronic inflammation
Kuttner’s disease: Chronic sclerosing sialadenitis,
Histopathological Features salivary ductal calculi causing subsequent pyogenic
bacterial infections, chronic inflammatory disease of the
Acute sialadenitis: There is accumulation of neutrophils
major salivary.
in ductal system and acini.
Subacute necrotizing sialadenitis: Mixed infla-
Chronic sialadenitis: There is scattered or patchy infiltra- mmatory infiltrate, neutrophils, lymphocytes
tion of salivary parenchyma by lymphocytes and plasma Management: Meticulous oral hygiene, parenteral
cells. antibiotics, patient hydrated.
Subacute necrotizing sialadenitis: There is heavy
mixed inflammatory infiltrate consisting of neutrophils, SJÖGREN’S SYNDROME
lymphocytes, histiocytes and eosinophils. Acinar cell are
lost and remaining exhibits necrosis. It is a chronic inflammatory disease that predominately
affects salivary, lacrimal and other exocrine glands. It was
Chronic sclerosing sialadenitis: It can lead to chronic first described by Henrik Sjögren in 1933. It predominately
inflammation, ensuing in the atrophy of mucous and affects middle aged and elderly women.
serous cells and hyperplasia of the involved connective
tissue leading to formation of the tumor like swollen Types
masses. • Primary Sjögren’s or sicca syndrome sicca: It also
called sicca syndrome and it consists of dry eyes
Management
(xerophthalamia) and dry mouth (xerostomia). Eye
Meticulous oral hygiene should be practiced. Soft diet lesion called keratoconjunctivitis sicca (sicca mean
should be given as chewing is painful. dry)
It is treated aggressively because even with antibiotics • Secondary Sjögren’s syndrome: It consists of dry
death can result in debilitated patients. Specimen of eyes, dry mouth and collagen disorders usually
purulent material should be immediately sent to laboratory rheumatoid arthritis or systemic lupus erythematous.
for sensitivity and culture.
Treatment usually starts with high dose of parenteral Etiology and Pathogenesis
antibiotics active against penicillin resistant staphylo-
Immunological findings: The lesion in this syndrome is
coccus.
immunologically mediated inflammatory exocrinopathy.
The patient must be adequately hydrated and the
It begins with periductal infiltration of the tissue by
electrolyte balance should be properly maintained with
mononuclear cells.
intravenous fluids. Salivation should be stimulated to
facilitate drainage by sucking the sour hard candy. Autoantibodies: The B cell hyperactivity may result from
Oral hygiene should be maintained by debridement and deficiency of suppressor T lymphocytes or B lymphocytes
irrigation. If improvement does not occur surgical drainage by producing autoantibodies against them. Antinuclear
of the affected gland should be performed. antibodies found in patients with Sjögren’s syndrome are
directed against many nuclear antigens, most commonly to

Etiology
DNA histone. Patients with secondary Sjögren’s syndrome
tend to develop antibodies against the EBV-associated • Immunological findings
nuclear antigen antibodies rheumatoid arthritis nuclear • Autoantibodies 425
antigen (RANA). • Polyclonal hyperglobulinemia
• B2 microglobulin
Polyclonal hyperglobulinemia: Serum immunoglobulin • Immune complex
levels of IgG and IgM are also raised. • Cell mediated immune response
B2 microglobulin: Serum salivary level of B2 microglobu- • Natural killer cell activity
lin is raised in minority of patients and correlates with sali- • Virologic aspect
vary lymphocyte infiltrate. • Genetic aspect
• Lympho-proliferative malignancy
Immune complex: Circulating immune complexes are
found in patients with primary and secondary Sjögren’s Clinical Features
syndrome.
Oral
Cell mediated immune response: Delayed hypersensitiv-
Symptoms: Xerostomia is a major complaint in most of the
ity response to skin testing is more depressed in patients
patients. But many patients do not complain of dry mouth,
with secondary Sjögren’s syndrome than in patient with
but rather of an unpleasant taste, difficulty in eating dry
primary Sjögren’s syndrome. Lymphokine production in
food, soreness or difficulty in controlling dentures. Pus may
response to antigen present in normal salivary tissue, is in-
be emitted from the duct. Angular stomatitis and denture
creased in patients with Sjögren’s syndrome.
stomatitis also occur. Dry mouth may be accompanied
Natural killer cell activity: Augmented natural killer by unilateral or bilateral enlargement of parotid gland,
cell activity is impaired in some patients with Sjögren’s which occurs in about one third of the patients and may
syndrome. Although natural killer cells provide a defense be intermittent. Enlargement of submandibular gland may
against viral infection and tumor, their role in Sjögren’s also occur.
syndrome is unclear.
Signs: Clinically, the mouth may appear moist in early
Virologic aspect: Culture of saliva does not show any stages of Sjögren’s syndrome but later, there may a lack
specific microorganisms and serologic studies have of the usual pooling of saliva in the floor of the mouth and
failed to show increased titers of antibodies, except to frothy saliva may form along the lines of contact with oral
cytomegalovirus (CMV). soft tissue. In advanced cases the mucosa is glazed, dry
and tends to form fine wrinkles. Soreness and redness of
Genetic aspect: Genetic effects of Sjögren’s syndrome
mucosa is usually the result of candidial infection.
depend on HLA-Linked and non HLA-Linked genes.
In some patients, there may be clicking quality of their
Relatives of a patient with Sjögren’s syndrome often show
speech, caused by sticking of the tongue to the palate.
a high incidence of connective tissue diseases. Primary
The tongue typically develops a characteristic lobulated,
Sjögren’s syndrome is associated with HLA-B8 and DR3 and
usually red surface with partial or complete depapillation.
secondary Sjögren’s syndrome is associated with HLA-B8
There is also decrease in number of taste buds, which leads
DR4 and BW44.
to an abnormal and impaired sense of taste.
Lympho-proliferative malignancy: Enlargement of Dental caries is severe and gross accumulation of
salivary glands in patients with Sjögren’s syndrome is plaque may be obvious. Periodontal disease can also occur.
occasionally massive and associated with enlargement Sjögren’s syndrome is the most common underlying
of regional lymph nodes, a condition known as ‘pseudo cause of acute bacterial sialadenitis in ambulated patients.
lymphoma’. Malignant B cell lympho-proliferation has Such infections are usually either staphylococcal or
been shown to affect patients with Sjögren’s syndrome. pneumoccocal and usually cause swelling of the salivary
island. Lymphocytic infiltration of the glands with ensuing

atrophy of glands also occurs.
426 Laboratory Investigations

Rose Bengal staining test: Keratoconjunctivitis sicca is
characterized by corneal keratotic lesion, which stains pink
when rose Bengal dye is used.
Schirmer test: The reduced lacrimal flow rate is measured
by this test. A strip of filter paper is placed in between the
eye and the eyelid to determine the degree of tears which
is measured in millimeter. When the flow is reduced to
less than 5 mm in a 5 minute sample, patient should be
considered positive for Sjögren’s syndrome.
Figure 17.16 Snowstorm appearance seen in Sjögren‘s Sialometry: Salivary flow rate estimation is a sensitive
syndrome indicator of salivary gland function. Parotid glands make
the major contribution to total salivary flow and are the
most consistently affected glands in patients with Sjögren’s
gland. The overlying skin is red, tender and shiny. The syndrome. Stimulated flow rate in symptomatic primary
regional lymph nodes may be enlarged and tender. and secondary Sjögren’s syndrome is usually below 0.5 to
On sialography it can show snowstorm or cherry 1.0 ml/minute (normal 1 to 1.5 ml/minute).
blossom appearance (Fig. 17.16).
Sialochemistry: Parotid saliva in Sjögren’s syndrome
General contains twice as much total lipid and has elevated content
Keratoconjunctivitis sicca: The patient usually complains of phospholipids and glycolipids than the normal saliva.
of dry eyes or continuous irritation in the eyes. Severe lac- The sodium chloride and phospholipids levels are higher in
rimal gland involvement may lead to corneal ulceration as saliva of Sjögren’s syndrome patient.
well as conjunctivitis. Immunologic: A routine autoantibody profile can
Connective tissue disorders: In patients with secondary usually be carried out with the particular aim of detecting
Sjögren’s syndrome, rheumatoid arthritis is typically rheumatoid and antinuclear factors.
long standing and clinically obvious. Patients may have Hematological investigations: It is necessary, particularly
small joint and ulnar deviation of fingers and rheumatoid to exclude anemia. ESR or plasma viscosity, leukopenia
nodules. occasionally may also be found.
Dryness of pharynx, larynx and nose are noted by some
patients. This is accompanied by lack of secretion in the Microbiological investigations: A swab from oral mucosa
upper respiratory tract, may lead to pneumonia. Vaginal should be taken to confirm candidiasis, if there is soreness
dryness may be also complained by some females. and erythema. Examination of pus is also of course essential
as a guide to antimicrobial treatment, if acute sialadenitis
Histopathological Features develops.
There may be intense infiltration of the glands by Salivary gland biopsy: The changes in minor glands of
lymphocyte cells replacing all acinar structure. lower lip show close correlation with those in the major
In some cases, there may be proliferation of ductal salivary glands and provide a safe and convenient source
epithelium and myoepithelium to form epimyoepithelial of material.
Diagnostic Criteria MIKULICZ’S DISEASE OR BENIGN

I. Ocular symptoms: Dry eyes for more than 3 months, LYMPHOEPITHELIAL LESION
recurrent sensation of sand or gravel in the eyes and It was first described by Mikulicz in 1888 as symmetric 427
use of tear substitute more than 3 time per day. or bilateral, chronic, painless enlargement of lacrimal
II. Oral symptoms: Dry mouth more than 3 months, and salivary glands. It exhibits both inflammatory and
recurrent or swollen salivary gland and drinking neoplastic characteristics. Initially, Mikulicz’s disease
liquid to aid in swallowing. was confused with disease processes such as leukemia and
III. Ocular sign: Positive Schesmer test, positive rose tuberculosis. In 1909 Campbell Howard went further and
Bengal score or other ocular dye score. limited Mikulicz’s disease to merely a clinical syndrome.
IV. Histopathology in minor salivary gland focal
lymphocytic sialadenitis with focus score (number Classification
of lymphocytic foci per 4 mm2) more than 1.
• Mikulicz’s disease proper: lacrimal and salivary
V. Salivary gland involvement: unstimulated whole
gland swelling only.
salivary flow ≤ 1.5 ml in 15 minutes. Parotid
• Pseudoleukemia: Lacrimal and salivary gland
sialography showing diffuse sialectasias and
swelling plus lymphatic system involvement.
salivary scintigraphy showing delayed uptake and
• Leukemia: Lacrimal and salivary gland swelling plus
delayed excretion.
hematopoietic involvement.
VI. Autoantibodies to Ro (SS-A) or La (SS-B) antigen.
Primary Sjögren syndrome: Present of any four of six Clinical Features
items presented above as long as item IV, VI is positive.
Age and sex distribution: It more commonly occurs in
Presence of any three of the four objective criteria.
women in middle and later life.
Secondary Sjögren syndrome: Connective tissue disease
with item I and Item II plus any two from III, IV, V, VI. Location: It is manifested as a unilateral or bilateral
enlargement of parotid and/or submandibular gland.
Management Symptoms: The onset of the lesion is sometimes associated
Most of the patients are treated symptomatically. with fever, upper respiratory tract infection, oral infection,
Keratoconjunctivitis is treated by instillation of ocular tooth extraction or some local inflammatory disorders. In
lubricants, such as artificial tears coating methylcellulose some cases there is mild local discomfort, occasional pain
and xerostomia is treated by saliva substitutes. and xerostomia.
Scrupulous oral hygiene and frequent fluoride Signs: There is often diffuse, poorly outlined enlargement
application is indicated to reduce these problems. of salivary gland rather than formation of a discrete tumor
Bromhexine can be used in some cases of Sjögren’s nodule. The enlargement varies in size but generally few
syndrome. Surgery for enlargement of salivary gland centimeters in diameter. There is history of alternating
is only recommended when the enlargement is causing increases and decreases in the size of mass, from time to
discomfort to the patient. time. The duration of the tumor mass may be only a few
months or many years.
Points to Remember
Xerostomia, unpleasant taste, lack of the usual pooling Histopathological Features
of saliva, clicking’ quality of their speech, dental It is characterized by orderly lymphocytic infiltration of
caries, acute bacterial sialadenitis, snowstorm or the salivary gland tissue, destroying or replacing the acini,
cherry blossom appearance, keratoconjunctivitis sicca, with presence of islands of epithelial cells, which probably
connective tissue disorders, dryness of pharynx, larynx represent residual of gland. The epithelium consists of
and nose, infiltration of the glands by lymphocyte cells, ducts showing cellular proliferation and loss of polarity.
epimyoepithelial island, rose Bengal staining test, As the disease persist solid nest or clumps of poorly
schirmer test, sialometry, sialochemistry, immunologic, defined epithelial which termed epimyoepithelial islands is
ocular lubricants, bromhexine, scrupulous oral hygiene. also present. Such islands arise as result of proliferation of
ductal cells and peripheral myoepithelial cells. In advancedNerve involvement: The most common nerve involved is
cases there is deposition of eosinophllic hyaline material in
facial nerve. There is unilateral or bilateral seventh nerve
the epithelial islands. paralysis. The neurological signs may precede, follow or
428 appear simultaneously with parotid swelling. Trigeminal
Management paresthesia, eyelid ptosis, polyneuritis, intercostal neural-
Surgical excision and radiation can be given. Prognosis is gia and spinal nerve impairment accompanied by weakness
good. and muscle atrophy have been reported.
Points to Remember
It will reveal a characteristic sarcoid nodule.
Unilateral or bilateral enlargement of parotid, sub-
mandibular gland, fever, upper respiratory tract Management
infection, oral infection, diffuse, poorly, enlargement of
It is largely symptomatic as it may undergo spontaneous
salivary gland, lymphocytic infiltration, cellular prolif-
remission. Corticosteroid can be used in cases of acute
eration and loss of polarity, ‘epimyoepithelial islands’.
exacerbation.
UVEOPAROTID FEVER Points to Remember

Heerfordt’s syndrome, prodormal symptoms, subman-
It is a form of sarcoidosis and it is also called Heerfordt’s dibular, sublingual and lacrimal gland swelling, sarcodi-
syndrome. al lesion, an inflammation of the uveal tract, facial nerve
involvement causing paralysis, sarcoid nodule.
Triad
• Uveitis: Inflammation of uveal tract of the eye.
• Parotid swelling: Firm, painless and bilateral
TUMORS OF SALIVARY GLANDS
enlargement of parotid gland. Salivary gland tumors are considered to have a special
• Facial palsy interest by pathologist because of its challenging variation
in the appearance. No other tissue in the body produces the
Etiology diversity in histopathological appearance as salivary gland
tumor does.
Tuberculosis was earlier thought to be the causative agent.
Salivary tumors are said to be relatively uncommon
Hereditary factor, autoimmune mechanisms are also lesions. The incidence is 3 to 5% of the tumors. It is
considered etiological factors for it. important to note that neoplasms arise not only in major
salivary glands, but also in minor salivary glands.
Clinical Features
In major salivary glands the most common site is parotid
It usually occurs in 3rd and 4th decades of life. (64–80%) whereas submandibular gland and sublingual
Prodromal symptoms lasting from a few days to glands show incidence of 8 to 11% and 1% respectively.
several weeks are the usual initial signs of the disease and It is important to note that neoplasms arise not only in
complains include fever, malaise, weakness, nausea and major salivary glands, but also in minor salivary glands.
night sweat. It appears as a bilateral, firm, painless parotid Neoplasms in minor salivary glands are 9 to 23% of all
swelling. tumors, palate being the most frequent site (42–54%).
Signs: Submandibular, sublingual and lacrimal gland
swelling may develop independently or during the course HISTOGENESIS
of the parotid swelling. The parotid swelling lasts from The basal cells of the excretory duct and intercalated duct
several months to several years. Sarcodial lesion may also play a major role in the development of salivary gland.
be found in the oral cavity. Basal cell of excretory duct forms columnar and squamous
Uveitis: It is an inflammation of the uveal tract, is a cell of the duct. The basal cells of intercalated duct gives
feature of this disease. Although ocular symptoms are rise to acinar cell, striated duct cells and myoepithelial cells.
usually bilateral, they become more apparent before the These two basal cells are said to be reserve cells or
appearances of parotid swelling. progenitor cells in salivary gland development (Fig. 17.17).
Ductal displacement: The ducts adjacent to the tumor

are usually stretched around this is known as ball in hand
appearance.
Retention of contrast media in the displaced ducts 429
during the emptying phase is seen.
Palpation of Tumor
Firmness of the tumor: Firmness results from dense
aggregates of nests and cords of closely packed tumor.
Fibrous tissue and hyaline area as well as cartilage like and
bone like tissue. Softness results from fluid produce and
Figure 17.17 Schematic diagram of salivary tree showing retention phenomenon.
acinus (A), intercalated ducts (ID-pink), striated ducts (SD-green),
terminal secretory ducts (TSD-yellow) and myoepithelial cells (M) Firm Tumor
• Pleomorphic adenoma
THEORIES OF SALIVARY GLAND • Adenoid cystic carcinoma
TUMOR HISTOGENESIS • Mucoepidermoid tumor of high grade
• Carcinoma in pleomorphic adenoma
There are two hypothesis suggested in the development of • Acinic cell carcinoma
salivary gland neoplasms. • Oncocytoma
Well differentiated cells of the salivary gland unit Soft Tumor
form the neoplasm of their differentiated counterparts. • Well differentiated mucoepidermoid tumor
According to this; • Papillary cyst adenoma
Acinar cell → Acinar cell carcinorma • Mucus producing adenocarcinoma
Striated duct cells → Oncocytic tumors • Warthin’s tumor
Excretory duct cells → Squamous cell carcinoma
and mucoepidermoid
carcinoma CLINICAL STAGING OF SALIVARY
Intercalated duct → Adenocarcinomas GLAND TUMORS
Bicellular theory: These theories suggest that undiffer-
By Spiro
entiated ‘reserve’ cell, i.e. basal cells of the excretory
and intercalated duct are responsible for formation of Staging of salivary gland neoplasms appear to have been
the majority of neoplasms. According to this theory, initiated by Spiro. It is as follows:
dedifferentiation of already specialized cells such as acinar • T1-0 to 3 cm and solitary and freely mobile and
and striated duct cells duct not required for development of CRVII intact
salivary gland neoplasms. • T2-3.1 to 6 cm and solitary and freely mobile or skin
fixation and CRVII intact
GENERAL FEATURES OF SALIVARY • T1- 6 cm or multiple nodules or ulceration or deep
fixation or CRVII dysfunction
GLAND TUMORS • Patient with T1 and T2 lesion are placed into stage I
Sialographic Appearance of Intrinsic and II respectively
Tumor of Salivary Glands • Any patient with clinical evidence of metastasizes to
lymph nodes or with T3 lesion is considered to be in
An area of under filling within the gland, due to ductal
stage III
compression by the tumor is seen.
The nomenclature of this tumor as mixed tumor and

By American Joint Committee
pleomorphic adenoma describe the histologic appearance
Primary Tumor and not the clinical behavior and manifestation. The
430 • Tx: Tumor that cannot be assessed by the rules. ability of the tumor cells to differentiate into epithelial and
• T0: No evidence of primary tumor mesenchymal cells leads to formation of mixed picture
• T1: Tumor 2 cm or less in diameter, without showing fibrous, hyalinised, chondroid, myxoid areas
significant local extension along with areas of epithelial cells with metaplasia. Thus
• T2: Tumor 2-4 cm in diameter without significant the term mixed tumor means the varied histopathological
local extension appearance due to differentiation of the tumor cells and it
• T3: Tumor more than 4 cm but not more than 6 cm in does not signify teratomatous origin.
diameter without significant local extension. Other names suggested for this tumor are iceberg tumor,
• T4a: Tumor over 6 cm in diameter without significant endothelioma, branchioma, or enchondroma, enclavoma.
local extension.
• T4b: Tumor of any size with significant local exten- Histogenesis and Pathogenesis
sion. The suggested theories of origin are as:
Nodal Involvement (N) Pleomorphic adenomas arise from myoepithelial cell
and intercalated duct reserve cells. There are evidences
• Nx: Regional lymph node cannot be assessed
for ulstrastructural similarities between the neoplastic cells
• N0: No regional lymph node metastasis
and the suggested cells of origin. The proposed There is
• N1: Clinical or histologically positive regional lymph
presence of myoepithelial cells and reserve cells, arranged
nodes.
in intercalated duct. Also the morphologic diversity of
Distant Metastasis (M) the tumor showing mixed tumor appearance with areas
• Mx: Distant metastasis cannot be assessed of fibrous mucinous chondroid and osteoid areas seen are
• M0: No distant metastasis suggested due to myoepithelial cells. The appearance of
• M1: Distant metastasis ductal pattern and areas of squamous cells are suggested to
Stage grouping is performed as follows: be from intercalated duct reserve cells.
• Stage I: T1N0M0 or T2N0M0 Batsakis theory: The reserve cells can differentiate into
• Stage II: T3N0M0 ductal and myoepithelial cells and undergo subsequent
• Stage III: T1 or T2, N1 M0, or T4a or T4b N0 M0 mesenchymal metaplasia. Thus the intercalated duct reserve
• Stage IV: T3 N1 M0, T4a or T4b N1 M0, any T any N M1 cell is the histogenetic precursor of pleomorphic adenoma.
Dardick’s theory: This theory suggests that any
BENIGN TUMORS neoplastically altered epithelial cells can differentiate
multidirectionally and the origin cannot be from intercalated
and myoepithelial cells.
PLEOMORPHIC ADENOMA
Pleomorphic adenoma is most common of the entire Clinical Features
salivary gland tumor. The term pleomorphic adenoma was Sex and age: Women to men ratio is 6:4. It is common
suggested by Willis characterizing the unusual histological in 4th to 6th decades but also seen in young adults and
pattern of the lesion (pleomorphic or mixed appearance). children.
The morphologically diverse appearance of tumor is its Site: Parotid 90 percent and intraoral palatal gland on lip.
pathognomic feature. Lower pole of superficial lobe of parotid gland is most
This tumor is derived from a mixture of ductal and commonly affected site. (Fig. 14.2) upper lip and buccal
myoepithelial elements. It is said that the pleomorphic mucosa are very uncommonly affected.
adenoma is the only tumor which shows remarkable
diversity from one tumor to the next, as well as in different Appearance: Palatal tumors present as smooth surfaced
areas of the same tumor. dome shaped lesions or tumor mass. Ulceration may be
431
Figure 17.18 Pleomorphic adenoma of parotid gland Figure 17.19 Pleomorphic adenoma gross specimen showing
showing huge swelling firm to hard mass exhibiting a cut potato appearance
seen if traumatized. Palatal tumors are immobile due to scribed, encapsulated and sometimes shows infiltration of
their location and microanatomy but lip and buccal mucosa capsule by tumor cells.
tumors are mobile (Fig. 17.18). Palatal pleomorphic adenoma usually shows infiltra-
tion of capsule beneath the epithelial surface. There are
Symptoms: Small, painless, quiescent nodule which
considerable variations with arrangement of epithelial and
slowly begins to increase in size, sometimes intermittently.
stromal components between different areas of tumor.
The growth is a slow growing firm mass and the patient will
The tumor can be described on the basis of the
be usually aware of the lesion for months and years before
following 3 components: an epithelial cell component, a
seeking professional help in diagnosis and treatment. If
myoepithelial cell component and a stromal (mesenchymal)
neglected the tumor can grow to very huge size.
component.
Shape: The tumor tends to be round or oval when it is
Epithelial component: The epithelial component consists
small, as it grows bigger it becomes lobulated.
of epithelial duct like cells, polygonal cells, cuboidal cells,
Size: It may increase to cricket ball size or even more, spindle cells arranged in different patterns. The epithelial
weighing in pounds and in intraoral cases, not more than 1 cells may be arranged in sheets, clumps, islands or interlace
to 2 cm in diameter. strands. Cuboidal cells shows duct like arrangement. These
Surface: Its surface is smooth. Sometime it is bosselated ducts like spaces may contain eosinophilic coagulum and
and is occasionally crossed by deep furrows. mucoid material. Epithelial cells resembling squamous
cells have distinct intercellular bridges. Cystic spaces are
Consistency: It is firm and rubbery to feel. Sometimes also uncommonly seen.
cystic degeneration may be seen.
Myoepithelial component: Few stellate cells or spindle
Histopathological Features cells called myoepithelial cells are also seen with variable
morphology. These cells have rounded eccentric nucleus
Macroscopic (Fig. 17.19): Encapsulated lesion with firm
and eosinophilic hyalinized cytoplasm resembling
rubbery consistency. Minor salivary gland tumor may not
plasma cells. These cells are called plasmacytoid cells.
show a well defined capsule. The gross cut surface of the
Hyaline cells are also seen with dense eosinophilic
firm mass shows a cut potato surface sometimes associated
cytoplasm.
with small cystic areas with mucoid, hemorrhagic areas.
Pleomorphic adenoma exhibits wide cytologic and ar- Stromal component: The stromal changes are highly
chitectural diversity. The tumor is typically well circum- variable and are believed to be produced by myoepithelial
cells. Loose myxoid material may be seen with wide

intercellular spaces and faint staining characteristics, foci
of hyalinized C.T, areas of chondroid or cartilage like
432 material.
Osteoid or bone like material may be seen in between
tumor cells (Figs 17.20 to 17.24).
When the tumor is highly cellular or having
predominantly epithelial component then it is often referred
to as cellular adenoma.
If myoepithelial cell component predominant, it is often
referred to as myoepithelioma.
The background of the tumor is made by stroma
Figure 17.22 Pleomorphic adenoma showing ductal spaces

(D) filled with eosinophilic material. Chondroid areas (C) are
seen
Figure 17.20 Pleomorphic adenoma showing presence of

duct like spaces (D), eosinophilic material (E), and mucous
acini (M) peripherally capsule (C) is seen
Figure 17.23 Pleomorphic adenoma showing chondroid

areas (C) and island of squamous metaplasia (SM)
containing areas with mucoid deposition giving

myxomatous appearance.
Sometimes they show plasma cell like appearance
called plasmacytoid myoepithelial cells.
Types (Histological) of Pleomorphic Adenoma (Foot

and Frazel 1954)
• Principally myxoid
• Myxoid–cellular components in equal proportions
Figure 17.21 Pleomorphic adenoma showing chondroid area
• Predominantly cellular
(Courtesy: Dr Sangamesh Halawar, Reader, Department of Oral
Pathology, VPDC and H, Kavalapur, Sangli, Maharashtra, India)
• Extremely cellular
BASAL CELL ADENOMA

It is a types of monomorphic adenoma. It was first
described by Kleinsasser and Klein in 1967. It is a 433
neoplasm of uniform distribution of basaloid epithelial
cells. Characteristic features of monomorphic adenoma are
the composition of the tumor by isomorphic/monomorphic
cells distributed in various pattern of arrangement.
Histogenesis
The isocellular cells resemble the reserve cells of inter-
calated duct. So the histogenetic source according to
Batsakis is suggested to be from these basal cells.
Figure 17.24 Pleomorphic adenoma myxoid area (Courtesy: Dr Clinical Features

VPDC and H, Kavalapur, Sangli, Maharashtra, India)
Age and sex: It is more common in females with 2:1
predilection. Older age group, usually over 60 years of age
Management are affected.
Surgical excision: Tumor and the involved lobe of gland Site: It occurs primarily in major salivary glands
is removed. particularly in the parotid gland and intraorally, upper lip.
Recurrence rate is 5 to 30 percent due to hypocellularity,
Signs and symptoms: Size is less than 3 cm in diameter.
incomplete resection and encapsulation.
The tumor shows painless slow growth and presents as
Signs of Malignant Transformation include freely movable mass like pleomorphic adenoma.
• Accelerated growth rate Membranous basal cell adenoma: This occur in
• Tumor irregularity on palpations association with skin appendage tumors like dermal
• Necrosis and painful ulceration cylindromas and trichoepitheliomas.
• Facial nerve involvement.
Points to Remember It is encapsulated well circumscribed tumor. The
Adenomas arise from myoepithelial cell and intercalated isomorphic cells are arranged into different patterns further
duct reserve cells, Batsakis Theory, Dardick’s theory, classifying tumor into four types.
parotid gland, smooth surfaced dome shaped lesions, Solid variant: The tumor cells are arranged into nests
painless, quiescent nodule, bosselated surface, firm sheets islands with minimal connective tissue stroma. The
consistency, firm rubbery consistency of excised section, cells have a basaloid appearance and resemble basal cell
infiltration of capsule beneath the epithelial surface, carcinoma. Confirmative diagnosis is made on the bases of
epithelial component consist of epithelial duct like cells, clinical behavior and degree of dysplasia (Figs 17.25 and
polygonal cells, cuboidal cells, spindle cells arranged in 17.26).
different patterns, myoepithelial component consist of
myoepithelial cells which have rounded eccentric nucleus Trabecular types: Epithelial cells form narrow cord like
and eosinophilic, stromal component shows loose strands in a background of loose fibrillar stroma.
myxoid material, cellular adenoma, Myoepithelioma, Tubular variant: The epithelial cells form small round
myxomatous appearance, plasmacytoid myoepithelial duct like structures which may be in combination with
cells, surgical excision. trabecular pattern.
Management
It is treated by surgical excision and recurrence is seldom
434 seen.
Points to Remember
Reserve cells of intercalated duct, parotid gland, painless
slow growth freely movable mass, membranous basal
cell adenoma, isomorphic cells, solid variant arranged
into nests sheets islands with minimal connective tissue
stroma, trabecular types form narrow cord like strands,
tubular Variant cells form small round duct like structures,
membranous Variant arranged in zig saw puzzle pattern.
Figure 17.25 Basal cell adenoma showing uniform CANALICULAR ADENOMA

appearing basaloid cells (B) in supporting stroma (S)
It is types of monomorphic adenoma. The name itself
implies the histologic appearance of the tumor. The cells
are arranged in canalicular pattern or branching cord like
pattern, hence the name. It is an uncommon neoplasm of
columnar epithelial cells.
Clinical Features
Age and sex: It is common in patients in 3rd to 6th decade.
There is predilection in females.
Site: It originates primarily in the intraoral accessory
glands. It occurs in upper lips followed by palate, buccal
mucosa and lower lip.
Symptoms: It presents as a slowly growing, well
circumscribed, firm nodule, which is particularly on the lip
Figure 17.26 Basal cell adenoma showing cells arranged in and is not fixed. It may be moved through the tissues for
nests sheets Island (Courtesy: Dr Sangamesh Halawar, Reader, some distance.
Department of Oral Pathology, VPDC and H, Kavalapur,
Sangli, Maharashtra, India) Histopathological Features
It is composed of long strands or cords of epithelial cells,
Membranous variant: Multiple large islands of tumor almost arranged in a double row and usually showing a
cells are arranged in zig saw puzzle pattern. These islands party wall (Figs 14.10 and 14.11).
are surrounded by hyaline material resembling a double Cystic spaces of varying sizes are enclosed by these
basement membrane. cords. The cystic spaces are usually filled by an eosinophilic
coagulum. The supporting stroma is loose and fibrillar with
Histological Types of Basal Cell Adenoma
delicate vascularity (Figs 17.27 and 17.28).
• Solid variant
• Trabecular types Management
• Tubular variant It can be treated by simple enucleation and surgical
• Membranous variant excision. Recurrence rate is rare.
Development
Heterotopic salivary rest theory: Tumor arises from
salivary gland tissue entrapped with para-parotid or intra- 435
parotid lymph nodes during embryogenesis.
Another theory suggests that there is neoplastic prolif-
eration of parotid ductal epithelium and concomitant sec-
ondary proliferation of lymphoid tissue.
Hypersensitivity theory: Allegra suggested that it is
most likely a delayed hypersensitivity response. The
lymphocytes being an immune reaction to the salivary
ducts which undergo oncocytic change.
Clinical Features
Figure 17.27 Canalicular adenoma showing anastomosing Age and sex: It is common in men (male to female ratio is
narrow cords of epithelium (E) in loose stroma (S) 5:1). It is common in 6th decade.
Site: The tumor occurs almost exclusively in the parotid
gland. It always occurs in the lower portion of the parotid
gland. The tumor is generally superficial. lying just beneath
the parotid capsule or protruding through it.
Symptoms: The usual complaint is painless slow growing
tumor over the angle of jaw. Involvement may be bilateral
or may be multifocal.
Sign: The tumor does not attain a large size and the
usual size is 1-3 cm in diameter. It is spherical in shape.
Surface is smooth and it is well circumscribed, movable. It
classically feels doughy and compressible on palpation. It
is firm on palpation and is clinically indistinguishable from
other benign lesions of parotid gland.
Figure 17.28 Canalicular adenoma showing double row of
cells of showing party wall appearance. Stroma shows loose The tumor is made up of epithelial and lymphoid tissue.
stroma. Epithelial chords (EC), Stroma (S) It is an adenoma exhibiting cyst formation, with papillary
projections into the cystic spaces and lymphoid matrix
forming the connective tissue core of the papillae (Figs
WARTHIN’S TUMOR 17.29 to 17.33).
It is also called adenolymphoma and papillary cystadenoma The epithelial cells, covering the papillary projection,
lymphomatosum and brachial cyst of parotid. This tumor are columnar or cuboidal cells usually arranged in two
was first described by Hildebrad in 1895, later by Albrecht rows.
and Arzt in 1910 reported few cases. The name papillary Outer cell layer is made up pseudociliated columnar
cystadenoma lymphomatosum was given by Warthin in cells with eosinophilic granular cytoplasm. Nucleus is
1929 in USA. Now it is recognized as Warthin’s tumor. polarized away from the basement membrane. The inner
The tumor shows papillary projection of the epithelium layer is made up of low cuboidal cells. Basement membrane
into the cystic cavity formed in the existing adenoma. Thus distinctly separates epithelium from the lymphoid tissue.
it is a cyst in an adenoma showing papillary folds of the There is frequently an eosinophilic coagulum present
epithelial lining. within the cystic spaces which appears as a chocolate
436
Figure 17.29 Warthin’s tumor showing papillary projections Figure 17.31 Warthin’s tumor showing cystic space (CS) and
with lymphoid cores cystic cavity seen in background double row of cells. Outer cells (PC) are tall columnar with
nucleus polarized away from basement membrane. Inner cells
(IC) are cuboidal. These cells enclose lymphoid stroma (LS)
Figure 17.30 Warthin’s tumor showing cystic space (CS)

containing papillary projection (PP) lined by epithelium (E).
Lymphoid stroma (LS) containing germinal center (GC) is Figure 17.32 Papillary projections in Warthin’s tumor are
present beneath the epithelium lined by outer columnar cells and inner cuboidal cells
colored fluid in the gross specimen. The lymphoid tissue

Points to Remember
beneath the epithelium shows germinal center formation.
The abundant lymphoid component may represent the Adenolymphoma, heterotopic salivary rest theory,
normal lymphoid tissue of the lymph node, within which neoplastic proliferation of parotid ductal epithelium,
the tumor developed or it may actually represent a reactive hypersensitivity theory, parotid gland, painless slow
cellular infiltrate which involves both, humoral and cell growing tumor, spherical in shape, surface is smooth,
mediated mechanism. columnar or cuboidal cells, pseudociliated columnar
cells with eosinophilic granular cytoplasm, eosinophl-
Management lic coagulum, reactive cellular infiltrate, superficial
Superficial parotidectomy and it seldom recurs after parotidectomy.
removal.
Size: The tumor usually measures 3 to 5 cm in diameter

and appears as a discrete encapsulated painless mass which
is sometimes nodular. Pain is generally absent.
Oncocytosis or nodular oncocytic hyperplasia: an 437
interesting condition called oncocytosis of parotid gland
has been described which is characterized by nodules of
oncocytes involving the entire gland or a large portion.
In this there is transformation of ductal and acinar cells
into oncocytes. In some cases entire gland is replaced by
oncocytes (diffuse hyperplastic Oncocytosis).
Oncocytoma is a well circumscribed tumor. It is composed
of oncocytes, which are large cells with eosinophilic
Figure 17.33 Warthin’s tumor showing cystic space (CS) and granular cytoplasm and distinct cell membrane (Fig. 17.34).
double row of cells. Outer cells (PC) are tall columnar with The cytoplasmic granularity is because of exclusion of
nucleus polarized away from basement membrane. Inner cells other cell organelles and their replacement by abundant
(IC) are cuboidal. These cells enclose lymphoid stroma (LS) uniform mitochondria which makes the cell to appear
(Low magnification) swollen.
Oncocytes are arranged in solid sheets and sometimes
tend to be arranged in narrow rows, cords and may
ONCOCYTOMA demonstrate alveolar or lobular pattern.
These cells exhibits few mitotic figures are closely
Oncocytoma is a tumor of oncocytes. Oncocytes are large packed and there is little supportive stroma. Lymphoid
epithelial cells that contain brightly eosinophilic granular tissue is frequently present.
cytoplasm. This is due to abundant mitochondria packing
in the cell cytoplasm. It is also called oxyphilic adenoma, Management
acidophilic adenoma. It is an uncommon tumor composing
Surgical excision should be done. Tumor does not tend to
less than 1 percent of salivary neoplasms. These cells are
recur. Malignant transformation is very rare.
predominately seen in duct lining of glands in elderly
persons.
Pathogenesis
As the tumor is exclusively seen in older age group it is
suggested that the oncocyte are the result of degeneration
of the salivary gland parenchyma.
Another school of thought is that it is a metaplastic
process seen in hyperplasia of salivary parenchyma. This
further could proliferate into neoplastic state.
Some suggest that it is not neoplasm but merely a
nodular hyperplasia.
Clinical Features
Age and sex: It is more common in women than in men
and occurs almost exclusively in older persons.
Figure 17.34 Oncocytoma with oncocytes showing dense
Site: It usually occurs in the parotid gland. eosinophilic cytoplasm
Points to Remember
Oxyphilic adenoma, acidophilic adenoma, degenera-
438 tion of the salivary gland parenchyma, hyperplasia of
salivary parenchyma, oncocytosis or nodular oncocytic
hyperplasia, diffuse hyperplastic oncocytosis, discrete
encapsulated painless mass.
Oncocytes are large cells with eosinophilic granular
cytoplasm and distinct cell membrane abundant uniform
mitochondria, arranged in solid sheets, few mitotic
figures.
MYOEPITHELIOMA
It is an uncommon salivary gland tumor. Sheldon in 1943
Figure 17.35 Myoepithelioma showing capsule (C) and myoe-
seperated this as a different entity from pleomorphic
pithelial cells (EC) producing hyaline eosinophilic material (E)
adenoma as these tumors were composed predominantly of
basket cells or myoepithelial cells.
Clinical Features
Age and sex: It occurs in adults and has equal sex
distribution.
Site: Parotid gland is most commonly involved and the
palate is the most frequent intraoral site of occurrence.
The clinical features are same as pleomorphic adenoma.
The tumor is composed of spindle shaped myoepithelial
or plasmacytoid or combination of the two cell types (Figs
14.35 and 14.37).
These cells may be set in myxomatous background,
which vary from scanty to copious. Figure 17.36 Myoepithelioma showing plasmacytoid cells (P)
and material (E)
Management
Surgical excision should be carried out. Clinical Features
Intraductal papilloma: It is present as an exophytic lesion
Points to Remember
with a papillary surface and is pedunculated. It is usually
Parotid gland, spindle shaped myoepithelial or reddish in color and present on the buccal mucosa or palate
plasmacytoid, myxomatous background.
Inverted ductal papilloma: It appears as a submucosal
nodule beneath normal appearing overlying surface mucosa.
DUCTAL PAPILLOMAS It is commonly seen on lower lip. It is asymptomatic and
sometimes may present with a surface opening or pit or
It is salivary gland tumor which is characterized by
indentation in surface.
papillomatous pattern. Many times squamous papilloma
will arise at the site where minor salivary gland duct merge Sialadenoma papilliferum: It is a salivary gland analogue
with the surface epithelium. So this type of squamous of the syringo-cystadenoma paipilliferum of skin. The
papilloma will contain scattered mucous cells within the lesion occurs in adults as an exophytic papillary lesion of
exophytic growth. This type is called ductal papilloma. the hard palate.
439
Figure 17.37 Myoepithelioma showing spindle cell Figure 17.39 Inverted ductal papilloma showing papillary
proliferation projections into the stroma
consist exophytic papillary projection covered by stratified

squamous epithelium. These ductal cells have oncocytic
appearance. Inflammatory infiltrate of plasma cells,
lymphocytes and neutrophil is present.
Inverted ductal papilloma: There is proliferation of
squamoid epithelium with multiple thick bulbous papillary
projections that fill the ductal lumen. This epithelium
communicated with surface through small pore like
opening (Fig. 17.39).
Management
It is treated by excision, including the base and it does to
recur after completely removed.
Figure 17.38 Ductal papilloma showing papillary Points to Remember

proliferations from ductal lining
• I ntraductal papilloma: Exophytic lesion with a pap-
illary surface non keratinized epithelium of the co-
Histopathological Features lumnar type, single or double row cuboidal or colum-
Intraductal papilloma: It consists of non keratinized nar epithelium.
epithelium of the columnar type, supported by cores of • Inverted ductal papilloma: Submucosal nodule, low-
vascular fibrous connective tissue. It is lined by single er lip, proliferation of squamoid epithelium, bulbous
or double row cuboidal or columnar epithelium. There papillary projections.
is multiple arborizing papillary projection into the cystic • Sialadenoma papilliferum: It is a salivary gland ana-
lumen (Fig. 17.38). logue of the syringo-cystadenoma paipilliferum skin,
luminal layer of columnar cells, exophytic papillary
Sialadenoma papilliferum: It consists of luminal layer projection, plasma cells, lymphocytes.
of columnar cells resting on a cuboidal basal layer. It
Onset: It appears as a slowly enlarging painless mass,

MALIGNANT SALIVARY GLAND which simulates pleomorphic adenoma.
TUMORS It is usually not completely encapsulated and often
440 contains cysts which may be filled with viscid mucoid
MUCOEPIDERMOID CARCINOMA material.
The tumor of low grade malignancy usually appears
The term mucoepidermoid carcinoma (MEC) was as a slowly enlarging, painless mass, which simulates
introduced in 1945 by Stewart, Foote and Becker. MEC pleomorphic adenoma. It seldom exceeds 5 cm in diameter.
comprises between 2.8 percent and 15.5 percent of all The tumor of high grade malignancy grows rapidly and
salivary gland tumors, between 12 percent and 29 percent of does produce pain as an early symptom. Ulceration is seen
malignant salivary gland tumors, and between 6.5 percent in cases which have an aggressive clinical course (Figs
and 41 percent of minor salivary gland tumors, representing 17.40 and 17.41).
the most common type of malignant minor salivary gland
tumor in most series. About half the cases occur in the
major salivary glands, >80 percent of these occur in the
parotid, 8 to 13 percent occur in the submandibular gland,
and 2 to 4 percent involve the sublingual gland.
In the minor salivary glands MEC most commonly
arises on the palate, but a significant number may also be
found in the retromolar area, floor of the mouth, buccal
mucosa, lip, and tongue.
Rarely, MEC can arise as primary jaw or heterotopic
intranodal tumors, or as laryngeal, lacrimal, nasal, paranasal,
tracheal, or pulmonary tumors (Figs 14.18 and 14.19).
It consists, of both, mucus secreting as well as
epidermoid type of cells as its name suggests.
The biologic behavior of the mucoepidermoid carcinoma
is graded on the basis of clinical and histological features.
Figure 17.40 Intraoral ulceration seen on left side due high
Histogenesis
grade mucoepidermoid tumor
It is an accepted hypothesis that the tumor develops from
an excretory duct reserve cell (Dardick’s theory).
Etiology
The most commonly implicated etiologic factor is
radiation; as according to a study many as 44 percent of
patients with a history of a radiation-associated salivary
tumor developed MEC (Kaste et al. 1994). Latency period
in this group is reported to range from 7 to 32 years.

Age and sex: It is most frequently seen in the 35 to 65
year age group but may be found at any age. It has a slight
predilection for women.
Site: About 60 percent occur in parotid gland and 30 per-
cent in the minor salivary glands, especially those of the
palate. Other common intraoral sites are buccal mucosa, Figure 17.41 Mucoepidermoid carcinoma of left parotid
tongue and retromolar area. gland showing ulceration of skin
It tends to infiltrate the surrounding tissues and in

high percentage of cases it metastasizes to the regional
lymph nodes. Distant metastases to lungs, bones, brain and
subcutaneous tissue are common. 441
Much more various findings such as lacrimation,
trismus, nasal discharge, and blood tinged saliva and facial
nerve paralysis are seen in high grade malignant lesions.
Intraoral lesions with bony erosion may show numbness
of teeth.
Radiological features: CT scan is more useful in deline-
ated margin of the lesion (Fig. 17.42).
(Figs 17.43 to 17.51)
The term mucoepidermoid itself signifies that it is Figure 17.43 Mucoepidermoid carcinoma showing presence
of clear cells (CC)
composed of mucus-secreting cells and epidermoid type
cells. Third type of cells commonly seen is intermediate
cells.
Mucous cells are cells with foamy cytoplasm filled with
mucin.
Epidermoid cells are large polygonal cells with
prominent nuclei, distinct cell membranes, intercellular
bridges, abundant eosinophilic cytoplasm. Epidermoid
cells are occasionally associated with keratin production
including pearl formation. These tumors show great
variability in the composition of cell types in a given tumor.
Cellular pleomorphism and atypia may be found and
range from minimal to severe.
The intermediate cells are small, round basaloid
cells. These cells are seldom the dominant cell, although
Figure 17.44 Mucoepidermoid carcinoma low power
it appears that it may undergo transformation into either

mucus or epidermoid cells.
Sometimes cluster of clear cells, often in abundance,
may be present which are generally containing mucinous
material and glycogen. If the clear cell population
predominate then it is called clear cell variant of MEC.
Occasional MECs will be associated with a prominent
oncocytic component and are referred to as the oncocytic
variant.
Ductal proliferation adjacent to the tumor is common.
These tumors show sheets or nests of epidermoid cells
Figure 17.42 CT scan showing extent of palatal and similar nests of mucous cells, usually arranged in a
mucoepidermoid carcinoma palate glandular pattern and showing microcyst formation. These
442
Figure 17.45 Low grade mucoepidermoid carcinoma showing Figure 17.47 High grade mucoepidermoid carcinoma
presence of mucous cells (MC) and epidermoid cells (EC) in showing predominate epidermoid cell (EC) only few mucous
equal proportions. Large mucous containing cystic space (CS) cells (MC) and few cystic spaces (CS)
are seen
Figure 17.46 Mucoepidermoid carcinoma showing large Figure 17.48 Cystic areas in MEC
cystic areas containing mucin
cysts may rupture liberating mucus, which may pool in the cells are seen in the tumor mass. Few epithelial nests are
connective tissue and evoke inflammatory reaction. In such seen.
cases lymphocytes are seen. Intermediate grade: The cystic transformation is less
Tumor is graded on the bases of proportion of epidermoid as compared to low grade. There is predominance of
and mucous cells, degree of cystic transformation of the intermediate cells although areas of mucous and epidermoid
tumor, and degree of cellular atypia. It graded into three cells are seen. Cellular atypia is minimal.
types:
High grade: The predominant cellularity is of epidermoid
Low grade: It predominantly shows mucous cells and cells with varying dysplastic features. These cells show
abundant extracellular mucin. Several cystic areas lined by considerable cellular atypia.
443
Figure 17.49 Mucoepidermoid carcinoma showing Figure 17.51 Clear cells showing vacuolated cytoplasm in
epidermoid cells and mucous cells mucoepidermoid carcinoma (high power)
Grading Criteria
• The degree of tumor invasion,
• Anaplasia pattern of invasion,
• Degree of maturation of the-various cellular compo-
nents,
• Mitotic rates,
• Presence or absence of necrosis,
• Neural or vascular invasion,
• Proportion of tumor composed of cystic spaces
relative to solid growth.
Management
Surgical excision followed by radiotherapy is recommend-
Figure 17.50 Intermediate cells in mucoepidermoid ed for intermediate grade tumors and high grade tumors.
carcinoma Low grade tumors can be managed by surgery alone.
Points to Remember
Grading (Brandwein et al) Parotid gland, minor salivary glands, slowly enlarging
• Grade I-Prominent goblet cell component, cyst painless mass, low grade malignancy, high grade
formation intermediate cells may be prominent malignancy grows rapidly, ulceration, distant
circumscribed growth pattern metastases to lungs, bones, brain, lacrimation, trismus,
• Grade II-Intermediate cells predominate over nasal discharge, blood tinged saliva, facial nerve
mucinous cells mostly solid tumor squamous cell paralysis, numbness of teeth, mucous cells, epidermoid
may be seen cells, cellular pleomorphism, intermediate cells are
• Grade III- Squamous cells predominate intermediate small, round basaloid cells, cluster of clear cells, clear
and mucinous cells must also be present, mostly cell variant of MEC, oncocytic variant, microcyst
solid variant. formation.
Clinical Features
Sex: It is more common in females than males.
444 Site: It occurs in mandible in premolar-molar area. It does
not extend anteriorly beyond the premolar region.
Symptoms: Patient complain of painless swelling. There
may be paresthesia of inferior alveolar nerve.
Sign: Swelling may cause facial asymmetry. Tenderness is
present. Regional lymph nodes are enlarged.
Radiological features: It present as a unilocular or
multilocular expansile mass. Margin often well defined,
well corticated and often crenated or undulating in nature.
A It has got soap bubble or honey comb internal structure.
It is similar to that of intraosseous mucoepidermoid
carcinoma. Most of the lesions are low grade tumor.
Management
It is treated surgically with en block resection. Neck
dissection and postoperative radiation therapy may be
required for control of nodal disease.
Points to Remember
Intraosseous mucoepidermoid carcinoma, entrapment
of retromolar mucous glands, premolar-molar area in
B mandible, painless swelling, facial asymmetry, soap
Figures 17.51A and B Acinic cell carcinoma showing clear bubble or honey comb, multilocular expansile mass,
cells arranged acinar pattern neck dissection, postoperative radiation therapy.
CENTRAL MUCOEPIDERMOID ACINIC CELL ADENOCARCINOMA

CARCINOMA It is also called acinic cell or serous cell adenoma. This
It is also called an intraosseous mucoepidermoid is low grade malignancy. It accounts for approximately
carcinoma. It is an extremely rare tumor originating in the 1 percent of all salivary gland tumors.
bone and is believed to be derive from salivary gland tissue
Clinical Features
entrapment in developing bone.
Age and sex: It occurs in middle age and twice common
Origin in women.
It may originate from entrapment of retromolar mucous Site: It arises exclusively in the superficial lobe and tail of
glands within the mandible, which subsequently undergo the parotid gland. The most common intraoral sites are the
neoplastic transformation. buccal mucosa and lip.
It may also form from developmentally included
Symptoms: It is painless and grows slowly.
embryonic remnants of the sub-maxillary gland within
the mandible and neoplastic transformation of the mucous Sign: Exact delineation of the lesion is difficult and
secreting cells commonly found in the epithelial lining of attachment to the overlying skin and muscle may occur.
dentigerous cyst. Some of these lesions run a rapid course with hematogenous
and lymphatic metastases, while others are more slowly Symptoms: The most common initial symptom is presence
progressive. Locally invasive growth may be encountered of mass followed by local pain, facial nerve paralysis in
in some lesions. It resembles to pleomorphic adenoma and case of parotid tumor and tenderness.
is encapsulated and lobulated in appearance. 445
Sign: Some of the lesions exhibit surface ulceration. Other
Histopathological Features findings include nasal obstruction, proptosis, sinusitis, ear
infection, epistaxis, signs of cranial nerve involvement and
It is surrounded by a thin capsule, may be composed of visual disturbances.
cells of varying degrees of differentiation.
Well differentiated cells bear remarkable resemblance Histopathological Features
to normal acinar cells, whereas less differentiated cells (Figs 17.52 to 17.58)
resemble embryonic ducts and immature acinar cells (Fig.
FNA cytology: It presents characteristic appearance of the
17.53).
tumor. Smears are cellular with a monomorphic population
The tumor cells can be arranged in solid masses or in
acini like groups. There are four types of growth pattern
seen: solid, papillary-cystic, follicular and microcystic.
Lymphoid elements are commonly found in parotid acinic
carcinomas.
Management
Surgical excision is done. Recurrence rate varies form 8 to
59 percent.
Points to Remember
Acinic cell or serous cell adenoma, exclusively in the
superficial lobe, painless, locally invasive growth,
well differentiated cells, immature acinar cells, solid,
papillary-cystic, follicular and microcystic.
ADENOID CYSTIC CARCINOMA Figure 17.52 Adenoid cystic carcinoma showing presence of
cribriform pattern (low power)
It is also called cylindroma, adenocystic carcinoma and
basaloid mixed tumor. Adenoid cystic carcinoma arises
from major and minor salivary glands and accounts for one
fourth of malignant salivary gland tumors.
It is a slow-growing malignant tumor that might be
aggressive, with a high incidence of distant metastasis.
Apart from the salivary glands, adenoid cystic carcinoma
also has been reported in other locations, including the
breast, lung, larynx, and trachea. The morphologic features
of adenoid cystic carcinoma are similar, regardless of site.
Adenoid cystic carcinoma spreads by direct extension,
perineural invasion, and hematogenous metastasis.
Lymphatic spread is uncommon.
Clinical Features
Age: It occurs in the 5th and 6th decade of life.
Site: Most common glands involved are the parotid, sub Figure 17.53 Adenoid cystic carcinoma showing tubular and
maxillary and the accessory glands in palate and tongue. cribriform pattern (Courtesy: Dr Sangamesh Halawar)
446
Figure 17.54 Solid pattern of ACC showing sheets of uniform Figure 17.56 Characteristic feature of ACC is perineural
appearing cells invasion by tumor cells (TC); (N) Nerves
Figure 17.55 ACC showing tubular pattern (T). Hyperchromatic Figure 17.57 Low power view of adenoid cystic carcinoma
epithelial cells (E) showing no dysplasia form ducts (D) solid pattern showing large islands or sheets of tumor cells with
little tendency for cyst formation
of small basaloid cells with high nuclear/cytoplasmic ratios, Tubular pattern: The stromal connective tissue becomes
coarse chromatin, and small nucleoli. Also present are hyalinized and surrounds the tumor cells, forming a
variable amounts of acellular hyaline stroma in globular or structural pattern of cylinder or tubular from which the
cylindromatous formations typically identified in aspirates lesion originally derived the name cylindroma.
of the cribriform and tubular subtypes.
Solid variant: It consist of larger island or sheets of
Cribriform pattern: It is composed of small, deeply tumor cells. There is also cellular pleomorphism and
staining uniform cells resembling basal cells that are mitotic activity and focal necrosis in the center of the
commonly arranged in anastomosing cords or may contain tumor island.
a mucoid material producing the typical cribriform honey In some cases delicate anastomosing cords of neoplastic
comb or Swiss cheese pattern. cells are dispersed throughout an abundant stroma.
447
Figure 17.58 High power view of adenoid cystic carcinoma Figure 17.59 Polymorphous low grade adenocarcinoma
solid type-showing cells arranged in sheets. Cells are showing intraoral ulceration
hyperchromatic with little atypia. Few cystic spaces are seen
Management
Surgical and in some cases it is accompanied by X-ray
radiation. Recurrence rate is about 60 to 92 percent.
Long-term follow-up is hence essential. The incidence of
metastases is more and the organs involved include cervical
lymph nodes, lungs, brain, liver and kidneys.
Points to Remember
Cylindroma, adenocystic carcinoma, parotid, sub
maxillary, presence of mass, local pain, facial nerve
paralysis, surface ulceration, FNA cytology shows
monomorphic population of small basaloid cells with
high nuclear/cytoplasmic ratios, coarse chromatin, and
small nucleoli, cribriform pattern composed of small, Figure 17.60 CT scan of polymorphous low grade carcinoma
deeply staining uniform cells resembling basal cells
cribriform ‘honey comb’ or ‘Swiss cheese’, tubular
pattern becomes hyalinized tumor cells, solid variant Clinical Features
consist of larger island or sheets of tumor cells. Location: It is seen exclusively in minor salivary gland.
Mostly occur on hard and soft palate, upper lip and buccal
POLYMORPHOUS LOW-GRADE mucosa.
ADENOCARCINOMA Age and sex distribution: It is more common in adults
It is also called lobular carcinoma, terminal duct carcinoma. with female predilection.
It is a malignant epithelial tumor showing which is highly Sign and symptoms: There is painless mass which can be
malignant and metastasize to regional lymph nodes and associated with bleeding and discomfort. Tumor can erode
general viscera. underlying bone (Figs 17.59 and 17.60).
Histopathological Features MALIGNANT MIXED TUMOR

(Figs 17.61 and 17.62)
It is uncertain that these tumors represent the previously
448 Tumor cells are round, polygonal shape with indistinct benign lesions which have undergone transformation into
margin and pale eosinophilic cytoplasm. malignant form or malignant lesion right from the onset.
Polymorphous growth of cell occurs like solid pattern, It accounts for 1% of all parotid tumors and 7% of all
cords, duct, cystic or cribriform pattern. malignant tumors.
Peripheral cells infiltrative invading adjacent tissue in
single line fashion. Perineural invasion can also be seen. Types
• Carcinoma ex-pleomorphic adenoma (carcinoma
Management
ex-mixed tumor)
Wide Surgical excision of the tumor should be carried out. • Carcinosarcoma
• Metastasizing mixed tumor.
Points to Remember
Lobular carcinoma, terminal duct carcinoma, painless
mass, bleeding, discomfort, round, polygonal shape Clinical Features
tumor cells, solid pattern, cords, duct, cystic or Carcinoma Ex-Pleomorphic Adenoma
cribriform pattern, perineural invasion.
(Fig. 17.63)
Age: The tumor occurs from 2nd to 9th decades, but
most frequently in 5th and 6th decade. The average age
of patients with malignant pleomorphic adenoma is about
ten years older than the patients with benign form of the
disease.
Location: It is most commonly seen in parotid gland. Other
less common location is minor salivary gland on the palate.
Sign and symptoms: The tumors are usually larger
than benign ones. There is often fixation of the tumor
to underlying structures as well as to overlying skin or
mucosa. Pain is more frequently a feature of malignant,
than the benign pleomorphic adenoma. Parotid tumor may
Figure 17.61 Histopathological picture of polymorphic low
produce facial palsy (Fig. 17.64).
grade carcinoma (Courtesy: Dr Sangamesh Halawar, Reader,
Department of Oral Pathology, VPDC and H, Kavalapur,
Sangli, Maharashtra, India)
Figure 17.63 Carcinoma ex-pleomorphic adenoma involving

Figure 17.62 Polymorphous low grade adenocarcinoma facial nerve causing Bell’s palsy masked eyes of patient
449
Figure 17.64 Malignant pleomorphic adenoma showing Figure 17.65 Malignant pleomorphic adenoma (Courtesy: Dr
cellular atypia Sangamesh Halawar, Reader, Department of Oral Pathology,
Carcinosarcoma Management
It is rare and most of the cases seen in parotid gland Surgical excision should be done. These neoplasms
with some lesion seen in submandibular as well as minor exhibit a high recurrence rate after surgical removal
salivary gland. Other signs and symptoms are similar to as well as a high incidence of regional lymph node
that of carcinoma ex-pleomorphic adenoma. involvement.
Carcinosarcoma can be treated with radical surgical
Metastasizing Mixed Tumor resection with radiation therapy.
Primary tumor seen in parotid gland. Metastasis occurs in
bones and lungs, regional lymph nodes, skin and liver. Points to Remember
Carcinoma ex-pleomorphic adenoma: Parotid gland,
usually larger than benign, pain, facial palsy, malignant
Carcinoma Ex Pleomorphic Adenoma component may over grow, nuclear hyperchromatism,
In some cases malignant component may overgrow the pleomorphism, abnormal mitosis, infiltrative growth at
benign component so that the benign component is difficult the periphery.
to demonstrate and in some cases benign component is Carcinosarcoma: Parotid gland, signs and symptoms
more with few malignant foci may be found. same as above, sarcomatous as well carcinomatous
There is presence of nuclear hyperchromatism changes.
and pleomorphism, increased or abnormal mitosis and Metastasizing mixed tumor: Parotid gland, metastasis
destruction of normal tissues. There is destructive infiltrative occurs in bones and lung, features of benign pleomorphic
growth at the periphery, with excessive hyalinization. adenoma seen at primary and metasize site.
Carcinosarcoma
It is biphasic tumor which can show sarcomatous as well CONNECTIVE TISSUE TUMORS
carcinomatous changes. Epithelial changes resemble that Hemangioma is the only common tumor of this group and
of adenocarcinoma and connective tissue resembles that of the most frequently seen in young infants. The involve
chondrosarcoma. gland appears hypertrophied.
There is blue discoloration of the overlying skin.
Metastasizing Mixed Tumor Lipoma may occur in the parotid gland. The facial nerve,
This has got features of benign pleomorphic adenoma both occasionally gives rise to a neural tumor. True sarcoma is
in primary as well as in metastatic sites. extremely rare.
NECROTIZING SIALOMETAPLASIA or bilateral. The ulcer is well demarcated from surrounding

normal tissue and often has an inflammatory reaction
It is a non-neoplastic inflammatory self healing reaction around the edge of the lesion. Margins of ulcer in some
450 of salivary gland tissues, which both clinically and cases may be indurated and inflamed.
histologically mimics a salivary gland malignancy. It Size: The lesion itself is most often a deep, craterlike, non
usually affects the minor salivary glands. It was predicted draining ulcer 1 to 3 cm in diameter.
that trauma causes ischemia of the minor salivary glands. The lesion is covered by an inflammatory exudate and
This benign self limiting lesion has been often confused necrotic debris however the margins of ulcer are usually
with malignancy thereby causing unnecessary surgery. clean, sharp and the granulation tissue often can be seen at
the superficial aspect.
Etiology and Pathogenesis (Flow chart 17.1)
Local ischemia, which may be due to physical, chemical, Histopathological Features
infective or local vasculitis. It is characterized histologically by an ulcerated mucosa,
Trauma form various factors such as denture wearing pseudoepitheliomatous hyperplasia of the mucosal
and recent surgery may be a causative factor. In some cases, epithelium, acinar necrosis and squamous metaplasia
smoking and alcohol intake can lead to this condition. of salivary ducts. There is preservation of the lobular
architecture, despite necrosis and inflammation.
Clinical Features Inflammatory cells may be found in and around the
Age and sex: It is more common in males than females. It lobular areas of necrosis. Granulation tissue and fibrosis
occurs in 4th and 5th decade. present in variable amounts.
Location: Most of the cases occur in palate, although cases
Management
of lip or retromolar pad also have been reported.
It is a self limiting condition. It does not require any
Symptoms: It is usually painless or may cause only slight treatment. The healing occurs in six to twelve week via
pain. The patient may have numbness in the palate, or area secondary intention. Debridement and saline rinses may
of looseness in palate. Pieces of tissue may fall out from aid the healing process.
the palate. There may be referred pain to ear or pharynx.
Signs: There may be early mild swelling to more advanced Points to Remember
and cancerous appearing ulcer. The lesion begins as a Trauma causes ischemia of the minor salivary glands,
larger ulcer or ulcerated nodule, which may be unilateral painless, numbness in the palate, pieces of tissue
may fall out from the palate, ulcer is well demarcated
from surrounding normal tissue, deep, craterlike,
Flow chart 17.1 Events in necrotizing sialometaplasia
pseudoepitheliomatous hyperplasia of the mucosal
epithelium, lobular areas of necrosis, debridement and
saline rinses.
BIBLIOGRAPHY
1. Batsakis JG, Luna MA. Undifferentiated carcinomas of
salivary glands. J Laryngol Otol. 1987;101(9):962-6.
2. Batsakis JG. Salivary Gland neoplasm: an outcome of
modified morphogenesis and cytodifferenciation. Oral Surg
Oral Med Oral Pathol. 1980;49:229-32.
3. Chidzonga MM, Perez VML, Alvarez ALP. A clinico-
pathologic study of parotid tumors. J Oral Maxillofac Surg.
1994;52:1253-6.
4. Cohen MA. Pleomorphic adenoma of the cheek. Int J Oral
5. Eveson JW, Cawson RA. Salivary gland tumours: a review an evaluation of 237 cases. Acta Otolaryngol. 2005;125:207-
of 2410 cases with particular reference to histological 14.
types, site, age and sex distribution. J Pathol. 1985;146: 16. Margaret SB, Katya I, Derrick IW. Muco epidermoid
51-8. carcinoma. A clinicopathologic study. Am J Surg Pathol. 451
6. Gerhard Seifert, Leslie H Sobin. The world health 2001;25:835-45.
organization’s histological classification of salivary gland 17. Naunheim MR, Lin HW, Faquin WC. Intercalated duct
tumors. A commentary on the second edition. Cancer. lesion of the parotid. Head Neck Pathol. 2012;6(3):373-6.
1992;70(2): 379-85. 18. Noguchi M, Onizawa K, Bukawa H. Basal cell adenoma
7. Gnepp DR. My journey into the world of salivary gland arising in a minor salivary gland of the palate. Oral
sebaceous neoplasms. Head Neck Pathol. 2012;6(1):101-10. Maxillofac Surg. 2012;16(1):111-4.
8. Goode RK, Auclair PL, Ellis GL. Mucoepidermoid carcinoma 19. Plambeck K, Friedrich RE, Bahlo M, Bartel-Friedrich
of the major salivary glands: clinical and histopathologic S, Klapdor R. TNM staging, histopathological grading,
analysis of 234 cases with evaluation of grading and tumor-associated antigens in patients with a history
criteria. Cancer. 1998;82:1217-24. of mucoepidermoid carcinoma of the salivary glands.
9. Gustafsson H, Dahlqvist A, Carlsoo B. Mucoepidermoid Anticancer Res. 1999;19:2397-2404.
carcinoma in a minor salivary gland in childhood. Laryngol 20. Przewony T, Stodulski D, Stankiewicz C. Major salivary
Otol. 1987;101:1320-3. gland disorders in children and adolescents]. Otolaryngol
10. Guzzo M, Andreola S, Sirizzotti G, Cantu G. Mucoepidermoid Pol. 2011;65(5):350-6.
carcinoma of the salivary glands: clinicopathologic 21. Rodriguez-Cuevas S, Labastida L, Baena L, Gallegos F.
review of 108 patients treated at the National Cancer Risk of nodal metastases from malignant salivary gland
Institue of Milan. Ann Surg Oncol. 2002;9:688-95. tumors related to tumor size and grade of malignancy. Eur
11. Healey WV, Przin KH, Smith L. Mucoepidermoid carcinoma Arch Otorhinolaryngol. 1995;252:139-42.
of salivary gland origin. Classification, clinical-pathologic 22. Seethala RR. Histologic grading and prognostic biomarkers
correlation, and results of treatment. Cancer. 1970;26:368-88. in salivary gland carcinomas. Adv Anat Pathol. 2011;18(1):
12. Hicks MJ, el-Naggar AK, Flaitz CM, Luna MA, Batsakis 29-45.
JG. Histocytologic grading of mucoepidermoid carcinoma 23. Spiro RH. Salivary Neoplasms: overview of a 35 year
of major salivary glands in prognosis and survival: a experience with 2807 patients. Head Neck Surgery. 1986;
clinicopathologic and flow cytometric investigation. Head 8:177-84.
Neck. 1995;17:89-95. 24. Vaidya AD, Pantvaidya GH, Metgudmath R, Kane SV,
13. Hübner G, Klein HJ, Kleinsasser O, Schiefer HG. Role of D’Cruz AK. Minor salivary gland tumors of the oral cavity:
myoepithelial cells in the development of salivary gland a case series with review of literature. J Cancer Res Ther.
tumors. Cancer. 1971;27(5):1255-61. 2012;8 Suppl 1:s111-5.
14. Ishibashi N, Yanagawa T, Yamagata K, Karube R, Shinozuka 25. Vander Poorten VL, Balm AJ, Hilgers FJ, Tan IB, Loftus-
K, Nagata C. Coll BM, Keus RB, et al. The development of a prognostic
15. Luukkaa H, Klemi P, Leivo I, Koivunen P, Laranne J, score for patients with parotid carcinoma. Cancer. 1999;85:
Makitie A, et al. Salivary gland cancer in Finland 1991-96: 2057-67.
1. The first gland appear during intrauterine life is: 4. Wharton’s duct is associated with:
a. Sublingual gland b. Parotid gland a. Parotid gland b. Sublingual gland
c. Submandibular gland d. Minor glands c. Submandibular gland d. Both b and c
2. The largest salivary gland is: 5. The most common gland involved in sialolithiasis is:
a. Parotid gland b. Submandibular gland a. Parotid gland b. Submandibular gland
c. Sublingual gland d. Accessory ducts c. Sublingual gland d. Both a and b
3. The duct associated with parotid gland is: 6. ‘Mucinophages’ a histopathological feature seen in:
a. Bartholin’s duct b. Wharton’s duct a. Sialosis b. Stenosis
c. Stensen’s duct d. None c. Strictures d. Mucocele
7. ‘Epimyoepithelial island’ a histopathological feature 13. Tender submandibular swelling is mostly due to:
seen in: a. Ludwig’s angina
a. Sjögren’s syndrome b. Pleomorphic adenoma b. Stone or sialolithiasis
452 c. Uveoparotid fever d. Mumps c. Enlarged lymph nodes
8. A chocolate colored eosinophilic coagulum seen in: d. All of the above
a. Brachial cyst of parotid 14. The most common complication of mumps is:
b. Warthin’s tumor a. Myocarditis b. Uveitis
c. Monomorphic adenoma c. Orchitis d. Conjunctivitis
d. Both a and b 15. A parotid fatty change is sign of:
9. Histopathological feature showing nests of epidermoid a. Aging b. Alcoholism
cells and mucous cells present in: c. Malnutrition d. None of the above
a. Mucoepidermoid carcinoma 16. Which of the following is of salivary gland origin?
b. Warthin’s tumor a. Acinic cell carcinoma
c. Ductal papillomas b. Chondrosarcoma
d. Myoepithelioma c. Granular cell tumor
10. Numbness in the palate, or area of ‘looseness’ in palate d. None of the above
is the clinical feature of: 17. The most common site for ectopic salivary gland tumor:
a. Small cell carcinoma a. Tongue b. Cheek
b. Necrotizing sialometaplasia c. Palate d. Neck
c. Salivary duct carcinoma 18. Sialography is used to detect anomaly of:
d. None a. Salivary duct only
11. Mikulicz’s disease is: b. Salivary gland
a. An inflammatory disease c. Salivary gland and duct
b. Neoplastic disease d. Salivary gland tumors
c. An autoimmune disease 19. Ranula is associated with:
d. Viral infection a. Mucus retention phenomenon only
12. The histogenesis of pleomorphic adenoma is from b. Mucus retention cyst only
which cell: c. Mucus extravasation cyst only
a. Myoepithelial cell d. Both (b) and (c)
b. Intercalated duct cell 20. Mucoceles are most commonly found in:
c. Epithelial cell a. Frontal sinus b. Maxillary sinus
d. Neural crest cell c. Ethmoid sinus d. None.
18 Bacterial Infection
Chapter Outline
Â Impetigo Â Scarlet fever

Â Erysipelas Â Diphtheria
Â Syphilis Â Tularemia
• Primary syphilis Â Rhinoscleroma
• Secondary syphilis (disseminated syphilis) Â Granuloma inguinale
• Tertiary syphilis Â Streptococcal tonsillitis and pharyngitis
• Congenital syphilis Â Tonsillar concretion and tonsillolithiasis
Â Gonorrhea Â Lymphogranuloma venereum
Â Leprosy (Hansen disease) Â Myiasis
Â Tuberculosis Â Cat scratch disease
Â Actinomycosis Â Pyostomatitis vegetans
Â Noma Â Sinusitis
The literal meaning of the word ‘disease’ is ‘loss of ease’. have an important note in preventive medicine as many
The oral cavity reflects the state of systemic health more systemic diseases have primary oral manifestations.
frequently than other parts of the body. Even in ancient
time, examination of mouth and tongue was given great IMPETIGO
importance. The oral tissues are in direct physical continuity It is also called ‘impetigo vulgaris’. It is acute superficial,
with rest of the body and they are also related via blood, purulent infection of the skin. It is caused by Streptococcus
lymphatics and nerve pathways. Furthermore, systemic pyogenes and Staphylococcus aureus.
influence such as endocrinological, immunological and In many patients with impetigo bacteria remain in nose
psychological factors has an important role in the balance and spread on skin at the site of scratches and abrasion. It
between oral health and disease. is contagious and spread in crowded condition.
During both, development and maintenance, local and
systemic factor are concerned in disease process of mouth. Predisposing Factors
Oral health must be considered in relation to general health. Poor hygiene, crowded living conditions, pre-existing
The dentist’s role in general health is based on the fact that, eczema and scabies and reduced resistance due to
he is the first person to see the oral lesion. In preventive preceding influenza or herpes simplex infection can lead
dentistry, the utmost principle is of early diagnosis. Dentist to this disorder.
Types
• Non-bullous impetigo (impetigo contagiosa)
454 • Bullous impetigo (staphylococcal impetigo)
• Impetiginized dermatitis.
Clinical Features
Age: The disease is mainly seen in pre-school children and
young adults.
Site: The face (angle of mouth, lips and nose) is most
common location.
Non-bullous impetigo: It is also called impetigo contagiosa.
Facial lesion have linear pattern that corresponds to fingernail
scratches. The lesions frequently begin as red, itchy spots. Figure 18.1 Histopathological features of impetigo
The close set, round or oval, flat, pustular vesicles with a
characteristic stuck on appearance subsequently develop. Antibiotics: Antibiotic like cephalexin, trimethoprim,
Vesicles which are formed quickly rupture and covered sulfamethoxazole, dicloxacillin, flucloxacillin and amoxicillin
with adherent thick ambar crust. This is described as clavulanic acid are shown good results. This should be given
‘cornflakes glued to surface’. Some of the lesions may for one week.
become confluent. The surrounding skin is erythematous
with lymphadenopathy, which becomes tender. Points to Remember
Bullous impetigo: It is caused by S. aureus. There is • Impetigo vulgaris, Streptococcus pyogenes
superficial vesicle which rapidly enlarged to form larger • Non-bullous impetigo: Impetigo contagiosa, stuck on
flaccid bullae. Bullae may rupture and develop thin appearance, cornflakes glued to surface.
brown crust which is described as ‘lacquer’. Cellulitis and • Bullous impetigo: Superficial vesicle, lacquer,
pneumonia can occur in some cases. cellulitis
• Impetiginized dermatitis: Secondary involvement of
Impetiginized dermatitis: There is secondary involvement areas of dermatitis
of areas of dermatitis. • Histopathological: Neutrophils leukocytes spongiosa,
Histopathological Features (Fig. 18.1) migration of leukocytes, severe inflammatory
infiltrate, fibrin and neutrophils, topical mupirocin,
Pustules filled mainly with neutrophils leukocytes are seen fusidic acid, antibiotics.
directly beneath the horny layer of the epithelium.
The spinous layer below show spongiosis and migration
of leukocytes from the connective tissue, where a slight to ERYSIPELAS
moderately, severe inflammatory infiltrate of neutrophils It is an acute, superficially spreading infection of the
and lymphocytes is seen. dermis, usually of the face, with a well demarcated, slightly
At the later stage when the bulla ruptures, the horny layer indurated erythema and progressive lymphangitis.
is absent and a crust composed of fibrin and neutrophils,
leukocytes may be found resting on the remaining epithelial Causes
layers. It is caused by group A streptococci. The microorganisms
are thought to enter the tissues through a small break in the
Management mucosa or the skin, such as fissure, abrasion, erosion or
Topical therapy: Topical mupirocin, fusidic acid are excoriation.
effective in dealing non bullous impetigo. Before topical Postsurgical erysipelas is caused by transmission of
application removal of crusts with clean cloth sock in streptococci from the nose, throat, or hands of the patient
warm soapy water is recommended. or from, the attendant or visitors.
Bacterial Infection
Nephrotic edema, lymphedema, dysgammaglobuline- The submandibular lymph nodes become markedly
mia, malnutrition and alcoholism are known predisposing tender and swollen.
factors.
Clinical Features Edema and dilatation of the lymphatics and capillaries
Age and sex distribution: The newborn and infants are occurs.
highly susceptible, but elderly are also affected. It is more A marked and diffuse inflammatory infiltrate, pre-
common in women in 5th decade and in men, in 7th decade. dominately of neutrophils, is seen throughout dermis,
occasionally extending into the subcutaneous fat.
Location: The most common location is on abdomen, face,
scalp and legs. The incubation time it thought to be from Management
few hours to several days. Penicillin is the drug of choice. Other antibiotics which can
Prodormal symptoms: It is characterized by malaise, be given are erythromycin, cephalexin, fluoroquinolones
vomiting, headache, pyrexia and chills. can be given.
After giving antibiotics therapy initially skin involvement
Symptoms: Erysipelas begins abruptly with a local sensation enlarges due to release of toxins from dying streptococci.
of burning and itching. The general condition of the patient
worsens with toxemia, high fever, insomnia and restlessness. Points to Remember
Appearance: A small area of the skin then becomes Group A streptococci, postsurgical erysipelas abdomen,
intensely red and swollen. Subsequently, bright red plaques face, scalp and legs, prodormal symptoms, local
develop and spread rapidly. Blisters, which breakdown to sensation of burning, skin intensely red and swollen,
form large ulceration, may develop on the red lesion. The Saint Anthony’s fire, facial lesion butterfly distribution,
lesion has an elevated, edematous, sharp border and an regional lymph nodes enlarged, complication like
irregular outline. necrotizing fasciitis, toxic shock syndrome, edema
involve the tongue, submandibular lymph nodes, edema
Saint Anthony’s fire: Many times this term is used to and dilatation of the lymphatics and capillaries, diffuse
describe erysipelas. The reason for this is that French inflammatory infiltrate, penicillin.
house of St. Anthony; an 11th century hospital had a fiery
red wall similar to color of erysipelas.
SYPHILIS
Facial lesion: It has got butterfly distribution resembling
lupus erythematous. Many times affected skin have surface It is also called ‘Lues’. It occurs most exclusively by
texture that resemble orange peel. venereal contact, in overcrowded living and primitive
Regional lymph nodes become enlarged and tender. housing conditions. It is cause by Treponema pallidum.
The untreated acute stage of erysipelas resolves after 3-10 This organism is vulnerable to drying so primary mode of
days, leaving dry, desquamating and sometimes partially transmission is from sexual contact.
ulcerated skin. Oral sex has important role in transmission of this
disease nowadays. Syphilis has got three stages out of
Complication: If therapy is not initiated then complications which two stages are contagious.
like gangrene, necrotizing fasciitis, toxic shock syndrome
with multiple organ failure, thrombophlebitis can occur. Types
Acquired syphilis
Oral Manifestations • Primary
It is uncommon in oral, pharyngeal and nasal mucosa. • Secondary
Erysipelas which develops in the oral, pharyngeal or upper • Tertiary
respiratory tract mucosa result in the same constitutional • Quaternary
reaction, as described for skin lesion. There is severe local • Latent phase
pain, redness and swelling. Congenital
Edema may involve the tongue, uvula, and epiglottis Early syphilis
and may lead to serious consequences. Late syphilis
Classification round, indurated and with rolled raised edges. It begins

as a papule, before proceeding to ulceration and varies in
Acquired syphilis: Contacted primarily as venereal
size from 5 mm to several centimeters. It is painless, unless
456 disease due to sexual intercourse with infected partner
superinfected. It disappears without therapy after 10 days.
∙ Primary: This occur usually after 3 to 9 days after
Regional lymph nodes become firm enlarged, rubbery
exposure.
in consistency and non tender.
∙ Secondary: It occur after 4 to 10 weeks of primary
infection. Classic signs: The combination of indurated ulcer on
∙ Tertiary: It appear after secondary after third year to appropriate mucosal surface and enlarged, nontender,
patient life. regional lymph nodes which are painless, discrete and
∙ Quaternary syphilis: The atypical malignant progression rubbery in consistency-constitute the classic signs of
of tertiary neurosyphilis in immunocompromised HIV primary syphilis.
individuals is referred to as quaternary syphilis.
∙ Latent phase: This phase occur after secondary Oral Manifestations
syphilis. It last from 1 to 30 years without any sign and Location: Oral lesions of primary syphilis are rare and
symptoms. occur at the site of entry of Treponema. Chancre has been
Congenital: It is manifested within 2 years of life. described on lips, in males (upper lip) and in females
(lower lip), oral mucosa, lateral surface of tongue, soft
Early syphilis: Primary syphilis, secondary syphilis and
palate, tonsillar area, pharyngeal lesion and gingiva.
the early latent phase of the disease are grouped as early
Transmission can occur during kissing as a consequence
syphilis. Early syphilis may last up to two years and is
of sexual practice among homosexual and heterosexuals,
infectious.
or by contact with objects such as mouth piece of musical
Late syphilis: While late latent and tertiary are grouped instruments and medical or dental instruments.
as late syphilis. Late syphilis is locally destructive and
Appearance: It has narrow copper colored, slightly raised
noninfectious.
borders with reddish brown base in center. It measures
Epidemiology from 0.5 to 2 cm in diameter. Intraoral chancres are slightly
painful due to secondary infection and are covered with
There is decreased incidence after introduction of penicillin grayish white film. Occasionally, it retains white sloughy
in late 1940s. Patient infected with Treponema pallidum material. They are contagious.
and HIV may exhibit a malignant form of syphilis with Primary involvement of tonsils is manifested by
slow development of standard serologic response to considerable edema, redness, ulcerated and eroded lesion.
syphilis. The tertiary manifestations lead to considerable Regional lymphadenopathy occurs.
morbidity and mortality. Extraoral lip chancre may have more typical brown
crusted appearance which may be multiple (Fig. 18.2).
Primary Syphilis
Lower lip involved more frequently. Oral chancre heals
Clinical Features spontaneously in 3 to 6 weeks leaving small scars.
Lesion develops at the site of inoculation approximately
3 to 90 days after contact with infection. Histopathological Features (Fig. 18.3)
Surface epithelium is ulcerated in this stage. The chancre
Location: It occurs most frequently on penis in males
is microscopically characterized by presence of dense
and vulva or cervix in females. Recently, occurrence on
infiltrate of plasma cells, lymphocyte, macrophages and a
extragenital sites have increased as a result of increase in
proliferative granulation tissue.
orogenital sex and increased contact among the infected
Obliterative endarteritis, characterized by perivascular
homosexuals. Extragenital sites of involvement include
infiltration of inflammatory cells is seen.
fingers, perianal region, nipples and lips, tonsils and
Deeper tissues show endothelial proliferation, swelling
intraoral structures such as tongue and palate.
and perivascular cuffing by inflammatory cell infiltrate.
Appearance of chancre: It is slightly raised, ulcerated, Proliferative granulation tissue is present at the margin
nontender, nonbleeding, firm plaque which is usually of ulcer. Plasma cells are numerous.
Bacterial Infection
Symptoms: Fever, headache, anorexia, pain in joints and

muscles occurs.
Generalized lymphadenopathy, which is painless,
discrete and nonadherent to the surrounding tissues, may be 457
seen. Generalized symmetrical enlargement of the lymph
nodes in posterior cervical, suboccipital, supratrochlear
and inguinal regions is seen.
Lues maligna: This is explosive and widespread form
characterized by necrotic ulceration on face and scalp.
Mucous patches are small, smooth, erythematous areas
or superficial grayish erosions found on mucus membrane
of vulva, penis, or in oral cavity, on palate and tonsils.
Condyloma latum are grayish, moist, flat topped, extra
large plaque which sometimes coalesce into larger plaques,
Figure 18.2 Syphilitic ulcer on the lip which has got ill found on moist mucocutaneous surfaces such as vulva,
defined border anus, scrotum, thigh and axilla.
Split papule is a double papule which occurs at skin
folds and angle of mouth.
There is positive serological test. Patient may enter the
phase of latency without treatment.
Oral Manifestations
Mucous patches: Mucous membrane analog of papular
or macular skin eruptions. If it found on tongue, buccal
mucosa, tonsillar and pharyngeal region and lips. It
appears as slightly raised grayish white lesions surrounded
by erythematous base. They are covered by grayish white
membrane. Trauma results in raw bleeding surface.
Snail track ulcers: Confluence and coalescence of these
glistening mucous patches gives rise to the so called ‘snail
track ulcers’. It is often painless but mild to moderately
Figure 18.3 Nonspecific infection of the syphilis (IC) and an
painful.
ulcerated surface epithelium (U)
Split papule: Split papule is a raised papular lesion
developed at the commissure of lip and with a fissure
Secondary Syphilis (Disseminated Syphilis)
separating the upper lip portion from lower lip portion.
Clinical Features They are called split papules as they are cracked in the
Organisms proliferate and spread by the way of blood middle giving a ‘split pea appearance’. They are highly
stream to produce lesions elsewhere. It usually appears infectious.
within 4 to10 weeks after primary lesion. Condyloma latum: They are flat silver gray wart like
Appearance: When appear on skin, they manifest as papule, sometimes having ulcerated surface. They are
fine macular or papular rash, sometimes accompanied by painless. Regional lymphadenopathy is usually present.
alopecia.
Circinate lesions on face are characteristic of secondary Histopathological Features
syphilis. The lesions either resolve completely or leave The macular lesion shows inflammatory cell infiltration
residual areas of hypo- or hyperpigmentation. and obliterative endarteritis.
The papular lesion exhibits endothelial proliferation, irritability, fatigue, mental sluggishness and carelessness
swelling and perivascular chronic inflammatory cell in personal habits. Loss of fine muscular coordination as
infiltration. indicated by inability to enunciate clearly or to perform
458 Condyloma latum reveals hyperplastic epithelium with delicate tasks with the hands. Involvement of spinal cord
hyperkeratosis and acanthosis. is late manifestation characterized by paresthesia, burning
and prickling sensation in the extremities. Patient may get
Tertiary Syphilis unrealistic ideas of wealth or ability.
Clinical Features Cardiovascular syphilis: It occurs in 10 percent cases
Age: It may occur at any age from the third year up to the of late syphilis. Involvement of CVS in tertiary syphilis
patient’s life. affects aorta and aortic valve and 80 percent of deaths
In tertiary syphilis, 1/3rd develop benign or gummatous occur due to it. Medial necrosis and destruction of elastic
form, 1/3rd cardiovascular form and 1/3rd neurosyphilis, tissue occurs in the wall of large blood vessels. Dilatation
i.e. general paresis and tabes dorsalis. and aneurysm occurs.
Gumma: It is due to a chronic destructive granulomatous Oral Manifestations
process which occurs anywhere in the body. Gumma is
the result of hypersensitivity reaction between hyperergic They manifest any time during 3 to 10 years after the
host and Treponema. There are two types of gumma, i.e. primary infection. Gumma can occur anywhere in the jaw
central and cortical. The characteristic gumma is a chronic but are more frequently on palate, mandible and tongue.
granulomatous and usually localized lesion, which later Gumma: It may manifest as solitary, deep, punched out
ulcerates which may be nodular. Punched out ulcer with mucosal ulcer. It usually starts as small, pale, raised,
vertical walls and dull red granulomatous base is the typical nodular mass in the midline of the palate which ulcerates
clinical feature of ulcerative gummatous lesion. Cutaneous and rapidly progresses to the zone of necrosis. It may
lesions heal slowly and leave behind tissue paper like scars. cause perforation of palatal vault. Lesion is sharply
Single cerebral gumma may produce symptoms suggestive demarcated and the necrotic tissue at the base of the ulcer
of brain tumor. may slough away leaving punched-out defects. Breathing
Neurosyphilis: It occurs due to obliteration of small vessel and swallowing difficulty may be encountered by the
artery involving vasa vasorum of aorta and other large patient.
vessels of the central nervous system (neurosyphilis). Numerous small healed gummata in tongue results
There are saddle deformities of nose. Neurosyphilis is in series of nodules or scars in deeper areas of the organ,
manifested as tabes dorsalis and general paresis. Tabes giving the tongue an upholstered or tufted appearance.
dorsalis is the syphilitic involvement of dorsal column of Chronic superficial interstitial glossitis: The tongue
spinal cord and dorsal root ganglion. General paresis is may be involved diffusely with gumma presenting as
syphilitic involvement of cerebral tissue. lobulated large and irregular shaped pattern. This is called
Tabes dorsalis: Patient looses the positional sense of his as interstitial glossitis. It is exclusive found in males and
lower extremities and walks with a slapping step. Burning is considered as pre-cancerous because of predictions to
and pricking sensation of the extremities, paresthesia, or undergo carcinomatous transformation.
at times, actual anesthesia of the part may accompany Leutic glossitis: Diffuse atrophy and loss of dorsal
the characteristic gait. Positive Romberg’s sign—person tongue papillae is called leutic glossitis. Loss of papillae
is unable to stand erect unaided with his eyes closed. is probably due to endarteritis leading to circulatory
Short, shooting, knife-like pains may be experienced in deficiency of lingual vasculature.
the abdominal region called ‘tabetic crises’, which results
from involvement of the dorsal root ganglion. Charcoat Histopathological Features
joint – trophic changes consist of deep perforating ulcers Gumma is characterized by central zone of coagulation
and painless destruction of larger joints. necrosis and peripheral rim rich in fibroblasts, which
General paresis: Argyll Robertson pupil—pupils that appear plump and often resemble epithelioid cells. There is
react to accommodation but not to light. Increased also pseudoepitheliomatous hyperplasia.
Bacterial Infection
Fibroblasts are plump and they often resemble with maculopapular eruptions, other than mucocutaneous
epithelioid cells. Occasional presence of giant cells and lesion and loss of weight.
regular presence of chronic inflammatory cells like plasma
Location: The lesions can be seen in spleen, kidney, bones 459
cells, lymphocytes and histiocytes.
and CNS. Bullae, vesicle and superficial desquamation
Points to Remember with cracking and scaling of reddened soles and palms,
petechiae, mucous patches and condyloma latum.
Lues, Treponema pallidum. After 2 years, interstitial keratitis, vascularization of
• Primary syphilis: Penis, vulva or cervix in females, cornea, 8th nerve deafness, arthropathy, signs of congenital
chancre are raised, ulcerated, nontender, non- neurosyphilis, gummatous destruction of palate and nasal
bleeding, firm plaque, regional lymph nodes are septum develop. Saber shins or anterior tibial bowing.
nontender with rubbery in consistency, chancre
can be seen on lips, transmission kissing, colored, Higoumenakis’s sign: Irregular thickening of sterno-
slightly raised borders with reddish brown base, clavicular portion of clavicle. Unexplained nerve deafness,
involvement of tonsils, extraoral lip chancre, dense retinal and corneal damage can also occurs.
infiltrate of plasma cells, lymphocyte, macrophages,
perivascular infiltration of inflammatory cells, Oral Manifestations
endothelial proliferation Postrhagadic scarring and syphilitic rhagades: Post-
• Secondary syphilis: Fine macular or papular rash, rhagadic scars are linear lesions found around oral and
circinate lesions on face, generalized lympha- anal orifices. They result from diffuse leutic involvement
denopathy, lues maligna, mucus patches, condy- of the skin in these areas from 3rd to 7th week after birth.
loma latum, split papule, positive serological test, They appear as red or copper colored linear areas covered
snail track ulcers, inflammatory cell infiltration, with a soft crust. Rhagades are said to be more frequent
obliterative endarteritis, endothelial proliferation, on the lower lip. Healed syphilitic rhagades appear as
chronic inflammatory cell infiltration, hyperplastic ordinary cicatrices. The linear scars are radially arranged
epithelium with hyperkeratosis and perpendicular to the mucocutaneous junction, which
• Tertiary syphilis: Gummatous form, cardiovascular are more prominent on lower lip near angle of mouth.
form, neurosyphilis, tabes dorsalis, general paresis, Diminished coloring of lip is evident and mucocutaneous
argyll Robertson pupil, aorta and aortic valve, border is indistinct.
gumma on palate, upholstered or tufted appearance
tongue, chronic superficial interstitial glossitis, Hutchinson’s triad: It consist of hypoplasia of permanent
leutic glossitis, central zone of coagulation necrosis incisors and 1st permanent molars, eight nerve deafness
and peripheral rim rich in fibroblasts, fibroblasts are and interstitial keratitis.
plump, giant cells, plasma cells, lymphocytes and Changes in dentition: Retarded root resorption of
histiocytes. deciduous dentition. There may be ‘marring’ of permanent
incisors present in congenital syphilis. 6 to 28 percent of the
Congenital Syphilis incisors and 3 to 37 percent of the molars have hypoplasia.
It is infection of fetus established by the passage of The crown of the first molar in congenital syphilis is
spirochetes from mother, through the placenta. Maternal irregular and enamel of the occlusal surface and occlusal
transmission during first two stages results in stillbirth, third of the tooth appears to be arranged in agglomerate
miscarriage and infant with congenital anomalies. mass of globules, rather than in well formed cusp.
Screw drive shaped incisors: Constriction of crown
Clinical Features toward incisal edge screw results in driver or peg shaped
Transplacental infection after 18-week gestation is related incisor. In addition, incisal edge is usually notch which may
to development of immune complement rather than any be due to the absence of central tubercle or calcification
toxic effect on organism. center.
It is manifested within the first 2 years of life (neonatal Rounding of mesial and distal incisal line angles occurs.
congenital syphilis) as rhinitis and chronic nasal discharge There is spacing between cuspid and incisors.
Moon molars: In molars positioning of the cusps toward Management

the central portion of the crown, gives the tooth a bud
Benzathine penicillin: Single dose of parenteral long
shaped or a shrunken occlusal form called as mulberry
460 acting benzathine is given for primary and secondary
molars or Moon’s molars. Affected molars are dirty yellow
syphilis. In case of tertiary syphilis IM penicillin is given
in color due to hypocalcification.
weekly for three weeks. Patient who are allegoric to
Carabelli cusp: Prominent accessory mesiolingual cusp penicillin doxycycline is given.
of upper molar (Carabelli cusp). Malocclusion and open
Jarisch Herxheimer reaction: This is occur after
bite is present. In congenital syphilis characteristic shape
administration of penicillin to patient. This occurs
of tooth crown can be identified on radiograph.
secondary to release of endotoxin when antibiotics kill
large number of organism. There is mild fever, malaise,
Points to Remember headache and increase in skin and mucosal lesion.
Transplacental infection, neonatal congenital syphilis,
rhinitis, chronic nasal discharge, interstitial keratitis, GONORRHEA
vascularization of cornea, 8th nerve deafness, saber
shins or anterior tibial bowing, Higoumenakis’s It is primarily an infection of the genitourinary tract
sign, Postrhagadic scarring and syphilitic rhagades, mucosa. Occasionally it can involve extra-genital sites.
Hutchinson’s triad, i.e. hypoplasia of permanent incisors Local infection may occur at extra-genital site (Rectal and
permanent molars, eight nerve deafness, retarded root pharyngeal gonorrhea).
resorption of deciduous dentition, screw drive shaped Rectal mucosa is affected in 30 to 50 percent of women
incisors, Moon molars, Carabelli cusp. with urogenital gonorrhea. Uncomplicated local infection at
other extra-genital sites is rare in adults. Initially, urogenital
infection is symptomless and associated with purulent
Diagnosis of Syphilis
urethral discharge, which responds to antibiotic therapy.
The presence of clinical manifestations together with
history of a sexually active person should give clue to the Etiology
diagnosis of acquired syphilis. It is caused by gram-negative intrabacillary located
Dark field examination microscopy: It is the most useful diplococcus Neisseria gonorrheae. It is an oval, paired,
method of identifying spirochete in primary acquired and gram-negative microorganism. Early sexual awakening,
occasionally, secondary syphilis. Not reliable for oral prostitution and varied sexuality are believed to be
responsible for the increase incidence of gonorrhea.
lesions, since the normal flora contains nonpathogenic
treponema which are difficult to distinguish from T. Pathogenesis
pallidum.
Once the gonococci are directly deposited on the
Lesion biopsy: Histopathological examination of suspected genitourinary tract mucosa during sexual intercourse, they
lesion, stained by silver impregnation technique, is useful penetrate through the intercellular spaces of the epithelium
particularly when the lesion contains few organisms, and reach the subepithelial connective tissue. Within 2
as may be in case of tertiary lesion. For oral lesions this to 3 days of infection, an intense inflammatory reaction
technique is of considerable value. occurs resulting in characteristic mucopurulent discharge
through urethral lumen. A chronic stage may be reached, if
VDRL and RPR: Venereal disease research laboratory untreated and spread is either by direct extension through
and rapid plasma regain test are can also be done. These lymphatics or hematogenous route.
tests are positive after 3 weeks of infection.
Fluorescent treponeal antibody absorption (FTA- Clinical Features
ABS), T. pallidum paticle agglutination assay (TPPA) and Age and sex distribution: It is primarily a disease of
microhemagglutination assay for antibody T. pallidum young adults between the age of 15 to 24 years and is more
(MHA-TP) are some highly sensitive serological test. common in males, as compared to females.
Bacterial Infection
Symptoms occur in 1 day to 2 weeks, following contact hematogenous spread. Difficulty in jaw movements due to
with infected persons. There is profuse purulent urethral pain and swelling of single or both joints is the presenting
discharge with frequent micturition, followed by dysuria. symptom. Cervical lymphadenopathy and fever may be
In some of the patients, there may be fever and headache. present. Rarely, perforation to the tympanic plate occurs. 461
In females, acute gonorrhea includes urethral, cervical and It may lead to fibrous ankylosis because articular cartilage
vaginal discharge. is destroyed.
Pelvic inflammatory disease: This is complication occur
in females and it results due to organism involving uterus Diagnosis
and ovarian tubes. Patient complaints of abdominal cramps In all forms of it, including those of oral cavity and pharynx,
and abnormal bleeding. the diagnosis rests on the identification of organism.
Disseminated gonorrhea: Some patient will have Method used include gram stained smear, culture studies
disseminated gonorrhea which results in myalgias, and direct fluorescent antibody test (Fig. 18.4).
arthralgias, polyarthritis and dermatitis. The skin lesion
consists of discrete papule with hemorrhagic component Management
and seen on extremities. Oral ciprofloxacin: This if first line therapy for gonorrhea.
Gonococcal ophthalmia neonatorum: This occur due Other fluoroquinolones like levofloxacin and ofloxacin can
infection of eye due to infected mother. There is perforation also be used.
of globe of eye and blindness. There is conjunctivitis and Other drugs which used are broad-spectrum
mucopurulent discharge. cephalosporin antibiotic like ceftriaxone 125 to 250 IM
plus doxycycline 100 mg orally; twice a day for 7 days
Oral Manifestations should be given.
Pharyngeal gonorrhea: It is higher in pregnant women
(2–15%), sexually active homosexuals (2–25%) and Points to Remember
heterosexuals practicing oral sex. History of fellatio is Intrabacillary located diplococcus Neisseria gonorrhea,
more associated with pharyngeal gonorrhea. Sore throat profuse purulent urethral discharge, pelvic inflammatory
and evidence of pharyngitis. Pharyngeal gonorrhea is a disease, disseminated gonorrhea which results in myal-
term used for patients in whom Neisseria gonorrheae is gias, arthralgias, gonococcal ophthalmia neonatorum,
isolated from nasopharynx. pharyngeal gonorrhea, gonococcal stomatitis with or
Gonococcal stomatitis: Incidence is very rare and often without pseudomembrane formation, lips develop acute
shows multiple, painful and round elevated gray white painful ulcerations, temporomandibular joint involve-
eroded spots, with or without pseudomembrane formation. ment, ceftriaxone, oral ciprofloxacin.
Regional lymphadenopathy may be seen. Acute gingivitis
develops around extraction site in patient who practices
fellatio repeatedly for days after extensive dental extraction.
The wide range of lesion may develop in gonococcal
stomatitis, i.e. isolated ulcers, gingivitis and membranous
gingivostomatitis.
Lips may develop acute painful ulcerations, limiting
the motion. Gingiva may become erythematous, with
or without necrosis. The tongue may present red, dry
ulcerations or become glazed and swollen with painful
erosion with similar lesions on buccal mucosa and palate.
Temporomandibular joint involvement: Gonococcus
infection involving articulating joint is most common
form of extragenital gonorrhea. Any joint may be affected,
the commonest being knee, ankle and wrist. TMJ is
affected in 14 percent of patients. It occurs as a result of Figure 18.4 Neisseria gonorrhea
LEPROSY (HANSEN DISEASE) Sign and symptoms: Toward the end of this stage the
symptoms are those of irritation of nerve ending in the skin,
It is a chronic infectious disease which has predilection persistent or recurrent paresthesia and numbness- localized
462 for the skin, nerves and mucous membrane. It probably to certain area with no accompanying visible alteration in
originated in tropic and spread to the east. the corresponding skin.
Leprosy has always been considered in superstitious
dread and the person suffering from leprosy was considered Tuberculoid Type
unclean and socially outcasted. It is a benign form of leprosy involving the skin nerves and
regional lymph node.
Etiology
Appearance: Lesions are hypopigmented, erythematous
The leprae bacillus, Mycobacterium leprae, first observed
and flat or raised cutaneous lesions. Nerve involvement
by Hansen in 1868. It is not been possible to grow the
with loss of different types of sensation is also manifest.
bacillus in culture media. It is an acid fast, gram positive,
non motile bacterial with affinity for Schwann cells and Early tuberculoid leprosy: It is manifested by hypo-
cells of reticuloendothelial system. pigmented macules which are sharply demarcated and
hypesthetic.
Types Intermediate tuberculoid lesion: Later the lesions
• Tuberculoid (TT) are larger with elevated and circinate margin. There is
• Lepromatous (LL) peripheral spread and central healing.
• Borderline tuberculoid (BT) Fully develop lesion: In this lesions are densely anesthetic
• Borderline lepromatous (BL) and loose normal skin organs (sweat glands and hair
• Polyneuritic follicles). There may be severe neuritic pain.
• Maculoanesthetic Loss of eyebrows and eyelashes is prominent feature of
• Indeterminate later involvement.
• Erythema nodosum leprosum. The sequelae of peripheral nerve involvement may
develop in some cases and this may give rise to muscle
Pathogenesis atrophy, like contracture of hands and feet, loss of
Entry of bacilli that reaches the lymphatic and bloodstream phalanges, lagophthalamus, exposure keratitis and corneal
are taken up by Schwann cells peripheral nervous system, ulceration leading to blindness.
where they start multiplying. If the host cell-mediated
immunity is adequate bacilli are destroyed and there is Borderline Type
no disease. Host immunity is unstable and suboptimal. It represent wide clinical picture and it is subdivided into
Restricted multiplication of bacilli. Lesion will develop, borderline tuberculoid and borderline lepromatous types.
when there is relatively good immunity but not enough Skin and nerve involvement is characterized by flat
to eliminate the infection, a localized type of disease and raised asymmetrical areas of hyper-pigmentation with
called as tuberculoid type. When the host cell immunity well defined or ill defined margins. The raised lesions
is deficient a generalized form of the disease lepromatous are rubbery or soft in consistency and erythematous. The
leprosy develops. In between these two polar varieties of edges of skin lesion are sharply demarcated, in tuberculoid
the disease there is a wide spectrum of manifestations, type and sloping in cases of lepromatous type. The surface
categorized as borderline leprosy. of lesion is dry and rough in tuberculoid type, while it is
smooth and shiny in lepromatous form.
Clinical Manifestations
General Features Lepromatous Leprosy
This form is more commonly seen in children and female
Age and sex distribution: Males are affected more
are affected more as compared to males.
commonly than females with ratio of 3:1.
It has got incubation period of 2 to 5 year during which Location: This malignant form of the disease produced
patient passes through silent or latent period. widespread involvement of body skin, peripheral nerves,
Bacterial Infection
mucous membrane, lymph nodes, eyes, skeleton tastes and

other internal organs.
It develops early as erythematous macules or papules
without subsequently lead to progressive thickening of 463
skin and the characteristic nodules (Fig. 18.5). The borders
of the lesion are ill defined and centers of the lesion are
indurated and convex.
Leonine facies: Loss of lateral portion of eye brow is
common. Much later, the skin of face and forehead become
thickened and corrugated (leonine facies) and earlobe
becomes pedunculous; nasal stiffness, epistaxis, hoarseness
and saddle nose also occur. Painless inguinal and axillary
lymphadenopathy is common along with sterility and
gynecomastia. Nerve involvement is a late phenomenon. It Figure 18.6 Claw hand seen in leprosy patient
runs chronic course and seldom causes sudden death.
Claw hand: This is one of the typical features of leprosy
(Fig. 18.6).
Erythema Nodosum Leprosum

They occur in lepromatous and borderline lepromatous
patients, most frequently in the later half of the initial year
of treatment.
Tender, inflamed subcutaneous nodules develop,
usually in crops. Each nodule lasts a week or two, but
new crop may appear. Low grade fever, lymphadenopathy
and arthralgia can accompany severe erythema notosum
leprosum (ENL).
Leporma: This is common feature of erythema nodosum
leprosum (Fig. 18.7). Figure 18.7 Leproma manifested as nodular swelling in
leprosy
Orofacial Manifestations (Fig. 18.8)

Depending on the type and duration of the disease, all
patients with lepromatous leprosy show facial and oral
manifestations and it is rare in tuberculoid and borderline
leprosy.
Appearance: The lesions are macular or raised, well-
defined, hypopigmented, unhydrotic and hyperesthetic or
anesthetic. Lepromatous infiltration of the helical and lobes
of ear are characteristic of the rare stage of the disease.
Advanced cases are characterized by nodular involvement
of facial, cutaneous and subcutaneous structure.
Neural changes involving face: Peripheral nerves such
as facial and trigeminal nerve are frequently involved.
Figure 18.5 Macular lesion seen on leg in leprosy Facial paralysis is observed in 3 percent of cases, complete
the tooth diameter is suddenly and concentrically reduced,

while the less marked cases exhibit a well demarcated,
tapering and shortening of root. Transient interruption of
464 odontogenesis results in slight, circumferential hypoplasia
of enamel and cementum. In long standing lepromatous
leprosy invasion of the pulp by granulomatous tissue
causes pulpal necrosis leading to a pinkish discoloration of
crown. Anterior teeth are most commonly affected.
Facies leprosa: It is triad of consist of atrophy of anterior
nasal spine, atrophy of anterior maxillary alveolar ridge
and endonasal inflammatory changes.

The typical granulomatous nodules show collection of
Figure 18.8 Facial appearance in leprosy epithelioid cells and lymphocytes, multinucleated giant
(note the saddle nose) cells in a fibrous stroma. Langerhans type giant cells are
present.
or partial facial paralysis may occur either unilaterally
Vacuolated macrophages called lepra cells are scattered
or bilaterally. Paralysis of the orbicularis muscle results
throughout the lesion and often contain the bacilli.
in facial disfigurement, difficulties in phonation and
mastication as well as drooling. Involvement of trigeminal Diagnosis
nerve results in hypoesthesia and anesthesia.
Skin smears should be examined as a routine with ziehl-
Skeletal changes involving the face: Rhino maxillary nelson stain. The percentage of solid bacilli in a smear is
changes termed facies leprosa consist of atrophy of anterior known as morphological index. Skin, mucosal and nerve
nasal spine, atrophy and recession of the maxillary alveolar biopsy for histopathological examination are helpful in
process confined to the incisor region and endonasal doubtful cases.
inflammation. Destruction of facial cartilage and bone is Lepromin test is non-specific test to determine the
caused by direct infiltration and secondary infection. From hypersensitivity reaction and is useful in determining
nasal mucosa, granulomatous tissue invades cartilage and the immunological status of patient for classification of
bone resulting in nasal collapse and a specific chronic leprosy.
osteomyelitis. Collapse of the nose (saddle nose) is also
observed. Loss of anterior nasal spine and resorptive
changes of premaxilla occur only in later stage of the
disease.
Oral and dental lesions: It is more commonly seen
in lepromatous leprosy although clinical changes of
oral mucosa have been described in borderline leprosy.
Involvement of oral mucosa, premaxilla, soft palate, uvula,
tongue, gingiva and periodontium has been reported.
Lepromas: Small tumor like masses called lepromas
develop on the tongue, lips or hard palate. These nodules
have a tendency to break down and ulcerate.
Gingival hyperplasia with loosening of teeth has been
also reported.
Tooth changes: In association with severe and Figure 18.9 Leprosy showing epithelioid cells (EC) and giant
granulomatous infiltration of pre-maxilla in childhood, cells (GC)
Bacterial Infection
Management acquiring this disease. It can rarely be transmitted through

the placenta from the diseased mother to fetus.
Tuberculoid type of leprosy is treated with 6 months
Microorganisms may become disseminated by either
regimen of rifampin and dapsone. 465
blood stream or lymphatic spread. Infection can occur
Lepromatous leprosy is treated with 24 months of
through ingestion of unpasteurized infected cow milk.
rifampin, dapsone, and clofazimine.
Physiotherapy: This is given after lesion is resolve for Etiology
the reconstruction of damage.
Causative organism: The first member identified as tuber-
Points to Remember culous bacillus and designate as mycobacterium tubercu-
Mycobacterium leprae losis. Other microorganisms associated are Mycobacterium
• Tuberculoid type: Hypopigmented macules sharply bovis, Mycobacterium kansasii and Mycobacterium xenopi
demarcated, lesion circinate margin, lesions are and Mycobacterium malmoense. The organism is anaero-
densely anesthetic and, loss of eyebrows, eyelashes, bic, non-motile, Ziehl-Neelsen, rod shaped and is stained
peripheral nerve involvement with special Ziehl Neelsen stain.
• B
orderline type: Skin and nerve involvement, Constitutional factor: Low income group, low and
asymmetrical areas of hyperpigmentation, lesions unhygienic living conditions, malnutrition and overcrowding
are rubbery or soft are constitute for tuberculosis infection.
• Lepromatous leprosy: Erythematous macules, loss of
lateral portion of eyebrow, nasal stiffness, epistaxis, Pathogenesis
hoarseness, saddle nose, claw hand Initial tuberculosis usually occurs in lungs but occasionally
• Erythema nodosum leprosum: Tender, inflamed occurs in tonsil or alimentary tract. Patients primary
subcutaneous nodules, Leproma infection and the associated lymph node lesions heal and
• Orofacial manifestations: Lesions are macular or calcify. In some cases, caseous tuberculosis focus ruptures
raised, well-defined, hypopigmented, unhydrotic and into vein and produces acute dissemination throughout
hyperesthetic, facial paralysis, rhino maxillary changes the body, a condition called as acute miliary tuberculosis.
termed facies leprosa, oral and dental lesions, leproma Meningitis often complicates this condition. Progressive
on the tongue, gingival hyperplasia, tooth diameter is pulmonary tuberculosis may develop directly from a primary
suddenly and concentrically reduced, facies leprosa lesion or may occur following reaction of an incompletely
• Histopathological features: Collection of epithelioid healed primary focus. Postprimary pulmonary tuberculosis
cells, lymphocytes, multinucleated giant, Langerhans is a condition in which liquefied center of tuberculosis
type giant cells, lepra cells pulmonary infection is discharged into sinus. Extension of
• Management: Tifampin and dapsone. infection into pleura causes tuberculosis pleurisy.
Types
TUBERCULOSIS
• Miliary tuberculosis
It is a systemic infectious disease of worldwide prevalence • Pott’s disease
and of varying clinical manifestations. It is an infectious • Scrofula
granulomatous disease caused by acid fast bacilli Myco- • Primary tuberculosis
bacterium tuberculosis or rarely, Mycobacterium bovis. • Secondary tuberculosis.
Transmission and Prevalence
Types
Prevalence is more in the low income group with low
socioeconomic and unhygienic condition. Most infection Miliary tuberculosis: It spreads through blood stream
results from direct person to person spread through airborne and there is wide involvement of many organs like kidney,
droplet from patient with active disease. liver and is called as miliary tuberculosis.
The common cause of entry of the bacillus in body is Pott’s disease: If tubercular involvement of spine occurs
by inhalation. Infants and young children are at risk in in children, then it is called Pott’s disease.
Scrofula: If it spreads by lymphatics to lymph nodes, it is Scrofula: In glandular form of the disease, there is marked
called scrofula. enlargement of the cervical lymph nodes with caseation
and frequent breakdown of the gland. Such tuberculosis
466 Primary tuberculosis: It occur in unexposed people
infection is called ‘scrofula’. These types of infection
and involved lung. Primary infection results in localized
occur due contaminated milk from the cows.
fibrocalcified nodule at the site of involvement.
Cold abscess: The chronicity of the infection and the lack
Secondary tuberculosis: Reactivation of infection occur
of marked pain or acute inflammatory symptoms have
in compromised host.
resulted in the term ‘cold abscess’.
Clinical Features
Oral Manifestations
Symptoms: Patient may suffer episodes of fever and chills, They are relatively uncommon and seen in middle and
easy fatigability and malaise. There may be gradual loss of older age groups, as cleansing action of saliva and its
weight accompanied by persistent cough with or without antibacterial properties, in general also provide protection
hemoptysis. Local symptoms depend upon the tissue or against tubercle bacilli.
organs involved.
Location: Tongue is most commonly affected followed by
Consumption: This is wasting syndrome occur in palate, lips, buccal mucosa and gingiva. Majority of oral
tuberculosis with patient’s body was being consumed or lesions are secondary to infection in some other parts of
destroyed. body.
Tubercular lymphadenitis may progress to acute abscess
Tubercular ulcer: The lesion may be preceded by an
or remain as granulomatous lesion. In any case, swelling
opalescent vesicle or nodule, a result of caseation necrosis.
of neck is present which is tender, painful and often show
It breaks down into an ulcer which is usually superficial or
inflammation of the overlying skin. When abscess forms, it
deep and painful. It tends to increased slowly in size. In area
perforates and discharges pus (Fig. 18.10).
of trauma may be mistaken as traumatic ulcer or carcinoma.
Pulmonary tuberculosis: A persistence cough, hemo- Ulcers are nonspecific in their clinical presentation and
ptysis abundant sputum is usual features of pulmonary for this reason they are overlooked by the clinician. The
tuberculosis. There is also evening rise in temperature of typical tuberculosis lesion is an irregular lesion with
0.5° to 2°F, night sweats. ragged undermined edges, minimum induration and often
Lupus vulgaris: It is term used for the involvement of skin with yellowish granular base. The mucosa surrounding the
of the patient. ulcer is inflamed and edematous.
Primary lesion: It develop when bacteria are directly
inoculated in the oral tissue of a person who has not
acquired immunity to the disease. It involves gingiva, tooth
extraction socket and buccal fold.
Secondary lesion: Infection is carried in by hematogenous
route or through break in the tissue surface, is deposited in
the submucosa, subsequently proliferates and ulcerates the
overlying mucosa. It occurs more frequently in cases of
extra-pulmonary tuberculosis.
Sentinel tubercle: Tiny, single and multiple nodules called
‘sentinel tubercle’ may also be seen surrounding the ulcer.
At the mucocutaneous junction, tubercular ulcers
are usually extremely shallow with granulating base.
Crusting and oozing is seen when the lesion involves
Figure 18.10 Tubercular lymphadenitis showing swelling in adjacent cutaneous surface. They are usually painful. The
the submandibular region (Courtesy: Dr Chole) nodular form of tuberculosis presents as single or multiple
Bacterial Infection
nodules of variable sizes, which initially may appear a

semitransparent lesion of pinhead size. They are gray in
color and size of variable consistency.
467
Miliary tubercle: Miliary tubercle of oral mucosa are
occasionally seen in miliary tuberculosis which is a result of
acute dissemination of the infection through hematogenous
and lymphatic channel. The oral lesion appears as small,
gray tubercle with the tendency to break down and ulcerate.
Tubercle involvement of the periapical tissue and tooth
socket has been reported. The socket may be filled with so
called tuberculosis granulation tissue, consisting of many
small, pink and red elevations. Tuberculosis gingivitis is
an unusual form which may appear as diffuse, hyperemic
or nodular papillary proliferation.
Figure 18.11 Tuberculosis showing giant cells and
Jaw involvement: It may involve maxilla or mandible. lymphocyte with tubercle follicle
Jaw bone involvement occur as a result of either deep
extension of a gingival lesion, from infected extraction
socket, an extension of tubercular granuloma at the apex
of tooth or by means of hematological spread. It may result
in more diffuse form of osteomyelitis and is normally more
serous than infections from periapical lesion. Tubercular
involvement of jaw bone causes swelling and the symptoms
include difficulty in eating, trismus, paresthesia of the
lower lip and enlargement of the regional lymph nodes.
Fistulae drain either intraorally or extraorally often with
blue margins.
In some case destructive involvement may reach to the
TMJ area.
(Figs 18.11 to 18.14)
Areas of infection demonstrate the formation of granuloma, Figure 18.12 Tubercular granuloma presented (Courtesy: Dr
Sangmesh Halawar, Reader, Department of Oral Pathology,
which are circumscribed collection of epithelioid
CDCRI, Rajnandgao, Chhattisgarh, India)
histiocytes, lymphocytes and multinucleated giant cells.
There is also central caseous necrosis. This granuloma is
called tubercular granuloma. Tuberculin skin test: The Mantoux test is the preferred
skin test for detecting tuberculosis. It involves the injection
Diagnosis of 5 tuberculin units of purified protein derivatives,
Microscopy: A pulmonary TB suspect should submit 3 usually 0.1 mL intradermal. The skin test is read on the
sputum samples for microscopy. Morning sample is ideal. basis of millimeters of induration produced by PPD. Ten
to fifteen millimeters induration is required for the test to
Staining method: Ziehl-Neelsen, carbol fuschin be positive.
or kinjouncarbol fuschin have been use for staining
mycobacterium. Management
Polymerase chain reaction: This technique amplifies Eight weeks course of isoniazid, rifampin, and
even very small proteins of predetermined target region of pyrazinamide which is followed by 16 week course of
Mycobacterium tuberculosis complex DNA. rifampin and isoniazid.
ACTINOMYCOSIS
It is a chronic granulomatous suppurative and fibrous
468 type of disease caused by anaerobic, gram +ve, non-acid
fast bacteria. Most common are Actinomycosis israeli, A.
naeslundii, A. viscosus and A. odontolyticus.
The organism is considered to be transitional form
between bacteria and fungi. The term Actinomyces was
given by Harz to refer the ‘ray like appearance’ of the
organism in the granule. The breach in the continuity
of mucosa caused either by trauma or surgery, if the
prerequisite for majority of actinomycosis infections.
Types
Figure 18.13 Tuberculosis

• Cervicofacial
• Abdominal
• Pulmonary
• Cutaneous
• Central: Here, the infection is from the tooth or
its membrane and is accompanied by radiographic
changes.
• Peripheral: The peripheral types originate in the soft
tissues and do not involve bone.
Predisposing Factors
There are various predisposing factor which may lead to
actinomycosis. They are trauma, presence of carious teeth,
secondary bacterial invasion and hypersensitivity reaction.
Clinical Features
Cervicofacial Form
Figure 18.14 Langhans giant cell showing horse shoe shaped
arrangement of nuclei at periphery in tuberculosis Age and sex distribution: It is most common type of
actinomycosis and is commonly seen in adult males.
Cervicofacial actinomycosis infections are endogenous
in origin and occur when dental plaque, calculus or gingival
Points to Remember
debris contaminate the relatively deep wounds around the
Acid fast bacilli Mycobacterium tuberculosis, low mouth.
income group fever and chills, gradual loss of weight
persistent cough, consumption, tubercular lymphadenitis, Location: Submandibular region is the most frequent site
pulmonary tuberculosis, lupus vulgaris, scrofula, cold of infection. It usually spreads by direct tissue extension.
abscess, tubercular ulcer with ragged undermined Cheek and masseter region and parotid gland may also be
edges oral tissue, sentinel tubercle, miliary tubercle, involved.
tubercle involvement of the periapical tissue, diffuse Symptoms and signs: The classical signs are chronic, low,
form of osteomyelitis, TMJ involvement, formation grade persistence infection. Trismus is a common feature,
of granuloma, central caseous necrosis, tubercular before the formation of pus.
granuloma, tuberculin skin test, 8 weeks course of The first sign of infection is characterized by the
isoniazid, rifampin and pyrazinamide. presence of a palpable mass. Mass is wooden indurated
Bacterial Infection
area of fibrosis which later on form central softer area of Subcutaneous Form
fibrosis.
Infection result from traumatic transplantation of
There may associate changes detectable at the portal
organism, usually due to human bites. 469
of entry such as nonhealing tooth socket, exuberant
Lesion seen as subcutaneous swelling which enlarges
granulation tissue or periosteal thickening of the alveolus.
slowly softens and ruptures through the sinuses. Occasionally,
Development of fistula is common (Fig. 18.15). Skin
these lesions burrow through deeper tissue and invade bones.
surrounding the fistula is purplish. Adjacent tissues have
doughy consistency.
Oral Manifestations
Sulfur granules: Several hard circumscribed tumor like Location: Organism may enter the tissue through oral
swelling may develop and undergo breakdown, discharging mucous membrane and may either remain localized in the
a yellow fluid containing the characteristic submicroscopic adjacent soft tissue or spread to involve salivary glands,
sulfur granules. bone or skin of face and neck.
Abdominal Form Signs: It produces swelling and induration of tissue. It may
Abdominal actinomyces is extremely serious form of the develop into one or more abscesses, which tend to discharge
disease. upon the skin surface liberating pus, which contains typical
sulfur granules.
Cause: Trauma, due to surgery or other causes such as fish There may be nonhealing tooth socket, exuberant
bone or chicken bone injury, usually precedes the onset of granulation tissue and periosteal thickening of alveolus.
the disease. Skin overlying abscess is purple red and indurate or
Symptoms: Generalized symptom of fever, chills, fluctuant. It is common for sinus, through which the abscess
vomiting, develop followed by symptoms of involvement has drained, to heal but due to chronicity, new abscesses
of organs, such as liver and spleen. are formed and perforate through skin surface. There is
disfigurement of face. Infection may involve maxilla and
Sign: There is palpable abdominal mass. Intestinal mandible.
manifestations develop later. There is formation of periapical granuloma.
On the tongue, the lesion is a painful nodule which
Pulmonary Form
eventually ulcerates. Untreated cases may reach to the
It produces findings such as fever and chills, accompanied point where the tongue may become fixed.
by a productive cough and pleural pain.
Pleural invasion resulting in empyema and there may Tonsillar hyperplasia: Involvement of tonsillar crypts
be formation of sinus. produce hyperplasia of tonsil secondary actinomycosis
infestation of the crypts.
Actinomycotic osteomyelitis of mandible and maxilla
can occur. It is described as ill-defined radiolucency
surrounded by radiopacity.
(Figs18.16 to 18.19)
Ray fungus appearance: Round or lobulated colony
meshwork of filaments stain with hematoxylin and
peripheral club shaped ends of filaments stain with eosin.
The typical lesion, whether in the soft tissue or in bone,
is granulomatous one showing central abscess formation
within which there are characteristic colonies of micro-
organisms are present.
Colonies consist club shaped filament that form
Figure 18.15 Fistulae seen in actinomycosis patient radiating rosette pattern.
470
Figure 18.16 Actinomycosis showing central abscess Figure 18.18 Actinomycosis

formation (sulfur granules)
Figure 18.17 Actinomycosis colony (AC) in a sea of Figure 18.19 Actinomycosis (low power)
lymphocytic infiltration (IC)
supply to the affected region and hence makes penetration

Colonies appear to be floating in a sea of polymorpho- of antibiotics difficult. Therefore, two fold therapy
nuclear leukocytes, often associated with multinucleated including antibiotics and surgery is necessary.
giant cells and macrophages. Antibiotics which are used are penicillin, amoxicillin,
and tetracycline. Cervicofacial actinomycosis require
Management course of 5 to 6 weeks while deep seated infection require
Actinomyces infection produces a reactive inflammatory course of 12 months.
response which causes an area of necrosis and scar tissue Surgical excision of sinus tract with drainage of abscess
around the abscess. This results in decrease in the vascular should be done.
Bacterial Infection
Common sites are areas of stagnation around fixed bridge

Points to Remember
or crown.
Anaerobic, gram +ve, nonacid fast bacteria Actinomy-
cosis israeli First sign: The commencement of gangrene is denoted by 471
• Cervicofacial form: Endogenous in origin, submandi- blackening of skin. Small ulcers of gingival mucosa spread
bular region, chronic, low, grade persistence rapidly and involve the surrounding tissues of jaws, lips and
infection, trismus, palpable mass, sulfur granules cheeks by gangrenous necrosis. This is called necrotizing
• Abdominal form: Serious form, generalized symptom ulcerative mucositis.
of fever, chills, vomiting, palpable abdominal mass Symptoms: Odor is foul. Patients have high temperature
• Pulmonary form: Fever, chills, productive cough, during the course of the disease, suffer secondary infection
pleural pain. and may die from toxemia or pneumonia.
• Subcutaneous form: Traumatic transplantation of Overlying skin is inflamed, edematous and finely
organism, subcutaneous swelling necrotic which results in formation of line of demarcation
• Oral manifestations: Swelling and induration between healthy and dead tissue.
of tissue, exuberant granulation tissue, red skin, In advanced stage, there is blue-black discoloration of
periapical granuloma, painful nodule on tongue, the skin. As gangrenous process advances, slough appears
tonsillar hyperplasia, actinomycotic osteomyelitis of and soon separated, leaving a perforating wound in the
mandible involved area. Large masses may be sloughed out leaving
• Histopathological features: Ray fungus appearance, the jaws exposed. Infection spread through anatomic
central abscess formation, club shaped filament, barrier such as muscle (Fig. 18.20).
radiating rosette pattern, polymorphonuclear leuko- Noma neonatorum is arises in first month of life in low
cytes, multinucleated giant cells, antibiotics, surgery. birth weight infants who also demonstrate malnutrition and
debilitating illness.
NOMA
Management
It is also called ‘Cancrum oris’, ‘gangrenous stomatitis, Parenteral fluid should be given urgently to correct
‘orofacial gangrene’ and necrotizing stomatitis’. dehydration and electrolyte balance.
It is rapidly spreading gangrene of oral and facial Penicillin and metronidiazole are first line of antibiotics
tissues occurring usually in debilitated or nutritionally which are used in case of noma.
deficient person. The word noma is derived from Greek Conservative debridement of necrotic area should also
word nomein meaning to devour. be carried out.
It is cause by Fusobacterium necrophorum and
Prevotella intermedia. Other organism which are associated
with noma are Borrelia vincentii, Porphyromonas
gingivalis and Staphylococcus.
It occurs in persons who are undernourished, debilitated
from infections such as diphtheria, dysentery, measles, and
pneumonia, scarlet fever, syphilis, tuberculosis and blood
dyscrasias.
It can also occur due to excessive mechanical injury,
infection by Vincent’s organisms, leukemia, sickle cell
trait, stress and chemotherapeutic agents can cause noma.
Clinical Features
Age: It is seen chiefly in children, but can be found in Figure 18.20 Blackish discoloration with ulceration seen in
adults in certain conditions like in malnourished states. case of gangrenous stomatitis (Courtesy: Dr Tapasya)
erythema giving skin of trunk and extremities sandpaper

Points to Remember
texture. After 3 to 4 days, the rash fades. This rash is due
Cancrum oris, Fusobacterium necrophorum, Prevotell to toxic injury to the vascular endothelium which produces
472 intermedia, blackening of skin, necrotizing ulcerative dilation of the small blood vessels and consequent
mucositis, skin is inflamed, edematous, necrotic, blue- hyperemia.
black discoloration of the skin, noma neonatorum,
penicillin, metronidiazole. Pastia’s line: These are transverse red streaks occur in
the skin fold secondary to capillary fragility in the zone of
stress.
SCARLET FEVER After rash is fade desquamation of face produce small
It is also called ‘scarlatina’. It is predominately occurs in flakes and that of skin produce thicker and larger flakes.
children during winter months, caused by infection with Complication like retropharyngeal abscess, sinusitis,
group-A streptococci of beta hemolytic type that elaborate pneumonia, otitis media, acute rheumatic fever, glomeru-
erythrogenic toxins. lonephritis, arthralgias, meningitis and hepatitis can
occur.
Clinical Features
Oral Manifestations
Incubation period is 3 to 5 days.
‘Stomatitis scarlatina’ accounts for the chief oral
Sign and symptoms: The desquamation of skin begins manifestation of scarlet fever. Mucosa of palate may
around the middle of the second week after the onset. appear congested and throat is often fiery red. The face,
Patient exhibits severe pharyngitis and tonsillitis, chills, especially the temples and cheeks are flushed and red,
fever and vomiting. but pale area of circumoral pallor is often seen around the
Throat becomes highly erythematous and exudation is mouth. The tonsils and faucial pillars are usually swollen
common. and sometimes covered with grayish exudate.
There may be enlargement and tenderness of regional
lymph nodes. White strawberry tongue: Tongue exhibits white coating
Characteristic diffuse, bright scarlet to dusky red skin and fungiform papillae are edematous and hyperemic,
rash which appears on the second or third day of the illness projecting above the surface as small red knobs and it is
(Fig. 18.21). It is described as ‘sunburn with goose pimple’. called as ‘strawberry tongue’.
Pinhead punctuates areas of normal color project through Red raspberry tongue: Coating of tongue is soon lost,
beginning at the tip and lateral margins and the organ
becomes deep red, glistening and smooth, except for
swollen hyperemic papilla. The tongue in this phase is
called ‘raspberry tongue’.
At the height of skin eruption oral mucosa is uniformly
congested and the breath is fetid. In severe cases of scarlet
fever, ulceration of buccal mucosa and palate have been
reported. In some cases hypoplasia of teeth is seen in
permanent teeth, if conditions occur at the time of tooth
developments.
The pharyngeal mucosa may display superficial necrosis,
pseudomembranous exudate and microbial colonies.
The submucosa will evidence an acute or subacute
inflammatory cell infiltration. Tongue will show vasodi-
Figure 18.21 Rash of scarlet fever seen on the skin of the lation with inflammatory cell infiltration, being non-
hand (Courtesy: Dr Pincha) specific mucositis.
Bacterial Infection
Management Symptoms and sign: There is restlessness, malaise,

headache, fever and occasional vomiting. Within a short
Antibiotics: Penicillin is the drug of choice, since group
time, patient complaint of sore throat. Mild redness and
A streptococci are generally, highly sensitive to this 473
edema of pharynx with cervical lymphadenopathy occur.
antibiotics.
Nasal regurgitation of liquids during drinking.
The species are also sensitive to other antibiotics like
erythromycin, tetracycline and Chloramphenicol. Bull neck: There may be swelling of the neck, called ‘bull
neck’.
Points to Remember Larynx is edematous and is covered by pseudo-
Scarlatina, group-A streptococci of beta hemolytic type, membrane. It produces a mechanical respiratory obstruction
throat erythematous, bright scarlet to dusky red skin rash, and typical croup.
sunburn with goose pimple, sandpaper texture, Pastia’s There is generalized polyneuritis with weakness;
line, retropharyngeal abscess, sinusitis, pneumonia, sto- paresthesia may follow in the next 10 to 14 days.
matitis scarlatina, white strawberry tongue, red raspberry Cutaneous diphtheria is characterized by chronic skin
tongue, ulceration of buccal mucosa, superficial necrosis, ulcer.
pseudomembranous exudate, microbial colonies, inflam-
Oral Manifestations
matory cell infiltration, vasodilation, erythromycin, tet-
racycline. There is formation of patchy ‘diphtheritic membrane’
which begins on tonsils and enlarges, becoming confluent
over the surface. It is thick and grayish in color. It tends to
DIPHTHERIA adhere and leave a raw bleeding surface on removal. After
It is an acute contagious disease caused by gram +ve sometime this membrane develop patches of green and
bacillus Corynebacterium diphtheriae, also called klebs black necrosis.
loeffler bacillus. It is transmitted by droplet infection or A soft palate temporary paralysis usually during 3rd
direct contact. and 5th week of the disease occurs. The paralysis usually
disappears in a few weeks or few months, at the most.
Pathogenesis Diptheric membrane covering may involve entire soft
The portal of entry is the upper respiratory tract and rarely palate, uvula, larynx or trachea resulting in stridor and
skin, genitalia, eye and middle ear. The bacilli settle on the respiratory difficulty.
mucous membrane or upper respiratory tract and lead to
Prevention
inflammation and necrosis of mucosal cells. The infection
may spread to adjacent areas. In the primary invasive region, The disease may be prevented by prophylactic active
it forms a thick, firm, leathery, blue white pseudomembrane immunization with diphtheria toxoid.
composed of bacteria, necrotic epithelium, macrophages Antitoxin should be administration in combination with
and fibrin. A narrow zone of inflammation surrounding antibiotics to prevent further toxin production. Antibiotics
the area is seen. When the diphtheria bacteria multiply in used are erythromycin, procaine penicillin, IV penicillin.
the local tissues, they produce powerful exotoxins. This
exotoxin diffuses through the body through a hematogenous Management
route. Heart, muscle, kidney, peripheral nerves and adrenal The patient should be isolated and bed rest is very essential.
glands are thus involved. Death may be caused by heart
failure, airway obstruction which is cause by edema or by Points to Remember
the effect of toxin. • C orynebacterium diphtheriae, klebs loeffler bacillus,
listlessness, malaise, headache, fever, sore throat
Clinical Features • Bull neck, larynx is edematous, polyneuritis, diph-
theritic membrane, green and black necrosis, soft
Age and incubation period: It occurs most frequently
palate temporary paralysis, stridor, active immuni-
in children, during the fall and winter months. Incubation
zation, antitoxin.
period is two days.
TULAREMIA Laboratory Diagnosis

Serology: An agglutination test is used to demonstrate
It is caused by gram negative bacillus Francisella
474 a rising titer of antibody in the serum of patients of
tularensis. also known as rabbit fever, deer fly fever. It
F. tularensis.
is contacted through infected rabbits, muskrats, ground
squirrels and other wild germ, particularly of rodent Skin testing: Intradermal injection of an extract of F.
family. tularensis gives a positive delayed hypersensitivity reaction
in 1st or 2nd week of illness.
Types
Management
• Cutaneous
• Ophthalmic Antibiotics of choice are streptomycin and tetracycline,
• Pleuropulmonary either alone or in combination.
• Oral
Points to Remember
• Abdominal
Francisella tularensis, sudden headache, nausea,
Clinical Features suppurative ulcer, conjunctivitis, on soft palate, fibrinous
pseudomembrane, generalized stomatitis, grayish
Incubation period: Incubation period is up to seven days. white membrane, regional lymphadenitis, skin testing,
Symptoms: Patient usually suffers a sudden headache, streptomycin, tetracycline.
nausea, bony pain, profuse sweat, vomiting, chills and
fever. RHINOSCLEROMA
Signs: A single cut or sore on the skin develops into It is unusual chronic infection caused by bacillus Klebsiella
a suppurative ulcer. Lymphatic vessels become swollen rhinoscleromatis and it is common in Europe and central
and painful and the lymph nodes are remarkably and South America. Rhinoscleroma is a chronic, slowly
enlarged. progressive, inflammatory disease of the upper respiratory
The eyes also become involved with conjunctivitis tract. Rhinoscleroma is found predominantly in rural areas
developing through localization of disease in the with poor socioeconomic conditions.
conjunctival sac.
Clinical Features
Oral Manifestations Age: It is common between the ages of 20 to 40 years.
Primary infection of the mouth usually occurs from eating
Location: Usually found in upper respiratory tract, often
infected meat.
originating from nose, but involvement of lacrimal glands,
Location: It is common on soft palate, tongue gingiva and orbit, skin, paranasal sinus and intracranial invasion have
angle of mouth. It is usually accompanied by sever pain. been describe.
Appearance: The ulcer is shallow with whitish fibrinous Rhinitis stage: In this stage, there is nasal obstruction,
pseudomembrane, but may extend more deeply with nasal deformity and epistaxis. There is also swelling of
superinfection of Vincent’s organism. upper lip and sore throat.
There may be generalized stomatitis develops.
Infiltrative stage: It is characterized by nasal obstruction,
Single nodular masses eventually develop into abscess.
due to the presence of exuberant granulation tissue.
The tonsil, posterior pharyngeal wall, soft palate, base
Hoarseness results due to laryngeal involvement. Anesthesia
of the tongue and buccal mucosa may be covered by a
of soft palate is common in this stage.
grayish white membrane simulating the appearance of
diphtheria. Nodular stage: The proliferation of nasal masses may
Regional lymphadenitis may arise in sub-maxillary and produce configuration known as ‘Hebra nose’. Posterior
the cervical groups of nodes. Cervical lymph nodes are extension of the lesion may produce laryngeal and tracheal
tender, enlarged and may suppurate. obstruction of varying degrees.
Bacterial Infection
Complications include scleromatous infiltration of Inguinal ulceration is commonly secondary to the

eustachian tube and unilateral scleroma of maxillary sinus. genital lesion and arises initially as fluctuant swelling
known as pseudo-bubo. Metastatic spread to bone and soft
Oral Manifestations subcutaneous tissue has been reported. 475
Oral lesion appears as proliferative granulomatous lesion
of lip, soft palate and tonsil. There is also anesthesia soft ORAL MANIFESTATIONS
palate and enlargement of uvula. Taste sensation is also
Oral lesion appears to be the most common extragenital
impaired.
form of granuloma inguinale. Oral lesions occur either
Histopathological Features as a result of autoinoculation through infected fingers or
through oral coitus.
Epithelial squamous metaplasia with subepithelial infiltrate
of polymorphonuclear cells and granulation tissue are seen. Location: It is most commonly found on lips, buccal
There is presence Mikulicz cells and Russell bodies. mucosa or palate or they may diffusely involve the mucosal
surface.
Management Lesions of lip are characterized by extensive superficial
Antibiotics like ciprofloxacin, doxycycline, and fluoroqui- ulceration with well-defined elevated, granulomatous
nolones can be given. Usually combination therapy is ad- margins.
vice. It may be of three types, i.e. ulcerative, exuberant and
Surgical debridement could also be considered if there cicatricial.
is significant airway obstruction or cosmetic deformity. Ulcerative: It is painful sometimes bleeding is associated
with it.
Points to Remember
Bacillus Klebsiella rhinoscleromatis, upper respiratory Exuberant: It appears as proliferative granular mass with
tract, rhinitis stage, infiltrative stage nasal obstruction, an intact epithelial covering. The mucous membrane is
nodular stage Hebra nose, scleromatous infiltration of inflamed and edematous.
eustachian tube, proliferative granulomatous lesion Cicatrical: Fibrous scar formation may be come extensive.
of lip, anesthesia soft palate, impaired taste sensation,
epithelial squamous metaplasia, polymorphonuclear Histopathological Features
cell, mikulicz cells, fluoroquinolones, ciprofloxacin, Donavon bodies present in smears which are recognized
doxycycline. as large, gram negative oval bacteria with intense bipolar
staining (safety pin appearance).
GRANULOMA INGUINALE There is granulation tissue with infiltration of
polymorphonuclear leukocytes and plasma cells. There is
It is also called ‘granuloma venereum’, ‘donovanosis’. It marked overlying pseudoepitheliomatous hyperplasia.
is found in inguinal and anogenital region and is caused
by Donavan granulomatosis and is a chronic slowly Management
progressing, mildly contagious disease. The recommended antibiotic for granuloma inguinale is
either trimethoprim/sulfamethoxazole or doxycycline. Alter-
Clinical Features
natives include ciprofloxacin, erythromycin, or azithromycin.
Age and incubation period: It chiefly affects adult black The antibiotic should be given for at least a 3-week.
of either sex, but may occur in any race. Although the exact
incubation period for granuloma inguinale is unknown, it Points to Remember
ranges from a day to a year, with the median time being Granuloma venereum, donavonosis, small papule that
50 days. ulcerates, inguinal ulceration, autoinoculation through
Appearance: Lesion begins as a small papule that ulcerates, infected fingers, oral coitus, lesions of lip, ulcerative,
increases in size and eventually gives rise to velvety, beefy, exuberant, and cicatrical types, donavon bodies, safety
granulating and spreading ulcerative lesion of inguinal and pin appearance, infiltration of polymorphonuclear
anogenital region. leukocytes, trimethoprim or sulfamethoxazole.
STREPTOCOCCAL TONSILLITIS AND TONSILLAR CONCRETION AND

PHARYNGITIS TONSILLOLITHIASIS
476 It is caused by a, b hemolytic streptococci. Viruses can also Usually tonsil demonstrated crypts which are deep to
cause this disease. They are Epstein Barr virus, adenovirus, increase surface area for interaction between immune cells
and enteroviruses. of lymphoid tissue and oral environment. These crypts
Spread of this infection occur by person to person filled with keratin and foreign material which sometime
contact, from children to parent by means respiratory become mass of foul smelling material. This is called
droplet and oral secreation. tonsillar concretion. If there is calcification occur it is
called tonsillolith.
Clinical Features
Age: It is commonly seen children of 5 to 15 years of age: Clinical Features
Age and sex distribution: It can be seen in childhood to
Sign and symptoms: There is sudden onset of sore
old age. Male affected more commonly than female.
throat, fever, dysphagia, tonsillar hyperplasia, cervical
lymphadenopathy and yellowish tonsillar exudate. Sign and symptoms: Patient complaint of foul smell. In
There is also beefy red and swollen uvula, excoriated some cases there may be recurrent infection leading pain,
nares and scarlatiniform rash (Fig. 18.22). ulceration, chronic sore throat, irritable cough, and otalgia.
There is sensation of foreign object seen in the throat
Management which get relieved after removal of tonsillar plug.
It is self-limiting and resolve in 3 to 4 days. Radiographic feature: On panoramic radiograph
Penicillin is drug of choice as group A streptococci are radiopaque object is superimposed on the midportion of
sensitive to it. mandibular ramus.
Other antibiotics like cephalosporin drug (cephalexin,
cefadroxl and cefouroxime) can also be given. Management
Points to Remember They are not treated unless significant hyperplasia of tonsil
is present.
a, b hemolytic streptococci, sore throat, fever, dysphagia, Local excision can be done of deeper tonsillolith. If
beefy red and swollen uvula, penicillin. there is evidence of recurrent tonsillitis tonsillectomy can
be performed.
Points to Remember
Foul smell, sore throat, irritable cough, radiopaque
object, local excision, tonsillectomy.
LYMPHOGRANULOMA VENEREUM
It is a venereal disease caused by one of the three strains of
Chlamydia trachomatis.
Clinical Features
Symptoms: There may be fever, chills, headache and
malaise.
It persists as firm, tender enlargement of inguinal lymph
nodes. Nodes are tender and adherent to the underlying
Figure 18.22 Swollen uvula seen in streptococcal pharyngitis tissues.
Bacterial Infection
Enlargement of lymph nodes both above and below the

Types
inguinal ligament, is characteristic feature called ‘groove
sign’. • Cutaneous or dermal myiasis: It affects skin.
Overlying skin becomes reddened and dusky and • Myiasis of external orifices: It includes nasal, oral, 477
multiple purulent fistulae develop over enlarged glands. vaginal and anal myiasis.
In females, placenta is frequently involved. Marked • Myiasis of internal organs: It includes intestinal
scarring and local edema frequently develops secondary to and urinary myiasis.
suppurative lymphadenitis. • Accidental myiasis: Larvae ingested along with food.
• Semi specific: When larvae are laid on necrotic
Oral Manifestations tissue.
• Obligatory: When larvae affect undamaged skin.
It results due to orogenital contact or anti-inoculation.
Tongue is the most common site.
Clinical Features
Appearance: Lesion consists of small, slightly painful
It is characterized by papular or migratory lesions. Papular
superficial ulceration with non-indurated borders which
lesions are itchy and occasionally painful.
appear on lip. In long standing infection, there is zone
Open lesion may produce serious discharge and larvae may
of cicatrical refraction, dark red area with loss of
be detected through the opening (Figs 18.23 and 18.24).
superficial epithelium, or opaque lichenoid grayish
Migratory lesions are superficial, red tunnel like
papules.
lesions, which form creeping eruptions.
Dysphagia, red soft palate and small red granulomatous
lesions accompanied by regional lymphadenopathy, are Oral Manifestation
commonly associated symptoms.
Cervical lymphadenopathy is present. Skin covering Appearance: It presents as an erythematous, edematous or
the swollen nodes is violaceous and indurated usually with granulomatous lesion.
one or more draining sinuses. Symptoms: Itching or pain may be present. These lesions
pulsate with movement of larvae.
Management
Antibiotics which are given are doxycycline, erythromycin,
and azithromycin.
Points to Remember
Chlamydia trachomatis, enlargement of inguinal lymph
nodes, groove sign, cicatrical refraction, dark red area
with loss of superficial epithelium, cervical lymph-
adenopathy, doxycycline, erythromycin.
MYIASIS
It is referred to the invasion of living tissues by the larvae
of certain species of flies. Oral myiasis is a rare condition
that refers to the invasion of tissue of the oral cavity by
fly larvae. The term myiasis (Greek: myia= fly, iasis =
disease) was coined by Hope in 1840. Figure 18.23 Larvae seen in lesion of oral cavity
centimeter in diameter. The overlying skin may be inflamed.

The lymph nodes gradually become soft and fluctuant,
owing to necrosis and suppuration. The lymphadenopathy
478 may persist for one to six months.
Symptoms: In early stage, low grade fever, headache,
chills, nausea, malaise or even abdominal pain may occur.
Other manifestations include nonpruritic macular or
maculopapular rash, parotid swelling, conjunctivitis and
grad mal seizures.
Oculoglandular syndrome of parinuad: Primary lesion
of eye can result in conjuctival granuloma with preauricular
lymphadenopathy. This occur when person touches fur
moistened with cat saliva during grooming. After this if
Figure 18.24 Oral myiasis showing detachment of palate
individual rub eye this syndrome results.
and larva in the palate In dental point of view there may be involvement of
preauricular, submaxillary or cervical chain of nodes.
Signs: An opening is present from which larvae can come Histopathological Features
to surface of the lesion.
Involved lymph nodes manifest reticuloendothelial
Management hyperplasia, focal granuloma, suppuration and necrosis
with capsular thickening. Necrosis is surrounded by band
Surgical removal of larvae and irrigation of the tissue with of histiocytes and neutrophils.
hydrogen peroxide is effective method of choice. Epithelioid cells and multinucleated giant cells are
occasionally seen.
Points to Remember
In some cases necrosis is absent but areas of karyorrhexis
Larvae, open lesion serious discharge larvae may be are present with proliferation of plump vascular channels
detected, migratory lesions, itching or pain, hydrogen which also exhibits thickened eosinophils walls.
peroxide. Organism can be demonstrated by immunoperoxidase
technique.
CAT SCRATCH DISEASE
Management
It is also called ‘cat scratch fever’. Disease is recognized in
The disease has a benign course and treatment is
1931 but the exact cause is unknown.
symptomatic including aspiration of the nodes, if it
Viral as well as bacterial agents have been proposed
becomes necessary. The application of local heat and
as the cause of this disease. But in 1988 the causative was
analgesic should be carried out.
named which initially called Rochalimaea henselae but
In case of severe and infection of immunocompromised
was reclassified as Bartonella henselae.
patient antibiotics like azithromycin, erythromycin,
Clinical Features doxycycline, rifampin, ciprofloxacin and gentamicin can
be used.
Age and incubation period: The incubation time of the
disease ranges from 3 to 30 days. It occur at any age, but Points to Remember
predominant in children and young adults. Rochalimaea henselae, after traumatic break in skin due
It is thought to arise after traumatic break in skin due to scratch or bite of household cat, lymphadenitis, low
to scratch or bite of household cat. Within few days of grade fever, headache, chills, oculoglandular syndrome of
indolent primary lesion, often papules or vesicle develop at parinuad, preauricular, submaxillary or cervical chain of
the site of injury. nodes, reticuloendothelial hyperplasia, focal granuloma,
Lymphadenitis: Within one to three weeks, lymphadenitis suppuration, histiocytes, neutrophils, epitheloid cells,
develops. The nodes are painful and may be several karyorrhexi, azithromycin, erythromycin, doxycycline.
Bacterial Infection
PYOSTOMATITIS VEGETANS Management

It is an uncommon inflammatory disease of the oral cavity. It is not specific. It is found that the oral lesions are regressed
The name is given as there is a similarity between it when the intestinal disturbance is brought under control 479
and the skin lesions in a dermatologic disease known as with the help of sulfasalazine or systemic corticosteroids.
pyodermatitis vegetans.
Points to Remember
Pyostomatitis vegetans is an inflammatory stomatitis
and most often seen in association with inflammatory Broad base papillary projections, snail tract ulceration,
bowel disease, namely ulcerative colitis and Crohn’s buccal mucosa show ‘cobblestone’ appearance, intact
disease. It may occur due to intestinal disturbances. stratified squamous epithelium, loose connective
tissue, plasma cells, lymphocytes, polymorphonuclear
Clinical Features leukocytes, focal areas of degeneration, microabscess
Location: This lesion may occur in any area of oral cavity, formation, sulfasalazine systemic corticosteroids.
except in tongue. It is multiple in numbers. It is painless.
Appearance: Oral lesions consist of large number SINUSITIS
of broad base papillary projections, tiny abscess or Inflammation of the mucosa of the paranasal sinuses is
vegetations developing in areas of intense erythema. referred to as sinusitis. When maxillary sinus is involved
These small projections are red or pink in color, but on it is called maxillary sinusitis. When all the sinuses are
careful examination may show tiny pustules beneath involved it is called a pansinusitis.
the epithelium, which liberate a purulent material when The most common organism responsible for acute
ruptured. This is called ‘snail tract’ ulceration. sinusitis is Streptococcus pneumonia, Haemophilus
When, condition is present, buccal and labial mucosal influenzae and Moraxella catarrhalis.
lesions have many folds and papillary projections may Oraganism responsible for chronic sinusitis are
develop on these folds. Yellow vesicles discharged small Streptococcus, Bacteroides or Veillonella spp. If sinusitis
amount of purulent material. These leave ulceration, which occur secondary to odontogenic infection causative
may coalesce into larger areas of necrosis. organism is Peptostreptococcus spp., Fusobacterium spp.,
Buccal mucosa show ‘cobblestone’ appearance, while and Prevotella spp.
vestibular lesions appeared as folds and ulcers, the lips
are diffusely swollen and indurated, gingival and alveolar Etiology
mucosal lesions are granular with erythematous swelling • Periapical infection from the teeth
and palatal lesions appear as multiple aphthous ulcers. • Oroantral fistula
• Periodontitis
Histopathological Features • Traumatic
The papillary projections generally show an intact • Dental material in the antrum
stratified squamous epithelium with an underlying loose • Implant
connective tissue, which is generally densely infiltrated by • Infected dental cyst
large number of plasma cells, lymphocytes and occasional • Common cold
polymorphonuclear leukocytes. • Allergic rhinitis
It is arranged in pattern of small abscess or spreads • Deviated nasal septum
diffusely throughout the tissue. Tiny areas of focal necrosis • Direct bacterial contamination
and micro abscess formation, either intraepithelial or sub- • Immune deficiency
epithelial, are common features of this lesion. • Influenza
Focal areas of degeneration and necrosis of the • Blood borne infection
overlying epithelium are present. • Disease of maxillary sinus
Etiology during diving, when pressure changes in the nose force

nasal secretion into the sinus.
Dental Causes
480 Periapical infection from the teeth: It may follow dental Immune deficiency: Sinusitis can occur in immune
infection particularly from upper molar and premolar deficiency state, like leukemia, lymphoma and AIDS.
teeth. The anatomic proximity of the roots of the maxillary Influenza: It can also occur in influenza when bacteria
bicuspids and molar teeth to the floor of the sinus leads to invade as secondary microorganisms.
potential infection of the sinus by direct extension of an
Blood borne infection: It can also occur in some cases of
apical abscess.
blood borne infection.
Oroantral fistula: The accidental opening in the floor
Disease of maxillary sinus: Benign mucosal cyst or tumors
of the maxillary sinus during dental extraction is called
of the maxillary sinus can also lead to maxillary sinusitis.
oroantral opening.
Periodontitis: It may spread from a deep pocket of Clinical Features
marginal periodontitis. Acute Maxillary Sinusitis
Traumatic: Injury of facial bones especially nasal bones Symptoms: The main symptom is severe pain which is
and malar bones. constant and localized. Pain may be felt in the area of
Dental material in the antrum: Perforation of endodontic eyeball, cheek and frontal region. Pain may be exacerbated
filler substance. If root canal is overfilled then there are by stooping or lowering the head. Postnasal drip causing
more chances of gutta purcha point to be inserted into the irritation, stuffiness and nasal discharge. Pain may be
maxillary sinus. referred to various areas including the teeth, orbit, head and
ear. Pain in the teeth may be referred to the premolars and
Implant: Implants are used in the upper edentulous jaw to molars on the affected side. Pain is increased by biting on the
aid the retention of dentures or bridges or replace missing affected side but unaffected by drinking hot, cold or sweet
teeth. Implants are also used when there is insufficiency of fluids. Teeth in the involved side become sore and painful.
bone to support the denture. In these cases as bone is thin Difficulty in breathing is common. Generalized toxemia
implant can penetrate the nose or sinus. develops, i.e. fever with chills, dizziness, malaise and nausea.
Infected dental cyst: Cyst which have become infected Nasal discharge is watery in the beginning but later
and involve the maxillary sinus can also cause sinusitis. becomes mucopurulent. The nasal mucosa of the anterior
nares may show reddening and inflammation and there
Nondental Causes may be presence of pus. In cases of sinusitis from infected
teeth, the discharge has a foul odor.
Mechanical Obstruction of Ostium
There is tenderness to pressure or swelling over the
Common cold: It is most common cause of mechanical involved sinus. Since, the anterolateral and posterolateral
obstruction of ostium. It will produce inflammatory edema sinus walls are thinnest in the area above the tooth root,
of the nasal mucosa which obstruct the antronasal duct and thumb pressure on the cheek here is the best way to elicit
cause mucus to accumulate in the sinus. Trapped mucous tenderness externally. Premolar and molars on the affected
becomes secondarily infected by local commensal bacteria. side may be sensitive to percussion.
Allergic rhinitis: It may cause maxillary discomfort due to Intraorally, there may be a mucobuccal swelling,
edema round the ostium and retention of secretion. reddening and pain near the sinus region.
Other conditions: Deviated nasal septum, presence of Chronic

nasal polyp and prolonged nasotracheal intubation can
Chronic sinusitis is recurring episode of acute sinusitis or
cause stagnation and relative anaerobiasis.
symptomatic sinus disease lasting longer than 3 months.
Direct bacterial contamination: Infected material may It develops as a result of neglected and overlooked
also be introduced directly by jumping or hydrosliding feet dental focus of infection. The lining becomes thicker and
first into contaminated water without holding the nose or irregular.
Bacterial Infection
General symptoms of chronic sinusitis include sense lymphocytic infiltration of the lining tissue with squamous
of tiredness, low grade fever and feeling of being unwell. metaplasia of the epithelium.
Stuffy sensation over the affected side of the face is present.
Nasal obstruction, nasal discharge and headache are the Management 481
related symptoms. Removal of the cause: Removal of dental infection and
other causes which are causing sinusitis.
Antibiotic: Antibiotics of choice are amoxicillin,
There is increase radiodensity in the maxillary sinus (Fig.
amoxicillin-clavulanate, trimethoprim, sulfamethoxazole.
18.25).
Other therapy like analgesic, nasal decongestant, steam
Lab Findings inhalation can be used.
In case of chronic sinusitis surgical management like
Elevated leukocyte count is seen. antral lavage, antrostomy and transnasal endoscopic
Histopathological Features surgery is performed.
Acute Sinusitis Points to Remember

The lining of the maxillary sinus may show a typical acute Pansinusitis. Streptococcus pneumoniae, Haemophilus
inflammatory infiltrate with edema of the connective tissue influenzae
and often hemorrhage. • Acute: Postnasal drip, severe pain, teeth in the
Sometimes squamous metaplasia of the specialized involved side become sore and painful, nasal
ciliated columnar epithelium occurs. discharge, reddening of nasal mucosa, tenderness to
pressure, premolar, molars sensitive to percussion,
Chronic Sinusitis
acute inflammatory infiltrate with edema of the
The fundamental pathologic changes are that of cellular connective tissue and squamous metaplasia.
proliferation. The mucosal lining of the maxillary sinus • Chronic: Sense of tiredness, low grade fever, feeling
shows remarkable thickening and the development of of being unwell, increase radiodensity in the maxillary
numerous sinus polyps. sinus, cellular proliferation, thickening and the
Polyps are simply hyperplastic granulation tissue development of numerous sinus polyps, hyperplastic
with lymphocytes and sometimes plasma cell infiltration. granulation, ciliated columnar epithelium.
Tissues covered by ciliated columnar epithelium tend to fill • Management: Analgesic, decongestant, steam inhala-
the sinus and obliterate it and in some cases there is mild tion, antral lavage, antrostomy.
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Bacterial Infection
1. Red itchy spots affecting face with round or oval 6. Leonine facies, pedunculus earlobe is the feature of 483
pustular vesicles caused by S. pyogenes is a. Lepromatous leprosy
a. Syphilis b. Impetigo contagiosa b. Tuberculosis
c. Impetigo vulgaris d. Both b and c c. Actinomycosis
2. Painless regional lymph nodes, discrete and rubbery in d. Scarlet fever
consistency is the classical signs of 7. Langerhan’s type giant cells and lepra cells are the
a. Late syphilis b. Primary syphilis histopathological feature of
c. Congenital syphilis d. Impetigo vulgaris a. Tuberculosis b. Leprosy
c. Noma d. None
3. Circinate lesions on face accompanied by alopecia is
the characteristic feature of 8. Langerhan’s giant cells having horse-shoe shaped
a. Primary syphilis b. Congenital syphilis nuclear arrangement is seen in
c. Late syphilis d. Secondary syphilis a. Gonorrhea b. Leprosy
c. Tuberculosis d. Tertiary syphilis
4. Snail track ulcers, split papule, and condyloma latum
9. The characteristic submicroscopic sulfur granules is
is the oral manifestation of
the feature of
a. Secondary syphilis b. Primary syphilis
a. Lepromatous leprosy b. Tuberculosis
c. Tertiary syphilis d. Gonorrhea
c. Actinomycosis d. Scarlet fever
5. Gumma formation, neurosyphilis and tabes dorsalis is 10. Strawberry and raspberry tongue is the oral
the feature of manifestations of
a. Gonorrhea b. Leprosy a. Tuberculosis b. Leprosy
c. Noma d. Tertiary syphilis c. Noma d. Scarlet fever
19 Fungal or Myocotic Infection
Chapter Outline
Â Candidiasis Â Mucormycosis
Â Oral candidiasis Â Cryptococcosis
• Thrush or pseudomembranous candidiasis Â Coccidioidomycosis
• Acute atrophic candidiasis or erythematous candidiasis Â Geotrichosis
• Chronic hyperplastic candidiasis Â Sporotrichosis
• Id reaction Â Rhinosporidiosis
• Candida associated lesion Â Aspergillosis
Â Chronic mucocutaneous candidiasis Â Paracoccidioidomycosis
Â Forms of candidiasis Â Toxoplasmosis
Â Histoplasmosis Â Leishmaniasis
Â Blastomycosis Â Trichinosis
CANDIDIASIS Chronic mucocutaneous candidiasis

∙ Familial CMC
It is also called as ‘candidosis’.
∙ Localized CMC
Candidiasis is the disease caused by infection with
∙ Diffuse CMC
yeast like fungus Candida albicans.
∙ Candidiasis endocrinopathy syndrome
Classification 1st Extraoral candidiasis
∙ Oral Candidiasis associated with extraoral lesions oro-
Oral candidiasis facial and intertriginous sites (candidal vulvovaginitis,
Acute intertriginous candidiasis)
∙ Acute pseudo membranous Candidiasis (thrush) ∙ Gastrointestinal candidiasis
∙ Acute atrophic Candidiasis (antibiotics sore mouth) ∙ Candida hypersensitivity syndrome
Chronic Systemic candidiasis
∙ Chronic atrophic Candidiasis. ∙ Mainly affect the eye, kidney and skin.
– Denture stomatitis
– Median rhomboid glossitis. Classification 2nd
– Angular cheilitis Candidiasis of nails and skin
∙ Id reaction. ∙ Candidal onychia
∙ Chronic hyperplastic Candidiasis. ∙ Candidal paronychia.
Fungal or Myocotic Infection
Candidiasis confined to skin ∙ In condition with minor immunological or other

∙ Interdigital candidiasis systemic defect
∙ Intertriginous candidiasis ∙ Chronic mucocutaneous candidiasis (CMC) syndromes
∙ Candidids (moniliids). – Familial mucocutaneous candidiasis 485
Candidiasis confined to mucosa – Candidiasis endocrinopathy syndrome
Oral mucosa – Localized chronic mucocutaneous candidiasis
∙ Acute oral candidiasis – Diffuse chronic mucocutaneous candidiasis
– Acute pseudomembranous candidiasis (thrush) – Chronic mucocutaneous candidiasis in association
– Acute atrophic candidiasis (antibiotics sore mouth) with thymoma.
∙ Chronic oral candidiasis Confined to mucocutaneous junctions
– Chronic atrophic candidiasis (denture sore mouth) ∙ Candidal angular cheilitis
– Chronic hyperplastic candidiasis. ∙ Perianal candidiasis.
Gastrointestinal mucosa Systemic candidiasis
∙ Pharyngeal candidiasis ∙ Candidal endocarditis
∙ Esophageal candidiasis ∙ Candidal septicemia
∙ Intestinal candidiasis. ∙ Candidal meningitis.
Respiratory mucosa Causative Organisms
∙ Bronchial candidiasis. It is most commonly cause by Candida albicans (the yeast
Genitourinary mucose like fungus occur in yeast and mycelial forms).
∙ Candidal vulvovaginitis. Other organisms which are responsible for candidiasis
Mucocutaneous candidiasis are Candida stellatoidea, Candida tropicalis, Candida
Confined to mucocutaneous surface parapsillosis, Candida pseudotropicalis, Candida famata,
∙ In condition with major immunologic defect Candida rugosa, Candida krusei and Candida guilliermondi.
– Swiss-type agammaglobulinemia Morphologically Candida can exist in three forms, i.e.
– Hereditary thymic dysplasia yeast cells, pseudohypha and chlamydospore forms.
– Di George syndrome Candida albicans is the commonest pathogen of all
– Acquired immunodeficiency syndrome (AIDS) Candida species and it appears as moist creamy colonies
Conditions associated with increased vulnerability of oral candidiasis and their mechanism
Category Condition Mechanism
Altered local resistance to Poor oral hygiene Promotes organism adherence and colonization
infection Xerostomia Absence of antimicrobial and flushing effect of saliva
Recent antibiotics treatment Inhibits competitive oral bacteria
Dental appliance Isolated mucosa from saliva and functional cleansing serve
as organism reservoir
Compromised immune Early infancy Immune competence has not completely developed
system function Genetic immune deficiency Specific humoral or cellular immune defects
AIDS Deficient cellular immune response
Corticosteroids therapy Inhibition of immune function
Pancytopenia Depletion of circulating leukocytes cause by chemotherapy,
aplastic anemia and similar hemopoietic disorders
Generalized patient Anemia, malnutrition, malabsorption Epithelial thinning and altered maturation, poor tissue
debilitation oxygenation
Diabetes mellitus Recurring hyperglycemia and mild ketoacidosis
Advanced systemic disease Metabolic toxicity or limited blood perfusion of tissue
and on blood agar as dull grey colonies. Candida albicans

is component of normal oral microflora with 50 percent of
people carried it without clinical evidence of infection.
486
Marked changes in oral microbial flora owing to
administration of antibiotics (broad spectrum), corticos-
teroids, immunosuppressive agents can lead to candidiasis.
Changes in microbial flora also can occur due to excessive
use of antibacterial mouth rinses, xerostomia secondary
to anticholinergic agent or salivary gland disease and and
radiation to head and neck.
Figure 19.1 Candidiasis presented as white plaque in the
Chronic local irritant like denture, orthodontic
palate
appliance and heavy smoke may predispose to candidiasis.
Acute and chronic disease such as leukemia, lymphoma,
diabetes and tuberculosis can also lead to candidiasis.
Malnutrition states such as low serum vitamin A,
pyridoxine and iron levels, age (infancy, pregnancy,
old age), hospitalization and oral epithelial dysplasia,
endocrinopathies such as hypoparathyroidism, hypo-
thyroidism and Addison’s disease also leads to candidiasis.
Primary and acquired immunodeficiency states such as
hypogammaglobulinemia also act as predisposing factors.
Tight and close fitting garments encourage the growth of
Candida. Areas around the indwelling catheter are also
involved.
ORAL CANDIDIASIS
Oral involvement is probably the most common Figure 19.2 Thrush showing white patches on tongue
manifestation of human candidal infection. It can occur
either solely confined to the oral mucosa or as a part of They are painless and noticed on careful examinations.
any of the several mucocutaneous candidiasis syndromes. They may be removed with little difficulty.
Different types of oral candidiasis can occur. This are
described below. Location: Common sites are roof of the mouth, retromolar
area, and mucobuccal fold. But it is common on any other
Thrush or Pseudomembranous Candidiasis mucosal surface and it is common in women as compared
It is the superficial infection of upper layer of oral mucus to male.
membrane and results in formation of patchy white plaque Prodormal symptom like rapid onset of bad taste may
or flecks on mucosal surface. be there. Spicy food will cause discomfort. Patient may
complain of burning sensation and there may be history of
Clinical Features dryness of the mouth.
In neonates, oral lesions start between the 6th and 10th Signs: Inflammation, erythema, and painful eroded areas
day after birth. Infection is contracted from the maternal may be associated with this disease. Sometimes typical,
vaginal canal where Candida albicans flourishes during pearly white or bluish white plaque curdy, loosely adherent
the pregnancy. The lesions in infants are described as soft patches are present on part of oral mucosa.
white or bluish white, adherent patches on oral mucosa Mucosa adjacent to it appears red and moderately
which may extent to circumoral tissue (Figs 19.1 and 19.2). swollen. Lesions are relatively inconspicuous.
487
Figure 19.3 Red area in palate after removal of patches Figure 19.4 Candidiasis showing organism in the corneum
layer taking up magenta red color (Arrow)
White patches of it are easily wiped out with wet gauze
which leaves either a normal or erythematous area or
atrophic area (Fig. 19.3).
Deeper invasion by the organism leaves an ulcerative
lesion upon the removal of patch. Erythematous or white
area may develop beneath the complete denture or partial
denture. It is occasionally associated with (coexist with)
dysplastic or carcinomatous change.

Fragments of the plaque material may smear on a
microscopic slide, with 20 percent potassium hydroxide
and examined for hyphae.
There is presence of yeast cells and hyphae or mycelia
in the superficial and deeper layer of involved epithelium. Figure 19.5 Candidiasis
The submucosa may be free from any infection or may
contain a chronic inflammatory cell infiltrate.
On staining with PAS organism was identified by
bright magenta color.
Hyphae are varying in length showing branching.
Hyphae are accompanied by yeasts, squamous epithelial
cells.
Potassium hydroxide (KOH) can also be use for rapid
evaluation of Candida species. In this background of
epithelial cells lyses allowing resistant yeasts and Hyphae
to visualize.
Identification of organism can be made by culture.
Culture is made by rubbing sterile cotton swab over
the lesion and then streaking the swab on the surface of
Sabouraud’s agar slant. Candida albicans is seen as creamy
smooth surface colonies after 2 to 3 days of incubation at Figure 19.6 Filamentous organism of Candida in magenta
room temperature. color PAS stain
Points to Remember
Soft white or bluish white, adherent patches on oral
488 mucosa, rapid onset of bad taste, mucosa, moderately
swollen, white patches of it are easily wiped out, presence
of yeast, hyphae or mycelia, yeasts, squamous epithelial
cells, potassium hydroxide (KOH), rapid evaluation of
Candida species, Candida albicans is seen as creamy
smooth surface colonies.
Acute Atrophic Candidiasis or Erythematous

Candidiasis
It is also called as antibiotics sore mouth. When the white
plaque of pseudomembranous candidiasis is removed,
Figure 19.7 Atrophic candidiasis showing red lesion in the
often red atrophic and painful mucosa remains. center of tongue
Clinical Features
Appearance: In this type, lesion appears as red or Chronic Hyperplastic Candidiasis
erythematous rather than white, thus resembling the It is also called as candidal leukoplakia because of its firm
pseudomembranous type in which white membrane has presentation and adherent white patches occurring in the
been wiped off. oral mucosa. This is rare type of candidiasis.
Location: It can occur at any site but it usually involves the
tongue and tissue underlying an appliance. Clinical Features
Age: It is predominantly occurs in men of middle age or
Symptoms: Patient usually described vague pain or a
over.
burning sensation. If the discomfort is not spontaneous, pain
can be elicited by mild abrasive pressure with cotton gauze. Cause: The majority of these patients are heavy smokers.
It occurs on cheek, lip and tongue.
Sign: Careful examination reveals a few white thickened
foci that rub off leaving a painful surface. Lesion closely Appearance: There is firm, and white leathery plaques are
resembles erosive lichen planus and erythroplakia. found.
Bald tongue: There is diffuse loss of filiform papillae Symptoms: Lesions may persist without any symptoms
resulting in bald appearance of tongue (Fig. 19.7). for years. Many women with oral candidiasis also report
symptoms of vaginal itching and discharge indicative of
Histopathological Features vaginal candidiasis.
Atrophic epithelium containing few hyphae in the Sign: It does not rub off with lateral pressure. Lesion range
superficial layer is present. from slightly white to dense white with cracks and fissures
Lamina propria shows mild acute inflammatory occasionally present. The borders are often vague, which
infiltrate and increased vascularity. mimic the appearance of epithelial dysplasia (Fig. 19.8).
Occasionally, microabscesses may be seen superficial It may occur as a part of chronic mucocutaneous
epithelial areas. candidiasis.
Antibiotics sore mouth, red or erythematous rather Epithelial dysplasia is seen but it is reversible. There is
than white, vague pain or a burning sensation, white mycelial invasion of the deeper layers of mucosa and skin
thickened foci, bald tongue, atrophic epithelium, mild occurs. Epithelium is usually parakeratinized.
acute inflammatory infiltrate, microabscesses increased There is acanthosis of spinous layer and bulbous
vascularity. elongated retepegs.
489
Figure 19.8 Chronic hyperplastic candidiasis showing Figure 19.9 Denture stomatitis presented as erythematous
leathery plaque area in maxillary area
There is also pseudoepitheliomatous hyperplasia Appearance: It exhibits patchy distribution often asso-
and microabscesses formation. It contains inflammatory ciated with speckled curd like white lesion (Fig. 19.9).
exudates and chronic inflammatory cell infiltration of
Symptoms: There is soreness and dryness of mouth.
polymorphonuclear neutrophils in the cornium.
Sign: Palatal tissue is bright red somewhat edematous and
Points to Remember granular. Red patches may be erythematous or speckled.
Candidal leukoplakia, heavy smokers, firm, and white The redness of mucosa is rather sharply outlined and
leathery plaques, white to dense white with cracks restricted to the tissue actually in contact with the denture.
and fissures, epithelial dysplasia, mycelial invasion, The multiple pinpoint foci of hyperemia usually involving
acanthosis of spinous layer, bulbous elongated retepegs, the maxilla frequently occur.
pseudoepitheliomatous hyperplasia. Histopathological features: Epithelium is atrophic and
there are superficially oriented mycelia. Intense chronic
Id Reaction inflammatory infiltrate in lamina propria is seen.
A person with chronic Candida infection may develop Management: Troches containing clotrimazole and
secondary response characterized by localized or Nystatin 4 to 5 times after meal and bed time.
generalized sterile vesicopapular rash that is believed to be
allergic response to Candida antigen (also called monolids). Median Rhomboidal Glossitis
Candida Associated Lesion There is debate that it is form of chronic atrophic
candidiasis but area of anterior 2/3 of tongue affect by
Denture Stomatitis median rhomboidal glossitis is frequently followed by
It is also called chronic atrophic candidiasis. It is common Candida infection. It is described in detail in chapter of
clinical manifestation of erythematous candidiasis. tongue disorders.
Candida albicans is always found in this lesion but the
typical white patch of thrush does not usually develop in Points to Remember
it. It occurs due to tissue invasion but organism effect of • D enture stomatitis: Chronic atrophic candidiasis,
fungal toxin hypersensitivity to fungus. speckled curd like white lesion, soreness, palatal
tissue is bright red, epithelium is atrophic
Location: It is usually found under complete denture and
• Median rhomboidal glossitis: Anterior 2/3 of tongue,
partial denture and found mostly in women and always
Candida infection.
include palate.
Management mycostatin cream and ointment accelerate the symptomatic

effect.
Removal of the causes: Replacement of denture or
Amphotericin B 0.08 mg/kg 2 to 3 hourly in ointment or
490 relining or adding mycostatin suspension below it while
cream base, suspension. Elixir containing both tetracycline
insertion in mouth in case of angular cheilitis and denture
and amphotericin B may also prove to be beneficial in
sore mouth. The denture must be cleaned thoroughly and
acute atrophic candidiasis.
regularly and should be kept in hypochlorite solution in
right. Withdrawal or change of antibiotics use if feasible. Rinses: Mycostatin rinses for 7 to 10 days 3 to 4 times a
Oral candidiasis may be treated either topically or day. 0.2 percent chlorhexidine solution, use of 1 percent
systemically. Treatment should be maintained for 7 days. chlorhexidine gel in denture stomatitis.
Response to treatment is often good; oral lesions and
Systemic Treatment: Several agents are effective for
symptoms may disappear in a fairly short period (ranging
systemic treatment.
from 2–5 days), but relapses are common because of the
Nystatin 250 mg TDS for 2 week followed by 1 troche
underlying immunodeficiency. As with other causes of
per day for third week.
oral candidiasis, recurrences are common if the underlying
Ketoconazole is a 200 mg tablet taken with food
problem persists.
once daily. Patient compliance is usually good. Careful
Topical treatments are preferred because they limit
monitoring of liver function is necessary for long-term use
systemic absorption, but the effectiveness depends entirely
because of reported side effects, including hepatotoxicity.
on patient compliance. Following are most commonly used
Lack of efficacy of ketoconazole may occur because of poor
topical treatment.
absorption in those with an abnormally high gastric pH.
Clotrimazole: It is an effective topical treatment (one oral Fluconazole is a triazole antifungal agent effective in
troche [10 mg tablet]) when dissolved in the mouth five treating candidiasis (100 mg tablet taken once daily for 2
times daily. Used less frequently, one vaginal troche can be weeks). Several studies suggest fluconazole is effective
dissolved in the mouth daily. 1 percent gentian violet can as a prophylactic agent, although the most effective
be use but it is not ideal because of the superficial necrosis prophylaxis dosing regimen is still unclear. Numerous
of mucosa it may produce and also because of the unsightly reports, however, describe oral and esophageal candidiasis
staining. failing to respond to treatment with fluconazole, and in
some of these cases investigators isolated resistant strains.
Nystatin preparations: It includes a suspension, a vaginal
Itraconazole (100 mg capsules) may be used for the
tablet, and an oral pastille. Nystatin vaginal tablets (one
treatment of oral candidiasis (200 mg daily orally for 14
tablet, 100,000 units, dissolved in the mouth three times a
days). Itraconazole oral suspension is now available (200
day). Nystatin oral pastille (available as a 200,000 unit oral
mg daily for 2 weeks. Salivary levels of itraconazole are
pastille, one or two pastilles dissolved slowly in the mouth
maintained for several hours after administration.
five times a day). Nystatin oral suspension 100,000 units/cc,
Ketoconazole, fluconazole, and itraconazole may
1 teaspoon of which is mixed with 1/4 cup of water and
interact with other medications including rifampin,
used as oral rinse.
phenytoin, cyclosporine A, terfenadine, digoxin, coumarin-
Topical creams and ointments: Containing nystatin, like medications, and oral hypoglycemic medications.
ketoconazole, or clotrimazole may be useful in treating
angular cheilitis. Another therapeutic choice is amphotericin Points to Remember
B (0.1 mg/mL). Five to 10 mL of oral solution is used as a Removal of the causes, clotrimazole, nystatin pre-
rinse and then expectorated three to four times daily. parations, topical creams and ointments, mycostatin
Mycostatin cream 1 lacks unit or lactose containing cream, amphotericin B, mycostatin rinses, nystatin,
vaginal tablet keeps under the tongue. The addition ketoconazole, itraconazole, fluconazole.
of absorbable corticosteroids and antibiotic agents to
CHRONIC MUCOCUTANEOUS and often laryngeal involvement. Cutaneous involvement

is characterized by raised, crusty proliferative lesions
CANDIDIASIS predominately on the skin of face and scalp. These lesions
Classification are also called candidal granuloma. Oral lesions are 491
widespread, proliferative and raised white lesions.
Types Chronic mucocutaneous candidiasis in association with
• Chronic familial mucocutaneous candidiasis thymoma: Rarely patient with thymoma manifest chronic
• Chronic localized mucocutaneous candidiasis mucocutaneous candidiasis. Oral and skin surface are
• Endocrinopathy candidiasis syndrome usually involved.
• Chronic diffuse mucocutaneous candidiasis
• Chronic mucocutaneous candidiasis in association Management
with thymoma. For resistance form of CMC and for systemic candidiasis
miconazole (250 mg 6 hourly), 5-flucytosine (200 mg daily)
Etiology in addition to amphotericin B is available. Transfusion of
It occur due to defect in cellular immunity, defect in transfer factor and transplantation of culture of thymic
structure of epidermis, diabetes, pregnancy and steroids. fragments have been used to correct T cell deficiency
associated with CMC.
Clinical Features
Chronic familial mucocutaneous candidiasis: It is Points to Remember
an inherited disorder, probably an autosomal recessive • C hronic familial mucocutaneous candidiasis: Candi-
disorder and affects both sexes. It is characterized by diasis of mouth, nails, and skin
candidiasis of mouth, nails, and skin. Children under the • Chronic localized mucocutaneous candidiasis:
age of 10 years show superficial candidal infection. Oral Chronic oral candidiasis, hypoplastic infection of
candidiasis is chronic hyperplastic exhibiting firm white nails
patches involving either buccal or lingual mucosa. • Candidiasis endocrinopathy syndrome: Candida
lesion of skin, scalp, nails and mucous membrane,
Chronic localized mucocutaneous candidiasis: This hypoparathyroidism, hypoadreno-cortism
form occurs early in life with oral mucosal, skin and • Chronic diffuse mucocutaneous candidiasis: Mu-
nail involvement. There is also presence of chronic oral cocutaneous candidiasis, extensive raised crusty
candidiasis and hypoplastic infection of nails fold in sheets, candidal granuloma
infancy. Oral lesion includes white patches characterized • Chronic mucocutaneous candidiasis in association
by horny masses mainly found on face and scalp. Patient with thymoma: Thymoma manifest chronic muco-
show increase tendency to fungal and bacterial infection in cutaneous candidiasis,
the absence of immunological or genetic defect. • Management: Miconazole, 5-flucytosine, ampho-
Candidiasis endocrinopathy syndrome: It is also tericin B.
genetically transmitted characterized by early onset and
Candida lesion of skin, scalp, nails and mucous membrane.
FORMS OF CANDIDIASIS
There is subsequent appearance of hypoparathyroidism,
hypoadrenocorticism and other endocrine abnormalities. Candidal onychia and paronychia: Candidal infection of
There is also occurrence of dental hypoplasia and severe nail and of the soft tissue on the sides and at the base of
caries. There may be balanitis and vulvovaginitis, the nail is common in housewife, nurses, and dishwasher,
keratoconjunctivitis, alopecia and juvenile cirrhosis can bartenders, and fruit pickers. Involved nail show green
also occurs. black discoloration and transverse ridges. Soft tissues
are inflamed and tender. Treatment is by daily topical
Chronic diffuse mucocutaneous candidiasis: It is of late
application of amphotericin B lotion or nystatin ointment.
onset and exhibits extensive raised crusty sheets involving
the limbs, groin, face, scalp and shoulders. Mucocutaneous Interdigital candidiasis: It is also called erosion inter
candidiasis is characterized by extensive oral, pharyngeal digitalis. It is commonly involved 3rd and 4th fingers. It
is characterized by pruritic, inflamed, and scaling lesions Candidal meningitis: It is predominately disease of male
between fingers. children characterized by variable features ranging form
stiffness of the neck, hemiplegia and other neurological
492 Intertriginous candidiasis: Candida infection of skin
signs. The finding of Candida in spinal fluid is of diagnostic
surface such as waist, groin, armpit, elbows, submammary
importance. The condition is fatal in half of the cases.
area, gluteal fold, axilla, scrotum. Opposing skin surface in
these sites, particularly in obese person prevent adequate Candidal septicemia: It occurs in those with severe oral
ventilation and remain moist favoring candidal growth. and esophageal thrush. Features include fever, chills, shock
Candida has characteristic way of causing dry pustulation and coma. Condition can be fatal if not treated in time.
with desiccation in lower level of stratum cornium of skin.
Lesions are very tender. Lesion spread from affected skin HISTOPLASMOSIS
as an area of glaze red skin or an easily detached overlying
It is also called Darling’s disease. It is caused by
epidermis, i.e. often invaded leaving paper like fungal
Histoplasma capsulatum, a dimorphic fungus that grows in
lesion along the margin. Satellite lesion may develop
the yeast form in infected tissue.
around deeper denuded tissue.
Humid areas with soil enriched by bird or bat excrement
Gastrointestinal candidiasis: An extension of oral are suited for growth of this organism. This is reason this
infection may occasionally lead to either pharyngeal or disease is more commonly found in fertile valley region.
esophageal involvement. It presents as acute enterocolitis, Infection results from inhalation of dust contaminated with
diarrhea, as proctitis with anal puritis of perineal dropping, particularly from infected birds.
eczemation. Dysphagia, chest pain, and gastrointestinal
tract (GIT) bleeding may be presenting symptoms. Types
Esophageal candidiasis: Esophageal candidiasis with • Acute primary histoplasmosis
or without gastric ulceration are common forms of GIT • Progressive disseminated histoplasmosis
candidiasis. It is diagnosed by characteristic appearance of • Chronic cavitary histoplasmosis.
edematous ulcerated mucosa on barium swallow.
Bronchial candidiasis: It is a chronic infection of Clinical Features
bronchial mucosa which may last for years without Acute primary histoplasmosis: There is chronic low grade
producing severe discomfort. It mimics chronic bacterial fever, malaise, headache and productive cough. There may
infection of the bronchus and chronic cough with mucoid be pleuritic pain. Primary infection is mild, manifesting as
sputum is common symptoms experience by the patients. self limited pulmonary disease that heals to leave fibrosis
Diagnosis is established by the demonstration of Candida and calcification. Chest radiograph may show patchy
in sputum and through culture studies. It includes systemic infiltrate which may exhibit signs of calcification.
use of antifungal agents. Progressive disseminated histoplasmosis: It is common in
Candidal vulvovaginitis and balanitis: Candida children and elderly. It is manifested by hepatosplenomegaly
vulvovaginitis is a common form of candidiasis, which and lymphadenopathy. Patients with disseminated form
increases during pregnancy, diabetes, and uses of show evidence of bone marrow involvement by anemia
antibiotics. Candidal inflammation of glans penis may and leukopenia. There is also kidney involvement with
occur due to sexual contact. Diabetic women and those gastrointestinal and oropharyngeal ulcerative lesions.
on long term drug therapy are prone to develop vaginal Chronic cavitary histoplasmosis: It closely mimics
infection. It is characterized by erythematous plaques on chronic cavitary tuberculosis. The cavitary lesions are
the glans, penis and around the prepuce. Symptoms usually bilateral and are found in the upper lung fields. Symptoms
clear up on topical application of antifungal agents. include cough, dyspnea and weight loss.
Candidal endocarditis: It is characterized by fever,
dyspnoea and edema of congestive cardiac failure. Vascular Oral Manifestations
vegetations of candidal growth often result in embolization Oral lesions are common in the progressive disseminated
to major vessels. The disease is fetal in majority of cases. form.
Location: It is seen on buccal mucosa, gingiva, tongue,

palate or lip.
Symptoms: Patient may complain of sore throat, painful 493
chewing, hoarseness, difficulty in swallowing.
Signs: Oral lesions are nodular, ulcerative or vegetative.
If left untreated it will progress to form firm papule or
nodules which ulcerate and slowly enlarge. Ulcerated area
covered by nonspecific gray membrane and is indurated
(Fig. 19.10).
The organisms are found in large numbers in phagocytic
cells and appear as tiny intracellular structures measuring
little more than 1 micron in diameter (Fig. 19.11). Figure 19.11 Histoplasma capsulatum organism
Biopsy shows small oval yeasts with in macrophages
and reticuloendothelial cells as well as chronic granuloma,
epithelial cells, giant cell with caseation necrosis. Supportive cares: In case of acute histoplasmosis
The mucosal epithelium shows ulceration, in majority supportive care with analgesic and antipyretic should be
of the cases. given.
In nonulcerated areas, pseudoepitheliomatous hyper Amphotericin B: This is indicated in case of disseminated
plasia is often seen. histoplasmosis.
The submucosa shows a dense infiltrate of granulocytes,
lymphocytes, plasma cells and histiocytes. Multinucleated Points to Remember
giant cells and caseation necrosis are often seen. Causative • A cute primary histoplasmosis: Pleuritic pain,
organism is identified with PAS stain. fibrosis, calcification, patchy infiltrate
• Progressive disseminated histoplasmosis: Hepato-
Management
splenomegaly, lymphadenopathy, bone marrow
Ketoconazole: Ketoconazole is given for 6 to 12 months. involvement by anemia
• Chronic cavitary histoplasmosis: Cavitary lesions
are bilateral cough, dyspnoea and weight loss
• Oral manifestations: Buccal mucosa, sore throat,
painful chewing, hoarseness, nodular, ulcerative or
vegetative, nonspecific gray membrane
• Histopathological: Tiny intracellular structures, small
oval yeasts, macrophages, reticuloendothelial cells,
ulceration, pseudoepitheliomatous hyperplasia, dense
infiltrate of granulocytes, lymphocytes, plasma cells
• Ketoconazole, amphotericin B.
BLASTOMYCOSIS
It is cause by blastomyces dermatidis. Organism is a normal
inhabitant of soil and that is the reason for it to be common
in agricultural worker.
Figure 19.10 Ulcerative lesion seen in female patient in It is transmitted through the respiratory tract. Infection
histoplasmosis. It is mistaken as malignancy of mandibular with blastomycosis begins in a vast majority of cases by
vestibule inhalation, as primary pulmonary infection.
Types Histopathological Features

• Acute blastomycosis The inflamed connective tissue shows occasional giant
494 • Primary pulmonary blastomycosis cells, macrophages and the typical round organisms, often
• Cutaneous blastomycosis budding, which appear to have a doubly refractile capsule
• Disseminated or systemic blastomycosis. (Fig. 19.12).
Organism is characterized by doubly refractive cell
wall with a broad attachment between budding daughter
Clinical Features
cells and parent cell.
Age and sex distribution: It is more commonly seen in Microabscesses are frequently found and if the lesion is
male as compared to female in the ratio of 9:1. It is seen in not ulcerated overlying pseudoepitheliomatous hyperplasia
middle age group. may be prominent.
Acute blastomycosis: It resemble pneumonia and patient
Diagnosis
complaint of high fever, chest pain, malaise and productive
cough. The index of suspicion should increase when chronic,
painless, oral ulcer appears in an agricultural worker or
Primary pulmonary blastomycosis: It follows a chronic when review of system reveals pulmonary symptom.
course with malaise, low grade fever and mild cough. If Diagnosis is made on the basis of biopsy and on culturing
untreated, shortness of breath, weight loss and blood tinged the organism from tissue.
sputum are encountered. It mimic tuberculosis.
Cutaneous blastomycosis: Infection of skin, mucosa and Management
bone may also occur, resulting from metastatic spread of Intravenous amphotericin B is indicated only if the patient
organism through lymphatic system. Skin and mucosal is seriously ill and not improving clinically. This is given
lesion starts as subcutaneous nodule and progresses to well for 8 to 10 weeks causes’ resolution of the disease.
circumscribed indurated ulcer. Elevated, verrucous, crusted Itraconazole – It is indicated in chronic cases.
and single or multiple lesions developing on exposed part of
the body, particularly on hands and face. Lesion leaves a Points to Remember
slanted serpiginous border with a tendency towards healing Blastomyces dermatidis
in the center. When the crust is lifted, pus exudes. • Acute blastomycosis: Pneumonia, high fever, chest
Disseminated or systemic blastomycosis: It results from pain, malaise and productive cough
the spread of infection from pulmonary form. Bones are • Primary pulmonary blastomycosis: Malaise, low
involved in larger number of cases. Liver, kidney, spleen grade fever, mild cough, shortness of breath
and gastrointestinal tract may be involved. Cerebral abscess • Cutaneous blastomycosis: Subcutaneous nodule, well
are rare, but may occur as a result of brain involvement. circumscribed indurated ulcer, slanted serpiginous
border
Oral Manifestations • Disseminated or systemic blastomycosis: Bones are
involved, cerebral abscess
It may be primary or secondary to some infection elsewhere
• Oral manifestations: Enlargement of cervical lymph
in the body.
nodes, non-specific, painless verrucous ulcer with
Symptoms and sign: Oropharyngeal pain, accompanied indurated borders
by the enlargement of cervical lymph nodes, may be a • Histopathological features: Occasional giant
presenting sign of oral disease. cells, macrophages, typical round organisms, often
Appearance: There is nonspecific, painless verrucous budding, doubly refractive cell wall, microabscess,
ulcer with indurated borders often mistaken for squamous pseudoepitheliomatous hyperplasia
cell carcinoma. Other lesions are hard nodules and appear • Management: Traconazole, Intravenous ampho-
as sessile projection, granulomatous appearing plaque. tericin B.
There is increased lethargy, progressive neurologic deficit

and ultimately death. Fungus invades artery to cause
fibrosis and ischemia. There is appearance of a reddish
black nasal turbinate and septum. Nasal discharge caused 495
by necrosis of nasal turbinate.
Oral Manifestations
If there is involvement of maxillary sinus patient will
exhibit intraoral swelling in the region of alveolar process
or palate. It this is untreated then ulceration of palate, due
to necrosis and invasion of palatal vessels can occur (Fig.
19.13).
Ulcer may be seen on gingivae, lip and alveolar bone. It
is large and deep, causing denudation of underlying bone.
Figure 19.12 Blastomyces dermatidis Radiological features—There is opacficiation of sinus
wall with patchy effacement of bony walls of the sinus.
MUCORMYCOSIS Histopathological Findings

It is also called as phycomycosis, Zycomycosis. It is caused The tissue involved by mucormycosis shows necrosis and
by saprobic fungus of class Zygomycetes like Absidia, chronic inflammatory infiltrate. The vessels in the area
Mucor, and Rhizopus. may be thrombosed with organisms in the lumen.
It is more common in patients with decreased resistance, Culture studies are essential to identify the order family
due to diseases like diabetes, tuberculosis, renal failure, and genus of fungus. The organism appears as large,
leukemia, cirrhosis and in severe burn cases. nonseptate hyphae with branching at obtuse angle. Round
Growth of these fungi is enhanced by iron and patient and ovoid sporangia are also seen.
who are taking iron chelating agent like deferoxamine are There is extensive tissue destruction which disturbs
also at increase risk of mucormycosis. normal blood flow resulting in infarction and necrosis.
Types
Management
• S uperficial: It involves external ear, fingernails and Surgical debridment is the treatment of choice.
skin.
• Visceral:
– Pulmonary
– Gastrointestinal
– Rhinocerebral or rhinomaxillary form.
Clinical Features
Rhinomaxillary form begins with inhalation of fungus by
susceptible individual. Infection usually arise in lateral
wall of nose and maxillary sinus; may rapidly spread by
arterial invasion to involve the orbit, palate, maxillary
alveolus and ultimately the cavernous sinus and brain
through hematogenous spread and may cause death. Ptosis,
proptosis, fever, swelling of cheek and paresthesia of face
can occur.
Intracranial involvement may be manifested by cranial Figure 19.13 Mucormycosis showing deep ulceration of the
neuropathy, especially of the trigeminal and facial nerve. palate (Courtesy: Dr Ashok L)
Systemic Amphotericin—It should be given in high Cryptococcal meningitis: Meningoencephalic lesions

doses. produce a variety of neurologic signs and symptoms,
generally associated with increased intracranial pressure.
496 Points to Remember
Radiographic features: The radiograph of chest shows
Rhinomaxillary form, arterial invasion to involve infiltrates and occasionally ‘coin’ lesion.
the orbit, ptosis, proptosis, fever, swelling of cheek,
paresthesia of face, intracranial involvement, cranial Oral Manifestations
neuropathy, reddish black nasal turbinate, nasal
Location: Lesion of hard palate, soft palate, gingiva,
discharge, involvement of maxillary sinus, denudation
extraction socket, tongue and tonsillar pillar are common.
of underlying bone, opacification of sinus wall, necrosis,
chronic inflammatory infiltrate, large, nonseptate Appearance: They appear as simple nonspecific, single
hyphae, systemic amphotericin. or multiple ulcers. They are nodular and granulomatous,
which may ulcerate during the course of disease.
CRYPTOCOCCOSIS Histopathological Features
It is also called torulosis. It is a chronic fungal infection In tissue section it appears as a small organism with round
caused by Cryptococcus neoformans and Cryptococcus or ovoid structure with a large clear halo, sometimes
bacillispora. described as tissue microcyst (Fig. 19.14).
The causative organism is gram positive, budding, The tissue reaction is generally granulomatous type;
yeast-like cell with an extremely thick, gelatinous capsule, epitheloid cell proliferation is minimal.
measuring 5 to 20 microns in diameter. Organism lives in Multinucleated giant cells as well as inflammatory cell
deposit of excreta left by pigeon bird. It can grow in soil infiltrate are common.
and in infected tissue.
Infection occurs due to inhalation of airborne Diagnosis
microorganism. It has increased incidence in immuno- The organisms can be cultured on Sabouraud’s glucose
suppressive patients. agar.
Types Management
• Primary cryptococcal infection Mild to moderate cases can be treated with ketoconazole
• Disseminated cryptococcal infection for 6 to 12 weeks.
• Cryptococcal meningitis
• Cutaneous cryptococcal infection.
Clinical Features
There is slight predilection for middle aged males.
Primary cryptococcal infection: The infection usually
occurs in lungs. It may be asymptomatic and in some cases,
patient may complain of cough with mucoid expectoration.
Occasionally, pleuritic pain and hemoptysis can also occur.
Disseminated type: Disseminated infection common
in patients who are immunocompromised. There is
involvement of meninges, skin, bone and prostate gland.
Cutaneous type: The skin lesion appears as multiple brown
papules which ultimately ulcerate, the clinical picture is
nonspecific. The lesion discharge pus like material which
is rich in organisms. Figure 19.14 Cryptococcus organisms
The severe form requires amphotericinB, intrave- Primary cutaneous coccidioidomycosis: Skin lesions
nously for up to 10 weeks. This can be combined with are also present, like erythema nodosum of erythema mul
flucytosine in case of cryptococcal meningitis. tiforme. Primary lesions, when they occur, are associated
with regional lymphadenopathy. Granulomatous, verrucous 497
Points to Remember or necrotic ulcers exuding thick pus are seen on involved
Torulosis, Cryptococcus neoformans, primary cry- skin surface.
ptococcal infection in lungs cough with mucoid Valley fever: This is hypersensitivity reaction which occur
expectoration and pleuritic pain, disseminated type in in conjunction with coccidioidomycosis is term as valley
immunocompromised with involvement of meninges, fever.
skin, bone, cutaneous type with multiple brown papules
and puslike material, cryptococcal meningitis, coin’ Progressive disseminated coccidioidomycosis: The
lesion, lesion of hard palate are simple non-specific, disease usually runs rapid course and the dissemination
single or multiple ulcers, tissue microcyst, tissue extends from the lungs to various viscera, bones, joints,
reaction granulomatous type, multinucleated giant cells, skin and central nervous system, where meningitis is the
flucytosine and ketoconazole. most frequent cause of death.
Oral Manifestations
COCCIDIOIDOMYCOSIS Lesions of head and neck, including the oral cavity, occur
It is also called valley fever, desert fever or coccidiodal with some frequency.
granuloma. The disease appears to be transmitted to man
Appearance: The lesions of oral mucosa and skin are
and animals by inhalation of dust contaminated by the
proliferative, granulomatous and ulcerated lesions that are
spores of the causative organism, Coccidioides immitis.
nonspecific in their clinical appearance. These lesions tend
Coccidioides immitis is dimorphic organism which
to heal by hyalinization and scar formation.
appears as mold in soil and as yeast in tissue of infected
host. Radiological features: Lytic lesions of bones of jaw may
Infection is spread by means of inhalation of arthro- develop.
spores.
Types The tissue reaction is similar to any specific granuloma.
• Primary nondisseminated coccidioidomycosis There is accumulation of large mononuclear cells,
– Primary pulmonary coccidioidomycosis lymphocytes and plasma cells.
– Primary cutaneous coccidioidomycosis Foci of coagulation necrosis are often found in the
• Progressive disseminated coccidioidomycosis. center of small granulomas and multinucleated giant cells
are scattered throughout the lesion.
Clinical Features The organism is found within the cytoplasm of giant
cells, as well as is lying free in the tissue. Organism is
Age and sex distribution: It is common in all age groups round spherules which may contain numerous endospores.
and predominately seen in males.
Symptoms occur usually 14 days after the inhalation of Management
fungus. Infection is common in summer months, especially
Amphotericin B has been found to be an effective
after periods of dust storm. It is self limiting and runs its
chemotherapeutic agent for the disease. It is given in
course within 10 to 14 days.
case of immunosuppressed patient, severe pulmonary
Primary pulmonary coccidioidomycosis: The patient infection, pregnant patient and patient who appear to be
generally develops manifestations suggestive of respiratory life threatening situation. Long-term therapy is required for
disease such as cough, pleural pain, headache and complete cure.
anorexia. Patient may also complaint of low grade fever Other drugs like fluconazole, itraconazole can also be
and joint pain. used.
Points to Remember
Valley fever, desert fever, coccidioides immitis, cough,
498 pleural pain, headache, anorexia, erythema nodosum of
erythema multiforme, regional lymphadenopathy, valley
fever, disseminated extends from the lungs to various
viscera, bones, joints, lesions of oral mucosa and skin
are proliferative, granulomatous, lytic lesions of bones
of jaw, large mononuclear cells, plasma cells, foci of
coagulation necrosis, round spherules, amphotericin B,
fluconazole, itraconazole.
GEOTRICHOSIS
Geotrichosis is an infrequent opportunistic mycosis
caused by yeasts. The main etiologic agent is Geotrichum Figure 19.15 Rectangular shaped organism of geotrichosis
candidum, which belongs to the class Hemiascomycetaceae.
Geotrichum candidum is a cosmopolitan micro-
organism and habitual contaminant. It has been isolated Points to Remember
from various sources such as fruits and vegetables, soil, and Geotrichum candidum, lung involvement pneumonitis
plants. Several studies have proven that it is a commensal or bronchitis, candidiasis or thrush, being white, velvety,
in humans and part of the normal flora of the skin, mouth patch like covering of the oral mucosa, small, rectangular
and gastrointestinal tract. shaped; spores.
It is found in patients with debilitating diseases. Most
of the reported clinical cases have occurred in immuno-
suppressed patients or immunosuppressive disorders.
SPOROTRICHOSIS
It is also called Rose gardener’s disease. It is a fungal
Clinical Features infection caused by Sporotrichum schenckii. The disease
Location: It has been reported to pathologically affect the predominately affects skin. Because roses can spread the
bronchi, lungs, and bowel, and only seldom the mouth, disease, it is one of a few diseases referred to as rose-
skin and nails. thorn or rose-gardeners’ disease, because Sporotrichum
Lung involvement produces symptoms of pneumonitis schenckii is naturally found in soil, hay, sphagnum moss,
or bronchitis. The expectoration is often tinted with blood. and plants, it usually affects farmers, gardeners, and
agricultural workers.
Oral features: They are similar to candidiasis or thrush,
It is caused by exposure to a wide variety of animals,
being white, velvety, patch like covering of the oral
both domestic and wild. It may be cause by accidental
mucosa, isolated or diffuse in distribution. Tonsillar lesions
injury from the thorns of some plants or bushes, accidental
are common in association with oral lesions.
laboratory or clinical inoculation in hospital workers.
The organism is small, rectangular shaped; spores
Location: The incubation period is from week to 3 weeks.
measuring approximately 4 to 8 microns, often with
It involves the skin, subcutaneous tissues and oral nasal
rounded ends (Fig. 19.15).
and pharyngeal mucosa.
The tissue reaction is nonspecific and of acute
inflammatory type. Cutaneous or skin sporotrichosis: It most common form.
There is nodular lesions or bumps in the skin, at the point of
Management entry and also along lymph nodes and vessels. The lesion
It includes topical and systemic application of nystatin and initially small and painless, and ranges in color from pink
amphotericin B. to purple. It the lesion left untreated, the lesion becomes
larger and look similar to a boil and more lesions will

appear, until a chronic ulcer develops. Usually, cutaneous
sporotrichosis lesions occur in the finger, hand, and arm.
The skin lesion often described as sporotrichotic chancre. 499
Regional lymphadenopathy is generally developed and it
may ulcerate and drain.
The fungus follows lymphatic channels in the body.
Small ulcers appear in lines on the skin as the infection
goes up an arm or leg. These sores do not heal unless they
are treated and may remain for years. The nodules may
drain small amounts of pus from time to time.
Pulmonary sporotrichosis: This rare form of the disease
occur when Sporotrichum schenckii spores are inhaled.
Symptoms of pulmonary sporotrichosis include productive
coughing, nodules and cavitations of the lungs, fibrosis, Figure 19.16 Hyphae of sporotrichosis
and swollen hilar lymph nodes. Patients with this form of
sporotrichosis are susceptible to developing tuberculosis Management
and pneumonia.
It includes oral administration of potassium iodide in
Disseminated sporotrichosis: The infection can spread suitable doses.
to joints and bones (called osteoarticular sporotrichosis) The skin infection is usually treated with an antifungal
as well as the central nervous system and the brain (called medicine called itraconazole.
sporotrichosis meningitis). The symptoms of disseminated
sporotrichosis include weight loss, anorexia, and appearance Points to Remember
of bony lesions. Rose Gardner disease Sporotrichum schenckii, cutaneous
or skin sporotrichosis, sporotrichotic ‘chancre’. Regional
Oral Manifestations lymphadenopathy, fungus follows lymphatic channels,
Nonspecific ulceration of the oral, nasal and pharyngeal pulmonary sporotrichosis, disseminated sporotrichosis,
mucosa also occurs. Long standing lesions become osteoarticular sporotrichosis, non-specific ulceration of
granulomatous, vegetative or papillomatous. the oral, nasal pharyngeal mucosa, septate hyphae bud-
Pain is present and the cervical lymph nodes are always ding form, granulomatous one with epihtheloid cells,
enlarged. polymorphonuclear leukocytes, pseudoepitheliomatous
hyperplasia, oral administration of potassium iodide.
The fungus is small, ovoid branching organism with septate RHINOSPORIDIOSIS
hyphae showing budding form. It is only 3 to 5 microns in Rhinosporidiosis is a mucosal and cutaneous mycosis
diameter and can be cultured on Sabouraud’s medium (Fig. caused by Rhinosporidium seeberi.
19.16). Laryngeal rhinosporidiosis too has been described
The tissue reaction is a granulomatous one with and may be due to inoculation from the nose during
epithelioid cells, multinucleated giant cells of Langhans endotracheal intubation. After inoculation the organism
type and lymphocytes, often surrounding a central area of replicate locally resulting in the hyperplasia of host tissue
purulent or caseous necrosis. and localized immune response.
Polymorphonuclear leukocytes are prominent with The infection results from a local traumatic inoculation
pseudoepitheliomatous hyperplasia of the overlying with the organism while swimming or bathing in freshwater
epithelium of skin or mucosal lesion. ponds, lakes or rivers.

Location: It is more common on oropharynx, naso- Rhinosporidium seeberi, local traumatic inoculation
500 pharynx, larynx, skin, eyes and genital mucosa. Though, with the organism while swimming or bathing nasal
the floor of the nose and inferior turbinate are the most irritation, epistaxis, local pruritus, rhinorrhea, coryza,
common sites, the lesions may appear in elsewhere small verrucae or warts, strawberry like appearance,
too. Traumatic inoculation from one site to others is soft, reddish pink, polypoid growth of tumor like nature
common which spreads to the pharynx and larynx, focal abscess
Symptoms: Initial symptoms include nasal irritation, formation acute and chronic inflammatory cells.
accompanied by mucoid discharge. Other symptoms
includes epistaxis, local pruritus, rhinorrhea, coryza with ASPERGILLOSIS
sneezing, post nasal discharge with cough, foreign body
sensation, and increased tearing and photo phobia in cases It is a fungal infection caused either by sensitization to,
of infection of palpebral conjunctiva. parasitic colonization of, or tissue invasion by species
of genus Aspergillus. It is second to candidiasis, as an
Sign: The skin lesion appears as small verrucae opportunistic infection, in immunocompromised patients
or warts, which ultimately become pedunculated. like AIDS, uncontrolled diabetes mellitus, leukemia etc.
Posteriorly, these polypoid masses may extend into Aspergillus species stays in soil, water, and decaying
the pharynx. The lesions are soft, friable and highly organic debris. Most commonly species are Aspergillus
vascular. Genital lesion resembles condyolomas. Lesion flavus and Aspergillus fumigates.
bleeds easily upon manipulation. It has got strawberry
like appearance. Clinical Features
Location: The respiratory tract, external auditory canal,
Oral Manifestations nasopharynx, cornea, gastrointestinal tract and occasionally
Location: The soft palate appears to be the most frequent the skin may be the primary sites of infection.
site of oral involvement.
Symptoms: In some cases, there may be asthmatic episode
Signs: It is accompanied by a mucoid discharge and in sensitive persons.
appears as soft, reddish pink, polypoid growth of tumor
Sign: It may present swelling, ulceration, crusting and
like nature which spreads to the pharynx and larynx. The
necrosis of anterior turbinates, nasal septum and nasal wall.
lesions are vascular and bleed readily.
Allergic fungal sinusitis: In some patient, allergy affecting
Histopathological Features sinus is present.
The organisms appear as sporangia containing large Disseminated aspergillosis: It occur in immuno-
number of round or ovoid endospores with size of 5 to 7 compromised patient. Patient complaint of chest pain,
microns in diameter. cough, fever.
The surrounding tissue reaction itself is a nonspecific,
consisting of a vascular granulation tissue with focal Oral Manifestations
abscess formation and occasional multinucleated giant Location: It is rare but in some cases, involvement of palate
cells. Both, acute and chronic inflammatory cells are and tongue can occur. Soft palate involvement seems to be
present in variable number. more common in upper respiratory tract involvement.
Management Appearance: A lesion on the palate is manifested as a
painful ulcer, surrounded by a ring of black necrotic tissue.
Surgical excision—Wide excision with electro-coagu-
lation of the lesion base. Oropharyngeal aspergillosis: In patients with hemato
Medical treatment is not so effective but treatment with logical malignancies, presents as yellowish-black ulcera-
a year long course of dapsone has been reported. tion of soft palate and posterior part of tongue. These
patients complain of intense local pain, oral bleeding and

Points to Remember
dysphagia.
Genus Aspergillus, asthmatic episode, ulceration, crust-
Aspergilloma: Sometime low grade infection of maxillary ing and necrosis of anterior turbinates, allergic fungal 501
sinus may results in mass of fungal hyphae which is called sinusitis, disseminated aspergillosis, oropharyngeal as-
as aspergilloma. After some time mass will undergo pergillosis, aspergilloma, granulomatous reactions, giant
calcification resulting in antroliths. cells, chronic inflammatory infiltrate, branching septate
hyphae, voriconazole, amphotericin B.
Granulomatous reactions are seen in majority of the lesions
and consist of central necrosis surrounded by epitheloid PARACOCCIDIOIDOMYCOSIS
cells and fibrosis. It is also called ‘South American blastomycosis’. It is
Giant cells and chronic inflammatory infiltrates are caused by Paracoccidioides brasiliensis. It is usually found
seen in connective tissue. in South American population of farmers.
Organism shows varying number of branching septate
hyphae 3 to 4 mm in diameter. These hyphae have tendency Clinical Features
to branch at acute angle and to invade adjacent small blood Age and sex distribution: It is seen in male in the ratio
vessels (Fig. 19.17). of 15:1. This can be due to protective effect of female
hormone. It is more commonly seen in middle age grouped.
Management
Pulmonary infection: Initial infection is usually
Amphotericin B is the treatment of choice in case of
pulmonary in nature
aspergillosis.
Recent studies show that voriconazole is more effective Addison disease: Adrenal involvement can occur
in treating this patients. due to hematgenous or lymphatic spread resulting in
Disseminated aspergillosis in immunocompromised hypoadrenocorticism.
patients should be treated on an individual basis with
intravenous antifungal agents and surgical debridement. Oral Manifestations
Location: It is occur on alveolar mucosa, gingiva, palate,
buccal mucosa and pharynx.
Appearance: There is mulberry like ulceration affecting.
There is pseudoepitheliomatous hyperplasia with ulceration
in surface epithelium.
There is also presence of epihtheloid macrophages and
Organism are scatter and large showing multiple
daughter buds on the parent cells which resemble
appearance as ‘Mickey mouse ears’ or the spokes of ship’s
steering wheel’ (mariner wheel).
Management
Sulfonamide derivative—These are used since long back
Figure 19.17 Branching organisms of aspergillosis
to treat this infection
Other drugs which can be use are intravenous

Points to Remember
amphotericin B, oral itraconazole and ketoconazole.
Toxoplasm gondii, cervical lymphadenopathy, dissemi-
502 Points to Remember nated type, congenita toxoplasmosis, accumulation of
eosinophilic macrophages, macrophage accumulate, sul-
Paracoccidioides brasiliensis, pulmonar infection, Ad-
fadiazine, pyrimethamine.
dison disease, mulberry like ulceration, pseudoepitheli-
omatous hyperplasia, epihtheloid macrophages, Mickey
mouse ears’ or the spokes of ship’s steering wheel’ (mari- LEISHMANIASIS
ner wheel), sulfonamide derivative.
It is caused by the genus Leishmania which consist of
three flagellate protozoa, which cause variety of distinct
TOXOPLASMOSIS infections in man and are transmitted by sandfly bites.
It is cause by obligate intracellular protozoal organism
Types
Toxoplasma gondii. Cats are considered to be host for this
organism. • V isceral leishmaniasis: It is also called Kalaazar. It
It is more commonly seen in immunocompromised is caused by Leishmania donovani.
individual like AIDS, transplant recipient and cancer • Cutaneous leishmaniasis: It is caused by Leishmania
patients. braziliensis.
Clinical Features Clinical Features

Symptoms: They are mild and consist of low grade fever, Visceral leishmaniasis: Incubation period is 2 weeks to
cervical lymphadenopathy, fatigue, and muscle and joint 2 years. Onset may be insidious with a low grade fever
pain. In some cases submental and buccal lymph nodes can or it may be abrupt with sweating and high intermittent
be enlarged. fever. Cough and diarrhea can also develop. The spleen
Disseminated type: This occurs in immunocompromised becomes enlarged, often massively. If not treated, patient
individual and manifested as encephalitis, pneumonia, will become anemic and wasted. There is also abdominal
myositis and myocarditis. There may be disorientation, discomfort, fever that lasts for weeks; may come and go in
lethargy and headache. cycles, night sweats, thinning hair and weight loss.
Congenita toxoplasmosis: This occur when mother Mucocutaneous leishmaniasis: Incubation period is 1
contract disease during pregnancy with organism week to 1 month. It is usually seen in young men. There is
crossing the placental barrier. In this case there may be past history of superficial ulcer of skin, caused by bite of
blindness, mental retardation and delayed psychomotor an infected sandfly, which heals with depressed scar. Nasal
development. mucosa becomes congested and ulcerates. Later, all the
soft tissues of nose may be destroyed.
Oral Manifestations
Lymph nodes show reactive germinal center exhibiting
accumulation of eosinophilic macrophages. Visceral leishmaniasis: There may be increase
Macrophage accumulate at subcapsular and sinusoidal pigmentation of face. There may be spontaneous bleeding,
region of node. edematous gingiva and loose teeth.
Mucocutaneous leishmaniasis: Mucosal lesion usually
Management occurs 1 to 2 years after skin lesion. Lips, soft palate and
If exposure is suspected during pregnancy combination of larynx may be involved. The mucosal lesions are long
sulfadiazine and pyrimethamine should be given. This will standing, destructive, granulating ulcers which in many
prevent transmission of organism to fetus. instance cause severe mutilation of structure involved.
Severe cases can also be treated by same combination. Regional lymphadenopathy is common.
Histopathological Features Symptoms: Trismus, muscular cramps of the facial

muscle, jaw and tongue can occur. There is also monotony
Dense dermal infiltrate of lymphocytes, plasma cells,
of speech.
histiocytes, epithelioid cells and occasionally eosinophils 503
and giant cells. Sometimes neutrophils are present Signs: There may be petechiae of buccal mucosa, palate
throughout the reticular dermis. and floor of mouth. There is also bleeding from gingiva,
The organisms are round to oval basophilic structures lips and nose.
with eccentrically located kinetoplast.
In case of chronic lesion there is mild to moderate
mononuclear infiltrate (lymphocytes and plasma cells) near Larva cause inflammation and destruction of muscle fibers.
the granuloma together with fibrosis and telangiectasia. A fibrous hyaline develops around a single coiled larva.
The histiocytes and giant cells may surround it.
Management
Amphotericin is the drug of choice for visceral leishmaniasis.
Management
In mucocutaneous lesion, small lesion may be treated There is no specific treatment for trichinosis and in severe
by freezing with liquid carbon dioxide, curettage or cases, prognosis is poor.
infiltration with 1 to 2 mL sodium stib gluconate.
If the lesions are multiple parenteral injection of Points to Remember
amphotericin B should be given. Trichinella spiralis, striated muscle, masseter, fever,
facial and periorbital edema, tongue, trismus, muscular
Points to Remember cramps, petechiae of buccal mucosa, inflammation,
Leishmania three flagellate protozoa, visceral leishmani- destruction of muscle fibers.
asis, cough, diarrhea, low grade fever, abdominal discom-
fort, mucocutaneous leishmaniasis, history of superficial BIBLIOGRAPHY
ulcer of skin, bite of an infected sandfly, pigmentation of
1. Aarestrup FM, Guerra RO, Vieira BJ. Oral manifestation
face, lips, soft palate and larynx shows destructive, gran-
of sporotrichosis in AIDS patients, et al. Oral Dis.
ulating ulcers, dense dermal infiltrate of lymphocytes, 2001;7(2):134-6.
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cated kinetoplast, mononuclear infiltrate, freezing with associated with kala-azar. A case report: Oral Surg Oral Med
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3. Ajit Daharwal, Hansa Banjara, Digvijay Singh, et al. A
rare case of laryngeal rhinosporidiosis. J Laryngol Voice.
TRICHINOSIS 2011;1:30-2.
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J AM Dent Assoc. 1992;123:77-82.
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neck manifestation of disseminated coccidioidomycosis.
Most common site involved are striated muscle, masseter, Laryngoscope. 114:747-2
neck muscle and diaphragm. 7. Azaz B, Milhem I, Hasson O. Acquired toxoplasmosis of
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Pediatric Dent. 1994;16:378-80.
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8. Barbeau J, Seguin J, Goulet J, et al. Reassessing the presence
Oral Manifestations of Candida albicans in denture related stomatitis. Oral Surg
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504 11. Falworth MS, Herold J. Aspergillosis of the paranasal sinus: with aspergillosis of the maxillary sinus: a report of case.
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Oral Pathol Oral Radiol Endod. 1996;81:255-60. 22. Reder PA, Neel B. Blastomycosis in otolaryngology: a
12. Fotos PG, Vincent SD, Hellstein JW. Oral candidosis: a review of large series. Laryngoscope. 1993;103:53-8.
clinical historical and therapeutic features of 100 cases: Oral 23. Rose HD, GIngrass DJ. localized oral blastomycosis
Surg Oral Med Oral Pathol. 1992;74:41-9. mimicking actinomycosis. Oral Surg Oral Med Oral Pathol.
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sequestration resulting from mucormycosis: Br J Oral Dis. 1995;1:77-9.
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1. Antibiotics sore mouth refers to: 3. ‘Darling’s disease refers to:

a. Median rhomboidal glossitis a. Blastomycosis
b. Acute atrophic candidiasis b. Cryptococcosis
c. Thrush c. Histoplasmosis
d. Bronchial candidiasis d. Systemic candidiasis
2. Presence of yeast cells and hyphae or mycelia in the 4. Fungal infection Sporotrichum schenckii causes:
epithelium of: a. Coccidioidomycosis b. Sporotrichosis
a. Oral thrush c. Rhinosporidiosis d. Aspergillosis
b. Median rhomboidal glossitis 5. Tissue microcyst or a large clear halo is the feature of:
c. Acute atrophic candidiasis a. Blastomycosis b. Cryptococcosis
d. Bronchial candidiasis c. Histoplasmosis d. Systemic candidiasis
20 Viral Infection
Chapter Outline
Â Human herpes virus Â Enteroviruses

Â Herpes simplex infection • Herpangina
• Primary herpes simplex infection • Acute lymphonodular pharyngitis
• Recurrent or secondary or recrudescent herpetic • Hand foot mouth disease
infection Â Foot and mouth disease
Â Measles Â Condyloma acuminatum
Â Varicella zoster infection Â Verruca vulgaris
• Chicken pox Â Focal epithelial hyperplasia
• Herpes zoster Â Molluscum contagiosum infection
• James Ramsey Hunt syndrome Â Cytomegalovirus infection
Â Rubella Â Infectious mononucleosis
Oral cavity is prone for viral infection. Many viruses can All of above virus cause infection and remain latent
cause distinct clinical and pathological features in the oral for the life of individuals. These viruses shed in saliva or
cavity. Virus which can affect the oral cavity are described genital secretion providing avenue for infection of new
in Table 20.1. hosts.
HUMAN HERPES VIRUS HERPES SIMPLEX INFECTION

The word herpes means to creep or crawl. Herpes virus It is the most common viral disease which can affect the
family is called as Herpetoviridae. Human acts as natural human being and which can cause vesicular eruption on
source. All herpes contain DNA nucleus which can remain the skin. This is usually cause by HSV 1. Infection caused
latent in host neural cells, thereby evading host immune by HSV 2 usually occurs below the waist, i.e. in genital
response. region.
Member of herpes family are herpes simplex virus type But due to change in sexual habit (oro-genital sex) now a
I (HSV I or HHV 1), type II (HSV2 or HHV 2), Varicella day HSV 2 can be seen causing the infection above the waist. It
zoster virus (VZV or HHV 3), Epstein Barr virus (EBV is primarily occur in early childhood due to frequent exchange
or HHV 4), cytomegalovirus (CMV or HHV5), HHV6, of salivary and nasal secretions. HSV II viruses remain latent
HHV7 and HHV 8. in lumbosacral ganglion.
Table 20.1 Viruses causing disease in oral cavity ganglion of vagus nerve, dorsal root ganglion and brain. Here
it remain latent or in sequestered state. During this phase no
Name of virus Disease cause
major histocompatibility (MHC) antigen is expressed, so there
506 HSV1 or HHVI Herpetic gingivostomatitis is no T cell response during this phase.
(primary and secondary)
HSV II or HHV II Genital infection Reactivation of virus: It may occur due to sunlight (fever
blister), cold (cold sore), trauma, stress, or immunosuppression.
Varicella-zoster HHV II Chickenpox, shingles
It usually occurs due to breakdown of focal immuno-
EBV HHV IV Mononucleosis, Burkitt’s surveillance or there is alternation in local inflammatory
lymphoma, hairy leukoplakia, mediators which allows virus to replicate. This will results in
nasopharyngeal carcinoma recurrence of lesion at the site of primary infection. This virus
Cytomegalovirus HHV Salivary gland enlargement travel by the same path which is used while going in latency
V phase. At this site replication of virus occur resulting in focal
HHV6 Roseola infantum infection. There is no systemic symptoms occur as humoral
HHV8 Kaposi Sarcoma and cell mediated immunity are sensitized for HSV antigen.
Papilloma viruses Oral papilloma, wart, heck
disease, condyloma acuminatum Types
Coxsackie viruses Herpangina, hand foot and mouth • Primary herpes simplex infection
disease • Recurrent herpes labialis (RHL)
Measles virus Measles • Recurrent intraoral herpes (RIH) simplex infection
Mumps virus Mumps, parotitis. • Herpetic whitlow
• Herpes gladiatorum or scrumpox
• Herpes barbae
Herpes simplex virus does not survive in external • Eczema herpeticum or Kaposi’s varicelliform eruption.
environment and infection results from contact with infected
person. Crowding and poor oral hygiene can lead to infection.
Herpes simplex virus nowaday has been implicated in Clinical Features
number of other disease like erythema multiforme, aphthous Primary Herpes Simplex Infection
ulceration, cluster headache, number of cranial neuropathies.
Herpes simplex virus may aid in carcinogenesis through
It is exposure of individual without antibodies to the virus.
promotion of mutations. Oncogenic role of HSV remain It occurs at young age. Development of lesion before ages
doubtful. of 6 months is rare as it is protected by maternal anti-HSV
antibodies.
Pathogenesis Virus is taken by sensory nerves and transported to
Contact with individual: There is direct physical contact autonomic ganglion where it remains latent. Ganglions
between infected individual and seronegative host (not where it can remain latent are trigeminal ganglion, nodose
previously infected with the virus). ganglion of vagus nerve, dorsal root ganglion.
Binding of virus to cell surface: After the contact virus binds Transmission: It occurs during close personal contact.
with surface of cell with the help of heparin sulfate. Primary infection of newborn is believed to be caused by
vaginal secretions during birth, which results in viremia and
Activation of genes: After binding there is activation of
disseminated infection of brain, liver, adrenals and lungs.
specific genes (immediate early (IE), early (E), and late (L)
genes). Prodormal signs and symptoms: Prodromal symptoms
Incubation period: This range from several days to 2 weeks. precede local lesion by 1 to 2 days and it includes fever,
After this individual can experience primary gingivostomatitis headache, malaise, nausea, vomiting and within a few days,
with focus at the site of contact. mouth becomes painful. There is also irritability, pain upon
swallowing and regional lymphadenopathy.
Latent phase: One primary infection is over virus moves along
the periaxon sheath of trigeminal nerve to trigeminal ganglion. Appearance: Small vesicles, which are thin walled,
Other ganglion where this virus can remain latent are no dose surrounded by inflammatory base are formed. They quickly
Viral Infection
rupture leaving small, shallow, oval shaped discrete ulcers. RECURRENT OR SECONDARY
The base of the ulcer is covered with grayish white or
OR RECRUDESCENT HERPETIC
yellow plaque. The margins of the sloughed lesions are
uneven and are accentuated by bright red rimmed, well INFECTION 507
demarcated, inflammatory halos. Recurrent infections are limited to localized portions of
The individual ulcer differs in size from 2 to 6 mm. skin and mucous membrane. Spontaneous recurrence can
As the disease progresses several lesions may coalesce, occur and affect the area supplied by sensory ganglion.
forming larger, irregular lesions. Antibodies can shed infectious viral particle without active
In severe cases, excoriation involving the lips may infection. In some cases virus may spread to other site in
become hemorrhagic and matted with serosanguinous same host to become latent at the sensory ganglion of the
fibrin like exudate and parting of the lips during new location.
mastication and speech, may become extremely painful If it occurs on lip, it is called as recurrent herpes labialis
and difficult. (cold sore, fever blister). If occurs intra-orally it is called as
Marginal acute gingivitis: There is appearance of recurrent intra-oral herpes infection (Fig. 20.2).
generalized marginal acute gingivitis. Entire gingiva is Predisposing factors: Condition such as old age,
edematous and swollen and small gingival ulcers are seen. Ultraviolet light, stress, fatigue, heat cold, pregnancy,
Examination of posterior pharynx reveals inflammation. allergy, trauma and dental therapy and menstruation can
Cervical and submandibular lymphadenopathy can also predispose this condition.
occur. Lesions begin healing in a week to 10 days and Recurrent herpes simplex infection may occur at
leave no scar. HSV may confine to saliva for up to 1 month widely varying intervals, from nearly every month in some
after onset of disease (Fig. 20.1). patients to only about once a year or even less in others.
Pharyngotonsillitis can also occur as primary infection. Lesions may develop lips or intraorally.
Numerous vesicle develops on the tonsils and posterior
Prodormal symptoms: Lesion is preceded by tingling and
pharynx. Vesicle rupture to form shallow ulceration.
burning sensation and feeling of tautness, swelling or slight
There is formation of diffuse gray yellow exudate over the
soreness subsequent development of vesicle.
ulcer.
In some cases satellite vesicle of perioral skin is Appearance: It is accompanied by edema at the site of the
seen. lesion, followed by formation of clusters of small vesicles.
Figure 20.1 Marginal gingivitis with lip lesion in primary Figure 20.2 Secondary infection due to herpes virus causing
herpes simplex infection vesicle present on lower lip
It ranges from 1 to 3 mm in diameter, to 1 to 2 cm. But

sometimes, it is large enough to cause disfigurement.
These gray or white vesicles rupture quickly leaving small
508 red ulcerations, sometimes with slightly erythematous halo
on lip covered by brownish crust on lips.
In RIH vesicles break rapidly to form small red
ulceration, sometimes with slight erythematous halo.
Cluster of small vesicles or ulcers 1 to 2 mm in diameter are
commonly found on gingivae, palate and alveolar region.
The lesions gradually heal within 7 to 10 days and leave no
scars.
Secondary infection in immunodeficiency patient
results in more severe symptoms as compared to normal
person. These infections also can spread to many site and
not restricted to one sight.
Different Forms of Herpes Simplex Infection

Herpetic whitlow: It is also called herpetic paronychia. Figure 20.3 Simplex infection showing ballooning
Dentist may experience primary or secondary lesion of degeneration (BD), vesicle formation (V) inflammatory cells (IC)
fingers from contact with lesions of the mouth or saliva of
the patients who are asymptomatic carriers of HSV; called Multinucleated giant cells: This can be seen after rupture
as herpetic whitlow. Incidence varies according to socio- of vesicle at the edge of ulceration.
economic group. In case of herpetic whitlow axillary or
epitrochlear lymphadenopathy may be present. Intraepithelial vesicle: Intercellular edema may lead to
development of intraepithelial vesicle which is made up
Herpes gladiatorum or scrumpox: This usually occurs of fibrin polymorphonuclear leukocytes and degenerated
in wrestlers and rugby players by the contaminated areas cells.
of abrasion.
Herpes barbae: Herpes simplex infection spreading over Diagnosis
the bearded region of the face due to minor injury cause by HSV isolation or culture: Isolation and neutralization
daily shaving. of virus in tissue culture is most positive method of
identification. This can be done by direct fluorescence
Eczema herpeticum or Kaposi’s varicelliform eruption:
assay or polymerase chain reaction. Rabbit kidney and
This is development of life threatening HSV infection
human amnion are sensitive to HSV.
occur with skin disease like eczema, pemphigus, darier’s
disease. Antibody titer: Antibodies to HSV appear in a week and
react peak in 3 weeks.
Intra-nuclear inclusions: Lipschutz bodies which are Management
eosinophilic, ovoid, homogenous structure within the Acyclovir suspension rinses and swallow method: This
nucleus, tend to displace the nucleolus and nuclear is more useful in primary infection with HSV. In this 200
chromatin peripherally. The displacement of chromatin mg of adults dose is given (children 15 mg/kg) five times
often produces a peri-inclusion halo. daily. Significant resolution of infection can be seen.
Ballooning degeneration: Infected cells in HSV infection Topical antiviral drug: Topical agents interrupts viral
exhibits acantholysis, nuclear enlargement. This is called replication through inhibition of DNA polymerization.
as ballooning degeneration. The acantholytic epithelial Acyclovir when used topically is convert into form that
cells are termed as Tzanck cells (Fig. 20.3). inhibit viral DNA polymerase. It is done by thymidine
Viral Infection
kinase (virus induced enzyme). Topical acyclovir can be MEASLES

initiated in given in 5 percent dose.
It is also called rubeola or morbilli. It is an acute
Systemic antiviral drugs: Acyclovir is given in the dose
contagious dermatotropic viral infection, primarily 509
of 200 to 400 mg tablet 5 times daily. Other antiviral drugs
affecting children and occurs many times in epidemic
which can be used are valacyclovir, famciclovir. Valacyclovir
form. It is cause by family paramyxovirus genus morbilli-
should be given initially 2g stat after the symptoms appear,
virus.
after this another 2 g can be given after 12 hours.
Spread of disease occurs by direct contact with a
Symptomatic treatment: Topical anesthetics spray person or by droplet infection, the portal of entry being the
1 percent dyclonine hydrochloride decreases the mucosal respiratory tract.
discomfort. Tetracaine lollipops can also be used for
numbing of affected mucosa. Topical anti-infective agents Clinical Features
can also be given for relief of the patient.
Incubation period is 8 to 10 days and affected patient are
Recurrent herpes labialis: Acyclovir ointment, penciclovir infectious 2 days before symptoms appear and 4 days after
ointment can give minimum reduction in the healing time. appearance of rash.
We can also give 10 percent n-docosanol cream.
Symptoms: There is onset of fever, malaise, cough,
Prophylactic use antiviral: This can be done when conjunctivitis, photophobia, lacrimation and eruptive
condition causing recurrence are identified, e.g. dental lesions of skin and oral mucosa occurs. Otitis media and
procedure. In this case acyclovir 400 mg BD, valacyclovir sore throat can occur.
1 g daily or famciclovir 250 mg BD can be given. This can Skin eruption begins on face, in the hair line and behind
be given one day prior to the procedure or any known trigger. the ear and spread to neck, chest, back and extremities.
Immunocompromised host with HSV infection often
required intravenous antiviral medication. Appearance: It appears as tiny red macules or papules
which enlarge and coalesce to form blotchy discolored
Points to Remember irregular lesions, which blanch on pressure. Fade away in
HSV 1 4 to 5 days with fine desquamation.
• Primary herpes simplex infection: Young age, There are three stages of this infection with each stage
prodromal symptoms precede local lesion by 1 to lasting for 3 days and justifying the designation of nine
2 days, fever, headache, thin walled, small vesicles days measles.
surrounded by inflammatory base, base of the ulcer
grayish white or yellow plaque, several lesions may First 3-day stage: There are three Cs in this stage. These
coalesce, forming larger, irregular lesions, marginal are Coryza (runny nose), cough and conjunctivitis. There is
acute gingivitis, pharyngotonsillitis also presence of koplik spot in this stage.
• Recurrent or secondary or recrudescent herpetic Second 3-day stage: In this, fading of koplik spot
infection: As recurrent herpes labialis (cold sore, with appearance of morbilliform (maculopapular and
fever blister), recurrent intra-oral herpes infection, erythematous) rash present on face followed by trunk.
predisposing factors old age, ultraviolet light, stress,
edema at the site of the lesion, formation of clusters Third 3-day stage: Fever ends with rash fading and it
of small vesicles, vesicles break rapidly, small red demonstrated brown pigmentatory staining. Desquamation
ulceration of skin is present in the area of rash.
• Histopathological: Lipschutz bodies which are Complication like otitis media, pneumonia, persistence
eosinophilic, ovoid, ballooning degeneration, Tzanck bronchitis, acute appendicitis and sub-acute sclerosing
cells, multinucleated giant cells, intercellular edema pan-encephalitis can occur.
may lead to development of intraepithelial vesicle
• Management: Acyclovir suspension rinses and Oral Manifestations
swallows method, topical antiviral drug, valacyclovir, Oral lesions precede 2 to 3 days before cutaneous rash and
famciclovir. are pathognomonic of this disease.
Points to Remember
Rubeola, paramyxovirus genus morbillivirus, otitis
510 media, malaise, cough, conjunctivitis, photophobia, tiny
red macules or papules which enlarge, nine days measles,
first 3-day stage coryza, cough, conjunctivitis, second
3 day fading of Koplik spot with appearance of morbilli-
form (maculopapular and erythematous) rash, third 3 days
stage fever ends with rash fading, complication like otitis
media, pneumonia, orally buccal mucosa shows Koplik
spots which are small, irregularly shaped flecks, bluish
white specks, grain of salt, of focal hyperparakeratosis,
spongiosis, intercellular edema, dyskeratosis, epithelial
syncytial giant cells, pink staining inclusion, micro
abscess formation, epithelial necrosis, vaccine, ribavirin,
Figure 20.4 Koplik spot seen in measles immunoglobulin, interferon and vitamin A.
Location: The most common site is buccal mucosa.

VARICELLA ZOSTER INFECTION
It is an acute disease caused by varicella zoster virus, which
Koplik spots (Fig. 20.4): Intraoral lesions are called as
is a DNA virus similar to HSV and causes both, primary
Koplik’s spots and occur in 97 percent of cases. They are
and recurrent infection.
small, irregularly shaped flecks which appear as bluish
After primary disease is healed, VZV becomes latent in
white specks surrounded by bright red margins. It has been
the dorsal root ganglion of spinal nerve or extra-medullary
describe as ‘grain of salt’ of red background.
ganglion of cranial nerve. VZV becomes reactivated
Generalized inflammation, congestion, swelling and
causing lesions of localized herpes zoster.
focal ulceration of gingiva, palate, throat may occur.
Patients with HIV infection, leukemia and those on
immunosuppressive therapy have an increased suscep-
tibility to severe or potentially fatal herpes zoster. Herpes
Koplik spot represent areas of focal hyperparakeratosis zoster infection can be deep seated and disseminated
which exhibits spongiosis, intercellular edema, dyskerato- causing pneumonia, meningocephalitis and hepatitis.
sis, and epithelial syncytial giant cells in epithelium.
There is pink staining inclusion in the nuclei or less Types
commonly in the cytoplasm. This inclusion represents • Chickenpox (Varicella)
microtubular aggregates characteristic of the paramy- • Shingles (herpes zoster) or zona
xovirus. There is also micro-abscess formation, epithelial • James Ramsey Hunt syndrome.
necrosis and ulceration.
Chickenpox
Management
It is also called as ‘varicella’. It is an acute viral disease
The patient should be isolated, if possible as it is contagious occurring in children and most commonly in winter and
disease. spring months.
Vaccine: This should be given to children between the age
of 12 and 15 months. Clinical Features
Ribavirin, immunoglobulin, interferon and vitamin A Transmission: Incubation period is two weeks and mode
should be given. of transmission is by air borne droplet or direct contact
Viral Infection
with infected persons, with the probable port of entry being

respiratory tract.
Prodromal symptoms: It is characterized by prodromal 511
occurrence of headache, nasopharyngitis and anorexia,
followed by maculo-papular or vesicular eruptions on skin
and low grade fever. This exanthema is very pruritic.
Appearance: These eruptions usually begin on the trunk
and spread to involve the face and extremities. They occur
in successive crops so many vesicles in different stages of
formation or resorption may be found.
Dewdrop on rose petal: The centrally located vesicle is
surrounded by zone of erythema. This is called as dewdrop
on rose petal appearance.
Figure 20.5 Chickenpox virus
Skin lesion: The skin eventually ruptures, forming a
superficial crust and heals by desquamation. The disease
runs its clinical course in a week to ten days, seldom Management
leaving any after effects. Symptomatic: Warm baths with soap or baking
Occasionally, secondary infection of vesicle results in soda, application of calamine lotion and systemic
the formation of pustules which may leave small pitting diphenhydramine are used for management of pruritus.
scar upon healing. Antipyretic other than aspirin is given.
Complication like Reye’s syndrome, secondary skin Antiviral medication like acyclovir, valacyclovir and
infection, encephalitis, cerebellar ataxia, pneumonia, famciclovir if administer within 24 hours of rash has reduce
gastrointestinal disturbance and hematologic event can duration and severity of infection.
occur. Intravenous administration is done in case of
immunocompromised patients.
Oral Manifestations
Purified varicella zoster immune globulin can be given
Location: Small blister like lesions occasionally involve to modify clinical manifestation of disease.
the oral mucosa chiefly buccal mucosa, tongue, gingiva,
lip, palate as well as the mucosa of pharynx. Vaccine: Vaccine for chickenpox is recommended for
children between 12 to 18 months of age. Vaccine use is
Appearance: The mucosal lesion, initially a slightly raised live attenuated varicella vaccine.
vesicle with a surrounding erythema, ruptures soon after
formation and forms a small eroded ulcer with red margins, Points to Remember
closely resembling aphthous lesion.
Varicella, prodromal symptoms headache, naso-
Histopathological Features pharyngitis, anorexia, eruptions begin on the trunk,
dewdrop on rose petal appearance, skin lesion forming
The cytological studies are identical to that of herpes
a superficial crust, complication like Reye’s syndrome,
simplex infection. These viruses cause acanthylosis with
secondary skin infection, encephalitis, mucosal lesion
formation of numerous free floating Tzanck cells.
slightly raised vesicle, acanthylosis, Tzanck cells,
There is also nuclear margination of chromatin and
nuclear margination of chromatin, multinucleation,
occasional multinucleation.
virus isolation, systemic diphenhydramine, acyclovir,
Virus isolation (Fig. 20.5) in cell culture is done by
valacyclovir and famciclovir, purified Varicella zoster
fluorescein conjucted VZV monoclonal antibodies can be
immune globulin vaccine.
performed.
HERPES ZOSTER
It is also called shingles or zona. It is an acute infectious
512 viral disease of extremely painful and incapacitating nature,
characterized by inflammation of dorsal root ganglion,
associated with vesicular eruptions of skin and mucous
membrane of the area supplied by the affected sensory nerve.

Predisposing factors: Herpes zoster may be predisposed by
trauma, malignancy or tumor involvement of dorsal root gan-
glion, local X-ray radiation or immunosuppressive therapy.
Prodormal period of 2 to 4 days in which shooting
pain, paresthesia, burning and tenderness appears along
the course of affected nerve. During initial viral replication Figure 20.8 Healed vesicle of herpes zoster causing
active ganglionitis develop which result in neuronal discoloration
A B
Figure 20.6 Intact vesicle seen in the case of herpes zoster Figures 20.9A and B Lesion of herpes zoster on face
infection
Figure 20.7 There is corneal scarring and ulceration occur

on the face in case of herpes zoster Figure 20.10 Healed lesion of herpes zoster
Viral Infection
necrosis and severe neuralgia. This is responsible for

intense pain that precede the rash.
Appearance: Unilateral vesicles on an erythematous base 513
appear in clusters, chiefly along the course of nerve and giving
picture of a single dermatome involvement. Vesicle turns into
scab in 1 week and healing takes place in 2 to 3 weeks.
Nerves commonly affected are C3, T5, L1, L2 and 1st
division of trigeminal nerve. It may affect motor nerve.
Herpes zoster ophthalmicus: Involvement of 1st
division leads to corneal scarring and blindness which
are presumably related to viral spread, neural damage,
vasculitis and inflammatory immune response.
If Hutchison’s sign (cutaneous zoster of the side of tip of
nose) is present, then the probability of ocular involvement Figure 20.11 Vesicle seen onto the lip and buccal mucosa
is more.
Zoster sine herpete (zoster without rash) in some cases
there is recurrence of zoster infection without vesicle of
skin or mucosa. Patient had severe pain and hyperesthesia
of specific dermatome.
Exanthema resolves within 2 to 3 weeks. On healing,
scarring with hypo or hyperpigmentation can also occur.
Post-herpetic neuralgia: Pain may continue for weeks
to months. This unfortunate sequel called as post-herpetic
neuralgia occurs in elderly persons due to inflammation,
fibrosis and scarring of nerve and may cause severe
stabbing pain after the skin lesions has healed. Light touch
over the involved area may give elicit painful response
which is called a tactile allodynia.
Oral Manifestations (Figs 20.11 and 20.12) Figure 20.12 Ulcer occurring on dorsal surface of tongue
Location: It results from involvement of 2nd and 3rd

divisions of trigeminal nerve. It may be found on buccal fluorescent conjugated monoclonal antibody. Antibody
mucosa, tongue, uvula, pharynx and larynx. titer increased.
Symptoms: Lesions of oral mucosa are extremely painful.
Points to Remember
The lesions rupture to leave areas of erosion.
Trigeminal herpes zoster occurring during tooth Shingles, zona, predisposing factors trauma, malignancy,
formation causes pulpal necrosis and internal root prodormal period of 2 to 4 days shooting pain, paresthesia,
resorption. Findings are similar to herpes infection, but it burning, unilateral vesicles on an erythematous base appear
is associated with neurogenic pain of unilateral nature and in clusters, single dermatome involvement, nerves affected
segmental distribution of the lesion. are C3, T5, L1, L2 and 1st division of trigeminal nerve,
Involvement of bone can occur which result in necrosis. herpes zoster ophthalmicus, Hutchison’s sign, zoster
This can occur due to damage of the blood vessel supplying sine herpete, scarring with hypo or hyperpigmentation,
alveolar ridge and teeth leading to focal ischemic necrosis. postherpetic neuralgia, tactile allodynia, lesions of oral
mucosa are extremely painful, trigeminal herpes zoster,
Histopathological Features pulpal necrosis, internal root resorption, bone can occur
These are similar to primary infection. Viral isolation which result in necrosis, viral isolation, acyclovir,
can be done with fluorescent antibody stained smear or diphenhydramine, live attenuated VZV vaccine.
JAMES RAMSEY HUNT SYNDROME Congenital rubella syndrome (CRS): This is present in
children after birth and occur due to transmission from
It is zoster infection of geniculate ganglion with involve- infected mother. Classic triad consists of deafness, heart
514 ment of the external ear and oral mucosa and facial disease and cataracts.
paralysis.
The clinical manifestation of it is facial paralysis as Complication: It includes arthritis, encephalitis, and
well as pain of the external auditory meatus and pinna of thrombocytopenia.
the ear. Oral Manifestations
In addition, vesicular eruption occurs in the oral cavity
and oro-pharynx with hoarseness of voice, tinnitus, vertigo Forchheimer sign: It consist of small, discrete, dark red
and occasional other disturbances. papule which develop on soft palate and extend to hard
palate. This occur simultaneously with rash and last only
Management to 12 to 14 hours.
Acyclovir: 800 mg five times daily which is associated In some cases palatal petechiae can also occur.
with significantly accelerated healing within 48 hours of Management
the onset of rash.
Non-aspirin antipyretics and antiprutitics may be given.
Symptomatic: Diphenhydramine can be given for itching, Passive immunity is acquired by giving human rubella
antibiotics for secondary infection. immunoglobulin. It should be given within two days of
Postherpetic neuralgia: To control postherpetic neuralgia, exposure.
prednisone 40 to 60 mg daily for 1 to 2 weeks. Steroid
injection can be given in a patient with age more than 60 Points to Remember
years, for the treatment of post-herpetic neuralgia. German measles, rubivirus of family Togavirus,
prodromal symptoms includes fever, headache, malaise,
Live attenuated VZV vaccine: It can be given in anorexia, malagia, rash present on face, discrete pink
adults after the age of 60 years. It prevent prevalence of macules, congenital rubella syndrome, complication
postherpetic neuralgia. includes arthritis, encephalitis, Forchheimer sign consist
of small, discrete, dark red papule, antiprutitics, non-
RUBELLA aspirin antipyretics.
It is also called German measles. It is cause by Rubivirus
of family Togavirus. This infection occurs in spring and ENTEROVIRUSES
winter by respiratory droplet.
Human enteroviruses are classified into echoviruses,
Clinical Features coxsackieviruses A and B, polioviruses and enteroviruses
Age and incubation period: It is more adolescents and 71. Coxsackieviruses are named after town in upper New
adults. Incubation period is form 14 to 21 days and infected York where they were first discovered. They are divided
patient is contagious for 1 week before exanthema to 5 into 2 groups.
days after rash. Type A - 24 types
Type B - 6 types.
Prodromal symptoms: It includes fever, headache, These viruses can cause hepatitis, meningitis,
malaise, anorexia, malagia, mild conjunctivitis, coryza, myocarditis, pericarditis and respiratory disease.
pharyngitis and lymphadenopathy. Frequently occurs in epidemic, with highest frequency
Appearance: Rash present on face and neck and spread from June to October. It appears to be transmitted from one
to entire body. It is presented as discrete pink macules, person to another through contact. Most cases transmitted
then papules and ultimately results in flaky desquamation. from fecal oral route. It is occur in poor hygiene and
Rash is resolve by three day giving designated 3 days crowding condition. Frequent hand washing is required to
measles. diminish spread of infection in epidemic.
Viral Infection
Types
• Herpangina
• Hand foot and mouth disease 515
• Acute lymphonodular pharyngitis.
Herpangina
It is also called as ‘aphthous pharyngitis’, ‘vesicular
pharyngitis’.
Herpangina is cause by A1 to A10, A16, and A22. It
can also be cause by coxsackieviruses B2 to B6, echovirus
9, 16 or 17 and enteroviruses 71.
Clinical Features
Age and incubation period: Majority affected are young Figure 20.14 Same patient after treatment
children aged 3 to 10 years. Incubation period is of 2 to 10
days.
Prodormal symptoms: Initially, generalized symptoms
of fever, chills, headache, anorexia, prostration, abdominal In these areas of affected epithelium exhibits intracellular
pain and sometimes vomiting. Sore throat, dysphagia and and intercellular edema, that leads to spongiosis and
occasionally, sore mouth can occur. formation of intraepithelial vesicle.
There is rupture of vesicle with formation of
Location: It occur on posterior pharynx, tonsil, faucial subepithelial vesicle. There is also epithelial necrosis and
pillars and soft palate. ulceration.
Appearance: Lesion starts as punctuate macule which
Laboratory Diagnosis
evolves into papules and vesicles. Within 24 to 48 hours,
vesicles get ruptured forming small 1 to 2 mm ulcers. No ballooning degeneration seen in this condition which is
Ulcers show a gray base and inflamed periphery. They helpful to distinguish herpangina from herpes simplex and
generally heal without treatment in 1 week (Figs 20.13 herpes zoster.
and 20.14).
Management
Self limiting and supportive treatment by proper hydration
and topical anesthetic, when eating or swallowing is
difficult.
Points to Remember
Aphthous pharyngitis, generalized symptoms of fever,
chills, headache, anorexia, prostration, posterior
pharynx, punctuate macule which evolves into papules
and vesicles, intracellular and intercellular edema,
spongiosis, formation of intraepithelial vesicle, no
ballooning degeneration seen, supportive treatment.
Acute Lymphonodular Pharyngitis

It is caused by A10 and is same as herpangina. It is self
Figure 20.13 Herpangina showing lesion in palate limiting and only supportive care is indicated.
Clinical Features Oral Manifestations

Age: The disease affects predominantely children and Location: The most common sites for oral lesions are hard
516 young adults, occasionally older adults can also be affected. palate, tongue and buccal mucosa.
Location: The lesion appears on uvula, soft palate, anterior Symptoms: A sore mouth with refusal to eat is one of the
pillars and posterior oropharynx. most common findings in this disease.
It has got 5 days incubation period and course may run
Signs: The tongue may become red and edematous. Oral
for 4 to 14 days, with local oral lesions resolving within 6
lesions are more extensive than herpangina. Clinical
to 10 days.
manifestations last for 3 to 7 days.
Symptoms: The chief complain is of sore throat, 41°C
temperature, mild head ache, anorexia and loss of appetite. Laboratory Findings
Appearance of lesion: It consists of raised, discrete, and Intracytoplasmic viral inclusion can be seen in vesicular
whitish to yellowish solid papules of 3 to 6 mm in diameter, scrapping of the lesions. There is rise in acute or
surrounded by narrow well defined zone of erythema. convalescent serum antibody titer to Coxsackie A16.
Lesion is non-vascular, non-ulcerated, tender, superficial Management
and bilateral.
No specific treatment is necessary since the disease is self
Histopathological Features limiting and generally regresses within one to two weeks.
The papular lesion consists of densely packed nodules of
lymphocytes. Points to Remember
In some cases, overlying epithelium has shown inclusion Appearance of maculo-papular, exanthematous and
bodies which in some instance were intra-nuclear, but in vesicular lesions of skin, anorexia, low grade fever, sore
other cytoplasmic. mouth, tongue may become red, intracytoplasmic viral
inclusion can be.
Points to Remember
Uvula, soft palate, anterior pillars, 5 days incubation FOOT AND MOUTH DISEASE
period, sore throat, anorexia, loss of appetite, raised,
discrete, and whitish to yellowish solid lesion, narrow Foot and mouth disease (FMD) is an animal disease caused
well defined zone of erythema, densely packed nodules by an RNA aphthovirus, belonging to the picornaviridae
of lymphocytes, inclusion bodies. family.
Foot and mouth disease is the most contagious of all
Hand Foot Mouth Disease infectious diseases in animals, affecting cattle and swine
most severely. It also affects sheep, goats, deer and other
It is cause by A16, A5, A9, A10, echovirus 9, and cloven-hoofed ruminants.
enteroviruses 71. Infected animals present fever and blister-like sores in
the mouth, on the teats and between the hooves.
Clinical Features
Disease in humans has been reported mainly in
Age: It primarily affects children between the age of 6 connection with consumption of unpasteurized milk, dairy
months and 5 years. or unprocessed meat products from infected animals or as
Appearance: It is characterized by appearance of maculo- a result of direct contact with infected animals (e.g. farmers
papular, exanthematous and vesicular lesions of skin, and veterinarians).
particularly involving the hands, feet, legs, arms and
occasionally buttocks.
Clinical Features
The incubation period in humans is two to six days.
Symptoms: There is anorexia, low grade fever and
sometimes lymphadenopathy, diarrhea, nausea and Symptoms: Symptoms are mostly mild and self-limiting.
vomiting. It is manifested by fever, nausea, vomiting, malaise and
Viral Infection
appearance of ulcerative lesions of oral mucosa and Appearance: Small keratotic warts occurring alone or in
pharynx. clusters on oral mucosa can be seen. The enlargement may
be larger than 1 cm in diameter.
Sign: Development of vesicle on skin also occurs in some 517
cases, usually on the palms of hands and soles of feet. Signs: The lesion is sharply delineated and may
appear sessile and pedunculated. It presents as discrete
Oral Manifestations papillomatous growth.
Location: It can occur at any site, but lips, tongue, palate
and oro-pharynx appear to be affected. Laboratory Investigation
Virus isolation can be done by staining of viral antigen
Appearance: These lesions begin as small vesicles which
DNA by hybridization restriction, endo-nuclease analysis
rapidly rupture but heal within two weeks.
and polymerase chain reaction.
Management
No treatment is necessary and recovery commonly occurs
The papillomatous projection making up the verrucoid
within a week of the last blisters forming.
lesion generally shows a parakeratotic surface with
Points to Remember marked underlying acanthosis. Thin connective tissue
support papillary projection which are blunted and broader,
RNA aphtovirus, most contagious, fever, nausea,
impairing appearance of keratin filled crypts between
vomiting, malaise, development of vesicle on skin, oro-
prominences.
pharynx, small vesicles which rapidly rupture.
Vacuolated cells in the spinous layer are common.
The supporting connective tissue is usually edematous,
CONDYLOMA ACUMINATUM with dilated capillaries and a chronic inflammatory cell
infiltrate (Fig. 20.15).
It is also called as genital wart, venereal wart or verruca
Prickle cells shows pyknotic, crinkled or raisin-like
acuminate. It is caused by human papillomavirus (HPV)
nuclear surrounded by clear zones (koilocytes).
2,6,11, 16, 18, 31, 53, and 54.
It can be transmitted from mother to infant, at birth and Management
resulting syndrome is called as juvenile onset respiratory
Genital warts are treated by excision, electro or cryosurgery,
papillomatosis.
CO2 laser therapy.
Clinical Features
Age and incubation period: Incubation period is around
1 to 3 months after sexual contact. It is commonly seen in
teenager and young adults.
Location: It develop on external genitals, perianal
mucosae, and adjacent skin as well as in vaginal and anal
canal. Wart growth is favored by moist warm environment
of perianal skin and mucosal surface.
Appearance: They are pink, fleshy, sessile, blunted
surface papillomatous lesions. It proliferates and coalesces
rapidly to form diffuse papillomatous clusters of varying
size. Recurrence is common.
Oral Manifestations
Location: It may involve gingiva, cheek, lip, hard palate, Figure 20.15 Vacuolated cells seen in condyloma
tongue and floor of mouth. acuminatum
Application of chemical agents such as podophyllin, Cupping effect: Elongated rete ridges tend to converge
cantharidin and 5-fluorouracil is also helpful. toward the center of lesion.
Immuno-modulating agent such as interferons can also Other features include pyknosis (small dark nuclei),
518 be used. hypergranulosis (prominient granular cell layer), clumped
keratohyaline granules and abundant koilocytes seen in
Points to Remember superficial spinous layer.
Genital wart, venereal wart, juvenile onset respiratory
papillomatosis, perianal mucosae, pink, fleshy, sessile, Management
blunted surface papillomatous lesions, involve gingiva, Topical salicylic acid, topical lactic acid or liquid nitrogen
small keratotic warts occurring alone or in clusters on cryotherapy.
oral mucosa, lesion is sharply delineated, verrucoid Surgical excision indicated in large and atypical lesion.
lesion, parakeratotic surface, acanthosis, keratin filled
crypts between prominences, vacuolated cells, dilated Points to Remember
capillaries and a chronic inflammatory cell infiltrate, Human papillomavirus, skin of hands, painless papule
prickle cells, pyknotic, crinkled or raisin-like nuclear or nodule with papillary projection, skin lesion are
excision, electro or cryosurgery, CO2 laser therapy pink, yellow or white, keratin horn or cutaneous horn,
podophyllin, cantharidin, 5-fluorouracil. proliferation of hyperkeratotic stratified squamous
epithelium, cupping effect, pyknosis, hypergranulosis,
VERRUCA VULGARIS clumped keratohyaline, topical salicylic acid, topical
lactic acid or liquid nitrogen cryotherapy.
It is also called as ‘common wart’. It is benign viruses
induce cause by human papillomavirus (HPV) types 2,4,6,
and 40. It can spread to other area by auto inoculation. FOCAL EPITHELIAL HYPERPLASIA
Clinical Features It also called as Heck disease, multifocal epithelial
hyperplasia, multifocal papilloma virus epithelial
Age: It is seen in children as well as some lesion seen in
hyperplasia’.
adults.
It is viral induced oral mucosal hyperplastic response
Location: The skin of hands, and in case of oral cavity characterized by multiple, more or less papillomatous like
it is seen on vermilion border, labial mucosa and anterior lesion. It is possibly cause by virus (may be papovavirus
tongue. group). Some lesion may be seen in HIV seropositive
patient.
Appearance: It appear as painless papule or nodule with
papillary projection or rough pebbly surface. It can be Clinical Features
pedunculated or sessile.
Age and sex distribution: It occurs predominately in
Sign and symptoms: Skin lesion are pink, yellow or white children between ages of 3 to 18 years.
and oral lesion are always white. Common wart can enlarge
rapidly to its maximum size of less than 5 mm. Location: Common sites are lip, buccal mucosa,
commissure, tongue and less commonly on the gingiva,
Keratin horn or cutaneous horn: Extreme accumulation and anterior faucial pillar.
of compact keratin may results in hard surface projection
several millimeters in height. This is called as cutaneous or Appearance: It present as multiple nodular lesions with
keratin horn. sessile base. It can occur in cluster or in a isolated crops.
Sometime it is present as flat, slightly raised whitish plaque
Histopathological Features on roughened surface.
There is proliferation of hyperkeratotic stratified squamous Sign: They become less conspicuous when the mucosa
epithelium arranged in finger like or pointed projection is stretched. It is non-tender. After drying, the lesion it
with connective tissue cores. reveals finely granular surface texture. These lesions are
Viral Infection
soft having size of 1 to 5 mm in diameter with same color at the time of onset of sexual activity, poverty, overcrowding
as adjacent mucosa. and poor hygiene. These cases are also reported in HIV
infection, atopic dermatitis and Darier’s disease.
Cobblestone or fissured appearance: Sometimes cluster 519
so closely together that they appear as cobblestone or Clinical Features
fissured appearance.
They are pale to normal in color. They often appear to Age and incubation period: Incubation period is 14 to 50
undergo spontaneous regression after 4 to 6 months. days. It is more common in children and young adults.
Site: It is more common on skin or inner thigh lower
Histopathological Features abdomen or external genitals.
There is thickening of spinous layer with presence of
Appearance: It manifests as multiple or isolated discrete
mitosoid cells (altered nucleus which resembles mitotic
elevated nodules, or sometimes papules, with depressed
figure) in the upper layer.
centers, which may be keratinized and are normal or
It compromised hyperplastic epithelium without
slightly red in color.
keratosis and thickening or elongation of rete pegs. The
lamina propria of the underlying connective tissue may Signs: These lesions are hemisphere in shape, usually
occasionally show some signs of inflammation. about 5 mm in diameter.
There is acanthosis with mild hyperparakeratosis Some of lesion are surrounded by mild inflammatory
usually present over the surface. erythema and slightly tender or pruritic.
Lymphocyte infiltration with occasional collection of
polymorphonuclear leukocytes with some focal areas of Oral Manifestations
liquefaction degeneration of the basal layer may be found. Site: It usually occurs in children, on the face, due to shared
There is lack of pronounced elongation of thin rete sleeping accommodation. Most commonly involved sites
ridges. The ridges are themselves are widened, which are are lips, tongue and buccal mucosa.
confluent and club shaped.
Appearance: Lesions are similar to skin lesions.
Management
These are harmless lesion and it will regress spontaneously.
It shows thickening and down growth of epithelium with the
Surgical approach: Lesion can be removed with the help formation of large eosinophilic intra-cytoplasmic inclusion
of cryotherpay, laser ablation or simple excision. bodies known as Henderson-Paterson inclusion or simply
Topical interferon-b and systemic interferon-α can molluscum bodies, measuring approximately 25 microns
also produce results in some cases. in diameter (Fig. 20.16). These bodies characteristically
Points to Remember
Heck disease, papovavirus group, multiple nodular
lesions with sessile base, less conspicuous when the
mucosa is stretched, cobblestone or fissured appearance,
thickening of spinous layer, mitosoid cells (altered
nucleus which resembles mitotic figure), hyperplastic
epithelium, signs of inflammation, lymphocyte
infiltration, lack of pronounced elongation of thin rete
ridges, topical interferon-b.
MOLLUSCUM CONTAGIOSUM
INFECTION
It is caused by virus molluscum contagiosum of pox group. It
occurs due to intimate skin contact. There is marked increase Figure 20.16 Molluscum bodies in contagiosum
are accumulated in the crater formed by the distinctive Sign: There is also hepatosplenomegaly, jaundice,
central umblicated or the dome shaped lesion. petecheal hemorrhages, pneumonia, cerebral calcification
and hearing defect can also occur.
520 Management
It is self limiting and regresses spontaneously within 1 to Oral Manifestations
2 months. Signs: There is presence of gingivitis, gingival hyperplasia
It includes curettage, followed by local cautery, and oral ulcer (Fig. 20.17).
cryotherapy.
Acute sialadenitis: This can be present in patients who are
Topical application of caustic acid and irritants such as
immunocompromised. In this case xerostomia is present
phenol, TCA, podophyllin and cantharidin can be done.
and gland is painful.
In immunocompromised patient antiviral agents like
cidofivir can be effective. Developmental tooth defect: This is present due
to neonatal CMV infection. There is diffuse enamel
Points to Remember hypoplasia, attrition, hypomaturation and yellow coloration
Molluscum contagiosum of pox group, multiple or due to underlying dentin.
isolated discrete elevated nodules, hemisphere in shape,
down growth of epithelium, intra-cytoplasmic inclusion, Histopathological Features
Henderson-Paterson inclusion, molluscum bodies, There are changes within the vascular endothelial cells.
central umblicated or the dome shaped lesion, caustic Scattered infected cells are swollen showing intra-
acid, cidofivir, phenol, TCA, podophyllin. cytoplasmic and intranuclear inclusion and prominent
nucleoli. This enlarge cells are called owl eye cells. Sali-
vary gland epithelium can also show owl eye cells.
CYTOMEGALOVIRUS INFECTION
It is cause by cytomegalovirus (CMV or HHV-5). It may Management
be clinically expressed or latent. Prevention by passive immunization with hyper immune
This virus remains latent in salivary gland cells, gamma globulin can be successful.
endothelium, macrophages and lymphocytes. When Ganciclovir is recommended for management of
expressed, it results in characteristic enlargement of cells CMV infection. It gives relief in 75 percent of cases of
with prominent and pathognomonic, intranuclear and
intracytoplasmic inclusions.
It is widespread with prevalence rates based on the
presence of CMV antibodies, ranging from 80 to 100
percent, in most adult population.
Blood and transplanted tissues are also potential means
of transmission of virus to susceptible individuals, rather
than saliva, urine, serum, vaginal secretion. Infants can
acquire it in utero from maternal virus reactivation during
pregnancy.
Clinical Features
Age: It is seen in infant and young age group.
Symptoms: It may be accompanied by mononucleosis
like illness or severe illness with neurologic abnormalities.
Patient complaint of fever, joint and muscle pain, shivering,
abdominal pain, nonproductive cough, macular rash and Figure 20.17 Gingivitis in patient with cytomegalovirus
diarrhea. infection
Viral Infection
immunocompromised patient. Other antiviral drug which headache, arthralgias, paresthesia, depression and cognitive
can be used are foscarnet, cidofivir and valganciclovir. deficit.
Symptomatic treatment is given in patient who are
not immunocompromised. Antipyretic medication with Oral Manifestations 521
NSAIDs can be recommended. Location: Tiny petechiae appear on the soft palate, labial
and buccal mucosa during the course of the disease (Fig.
Points to Remember 20.18).
Cytomegalovirus, latent in salivary gland cells, mono- Acute gingivitis and stomatitis may be present and the
nucleosis like illness, hepatosplenomegaly, jaundice, lesion normality persisting for 3 to 11 days. The organism
petecheal hemorrhages, acute sialadenitis, developmental responsible for gingivitis is fusospirochetal.
tooth defect, vascular endothelial cells, intracytoplasmic, Intraorally, the most prominent sign is enlargement
intranuclear inclusion, prominent nucleoli, owl eye cells, and inflammation of the tonsils along with sore throat and
hyper immune gamma globulin, ganciclovir, foscarnet, difficulty in swallowing. Quite commonly the tonsils are
cidofovir. covered by a white or grayish pseudomembrane.
1/3rd of the patients with hemorrhagic tendency exhibit
oronasopharyngeal bleeding, including bleeding from
INFECTIOUS MONONUCLEOSIS
gingiva. Transient oral ulcerations and lymphadenopathy
It is also called as glandular fever, kissing disease, mono. can also occur.
It is a benign acute infectious disease caused due to
the Epstein Barr virus, a herpes virus which infects the Hematological Findings
B-lymphocytes. There is an absolute and relative increase in mononuclear
The mechanism of human transmission is not entirely cells which exhibit pleomorphism and an oval and kidney
known but one important mean is thought to be through shaped nucleus with a non granular or foamy cytoplasm.
deep kissing so this condition is also called as kissing The hemoglobin and platelet counts are normal. An
disease. EBV is present in oropharyngeal secretions and increase in white blood cell count and positive Paul Bunnel
mixed saliva during active phase. test are pathognomonic of this disease.
Clinical Features Diagnosis

Age and incubation period: It varies from 10 to 40 days Paul Bunnel test for infectious mononucleosis. Leukocyte
and the disease occurs chiefly in children and young adult count is between 4000 to 15000/dL.
in 15 to 30 age groups.
Location: Anterior and posterior cervical nodes are
enlarged and only slightly tender on palpation.
Symptoms: The patient usually complaint of sore throat
accompanied by fever usually 101°F to 103°F and extreme
fatigability.
Occasionally, there is a complaint of headache,
photophobia, nausea, vomiting, diarrhea and presence of
erythematous macular rash (morbilliform skin rash).
Signs: Physical examination reveals enlarged palatine
tonsils with copious amount of cheesy yellow exudate
filling tonsillar crypt. Enlargement of the superficial lymph
nodes, particularly the posterior cervical and splenomegaly
are common manifestations.
Chronic fatigue syndrome: In these syndrome patient Figure 20.18 Petechiae seen in palate in patient with
complaints of chronic fatigue, fever, pharyngitis, myalgias, infectious mononucleosis
Heterophill antibody test positive, atypical lymphocytes 9. Epstein JB, Scully C. Cytomegalovirus a virus of increasing
and clinical signs and symptoms make a diagnostic triad. relevance to oral medicine and pathology. J Oral Pathol
Med. 1993;22:348-53.
522 Management 10. Epstein JB, Sherlock CH, Wolber RA. Oral manifestation of
cytomegalovirus infection. Oral Surg Oral Med Oral Pathol.
Symptomatic: Nonaspirin containing antipyretics and 1993;75:443-51.
NSAIDs can be used to reduce common symptoms. 11. Fornatora ML, Reich RF, Gray RG, et al. Intraoral molluscum
Corticosteroid: This should be used properly and in life contagiosum: report of cases and review of literature: Oral
Surg Oral Med Pathol Oral Radiol Endod. 2001;92:318-20.
threatening situation. It reduces the duration of fever and
12. Guggenheimer J, Nowak AJ, Michaels RH Dental
shrinkage of tonsil.
manifestations of the rubella syndrome. Oral Surg Oral Med
Antiviral agents like acyclovir, valacyclovir and Oral Pathol. 1971;32(1):30.
famciclovir can be used. 13. Katz J, Guelmann M, Stavropolous F, et al. Gingival and
Acute tonsillectomy can be performed if it causing other oral manifestations in measles virus infection. J Clin
airway obstruction. Periodontol. 2003;30(7):665-8.
14. Laskaris G. Oral manifestations of infectious diseases. Dent
Points to Remember Clin North Am. 1996;40(2):395-423.
Glandular fever, kissing disease, mono, anterior and 15. Ledesma-Montes C, Vega Memije E, Garces-Ortiz M. et al.
Multifocal epithelial hyperplasia: report of nine cases. Med
posterior cervical nodes are enlarged, sore throat, fever,
ral Patol Oral Cir Buccal. 2005;10:394-401.
morbilliform skin rash, enlarged palatine tonsils, chronic 16. López-Sánchez A, Guijarro B, Hernández Vallejo G. Human
fatigue syndrome, tiny petechiae on soft palate, acute repercussions of foot and mouth disease and other similar
gingivitis, stomatitis, enlargement and inflammation of viral diseases. Med Oral. 2003;8(1):26-32.
the tonsils, oronasopharyngeal bleeding, mononuclear 17. Meer S, Coleman H, Altini M, et al. Mandibular osteomyelitis
cells, increase in white blood cell count, positive Paul and tooth exfoliation zoster CMV co infection: Oral Surg
Bunnel test, corticosteroid, acute tonsillectomy, acyclovir. Oral Med Oral Pathol Oral Radiol Endod. 2006;101:70-5.
18. Mendieta C, Miranda J, Bruet LI, et al. Alveolar bone necrosis
and tooth exfoliation following herpes zoster infection: a review
BIBLIOGRAPHY of literature and case report. J Periodontal. 2005;76:148-53.
19. Nesbit SP, Gobetti JP. Multiple recurrence of oral erythema
1. Anderson KM, Perez-Montiel D, Miles L, et al. The multiforme after secondary herpes simplex: a report of two
histologic differntriation of oral condyloma acuminatum case: J AM Dent Assoc. 1986;112:348-52.
from its mimics. Oral Surg Oral Med Oral Pathol Oral 20. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and
Radiol Endod. 2003;96:420-8. maxillofacial pathology, 3rd edn. Saunder Elsevier, 2009.
2. Arduino PG, Porter SR. Oral and perioral herpes simplex 21. Reborn GW, Grace MGA. Recurrent herpes simplex
virus type I (HSV-1) infection review of its management: labialis: selected therapeutic options. J Can Dent Assoc.
Oral Dis. 2006;12:254-70. 2002;133:303-9.
3. Barrett AP, Katelaris CH, Morris JGL, et al. Zoster sine 22. Sabella C. Measles: not just a childhood rash. Cleve Clin J.
herpete of the trigeminal nerve. Oral Surg Oral Med Oral 2010;77(3):207-13.
Pathol. 1993;75:173-5. 23. Simon PA. Oral condyloma acuminatum as an indicator
4. Buchner A. Hand foot and mouth disease: Oral Surg Oral sexual abuse: dentistry role. Quintessence Int. 1998;29:455-8.
Med Oral Pathol. 41:333-7. 24. Slote J, Saygun I, Sabeti M, et al. Epstein barr virus in oral
5. Carlos R, Sedano HO. Multifocal papilloma virus epithelial disease. J Periodontal Res. 2006;41:235-44.
hyperplasia. Oral Surg Oral Med Oral Pathol. 1994;77:631-5. 25. Torres R, Cottrell D, Reebye UN. Ulcerative tongue lesion
6. Cohen SG, Greenberg MS. Chronic oral herpes simplex secondary to cytomegalovirus. J Mass Dent Soc. 2004;53:36-7.
virus infection in immunocompromised patient. Oral Surg 26. Whitaker SB, Wiegand SE, Budnick SD. Intraoral molluscum
Oral Med Oral Pathol. 1985;59:465-71. contagiosum. Oral Surg Oral Med Oral Pathol. 72:334-6.
7. Courant P, Sobkov T. Oral manifestation of infectious 27. Woo SK, Challacombe SJ. Management of recurrent oral
mononucleosis. J periodontal. 1979;40:279-83. herpes simplex infection. Oral Surg Oral Med Oral Pathol.
8. Dewan P, Gupta P burden of Congenital Rubella Syndrome Oral Radiol Endod. 2007;S12.e1-S12.e18.
(CRS) in India: a systematic review; Indian Pediatr. 28. Zenner D, Nacul L. Predictive power of Koplik’s spots for the
2012;49(5):377-99. diagnosis of measles’. Infect Dev Ctries. 2012;6(3):271-5.
Viral Infection
1. Ballooning degeneration, vesicle formation and 6. Following are the Coxsackievirus infection, except: 523
inflammatory cells seen in: a. Herpangina
a. Herpes simplex b. Acute lymphonodular pharyngitis
b. Measles c. Zona
c. Herpes zoster d. Hand foot mouth disease
d. Herpangina 7. A characteristic corrugated and white appearance of
2. Presence of Koplik spot and microtubular aggregates tongue which cannot be rub off is:
is the histopathological feature of: a. Kaposi’s sarcoma
a. Herpes simplex b. Candidiasis
b. Measles c. Hand foot mouth disease
c. Herpes zoster d. Hairy leukoplakia
d. Herpangina 8. Most common tumor associated with AIDS is:
3. Floating Tzanck cells is the characteristic feature of: a. Kaposi’s sarcoma
a. Herpes simplex b. AOT
b. Measles c. CEOT
c. Herpes zoster d. Melanoma
d. Chicken pox 9. Interweaving band of spindle shaped and plump
4. Herpes zoster is also called as: endothelial cell is the feature of:
a. Morbilli a. Kaposi’s sarcoma
b. Shingles b. Candidiasis
c. Zona c. Hand foot mouth disease
d. Both b and c d. Hairy leukoplakia
5. A zoster infection of geniculate ganglion with 10. Tzanck cells, ballooning degeneration and Lipschutz
involvement of the external ear and oral mucosa is: bodies are present in:
a. Fanconi’s syndrome a. Herpangina
b. First arch syndrome b. Acute lymphonodular pharyngitis
c. James Ramsay Hunt syndrome c. Zona
d. Eagle’s syndrome d. Secondary herpetic infection
21 Acquired Immunodeficiency
Syndrome
Chapter Outline
Â Definition Â Herpes zoster

Â AIDS related complex Â HIV associated salivary gland diseases
Â Prevalence Â Idiopathic thrombocytopenic purpura
Â Etiology Â Mycobacterium infection
Â Characteristic of HIV virus Â Hyperpigmentation
Â Mechanism of action Â Histoplasmosis
Â Transmission Â Recurrent aphthous stomatitis
Â Clinical features Â Molluscum contagiosum
Â Oral manifestations Â Oral squamous cell carcinoma
Â Candidiasis Â Diagnostic tests
Â Kaposi’s sarcoma Â Screening test for AIDS
Â Hairy leukoplakia Â Enzyme-linked immunosorbant assay
Â Periodontal disease associated with HIV Â The Western Blot method
Â Non-Hodgkin lymphoma Â Viral culture and polymerase chain reaction
Â Recurrent herpes labialis Â Surrogate marker for progression of HIV-I infection
Â Oral human papilloma virus lesions Â Management
Acquired immunodeficiency syndrome (AIDS) is a and HTLV-II) that are capable of oncogenic transformation
devastating fatal disease, which is in epidemic form and are usually associated with leukemia or lymphoma.
throughout the world. It is an incurable viral STD caused The case of AIDS was detected in June 1981 when
by human immunodeficiency virus (HIV). It stands for: 5 young homosexuals men came with the suffering
∙ A: Acquired, i.e. contagious not inherited from rare lung infection due to microorganism called
∙ I: Immune, i.e. power to receive disease Pneumocystis carinii. In India, the first description of
∙ D: Deficiency AIDS came in Madras where 6 women out of 125 who
∙ S: Syndrome, i.e. number of signs and complains were screened were HIV positive in high-risk group of
indicative of particular disease. prostitutes.
Four identified etiological agents are of substantially The AIDS appear to be endemic in central and
lenti virus (HIV-I an HIV-II) that cause slow infection in equatorial Africa and it may be old disease of Africa that
which sign and symptoms only appear after many months or has gone unrecognized. The HIV-1 infection has also
years of infection and two member of oncovirus (HTLV-I become the primary emphasis of effort at controlling
Acquired Immunodeficiency Syndrome
sexually transmitted diseases (STDs). Moreover, the DEFINITION

knowledge gain about sexual and other behavior associated
with transmission of HIV, as well as strategies that have World Health Organization (WHO) has given following
been effective in modifying those behaviors, is transferable definition of AIDS: 525
to other sexually transmittable and bloodborne infections One or more opportunistic infections listed in clinical
and has revolutionized standard approaches to the control features that are at least moderately indicative of underlying
of these infections. cellular immune deficiency.
Oral and perioral lesions are common presenting Absence of all known underlying causes of cellular
features in patient with human immunodeficiency virus immune deficiency (other than HIV infection) and absence
and may have deterioration of general health and a poor of all other causes of reduced resistance reported to be
prognosis. associated with at least one of those opportunistic diseases.
CLASSIFICATION
1st Classification (given in 1993) by Center for Disease Control (CDC)
CD4 + T-cell categories A B C
Asymptomatic, acute HIV Symptomatic, not A or C AIDS indicator condition
and PGL conditions
More 500/µL A1 B1 C1
200 to 499/µL A2 B2 C2
Less than 200/µL A3 B3 C3
AIDS indicator T-cell count
Category A: In adolescent less than 13 years with ∙ Invasive cervical cancer

documented HIV infection: ∙ Disseminated or extrapulmonary coccidioidomycosis,
∙ Persistence generalized lymphadenopathy extrapulmonary cryptococcosis
∙ Active condition. ∙ Chronic intestinal cryptosporidiosis more than 1 month
duration
Category B: Condition is attributed to HIV infection or
∙ Cytomegalovirus retinitis with loss of vision
indicative of defect in the cell-mediated immunity.
∙ HIV related encephalopathy
∙ Bacillary angiomatosis
∙ Herpes simplex bronchitis, pneumonitis and esophagitis
∙ Oropharyngeal and vulvovaginal candidiasis
∙ Kaposi sarcoma, Burkitt’s lymphoma and immunoblastic
∙ Cervical carcinoma in situ
lymphoma
∙ Constitutional symptoms like fever (38.5˚C) and
∙ Mycobacterium tuberculosis infection at any pulmonary
diarrhea
or extrapulmonary sites
∙ Oral hairy leukoplakia and herpes zoster
∙ Pneumocystic carinii pneumonia and recurrent
∙ Idiopathic thrombocytopenic purpura.
pneumonia
Category C: AIDS indicative condition ∙ Progressive multifocal leukoencephalopathy
∙ Candidiasis of bronchi, trachea or lung and esophageal ∙ Toxoplasmosis of brain and wasting syndrome
candidiasis ∙ Recurrent Salmonella septicemia.
2nd Classification by USPHS-CDC PREVALENCE

∙ Group I: Acute infection It is more common in Western countries particularly
526 ∙ Group II: Asymptomatic infection in the United State. Largest population of AIDS is in
∙ Group III: Persistence generalized lymphadenopathy. homosexuals, intravenous drug users and, heterosexuals
∙ Group IV: Other disease with sexual contact with AIDS patient. Patients who
– Subgroup A: Constitutional diseases received transfusion of blood or blood pigments donated by
– Subgroup B: Neurological diseases the person with risk factors. Ninety-two percent of victims
– Subgroup C: Secondary infectious diseases are males, 6.5 percent female with 1 percent children. It is
- C1: Specified secondary infectious diseases common at the age of 25 to 49 years.
listed in CDC surveillance definition for
Etiology
AIDS.
- C2: Other specified secondary infectious T lymphocytes: There is quantitative and qualitative
stages. deficiency of T4 helper cells in AIDS patients, which lead
– Subgroup D: Secondary cancer to certain investigators to focus their efforts on determining
– Subgroup E: Other conditions. if etiologic agent was a virus that manifested a particular
tropism for T4 helper lymphocytes.
AIDS RELATED COMPLEX HTLV-III virus: Dr Robent C Galleo determined

that type C retrovirus was tropic for T4 lymphocytes in
For clinical and research studies, persons exhibiting adult T-cell leukemia/lymphoma. He named the virus,
complex clinical problems and immunological or Humans T-cell leukemia/lymphoma virus (HTLV–I). So
hematological abnormalities on the laboratory tests, have it is considered to be etiological agent for AIDS. But as
been classified as having AIDS related complex (ARC). it causes lymphoproliferation in T-cell leukemia, where as
The ARC requires any two or more symptoms and two or AIDS is a disease of lymphodepletion. The answer came in
more abnormal laboratory findings. It must be present for the discovery of type D retrovirus of HTLV family that has
at least 3 months. been termed as HTLV-III.
LAV virus: On the other hand, virus called lymphadeno-
pathy associated virus (LAV), was being isolated from the
Symptoms Laboratory findings AIDS patient in Europe.
• Lymphadenopathy • Decreased number of T HTLV-III and LAV is closely related members of same
• Weight loss of 15 lbs or helper cell class of virus. Finally, it is proved that HTLV and LAV
10% of body weight • Decreased ratio of T helper are cytopathic human T–lymphocytotropic viruses that
• Fever of 38.5˚C which is cells to T suppressor cells manifested selective infectivity for the helper/inducer
intermittent or continuous • Anemia or leukopenia
subset of T-cells that as phenotypically designated
• Diarrhea, fatigue and or thrombocytopenia or
reactivity with monoclonal antibody T4 or Leu3.
malaise lymphopenia
• Night sweats • Increased serum globulin HIV: In order to avoid different nomenclatures
level retrovirus responsible for the AIDS are named ‘Human
• Decreased blastogenic immunodeficiency virus’ which belong to family of
response of lymphocytes to
retroviruses.
mitogen
• Increased level of Risk person: Six groups are at risk of developing AIDS.
circulating immune These are homosexuals or bisexuals—71.4 percent,
complex intravenous drug users—18.4 percent, hemophilia,
• Cutaneous anergy to recipient of multiple blood transfusion, infant born of
multiple the skin test parents belonging to first three high-risk groups and
antigens
heterosexual contacts of high-risk group.
CHARACTERISTIC OF HIV VIRUS T4 lymphocyte → this viral DNA then becomes integrated
into the host chromosomes → the chromosomal integration
The HIV is a spherical enveloped virus, about 90 to is prerequisite for replication of retroviruses, but also for the
120 nm in size (Fig. 21.1). The nucleocapsid has an outer latency → once the viral genes are integrated into cells of own 527
icosahedral shell and inner cone shaped core, enclosing the DNA, they can apparently remain dormant for an indefinite
ribonucleoproteins. The genome is diploid, composed of period of time, without causing its affects. This is called
two identical single stranded, positive sense RNA copies. ‘incubation period’ → once the viral gene is activated, virus
Inside the envelope is a protein core, which contain enzymes particles convert T4 lymphocytes into AIDS virus factory →
reverse transcriptase, intregrase, protease, etc. all essential for when the number of T4 lymphocyte is severely depleted, the
viral replication and maturation. When the virus infects a cell, immune system collapses and variety of infections occur → at
the viral RNA is transcribed by the enzymes, first into single this stage patient is said to have AIDS.
stranded DNA and then to double stranded DNA (provirus),
which is integrated into the host cell chromosomes. The virus Transmission
is extremely sensitive to heat, thus boiling and autoclaving are Repeated intimate contact: It is in 90 percent of cases.
very effective measure of inactivating the virus. It depends upon number of sexual partners, receptive anal
intercourse and presence of other STDs. All these are in
Mechanism of Action high-risk group. Prostitution is a major heterosexual factor
The HIV attacks the immune system of the body. Due associated with AIDS.
to that an individual is not able to protect himself from Use of contaminated blood products: Intravenous drug
potentially harmful organism. users, HIV contaminated blood transfusion, blood clotting
concentrate and organ transplantation.
Normal mechanism: Pathogenic viruses → identified
by macrophage → it activates T lymphocytes → it get Perinatal transmission: It occurs in 13 percent among
differentiated into effecter cell like T helper cell or T4 children born to HIV seropositive mother.
and T suppresser cell or T8 → T4 cells secrete various Other nosocomial routes: Transmission from patient to
lymphokines which induce lymphocyte to differentiated patient due to reuse of contaminated and shared needles.
into plasma cell → it secrete specific antibodies against
viral antigen → it destroy the virus. Professional hazards: The risk of transmission from HIV
infected patient to health care workers is more than health
Mechanism in AIDS: HIV virus is lymphotropic virus care workers to patient.
→ its primary target is T4 cell → when the virus enters the
bloodstream, it integrates into gene into DNA of some primary CLINICAL FEATURES (FIG. 21.2)
Protozoan and helminthes infection: Cryptosporidiosis
(intestinal) causing diarrhea for over one month. The
most common opportunistic infection is by Pneumocystis
carinii which causes pneumonia. CNS infection or other
disseminated infections and toxoplasmosis.
Fungal infection: Candidiasis causing esophagitis,
cryptococcosis causing CNS infection, disseminated
histoplasmosis and bronchial or pulmonary candidiasis.
Bacterial infections: Mycobacterium avium intracellulare
causing infection disseminated beyond lung and lymph
node. Mycobacterium tuberculosis will cause tuberculosis.
Viral infections: Cytomegalovirus causing infection in the
internal organs other than liver, spleen and lymph nodes.
Herpes simplex virus, causing chronic mucocutaneous
Figure 21.1 HIV virus infection with ulcers persisting more than one month.
528
Figure 21.2 Features of HIV infection
Malignancy: Kaposi’s sarcoma and squamous cell Oral Disorders in HIV Disease
carcinoma. Lymphoma limited to bronchi and non- ∙ Fungal
Hodgkin’s lymphoma. More common
AIDS dementia complex: This occur in patient with HIV – Candidiasis
infection and causes progressive encephalopathy. Less common
– Aspergillosis
ORAL MANIFESTATIONS – Histoplasmosis
– Cryptococcus neoformans
Oral manifestations of HIV disease are common and – Geotrichosis
include oral lesions and novel presentations of previously ∙ Bacterial
known opportunistic diseases. More common
Careful history taking and detailed examination – HIV gingivitis
of the patient’s oral cavity are important parts of the – HIV periodontitis
physical examination and diagnosis requires appropriate – Necrotizing gingivitis
investigative techniques. Less common
Early recognition, diagnosis and treatment of HIV- – Mycobacterium avium intracellulare
associated oral lesions may reduce morbidity. The – Klebsiella pneumoniae
presence of these lesions may be an early diagnostic – Enterobacter cloacae
indicator of immunodeficiency and HIV infection may – E. coli
change the classification of the stage of HIV infection and – Salmonella enteritidis
is a predictor of the progression of HIV disease. Around 95 – Sinusitis
percent of AIDS patients have head and neck lesions and – Exacerbation of apical periodontitis
about 55 percent have important oral manifestation. They – Submandibular cellulitis
are described below.
∙ Viral Group II—less commonly associated with HIV

More common infection
– Herpes simplex ∙ Bacterial infection (Mycobacterium)
529
– Varicella zoster ∙ Melanotic hyperpigmentation
– Epstein-Barr virus including hairy leukoplakia ∙ Necrotizing ulcerative stomatitis
Less common ∙ Salivary gland disease
– HPV virus ∙ Thrombocytopenia purpura
– CMV virus ∙ Oral ulceration NOS (not otherwise specified)
∙ Neoplasm ∙ Viral infections like herpes simplex, zoster,
More common papillomavirus.
– Kaposi’s sarcoma
Group III—seen in HIV infection
Less common
∙ Bacterial infection (Actinomyces israelii, E. coli,
– Non-Hodgkin lymphoma
Klebsiella pneumonia)
– Squamous cell carcinoma
∙ Cat scratch disease
∙ Lymphadenopathy
∙ Epithelioid angiomatosis
∙ Neurologic disorders
∙ Drug reaction
Less common
∙ Fungal infection other than candidiasis
– Paresthesia
∙ Neurologic disturbance
– Facial palsy
∙ Recurrent aphthous stomatitis
– Hyperesthesia
∙ Viral infection like cytomegalovirus, molluscum
– Dysphagia
contagiosum.
∙ Miscellaneous
Less common
– Recurrent apthous ulceration
Candidiasis
– Progressive necrotizing ulceration Oral candidiasis is most commonly associated with Candida
– Toxic epidermolysis albicans, although other species, such as C. glabrata and
– Delayed wound healing C. tropicalis, are frequently part of the normal oral flora.
– Thrombocytopenia A number of factors predispose patients to develop can-
– Xerostomia and sicca type syndrome didiasis: infancy, old age, antibiotic therapy, steroid and oth-
– HIV embryopathy er immunosuppressive drugs, xerostomia, anemia, endocrine
– Hyperpigmentation disorders and primary and acquired immunodeficiency.
– Granuloma annulare Candidiasis is a common finding in people with HIV
– Exfoliative cheilitis infection. Reports describe oral candidiasis during the
– Lichenoid and other drug reaction. acute stage of HIV infection, but it occurs most commonly
with falling CD4+ T-cell count in middle and late stages of
HIV disease.
EC-Clearinghouse Classification of Oral Several reports indicate that most persons with HIV
Manifestation of HIV infection carry a single strain of Candida during clinically
Group I—strongly associated with HIV infection apparent candidiasis and when candidiasis is quiescent.
∙ Candidiasis
∙ Hairy leukoplakia Clinical Features
∙ Kaposi’s sarcoma Location: Patient with a HIV usually has lesion of hard
∙ Non-Hodgkin’s lymphoma palate and soft palate.
∙ Periodontal disease (linear gingival erythema,
Appearance: The clinical appearances of oral candi-
necrotizing gingivitis and necrotizing periodontitis).
diasis vary. The most common presentations include
clinical lesions are examined using potassium hydroxide

(KOH), PAS, or Gram’s stain.
Smear shows candidal organism embedded in
530 superficial keratin.
Management
Topical clotrimazole is treatment option in case of
candidiasis associated with HIV infection.
Systemic fluconazole is given if the CD4+ count is below
50 cell/mm3. In some patient itraconazole and intravenous
amphotorecin B are given to combat severe infection.
Points to Remember
C. glabrata and C. tropicalis, falling CD4+ T-cell count,
Figure 21.3 Candidiasis in AIDS patient pseudomembranous, hyperplastic, angular, and erythe-
matous candidiasis, burning mouth, smears, embedded
in superficial keratin, topical clotrimazole, systemic flu-
pseudomembranous, hyperplastic, angular, and erythe- conazole, itraconazole and intravenous amphotorecin B.
matous candidiasis, which are equally predictive of the
development of AIDS and angular cheilitis (Fig. 21.3). Kaposi’s Sarcoma
It is also called angioreticulo-endothelioma. It is the most
Symptoms: These lesions may be associated with a variety
common tumor associated with AIDS and occurs in 1/3rd
of symptoms, including a burning mouth, problems in
of AIDS patients.
eating spicy food and changes in taste. All three of these
common forms may appear in one individual. Etiology
Pseudomembranous candidiasis (Thrush): Charac- There is higher incidence of Kaposi’s sarcoma is in
teristic creamy white, removable plaques on the oral homosexual men with AIDS as compared to heterosexuals
mucosa are caused by overgrowth of fungal hyphae mixed with AIDS. It has been suggested that there is transmissible
with desquamated epithelium and inflammatory cells. The agent prevalence in homosexual population, which
mucosa may appear red when the plaque is removed. This stimulate certain factor such as angiogenesis protein that
type of candidiasis may involve any part of the mouth or may be critical in the pathogenesis of neoplasm.
pharynx. The patient with AIDS often shows clustered lesion
in the oral cavity, which suggests direct inoculation of
Erythematous candidiasis: Erythematous candidiasis mucosa with sexually transmitted agent.
appears as flat, red patches of varying size. It commonly Some theories suggests role of cytomegalovirus in
occurs on the palate and the dorsal surface of the tongue. the pathogenesis of Kaposi’s sarcoma, but studies on
Erythematous candidiasis is frequently subtle in appearance prevalence of antibodies to cytomegalovirus in patient
and clinicians may easily overlook lesions, which may with classic and epidemic Kaposi’s sarcoma have failed to
persist for several weeks if untreated. demonstrate role of cytomegalovirus.
Angular cheilitis: Angular cheilitis appears clinically Nowadays, it has been stated that Kaposi’s sarcoma is
associated with human herpes virus (HHV 8). The HHV 8
as redness, ulceration and fissuring, either unilaterally or
is detected in oral epithelial cells and in oropharynx.
bilaterally at the corners of the mouth. It can appear alone
or in conjunction with another form of candidiasis.
Types
Histopathological Features • Classic
• African (cutaneous variant)
Candida is a commensal organism in the oral cavity.
• African (lymphadenopathy variant)
Candidiasis is diagnosed by its clinical appearance and
• Kaposi’s sarcoma associated with AIDS.
by detection of organisms on smears. Smears taken from
Epidemiology and Form on any part of the oral mucosa including the gingiva, soft
palate, buccal mucosa and in the oropharynx. It can involve
Kaposi’s sarcoma appears in various forms like classic,
either alone or in association with skin and disseminated
African (cutaneous variant), African (lymphadenopathy 531
lesions. It may be the first symptom of AIDS.
variant) and Kaposi’s sarcoma associated with AIDS.
Classic type is a rare neoplasm and occurs in the older Appearance: It can appear as a red, blue, or purplish lesion.
man. Usually, it appears as blue-black macule on the lower It may be flat or raised solitary or multiple. Occasionally,
extremities. It is slow growing and rarely involves the yellowish mucosa surrounds the lesion. The lesions may
lymph nodes and visceral organs. enlarge, ulcerate and become infected. Good oral hygiene
African Kaposi’s sarcoma is considered an endemic is essential to minimize these complications.
disease and affects children, 10-year-old or younger
Sign: It may vary in size from few millimeter to a centimeter
patients, more common in men than women. It appears as
or more in diameter and are tender and painful.
exophytic growth located in legs and arms. This form is
locally aggressive and lymph nodes involvement is rare. Histopathological Features
The lymphadenopathic form occurs in children of 10 years
It consists of interweaving band of spindle shaped and
age and younger with same frequency in men and women.
or plump endothelial cell and atypical vascular channels,
The visceral and massive nodal involvement is common.
enmeshed in reticular or collagen fibers.
Kaposi’s sarcoma is observed in patients with kidney
It consists of numerous, small capillary type blood
transplantation and in patients who receive the immuno-
vessels, which may or may not contain blood. Inflammatory
suppressive drugs for variety of diseases. Drugs such as
cell infiltration is common (Fig. 21.4).
prednisolone, cyclosporine and cyclophosphamide have
In late stage, lesion consists of well defined nodules or
been associated with development of Kaposi’s sarcoma.
lesions with diffuse involvement of the lamina propria.
It usually affects legs, arms, lymph nodes and visceral
organs.
Histopathological Stages
Clinical Features • P atch stage (macular): In this proliferation of
miniature vessels
Age and sex distribution: It is more common in male as
• Plaque stage: It shows further proliferation of
compared to female in ratio of 20:1. It can occur at any age
vascular channels with development of spindle cells
but most common in 5th, 6th, 7th decade except in Africa
• Nodular stage: Spindle cell increase to form nodular
where it more common in children.
tumor like mass.
Site: Commonly affects skin, oral and visceral organs. It
occurs commonly in head and neck region. Tip of nose is
peculiar and frequent location of it. It can involve lymph
nodes, soft tissue, extremities, GIT, lung, liver, pancreas,
spleen and adrenal gland.
Appearance: It begins as multinucleated neoplastic
process that manifests as multiple red or purple macules
and in more advanced stage, a nodule occurring on the skin
or mucosal surface.
Sign: Size of it ranges from a few millimeters to a centimeter
or more in diameter and are usually tender on palpation. It
is slow growing but can behave as a very aggressive lesion
with rapid visceral involvement.
Oral Manifestations
Location: It has tendency to involve the oral cavity, with
hard palate as the most common site. But lesions may occur Figure 21.4 Capillary blood cell seen in Kaposi’s sarcoma
Management Clinical Features

Treatment is determined on the basis of the number, size Location: It is unique and significant lesion which
532 and location of the oral lesions. The choice of therapy primarily occurs unilaterally or bilaterally on the lateral
depends on the effect of treatment on the adjacent mucosa, border of tongue. It can also occur on dorsum of the tongue,
pain associated with treatment, interference with eating buccal mucosa, floor of mouth, retromolar area and soft
and speaking and the patient’s preference. palate. Many time lesion start on lateral border and spread
It is important to perform thorough dental prophylaxis to entire dorsum of tongue.
before initiating therapy for lesions involving the gingiva.
Appearance: There is characteristic corrugated and
Response to therapy is improved if all local plaque and
white appearance. It does not rub off and may resemble
calculus are removed. Local application of sclerosing
the keratotic lesion. The surface is irregular and may
agents may reduce the size of oral lesions.
have prominent folds or projections, sometimes markedly
Local treatment is appropriate for large oral lesions that
resembling hairs. Occasionally, however, some areas may
interfere with eating and talking. Oral lesion can be treated
be smooth and flat (Fig. 21.5).
surgically or with localized intralesional chemotherapy.
Intralesional vinblastine, radiation therapy, intra- Pseudo hairy leukoplakia: Sometimes, the white lesion
venously interferon alpha and sclerotherapy with 3 percent satisfies many criteria for diagnosis of hairy leukoplakia,
sodium tetradecyl sulfate. but if EBV not present this is called ‘pseudo hairy
leukoplakia’. Presence of hairy leukoplakia is fairly
Points to Remember indicator of HIV pro-sensitivity and is predictor of
Angioreticulo-endothelioma, affects skin, multinucle- deficiency immunocompetence.
ated neoplastic process that manifests as multiple red or
purple macules, hard palate, most common site, red, blue, Histopathological Features
or purplish lesion, yellowish mucosa surrounds the lesion, Histologically lesion shows hyperkeratosis, acanthosis,
spindle shaped and or plump endothelial cell, numerous, contain Epstein-Barr virus and no or minimum infla-
small capillary type blood vessels, Inflammatory cell mmation.
infiltration, thorough dental prophylaxis, intralesional
vinblastine, radiation therapy, intravenously. Balloon cells: The epithelial exhibits band-like zone of
lightly stained cells with abundant cytoplasm in the upper
spinous layer.
Hairy Leukoplakia
Oral hairy leukoplakia, which presents as a non-movable, Nuclear beading: There is presence of scattered cells with
corrugated or “hairy” white lesion on the lateral margins nuclear clearing with pattern of peripheral margination of
of the tongue occurs in all risk groups for HIV infections,
although less commonly in children than in adults.
It occurs in about 20 percent of persons with
asymptomatic HIV infection and becomes more common
as the CD4+ T-cell count falls.
Etiology
Exact etiology is not known but Epstein-Barr virus has
identified in these lesions.
One hypothesis is that basal epithelial cells of lateral
margin of tongue normally harbors EBV in majority of
adult population, who are EBV sero-positive and carrier of
that disease.
It is found primarily in homosexual male. Direct
infection of Langerhans cell due to HIV induced loss of
factor essential for their integrity and function, permit Figure 21.5 Hairy leukoplakia showing characteristic
reactivation of EBV with frequent epithelial hyperplasia. corrugated appearance
chromatin on superficial epithelium. This is called nuclear Clinical Features

beading.
Site: It often occur in clean mouths, where there is very
Immunochemistry tissue in situ hybridization, non-
little plaque or calculus to account for the gingivitis. 533
invasive tissue in situ hybridization, or electron microscopy
does demonstrate of Epstein-Barr virus. The lesion of Sign: The onset is often sudden, with rapid loss of bone and
leukoplakia consists of Langerhans cells. soft tissue. Patients sometimes complain of spontaneous
bleeding.
Management
Linear gingival erythema: In this condition gingiva may
Hairy leukoplakia usually is asymptomatic and does not be reddened involving the free gingival margin. There is
require treatment. Hairy leukoplakia is almost always a linear band of erythema which can extend 3 mm apically.
manifestation of HIV infection and clinicians should arrange The diagnosis of this should be done if gingivitis does not
evaluation of HIV disease and appropriate treatment for respond to improved plaque control (Fig. 21.6).
patients with hairy leukoplakia.
Hairy leukoplakia has disappeared in patients receiving Necrotizing ulcerative gingivitis: In these ulcers
high-dose acyclovir for herpes zoster, presumably because occurs at the tips of the interdental papilla and along the
of the anti-EBV activity of acyclovir. Doses of acyclovir gingival margins and often elicits complaints of severe
(2.5–3 mg per day for 2–3 weeks) usually eliminate HL, but pain. The ulcers heal, leaving the gingival papillae with a
the lesion usually recurs with cessation of treatment. characteristic cratered appearance.
Elimination or almost complete clinical resolution of Necrotizing ulcerative periodontitis may present as
the lesion has occurred in patients treated with agents such rapid loss of supporting bone and soft tissue. Typically,
as desciclovir (an analog of acyclovir), phosphonoformate, these losses occur simultaneously with no formation of
Retin A and podophyllin resin, although lesions tend to gingival pockets, sometimes involving only isolated areas
recur within few months. Occasionally, Candida albicans of the mouth. Teeth may loosen and eventually fall out, but
may be found in HL lesions. uninvolved sites can appear healthy. There is loss of more
Antifungal medications like topical clotrimazole, than 6 mm attachment.
topical nystatin 10000 unit/g 5 times a day can be given. Necrotizing stomatitis may develop and areas of
Systemic agent like ketoconazole 200 mg BD a day, necrotic bone may appear along the gingival margin. The
acyclovir, azidothymidine and retinoid acid podophyllin bone may eventually sequestrate. Patients with necrotizing
resin can also be given. ulcerative periodontitis and necrotizing stomatitis
frequently complain of extreme pain and spontaneous
Points to Remember bleeding.
Nonmovable, corrugated or hairy white lesion on the
lateral margins of the tongue, Epstein-Barr virus, does
not rub off, pseudo hairy leukoplakia, hyperkeratosis,
acanthosis, balloon cells, nuclear beading, demonstrate
of Epstein-Barr virus, Langerhans cells, high-dose
acyclovir, desciclovir, phosphonoformate, Retin A,
podophyllin resin, antifungal medications like topical
clotrimazole, topical nystatin 10000.
Periodontal Disease Associated with HIV

Periodontal disease is a fairly common problem in both
asymptomatic and symptomatic HIV-infected patients.
Form
• Linear gingival erythema
• Necrotizing ulcerative gingivitis
• Necrotizing ulcerative periodontitis. Figure 21.6 Linear gingival erythema
Management Appearance: Herpes labialis occurs as characteristic lip

lesion consisting of vesicles on an erythematous base that
Clinicians should refer patients to a periodontist or dentist
heals within 7 to 10 days. The recurrent herpes labialis
534 for management.
(RHL) are small, shallow, irregular and erosive-like lesion
The following protocol has achieved reasonable
that may coalesce and seen to occur only on keratinized
success: plaque removal, local debridement, irrigation
epithelium like that of gingiva, hard palate or dorsal surface
with povidine-iodine, scaling and root planning and
of tongue.
maintenance with a chlorhexidine mouth rinse once or
Diagnosis is made by isolation of virus from ulcer.
twice daily.
Antiviral drugs like acyclovir 30 mg/kg/day should be
The use of systemic antibiotics like metronidazole has
given. In case of resistant infection foscarnet is preferred.
been given in patient which has got extensive involvement.
Points to Remember
Points to Remember
HSV, vesicles on an erythematous base, antiviral drugs.
Linear gingival erythema, necrotizing ulcerative
gingivitis, necrotizing ulcerative periodontitis, necro-
tizing stomatitis, plaque removal, local debridement, Oral Human Papilloma Virus Lesions
irrigation with povidine-iodine, scaling and root It is cause by human papilloma virus. Oral warts,
planning, metronidazole. papillomas, skin warts and genital warts are associated
with the human papilloma virus (HPV) lesions. Because
Non-Hodgkin Lymphoma the HPV types found in oral lesions in HIV-infected
This is the second most common malignancy in HIV persons are different from the HPV types associated with
infection. It may cause by EBV virus infection and HHV 8. anogenital warts, clinicians should probably not use the
term condyloma acuminate to describe oral HPV lesions.
Clinical Features
Location: Lesions caused by HPV are common on the skin
Site: It is seen in extranodal location. CNS is the most and mucous membranes of persons with HIV disease. Anal
common site of involvement. Intraorally, it can be seen on warts have frequently been reported among homosexual
gingiva, palate, tongue, tonsil, and maxillary sinus. men. It can be found on any mucosal surface and are
Sign: There is loss of periodontal attachment and loosening contagious to both host and sex partner.
of teeth. Appearance: HPV lesions in the oral cavity may appear
Appearance: There is erythematous and soft tissue as solitary or multiple nodules. They may be sessile or
enlargement seen in oral cavity. pedunculated and appear as multiple, smooth-surfaced
raised masses resembling focal epithelial hyperplasia or as
Management multiple, small papilliferous or cauliflower-like projections.
Combination of chemotherapy and radiotherapy is Histopathologically lesion is sessile or papillary
effective. which is covered by acanthotic or hyperplastic stratified
squamous epithelium. Affected epithelium demonstrated
Points to Remember vacuolization of numerous epithelial cells which is called
‘koilocytosis’.
EBV virus infection and HHV 8, CNS involvement,
gingiva, loss of periodontal attachment, erythematous and Management
soft tissue enlargement, chemotherapy, radiotherapy.
Simultaneous irradiation of all lesions and care of infected
partner. Local excision and cauterization of base.
UNCOMMON ORAL MANIFESTATION
Points to Remember
OF HIV
Oral warts, papillomas, skin warts, homosexual men,
Recurrent Herpes Labialis solitary or multiple nodules, small papilliferous,
Recurrent herpes labialis mainly appears as herpes labialis cauliflower-like projection, koilocytosis, acanthotic or
and recurrent intraoral herpes. It is cause by HSV. hyperplastic stratified squamous epithelium.
Herpes Zoster individuals with xerostomia, the use of salivary stimulants

such as sugarless gum or sugarless candies may provide
It occurs more frequently in HIV infected patients and
relief. Candies that are acidic should be avoided as
carries poor prognosis. 535
frequent use may lead to loss of tooth enamel. The use
The occurrence of unilateral vesicles that break and
of salivary substitutes may also be helpful. An increase
scab is characteristic of this infection. They are self-
in caries can occur, so fluoride rinses (that can be bought
limiting. When herpes zoster infection involvements occur
over the counter) should be used daily and visits to the
intraorally, it will lead to sequestration of bone with loss of
dentist should occur two to three times per year.
teeth.
Main complication is neuropathy after inflammation.
Points to Remember
Diagnosis is made by cytological smear and finding of
multinucleated giant cells. Xerostomia, parotid gland, cytomegalovirus infection,
enlarged salivary glands, diffuse infiltrative lymphocytosis
Management syndrome, antiretroviral therapy.
Systemic acyclovir 800 mg orally or 15 to 30 mg/kg/day
IV 8 hourly for 10 to 15 days. Idiopathic Thrombocytopenic Purpura
Reports have described idiopathic thrombocytopenic
Points to Remember purpura (ITP) in HIV-infected patients. It can be cause by
Occurrence of unilateral vesicles, sequestration of bone, direct infection by HIV, immune dysfunction, alteration of
loss of teeth, neuropathy, systemic acyclovir. platelet production and drug reaction.
Oral lesions may be the first manifestation of this
HIV Associated Salivary Gland Diseases condition. Petechiae, ecchymosis and hematoma can occur
anywhere on the oral mucosa.
Salivary gland disease associated with HIV infection (HIV-
Spontaneous bleeding from the gingiva can occur and
SGD) can present as xerostomia with or without salivary
patients may report finding blood in their mouth on waking.
gland enlargement. Reports describe salivary gland
enlargement in children and adults with HIV infection Management
usually involving the parotid gland.
HAART has reduced the prevalence of thrombocytopenia.
The etiology of HIV-SGD is as yet unknown. It has
Other approach like splenectomy, intravenous immuno-
been postulated that cytomegalovirus infection has got
globulin, platelet transfusion is given.
predilection for salivary glands. Cytomegalovirus infection
produces inflammation which causes reduced salivary
Points to Remember
production.
Infection by HIV, immune dysfunction, alteration of
Sign: The enlarged salivary glands are soft but not platelet production, petechiae, ecchymosis and hematoma,
fluctuant, but the enlarged parotid glands can be a source spontaneous bleeding.
of annoyance and discomfort.
Xerostomia is sometimes seen in individuals with
Mycobacterium Infection
HIV-SGD. HIV-infected patients may also experience dry
mouth. It can results in tuberculosis which is cause by
Mycobacterium tuberculosis.
Diffuse infiltrative lymphocytosis syndrome: It is seen It involves tongue, gingiva, and buccal mucosa, floor of
in HIV infected salivary gland disease. There is salivary mouth, lip and palate.
gland enlargement. The glandular involvements result from
infiltration of CD8 lymphocytes. Appearance: There is chronic ulceration, exophytic
proliferation
Management Diagnosis: It is difficult as maximum cases do respond
Antiretroviral therapy is useful in this case. Removal of to tuberculin skin test. Confirmative diagnosis is done by
the enlarged parotid glands is rarely recommended. For AFB stained section of biopsy material.
Management Histopathologically surface epithelium forms several

hyperplastic down growth. There is presence of molluscum
Triple drug regimen is used. It includes rifampicin,
bodies which are large intracytoplasmic inclusion.
536 isoniazid, and pyrazinamide with ethambutol.
Management
Points to Remember
There is resolution of lesion after giving HAART therapy.
Chronic ulceration, exophytic proliferation, triple drug
regimen. Oral Squamous Cell Carcinoma
Squamous cell carcinoma of oral cavity, pharynx, and
Hyperpigmentation larynx has been seen in the HIV infected patients. The HIV
Hyperpigmentation of skin, nail and mucosa is seen in HIV infection accelerated the development of squamous cell
patient. carcinoma due to impaired immune surveillance.
There is increase melanin pigmentation occur in basal
layer of affected epithelium. Management
In some cases, adrenocortical destruction occur due to It is same as that of squamous cell carcinoma.
HIV resulting in addisonian pattern of pigmentation.
DIAGNOSTIC TESTS
Histoplasmosis
It is cause by Histoplasma capsulatum. It is most common Diagnosis of AIDS can be made by history and medical
type of fungal infection seen in patient with HIV other than examination for symptoms and signs. Individual is
candidiasis. generally young and in high risk group. There are several
circumstances in which, it is important for dentist to know
Sign: There is fever, splenomegaly and pulmonary HIV antibody status.
infiltrate. To ensure accurate differential diagnosis and appro-
Appearance: Orally, it is presented as indurated mucosal priate treatment of the oral lesions those are associated
ulceration with raised border. with development of AIDS.
Histopathologically, fungal organism are visible within To assess the risk to dental health care worker (HCW)
the cytoplasm of histiocytes and multinucleated giant cells. after needle stick or other injury from contaminated once
by contact with patients blood and saliva.
Management To convey with communicable disease control (CDC)
Intravenous amphotericin B is useful in this case. guideline that all HCW engaging in invasive dental practice
should be aware of HIV antibody status.
Recurrent Aphthous Stomatitis
In HIV infection all three forms of aphthous ulceration like SCREENING TEST FOR AIDS
minor, major and herptiform is seen. Enzyme-linked Immunosorbent Assay
There is well defined ulceration seen in the oral cavity. (Fig. 21.7)
Management Enzyme-linked immunosorbent assay (ELISA) is a color
reaction test in which prepared whole HTLV III virus
It is done with the help of topical and intralesional
particle which acts as antigen, will bind with antibodies to
corticosteroids.
HTLV III in an infected human serum.
Molluscum Contagiosum Then this complex is added to goat antihuman antibody
labeled with chemical enzymes, which gives color reaction
It is infection of skin cause by pox virus.
with particular chemicals e.g. orange color with peroxides
Location: It is seen on genital, trunk and facial region. labeled goat antihuman antibody.
Appearance: Lesion are small, waxy, dome-shaped ELISA test is simple and convenient to perform; it is
papules with center depressed crater. In HIV infection, this very sensitive for small amount of HTLV III antibodies
are hundred in number. and in initial screening tests.
antigen or reverse transcriptase assay provide method that

specifically demonstrates the presence of virus in given
patient or specimen.
The variable amount of virus present in peripheral 537
blood monocytes at different stage of infection and degree
of immunosuppression both influence the extent HIV-I
viremia.
Polymerase chain reaction technique has provided
an opportunity for detection of very small amount of an
infectious agent like HIV-I based on reported cycle of
enzymatic duplication of number of copies of either DNA
or RNA specific for microorganism.
Surrogate Marker for Progression of

Figure 21.7 ELISA kit for detection of AIDS HIV-I Infection
It is not 100 percent reliable because it is not completely The absolute CD4+ T-cell lymphocyte count correlate best
specific only for HTLV II antibodies and reacts with other with progression of HIV-I related immune dysfunction.
related viral antibodies and other nonspecific chemicals Other serum neoprotein beta-2-microreceptor HIV P24
giving false positive result of about 5 to 25 per 1000 antigen interleukin-2 receptor IgA and impaired delayed
patients. type of sensitivity are also used.
In case of positive test, there is strong indication of past
exposure and infection with virus but this does not show MANAGEMENT
whether the patient is infectious because it does not show Various drugs are used for immunotherapy are as follows:
whether virus is still active or has been destroyed.
Interferon: It is a useful therapeutic agent in this
The Western Blot Method syndrome of infection and neoplasms in view of their
antiviral antiproliferative and immunomodulater activity.
The prepared HTLV III virus particles consists of specific
The interferon is a glycoprotein produced by a number of
HTLV III specific proteins separated by a process called
different types cells. Type I interferon (alpha and beta) are
electrophoresis and subsequent transfer of nine protein
produced by leukocytes and fibroblasts. Type II interferon
bands to nitrocellulose membrane strip, which are reacted
(gamma) is produced by lymphocytes and monocytes.
with patient’s serum followed by an enzyme linked
Low doses of interferon enhance the antibody formation
antihuman antibody and enzyme substance.
and lymphocyte blastogenesis. They also prolong cell
Positive test is indicated when the treated, i.e. HTLV-
cycle and cause inhibition of intracellular enzyme system
III specific nitrocellulose strip are exposed to infected
(antineoplastic effect). The gamma interferon stimulate
human serum and a goat antihuman antibody strip display
macrophage oxidative metabolism and have antimicrobial
the characteristic band detected for each of three (env, pol
effect.
and gag) group of viral protein on exposure to X-ray film.
Those that react with only one of these three groups Thymic replacement therapy: The thymic epithelium
of antigens are termed indeterminate and those react with plays an important role in transformation of blood borne
only non viral proteins are negative. precursor cell into mature T-cells. Thymic hormones or
It is more specific for HTLV III antibodies and is used factor mediates this effect, since the immune system in
to eliminate false positive result. AIDS is characterized by numerical and functional defects
of T-cell lymphocytes, it will correct the immune defect.
Viral Culture and Polymerase Chain Reaction Transplant of fetal thymus of cultural thymic epithelium
Cultivation of HIV from blood and other body fluids and and injection of thymic hormone have been successfully
tissues with detection of the increased filter by either HIV utilized in treatment of AIDS.
Lymphokines and cytokines: Lymphokines are materials reverse transcriptase inhibitors, which are used are abacavir,
produced by lymphocyte. Interleukin-I is macrophage didanosine, emtricitabine, lamivudine, stavudine. Non-
product. In in vitro system interleukin-I enhance plague nucleosides used are capravirine, delavirdine, efavirenz
538 forming cells responses and the generation of cytotoxic and nevirapine. Protease inhibitor used is amprenavir,
T-cell alloantigen. In the presence of macrophage, darunavi, indianvir, tipranvir.
interleukin-1 stimulates the production of interleukin-2,
which stimulates and maintains the growth of T-cell PREVENTION
activated by antigens. Various studies have conformed
∙ Educational counseling of general public
that purified interleukin-2 (which stimulate and maintain
∙ Avoid sexual contact with suspect and in high-risk
growth of T-cell activated by antigen), preparation in
group
in vitro system can normalize lymphocyte reaction
∙ Use of disposable syringes and needles
in high percentage of individuals with unexplained
∙ Blood donor should be properly screened
lymphadenopathy and immunologic abnormalities, but the
∙ Avoid multiple sex partners, intimate kissing and oral
result are not significant in patients with AIDS.
contact
Bone marrow transplantation: Syngeneic (identical ∙ Educate healthcare workers on safety measures.
twin) allogenic (HLA/NHC matched) bone marrow
transplantation has been successful in reconstituting BIBLIOGRAPHY
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1. HIV is: 5. Causative organism for hairy leukoplakia is: 539

a. 90–120 nm b. 80–120 nm a. EBV b. HPV
c. 70–120 nm d. 50–100 nm c. Rota virus d. None of the above
2. AIDS dementia complex causes: 6. ELISA is a:
a. Progressive encephalopathy a. Color reaction test b. Electrophoresis test
b. Regressive encephalopathy c. PCR d. None of the above
c. Both a and b
d. None of the above 7. Balloon cells and nuclear beading are seen in:
a. Hairy leukoplakia
3. Most common fungal oral manifestation of HIV is:
b. Pseudo hairy leukoplakia
a. Kaposi’s sarcoma b. Candidiasis
c. Non-Hodgkin’s lymphoma
c. RAS d. Dysphagia
d. All of the above
4. Most common tumor associated with AIDS is:
a. Squamous cell carcinoma 8. The following are the histopathological stages of
b. Hodgkin’s lymphoma Kaposi’s sarcoma except:
c. Non-Hodgkin’s lymphoma a. Patch stage b. Plaque stage
d. Kaposi’s sarcoma c. Nodular stage d. Bullous stage
22 Odontogenic Infection
and Pulp Pathology
Anil Govindrao Ghom, Shubhangi Mhaske (Jedhe), Seema Vaidya
Chapter Outline
Â Effect of infection on host Â Infantile osteomyelitis

Â Pathophysiology of infection Â Diffuse sclerosing osteomyelitis
Â Pulp Â Primary chronic osteomyelitis
Â Classification of pulpitis Â Chronic tendoperiostitis
Â Pulpitis Â SAPHO syndrome
• Reversible pulpitis Â CRMO
• Chronic hyperplastic pulpitis Â Focal sclerosing osteomyelitis (condensing osteitis)
• Irreversible pulpitis Â Osteomyelitis with proliferative periostitis
Â Pulp degeneration Â Radiation osteomyelitis
Â Pulp calcifications Â Cellulitis
Â Necrosis of pulp Â Ludwig’s angina
Â Cracked tooth syndrome Â Fatal complications of oral infection
Â Periapical abscess • Bacterial meningitis
Â Periodontal abscess • Brain abscess
Â Acute exacerbation of a chronic lesion • Cavernous sinus thrombosis
Â Periapical granuloma • Odontogenic infection of orbit
Â Periapical scar • Mediastinitis
Â Osteomyelitis • Necrotizing fascitis
Â Acute suppurative osteomyelitis Â Oral foci of infections
Â Chronic suppurative osteomyelitis Â Dry socket
Infection is a clinicopathological entity involving invasion the microorganisms, the host resistance and anatomic
of the body by pathogenic microorganisms and reaction of geography.
the tissues to microorganisms and their toxins. These odontogenic infections can become severe, life-
Soft tissue infections of head and neck are commonly threatening facial space infections. These infections have
encountered in routine practice in dentistry. These the potential to spread through facial planes of head and
infections may be odontogenic or nonodontogenic in neck there by compromise the vital structures in these
origin. Once the infection extends past the apex of the regions, e.g. intracranial odontogenic infection leading to
tooth the pathophysiology of the infectious process necrotizing fascitis of head and neck. Most odontogenic
can vary, depending upon the number and virulence of infections can be managed successfully with minimal
Odontogenic Infection and Pulp Pathology
complications. The key to successful management is sound occur due to neutralization of host defence. There is
surgical principles. fever, endotoxic shock and intravascular coagulation.
The relationship between the host and microbes is a The pathogen or its products, in certain situations, may
dynamic one. Usually, host resistance is the dominant combine with antibodies or sensitized mononuclear 541
factor. On the other hand, when the host resistance is leukocytes to produce harmful immunologic effects called
lowered, microbes predominate and clinical infection hypersensitivity reaction.
occurs. In establishing the presence of infection there is Compromised host: It is a person whose defense
interaction between three viz factors host, environment and mechanisms have been lowered as a result of diabetes,
microbes. tuberculosis, rheumatic fever, malignancy, radiation
A compromised patient is more likely to have infection therapy, use of therapeutic immunosuppressive drug or
and this infection can rapidly acquire a serious form. Hence, antibiotics, extensive skin burns, genetic deficiency of
patient history is more important so as to recognize the immune system and malnutrition.
patient ability to defend himself against the infection. The
adverse relationship between the host and the infectious Spread of Infection
microorganism can be best understood by imagining a • Direct invasion or extension
balance on which the pathologic attribute of microbes are • Spread by lymphatic system
weighed against the protective mechanisms of the host. • Spread by blood vessels.
Body defends against the microbial invasion by three
major defenses local defense, cellular defense and humoral
defense. The microorganisms on the other hand use PATHOPHYSIOLOGY OF INFECTION
two weapons in this battle, i.e. virulence and number of The body’s response to infectious agent is inflammatory,
microbes. which is essentially a protective phenomenon. Hence, the
cardinal signs of inflammation are present, to some degree,
Factors for Host Defense in nearly all patients with infection.
Local defense Redness (rubor) is seen when the infection is close
• Epithelial lining to the tissue surface, which is secondary to the intense
• Secretory and drainage system hyperemia caused by increased vasodilation of arterioles.
• Microbial floral interference Calor or heat is due to inflow of relatively warm blood
• Mucosal immune system from deeper tissues, increased velocity of blood flow and
Humoral factor increased rate of metabolism.
Dolor or pain results from pressure on sensory nerve
• Immunoglobulin endings, from distension of tissues caused by edema or
• Complement system spread of infection. Release of substance like kinins,
Cellular component histamines or bradykinin is also responsible for pain. It is
• Polymorphonuclear leukocytes the most universal sign of infection.
• Lymphocyte. Swelling accompanies infection, unless the infection
is confined to bone which cannot swell. It is due to the
accumulation of fluid, exudate or pus.
EFFECT OF INFECTION ON HOST Loss of function is another sign of infection. A patient
Infectious agents initiate, in the host, a series of reactions immobilizes the painful part in the most comfortable
that are collectively called inflammatory reaction. This position he can find. Hence, when the masticatory muscles
response results in generation and release of mediators, are involved, there is limitation of jaw movement.
microvascular changes and mobilization and activation Fever occurs in some cases, which reflect a non-
of leukocytes, all designed to eliminate the infectious specific physiologic response of host to tissue injury.
pathogens and repair tissue injury. Therefore, these This injury results in increase of substance called pyrogen
reactions are protective in nature. from endogenous (injured tissue) and exogenous source
Direct injury to the host cells, enhancement of the (infecting agent). In clinical fever, it appears that the
parasite’s invasiveness and amplification of these effects hypothalamic regulating center is stimulated by endogenous
pyrogen, which is activated by bacterial endotoxin release

2nd Classification
from granulocytes, monocytes and macrophages.
According to involvement
542 Cardinal Sign of Inflammation • Focal or subtotal or partial pulpitis: In it
inflammatory process is confined to a portion of the
• Redness (rubor)
pulp, usually a portion of the coronal pulp.
• Calor or heat
• Total or generalized pulpitis: In it most of the pulp is
• Dolor or pain
involved.
• Swelling
• Loss of function According to severity
• Fever. • Acute
• Chronic
According to presence or absence of direct communication
PULP
between the dental pulp and oral environment
The pulp is the formative organ of the tooth. It builds • Pulpitis aperta (open pulpitis): In it pulp is
primary dentin during the development of the tooth, communicated with the oral cavity.
secondary dentin after tooth eruption and reparative dentin • Pulpitis clausa (closed pulpitis): No communication
in response to stimulation as long as the odontoblasts exists.
remain intact. The pulp has been described as both a
highly resistant organ and as an organ with little resistance
or recuperating ability. It is a delicate connective tissue
PULPITIS
liberally interspersed with tiny blood vessels, lymphatics, The initial response to injury to pulp is same as that of other
myelinated and unmyelinated nerves, and undifferentiated tissue, but the end results is different due to rigid dentinal
connective tissue cells. wall of pulp chamber.
CLASSIFICATION OF PULPITIS Pathogenesis

Some stimuli of short duration, such as cutting dentin may
1st Classification cause short-term vasodilation and a reversible increase in
vessel wall permeability.
Pulpitis (Inflammation)
More severe stimuli and a greater degree of cell
Reversible
damage cause more marked vasodilatation and the
• Symptomatic (acute)
movement of polymorphonuclear leukocytes into the
• Asymptomatic (chronic)
injured tissues.
Irreversible These acute inflammatory reactions are usually limited
• Acute to the odontoblast and subodontoblastic regions adjacent to
– Abnormally responsive to cold the dentinal tubules involved.
– Abnormally responsive to heat Odontoblastic nuclei may be displaced into the tubules,
• Chronic due to either increased local tissue pressure or dentinal fluid
– Asymptomatic with pulp exposure during the injury. It is reversible, if the etiological factors
– Hyperplastic are removed. Repair involves the return to normal tissue
– Internal resorption fluid dynamics, exit of polymorphonuclear leukocytes
Pulp degeneration from the area and the re-differentiation of odontoblasts
if they have damaged. The pulpal tissue after repair is
• Calcific
usually less vascular, more fibrous and less cellular than
• Others
before and may be less able to withstand a subsequent
Necrosis insult.
If stimuli are not removed, pulpal damage can i.e. change of position, exacerbates the pain which is due to
overwhelm the system and spread progressively to apical change in intrapulpal pressure.
portion of pulp. This will result in pulpal necrosis.
Referred pain: Patient may complain of pain referred to 543
Causes adjacent teeth to the temporal region or sinuses when an
upper posterior tooth is involved, or to the angle, when
Mechanical cause: It includes traumatic accident,
lower posterior tooth is affected.
iatrogenic damage for dental procedure, attrition, abrasion.
In later stages, pain is more severe and is generally
Thermal cause: It may cause through uninsulated metallic described as boring, gnawing or throbbing or as if tooth is
restoration, during cavity preparation, polishing. under constant pressure. Patient is often awake at night due
to pain. Pain is increased by heat and is sometimes relieved
Chemical cause: It arises from erosion or inappropriate
by cold, although continued cold may intensify the pain.
use of acidic dental material.
Bacterial cause: It can damage the pulp through the toxins Chronic Hyperplastic Pulpitis
secreted by bacteria from caries. It is also called pulp polyp or pulpitis aperta. It is essentially
an excessive, exuberant proliferation of chronically
Clinical Features inflamed dental pulp tissue. It occurs due to long standing
Reversible Pulpitis low grade infection. Mechanical irritation from chewing
It is a mild-to-moderate inflammatory condition of the pulp and bacterial infection often provides the stimuli.
caused by noxious stimuli in which the pulp is capable of Teeth involved: Teeth most commonly involved are
returning to un-inflamed state following removal of the deciduous molars and first permanent molars as they have
stimuli. an excellent blood supply because of a large root opening,
and this coupled with high tissue resistance and reactivity
Pulp hyperemia: It is the earliest form which is sometimes
in young person’s accounts for unusual proliferative
known as pulp hyperemia. There is excessive accumulation
properties of the pulp tissue. It is asymptomatic and it is
of the blood within the pulp tissue leading to vascular
seen only in teeth of children and young adults.
congestion.
Appearance: Polypoid tissue appears as a fleshy, reddish
Symptoms: It is characterized by sharp pain lasting for a
pulpal mass filling most of the pulp chamber or cavity
moment. It is more often brought on by cold than hot food or
or even extending beyond the confines of the tooth (Fig.
beverages and by cold air. It does not occur spontaneously
22.1). Sometimes, mass if large enough interferes with
and does not continue when the cause has been removed.
Tooth responds to electric pulp testing at lower current.
Irreversible Pulpitis
It is a persistent inflammatory condition of the pulp, which
may be symptomatic or asymptomatic and is caused by a
noxious stimulus.
Symptoms: In early stages of irreversible pulpitis, a
paroxysm of pain may be caused by the following: sudden
temperature changes like cold, sweet and acid foodstuffs.
The pain often continues when the cause has been removed
and it may come and go spontaneously.
Nature of pain: Pain is sharp, piercing or shooting and
is generally severe. It may be intermittent or continuous,
depending on the degree of pulpal involvement and
depending on whether it is related to an external stimulus. Figure 22.1 Chronic hyperplastic candidiasis showing reddish
The patient may also state that bending over or lying down pulpal mass
comfortable closure of teeth. Polypoid tissue is less

sensitive than normal pulp tissue and more sensitive than
the gingival tissue.
544
Signs and symptoms: It may cause discomfort during
mastication, due to pressure of food bolus. This tissue
bleeds easily because of rich network of blood vessels.
The tooth may respond feebly or not at all to the thermal
test. There is presence of squamous covering as a result
of grafting of exfoliated epithelial cells from adjacent oral
mucosa.

It may range from hyperemia to mild-to-moderate Figure 22.2 Necrosis of pulp in pulpitis
inflammatory changes to the area of the involved dentinal
tubules such as dentinal caries.
Microscopically one sees involved reparative dentin,
disruption of the odontoblastic layer, dilated blood
vessels, extravasation of fluid (edema) and the presence
of immunological response. Chronic inflammatory cells
predominate. In some cases acute inflammatory cells can
also be seen.
In irreversible pulpitis the postcapillary venules
become congested and affect the circulation within the
pulp, causing pathologic changes such as necrosis. These
necrotic areas attract polymorphonuclear leukocytes by
chemotaxis and start an acute inflammatory reaction.
After phagocytosis, the polymorpho-nuclear leukocytes,
which have a short life span, die and release lysosomal Figure 22.3 Pulp hyperemia showing cellular infiltrate and
enzymes. The lysosomal enzymes lyse some of the pulpal inflammatory cells
stroma and together with the cellular debris of the dead
polymorphonuclear leukocytes form purulent exudates
(pus).
The inflammatory reaction produces micro-abscess
(acute pulpitis). The pulp trying to protect itself walls off
the areas of the micro-abscess with fibrous connective
tissue. The surrounding pulp tissue exhibits fibrosis and
mixture of plasma cells, lymphocytes and histiocytes.
In some cases within a few days, the acute inflammation
spreads to involve most of the pulp so that neutrophilic
leukocytes fill the pulp. The entire odontoblastic layer
degenerates. As pulp is closed, there is rise in pressure
and the entire pulp tissue undergoes rapid disintegration,
forming numerous small abscesses. Eventually the entire
pulp undergoes liquefaction and necrosis which is called Figure 22.4 Pulp hyperemia showing many dilated blood
acute suppurative pulpitis. vessels and necrotic area in the center
In chronic hyperplastic pulpitis the surface of the Points to Remember

pulp polyp is covered by stratified squamous epithelium.
Stimuli of short duration, mechanical cause, thermal
Such epithelium may be derived from gingiva or freshly
cause, chemical cause, bacterial cause: 545
desquamate epithelial cells of the oral mucosa or tongue.
The tissue in the pulp chamber is often transformed into • Reversible pulpitis: Mild noxious stimuli, pulp
granulation tissue which projects from the pulp into the hyperemia, sharp pain lasting for a moment, brought
carious lesion. on by cold
The granulation tissue is a young vascular tissue • Irreversible pulpitis: Paroxysm of pain, sudden
containing polymorphonuclear neutrophils, lymphocytes temperature changes like cold, pain often continues
and plasma cells. The granulation tissue may become cause has been removed and it may come and pain
is sharp, piercing or shooting, referred pain, later
epithelized as a result of implantation of epithelial
stages, pain is more severe, patient is often awake at
cells on its surface. Granulation tissue is made up of
night due to pain
delicate connective tissue fibers interspersed with a
• Chronic hyperplastic pulpitis: Pulp polyp, pulpitis
variable number of small capillaries. Inflammatory cell
aperta, polypoid tissue appears as a fleshy, reddish
infiltration chiefly lymphocytes and plasma cells are also
pulpal mass filling most of the pulp chamber,
seen.
discomfort during mastication
• Histopathological features: Hyperemia, mild-
Clinical Differences between Reversible and to-moderate inflammatory changes, disruption
Irreversible Pulpitis of the odontoblastic layer, dilated blood vessels,
The pain of irreversible pulpitis is more severe and extravasation of fluid (edema), chronic inflammatory
lasts longer. In reversible pulpitis, the cause of pain is cells predominate, polymorphonuclear leukocytes by
generally traceable to a stimulus such as cold water or chemotaxis, lysosomal enzyme, microabscess, chronic
air whereas in irreversible pulpitis, the pain may come hyperplastic pulpitis, stratified squamous epithelium,
without any apparent stimulus. delicate connective tissue fibers interspersed with a
variable number of small capillaries
• Management: Removal of noxious stimuli, pulpotomy,
Management
pulpectomy, elimination of polypoid tissue.
Prevention is the best management for it. It is done by
periodic care, early insertion of a filling if a cavity has
developed. Removal of noxious stimuli should be done. PULP DEGENERATION
In early stages pulpotomy (removal of the coronal Degeneration is usually present in older aged people. It
pulp) and placing material that favors calcification such as is a result of persistent, mild irritation in teeth of younger
calcium hydroxide over the entrance of root canals. Root people.
canal filling with inert material like gutta percha should be
done. Clinical Features
Complete removal of the pulp or pulpectomy and In early stages, there are no symptoms and no clinical
placement of an intracanal medicament to act as a findings. As degeneration progresses, the tooth may
disinfectant or obtundent such a cresatin, eugenol or become discolored and the pulp within does not respond
formacresol is carried out in irreversible pulpitis. to stimuli.
In case of hyperplastic pulpitis elimination of
polypoid tissue, followed by extirpation of the pulp is Types
done. After removing the hyperplastic tissue bleeding Calcific degeneration: A part of pulp tissue is replaced
can be controlled by pressure. Extraction of tooth can by calcific material that is pulp stones and denticles are
also be done. formed. The calcific material has a laminated structure like
the skin of an onion and lies unattached within the body of Etiology
pulp. In another type of calcification the calcified material
There is no clear-cut etiology. There is strong association
is attached to the wall of the pulp cavity and is an integral
546 between chronic pulpitis and presence of pulpal
part of it which is called diffuse calcification.
calcification. But pulp calcification can be found in
Atrophic degeneration: It is observed in older people. unerupted teeth.
The pulp tissue is less sensitive than normal. Extremely high percentage of pulp stones yield pure
growth of streptococci on culture but often the affected
Fibrous degeneration: It is characterized by replacement
teeth are normal.
of the cellular element by fibrous connective tissue. On
removal from the root canal, the pulp has the characteristic Sundell Schematic Presentation
appearance of a leathery fiber (Fig. 22.5).
Local metabolic dysfunction = trauma = hyalinization of
Pulp artifacts: Vacuolization of the odontoblasts was once injured cells = vascular damage = thrombosis = vessels
thought to be a type of pulp degeneration characterized by wall damage = fibrosis = minerlization (nidus formation)
empty spaces formally occupied by odontoblasts. It is an = growth = pulp stone
artefact caused by poor fixation of the tissue specimen.
Tumor metastasis: It is rare except possibly in the terminal Classification of Pulp Stone
stages. It may occur due to direct local extension from the • Denticle
jaw. • Pulp stones
– True
Points to Remember - Free
No symptoms, no clinical findings, calcific degeneration, - Attached
atrophic degeneration, fibrous degeneration, pulp – False
artifacts, tumor metastasis. - Free
- Attached
• Interstitial
PULP CALCIFICATIONS
Various forms of pulp calcifications are found within the Classification
pulp which may be located in the pulp chamber or in the
There are of following types:
root canals. It can occur in any sex and in any teeth in the
dental arch. Denticle: It occur due to epitheliomesenchymal interaction
within developing pulp. They develop during the formation
and root and form before completion of primary dentin. So
denticle will become attached to or embedded in the dentin.
Pulp stone: Also called pulp nodules. It is of following
types:
∙ True: They are made of localized masses of calcified
tissue that resembles dentin due to their tubular
structure. Tubules are irregular and few in number. It is
more common in pulp chamber than root canals. They
are subdivided into:
– Free: It lies entirely within pulp tissue and is not
attached to the dentinal wall.
– Attached: These are continuous with the dentinal
wall.
∙ False: It is composed of localized mass of calcified
Figure 22.5 Fibrosis of pulp seen as replacement of cellular material and they do not exhibit dentinal tubules.
element by fibrous connective tissue Nodule appears to be made up of concentric layers
or lamellae deposited around a central nidus. It is

composed of cells around which laid down is a layer
of reticular fibers that subsequently calcifies. They are
again classified as free or attached. They are larger 547
than true denticles and they may fill nearly the entire
pulp chamber.
∙ Interstitial: As the concentric deposition of calcified
material continues it approximates and finally is in
apposition with the dentinal wall where it may be
surrounded by secondary dentin then it is called an
interstitial pulp stone.
Diffuse linear calcification: It is also called calcific
degeneration. It is amorphous unorganized linear strands
or columns parallel with blood vessels and nerves of pulp.
It contains fine fibrillar irregular calcification. Figure 22.6 Radiopaque pulp stone seen in the pulp chamber
Clinical and Radiographic Significance

As such they have very little clinical significance. Usually
it is discovered on the radiograph only as radiopacity.
Sometimes, it may cause pain from mild pulpal
neuralgia to severe excruciating pain resembling that of tic
douloureux as the denticle can impinge on the nerve of the
pulp.
Difficulty may be encountered in extirpating the pulp
during root canal therapy.
In some cases pulp calcification may become large
enough to interfere with root formation which may lead to
periodontal destruction and tooth loss.
In some condition like dentin dysplasia (type II),
tumoral calcinosis, calcinosis universalis and Ehlers
Danlos syndrome you may observe calcification.
Figure 22.7 Pulp calcification
Radiographic features: It is radiopaque on the resultant
radiograph. It can be seen hanging in pulp chamber or it is
attached to wall of pulp chamber (Fig. 22.6). Management
Histopathological Features (Figs 22.7 to 22.9) No treatment is necessary as tooth is asymptomatic. Care
should be taken while undergoing root canal therapy for
Denticle consists of tubular dentin which surround central
tooth having pulp stone.
nest of epithelium. After some time it degenerated and
tubules undergo necrosis. Points to Remember
Pulp stone shows central amorphous mass of
Discovered on the radiograph, difficulty during root
calcification which is surrounded by concentric lamellar
canal therapy, periodontal destruction, radiopaque on the
rings. In some cases fibrillar irregular calcified material
resultant radiograph, denticle consists of tubular dentin,
can be seen at the periphery of pulp stone.
pulp stone, central amorphous mass of calcification,
Diffuse calcification consists of fine fibrillar and
diffuse calcification consists of fine fibrillar and irregular
irregular calcification which developed in pulp chamber
calcification.
and canals. This is deposited in linear fashion.
Liquefaction necrosis: It results when proteolytic enzymes

convert the tissue into softened mass, liquid or amorphous
debris.
548
Clinical Features
It causes no painful symptoms.
Sign: Discoloration of the tooth is the first indication
that the pulp is dead (Fig. 22.10). The tooth with partial
necrosis can respond to thermal changes owing to presence
of vital nerve fibers passing through the adjacent inflamed
tissue.
History of severe pain lasting from a few minutes to a
few hours, followed by complete and sudden cessation of
pain.
Figure 22.8 Pulp calcification (Courtesy: Dr Sangamesh
Halawar, Reader, Department of Oral Pathology, VPDC and Histopathological Features
H, Kavalapur, Sangli, Maharashtra, India) Necrotic pulp tissue, cellular debris and microorganisms
may be seen in the pulp cavity.
The periapical tissue may be normal or slight evidence
of inflammation of the apical periodontal ligament may be
seen.
Management
Preparation and obturation of root canals should be carried
out.
Points to Remember
Discoloration of the tooth, history of severe pain,
necrotic pulp tissue, cellular debris, microorganisms,
obturation of root canals.
Figure 22.9 Diffuse calcification presented as linear strands
NECROSIS OF PULP
It is the death of pulp. It may be partial or total depending
on whether a part or the entire pulp is involved.
It is sequelae of inflammation and can also occur
following trauma, in which the pulp is destroyed before an
inflammatory reaction.
Types
Coagulation necrosis: The soluble portion of tissue is
precipitated or is converted into a solid material. Caseation
is a form of coagulation necrosis in which the tissue
is converted into a cheesy mass consisting chiefly of
coagulated proteins, fats, and water. Figure 22.10 Necrotic pulp showing discoloration of tooth
CRACKED TOOTH SYNDROME Bite test: Crack can be confirmed by selective biting
pressure using a cotton roll or a small wooden stick to
The term cracked tooth syndrome (CTS) refers to an allow selective localization of such pressure. Pain increases
incomplete fracture of a vital posterior tooth that involves as the occlusal force increases, and relief occurs once the 549
the dentine and occasionally extends into the pulp. pressure is withdrawn.
Parafunctional habits such as bruxism are also
associated with the development of this condition. Management
It is treated by splinting of the offending cusp with a cusp
Clinical Features
protecting restoration or by removing the split cusp and
Age: The condition presents mainly in patients aged then restoring the tooth.
between 30 and 50 years.
Points to Remember
Location: Mandibular second molars, followed by
mandibular first molars and maxillary premolars. Mandibular second molars, pain ranging from mild to
excruciating, fluid movements are induced by pressure
Symptoms: Patient complains of pain ranging from change, fiberoptic examination, bite test, splinting of the
mild to excruciating, at the initiation or release of the offending cusp.
biting pressure. It can mimic the condition as severe
as trigeminal neuralgia. A crack may involve enamel
and dentine only or it may also involve the pulp and PERIAPICAL ABSCESS
symptoms will vary accordingly (Fig. 22.11). Pain An abscess is a localized collection of pus, surrounded
occurs due to fluid movement within the dentinal tubules by an area of inflamed tissue in which hyperemia and
causing stimulation of sub-odontoblastic nerve fibers. infiltration of leukocytes is marked.
The fluid movements are induced by pressure changes
when biting with the offending cusp. Bacteriology
Sign: If the crack involves dental pulp, direct bacterial Staphylococci are frequently associated with abscess
invasion will occur with predictable pulpal inflammation formation. They produce the enzyme called coagulase
and resultant pulpitic pain. which causes fibrin deposition and thus helps in walling
off the lesion. Coagulase promotes virulence by inhibiting
Fiberoptic examination: Close examination of the crown
phagocytosis.
of the tooth may disclose a crack in enamel, which may be
Small pockets of necrotic tissue are formed within
better visualized by using a dye or by trans-illuminating
cellulites, which coalesce and enlarge, compressing the
the tooth with fiberoptic light.
surrounding fibrous connective tissue. Thus an abscess
is generated, which is a collection of pus surrounded by a
wall of fibrous connective tissue.
Types
∙ Acute periapical abscess: It is also called acute
alveolar abscess.
∙ Chronic alveolar abscess: It is a long standing, low
grade infection of the periradicular tissues.
But as this acute and chronic process both have acute
inflammatory reaction so this term may be wrong to used.
Instead it should be used as symptomatic and asymptomatic.
Etiopathogenesis
It may be result of trauma or chemical or mechanical
Figure 22.11 Cracked tooth syndrome irritation. The immediate cause is the bacterial invasion of
dead pulp. When inflammatory response may extend into The tissues at the surface of swelling appear taut and
adjacent periapical alveolar bone, it will initiate necrosis inflamed. The surface of tissue become distended from
of periapical tissue and diffuse rarefaction of bone, leading the pressure of underlying pus and finally ruptures due
550 to formation of periapical abscess with symptoms of acute to pressure and lack of resistance caused by continued
inflammation. liquefaction.
Primary or necrotic abscess are pulpo-periapical When the maxillary anterior teeth are involved,
inflammatory conditions associated with teeth, which swelling of upper lip may extend to one or both eyelids.
have not developed apparent periapical radiolucent lesion; When the maxillary teeth are involved, the cheek may
usually described as acute apical periodontitis or acute swell to an immense size, distorting the patient’s face. It
periapical abscess. may results in buccal space infection (Fig. 22.12).
The surrounding tissue attempt to localize the pyogenic When the maxillary posterior teeth are involved, there
infection by forming enclosure of granulation tissue; this in is possibility of maxillary sinus to involvement. When the
turn is surrounded by fibrous connective tissue; this results mandibular posterior teeth are involved, swelling of the
in well circumscribed lesion containing necrotic tissue. cheek may extend to ear.
Well circumscribed lesion may form sinus due to Sudden decrease in pain signals the formation of sinus.
inability of the body to completely contain or localized Tooth is tender, vitality test is negative. Draining fistulas
the causative organisms, increase in number of causative are also commonly associated with chronic alveolar
organisms, lowering of patient’s general resistance and abscess. Majority opens on labial and buccal aspect of
trauma or surgical intervention. alveolus, as apices of both maxillary and mandibular teeth
Enlarging dentoalveolar abscess contains purulent are located nearer to the buccal than the lingual cortical
material that is under pressure due to the production of pus— plate. In maxilla, roots of lateral incisors and molars are
the purulent material travels along path of least resistance, close to palatal cortical plate, so sinus appear there (Fig.
until it reaches the surface, where due to limitation of 22.13). Most root tips lie below the mylohyoid muscle, so
periosteal layer, it temporally forms subperiosteal abscess. pus drains into the submandibular space.
Eventually, it erodes through the periosteum and Sinus opening appears as a small ulcer. At the opening
penetrates the soft tissue, again, following the path of least of sinus mass of inflamed granulation tissue is found, it is
resistance. Path of least resistance is determined by the called parulis or gum boil.
location of breakthrough in the bone and the anatomy of Occasionally, after temporary emphysema, sinus heals
muscles and fascia plane in the area. and form slightly raised pale papule. As the pus accumulates,
another signs formation may take place eventually.
Symptoms: Pain is severe and of throbbing type. Periapical
abscess may confine to osseous structures and during the
early period of abscess formation, may cause excruciating
pain without observable swelling. The patient may appear
pale, irritable and weak from pain, loss of sleep as well
as from absorption of septic products. He may have slight
fever (99 to 100°F).
Sign: Patients experience sensitivity or pressure in the
affected area. Ice relives the pain and heat intensifies it
aspiration yield yellowish pus. The tooth becomes more
painful, appears elongated and mobile. In acute periapical
infection, tooth is sensitive to percussion and movement.
There is also painful lymphadenopathy. After some period
the affected pulp is necrotic and does not respond to electric
current or to application of cold. Swelling is usually seen in Figure 22.12 Periapical infection from tooth which involve
adjacent tissues adjacent to the affected tooth. buccal space
Fibroblast may start to form capsule at the periphery.

Sinus tract are generally lined by granulation tissue. In
addition, chronic inflammatory cells are also present.
551
Management
Establish drainage immediately, if possible: It may be
done by opening the pulp chamber and passing file through
the canal into the periapical region. Trephination opening
through mucosa and bore to the abscess at apex. Through
and through drain is placed in the abscess and irrigated
with 1:1 mix of 3 percent H2O2 and normal saline solution.
Antibiotics like Penicillin 500 mg, QID, for 5 days and
analgesics should be given. In 24 to 48 hours, it can be
determined if the tooth can be treated endodontically or
Figure 22.13 Intraoral swelling seen on palatal side in patient extraction is necessary.
Warm saline mouth rinse often aid in localizing the
infection and maintaining adequate drainage, before
endodontic treatment or extraction.
If there is need of retention of offending tooth, necrotic
pulp should be opened and tooth should be treated
endodontically.
Points to Remember
Staphylococci, acute periapical abscess, chronic
alveolar abscess, bacterial invasion of dead pulp, pain
is severe throbbing type, pale, irritable and weak from
pain, painful lymphadenopathy, tooth becomes more
painful, appears elongated, tissues at the surface of
swelling appear taut, sudden decrease in pain signals the
formation of sinus, draining fistulas, parulis or gum boil,
there is loss of lamina dura with destruction of bone,
Figure 22.14 Periapical loss of bone due to periapical polymorphonuclear leukocytes, inflamed connective
abscess canal appears to be devoid of tissue macrophages
and granulation tissue, fibroblast, establish drainage
Radiological features: There is loss of lamina dura with immediately, antibiotics, warm saline mouth rinse.
destruction of bone in periapical area (Fig. 22.14)
Histopathological Features PERIODONTAL ABSCESS

Central region of necrosis contain dense accumulation of It is usually culmination of a long period of chronic
polymorphonuclear leukocytes, surrounded by inflamed periodontitis.
connective tissue wall of varying thickness.
Acute abscess contain necrotic and unidentical soft Causes
tissues. There is an empty space, where suppuration has Many cases of periodontitis arise in the patients who are
occurred. The root canal appears to be devoid of tissue and going periodontal therapy due to incomplete removal
instead, microorganism and debris may be observed. deep of calculus. There is also microbial penetration of
In case of chronic condition macrophages and surrounding tissue.
granulation tissue are present. Lymphocytes and plasma Other factors which can cause periodontal abscess are
cells are found at the periphery of the abscessed area. diabetes, trauma, enamel pearls.
Pathogenesis tissue, as the epithelial lining of the crevice has been

destroyed by the inflammatory process.
It usually occurs in pre-existing periodontal pocket. When
There is also large colonies of microorganism can also
552 such pocket reaches sufficient depth of about 5 to 8 mm, the
be present.
soft tissues, around the neck of the tooth may approximate
the tooth so tightly that orifice of the pocket is occluded. Management
Bacteria multiply in the depth of pocket and cause sufficient
irritation to form an acute abscess, with exudation of pus Incision and drainage: Primary treatment for relief of
into this area. It results in sufficient swelling to destroy the acute symptoms is incision of the fluctuant abscess, from
cortical plates of bone. the depth of the abscess cavity to the gingiva. The incision
should extend into the soft tissues of the root surface. If the
Clinical and Radiological Features surrounding tissue is normal, the tooth may be retained and
debridement of the root surface by removal of granulation
It starts at the gingival crevice and extends down on one or
tissue should be done.
more surface of the root, frequently as far as apical region.
Treatment for new attachment and new tissue
Symptoms: Acute episode usually has sudden onset with regeneration should be performed. However, if the roots
extreme pain. There is also distension and discomfort. are denuded beyond the apical thirds of the root, the tooth
Signs: They are associated with swelling of the soft tissues should be extracted and curettage should be carried out to
overlying the surface of the involved root (Fig. 22.15). remove the granulation tissue from the socket.
Tooth is tender and mobile. Pus usually exudes from the Antibiotics: Antibiotics of choice is penicillin.
gingival crevice.
Other features includes lymphadenopathy, fever,
Points to Remember
leukocytosis, foul taste.
Incomplete removal deep of calculus, sudden onset with
Radiographic features: There is bone loss associated extreme pain, soft tissues overlying the surface of the
with affected teeth. In some cases infection can spread to involved root, lymphadenopathy, fever, leukocytosis,
periapical region causing periodontal-endodontic lesion. foul taste, bone loss associated with affected teeth,
central cavity filled with pus walled off on one side by
Histopathological Features the root, large colonies of microorganism, incision and
It consist of a central cavity filled with pus walled off on drainage, new attachment and new tissue regeneration,
one side by the root and on the other side by connective antibiotics.
ACUTE EXACERBATION OF A
CHRONIC LESION
It is an acute inflammatory reaction superimposed on an
existing chronic lesion, such as on cyst or granuloma. It is
also called phoenix abscess due to mythical bird that would
die only arise again from its own ashes.
Causes
The peri-radicular area may react to noxious stimuli form a
diseased pulp with chronic peri-radicular disease. At times,
because of an influx of necrotic product from a diseased
pulp or because of bacteria and there toxins, this apparently
Figure 22.15 Swelling in soft tissue due to periodontal dormant lesion may react and cause an acute inflammatory
abscess response.

Symptoms: At the onset, tooth may be tender to touch. Phoenix abscess, tooth may be tender to touch, mucosa
Patients complain of intense pain, local swelling and over the radicular area may be sensitive to palpation, 553
possibly associated cellulitis. lack of response to vitality test, radiolucency seen at the
Signs: Mucosa over the radicular area may be sensitive apex of tooth, area of liquefaction necrosis, disintegrating
to palpation and may appear red and swollen. The patient polymorphonuclear neutrophils, drainage, antibiotics and
has history of traumatic accident that turned the tooth dark analgesic.
after a period of time or of postoperative pain in a tooth that
had subsided until the present episode of pain. PERIAPICAL GRANULOMA
Lack of response to vitality test points to diagnosis
necrotic pulp. It is the most common type of pathologic radiolucency
encountered in dentistry. It is a growth of granulation
Radiological features: There is radiolucency seen at the tissue continuous with the periodontal ligament resulting
apex of tooth (Fig. 22.16). from the death of the pulp and diffusion of bacteria and
bacterial toxins from the root canals into the surrounding
periradicular tissues through the apical and lateral foramina.
Area of liquefaction necrosis with disintegrating polymor-
phonuclear neutrophils and cellular debris is seen. Etiopathogenesis
These are surrounded by infiltration of macrophages It occurs as a response to intense and prolonged irritation
and some lymphocytes. These abscesses can maintain soft from infected root canals producing extension of chronic
tissue component. apical periodontitis beyond the periodontal ligament. The
expanding inflammation and increased vascular pressure
Management
result in abscess formation and resorption of the bone in
Drainage, either via the root canal or by incision, if there the affected area, which in cause of time is replaced by
is localized swelling. granulation tissue. It is the result of a successful attempt by
Antibiotics and analgesic: Appropriated antibiotics the periapical tissues to neutralize and confine the irritating
and analgesic should be given. toxic product that is escaping from the root canal.
But continuous discharge into the periapical tissues
induces a vascular inflammatory response. Insult from
diseased pulp represents broad spectrum of inflammatory
mediations like prostaglandins, kinin and endotoxins.
Elevated level of IgG in pulpoperiapical lesion.

The tooth is nonvital, i.e. it does not respond to thermal and
electric pulp test.
Symptoms: Mild pain can be occasionally experienced
while biting or chewing on solid foods. Sensitivity occurs
due to hyperemia, edema and inflammation of the apical
periodontal ligament.
Sign: Tooth may be darker in color, because of the blood
pigments that diffuse into the dentinal tubules. There is,
Figure 22.16 Phoenix abscess seen as radiolucency in seldom, swelling or expansion of the overlying cortical
relation to first molar bone. Tooth may feel to be slightly elongated in the socket.
Radiological features: The lesion is radiolucent well occasionally, nests of odontogenic epithelium, Russell’s
circumscribed less than 1.5 cm in diameter. Margin are bodies (scattered eosinophilic globules of gamma globulin),
well defined usually but in some cases it can be ill defined pyronine bodies (cluster of lightly basophilic particles),
554 (Fig. 22.17). foam cells and cholesterol clefts. New capillaries are lined
by swollen endothelial cells.
Types of Granuloma According to Histopathology There is more inflammation in the center with fibrosis at
• Exudative the periphery. Connective tissue is more prominent on the
• Granulomatous periphery and the bundles of collagen become condensed
• Granulofibrosis there, apparently as a result of the slow expansion of the
• Fibrous. soft tissue mass, resulting in formation of a continuous
capsule separating the granulation tissue from the bone.
Histopathological Features (Fig. 22.18) Epithelial rest of Malassez may be seen with granulation
tissue. Lymphocytes, plasma cells, macrophages and
It consists of proliferating endothelial cells capillaries,
foreign body multinucleated giant cells may also be
young fibroblasts minimum amount of collagen and
present. Occasionally, cholesterol clefts may form the
major portion; then it is called as cholesterol granuloma of
the jaw.
Management
Extraction of the involved tooth should be done.
Under certain conditions, root canal therapy, with or
without subsequent apicectomy are the treatment options.
NSAID should be given in periapical granuloma for
combating inflammation.
Reason for Failure of Treatment

• Transforming into cyst
• Persistent pulpal infection
• Extraradicular infection
• Accumulation of endogenous debris
Figure 22.17 Periapical granuloma showing well defined
• Periapical foreign material
radiolucency less than 1.5 cm in diameter
• Fibrous scar formation
• Maxillary sinus involvement
• Periodontal disease.
Points to Remember
Growth of granulation tissue, nonvital tooth, mild
pain, tooth may be darker in color, radiolucent well
circumscribed less than 1.5 cm in diameter, proliferating
endothelial cells capillaries, Russell’s bodies, pyronine
bodies, foam cells, cholesterol clefts, more inflammation
in the center, epithelial rest of Malassez, multinucleated
giant cells, cholesterol granuloma.
Periapical Scar
It is a possible end point of healing. It is composed of
Figure 22.18 Periapical granuloma dense fibrous tissue and is situated at the periapex of pulp
less tooth, in which usually, the roots canal have been

Points to Remember
successfully filled.
After endodontic treatment, tooth is nonvital, periapical
Formation of Scar radiolucency, spindle shaped fibroblast, hyalinization. 555
Irritant substance confined in the periapical area. It leads
to accumulation of chronic inflammatory cells. Young OSTEOMYELITIS
fibroblasts, endothelial cells and capillaries proliferate, which
It is the inflammation of the bone marrow that produces
lead to granuloma formation. After endodontic treatment,
clinically apparent pus and secondarily affects the calcified
the granuloma resolves, but in some cases, granulation tissue
components. It is infection of the bone that involves all the
gets slowly organized with the production of more and more
three components viz. periosteum, cortex and marrow.
collagen fibers, which in turn leads to scar formation.
It may be defined as an inflammatory condition of the
Clinical Features bone that begins as an infection of medullary cavity and the
haversian system and extends to involve the periosteum of
It occurs usually after endodontic treatment and in patients
the affected area.
treated by periapical curettage or root resection.
It is more common in anterior region of maxilla. Tooth Predisposing Factors
is nonvital and the patient is asymptomatic.
Certain predisposing factors play an important role in the
Radiologically periapical radiolucency is seen (Fig.
onset and severity of the osteomyelitis, in addition to the
22.19).
virulence of microorganism.
Conditions affecting host resistance: Diabetes mellitus,
It will show spindle shaped fibroblast scattered throughout tuberculosis, severe anemia, leukemia, agranulocytosis,
the dense collagen bundles, which show advanced degree acute illness such as influenza, scarlet fever, typhoid and
of hyalinization. exanthematous fever, sickle cell anemia, malnutrition and
chronic alcoholism.
Management
Conditions affecting jaw vascularity: Metastasis from
No treatment is necessary for this periapical scar.
remote area of infection such as another bony site, skin
and kidneys, radiation, osteoporosis, osteopetrosis, fibrous
dysplasia, bone malignancy and peripheral vascular
disease.
Etiology of Osteomyelitis
• Odontogenic infections
• Compound fractures of the jaws
• Traumatic injury
• Middle ear infection and respiratory infection
• Furunculosis of chin
• Peritonsillar abscess.
Etiology
Odontogenic infections which can be periapical or
periodontal infection, pericoronal infection and infection
from infected dental cyst.
Figure 22.19 Periapical scar seen as radiolucency at the apex
of central incisor (Courtesy: Dr RN Modi, Professor and Head, Compound fractures of the jaws: Generally, these
Department of Oral Medicine and Radiology, Hitkarini Dental fractures are compound through the tooth socket into the
College, Jabalpur, Madhya Pradesh, India) mouth and rarely, to the skin.
Traumatic injury: Local traumatic injury of the gingiva rise to increased intra-medullary pressure, which results
leads to periostitis, in patients with low resistance to in compression of vasculature, vascular collapse, venous
infection and later to osteomyelitis. stasis and ischemia.
556
Middle ear infection and respiratory infection: Via Elevation of periosteum: Pus travels through the
hematogenous route, either from middle ear infection or Haversian and Volkmann’s canals and accumulates beneath
from infection of the upper respiratory tract. the periosteum, elevating it from the cortex, thereby further
reducing the blood supply.
Furunculosis of chin: Furunculosis of chin, i.e. spread
through lymphatic channel via infected lymph nodes. Necrosis of bone: The reduced blood supply leads to slow
necrosis of the bone.
Peritonsillar abscess: Peritonsillar abscess has also
been reported to cause osteomyelitis of the ramus of Penetration of periosteum: If the pus continues to
mandible. accumulate the periosteum is penetrated and mucosal and
cutaneous fistulae develop and thereby discharging the
Pathogenesis of Osteomyelitis purulent pus.
Compromised blood supply = inflammatory reaction = After therapy: As the therapy begins to be effective and
disorganization of clot = mechanical trauma = the host resistance increases, the process become chronic.
accumulation of pus = elevation of periosteum = necrosis Inflammation regresses, granulation tissue forms and
of bone = penetration of periosteum = involucrum = new blood vessels are formed which cause lysis of bone;
sequestra = cloacae = systemic spread of infection = thus causing fragments of necrotic bone from the viable
necrosis of bone. bone.
Involucrum: Small sections of necrotic bone may be
Pathogenesis
completely lysed, while large one get localized and get
Compromised blood supply is a critical factor in the separated from the shell of the new bone by a bed of
establishment of osteomyelitis. The virulent micro- granulation tissue. This dead bone surrounded by viable
organisms get entry in the medullary cavity via many bone is called involucrum.
routes like odontogenic infections, compound fractures,
periostitis, hematogenous route and lymphatic channel. Sequestra: Small pieces of necrotic bone are called
as sequestra, which are avascular and which harbor
Inflammatory reaction: These microorganisms cause microorganisms. These sequestra need to be removed,
intense inflammatory reaction within the marrow of the otherwise they continue to be chronically infected and
bone. Pain is a feature of this stage. infect the surrounding granulation tissue and cause further
sequestration, which weakens the bone and may cause
Localization of infection: Most of the odontogenic
pathologic fracture.
infections, like periapical and periodontal infections, are
localized by pyogenic membrane or soft tissue abscess Cloacae: An involucrum contains one or more holes on
walls. the surface which lead into channels, which can be traced
to end in the depth of the bone at the site of an area of
Disorganization of clot: However, disorganization of this
bone destruction around the sequestrum. These orifices are
pyogenic membrane occurs by virulent microorganism or
termed ‘cloacae’. Pus finds its way from the depth of the
by chronic movement of the unreduced fractures of jaws.
bone to the surface, through the cloacae. The presence of
Mechanical trauma: Mechanical trauma, due to chronic cloacae indicates that there is a dead bone or a foreign body
movement of unreduced fractures, burnishes the bone and at the deep end.
causes ischemia, thereby introducing the microorganisms
Systemic spread of infection: Besides the pathogenic
deep into the underlying tissues.
activity of the virulent microorganisms, these micro-
Accumulation of pus: When this protective barrier organisms precipitate thrombi formation by virtue of
breaks, the pus accumulated in the medullary cavity gives their destructive lysosomal packages. The coagulum
provides the host medium for further pathogenic Classification

proliferation as well as an isolating barrier from the
According to anatomic location of infectious process:
host immune response. It also allows systemic spread
• Intra-medullary 557
of infection.
• Sub-periosteal
Necrosis of bone: The necrosis of bone is brought about by • Periosteal
thrombosis of the vessels or compression of the vasculature. According to duration and severity:
The necrosis of bone, with superadded infection forms a • Acute
base line pathogenesis of the osteomyelitis. Compression • Chronic
of neurovascular bundles can result in osteomyelitis Depending upon the presence or absence of suppuration:
mediated anesthesia. Suppurative
• Acute suppurative osteomyelitis
Occurrence • Chronic suppurative osteomyelitis
– Primary
It is more common in men, than women. It occurs in – Secondary
mandible in premolar area because cortical plate of Infantile osteomyelitis
the bone in mandible is dense it takes longer for sinus Nonsuppurative
formation and release and hence, the infection gets directs • Chronic nonsuppurative
into spongiosa and spreads. Other reason for occurrence – Focal sclerosing
in mandible are removal of posterior mandibular teeth – Diffuse sclerosing
attended by more damage to the bone, mandible is less • Radiation osteomyelitis
vascular than maxilla and thin cortical plates and relative • Garre’s sclerosing osteomyelitis
paucity of medullary tissue in the maxilla precludes • Osteomyelitis due to specific infection:
confinement of infection within the bone and permits – Actinomycosis
dissipation of edema and pus into the soft tissues and – Tuberculosis
paranasal sinuses. – Syphilis.
Infantile osteomyelitis is more common in maxilla than
mandible, as it spreads by hematogenous route and maxilla Clinical Staging of Osteomyelitis
has more blood supply than mandible. • Initial stage: Spontaneous pain (localized).
• Acute stage (Suppurative stage): In this stage, there
Microbiology of Osteomyelitis is severe pain, soreness and looseness of the involved
Staphylococcus aureus and Staphylococcus albus, teeth.
hemolytic streptococci, gram negative organisms – Early acute stage: In reference to the involved
like Klebsiella, Pseudomonas, Proteus, E. coli. tooth, progressive sensitivity of the adjacent teeth
Anaerobic microorganisms like pepto-streptococci, to percussion and pain of the involved side
Bacteroides and fusobacteria. Some specific forms like – Late acute stage: Paresthesia or anesthesia of the
Mycobacterium tuberculosis, Treponema pallidum and lip region supplied by the mental nerve. Other
A. israelii. systemic symptoms can occur.
• Osteonecrotic stage: Diminished spontaneous pain,
Following parameters should be used for recognition of abscess formation and pus discharge.
• Sequestrum stage: Lack of symptoms sequestrum
pure anaerobic or mixed anaerobic infection:
formation visible on the radiograph.
∙ Presence of foul smelling exudate.
∙ Sloughing of necrotic tissues or gas in the necrotic
tissue.
ACUTE SUPPURATIVE
∙ Gram stain revealing multiple organisms of different OSTEOMYELITIS
morphological character. It is serious sequelae of periapical infection there is often
∙ Presence of sequestra. a diffuse spread of infection throughout the medullary
spaces, with subsequent necrosis of variable amount of

bone. There is insufficient time for body to react to the
presence of inflammatory infiltrate. This process is of less
558 than one month of duration.
Clinical Features
In early acute suppurative osteomyelitis: It has rapid
onset and course, severe pain, paresthesia or anesthesia of
the mental nerve. At this stage the process is truly intra-
medullary, therefore swelling is absent, teeth are not
mobile and fistulae are not present.
In established suppurative osteomyelitis: In this type
there is deep intense pain, anorexia, fetid oral odor malaise
and fever, regional lymphadenopathy. There is also soreness
of involved teeth which become loose within 10 to 14 days. Figure 22.20 Necrotic bone seen in case of osteomyelitis
Pus exudes around the gingival sulcus or through
mucosal and cutaneous fistula. There is firm cellulitis
of cheek and abscess formation with localized warmth
and tenderness on palpation. The patient feels toxic and
dehydrated.
Radiological features: The radiograph shows ill defined
radiolucency. In some cases periosteal new bone formation
can be seen. Sequestration can be seen as radiopaque
structure in the radiolucency.
It usually consists of necrotic bone. The medullary spaces
are filled with inflammatory exudate that may or may not
progress to the actual formation of pus (Figs 22.20 and Figure 22.21 Acute suppurative osteomyelitis showing
22.21). inflammatory infiltrate (Courtesy: Dr Sangamesh Halawar,
The inflammatory cells are chiefly neutrophilic Reader, Department of Oral Pathology, VPDC and H,
polymorphonuclear leukocytes, but may show occasional Kavalapur, Sangli, Maharashtra, India)
lymphocytes and plasma cells.
The osteoblastic rimming of the bony trabeculae is antibiotic cover. After pus is evacuated, drains are placed.
generally destroyed. Depending upon the duration or the The consistency, color and odor of the pus may provide
process, the trabeculae may loose their viability and begin important clues to the diagnosis and initial treatment.
to undergo slow resorption.
The periphery of the bone and haversian canals Points to Remember
contains necrotic debris with acute inflammatory infiltrate. Rapid onset and course, paresthesia or anesthesia of
the mental nerve, deep intense pain, anorexia, fetid oral
Management
odor, pus exudes around the gingival sulcus, cellulitis
Antibiotics therapy: If abscess formation is seen of cheek, ill defined radiolucency, necrotic bone,
antibiotics medication like aqueous penicillin, clindamycin, inflammatory exudate, neutrophilic polymorphonuclear
cephalexin, cefotaxime, tobramycin and gentamicin. leukocytes, lymphocytes, plasma cells, trabeculae may
Incision and drainage: When early diagnosis is made, loose their viability, haversian canals contains necrotic
drainage of the fluctuant areas should be carried out under debris, antibiotics therapy, incision and drainage.
CHRONIC SUPPURATIVE
OSTEOMYELITIS
Chronic osteomyelitis develops without initial acute stage, 559
if the virulence is of low grade.
Chronic osteomyelitis is persistent abscess of the
bone that is characterized by usual complex inflammatory
process including necrosis of mineralized and marrow
tissues, suppuration, resorption sclerosis and hyperplasia.
It occurs as defensive response lead to production of
granulation tissue which forms dense scar tissue to wall of
infection.
Clinical Features
Figure 22.23 Extraoral discharging sinus in osteomyelitis
Virulence of the microorganism is low and the host
resistance is high. This type is not preceded by an episode
of acute symptoms.
Symptoms: It has insidious onset with slight pain, slow
increase in jaw size and a gradual development of sequestra
without fistula.
Signs: Local tenderness and swelling develop over the
bone in the area of abscess.
Intraorally and extraorally sinus develops intermittently
and drains small amount of pus and then gradually heals.
Sinus extends from medullary bone, through cortical plate,
to mucus membrane or skin. Sinus may be at a considerable
distance from the offending infection. It is painless unless
there is an acute or sub-acute exacerbation (Figs 22.22 and
22.23).
Involvement is single feeder vessels may lead to Figure 22.24 Sequestra seen as radiopaque structure in
necrosis of entire quadrant of jaw in long standing chronic osteomyelitis
osteomyelitis.
Radiographic features: It shows patchy, ragged and ill-
defined radiolucency which contain central radiopaque
sequestra. Surrounding bone show increase radiodensity
(Fig. 22.24).
There is significant soft tissue component which consist
of inflamed fibrous connective tissue in areas of inter-
trabecular bone.
Scattered sequestra and pockets of abscess formation
are common (Figs 22.25 and 22.26).
Management
It is difficult to manage by drugs as dead bone and organism
Figure 22.22 Exposed bone seen clinically as sequestra are surrounded by wall of fibrous connective tissue.
Supportive therapy: Patients is suffering from

osteomyelitis required adequate rehydration in the form of
fluids. Rich nutritional diet should be given. Multivitamin
560 supplements should be given.
Sequestrectomy: It is the removal of sequestra which are
small pieces of necrotic bone that are avascular and harbor
microorganisms.
Saucerization: It means excision of the margins of necrotic
bone, overlying the focus of osteomyelitis which allows
better visualization of sequestra and excision of margins of
the affected bone.
Decortication: Decortication of mandible refers to removal
of the chronically infected and inferior cortical plates, 1
Figure 22.25 Suppurative osteomyelitis showing to 2 cm beyond the area of involvement. Thus, access is
inflammatory cells provided to the medullary cavity.
Hyperbaric oxygen therapy: In this patient is placed in
multiplace/monoplace chamber and a concentration of 100
percent O2 is given. Duration of treatment is of 1.5 to 2
hours for 5 to 6 days in a week, for a total of 60 treatments.
Points to Remember
Slight pain, slow increase in jaw size, local tenderness,
intraorally and extraorally sinus, shows patchy, ragged
and ill defined radiolucency, soft tissue component
which consist of inflamed fibrous connective, scattered
sequestra, pockets of abscess formation, antibiotics,
irrigation and debridement of the necrotic areas,
extraction of offending tooth, supportive therapy, seq-
uestrectomy, saucerization, decortications, hyperbaric
oxygen therapy.
Figure 22.26 Chronic suppurative osteomyelitis seen as
scattered sequestra and pockets of abscess formation INFANTILE OSTEOMYELITIS
It is a rare type of osteomyelitis seen in infants few weeks
Antibiotics: These are similar to used in case acute after the birth. It usually involves the maxilla.
osteomyelitis.
Irrigation and debridement of the necrotic areas— Route of Infection
thorough debridement of the affected areas should be Hematogenous route: Infantile osteomyelitis is usually
carried out. Debride any foreign bodies, necrotic tissue transferred through the hematogenous route.
or sequestra. These areas may be irrigated with hydrogen
Trauma: Prenatal trauma of oral mucosa from
peroxide and saline.
obstetrician’s finger.
Extraction of offending tooth: Extraction of carious teeth
Infection: Infection from mucous bulb used to clear the
with periapical infection, should be done. It should be
airway immediately after birth.
carried out to remove the source of infection from the oral
cavity. Infected nipple: Infected human or artificial nipple.
Clinical Features Radiographic features: Increase radiodensity seen around

sites of chronic infection. This area can be multifocal
Location: It is more common in maxilla due to
involving the entire quadrant.
hematogenous route. The infection is thought to arise in 561
maxillary antrum or lacrimal sac. It appears to center in the Histopathological Features
region of first deciduous molars and the adjacent portion
of maxilla, although it may involve the inferior aspect of It shows dense irregular trabeculae of bone, some of which are
bordered by an active layer of osteoblasts. The bone, in some
the orbit.
lesions, shows a pronounced ‘mosaic’ pattern, indicative of
Symptoms: There is fever, anorexia and dehydration. In repeated periods of resorption followed by repair.
some cases, convulsions and vomiting may occur. The haversian canals are scattered with little marrow
spaces. The soft tissue between the individual trabeculae is
Signs: There is redness and edema of eyelids, alveolar
fibrous and show proliferative fibroblasts and occasional
bone and palate of the affected side. Intracanthal swelling,
small capillaries focal collections of lymphocytes and
palpebral edema, conjunctivitis and proptosis may result.
plasma cells.
Maxilla on affected side is swollen both buccally and
Polymorphonuclear leukocytes may be present, if the
palatally, especially in the molar region. Sinus develops
lesion is undergoing an acute phase.
and discharges pus intraorally and extraorally.
Necrotic bone surrounded by inflamed granulation
Complications may occur like TMJ infection and
tissue.
devitalization of adjacent tooth germs may occur.
Management
Points to Remember
Resolution of infection—this will help in remodeling in
Hematogenous route, common in maxilla, fever, anorexia some patients.
and dehydration, redness and edema of eyelids, alveolar If secondary osteomyelitic occur in patients with long
bone, intracanthal swelling, complications may occur term alveolar resorption, the patient should be treated in
like TMJ infection. same way as that of acute and chronic osteomyelitis.
Diffuse Sclerosing Osteomyelitis Points to Remember

Reactive proliferation of bone is the primary response in Reactive proliferation of bone, mandibular jaw,
diffuse sclerosing osteomyelitis due to a balance between complains of pain and tenderness, jaws may be slightly
the virulence of infection and resistance of host. It is enlarged, increase radiodensity, mosaic pattern, haversian
analogous to focal form of the disease. It occurs due to low canals are scattered, proliferative fibroblasts, capillaries
grade infection. focal collections of lymphocytes, polymorphonuclear
This term should be used when an obvious infectious leukocytes, resolution of infection.
process directly responsible for sclerosis of bone. In this
chronic bacterial infection of intraosseous nature creates Primary Chronic Osteomyelitis
mass of chronically granulation tissue which incites It gets often confused with chronic suppurative osteo-
sclerosis of bone. myelitis. In this case association with bacterial infection is
not so obvious and suppuration and sequestration is absent.
This condition does not respond long term antibiotics
Age and sex distribution: It can occur at any age but most therapy.
common in older persons. It has got no sex predilection.
Location: It is common especially in mandibular jaw in an
edentulous area. Location: It is isolated process seen in the mandible.
Symptoms are very mild to absent. During the period of Age: It is seen in two peaks one seen in adolescence and
growth the patient complains of pain and tenderness. Pain other in adults after 5th decade.
persists for few weeks to months to even years.
Symptoms: Recurrent pain, swelling, local induration and
Signs: Jaws may be slightly enlarged on the affected side. limited mouth opening is present.
Sign: Regional lymphadenopathy and reduce sensation in muscle mainly masseter and digastric. Many people believe
the distribution of inferior alveolar nerve can be seen. that this is variation of primary chronic osteomyelitis in
Fascial asymmetry: It is seen and takes year to resolve which parafunctional habits exacerbate.
562 Microbiological culture is negative in chronic
secondary to slow remodeling.
tendoperiostitis.
Radiographic features: It demonstrated areas of
radiolucent osteolysis mixed with zone of sclerosis. Clinical and Radiological Features
Osteolytic area are not continuous and alternate with zones
Age: It is more commonly seen in 3rd and 4th decade of
of sclerosis. Bone is thickened with periosteal reaction
life.
which is more solid as compared to laminated proliferate
periostitis of inflammatory origin. Symptoms: Recurrent pain, swelling of cheek and trismus
are present.
Radiological features: Sclerosis is usually found on the
In the areas of sclerosis irregular trabecular of pagetoid
anterior region of mandibular angle and posterior portion
bone are present with prominent reversal line, osteoblastic
of mandibular body. These are the area where attachment
rimming and focal areas of osteoclastic activity.
of masseter and digastric muscle occur. In the area of
Intra-trabecular fibrosis with scattered lymphocytes
sclerosis radiolucent zone appear. There is erosion of
and plasma cells is present.
inferior border of mandible occur.
Other features include microabscess formation,
hyalinization around small blood vessels and subperiosteal Histopathological Features
bone formation also occurs.
There is sclerosis and remodeling of the cortical and
Management subcortical bone with increase in bone volume.
Elimination of infection should be carried out. Management
Surgical decortication: It should be done and it will help Treatment should be done to resolution of muscle overuse.
decrease the intensity and frequency of symptoms. It should be done by muscle relaxation, rotation exercise,
IV bisphosphonates: IV bisphosphonates like alendronate, occlusal splint therapy, myofeedback and muscle relaxant
disodium clodronate and pamidronate can results in drug like diazepam and mefenoxalon should be used.
complete disappearance of pain and dramatic suppression
bone turnover. Points to Remember
Other drugs like corticosteroids, NSAIDs and calcitonin Reactive alternation in bone, overuse of masticatory
can also help in relieving the symptoms. muscle, recurrent pain, swelling of cheek and trismus,
sclerosis, sclerosis and remodeling of the cortical and
Points to Remember subcortical bone with increase in bone volume, muscle
Recurrent pain, swelling local induration, regional relaxation, rotation exercise, occlusal splint therapy,
lymphadenopathy, Fascial asymmetry, radiolucent myofeedback.
osteolysis mixed with zone of sclerosis, trabecular of
pagetoid bone, intra-trabecular fibrosis, microabscess
formation, hyalinization around small blood vessels, SYNOVITIS, ACNE, PUSTULOSIS,
subperiosteal bone formation, surgical decortications, HYPEROSTOSIS AND OSTEOMYELITIS
IV bisphosphonates. SYNDROME
It consists of synovitis, acne, pustulosis, hyperostosis and
Chronic Tendoperiostitis osteomyelitis.
Clinical presentation of chronic tendoperiostitis is similar Cause of SAPHO syndrome is not exactly known, but it
that of primary chronic osteomyelitis. is thought to be genetic in origin. There is abnormal immune
It occur due to reactive alternation in bone occur due response to microorganism cross reacts with normal bone
to parafunctional habits. There is overuse of masticatory or joint leading to variety of clinical manifestation.
This syndrome occurs usually younger than 60 years of

age. Osteolytic area are scattered randomly within areas of
sclerotic bone.
Bones involved are anterior chest wall, clavicles, ribs, 563
spine, pelvis, and long bone.
Periosteal new bone formation is also present which
leads to enlargement of affected bone. After some days
bone become more sclerotic with less periosteal apposition
resulting in decrease in size of the bone. External bone
resorption and deformity of the mandible are characteristic
of these syndrome.
Points to Remember
Synovitis, acne, pustulosis, hyperostosis, osteomyelitis,
Figure 22.27 Condensing osteitis seen as increase
osteolytic area are scattered randomly, periosteal new
radiodensity
bone formation, external bone resorption and deformity
of the mandible.
Symptoms: Tooth is usually asymptomatic. But in some
cases, patient may report pain or tenderness on percussion
CHRONIC RECURRENT MULTIFOCAL or palpation.
OSTEOMYELITIS
Radiological features: There is uniform zone of increase
It is chronic recurrent multifocal osteomyelitis. In this radiodensity in the periapical area of tooth (Fig. 22.27).
there is involvement of multiple bones and it is widespread There is also widening of periodontal ligament space.
variant primary chronic osteomyelitis.
There is metachronous involvement of multiple bone Histopathological Features
like clavicle, humerus, radius, femur, or tibia. Mandibular It appears as an area of dense bone with trabeculae borders
involvement in CRMO occur in less than 10 percent of lined by osteoblasts. Chronic inflammatory cells, plasma
cases. cells and lymphocytes are seen in the scanty bone marrow.
Focal Sclerosing Osteomyelitis Management

(Condensing Osteitis) Endodontic therapy: Endodontic therapy should be
It is nonsuppurative inflammatory condition often seen in carried out.
dentulous jaw. When the resistance of the alveolar bone
to odontogenic infection is high or the virulence of the Points to Remember
organisms is low, a chronic condition characterized by the Formation of focal areas of sclerosis around the
formation of focal areas of sclerosis around the roots of roots of the teeth, mandibular first molar with a large
the teeth can possibly result. There is deposition of new carious lesion, asymptomatic, uniform zone of increase
bone along the existing trabeculae, a process known as radiodensity, osteoblasts, chronic inflammatory cells,
appositional bone deposition. plasma cells, lymphocyte, endodontic therapy.

Osteomyelitis with Proliferative Periostitis
Age: It occurs almost in young person before the age of
It is also called periostitis ossificans. There is bone
20 years.
formation within periosteal reaction. It is characterized by
Location: The tooth commonly affected is mandibular formation of hard bony swelling at the periphery of the jaw.
first molar with a large carious lesion. It is associated It is essentially a periosteal osteosclerosis analogous to the
with nonvital teeth or in teeth undergoing the process of endosteal sclerosis of chronic, focal and diffuse sclerosing
degeneration. osteomyelitis.
Initially it was called Garre’s osteomyelitis by the

reported case in literature by Carl Garre in 1893. It has
been stated that he stated case report with inflammatory
564 periosteal hyperplasia demonstrating the onion skin like
reduplication of cortical plate. But has Garre does not have
specimen and at that time X-rays are not discovered. So
use of this term should be discarded and it should be called
osteomyelitis with proliferative periostitis.
These types of lesion occur when periosteum possesses
high potential for osteoblastic activity, infection is mild,
and there is fine balance between resistance of host and
number and virulence of organisms.
Causes
It is cause by dental caries, fracture, buccal bifurcation Figure 22.28 Osteomyelits with proliferative periostitis
cyst, and nonodontogenic infection.
Clinical and Radiological Features Management

Age and sex distribution: It occurs mainly below 30 Removal of cause: Extraction of offending tooth or
years; males are affected more commonly than females. endodontic therapy should be done.
Location: Most frequently involve the anterior surface Points to Remember

of tibia and femur. Mandible is affected more commonly Periostitis ossificans, hard nontender swelling with
than maxilla. It commonly occurs at the inferior border of medial and lateral expansion of jaw, secondarily infected
mandible, in first molar region. discomfort, lymphadenopathy, hyperpyrexia, radiopaque
Sign: It is presented as hard nontender swelling with medial lamination of bone parallel to each other, supracortical
and lateral expansion of jaw. Mass varies in size from 1 or and sub-periosteal mass, osteoblasts bordering many
2 cm to the involvement of the entire length on the affected of the trabeculae, trabeculae arranged parallel to each
side. Cortex may become 2 to 3 cm thick. other, retiform fibrous, diffuse or patchy sprinkling of
It may become secondarily infected and cause lymphocytes, removal of cause.
considerable discomfort. Lymphadenopathy, hyper pyrexia
and leukocytosis are common findings. Radiation Osteomyelitis
Radiological features (Fig. 22.28): There is radiopaque It is an infection of the irradiated bone.
lamination of bone which runs parallel to each other. The
radiolucent separation is seen between new bone and Etiopathogenesis
original cortex. Within new bone area of small sequestra After exposure to radiation (40 to 80 Gy), bone undergoes
osteolytic radiolucencies is present. marked decreased in vascularity. Such bone has poor
defensive process and susceptible to traumatic injury. It
Histopathological Features draws infection from extraction wound and infected pulp,
The supracortical and sub-periosteal mass is composed of severe periodontitis and denture stomatitis. It will cause
much reactive new bone and osteoid tissue, with osteoblasts death of bone cells and result in progressive obliterative
bordering many of the trabeculae. These trabeculae often arteritis. This results in aseptic necrosis of the portion of bone
are oriented perpendicular to the cortex, with the trabeculae directly in beam of radiation, with compromised vascularity
arranged parallel to each other or show a retiform pattern. in the adjacent bone. Pathogenic organisms are introduced
The connective tissue between the bony trabeculae is into this irradiated bone through odontogenic infections,
rather fibrous and shows a diffuse or patchy sprinkling of compound fractures of the jaws and mucosal lacerations.
lymphocytes and plasma cells. The organisms most commonly found are Staphylococcus
aureus, Staphylococcus epidermidis. Higher incidences These enzymes break down fibrin, connective tissue
in jaw are recorded due to higher degree of infections and ground substance and cellular debris, thus facilitating rapid
frequent trauma to these bones. spread of bacterial invaders.
In some cases cellulitis of face can occur secondary to 565
Clinical Features infection from dens evaginutus teeth.
It occurs with a triad of radiation, trauma and infection. At least 50 percent of facial cellulitis cases in the
Effective response to infection becomes markedly pediatric population have been reported to be caused by
diminished. odontogenic infections.
Location: It is common in mandible than maxilla as Clinical Features

irradiation is often directed to mandible. Sign and symptoms: There is widespread swelling,
Sex distribution: It is common in males due to their redness and pain without definite localization. There is
probable involvement in traumatic events and increased presence of tenderness on palpation.
risk of oral cancer. Tissues are grossly edematous (Fig. 22.29). There
is marked induration, hence tissues are firm to hard on
Symptoms: Intense pain and facial fistula develop from palpation. Tissues are often discolored; temperature is
the subperiosteal tissues. Spread is diffuse and throughout elevated with malaise and lethargy. Usually massive
with signs of inflammation and swelling. cellulitis will ultimately suppurate, particularly if bacteria
Management are staphylococcal.
Depending upon the location and proximity to anatomic
It is directed at control of infection and penicillin is the structures that guide the progress, the pus may evacuate
antibiotic of choice. into nose, maxillary sinus, oral vestibule, floor of mouth,
infra-temporal fossa and into fascial spaces (Fig. 22.30).
Points to Remember
Infections arising in maxilla perforate the outer cortical
Common in mandible, intense pain, and facial fistula, layer of bone, above the buccinators attachment and, cause
penicillin. swelling of upper half of face.
If infection in mandible perforates the outer cortical
CELLULITIS palate, below the buccinators attachment, there is diffuse
It is also called Phlegmon. Occasionally, the infectious

process progresses out of the bone, despite the use of
supportive therapy and the patient develops cellulitis,
either in vestibular region or extraorally. It is a potential
complication of acute dental infection.
Cellulitis may be defined as a nonsuppurative
inflammation of the subcutaneous tissues extending along
the connective tissue planes and across the intercellular
spaces.
If improperly managed, cellulitis of the head and neck
region can lead to more severe systemic complications
including sepsis, airway obstruction, central nervous
system involvement and mortality.
Bacteriology and Causes

The alpha hemolytic streptococci are the classic etiologic Figure 22.29 Patient having cellulitis showing swelling on left side
agent. They produce enzymes like streptokinase and face which involving submandibular space, pterygomandibular
hyaluronidase. space
tooth and specific antibiotic cover bring about resolution

of the process.
Antibiotics like clindamycin was administered
566 intravenously.
Points to Remember
Phlegmon, widespread swelling, redness and pain, tissues
are grossly edematous, marked induration, temperature
is elevated, pus may evacuate into nose, maxillary sinus,
oral vestibule, floor of fascial spaces, swelling of upper
half of face, diffuse swelling of the lower half of face,
diffuse exudation of polymorphonuclear leukocytes,
occasional lymphocytes, surgical incision and drainage.
Figure 22.30 Submandibular space infection causing cellulitis LUDWIG’S ANGINA

It is a condition which was first described by Ludwig in
1936. The word angina means sensation of choking or
swelling of the lower half of face, which then spreads
suffocation. It is the most commonly encountered neck
superiorly as well as cervically. If maxillary tooth is
space infection.
involved, there may be redness of eye.
Laboratory Findings Definition

This condition may be defined as an overwhelming, rapidly
ESR and white blood cell count are raised.
spreading, septic cellulitis involving submandibular,
Histopathological Features submental and sublingual spaces bilaterally.
Once infection enter submandibular space it can extent
Diffuse exudation of polymorphonuclear leukocytes and
into lateral pharyngeal space and retropharyngeal space
occasional lymphocytes, with considerable serous fluid and
which later may spread into mediastinum.
fibrin, causing separation of connective tissue or muscle
fibers. It presents only a nonspecific picture of diffuse Etiology
acute inflammation.
• Odontogenic infection
Management • Trauma
• Sialadenitis
Surgical incision and drainage: It is performed when the
• Calculi
presence of pus is diagnosed. In case of large cellulitis,
• Osteomyelitis.
a superficial erythematous spot develops, which is
pathognomonic of pus near the superficial surface. These
superficial fluctuant areas can be incised and drained under Etiology
local anesthesia. Usually ring block of peripheral skin, as Odontogenic infection: It is usually an extension of
a wheel is made for skin anesthesia. Knife is introduced odontogenic infection from mandibular molar teeth into
in the most inferior portion of the fluctuant area. A small the floor of mouth. The 2nd and 3rd molars are the teeth
sinus forcep is introduced in the wound, opened in several most commonly involved.
directions and pus is drained. A rubber drain is placed in
Trauma: It may also be caused by oral soft tissue laceration
the deepest portion of the wound, so that just 12 cm lie
and punctured wounds of the oral floor.
above the source of the skin, where it is sutured. A large
dressing is applied. When no superficial spot is present, Sialadenitis: Submandibular gland sialadenitis and
fluctuance is more difficult to determine, particularly if infected malignancy may be sometimes contributory to
deep pus is suspected. Usually, extraction of the offending Ludwig’s angina.
Calculi: Salivary calculi or from intravenous injection of the roof of the mouth and the posterior pharyngeal wall;
the internal jugular vein, especially in drug abusers. when this occurs, acute respiratory obstruction is likely to
occur. Two large potential spaces at the base of the tongue
Osteomyelitis: Osteomyelitis in compound mandibular 567
are involved, i.e. submental and sublingual.
fracture.
Woody tongue: Involvement of sublingual space results
Bacteriology in elevation and posterior enlargement and protrusion of
Streptococci are the most commonly reported organism tongue. This is called woody tongue.
from the culture. Other microorganisms are a hemolytic Floor of mouth appears erythematous and edematous.
streptococci, Bacteroides, Klebsiella, Fusiform bacilli and Stiffness in tongue movement generally develops. The
E. coli. patient develops a toxic condition and speech becomes
impaired.
Clinical Features Larynx and glottis become edematous. As the disease
There are three typical appearances of Ludwig’s angina: continues the swelling starts involving the neck above the
level of hyoid which is called as bull neck.
First: It is characterized by brawny indurations. Tissues The patient always has fever and there is considerable
are board like and do not pit on pressure. No fluctuance salivation, as the patient is unable to swallow. There are
is present. The tissues may become gangrenous and when chills accompanied with fever. There is an inability to
cut, they have a peculiar lifeless appearance. A sharp swallow and to eat.
limitation is present between the infected tissues and Respiratory distress is common. There is also neck
surrounding normal tissues. pain, redness of neck, fever, weakness, fatigue, excessive
Second: Three facial spaces are involved bilaterally, tiredness, confusion or other mental changes, difficult
i.e. submandibular, submental and sublingual. If the breathing and earache. There is an intense pain on tongue
involvement is not bilateral, the infection is not considered movement and the patient may be severely dehydrated,
a typical Ludwig’s angina. owing to inability to take anything by mouth. If the swelling
has spread into the pterygoid region, then there is difficulty
Third: The mouth is open (Fig. 22.31) and the tongue is
in opening the mouth.
lifted upwards and backwards, so that it is pushed against
Fatal Complications
Respiratory obstruction: The infection of Ludwig’s
angina tends to spread through the connective tissues
which cover the small muscles of the larynx and between
the muscles of the floor of mouth. The epiglottis and larynx
become edematous along with the posterior aspect of the
tongue. The tongue gets elevated and gets pressed upward
and backward, causing pressure on the larynx. Therefore,
dyspnea occurs in paroxysm. Ultimately, death occurs due
to respiratory embarrassment.
Generalized Septicemia
Erosion of the carotid artery: Late spread of infection
to lateral pharyngeal space can also cause erosion of the
carotid artery.
Cavernous sinus thrombosis with subsequent meningitis
may be sequelae of it.
Figure 22.31 Patient of Ludwig angina showing typical open Others: Mediastinum extension, pharyngomaxillary space
mouth appearance extension, osteomyelitis and airway obstruction.
Prevention between the chin and hyoid bone, is a classic approach to

surgical drainage of Ludwig’s angina. It may be carried out
Regular visits to the dentist and prompt treatment of oral/
under local anesthesia.
568 dental infections can prevent the conditions that increase
the risk of developing Ludwig’s angina. Supportive therapy: Parenteral hydration, high protein
diet and vitamin supplements.
Laboratory Findings Extraction of offending tooth.
A moderate leukocytosis is found. Fusiform bacilli and
spiral forms, various staphylococci, diphtheria and may Points to Remember
other microorganisms have been cultured on different Streptococci, brawny indurations, tissues are board like,
occasions. peculiar lifeless appearance, facial space, submandibular,
submental and sublingual, mouth is open, woody tongue,
Management floor of mouth appears erythematous, larynx and glottis
Intense and prolonged antibiotic therapy: Penicillin become edematous, bull neck, respiratory obstruction,
is to be administered IM or IV, in high doses, because erosion of the carotid artery, cavernous sinus thrombosis,
it is the empirical antibiotic of choice and the oral flora, intense and prolonged antibiotic therapy, establishment
including most of anaerobes, are sensitive to it. Recently, and maintenance of an adequate airway are the essentials
combination of gentamicin and cloxacillin has been proved of therapy incision and drainage, supportive therapy.
successful.
Establishment and maintenance of an adequate FATAL COMPLICATIONS OF
airway are the essentials of therapy: Tracheostomy ORAL INFECTION
is a routine procedure, but it is often difficult to perform
in the late stages. Attempt at blind intubations is often Bacterial Meningitis
time consuming. Recently, successful intubations with It is the most common neurologic complication resulting
fiberoptic laryngoscope have been introduced as a from oral and maxillofacial infections.
worthwhile technique in the therapy of Ludwig’s angina. In this condition bacteria infect arachnoid pia mater
In the late stage, cricothyroidotomy may be performed as and CSF. The infection quickly spreads from its point of
an emergency procedure. origin, via CSF, to the entire subarachnoid space.
Incision and drainage (Fig. 22.32): It is done for the Bacteriology
release of tissue tension. A horizontal incision, midway
Microorganisms responsible are Staphylococcus; Strep-
tococcus, pneumococci, Proteus, Klebsiella and H.
influenzae are common pathogens causing meningitis
secondary to head and neck infection.
Clinical Features
Symptoms: Patient complaint of headache, chills, fever
and nausea, pain in back and stiffness of neck.
Kernig’s sign: It is assessed with the patient lying supine,
with the hip and knee flexed to 90 degrees. In a patient with
a positive Kernig’s sign, pain limits passive extension of
the knee.
Brudzinski’s sign: It occurs when flexion of the neck
causes involuntary flexion of the knee and hip.
Diagnosis is made by lumbar puncture and CSF
Figure 22.32 Ludwig’s angina is drained surgically examination.

It is a medical emergency and prompt intravenous Headache, dysphagia, visual defects, papilledema, hemi-
antibiotics should be started, after antibiotic sensitivity paresis, confusion and stupor, drainage of the abscess. 569
testing.
Cavernous Sinus Thrombosis
Points to Remember
Staphylococcus, Streptococcus, pneumococci, Proteus, It is one of the most dreaded and life-threatening
Klebsiella, headache, chills, fever and nausea, pain complication due to intracranial spread of infection from
in back, Kernig’s sign, Brudzinski’s sign intravenous odontogenic source.
antibiotics. Etiology
The bacteria can reach cavernous sinus through anterior
Brain Abscess
pathway, posterior pathway or direct extension.
It can develop from bacteremia associated with odontogenic
infections. Anterior pathway: It results from maxillary anterior teeth,
perforation of maxillary bone, canine space involvement
Pathogenesis formation of septic thrombus, it transfer from retrograde
fashion from angular vein, inferior ophthalmic vein through
Once the bacteria’s reach the brain tissues. It results in
inferior orbital fissure into cavernous sinus.
local cerebritis. Inflammatory exudates collects along
with degenerative leukocytes. Septic thrombosis of blood Posterior pathway: Infection from maxillary premolar
vessels occurs. Cerebral edema develops around the and molar, buccal and intratemporal space involvement,
infected area. As the abscess grows, its center becomes infection spread through emissary vein from pterygoid
pus filled with a capsule of granulation tissue at its venous plexus to inferior petrosal sinus and cavernous sinus.
periphery. As the abscess increases in size, it may rupture Direct extension through skull, by an abscess located
into the ventricular system and the result is usually fatal. in deep spaces such as in intratemporal space. The direct
If it extends to subarachnoid space, meningitis may antral infection may also give rise to this disease.
develop.
Bacteriology
Clinical Features Many organisms have been found in culture of CSF inclu-
Symptoms: Headache due to rise in intracranial pressure ding Pseudomonas, Corynebacterium, Staphylococcus
can occur. There is also nausea, convulsions and vomiting albus, Streptococcus, Diplococcus pneumoniae and Proteus.
may occur. Most common organism is Staphylococcus aureus.
Signs: Dysphagia and visual defects, if it involves the Clinical Features
temporal lobe. Papilledema and convulsions are common
Eye lesion: Proptosis or protrusion of eye is seen as a
features.
result of decreased venous drainage, chemosis and edema
If abscess is present on motor cortex then patient will
of eyelid, which is secondary to venous stasis. Limitation
suffer from hemiparesis, and if abscess is in frontal lobe,
of extra-ocular movements occurs because of involvement
confusion and stupor will occur.
of 3rd, 4th and 6th cranial nerves.
Diagnosis is made by radionuclide scanning and CT
Cranial nerve palsy of 3rd, 4th and 6th nerve is usually
scanning.
evident due to irritation caused by pressure of venous
Management congestion.
There is rapid progression of signs and symptoms from
Drainage of the abscess via catheter through a bur hole
one eye to other eye, due to spread of infection through
is the treatment of choice. Once the size of the abscess is
inter-cavernous sinus.
decreased the complete capsule should be excised.
Some surgeons prefer to do craniotomy initially and Symptoms: There is high spiking fever, severe headache,
then excise the entire abscess. stiffness of neck, ocular palsy and facial weakness.
Investigations The maxillary anterior teeth can produce orbital

cellulitis by retrograde spread through vessels like anterior
Lumbar puncture should be made. CSF shows neutrophils,
facial, angular and ophthalmic vein.
decreased glucose concentration and elevated protein
570 Progress of orbital infection posteriorly may involve
concentration. Blood culture and sensitivity should be
the superior orbital fissure and spread to cavernous sinus
done.
via superior ophthalmic vein.
Management
Clinical Features
Massive doses of antibiotics, with proper surgical
Sign and symptoms: It may result in temporary loss of
intervention at the primary site of infection are essential.
visual acuity. Long term ophthalmologic sequelae include
The initial drug of choice is IV chloramphenicol 1 gm, after
permanent loss in visual acuity, residual proptosis, diplopia
every 6 hours.
and blindness.
An attempt should be made to identify the causative
When later CNS is involved, hemiparesis, seizures and
organism by culture from the source of infection or from
death have also been reported.
blood culture, so that precise antibiotic sensitivity can be
established. Management
Anticoagulant therapy to prevent further thrombosis
and dissemination of septic emboli is given. When orbital infection is suspected, early aggressive
antibiotic therapy and surgical intervention, following
Points to Remember appropriate consultation, should be initiated to prevent
serious ocular complications and CNS involvement.
Proptosis or protrusion of eye, cranial nerve palsy,
high spiking fever, severe headache, neutrophils,
Points to Remember
decreased glucose concentration, IV chloramphenicol,
anticoagulant therapy. Infection of premolars and molars temporary loss of
visual, CNS, hemiparesis, early aggressive antibiotic
therapy.
Odontogenic Infection of Orbit (Fig. 22.33)
Odontogenic infection can spread to orbit through several Mediastinitis
routes. Infection of premolars and molars can perforate the
maxillary buccal plate and can spread to pterygopalatine It is usually a late complication of facial infection. The
and infra-temporal fossa and reach the orbit, via inferior process occurs because it gives a pathway for spread of
orbital fissure. pus and infection from submandibular region, floor of
mouth and from all the related spaces in neck, beneath the
investing layer to deep cervical fascia.
Following this pathway, infection can come into close
relationship with trachea, larynx and great vessels and
eventually reach the mediastinum.
The most common anatomic pathway is lateral
pharyngeal space, through visceral space inferiorly, which
may occur causing weakening and rupture of pleura.
Clinical Features
Symptoms: Usually there is chest pain and severe dyspnea
with unremitting fever and evidence of swelling on the
lateral aspect of neck of the affected side. Involvement of
one or two facial spaces is usually preceded by these signs
and symptoms.
Figure 22.33 Infection of orbit as a complication of Progressive septicemia, mediastinal abscess, pleural
odontogenic infection effusion, empyema, compression of mediastinum veins
with decreased venous return to heart and pericarditis may The progression of disease can be alarmingly rapid
occur, with death as the final step. with skin color changing from red blue to gray in as early
Hemorrhage secondary to erosion of internal carotid as 36 hours leading to frank cutaneous gangrene due to
artery or one of its branches is the most common cause of thrombosis of nutrient vessels, usually by 4th or 5th day. 571
death in deep space infection. Skin bullae may develop, but lymph adenitis is usually
not seen. Skin death subsequent to subcutaneous necrosis
Management is common.
Utmost priority is to be given for airway control. Systemic complications such as neck organ involvement,
In such cases, either emergency tracheostomy or pneumonia, pulmonary abscess, vascular erosion, venous
cricothyroidectomy may be performed. thrombosis and cranial neuropathies occurs.
Specific antibiotic therapy—in high doses intensive
antibiotic therapy instituted. Histopathological Features
Surgical drainage of mediastinum should be done. The pathological changes of necrotizing fasciitis (NF)
include thrombosis of blood vessels, suppuration and
Points to Remember necrosis of the superficial fascia with subcutaneous fat.
Submandibular region, floor of mouth, lateral pharyngeal Management
space, chest pain and severe dyspnea, progressive
Spectrum of antibiotics should include drugs active against
septicemia, hemorrhage secondary to erosion of internal
anaerobic organisms such as metronidiazole.
carotid artery, tracheostomy or cricothyroidectomy,
Continuous wound care is of utmost important.
specific antibiotic therapy.
Irrigation with hydrogen peroxide, followed by dressing of
charcoal lime and boric acid solution soaked gauze should
Necrotizing Fasciitis be applied.
This condition was first recognized in 1924 by Meleney. It Hyperbaric oxygen therapy has been used, but its value
is defined as rapidly progressing necrosis of subcutaneous is not proven.
tissue and fascia, usually sparing the muscles and Blood transfusion and general supportive measures
accompanied by toxicity, high fever and apathy. must be given. As soon as disease is controlled split skin
It is multimicrobial infection which spread very quickly. graft should be applied, if necessary for reconstruction.
Importance of Streptococcus pyogenes, a major cause of It is important that airway be maintained open, since
severe necrotizing and fulminating infection, must not be they may be compromised as a result of inflammation.
forgotten. Intubation may be difficult and tracheostomy may be
It is characterized by the formation of large necrotic required.
lesions and gas, located in the subcutaneous tissue and
superficial fascia. As the disease progresses, muscles and Points to Remember
skin involvement develop, giving rise to myonecrosis, that Multimicrobial infection, large necrotic lesions, tender
passes through the infected fascia. erythematous cellulitis with ill defined margins, red
Immunosuppressed states of peripheral vascular disease blue to gray, skin bullae, systemic complications such
like diabetes mellitus have been reported the most common as neck organ involvement, pneumonia, thrombosis of
predisposing factors. Other diseases which can cause are blood vessels, necrosis of the superficial fascia with
malignancy, alcoholism. subcutaneous fat, metronidiazole, continuous wound
care, hyperbaric oxygen therapy, blood transfusion.
Clinical Features
Symptoms: A tender erythematous cellulitis with ill
defined margins presents the patient with high fever
ORAL FOCI OF INFECTIONS
and apathy. Pain can be severe, but the affected area of Infected periapical lesion: Particularly those of chronic
skin becomes anesthetic, secondary to cutaneous nerve nature an area usually surrounded by the fibrous capsule,
destruction, which can occur before clinical gangrene. which effectively walls off or separates the area of
infection from the adjacent tissues but do not prevent the result is found and sulfonamide, antibiotics and vaccine
absorption of bacteria or toxins. Periapical granuloma has have failed to produce desirable results.
been described as a manifestation of vigorous body defense Valvular heart diseases: Sub-acute bacterial endocarditis
572 and repair reaction, while cysts merely a progressive form is without doubt related to oral infections. There is close
of granuloma. Abscess occurs when the reparative and similarity, in most instances, between the etiologic agent of
defensive phase is minimum. Majority of investigators the disease and microorganisms in the oral cavity, dental pulp
indicate that an unusually high percentage of periapical and in periapical lesions. Symptoms of subacute bacterial
granuloma are the biologically sterile and for this reason endocarditis have been observed in some instances shortly
the possibility such lesions giving rise to focal infection is after extraction of teeth. Transient bacteremia frequently
minimum. follows tooth extraction. Streptococci of viridans type
Teeth with infected root canals: These are potential cause majority of sub-acute bacterial endocarditis. After
sources of dissemination of microorganisms and toxins. tooth extraction, there is streptococcal bacteremia, so there
Most commonly it shows occurrence of a hemolytic is occurrence of subacute bacterial endocarditis after dental
Streptococcus; which is the most important in etiology of operations, dental extractions. Premedication of the patient
rheumatoid arthritis and rheumatic fever. should be done before extraction.
Periodontal disease: It is equally significant as potential Gastrointestinal disease: Some workers state that constant
source of infection. The usual organism recovered is swallowing of microorganisms might lead to variety of
Streptococcus viridans. Simple massage of gingiva may gastrointestinal diseases. Gastric and duodenal ulcers are
result in transitory bacteremia. The rocking of teeth in their produced by injection of streptococci.
socket by forceps, before extraction, has been shown to Ocular diseases: Factor supporting the hypothesis of
favor bacteremia in patients who have periodontal disease. Woods the role of foci of infection in ocular diseases.
Due to pumping action during extraction microorganism Many ocular diseases occur in which no systemic cause,
may be forced from the gingival cervix into the capillary other than presence of remote foci of infection can be
of gingiva as well as into the pulp of tooth and thus, will demonstrated. Numerous instances of prompt and dramatic
results in bacteremia. Oral prophylaxis may be followed by healing of ocular diseases are reported following the
bacteremia. So it is mandatory to administer the antibiotics removal of these foci. Occasionally, sudden transient
to the children who area diagnosed rheumatic or congenital exacerbation is observed, after the removal of teeth and
heart disease; to prevent the positive consequences of tonsils. Presence of blood stream infection in early stages
bacterial endocarditis. of ocular disease, are evident. Iritis may be produced by
intravenous injection of microorganisms, e.g. streptococci.
Significance of Oral Foci of Infection There is some objection to these points, i.e. many healthy
There are reports that the oral foci of infection either cause, people have focal infections, but do no have ocular diseases,
or aggravate many systemic disorders. Most common are spontaneous care may occur if nothing is done and positive
as follows: blood cultures are rare in acute iritis.
Arthritis: It is of mainly rheumatoid and rheumatic fever Skin diseases: Some forms of eczema and possibly urticaria,
type. Arthritis of rheumatoid type is of unknown etiology. can be related to oral foci of infection. If the relationship
These patients have high antibody titer to group of hemolytic does not exist, the mechanism is probably sensitization,
streptococci. It is tissue hypersensitive reaction. There are rather than metastatic spread of the microorganisms.
some points in favor of septic foci theory: streptococcal Renal disease: Microorganism most commonly involved
infection of throat, tonsils or nasal sinus may precede the in urinary infection are E.coli, staphylococci and
initial or recurrent attack, dramatic improvement occurs streptococci. Streptococci hemolyticus seems to be the
sometimes after the removal of septic foci and temporary most common. Streptococci are uncommon inhabitants of
bacteremia may occur immediately after tonsillectomy, dental root canals or periapical and gingival areas. Since
tooth extraction or after vigorous massage of gums. Against the microorganisms commonly involved in renal infection
the theory, there are some points: often no infective focus so it appear that there is little relationship between oral foci
can be found, usually when focus is extirpated, no dramatic of infection and renal disease.
DRY SOCKET Analgesic: Potent analgesic should be given.
It is also called alveolar osteitis and fibrinolytic alveolitis. Obtundent dressing: Dressing of idoform gauze with
It occurs due to disruption of clot which occur secondary eugenol reduce the symptoms. This dressing should be 573
to transformation of plasminogen to plasmin with lysis of change every 24 hours.
fibrin and formation of kinin. Antibiotics: This should be place intra-alvolar. Antibiotics
used are tetracycline, lincomycin, clindamycin and
Cause (Fig. 22.34) metronidiazole.
Local factors like trauma, poor oral hygiene, traumatic
extraction, use of oral contraceptive, and presurgical Points to Remember
infection can lead to dry socket. Fibrinolytic alveolitis, severe pain, swelling, bare bony
Use of tobacco produce like smoking, also lead to dry socket, sensitive to percussion, irrigation of socket,
socket. Heavy sucking, splitting and inadequate irrigation analgesic, obtundent dressing, antibiotics.
can also be causative organism.
Clinical Features BIBLIOGRAPHY

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1. ‘Phlegmom’ refers to: 9. A productive pulpal inflammation due to an extensive

a. Ludwig’s angina b. Cellulitis carious exposure of a young pulp is:
c. Brain abscess d. Both a and b a. Pulpitis aperta
2. Brawny indurations, board like tissues which do not b. Pulp polyp
pit on pressure is the classical feature of: c. Chronic hyperplastic pulpitis
a. Septicemia b. Cellulitis d. All of the above
c. Ludwig’s angina d. Sinusitis 10. In pulp polyp the teeth most commonly involved are:
a. Central incisors b. 2nd molars
3. Pain in the area of eyeball, postnasal drip and stuffiness
c. 1st molars d. Premolars
is the feature of:
a. Cellulitis b. Septicemia 11. Localized mass of calcified material which do not
exhibit dentinal tubules is:
c. Maxillary sinusitis d. Mediastinitis
a. False denticle b. True denticle
4. Kernig’s and Brudzinski’s signs are positive in: c. Attached denticle d. Free denticle
a. Bacterial meningitis b. Necrotizing fasciitis
12. Cracked tooth syndrome can be confirmed by:
c. Bacteremia d. Mediastinitis
a. Using a dye
5. Cranial nerve palsy of 3rd, 4th, 6th nerve is the clinical b. Fiber optic light
feature of: c. Selective biting pressure
a. Cavernous sinus thrombosis d. All of the above
b. Bacterial meningitis 13. An acute inflammatory reaction superimposed on an
c. Bacteremia existing chronic lesion is called:
d. Maxillary sinusitis a. Acute periapical abscess
6. Dilation of pulp vessels, edema fluid collection and b. Phoenix abscess
hemoconcentration is the histopathological feature of: c. Condensing osteitis
a. Chronic pulpitis b. Focal reversible pulpitis d. None
c. Pulp hyperemia d. Both b and c 14. The infection of bone that involves all the three
components viz periosteum, cortex and marrow is
7. Acute suppurative pulpitis where the entire pulp
called:
undergoes liquefaction and necrosis is the feature of:
a. Osteomyelitis b. Periapical granuloma
a. Acute pulpitis b. Reversible pulpitis c. Osteosclerosis d. Condensing osteitis
c. Chronic pulpitis d. All of the above
15. Scattered sequestra and pockets of abscess formation
8. Formation of granulation tissue and deposition of is the feature of:
collagen on the surface of the pulp tissue in awide open a. Radiation osteomyelitis
exposure is called as: b. Chronic suppurative osteomyelitis
a. Pulp abscess b. Suppurative pulpitis c. Garre’s osteomyelitis
c. Ulcerative pulpitis d. Pulp calcification d. Periapical granuloma
23 Bone Disease
Manifested in Jaw
Anil Govindrao Ghom, Shubhangi Mhaske (Jedhe), Pranoti Pradhan
Chapter Outline
Â Fibro-osseous lesions Â Juvenile ossifying fibroma

Â Classification Â Osteoporosis
Â Fibrous dysplasia Â Infantile cortical hyperostosis
Â Cherubism Â Osteopetrosis
Â Central giant cell granuloma Â Osteogenesis imperfecta
Â Paget’s disease Â Pierre Robin syndrome
Â Cemento-osseous dysplasia Â Marfan’s syndrome
• Periapical cemento-osseous dysplasia Â Down’s syndrome
• Florid osseous dysplasia Â Achondroplasia
• Focal cemento-osseous dysplasia Â Osteosclerosis
Â Familial gigantiform cementoma Â Massive osteolysis
Â Ossifying fibroma, cementifying fibroma and cemento- Â Gardner’s syndrome
ossifying fibroma
Bone is a dense calcified tissue which is specifically tissue containing a newly formed mineralized tissue.
affected by a variety of disease that often causes it to react Broadly they can be of the following categories:
in a dynamic fashion. These diseases of bone may arise at Developmental or hamartomatous lesions.
any age; some are congenital and present at birth, while Reactive or dysplastic lesions.
other develop in early childhood or in young adulthood. Neoplasms.
FIBRO-OSSEOUS LESIONS CLASSIFICATION

In it normal bone is replaced by benign fibrous tissue showing 1st
varying amounts of mineralization. The subject of benign
fibro-osseous enlargement of the jaws has many facets, the Fibro-osseous Lesions of Medullary Bone Origin
most sinister of which is the possibility that a healthy good- ∙ Fibrous dysplasia
looking person may take on a monstrous appearance. ∙ Fibro-osteoma
Fibro-osseous lesion is not a specific diagnosis but a ∙ Cherubism
process. They are a diverse group of processes that are ∙ Juvenile ossifying fibroma
characterized by replacement of normal bone by fibrous ∙ Giant cell tumor
Bone Disease Manifested in Jaw
∙ Aneurysmal bone cyst Types

∙ Jaw lesions in hyperparathyroidism
1st
∙ Paget’s disease.
Types 577
Fibro-osseous Lesions of Periodontal Origin • M onostotic fibrous dysplasia: In it, only one bone is
∙ Periapical cemental dysplasia involved.
∙ Florid osseous dysplasia • Polyostotic fibrous dysplasia: In it, more than one
∙ Cemento-ossifying fibroma bone is involved
∙ Cementifying fibroma – Jaffe-Lichtenstein syndrome: Pigmented lesions
∙ Ossifying fibroma. of the skin or ‘café au lait’ spots.
– McCune-Albright’s syndrome: Pigmented lesions
FIBROUS DYSPLASIA of the skin plus endocrine disturbances of various
types.
Definition: Fibrous dysplasia of bone, a disturbance of
medullary bone maintenance in which the bone undergoing
physiologic lysis is replaced by abnormal proliferation 2nd according to Stewart
of fibrous tissue, resulting in asymmetric distortion Types
and expansion of bone, maybe confined to one bone
• M
onostotic: Indicates involvement of a single bone.
(monostotic fibrous dysplasia) or involve multiple bones
• Monomelic: It refers to the involvement of one
(polyostotic fibrous dysplasia). Dysplasia means abnormal
extremity and is rarely found.
tissue development.
• Polyostotic: Many bones involved.
It arises from the bone forming mesenchyme in
• Albright’s syndrome.
the spongiosa and develops by proliferation of fibrous
tissue. Lichtenstein in 1938 coined the term ‘fibrous Subclinical fibrous dysplasia: Many a times an unsuspected
dysplasia’. It is also called fibrocystic disease, osteitis lesion of fibrous dysplasia comes to light accidentally on
fibrosa localisata, focal osteitis fibrosa and fibro- routine radiographic examination, without any clinical
osteodystrophy. evidence of the suspected disease. Such examples are termed
as sub-clinical fibrous dysplasia.
Etiology
FD is a genetic non-inherited condition caused by genetic Clinical Features
mutation in the gene GNAS1 on chromosome 20, that
Monostotic Fibrous Dysplasia
encodes the alpha subunit of the stimulatory G protein-
coupled receptor, Gsa. The activating mutations occur Age and sex distribution: Fibrous dysplasia discovered in
post-zygotically, replacing the arginine residue amino young patients, usually in children younger than 10 years
acid with either a cystein or a histidine amino acid. The affecting both the sexes equally.
mutation selectively inhibits GTPase activity, resulting Sites: Monostotic fibrous dysplasia involves only one bone
in constitutive stimulation of AMP-protein kinase A and presents no extra-skeletal effects, other than occasional
intracellular signal transduction pathways. pigmented skin lesions. This type accounts for about 80 to
The systemic manifestations of the mutated Gsa 85 percent of all cases. Most frequent sites are ribs, femur,
protein-coupled receptor complex include autonomous maxilla and mandible.
function in bone through parathyroid hormone receptor; Symptoms: Painless swelling of the bone is most common
in skin through melanocyte-stimulating hormone complaint patient gives (Fig. 23.1).
receptor; in ovaries through the follicle-stimulating
hormone receptor; and in the thyroid and the pituitary Polyostotic fibrous dysplasia (Jaffe’s type):
gland, through the thyroid and growth hormone receptors Sex predilection: Polyostotic fibrous dysplasia involves
respectively. multiple bones, with female to male ratio of 3:1.
Oral Manifestations
Monostotic
578 Site: Maxilla is more commonly affected than mandible,
with most changes occurring in the posterior region. Most
common area involved is premolar-molar area.
Symptoms: There may be unilateral facial swelling, which
is slow growing with intact overlying mucosa. Swelling is
usually painless but patients may feel discomfort in some
cases and while others complain of frank pain.
Enlarging deformities of alveolar process mainly buccal
and labial cortical plates is seen (Fig. 23.1). In mandible,
it causes protuberant excrescence of the inferior border of
mandible.
Figure 23.1 Fibrous dysplasia showing swelling in the palate The teeth present in the affected area are either
malaligned and tipped or displaced. Dental anomalies
Café au lait spot: The skin lesions consist of irregularly such as supernumerary teeth have been reported in
pigmented, light brown melanotic spots, described as ‘cafe connection with the monostotic fibrous dysplasia. These
au lait spot’. These pigmented lesion maybe congenital and supernumerary teeth often remain impacted and may affect
pigmented oral mucosal macules also may be present. The the eruption of normal teeth.
margins of the café au lait spots are typically very irregular,
Craniofacial fibrous dysplasia: If fibrous dysplasia
resembling a map of coast line of Maine.
extends to involve the maxillary sinus, the zygomatic
Recurrent bone pain is the most common presenting
process, floor of orbit and sometimes toward the base of
skeletal symptom. Skeletal lesions may be unilateral in
the skull, it is known as craniofacial fibrous dysplasia.
distribution or may involve nearly all bones of the body.
It results in severe malocclusion and marked facial
Spontaneous fracture is a common complication. In
deformity. Craniofacial lesions may lead to anosmia (loss
rare cases, continuous and inexorable extension results in
of sense of smell), deafness and blindness. There may be
great deformity and blindness.
proptosis of the affected eye.
Albright’s syndrome
Polyostotic (Jaffe’s type): Expansion and deformities
Albright’s syndrome, in addition show, endocrinal distur-
of jaws occurs. The eruption pattern of teeth is disturbed
bances like precocious puberty, goiter, hyperthyroidism,
because of loss of support of the developing teeth. There is
hyperparathyroidism, Cushing’s syndrome and acromegaly.
asymmetry of facial bones.
Albright’s syndrome is exclusively found in females.
There is ballooning of jaws, so there is gross enlargement
Vaginal bleeding has been noted.
and deformity. In some cases, intraoral pigmentation can
Secondary sexual characteristics such as pubic and
be seen.
axillary hair and development of breasts are evident by the
age of 5 years.
It may result in crippling deformities or fracture.
Precocious puberty is rare in boys and is manifested as Ground glass appearance: Patient usually get ground
gynecomastia. Long bones are frequently affected. Bone glass appearance which results from superimposition of
and skin lesions found in polyostotic form are unilateral. myriad of poorly calcified bone trabecular arranged in
Skeletal lesions become static with the cessation of disorganized pattern (Fig. 23.2).
growth but proliferation may continue, particularly in the Margin of the lesion is not well demarcated and it blends
polyostotic form. imperceptibly into the adjacent bone. There is expansion of
Another disorder characterized by fibrous dysplasia lingual and buccal cortical plates. In some cases superior
with intramuscular myxomas is termed Mazabraud displacement of inferior alveolar canal is seen.
syndrome. In earlier stages lesion may be radiolucent or mottled.
579
Figure 23.2 Fibrous dysplasia showing ground glass Figure 23.3 Chinese letter pattern bony trabaculae seen in
appearance of the lesion fibrous dysplasia
In case of involvement of maxilla lesion may cause

displacement of sinus floor superiorly and obliteration of
maxillary sinus can occur.
Laboratory Examination
Serum calcium and serum phosphorus concentration, as
well as serum alkaline phosphatase activity usually are
within normal limits.
But in rare instances particularly when the polyostotic
lesions are numerous and active, the serum alkaline
phosphatase levels may be elevated in 50 percent of the cases.
Monostotic fibrous dysplasia: The lesion is essentially
a fibrous bone made up of proliferating fibroblast in a
Figure 23.4 Fibrous dysplasia showing irregularly shaped
compact stroma of interlacing collagen fibers.
trabecular of woven bone in fibrous stroma
Irregular trabeculae of bone are scattered throughout
the lesion, with no definite pattern of arrangement. Some
of these trabeculae are C-shaped and described as Chinese
trabeculae of coarse, woven bone, irregular in shape but
character shaped (Fig. 23.3).
evenly spaced, showing no relation to functional pattern.
These trabeculae are usually woven bone, but may
The osteocytes are quite large and collagen fibers of
be lamellar. In some cases it has been shown that classic
these trabeculae can often be seen into the fibrous tissue.
fibrous dysplasia of jaws undergoes progressive maturation
These trabeculae have typically wide osteoid seams.
to a lesion consisting of lamellar bone in a moderately
Osteoclastic activity may be seen where the calcification
cellular connective tissue stroma. The bony trabeculae in
of osteoid extends to the surface of the trabeculae.
these mature lesions tend to run parallel to one another
(Figs 23.4 and 23.5). Management
Polyostotic fibrous dysplasia: The lesions are composed Surgical removal of lesion should be carried out if
of fibrillar connective tissue within which are numerous necessary.
appearance of affected children. It is autosomal dominant

inherited fibro-osseous disease that affects only the jaws
causing bony expansion.
580 Cherubism is a rare hereditary developmental condition,
manifested in childhood by bilateral enlargement of jaws
giving a cherub like appearance to the face. “Cherubs” are
referred to the plumped cheek little angels in the paintings
of Renaissance.
Classification
It depends on the severity and location of the lesion and the
extent to which jaws are affected:
∙ Grade I: The fibro-osseous expansion tends to be
bilateral and symmetrical. It is primarily in the ramus
Figure 23.5 Fibrous dysplasia showing broad trabeculae of of the mandible.
bone within fibrous connective tissue (Courtesy: Dr Sangamesh ∙ Grade II: In more severe cases the ramus and the body
Halawar, Reader, Department of Oral Pathology, VPDC and of the mandible are involved resulting in congenital
H, Kavalapur, Sangli, Maharashtra, India) absence of the third and occasionally the second
mandibular molar teeth. In this group the tuberosity
Osseous contouring: It is necessary for correcting the
region of the maxillae are also affected.
deformity for esthetics or preesthetic purposes. Smaller
∙ Grade III: In these cases, the lesions affect the mandible
lesion of mandible is surgically resected entirely.
and maxilla entirely and may result in considerable
In large and diffuse lesions particularly of maxilla
facial deformities.
may require surgical reduction of lesion to an acceptable
contour without complete removal. But the regrowth of
Etiology
lesion usually occurs with time.
It is autosomal-dominant, fibroblast/giant cell-containing
Points to Remember condition. It may be secondary to somatic mutation,
Monostotic, polyostotic, GNAS1 on chromosome 20, mapping to chromosome 4p16.3. There is possible linkage/
Café au lait spot, map of coast line of Maine, recurrent association with Noonan’s syndrome.
bone pain, spontaneous fracture, endocrinal disturbances, It is caused by anomalous development of dental struc-
secondary sexual characteristics, crippling deformities, tures, latent hyperparathyroidism, a hormone dependent
precocious puberty, Mazabraud syndrome, protuberant benign neoplasm, trauma, an aberration in ossification, and
excrescence, supernumerary teeth, severe malocclusion, disturbance in the development of bone forming mesen-
anosmia, ballooning of jaws, ground glass appearance, chyme.
displacement of inferior alveolar canal, displacement of
sinus floor, proliferating fibroblast, C-shaped, Chinese Clinical Features
character shaped, trabeculae parallel to one another Age and sex distribution: Early childhood between the
woven bone, osseous contouring. ages of 2 to 4 years males are affected about twice as
frequently as females.
CHERUBISM Site: It shows predilection for angle of mandible bilaterally
(Fig. 23.6) and occasionally posterior maxilla.
It is also known as familial fibrous dysplasia of the
jaws, disseminated juvenile fibrous dysplasia, familial Chubby appearance: In the rapidly increasing stage,
multilocular cystic disease of the jaws and Hereditary the child assumes a chubby, cherubic facial appearance,
fibrous dysplasia of the jaws. The clinical entity was especially if combined with involvement of the orbital
first described by Jones in 1933 who coined the term floor with upward displacement of the globe and exposure
‘Cherubism’ reflecting the characteristic chubby facial of the scleral rims (Fig. 23.7).
Swelling is firm and hard on palpation. Overlying

mucosa is intact and non-painful. The alveolar process are
so wide, as to occupy almost the whole of the roof of the
mouth; the actual palate being reduced to a narrow fissure 581
between the two approximating alveolar process. There
may be enlargement of sub-mandibular lymph nodes.
There is rapid increase in size up to 7 to 8 years of
age, after which the lesions become static or progress very
slowly until puberty. After puberty the maxillary lesions
tend to regress. The mandibular lesions progress slowly
up to the age of 20 years and then regress; the facial
appearance almost returns to normal in the 4th and 5th
decade of life.
The fibrous replacement of bone displaces the
Figure 23.6 Cherubism showing swelling intraorally
deciduous dentition. The primary teeth may be irregularly
spaced and some may be absent. There is premature loss
of primary teeth. The developing permanent teeth are
affected, giving rise to displaced unerupted or absent teeth
along with malocclusion.
There is multilocular expansile radiolucency which is
located bilaterally in the mandibular posterior location.
Laboratory Findings
Serum calcium, phosphorus and alkaline phosphatase
levels are within normal limits. But in active cases, serum
alkaline levels may be raised.

Figure 23.7 Chubby face appearance seen in cherubism Histopathology is characterized by similar picture as that
of central giant cell granuloma. Vascular fibrous tissue
consists of giant cells. The giant cells are small and
Symptoms: The patient may have difficulty in speech, aggregated focally. In some cases perivascular cuffing by
deglutition, mastication, respiration and limited jaw the eosinophils may be seen.
movement. It also contain large number of fibroblast and small
blood vessels. Sprinkling inflammatory cells can be found
Bilateral enlargement of mandible in this condition in this condition. Epithelial remnants of developing teeth
produces full, round lower face. Bilateral enlargement of are sometimes scattered throughout the lesion.
maxilla gradually follows.
Eye raised to heaven: Pulling or stretching of skin of the Management
cheek, depresses the lower eyelid, exposing a thin line of Surgical procedures should be delayed, as long as possible,
sclera and resulting in the so called “eyes raised to heaven” as the cystic lesion defect usually becomes static and
look. regresses during adulthood.
Tumor encroachment on the orbital floor often causes Occasionally, surgical contouring of the lesions
partial obliteration of the palatal vault with resulting V is necessary to improve esthetics and in case of active
shaped cleft. growth.
74 percent cases in younger than 30 years. It is more

commonly seen in females.
582 Site: Mandible is twice as frequently involved than

maxilla, with anterior half showing greatest incidence with
fairly high percentage crossing the symphysis. The most
common sites are anterior and bicuspid region in mandible,
the canine fossa, and ethmoid region in maxilla.
Expansion of bone: The earliest sign of the lesion may be
expansion of bone with premature loosening and shedding
of deciduous teeth. There is jaw swelling associated with
facial asymmetry.
Symptoms: Usually painless, but local discomfort may
Figure 23.8 Cherubism (Courtesy: Dr Sangamesh Halawar,
be noted. Palpation may elicit tenderness. Growth is slow.
Reader, Department of Oral Pathology, VPDC and H,
Kavalapur, Sangli, Maharashtra, India) Teeth in the area may become mobile but maintain their
vitality, until they are exfoliated.
Points to Remember Sign: The swelling is smooth, and palpation can reveal a
rubbery, elastic sensation where the bone has thinned.
Familial fibrous dysplasia of the jaws, autosomal-domi-
nant, angle of mandible bilaterally, chubby appearance, Radiological Features
round lower face, eye raised to heaven, ‘V’ shaped cleft,
Radiologically it appears as radiolucent area which can be
premature loss of primary teeth, multilocular expansile
unilocular or multilocular. Margin of the lesion are non-
radiolucency, giant cells, perivascular cuffing, fibro-
corticated. This lesion crosses the midline (Fig. 23.9).
blast, sprinkling inflammatory cells, surgical contouring.
CENTRAL GIANT CELL GRANULOMA It is made up of a loose fibrillar connective tissue stroma
The WHO has defined it as an intraosseous lesion consisting with many interspersed proliferating fibroblast and small
of cellular fibrous tissue that contains multiple foci of capillaries.
hemorrhage, aggregations of multinucleated giant cells Multinucleated giant cell are prominent thorough the
and occasionally trabeculae of woven bone. Some authors connective tissue which may vary in size from case to case
separate central giant cell granuloma (CGCG) into two and may contain several dozen nuclei. The multinucleated
types, referring to its clinical and radiographic features as. giant cells show a patchy distribution and are usually
Nonaggressive lesion which is usually slows growing associated with areas of hemorrhage.
and asymptomatic.
Aggressive lesion which is usually found in younger
patients and is painful, grows rapidly, is larger overall, and
often causes cortical perforation and root resorption and
has a tendency to recur.
It is a non-neoplastic bone disease reactive to some
unknown stimulus. It was first described in the jaws by
Waren 1837. It has been called osteoclastoma, myeloid
sarcoma, chronic hemorrhagic osteomyelitis and giant cell
reparative granuloma.
Clinical Features
Age and sex distribution: Lesion of adolescent and young Figure 23.9 Radiolucent defect seen in mandible which
adult with 60 percent cases in younger than 20 years and crosses the midline of central giant cell granuloma
583
Figure 23.10 Central giant cell granuloma giant cells Figure 23.13 Giant cell granuloma showing few giant cells and
bony trabeculae
There are also numerous foci of old extravasated blood

and associated hemosiderin pigment. The majority of
giant cells contain round nuclei but, some cells may have
pyknotic appearance.
Older lesions show considerable fibrosis of stroma.
Foci of osteoid and newly formed bone are occasionally
present within the lesion.
Ultra structurally, the proliferating cells include spindle-
shaped fibroblasts, myofibroblasts, and inflammatory
mononuclear cells.
Management
It is treated by enucleation, curettage and partial resection.
Figure 23.11 Central giant cell granuloma The lesions considered on clinical and radiological grounds
to be potentially aggressive show a higher frequency for
recurrence.
Different modalities have been investigated for
aggressive lesions alternative to surgery as corticosteroids,
calcitonin and interferon alfa-2a.
Corticosteroids: Weekly injection in the tumor for 6
weeks has been use successfully.
Points to Remember
GNAS1 on chromosome 20, Gsaα protein-coupled
receptor complex, painless swelling, expansion of
bone, facial asymmetry, local discomfort, unilocular,
multilocular, margin non-corticated, loose fibrillar
connective tissue stroma, proliferating fibroblast,
multinucleated giant cell, hemosiderin pigment, fibrosis
Figure 23.12 Giant cell granuloma of stroma.
PAGET’S DISEASE Complication of Paget’s disease is development of

osteosarcoma which is malignant tumor of the jaw bone.
It is also called osteitis deformans. It was discovered in
584 1877 by Sir James Paget. It is a condition characterized by Oral Manifestations
abnormal and anarchic resorption and deposition of bone Maxilla is involved three times more commonly than
resulting in distortion and weakening of affected bone. mandible. It is bilaterally symmetrical in the involved jaw.
The disease is initiated by an intense wave of osteolytic There is movement and migration of affected teeth,
activity with resorption of normal bone resulting in irregularly malocclusion and in edentulous patient poor fit of denture.
shaped resorption followed by vigorous osteoblastic activity Increase in alveolar width associated with flattening of
forming woven bone after variable period. palate when maxilla is involved. As the disease progresses
The etiology of Paget’s disease is unknown but the mouth may remain open exposing the teeth as the lips
inflammatory, genetic and endocrine may be contributing are too small to cover the enlarged jaws (Fig. 23.14).
factors. Recurrent mutation in sequestosome 1 gene Leontiasis ossea: Maxillary involvement results in
(SQSTM1) has been identified in both familial and sporadic enlargement of middle third of face which results in lionlike
cases. facial deformity (leontiasis ossea).
Extraction sites heal slowly and incidences of
Clinical Features
osteomyelitis are higher. Extraction may be further
It is seen most frequently in Britain and less frequently in complicated by excessive bleeding from highly vascular
North America and Western Europe. abnormal bones in the lytic phase of disease.
Age and sex distribution: It occur predominantly in
patients over 40 years of age with a slight predilection for Radiological Features
men. Male to female ratio is 2:1. In early stage there is decrease radiodensity of the bone
Sites: It is prone to occur in the axial skeleton especially with alteration in the trabecular pattern. In the skull large
the skull, femur, sacrum and pelvis. The disease maybe area of radiodensity can be see which is called osteoporosis
monostotic (i.e. limited to one bone), most cases of Pagets circumscripta.
disease are polyostotic (i.e. more than one bone is affected) After this area of sclerotic bone is formed which tend to
become confluent and it appear as cotton wool appearance.
Symptoms: First complains may be that patient needs
to buy a hat of larger size because of skull enlargement. Laboratory Investigations
Bone pain is a consistent symptom and most often directed
Serum alkaline phosphatase level is increased, occasionally,
towards weight bearing areas. Deafness occurs due to
attending the level of 200 or more KA units.
involvement of the petrous portion of temporal bone with
compression of cochlear nerve in the foramen. Mental
disturbance, dizziness also occurs.
Neuralgic pain: The patients may have ill defined
neuralgic pain as a result of restriction of foramina and
canals, which occurs due to pressure created by mass on
structures passing through the foramina.
Bowing of legs, curvature of spine and enlargement of
skull occur in some cases. The involved bones are warm
to touch because of increased vascularity and are prone to
fracture.
Involvement of weight bearing bones which lead to
bowing deformity which results in simian (monkeylike)
stance.
Grotesque facial appearance caused by bony
deformities. Broadening and flattening of the chest and Figure 23.14 Increase in alveolar width seen patient with
spinal curvature. The patient assumes waddling gait. Paget’s disease
Serum calcium and serum phosphorus levels are within

normal limits.
Urinary hydroxyproline levels increase from normal
levels of 440 mg/24 hours to 1 g/24 hours. Newer 585
and more sensitive markers of bone resorption are
N-telopeptides, C-telopeptides and pyridonoline cross
linked assays.

Collagen fibers within the lamellar bone loose their normal
orientation and run in many directions.
Figure 23.17 Paget disease showing sclerotic bone
Normal hematopoietic marrow is replaced by cellular

large vascular channels resulting in marked increase in
vascularity.
A highly fibrous connective tissue replaces the marrow.
Basophilic reversal lines are seen in the bone.
These lines indicate the junction between the alternating
resorptive and formative phases of bone and result in jigsaw
puzzle or mosaic pattern of bone.
One of the most characteristic feature of this bone
if the formation of ‘mosaic’ bone, a descriptive term
used to indicate the appearance of bone which has been
Figure 23.15 Paget disease showing reversal line
partially resorbed and then repaired leaving deeply staining
hematoxyphlic reversal lines.
Osteoclasts increase in size and number and are
morphologically abnormal. Later in the disease process
osteoblastic activity increases while osteoclastic activity
decreases and the bone becomes sclerotic.
Inflammatory edema of marrow is common and focal
collection of lymphocytes may be observed.
The proliferation of bone and concomitant hyper-
cementosis result in obliteration of periodontal ligament.
Management
Paget’s disease is a chronic and slow progressing disease. In
asymptomatic patients, treatment is often not initiated until
the serum alkaline phosphatase is more than 25 to 50 percent.
Antiresorptive therapy is recommended in patients with
bone pain, headache related to skull involvement, deafness
Figure 23.16 Paget disease (Courtesy: Dr Sangamesh or visual disturbances, bone fractures.
Halawar, Reader, Department of Oral Pathology, VPDC and Calcitonin and bisphosphonates etidronate, a parathy-
H, Kavalapur, Sangli, Maharashtra, India) roid hormone antagonist produced by the thyroid gland,
suppresses bone resorption and also relieves pain and Periapical Cemento-osseous Dysplasia
decrease serum alkaline. Now this agents are supplanted
It is also called osseous dysplasia, cemental dysplasia or
by newer bisphosphonates alendronate and risedronates.
cementomas.
586 Single infusion therapy with zoledronic acid can give
good results. Age and sex distribution: It is seen in age group of 30 to
50 years. Females are affected more commonly in the ratio
Points to Remember of 10:1.
Osteitis deformans, polyostotic, hat of larger size, Site: It has predilection for periapical region of anterior
neuralgic pain, bones warm to touch, simian (monkey region of the mandible.
like) stance, Grotesque facial appearance, osteosarcoma, Symptoms: It is asymptomatic and discovered routinely
migration of affected teeth, increase in alveolar width, on radiographic examination. Teeth are vital.
lion like facial deformity (leontiasis ossea), osteomyelitis,
osteoporosis circumscripta, cotton wool appearance, Florid Osseous Dysplasia
serum alkaline phosphatase increased, collagen fibers, Age and sex distribution: Females are exclusively
marked increase in vascularity, basophilic reversal affected. Most common age is middle age, with a mean age
lines, jigsaw” puzzle, mosaic pattern, osteoclasts, 42 years with predilection for blacks.
inflammatory edema of marrow, hypercementosis anti-
resorptive therapy, calcitonin and bisphosphonates Site: The lesion is restricted to the jaw bone with mandible
etidronate, a alendronate, risedronates, zoledronic acid. being most commonly affected. Lesion is multifocal with
bilateral involvement. In some extensive cases all the four
quadrants are get involve.
Cemento-osseous Dysplasia
Symptoms: Patients may complain of intermittent poorly
These occur in tooth bearing region of the jaws and they are
localized pain in the affected bone area, with or without an
most common fibro-osseous lesion seen in clinical practice.
associated bony swelling.
These occur in close approximation with periodontal
ligament and exhibits histopathological similarities with it. Signs: There is a painless expansion of the alveolar process
Some author believes that it is defect in extra ligamentary of mandible. Mucosal ulceration with fistulous tract may
bone remodeling that may be triggered by local factors. be present. Teeth are vital.
Hormonal imbalance may be causative factors for this
disease. Radiological Features
Histopathological features of all three variant are same. Focal cemento-osseous dysplasia: It can appear as
completely radiolucent lesion to dense radiopaque lesion.
Types Radiopaque lesion has got thin radiolucent rim.
• Focal cemento-osseous dysplasia Size of lesion is not more than 1.5 cm. Lesion is well
• Periapical cemento-osseous dysplasia defined with irregular border.
• Florid cemento-osseous dysplasia. Periapical cemento-osseous dysplasia: Early lesions are
circumscribed area of radiolucency in the apex of tooth.
Clinical Features This can affect many teeth in the same region.
Focal Cemento-osseous Dysplasia In later stage lesion gets mature and pattern of mixed
radiolucent-radiopaque appear.
Age and sex distribution: Over 90 percent of cases occur
In the end stage there is circumscribed radiopaque
in female as compared to male with mean age of occurrence
lesion surrounded by radiolucent rim. Size is usually less
38 years.
than 1 cm in diameter.
Site: It exhibits single site of involvement. Posterior region
Florid osseous dysplasia: It has got initially radiolucent
of the mandible is the most common site of involvement.
lesion which later on become mixed and completely
Symptoms: It is asymptomatic and discovered routinely radiopaque. Radiopaque lesion is surrounded by thin
on radiographic examination. radiolucent rim.
In some cases of florid dysplasia cemento-osseous Management

material may get deposited directly on the surface of
If teeth present effective oral hygiene should be maintained
the root of teeth resulting in thickened root apices. It is
since with this disease, patients exhibit poor healing and
surrounded by radiolucency causing hypercementosis like 587
osteomyelitis may develop after tooth loss.
appearance (Fig. 23.18).
As there formation of sequestration which occurs due
Histopathological Features to osteomyelitis saucerization of bone can result speeding
of healing.
All the three variant of cemento-osseous dysplasia have Many times onset of symptoms occurs after extraction
common histopathological features. of teeth as sclerotic masses appear in the oral cavity,
The tissue specimen consists of cellular mesenchymal elective extraction of teeth should be avoided.
tissue which consists of spindle shaped fibroblast and
collagen fibers. Numerous blood vessels may be present. Points to Remember
In the fibrous connective tissue there is present of Focal cemento-osseous dysplasia
woven bone, lamellar bone and cementum like particle
Over 90 percent female, posterior region of the
(Fig. 23.19).
mandible, asymptomatic, completely radiolucent to
With maturation bone trabecular pattern become thick
dense radiopaque lesion, size not more 1.5 cm.
curvilinear structure which resembles to shape of ginger
roots. Periapical cemento-osseous dysplasia
In the final stage there is trabecular fuse and lobular Osseous dysplasia, females 10:1, periapical region
masses which composed of sheets of fused globules of of anterior region of the mandible, asymptomatic,
acellular and disorganized cemento-osseous material. circumscribed area of radiolucency, mixed radiolucent-
radiopaque, radiopaque lesion with radiolucent rim.
Florid osseous dysplasia
Females, multifocal with bilateral involvement, inter-
mittent poorly localized pain, a painless expansion,
mixed and completely radiopaque, hypercementosis
like appearance.
Histopathology and management
Spindle shaped, collagen fibers, woven bone, lamellar
bone, cementum like particle, shape of ginger roots, lobular
Figure 23.18 Radiopaque lesion of florid dysplasia masses, effective oral hygiene, saucerization of bone.
FAMILIAL GIGANTIFORM
CEMENTOMA
Gigantiform cementoma earlier use as the synonym
for florid osseous dysplasia. But nowaday this is called
separate entity.
It is disorders of gnathic bone which lead to formation
of massive sclerotic mass of disorganized mineralized
material. It is autosomal dominant disorders which
demonstrated high penetrance with variable expressivity.
Clinical Features
Figure 23.19 Cemento-osseous dysplasia showing cemental Age and sex distribution: It is most commonly seen in
masses first and second decade of life without any sex predilection.
Facial asymmetry: Gnathic enlargement in patient results cementifying fibroma and tumors characterized by both
in significant facial deformity. If not treated this osseous types of cells, but probably the same progenitor cells, thus
involvement ceases during fifth decade. giving rise to the well recognized hybrid form of the tumor,
588 i.e. the cemento-ossifying fibroma.
Symptoms: There is impaction, malposition, and
It is understood that these are three sub-classifications
malocclusion of involved dentition.
of an otherwise identical lesion.
Patient also complaint of anemia, multifocal polypoid
adenomas of uterus, bone fragility and tendency to long Clinical Features
bone fracture.
Ossifying fibroma (Figs 23.20 and 23.21)
Radiological Features Age and sex distribution: It is a rare neoplasm and occurs
There are multiple radiolucencies in the periapical region at any age, but usually is found in the young adult with
which with progression appears as mixed radiolucent females more commonly affected than males.
radiopaque region within the involved quadrant. With
maturation lesion become more radiopaque with thin
radiolucent line surrounding the radiopacities.
These are same as that of cemento-osseous dysplasia.
Management
Improvement of esthetic by shave down surgical procedure
has been attempted.
In some cases extensive resections of altered bone with
reconstruction of the facial skeleton with produce good
results.
Points to Remember
Gnathic bone, massive sclerotic mass, facial asymmetry, Figure 23.20 Ossifying fibroma seen in upper maxillary
impaction, malposition, anemia, multifocal polypoid region of the jaw
adenomas, radiolucencies, mature lesion radiopaque
with radiolucent line, shave down surgical procedure.
OSSIFYING FIBROMA, CEMENTIFYING

FIBROMA AND CEMENTO-OSSIFYING
FIBROMA
These are characteristically encapsulated neoplasms
consisting of highly cellular fibrous tissue in which bone
formation occurs. They may show a locally aggressive
behavior. There is remarkable similarity in clinical
and radiological features of these lesions. There is also
considerable similarity and even overlap in histological
features of these lesions. For this reason it is suggested that:
They are three separate/distinct benign tumors identical
in nature, except for the cells undergoing proliferation. Figure 23.21 Ossifying fibroma (Courtesy: Dr Aparna Thombre,
The osteoblasts with bone formation in ossifying Reader, Department of Oral Pathology, VSPM Dental College
fibroma, cementoblasts with cementum formation in and Hospital, Nagpur, Maharashtra, India)
Symptoms: There are mild deformities and displacement

of teeth. It is slow growing. Cortical plates of bone and
overlying mucosa or skin are almost invariably intact.
589
Cemento-ossifying Fibroma
Signs and symptoms: Pain and paresthesia may result
from pressure on adjacent nerves. The lesion grows
spherical in shape buccally and lingually causing bulging
of lower border of the mandible. Cortical erosion is rare.
The lesion is well-defined and unilocular with some cases
showing sclerotic border. Resorption of root is common
features of this disease (Fig. 23.22).
Figure 23.22 Well-defined and unilocular lesion of ossifying Large lesion shows downward bowing of the inferior
fibroma (Courtesy: Dr Aparna Thombre, Reader, Department of cortex of the mandible.
Maharashtra, India) Histopathological Features
Ossifying fibroma (Figs 23.23 to 23.25): Large number of
Site: It shows a predilection for premolar-molar area fibroblasts, with flat elongated nuclei is present within the
of the mandible. Mostly in facial bones in mandible and network of interlacing collagen fibers.
in maxilla, it is often located in the canine fossa and Chinese letter shaped islands of bone or calcification
zygomatic arch. In some cases, there may be involvement distributed throughout the connective tissue. As the lesions
of the anterior base of skull and of temporal bone. mature, the islands of ossification increase in number
enlarge and ultimately coalesce.
Facial asymmetry: Occasional facial asymmetry is seen
in some of the cases. Cementifying fibroma (Fig. 23.26): Cementifying
fibroma is composed of cellular fibrous connective tissue
Symptoms: When in maxilla, symptoms may include
that contains cementum like spherules.
nasal stuffiness and epiphora on the affected side. There
It is composed of many delicate interlacing collagen
may be associated exophthalmos, with visual disturbances,
fibers arranged in discrete bundles, interspersed by large
depending on the extent of compression of its orbital
number of active, proliferating fibroblasts or cemen-
content by the tumor.
toblasts.
The lesion is slow growing and in some cases, there is
The connective tissue characteristically presents many
displacement of teeth. Bony cortex and covering mucosa
small foci of basophilic masses of cementum like tissue.
remain intact. The lesion may be slow growing initially,
These are irregularly round, ovoid or slightly elongated, often
with a rapid increase in size in a relatively short time.
lobulated. As the lesion matures, the islands of cementum
If sinus is affected it may fill the sinus completely and
increase in number enlarge and ultimately coalesce.
expands the sinus wall.
Management
Cementifying Fibroma
Enucleation: Small, clinical encapsulated lesion are
Age and sex distribution: It may occur at any age, but it is
treated by conservatively enucleation.
more common in the young and middle aged adults with a
mean age of 35 years. Female are affected more commonly Resection: It is recommended if the there is involvement
than males by ratio of 2:1. of inferior border, extension into maxillary sinus occurs.
590
Figure 23.23 Ossifying fibroma showing Chinese shaped Figure 23.24 Ossifying fibroma (Courtesy: Dr Sangamesh
island of bone Halawar, Reader, Department of Oral Pathology, VPDC and H,
Kavalapur, Sangli, Maharashtra, India)
Figure 23.25 Ossifying fibroma (Courtesy: Dr Aparna Thombre, Reader, Department of Oral
Pathology, VSPM Dental College and Hospital, Nagpur, Maharashtra, India)
Points to Remember
Young adult, premolar-molar area of the mandible, facial
asymmetry, nasal stuffiness, epiphora, slow growing,
displacement of teeth, pain, paresthesia, well defined,
unilocular, downward bowing of the inferior cortex of
the mandible, fibroblast, elongated nuclei, Chinese letter
shaped islands of bone, cellular fibrous connective tissue,
cementoblasts, proliferating fibroblasts, cementum like
spherules.
JUVENILE OSSIFYING FIBROMA

It is also called juvenile active ossifying fibroma, ‘juvenile
Figure 23.26 Cementifying fibroma (Courtesy: Dr Sangamesh aggressive ossifying fibroma. It is differentiated from
Halawar, Reader, Department of Oral Pathology, VPDC and H, ossifying fibroma on the basis of age, site and clinical
Kavalapur, Sangli, Maharashtra, India) behavior.
Types
• Trabecular
• Psammomatoid. 591
Clinical Features
Age and sex distribution: It occur in patient younger
than 6 months to older than 70 years. There is slight male
predilection.
Trabecular form: It is seen in younger patient with mean
age 11 years.
Psammomatoid form: Mean age of occurrence is 22
years.
Sign: Cortical expansion may results in facial enlargement. Figure 23.27 Juvenile ossifying fibroma with cellular fibrous
tissue with ossicle
Lesion in paranasal sinus: Lesion in sinus may results
in penetration of orbital, nasal and cranial cavities. It
can results nasal obstruction, exophthalmos or proptosis. Points to Remember
Rarely temporary and permanent blindness occur.
Juvenile active ossifying fibroma, lesion in paranasal
Intracranial extension: Lesion derived from cribriform sinus, intracranial extension, well circumscribed
plate may extent intracranialy. Rarely patient may suffer radiolucency, ground glass appearance, clouding of
from meningitis. sinus, cellular fibrous connective tissue, myxomatous
foci, trabecular pattern, psammomatoid pattern, complete
Radiological Features local excision.
The lesions are well circumscribed radiolucency which
may contain central radiopacities.
OSTEOPOROSIS
In some cases ground glass appearance can be seen. If
it involve sinus there is clouding of sinus can occur. There is reduction in the inorganic constituent. There
is abnormal persistence of calcified cartilage. Spongy
Histopathological Features portion of affected bone ultimately becomes a solid
Tumors consist of cellular fibrous connective tissue. block of calcified cartilage leaving inadequate space for
Cytoplasm of individual cells is hard to discern. Myxomatous hemopoiesis. It is characterized by low bone mass and
foci are associated with pseudocystic degeneration. micro-architectural bone fragility. It is usually rarefaction
Areas of hemorrhage and small cluster of multinucleated of bone resulting from deficiency of bone matrix rather
giant cells are seen. than deficit mineral (Fig. 23.28).
Trabecular pattern: It shows irregular strands of highly Types
cellular osteoid encasing plump and irregular osteocytes.
Primary (associated with aging).
These are lined by plump osteoblasts and multinucleated
Secondary (reduction of histologically normal bone that
osteoclasts.
has been accelerate by abnormal or iatrogenic circumstance
Psammomatoid pattern: Concentric lamellated and such as corticosteroid or heparin therapy or condition such
spherical ossicle that have basophilic centers with as malnutrition and scurvy.
peripheral eosinophils osteoid rims (Fig. 23.27).
Mechanism of Bone Loss
Management It is caused by imbalance between bone resorption and bone
For smaller lesion complete local excision with curettage formation with an exaggeration of resorption, reduction in
is sufficient. In large lesion wider resection can be done. bone formation or combination of both.
Contraceptive drug can also cause osteoporosis; as after

administration of contraceptive drug, there is increase in
level of cortisol. Combination of these two compounds
592 leads to production of abnormal megakaryocytes, which in
turn leads to formation of abnormal sticky platelets. These
sticky platelets fuse to form thrombi which occlude small
vessels in the tissue due to which the bone dies and get
resorbed.
Malnutrition state: Sufficient protein must be absorbed
from intestine to supply constant need for matrix formation.
Deficiency causes osteoporosis and may result from protein
poor diet or from GIT disturbances such colitis, regional
arteritis. Vitamin C deficiency may weaken sinusoidal
vessel walls in medullary bone which tend to dilate and
rupture, resulting in pooling of blood and hypoxia, which
leads to loss of vitality and removal of bone by physiologic
osteoclast.
Figure 23.28 Left side showing normal bone matrix and right
side showing osteoporotic bone Progesterone: There is interaction between the
progesterone compounds and increased level of cortisol
by stress, leading to formation of multiple small thrombi.
Postmenopausal and senile: It occurs due to overall The thrombi occlude small vessels of osseous tissue,
decrease in anabolic hormones (estrogen) in postmenopausal which results in death and resorption of bone, leading to
women. There may be lag in formation of bone and since osteoporosis.
bone resorption is continued, it results in osteoporosis.
After the age of 60 years, generalized atrophy of bone Thyrotoxic osteoporosis: It is usually seen in children
occur (senile osteoporosis). Senile osteoporosis is caused with hyperthyroidism. Thyroxin mediates the action of
by decrease in calcium absorption, vitamin D absorption cortisol on bone and an excess of thyroxin results in a more
and metabolism with age, which in turn decreases the efficient utilization of steroids. Thus, there is increased
anabolic hormones, muscular proteins and flow of blood to resorption.
bone. By virtue of this condition, bone resorption occurs.
Another cause of bone resorption in adults is that, in elderly
Clinical Features
person altered hormonal function lead to formation of small It is either present at birth (congenital) or developed in
thrombi which plug small vessels in bone and cause loss of early life (infantile). Bones are fragile and susceptible
bone vitality and hence resorption. to fracture. These fractures typically affect the forearm
(Colle’s fracture), spine (vertebral fracture) and hip (femur
Cushing’s syndrome: The symptoms are caused by
fracture).
an increased output of glucocorticosteroids, especially
Congenita form inherited as an autosomal recessive
cortisol. The excess cortisol acts to produce osteoporosis
disorder is invariably fatal in early life due to massive
by two ways:
hemorrhage, anemia and rampant bone infections occurring
1. It contributes to the degradation of proteins and severely
due to progressive loss of bone marrow and their cellular
limits the formation of bone matrix by reducing the
products. Percussion over the affected vertebrae is painful.
amount that each osteoblast synthesizes.
Symptoms: Osteoporotic patient may notice gradual
2. It promotes the formation of osteoclasts from the
loss of height due to shortening of trunk. In advanced cases
osteogenic undifferentiated cells and thus enhances
clinical onset is often characterized by attack of severe
bone resorption.
pain which is aggravated by movements and occurs after
Drug induced osteoporosis: Prolonged administration of trauma.
cortisol and cortisone show Cushing like syndrome and cause Osteomyelitis may occur due to relatively avascular
osteoporosis in the same way as that in Cushing syndrome. and there may be bone pain.
Oral Manifestations
There is marked predilection for development of
osteomyelitis. Patient can also suffer from fracture of jaws 593
during tooth extraction.
Other features include enamel hypoplasia, arrested
root development, retardation of tooth eruption, early loss
of teeth, missing teeth and malformed teeth. Teeth are
poorly calcified and are prone to caries.
Radiographs may show osteopenia. This finding indicates
that at least 30 percent of the bone mass has been loss (Fig.
23.29).
Laboratory Findings Figure 23.30 Osteoporotic bone showing atrophic trabeculae

Patients manifest anemia due to replacement of hemolytic
marrow by bone. Hepatomegaly may be present. Bisphosphonates are the primary drugs used to both
Red blood cell (RBC) count below 1,000,000 per cubic prevent and treat osteoporosis in postmenopausal women.
meter. Increased serum phosphatase levels. Calcitonin is a medicine that slows the rate of bone
loss and relieves bone pain. It comes as a nasal spray or
Histopathological Features injection.
There is endosteal production of bone with an apparent Hormone replacement therapy estrogens are rarely
concomitant lack of physiologic bone resorption. used anymore to prevent osteoporosis and are not approved
Osteoblasts are prominent but osteoclasts are seldom to treat a woman who has already been diagnosed with the
found. condition.
Trabeculae are in disorder arrangement and atrophic
(Fig. 23.30). Marrow tissue is fibrous. Bone is markedly Points to Remember
deficient in collagen matrix. Postmenopausal and senile, Cushing’s syndrome, drug
induced osteoporosis, malnutrition state, progesterone,
Management thyrotoxic osteoporosis, Colle’s fracture, vertebral fracture,
The goals of osteoporosis treatment are to control pain gradual loss of height, osteomyelitis, enamel hypoplasia,
from the disease, slow down or stop bone loss and prevent arrested root development, retardation of tooth eruption,
bone fractures with medicines that strengthen bone. osteopenia, anemia, increased serum phosphatase levels,
osteoblasts, fibrous marrow tissue, bisphosphonates,
calcitonin, hormone replacement therapy.
INFANTILE CORTICAL HYPEROSTOSIS

It is also called Caffey’s disease, Caffey-Silverman syndrome.
It was first described in detail in 1945. It is characterized by
unusual cortical thickening of certain bones and it occurs in
two forms, i.e. autosomal dominant form and sporadic form.
It can represent as autosomal dominant trait.
Etiology
It may be an embryonic osteodysgenesis consequent to
Figure 23.29 Osteopenia in patient with osteoporosis local defect in blood supply to the area.
Inherited defects of arterioles supplying the affected Histopathological Features

part results in hypoxia producing focal necrosis of overlying
In the early stages of infantile cortical hyperostosis shows
soft tissue and periosteal proliferation.
inflammation of the periosteum and adjacent soft tissues.
594 Allergy can be basis of disease, edema and infla-
After infection resolves, the periosteum remains
mmation producing periosteal elevation and subsequent
thickened, and subperiosteal immature lamellar bone can
deposition of calcium.
be seen on biopsy, while the bone marrow spaces contain
Clinical Features vascular fibrous tissue.
Eventually, the inflammation and subperiosteal changes
Age and sex distribution: It is equal in males and females, resolve, and hyperplasia of lamellar cortical bone can be
with average onset at 9 weeks of age, with most of the cases seen.
arising before 6 months.
Sites: Bones commonly affected are mandible, clavicle, Management
scapula, frontal bone and ulna. There is bilateral No specific treatment exists for infantile cortical
involvement in every case. Of all these bone clavicle and hyperostosis. The disease is self-limited and usually
mandible are the most common sites. resolves without sequelae.
Symptoms: Infants will develop fever and become hyper-
Points to Remember
irritable.
Soft tissue swellings have a sudden onset, especially in Caffey’s disease, inherited defects, allergy, infants fever,
the facial area and early in disease, they may be warm and hyperirritable, soft tissue swellings, pseudo-paralysis,
tender. Swelling is devoid of clinical signs of inflammation pleurisy, dysphasia, mandible, malocclusion, enamel
on the overlying skin. Swelling disappears slowly, but hypoplasia, periosteal new bone, anemia, leukocytosis,
may suddenly recur at the same place or new site may be elevated erythrocyte sedimentation rate, inflammation of
involved. the periosteum, subperiosteal immature lamellar bone,
Other features are pseudo-paralysis, pleurisy and hyperplasia of lamellar cortical bone.
dysphasia.
Oral Manifestations OSTEOPETROSIS

Mandible is one of the most common bones affected in It is also called Marble bone disease, Albers-Schonberg
infantile cortical hyperostosis. disease, osteosclerosis fragilis generalisata.
Patient may have malocclusion and enamel hypoplasia It is a rare disorder characterized by an increase in
may be present. density of bones, resulting from a defect in remodeling
caused by failure of normal osteoclast function in which
Radiological Manifestation becomes hard and brittle.
Radiographs initially show layers of periosteal new bone
formation with cortical thickening. Types
Periosteal new bone may cover the diaphysis of the • Infantile: Severe disease that is termed as malignant
bone, causing an increase in diameter of the bone. Over osteopetrosis.
time, the periosteal new bone density increases, becoming • Intermediate osteopetrosis: This is less severe variant
homogenous with the underlying cortex. Eventually the of malignant osteopetrosis.
bone remodels and resumes a normal appearance. • Transient osteopetrosis: There is radiographic
evidence of diffuse sclerosis and associated marrow
Laboratory Findings failure but resolve without specific therapy.
Anemia, leukocytosis, elevated erythrocyte sedimentation • Adult osteopetrosis: It is discovered later in life and
rate, monocytosis and increased serum alkaline phos- is termed as benign osteopetrosis.
phatase levels.
Clinical Features Laboratory Findings

Malignant Osteopetrosis Red blood cell count below 1,000,000 cells per cu mm.
It is developed as autosomal dominant trait. Almost The serum calcium and phosphate levels are usually 595
everyone has a varying degree of poor skeletal development, within normal limits. Elevated serum acid phosphate
if the disease appears early in life. levels have been reported in patients with benign dominant
Subsequent skeletal deformities occur later due to osteopetrosis.
bending of the long bones and improper healing after Histopathological Features
pathological fractures.
As a result of continuous bone deposition and lack It is characterized by the endosteal production of bone,
of bone resorption, the foramina of cranial nerves are with an apparent concomitant lack of physiologic bone
constricted; hence there is loss of hearing, disturbed vision, resorption.
which diminishes progressively. Facial nerve palsy is also Osteoblasts are prominent, but osteoclasts are seldom
seen. found. The predominance of bone formation over resorption
Due to displacement of hematopoietic bone marrow typically leads to the persistence of cartilage cores of bony
normocytic anemia ensues and the hematopoietic function trabeculae, long after their replacement should occurred in
is assumed by the liver, spleen and the lymphnodes resulting endochondral bones.
in hyperplasia of lymphoid tissues and hepatosplenomegaly. The trabeculae are disorderly in arrangement and the
There is also frontal bossing, obliteration of maxillary marrow tissue present is usually fibrous.
sinus and possible hydrocephalus and mental retardation. There is globular amorphous bone deposition in marrow
space.
Benign Osteopetrosis
Management
It discovered later and have less severe manifestation. It is
inhibited as autosomal dominant trait. There is as such no effective treatment for osteopetrosis.
There is significant sclerosis in the axial skeleton as Prognosis of adults form is good and survival is long as
compare to long bone. compared to infantile form where patient dye during first
Forty percent of patients are asymptomatic and marrow decade of life.
failure is rare. Bone marrow transplantation can give good results.
Cranial nerve compression and fracture of bone can Therapy like interferon gamma-Ib in combination with
occur. calcitriol shown to reduce bone mass.
Other treatment modalities like administration of
Oral Manifestations corticosteroid, parathormone, macrophage colony stimu-
Osteomyelitis of jaws associated with osteopetrosis lating factors and erythropoietin has also been suggested.
probably follows the obliteration and fibrosis of marrow is Performing dental extraction has a risk of osteomyelitis
caused by reduced osseous circulation. and jaw fracture.
It is most common in mandible and occasionally, in
Points to Remember
maxilla. Paranasal sinus may become readily obliterated.
There may be enamel hypoplasia, defective dentine, Marble bone disease, bending of the long bones, patho-
disturbed tooth development, small pulp and tendency logical fractures, constricted foramina of cranial nerves,
towards caries. loss of hearing, facial nerve palsy, normocytic anemia,
hyperplasia of lymphoid tissues, hepatosplenomegaly,
Radiological Features frontal bossing, osteomyelitis of jaws, paranasal sinus
There is widespread increase in skull density distinction obliterated, enamel hypoplasia, small pulp, increase skull
between cortical and cancellous bone is lost. density, red blood cell below 1,000,000, osteoblasts,
Roots of teeth are difficult to visualize due to density of fibrous marrow tissue, bone marrow transplantation,
surrounding bone. calcitriol, interferon gamma-Ib.
OSTEOGENESIS IMPERFECTA
It is also called brittle bone, Lobstein disease. It is a serious
596 disease of unknown etiology. It represents a hereditary
autosomal dominant trait.
Types
Type 1 Osteogenesis imperfecta
Type 4 Osteogenesis imperfecta.
Clinical Features
Many infants with this disease are stillborn or die shortly
after birth.
There is extreme fragility and porosity of bones with
an attendant proneness of fracture. Fracture heals readily
Figure 23.31 Patient with osteogenesis imperfecta showing of
but new bone is of similar imperfection. It is common for
bowing of leg
fracture to occur while the infant is walking or crawling.
There is formation of hyperplastic callus, which may
mimic osteosarcoma, take place. pale blue in early childhood, but the blue color fades later
I life (Fig. 23.31).
Blue sclera: There is occurrence of pale blue sclera which
is thin; pigmented choroids show through and produce the Oral Manifestations
blue color.
There is deafness due to osteosclerosis; laxity of Most of the times, osteogenesis imperfecta is associated
ligament and peculiar shape of the skull. There is also with dentinogenesis imperfecta.
abnormal electrical reaction of muscle, increased tendency Sometimes, there is also hypoplasia of teeth, class I
for capillary bleeding. malocclusion and greater incidence of impacted 1st and
2nd molars. Deciduous teeth are poorly calcified and semi-
Type 1 Osteogenesis imperfecta: It is most common and translucent or waxy.
mildest form. It is inherited as autosomal dominant trait Appearance of teeth is faint dirty pink, half normal
patients have mild-to-moderate bone fragility. The sclera size, with globular crowns and relatively short roots in
is blue in all ages and these aids in diagnosis. proportion to other dimension.
Type 2 Osteogenesis imperfecta: This type is severe
form. It has extreme bone fragility and frequent fractures.
It is inherited both as autosomal dominant and recessive The radiographic features of osteogenesis imperfect
traits. Many patients are stillborn and 90 percent die before include osteopenia, bowing, angulation or deformity of
4 weeks of age. long bones, multiple fractures and wormian bones in skull.
Type 3 Osteogenesis imperfecta: This type has moderate- Histopathological Features

to-severe bone fragility. It can be inherited both autosomal It exhibits thin cortices, sometimes being composed of
dominant and recessive patterns. The sclera is normal or immature spongy bone, while the trabeculae of cancellous
blue, if discoloration is present then it fades as the child bone are delicate and often show micro-fractures.
grows. Osteoblastic activity appears retarded and imperfect
Type 4 Osteogenesis imperfecta: This type is associated and for this reason thickness of long bones is deficient.
with mild to moderately severe bone fragility. This variant There is failure of fetal collagen to be transformed into
appears as an autosomal dominant trait. The sclerae may be mature collagen.
Progressive intermolecular cross-linkage of adjacent

collagen molecule is only sometimes evident, which a
characteristic of normal collagen maturation.
597
Management
Management consist physiotherapy, rehabilitation, and
orthopedic surgery.
Use of bisphosphonates can provides relief from pain,
reduces risk of fracture and improved mobility.
Points to Remember
Brittle bone, extreme fragility, porosity of bones, fracture
infant while walking, blue sclera, deafness, hypoplasia,
impacted, faint dirty pink teeth, osteopenia bowing,
angulation, multiple fractures, wormian bones, immature Figure 23.32 Bird facies seen in Pierre Robin syndrome
spongy bone, trabeculae show micro-fractures, cross-
linkage, collagen molecule, physiotherapy, rehabilitation,
bisphosphonates. the characteristic bird facies (Fig. 23.32). Mandibular
hypoplasia yields a retrognathic appearance.
PIERRE ROBIN SYNDROME Cleft palate: There is abnormal descent of tongue between
the palatal shelves resulting in cleft palate.
It is also called Robin anomalad Pierre Robin sequence.
It result arrested development. It is discovered in 1923 by Breathing difficulty: There is respiratory difficulty
physician name Pierre Robin. due to failure of support of tongue musculature because
of micrognathia, allowing the tongue to fall down and
Pathogenesis backwards, partially obstructing the epiglottis.
Mechanical theory: This theory is the most accepted. The There may be congenital heart defects, other skeletal
initial event, mandibular hypoplasia, occurs between the abnormalities and ocular lesions. Mental retardation is also
7th and 11th week of gestation, which keeps the tongue a common finding.
high in the oral cavity, causing a cleft in the palate by
preventing the closure of the palatal shelves. This theory Management
explains the classic inverted U-shaped cleft and the absence Breathing support and feeding assistance are necessary
of an associated cleft lip. during infancy.
Surgical closure of cleft palate and orthodontic
Neurological maturation theory: A delay in neuro-
treatment are indicated.
logical maturation has been noted on electromyography
of the tongue musculature, the pharyngeal pillars, and the Points to Remember
palate, as has a delay in hypoglossal nerve conduction. The
Pierre Robin sequence, mechanical theory, neurological
spontaneous correction of the majority of cases with age
maturation theory, triad mandibular hypoplasia, cleft
supports this theory.
palate, glossoptosis.
Clinical Features
Triad: It consists of cleft palate, micrognathia and MARFAN’S SYNDROME
glossoptosis.
It is also called Marfan-Achard syndrome, arachno dactyly.
Mandibular hypoplasia: Arrested development and It is hereditary disease transmitted as autosomal dominant
ensuing hypoplasia of mandible ultimately produce trait. It is basically a disease of connective tissue related
to defective organization of collagen which is abnormally

Points to Remember
soluble.
Arachnodactyly, long thin extremities, spider finger, long
598 Clinical Features and narrow face, bilateral ectopia lentis, cardiovascular
complications, high arched palatal vault, multiple
There is excessive length of the tubular bones resulting in
odontogenic cysts of maxilla, bifid uvula.
disproportionately long thin extremities.
The finger and toes are long, thin and tapering so that
the name spider finger has been applied (Fig. 23.33). DOWN’S SYNDROME
The shape of face and skull is characteristically long
It is also called Trisomy 21 syndrome and mongolism. It is
and narrow.
associated with subnormal mentality in which an extremely
There is hyperextensibility of joint with habitual
wide variety of anomalies and functional disorders may
dislocations, kyphosis or scoliosis and flatfoot.
occur. It results from excessive chromosomal material
Bilateral ectopia lentis: It caused by weakening or
involving all or a portion of chromosome 21.
rupture of the suspensory ligaments.
Cardiovascular complications like aortic aneurysm and Types
aortic regurgitation, valvular defects and enlargement of
the heart are common. • Typical type – trisomy–21 with 47 chromosomes (95
percent of cases).
Oral Manifestations • Translocation type – 46 chromosomes.
• Chromosomal mosaicism.
High arched palatal vault is very prevalent. Bifid uvula,
malocclusion and multiple odontogenic cysts of maxilla
and mandible can also occur. Clinical Features (Fig. 23.34)
There may be temporomandibular dysarthrosis. In some Facial characteristic: There is midface dysplasia, nose
cases of Marfan calcification in the pulp can also occur. malformations including a flat broad bridge of the nose is
present. Ear malformations, including “lop” ears, low-set
Management ears and ears with a flat or absent helix have been reported.
People with Marfan syndrome should take antibiotics before Eye malformations like epicanthal folds with slanting
dental procedures to prevent endocarditis. Pregnant women almond-shaped eyes (narrow palpebral tissue slanting
with Marfan syndrome must be monitored very closely toward the midline), which was responsible for the term
because of the increased stress on the heart and aorta. mongoloid, are also reported. It exhibit brachycephaly
Figure 23.33 Radiograph of hand of patient with Figure 23.34 Clinical features of Down syndrome
Marfan syndrome
(broad, short head) and lack of supraorbital ridges and Other problems: The incidence of macroglossia, fissured
hypotelerism (secondary to hypoplasia of the central face) tongue and protruding tongue due to forward position of
are common findings. the mandible and open mouth is a common finding. Also,
increases in bifid uvula and submucous clefts and cleft 599
Congenital cardiopathies: Incidence of mitral valve
palates have been reported in this population.
prolapse, aortic regurgitation is reported.
Upper respiratory tract infections: The higher incidence Management
of upper respiratory tract. Infections seen in persons with Down syndrome is a permanent genetic disability.
Down Syndrome is thought to be due to their impaired Management consist of pursuing mega-vitamin therapy in
immunologic response to infectious or inflammatory addressing the lowered immune response to infection seen
diseases. in persons with Down Syndrome.
Alzheimer dementia: Neurological signs of Alzheimer Cosmetic facial surgery: These procedures would
disorder, as evidenced by post-mortem findings. include augmentation of the nasal, cheek and chin areas,
Atlantoaxial instability: The incidence of atlanto-axial glossectomies and lateral canthoplasty.
instability in persons with Down syndrome has been
Points to Remember
reported as ten to twenty percent. This condition refers to
an abnormal increase in mobility of the upper two cervical Trisomy 21 syndrome, midface dysplasia, lop ears, al-
vertebra (Cl/C2) due to congenital ligamentous laxity. mond-shaped eyes, brachycephaly, hypotelerism, con-
genital cardiopathies, upper respiratory tract infections,
Other problems: Patient complaint of delayed speech Alzheimer dementia, atlanto-axial instability, periodontal
and a husky quality of voice, hearing impairments, risk for disease, NUG, malocclusion, delayed eruption.
cataracts.
Oral Manifestation ACHONDROPLASIA

Periodontal disease: Inadequate oral hygiene may It is also called chondrodystrophia fetalis. It is a distur-
lead to early onset fulminating periodontal disease in bance of endochondral bone formation, which results in a
these individuals. It is common to see alveolar bone loss characteristic form of dwarfism. It is a hereditary condi-
in persons with DS at age 6 to 16 years. This increased tion, which is transmitted as an autosomal dominant trait.
incidence of periodontal disease is directly related to Clinical Features
the reduced immunologic response to infections and
inflammatory disease reported in these individuals. Signs: Patient is quite short, usually less than 14 meters in
height and thickened muscular extremities, brachycephalic
NUG: Several reports have indicated a high incidence of skull and bowed legs.
necrotizing ulcerative gingivitis (NUG) in these individuals. Hands are usually small and fingers are stubby. The
Malocclusion: There is an increased incidence of base of the skull is small and constricted as a result of
malocclusion in individuals with DS, particularly Class retarded growth of the cartilaginous portions.
III malocclusions. The higher incidence of Class III The calvarium is large and bulges frontally and
malocclusions is due to the underdevelopment of the laterally. There is also depression of the bridge of nose.
midface (nasal, premaxillary and maxillary bones) not to Lumbar lordosis with prominent buttocks and
prognathism. protruding abdomen is often present. Joints exhibit
limitation of motion. Arms do not hang freely at the sides
Delayed eruption: The teeth of people with Down and the elbows cannot be straightened.
syndrome, both baby teeth and permanent teeth, may come
in late compared to children without Down syndrome. Oral Manifestations
Small and missing teeth: Frequently, people with Down Maxilla is often retruded because of restriction of growth
syndrome have smaller than average teeth, and missing at the base of skull which may produce relative mandibular
teeth. proganthism.
Congenital missing teeth with disturbances in shape of Radiographic features: Seen most commonly in
teeth may also occur. mandibular first molar area. There is presence of radiopaque
sclerotic bone. It is well-defined, rounded elliptic mass.
Retardation or even aplasia of zone of provisional Histopathological Features
calcification of endochondral growth occurs. It consists of thickened trabeculae and concomitant
Cartilage columns lack orderly arrangement, fail to decrease in the size and number of marrow spaces,
calcify properly and are not resorbed and replaced by bone vascularity and number of lacunae.
in the usual fashion.
Chondrocyte development is defective; the orderly Management
longitudinal growth of bone is disrupted, resulting in
stunting of bones. No treatment is required for the disease.
Management MASSIVE OSTEOLYSIS

There is no specific treatment but it is desirable to It is also called vanishing bone, disappearing bone, phantom
monitor the progress of affected children to offer support bone, progressive osteolysis or Gorham syndrome. It is
and to detect early the occurrence of any complications. characterized by spontaneous, progressive resorption of
Occupational therapists can be helpful in advising on such bone with ultimate total disappearance of bone. Destroyed
issues. bone is replaced by vascular proliferation.
Points to Remember
Cause
Chondrodystrophia fetalis, stubby fingers, large
calvarium, lumbar lordosis, retruded maxilla, missing Cause is unknown. Initially, it is thought that hyperactivity
teeth, provisional calcification of endochondral growth, of osteoclast may lead to destruction of the bone. Some
defective chondrocyte. author believed that massive osteolysis may relate to
proliferation of blood and lymphatic vessels which can
become multicentric. It has been termed as angiomatosis
OSTEOSCLEROSIS of bone.
There is a focal area of increase radiodensity due to
unknown cause. So it is called idiopathic osteosclerosis, Clinical Features
dense bone island, bone ebnuration, bone whorl, bone Age and sex distribution: It is most common in older
scar, focal periapical osteosclerosis, enostosis. children and young and middle age adults, affecting both
They are localized growth of compact bone that extends sexes equally.
from the inner surface of cortical bone into the cancellous
Sites: The most commonly affected bones are the clavicle,
bone.
scapula, humerus, ribs, ilium, ischium and sacrum.
Clinical Features Symptoms: The disease may or may not be painful, begins
Age: Patient is usually over the age of 12 years. suddenly and advance rapidly, until the involved bone is
replaced by a thin layer of fibrous tissue surrounding a
Location: It is asymptomatic and more common in
cavity.
mandible than maxilla and commonly found in premolar
molar area. Oral Manifestations
Sign: It is a mass of compact bone within the spongiosis, The patients may present with pain or facial asymmetry,
which does not cause expansion. or both and there is pathologic fracture of bone following
A rare condition in which there are thousands of dense even minor trauma.
islands of bone scattered through the skeleton is known as There may be complete destruction of mandible;
osteopoikile or osteopoikilosis. maxilla is less commonly affected.
Radiological Features GARDNER’S SYNDROME

It consist intramedullary radiolucent foci varying size with Gardner’s syndrome is an autosomal dominantly inherited
irregular and diffuse margin. After some period large area disorder with variable penetrance, characterized by 601
bone disappears. There is loss of lamina dura and thinning multiple supernumerary teeth, multiple osteomas of bony
of cortical plates occurs. skeleton, intestinal polyposis, and various skin and soft
Histopathological Features tissue tumors. Multiple sebaceous cysts are also associated.
Manifestation of Gardner’s syndrome is thought to be
There is replacement of bone by connective tissue due to abnormal growth of all three primordial germ layers.
containing many thin walled blood vessels or anastomosing
vascular spaces lined by endothelial cells (Fig. 23.35). Clinical Features
There chronic inflammatory infiltrate of lymphocytes Multiple polyps of the colon appear in the second decade
and plasma cells. of life. Although asymptomatic, malignant changes or risk
of colonic cancer is almost 100 percent by the age of fifty.
Management
Skeletal abnormalities are localized cortical skeletal
There is no specific treatment; although surgical resection thickening of long tubular bones and osteomas of the
ceases the progress of the disease. mandibular ramie are characteristic of this syndrome.
In some cases estrogen, magnesium, calcium, Osteomas are noted during puberty.
vitamin D, fluoride, calcitonin, alpha-2b interferon and In some cases increase incidence of thyroid carcinoma
chemotherapeutic agents can also be given. is also seen.
Radiation therapy is most successful and widely
accepted mode of therapy. Oral Manifestation
In some cases zoledronic acid can also be given. Large osteoma in condylar region may limit the mandibular
opening.
Points to Remember Dental anomalies noted are supernumerary teeth,
Vanishing bone, angiomatosis of bone, involved bone odontomas, multiple unerupted teeth and multiple caries.
is replaced by a thin layer of fibrous tissue, facial Sebaceous or epidermoid cyst appear later in life over
asymmetry, complete destruction of mandible; maxilla, the face, scalp and extremities.
loss of lamina dura, intramedullary radiolucent foci, Lipomas are frequently noted in the subcutaneous
many thin walled blood vessels or anastomosing vascular tissue. Fibromas are also reported.
spaces, zoledronic acid, estrogen, magnesium, calcium,
vitamin D, fluoride, calcitonin, alpha-2b interferon. Radiographic Features
Osteoma appears as areas of increase density which varies
in size.
Osteomas are generally of compact type.
Management
Prophylactic colectomy is indicated in Gardner syndrome
as it has got high incidence of malignant transformation
Removal of osteomas of jaw and epidermoid cyst can be
carried out for esthetic reason.
Points to Remember
Multiple polyps, osteoma, supernumerary teeth, odonto-
mas, sebaceous or epidermoid cyst, lipomas, prophylac-
Figure 23.35 Vascular channels seen in massive osteolysis tic colectomy.
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sequence: appearances and 25 years of experience with maxillofacial pathology, 3rd edn. Saunder Elsevier; 2009.
an innovative treatment protocol. J Pediatric Surg. 28. Oreland A, et al. Malocclusions in physically and/or mentally
2009;44(11):2112-8. handicapped children Swed Dent Jour. 1987;11(3):103-19.
11. Carrillo R, Morales A, Rodriguez-Peralto JL, et al. Benign 29. Otomo-Corgel J. Osteoporosis and osteopenia: implications
fibroosseous lesion in paget disease of the jaws. Oral Surg for periodontal and implant therapy, Periodontol 2000.
Oral Pathol. 1991;72:588-592. 2012;59(1):111-39.
12. Cheng YSL, Wright JM, Walstad WR, et al. Osteosarcoma 30. O’Connel AC, Marini JC. Evaluation of oral problems in an
arising in Paget disease of bone. Oral Oncol. 2002;38:785- osteogenesis imperfecta population. Oral Surg Oral Pathol
Oral Radiol Endod. 1995;887:189-96.
92.
31. Penarrocha M, Bonet J, Minguez M. Cherubism: A clinical,
13. Cohen MM, et al. Dental and Facial Characteristics in
radiographic, and histopathological comparison of 7 cases:
Down’s syndrome. J Dent Res. 1965;44(suppl):197-208.
J Oral Maxillofac Surg. 2006;64:924-30.
14. DeLange J, van den Akker HP. Clinical and radiological 32. Rauch F, Glorieux FH. Osteogenesis imperfecta. Lancet.
features of central giant cell lesion of the jaw. Oral Surg 2004;363:1377-85.
Oral Pathol Oral Radiol Endod. 2005;99:464-70. 33. Ricalde P, Ord RA, Sun CCJ. Vanishing bone disease in
15. El MOfty S. Psammomatoid and trabeculae juvenile five year old: report of case and review of literature. Int J
ossifying fibroma of the craniofacial skeleton two distinct Oral Maxillofac Surg. 2003;32:222-6.
entities. Oral Surg Oral Pathol Oral Radiol Endod: 34. Rossbch HC, Letson D, Lacson, et al. Familial gigantiform
2002;93:296-304. cementoma with brittle bone disease, pathologic fracture
and osteosarcoma: a possible explanation of ancient 38. Waldoron CA. Fibro-osseous lesion of the jaw: J Oral
mystery: pediatric blood cancer. 2005;44:390-6. Maxillofac Surg. 1993;51:828-35.
35. Smith J, Eveson J. Paget disases of bone with particular 39. Williams HK, Mangham C: SpeIght PM. Juvenile ossifying
reference to dentistry. J Oral Pathol. 1981;10:233-47. fibroma: an analysis of eight cases and comparison with 603
36. Starropoulos F, Katz J. Central giant cell granulomas: A other fibro-osseous lesion: J Oral Pathol Med. 2000;29:
system review of the radiographic characreristic with addition 13-8.
of 20 new cases. Dentomaxilllofac Radiol. 2002;31:213-7. 40. Yonetsu K, Yuasa K, Kanda S. Idiopathic osteosclerosis of
37. Von Wowern N. Cherubism: A 36 years long term follow the jaw; panoramic radiographic and computed tomographic
up 2 generation in different families and review of literature. finding. Oral Surg Oral Med Oral Pathol Oral Radiol Endod.
Oral Surg Oral Pathol Oral Radiol Endod. 1981;10:233-47; 1997;83:517-21.
2000;90;765-72.
1. In monostotic fibrous dysplasia there is an involvement 6. ‘Jigsaw puzzle’ or ‘mosaic pattern’ of bone seen in:
of: a. Cherubism
a. Multiple bone b. Paget’s disease
b. Multiple bone with pigmentation c. Fibrous osseous dysplasia
c. Single bone d. Achondroplasia
d. Single bone and endocrinal disturbance 7. ‘Marble bone disease’ refers to:
2. Multiple bone involvement accompanied by pigmented a. Osteopetrosis
lesion plus endocrine disturbance seen in: b. Osteoporosis
a. Albright’s syndrome b. Down’s syndrome c. Albers-Schonberg disease
d. Both a and c
c. Cherubism d. Marble bone disease
8. Extreme fragile bones, pale blue sclera is the clinical
3. C–shaped or Chinese character shaped trabeculae of
feature seen in:
bone seen in:
a. Osteogenesis imperfecta
a. Polystotic fibrous dysplasia b. Lobstein disease
b. Monostotic fibrous dysplasia c. Robin anomalad
c. Both d. Both a and b
d. None
9. ‘Spider finger’ is a clinical feature of:
4. ‘Eye raised to heaven’ look is the clinical feature seen a. Marfan’s syndrome b. Down’s syndrome
in: c. Brittle bone disease d. Lobstein disease
a. Marfan’s syndrome b. Fibrous dysplasia
10. Down’s syndrome is associated with:
c. Albright’s syndrome d. Cherubism
a. Trisomy of 21 chromosome
5. ‘Osteitis deformans’ refers to: b. Deletion in 5 chromosome
a. Paget’s disease b. Cherubism c. Trisomy of 13 chromosome
c. Osteoporosis d. Osteopetrosis d. None
24 Diseases of Lip
Chapter Outline
Â Anatomy Â Contact cheilitis
Â Congenital lip pits Â Actinic cheilitis
Â Commissural pits Â Exfoliative cheilitis
Â Double lip Â Plasma cell cheilitis
Â Cleft lip and palate Â Carcinoma of lip
Â Glandular cheilitis Â Chapping of the lips
Â Granulomatous cheilitis Â Actinic elastosis
Â Angular cheilitis Â Lip ulcers due to caliber persistent artery
Â Eczematous cheilitis
CLASSIFICATION OF LIP DISORDERS ANATOMY

∙ Developmental: Lips are fleshy folds lined by skin externally and mucous
– Congenital lip pits membrane internally. The upper and lower lips close
– Commissural lip pits along the red margin which represents the mucocutaneous
– Double lip junction. The lips are covered with skin on the external
– Cleft lip and cleft palate surface and mucous membrane on the inner surface, which
∙ Cheilitis: has profuse salivary glands. The lip extends from the lower
– Glandular cheilitis end of the nose to the upper end of the chin. The upper lip
– Granulomatous cheilitis borders onto the nose and is separated form the cheek by
– Angular cheilitis a variably deep groove called as nasolabial groove. The
– Contact cheilitis lower lip is separated from the chin proper by a more or
– Eczematous cheilitis less sharp and deep groove that is convex superiorly called
– Actinic cheilitis as labiomental groove.
– Exfoliative cheilitis Oral commissural is the angle where the upper and lower
– Plasma cell cheilitis lip meets. The upper lip includes the philtrum, a midline
– Cheilitis due to drugs depression, which can be followed to the nose. Each lip
∙ Carcinoma of lip mainly consists of bundles of striated muscle, orbicularis
∙ Miscellaneous: oris, superficial fascia and submucosa. The skin of the
– Chapping of lips lip contains sweat glands, hair and sebaceous gland. The
– Actinic elastosis dermal papillae are numerous, with rich capillary supply,
– Lip ulcers due to caliber persistent artery. which produce reddish pink color of the lips.
Diseases of Lip
Vermilion zone: It is the transitional zone between syndrome (popliteal webbing, cleft lip/palate, genital
the skin and the mucous membrane. The vermilion zone abnormalities, and congenital bands connecting the upper
contains no hair or sweat glands and contains a few and lower jaw).
sebaceous glands. In some people, sebaceous glands may 605
be seen as creamy yellow dots. Fordyce’s spots along the Histopathological Feature
border between vermilion border and the oral mucosa can It shows the tract lined by stratified squamous epithelial.
also occur. Chronic inflammatory cell infiltrate is noted in the
Submental artery to the lower lip and inferior and surrounding connective tissue.
superior labial arteries to the upper lip supply the lip.
Anterior facial vein and its branches corresponding to the Management
facial artery provide the venous drainage of lips. Lymphatic Surgical excision for cosmetic purpose should be carried
drainage is from central part of lower lip lymphatics drain out.
to the submental node and rest of the lip to submandibular
nodes. Mental nerve and superior labial nerve are the nerve Points to Remember
supply of the lip. Paramedian lip pits, congenital fistulas, autosomal
dominant trait, vermilion border, sparse mucus secretion,
DEVELOPMENTAL DISTURBANCE Van der Woude’s syndrome, popliteal pterygium
OF LIP syndrome, stratified squamous epithelial, chronic
inflammatory cell infiltrate, surgical excision.
Congenital Lip Pits
It is also called as paramedian lip pits, congenital fistulas Commissural Pits
of the lower lip. They arise from persistent lateral sulci on
Commissural lip pit occur due to failure in fusion of
the embryonic mandibular arch.
embryonic process. These are invagination occur on lip.
Etiology Clinical Features
It is inherited as autosomal dominant trait. There is
Age and sex distribution: It is more common amongst
notching of lips at an early stage of development with
males and black people are affected more than white
fixation of tissues at the base of the notch. It may occur due
people. It is more common in male as compare to female.
to failure of complete union of embryonic sulci of the lip.
Location: Commissural pit appears as a unilateral or
Clinical Features bilateral pit at the corner of the mouth on the vermilion
Sex distribution: It is more commonly seen in females. surface. If it is unilateral, it occurs on the right side of the
lip. It is localized at angle of mouth with the tract diverging
Location: It is common on vermilion border of either lips dorso-laterally into the cheek (Fig. 24.1).
and most commonly on lower lip. Lip pits or fistula is
unilateral or bilateral depression. Sign: In some cases small amount of fluid can be seen from
the pit when the pit is squeezed which may occur from
Size: It may be up to 3 to 4 mm in diameter and may extend minor salivary gland.
as deep as 2 cm and communicated with underlying minor
salivary glands. Size: Ranges from a shallow dimple to a tract measuring 4
mm in length and tissue slightly raised above the opening.
Sign: In some cases, sparse mucus secretion may be visible
from the base of the pit. Lips, sometimes appear swollen, Histopathological Features
accentuating the appearance of the pit.
There is narrow invagination line by stratified squamous
Syndrome associated: Congenital lip pits may occur in epithelium.
association with Van der Woude’s syndrome (cleft lip, Ducts from minor salivary gland may drains into the
cleft palate and congenital lip pits) popliteal pterygium invagination.
hangs loosely over the margin of the lid) and nontoxic

thyroid enlargement.

There is abundance of minor salivary gland.
Ascher’s syndrome shows hyperplasia of the lacrimal
gland or prolapse of orbital fat.
Management
Surgical excision of excess tissue can be perform for
esthetics purpose.
Points to Remember
Cupid bow, Ascher’s syndrome, abundance of minor
Figure 24.1 Commissural pit seen at angle of mouth salivary gland.
Cleft Lip and Palate

Management It occurs along many planes as a result of fault or defect in
No treatment is necessary as it is asymptomatic. In cases of the development.
secondary infection surgical excision should be carried out.
Etiology
Points to Remember Hereditary: It is one of most important factors to be
Failure in fusion of embryonic process, unilateral or considered in the etiology.
bilateral pit, the corner of the mouth on the vermilion
Genetic: The main possible mode of transmission is by a
surface, shallow dimple to a tract measuring 4 mm,
single mutant gene; producing a large effect or by number
narrow invagination, stratified squamous epithelium.
of genes (polygenic inheritance), each producing small
effects which together create this condition.
Double Lip
Nutritional disturbances: Riboflavin deficient diet can
It is an anomaly characterized by a fold of excess tissue on produce cleft palate and cleft lip.
the inner mucosal surface of the lip.
It may be congenital or acquired because of trauma to Developmental: Physiological, emotional and traumatic
the lip. stress during developmental stages.
The congenital variety occurs as a result of persistence Defective vascular supply: Defective vascular supply to
of horizontal sulcus which is situated between pars glabrosa the area may lead to ischemia which in turn may lead to
and pars villosa of the lip. cleft formation.
The acquired variety occur due sucking of lip inside the
Mechanical disturbances: Here, the size of tongue may
oral cavity.
prevent union of the parts.
Clinical Features Infection: Infection and lack of inherent developmental
Location: Upper lip is more commonly affected than a force.
lower lip. It usually occurs on inner aspect of upper lip. Miscellaneous: Steroid therapy during pregnancy, alcohol,
Cupid bow: When upper lip is tensed double lip resembles toxins in the circulation.
cupid bow.
It is associated with Ascher’s syndrome which consists Development of Cleft
of double lip, blepharochalasis (it is drooping of the tissue Mandibular cleft lip occurs due to failure of copula to give
between eyebrow and edge of the upper eyelid so that it rise to mandibular arch or persistence of the central groove
Diseases of Lip
of mandibular process. Maxillary cleft lip occurs due to Cleft palate: Cleft palate is a birth defect characterized by
failure of mesodermal penetration and obliteration of the an opening in the roof of the mouth caused by a lack of
ectodermal groove separating that mesodermal mass which tissue development.
actually constitutes the facial process. Either absence or 607
Lateral facial cleft: It is cause by lack of fusion of maxillary
deficiency of these mesodermal masses or their failure to
and mandibular process. It extends from commissure
penetrate the ectodermal grooves leads to breakdown of the
toward the ear. This will results in macrostomia. This
ectoderm, causing cleft formation.
The cleft of lip occurs earlier and inhibits tongue can occur in syndrome like mandibulofacial dysostosis,
migration, which may then prevent horizontal alignment hemifacial macrostomia.
and fusion of the palatal shelves. Oblique facial cleft: It extend from upper lip to eye. It
In unilateral cleft lip, the floor of the nose communi- may involve nostril or may bypass nose laterally to reach
cates freely with the oral cavity, maxilla on the cleft side is to eye. This may results due to failure of fusion of lateral
hypoplastic, columella is displaced to the normal side and nasal process with maxillary process.
the nasal ala on the cleft side is laterally, posteriorly and
inferiorly displaced. The lower lateral cartilage of the nose Median cleft of lip: It occur due to failure of fusion of
is lower on the cleft side, its lateral cruz is longer and the medial nasal process. It may be associated with oro-facial-
angle between the medial and lateral cruz is more obtuse. digital syndrome and Ellis-van Creveld syndrome.
The muscles of the orbicularis oris do not form a complete
sphincter but instead are directed superiorly to the ala nasi, Classification
laterally and the base of the columella. medially. 1st
In bilateral cleft lip, the central portion of the alveolar • Unilateral incomplete
arch is rotated anteriorly and superiorly. The medial or pro- • Unilateral complete
labial segment of skin contains no muscle or vermilion. In • Bilateral incomplete
palatal clefts, the muscles of soft palate are hypoplastic and • Bilateral complete.
insert in the posterior margin of the remaining hard palate
2nd by Veau’s
rather than the midline raphe.
• Cleft lip
Cleft palate occurs due to disturbances in normal fusion
– Class I: A unilateral notching of vermilion not
of palatal shelves; failure to unite due to lack of force,
extending into the lip.
interference by the tongue or a disparity in the size of parts
– Class II: A unilateral notching of vermilion
involved.
Cleft of soft palate and uvula do not appear to be formed with cleft extending into lip but not including
as a result of nonfusion of parts but rather as posterior the floor of the nose.
extension of palatal process; thus cleft of these parts is – Class III: A unilateral cleft of vermilion
basically extension of a cleft of hard palate. extending into the floor of the nose.
Clefts of the primary palate occur anterior to the incisive – Class IV: Any bilateral cleft of the lip, whether
foramen. Clefts of the secondary palate are due to lack of this is complete or incomplete.
fusion of the palatal shelves and always occurs posterior to • Cleft palate
the incisive foramen. The secondary palate closes 1 week – Class I: Involving only soft palate.
later in females, which may explain why isolated clefts of – Class II: Involving soft and hard palate but not
the secondary palate are more common in females. A cleft alveolus.
lip increases the probability of development of cleft palate – Class III: Involving soft and hard palate and
(Fig. 24.2). alveolus of one side.
– Class IV: Involving both the soft and hard palate
Types of Cleft in Orofacial Region and alveolus on both sides of the pre-maxilla.
Cleft lip: It is a birth defect that results in a unilateral or
bilateral opening in the upper lip between the mouth and Clinical Features
the nose. It is also called as harelip. It is wedge shaped
defect resulting from failure of two parts of the lip to fuse General: It is more common in boys than in girls. It is
into a single structure. more frequently seen on the left side than on the right
Speech problem is serious and tends to increase due

to mental trauma. There is defect in smelling due to
contamination of the nasal mucous membrane with the
608 oral organism through the cleft palate. The alveolar cleft
interferes with the dental lamina and the upper lateral
incisors may be small, absent or even duplicate.
Cleft of palate may also vary in severity, involving
uvula or soft palate or extending all the way through the
palate and indirectly to the alveolar ridge on one or both
sides (Figs 24.4 and 24.5).
Isolated cleft palate is associated with other develop-
mental abnormalities like congenital heart disease, poly-
dactyly and syndactyly, hydrocephalus, micro-cephalus,
clubfoot, supernumerary ear, spina bifida, hypertelorism
Figure 24.2 Cleft involving lip as well as palate in a child
side. Left side is involved in 70 percent of the cases.

A typical patient with cleft palate, cleft lip and ridge
exhibits a large defect with a direct opening in the nasal
cavity. Disturbances in the dental structures are seen
in this region so that teeth may be missing, deformed,
displaced or divided, thus producing supernumerary
teeth.
Cleft lip: A unilateral cleft involves only one side of the
lip; a bilateral one involves both sides and later gives rise
to hair lip.
Incomplete cleft lip extends for varying distances
forward to the nostril, but not up to the nostril. The upper
part of lip has fused normally. Figure 24.3 Cleft of lip without involvement of palate
Complete cleft lip extends into nostril and palate is
commonly involved. It is often associated with flattening
and widening of the nostril of the affected side. Sucking
become difficult to some extent but not greatly. There
is defective speech particularly with the labial letters B,
F, M, P and V. Cleft of mandibular lip or jaws are rare.
There is soft tissue mass between the ends of the bone,
uniting the tongue to the lip, so that tongue is bound
down (Fig. 24.3).
Cleft palate: There may be cleft of the hard and soft palate
or in some cases, cleft of soft palate alone.
Entire pre-maxillary portion of bone may be missing
and in such instances, the cleft appears to be entirely a
midline defect. Cross bite due to medial collapse of pre-
maxilla. Eating and drinking is difficult due to regurgitation
of food and liquid through the nose. Figure 24.4 Cleft involving palate see as defect
Diseases of Lip
609
A B
Figures 24.5A and B Cleft lip and palate
and mental deficiency. Isolated cleft palate is more com- Cheiloplasty: It is surgical closure of the lip. A general
mon in females as compared to male. ‘rule of tens’ is used in determining optimal timing of lip
Airway problems may arise in children with cleft pal- closure, i.e. 10 weeks of age, 10 pounds of body weight
ates, especially those with concomitant structural or func- and 10 gm of Hb. At the time of lip closure, when an infant
tional anomalies. For example, Pierre-Robin syndrome is is under general anesthesia, an impression is made for the
the combination of micrognathia, cleft palate and glossop- new obturator.
tosis. Affected patients may develop airway distress from
Obturator: Between 3rd and 9th months of age, an
their tongue becoming lodged in the palatal defect. Ear in-
obturator is used to provide cross-arch stability, support
fection and respiratory tract infection.
and to prevent collapse of maxillary arch.
Submucous palatal cleft: In this defect exists in Palatoplasty: It is performed to close an opening in the
underlying musculature of soft palate. The overlying palate. Surgeons may close the palate in one surgery, when
mucosa is intact in this case. It has been seen as bluish the child is about one year of age or the palate may be
midline discoloration. closed in two stages, the soft palate first followed by the
hard palate.
Syndrome Associated with Cleft Palate
Bone grafting: Sometimes closure of palatal cleft may be
• Pierre-Robin syndrome
done by bone grafting.
• Goldenhar syndrome
• Median cleft face syndrome Orthodontic therapy: Orthodontic therapy is done to
• Oral facial digital syndrome correct malocclusion.
• Apert’s syndrome Cleft rhinoplasty: To improve nasal function and correct
• Nagar syndrome the distortion.
• Otopalatodigital syndrome
• Down’s syndrome Speech therapy: Speech therapy is given to improve
• Marfan syndrome pronunciation of the words.
Psychotherapy: Psychological management is necessary.
Management
Feeding plate: To overcome initial feeding problems,
The complete rehabilitation of the condition requires a feeding plate is used which acts as an obturator to prevent
multi-disciplinary approach. nasal reflux.
Points to Remember Clinical Features

Unilateral cleft, bilateral hair lip, incomplete cleft Age distribution: It is more common in adults but
610 lip, complete cleft lip extends into nostril and palate, sometimes it can also occur in children.
defective speech B, F, M, P and V, cleft palate pre- Site: Lower lip is involved more often than the upper lip.
maxillary portion, cross bite, eating and drinking,
speech problem, isolated cleft palate, airway Signs: Labial salivary glands become enlarged and
problems, Pierre-Robin syndrome, ear infection and sometimes nodular. Orifices of secretory ducts are
respiratory tract infection, submucous palatal cleft, inflamed and dilated appearing as small red macules over
cheiloplasty, obturator, palatoplasty, bone grafting, the mucosa. Viscid mucous secretion may seep from these
orthodontic therapy, cleft rhinoplasty, speech therapy, openings of everted hypertrophic lips.
psychotherapy, feeding plate. Volkmann’s cheilitis: It is more severe suppurative form of
glandular cheilitis. The lip is considerably and permanently
CHEILITIS enlarged and is subjected to episodes of pain, tenderness
and increased enlargement. The surface is covered by crust
Glandular Cheilitis and scales beneath which the salivary duct orifice may be
It is also called as cheilitis glandularis. It is an uncommon discovered (Fig. 24.6).
condition in which lower lip becomes enlarged, firm and Malignant transformation: It is apparently pre-malignant
finally everted. and epidermoid carcinoma can be associated with it in 18
to 35 percent of cases.
ETIOLOGY
It occurs due to chronic exposure to sun, wind and dust as
well as use of tobacco. There is presence of stratified squamous epithelium which
In several cases, emotional disturbances, as well as covers inflammatory connective tissue.
familial occurrence, suggesting a hereditary pattern is In a milder form, there is some fibrosis surrounding the
observed. salivary glands and in the more severe forms, there may be
Inflammation of enlarged heterotopic salivary glands a dense, inflammatory infiltrate.
may also leads to glandular cheilitis. The underlying salivary gland tissue shows hypertrophy
and inflammation. In some cases there is dysplastic changes
Types can be seen in the epithelium.
• Simple
• Superficial suppurative type (Baelz’s disease)
• Deep suppurative type (cheilitis glandularis aposte-
matosa, myxadenitis labialis).
Types
Simple: Multiple, painless, pinhead sized lesions with
central depression and dilated canals present.
Superficial suppurative type (Baelz’s disease): It is
characterized by painless swelling, induration, crusting and
superficial ulceration of lip.
Deep suppurative type (Cheilitis glandularis aposte
matosa, myxadenitis labialis): Deep seated infection with
abscess and fistula tract that eventually forms a scar. Figure 24.6 Volkmann’s cheilitis seen as crusted area
Diseases of Lip
Management is accompanied by fever and mild constitutional symptoms

including headache and even visual disturbances. Enlarged
Vermilionectomy: Due to high incidence of associated
lip can create cosmetic problems, difficulty during eating,
malignancy, a vermilionectomy or surgical stripping of lips 611
drinking or speaking.
has been recommended.
In some cases, scaling, fissuring and vesicles or
If the lips are grossly enlarged, excision of an elongated
pustules have been reported. The swelling is usually soft
ellipse of tissue may be required.
and exhibits no pitting on pressure. Swelling eventually
Points to Remember becomes firmer and acquires the consistency of that of hard
rubber.
Cheilitis glandularis, inflammation of enlarged hetero-
The regional lymph nodes are enlarged in some cases,
topic salivary glands, secretory ducts are inflamed,
but not always. The skin and adjacent mucosa may be of
Volkmann’s cheilitis, stratified squamous epithelium,
normal color or erythematous.
fibrosis surrounding the salivary glands, dense,
It is associated with Melkersson-Rosenthal syndrome
inflammatory infiltrate, vermilionectomy, dysplastic
which consists of fissured tongue and facial paralysis.
changes.
Granulomatous Cheilitis It consists of chronic inflammatory cell infiltrate particularly
It is also called as Miescher’s syndrome or cheilitis peri and paravascular aggregation of lymphocytes, plasma
granulomatosa. cells and histiocytes.
It is a condition of unknown etiology that is not related There is focal noncaseating granuloma formation with
to cheilitis glandularis except by the similarity in the epitheloid cells and Langhans type of giant cells. There is
clinical appearance of the two diseases. generalized edema and dilated blood vessels present in the
connective tissue.
Clinical Features
Age and sex distribution: It is seen in adults as well as in Management
children and there is female predilection. Corticosteroid injection: Repeated injection of triamci-
nolone into the lips every few weeks may be effective.
Sign and symptoms: There is diffuse swelling of the lips,
especially the lower lip (Fig. 24.7). In some cases, an attack Cheiloplasty: Surgical stripping of lip can be done.
Figure 24.7 Cheilitis granulomatosa showing Figure 24.8 Histopathological picture of granulomatous
diffuse swelling of lip cheilitis
Diseases of skin: Atopic dermatitis involving the face is

Points to Remember
often associated with angular cheilitis. The incidence also
Miescher’s syndrome, fever, headache, cosmetic prob- appears to be increased in seborrhoeic dermatitis.
612 lems, scaling, fissuring and vesicles or pustules, regional
lymph nodes, Melkersson-Rosenthal syndrome, chronic Other factors: Hypersalivation, Down’s syndrome, large
inflammatory cell infiltrate, lymphocytes, plasma cells and tongue and constant dribbling being the contributory factors.
histiocytes, focal noncaseating granuloma, corticosteroid A rare cause is the presence of a sinus of developmental
injection. origin at the angles of the mouth.
Clinical Features
Angular Cheilitis
Age: It occurs in young children as well as in adults.
It is also called perleche, angular cheilosis.
Signs: It is characterized by feeling of dryness and a
Causes burning sensation at the corners of the mouth. It is usually
Microorganisms: It is caused by Candida albicans, but a roughly triangular area of erythema and edema at one or
also staphylococci and streptococci. more, commonly both the angles of mouth (Fig. 24.9).
Epithelium at the commissures appears wrinkled and
Mechanical factors: Over closure of jaws such as in somewhat macerated. In time, wrinkling becomes more
edentulous patients or in patients with artificial dentures pronounced to form one or more deep fissures or cracks
which lack proper vertical dimensions. In it, folds are which appear ulcerated but which do not tend to bleed,
produced at the corners of the mouth in which saliva although a superficial exudative crust may form.
tends to collect and the skin becomes macerated, Linear furrow or fissures radiating from the angle of
fissured and secondarily infected. Prognathism may give mouth (rhagades) are seen in more severe forms, especially
rise to a similar state of affair in young. The recurrent in denture wearers. If the lesion is not treated, they often
trauma from dental flossing may occasionally be also show a tendency for spontaneous remission.
implicated.
Nutritional deficiency: It can also occur due to riboflavin, Management
folate and iron deficiency with a superimposed fungal or Removal of cause: Underlying primary cause should be
bacterial infection. General protein deficiency can also identified and treated. A course of vitamin B and iron
cause cheilitis. supplements is useful in these cases.
Fusidic acid ointment: It is used in staphylococcal
infection. The lesions should be swabbed first and then
fusidic acid ointment or cream should be applied at least
four times a day.
Miconazole may be preferred, if angular cheilitis is due
to candidiasis (cream applied locally together with an oral
gel).
Gentian violet application: In some cases it is useful.
Points to Remember
Perleche, epithelium macerated, rhagades, dryness and
a burning sensation, fusidic acid ointment, Miconazole,
gentian violet application.
Eczematous Cheilitis
Figure 24.9 Angular cheilitis presented as fissuring and crack The lips are involved secondary to atopic eczema but
at the corner of mouth possibility of contact dermatitis must also be considered.
Diseases of Lip
The Management of atopic eczema of the lips is with an

emollient and topical steroids.
Contact Cheilitis 613

Contact cheilitis is an inflammatory reaction of the lips
provoked by the irritants or sensitizing action of chemical
agents in direct contact with them.
Causes
Lipsticks: They are composed of mineral oils and waxes
which form the stick; castor oil as a solvent for the
dyes, lanolin as an emollient preservative, perfumes and
color. The color includes azo dyes and eosin, which is a
bromofluorescein derivative. Sunscreen applied in the
form of lipstick can also cause contact cheilitis. Some also Figure 24.10 Cheilitis occurs due to lipstick allergy
contain ditrimethylolpropane triethylhexanoate which can
cause allergic reaction.
Pigmented contact cheilitis: It is cause due to paraphenulen
Lipsalves and other medicaments: Lipsalves containing diamine use in hair dye.
lanolin are frequently applied for dryness or chapping.
Phenyl salicylates and antibiotics have also been Management
incriminated as a cause of cheilitis. Topical steroids will give symptomatic relief.
Mouthwashes and dentifrices: Essential oils such as Avoidance of specific allergens: The offending
peppermint, cinnamon, clove, spearmint and bactericidal substance must be traced and avoided.
agents can cause cheilitis. Propolis, derived from resin and
collected by bees, is a well known sensitizer which has Points to Remember
been used in toothpastes. Lipstick cheilitis, persistent irritation, pigmented contact
cheilitis, topical steroids, avoidance of specific allergens.
Dental preparations: Mercury and eugenol may cause
cheilitis in the absence of stomatitis. Allergy to epimine
Actinic Cheilitis
containing materials used for crowns and bridges can cause
cheilitis. It is also called as actinic keratosis or solar cheilosis,
farmer’s lip, sailor’s lip.
Foods: Oranges, mangoes and artichokes are among the It is a pre-malignant sq. cell lesion resulting from long
food plants which occasionally cause allergic cheilitis and term exposure to solar radiation and may be found at the
dermatitis of the skin around the lips. vermilion border of lip as well as other sun exposed surfaces.
Miscellaneous objects: Metal hair clips, metal pencils,
cobalt paint on blue pencil can also cause cheilitis. Etiology
Paraphenylene diamine (PPD) is a coal-tar derivative Chronic sun exposure is the main cause so it usually occurs
that is widely used as a permanent hair dye. in hot, dry regions, in outdoor workers and in fair skinned
people.
Clinical Features
Location: Lipstick cheilitis is usually confined to the Clinical Features
vermilion borders but more often extends beyond that (Fig. Site: The lower lip is more commonly affected than the
24.10). upper lip as it receives more solar radiation than the upper
lip.
Sign and symptom: There may be persistent irritation and
scaling or a more acute reaction with edema and vesiculation. Age and sex distribution: It has got strong male
There is also dryness, crusting, fissuring, erythema. predilection in the ratio of 10:1 than female. The reason
614
Figure 24.11 Actinic cheilitis showing scaling and dry Figure 24.12 Actinic cheilitis atrophic epithelium
for this is due to fact that females had sunscreen effect of Nuclear atypia and abnormal mitoses can be seen
lipstick. It is also less common in blacks due to protective in more severe cases and some develop into invasive
effect of melanin. It is more seen in older individual than squamous cell carcinoma.
45 years of age. The collagen generally shows basophilic degenera-
tion.
Sign: In the early stages, there may be redness and edema
but later on, the lips become dry and scaly (Fig. 24.11). If
Management
scales are removed at this stage, tiny bleeding points are
revealed. With the passage of time, these scales become Topical fluorouracil: For mild cases, application of
thick and horny with distinct edges. 5 percent fluorouracil three times daily for 10 days is
Epithelium becomes palpably thickened with small suitable. It produces brisk erosion but lips heal within
grayish white plaques. Vertical fissuring and crusting 3 weeks. Application of 5-fluorouracil to the lip will
occurs, particularly in the cold weather. At times, vesicle produce erythema, vesiculation, erosion ulceration,
may appear which rupture to form superficial erosions. necrosis and epithelization. In some cases, podophyllin
Secondary infection may occur. Eventually warty is also used.
nodules may form which tend to vary in size with Rapid freezing with CO2 snow or liquid nitrogen on
fluctuation in the degree of edema and inflammation. swab stick is used to remove superficial lesions.
Signs of Malignant Transformation Vermilionectomy (Lip shaves): Under local anesthesia,

• Ulceration in actinic cheilitis the vermilion border is excised by a scalpel and closure
• Red and white blotchy appearance is then achieved by advancing the labial mucosa to the
• Indistinct vermilion border skin. Postoperative complications include paresthesia, lip
• Generalized atrophy or focal areas of whitish pruritis and labial scar tension.
thickening Laser ablation: Carbon dioxide laser therapy has been
• Persistent flaking and crusting and indurations at the used to vaporize the vermilion. Good results with no
base of keratotic lesion. postoperative paresthesia or significant scarring have been
reported.
Histopathological Features Following Management, prevention of recurrence by
It shows flattened or atrophic stratified epithelium beneath regular use of sunscreen lip salves is advisable. Liquid or
which is a band of inflammatory infiltrate in which plasma gel waterproof preparation containing para-aminobenzoic
cells may predominate (Fig. 24.12). acid probably gives the best protection.
Diseases of Lip
Points to Remember
Actinic keratosis, solar cheilosis, redness and edema,
tiny bleeding points, grayish white plaques, secondary 615
infection, vertical fissuring and crusting, atrophic
stratified epithelium, nuclear atypia and abnormal
mitoses, basophilic degeneration, topical fluorouracil,
rapid freezing with CO2, vermilionectomy (lip shaves),
laser ablation.
Exfoliative Cheilitis
It is a chronic superficial inflammatory disorder of the
vermilion border of lips characterized by persistent scaling.
Causes Figure 24.13 Exfoliative cheilitis

This can occur due to excessive production and subsequent
desquamation of superficial keratin.
Other therapy like corticosteroids, topical tacrolimus,
Some cases may occur due to repeated lip sucking,
sunscreen and moisturizing preparation is useful.
chewing or other manipulation of the lips. Cases which
occur due to chronic injury is called factitious cheilitis. Points to Remember
In some cases diffuse infection secondary to Candida
Desquamation of superficial keratin, scaling and crusting,
infection occur at the areas of low grade trauma of vermilion
more or less confined to the vermilion borders, irritation
border of lip. This type is called cheilocandidiasis.
or burning, circumoral dermatitis, psychotherapy,
Clinical Features corticosteroids, topical tacrolimus, sunscreen.
Age and sex distribution: Most cases occur in girls and
young women and majority have personality disorders.
Plasma Cell Cheilitis
It is an idiopathic benign inflammatory condition
Location: The process starts in the middle of the lower lip
characterized by dense plasma cell infiltrate in the mucosa
and spreads to involve the whole of the lower lip or both
close to the body orifice.
the lips.
It is thought to be cause by traumatic, genetic, hormonal,
Sign: It consists of scaling and crusting, more or less and autoimmune factors have been regarded as possible
confined to the vermilion borders and persisting in varying etiologic factors.
severity for months or years. Later vermilion border is
covered with thickened yellowish hyperkeratotic crust that Clinical Features
can be hemorrhagic (Fig. 24.13). Site: It can affect penis, vulva, lips, buccal mucosa, palate,
gingiva, tongue, epiglottis and larynx.
Symptoms: The patient complains of irritation or burning
and can be observed frequently biting or sucking the lips. Signs: It presents as circumscribed patches of erythema,
usually on the lower lip in elderly persons.
Circumoral dermatitis: There is involvement of perioral
skin which is presenred as areas of crusted erythema. Histopathological Features
Management It is histologically characterized by plasma cell infiltrates into
the mucosa. There is also presence of dilated blood vessels.
Elimination of cause: This will result in resolution of the
lesion. Management
Psychotherapy: This can be given for stress reduction. It Steroid: It responds to topical application of powerful
is given in combination with tranquilizers. steroids or to intradermal injection of triamcinolone.
Other therapy: It includes oral griseofulvin, and topical

cyclosporine
Topical calcineurin inhibitors are effective for plasma
616 cell cheilitis. Agents used are pimecrolimus and tacrolimus.
Points to Remember
Circumscribed patches of erythema, plasma cell
infiltrates topical calcineurin inhibitors, steroid.
Carcinoma of Lip
Squamous cell carcinoma is the most common malignancy
to affect the vermilion zone.
Clinical Features
Age and sex distribution: There is peak appearance in Figure 24.14 Malignancy of lip presented as ulcerative
growth on lower lip
6th and 7th decade of life. It is more common in males as
compared to females.
Location: It is most common on the lower lips of fair
skinned people and persons who work in outer climate. It
usually begins on vermilion border of the lip to one side
of the midline and it may be covered with a crust due to
absence of saliva.
Preceding factors: It is preceded by actinic cheilitis which is
characterized by innocuous looking white plaque on the lip.
Symptoms: Patient may complain of difficulty in speech,
difficulty in taking food and inability to close the mouth.
There is also pain, bleeding and paresthesia.
Sign: It often commences as a small area of thickening,
induration and ulceration or irregularity of the surface. In
some cases it commence as a small warty growth or fissure
Figure 24.15 Granulomatous ulcerative growth seen in
on the vermilion border of the lip. Crater like lesion having malignancy
a velvety red base and rolled indurated borders are present.
As the lesion enlarge it takes papillary or an ulcerative
form. In untreated cases there is total destruction of lip and surrounding structures and by metastasis which is through
invasion of cheek, the gums and the mandible (Figs 24.14 lymphatic channels.
and 24.15).
Papillary lesion grows slowly and infiltrated the deeper Histopathological Features
tissue relatively late whereas ulcerative growths invade They are mostly well differentiated malignancies.
early. It may metastasize and it usually ipsilateral.
Carcinoma of the upper lip metastasizes earlier and Management
more frequently than carcinoma of the lower lip. It involves Surgical: Prognosis is good if the treatment is done before
submaxillary and submental lymph nodes first and then metastasis. The best results are seen when the entire lip
deep cervical nodes. It is spread by direct extension into mucosal field is removed with early lesion.
Diseases of Lip
Points to Remember Clinical Types

Lower lips, preceded by actinic cheilitis, difficulty • C
utis rhomdoidalis: Thickened skin with furrow
in speech, thickening, induration and ulceration or giving an appearance of rhomboidal network. 617
irregularity of the surface, papillary lesion grows slowly, • Dubreuilh’s elastoma: Diffuse plaque like lesions.
metastasizes, submaxillary and submental nodes, well • Nodular elastoidosis: Nodular lesion.
differentiated malignancies, surgical.
MISCELLANEOUS The underlying connective tissue shows band of amorphous,
acellular, basophilic changes.
Chapping of Lips
It is a reaction to adverse environmental conditions in
Management
which keratin of the vermilion zone loose its plasticity, so Topical application of retinoic acid is helpful.
that lip becomes sore, cracked and scaly.
The affected subjects tend to lick the lips or to pick at Points to Remember
the scales which may make conditions worse. It is caused Solar elastosis, UV light, white area of atrophic
by exposure to freezing cold or to hot, dry wind, but acute epithelium, which vermilion border blends with the skin
sunburns can cause very similar changes. surface, pigmentary changes, leathery appearance, band
Management is by application of petroleum jelly and of amorphous, acellular, basophilic changes, topical
avoidance of the causative environmental conditions. application of retinoic acid.
Points to Remember
Lip Ulcers due to Caliber Persistent Artery
Lip becomes sore, cracked and scaly exposure to freezing
cold or to hot petroleum jelly. A caliber persistent artery is defined as an artery with a
diameter larger than normal near a mucosal or external
surface.
Actinic Elastosis Such artery in the lip causes chronic ulceration which
It is also called solar elastosis or senile elastosis. can be mistaken for squamous cell carcinoma. The ulcer
is attributed to continual pulsation from the large artery
Causes running parallel to the surface.
It is caused by prolonged exposure to UV light. UV
radiation can produce collagen degeneration in the dermis BIBLIOGRAPHY
and extent of this effect is dependent upon factors such
1. Baker SR, Krause CJ. Carcinoma of lip: Laryngoscope.
as the thickness of stratum corneum, melanin pigment,
1990;90:19-27.
clothing or chemical sunscreens. 2. Carinci F, Pezzetti F, Scapoli L, et al. Recent development in
orofacial cleft genetics. J Craniofacial Surg. 2003;14:130-43.
Clinical Features
3. Cohen DM. Green JG, Dickmann SL. Concurrent anomalies
On the labial mucosa exposed to sun, white area of atrophic cheilitis glandularis and double lip: report of case. Oral Surg
epithelium develops with underlying scarring of the lamina Oral Med Oral Pathol. 1988;66:397-9.
propria. 4. Connolly M, Kennedy C. Exfolitivateive cheilitis
In outdoor elderly population, lips may show actinic successfully treated topical tacrolimus. Br J Dermatol
elastosis in which vermilion border blends with the skin 2004;151:241-242.
surface. 5. Daley TD, Gupta AK. Exfoliative cheilitis. J Oral Pathol
Med 1995;24:177-9.
Signs: Clinical features include leathery appearance, laxity 6. Derijcke A, Eerens A, Carels C. The incidence of oral clefs:
with wrinkling and various pigmentary changes. a review. Br J Oral Maxillofac Surg. 34:488-94.
7. Eski M, Nisanci M, Atkas A, et al. congenital double lip: a 18. Ohman SC, Dahlen G, Molelr A, et al. Angular cheilitis: a
review of 5 cases. Br J Oral Maxillofac Surg. 2007;45:68-70. clinical and microbial study: J Oral Pathol. 1986;15:213-
8. Everett FG, Wescott WB. Commissural lip pit. Oral Surg 217.
618 Oral Med Oral Pathol.1961;14:202-209. 19. Onfre MA, Brosco HB, Taga R. Relationship between lower
9. Gomez Dusao AJ, Seoane J, Vazquez-Garcia, et al. Ascher lip fistulae and clef lip and/or palate in van der Woude
syndrome: report of two cases. J oral Maxillofac Surg. syndrome. Cleft palate craniofac J. 1997;34:261-5.
1997;55:88-90. 20. Park KK, Brodell RT, Helms SE. Angular cheilitis, part 1:
10. Gurvinder PT, Amrinder JK, Harshmohan. Plasma cell local etiologies: Cutis. 2011;87(6):289-95.
cheilitis: Indian journal of dermatology: 1999;44(3)135-7. 21. Park KK, Brodell RT, Helms SE. Angular cheilitis, part 2:
11. Hanami Y, Motoki Y, Yamamoto T. Successful treatment nutritional, systemic, and drug-related causes and treatment:
of plasma cell cheilitis with topical tacrolimus: report of two Cutis. 2011;88(1):27-32.
cases. Dermatol Online J. 2011;17(2):6. 22. Precious DS, Delaire J. Clinical observations of cleft lip and
12. Harada K, Sato M, Omura K. Long term maxillomandibular palate. Oral Surg Oral Med Oral Pathol. 1993;75(2):141-51.
skeletal and dental changes ij children with cleft lip and 23. Sharon E Jacob, Elise M Herro. Allergic Contact Cheilitis:
palate after maxillay distraction. Oral Surg Oral Med Oral the dermatologist. 2011;19(8):18-22.
Pathol Oral Radiol Endod. 2006;102:292-9. 24. Souissi A, El Euch D, Mokni M, Badri T, et al. Congenital
13. Kaugars GE, Pillon T, Sirsky JA, et al. Actinic cheilitis: a lower lip pits: a case report. Dermatol Online J. 2004;
review of 152 cases. Oral Surg Oral Med Oral Pathol Oral 10(2):10.
Radiol Endod. 1999;88:181-6. 25. Stoopler ET, Carrasco I, Stanton DC, et al. Cheilitis
14. Markopoulos A, Albanidou-Farmaki E, Kayavis I. Actinic glandularis: an unusual histopathological presentation.
cheilitis: clinical and pathological characteristic in 65 cases: Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;
Oral Dis. 2004;10:212-216. 95:312-7.
15. Mehta V, Nayak S, Balachandran C. Pigmented contact 26. Visscher JG, Schapveld M, Otter R, et al. Epidermology of
cheilitis to paraphenylenediamine. Indian J Dermatol. caner of lip in the netherland. Oral Oncology. 1998; 34:421-
2010;55(1):119-20. 6.
16. Miura M, Isami M, Yagami, et al. A allergic contact cheilitis 27. Worsaae N. Christensen KC, Schiodt M, et al. Melkersson-
caused by ditrimethylolpropane triethylhexanoate in a Rosenthal and cheilitis granulomatous: a clinicopathologic
lipstick. Contact Dermatitis. 2011;64(5):301-2. study of thirty-three patient with special reference to their
17. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and oral lesion. Oral Surg Oral Med Oral Pathol. 1982;54:404-
maxillofacial pathology, 3rd edn, Saunder Elsevier, 2009. 13.
1. Arteries supplying blood to lower lip is: 4. Following are the syndromes associated with cleft
a. Submental artery b. Superior labial artery palate ,except:
c. Inferior labial artery d. Sublingual artery a. Pierre Robin syndrome
b. Apert’s syndrome
2. Congenital lip pits are associated with:
c. Rabbit syndrome
a. Rabbit syndrome
d. Nagar syndrome
b. Van der Woude’s syndrome
c. Kawasaki syndrome 5. More severe suppurative form of glandular cheilitis is
d. Both a. and b. called as:
a. Miescher’s syndrome
3. Double lip is associated with: b. Perleche
a. Ascher’s syndrome b. Down’s syndrome c. Exfoliative cheilitis
c. Miescher’s syndrome d. None d. Volkmann’s cheilitis
25 Tongue Disorders
Chapter Outline
Â Embryology of tongue Â Lingual thyroid nodule

Â Anatomy of tongue Â Patent thyroglossal duct cyst
Â Surface Â Lingual polyp
Â Papillae Â Lingual cyst
Â Muscle Â Fissured tongue
Â Arterial supply Â Median rhomboid glossitis
Â Venous drainage Â Benign migratory glossitis
Â Nerve supply Â Hairy tongue
Â Lymphatic drainage Â Crenated tongue
Â Functions of tongue Â Foliate papillitis
Â Classification of tongue disorders Â Leukokeratosis nicotina glossi
Â Aglossia and microglossia Â Depapillation of the tongue
Â Macroglossia Â Dysgeusia and hypogeusia
Â Ankyloglossia Â Dyskinesia
Â Cleft tongue Â Paralysis of tongue
Â Ankyloglossum superius syndrome Â Squamous cell carcinoma
Â Lingual varicosities Â Pigmentation of tongue
INTRODUCTION filled with striated muscle. It is derived principally from

the primordia of the branchial arches in such a way that
The tongue makes up a large part of the oral cavity and the body of the tongue comes from the 2nd arch, whereas
can be affected in numerous lesions. The tongue may be the root comes from the 3rd, 4th and possibly the 5th arch.
affected as a part of oral disease or as a signs of a systemic During the 4th week of development, paired lateral
disease. The word tongue is derived from Latin word lingua thickening of mesenchymal appears on the internal aspect
and Greek word glossa. It is partly oral (anterior 2/3rds of of 1st branchial arch, to form the lingual swelling. Between
tongue) and partly pharyngeal (posterior 1/3rd of tongue). and behind the swelling there appears median eminence
called tuberculum impar. But soon, elevations of the
EMBRYOLOGY OF TONGUE ventromedial portion of the 1st arch arise on each side of
tubercle and merges with each other.
The tongue develops from the ventral wall of the primitive The tuberculum impar is a transient elevation with its
oropharynx and may be conceived as a mucosal pocket caudal border marked by a blind pit, the foramen caecum,
from which the thyroid glands develop as an endodermal by a sickle shaped fold of mucous membrane called lingual
outgrowth. Anterior 2/3rds is formed by fusion of tuberculum frenulum. On the ventral surface, lingual veins are often
impar and two lateral lingual swelling. The posterior 1/3rd visible as bluish streaks. At the lateral side of the vein
620 of the tongue has a more complicated developmental origin. is a fringed fold of mucous membrane called the plica
It first exists as a central mound called the copula, which is fimbriata or fimbriated fold. Anteriorly, on either side of
the result of fusion of the 3rd branchial arches. the frenulum, the caruncles opening for the submandibular
The endodermally derived mucosa of the 2nd to 4th ducts are visible.
branchial arches and the copula, provide covering for the Taste buds: These are peripheral gustatory organs
posterior thirds of the tongue. A V-shaped terminal sulcus, which are composed of modified epithelial cells. They are
whose apex is the foramen caecum, signifies the mobile most numerous on the sides of circumvallate papillae and
body of the tongue from its fixed root. less on the walls surrounding the foliate papillae. They are
more numerous in infants than in adults. With age, they
ANATOMY OF TONGUE undergo atrophy.
Surface PAPILLAE
The tongue is a muscular organ situated in the floor of There are four types of papillae: circumvallate, fungiform,
mouth, associated with the function of deglutition, taste filiform and foliate papillae.
and speech. It lies partly in the mouth (oral part), which
compromises the anterior 2/3rds and in the pharynx Circumvallate papillae: They are usually 8 to 12 in number
(pharyngeal part), which comprises the posterior 1/3rd. and are the largest of the papillae. They are situated in a
Both the parts are separated by the inverted V shaped row parallel to and close to the sulcus terminalis. Papillae
sulcus called the sulcus terminalis. are 1 to 3 mm in diameter and are flattened with a circular
Tongue has a base, body and circum tip. It has two depression. They are surrounded by a moat-like trough.
surfaces, a dorsal and a ventral surface. The dorsal surface
The fungiform papillae: They are smaller than the vallate
is divided into an oral and pharyngeal part and the ventral
papillae and are distributed over the dorsal surface of the
surface is confined to the oral cavity only. At the apex of
tongue, being most numerous on the anterior part. They are
sulcus terminalis, there is a depression, called the foramen
round and mushroom-shaped and is distinguished from the
cecum.
filiform papillae by their larger size and bright red color.
In the anterior part of the tongue, the mucous membrane
Their number is about 100/cm2 on the tip and 50/cm2 in the
is thin with reduced lamina propria and is closely attached to
middle. They carry taste buds.
the underlying muscular tissue. The color of the anterior part
of the mucous membrane is pink and is marked by a variety The filiform papillae: These are smallest, but most
of papillae that gives the tongue a characteristic roughness. numerous and are evenly distributed over the dorsum and
The anterior part of the tongue is divided in half by are often arranged in rows parallel to the sulcus terminalis,
the median lingual sulcus. The posterior part also called except for the tip where they run transversely. The papillae
pharyngeal part or base of the tongue is located posterior to are conical, broadest at the base and whitish due to marked
the palatoglossal arch. The surface without papillae shows degree of keratinization. The concentration of papillae in
a slightly corrugated appearance, due to the underlying man is calculated about 500/cm2. They are more heavily
lymphoid tissue called the lingual tonsil. concentrated in center of dorsum of tongue.
The root of the tongue is attached to the epiglottis by a
The foliate papillae: They are vertical folds of the mucosa
medial fold (the glossoepiglottic fold). Laterally, pharyngo-
located at the margins of the tongue, just anteriorly to the
epiglottic (glossopharyngeal) folds pass from the sides of
palatoglossal arch.
the tongue and pharyngeal wall to the epiglottis. The root
of tongue is attached to the hyoid bone, below and the Papillae simplices: They are connective tissue papillae,
mandible above. which are similar to the papillae of dermis of skin. They
The ventral surface is smooth and purplish with no are present beneath the entire tongue surface, including the
papillae. The tongue is connected to the floor of the mouth mucosal papillae described above.
Tongue Disorders
MUSCLE Vertical muscle: It is found at the borders of anterior part

of tongue. Its fibers extend from dorsal to ventral surface,
The muscles of tongue are grouped into two sets: (1) mainly near its lateral borders, but fibers are interspersed
extrinsic set (2) intrinsic set. The extrinsic muscles include through the tongue. It makes the tongue broad and flattened. 621
genioglossus, hyoglossus, styloglossus and palatoglossus.
Genioglossus: It is the largest and arises from the upper

ARTERIAL SUPPLY
mental spine and spread in a fan-like fashion and is inserted The lingual artery, a branch of the external carotid, is
into the tongue from its tip to the root. It draws the tongue the main vessel supplying the tongue. Before the artery
forward and acting together this muscle is able to flatten reaches the posterior edge of the hyoglossus muscle, it
the tongue, making a concavity from side to side. gives off a branch to the hyoid bone area. In its course
below the hyoglossal muscle, it gives off a lingual dorsal
Hyoglossus: It is a flat, quadrilateral muscle arising from
artery, which runs steeply upward dividing into many
the hyoid bone. It runs as thin plate upward, connect with
branches supplying the base of tongue and posterior part
fibers from the styloglossus and enter the tongue lateral to
of the dorsum. The root of the tongue is also supplied by
the genioglossus. It depresses the tongue.
the ascending pharyngeal and tonsillar arteries. At the
Styloglossus: It originates from the styloid process, passes lower border of the anterior part of the hyoglossal muscle,
downwards and forwards and inserts into the side of the the lingual artery gives off the sublingual artery, which
tongue, connecting with fibers from the hyoglossus. The supplies the sublingual region medial to the sublingual
styloglossus draws the tongue upwards and backwards. gland.
Palatoglossus: It originates from the palate and runs in the
palatoglossus arch, continuing into the side and dorsum of VENOUS DRAINAGE
the tongue. Deep lingual vein is the largest and the principal vein of
Intrinsic muscles: These are situated inside the tongue and the tongue. Deep lingual vein originates near the tip of the
constitute a greater part of the organ. They are divided into tongue and runs backward, close to the mucous membrane
the superior longitudinal, inferior longitudinal, transverse on the ventral surface of the tongue. It joins the sublingual
and the vertical muscles. vein, at the posterior border of the hypoglossal muscle to
form the vena comitans of the hypoglossal nerve, which
Superior longitudinal: It arise from submucous fibrous drains to the facial or internal jugular vein. The dorsal
layer close to the epiglottis and from the median fibrous lingual vein drains the dorsum and sides of the tongue. It
septum. If runs forward to the edges of tongue, some its joins the sublingual veins, which follow the artery deep to
fibers being inserted into mucous membrane. It shortens the hypoglossal muscle and enter the internal jugular vein,
the tongue and makes the dorsum concave, by the turning near the hyoid bone.
the tip and side of the tongue upward.
Inferior longitudinal: It is a narrow band lying close to NERVE SUPPLY
the inferior surface of tongue, between genioglossus and
All the muscles of the tongue, except the palatoglossus
hyoglossus. It extends from the root apex of the tongue,
are supplied by the hypoglossal nerve. The palatoglossus
some of its posterior fibers being connected with the
is supplied by the pharyngeal plexus. Lingual branch
body of hyoid bone. In front, it blends with the fibers of
of mandibular nerve is nerve for general sensation for
styloglossus. It shortens the tongue and makes its dorsum
anterior 2/3rds of tongue. Glossopharyngeal nerve is
convex by pulling the tip of tongue downwards.
the nerve for general sensation for posterior 1/3rd of the
Transverse muscle: It originates from the median fibrous tongue. Posterior most part of tongue is supplied by vagus
septum and runs in horizontal course, laterally to be nerve, through internal laryngeal nerve. Taste sensation is
inserted into submucous fibrous tissue. By their action, carried out by chorda tympani branch of facial nerve for
intrinsic muscles alter the shape of the tongue making it anterior 2/3rds and glossopharyngeal nerve for posterior
narrow and elongated. 1/3rd.
LYMPHATIC DRAINAGE Taste: It acts as a special sense organ of taste, by virtue

of presence of numerous taste buds. The tip of the tongue
The tip of the tongue drains bilaterally into the submental is most sensitive to substances eliciting a sweet sensation.
622 nodes. Right and left halves of rest of the anterior 2/3rds The lateral margins are most sensitive to substances causing
of the tongue, drain unilaterally to submandibular lymph sour sensation. The base of the tongue is most sensitive to
nodes. A few central lymphatic, drains laterally into the substances eliciting a bitter sensation. The salty quality is
same nodes. Some of the lymph vessels, from the lateral more widespread, but is greatest at the tip.
margins of the tongue, drain to the jugulodigastric nodes.
The posterior 1/3rd of the tongue drain bilaterally to the Barrier function: Mucosa covering the tongue acts as
juguloomohyoid nodes, in which most of the lymph drains a barrier protecting the deeper tissues from mechanical
from the tongue. damage. It also prevents entry of microorganisms and toxic
substances.
FUNCTIONS OF TONGUE Jaw development: Muscular pressure from the tongue
Speech: It is the result of interaction between different is an important factor in determining the shape of the
organs. Even small changes in the position or shape of mandibular arch.
the tongue may cause disturbance in speech. Tongue is Thermal regulation: It is more pronounced in dogs, where
one of the organs in the oral cavity, which interrupts the there is a considerable loss of heat from the tongue.
air passage through mouth or pharynx thereby producing
consonants. Certain consonants like c, d, j, i, n, t, z, l, g, etc. Secretion: Major secretion of tongue is provided by
requires movement of tongue. salivary glands activity which maintains the moist surface
of oral mucosa.
Mastication: The tongue has a direct crushing effect on
food by pressing it against the hard palate. The tongue Defense mechanism: Secretory immunoglobulin system
pushes the food onto the occluding surfaces and helps to of tongue plays an important role in body defense.
mix in the saliva. The sensory ending on the tongue enable Maintenance of oral hygiene: By virtue of its movement
to select those parts of the food mass that are sufficiently it can reach all parts of the oral cavity removing food debris
well masticated to be ready for swallowing. from the gums, vestibule and floor of mouth. Thus it helps
Deglutition: When the food bolus is placed on dorsum in maintenance of oral hygiene.
of tongue, it is pressed lightly against the hard palate just
Sucking: Tongue also plays an important role in sucking in
behind the incisors. It is a coordinated muscular activity
both bottle feeding and breast feeding.
involving the tongue and constrictor muscle of the
pharynx, to close the palatal velum and the epiglottis. It General sensitivity: Due to extreme sensory innervations
allows the passage of the bolus into the esophagus, without terminating in both simple and organized nerve endings,
regurgitation into the nose or lower respiratory tract. The there is perception of heat, cold, pressure and chemical
process is initiated by the voluntary action of collecting discrimination.
food onto the tongue and propelling it backwards into
Symbolic function: Functions that are traditionally
the pharynx. The muscles involved in this process are
associated with the tongue, but that have no anatomic and
the mylohyoid and the pharyngeal constrictors. Bolus is
physiologic basis should be mentioned because images of
pushed backwards by raising the back of tongue. Food
this type are well established cultural and literary tradition.
bolus is sucked from mouth into pharynx, by creating a
It must frequently influences a patient perception of a
negative pressure, while airways are still closed by rapid
lingual abnormality. Expressions such as ‘speaking with
relaxation of muscles of tongue and pharynx.
a forked tongue’ or ‘speaking in different tongue’ all
Digestion: Tongue has a slight digestive function by virtue describe the mental attitude and behaviors, by which they
of salivary lipase, present in serous lingual salivary glands. are expressed.
Tongue Disorders
CLASSIFICATION OF TONGUE ∙ Neurological disease:

DISORDERS – Glossodynia
– Dyskinesia 623
∙ Congenital and developmental disorders:
– Paralysis
– Aglossia and microglossia – Oropharyngeal dysphagia
– Macroglossia
∙ Cyst:
– Ankyloglossia
– Cleft tongue – Anterior median lingual cyst
– Ankyloglossum superius syndrome – Bronchogenic cyst
– Lingual varices – Epidermoid and dermoid cyst
– Lingual thyroid nodule – Gastric mucosal cyst
– Variations in tongue movement – Parasitic cyst
– Patent thyroglossal duct cyst – Thyroglossal duct cyst
– Tongue thrusting ∙ Benign tumor:
– Lingual polyp – Fibroma
– Reactive lymphoid aggregate – Glomus tumor
– Lingual cyst – Granular cell tumor
∙ Local tongue ditsorders: – Leiomyoma
– Rhabdomyoma
– Fissured tongue – Plasmacytoma
– Median rhomboidal glossitis
∙ Premalignant lesion and conditions
– Benign migratory glossitis
– Hairy tongue – Leukoplakia
– Crenated tongue – Lichen planus
– Oral submucous fibrosis
– Foliate papillitis
– Leukokeratosis nicotine glossi ∙ Malignant tumor:
∙ Depapillation of tongue: – Squamous cell carcinoma
Local disease – Malignant lymphoma
– Malignant melanoma
– Eosinophilic granuloma – Metastatic tumor
– Traumatic injuries – Sarcoma
– Lesions due to automutilation
∙ Miscellaneous:
– Allergic stomatitis
– Facial hemiatrophy – Pigmentation of tongue
– Cranial arteritis – Phlebectasia.
– Chronic candidiasis
Systemic disease
AGLOSSIA AND MICROGLOSSIA
– Iron deficiency anemia
– Plummer Vinson syndrome Definition
– Pernicious anemia Aglossia: It is the complete absent of tongue at birth.
– Niacin deficiency
– Folic acid deficiency Microglossia: It is the presence of small rudimentary
– Peripheral vascular disease tongue.
– Dermatomyositis
– Diabetes Causes
– Syphilis It usually occurs in syndromes such as hypoglossia-
– Zoster infection hypodactylia syndrome, Pierre Robin syndrome. It is also
– Tuberculosis associated with cleft lip and palate.
Clinical Features Classification

Symptoms: Patient encounters difficulty in eating and Congenital Macroglossia
624 speaking. It is present since birth.
Signs: Patient may have high arched palate and a narrow
constricted mandible. It is usually associated with other Acquired Macroglossia
oral or generalized malformations. There may be an Hypertrophic: In it, muscles of the tongue are hypertrophic.
airway obstruction, due to negative pressure generated by It usually occurs in mentally retarded patients.
deglutition and inspiration.
Inflammatory: It may involve the tongue partially or
Microglossia is frequently associated with hypoplasia
completely. It is due to various causes like syphilitic,
the mandible and lower incisor may be missing.
Ludwig’s angina, etc.
Management Neoplastic: It can be based on benign and malignant
Nonsurgical technique such as positioning, nasogastric tumors.
intubution and temporary endotracheal intubation can be
carried out to prevent airway obstruction. Relative Macroglossia
It is a condition, in which a normal sized tongue appears
Points to Remember abnormally large, if it is particularly enclosed within a
Hypoglossia-hypodactylia syndrome, Pierre Robin small oral cavity.
syndrome, difficulty in eating and speaking, high arched
palate, narrow constricted mandible, hypoplasia the Apparent Macroglossia
mandible, lower incisor may be missing positioning, It is a condition where the tongue appears large due to
nasogastric intubution. poor muscular control of the tongue, although there is no
increase in the bulk of tongue tissue.
MACROGLOSSIA Etiology
Macroglossia is tongue enlargement, which leads to func- Congenital: It includes hemangioma, lymphangioma and
tional and cosmetic problems. Although this is a relatively lingual thyroid.
uncommon disorder, it may cause significant morbidity.
Normal speech and swallowing reflexes require normal Inflammatory: Inflammatory causes include tuberculosis,
tongue anatomy and function. Swallowing begins as the actinomycosis, dental infection, syphilitic gumma, Riga
tongue mixes food with saliva to form a food bolus, which is disease, ranula and sublingual calculus.
then propelled into the pharynx by the tongue. Articulation Traumatic: Traumatic causes include dental irritation,
also depends on the tongue’s ability to alter the impedance hematoma and postoperative edema.
of air and change the resonant characteristics of the upper
Neoplastic: The neoplastic causes can be divided into
airway. In macroglossia, increased tongue bulk may impair
malignant and benign lesions; with the malignant lesions
these functions.
including carcinoma and sarcoma. The benign lesions
Types include granular cell tumor, neurofibroma, leiomyoma and
lipoma.
Congenital macroglossia
Acquired Metabolic: Metabolic causes are myxodema, amylodo-
• Hypertrophic sis, lipoid proteinosis, chronic steroid therapy and acro-
• Inflammatory megaly.
• Neoplastic
Muscular hypertrophy: Overdevelopment of musculature,
Relative macroglossia
this may or may not be associated with generalized muscular
Apparent macroglossia
hypertrophy or hemi hypertrophy.
Tongue Disorders

Age: Macroglossia is most prominent in infants, but Orofacial therapy uses a palatal device to stimulate
tongue size may remain above normal in childhood and muscular tone and proper tongue position. 625
adolescence (Fig. 25.1). As hyoid descends with age, Surgical approach: Majority of the cases of
macroglossia may improve. macroglossia are treated surgically. Indications for surgery
include airway obstruction, speech difficulties, dysphagia
Symptoms: This includes tongue protrusion, which
and cosmetics. The procedure of choice is partial
exposes the tongue to trauma. This exposure also leads
glossectomy. Surgical goal is to reduce the tongue size and
to mucosal drying and recurrent upper respiratory tract
thus improve the condition.
infections.
Removal of primary cause and correction of the dental
Other symptoms include swallowing difficulties, airway
arch deformity and malocclusion by orthodontic treatment
obstruction, drooling and failure to thrive.
is done. Correction of defective articulation by speech
The enlargement is generalized and may cause variety
therapy is carried out.
of difficulties with speech, feeding and airway problems.
Patient is having lisping speech. Points to Remember
Signs: It may produce displacement of teeth and Tongue enlargement, tongue protrusion, swallowing
malocclusion, due to the strength of muscles involved difficulties, airway obstruction, drooling, lisping speech,
and pressure exerted by the tongue on teeth. Crenation or crenation or scalloping of the lateral borders of the
scalloping of the lateral borders of the tongue; the tips of tongue, Beckwith’s Wiedemann syndrome, hypertrophy
scalloping fit into the interproximal spaces between the and hyperplasia muscle cells, palatal device.
teeth occurs.
In edentulous patient tongue appear as elevated and
spread out laterally causing difficulty in wearing a denture. ANKYLOGLOSSIA
It is associated with syndromes like Beckwith’s It is also called tongue-tie. It is a condition in which the
Wiedemann syndrome which includes neonatal hypogly- lingual frenulum is either too short or anteriorly placed
cemia, mild microcephaly, umbilical hernia, fetal limiting the mobility of the tongue. Reports of partial
visceromegaly and postnatal somatic gigantism. ankyloglossia affecting several generations, suggest
a possible genetic basis for the minor variation in the
Histopathological Features attachment of the genioglossus muscle. It may be traumatic
It shows hypertrophy and hyperplasia of muscle cells. or congenital.
Types
Complete: Fusion of tongue and the floor of mouth.
Partial: Short lingual frenum.
Clinical Features
Symptoms: It may limit the movement of the tongue.
In extreme cases of ankyloglossia, nursing and feeding
problems can occur. There is also recurrent tongue biting,
poor sucking and inability to chew some food.
Speech abnormalities: It was felt that tongue-tie was
associated with speech abnormalities, especially lisping
and inability to pronounce certain sounds and words, viz. t,
d, n, l, as, ta, te, time, etc.
Signs: When there is an attempt to stick the tongue
Figure 25.1 Macroglossia showing large tongue out, there may be a V shaped notch at the tip. Physical
CLEFT TONGUE
It is also called bifid tongue. It is the condition in which
626 there is cleavage of the tongue due to lack of fusion of the
lateral halves. Completely bifid or cleft tongue is rare.
Pathogenesis
Development of tongue occur in 4th week of intrauterine
life. It originate from a median swelling, the tuberculum
impar and two lateral lingual swellings joining this central
structure. These lateral lingual structures grow rapidly
to cover the tuberculum impar to form the anterior two-
thirds of the tongue. When this process is disturbed, tip of
the tongue is divided longitudinally for a certain distance
Figure 25.2 Short frenum due resulting in ankyloglossia
giving rise to cleft tongue/bifid tongue.
Clinical Features
examination will easily demonstrate the short or anteriorly
placed lingual frenulum (Fig. 25.2). Sign: Partially cleft tongue is manifested as deep
grooves in the midline of dorsal surface. Food debris and
Patients have midline mandibular diastema and inability to microorganisms may collect in the base of cleft and cause
clean the teeth and lick the lips with tongue. irritation.
Dyspnea: Recently, it is suggested that ankyloglosssia can Syndromes associated: Seen with oral-facial-digital
cause upward and forward displacement of epiglottis and syndrome, with thick fibrous bands in lower anterior
larynx which results in dyspnea. mucobuccal fold, which eliminate the sulcus and is
Syndromes associated with ankyloglosssia are anky- associated with clefting of hypoplastic mandibular alveolar
loglossum superius syndrome, rainbow’ syndrome, process.
Fraser’s syndrome and orofacial digital syndrome. Bifid tongue has also been associated with diabetic
mother syndrome, tongue piercing.
Management
Physician education, parental education and reassurance Management
should be given to the patient. Surgical correction of defect can be carried out.
Surgery: Indication for surgery, i.e. frenectomy are as
follows:
Points to Remember
If complete fusion of tongue is present then it requires
surgery. Bifid tongue, deep grooves in the midline, oral-facial-
When nursing and feeding become a problem, surgery digital syndrome, diabetic mother syndrome.
is indicated.
Children between 2 to 4 years, with poor development ANKYLOGLOSSUM SUPERIUS
of speech and anxious parents desire for the necessary
treatment.
SYNDROME
In cases, where tongue tie has recurred after snipping. It is characterized by the attachment of the tongue to the
hard palate and by limb malformation.
Points to Remember
Tongue-tie, nursing and feeding problems, speech abnor- Etiology
malities, V shaped notch at the tip, midline mandibular A genetic disorder is the most important cause of this
diastema, dyspnea, ankyloglossum superious syndrome, syndrome. Intrauterine environmental factors can also
rainbow’ syndrome. cause this syndrome.
Tongue Disorders
Clinical Features There appears to be no significant relationship between

the presence of leg varicosities and sublingual varices.
It is a very rare disorder and is associated with jejunal
and ileal Atresia. Patient has difficulty in speech and Histopathological Features 627
mastication. Patient may have difficulty in swallowing.
Vessels are dilated with presence of smooth muscle and
Management elastic fibers.
Secondary thrombosis may lead to occluded lumen
Surgical separation of the tongue from the palate.
with platelets and erythrocytes.
Points to Remember Older thrombi may result in dystrophic calcification
resulting in formation of phlebolith.
Jejunal, ileal atresia, difficulty in swallowing.
Management
LINGUAL VARICOSITIES As these are asymptomatic no treatment is necessary for this.
A varix is a dilated, tortuous vessel, most commonly a vein, Points to Remember
which is subjected to increased hydrostatic pressure and is
Varix, varicosities blue or purple shot-like clusters,
poorly supported by the surrounding tissues.
presence of smooth muscle and elastic fibers, platelets
Varicosities involving the lingual veins are relatively
erythrocyte.
common, and are seen on ventral or lateral surface of
tongue.
These may occur due to loss tonicity of supporting LINGUAL THYROID NODULE
tissue.
The thyroid gland develops in the embryo from the
Clinical Features ventral floor of the pharynx, by means of an ectodermal
invagination of diverticulum. The tongue forms at the
Age and sex distribution: It is seen after the age of 50
same time from this pharyngeal floor and is anatomically
years without sex predilection.
associated with thyroid gland by connection through the
Signs: Patient is symptom free with lesion appearing as thyroglossal tract, the lingual remnant of which is known
blue or purple shot-like clusters of vessels on the ventral as the foramen caecum.
surface and lateral borders of the tongue as well as in the The lingual thyroid is an anomalous condition in which
floor of the mouth (Fig. 25.3). follicles of thyroid tissue are found in the substance of the
tongue, possibly arising from a thyroid anlage that failed to
migrate to its predestined position or from anlage remnants
that became detached and were left behind.
Etiology
Functional insufficiency of the chief thyroid gland in neck
and failure of the primitive thyroid anlage to descend are
the cause of the condition.
Clinical Features
Age and sex distribution: Females are more affected
as compared to males in a ratio of 7:1 due to hormonal
influence. The age of onset ranges from birth to the 6th
decade, with a peak in the 2nd decade.
Signs: It is manifested as a nodular mass in or near the base
Figure 25.3 Lingual varicosity seen on ventral of the tongue, in general vicinity of the foramen caecum. It
surface of tongue is often but not always, in the midline (Fig. 25.4).
as an autosomal dominant trait. It is the ability to fold back

the tip of the extended tongue, without the aid of the teeth.
628 Trefoil tongue: Clover leaf pattern.

Gorlin sign: Extensibility of the tongue, both, forward to
touch the tip of nose and backwards into the pharynx.
PATENT THYROGLOSSAL DUCT CYST

It is also called thyroglossal tract cyst.
Development
Thyroid gland develops from an anlage of endothelial cells
in the midline of the floor of pharynx, between the first and
Figure 25.4 Lingual thyroid nodules present on posterior second branchial arches, just posterior to tubercular impair.
aspect of tongue These cells sink into the base of developing tongue
descend into the neck and proliferate below the larynx to
Symptoms: There may be complains of dysphagia, form thyroid gland.
dysphonia, dyspnea, hemorrhage with pain or feeling of Along this path epithelial tract or duct is formed which
tightness or fullness in the throat. maintain an attachment to base of the tongue. Thyroglossal
duct become associated with hyoid bone. After hyoid bone
Thyroid scan: Diagnosis is well established by using
is mature duct passes in front and beneath hyoid bone.
iodine isotopes as it has affinity for the thyroid tissue.
These tracts undergo atrophy and obliterates. However,
Rarely there are incidence that carcinoma can arise in
some remnants of this epithelium may persist and give rise
the lingual thyroid nodules.
to cyst which is called thyroglossal duct cyst.
Histopathological Features Inflammatory conditions lead to reactive hyperplasia of
the lymphoid tissue adjacent to the remnants of thyroglossal
They resemble either normal thyroid tissue or thyroid tract may stimulate the epithelial remnants themselves
tissue of an embryonal or fetal type. have been mentioned, as has a blocked thyroglossal duct
It characteristically has an incomplete or poorly defined with an accumulation of secretion.
capsule. Follicular cells sometime atrophic in nature.
Clinical Features
Management
Age and sex predilection: It usually occurs in young
Thyroxin should be given to reduce the size of swelling. persons with no sex predilection.
When swelling is causing difficulty to the patient
in spite of thyroxin therapy, excision or ablation with Site: It is seen above the thyroid, in vicinity of the hyoid
radioiodine is indicated. bone, in midline of the neck. It can occur anywhere from
foramina caecum area of tongue to superasternal notch.
Points to Remember Cyst developing in an area of thyroid cartilage are deflected
Ectodermal invagination of diverticulum, nodular mass lateral to midline due to sharp anterior margin of thyroid
near the base of the tongue, dysphagia, dysphonia, cartilage.
dyspnea, iodine isotopes normal thyroid tissue, follicular Symptoms: Pain may occur if the cyst is infected. If they
cells, thyroxin. are located high in the tract they may cause dyspnea.
Sign: It is a firm cystic mass in which formation of fistula
VARIATIONS IN TONGUE may take place. It is compressible. It yields yellow fluid on
aspiration. Swelling is fluctuant and movable. The cysts
MOVEMENT are usually in the midline and produce a softer, movable
Ability to curl up the lateral borders of the tongue into a sometimes fluctuant or tender swelling. Consistency is
tube is noted in 65 percent of Caucasians and is inherited often firm or hard depending upon the tension of fluid
Tongue Disorders
within the cyst. Cyst moves with deglutition as swelling is It is a circumscribed, sessile or pedunculated lesion. It
attached to hyoid bone by fibrous tissue. It also moves with may sometimes cause asphyxia in neonates.
protrusion of tongue. Management: It consists of surgical removal.
629
Fistulous tract: In some cases fistulous tract to the skin or
mucosa develop which may be due infection. LINGUAL CYST
It is a rare lesion. It is also called gastric cyst or
Histopathological Features enterocystoma. It arises as a result of epithelial entrapment
They are lined by pseudo-stratified columnar epithelium during fissural closure of the lateral lingual processes.
which may be ciliated or by stratified squamous epithelium.
The connective tissue wall of the cyst frequently contain Clinical Features
small patches of lymphoid tissue, thyroid tissue and Site: It is located in the anterior midline of the tongue.
mucous glands (Fig. 25.5).
Signs: It is movable and compressible (Fig. 25.6).
Management
Sistrunk operation involves the removal of a 1 cm block of
It is lined by aberrant gastric epithelium hence it is referred
tissue surrounding the duct and the duct should be traced
to as gastric cysts. Some of the lesion are lined by columnar,
down to the pyramidal lobe of thyroid gland and to the
respiratory and stratified squamous epithelium.
foramen caecum at the base of tongue.
Management
Points to Remember
Surgical: Surgical removal of the cyst is carried out.
Thyroglossal tract cyst, in vicinity of the hyoid bone,
in midline of the neck, pain, located high dyspnea, firm
Points to Remember
cystic mass, yellow fluid on aspiration, fistulous tract,
pseudostratified columnar epithelium, small patches of Enterocystoma, anterior midline of the tongue, movable,
lymphoid tissue, Sistrunk operation. compressible, aberrant gastric epithelium, columnar,
respiratory and stratified squamous epithelium.
LINGUAL POLYP
FISSURED TONGUE
It can occur at any age, with no sex predilection.
It is also called scrotal tongue, plicated tongue and lingua
dissecta.
Figure 25.5 Thyroid follicles, mucous glands and duct in the

wall of thyroglossal duct cyst Figure 25.6 Lingual cyst of newborn seen as growth
A tongue with or without a central fissure shows double fissures, and transverse fissuring arising from a
parallel fissures lateral to the midline or fissures at central fissure, transverse fissuring with a central fissure
right angle to the long axis of the tongue. Fissure and lateral longitudinal fissuring.
630 tongue is characterized by furrows, one extending
Syndrome: It is associated with Melkerson Rosenthal
anteroposteriorly and others laterally over the entire
syndrome which includes cheilitis granulomatosa,
anterior surface.
facial paralysis, fissured tongue and non-pitting, non-
Types inflammatory painless edema of face.
• Foliaceus Histopathological Features

• Cerebriform
It shows hyperplasia of the rete pegs and loss of keratin on
• Plicated.
the surface of filiform papillae.
Papillae are separated by grooves. There is formation
Etiology of microabscess in the upper epithelial layer as polymor-
It is genetically determined and seen in mentally retarded phonuclear leukocytes are migrating into the epithelial.
and psychotic individuals. It is autosomal dominant trait Management
with incomplete penetrance. Extrinsic factors like chronic
trauma or vitamin deficiency. As food and debris can act as source of infection, patient
should be advice to clean the teeth very frequently.
Clinical Features
Points to Remember
Well marked fissuring increases with age, as does the
number, width and depth of the fissures in affected Plicated tongue, small furrows or grooves on the
individuals. dorsal surface, painless, plication, central longitudinal
It is manifested as small furrows or grooves on the fissuring, double fissures, transverse fissuring patterns,
dorsal surface, often radiating out from the central groove Melkerson Rosenthal syndrome, hyperplasia of the rete
along the midline of the tongue (Fig. 25.7). pegs, microabscess.
It is usually painless, except in some occasional cases
in which food debris tends to collect in the groove and MEDIAN RHOMBOID GLOSSITIS
produce irritation.
It is also called central papillary atrophy of tongue. It is a
Pattern: Six different patterns of tongue fissuring were developmental defect resulting from an incomplete decsent
observed, i.e. plication, central longitudinal fissuring, of tuberculum impar and entrapment of a portion between
fusing lateral halves of the tongue.
It is a benign lesion of the tongue, characterized by
rhomboid or oval shaped changes of the tongue mucosa in
the midline, just anterior to the foramen cecum.
Pathogenesis
It is a congenital abnormality of the tongue due to failure
of tuberculum impar to retract or withdraw before fusion
of lateral halves of the tongue, so that a structure devoid of
papilla is interposed between it.
Etiology
Developmental: The persistent tuberculum impar suggests
its developmental origin.
Fungal infection: Fungal infection with Candida albicans
Figure 25.7 Fissure tongue showing furrow can be causative factors.
Tongue Disorders
Diabetes: Median rhomboid glossitis is more common in a Histopathological Features

diabetic, than in nondiabetic subjects.
There is loss of papillae with varying degrees of
Other factors: Other factors such as smoking, denture hyperparakeratosis. There is proliferation of spinous 631
wearing can also cause. layer with elongation of rete pegs, which may branch and
anastomose. Lymphocytic infiltration within connective
Clinical Features tissue and numerous blood vessels are seen. Connective
Age and sex predilection: Males predominate over tissue also shows increased vascularity and chronic
females (3:1). It is common in adults with age ranging from inflammatory infiltrate.
15 to 84 years. Degeneration and hyaline formation, within underlying
muscles is seen. When present fungal hyphae are usually
Location: It is located just anterior to the foramen cecum
found in the parakeratin or in the very superficial spinous
on dorsal surface of the tongue, in midline and anterior to
layer of epithelium or in both. Epithelium is devoid of
the circumvallate papillae.
filiform papillae and is slightly thickened. There is also
Signs: It appears as an ovoid, diamond or rhomboidal elongation and branching of rete pegs.
shaped reddish patch or plaque on the dorsal surface of the
tongue, immediately anterior to the circumvallate papillae. Management
In some cases flat or slightly raised area stands out from If candidal organism is found, it is treated with an antifungal
rest of the tongue because it has no filiform papillae (Fig. agent.
25.8). Only in longstanding cases, cryosurgery or an excisional
The surface is dusky red and completely devoid of biopsy is indicated.
filiform papillae and usually smooth. The texture may
be varied from a reddish, smooth, granular surface to a Points to Remember
lobulated and indurated surface. Central papillary atrophy of tongue, failure of tuber-
Symptoms: While most of the cases are asymptomatic, culum impar anterior to the circumvallate papillae,
some patients complain of persistent pain, irritation, or ovoid, diamond or rhomboidal shaped reddish patch or
pruritus. plaque, lobulated and indurated surface, persistent pain,
irritation, or pruritis, kissing lesion, loss of papillae,
Kissing lesion: When lesion of median rhomboidal hyperparakeratosis, lymphocytic infiltration, degeneration
glossitis touched the palatal surface and it causes palatal and hyaline formation devoid of filiform papillae,
inflammation, which is called the kissing lesion. elongation and branching of rete pegs. antifungal agent.
BENIGN MIGRATORY GLOSSITIS

It is also called geographic tongue, wandering rash, glossitis
areata exfoliativa and erythema migrans, erythema areata
migrans, stomatitis areata migrans.
It refers to irregularly shaped reddish areas of
depapillation and thinning of dorsal tongue epithelium that
is surrounded by a narrow zone of regenerating papillae
which are whiter than the surrounding tongue surface.
Etiology
Immunological reaction, allergic, emotional stress, here-
ditary factors, infections and nutritional deficiencies can
be etiological factors.
Figure 25.8 Median rhomboidal glossitis showing ovoid- In patients with geographic tongue, there is a high
shaped lesion frequency of history of asthma, eczema and hay fever.
Types of Benign Migratory Glossitis

Type I
632 Type II
Type III
• Fixed form
• Abortive forms
Type IV
Classification
Type I: Lesion confined to the tongue, with both active
and remission phases. No other lesion elsewhere in the oral
cavity.
Type II: As type one with similar lesions elsewhere in the
mouth. Figure 25.9 Geographic tongue presented as
erythematous area
Type III: Lesions on the tongue that are not typical and that
may be accompanied by lesions elsewhere in the mouth. It or lesion sometimes appears inflamed, while the border is
consists of two forms: outlined by thin yellowish white line or band. Fungiform
1. Fixed form: A few areas of the tongue are affected, but papillae persist in the desquamated areas as small elevated
no movement is observed. They may disappear only to red rods. Area of desquamation remains for a short time in
recur at the same area. one location and then heals and appears in another location
2. Abortive forms: This form starts as yellow-white thus giving rise to the term migration.
patches, but disappear before acquiring the typical Condition may persist for weeks or months and then
appearance of geographic tongue. regress spontaneously only to recur at a later date.
Type IV: No tongue lesions are present, but geographic Ectopic geographic tongue: Lesion is not always
areas present elsewhere in the mouth. restricted to tongue and similar irregular or circinate
lesions occur elsewhere in oral cavity and are called
Clinical Features
ectopic geographic tongue or erythema circinate migrans
Age and sex distribution: It is common in young and or annulus migrans.
middle-aged adults, with an age range of 5 to 84 years with
a predilection for females. Histopathological Features
Site: Lesion confines to dorsal surface and lateral border Filiform papillae are lost (Fig. 25.10) and at the margins
of the tongue, but may occur on the ventral surface. It of the lesion, there is usually hyperparakeratosis and
is extremely variable in size and diameter and it may be some acanthosis. Parakeratin is desquamated with
single or multiple. marked migration of polymorphonuclear leukocytes and
lymphocytes into epithelium.
Symptoms: It is asymptomatic, but the patient may This produce degeneration of epithelial cells and micro-
complain of burning sensation that is made worse by spicy abscess formation, called Munro’s abscess, near the sur-
or citrous food. face. There is also an inflammatory cell infiltration in the
Signs: Initially appears as a small erythematous, non- underlying connective tissue, chiefly neutrophils, lympho-
indurated, atrophic lesion, bordered by a slightly elevated cytes and plasma cells. Due to neutrophil infiltrate there is
distinct rim that varies from gray white to light yellow (Fig. destruction epithelium which produces atrophic mucosa.
25.9). Loss of filiform papillae produces pink to red smooth
shiny surface except the residual fungiform papillae. Management
Multiple areas of desquamation of filiform papillae, Topical local anesthetic agents like lidocaine, dyclonine
in an irregular circinate fashion are seen. Central portion hydrochloride or diphenhydramine can be given.
Tongue Disorders
Oral use of certain drugs (sodium perborate, sodium

peroxide and antibiotics like penicillin and aureomycin)
can also cause hairy tongue.
Extensive X-ray radiation around head and neck for the 633
treatment of tumors may be cause for hairy tongue.
It also occurs in patient with intermaxillary fixation and
with disturbed orophysiology, due to recent surgery in the
oral cavity.
A lowered pH of oral secretion, which blocks the
normal desquamation of epithelial cells covering the
filiform papillae results in overgrowth of filiform papillae.
Debilitating diseases in which the tongue movement is
limited by some illness. Various foods, particularly coffee
and tea, probably contribute o the diffuse coloration.
Figure 25.10 Benign migratory glossitis showing loss of Hairy tongue is also found in patient who are heavy
filiform papillae smokers.
Clinical Features
Bland diet, elimination of irritants and psychological Location: The lesion involves the dorsum, particularly
reassurance is useful. the middle and posterior one-third. In heavily keratinized
Topical corticosteroids and topical application of surface layers of filiform papillae, continuous desquamation
salicylic acid and tretinoin (retinoic acid or Vitamin A) for through friction of tongue with food, palate and upper
external use can also be helpful. anterior teeth occurs and is replaced by new epithelial cells.
Points to Remember Symptoms: Papillae which are of considerable length will
Geographic tongue, wandering rash, dorsal surface and occasionally brush the palate and may produce gagging.
lateral border of the tongue, burning sensation, small There is hypertrophy of filiform papillae. The papillae may
erythematous, nonindurated, atrophic lesion, multiple reach a length of 2 cm.
areas of desquamation, irregular circinate fashion, mi- Signs: The papillae are elongated, sometimes markedly
gration, ectopic geographic tongue, filiform papillae are so and have the appearance of hair (Fig. 25.11). The
lost, hyperparakeratosis acanthosis, polymorphonuclear hyperplastic papillae then become pigmented by the
leukocytes, Munro’s abscess, inflammatory cell infiltra-
tion, local anesthetic agents, topical corticosteroids.
HAIRY TONGUE
It is also called lingua villosa, coated tongue and black
hairy tongue. It designates an overgrowth of the filiform
papillae on the dorsum of the tongue with accumulation of
keratin giving the tongue a superficial resemblance as that
of hairiness.
Etiology
There may be delayed shedding of the horny layer of the
filiform papillae or there may be an increase in the rate of
formation of keratin.
Fungal organisms like Candida albicans and systemic
disturbances (anemia, gastric upset). Figure 25.11 Hairy tongue showing elongated papillae
colonization of chromogenic bacteria, which can impart a FOLIATE PAPILLITIS

variety of colors ranging from green to brown to black. This
gives it a coated or hairy appearance and retains debris and One foliate papilla is present in newborn at each side
634 pigments from substances such as food, tobacco, smoke of the tongue, consisting of 4 to 8 leaves and located at
and candy. the junction of the anterior two-thirds of the tongue and
the base. The folds are separated by grooves of different
Earthy or encrusted tongue: Extreme degree of coating depths, perpendicular to the longitudinal axis of the tongue.
occurring in dehydrated, debilitated and terminally ill In adults, they are barely noticeable, but sometimes they
patient can lead to a very thick, leathery coating on the swell.
tongue which is referred to as ‘earthy’ or ‘encrusted’ Foliate papillae frequently become inflamed and
tongue. It is heavily coated with bacteria and fungi and enlarged, so that it is clinically evident. Such enlargement is
forms a thickened malted layer. usually bilateral. This reactive lingual tonsil has often been
Color may be varied from yellowish white to brown called foliate papillitis. Hypertrophy of lymphoid tissue
or even black depending upon their staining by extrinsic may be followed by secondary traumatization resulting in
factors such as tobacco, certain foods, medicines or a so-called foliate papillitis.
chromogenic organisms of the oral cavity.
Clinical Features
Symptoms may be partly due to upper respiratory tract
The filiform papillae are extremely elongated and infections and partly due to irritation.
hyperplastic with keratosis.
External colonization of the papillae by basophilic Age and sex distribution: It is more common in women,
microbial colonies is a prominent feature. usually in the second half of life. It is seen in children.
Site: The condition may be bilateral with a duration varying
Management from few weeks to many years.
Brushing of the tongue twice daily for 2 minutes, making Symptoms: Soreness, tenderness, pain and occasionally, a
gentle movements over the involved area towards the tip sour or metallic taste.
of the tongue.
The topical application of podophyllum in acetone and Sign: Clinically, the patient presented a pronounced
alcohol suspension seems to be quite effective. enlargement of one foliate papilla.
Application of topical keratolytic agents and
prescription of yogurt or other Lactobacillus acidophilus
cultures have been used in the treatment of this disease. The lesion had polypoid morphology and the mass appeared
microscopically as aggregates of lymphoid tissue showing
Points to Remember a reactive hyperplasia.
Lingua villosa, papillae of considerable length, brush the
Management
palate gagging, papillae appearance of hair, pigmented
green to brown to black colors, earthy or encrusted It consists of elimination of irritating factors such as sharp
tongue, hyperplastic, keratosis, basophilic microbial edges of teeth and dentures.
colonies, podophyllum, topical keratolytic agents. If necessary, surgical removal may be performed.
Points to Remember
CRENATED TONGUE Reactive lingual tonsil, bilateral, soreness, tenderness,
The term is applied to a condition in which indentations of pain, enlargement of one foliate papilla, aggregates of
teeth are observed at the lateral margins of the tongue. lymphoid tissue, elimination of irritating factors.
It may occur due to abnormal tongue pressure habits
and tongue thrusting habits. Any enlargement of the tongue
may cause indentations on the teeth.
LEUKOKERATOSIS NICOTINA GLOSSI
Often, impression of teeth is seen on the tongue. It is It is also called smoker’s tongue. It is homogeneous, like
usually an asymptomatic and harmless condition. leukoplakia with evenly distributed, pinpoint, hemispheri-
Tongue Disorders
cal depressions, showing a so-called golf ball appearance. a large permanent cleft of the tongue. Cotton roll ulcers
As a result of heavy smoking, there is loss of papillae. No are rare, but may occur on the borders of the tongue. Such
other clinical features are found in these patients. ulcers are not indurated and can be extremely painful.
635
DEPAPILLATION OF THE TONGUE Lesions due to Automutilation
Local Disease Injuries to the tongue can occur due to self inflicted bites. It
usually occurs in mentally handicapped persons.
Eosinophilic Granuloma
It is not related with eosinophilic granuloma of bone. It is Allergic Stomatitis
also called ulcerated granuloma eosinophilicum diutinum, It refers to edematous changes in part or all of the oral and
traumatic granuloma or reparative lesion. The cause of the lingual mucosa, due to hypersensitivity reaction.
lesion is unknown. It is characterized by a well demarcated
Cause: It can occur due to certain drugs like antibiotics,
proliferative ulceration covered by thick masses of fibrin
cancer chemotherapeutic agent and anticholinergic agents.
and detritus.
It can also occur due to variety of allergens such as
Age and sex distribution: It may occur at any age and monomer of the denture, mouthwashes, chewing gum and
does not show a sex predilection. lipstick.
Site: It is located on the dorsum, margin or inferior surface Signs: There is edematous swelling of the tongue. There is
of the tongue. Some of the cases are also found on labial depapillation of the tongue.
mucosa, floor of the mouth, alveolar ridge and gingiva.
Facial Hemiatrophy
Sign: The lesions are ulcerative, not indurated and rather
well circumscribed. There is putrid odor. The ulceration is It is characterized by unilateral atrophy of the skin,
probably due to moist environment and frequent traumati- subcutaneous tissues and muscle of the face. There is
zation. It can be confused with squamous cell carcinoma. atrophy of half of the tongue.
Histopathologically it will show masses of eosinophilic
Cranial Arteritis
granulocytes as well as neutrophils, plasma cells and
histiocytes. The presence of mast cells also has been Rheumatic polymyalgia and temporal arteritis is an
reported. Proliferation of capillaries and myoblasts are inflammatory condition of large and median sized arteries
other common findings. in elderly persons. There is no sex predilection
Symptoms include headache, fever, sweating, malaise,
Management: When one is dealing with an eosinophilic fatigue, anorexia and weight loss. Blindness is the most
granuloma, spontaneous healing can be expected in a severe complication.
matter of weeks. Sign: Several cases of painful ulceration and gangrene
of the tongue, as a result of arteritis, have been reported.
Traumatic Injuries
There is also lingual pain and intermittent blanching of the
Cause: The tongue may be repeatedly traumatized, either tongue also has been described.
mechanically or chemically. It is associated with jagged teeth, Early use of adequate corticosteroid therapy at the
rough margins of restorations and inadvertent contact of level of 40 to 60 mg daily is required for the treatment of
tongue with dental medicaments such as phenol and eugenol. suspected cranial arteritis.
Sign: Localized area of depapillation is often noted with
papillary regeneration around such areas. A sharp edge of Chronic Candidiasis
the tooth may cause a yellowish, not indurated and well Chronic atrophic candidiasis can be present on the dorsum
circumscribed ulcer at the borders of the tongue. of the tongue. It is difficult to distinguish it from median
Severe damage of the tongue may occur during rhomboidal glossitis. It is diagnosed by scraping and
epileptic seizures. Prolonged oral intubation may cause cytological examination.
Systemic Disease The filiform papillae are most sensitive and disappear
first. The fungiform papilla may become enlarged.
Iron Deficiency Anemia
In advanced cases, all the papillae are lost and reddening
636 There is inhibition of epithelial reproduction, secondary become intense. In this, tongue may become so swollen
candidiasis and chronic xerostomia. that indentation from teeth are found along borders of the
There are atrophic changes on the dorsum of the tongue.
tongue. It first appears at the tip and lateral borders with
loss of filiform papilla. Folic Acid Deficiency
In extreme cases, the entire dorsum becomes smooth There is marked glossitis. The tongue is fiery red and
and glazed. The tongue may be very painful and is either atrophy filiform and fungiform papillae.
pale or fiery red. The tongue is often swollen and small cracks may
appear on the dorsum of the tongue.
Plummer Vinson Syndrome
Sideropenic anemia shares atrophic glossitis, angular Peripheral Vascular Disease
cheilitis, generalized atrophic oral mucosa, oral ulceration
It includes scleroderma and lupus erythematous.
and secondary candidiasis.
Fibrosis of submucosal tissue secondary to the
The tongue may be red or pale, painful and fissured.
obliteration of small vessels by an autoimmune process is
There is also dysphagia and dystrophy of nails.
responsible for a scarred, shrunken and atrophic appearance
Pernicious Anemia of the tongue in scleroderma.
Isolated irregular areas of lingual mucosa, atrophy
The patient suffers from general weakness, burning or
and ulceration caused by arteritis, are seen in lupus
itching sensation from the oral mucous membrane with
erythematous. In scleroderma, the tongue shrinks, losing
disturbance of taste and occasional dryness of mouth.
its mobility and papillary pattern.
There may be paresthesia, atrophy of filiform and
The color of tongue changes to a vivid appearance due
fungiform papillae (Fig. 25.12). In advanced cases, dorsum
to circulatory disturbances. In the end stages, the tongue
of the tongue becomes completely atrophic, smooth and
lies as a stiff, reduced body in the floor of mouth.
fiery red surface. Tongue appears flabby because the
normal muscle tonus is reduced.
Dermatomyositis
Niacin Deficiency It is a clinical syndrome consisting of polymyositis
Deficiency of niacin results in a disease called pellagra. associated with skin lesions. The oral mucosa may show
The tongue become fiery red and devoid of papillae. dark red or bluish erythema.
In the early stages, tongue is markedly swollen and
later becomes harder. In the late phase, tongue is atrophic.
Diabetes
Decreased nutritional status of the lingual papillae, as a
result of vascular changes affecting subpapillary dorsal
capillary plexus supplying it, causes atrophic glossitis.
Central papillary atrophy of the dorsum in which
low flat papillae are noticed just anterior to the row of
circumvallate papillae, is associated with diabetes.
Syphilis
Depapillation of the tongue usually occurs in secondary
and tertiary syphilis. In secondary syphilis mucous patch
Figure 25.12 Depapillation of tongue seen in nutritional occur, which may be single or multiple on the tongue.
deficiency Tongue in tertiary syphilis may show gumma formation.
Tongue Disorders
A more diffuse, chronic, nonulcerating, irregular • F olic acid deficiency: Marked glossitis, tongue is
induration, with an asymmetrical pattern of grooves and often swollen
smooth atrophic field covering the entire dorsum is seen. • Peripheral vascular disease: Scleroderma, lupus
Gumma is often developed in chronic interstitial glossitis. 637
erythematous, Isolated irregular areas of lingual
There is atrophy of filiform and fungiform papillae. mucosa, atrophy and ulceration caused by arteritis,
vivid appearance
Zoster Infection
• Dermatomyositis: Tongue is markedly swollen and
It is a viral infection caused by herpes zoster virus. becomes harder
Numerous vesicles occur on the ventral surface of the • Diabetes: Low flat papillae
tongue. • Syphilis: Secondary and tertiary syphilis, asymme-
trical pattern of grooves and smooth atrophic field
Tuberculosis
• Zoster infection: Numerous vesicles
The most frequent involved area is dorsum of the tongue. • Tuberculosis: Ulceration with irregular outline.
There is ulceration with irregular outline and
undermined borders, covered by yellowish gray fibrinous
layer. There is usually pain associated with ulceration. DYSGEUSIA AND HYPOGEUSIA
It is also called phantom taste or distorted taste. Dysgeusia
Points to Remember is defined as persistent abnormal taste and hypogeusia is
• E osinophilic granuloma: Ulcerated granuloma, defined as diminished taste sensation.
traumatic granuloma, dorsum, margin or inferior
surface of the tongue, ulcerative, well circumscribed, Causes
eosinophilic granulocytes, neutrophils, plasma cells, Local factors: Loss of taste sensation may occur due to
histiocytes. candidiasis, oral trichomoniasis, xerostomia, periodontitis
• Traumatic injuries: Jagged teeth, rough margins of or gingivitis.
restorations, localized area of depapillation, papillary
regeneration, epileptic seizures cleft of the tongue, Systemic factor: Systemic disease like vitamin deficiency,
cotton roll ulcers zinc deficiency, liver dysfunction, pesticide ingestion,
• Lesions due to automutilation: Self inflicted bites, radiotherapy, chronic gastritis, lead poisoning, cystic
mentally handicapped persons fibrosis, and Sjögren’s syndrome.
• Allergic stomatitis: Antibiotics, cancer chemo- Drugs factors: There are many drugs which can cause dys-
therapeutic, edematous swelling of the tongue geusia. Some example are phenindione, chlorpheniramine
• Facial hemiatrophy: Atrophy of half of the tongue maleate, metronidiazole, vincristine, thiouracil, captopril,
• Cranial arteritis: Rheumatic polymyalgia, headache, amphotericin B, etc.
fever, sweating, malaise, fatigue, painful ulceration
and gangrene of the tongue Clinical Features
• Chronic candidiasis: Chronic atrophic candidiasis, Symptoms: Patient complaint of altered taste which
scraping sometime he described as metallic, foul and rancid.
• Iron deficiency anemia: Inhibition of epithelial
reproduction, atrophic changes on the dorsum of the Phantom taste: In this case no stimulus is required to
tongue, entire dorsum becomes smooth and glazed. define altered taste.
• Plummer Vinson syndrome: Atrophic glossitis, Management
angular cheilitis, generalized atrophic oral mucosa,
tongue may be red Correction of underlying cause: This should be done to
• Pernicious anemia: Weakness, burning or itching provide relief to patient.
sensation, paresthesia, atrophy of filiform and
Points to Remember
fungiform papillae, tongue appears flabby
• Niacin deficiency: Pellagra, filiform papillae, red- Distorted taste, altered taste, correction of underlying
dening cause.
DYSKINESIA Tardive Dyskinesia

It is defined as an impairment of voluntary motion, causing Vermicular movements: Fine tremors and fasciculations
638 movements that are incomplete or only partial. of the tongue are described as ‘vermicular movements’.
Fly-catcher’s tongue or Bon-Bon sign: Rapid darting
Generalized Neurological Disease movements of the tongue is ‘Fly-catcher’s tongue or ‘bon-
Huntington’s chorea: It is a hereditary disease manifesting bon’ sign.
progressive degeneration of the nerve elements and Rabbit syndrome: When Bon-Bon sign is associate with
characterized by choreiform hyperkinesia including smacking movement of the lips with constant lip tremor, it
grimacing of face. The speech may be affected early, due is referred as rabbit syndrome.
to the involuntary movements of the tongue.
Points to Remember
Gilles de la Tourette’s syndrome: It is characterized by
motor incordination and tics, with echolalia and coprolalia. • G eneralized neurological disease: Huntington’s
The patient exhibits bizarre facial tics, protrusion of the chorea, Gilles de la Tourette’s syndrome, chorea
tongue and the tendency for lip licking. minor
• Amyotrophic lateral sclerosis: Atrophy and
Chorea minor: It is a disease of the childhood, fasciculations of the wasted muscle, flaccidity,
characterized by the occurrence of rapid irregular, aimless slowness of movement and atrophy of tongue,
involuntary movements of the muscles of the extremities, unintelligible articulation
face and trunk. The tongue is protruded quickly and rapidly • Buccolingual masticatory syndrome: Repetitive,
withdrawn to prevent biting of the tongue by involuntary nonrhythmic abnormal movements, spontaneous
movements of the jaw muscle. dyskinesia
• Tardive dyskinesia: Vermicular movements, Fly-
Amyotrophic Lateral Sclerosis
catcher’s tongue or Bon-Bon sign, Rabbit syndrome.
It is a chronic progressive disease of an unknown cause
characterized by atrophy and fasciculations of the wasted
PARALYSIS OF TONGUE
muscle. It involves demyelination of the corticobulbar
tracts and degeneration of cranial nerve motor nuclei. It is also called glossoplegia. It usually occurs due to
The muscles of palate, pharynx and tongue are commonly unilateral injury of the nucleus in the medulla or the
affected. Speech may be slurred eating solid food and peripheral hypoglossal nerve.
drinking can cause choking.
Causes
Early stage: Hypoglossal nerve involvement results in It may be caused by acute anterior poliomyelitis, infectious
flaccidity, symmetric weakness, and slowness of movement polyneuritis, neurofibromatosis, syringobulbia, irradiation
and atrophy of tongue. of head and neck and compression by a tortuous internal
Middle stage: In the middle stage a gradual and generalized carotid artery in the neck.
weakening of the tongue occurs. This is accompanied by
spasticity, which results in reduced rate, range and force of Clinical Features
articulatory tongue movements. Sign: There is paralysis and atrophy of the muscles of
one half of the tongue (Fig. 25.13). The affected tongue
Late stage: In the late stage, there is virtually unintelligible
deviates towards the paralyzed side when protruded.
articulation.
The movements towards the normal side are weak or
Buccolingual Masticatory Syndrome absent. When the tongue lies on the floor of the mouth,
it may deviate or curl slightly toward the healthy side and
This syndrome consists of repetitive, nonrhythmic movements of the tongue towards the back of the mouth on
abnormal movements that occur at the speed of normal the healthy side are impaired.
voluntary movements. If the paralysis is not accompanied by atrophy, the
Spontaneous dyskinesia: It consists of movement of tongue may appear to bulge slightly and to be higher and
mandible and protrusion of the tongue or the lips. somewhat more voluminous on the paralyzed side.
Tongue Disorders
Clinical Types
• Ulcerative variety
• Warty growth 639
• An indurated plaque or mass
• A fissure.
Clinical Features
Age and sex distribution: Carcinoma of the tongue is
disease of middle and later decades of life, with mean
age at presentation being about 60 years. Males are more
commonly affected than females.
Location: The majority of tongue carcinoma occurs on lateral
border of anterior 2/3rds of the tongue and undersurface of
Figure 25.13 Paralysis of tongue showing deviation
the tongue. The lesions on the posterior border of the tongue
are usually of higher grade malignancy, metastasize earlier
When atrophy supervenes, paralyzed side becomes and often have a poor prognosis. Cancers located in the
smaller and the tongue may become curved towards the anterior 2/3rds of the tongue are detected in early stages, as
paralyzed side with sickle shaped deformities. In some compared to those in the posterior 1/3rd of the tongue.
cases, hypoglossal nerve may be affected bilaterally, Signs: The most common presenting signs of carcinoma
causing impairment of the tongue, mobility in lateral of tongue is a painless mass or ulcer, although in most
direction and atrophy of sides of the tongue. patients the lesion ultimately becomes painful, especially
It can be seen in syndromes like Dejerine (anterior when it becomes secondarily infected.
bulbar syndrome), Jackson-Mackenzie (vagoaccessory-
Symptoms: Excessive salivation gradually appears along
hypoglossal syndrome), Collet-Sicard, Duchenne, Mobius
with the growth. In late stages, saliva becomes blood
and Tapia.
stained. As the patient is unable to swallow saliva, offensive
Points to Remember smell in the mouth occurs due to bacterial stomatitis. There
is complained of sore throat and pain in case of lesions on
Glossoplegia, peripheral hypoglossal nerve, tongue
posterior border of the tongue.
deviates towards the paralyzed side, tongue may appear
to bulge, Dejerine (anterior bulbar syndrome). Immobility of tongue: Patient may complaint of immobility
of the tongue which occurs due to extensive carcinomatous
infiltration of the lingual musculature. It becomes worse
SQUAMOUS CELL CARCINOMA when floor of mouth is involved and ultimately, it causes
It is the most common oral carcinoma with 60 percent difficulty in speech. Hoarseness of voice and dysphagia is
cases arising from the anterior 2/3rds of the tongue and present when the carcinoma involves posterior 3rd with
remainder from the base. involvement of pharynx and larynx.
Carcinoma of the tongue may be seen in four varieties.
Etiology 1. Ulcerative variety: Is usually seen near the edge of
There are many etiological factors responsible for tongue the tongue. The ulcer looks irregular and the edges are
cancer. Most commonly associated factor is alcohol and raised and everted. The floor is covered by yellowish
tobacco smoke. These two has got maximum risk for all grey slough. Base is indurated.
head and neck cancer. 2. Warty growth: It usually possesses a broad and
Other factors which can be responsible are candidiasis, indurated base (Fig. 25.14). It is developed on excess
syphilis, chronic dental trauma and chronic superficial proliferating growth of filiform papillae. Rarely, does it
glossitis. take cauliflower type look.
Posterior 3rd: It drains into jugulodigastric group of the

upper deep cervical nodes on both sides of the neck.
640 Blood: It is rare and extremely late in occurrence. It is only

seen when the growth is in extreme posterior part of the
tongue.
Management
Surgery: If the growth is less than 1 cm in diameter, it
should be removed along with a wide margin of mucosa,
not less than 1 cm. If it is localized to anterior 2/3rds of the
tongue, partial glossectomy or subtotal glossectomy should
be carried out. When the growth reaches within 2 cm of
jaw, hemimandibulectomy may be required with excision
of the growth.
Figure 25.14 Carcinoma of tongue showing ulcerative and
indurated growth Radiotherapy: When the growth is more than 1 cm in
diameter in anterior 2/3rds, the preliminary treatment is
radiotherapy in the form of interstitial radiotherapy.
3. An indurated plaque or mass: In this case a typical
indurated submucous plaque, can be felt. Prognosis: The 5 years survival rate of cancer tongue is
4. A fissure: It is usually presented as a chronic fissure not more than 25 percent.
which does not to heal.
The tumor may begin as a superficially indurated ulcer Points to Remember
with a slightly raised border and may proceed either to Alcohol, candidiasis, syphilis, lateral border of anterior
develop a fumigating, exophytic mass or to infiltrate the 2/3rds of the tongue, painless mass or ulcer, excessive
deep layers of the tongue, producing fixation and induration salivation, immobility of tongue, ulcerative variety,
without much surface changes. At an early stage tongue warty growth, indurated plaque or fissure, local spread,
cancer may appear as thickened, leucoplakic patches, or as lymphatic spread, blood spread, surgery, radiotherapy.
a nodule.
Spread of Carcinoma Complications

Local spread: It spreads by infiltration and invasion. Patient usually dies due to cancerous cachexia, starvation,
inhalation bronchopneumonia, asphyxias and hemorrhage
Carcinoma of anterior 2/3rds of the tongue: It usually from involved cervical lymph nodes.
starts on the lateral margins of the tongue and invades the
floor of the mouth early, but it does not extend to the other PIGMENTATION OF TONGUE
side across the midline.
The tongue may exhibit various patterns of racial melanin
Carcinoma of posterior 1/3rd of the tongue: It tends pigmentation.
to spread to the corresponding tonsils, epiglottis and soft Endogenous pigmentation is rarely identifiable on the
palate. dorsum because of the thickness of the epithelium, but
Lymphatic Spread jaundice may be apparent under the thinner ventral mucosa.
In some cases extensive melanin pigmentation can occur
Tip of the tongue: Carcinoma of tip of the tongue may on tongue (Fig. 25.15).
drain to the submental nodes, but may also drain to the Exogenous pigmentation of filiform papillae of the
jugulo-omohyoid nodes. normal and coated or hairy tongue is very common and
Anterior 2/3rds: It drains into the submandibular lymph result from microbial growth, metabolic products, food
nodes. debris and dyes from candy, beverages and mouth rinses.
Tongue Disorders
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27. Southam JC, Ettinger RL. A histological study of sublingual 32. Yasuda Y, Kitai N, fujii Y, et al. Report of patient with
varices. Oral Surg Oral Med Oral Pathol. 1974;38;879-86. hypoglossia-hypodactylia syndrome and review of literature:
28. Sunira Chandra, Vaishali Keluskar, Anjana Bagewadi, et al. clef palate Craniofac J. 2003;40:196-202.
Extensive physiologic melanin pigmentation on the tongue: 33. Yifat Manor, Amos Buchner, Michael Peleg, et al. Lingual
An unusual clinical presentation, Contemp Clin Dent. cyst with respiratory epithelium: An entity of debatable
2010;1(3):204-6. histogenesis: Journal of Oral and Maxillofacial Surgery.
29. van der Waal I, Pindborg JJ. Diseases of the tongue. Chicago: 1999;57(2):124-7.
Quintessence; 1986.
1. The largest papillae are: 6. Geographic tongue is refer to:

a. Filiform papillae b. Fungiform papillae a. Hairy tongue
c. Circumvalate papillae d. Foliate papillae b. Glossitits
2. Most numerous and smallest papillae are: c. Crenated tongue
d. Benign migratory glossitis
a. Papillae simplices b. Filiform papillae
c. Fungiform papillae d. Foliate papillae 7. Loss of filiform papillae and munro’s abscess is the
histopathological feature of:
3. Muscle depresses the tongue is:
a. Erythema migrans b. Geographic tongue
a. Hyoglossus b. Genioglossus c. Both a and b d. Lingua dissecta
c. Palatoglossus d. Vertical muscle
8. In hairy tongue, there is a hypertrophy of:
4. All the muscle of tongue are supplied by hypoglossal a. Fungiform papillae b. Filiform papillae
nerve, except: c. Circumvalate papillae d. Foliate papillae
a. Hyoglossus b. Palatoglossus
9. Extensibility of tongue, both, forward to touch the tip
c. Genioglossus d. Styloglossus of nose and backward in to the pharynx refer to:
5. Fissured tongue is associated with: a. Gorlin sign b. Bon-Bon sign
a. Melkerson-Rosenthal syndrome c. Rabbit syndrome d. Vermicular movements
b. Apert’s syndrome 10. Rapid darting movement of tongue is:
c. Parkes Weber syndrome a. Bon-Bon sign b. Rabbit syndrome
d. Sturge-Weber syndrome c. Fly-catcher’s tongue d. Both a and c
26 Temporomandibular Joint
Pathology
Chapter Outline
Â Coronoid hyperplasia Â Rheumatoid arthritis

Â Condylar hyperplasia Â Ankylosis
Â Condylar hypoplasia Â Subluxation (hypermobility)
Â Bifid condyle Â Synovial chondromatosis
Â Osteoarthritis Â Temporomandibular joint dysfunction
Temporomandibular joint (TMJ) is a unique joint in Site: It is unilateral or bilateral. Bilateral seen more
which translatory as well as rotational movements are commonly than unilateral.
possible and where both the ends of bone articulate, in
Restricted mandibular movement: Coronoid process
the same plane, with that of other bone. It is also called
impinged on posterior surface of zygoma, deviation of
as ginglymodarthrodial type of joint, meaning that it has a
mandible towards affected site.
relatively sliding type of movement between bony surfaces,
in addition to hinge movement, common to diarthrodial Radiographic Feature
joint.
There is irregular nodular growth of tip of coronoid
process. In bilateral type there is regular elongation of
CORONOID HYPERPLASIA both process.
This is rare anomalies which may results in limitation of
mandibular movements. Management
Surgery: Surgical removal of elongated coronoid process.
Cause
It has got hereditary nature as cases are seen in family. Points to Remember
Other factors which can be causative factor for coronoid Unilateral or bilateral, restricted mandibular movement,
hyperplasia are endocrinal, tumor affecting coronoid deviation of mandible, irregular nodular growth regular
process. elongation.
Clinical Features
CONDYLAR HYPERPLASIA
Age and sex distribution: It is more common in male in
the ratio of 5:1. It is more commonly seen at the puberty. There is excessive growth of one condyle.
Cause Types
It is as such idiopathic in nature, but in some cases local Congenital: It is associated with head and neck syndrome
644 circulatory problems, endocrine disturbances and trauma like mandibulofacial dysostosis, Goldenhar syndrome.
are thought to be causes.
Acquired: Is results from defect in growth center of
Clinical Features condyle in developing stage. It is usually cause by trauma
during infancy or childhood.
Age: It is more commonly seen in adolescents and young
adults. Clinical Features
Signs: There is facial asymmetry, prognathism cross bite Site: It can be unilateral or bilateral.
and open bite. Signs: There is class II malocclusion with smaller size of
Radiographic features: There is enlargement of condylar mandible.
head and elongation of neck of condyle (Fig. 26.1). Unilateral hyperplasia shows depression on the affected
side. There is also deviation of midline towards the affected
side while opening of the mouth.
During active phase there is proliferation of condylar
cartilage is seen. Radiographic features: There is short condylar process,
small sigmoid notch, poorly formed condylar head (Fig.
Management 26.2).
Unilateral condylectomy: It can be done in some patient. Management
Costochondral rib graft can be place to establish active
Points to Remember
growth center.
Proganthism cross bite, enlargement of condylar
head, proliferation of condylar cartilage, unilateral Points to Remember
condylectomy. Mandibulofacial dysostosis class II malocclusion,
depression on the affected side, small sigmoid notch,
CONDYLAR HYPOPLASIA costochondral rib graft.
It is the underdevelopment of mandibular condyle.
Figure 26.1 CT scan of hyperplasia of condyle Figure 26.2 Hypoplasia of condyle on left side
Temporomandibular Joint Pathology
Etiopathogenesis
The lesion is brought about either by an increase in the
functional demands of the healthy tissue or by deterioration 645
in the functional capacity of the tissue. Breakdown of the
joint may occur when the tissues are subjected to repetitive
overload in excess of their functional capacity or with
normal load when the capacity is reduced as a part of aging
process.
By another theory, bone growth does not cease
completely after puberty and remodeling of the joint
progresses under functional demands. Degenerative joint
disease may develop when the remodeling rate of bone
exceeds that of the cartilaginous repair. The gross evidence
of these changes is the formation of marginal osteophytes
Figure 26.3 Bifid condyle showing bilobed pattern with development of new bone in the area adjacent to the
cartilage.
There is slower replacement of chondroblast and
BIFID CONDYLE chondrocytes in joint cartilage. Fibrocartilage of TMJ force
fibers to work longer and becomes susceptible to fatigue.
In this double headed mandibular condyle. They have The matrix is having less water become desiccated and
medial and lateral head divided by anteroposterior groove. brittle. There is diminished blood flow to TMJ producing
Cause of this thought to be traumatic in origin. Trauma poor nutrition to the joint. After continuous joint use surface
can occur in childhood. Other factor which though to fiber break down exposing underlying bone. This bone
causing it are abnormal muscle attachment, teratogenic then undergoes degenerative destruction and proliferation.
agent and persistence of fibrous septum.
Bifid condyle is asymptomatic and discover on routine Clinical Features
radiograph. Radiograph will show bilobed pattern of the Age and sex distribution: It occurs in patients older than
condyle (Fig. 26.3). 40 years of age and 85 percent of them are older than 70,
No treatment is necessary for this case as it is with a mean age of 53 years. Females are affected 6 times
asymptomatic. as frequently as males.
Points to Remember Location: It is common in many joints, but it is not
Double headed mandibular condyle, traumatic, abnormal frequently found in TMJ.
muscle attachment, persistence of fibrous septum, Symptoms: Unilateral pain over the joint, which may
bilobed pattern of the condyle. be sensitive to palpation, occurs. Pain on movements or
biting occurs, which may limit mandibular function. Pain
is usually located to the immediate preauricular region.
OSTEOARTHRITIS
Signs: There is deviation of the jaw towards the affected
It is also called as osteoarthrosis or degenerative arthritis.
side. Stiffness of the joint is present. There is presence of
It is primarily a disorder of movable joints characterized
crepitation of the joint; the sound indicates degeneration
by deterioration and abrasion of the articular cartilage with
within the articulating surfaces of the joint or disc. There is
formation of new bone at the joint surface.
limitation of jaw movements, which becomes increasingly
There is destruction of the soft tissue component of the
apparent with function.
joint and subsequent erosion with hypertrophic changes in
bone. There is breakdown of the connective tissue covering Muscle guarding: Patient exhibits tenderness of the
of the condyle, articular eminence and the disk. Articular muscle due to constant strain on muscle. This strain is due
eminence shows resorption and the underlying bone to attempt to keep painful joint immobile. This is called as
becomes sclerotic. muscle guarding.
cartilage, in turn, causes erosion and perforation with

ultimate destruction of the inner articular disc.
Secondary arthritic changes in the articular disc may
646 result in flattening of the disc. Initial destruction of the
soft tissue component of the joint occurs with subsequent
erosion and hypertrophic changes in the bone. The
connective tissue covering the condyle eminence and disc
breaks down. The bony surface of the condyle and articular
eminence shows resorption with the underlying bone
becomes sclerotic.
The bone beneath the cartilage contains osteocytes,
fatty degeneration or necrosis of marrow, marrow
fibrosis, infiltration by chronic inflammatory cells,
large degenerative space (subchondral cyst). There is
Figure 26.4 Ely cyst in osteoarthritis of TMJ
also formation of metaplastic bone (ossicles), or hyaline
cartilage granules (chondral bodies).
Early signs may progress to spasm of the masticatory Management

muscles resulting in stiffness and locking of the jaw. If not Elimination of the cause: It includes occlusal adjustment
treated at this point it may lead to irreversible changes in or replacement of the missing teeth and ill fitting prosthesis,
the TMJ. Normal course is between 1 to 3 years. Severe grinding, treatment of caries and periodontal disease.
symptoms last for about nine months, but gradually burn Low dose doxycycline and NSAIDs can reduce the
out leaving little or no disability. symptoms of osteoarthritis.
We can also give physiotherapy, myotherapy,
Radiographic Features corticosteroids and occlusal splints.
Osteophytes: There is formation of protuberance of bone
in the joint. This is called osteophytes. Points to Remember
There is narrowing and obliteration of joint space. Degenerative arthritis, increase in the functional
There is also presence of radiolucent subchondral demands, unilateral pain over the joint, crepitation of the
cyst or ely cyst (Fig. 26.4), ossicles (ossification within joint, muscle guarding, spasm of the masticatory muscles,
the synovial membrane), osteosclerosis and flattening of osteophytes, subchondral cyst, ossicles, osteosclerosis,
articular surface. flattening of articular surface, proliferation of overlying
articular tissue, osteophytic lipping, flattening of the
Histopathological Features disc, fatty degeneration or necrosis of marrow, marrow
The earliest changes occur in the articular covering of the fibrosis, chronic inflammatory cells, large degenerative
condyle. It shows that the cells tend to clump together and space, hyaline cartilage granules,low dose doxycycline,
become unevenly distributed throughout the fibrous matrix. NSAIDs, physiotherapy, myotherapy, corticosteroids
At this stage, the subarticular bony end plate may still and occlusal splints.
be intact, but later osteoclastic resorption of the bone takes
place.
Vertical splitting of the articular layer takes place and this
RHEUMATOID ARTHRITIS
is followed by fragmentation and loss of articular surface, It is a debilitating systemic disease of unknown origin,
with exposure of the underlying bone. Attempted repair by characterized by progressive involvement of the joint,
proliferation of overlying articular tissue downward into particularly bilateral involvement of large joints.
the resorbing areas of subarticular bone is sometimes seen Bony components of the TMJ are affected secondary to
and this leads to uneven thickening of the articular surface. the granulomatous involvement of the synovial membrane
Osteophytic lipping was found only on the anterior that subsequently spreads to the articular surface of the
surface of the condyle. Destruction of the articular condyle.
Etiopathogenesis fatigue, weight loss, pain and stiffness in the limb are also
evident. Polyarthritis develops subsequently, large and
Its manifestations are probably due to a two phase process.
weight bearing joints are frequently affected.
Phase one result from some systemic infection, which 647
evokes an inflammatory response within the joint. Spindle appearance of finger: Swelling of the proximal
Phase two as an autoimmune reaction to the antigen but not the distal, interphalangeal joints gives the finger a
generated by the initial inflammation itself or it may be spindle appearance and swelling of the metatarsophalangeal
associated with derangement of the immune response to joints results in broadening of feet.
the exogenous antigen. It may results from cross reaction Subcutaneous nodules: There is formation of subcutaneous
of antibodies generated against hemolytic streptococci or nodules on the pressure points, sites of friction and various
other micro-organism. vascular lesions, both necrotizing and obliterative types.
In the active phase, TMJ may get involved bilaterally. Severe deformities of extremity can occur as a result of
The joint space enlarges with synovial effusion which joint collapse, tendon rupture and muscle involvement.
attacks the fibrocartilage and ultimately produces erosion
of the underlying bone. This causes pain, stiffness and Signs: The joint may become red, swollen and warm to
limitation of movement. touch. Muscle atrophy around the joint is common. In
The process then enters the healing phase, where hands, it may produce an ulnar drift. Bursitis can also occur.
the symptoms subside and remodeling of the articular Anvil shape joint: Joint involved in arthritis have got
surface occurs. Bony components of the TMJ are affected characteristic anvil shape with irregular flattening of central
secondary to the granulomatous involvement of its synovial articular surface and splaying of the lateral bone.
membrane that subsequently spreads to the articular surface
of the condyle. TMJ Involvement
However, chronic phase may follow before healing It can be acute or chronic and usually, it is bilaterally
occurs. Here, there is proliferation of the synovial membrane involved.
due to inflammation this is called as pannus formation. This
pannus then encroaches the joint space and causes destruction Acute case: In acute cases, there is bilateral stiffness, deep
of the articular cartilage. In this lipping of the condyle and seated pain, tenderness on palpation and swelling over the
marginal proliferation is seen, this result in narrowing of joint. There is limitation in opening of mouth.
joint space. Here, predominant clinical findings are crepitus, Chronic cases: In chronic cases, crepitus is the most
pain on biting and tenderness. The granulomatous tissue frequent finding. Functional disturbances like deviation
replaces the articular surface and small adhesions develop on opening and inability to perform lateral excursions are
between the articular surface and disk. common. Anterior open bite is present due to bilateral
Psychosomatic: Emotional trauma, anxiety and destruction and antero-posterior positioning of the
environmental strain can lead to the onset of rheumatoid condyle. Fibrous ankylosis of the joint which may be
arthritis. partial or complex occurs in long term. Pain on biting
Immunological: The presence of rheumatoid factor in is referred to the temporal region, ear and angle of
the serum and synovial fluid of affected patients suggests mandible.
immunological etiology. Also present are plasma cells and
lymphocytes on histological examination. Radiographic Features
Clinical Features There is flattened condylar head with irregular surface (Fig.
26.5). There is also anterior displacement of the condyle.
General
Age and sex distribution: It more commonly occurs in Histopathological Features
temperate climate and has its highest incidence in women
Proliferation of synovial lining cells with intense infiltration
from 20 to 50 years of age.
of lymphocytes, plasma cells and polymorphs.
Symptoms: It include bilateral stiffness, crepitus, As the inflammation becomes chronic, granulation
tenderness and swelling over the joint. Fever, malaise, tissue spreads across the cartilagenous articular surface
Points to Remember
Autoimmune reaction, pannus formation, psychoso-
648 matic, immunological, spindle appearance of finger,
polyarthritis, subcutaneous nodules, anvil shape joint,
red joint, muscle atrophy around the joint, anterior open
bite, fibrous ankylosis of the joint, flattened condylar
head, anterior displacement of the condyle, proliferation
of synovial lining cells, rice bodies, rose Waller test is
positive, intra-articular corticosteroid injections, rest to
the joint, systemic glucocorticoids therapy.
ANKYLOSIS
Ankylosis, a Greek word which means stiff joint. It is an
Figure 26.5 Flattened condylar head in rheumatoid arthritis
abnormal immobility and consolidation of the joint.
undermining the margins and eventually eroding the Types

adjacent bone. Later on, the fibrous tissue becomes dense • True (intra-articular)
and it may unite the head of the condyle to the articular • False (extra-articular)
fossa. • Bony
• Fibrous
Rice bodies: Synovial membrane protrude in the joint
• Partial
space as villi which undergo necrosis resulting in rice
• Complete.
bodes which are small whitish villi fragments composed of
cellular debris admixed with fibrin and collagen.
Classification
Investigations True (intra-articular): It is any condition that produces
Rose Waller test is positive in 70 percent of the patients fibrous or bony adhesion between the articular surfaces of
with rheumatoid arthritis. the TMJ.
Antinuclear antibodies are detected by indirect False (extra-articular): It is the one which results from
immunofluorescence. pathologic conditions outside the joint, that result in limited
Analysis of synovial fluid is essential for the immediate mandibular mobility.
diagnosis of joint infection, inflammation and degenerative
Bony: If bone is present between the articulating surfaces
disease.
and prevents movements, it is called as bony ankylosis.
Management Fibrous: If the medium which prevent, the movements is
Adequate rest to the joint, soft diet is advocated. Treatment fibrous, it is called as fibrous ankylosis.
should be given for suppression of the active process, Partial: If there is incomplete union between the
preservation of function and prevention of deformities. articulating surfaces, it is called as partial ankylosis.
Intra-articular corticosteroid injections, non-steroidal
anti-inflammatory drugs, immunomodulator, slow acting Complete: If there is complete union between the
anti-rheumatic drug can be given. articulating surfaces, it is called as complete ankylosis.
Systemic glucocorticoids therapy: This can give Etiology
relief to many patient.
Local treatment is done with heat, diathermy, jaw False
exercise or a mouth stretcher. Muscle strengthening Myogenic: The most common problem associated with
exercise and hydrotherapy. muscle origin is fibrosis, which may result from chronic
infection of the elevator muscles of mastication. Myositis

ossificans can also produce limitation of opening.
Neurogenic: They include epilepsy, brain tumor, bulbar 649
paralysis and cerebrovascular accidents.
Psychogenic: Here the affected persons exhibit no pain,
but cannot get the jaws separated also called as hysterical
trismus and is apparently produced due to fright.
Bone impingement: The most common is coronoid
impingement. Malformation of coronoid such as exostosis
or elongation can cause the mandible to impinge on the
posterior aspect of the zygoma, when opening is attempted.
The formation of fibrous adhesions or cicatrical bands
of scar tissues usually occurs after a traumatic accident or Figure 26.6 Intraoral view of patient who is having ankylosis
burns. showing trismus
Neoplastic disease like osteochondroma.
True
Congenital: Abnormal intrauterine development, birth
injuries and congenital syphilis.
Trauma: Trauma to the chin forcing the condyle against
the glenoid fossa, particularly with bleeding in the joint.
Malunion of condylar fractures. Injuries associated with
fracture of the malar-zygomatic compound. In the case of
injury to the joint, there is hemorrhage within and outside
the joint capsule. Injury occurs during the period of active
bone formation. There is immobility of the jaw due to pain
or treatment and ankylosis is take place.
Inflammatory: Primary inflammation of the joint.
Inflammation of the joint secondary to a local inflammatory
process (otitis media, osteomyelitis, etc). Inflammation of Figure 26.7 Extraoral view of unilateral ankylosis showing
the joint secondary to a blood stream infection (septicemia, fullness on affected side
scarlet fever, gonorrhea). Rheumatoid arthritis is the most
common cause of bilateral ankylosis. Gonococcal arthritis duration, there may poor oral hygiene, carious teeth and
can also cause ankylosis of TMJ. Inflammation secondary periodontal problems malocclusion.
to radiation therapy.
Others: Loss of tissue with scarring and metastatic Unilateral
malignancy. Unilateral ankylosis is more common than bilateral
ankylosis. Mouth opening is impossible, but the patient
Clinical Features (Figs 26.6 to 26.9) may be able to produce several mms of interincisal
General opening. Asymmetry of the face with fullness on the
affected side and relative flattening on the unaffected side.
Age: It is seen primarily in a young age or between 1 to
In unilateral ankylosis, patient’s face is deviated towards
10 years.
the affected side. The chin is retracted on the affected side
Symptoms: Pain and trismus is present which is directly and slightly bypasses the midline. There is slight gliding
related to the duration of ankylosis. Depending upon the movement towards the affected side. Cross bite is present.
650
Figure 26.8 Unilateral ankylosis showing deviation of Figure 26.9 Bilateral ankylosis showing bird face appearance
mandible on affected side
Bilateral fibrous cartilage → direct transformation into bone →

ankylosis.
Face is symmetrical with micrognathia. There is bird face
appearance. No gliding movement. With bilateral ankylosis, Management
neither protrusive nor lateral movements are possible. An
attempt at forced opening in bony ankylosis, there is no pain Brisement force, condylectomy, gap arthroplasty is
but in case of fibrous ankylosis there is pain. performed.
Due to long standing ankylosis, atrophy or fibrosis of
Points to Remember
muscles of mastication may result. In case of congenital
ankylosis, there is difficulty of introducing the nipple Stiff joint, myogenic, neurogenic, psychogenic, bone
into the mouth of newborn infants. Class II malocclusion, impingement, neoplastic disease, congenital, trauma,
protrusive incisors and anterior open bite are also the inflammatory, pain, trismus, asymmetry of the face, fullness
features. It is due to the patient’s attempt at forcing food on the affected side, relative flattening on the unaffected
through the anterior teeth for a period of years. side, patient’s face is deviated towards the affected side,
bird face appearance, class II malocclusion, protrusive
Histopathological Features incisors and anterior open bite, thick fibrous bands, loss of
Atrophic or destructive changes in the cartilagenous meniscus, brisement force, condylectomy, gap arthroplasty.
component of the joint with loss of meniscus are a constant
finding. SUBLUXATION (HYPERMOBILITY)
There is progressive destruction of the joint tissue
with narrowing of the joint space. Normal soft tissues are It is the unilateral or bilateral positioning of the condyle
replaced by thick fibrous bands, which blend in with the anterior to the articular eminence, with repositioning
periarticular tissue. to normal accomplished physiologic activity. It is self
An overall flattening of articulation, with glenoid fossa reducing incomplete dislocation, which generally follows
and articular eminence becoming less pronounced and stretching of the capsule and ligaments.
condylar process become enlarged. The enlarged condyle
is composed of dense sclerotic bone. Etiology
Apparent union of injured parts is by granulation It is caused by long continuous opening of mouth, oral
tissue → it is replaced by dense fibrous connective surgical procedures, osteoarthritis, psychiatric problem,
tissue → formation of fibrous cartilage → metaplasia of use of phenothiazine derivatives.
Clinical Features Predisposing Factors

It may be unilateral or bilateral. Drugs such as thiazide diuretics, operations, trauma,
Symptoms: Cracking noise, temporary locking of the alcohol and rapid weight loss can lead to gout. 651
condyle and immobilization of the jaw. Patient describes
weakness of the joint while yawning. Pain is associated Clinical Features
with last few millimeters of mouth opening. Acute Gouty Arthritis
Signs: The condyle may get locked when the mouth
Site: Initially, metacarpophalangeal joints are commonly
is opened widely and upon closing, it will return with a
involved. Later foot, ankles, hand, wrist and elbow may
jumping motion, accompanied by a sound caused by
be affected.
movement of the condyle over the articular eminence. On
palpation click on opening and sliding of condyle over the Symptoms: There is excruciating pain, which is worse at
articular eminence, are common. night. Sometimes it is associated with anorexia, fever and
general malaise.
Signs: Joint returns to normal after few days, with
Radiograph shows excessive excursion of the condyle desquamation of the overlying skin.
from rest position to the position when jaw is opened wide.
Chronic Tophaceous Gout
Due to long standing opening the ligament and capsule Symptoms: As the disease becomes chronic, pain and
are stretched. As the ligament does not contain elastic stiffness persist, with irregular swelling.
fibers, once it is stretched beyond its capacity, it does not Tophi: Tophi are found in cartilage of the ear, nose or
come back to its normal length. The lax capsule and over eyelids. In half the cases, palms of hands may show white
stretched ligament are responsible for subluxation. streaks along the creases.
Management TMJ Involvement
Shrinking of the capsule by a sclerosing agent will cause It is seen in middle age with equal sex distribution. It has a
fibrosis of the capsule. It will limit the condylar excursion hereditary tendency.
without deleterious effects on motion and cartilaginous
surface of the articulating surface of the condyle. The Symptoms: Sudden excruciating pain in the TMJ, followed
solution used is 5 percent sodium psylliate or 5 percent by rapidly developing swelling.
intracaine in oil base. Signs: Tenderness of the affected area with limitation of
movements occurs. Deviation to the affected side while
Points to Remember opening the jaws.
Positioning of condyle anterior to the articular eminence,
cracking noise, temporary locking of the condyle, Radiographic Features
weakness of the joint while yawning, click on opening, Punched out radiolucency can be seen on the condylar
excessive excursion of the condyle, lax capsule, over cartilage. Severe destruction of the cartilage can also be
stretched ligament, sclerosing agent, 5 percent sodium seen in some areas.
psylliate.
Gross examination of the articular cartilage reveals white
GOUT chalky deposits. This deposit is also often found in the
It is a chronic metabolic disorder characterized by acute synovial and adjacent tissues. The cartilage may be
exacerbations of joint pain and swelling associated with fragmented.
an elevated blood uric acid and deposition of crystals of Early findings in gout are crystalline deposits of uric
monosodium urate. acid which are diffuse in nature.
Microtophi: This is followed by small accumulations Symptoms: Facial pain, limitation of motion and deviation
(microtophi) with surrounding granulomatous foreign towards the affected side.
body reaction, which contains pleomorphic, non-nucleated
652 Signs: Crepitus, preauricular swelling, enlarged joint with
and foreign body giant cells.
effusion and local tenderness.
Management
Diet should be low in uric acid and fat, i.e. sweetbread,
Loose bodies: These are rounded, irregularly shaped and
meat, extract peas, beans.
variable sized radiopaque structure in the joint.
Increased elimination of uric acid by uricosuric agents
There is also widened joint space, and condylar head
like colchicine 0.5 mg every 2 hourly, to a maximum of
irregularities.
6 mg in 24 hours.
Points to Remember
Elevated blood uric acid, acute gouty arthritis, chronic There is nodule of loose cartilage in the joint space. There
tophaceous gout, excruciating pain, stiffness, tophi, is also presence of inflamed synovial membrane.
punched out radiolucency on the condylar cartilage, white Cartilage is consist of hyperchromatic and binucleated
chalky deposits, fragmented cartilage, crystalline deposits chondrocytes.
of uric acid, microtophi, uricosuric agents, colchicine.
Management
Surgery: These bodies, if symptomatic should be removed.
SYNOVIAL CHONDROMATOSIS Removal of metaplastic foci and synovectomy are the
It is also called chondometaplasia. It is a benign chronic preferred treatment.
progressive metaplasia that will not resolve spontaneously.
Points to Remember
Although it is non-neoplastic, it may resemble a malignant
condition histologically. Chondometaplasia, metaplasia of mesenchymal cell,
metastatic foci, trauma, facial pain, crepitus, preauricular
Pathogenesis swelling, loose bodies, nodule of loose cartilage,
It denotes the condition whereby a cartilaginous focus inflamed synovial membrane, hyperchromatic and
develops within the synovial membrane of the joint. binucleated chondrocytes surgery.
This is generally believed to occur through metaplasia of
mesenchymal cell rests in the underlying connective tissue
of the membrane. TEMPOROMANDIBULAR JOINT
There is formation of metastatic foci. Eventually, they DYSFUNCTION
are detached from the affected membrane and become
The syndrome of signs and symptoms of TMJ is termed
cartilaginous mobile bodies within the joint cavity. Many
as temporomandibular joint dysfunction (TMD). In TMD
of these cartilaginous foci then undergo calcification.
affected parts are teeth, jaws, joints and muscles.
These joint bodies acquire a perichondrium, which
enables them to grow by proliferation of chondrocytes. Clinical Features
Trauma may be a predisposing factor, others factors are
Age and sex distribution: It is seen in women as compare
malocclusion, occlusal habits, and subluxation or tension
to men. It is more common in middle age group.
sites.
Symptoms: Patient complaint of pain at the preauricular
Clinical Features area. This pain may get radiated to temporal, frontal or
Age and sex distribution: Female to male ratio is 3:1 with occipital areas. Pain may be presented as headache, tinnitus
greatest incidence at 40 to 60 years of age. (ringing in ear), otalgia (pain in ear) or toothache.
Site: This disease commonly affects large joint like knee, Signs: Palpation of TMJ may evoke pain. Pain is also
elbow, hip and shoulder. TMJ can also be involved. present on the mandibular movements.
Myospasm: Muscle splinting can lead to involuntary 9. Greene CS, Laskin DM. Long-term evulation of treatment
muscle contraction called as myospasm. for myofascial pain dysfunction syndrome: A comparative
study. J Am Dent Assoc. 1983;107:235-8.
Myofascial trigger point: These are the circumscribed 10. Gynther GFW, Tronje G, Holmlund AB. Radiographic 653
area within the muscle which causes referred pain on changes in the temporomandibular joint in patient with
palpation and it may be constant source of deep pain. generalized osteoarthritis and rheumatoid arthritis. Oral Surg
Oral Med Oral Pathol Oral Radiol Endod. 1996;81:613-8.
Radiological Features 11. Hall RE, Orbach S, Landesberg R. Bilateral hyperplasia of
There is widened joint space, anteriorly displaced meniscus. coronoid process: a report of two cases. Oral Surg Oral Med
Oral Pathol. 1989;67;141-5.
Osteophytes: These are irregular joint surface with
12. Izumi M, Isobe M, Toyama M, et al. Computed tomographic
protuberance.
features of bilateral coronoid process hyperplasia with
special emphasis of patents without interference between
Management
the process and the zygomatic bone. Oral Surg Oral Med
Conservative treatment: There should rest to the join, Oral Pathol Oral Radiol Endod. 2005;99:93-100.
application of cold or heat, occlusal splint and physical 13. Karlis V, Glickman RS, Zaslow M. Synovial chondromatosis
therapy. of temporomandibular joint with intracranial extension
Oral Surg Oral Med Oral Pathol Oral Radiol Endod.
Surgical management: It is done in severe cases of TMD. 1998;86:664-6.
14. Kleinman HZ. Gout of the TMJ: Oral Surg Med Oral Pathol.
Points to Remember 1969;27:281-2.
Headache, tinnitus, otalgia, toothache, myospasm, 15. Manganello Souza LC, Mariani PB. Temporomandibular
myofascial trigger point, osteophytes, conservative joint ankylosis: a report of 14 cases. Int J Oral Maxillofac
treatment, surgical management. Surg. 2002;32:24-9.
16. Matheus RA, Ramos-Perez FM, Menezes AV. The
relationship between temporomandibular dysfunction and
BIBLIOGRAPHY head and cervical posture, J Appl Oral Sci. 2009;17(3):204-
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radiological study. Dentomaxillfac Radiol. 2000;29:286-90. 1968;26:473-7.
2. Antoniades K, Hadijipetrou L, Antoniades V, et al. Bilateral 18. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and
bifid mandibular condyle: Oral Surg Oral Med Oral Pathol maxillofacial pathology, 3rd edn, Saunder Elsevier, 2009.
Oral Radiol Endod. 2004;97:535-8. 19. Palton DW. Recurrent subluxation of TMJ in psychiatric
3. Ardekian L, Faquin W, TRoulis MJ, et al. Synovial illness. Brit Dent J. 88:153(4):141-4.
chondromatosis of the temporomandibular joint: report of 20. Petito AR, Bennett, J, Assael LA, et al. Synovial
case and analysis of eleven cases. J Oral Maxillofac Surg. chondromatosis of the temporomandibular joint. Varying
2005;63:941-7. presentation of 4 cases. Oral Surg Oral Med Oral Pathol
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to perinatal trauma: J Oral Surg. 1977;35:578-82. 21. Sano T, Westesson PL, Larheim TA, et al. Osteoarthritis
5. Cacoppi JT, et al. Condyle destruction concomitant with and abnormal bone marrow of the mandibular condyle.
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Oral Surg Oral Med Oral Pathol. 1968;25:919-21. 1999;87:243-52.
6. Chidzonga MM. Temporomandibular joint ankylosis 22. Sarma UC, Dave PK. Temporomandibular joint ankylosis:
a review of thrity two case. Br J Oral Maxillofac Surg. An Indian experience. Oral Surg Oral Med Oral Pathol.
1999;14:136-8. 1991;72:660-4.
7. Dijkgraaf LC, Spijkervet FK, de Bont LG. Arthroscopic 23. Slootweg PJ, Muller H. Condylar hyperplasia: A clinico-
finding in osteoarthritis temporomandibular joint. J Oral pathological analysis of 22 cases. J Maxillofac Surg.
Maxillofac Surg. 1999;57:255-68. 1986;14:209-14.
8. Eskanu V, Behnia H, Javadi H, et al. Histopathological 24. Stefanou EP, Fanourakis IG, Vlastos K, et al. Bilateral bifid
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25. Wiberg B, Wanman A. Signs of osteoarthrosis of 26. Yatani H, Minakuchi H, Matsuka Y, et al. The long term
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654 Radiol Endod. 1998;86:158-64.
1. Following are the inflammatory disturbances of TMJ, 3. Resorption of articular eminence and sclerotic
except: underlying bone seen in:
a. Traumatic arthritis a. Osteoarthritis b. Rheumatoid arthritis
b. Osteoarthritis c. Costen syndrome d. Ankylosis
c. Ankylosis 4. Rose Waller test is done to investigate:
d. Rheumatoid arthritis a. Ankylosis b. Rheumatoid arthritis
2. Which one of the following is associated with extra- c. Hypermobility d. Both b and c
articular disturbances of TMJ: 5. Bird face appearance is the clinical feature of :
a. Costen syndrome b. Trotter’s syndrome a. Unilateral ankylosis b. Hypermobility
c. MPDS d. Both a and c c. Bilateral ankylosis d. Both a and c
27 Chemical and
Physical Injuries
Chapter Outline
Â Linea alba Â Resorption of teeth

Â Habitual cheek or lip biting • External resorption
Â Traumatic ulcer • Internal resorption
Â Electrical and thermal burns Â Hypercementosis
Â Anesthetic necrosis Â Cementicles
Â Chemical burns Â Bruxism
Â Smoker melanosis Â Traumatic lesion due sexual habit
Â Drug induced discoloration of oral mucosa Â Oral piercing and other body modification
Â Cutright lesion Â Fracture of teeth
Â Traumatic sequestration Â Amalgam tattoo
Â Methamphetamine abuse lesion Â Bismuthism
Â Submucosal hemorrhage Â Plumbism
Â Oral lesion as complication to anti-neoplastic therapy Â Mercurialism
(non-infectious) Â Argyria
Â Cervicofacial emphysema Â Arsenism
Â Myospherulosis Â Auric stomatitis
Â Attrition Â Inflammatory fibrous hyperplasia
Â Abrasion Â Inflammatory papillary hyperplasia
Â Erosion Â Epulis granulomatosum
Â Abfraction Â Nodular fasciitis
Â Dentinal sclerosis Â Uremic stomatitis
Â Secondary and tertiary dentin Â Traumatic keratosis
Â Bisphosphonates associated osteonecrosis
LINEA ALBA Etiology

Linea alba refers to the line of keratinization, found It occurs due to variation in dietary and oral hygiene
on the buccal mucosa parallel to the line of occlusion practice, frequent frictional contact with food and teeth.
expanding to a triangular area inside each labial commis Effect of smoking, food texture and other environmental
sure. irritants can cause linea alba.
HABITUAL CHEEK OR LIP BITING

It is also called Morsicatio buccarum. The Morsicatio
656 comes for Latin word morsus meaning bite.
Superficial lesions produced by frequent and repeated
rubbing, sucking or chewing movements that abrade the
surface of a wide area of lip or cheek mucosa without
producing discrete ulceration.
Etiology
Unconscious nervous habits, uncontrolled tongue thrusting
and neuromuscular disorders such as tardive dyskinesia.
Occlusal discrepancies, rough tooth surface, stress and
anxiety can also lead to habitual cheek biting.
Figure 27.1 Increase line of keratinization seen in linea alba Clinical Features
Age and sex distribution: It can occur at any age with no
Clinical Features sex predilection. It is usually occur on the buccal mucosa
Location: Common sites are buccal mucosa at line of at the level of occlusion.
occlusion and where the mucosa overlies the bone as in Appearance: Usually there is opaque white appearance
hard palate and gingiva. and is homogenous. In some cases, there is macerated and
reddened area usually with patch of partly detached surface
Appearance: It is white line extending horizontally from
epithelium (Fig. 27.2).
commissures to most posterior teeth (Fig. 27.1).
Palate and gingiva appear whiter than the adjacent Sign and symptoms: It may have sharply delineated
mucosa of the soft palate and alveolar gingiva, buccal and borders or in some cases it may be poorly outlined. In some
lingual sulcus. It is more prominent in people with little cases, contused margins present with transient whitish
overjet of molars and premolars. tags of necrotic tissue around the ulcer. It feels rough to
examiner’s fingers. Area becomes thickened, scarred and
Histopathological Features paler than surrounding mucosa.
Epithelium is keratinized under normal conditions and Morsicatio labiorum: When lesion of nibbling presents
exhibits stratum granulosum, well developed rete-pegs on labial mucosa it is called morsicatio labiorum.
and densely collagenous lamina propria that merges with
periosteum.
Increased thickness or hyperorthokeratosis is seen.
Sometime intracellular edema of the epithelium and mild
chronic inflammation of underlying connective tissue can
occur.
Management
No treatment is required for this condition.
Points to Remember
Buccal mucosa at line of occlusion, white line
extending horizontally from commissures, keratinized,
stratum granulosum, well developed retepegs, hyper
orthokeratosis, sometime intracellular edema of the
Figure 27.2 Habitual cheek biting showing lesion in
epithelium.
retromolar area
Chemical and Physical Injuries
Morsicatio linguarum: When lesion presents on tongue it Clinical Features

is called morsicatio linguarum.
Age and sex distribution: It occurs in persons of any age,
Histopathological Features with equal frequency in both the sexes. 657
Long established lesion may be associated with areas of Location: It may involve any region of the mouth but is
keratosis and increased thickness of epithelium. common on tongue, in mucobuccal fold, gingiva and palate.
There may be hyperorthokeratosis, hyperparakeratosis, Appearance: The appearance of the traumatic ulcer varies
and acanthosis. Depending upon degree of irritation, the markedly depending on the site of the injury, the nature and
underlying submucosa may show an inflammatory cell severity of trauma and the degree of secondary infection
infiltrate. In some cases clusters of vacuolated cells are present.
present in the superficial portions of the prickle cell layer.
Single uncomplicated ulcer: The most common variety
Management of traumatic ulcer is single uncomplicated ulcer which
Small doses of diazepam 5 to 10 mg at bed time for the generally is of moderate size (from several millimeters to
management of neurological disorders. a centimeter or more in diameter), round, oval or elliptical
Plastic occlusal night guard to control the habit of in shape and flat or slightly depressed. Its surface consists
cheek biting should be used. of a serosanguinous or grayish serofibrinous exudate.
It may be composed of a grayish necrotic slough which
Points to Remember when removed, reveals a red raw tissue base. The lesion
is surrounded by a narrow border of redness. There is
Morsicatio buccarum, opaque white appearance, sharply
tenderness and pain in the area of lesion and it will be
delineated borders, transient whitish tags, Morsicatio
helpful to identify the cause of lesion. It may persist for
labiorum, Morsicatio linguarum, keratosis and increased
few days and may last for weeks. Ulcer on vermilion border
thickness of epithelium, hyperorthokeratosis, hyper
may have crusted surface due to absence of saliva. Painful
parakeratosis, acanthosis, diazepam, plastic occlusal
regional lymphadenopathy also occurs.
night guard.
Complicated ulcer: In still other instances, the traumatic
lesions are large and irregular. These are the result of
TRAUMATIC ULCER unusually severe traumatic episodes, such as a blow or a
It is a frequently encountered ulcerative lesion of mouth. fall and are often accompanied by considerable edema,
The common term used to denote a traumatic ulcer is inflammation and swelling of the neighboring tissues (Fig.
decubitus ulcer, tropic ulcer, neutrons-tropic and Bednar’s 27.3).
ulcer.
Causes
Mechanical or physical: It includes biting, sharp or mal
posed teeth or roots, sharp food, stiff tooth brush bristles,
sharp margins of crown, fillings, denture, orthodontic
appliances and faulty instrumentation.
Chemical: It results from caustic substances such as silver
nitrate, phenol, TCA, formocresol, eugenol, eucalyptus oil,
phosphorus and acetylsalicylic acid.
Thermal: Excessive heat in the form of hot fluid or food,
on rare occasion the application of the dry ice, reverse
smoking and hot instrumentation.
Electrical current application to the oral tissues may result
in destruction and consequent ulceration, e.g. galvanism.
Others: Radiation burns, selfinflicted and iatrogenic. Figure 27.3 Soft tissue traumatic ulcer on upper lip
Infected ulcer: The infected ulcer is larger more irregular Vascular connective tissue deep into the ulcer can
and more protruding than the noninfected one and often become hyperplastic which may results in surface elevation.
it is covered with a thick layer of necrotic slough through Atypical eosinophilic ulceration shows deeper
658 which purulent exudate may be observed. tissue replaced by highly cellular proliferation of large
Eosinophilic ulceration: This type of ulceration appear lymphoreticular cells.
similar to traumatic but underlying proliferative granulation Management
tissue can results raised lesion.
Usually the simple and uncomplicated traumatic ulcer heals
Radiation burns: Acute reaction occurs during the uneventfully in 5 to 10 days after onset and even without
course of radiotherapy due to direct tissue toxicity. Ulcer treatment. However in the presence of secondary infection
resolves over several weeks following the completion of or repetitive trauma longer healing period is required.
therapy. Chronic complication or late radiation reaction Causative agent should eliminate.
occurs due to change in the vascular supply, fibrosis in Persistent ulcer: Triamcinolone acetonide in emollient
connective tissue and muscle and change in cellularity base before bed time and after meals. Chlorhexidine
of tissues. Mucositis occurs when the rate of epithelial mouth wash or even topical local anesthetic should be
growth and repair are affected by radiation, resulting in given to relive acute symptoms of pain. A persistent
epithelial thinning, erosion and ulceration. The first sign ulcer, not responding to the foregoing regimen, should be
of mucositis may be whitish appearance of the mucosa surgically excised and the entire tissue must be sent for
due to hyperkeratinization and intraepithelial edema or histopathological examination.
a red appearance due to hyperemia. Pseudomembrane
formation, most likely represents ulceration with fibrinous Points to Remember
exudate, oral debris and microbial component. Radiation Decubitus ulcer, tropic ulcer, single uncomplicated
has more marked effect on rapidly proliferating epithelium ulcer serosanginous or grayish serofibronous exudate
and therefore, mucositis involves the nonkeratinized with narrow border of redness, complicated ulcer consi
mucosa first. derable edema, inflammation and swelling, infected
Histopathological Features (Fig. 27.4) ulcer thick layer of necrotic slough, eosinophilic
ulceration, radiation burns, fibropurulent membrane,
Simple ulcers are covered by fibro-purulent membrane that hyperplasia with or without hyperkeratosis, highly
consists of fibrin intermixed with neutrophils. cellular proliferation of large lymphoreticular cells.
Adjacent surface epithelium may show hyperplasia
with or without hyperkeratosis. Inflammatory infiltrate
consist lymphocytes, neutrophils. ELECTRICAL AND THERMAL BURNS
Burns cause transient non-keratotic, white appearance
of the mucosa which is attributed to superficial
pseudomembrane composed of coagulated tissue with an
inflammatory exudate (saliva protects oral mucosa).
Chronic mild burns results in keratotic white lesions
while intermediate burns cause localized mucositis and
severe burns coagulate the surface of the tissue and produces
diffuse white lesions. If coagulation is severe, tissue can
not be scraped off easily leaving raw and bleeding painful
surface.
Clinical Features
Electrical burns can be contact type (electrical current
passing through the body from the point of contact to the
Figure 27.4 Traumatic ulcer showing proliferation with ground site) or arc type (saliva act as conducting medium
vascular connective tissue and an electrical arc flow between the electrical source and
mouth). Electrical burns usually occur in children younger Management

that 4 years of age with oral commissures is commonly
Tetanus immunization: It should be done in patient with
involved. Edema is developed which is followed by necrosis
electrical burn. 659
of affected area after 4 days. Bleeding may occur due to
exposure of vasculature which should be closely monitored Macrostomia prevention technique: Splinting is maintain
in this case. After some time adjacent teeth become non for 6 to 8 months to ensure proper scar maturation.
vital with or without necrosis of alveolar bone. There may Antibiotics and corticosteroid are given for control of
be contracture of mouth opening after healing. infection and inflammation.
Thermal burns can be cause by hot food, beverages.
There is pain which last for short duration. It is seen anterior Points to Remember
1/3rd of tongue and palate. It may produce coagulation
• E lectrical burn: Transient nonkeratotic, white
necrosis of superficial tissue that appears whitish. In
appearance of the mucosa, contact type, arc type,
some cases, there may be frank ulceration and stripping
bleeding may occur due to exposure of vasculature,
of mucosa. Red area is tender to painful; it may blanch on
teeth become nonvital with or without necrosis of
pressure and there is bleeding on manipulation. Surface
bone.
layer of epithelium is desquamated. It can be manage by
• Thermal burns: Coagulation necrosis of superficial
systemic analgesics and topical hydrocarbon in emollient
tissue, red area is tender, pizza burns, CO2
base.
burns (snow) (dry ice), frost bite of lip, popsicle
Pizza burns: Central palatal burns, whitish gray or panniculitis, tetanus immunization, macrostomia
ulcerated lesions of the middle third of the hard palate. prevention technique, antibiotics and corticosteroid.
These also present superficial necrosis and ulceration due
to combination of heat of the cheese and its adhesion to
blister epithelium (Fig. 27.5).
ANESTHETIC NECROSIS
In some cases after administration of local anesthesia there
CO2 burns (snow) (dry ice): Children affected commonly.
is ulceration and necrosis at the site of injection. This is
Tongue and lip are most common sites. Frost bite of lip—
cause by localized ischemia, which may occur due to faulty
it is also called Popsicle panniculitis. There is persistent
technique.
swelling and redness. It occurs due to prolonged contact
of ice cream and other frozen confectionaries or very cold Clinical Features
metal, glass object, with child’s lip. Epithelium becomes
dry and rougher than surrounding tissues. Location: Hard palate is common site for anesthetic
necrosis. It appear after several days after injection.
Appearance: Well circumscribed are of ulceration develop
at the site of injection.
Management
As such no treatment is needed as ulceration heal
automatically.
Points to Remember
Necrosis hard palate, well circumscribed are of
ulceration.
CHEMICAL BURNS
Burns due to caustic chemical agents will produce
Figure 27.5 Pizza burn seen in palate coagulation necrosis of the epithelium with subsequent
inflammation. Large number of chemical and drugs comes

in contact with the oral tissue. Many time patient place the
chemical like aspirins, sodium perborate to get relief from
660 oral problems.
Causes
• Aspirin and aspirin–containing compounds
• Tooth-ache drop burns
• Phenol
• Ethyl alcohol burns
• Vitamin C tablet
• Acid burns
• Ingestion
• Hydrogen peroxide
• Silver nitrate. Figure 27.6 Sloughing of tissue in chemical burn
Causes
Aspirin and aspirin: Containing compounds: it occur Clinical Features
when it is kept in mucobuccal fold to relive toothache. It
Appearance: Irregularly shaped, white pseudomembrane
is available as tablet as well as powder form.
covered lesion develops. The lesion is usually painful.
Tooth-ache drop burns: Such drops contain creosote, Gentle lateral pressure causes the white material to slide
guiacol and phenol derivatives. Extensive mucosal necrosis away exposing an exquisitely painful central ulceration and
with underlying bone loss has been seen in patient using more adherent patches of white material in the periphery
phenol derivative. (Fig. 27.6).
Phenol: It has been use as cavity sterilizing agents and Aspirin burns are focal with sharply delineated borders.
cauterizing material. Diffuse border may present if the burn is more extensive.
Ethyl alcohol burns: Topical application of ethyl alcohol In the toothache drop burn there may be sloughing of oral
solution which results in sloughing of the oral mucosa. mucosa.
Some of the mouth wash contain 25 percent ethyl alcohol. Cotton roll burns: It is also called cotton roll stomatitis.
Vitamin C tablet: In some cases, it can causes burns of Sometime when cotton is used, caustic material can leak in
oral mucosa. it and it held against the mucosa for longer period of time.
Acid burns: If the rubber dam placement is ineffective This will lead to injury to mucosa. In some cases mucosa
then acid used to etch tooth surface may come in contact can adhere to dry cotton role which will results in striping
with the oral mucosa. of the epithelium in the area.
Caustic material injected into bone during endodontic
Ingestion: Chemical burns of the oral cavity can result procedures can result in bone necrosis, pain and perforation
when the children mistakenly drink household chemical into the soft tissue.
and with suicide attempts by ingestion of caustic material.
Hydrogen peroxide: It is use for the treatment of Histopathological Features
periodontitis. Concentration higher than 3 percent will
It causes coagulation necrosis of the epithelium. The
result in mucosal damage.
upper layer shows a homogenous appearance with loss of
Silver nitrate: It is the treatment modality of aphthous structure and failure of nuclei to take stain.
ulceration. It will also results more mucosal damage so Inflammatory cell infiltrates with the underlying
its use should be discouraged. In some cases their use connective tissue can be seen. Hyperkeratosis and
can lead to necrotic defect in the oral cavity. acanthosis of oral mucosa is present.
Management
Palliative care with a topical anesthetic or a bland coating
suspension is used. These are accomplished by the use 661
of Orabase gel or palliation may result form anesthetics
effects of an antihistaminic mouth rinse in combination
with kaopectate. Painful lesion heals quickly.
Points to Remember
White pseudomembrane covered lesion, painful,
sharply delineated borders, cotton roll burns, cotton
roll stomatitis, by bone necrosis, pain and perforation,
coagulation necrosis, inflammatory cell infiltrates,
hyperkeratosis, acanthosis, topical anesthetic.
Figure 27.7 Smoker melanosis showing pigmentation
SMOKER MELANOSIS
Oral pigmentation increase in heavy smokers. Melanins
have ability to bind to noxious substance. Exposure
to polycyclic amines like nicotine and benzpyrene has
stimulated melanin production by melanocytes which are
also bind strongly with nicotine. This may be protective
mechanism of body to avoid harmful effect of tobacco
smoke.
Clinical Features
Age and sex distribution: It is seen in adults with female
predilection. The reason for female predilection occur due
to synergistic effect of female sex hormone when combined
with smoking.
Location: It is seen on anterior facial gingiva in cigarette
smoker and commissural and buccal mucosa in case of Figure 27.8 Smoker melanosis showing melanin pigmentation
pipe smokers. Reverse smoker show it in hard palate.
Appearance: There is areas of melanin pigmentation Points to Remember
which appear black or blue in color (Fig. 27.7). Nicotine and benzapyrene, anterior facial gingiva,
melanin pigmentation which appear black or blue,
Histopathological Features increase melanin pigmentation of basal cell layer of the
There is increase melanin pigmentation of basal cell surface epithelium.
layer of the surface epithelium (Fig. 27.8). There is also
collection of incontinent melanin pigmentation in the
DRUG INDUCED DISCOLORATION
superficial connective tissue
OF ORAL MUCOSA
Management Oral mucosal discoloration occurs due to much medication.
Biopsy should be done if change occur like surface elevation This can occur due to phenolphthalein, minocycline,
otherwise pigmentation cease after discontinuation of tranquilizers, antimalarial medication, estrogen, chemo
smoking. therapeutic agents.
CUTRIGHT LESION
It is also called reactive osseous and chondromatous
662 metaplasia. There is cartilaginous metaplasia secondary to
chronic denture trauma.
Clinical Features
Sign and symptoms: There is extremely tender localized
area on the alveolar ridge which can be having local
enlargement. Knife edge like crest will present as
susceptible for this lesion
Location: It is common on posterior mandible and rarely
seen in maxillary ride and anterior mandibular ridge.
Figure 27.9 Pigmentation of lower lip occur due to drug Histopathological Feature
There is mass of hypercellular periosteum which blends
Clinical Features into areas of osseous and chondromatous tissue.
Appearance: There is diffuse melanosis of the skin, Bone and cartilage show hypercellularity, pleomor-
mucosal surface (Fig. 27.9). phism, nuclear hyperchromatism and binuclear and multi
Sex: Females are more commonly affected due to nucleated cells.
interaction with sex hormones. Management
Phenolphthalein pigmentation: There are numerous Recontouring of thin mandibular ridge with graft material
small, well circumscribed areas with hyperpigmentation on will alleviated the symptoms.
the skin.
Minocycline pigmentation: This cause discoloration of Points to Remember
the bone and teeth. Affected bone is dark green which is Reactive osseous and chondromatous metaplasia,
presented as bluegray discoloration of the translucent oral extremely tender localized area, knife edge like crest,
mucosa. There is linear band above attached gingiva. hypercellular periosteum which blends into areas of
Pigmentation due to antimalarial drug: There is blue osseous and chondromatous tissue, hypercellularity,
black discoloration which is limited to hard palate. pleomorphism, nuclear hyperchromatism, recontouring
of thin mandibular ridge.
Estrogen pigmentation: It results in diffuse brown
melanosis of the skin.
TRAUMATIC SEQUESTRATION
Management
Discontinuation of drug will give reversal of the lesion. It is also called oral ulceration with bone sequestration,
spontaneous sequestration. It usually occurs in site where
Points to Remember bone prominence is covered by thin mucosal surface.
It may cause by loss of blood supply by periosteal
Phenolphthalein, minocycline, tranquilizers, phenol microvasculature to the bone which lead to focal bone
phthalein pigmentation shows small, well circum necrosis and sequestration.
scribed hyperpigmentation, minocycline pigmentation
shows dark green bone with bluegray discoloration Clinical Features and Radiological Features
of the translucent oral mucosa. pigmentation due to
Location: It is seen on lingual surface of posterior mandible
antimalarial drug is blue black, estrogen pigmentation is
along the mylohyoid ridge. Exostosis is often involved
diffuse brown melanosis.
with mandibular tori most commonly affected.
Appearance: Mucosa show ulceration for long period Parasitosis (formication): This is neurosis that produces
of time. Most cases are unilateral but some cases may be sensation of snakes and insects crawling on or under the
bilateral. skin. Patient will try to remove the insect by picking at the
skin with fingernail resulting in traumatic injury. 663
Symptoms: There is pain which is of variable intensity.
Speed bumps, meth sores or crank bugs: This term used
Radiological features: Occlusal radiograph may show
for facial appearance of the patient after factitial damage
faint radiopaque mass.
due to fingernail injury.
Histopathological Features Rampant dental caries: This occur due to drug related
The sequestra consist of well organized lamellar bone. xerostomia and poor oral hygiene of the patients.
There is also loss of osteocytes for their lacunae. Bacterial
colonization with peripheral resorption can also be seen. Management
Use of local anesthesia with epinephrine will lead
Management hypertensive crisis in this patient. So oral physician should
Surgical removal of dead bone results in healing. be very careful while dealing the patients.
Management of rampant dental caries and xerostomia
Points to Remember should be done accordingly.
Sequestration, spontaneous sequestration, lingual surface
of posterior mandible, ulceration for long period of time, Points to Remember
show faint radiopaque mass, well organized lamellar CNS stimulant drug, chalk, crank, crystal, glass, ice,
bone, bacterial colonization, peripheral resorption, meth and speed insomnia, aggressiveness, hyperactivity,
surgical removal. psychological addiction, violent behavior, parasitosis
(formication), sensation of snakes, speed bumps, meth
sores or crank bugs, rampant dental caries.
METHAMPHETAMINE ABUSE LESION
Methamphetamine, i.e. meth is a CNS stimulant drug. This
drug is used in case of narcolepsy and attention deficit SUBMUCOSAL HEMORRHAGE
hyperactivity. As this drug give greater energy, euphoria
This type of hemorrhage occurs after minor trauma.
and more physical ability, abuse of the drug is increasing.
This drug can be smoked, snorted, injected or taken
orally. Effect of this drug is around 12 hours and many Types
people used this drug more than 2 to 3 times per day. This • P etechiae: These are minute hemorrhage in the skin,
drug has many name like chalk, crank, crystal, glass, ice, mucosa or serosa
meth and speed. • Purpura: If large area is involved it is termed as
purpura
Clinical Features • Ecchymosis: Accumulation greater than 2 cm is
Age: Most of the user of this drug is between the ages of called ecchymosis
19 to 40 years. • Hematoma: If accumulation produce mass it is
termed as hematoma. It is caused by blunt trauma.
Short-term effect: It includes insomnia, aggressiveness,
hyperactivity, tachycardia, xerostomia, tremor and vomit
ing. Clinical Features
Long-term effect: Strong psychological addiction, vio Appearance: These are nonblanching flat or elevated
lent behavior, anxiety, confusion, depression, paranoia, area. Color of this lesion varies from red or purple to blue
delusion, mood change. to black.
There is also cardiovascular, CNS, hepatic gastrointestinal, Location: It is most commonly seen on labial or buccal
renal and pulmonary disorders. mucosa.
Sign and symptoms: Pain may be present. Hematoma discomfort and difficulty in intake of food. The surface
formation associated with surgical implant may present epithelium may show ulceration or atrophy. Later changes
with damage to soft tissue. show an inflammatory cell infiltrates in the submucosa
664 with an attenuated spinous cell layer (Fig. 27.11).
Antral hematoma: Patient with antral hematoma may
have nasal bleeding, headache, malar swelling, facial Pseudomembrane formation: After this the
paresthesia and hyposmia. mucous membrane begins to break down and leads
to the formation of white to yellow pseudomembrane
No treatment is for smaller lesion, if the lesion is large Taste buds: Dose in therapeutic range can cause extensive
surgical exploration with isolation and repair of damaged degeneration of the histological architecture of taste buds.
vessels. Patient usually notices loss of taste during 2nd or 3rd week
after radiotherapy. Bitter and acid flavors are severely
Points to Remember affected when the posterior third of the tongue is irradiated
Nonblanching flat or elevated area, pain, antral
hematoma.
ORAL LESION AS COMPLICATION TO

ANTI-NEOPLASTIC THERAPY (NON-
INFECTIOUS)
There is always death of normal cells occur when patient
undergoing radiation or chemotherapy. Both radiation
and systemic chemotherapy can cause significant oral
problems.
Painful mucositis and dermatitis are the most frequent
manifestation of radiation injury. Oral lesion seen in almost
all patient of radiation therapy of head and neck, around 75
percent of patient getting bone marrow transplantation and
chemotherapy. Figure 27.10 Dermatitis in patient after receiving radiotherapy
Clinical Features
Dermatitis: Excessive exposure will cause dermatitis.

Repeated exposures have a cumulative effect (the tissue
does not return to original state due to irreparable damage).
There is dryness, erythema, thickening, desquamation and
cracking of hands may also occur. Epilation can be cause
by radiation. It is often seen in association with dermatitis.
Hair loss can be permanent. Radiation dermatitis can also
become chronic and is characterized by dry, smooth, shiny,
atrophic, necrotic and ulcerated area (Fig. 27.10).
Mucositis: It is redness and inflammation of the mucous
membrane and it is commonly seen during radiation therapy
as well as patient receiving chemotherapy. It occurs due to
death of the cells of oral mucous membrane. By the end
of therapy, the mucositis is very severe, with maximum Figure 27.11 Mucositis in patient receiving radiotherapy
will show fibro atrophy after radiation. Radiation can also

affect developing facial bone resulting in micrognathia.
Osteoradionecrosis: It is the most serious clinical 665
complication that occurs in bone after irradiation. When
the changes in a bone are so severe that bone death results,
the condition is known as osteoradionecrosis. Bone
infection occurs either due to radiation induced breakdown
of the oral mucous membrane, mechanical damage to the
weakened oral mucous membrane, through periodontal
lesion or from radiation caries. Osteoradionecrosis is more
common in mandible than in maxilla due to rich vascular
supply of the maxilla.
Management
Figure 27.12 Pseudomembrane formation in patient who are Mucositis: A low cost salt and soda has been effective
receiving radiotherapy in management of mucositis. Cryotherapy (placement of
ice chips in the mouth 5 minutes before chemotherapy
and taste of salt and sweet are affected when anterior third and continuous 30 minutes after chemotherapy) has been
of tongue is irradiated. shown to reduce prevalence and severity of mucositis.
Xerostomia: The parenchymal component of salivary Xerostomia: Avoid use of tobacco product as it reduces the
gland is more radiosensitive (parotid gland is more salivary secretion. Use of salivary substitute and systemic
radiosensitive than submandibular or sublingual gland). therapy like bethanechol and anetholtrithione can be used.
Exposure to radiation leads to injury of these parenchymal Loss of taste: Zinc sulfate supplements appear to be
cells leading to xerostomia. The extent of reduced salivary beneficial.
flow is dose dependent and it is zero when dose reaches
60 Gy. The mouth becomes dry, tender, and swallowing Osteoradionecrosis: Therapy include antibiotics, debride
is difficult and painful since the residual saliva also loses ment, irrigation and removal of disease of bone.
its normal lubricating properties. Because of small amount Points to Remember
of thick, viscous, acidic saliva such patient are prone to
Dermatitis, mucositis, pseudomembrane formation, loss
carries which is known as radiation carries. After months,
of taste, xerostomia, hemorrhage, trismus, osteoradio
the inflammatory response becomes more chronic and
necrosis, developmental abnormalities.
the glands demonstrate progressive fibrosis, adiposis,
loss of fine vasculature and concomitant parenchymal
degeneration, resulting in xerostomia. CERVICOFACIAL EMPHYSEMA
Hemorrhage: It occur secondary to thrombocytopenia These occur due to introduction of air into subcutaneous
which develop due to bone marrow suppression. Oral tissue or fascial spaces. This air may spread through
petechiae and ecchymosis are present which can be seen retropharyngeal and mediastinal areas.
on labial mucosa, tongue, and gingiva.
Trismus: Tonic muscle spasm with or without fibrosis of Causes
muscle of mastication may lead to trismus in the patient. • C ompressed air: Compressed air from air driven
Developmental abnormalities: Irradiation of teeth during handpiece can cause it
developmental stage retards their growth severely. Children • Difficult extraction: It can occur after difficult
receiving radiation therapy to the jaws may show defect in extraction
the permanent dentition such as retarded root development, • Increase intraoral pressure: This can occur due to
dwarfed teeth or failure to form one or more teeth. Pulp sneezing or blowing after oral surgical procedure.
Clinical Features
Sign and symptoms: There is soft tissue enlargement
666 due to presence of air in deeper tissue. Enlargement can
increase and spread due to edema and inflammation. There
is also pain, facial erythema, dysphagia, and dysphonia and
vision abnormalities.
Pneumoparotid: This occur when air enter parotid gland
duct, leading to enlargement of parotid gland. Stensen
duct has fold which seals as soon as pressure increase
intraorally. If there is more pressure increase this sealing
does not takes place and pneumoparotid can occur.
Hamman’s crunch: Cardiac auscultation will reveal
crepitus synchronous with heart beat in case of mediastinal
involvement Figure 27.13 Myospherulosis showing bag of marbles
appearance
Management
Broad spectrum antibiotics should be given. Management
Points to Remember Surgical removal of foreign material and associated tissue
should be done.
Soft tissue enlargement due to presence of air, pneumo
parotid, Hamman’s crunch.
Points to Remember
Swelling, pain, purulent discharge, black, greasy, tarlike,
MYOSPHERULOSIS granulomatous inflammatory response, bag of marbles
appearance.
It occurs due to placement of topical antibiotics in
petrolatum base jelly at surgical site.
Clinical Features ATTRITION

Location: It can occur at any site in the bone or soft tissue. It is the physiologic wearing away of teeth because of
In bone it occurs at previous extraction site usually in the tooth to tooth contact, as in mastication. It develops from
mandibular region. It can also be seen in paranasal sinus area. word attritum (action of rubbing against another surface). It
plays an important physiological role as it helps to maintain
Appearance: There is swelling which can be associated an advantageous crownroot ratio and gains intercoronal
with pain and purulent discharge. On exploration lesion is space of 1 cm, which facilitates third molar eruption.
black, greasy, tarlike material.
Types
• P hysiological attrition: Attrition which occur due to
There is dense collagenous tissue which is mixed with normal aging process, due to mastication.
granulomatous inflammatory response showing macro • Pathological attrition: It occurs due to certain
phage and multinucleated giant cells. abnormalities in occlusion, chewing pattern or due to
Bag of marbles appearance: In the connective tissue some structural defects in teeth.
there are cyst like space which contain brown to black
staining spherules. Spherules are surrounded by outer
membrane called parent body. This appearance is called Etiology Factors for Pathological Attrition
bag of marbles. Spherules represent red blood cells Development: Malocclusion and crowing of teeth, may
which are altered by medication. The dark color is due to lead to traumatic contact during chewing, which may lead
degradation of hemoglobin (Fig. 27.13). to more tooth wear.
Acquired: Due to extraction of teeth. Extraction causes

increased occlusal load on the remaining teeth, as the
chewing force for the individual remains constant.
667
Abnormal chewing habits: Parafunctional chewing habit
like bruxism and chronic persistent chewing of coarse and
abrasive food or other substances like tobacco.
Occupation: In certain occupations, workers are exposed
to an atmosphere of abrasive dust and cannot avoid it
getting into mouth.
Salivary factor can also play role in attrition.
Structural defect: In defects like amelogenesis imperfecta
and dentinogenesis imperfecta, hardness of enamel and
dentin is reduced and such teeth become more prone to
attrition. Figure 27.14 Attrition of the posterior teeth in mandibular
region
Clinical Features
Sex distribution: Men usually exhibit more severe attrition compensatory growth (dentoalveolar compensation) to
than women due to greater masticatory force. some degree.
Site: It may be seen in deciduous as well as permanent Dentoalveolar compensation: If attrition affecting the
dentition. It occurs only on occlusal, incisal and proximal occlusal surfaces of teeth has occurred, then reduction
surfaces of teeth. Severe attrition is seldom seen in primary in occlusal face height (vertical dimension of occlusion)
teeth, as they are not retained for any great period. Lingual and increase in the freeway space could be anticipated.
cusps of maxillary teeth and buccal cusps of lower posterior This may be further complicated by forward posturing of
teeth show most wear. mandible. It is often observed, however, that despite overall
Appearance: The first clinical manifestation of attrition tooth surface loss, the freeway space and the resting facial
is the appearance of small polished facet on a cusp tip or height appear to remain unaltered primarily because of
ridge and slight flattening of an incisal edge. dentoalveolar compensation. This is important with respect
to patient assessment. If restoration of worn teeth is being
Physiological attrition begins with wearing of the incisal planned then the extent of dentoalveolar compensation
edge of an incisor, which is followed by the palatal cusp would appear to determine the dentist’s strategy; defining
of maxillary molars and buccal cusp of mandibular the need to carry out measures such as crown lengthening,
molars. It commences at the time of contact or occlusion. to ensure the same vertical dimension of occlusion and
Physiological tooth surface loss results in a reduction, in freeway space.
both vertical tooth height and horizontal tooth width.
Radiological features: Smooth wearing of incisal and
Sign and symptoms: Due to slight mobility of teeth in their occlusal surfaces of involved teeth is evident by shortened
socket (which is a manifestation of resiliency of periodontal crown image (Fig. 27.15). Sclerosis of pulp chamber and
ligament) similar facets occur at contact points. When the canals is seen due to deposition of secondary dentin which
dentin gets exposed it generally becomes discolored, i.e. narrows the pulp canals. Widening of periodontal ligament
brown in color. There is gradual reduction in cusp height space and hypercementosis. In some cases there is loss of
and consequent flattening of occlusal inclined plane. There alveolar bone.
is shortening of the length of dental arch, due to reduction
in the mesiodistal diameter of teeth. Secondary dentin Management
deposition occurs (Fig. 27.14).
Treatment of patient depends upon degree of wear relative
Pathological attrition: If pathologically vertical dimen to the age of patient, etiology, symptoms and patient’s
sion of tooth has reduced then, there is the possibility of desire. Patient should be advised of any possible bruxist
Etiology
It is caused by use of abrasive dentifrices, horizontal tooth
668 brushing, and habitual opening of bobby pins. It may also
occur due to holding nails or pins between teeth, e.g. in
carpenters, shoemakers or tailors. Improper use of dental
floss and tooth picks.
Clinical Types of Abrasion

• Habitual abrasion
• Occupational abrasion
• Ritual abrasion
• Tooth brush injury
• Dental floss or tooth pick injury.
Figure 27.15 Wearing of tooth surface due to attrition

Clinical Types
Habitual abrasion: Habitual pipe smoker may develop
habits. The provision of one of three different sorts of abrasion on the incisal edges of lower and upper anterior
splints could be considered. A soft bite guard can help in teeth. Improper and habitual use of tooth prick or dental
breaking a bruxist habit or simply will protect the teeth floss can cause abrasion on the proximal surface of teeth.
during the bruxist habit.
A localized occlusal interference splint is designed to Occupational abrasion: It occurs when objects and
break the bruxist habit and can be worn easily during the instrument are habitually held between the teeth by people
day. A stabilization splint reduces bruxism by providing during working.
an ideal occlusion: it also enables the clinician to locate Ritual abrasion: It is mainly seen in Africa.
and record centric relation. In case of bruxism, use of night
Tooth brush injury (Fig. 27.16): It usually occurs on
guards may be effective in reducing attrition.
exposed surfaces of roots of teeth. It occurs due to back
Correction of malocclusion, stoppage of tobacco
and forth movement of brush with heavy pressure causing
chewing habit and restriction of diet to noncoarse food are
bristles to assume wedge shaped arrangement between
useful in avoiding attrition. Noncarious loss of tooth tissue
crown and gingiva. In horizontal brushing there is
may require treatment for sensitivity, esthetics, function
and space loss in the vertical dimension.
Points to Remember
Occlusal, incisal and proximal surfaces of teeth, small
polished facet on a cusp tip, physiological attrition,
dentin gets exposed, brown in color, pathological
attrition, dentoalveolar compensation, smooth wearing
of incisal and occlusal surface, widening of periodontal
ligament space, localized occlusal interference splint,
correction of malocclusion.
ABRASION
It is the pathological wearing away of tooth substance
through abnormal mechanical process. It is develop from
Latin word abrasum (means scrape off). Figure 27.16 Abrasion seen in cervical area of teeth
usually a ‘V’ shaped or wedge shaped ditch on the root at

Points to Remember
cementoenamel junction. It is limited coronally by enamel.
It is more commonly seen on left side of right handed Abrasive dentifrices, horizontal tooth brushing, habitual
persons and vice versa. Patient develops sensitivity as abrasion, occupational abrasion, ritual abrasion, tooth 669
dentin becomes exposed. The angle formed in the depth brush injury, ‘V’ shaped or ‘wedge’, dental floss or tooth
of the lesion as well as that of enamel edge is a sharp one. pick injury, demastication, radiolucency in cervical area
Cervical lesions caused purely by abrasion have sharply of teeth.
defined margins and a smooth, hard surface. The lesion may
become more rounded and shallow, if there is an element of EROSION
erosion present. Exposed dentin appears highly polished.
Exposure of dentinal tubules and consequent irritation of It is the loss of tooth substance by chemical process that
the odontoblastic processes stimulates secondary dentin does not involve known bacterial action. This word is
formation which is sufficient to protect the pulp from develop from erosum (corrode).
clinical exposure. Dissolution of mineralized tooth structure occurs upon
contact with acids. Erosion is a chemical process in which
Dental floss or tooth prick injury: Cervical portion of the tooth surface is removed in the absence of plaque.
proximal surfaces, just above the gingival margin, is
affected. Grooves on distal surface are deeper than on Types
mesial surface. • I ntrinsic: Erosion that occur due to intrinsic cause,
Demastication: When tooth wear accelerated by chewing e.g. gastroesophageal reflux, vomiting.
an abrasive substance between opposing teeth and • Extrinsic: Erosion occurring from extrinsic sources,
exhibits features of attrition as well as abrasion is term as e.g., acidic beverages, citrus fruits.
demastication.
Radiological features: It will show radiolucency in Etiology
cervical area of teeth (Fig. 27.17). Local acidosis in periodontal tissue from damage due to
traumatogenic occlusion.
Management
Chronic vomiting: Complete loss of enamel on lingual
Examination and modification of teeth cleaning habits will
surfaces of teeth through dissolution by gastric hydrochloric
be indicated.
acid. Vomiting can also occur in alcoholics, peptic ulcer,
Elimination of causative agent should be carried out.
gastritis, pregnancy and drug side effect.
Restoration, for esthetics and to prevent further tooth
wear. Acidic foods and beverages: Large quantities of highly
acidic carbonated beverages or lemon juice can produce
erosion. Most of the fruits and fruits juices have a low pH
and can cause erosion. Frequent consumption of carbonated
drinks, which are acidic in nature, may result in erosion of
teeth.
Anorexia nervosa: It induces chronic vomiting often after
bouts of uncontrolled eating that is interspersed between
periods of starvation, because of inner rejection of food.
Occupational: Workers involving in manufacturing of
lead batteries, sanitary cleaners or soft drinks can develop
erosion.
Risk factors: Following factors are assume as risk factor
for erosion. They are citrus fruits intake, soft drink more
than 6 per weeks, bruxism habits, vomiting and symptoms
Figure 27.17 Radiolucency in cervical area due to abrasion of gastroesophageal reflux disease.
Clinical Features ABFRACTION

Location: It occurs most frequently on labial and buccal It is also called stress lesion. It is derived from Latin ab
670 surfaces of teeth; sometimes may occur on proximal (away), fractio (breaking).
surfaces of teeth. Usually it is confined to gingival thirds
of labial surface of anterior teeth. Erosion may involve Causes and Mechanism
several teeth of dentition.
It has been suggested that the stress lesion or abfraction is
From extrinsic source, it causes erosion on labial and a consequence of eccentric forces on the natural dentition.
buccal surface and from intrinsic source; it causes erosion The theory propounds tooth fatigue, flexure and
on lingual or palatal source. deformation via biomechanical loading of the tooth
structure, primarily at the cervical region.
Appearance: It is usually a smooth lesion which exhibits
Cusp flexure causes stress at the cervical fulcrum and
no chalkiness. Loss of enamel often causes hypersensitivity
results in loss of the overlying tooth structure.
in teeth and may also trigger secondary dentin formation.
Loss of tooth substance is manifested by shallow, broad, Clinical Features
smooth, highly polished and scooped out depression on Location: It usually affects buccal/labial cervical areas
enamel surface adjacent to cementoenamel junction. of teeth. Commonly affects single teeth with excursive
There may be pink spot on tooth which is attributable to the interferences or eccentric occlusal loads.
reduced thickness of enamel and dentin making the pink Appearance: It appears as deep, narrow Vshaped notch.
hue of pulp visible. The lesion is typically wedge shaped with sharp line
Perimolysis: Erosion from dental exposure to gastric angles, but occlusal abfraction may present as circular
secretion is called perimolysis. invaginations (Fig. 27.18).
The magnitude of tooth tissue loss depends on the size,
Erosive lesions cause cupping in dentine. When erosion
duration, direction, frequency and location of the forces.
affects the palatal surfaces of upper maxillary teeth, there
Other factors, such as erosion and abrasion may play a
is often a central area of exposed dentine surrounded by a
significant role in tooth tissue loss, but the initial force is of
border of unaffected enamel. In most cases, it results in little
biomechanical loading.
more than a loss of normal enamel contour, but in severe
cases, dentine or pulp may be damaged. Points to Remember
Stress lesion, excursive interferences or eccentric
Management
occlusal loads, deep, narrow Vshaped notch.
In a patient where loss of tooth surface is essentially caused
by erosive fluids, advise regarding diet and use of sugar
free chewing gum.
Prescription of a fluoride mouthwash is certainly
indicated here.
Modifying brushing habits and restoration of the
defect, usually by glass inomer cement should be done.
For systemic management of vomiting, patient should be
referred to the physician.
Points to Remember
Smooth lesion which exhibits no chalkiness, shallow,
broad, smooth, highly polished and scooped out
depression, pink spot, perimolysis, cupping, fluoride
mouthwash, glass inomer cement.
Figure 27.18 Abfraction seen at cervical area
DENTINAL SCLEROSIS Early widespread formation of secondary dentin seen in

progeria.
It is also called transparent dentin. It is a regressive
alternation in tooth substance that is characterized by Calcific metamorphosis: Significant traumatic injury can 671
calcification of the dentinal tubules. lead to early obliteration of the pulp chamber and canal is
called calcific metamorphosis.
Etiology Physiological secondary dentin: In functioning teeth
It is caused by injury to dentin by caries or abrasion, deposition begins in the coronal portions of tooth proceeds
normal aging process, abrasion or erosion of tooth. toward apical area.
Mechanism Tertiary dentin: Localized new dentin laid in areas of

focal injury. This is called tertiary dentin.
The mechanism of dentinal sclerosis or deposition of
calcium salts is not understood, although most likely Reactionary dentin: If the stimulus is mild to moderate
source of calcium salt is the fluid or dental lymph within tertiary dentin produce by surviving odontoblasts and is
tubules. Increased mineralization of the tooth decreases the termed as reactionary dentin.
conduction of odontoblastic process. Reparative dentin: If the stimulus is severe and lead to
death of primary odontoblast new generation of odontoblast
Clinical Features
may arise from undifferentiated cell within pulp. This is
Appearance: If it is examined under transmitted light, a called reparative dentin.
translucent zone is observed; which is due to the differences
between the refractive indices of the sclerotic or calcified Interface dentin: The initial layer reparative dentin is a
dentinal tubules and the adjacent normal tubules. tubular and this is called interface dentin or fibro dentin.
It shows advancing carious process. Sclerotic dentin Dead tract: When primary odontoblast die their dentinal
is harder than adjacent normal dentin. It is more highly tubules are filled with degenerated odontoblastic process
calcified than normal dentin. and this is called dead tract.
Clinical Features
SECONDARY AND TERTIARY DENTIN
Location: Anterior teeth exhibit higher incidence of
It is also called irregular dentin. It is the dentin which is secondary dentin formation than molar teeth.
formed after the deposition of primary dentin. It serves to
Sign and symptoms: Decrease in tooth sensitivity occurs
prevent involvement or exposure of the pulp cavity. It can
when secondary dentin formation is extensive. It forms an
be physiological (occur due to age) and reparative (occur
additional insulating layer of calcified tissue between the
due to injury).
pulp and the particular pathological process that initiate the
Etiology dentinal response.
Teeth affected by calcific metamorphosis shows yellow
Physiological: Normal aging process, with advancing
discoloration of teeth (Fig. 27.19).
age deposition of secondary dentin leads to smaller pulp
chamber and canal. Radiological features: There is accelerated closure of
the pulp chamber and canal when compared to adjacent or
Reparative: Dental caries, abrasion, attrition, erosion,
contralateral teeth.
tooth fracture, cavity preparation, chemical, thermal or
mechanical insult. Histopathological Features (Fig. 27.20)
Terminology It is well demarcated than primary dentin by deeply staining
resting lines. It exhibits fewer tubules.
Primary dentin: Dentin formed before completion of the
Adventitious secondary dentin is composed of few
crown is called primary dentin.
tubules that may be more tortuous in course.
Secondary dentin: Odontoblast that formed primary Sometimes, secondary dentin is formed at a rapid
dentin remains functional and produce secondary dentin. rate and odontoblasts may get entrapped producing
672
Figure 27.19 Ground section showing tertiary dentin Figure 27.20 Reparative dentin showing well demarcated resting
line with few tubular structure (Courtesy: Dr Sangamesh Halawar,
Reader, Oral Pathology, CDCRI, Rajnandgao, Chhattisgarh
structure resembling bone. Such calcified tissue is called

Etiology
osteodentin.
Tertiary dentin is localized to pulpal end of odontoblastic • Resorption associated with periapical infection
process. It is localized exclusively adjacent to irritated • Reimplanted teeth
zone. The tubules are very irregular, tortuous and reduced • Tumors and cysts
in number. • Excessive mechanical and occlusal forces
• Impacted teeth
Management • Overhanged root canal filling material
• Idiopathic.
Root canal treatment should be done.
Points to Remember Etiology

Irregular dentin, decrease in tooth sensitivity occurs, Resorption associated with periapical infection:
yellow discoloration of teeth, accelerated closure of periapical granuloma (arising due to pulpal infection or
the pulp chamber, fewer tubules, osteodentin. irregular, trauma), causes subsequent resorption of root apex. On the
tortuous and reduced in number. radiograph, it appears as slight raggedness or blunting of
the root apex in the early stages.
RESORPTION OF TEETH Reimplanted teeth: It may result in severe resorption of
It is chronic progressive damage or loss of tooth structure root. Tooth root is resorbed and replaced by bone which
due to the action of cells called odontoclasts. produces ankylosis. Many implanted teeth exhibit complex
It can be physiological as in case of root resorption of resorption of root and are gradually exfoliated.
deciduous teeth or pathological, which occurs in permanent Tumors and cysts: Resorption due to tumors and cysts
teeth. Pathological resorption may be external or internal. appears to be essentially by pressure phenomenon. In most
cases, tissue is present between the tumor and the tooth
External Resorption and it is from this tissue that cells, chiefly osteoclasts arise
It is lytic process occurring in the cementum or cementum and initiated root resorption. Cysts like apical periodontal
and dentin of the roots of teeth. It can be resorption cyst may exert such pressure on the apex of tooth the
occurring at the apex or along the lateral surface of the root intervening connective tissue may in turn get stimulated
or it can be external internal resorption. for osteoclasts formation and thus, resorption begins. The
apex may be lost leaving a flat or scalloped surface at the

distal end of the root.
Excessive mechanical and occlusal forces: Usually, due 673
to the force that is applied during orthodontic treatment,
the patient exhibits multiple areas of root resorption,
irrespective of the manner of treatment. Pressure from
occlusal forces results in destruction, primarily of bone,
then small lacunae often appear on the surface of cementum
and ultimately, it extends into the dentin.
Impacted teeth: Teeth which are completely impacted
or embedded in the bone, occasionally, will undergo
resorption of the crown and root. Impacted tooth also
may cause resorption of roots of adjacent teeth without
itself getting resorbed. This is commonly seen in case of Figure 27.21 External resorption of the root of molar
horizontally or mesioangularly impacted mandibular 3rd
molars impinging on roots of 2nd molar. Microscopically it varies from small area of cementum
Overhanged root canal filling material: In such cases resorption replaced by connective tissue or repaired by
not only the apex get resorbed but more or less, sides of new cementum; too large areas of resorption are replaced
the root are affected. In some cases, all or most of the root by osseous tissues and scooped out areas of resorption are
disappears and the regenerating bone eventually embraces replaced by inflammatory or neoplastic tissue.
the root canal filling intimately, so that it appears as if
Management
the crown of the tooth is held in position by the root
filling. Apicectomy is treatment of choice. If the area is broad and
on lateral surface, curettage and filling of resorbed area
Idiopathic: In this case, burrowing type of external should be carried out.
resorption can occur which is usually seen in relation with
single or multiple erupted teeth. Points to Remember
Lytic process occurring in the cementum, asymptomatic,
Clinical Features
upper incisors, root resorbed, tooth mobile, invasive
Symptoms: The affected tooth is usually asymptomatic. cervical resorption, radiographic resorption of teeth,
Location: The most frequent site for external resorption is osteoclastic activity, root surface of the involved scooped
upper incisors, upper and lower bicuspids. areas of are resorption, apicectomy.
Sign: When the root is completely resorbed, the tooth may

become mobile. If root resorption is followed by ankylosis Internal Resorption
then the tooth is immobile, in infraocclusion and with high It begins certainly in the tooth. It is a condition starting in
percussion sound. the pulp, in which the pulp chamber or the root canals or
both, of the tooth expand by resorption of the surrounding
Invasive cervical resorption: This type of resorption
dentin.
occur in cervical area extend to involve large area of dentin
It is an idiopathic, slow or fast progressive resorptive
through small opening.
process occurring in the dentin or pulp chamber or root
Radiographic features: It will show resorption of teeth canals.
(Fig. 27.21).
Etiology
Histopathological Features It can be caused by inflammatory hyperplasia of the pulp,
It is the result of osteoclastic activity on the root surface of direct and indirect pulp capping, pulpotomy, enamel
the involved tooth. invagination, acute trauma to teeth and pulp polyp.
Mechanism Sign: When the lesion is located in the crown of teeth, it

may expand to such an extent that the crown shows dark
Precipitating factor → vascular changes in pulp →
shadow due to necrosis of the pulp tissue. If the resorption
674 inflammation and production of granulation tissue
is in the root, it may weaken the tooth and result in fracture
→ metaplasia of embryonic connective tissue and
of the tooth. It may perforate the crown with hemorrhagic
macrophages → odontoclast like giant multinucleated cells
tissue projecting from the perforation and result in
→ resorption of internal wall of pulp.
infectious pulpitis.
Types Radiological features: There is enlargement of canal
• I nternal inflammatory resorption: It occurs due to which is balloon like dilation of canal (Fig. 27.23).
intense inflammatory reaction within the pulp tissue.
• Internal replacement or metaplastic resorption: It Histopathological Features
occurs due to absence of any inflammatory reaction There is resorption of the inner or pulpal surface of dentin
within the pulp. and proliferation of the pulp tissue, filling the defect. It
may show occasional osteoclasts or odontoclasts hence it
Clinical Features is called odontoclastoma.
Pulp tissue usually exhibits chronic inflammatory
Appearance: Pink hued area on the crown of tooth, which reaction. Alternating periods of resorption and repair are
represents hyperplastic pulp tissue fitting the resorbed manifested as irregular lacunae like areas in dentin that
area and showing through the remaining overlying tooth are partially or completely filled with irregular dentin or
substance. osteodentin.
Location: It may affect any tooth in primary and secondary When the root surface is perforated, it is very difficult
dentitions, with prevalence in permanent dentition. It is to determine whether the lesion began externally or
more common in central incisor, lateral incisors, premolar internally. Multinucleated giant cells and odontoclasts are
and canine and 3rd molar according decreasing frequency. also present.
Multiple tooth involvement may be there.
Management
Age and sex distribution: It occurs during 4th and 5th
decades of life and is more common in males. Extirpation of pulp with routine endodontic treatment or
retrograde filling stops the internal resorption process.
Symptoms: It is asymptomatic. Extraction of the tooth, if perforation occurs should be
Pink tooth mummery: The roots of teeth with internal carried out.
resorption may manifest a reddish area, called the ‘pink
spot’ (Fig. 27.22). This reddish area represents granulation
tissue showing through the resorbed area of crown.
Figure 27.22 Pink tooth due to internal resorption Figure 27.23 Internal resorption of incisor
as cementoblasts and their direct precursors in this area

Points to Remember
are lost. Instead, it occurs at some distance above the apex
Pink hued area, central incisor, reddish area, called as the inflammatory reaction acts as stimulation for the
the pink spot, enlargement of canal osteoclasts or cementoblasts. 675
odontoclasts, odontoclastoma, pulp tissue, chronic
inflammatory reaction, osteodentin, multinucleated Tooth repair: Occlusal trauma results in mild root
giant cells and odontoclasts, extirpation of pulp. resorption and it is then repaired by cementum formation.
Root fracture is also repaired on occasion by deposition of
cementum between the root fragments as well as in their
HYPERCEMENTOSIS periphery.
It is also called cementum hyperplasia or exostosis of root. Osteitis deformans or Paget disease of bone: It is a
It is characterized by deposition of excessive amount of generalized skeletal disease characterized by excessive
cementum on the root surface. New tissue formation is in amount of cementum formation on roots of teeth and by
direct contact with the cementum of roots of teeth. apparent disappearance of lamina dura of teeth.
Others: Hyperpituitarism, cleidocranial dysostosis can
Types also cause hypercementosis.
• Localized
• Generalized. Clinical Features
Age: It is predominately seen in adults.
Types Location: Premolar teeth are often affected and often teeth
are bilaterally affected and symmetrical in distribution.
Localized: Hypercementosis of single tooth. It is usually The permanent teeth are affected more commonly than
a reactive, inflammation dependent phenomenon seen on deciduous teeth. In multirooted teeth, one or more roots
singular teeth and usually in relation to periapical osteitis are involved.
or due to loss of occluding antagonistic teeth.
Sign and symptoms: There is no increase or decrease
Generalized: Generalized hypercementosis affecting many in tooth sensitivity, unless periapical infection is present.
(all) teeth, but which is seldom recognized as such, occurs Teeth are vital and not sensitive to percussion. There
with increasing age, i.e. as an age dependent factor. It is also may be difficulty in extraction of teeth. In some cases
be seen as a sign accompanying specific diseases, as for hypercementosis is so extensive that it causes fusion of two
instance, Paget’s disease of bone. or more adjacent teeth (Fig. 27.24). Roots appear larger in
diameter than normal and present rounded apices.
Etiology
• Accelerated elongation of a tooth
• Inflammation of the root
• Tooth repair
• Osteitis deformans or Paget disease of bone
• Hyperpituitarism
• Cleidocranial dysostosis.
Etiology
Accelerated elongation of a tooth: It occurs due to loss
of antagonist. It occurs due to inherent tendency of the
periodontium to maintain normal width of the periodontal
ligament.
Inflammation of the root: It does not occur at the apex
of the root directly adjacent to the area of inflammation, Figure 27.24 Fusion of root due to hypercementosis
the cementum surface. Cementicles may be composed of

fibrillar or afibrillar cementum, or a mixture of the two.
They are usually acellular.
676
Types
• F
ree cementicles: They are not attached to cementum.
• Attached or sessile cementicles: They are attached to
the cementum surface.
• Embedded cementicles: They are incorporated into
the cementum layer.
Etiology and Formation

They represent the areas of dystrophic calcification.
Figure 27.25 Hypercementosis Calcification of cell rest of Malassez occurs as a result
of degenerative changes. These bodies enlarge by further
deposition of calcium salts in the adjacent surrounding
Spike formation of cementum: It is characterized by connective tissue.
occurrence of small spikes or outgrowth of cementum on The continued proliferation of connective tissue may
root surface. It occurs in cases of excessive occlusal trauma lead to eventual union of cementicles. It may result from
probably due to deposition of irregular cementum in focal focal calcification of connective tissue between Sharpey’s
group of periodontal ligament fibers. bundles with no apparent central nidus. This occurs as
small round or ovoid globules of calcium salt.
Small cementicles tears or fragments of bone detached
Excessive amount of secondary or cellular cementum is from alveolar plate, if lying free in the periodontal
found to be deposited directly, over typically thin layer of ligament may resemble cementicles. Cementicles appear
primary acellular cementum. to arise through calcification of thromboses capillaries in
Secondary cementum is called osteocementum due to periodontal ligament.
its cellular nature and its resemblance to bone.
Cementum typically arranges in concentric layers Clinical Features
around the root and frequently shows numerous resting It may impart rough globular outline to the root surface;
lines indicated by deeply staining hematoxyphlic lines size is small, ranging from 0.2 to 0.3 in diameter.
parallel to the root surface.
Management
Cementicles appears in the periodontal ligament space
Treatment of the primary cause should be done. (Fig. 27.26). Size of the cementicle is usually varies.
Cementum hyperplasia, exostosis of root, premolar Small, spherical particles of cementum, rough globular
teeth, teeth are vital, fusion of two or more adjacent outline, cementicles appears in the periodontal ligament
teeth, spike formation of cementum, secondary or space, continued proliferation of connective tissue,
cellular cementum, osteocementum, cementum typically cementicles.
arranges in concentric layers.
BRUXISM
CEMENTICLES
The word bruxism is taken from the Greek word brychein:
Cementicles are small, spherical particles of cementum gnashing of teeth. Although the term bruxism is not
that may lie free in the periodontal ligament adjacent to generally known to lay people, it is shorter and more
677
Figure 27.26 Cementicles Figure 27.27 Bruxism causing severe attrition of teeth
convenient than teeth clenching or grinding. Bruxism can Clinical Features

perhaps be best defined as the involuntary, unconscious
In grinding and tapping, this is contact which involves
and excessive grinding, tapping, or clenching of teeth.
movements of the lower jaw and unpleasant sounds which
When it occurs during sleep, it may be best called sleep or
can often awaken housemates.
nocturnal bruxism. A few people, on the other hand, brux
Clenching (or clamping) on the other hand, involves
while they are awake, in which case the condition may be
inaudible, sustained, forceful teeth contact unaccompanied
called wakeful or diurnal bruxism.
by mandibular movements.
All forms of bruxism entail forceful contact between the
biting surfaces of the upper and lower teeth. It is also called Teeth: Chronic bruxism may lead to sensitive, wornout,
night grinding or bruxomania. It is a habitual grinding of decayed, fractured, loose or missing teeth. Grinding or
the teeth, either during sleep or as an unconscious habit clenching breaks down the enamel; sometimes in long
during waking hours. It is term used both for clenching term bruxers, reducing the teeth to stumps (Fig. 27.27).
habit during which pressure is exerted on the teeth and Instead of a white enamel cover, one often sees the more
periodontium by the actual grinding or clamping of the yellowish and softer dentin. The absence of enamel makes
teeth and, also to repeated tapping of the teeth. it easier for the bacteria to penetrate the softer part of the
teeth and produce cavities. As the teeth wear out, they
Etiology become shorter. As a result, when the mouth is closed,
Local: Mild occlusal disturbances, unconscious attempt the upper and lower jaws are nearer than they used to
by the patient to established a greater number of teeth in be and so are the nose and chin. The skin now may bag
contact or to counteract a local irritating situation. below the eyes and curl around the lips, causing the lips
to seemingly disappear. The chin recedes and the person
Systemic factors: Gastrointestinal disturbances, subclini
looks comparatively old.
cal nutritional deficiency and allergy or endocrine distur
bances have been reported to be the causative factors. Facial muscles: Bruxism involves excessive muscle use
leading to a buildup or enlargement (hypertrophy) of
Psychological factors: Emotional tension in which patient
facial muscles, especially those of the jaws (where the
is unable to express his emotion due to fear, rage, rejection
masseter muscle—the muscle that raises the lower jaw
and it becomes hidden in subconscious and later expressed
and enables closing the jaws—is located). In longterm
by variety of way like by grinding the teeth.
bruxers, this buildup may lead to a characteristic square
Occupational: Like in cases of watchmaker, persons who jaw appearance. Some patients resort to removing part
chew gum, tobacco or objects such as toothpicks or pencils. of the masseter muscle by surgery or injections of toxic
materials to reduce muscle size and thus partially regain tension, patients may develop techniques for reducing that
their former, more aesthetically pleasing look. tension and hence, bruxism.
Salivary glands: Another example of this spiral involves Exercise: Quinn suggested isokinetic and stretching
678
the occasional inflammation and blockage of some exercises of the mandible. Such exercises may or may
salivary glands. In this case, the masseter muscle becomes not help alleviate bruxism, but perhaps may be used to
disproportionately overdeveloped and blocks the opening of complement other approaches. However, it seems unlikely
the nearby parotid glands. They, thus interfere with the flow that they could ever be used as the sole therapeutic
of saliva into the mouth, causing the saliva to accumulate approach. Evidence that this approach is effective are non
existent.
in the glands. This in turn may lead to periodical swelling,
pain, inflammation and abnormal dryness of mouth. Drugs: Both, the stress and brain malfunction etiological
theories give at times, rise to the use of antianxiety agents,
TMJ: Bruxism may also damage the temporomandibular
muscle relaxant and other drugs. Most authorities however
joints. First few signs of temporomandibular joint disorders feels that at best, drugs in use now are of limited value
are TMJ discomfort or pain, soreness of jaws and muscles, in the treatment of great majority of chronic bruxers and
clicking or popping sounds when opening the jaws or while that they often involve moreover untoward side effects.
chewing and difficulties in opening the mouth fully. Evidence that this approach is effective: are nonexistent.
Malocclusion: Malocclusion or bad bite is more common Equilibration therapy: Some people believe that bruxism
among bruxers than in the general population. Bruxism is traceable to malocclusion (bad bite). They therefore
may often involve more pressure on one side of the mouth suggest eliminating this cause through orthodontic
than on the other, thereby causing malocclusion. As the adjustment.
teeth wear out, the distance between the upper and lower
Splints: By far, the most common treatment regime for
jaw decreases and overclosure may develop.
bruxism relies on the timehonored procedure of splints
Effect on periodontium: There may be loss of integrity of like nightguards, biteguards, occlusal splints, biteplates,
the periodontal structures resulting in loosening or drifting removable appliances or interocclusal orthopedic appli
of the teeth and even gingival recession occurs. ances and use of manufactured customized appliances.
Removable splints are worn at night to guide the movement
Management so that periodontal damage is minimal
Psychotherapy: The belief that bruxism is traceable
to stress and other emotional and psychological factors Points to Remember
give rise to a variety of psychotherapeutic approaches. Grinding and tapping, clenching (or clamping),
For instance, listening to progressive relaxation or sensitive, wornout, decayed, fractured, loose or missing
autosuggestion tapes just before going to sleep may foster teeth, produce cavities, enlargement (hypertrophy) of
calmness and selfconfidence. facial muscles, inflammation and blockage of some
salivary glands, temporomandibular joint disorders,
Wakeful EMG feedback: Another psychological
malocclusion, loosening or drifting of the teeth, psycho
approach to stress reduction resorts to instrumentation.
therapy, wakeful EMG feedback, exercise, equilibration
During bruxing, the relevant muscles are active and this
therapy, splints.
increased activity or tension can in turn be measured
with an electromyograph (EMG: electro electric; myo
muscle; graph record). During treatment sessions at home TRAUMATIC LESION DUE SEXUAL
or the laboratory, the patient sits or reclines comfortably.
HABIT
One or more pairs of recording electrodes are then attached
to the surface of the skin in close contact to appropriate Orogenital practice is common nowaday inspite it is illegal
muscles (e.g. masseter muscles). These electrodes transmit in many jurisdictions.
information about the level of muscle activity to a computer
monitor. The patient is instructed to consciously lower that Clinical Features
level below a threshold line (also visible on the screen). Appearance: There is submucosal palatal hemorrhage
Gradually, by becoming alert to the presence of muscle secondary to felatio. It appears as erythema, petechiae,
purpura, and ecchymosis of soft palate. The erythrocytic

extravasation is results from musculature of the soft palate
elevating and tensing against an environment of negative
pressure. 679
Oral lesion also occurs due to cunnilingus, resulting in
horizontal ulceration of the lingual frenum. As the tongue
is thrust forward the taut frenum rubs or rakes across the
incisal edges of the mandibular central incisor.
Linear fibrous dysplasia also seen in people who
repeatedly perform the act of cunnilingus.
There is subepithelial accumulation of red blood cells
which may separate the surface epithelial from underlying
connective tissue. Patchy degeneration of epithelial basal Figure 27.28 Oral piercing in tongue
cell layer can occur.
Management Acute complication: It includes pain, profuse bleeding,

No treatment is required. and infection like Ludwig angina, lingual nerve damage,
speech impediment and allergy to the jewellery.
Points to Remember
Chronic complication: Mucosal and gingival trauma,
Orogenital practice, felatio, erythema, petechiae, fractured teeth, aspiration of jewellery, gingival recession,
purpura, and ecchymosis of soft palate, horizontal hyper salivation and tissue hyperplasia around the posts.
ulceration of the lingual frenum, cunnilingum, linear
fibrous dysplasia, subepithelial accumulation of red Forked tongue: It is also called split tongue, bifid tongue.
blood cells. In this anterior one third of tongue is split down to middle.
It is done by pulling fishing line through pierced hole and
tightening a loop over the period of 3 weeks. It can also be
ORAL PIERCING AND OTHER done with the help of laser or surgical approach for quick
BODY MODIFICATION results. Complication of this may occur and it includes
prolonged hemorrhage, permanent neurovascular damage.
Body piercing is ancient practice with association with
religious, cultural, superstitious belief. Talisman (magical charm): Also called susuk (charm
The selected site is pierced with needle and then needles or charm pin). Susuk is placed by magician to
jewellery of choice is threaded through it, jewellery most enhance or preserve beauty, relived pain, bring success
often used is gold, silver and stainless steel. to business and provide protection against harm. Susuk is
made up of silver or gold of 0.5 mm in diameter and around
Clinical and Radiological Features 1 cm in length.
Age and sex distribution: It is more common in
Management
adolescents and young adults with female predilection.
Patient should be encouraged to remove the jewellery.
Location: It is more common seen on tongue, lips buccal If there is inflammation local debridement, antibiotics
mucosa, and uvula. therapy and chlorhexidine mouth wash.
Barbell: This is done in case of tongue consisting of metal
rod with ball that screw onto each end (Fig. 27.28). Points to Remember
Barbell, labret, pain, profuse bleeding, and infection,
Labret: This is seen on lip and consists of ring or rod with
mucosal and gingival trauma, fractured teeth, forked
flat end attached to the mucosal side and round ball of
tongue, susuk, talisman (magical charm) pain.
cutaneous surface.
680
Figure 27.29 Fracture of crown of tooth central incisor Figure 27.30 Root fracture
FRACTURE OF TEETH gingival attachment on the lingual surface. It frequently

involves pulp. There is pain during mastication. Tooth is
Dental Crown Fracture (Fig. 27.29) sensitive to occlusal force.
Anterior teeth are commonly involved. It may be caused by
fall, accident and blows from foreign bodies. Vertical Root Fracture
It is also called cracked tooth syndrome. It runs length
Cracks: It can be seen in indirect light (directing the beam
wise from crown towards apex of tooth. It occurs in
along the long axis of tooth).
endodontically treated tooth as there is weakening of tooth
Uncomplicated fracture: It does not involve dentin and in such cases. It may be caused by traumatic occlusion. It
is usually found on mesial or distal corner of maxillary is usually seen in posterior teeth in adults, especially in
central incisors. Dentin involvement is identified by mandibular molars. Dull pain of long duration which may
contrast of color between it and peripheral layer of enamel. vary from non existent to mild. It may have periodontal
Exposed dentin is very sensitive to chemical, thermal and lesions resembling chronic lesion. History of repeated
mechanical stimulation. failure of endodontic treatment.
Complicated fractures: There is bleeding from exposed
pulp or atleast drop of blood oozes from the pinpoint
Perforation of the Root
exposure. Exposed pulp will be sensitive to most forms of The root canal is sometimes penetrated during operative
stimulation. procedures and injury may extend to cause a perforation
of the root. The usual site of it is at the apex and the side
Dental Root Fracture (Fig. 27.30) of the root, but the floor of the pulp chamber and even
It is common with maxillary central incisors. Coronal the side of the crown; near the neck of the tooth may be
fragment is displaced lingually and is slightly extruded. If perforated. In some cases, the shadow of root canal can be
fracture line is close to the apex then tooth will be more seen approaching the side of the root and this can be the
stable. If only movement of crown is detected, root fracture evidence of a perforation.
is likely. There is temporary loss of sensitivity. It returns to
normal within 6 months. Histopathological Features of Tooth Fracture
The clot between root fragments is organized and this
Crown/Root Fracture connective tissue is subsequently the site of new cementum
Such fracture is likely to be intra and extra alveolar. They or bone formation. There is always some resorption of the
are a result of direct trauma. They have labial margin in ends of the fragment but these resorption lacunae ultimately
the gingival third and course obliquely to exist below the are repaired.
Points to Remember
• D ental crown fracture: Cracks, uncomplicated
fracture, complicated fractures 681
• Dental root fracture: Coronal fragment is displaced
lingually, temporary loss of sensitivity
• Crown/root fracture: Direct trauma, frequently
involves pulp
• Vertical root fracture: Cracked tooth syndrome,
posterior teeth, dull pain
• Perforation of the root: During operative procedures
• Histopathological: New cementum or bone
formation, resorption of the ends of the fragment.
AMALGAM TATTOO Figure 27.31 Blue black pigmentation seen due to amalgam
tattoo
Causes
It may be condensed in the abraded gingiva during routine Histopathological Features
amalgam restorative work. It may enter the mucosa lacerated
by rotary instruments during removal of old amalgam Amalgam presents as discrete fine dark growth and
fillings or crown and bridge preparations of teeth with large irregular solid fragments.
amalgam restorations. Broken pieces may be introduced into Dark granules arranged mainly along collagen bundles
the socket or beneath the periosteum during extraction of the and around blood vessels, nerve sheath, elastic fibers and
teeth. Particles may enter the surgical cut during root canal acini or minor salivary glands.
treatment with retrograde amalgam filling. Dark granules are present intracellularly within
Other cause of exogenous pigmentation are pencil macrophage multi-nucleated giant cell, fibroblasts.
implantation, fragment of broken carborundum disks, Large fragment are surrounded by dense fibrous
dental burs and charcoal denitrifies have also have similar connective tissue with mild inflammation.
appearing lesion.
Management
Clinical Features Treatment is not necessary. However, if required, excision
is done.
Location: The most common sites are gingiva and alveolar
mucosa with mandibular region being affected more Points to Remember
commonly than maxillary region. Gingiva and alveolar mucosa with mandibular region,
Age and sex distribution: It can occur at any age but it is flat macule or sometimes slightly raised lesion, blue
rarely seen below the 12 years as amalgam restorations are black, discrete fine dark growth and irregular solid
not used before the age of 12 years. Females are affected fragments, multinucleated giant cell, fibrous connective
more commonly than males in ratio of 1.8:1. tissue with mild inflammation.
Appearance: It is described as a flat macule or
sometimes slightly raised lesion with margins being
BISMUTHISM
well defined or diffuse in other. Pigmentation is
blue black in color. It may gradually increase in size Causes
(Fig. 27.31). Medicinal use of bismuth containing preparation can cause
Radiological features: Fragment are seen radiopaque. bismuthism. Many proprietary drugs contain bismuth
salt and bismuth containing pastes may result in bismuth when small piece of white paper is inserted in the gingival
pigmentation. sulcus, the presence of pigmented area is verified.
682 Mechanisms Points to Remember

This pigment is produced by the action of hydrogen sulfide Hydrogen sulfide, Bismuth grippe, Bismuth line,
on the bismuth compound. The hydrogen sulfide is formed metallic taste, annoying gingivostomatitis, blue black’
through bacterial degradation of organic material of food bismuth line gingival papillae, irregular black collection
retention. of pigment, paper test.
Clinical Features
PLUMBISM
Bismuth grippe: Vague gastrointestinal tract disturbances,
nausea, bloody diarrhea, bismuth grippe and jaundice can It occurs due to lead poisoning.
occur.
Causes
Bismuth line: Sometimes in the long bone, white bands
It is caused by lead in the paints, glazes, cooking vessels,
of increase density appear in the ends of the diaphyses
batteries, ointment and containers. Moonshine an illicit
immediately adjacent to the epiphyseal lines. This is called
alcoholic beverage distilled in car radiators has been shown
bismuth line.
to cause acute lead poisoning. Use of tetraethyl lead, an
Oral Manifestations antiknock compound in gasoline, has introduced a new
source of lead.
Sign and symptoms: Patients often complain of a
Excessive absorption of the lead from automobile
metallic taste with burning sensation in the oral cavity
exhaust and dust and dirt derived from house paint is
and annoying gingivostomatitis with symptoms similar to
known to affect a large number of poor children living in
Acute necrotizing ulcerative gingivitis (ANUG). Large,
urban areas.
extremely painful, shallow ulcerations are seen at times on
Acute exposure can occur in foundries, smelters,
the cheek mucosa in molar region.
battery plants, munitions and garages.
Regional lymphadenopathy may be present. Tongue is
frequently enlarged and sore. Mechanism of Action
Blue black bismuth line appears to be well demarcated
Absorption of lead from alimentary tract, lungs and gut
to eye on gingival papillae. Blue black bismuth sulfide
→ modulated by vitamin D and calcium status of the
granules formed by action of H2S produced by action of
individual → lead is taken up by circulating erythrocytes
bacteria on organic material remaining in areas of poor oral
and bound to reactive sulfhydryl group of proteins → from
hygiene.
the circulation, lead is transferred to all the soft tissues and
Histopathological Features in high concentration it will inhibit metabolic pathways
→ in the red cells, lead inhibits enzymes associated with
The granules of sulfide are seen in the tissue section as
hemoglobin synthesis → hence abnormal activity of the
small irregular black collection of pigment, sometimes
enzymes occur.
perivascular in location. The material may be present in
endothelial cells or in mononuclear phagocytes in the Clinical Features
tissue, but usually in intercellular tissue.
Nervous system: Lead has high affinity for cells in central as
Diagnosis well as peripheral systems. In acute poisoning, demyelination
and axon degeneration occurs. Lead encephalopathy,
An ulcerative gingivostomatitis accompanied by discrete
cerebral palsy, mental retardation, seizures, wrist or foot
blue black pigmentation of interdental papilla and marginal
drop and fatigue can occur.
gingiva in a patient receiving oral or anal administration of
bismuth compounds. Gastrointestinal tract: There may be serious gastro
Paper test: It will indicate whether the pigmentation intestinal disturbances like nausea, constipation, vomiting,
is actually in gingival tissue. If the pigmentation persists, and colic.
Neurological value: Abnormal neurological value for the

both peripheral and central nervous system.
Management 683
Lead can be removed from body by using a chelating agent
such as calcium edetate (EDTA) or penicillamine.
Points to Remember
Moonshine, demyelination and axon degeneration,
lead encephalopathy, cerebral palsy, gastrointestinal
disturbances, interfere with cellular metabolism, metallic
taste, Burtonian line gray black in color, pallor of lip,
aminolevulinic acid and aminolevulinic acid dehydratase
and synthetase level in blood are decreased, EDTA) or
Figure 27.32 Gray black color pigmentation due to lead
penicillamine.
Bone: When incorporated in the bone it can interfere with MERCURIALISM

cellular metabolism and changes the rate of bone resorption It may be chronic or acute. It is also called pink disease,
and apposition. swift’s disease, dermato-polyneuritis, and acrodynia. It is
an uncommon disease caused due to a mercurial toxicity
Oral Manifestations
reaction, either actual mercury poisoning or, more likely,
Oral tissues are exposed to lead through direct contact with an idiosyncrasy to the metal. Exposure of young children to
ingested lead and through secretion of lead in the saliva. minute amounts of mercury is responsible for a condition.
The exact etiology of it is unknown but it is thought that it
Symptoms: There is a metallic taste which is accompanied
may occur due to mercury toxicity.
by excessive salivation and dysphagia.
Burtonian line: When exposure to lead is very high and Causes
oral hygiene is very poor, a line known as ‘burtonian line’ As a result of occupational contact, drug overdose, suicide
is seen which is gray black in color and is present along attempt or self medication with mercurial compounds.
the gingival margin. Lead line is more diffuse than bismuth Mercury hazards exist in paints containing mercurial
line (Fig. 27.32). salts such as phenylmercuric propionate.
Sign: There is pallor of lip; poor muscle tone and the face Prolonged administration of mercurial diuretics
appears ashen in color because of associated anemia. There can also result in mercurialism. Frequent use of night
is bilateral parotid gland hypertrophy. cream containing inorganic salts may produce distinctive
discoloration.
Laboratory Finding Mercurial fumes which are produced industrially also
result in bone changes in the jaws, if inhaled in quantity.
Blood changes: These are those of hemolytic anemia with
Improper use of dental amalgam alloy can also cause
basophilic stippling of the RBCs.
mercurialism.
Lead level: In children, the level of exposure can be gained
by measurement of tooth lead level and in adults the hair Clinical Features
lead concentration is commonly used as an indicator. It occurs most frequently in young infants before the age of
2 years although children can occasionally affected up to
Enzymes level: Measurement of aminolevulinic acid and
age of five years or six years.
aminolevulinic acid dehydratase and synthetase level in
blood are decreased in lead poisoning as these enzymes Gastrointestinal symptoms: Intestinal colic and diarrhea.
are associated with hemoglobin synthesis and lead inhibits There is also pharyngitis, dysphagia, nausea, abdominal
hemoglobin synthesis. pain.
Nervous symptoms: Long continued exposure to mercury Management

vapor can result in permanent neurological changes.
Bed rest and suitable dietary regimen should be adjusted
Headache, insomnia, tremors of fingers and tongue and
684 for renal damage. Atropine or belladonna can be prescribed
mental depression.
to lessen the salivary flow.
Renal symptoms: Severe intoxication and it can be the Due discontinuation of possible exposure to mercury
cause of death. and administration of BAL (British antilewisite) and
dimercaprol has been proven successful in most cases
Hands, feet, nose and cheeks assume pink color. The nails
unless the disease is of long duration.
are shed at the same time with teeth lost prematurely and
alopecia is also present. The children will frequently tear Points to Remember
their hair out in patches. The skin over the affected area
Pink disease, swift’s disease, intestinal colic and diarrhea,
peels frequently during the course of the disease. The
neurological changes. Headache, insomnia, tremors of
patient also have maculopapular rash which is extremely
fingers, renal symptoms, pink color hands, feet, nose,
pruritic.
raw beef appearance, dribbling, oral mucosal ulceration,
Raw beef appearance: The skin of hands, feet, nose, ears diffuse grayish pigmentation, alveolar mucosa, sound
and cheek becomes clammy red or pink and has a cold teeth may exfoliate, administration of BAL (British anti
clammy feeling. The appearance is described as resembling lewisite).
raw beef.
Severe sweating, extreme irritability, photophobia with
lacrimation, insomnia, muscular weakness, tachycardia ARGYRIA
and hypertension It is also called argyrosis which occurs due to chronic
exposure to silver compound.
Oral Manifestations
Causes
Symptoms: There is marked increase in inflow of ropy
viscid saliva, hot mouth, itching sensation and metallic It is cause from local and systemic absorption of silver
taste are experience. Patient will exhibit profuse salivation compounds. It may result from the use of silver containing
and often much dribbling. Mastication is difficult due to nasal drops or sprays or silverarsphenamine injection used
pain. The gingiva becomes extremely sensitive or painful to treat syphilis.
and it may exhibit ulceration. Chewing pieces of photographic films over an extended
Oral mucosal ulceration occurs and spreads to the period can also result in argyria. Localized argyria can
palate, throat and pharynx. Salivary glands and lymph develop following long continued use of silver preparation.
nodes may be swollen and tongue is enlarged, painful and Clinical Features
ulcerated. Tongue tremors may be present.
Faint diffuse grayish pigmentation of alveolar mucosa Acute silver intoxication: It can produce coma, pleural
occurs. The gums are of a deeper hue than normal. Lips are edema, hemolysis and bone marrow failure.
dry, cracked and swollen. Appearance: Skin is slate gray, violet or cyanotic and in
Sound teeth may exfoliate and one or more teeth may be marked cases, there is even suggestion of metallic luster.
found on bed in the morning as patients awake. The reason The exposed body surfaces including the nail beds are
for it is that there is marked periostitis with loosening of deeply discolored.
the teeth which may lead to exfoliation of teeth. Many time
children extract his own teeth with the help of his finger. Pigmentation is distributed diffusely throughout the
There may be loosening and premature shedding of teeth gingival and mucosal tissue.
often occurs. The loss of teeth sometimes followed by Oral finding: In oral cavity slate blue silver line present
necrosis of bone and there may be sequestrum formation. along the gingival margin. In some cases diffuse blue black
Bruxism is a common finding. discoloration.
Management Causes
Source of contact should be eliminated. Gold is useful for the treatment of Rh arthritis, lupus
erythematous and leprosy. 685
Points to Remember
Argyrosis, silver arsphenamine, coma, pleural edema, Clinical Features
hemolysis, slate gray, violet or cyanotic, pigmentation, Dermatitis is the most common complaint which is preceded
slate blue silver line present along the gingival margin by pruritus. It will results in alopecia and loss of nails.
diffuse blue black discoloration. Purpura and malignant neutropenia can also occur.
Chrysiasis: Slate blue or purple discoloration of skin can

ARSENISM occur. This is called chrysiasis.
It occurs due to arsenic poisoning. Arsenic treatment is Oral mucositis: It is the most common complaint of the
useful in case of asthma and skin disorder such as psoriasis. patient who is receiving gold therapy. There is vesiculation
and ulcerations of the oral mucosa. This is common on
Causes lateral border of tongue, palate and pharynx.
Industrial exposure or intentional use or due to therapeutic Symptoms: Metallic test often precede the oral mucositis.
consumption can lead to arsenic poisoning.
Management
Clinical Features
Discontinuation of gold therapy and alkaline mouth washes
Symptoms: Patient may be having chronic gastritis, and should be prescribed.
colitis.
Arsenic keratosis: Keratosis of palms of the hand and Points to Remember
soles of feet. Rh arthritis, lupus erythematous and leprosy, dermatitis,
purpura, malignant neutropenia, chrysiasis, slate blue
Hyperpigmentation: Prolonged exposure to arsenic
or purple discoloration, oral mucositis, metallic test,
results in diffuse macular pigmentation or ulceration of the
alkaline mouth washes.
skin.
Oral lesion: Oral tissues are extremely painful, become
intensely inflamed and severe gingivitis may develop. INFLAMMATORY FIBROUS
Excessive salivation can be done. Tissues are deep red in HYPERPLASIA
color. Local contact with arsenic trioxide often produces It is also called denture injury tumor, denture epulis, and
ulceration. epulis fissuratum.
Management Causes
Surface anesthetic ointment or rinses such as lidocaine or Ill fitting denture is the most common cause for this
dyclonine solution. condition. It occurs due to overextended denture flanges.
Other factors which are responsible are ragged margins
Points to Remember
of teeth, overhanging restorations, sharp spicules of bone,
Industrial exposure, chronic gastritis, and colitis, arsenic badly fitting clasps and chronic biting of cheek and lips.
keratosis, hyperpigmentation, painful, become intensely
inflamed severe gingivitis, lidocaine or dyclonine Clinical Features
solution. Age and predilection: It occur in adult with female
predilection.
AURIC STOMATITIS Location: The anterior portion the jaw is more commonly
It is poisoning occur due to gold which has been used in affected that posterior portion. It may occur either in
medicinal treatment. maxilla or mandible.
686
Figure 27.33 Inflammatory fibrous hyperplasia due to denture Figure 27.34 Inflammatory fibrous hyperplasia
Appearance: There is development of elongated rolls infiltration in the subepithelial connective tissue. There is
of tissue in the mucolabial or mucobuccal fold area, into also mucopolysaccharide keratin dystrophy, also referred
which the denture flanges conveniently fit. The proliferation as plasma pooling.
of tissue is usually slow. Osseous or chondromatous metaplasia: In some case
there is formation of osteoid or chondroid which is reactive
There may be small nodular or polypoid overgrowth of
phenomenon due to chronic irritation of ill fitting denture.
fibrous tissue due to gingival irritation. When the lesions
occur in buccal sulcus due to denture flanges, it is called Management
epulis fissuratum. In it, there is concomitant overgrowth of
It should be treated with excisional biopsy. Elimination of
surrounding fibrous tissues with a groove in it (Fig. 27.33) .
irritation should be done.
Leaf like denture fibroma: It is seen on hard palate
beneath maxillary denture. This flattened pink mass that Points to Remember
is attached to palate by narrow stalk. The edge of lesion is Denture injury tumor, overextended denture flanges,
serrated and resembles a leaf. It is also called fibroepithelial elongated rolls of tissue in the mucolabial, mucobuccal
polyps. fold area, epulis fissuratum, leaf like denture fibroma,
The excess folds of tissue are usually inflamed clinically, excessive bulk of fibrous connective tissue, pseudoepi
although there may be irritation or even ulceration in the theliomatous hyperplasia, chronic inflammatory infil
base of the fold, into which the denture flange fits. The tration, osseous or chondromatous metaplasia.
lesion is firm on palpation.
Histopathological Features (Fig. 27.34) INFLAMMATORY PAPILLARY

HYPERPLASIA
The hyperplastic mass of tissue is composed of an excessive
bulk of fibrous connective tissue covered by a layer of It is also called palatal papillomatosis and palatal epithelial
stratified squamous epithelium, which may be of normal hyperplasia. It occurs in 3 to 4 percent of dentures wearers.
thickness or show acanthosis.
Pseudoepithelioumatous hyperplasia, hyperortho Causes
keratosis or parakeratosis are often found. Frictional irritation produced by loose fitting dentures on
The connective tissue is composed chiefly of coarse palatal tissues. It occurs in patients who sleep with their
bundles of collagen fibers with few fibroblasts or blood dentures on. Full dentures in which relief areas or suction
vessels, unless there is an active inflammatory reaction. chambers are cut in palatal seating surface appear to be the
There is moderate degree of chronic inflammatory strongest stimuli for the lesion.
It is much common in acrylic dentures than in those Histopathological Features

with metallic dentures. It is more common in patients with
It has got exophytic nature. There is varying degree of
poor oral hygiene. In some cases, irritation due to amalgam
branching and polypoid proliferation on the epithelial 687
filling may produce these lesions.
surface.
Clinical Features It shows numerous small vertical projections, each
composed of parakeratotic or sometimes orthokeratotic
Age and sex distribution: It can arise at any age in adults stratified squamous epithelium and a central core of
and has no definite sex predilection. connective tissue. Pseudoepitheliomatous hyperplasia is
Location: It occurs exclusively on palate beneath the seen.
complete or partial denture. It occurs predominately in
edentulous patients and the site of lesion corresponds to Management
the denture base. In rare cases, cheek may be involved. Remove the denture at night to provide rest to the tissue.
Whole palatal mucosa under the denture may be covered Conditioning liner should be applied.
with numerous small polypoid masses. In cases of fibrosis, surgical removal and curettage,
Appearance: The lesion presents as numerous closely electrosurgery and cryosurgery should be done. In case
arranged, red, edematous papillary projections often with superimposed candidal infection, topical application
involving nearly all of the hard palate and imparting to of antifungal agents should be used. Most commonly used
it a ‘warty appearance’. In some cases, it is swollen and is nystatin ointment.
papillary projection resembles the surface of ‘overripe
Points to Remember
berry’. In some cases it produces a ‘cobblestone’
appearance. Palatal papillomatosis, frictional irritation, palate
beneath the complete or partial denture, warty appear
Sign and symptoms: Lesions are friable, often bleed with
ance, overripe berry, cobblestone, friable, often
minimum trauma and may be covered with thin whitish
bleed with minimum trauma, branching and polypoid
exudate. The tissue exhibits varying degrees of inflammation,
proliferation on the epithelial surface, parakeratotic or
but seldom there is ulceration. These are seldom over 0.3
sometimes orthokeratotic stratified squamous, pseudoe
cm in diameter. The lesion may extend onto the alveolar
pitheliomatous hyperplasia.
mucosa. The individual papillae are seldom over a millimeter
or two in diameter. When complicated by Candida albicans
lesion appears as red to scarlet, soft and bleeds easily in the
EPULIS GRANULOMATOSUM
inflammatory or granulomatous stage (Fig. 27.35).
It is reactive hyperplasia that develops within a tooth socket
after the extraction or exfoliation of tooth. It is caused by
sharp spicules of bone in socket.
Clinical Features
Age: It can occur at any age and becomes apparent within
2 weeks after the loss of a tooth.
Appearance: It is exuberant dark red granulation tissue
extruding from a tooth socket. It is painless growth. The
enlargement is soft, hemorrhagic with an erythematous to
white, smooth surface.
Management
It is done to remove the granulation tissue and smoothing
Figure 27.35 Palatal papillary hyperplasia the socket borders is indicated. It should be done.
There is frequently patchy chronic inflammatory

Points to Remember
infiltration, consisting of lymphocytes and occasional
Exuberant dark red granulation, tissue extruding from a plasma cells and macrophages. Fibroblasts tend to be
688 tooth socket. larger than their normal size. The nuclei are plump with
prominent nucleoli.
NODULAR FASCIITIS Management
It is also called pseudosarcomatous fasciitis. It is a Local excision will yield excellent result.
proliferative fibroblastic lesion and presents as a tumor
like mass with infiltrative properties. It appears to be an Points to Remember
inflammatory reactive phenomenon. Pseudosarcomatous fasciitis, inflammatory reactive
phenomenon small lump, painful, proliferation of fibro
Clinical Features
blast, textured feathery appearance, chronic inflamma
Location: Half of the cases are reported on upper limb. tory infiltration.
Intraorally, the affected sites are subcutaneous tissues
overlying the mandible, zygoma, parotid sheath and oral
mucosa. UREMIC STOMATITIS
Age: It can occur at any age, but 3rd to 5th decades are Nonkeratotic white lesions caused due to elevated
common. creatinine or blood urea nitrogen. It has been suggested to
Appearance: It appears as a small lump, which may be be consequence of strongly alkaline saliva due to ammonia
painful. The lesion often enlarges rapidly, but only to formation from retained urea secreted in the saliva. This
maximum size of 4 cm, where it remains stationary or will damage oral mucosa.
regresses.
Clinical Features
Histopathological Features Age and sex distribution: It is common in young and
There is proliferation of fibroblast (Fig. 27.36) in a rather middle aged adults with no sex predilection.
haphazard manner in a vascular myxoid matrix which Location: It is seen on tongue, buccal mucosa
is rich in acid mucopolysaccharide. It results in a loose (Fig. 27.37), and floor of mouth.
textured feathery appearance.
Figure 27.36 Nodular fasciitis showing proliferation of Figure 27.37 Lesion of uremic stomatitis
fibroblast
Appearance: There is extensive pseudomembranous

white lesion. There is ammonical odor to breath. In some
cases, there is appearance of ulcer occurs. Oral ulceration
varies in size and is irregular in shape and usually shallow. 689
The epithelium is lacking, have been replaced by an
eosinophilic coagulum with an adjacent inflammatory cell
infiltrates.
Management
Oral ulcer, if painful may be treated by prescribing a
palliative oral rinse such as an antihistamine oral suspension
with kaopectate.
Figure 27.38 Traumatic keratosis
Points to Remember
Elevated creatinine or blood urea nitrogen, pseudo
membranous white lesion, ammonical odor to breath,
epithelium is lacking, adjacent inflammatory cell Points to Remember
infiltrates, oral suspension with kaopectate. Thickened whitish oral mucosa, local irritants,
glassblower’s white patch, hyperkeratosis, parakeratosis
and acanthosis.
TRAUMATIC KERATOSIS
It refer to isolated area of thickened whitish oral mucosa
that is clearly related to identifiable local irritant and BISPHOSPHONATES ASSOCIATED
resolves following elimination of irritant. OSTEONECROSIS
These drugs are used in the treatment of malignancy like
Etiology
multiple myeloma, metastatic breast carcinoma and bone
Local irritants like ill fitting denture, sharp clasp and rough disease like Paget disease. These drugs inhibits osteoclast
edges of restoration. Heavy cigarettes smoking. and interfere with angiogenesis. They also the vascular
endothelial factor. This are used to slow down osseous
involvement
Most common sites are lip and buccal mucosa. There is There are two generation of bisphosphonates, i.e. first
isolated thickened whitish area. generation with low potency and are readily metabolized
Glassblower’s white patch: It is variant of traumatic by osteoclast and second generation which are more potent
keratosis affecting the cheek and lips, which occur in glass and are designated as aminobisphosphonates.
factory.
Pathogenesis
Normal bone undergoing resorption and reapposition =
There is varying degree of hyperkeratosis, parakeratosis osteoclast maintain normal bone by repairing microfracture
and acanthosis. and resorbs areas of normal bone containing foci of
osteocytes = release of cytokines and growth factors which
Management induce formation of active bone forming osteoblasts =
Upon removal of the offending agent, the lesion should bisphosphonates treated bone induce osteoclastic apoptosis
resolve within 2 weeks. Biopsies should be performed on = reduction in recruiting additional osteoclast = stimulation
lesions that do not heal to rule out a dysplastic lesion. of osteoblasts to release osteoclast inhibiting factors.
Clinical and Radiological Features 4. Bamise CT, Esan TA, Ajayi JO, Olagundoye O, Oziegbe
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trauma to bony protuberance like tori and exostosis. oral mucosa related to dermatological disorders. J Cutan
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Radiographic features: There is increase radiopacity 6. BeckMannagetta J, Hutarew G. Pigmented lesions of the
before clinical evidence of frank necrosis. Panoramic oral mucosa. Hautarzt. 2012;63(9):7049.
radiograph show increase radiodensity of crestal portion 7. Beumer J 3rd, Curtis T, Harrison RE. Radiation therapy
of alveolar ridge. In severe cases there is moth eaten of the oral cavity: sequelae and management, part 1. Head
appearance with ill defined radiolucency. In some cases Neck Surg. 1979;1(4):30112.
sequestra formation can be seen. 8. Bolewska J, Holmstrup P, MøllerMadsen B, Kenrad B,
Danscher G. Amalgamassociated mercury accumulations
in normal oral mucosa, oral mucosal lesions of lichen planus
Histopathological Features and contact lesions associated with amalgam. J Oral Pathol
There are irregular trabeculae of pagetoid bone with Med. 1990;19:3942.
enlarged and irregular osteoclast which demonstrate 9. Bring support to bruxism sufferers. Br Dent J.
numerous intracytoplasmic vacuoles. There is also 2012;213(5):248. doi: 10.1038/sj.bdj.2012.814.
peripheral resorption with bacterial colonization. 10. Burton H. On a remarkable effect on the human gums,
produced by the absorption of lead. Med Chir Trans.
Management 1840;23:6379.
11. Carmona IT, Tejeiro JC, Dios PD, Leston JS, Ferreiro
Elimination of all dental infection and improve dental MC, Posse JL. Morsicatio linguarum versus oral hairy
health to avoid invasive procedure which may lead to leukoplakia. Dermatology. 2000;201:2812.
osteonecrosis. Manipulation of bone should be avoided. 12. Damm DD, Fantasia JE. Bilateral white lesions of buccal
Endodontic treatment should be done in place of extraction mucosa: morsicatio buccarum. Gen Dent. 2006;54:4424.
of teeth. 13. DeSesso JM. Teratogen update: inorganic arsenic.
In symptomatic patient systemic antibiotics like penicillin Teratology. 2001;64(3):1703.
with or without metronidiazole, ciprofloxacin, erythromycin 14. Donly KJ, Nowak AJ. Oral electrical burns: etiology,
and chlorhexidine mouth wash should be given. manifestations, and treatment. Gen Dent. 1988;36(2):1037.
15. Dubach P, Caversaccio M. Images in clinical medicine.
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16. D’Acunto C, Piccolo V, Neri I, Misciali C, Raone B, Russo
Drugs inhibits osteoclast and interfere with angiogenesis, T, Patrizi A. Pigmented lesion of the floor of oral cavity:
bone show area of necrosis, increase radiopacity, frank what is your diagnosis? Amalgam tattoo (AT). Clin Exp
necrosis, crestal portion of alveolar ridge, pagetoid Dermatol. 2012;37(2):2056.
bone with enlarged and irregular osteoclast, numerous 17. ElSaid KF, ElGhamry AM, Mahdy NH, ElBestawy NA.
intracytoplasmic vacuoles, penicillin metronidiazole, Chronic occupational exposure to lead and its impact on oral
ciprofloxacin, erythromycin. health. J Egypt Public Health Assoc. 2008;83(56):45166.
18. Ethunandan M, Shanahan D, Patel M. Iatrogenic mandibular
fractures following removal of impacted third molars: an
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1. Well developed rete pegs and densely collagenous 4. A characteristic square- jaw appearance is the feature of:
lamina propria is the feature of: a. Osteoradionecrosis
a. Linea alba b. Lip biting b. Bruxism
c. Chemical burns d. Both b and c c. Ankylosis
2. Whitish gray or ulcerated lesions of the middle third of d. None
the hard palate is due to: 5. ‘Burtonian line is seen in:
a. CO2 burn b. Pizza burn a. Copper poisoning b. Mercury poisoning
c. Acid burn d. Vitamin C tablet c. Lead poisoning d. Thermal burns
3. The salivary flow becomes zero when dose reaches: 6. Raw beef appearance is the clinical feature of:
a. 20 Gy b. 30 Gy a. Mercurialism b. Swift’s disease
c. 40 Gy d. 60 Gy c. Both a and b d. Argyria
28 Blood Pathology
Chapter Outline
Â Disease of lymph tissue Â Thrombotic thrombocytopenic purpura

Â Lymphoid hyperplasia Â Aldrich syndrome
Â Disease of red blood cells Â Familial thrombasthenia
Â Anemia Â Thrombocytosis or thrombocythemia
Â Thalassemia Â Disease due to clotting defect
Â Aplastic anemia Â Hemophilia
Â Hereditary elliptocytosis Â Von Willebrand’s disease
Â Hereditary spherocytosis Â Plasminogen deficiency
Â Acute post hemorrhagic anemia Â Factor V deficiency or Parahemophilia
Â Erythroblastosis fetalis Â Afibrinogenemia and hypofibrinogenemia
Â Erythropoietic porphyria Â Dysfibrinogenemia
Â Iron deficiency anemia Â Fibrin stabilizing factor deficiency
Â Plummer Vinson syndrome Â Macroglobulinemia
Â Pernicious anemia Â Malignancy involving blood tissue
Â Polycythemia Vera Â Hodgkin’s lymphoma
Â White blood cell disorders Â Non Hodgkin’s lymphoma
Â Agranulocytopenia Â Primary reticular cell sarcoma
Â Cyclic neutropenia Â Mycosis fungoides
Â Lazy leukocyte syndrome Â Burkitt’s lymphoma
Â Chronic idiopathic neutropenia Â Leukemia
Â Chediak Higashi syndrome Â Multiple myeloma
Â Disease of platelet Â Plasmacytoma
Â Idiopathic thrombocytopenic purpura Â Extranodal NK/T-cell lymphoma
DISEASE OF LYMPH TISSUE in enlargement called lymphoid hyperplasia. Lymphoid

hyperplasia is unusual in the head and neck region, but
Lymphoid Hyperplasia the diagnosis of it is of clinical importance as it may be
confused with malignant lymphoma, both on clinical
Lymph tissue is very important in the defense mechanism examination and pathologically. It is also called reactive
of the body. It process foreign antigen like viruses, fungi, lymphoid hyperplasia. This entity was first described in
and bacteria. The proliferation of lymphoid tissue results 1973 by Adkins.
Clinical Features
Site: Lymphoid hyperplasia is usually affect the lymph

694 nodes in the region anterior cervical chain. It can also
affect lymphoid tissue of Waldeyer’s ring, aggregates
of lymphoid tissue in the region of oropharynx, the soft
palate, the lateral tongue and the floor of the mouth.
Lymph nodes: In case of acute infection lymph nodes
are soft, tender, and are increase in size. In case of chronic
infection lymph nodes are enlarge with rubbery consistency.
In chronic cases lymph nodes are non-tender and movable
in consistency. This chronic cases may get confused with
lymphoma. History will give clue to the diagnosis. History of
infection and no progressive enlargement will goes in favor of
lymphoid hyperplasia. In some cases buccal lymph nodes are Figure 28.2 Histopathological photographs showing tingible
enlarge and present with non-tender, freely movable nodule, bodies in lymphoid hyperplasia
less than 1 cm in diameter. It is present in the cheek region.
Lingual tonsil (Fig. 28.1): It is located in posterior
centers are interspersed in sheets of well differentiated
part of tongue on the dorsolateral aspect. It is normally
lymphocyte. The cells of germinal center are transformed
prominent in the younger individual and it get decrease in
B lymphocytes.
size with the age. In some cases tonsil are so enlarge that
they can occlude the airway. This type of tonsil is called Tingible bodies: There is presence of phagocytized mate-
kissing tonsil. This patient are usually asymptomatic and rial called tangible bodies in the cytoplasm of macrophage.
enlargement is symmetrical. They engulf nuclear debris from proliferating lymphocytes
(Fig. 28.2).
Intraoral lymphoid aggregates: This are usually present
in lateral border of the tongue in the posterior region. The Management
color of this can be normal or dark pink (if the aggregates is
As lymphoid hyperplasia is the benign process, no
deeper) and yellowish orange (if it near to the surface). This
management is require. In some cases when palatal
usually bilaterally symmetrical.
lymphoid hyperplasia interferes with the prosthesis surgical
Histopathological Features excision of the lesion can be carried out.
Germinal center: There is collection of sharply demarcated Points to Remember
reactive lymphoblast called germinal center. These
Enlargement of lymph nodes, kissing tonsil, lingual
tonsil, intraoral lymphoid aggregates, germinal center,
tingible bodies.
DISEASE OF RED BLOOD CELLS

Anemia
Anemia is the general term which is used for decreases in
volume of red blood cells or concentration of hemoglobin.
There are various factor which can cause anemia like
disturbed iron metabolism (iron deficiency and sideroblastic
anemia), deficiency of factor like vitamin B12, folic acid.
Figure 28.1 Lingual tonsil due to lymphoid hyperplasia Anemia also can occur due to chronic infection, connective
Blood Pathology
tissue disorders, secondary to malignancy, in liver disease, Most of persons expire before the age of 40 years. There is
endocrine disease. Some disorders of hemoglobin (sickle also presence of leg ulcer and gall stones. When associated
cell and thalassemia) and anemia due to hemolytic factor with folate deficiency, there may be growth retardation and
can also occur. also delayed puberty. Hyperplasia of marrow in first year 695
Generally patient complaint of tiredness, headache and of life expands the marrow cavity producing bossing of the
lightheadedness. Pallor of the mucosa and conjunctiva is skull, prominent malar bones and protuberant teeth.
present in all types of anemia. Sickle cell crisis: There is a long quit spell of hemolytic
There are many types of anemia. Some important types latency occasionally punctuated by exacerbations called
are discussed below. sickle cell crisis.
Sickle Cell Anemia Acute chest syndrome: Pulmonary involvement which

is precipitated by fat embolism or community acquired
It is autosomal dominant. It was first described by Herrick in pneumonia is known as acute chest syndrome.
1910. This is the most common type of hemoglobinopathy
in which there is a substitution of amino acid glutamine Other symptoms: Infarction can only occur in bone and
on position 6 present in the chain of the HbA, by valine; spleen but, other tissues may also be involved. In infants,
giving rise to an abnormal Hb, i.e. hemoglobin S. In fingers and toes are commonly affected. Thrombosis of
homozygous individuals, whole of HbA is replaced by HbS vessels in brain cause severe neurologic disorders like
and this is known as sickle cell disease and in heterozygous stroke, convulsion, coma, drowsiness as well as speech
individuals, only 50 percent of HbA is replaced by HbS visual and hearing disturbances. Occlusion of smaller
and this is known as sickle cell trait. It is chronic hemolytic vessels results in headache and cranial nerve neuropathy
blood disorder characterized by abnormal hemoglobin including palsy and paresthesia.
(deoxygenated hemoglobin) which under low oxygen Oral manifestation: The oral mucosa will show pallor and
tension, results in sickling of cell. jaundice. There may be delayed eruption and hypoplasia
When HbS is deoxygenated, it forms structures know of the dentition, secondary to their general development.
as tactoids which distort the RBC membrane and produce Patient is more prone to develop osteomyelitis. This may
characteristic sickle shaped cell which are destroyed by be due to hypovascularity of the bone marrow secondary to
RE cells. Sickle cells increase blood viscosity and tend thrombosis. Patient may present with paresthesia of mental
to reduce blood flow leading to thrombosis and tissue nerve which may be secondary to occlusion involving the
infarction. nerves and blood supply. Mongoloid facies with high cheek
In addition these cells are phagocyte in large number bones and bimaxillary proganthism is present. It is due to
by mononuclear-phagocyte system which reduce their life marrow hyperplasia resulting in an increase in hard palate
span and give rise with hemolysis. Patients may develop length and palate alveolar ridge angle.
severe folic acid deficiency due to increased erythropesis.
Radiological features: There is reduced trabecular pattern
due to increases hematopoiesis of bone marrow. In some
Clinical and Radiological Features cases hair on end appearance is seen in the skull.
It is common in females and mostly the clinical symptoms
become evident before the age of 30 years. Hematological and Histopathological Findings
The RBC count may reach level of 10 lac cells or less
Symptoms: Clinical manifestations begin only after several
per cu mm with Hb levels from 5 to 12 g/dL. The sickle
months as fetal Hb protects against sickling phenomenon.
hemoglobin molecule undergoes gelation or crystalliza-
It includes dehydration, chills and infection but some time
tion, when deoxygenated and this typically distorts the
the attack occurs spontaneously. There is fatigue, weakness
erythrocyte, producing the sickle or boomerang shape (Fig.
and shortness of breath. Severe abdominal pain, muscle
28.3). This sickle shaped cells then ‘logjam’ and produce
and joint pain, at high temperature which may result in
stasis within the microvasculature. Damage to erythrocyte
circulatory collapse. There is painless hematuria.
membrane also occurs in sickle cells and leads to their
There is enlargement of heart and murmur is found in most fragmentation and intravascular hemolysis. Reticulocyte
of the patients. There is increased susceptibility to infection. count is raised.
some instance this is most common inherited disorders that

affect human beings.
696 Types
Alpha thalassemia: There is reduction or absence of
chain synthesis. Alpha chains of hemoglobin are required
not only for HbA but also for HbF, which is the main
hemoglobin type in fetal life. Therefore, major type of
alpha thalassemia is incompatible with life and results in
hydrops fetalis and intrauterine death of fetus.
Beta thalassemia: There is reduction or absence of beta
chains. Hemolysis is not primarily due to lack of β-globin
chains but it is because of the free alpha chains which form
insoluble aggregates that precipitate within the RBCs and
cause damage to the cell membranes. The red cells which
are present are very fragile and survive for only few days
Figure 28.3 Sickle cell shaped of red blood cells in peripheral circulation. In order to maintain the adequate
oxygenation the rate of hematopoiesis is increase 30 times
than normal to produce massive bone marrow hyperplasia,
Management hepatosplenomegaly.
Prevention of episode by avoiding the precipitating factors Thalassemia major or homozygous β-thalassemia:
is an important aspect of treatment. Patients should avoid Occurs when the patient is homozygous. It is also called
becoming chilled, dehydrated or exposed to hypoxia (high Cooley’s anemia.
attitude). Hemoglobin H disease: It is very mild form of the disease
Regular folic acid supplement (5 mg/daily) and blood in which the patient may live relatively normal life. There
transfusion should be given. is three altered gene.
Genetic counseling should be done. Termination of
pregnancy if fetus is affected by sickle cell disease. Now- Hemoglobin Bart disease: In which infants are stillborn
a-day molecular evaluation of DNA of single cell obtained or die shortly after birth.
from an embryo that was fertilized from in vitro allow Thalassemia intermedia: It is group of disorders
selection of non-affected embryo for uterine implantation. characterized by clinical manifestations between major and
minor.
Points to Remember
Sickle cell trait tactoids sickle cell crisis, acute chest Thalassemia minor or thalassemia trait: Occurs when
syndrome, paresthesia of mental nerve, mongoloid the patient heterozygous.
facies, hair on end appearance sickle or boomerang, Types
shape, genetic counseling, folic acid supplement.
Alpha thalassemia, beta thalassemia, thalassemia major
or homozygous β-thalassemia, hemoglobin H disease,
Thalassemia hemoglobin Bart disease, thalassemia intermedia,
It is also called Cooley’s anemia, Mediterranean anemia Thalassemia minor or thalassemia trait.
and erythroblastic anemia. Either alpha or beta globulin
genes may be affected. The resultant red blood cells have
reduced hemoglobin are thin and have shortened life span. Clinical, Oral and Radiological Features
It is autosomal dominant. It is an inherited impairment of Beta thalassemia
hemoglobin synthesis in which there is partial or complete It occurs between the ages of 6 to 24 months and after
failure to synthesize a specific type of globin chain. By the age of 6 to 8 months, development and growth of
Blood Pathology
the child is retarded. Survival time is short. The patient

first presents with pallor of skin, fever, chills, malaise,
generalized weakness, prominent cheek bone and mild
hepatosplenomegaly. 697
Mongoloid appearance: Bone marrow hyperplasia in

early life may produce frontal head bossing and there
may be marked overdevelopment of malar bone which
is associated with a short nose having a depressed bridge
giving the appearance of mongoloid. Deposition of iron
in various organs (due to multiple transfusions) leads to
signs and symptoms of organ failure. Most patients die in
childhood due to anemia and cardiac failure.
There is excessive overgrowth of maxilla causing excessive
lacrimation and nasal stuffness. The oral mucosa is pale
and has a lemon yellow tint because of chronic jaundice.
The color is best seen at the termination of hard palate and
in the floor of mouth. Figure 28.4 Hair on end appearance in patient with thalassemia
Rodent facies: There is marked over development of

maxilla associated with hyperplasia of alveolar process,
which results in anterior open bite and prominent cheek Histopathological Findings
bone producing characteristic ‘rodent facies’. The presence of typical safety pin cells and nucleated
red blood cells in circulation are characteristic.
The maxillary teeth are protrudes with spacing between
them with a short upper lip due to a lag between the growth Management
of the maxilla and the growth of upper lip. Due to high
concentration of iron discoloration of dentin and enamel Blood transfusion should be administered every two to
may be evident. three weeks. But major disadvantage of repeated blood
transfusion is iron overload (hemochromatosis). It may
Chip munk facies: There is also saddle nose, prominent cause death due to toxic accumulation on heart and liver.
malar bone and pneumatization of maxillary sinus. As a To overcome this problems iron chelating agent like
result of these skeletal changes, the upper lip is retracted deferoxamine should be given.
giving the child a ‘chip munk’ facies. There is poor healing Other therapy like splenectomy, folic acid supplement,
after dental treatment. surgical correction of abnormal facial appearance should
Radiological features: There is prominent hair on end be done.
appearance (Fig. 28.4).
Points to Remember
Alpha thalassemia Cooley’s anemia, Alpha thalassemia, Beta thalassemia,
The clinical findings are severe alveolar bone loss, Mongoloid appearance, Rodent facies, Chip munk facies,
pronounced spacing of maxillary anterior teeth and hair on end appearance, safety pin cells and nucleated
mongoloid appearance. red blood cells, blood transfusion, splenectomy, folic
acid supplement.
Laboratory Findings
The anemia is hypochromic and microcytic. The peripheral
smear shows abnormal RBCs. Reticulocyte count is Aplastic Anemia
increased. Bone marrow shows increased erythropoietic It is a rare disorder characterized by peripheral blood
activity. Serum bilirubin and fecal and urinary urobilinogen pancytopenia (anemia, leukopenia and thrombocytopenia)
are elevated because of severe hemolysis. An elevated fetal associated with bone marrow suppression. In most cases
hemoglobin is present. bone marrow suppression is not known and hence is known
as idiopathic aplastic anemia. Fanconi’s anemia is an ecchymosis. Large ragged ulcers covered by gray or black
inherited anemia that manifests in early childhood. In this necrotic membrane may be present, which are the result of
there is failure of hematopoietic precursor cells to produce generalized lack of resistance to infection and trauma.
698 adequate number of all types of blood cells.
Histopathological and Hematological Findings
Etiology RBC count is remarkably diminished, as low as 1 million
Common drugs which can cause aplastic anemia are cells/mm3. WBC count is as low as 2000/mm3 and platelet
benzene derivatives, chloramphenicol, amidopyrine, count may fall below 20000/mm3. The classical finding
organic arsenicals, colloidal silver, bismuth, mercury, is that of pancytopenia along with reduction of absolute
sulfonamides, penicillin and anticancer drugs. reticulocyte count. Bleeding time is prolonged and clotting
Patient with bacterial disease such as tuberculosis and time is normal. Anemia is normocytic with some degree
viral infections like hepatitis and infectious mononucleosis of macrocytosis. Bone marrow is fatty, acellular, and few
can cause pancytopenia. Long-term continuous exposure developing cells (Fig. 28.6).
to small amounts of external radiation or to internally Histopathological features shows acellular bone
deposited radium or thorium has been followed by the marrow with extensive fatty infiltrated. There is also
development of aplastic anemia. presence of many microorganism and inflammatory cells.

It can occur at any age, but is common in young adults. Withdrawal of offending agent: Withdrawal of offending
Patient may feel weakness after slight physical exertion agents may sometime lead to recovery.
and exhibits pallor of skin.
Antibiotics: Appropriated antibiotics should be given to
Symptoms: Breathlessness, headache, ankle edema are combat infection.
common clinical features. Numbness and tingling of Bone marrow transplantation: Now-a-day this therapy
extremities with edema are often encountered. Anemia has got acceptance to replace defective bone marrow.
may be severe enough to cause anginal pain or congestive
cardiac failure. Immunosuppressive therapy: Anti-hemocyte globulin
combine with cyclosporine produced response in majority
Bleeding: There may be bleeding from various sites like of cases.
skin, nose, vagina and gastrointestinal tract associated with
fever due to infection.
Oral features: The mucosa shows pallor. In some cases,
spontaneous hemorrhage may occur form the gingiva
can occur (Fig. 28.5). Petechiae, often are present on the
soft palate and in severe cases, there may submucosal
Figure 28.5 Diffuse gingival hyperplasia with bleeding Figure 28.6 Bone marrow of patient having aplastic anemia
Blood Pathology
Other therapy: Stimulation of hemopoiesis by androgens, Acute Posthemorrhagic Anemia

bone marrow transplantation and anti-fibrinolytic agents.
The anemia caused by blood loss may occur in variety
Points to Remember of conditions causing bleeding. When blood loss occurs 699
in large amounts in a short period of time, anemia may
Fanconis anemia, benzene derivatives, breathlessness,
develop even though iron stores remain adequate. It is
ankle edema, bleeding, hemorrhage in gingiva, RBC
called acute posthemorrhagic anemia.
count 1 million cells/mm3, acellular bone marrow, bone
marrow transplantation, immunosuppressive therapy. Clinical Features
The manifestations of hemorrhagic anemia depend on the
Hereditary spherocytosis rate and magnitude of bleeding; the time elapsed since it
In this disorder, red blood cells are excessively permeable to took place and the site-whether it is external or internal.
sodium ion. The osmostic fragility of red cells is abnormal When the blood loss is about 500 to 1000 mL, most
in these conditions. It will lead to loss of cell membrane of the patients do not present any symptoms, but few may
and gradually the cell becomes spherical in shape and is present with weakness and sweating.
destroyed by spleen, giving rise to hemolytic anemia. With rapid loss of 1000 to 1500 mL, a previously
healthy individual may experience lightheadedness and
Clinical Features hypotension.
Symptoms are usually present in childhood. There is mild- When 1500 to 2000 mL of blood is lost, symptoms like
to-moderate hepatosplenomegaly, jaundice and anemia. sweating, thirst, shortness of breath, clouding or loss of
A hemolytic and aplastic crisis may be precipitated by consciousness are seen. The pulse becomes rapid and low
infection or may occur with any cause. Such patients in volume, skin become cold and clammy and there is a fall
will have shivering, fever, marked weakness, vomiting, in blood pressure. When rapid loss of blood exceeds 2000
abdominal pain, dyspnea and palpitation. to 2500 mL, severe state of shock is reached.
Hematological Findings Hematological Features

The peripheral smear shows presence of spherocytes. Reti- Plasma volume and red cell mass are reduced in proportional
culocyte count is increased. Hb ranges from 5 to 12 g/dL. amount. Anemia is normocytic and normochromic.
Erythropoietin secretion is stimulated which in turn gives
Management rise to hyperplasia of marrow erythroid elements within 3
Splenectomy is the treatment of choice but should be done to 5 days. Increased in reticulocyte number. Neutrophilic
after the child is 6 years old. Folic acid 5 mg daily. leukocytosis often follows hemorrhage and is maximum
after 2 to 5 hours.
Points to Remember
Management
Permeable to sodium ion, hepato-splenomegaly, jaun-
Restoration of blood volume. Intravenous infusion of saline,
dice, spherocytes, splenectomy.
dextrin, albumin or plasma. Whole blood replacement is
the therapy of choice.
Hereditary elliptocytosis
It is a genetically determined abnormality of the red cell Points to Remember
shape associated with variable degree of hemolysis.
Blood loss, lightheadedness, shortness of breath, cold
and clammy skin, normocytic and normochromic
Clinical Features
anemia, whole blood replacement.
Most of the patients have no clinical manifestations but
few may show signs of chronic hemolytic anemia. Erythroblastosis fetalis
It occurs due to isoimmune antibodies. It is also called
Hematological Findings hereditary disease of new born (HDN). Congenital
The peripheral smear shows larger number of elliptocytes. hemolytic anemia due to Rh incompatibility results from
Mild anemia of normocytic, normochromic type. destruction of fetal blood brought about by a reaction
between maternal and fetal blood factors. The Rh factor, level of 100 units. The peripheral smear shows large number
named after Rhesus monkey, was discovered by Landsteiner of immature RBCs. There is also evidence of hemolysis.
and Wiener in 1940 as a factor in human RBC which reacts
700 with rabbit antiserum produced by administration of red Points to Remember
bloods cells from Rhesus monkey. Stillborn, Kernicterus, green, brown or blue hue teeth,
enamel hypoplasia, Rh hump, red blood count less the
Pathogenesis 1,000,000 cells per cubic millimeter.
It occurs due to inheritance, by the fetus, of a blood factor
from the father that acts as a foreign antigen to mother. Erythropoietic porphyria
The transplacental transfer of antigen and leak of red cells, Is a group of inherited or acquired disorders characterized
from fetus to mother results in immunization of mother by excessive production, accumulation and excretion of
and formation of antibodies. When the fetal red cells cross some porphyrins and their precursors or by-products. It is a
placenta they may stimulate the production of maternal metabolic defect within the maturing erythrocytes resulting
antibodies against the fetal antigens. in excessive production of uroporphyrinogen-I.
Some of these antibodies then cross into fetal
circulation and cause the destruction of fetal red cells. If Clinical Features
the father is Rh-positive and the mother is Rh-negative, It occurs soon after birth. The urine is burgundy red in
fetus inherits Rh-positive antigens, which may act as color or turns red on exposure to light. Photosensitivity
antigen to the mother and immunize her with resultant of exposed parts of body leads to formation of blister and
antibody formation. The problem is complicated by Rh scars. There is also hemolytic anemia and splenomegaly.
antigens which are termed as C, D and E. Out of this, D Oral features: There is deposition of porphyrins
antigen is the strongest and is responsible for the clinical in dentin, to a lesser extent in the enamel which imparts
manifestations of erythroblastosis fetalis. red or brown color to deciduous and permanent teeth
(erythrodontia). The staining by uroporphyrin can be
Clinical Features confirmed by ultraviolet light which will produce red
Some infants are stillborn. Those that are born alive have fluorescence. Bullous erosions of oral mucosa can be seen.
anemia with pallor, jaundice, compensatory erythropoiesis In advanced cases, pigmented atrophic scars on lip are seen
(both medullary and extra-medullary) and edema resulting resemble the keloid structures. The patient is hence, unable
in fetal hydrops. to close his mouth.
Kernicterus: It may manifest itself by apathy and poor Hematological Findings

feeding and later by mental retardation, irritability and
About 50 percent normoblasts, reticulocyte and 50 percent
cranial nerve palsies.
RBCs exhibit intense red fluorescence under fluorescence
Oral features: It may manifest in teeth by the deposition microscope.
of blood pigment in enamel and dentin of developing teeth
giving them a green, brown or blue hue. It occurs in those Management
portions of the teeth which are being laid down during the Splenectomy, in cases of severe hemolysis may be carried
time when icterus was at its height. out.
Enamel hypoplasia is also reported occurring in some
cases of erythroblastosis fetalis and usually involves the Points to Remember
incisal edges of the anterior teeth and middle portion of the Burgundy red urine, erythrodontia, bullous erosions, red
deciduous cuspid and first molar crown. There is ring like fluorescence, splenectomy.
defect called Rh-hump.
Laboratory Findings Iron Deficiency Anemia

The red blood count at birth may vary from less the Iron is essential for synthesis of ‘hem’ portion of
1,000,000 cells per cubic millimeter to near a normal hemoglobin. Iron deficiency anemia is caused by imbalance
level. The icterus index is very high and may reach a between iron intake and loss or inadequate utilization. In
Blood Pathology
this iron which is present in the body cannot keep pace with
need for iron in the production of red blood cells.
Causes 701
It is caused by inadequate intake of iron. It may occur
due to malabsorption of iron due to hypochlorhydria
and diarrhea. There is increased requirement of iron in a
growing child and in pregnancy. Other factors which can
cause iron deficiency anemia are increased loss of iron due
to injury, recurrent epistaxis, peptic ulcer, blood loss in
menstrual flow (menorrhagia), parturition and subtotal or
complete gastrotomy.
Clinical Features
It occurs chiefly in women in the 4th and 5th decade of life. Figure 28.7 Atrophy of tongue papillae seen in iron
The patient experiences tiredness, headache, paresthesia deficiency anemia
and lack of concentration.
Koilonychias: Nails become brittle, flattened and often

show spoon shape (koilonychia).
There may be tingling, pins and needle sensation in the
extremities.
Dysphagia: Some patients develop pharyngeal mucosal
thickening and mucosal web formation, giving rise to
dysphagia.
Gastrointestinal symptoms: Liver and spleen may be
palpable. There may be gastrointestinal bleeding and
menorrhagia there by setting a vicious circle.
Oral Manifestations
Pallor: In iron deficiency there is pallor of oral mucosa
and gingiva. The normal pink color is lost due to lack of
oxygenated blood in the capillary bed in lamina propria Figure 28.8 Gingival enlargement in iron deficiency anemia
and is associated with lowered levels of hemoglobin. The
generalized atrophy of oral mucosa can occur.
Tongue: There is redness, soreness or burning of tongue. Recurrent aphthous ulceration and candidal lesions
The filliform papillae over the anterior two-thirds of tongue can also occur in iron deficiency anemia. Patient may show
are the first to undergo atrophy (Fig. 28.7). In severe cases slow healing after oral surgical procedures. In some cases
fungiform papillae are also affected leaving the tongue there may be gingival enlargement (Fig. 28.8).
completely smooth and waxy or glistening in appearance.
There is cracking and fissuring at the corner of mouth Hematological Findings
(angular cheilitis). There is softening of epithelium which The anemia is microcytic and hypochromic (Fig. 28.9)
leads to linear ulceration of the skin, extending up to and and the peripheral smear shows abnormal forms of RBCs.
beyond the mucocutaneous junction. There may be pain on There is reduced hemoglobin level, as low as 4 g/100 mL.
opening or stretching and rarely, bleeding from ulcerated There is normal or slightly reduced RBC count. MCV,
tissues. MCH and MCHC are all reduced.
sore mouth and inability to retain dentures. A smooth, red,

occasionally enlarged and often sore tongue with fissuring
is occurs. The width of mouth is narrowed and the oral
702 mucosa is pale and painful. There is also dry mouth and
spoon shaped nails.
Dysphagia: There are atrophic changes in mucosa of

mouth, pharynx, upper esophagus and vulva. This will
leads to dysphagia.
Esophageal webs: With evaluation with endoscopy it
shows abnormal bands of tissue in the esophagus. This is
called esophageal web.
Koilonychia: There is alternation of the growth pattern
with spoon shaped configuration.
Figure 28.9 Hypochromic types of iron deficiency anemia
Histopathological and Hematological Features
Hematologic study show hypochromic microcytic anemia.
Histopathological Findings There is atrophic epithelium and atrophy of lamina propria
There is marked thinning of epithelium, absence of pa- and muscles. There is also presence of submucosal chronic
pillae in the lamina propria and absence of keratohyaline inflammation. In advance cases there may be evidence of
granules. epithelial atypia or dysplasia.
Management Management
Almost all patients can be treated by oral supplements Dietary iron supplement should be given for the correction
of iron by giving ferrous fumerate or ferrous sulfate. It is of iron deficiency anemia. Esophageal dilation should be
given in dose of 300 mg three to four times a day for a done to improve symptoms of dysphagia.
period of 6 months.
Points to Remember
Points to Remember Paterson-Brown-Kelly syndrome dysphagia, iron
Menorrhagia, Koilonychias, dysphagia, gastrointestinal deficiency anemia, dystrophy of nails (koilonychia) and
bleeding, angular cheilitis, redness of tongue, recurrent glossitis.
aphthous ulceration, candidal lesions, microcytic
hypochromic anemia, epithelium thinning, iron supple- Pernicious Anemia
ment. It is also called primary anemia, Addison’s anemia or
‘Biermer’s anemia’. The term pernicious anemia should be
Plummer Vinson syndrome reserved for patients who have B12 deficiency secondary
It is also called Paterson-Brown-Kelly syndrome or to intrinsic factor deficiency. If there is extrinsic factor
sideropenic dysphagia. It is characterized by dysphagia, deficiency it is called megaloblastic anemia.
iron deficiency anemia, dystrophy of nails (koilonychia)
and glossitis. Causes
It occurs due to atrophy of gastric mucosa resulting in
Clinical Features failure to secrete the still unidentified ‘intrinsic factor’.
It is exclusively found in middle aged women. Patient of Intrinsic factor is produced by parietal cells of the stomach
this syndrome have got characteristic asthenic appearance. lining. This intrinsic factor is responsible for absorption of
Vermilion borders of the lip are very thin and there is vitamin B12. It is suggested that it is autoimmune disorder,
often angular cheilitis. Patients complaint of ‘spasm in because autoantibodies to gastric parietal cells are often
throat’ or food sticking in throat. There is also complain of found in serum of patients.
Blood Pathology
Clinical Features
It is rare before the age of 30 years and increase in frequency
with advancing age. Males are more commonly affected 703
than females.
There is usually triad of symptom: Generalized
weakness, sore painful tongue and numbness and tingling
of the extremities. Other features are fatigability, headache,
dizziness, nausea, vomiting, diarrhea, with loss of appetite,
shortness of breath, loss of weight, pallor and abdominal
pain.
Nervous system: Nervous system disorders are also

present and manifested by sensory disturbances including
the paresthetic sensation of the extremities, weakness,
stiffness and difficulty in walking, general irritability, Figure 28.10 Atrophy of tongue papillae in megaloblastic
depression, drowsiness as well as incoordination on loss anemia
of vibratory sensation. There is also tingling sensation in
the fingers and toes that eventually progress to numbness.
Gastrointestinal complaint: Epigastric discomfort,
constipation or diarrhea can be seen in these patients.
Oral Manifestations
There is glossitis and patient complains of painful and
burning lingual sensation which may be so annoying that
the dentist is often consulted first. The tongue is generally
inflamed often described as ‘beefy red’ in color, either
entirely or in patches scattered over the dorsum and lateral
border of tongue.
There is gradual atrophy of the papillae of tongue that
eventuates in a smooth and bald tongue which is often
referred as Hunter’s glossitis or Moeller’s glossitis and is
similar to the bald tongue of sandwith seen in pellagra (Fig.
28.10).
The fiery red appearance of tongue may undergo
remission but recurrent attacks are common. Sometimes Figure 28.11 Megaloblastic anemia showing hyperchromatic
inflammation and burning involve the entire oral mucosa. nuclei
Tongue may show lobulations, which may be secondary
to decrease in saliva production. There is disturbance in
taste sensation with intolerance to dentures and occasional There is atrophy of epithelium with intra or subepithelial
dryness of mouth. Oral mucosa shows greenish yellow chronic inflammatory cell infiltration. Cellular atypia can
color (frequently observed on the skin) at the junction of be seen. Loss of rete ridges is also a features of pernicious
hard and soft palate, when daylight is used for illumination. anemia.
Histopathological Features Laboratory Findings

Histologically, oral epithelial cells in pernicious anemia The red blood count is decreased often to three or less per
reveal enlarged, hyperchromatic nuclei with prominent cubic millimeter. Many of the cells exhibits macrocytosis,
nucleoli and serrated nuclear membranes (Fig. 28.11). while some exhibit poikilocytosis. Decreased WBC count
and mean corpuscular hemoglobin. In advanced cases of

anemia, there are polychromatophilic cells, stippled cells,
nucleated cells, Howell-Jolly bodies and Cabot rings.
704 Leukocytes are also often reduced in number but are
increased in average size.
Bone marrow shows great number of immature red
cells or megaloblasts with few normoblasts, indicating
maturation arrest at the more primitive megaloblasts state.
Achlorhydria or lack of gastric hydrochloric acid secretion
is a constant feature and pH of the gastric content is usually
high. Achlorhydria is associated with atrophy of the gastric
mucosa, which commonly occurs in presence of chronic
inflammation. Schilling test detects the absence of intrinsic
factor.
Figure 28.12 Purplish red discoloration of eyes of the patient
Management
Most of the patients should be given vitamin B12 parenterally
but, some of the cases are treated with massive oral dose Symptoms: Common symptoms are lassitude, loss of
of vitamin B12. The standard dosage is 100 mg IM every concentration, headache, dizziness and blackout, slurring
30 days. of speech, pruritis, mental confusion and indigestion.
Paresthesia is common, usually involving the cranial nerves.
Points to Remember Itching: This is very characteristic complaint given by
Addison’s anemia’ generalized weakness, sore painful the patient is that there is itching particularly after bathing
tongue, numbness and tingling of the extremities, bald without evidence of rash.
tongue of sandwith, fiery red appearance of tongue, The skin appears flushed or diffusely reddened.
cellular atypia, loss of rete ridges, immature red cells or Superficial veins are dark enlarged and distended. The tip
megaloblasts, vitamin B12 parenterally. of the finger usually has a cyanotic appearance. There is
also splenomegaly.
There is marked purplish red discoloration especially
Polycythemia Vera of the head and neck region (Fig. 28.12). Feet and hands,
It is defined as abnormal increases in the number of red which give the patient an extremely angry appearance.
blood cells in the peripheral blood, usually with an increase Erythromelalgia is peripheral vascular event which
hemoglobin level. It is also called polycythemia rubra affects hands and feet. There is painful burning sensation
Vera, Osler’s disease, primary acquired erythrocytosis associated with erythema and warmth. This again will
‘Vaquez’s disease and erythremia. There is an uncontrolled lead to occlusion of vessels which in turn results in digital
proliferation of the erythroid stem cells leading to gangrene and necrosis.
excess of erythroid cell mass in the body (RBCs). Thrombotic complications may occur with increase
There is accompanying increase in the granulocyte and viscosity of blood with increase platelet number. It may
megakaryocytic elements though to a lesser degree. It is leads to ischemic attacks, cerebrovascular accidents,
believed that single progenitor marrow stem cells become myocardial infarction.
multiplying without regard to normal regulatory hormone Oral features: There is purplish red discoloration of
like erythropoietin. This will lead to overproduction red the ears, oral mucosa, gingiva and tongue. The tongue may
blood cells. appear as if it had been painted with crystal violet. The
gingiva is markedly swollen and bleeds spontaneously,
Clinical Features but with no tendency to ulcerations. Petechiae of the oral
The disease is more common in males and usually occurs mucosa are common. In such patients severe hemorrhage
in middle age or later. may follow surgical procedures.
Blood Pathology
Hematological Findings or antimetabolics. This results due to inhibition of normal

mitotic division and maturation of hematopoietic cells.
The hemoglobin level is greater than 18 g/dL with an
Deficiency of vitamin B12 and folic acid. Disease causing
associated elevation of WBC and platelet count. The 705
sequestration of neutrophils includes systemic lupus
bone marrow is hypercellular with erythroid hyperplasia,
erythematosus and Felty’s syndrome. It can be associated
increased number of megakaryocytes and granulocytes.
with leukemia, pancytopenia and hypersplenism.
Management
Clinical Features
Phlebotomy: It is removal of excess blood (500 mL) daily.
This will reduce the red blood cell. It can occur at any age but it somewhat more common
For the management of thrombotic event low dose aspirin, in adults, particularly in woman. It is also common in
for control of platelet level anagrelide hydrochloride (selective professional and in hospital as they have easy access of the
inhibitor of megakaryocytic maturation) should be given. offending drugs and often use drug sample injudiciously.
Sign and symptoms: The onset may be sudden or
Points to Remember gradual. The condition begins with sore throat, high fever
and often rigors, which may be followed by prostration.
Osler’s disease, itching, angry appearance, thrombotic
The skin appears pale and anemic and in some cases,
complications, erythromelalgia, purplish red discolora-
jaundiced. There is rapidly advancing necrotic ulceration
tion of oral mucosa, hemoglobin level < 18 g/dL, low
of throat and mouth with little evidence of pus formation.
dose aspirin, phlebotomy.
In most of the cases patient dies within 3 to 5 days due to
toxemia and septicemia.
WHITE BLOOD CELL DISORDERS
Oral Manifestations
Agranulocytopenia The most common sites are gingiva and palate, tonsil
It is also called granulocytopenia, agranulocytic angina, and pharynx. There may be associated pain, excessive
agranulocytosis. It is a serious disease characterized salivation and spontaneous oral hemorrhage. The oral
by marked leukopenia with reduction and absence of lesions appear as necrotizing ulcerations of oral mucosa,
neutrophilic leukocytes. tonsils or/and pharynx.
The lesions appear as ragged and necrotic and are
Types covered with a gray black membrane. The necrotic tissue
• Primary agranulocytosis: In this, etiology is is often foul smelling. There is lack of inflammation at the
unknown. margin of lesions.
• Secondary agranulocytosis: In it, cause is recognized. The disease spreads quickly in gingival tissues causing
• Congenital agranulocytosis: Also called Kostmann destruction of supporting structures and inevitable loss
syndrome. This occurs due to decreased level of the of deciduous and permanent teeth. This features also
cytokine granulocyte colony stimulating factor resembles pattern of necrotizing ulcerative gingivitis.
• Mild neutropenia: When 1000/mm3 to 2000/mm3
neutrophils are present. Histopathological and Hematological Findings
• Moderate neutropenia: When 500/mm3 to 1000/
Majority of patients show a leukocyte count below 2000
mm3 neutrophils are present.
• Severe neutropenia: When fewer than 500/mm3 cells per cumm and granulocyte count below 100 cells per
neutrophils are present. cumm. Hemoglobin and platelet counts are normal. The
• Agranulocytosis: When no neutrophils are seen in histopathological features of ulcer shows that reduce or
peripheral smear. absence of neutrophils. It may show bacterial invasion.
Management
Causes If hemoglobin is less than 10 g/dL, transfusion of red cell
It is caused by certain drugs like aminophylline, concentrate is given. In some cases white cell transfusion
chlorpromazine and phenylbutazone, benzene, bismuth, can be given. Septicemia can be controlled by parenteral
chloramphenicol, sulfonamides and use of cytotoxic drugs antibiotic therapy along with corticosteroid therapy.
A combination of carbenicillin, methicillin and

gentamicin is commonly used because of broad coverage
against most organisms.
706 Chlorhexidine containing mouth wash reduce the
severity of oral lesion.
Points to Remember
Aminophylline, sore throat, necrotic ulceration of throat
and mouth, excessive salivation, necrotizing ulcerative
gingivitis, spontaneous oral hemorrhage, leukocyte count
below 2000 cells per cumm, absence of neutrophils,
transfusion of red cell concentrate.
Cyclic Neutropenia
It is also called periodic neutropenia, and cyclic Figure 28.13 Severe gingivitis in patient with cyclic neutropenia
hematopoiesis. It is a rare disorder characterized by periodic
or cyclic diminution in circulating neutrophils due to
failure of stem cells of bone marrow. One-third cases are Hematological Findings
inherited as autosomal dominant trait and two-thirds appear
spontaneously during the first few year of life. The patient Normal blood count, over a period of 4 to 5 days, begins to
is healthy between neutropenic periods; but at regular show a decline in the neutrophil count compensated by an
intervals, the absolute neutrophils count falls below 500/ increased in monocytes and lymphocytes. At the peak of
mm3. In some patient it comes to zero. The cause for this the disease, the neutrophils may completely disappear for
disorders mutation of neutrophils elastase (ELA2) gene. one or two days.

The disease is frequently present during infancy and There is no specific treatment and monitoring the patient
childhood and both sexes appear to be equally affected. for infection during neutropenic period and vigorous early
The frequency of neutropenic episodes vary from once in management of infection. Antibiotics should be given to
2 to 4 weeks which last for 3 to 5 days with 21 days gap combat infection.
being the most common. In some cases use of corticosteroids, adreno-
corticotropin (ACTH) or testosterone modulates sharp
Symptoms: The patients manifest fever, sore throat, stomatitis reduction in marrow function.
and regional lymphadenopathy as well as headache, arthritis, Administration of cytokine granulocyte colony
cutaneous infection and conjunctivitis. Less frequently stimulating factor (G-CSF) several times in week will
patient experience lung, urinary tract infection as well as correct the lack of production of neutrophils.
rectal and vaginal ulcer. Oral hygiene should be maintained and patient should
be recalled for oral hygiene every 2 to 3 months.
In some patients, amyloidosis can occur which is due
to repeated, increased antigenic stimulation during Points to Remember
neutropenic episodes. The symptoms are milder as
Periodic neutropenia, stomatitis, regional lymphadenopa-
compared to agranulocytosis.
thy, amyloidosis, severe gingivitis, no specific treatment.
Oral features: Severe gingivitis and painful ragged ulcers
that have a core like center are found on the lip, tongue,
Lazy Leukocyte Syndrome
palate, gums and buccal mucosa which heal after about
two weeks with scarring (Fig. 28.13). Isolated painful ulcer It is a result of loss of chemotactic function of the
may occur which correspond to the period of neutropenia. neutrophils. The neutrophils present in the blood can not
Blood Pathology
migrate to the site of tissue injury although phagocytic and

Points to Remember
bactericidal activities are normal.
Decreased chemotactic and bactericidal activity, oculo-
Clinical features: It becomes apparent at the age of 1 to cutaneous albinism, photophobia, severe gingivitis, 707
2 years when complication occurs due to infections. The glossitis, giant abnormal granules, antibiotics.
most common clinical manifestations are stomatitis, otitis
media and bronchitis. Chronic Idiopathic Neutropenia
Oral manifestations: Gingivitis and stomatitis are It consists of group of diseases which includes familial
common oral finding of this disorder. In some cases neutropenia, chronic benign neutropenia, chronic neutro-
periodontal disease may be present. penia and hyperplastic neutropenia. Etiology is unknown,
Hematological findings: The total leukocyte count is but they are characterized by a decrease in production of
slightly low but the absolute neutrophil count is as low as neutrophils below 1500/mm3 in the bone marrow. Some of
100 to 200 cells/mm3. The bone marrow contains normal ethnic group like African and Middle Eastern background
number of mature neutrophils. will have neutrophils count constantly below 1200/mm3.
But this people live healthy life. This type of neutropenia is
Points to Remember called benign ethnic neutropenia.
Loss of chemotactic function, otitis media, gingivitis, Clinical Features
stomatitis, absolute neutrophil count is as low as 100 to
Predominantly in females with some cases being familial.
200 cells/mm3.
It is usually asymptomatic and free of infection which is
due to compensatory monocytosis, which accounts of
Chediak Higashi Syndrome normal number of phagocytes at the site of tissue injury.
It is a congenital autosomal recessive defect of granulocytes Some patients may exhibit recurrent bacterial
and melanocytes. Abnormal granules are seen in all blood infections like recurring upper respiratory tract infections,
granulocytes resulting in decreased chemotactic and bacteri- otitis media, bronchitis and furunculosis. When neutrophil
cidal activity, although phagocytosis remains intact. count falls below 500/mm3 pulmonary infection can occur.
Clinical features: The characteristic clinical feature of this Oral Manifestations

disease consists of oculo-cutaneous albinism, photophobia, Severe, rapidly advancing periodontal diseases. Gingivae
nystagmus and recurrent infections of the respiratory tract appear intensely red with granulomatous margins. Severe
and sinuses. There may be neurological and gastrointestinal gingival recession, advanced bone loss, mobility, denuded
disturbances. The disease may be associated with malignant roots and loss of teeth.
lymphoma. The second most common finding is recurring oral
Oral manifestations: Ulcerations of the oral mucosa, ulcers and in some cases, maxillary sinusitis can also occur.
severe gingivitis and glossitis are the common oral lesions.
Laboratory Findings
There may be rarely loss of teeth due to periodontal disease.
The bone marrow of patients shows a normal number
Hematological findings: Hematological studies show of immature cells but decrease in number of mature
presence of giant abnormal granules in the granulocytes in neutrophils. This is called maturation arrest. Absolute
the peripheral blood as well as in their precursors in the neutrophils count may below 500/mm3. Bacterial invasion
bone marrow. of the host tissue can be seen.
Treatment: Immediate and proper treatment with
antibiotics of the infection as soon as they occur is most Management
important. Vincristine, prednisolone and ascorbic acid Patient who is having infection can be given antibiotics
have been tried as the treatment of this disorder. therapy. Proper oral hygiene should be maintained.
Patient can be given recombinant human granulocyte Sequestration in the spleen: Usually platelet is sequestered
colony stimulating factor (G-CSF) which promotes growth in spleen. So when splenomegaly occurs large number of
and differentiation of neutrophils. platelet are sequestered.
708
Points to Remember Clinical Features
Familial neutropenia, benign ethnic neutropenia, Clinical evidence is seen when platelet count is below
asymptomatic, recurrent bacterial infections, advance 100,000/mm3. Thrombocytopenic purpura is characterized
periodontal diseases, recurring oral ulcers, decrease by spontaneous appearance of purpuric hemorrhagic
mature neutrophils, recombinant human granulocyte lesions of skin, which vary in size from tiny red pin point
colony stimulating factor. petechiae to large purplish ecchymoses and sometimes,
even massive hematoma.
Symptoms: The patient also exhibits a bruising tendency.
DISEASE OF PLATELET Epistaxis, hematuria and melena are common findings. Intrac-
ranial hemorrhage is rare, but can be seen in children and the
Idiopathic Thrombocytopenic Purpura symptoms are headache, dizziness and confusion.
It is also called Werlhof’s disease, purpura hemorrhagic and
primary thrombocytopenic purpura. It is a disease in which Oral Manifestations
there is an abnormal reduction in the number of circulating The first manifestation of the disease can be seen in
blood platelets with normal or raised number of megakaryo- oral cavity in the form of excessive bleeding after tooth
cytes in the bone marrow. It is thought to be an autoimmune extraction. Submucus petechiae and ecchymosis commonly
disorder in which a person becomes immunized and devel- occur especially at the junction of the hard and soft palate. It
ops antibodies against his own platelets. appears as numerous tiny, grouped clusters of reddish spots,
only a millimeter or less in diameter (Figs 28.14 and 28.15).
Causes Petechiae do not blanch on pressure which is the
Decrease production of platelets: It may be results of distinguishing feature between purpura and telangiectasia.
infiltration of bone marrow by malignant cells or toxic In severe cases, extensive spontaneous gingival bleeding
effect of cancer chemotherapeutic drugs. may be seen and this may form foci of secondary infection.
Increased destruction of platelets: It may be cause of Hematological and Histopathological Findings

immunological reaction which can be precipitated by many The platelet count is usually below 60,000 cells per
drugs. Most common drug is heparin. cumm. The bleeding time is prolonged but, the clotting
Figure 28.14 Idiopathic thrombocytopenic purpura showing Figure 28.15 Idiopathic thrombocytopenic purpura showing
hemorrhage purpuric lesion on the soft palate
Blood Pathology
There are widespread microthrombi in the arterioles,
venules and capillaries in all tissues and organs throughout 709
the body. The intravascular thrombi are composed of
loose aggregates or platelet that becomes organized into
amorphous plugs, which are often replaced by fibrin. This
fibrin deposit more readily seen on PAS stain.
Management
Corticosteroids, platelet aggregation inhibitors, splenectomy
and exchange transfusion.
Points to Remember
Immunologically mediated, thrombocytopenia, hemo-
Figure 28.16 Purpura showing megakaryocytic hyperplasia
lytic anemia, microthrombi, amorphous plugs, corticos-
teroids, platelet aggregation.
time is normal. The bone marrow reveals megakaryocytic
hyperplasia (Fig. 28.16). When severe bleeding occurs, Aldrich Syndrome
there may be associated iron deficiency anemia. Gingival
It is also called Wiskott-Aldrich syndrome. It is X-linked
biopsy shows presence of fibrin deposit in the small vessels.
recessive condition.
This can be seen more with PAS stain.
Clinical features: It is characterized by thrombocytopenia,
Management
eczema, increased susceptibility to infection and a prolonged
Corticosteroids, splenectomy, transfusion, local hemostatic bleeding time. Patients commonly manifest boils, otitis
agents should be given to control the bleeding. media, bloody diarrhea and respiratory infection. There
is common occurrence of malignant lymphoma, which is
Points to Remember an important feature of this disease. Bleeding occurs from
Werlhof’s disease, sequestration in the spleen, purpuric nose, skin and gastrointestinal tract.
hemorrhagic lesions of skin, bruising tendency, epistaxis,
Oral manifestations: Palatal petechiae are frequently
bleeding after tooth extraction, petechiae, platelet count
present. Spontaneous bleeding from the gingiva.
below 60,000 cells per cumm, presence of fibrin deposit in
the small vessels, corticosteroids, local hemostatic agents. Laboratory findings: Prolonged bleeding time and there
is considerable anisocytosis, alternation in the size and
shape of platelets with most platelets smaller than normal.
Thrombotic Thrombocytopenic Purpura There is decreased production and defective maturation of
It is immunologically mediated. It is characterized by platelets since normal megakaryocytes may be seen in the
occlusion of small arterioles and capillaries of many organs marrow.
by thrombi formed of fibrin and platelets.
Points to Remember
Clinical Features Wiskott-Aldrich syndrome thrombocytopenia, prolonged
It generally occurs in young adults and more commonly bleeding time, palatal petechiae, spontaneous bleeding
in females than in males. There is thrombocytopenia, from the gingiva, anisocytosis, platelets size and shape
hemolytic anemia, fever, transitory neurologic dysfunction alternation.
and renal failure.
Familial Thrombasthenia Laboratory Findings

It is also called Glanzmann’s disease. It is hereditary, The platelet count is increased and there is abnormal
710 chronic hemorrhagic disease transmitted as an autosomal aggregation in response to several aggregating agents. The
recessive trait. clotting time, prothrombin time are normal but bleeding
time is frequently prolonged.
Clinical features: There is excessive bleeding, either
spontaneous or following minor trauma. Both sexes may Management
be affected and onset of menarche may be a critical event. Certain cytotoxic drugs, heparin, corticosteroid and aspirin
Purpuric hemorrhages of skin are common, as are epistaxis in small dose may help to counter act the thrombactive
and gastrointestinal bleeding. Hemarthrosis is also noted in tendency. It responds to radioactive phosphorus.
some cases.
Oral manifestations: Spontaneous bleeding from the oral Points to Remember
cavity particularly gingival bleeding. Tendency to bleed, thrombic episodes epistaxis, spon-
taneous gingival bleeding, platelet count is increased,
Laboratory finding: Bleeding time is prolonged while
cytotoxic drugs, heparin.
clot retraction is impaired. Platelet count is normal as the
clotting time. The aggregation of platelet by epinephrine,
ADP and thrombin is defective. DISEASE DUE TO CLOTTING DEFECT
Management: No specific treatment but patient in oral Hemophilia
surgery can be given microfibriallar collagen preparation
with fibrinolytic inhibitors. Hemophilia (hemo-blood, philia-loving) represent bleeding
disorders with deficiency of clotting factor. It is a hereditary
Points to Remember disorder of blood coagulation characterized by excessive
Glanzmann’s disease, purpuric hemorrhages of skin, hemorrhage due to a prolonged coagulation time. It is also
bleeding time is prolonged. called Bleeder’s disease, disease of Hapsburgs, disease of
Kings.
Thrombocytosis or Thrombocythemia When you bleed, the body launches a series of reactions
that help the blood clot. This is called the coagulation
It is characterized by an increase in the number of platelets
cascade. The process involves special proteins called
in the blood in excess of 1000 × 106 /dL. It is of two types,
coagulation factors. When one or more of these clotting
i.e. primary and secondary.
factors are missing, there is usually a higher chance of
Causes bleeding.
It is caused by polycythemia, myeloid leukemia, anemia, Types

tuberculosis and sarcoidosis. Other diseases which can
cause this disorders are hyperadrenalism, rheumatoid Hemophilia A: Deficiency of factor VIII or anti-hemophilic
arthritis and bronchial carcinoma. factor is the cause of hemophilia. It is transmitted as
X-linked recessive character carried on X-chromosome.
Clinical Features The males are clinically affected and the females are
carriers of the trait. Hemophilia A occurs 10 times more
It is a rare disorder of the elderly associated with a tendency
commonly than hemophilia B.
to bleed and to have thrombic episodes. Epistaxis, bleeding
into the gastrointestinal tract as well as bleeding into the Hemophilia B or Christmas disease: It occurs due to
genitourinary tract and central nervous system is common. hereditary deficiency of factor IX or functionally defective
Hemorrhages in skin are also common. factor IX. It is transmitted as X-linked recessive character
through chromosome. It is very rare, compared to hemophilia
Oral Manifestations A. In the laboratory, hemophilia A may be differentiated
Excessive and prolonged bleeding after extraction of tooth from hemophilia B by modification of the prothrombin
is also common. consumption time or the partial thromboplastin time.
Blood Pathology
Hemophilia C: This is very rare form of hemophilia

and it occurs due to deficieny of factor XI or plasma
thromboplastin antecedent.
711
Types
Hemophilia A, Hemophilia B or Christmas disease,
Hemophilia C.
Clinical Features
Sex predilection: This disorder usually affect the male
and female usually carry the trait. This can be transmitted
through unaffected daughter to grandson. The sons are
hemophilic patient are normal and are not carrier. The
heterozygous daughter carry their defect to half of their son
and recessive trait to their daughters. Figure 28.17 Bleeding seen in patient with hemophilia
Bleeding manifestations usually begin after 6 months of

age, when the child begins to move about and tends to fall Hematological Findings
and sustain injuries, i.e. when spontaneous hemorrhage is
Clotting time is prolonged, but however the bleeding
noted by the parents.
time, platelet count and prothrombin time are all normal.
Hemarthrosis: The most common manifestation is The prothrombin consumption time and the partial
hemorrhage into joints which is spontaneous and associated thromboplastin time may be prolonged in severe cases.
with warmth and muscle spasm. Repeated episodes cause
damage to the joint with wasting of the related muscle, Management
leading to deformity and crippling. Replacement therapy: Various forms of replacement
therapy are available like plasma, cryoprecipitate and
Hemorrhage into subcutaneous tissues, internal organs and
factor VIII concentrates.
musculature also are frequent and potentially disabling
The use of aspirin is strictly contraindicated in patient
complications. Superficial trauma gives rise to uncontrolled
with hemophilia. Aspirin gives adverse effect on the blood
bleeding. Intracranial bleeding is relatively rare unless
platelet factor. Genetic counseling should be provided so
associated with trauma.
that families understand the mechanism of inheritance.
Pseudotumor of hemophilia: Sometimes when the Optimal dental care should be done while dealing with
hemorrhage in the tissue tumor like mass may be form. surgery in dentistry like periodontal surgery, oral surgery.
This is called pseudotumor of hemophilia. In this condition Consultation with the patient physician is mandatory while
there is formation of reactive new bone which cause dealing with this patient.
expansion of bone. Clotting factor replacement may cause many time com-
Oral manifestation: Traumatic injury of the oral plications. Most common while doing cryoprecipitation
cavity may lead to the diagnosis of hemophilia. (method of concentrating clotting factor from the serum)
The anatomic sites involved in persistent oral bleeding several viruses like hepatitis viruses, HIV can also become
are the frenum of lip and the tongue (Fig. 28.17). There concentrated. So many hemophilic patients are affected
is prolonged bleeding after tooth extraction. Hematoma with HIV infection. Now-a-day due recombinant DNA
of the floor of mouth may occur and blood may spread technology, this products can be produce without contami-
via the fascial spaces and produce a hematoma of the nation of viral organism.
larynx, with consequent respiratory embarrassment.
Points to Remember
Physiological processes of tooth eruption and exfoliation
may be associated with severe and prolonged hemorrhage. Bleeder’s disease, hemarthrosis, hemorrhage into subcuta-
Gingival hemorrhage is extremely rare and when it does neous tissues, pseudotumor of hemophilia, persistent oral
occur, it is the result of gingival injury. bleeding, prolonged clotting time, replacement therapy.
Von Willebrand’s Disease Points to Remember

It is also called pseudo-hemophilia, vascular hemophilia Pseudo-hemophilia, excessive bleeding, gingival bleed-
712 and vascular purpura. It is a rare disorder which is inherited ing, epistaxis, severe menorrhagia, prolonged bleeding
as an autosomal dominant trait in which there is defect in time, transfusion of plasma.
all three components of the hemostatic mechanism; the
capillaries, platelets and coagulation mechanism. It is most
Plasminogen Deficiency
common hereditary clotting disorder. This is discovered
by Erik Adolf von Willebrand in 1926. This is caused It is also called ligneous conjunctivitis or hypoplasmi-
by genetic deficiency of plasma glycoprotein called von nogenemia. It is rare autosomal recessive disorders
Willebrand’s factor. This glycoprotein helps in adhesion cause by mutation of genes responsible for production of
of platelet at the site of bleeding. This factor also binds to plasminogen the precursors to plasmin.
factor VIII acting as transport molecule. It is transmitted as
an autosomal-dominant trait and thus there may be evidence
Pathogenesis
of a family history of excessive bleeding. Most forms of the In clotting mechanism, activation of factor lead to the
disease show incomplete penetrance of the phenotype and formation of clot. At the same time, plasminogen is
variable expressivity of bleeding symptoms within families. converted into plasmin which is responsible for degradation
of clot. In the deficiency state of plasminogen, plasmin
Clinical Features cannot be formed so degradation of clot does not takes
place. So clot tend to grow and persist.
Excessive bleeding either spontaneously or following even
minor trauma, is the chief feature of the disease. The most Clinical Features
common sites of bleeding are nose, skin. Bleeding into the
Age and sex distribution: It is more common in women as
gastrointestinal tract, epistaxis and severe menorrhagia are
compared to men. First decade of life is age of occurrence.
also common. Bleeding tendencies usually appear early in
childhood and decrease in middle and old age. Site: Most commonly seen on conjunctiva, laryngeal
mucosa, oral mucosa and vaginal mucosa.
Oral features: Gingival bleeding and post-extraction
bleeding are the most common oral manifestations. Conjunctiva: Most common affected site is conjuctival
The disease may be discovered after dental extraction. mucosa. There is formation thick, firm plaque which is
Continuous oral bleeding over long periods of time fosters yellow in color. This resembles woodlike that is reason it is
deposits of hemosiderin and other blood degradation called ligneous (ligneous means woodlike).
products on the tooth surfaces, turning them brown.
Oral lesion: They are usually patchy ulcerated papule and
nodules with irregular surface seen on gingiva. They tend
Hematological Findings to wax and wane in severity.
Prolonged bleeding time ( in some cases over 300 minutes),
normal platelet count, failure of aggregation of platelets Histopathological Feature
impaired adherence of platelets and depressed levels of There is accumulation of fibrin appear as diffuse sheets of
factor VIII are suggestive of this disorder. Low AHG acellular eosinophils material which resembles amyloid.
levels and abnormal platelet retention to glass beads. The Inflammatory cells and granulation tissue is seen adjacent
platelet count and prothrombin consumption are normal. to fibrin deposit.
The clotting time usually is normal but capillary fragility
is increased. Management
Damage to tissue should be minimized.
Management Topical heparin with prednisone may be given.
Bleeding episode is best controlled by transfusion of plasma Topical or systemic plasminogen yields good results.
and or cryoprecipitate and by local control of homeostasis. But these agents are not available commercially.
Blood Pathology
Oral features: Orally there is spontaneous gingival

Points to Remember
bleeding and excessive bleeding after dental extraction.
Ligneous conjunctivitis, thick, firm plaque on conjunc-
tiva, patchy ulcerated papule on gingiva, accumulation Laboratory finding: Clotting and prothrombin time is 713
of fibrin, topical heparin, topical or systemic plasmi- infinite. Peripheral blood fails to clot even after addition
nogen. of thrombin. ESR is zero and cells remain suspended even
after 24 hours.
Factor V Deficiency or Parahemophilia Management
It is a rare htemorrhagic disorder, clinically similar Transfusion of concentrated fibrinogen during bleeding
to hemophilia, caused by a deficiency of factor V or episode. Prognosis is very poor and patient die in infancy
proaccelerin. It is inherited as an autosomal dominant trait or early childhood.
affecting both sexes.
Clinical features: Spontaneous epistaxis, bleeding into Points to Remember

the gastrointestinal tract and menorrhagia are common. Blood does not clot, excessive bleeding, spontaneous
Cutaneous ecchymosis and hamartomas are frequently gingival bleeding, clotting and prothrombin time is
seen, although petechiae are rare. Intraocular hemorrhage infinite, ESR is zero, transfusion, concentrated fibrinogen.
and hemorrhage into the central nervous system have been
also reported.
Oral manifestation: Spontaneous gingival bleeding occurs DYSFIBRINOGENEMIA
in some cases and prolonged bleeding after extraction of
This disease is transmitted as an autosomal dominant trait
tooth is observed.
which represent the group of familial disorders. In this
Laboratory finding: Both clotting and prothrombin time disease fibrinogen is present in normal amount but structure
are prolonged but, bleeding time is normal. The basic is defective. Due to this aggregation of fibrin monomers is
defect is reduction in plasma proaccelerin. impeded.
Clinically there is mild-to-severe bleeding tendency. In
Management: Transfusion and freshly frozen plasma are
some cases paradoxical thrombosis is also been reported.
given when there is excessive hemorrhage.
Prothrombin time and activated partial thromboplastin
Points to Remember time is prolonged.
Deficiency of factor V, spontaneous epistaxis, menor- Fresh frozen plasma or cryoprecipitate may be
rhagia, cutaneous ecchymosis, spontaneous gingival transfused in case of bleeding.
bleeding, clotting and prothrombin time are prolonged,
transfusion. Fibrin Stabilizing Factor Deficiency
It occurs due to factor XIII deficiency which is discovered
Afibrinogenemia and Hypofibrinogenemia by Duckert in 1960. It is autosomal recessive trait. Inherited
It is also called hypofibrinogenopenia. It is very rare disease factor XIII deficiency occur due to mutation in the gene
where blood does not clot as there is absence of fibrinogen. encoding located on chromosome 6.
This disease can be congenital or acquired. Congenital is Clinical features: There is severe postsurgical bleeding
transmitted as autosomal recessive trait occurring in both episodes which can be continuous for more than a day.
the sexes. Acquire occur secondary to defective fibrinogen There is also hemarthrosis, and defective wound healing.
formation. There is also bleeding in mouth during teething. Soft tissue
Clinical features: Patient usually suffer from excessive bleeding and bruising is also very common.
bleeding in the lifetime. It is very difficult to distinguish Laboratory finding: Bleeding and clotting time is normal.
it from hemophilia. There is also bleeding from nose, in Measurement of clot stability is diagnostic for deficiency
gastrointestinal tract and central nervous system. of factor XIII.
Management
Plasma, cryoprecipitate and factor XIII concentrate have
714 been used.
Points to Remember
Inherited factor XIII deficiency, severe postsurgical
bleeding episodes, hemarthrosis, measurement of
clot stability, plasma, cryoprecipitate and factor XIII
concentrate.
MACROGLOBULINEMIA
It is also called Waldenstrom hypergamma-globulinemia
Figure 28.18 Enlargement of lymph nodes in Hodgkin’s
or macroglobulinemia of Waldenstrom. This condition is
lymphoma
describe in 1948 by Waldenstrom. It occur due to excessive
proliferation of B lymphocytes which results in production
of large amount of electrophoretically homogeneous IgM
It was first described by British pathologist Thomas
globulins which is characteristic of the disease.
Hodgkin in 1832. It is characterized by painless enlargement
Clinical features: It is seen in older individual with no sex of lymphoid tissue throughout the body.
predilection. Patient complaint of pallor, weakness, weight
loss, lymphadenopathy, and hepatomegaly. There is also
Etiology
hemorrhage from nasal cavity, ocular hemorrhage. Particularly Epstein-Barr and oncorna virus are being
investigated as possible etiological agents. Sometimes, it
Oral manifestation: There is spontaneous gingival bleeding,
can occur without any etiological factor.
with continuous oozing of blood. Focal area of hypermia
with bleeding oral ulcer on the tongue can also occur. Clinical Features
Laboratory finding: There is macroglobulinemia and Age and sex distribution: It is characterized by a bimodal
hyperglobulinemia. Patient manifest severe anemia. Bone age incidence, peak one in young adults and the second
marrow smears shows increase mononuclear cells. in the 5th decade of life with equal distribution between
sexes.
Management
Location: The onset is insidious, usually with enlargement
Chlorambucil in high dose can produce remission in some
of one group of superficial nodes. The cervical lymph
patient. Supportive therapy with whole blood replacement
nodes are usually the first to be involved but the disease
is also a treatment of choice.
may start in the mediastinal, axillary, abdominal, pelvic or
Points to Remember inguinal lymph nodes.
Waldenstrom hypergamma-globulinemia, lymphadeno- Symptoms: The involved nodes are painless. Generalized
pathy, pallor, spontaneous gingival bleeding, macroglo- weakness, loss of weight, cough, dyspnea and anorexia
bulinemia, hyperglobulinemia, chlorambucil. are seen. There is pain in back and abdomen owing to
splenic enlargement, due to pressure of enlarged nodes or
involvement of vertebrae.
MALIGNANCY INVOLVING BLOOD
TISSUE Signs: The lymph nodes are discrete and rubbery in consist-
ency with overlying skin being freely mobile. Splenomegaly
Hodgkin’s Lymphoma is usually seen in later stage. Some patients may manifest
It is lymphoproliferative disorders arising from lymph pruritis. Characteristic features of this disease are:
nodes and from lymphoid components of various organs ∙ Pel-Ebstein fever, a cyclic spiking of high fever and
(Fig. 28.18). generalized severe pruritis of unknown etiology.
Blood Pathology
Pressure of enlarged lymph nodes on adjacent structures – M ixed cellularity: Lymphocytes, plasma cells,
may cause dyspnea, dysphagia, venous obstruction, eosinophils, easily identified Reed Sternberg cell.
jaundice and paraplegia. – Nodular sclerosis: Sparse lymphocytes, stromal
715
Clinical Stages (ANN Arbor Staging) cell, fibrosis and numerous but bizarre Reed
Sternberg cell, poor prognosis.
• S tage I: Involvement of single lymph node region or
– Lymphocyte depletion: Lymphocytes, plasma
extra-lymphatic sites.
cells, eosinophils with localized involvement.
• Stage II: Involvement of two or more lymph node
– Unclassified: Hodgkin’s lymphoma does not
regions or an extra-lymphatic site and lymph node
resembles any of the above criteria.
region on the same side of diaphragm.
• Stage III: Involvement of lymph node region on the
both sides or without extra-lymphatic involvement or Laboratory Investigations
involvement of spleen or both. Full blood count: Anemia which is normocytic and
• Stage IV: Diffuse involvement of one of more extra- normochromic is a common finding. The total WBC count
lymphatic tissues, e.g. liver or bone marrow. is normal but there may be mild eosinophilia. In the terminal
A-No systemic symptoms stage there, may be leukopenia and thrombocytopenia.
B-Systemic symptoms such as weight loss, fever, night
ESR and LDH is raised, liver function may be abnormal
sweats are present.
due to infiltration in liver.
Oral Manifestations Chest radiography: It is done to permit staging.

Primary jaw lesions are uncommon. Secondary effect
Management
can be seen in oral cavity in the form of infection due to
reduced host immune response. Radiotherapy: Irradiation treatment 3500 to 4000
rads/ week over the involved region plus all adjacent
Appearance: It may appear in the oral cavity as an ulcer sites been given in stage I and II. It is also given after
or a swelling or as an intra-bony lesion which presents as chemotherapy, to sites where there was originally bulk
a hard swelling. disease.
Histopathological Features Chemotherapy: It is given in stage III and IV. Usually
It is characterized by replacement of normal lymph node combination is given. First combination is MOPP, i.e.
architecture by an admixture of malignant lymphoid cells mustine HCl (6 mg/m 2 IV on day 1 and day 8), oncovin
and non-neoplastic inflammatory cells. which is also called vincristine (1.4 mg/m 2 IV on day 1
and 8), procarbazine (100 mg/m 2) orally from day 1 to 14)
Reed Sternberg cells: There are sheets of lymphoid and prednisone (40 mg/m 2 orally from day 1 to 14). MOPP
cells with interposed vacuolated spaces containing combination given in six courses with no drugs is given
characteristic bi-nucleated mononuclear cells (owl-eye from day 15 to 28. Second combination is ABVD regimen
nuclei). Multinucleated giant cells are also present. i.e. adriamycin (25 mg/m 2 IV bolus on day 1,8 and 14),
Popcorn cells: Malignant cells in lymphocytes predominant bleomycin (10 mg/m 12 bolus on day 1,14) vinblastine (6
resembles the nucleus to kernel of popped corn. mg/m 2 IV bolus on day 1,14) and decarbazine (375 mg/m
2 IV bolus on day 1,14). The cycle should be repeated on
Histological Types 20th day.
• N odular lymphocytes predominant Hodgkin’s Combination: A combination of radiotherapy and
lymphoma chemotherapy may increase the overall response and long-
• Classical Hodgkin–lymphoma term survival but, it is associated with delayed complications
– L
ymphocyte rich: Abundant lymphocytes, few like leukemia, gonadal atrophy and avascular necrosis of
plasma cells, occasional Reed Sternberg cell, bone.
localized involvement on one side of diaphragm
Splenectomy: Splenectomy is advocated in many patients,
and most favorable prognosis.
except with stage IV disease.
Lymph nodes: Painless lymph node enlargement of

Points to Remember
abdominal and mediastinal region is the most common
Lymphoproliferative disorders, enlargement of one finding. Very often the first group of lymph nodes affected
716 group of superficial nodes, painless, loss of weight, may be cervical, axillary or inguinal.
cough, dyspnea, lymph nodes are discrete and rubbery
in consistency, Pel-Ebstein fever, cyclic spiking of high Signs: Pressure effect of lymphoma may cause dysphagia,
fever, oral cavity as an ulcer or a swelling, admixture breathlessness, vomiting, intestinal obstruction or
of malignant lymphoid cells, Reed Sternberg cells, ascites and paraplegia. If liver and spleen are involved
owl-eye nuclei, Popcorn cells, ESR and LDH is raised, hepatosplenomegaly is present. The growth is fleshy and
radiotherapy, chemotherapy, splenectomy. is prone to ulceration.
Oral Manifestations
Non-Hodgkin’s Lymphoma Occurrence of malignant lymphoma in oral cavity is rare,
It is also called lymphosarcoma. In this group, there is when present it is more often found to arise from the tonsils,
neoplastic proliferation of lymphoid cells, usually affecting although other oral tissue may also be involved.
the B-lymphocytes. Unlike Hodgkin’s lymphoma, the Palatal lesions have been described as slow growing,
disease is frequently widespread at the time of diagnosis, painless, bluish soft tissues mass which may be confused
often involving not only the lymph nodes but also bone with minor salivary gland tumors.
marrow, spleen and other tissue. Early involvement of Paresthesia of mental nerve has been reported.
bone marrow is typical of this lymphoma. Sometimes there is pain and neuralgia in the region of 2nd
and 3rd division of 5th cranial nerve. In rare cases necrotic
Types proliferation of palate may also be seen. The swelling may
• Nodular ulcerate.
• Diffuse
Etiology It shows ill defined or ragged radiolucency. In later stage
it may caused expansion of the bone which can perforate
The etiology is unclear but Epstein-Barr and herpes the cortex.
virus etiology has been suggested. There may be induced
immunologic effect permitting a malignant clone to Histopathological Features
proliferate.
Nodular or follicular: In the nodular pattern, the neoplastic
Clinical and Radiological Features cells tend to aggregate in such a way that large clusters of
cells are seen.
Age and sex distribution: It affects persons of all ages Diffuse: Diffuse pattern is characterized by a monotonous
from infants to the elderly, but is most common in middle distribution of cells (Fig. 28.19) with no evidence of
age group. Males are affected more commonly than the nodularity or germinal center pattern.
females. If it arises in lymph node, then tumor destroyed normal
Location: In the oral cavity it frequently occurs in tonsils. architecture of nodes.
The other sites affected are salivary glands or jaws.
Symptoms: The onset of symptoms may be insidious. The
patient complaint of tiredness, loss of weight, fever and Blood count usually shows normal levels but if there is
sweating. Pain is the main symptom of bone involvement associated hypersplenism or hemolytic anemia the reduced
which may present as a pathological fracture. Patient may WBC and RBC counts are seen along with reduced
complain abdominal pain, nausea, vomiting, diarrhea or hemoglobin levels and reticulocytosis.
intestinal obstruction which may occur due to involvement In some cases there may be slight increase in lympho-
of gastrointestinal tract. cytes and thrombocytopenia.
Blood Pathology
PRIMARY RETICULAR CELL SARCOMA

It is also called primary lymphoma of bone.
717
Clinical Features
Age and sex distribution: It can occur in any age group
but it is common amongst young adults under the age of 40
years. It is common in males as compared to females.
Location: It is most commonly seen in femur, tibia and
humerus.
Signs and symptoms: It is usually asymptomatic except
for the presence of localized swelling of the involved bone.
Regional lymphadenopathy is usually present.
Figure 28.19 Diffuse distribution of monotonous abnormal
lymphocytes invading the connective tissues Oral Manifestations
It is not common, but it usually occurs more in mandible
Moderate degree of anemia will also present when than maxilla.
there is considerable bone marrow involvement. Some
Symptoms: Pain is common complaint and usually present
very aggressive high grade non-Hodgkin lymphomas are
for years and more.
associated with very high orate levels which can precipitate
renal failure. Signs: There is also presence of expansile mass. The oral
mucosa over the involved bone seldom gets ulcerated but
Management at times, appears diffusely inflamed. The teeth usually
Chemotherapy: When diagnosed in late stages, become exceedingly loose, owing to destruction of bone.
chemotherapy is the treatment of choice. In most cases When the lesion involves maxilla, there may be
single agent chemotherapy (chlorambucil) is usually given. evidence of expansion of the bone as well as symptoms of
Combination with prednisolone is also useful. A combination nasal obstruction due to superior growth of tumor into the
of cyclophosphamide, doxorubicin, vincristine, bleomycin floor of nasal cavity.
with prednisolone is commonly used.
Radiotherapy: Radiotherapy is used to treat local disease
and the patient is given a total dose of 150 rads spread over The primary cells of tumor are identical with that of the soft
a period of five weeks. tissue tumors. There may be inflammatory cell infiltration
which can lead to confusion in the diagnosis of the lesion.
Transplantation: Studies of autologous stem cell The individual cells are mixed with both mature
transplantation are in progress. appearing lymphocytes and large histiocytoid lymphoblast.
Points to Remember
Management
Lymphosarcoma, Epstein-Barr, herpes virus, tonsils,
tiredness, loss of weight, fever and sweating, painless Surgical excision or X-ray radiation, as it appears to be
lymph node enlargement of abdominal and mediastinal radiosensitive. The 5-year survival rate is between 50 and
region, dysphagia, breathlessness, vomiting, intestinal 60 percent.
obstruction or ascites and paraplegia, paresthesia of
mental nerve, slow growing, painless, bluish soft tissues Points to Remember
mass, ill-defined or ragged radiolucency, expansion of Primary lymphoma of bone, asymptomatic, localized
the bone, nodular or follicular, diffuse, moderate degree swelling, oral mucosa over the involved bone seldom
of anemia, slight increase in lymphocytes, chemotherapy, gets ulcerated, destruction of bone, inflammatory cell
radiotherapy, transplantation. infiltration, large histiocytosis lymphoblast.
MYCOSIS FUNGOIDES
It is also called cutaneous T-cell lymphoma. It usually affects
718 the skin. Mycosis fungoides exhibits epidermotrophism
(i.e. propensity to invade the epidermis of skin).
Clinical Features
Age and sex distribution: It is usually seen in adults with
male predilection in male in the ratio of 2:1.
Appearance: The disease commences with eczematous
lesion, which gradually develop into thickened plaques.
Size: It varies in size from few millimeters to centimeters
in diameter and finally spread to lymph nodes, spleen and
liver.
Figure 28.20 Erythematous lesion seen in myocosis fungoides
Eczematous stage (erythematous stage): There are well
demarcated, scaly erythematous patches with pruritus.
microabscess. In some cases, nuclei are so convoluted that
Plaque stage: Erythematous patches developed into they are described cerebriform due to infolding of nuclear
elevated red lesion membrane.
Tumors stage: Plaque become papules and nodules. In Tumors stage: There is a dense and diffuse infiltration of
this stage visceral involvement occur. lymphoid cells with irregularly shaped nuclei.
Sezary syndrome: There is generalized exfoliative
Management
erythroderma, lymphadenopathy, hepatomegaly,
splenomegaly with involvement of lung, kidney and CNS. Topical nitrogen mustard, topical carmustine, electron
It results in patient death in short period of time. beam therapy, topical corticosteroids and PUVA therapy
are effective in mycosis fungoides.
Oral Manifestation
Points to Remember
Oral lesions can be the first manifestations and sometimes
appear after the skin lesions have been treated and remitted. Cutaneous T-cell lymphoma, eczematous stage (erythe-
matous stage), plaque stage, tumors stage, Sezary syn-
Location: It is occur on tongue, palate, buccal mucosa, lip, drome, oral lesions can be the first manifestation, indurat-
gingiva and tonsil. ed areas, nodules or erythematous ulceration on tongue,
psoriasiform pattern of epithelial alteration, parakeratin
Appearance: The lesions appear as indurated areas,
production, mycosis cells or Sezary cells, Pautrier’s mi-
nodules or erythematous ulceration (Fig. 28.20).
croabscess, cerebriform, dense and diffuse infiltration of
Histopathological Features lymphoid cells, topical nitrogen mustard, topical carmus-
tine, electron beam therapy, PUVA therapy.
Eczematous stage: In this psoriasiform pattern of epithelial
alteration is seen. Scattered, slightly atypical lymphocytes
with parakeratin production seen in the connective tissue BURKITT’S LYMPHOMA
papilla of mycosis fungoides. There is also elongation of It was described by Dennis Burkitt in 1950. It is also
epithelial rete pegs. called African jaw lymphoma. It is a lymphoreticular
Plaque stage: There is infiltration by atypical lymphocytes cell malignancy. In the African form jaw involvement is
cells which are called mycosis cells or Sezary cells. Mycosis 75 percent and in cases of the American form, abdomen
cells form small intraepithelial aggregates called Pautrier’s involvement is more common. It is a B cell neoplasm.
Blood Pathology
Etiology of arch and occlusion. There may be large quantity of mass

protruding into the mouth, on the surface of which may be
Epstein-Barr virus (EBV) which also causes nasopharyngeal
seen rootless, developing permanent teeth.
carcinoma and infectious mononucleosis is considered to 719
be the etiological factor. There are higher EBV antibody Progress of lesion: Once the tumor perforate the bone it
levels patients of Burkitt’s lymphoma patients. is initially confined by the periosteum, but subsequently it
spreads to the soft tissues of the oral cavity and face where
Types rapid tumor growth soon obliterates the entire face and skin
• A frican Burkitt’s lymphoma: It is seen in African becomes tense and shiny.
children with predilection of jaw
• American Burkitt’s lymphoma: It is seen other Radiological Features
countries with predilection for abdominal There is radiolucent destruction of bone with ragged and ill-
• Immunodeficiency associated Burkitt’s lymphoma: It defined margin. There is also patchy loss of lamina dura.
is seen in HIV patients
• Endemic Burkitt’s lymphoma: It is seen in Brazil in Histopathological Features
increase prevalence. Monotonous sea of undifferentiated monomorphic
lymphoreticular cells, usually showing abundant mitotic
activity is seen. There is hyperchromatosis and loss of
Clinical Features cohesiveness.
Age and sex distribution: Peak incidences in children Macrophages with abundant clear cytoplasm often
between 6 and 9 years. Males are affected more commonly containing cellular debris scattered throughout the tumor
than the females, with a ratio of 2:1. producing a very characteristic starry sky appearance (Fig.
28.21). This appearance occurs due to less intensely stain
Location: It is more are found in maxilla than in mandible, macrophages in comparisons with surrounding tissue.
where it may spread rapidly to the floor of the orbit. Almost Macrophages appear as star against night sky of deeply
always occurs in molar area. In the African form more than hyperchromatic neoplastic lymphoid cells.
one quadrant is involved while in the American form, only Nuclei are uniform in size, mostly round but
one quadrant is involved. occasionally may be ovoid with a slight indentation. The
The most important hallmark of this tumor is readily of nuclear membrane is prominent.
growth with a tumor doubling time of less than 24 hours.
Signs and symptoms: The most common presenting
features are swelling of the jaws, abdomen and paraplegia.
It is painless. Peripheral lymphadenopathy is common.
Oral Manifestations
It begins generally as a rapidly growing tumor mass of
the jaws, destroying the bone with extension to involve
maxillary, ethmoid and sphenoid sinus as well as orbit.
There may be loosening or mobility of permanent teeth.
There is gross distortion of the face due to swelling.
Paresthesia and anesthesia of inferior alveolar canal or
other sensory facial nerves is common.
Gingiva and mucosa adjacent to the affected teeth
become swollen, ulcerated and necrotic. As the tumor mass
increases, the teeth are pushed out of their sockets. Swelling Figure 28.21 “Starry sky” appearance- Burkitt’s lymphoma
of the jaw occurs and it may cause facial asymmetry. showing uniform distribution of lymphocytes and macrophages
They are capable of blocking nasal passages, displacing with clear cytoplasm interspersed between giving the characteristic
orbital contents and eroding through skin. There is derangement appearance
Management 2nd
It is rapidly fatal in the absence of treatment, with • Acute lymphoblastic leukemia
720
death occurring within 6 months. Cytotoxic drugs like – L1 : Acute lymphoblastic (principally pediatric)
cyclophosphamide 40 mg/kg in single IV administration – L2 : Acute lymphoblastic (principally adults)
and repeated about 2 weeks later. – L3 : Burkitt’s lymphoma
Vincristine and methotrexate have been successful in • Acute non-lymphoblastic or myeloid leukemia
some cases. – M0: Myeloblastic (without maturation)
Combination of drugs such as cyclophosphamide,
– M1: Myeloblastic (with little maturation)
vincristine and methotrexate give better results than any
– M2: Myeloblastic (with maturation)
single drug. Majority of patients show dramatic response to
the therapy. The swelling regresses and the displaced teeth – M3: Promyelocytic
return to their normal position within 1 to 2 weeks. – M4: Myelomonocytic
– M5: Monocytic
Points to Remember – M6: Erythroleukemia – bizarre, multinucleated,
– M7–Megakaryocytic
African jaw lymphoma, EBv virus, maxilla than in • Chronic lymphatic leukemia
mandible, peripheral lymphadenopathy, swelling of the • Chronic myeloid leukemia
jaws, abdomen and paraplegia, doubling time of less
than 24 hours loosening or mobility of permanent teeth.
There paresthesia and anesthesia of inferior alveolar Types
canal is teeth are pushed out, derangement of arch and ∙ Stem or blast cell leukemia: When the leukemic cells
occlusion, patchy loss of lamina dura, radiolucent de- are too immature to be classified as to cell type, the
struction of bone with ragged and ill-defined margin, leukemia is termed as ‘stem’ or ‘blast’ cell leukemia.
undifferentiated monomorphic lymphoreticular cells, ∙ Subleukemia: When the total WBC is normal and
usually showing, hyperchromatosis, loss of cohesive- leukemic cells are seen in the peripheral blood is termed
ness, macrophages, starry sky appearance, vincristine as subleukemia.
and methotrexate, cyclophosphamide, vincristine and ∙ Aleukemia: When no abnormal leukocytes can be
methotrexate. found in the peripheral blood (i.e. they can be found
only in the bone marrow) the term aleukemia is used.
Leukemia ∙ Leukemoid reaction: When the peripheral blood
picture in non-leukemic patient resembles that of
It is also called leukosis. It is defined as a neoplastic leukemia it is called a leukemoid reaction. In this,
proliferation of WBC in bone marrow, usually in circulating absolute neutrophil count remains above 30,000/mm3.
blood and sometimes in other organs such as liver, spleen
and lymph nodes. Presence of leukemic cells in bone Classification
marrow results in impairment of normal hemopoiesis with
Acute
resultant anemia, granulocytopenia and thrombocytopenia.
Leukemia is a progressive and fatal condition causing Acute lymphoblastic leukemia
death from hemorrhage and infection. There is presence ∙ L1: Acute lymphoblastic (principally pediatric)–in it,
of excessive number of abnormal cells in the peripheral small cells predominate and nuclei are generally round.
blood but leukemia is considered a primary disorder of ∙ L2: Acute lymphoblastic (principally adults)–cells are
bone marrow. heterogeneous in size and sharp in features, nuclei
often show cleft.
Classification ∙ L3: Burkitt’s lymphoma–there is homogeneous
population of large cells. Nuclei are round to oval with
1st
prominent nucleoli.
• Stem or blast cell leukemia
• Subleukemia Acute non-lymphoblastic or myeloid leukemia
• Aleukemia ∙ M0-Myeloblastic (without maturation): Myoblasts
• Leukemoid reaction predominate with distant nucleoli, few granules are
present.
Blood Pathology
∙ M1: Myeloblastic (with little maturation) Immunological deficiency syndrome: The persons
∙ M2–Myeloblastic (with maturation): Myeloblast and suffering with Wiskott-Aldrich syndrome can develop
promyelocytes predominate and Auer rods are seen. leukemia.
∙ M3–Promyelocytic: Hypergranular promyelocytes 721
often with Auer rods are seen. Acute Leukemia
∙ M4–Myelomonocytic: Myelocytic and monocytic Acute leukemia is a disorder in which there is a failure
differentiation evident, myeloid elements resemble of maturation of leukocytes. As a results, there is an
peripheral monocytosis. accumulation of immature cells within the bone marrow
∙ M5–Monocytic: Promonocytes or undifferentiated and later in the blood. It is the most common type of
blast. leukemia.
∙ M6–Erythroleukemia: Bizarre, multinucleated, mega-
loblastoid erythroblast predominate. Pathophysiology
∙ M7–Megakaryocytic: Pleomorphic undifferentiated There is a block in differentiation of leukemic and stem cells
blast cells with anti-platelet antibodies, myelofibrosis and leukemic blasts have prolonged, rather than shortened
is present. generation time. Thus, accumulation of leukemic blast in
acute leukemia results primarily from failure of maturation
Chronic into functional stage. As leukemic blast accumulates in
∙ Chronic lymphatic leukemia (lymphogenous, lympho- the marrow, they suppress the normal hematopoietic stem
cytic) involving lymphocytes series. cells. The mechanism is not fully understood. Suppression
∙ Chronic myeloid leukemia (myelogenous, myelocytic) part is related to physical replacement of normal precursor
leukemia involving granulocyte series. cells by expanded clones of leukemic cells. Clinical
manifestations result from paucity of normal red cells white
Etiology cells and platelets, this occur due to myelophthisic anemia,
Virus: Epstein-Barr virus, herpes like virus and HTLV i.e. crowding out of the normal hematopoietic stem cells by
(human T-cell leukemic virus) have been considered to be malignant proliferation.
the etiological agents responsible for leukemia.
Clinical Features
Radiation and atomic energy: If given over the dose
Age and sex distribution: It is more common in children
of 100 rads, it is known to significantly increase the risk
and young adults between the age of 15 and 39 years.
of leukemia. Leukemia among radiologists and Japanese
Males are affected more commonly than females with a
exposed to the atomic blast are more, as compared to
ratio of 3:2. There is abrupt stormy onset with pyrexia and
other population. It is also common in patients receiving
enlargement of spleen.
X-radiation for rheumatoid spondylitis.
Symptoms: usually results, from bone marrow suppression
Chemical agents: Chronic exposure to aniline dyes;
and infiltration of other organs and tissues by leukemic
benzene and phenylbutazone have been recognized to
cells. Weakness, fever, headache, generalized swelling of
be associated with leukemia. Usually in these patients
lymph nodes, petechiae or hemorrhage in skin and mucous
pancytopenia due to marrow hyperplasia occurs prior to
membrane are seen. There is bone pain and tenderness,
leukemia.
resulting from marrow expansion, with infiltration of
Anticancer drugs: Patient treated with anticancer drug subperiosteum. Central nervous manifestations such as
like melphalan and chlorambucil have an increased risk headache, vomiting, nerve palsies resulting form meningeal
of developing leukemia, usually of acute myelocytic spread which more common in children than in adults; and
variety. more common in ALL than AML.
Genetic and chromosomal factors: Philadelphia Signs: The clinical features are due to anemia and
chromosome is found in about 15 percent of cases of thrombocytopenia viz pallor, dyspnea, fatigue, petechiae,
acute lymphocytic leukemia. It suggests that if one set of ecchymosis, epistaxis and melena. Hepatosplenomegaly is
identical twins develop leukemia before the age of 6 years, present in later stages. There is an increased susceptibility
the risk of disease in other twins is 20 percent. to infection.
Cervical lymphadenopathy, secondary to pharyngeal Hematological Findings

sepsis is seen. Intracranial and subarachnoid hemorrhage
The total WBC count may vary from a very low count less
may result from thrombocytopenia and leukastasis
722 than 1X106 per cumm to as high as 5000 X 106 per cumm
(intravascular clumping of leukemic blasts in the small
or more.
blood vessels of brain). There is recurrent infection
The peripheral smear shows significant number of
of lungs, urinary tract skin, mouth, rectum and upper
immature granulocytes or lymphatic precursors or even
respiratory tract, which may result in fever.
stem cells.
Chloroma: Localized tumors consisting of leukemic Bone marrow is hypercellular with replacement of
cells are called chloromas, surface of which turn green normal marrow elements by leukemic blast cells in varying
(chlor) when exposed to light because of the presence of degree. There is an associated normochromic anemia,
myeloperodioxase. thrombocytopenia and decrease in normally functioning
neutrophils.
Oral Manifestations
The submental, cervical and pre- and post-auricular lymph Management
nodes may be enlarged and tender. Phase of induction: It is treated using combination
Paresthesia of lower lip and chin may be present. There of vincristine (1.4 mg/m 2 every week of 1 month),
may be toothache due to leukemic cell infiltration of dental L-asparaginase (600 units/m 2 biweekly for 1 month) and
pulp. prednisone (40 mg/m 2 orally daily for 1 month). As the
The oral mucous membrane shows pallor, ulceration leukemic cells regress, regrowth of normal cells occurs and
with necrosis, petechiae, ecchymosis and bleeding tendency the patient goes rapidly into remission.
(Figs 28.22 and 28.23). There may be massive necrosis of
Phase of consolidation: In it, drugs uses include
lingual mucosa with sloughing. There may be crusting of
daunorubicin, mercaptopurine, cytarabine and methotrexate
lip occur due to repeated bleeding from lip.
with intra-thecal therapy using the last two drugs, together
Gingival hypertrophy: Gingiva shows hypertrophy and with irradiation of the cranium to eradicate, the disease from
cyanotic discoloration. The hypertrophy may be due to central nervous system. Radiation reduces the risk of relapse
leukemic cell infiltration within gingiva or due to local in central nervous system when given with methotrexate.
irritants. The gingiva appears boggy, edematous and deep
red bleed easily due to ulceration of sulcus epithelium and Phase of maintenance: In this phase, patient receives a
necrosis of underlying tissue. repeating cycle of above drugs until two or three years
Mobility of permanent teeth may be present. Oral have been completed.
infections (candidal, viral and bacterial) are serious and Transplantation: Allogenic bone marrow transplantation
potentially fatal complication in leukemic patients. from HLA identical twin is done. Ablative therapy has
Figure 28.22 Bleeding occur from lip in patient of leukemia Figure 28.23 Crusting of lip occur in patient of leukemia
Blood Pathology
been utilized to eradicate the patient’s residual leukemic Pathophysiology

cells and normal cells with intensive irradiation and
It is associated with the presence of a distinctive
chemotherapy. After this reconstruction of the hemopoietic
chromosomal abnormality, i.e. philadelphia chromosome. 723
tissue is done with precursor cells from the donor.
Acutenon-lymphoblastic leukemia is treated with Clinical features
daunorubicin, cytarabine and 6-thioguanine. All these Age and sex distribution: The disease occurs chiefly
drugs are very toxic to the normal as well as leukemic between the age of 35 to 60 years. The disease may be
cells and therefore, treatment is given in pulses to reduce discovered during routine examination, when splenomegaly
toxicity. or an elevated count is noted.
Catabolic products of leukemic cells produce uric
Symptoms: There may be slowly advancing anemia with
acid and cause hyperuricemia, which is prevented by
loss of weight, prominence of abdomen and discomfort
allopurinol.
in the left upper quadrant due to splenomegaly. Attacks
Supportive therapy: Transfusion of red cells and of acute left upper abdominal pain may develop due to
platelets may be required in cases of severe anemia and infarction of spleen. Anemia causes weakness, fatigue
thrombocytopenia. Combination of higher antibiotics and dyspnea on exertion.
like aminoglycosides with cephalosporin, allopurinol,
Signs: As the disease progress thrombocytopenia can
before starting anti-leukemic agents are given to prevent
cause petechiae, ecchymosis as well as hemorrhage from
hyperuricemia.
the skin and mucous membrane. Liver may be enlarged but
Topical treatment to stop gingival bleeding includes
lymph nodes are normal.
removing obvious local irritants and direct pressure. Use
of absorbable gelatin or collagen sponge topical thrombin Hematological Findings
is helpful.
Examination of blood shows a normocytic and
The management of oral ulcers includes topical anti-
normochromic anemia. WBC count is considerably
bacterials with povidone iodine solution, chlorhexidine
increased and may be between 50 × 106 and 500 × 106 cells
rinses or tetracycline rinses.
per cu mm.
Psychological support is very important as delusion,
Peripheral smear shows mature leukocytes but, few
hallucinations and paranoia are not uncommon during
immature forms may also be present (Fig. 28.24). The
periods of severe bone marrow failure.
platelet count is often high initially but with treatment, it
Points to Remember comes down.
Failure of maturation of leukocytes, marrow suppression
and infiltration of other organs, headache, vomiting, nerve
palsies, pallor, dyspnea, fatigue, petechiae, ecchymosis,
cervical lymphadenopathy, chloroma, paresthesia of lower
lip, bleeding tendency, pallor, ulceration with necrosis,
petechiae, ecchymosis, gingival hypertrophy, WBC count
less than 1 × 106 per cumm, immature granulocytes,
bone marrow is hypercellular, phase of induction, phase
of consolidation, phase of maintenance, transplantation,
allopurinol, topical treatment to stop gingival bleeding,
psychological support.
CHRONIC MYELOID LEUKEMIA

Chronic leukemias are characterized by the presence
of large leukemic cells and differentiated WBCs in the
bone marrow, peripheral blood and other tissues. It has a Figure 28.24 Chronic myeloid leukemia showing abundant
prolonged clinical course even without therapy. mature monocytes (mo) in the blood smear
Management compression of the central or peripheral nervous system.

The most common groups of lymph nodes involved are
The treatment of choice is chemotherapy using the drug
cervical, axillary and inguinal group.
724 busulfan given orally in a dose of 4 mg daily or in large
doses of 50 to 100 mg spaced 2 to 3 weeks apart. The Clinical Staging
treatment is continued for 12 to 18 weeks and should be
• S tage A: Lymphocytosis is less than three
discontinued when WBC count is between 10 x×106 and
areas of lymphoid enlargement, no anemia or
20 × 106/cumm, other wise busulphan may cause aplasia of
thrombocytopenia.
the bone marrow.
• Stage B: More than three areas of lymphoid
A combination chemotherapy using a small dose of
enlargement, no anemia or thrombocytopenia.
busulphan 2 mg daily along with mercaptopurine 50 mg
• Stage C: Anemia or thrombocytopenia, regardless of
daily or thioguanine 80 mg daily is given.
number of area of lymphoid enlargement.
Radiotherapy and splenectomy are other treatment of
choice.
Oral Manifestations
Points to Remember
The most common oral finding is hypertrophy of gingiva.
Slowly advancing anemia with loss of weight,
Sign and symptoms: There may be ulceration with necrosis
prominence of abdomen, weakness, fatigue, petechiae,
and gangrenous degeneration; a dark brown exudate and
ecchymosis as well as hemorrhage, WBC count is
foul fetor oris are present. The tongue is frequently swollen
considerably increased, mature leukocytes, busulfan,
and dark. There is regional lymphadenopathy.
combination chemotherapy, mercaptopurine 50 mg daily
Rapid loosening of teeth due to necrosis of periodontal
or thioguanine 80 mg daily.
ligament has been reported. Destruction of alveolar bone
also occurs in some cases.
CHRONIC LYMPHATIC LEUKEMIA Hematological Findings
It is a slowly progressing malignancy involving Peripheral blood smear shows mild anemia and a large
lymphocytes. number of small lymphocytes.
Lymphoblasts are rare but increase in the terminal
Pathophysiology stages of disease.
It is characterized by the accumulation of long lived, non- WBC count may increase up to 1000 × 106 per cumm.
functional B lymphocytes.
Management
Clinical Features General measure to maintain good health, adequate rest,
Age and sex distribution: It occurs more frequently in good food and exercise should be advised.
males and majority of the patients are over 45 years. The
Chemotherapy: Chlorambucil 6 to 10 mg/day for 14 days
onset is very insidious.
with break of 14 days and cyclophosphamide 2 to 3 mg/kg
Symptoms: Tiredness and ill health are common, although IV for 6 days.
some patients are symptom free and the disorder is found
Combination therapy: Cyclophosphamide doxorubicin,
incidentally. Bone marrow infiltration causes anemia and
vincristine and prednisone have been recommended.
thrombocytopenia and results in pallor, weakness, dyspnea
and purpura. Radiotherapy: It is useful for large granular masses,
if they cause symptoms. Radiotherapy with very small
Signs: There is a moderate enlargement of lymph nodes
doses, of only 150 rads over a period of five weeks, is very
which are firm, rubbery and discrete. Liver and spleen
effective and may induce satisfactory remission.
are usually enlarged and palpable. There is an increased
susceptibility to infection as the leukemic B cells are non- Steroids: If the bone marrow is severely involved initial
functional. Leukemic infiltration results in skin nodules, treatment with prednisone 40 mg daily and 25 to 50 mg
intestinal malabsorption, pulmonary obstruction or daily later should be given.
Blood Pathology
Variation of Leukemia In some cases, only light chains are produced and these
appear in urine as Bence Jones proteinuria.
Hairy leukemia: It a variant of chronic lymphatic
leukemia in which there is splenomegaly, severe neutro-
Clinical Features 725
penia, monocytopenia and the characteristic appearance
of hairy cells in blood and bone marrow. These hairy Age and sex distribution: The most common age group
cells appear to be a cross between the lymphocytes and affected is between 40 and 70 years with male to female
monocytes. It occurs mainly in adults and show male ratio 4:1. The skull, clavicle, vertebrae, ribs, pelvis, femur
predilection. Manifestations result from infiltration of bone and jaws are involved.
marrow, liver, and spleen. Splenomegaly is massive and Symptoms: Skeletal pain associated with motion or
hepatomegaly is less common. Hairy cell can be identified pressure over the tumor masses, is an early symptom.
on the peripheral smear. Spontaneous pathological fracture with acute pain may
Prolymphocytic leukemia: It is another variant of be present. Weakness and pain of back and thorax also
chronic lymphatic leukemia in which there is massive may be presenting symptoms. Pain in the involved bone
splenomegaly with little lymphadenopathy and a very high may be aggregated by exercise and relieved by rest. The
WBC count. The characteristic cell is a large lymphocyte patient may also complain of tiredness, bleeding tendency
with prominent nucleus. and bruising of skin due to anemia and thrombocytopenia.
The cause of bleeding is that the abnormal globulins bind
Aleukemic leukemia: It is the sub-leukemic form of with coagulation factors which also increase the viscosity
leukemia in which the WBC count of the peripheral blood of blood. Patient may complain of vomiting due to increase
is normal or even subnormal and abnormal or immature serum calcium level.
leukocytes may be present.
Signs: Swelling over the areas of bone involvement may
Points to Remember be detectable. There is an increased susceptibility to
Tiredness and ill health, anemia and thrombocytopenia, infection due to abnormal immunoglobulin production by
moderate enlargement of lymph nodes, increased the plasma cells.
susceptibility to infection, hypertrophy of gingiva, Hypercalcemia: Mobilization of calcium from the skeleton
rapid loosening of teeth, mild anemia, lymphoblasts, may cause hypercalcemia resulting in nephrocalcinosis,
WBC count may increase up to 1000 × 106 per cumm, lethargy, drowsiness and eventually coma, if untreated.
chemotherapy, combination therapy, radiotherapy,
steroids. Complication: The common cause of death is renal failure,
caused by accumulation of abnormal proteins in the renal
tissue.
MULTIPLE MYELOMA
It is also called myelomatosis. It is a malignant neoplasm Oral Manifestations
of plasma cells of the bone marrow with widespread Location: Mandible is more commonly involved than the
involvement of the skeletal system, including the skull and maxilla and particularly angle of the mandible, because
jaws. of its greater content of marrow. Lesions have also been
reported in temporomandibular joints.
Origin
Symptoms: The patient may experience pain, swelling
It is thought to be multicentric in origin. There is
and numbness of the jaw. Epulis formation or unexplained
proliferation of a single clone of abnormal plasma cells in
mobility of teeth are also detectable.
the bone marrow.
Normal plasma cells are derived from B cells and Signs: Intraoral swelling tends to be ulcerated, rounded
produce immunoglobulin, which contain heavy and light and bluish red similar to a peripheral giant cell lesion.
chain. In myeloma, plasma cells produce immunoglobulin Sometimes, swelling may erode buccal plate and produce
of single heavy and light chain, a monoclonal protein rubbery expansion of jaw. Chronic trauma produces an
commonly referred as para protein. inflamed and ulcerative necrotic surface.
Secondary signs of bone marrow involvement such as pallor in cartwheel (Fig. 28.26) or checkerboard pattern. Russell
of oral tissue, intraoral hemorrhage and susceptibility to bodies are common finding in multiple myeloma.
infection may also be seen.
726 Hematological Findings
Excessive hemorrhage may be caused by thrombocytopenia,
secondary to increased proliferation of the plasma cells Bone marrow examination shows an increased number of
in marrow. On palpation, swelling is tender and eggshell abnormal plasma cells.
cracking may be elicited. There is usually an associated anemia but, WBC and
platelet counts are normal.
Oral amyloidosis: It is a complication of this disease. The There is increased ESR, serum monoclonal immuno-
tongue may be enlarged and studded with small garnet- globulin with reversal of the albumin globulin ratio and
colored enlargements, including nodes on lip and cheeks. increase in total serum protein to a level of 8 to 16 gm%.
Tongue enlargement may cause impairment of speech, Bence Jones proteins, which coagulate when the urine
mastication and deglutition. Amyloid can also deposit in is heated to 40°C can be demonstrated in the urine of
the gingivae, where it can cause soreness and inability to patients suffering from multiple myeloma.
wear dentures. There may be hyperproteinemia due to increase in
globulins.
There is multiple well-defined punch out radiolucency. Management
The margins of radiolucency are usually well-defined
Chemotherapeutic agents: General disease is treated with
(Fig. 28.25).
chemotherapeutic agents like melphalan and cyclophos-
Histopathological Features phamide.
Microscopically, it can be classified as plasmocytic or Management of anemia and hypercalcemia: Patients
plasmoblastic. who present with anemia, hypercalcemia, evidence of renal
The plasmocytic is characterized by small normal damage require urgent management with alkalization of urine
appearing plasma cells with a low mitotic index as is with oral bicarbonates, high fluid intake, corticosteroids and
associated with a comparatively better prognosis than the possibly mithramycin to reduce calcium level.
plasmoblastic type. Blood transfusion: It may be required if Hb is less than
The plasmoblastic type shows infiltration by immature, 10 g/dL.
nucleated plasma cell precursors and has a less favorable
prognosis. Plasma cells are round or ovoid cells with Cell transplantation: Stem cell auto transplant may
eccentrically placed nuclei exhibiting chromatin clumping improve quality of life and prolong survival.
Figure 28.25 Punched out lesion seen in multiple myeloma Figure 28.26 Multiple myeloma showing cartwheel pattern
Blood Pathology
Radiotherapy: This is effective for localized pain not in mucous membranes, which become lobulated as they
responding to simple analgesics and for pathological enlarge but exhibits the tendency to ulcerate.
fractures. Pain is not prominent symptom unless bone is invaded.
There may be bleeding and ulceration of oral mucosa. 727
Bi-phosphonate therapy: It may reduce bone pain and
skeletal events. Radiological Features
Others drugs: Alpha interferons may prolong the plateau There is unilocular radiolucency with no evidence of
phase. Inorganic fluoride phosphate to reduce bone pain. sclerotic border as seen in multiple myeloma.
Myelomatosis, bence Jones proteinuria, para protein, It contains densely packed plasma cells and is
skeletal pain, swelling over the areas of bone involvement, indistinguishable from the bony lesion in multiple
hypercalcemia, renal failure, mandible is more commonly myeloma. There may be considerable nuclear and cellular
involved, pain, swelling and numbness of the jaw, intraoral pleomorphism. Russell bodies (Fig. 28.27) can also be
swelling tends to be ulcerated, rounded and bluish red, found. It is similar to multiple myeloma.
secondary signs of bone marrow involvement, excessive
hemorrhage, oral amyloidosis, multiple well-defined punch Laboratory Findings
out radiolucency, plasmocytic or plasmoblastic, normal Bence Jones proteins are found in urine, there is also
appearing plasma cells with a low mitotic index, infiltration hyperglobulinemia and anemia.
by immature, nucleated plasma cell precursors, cartwheel,
checkerboard pattern, russell bodies, abnormal plasma Management
cells, chemotherapeutic agents, management of anemia
It should be treated by conservative process to eradicate
and hypercalcemia, blood transfusion, cell transplantation,
the single lesion and this can be accomplished by surgery.
radiotherapy, Bi-phosphonate therapy.
Points to Remember
PLASMACYTOMA Plasma cell myeloma, nares, tonsil, palate, pain and
swellings, pathologic fractures, sessile or polypoid
Extramedullary location without bony involvement may
reddish masses in mucous membranes, unilocular
occur in the nasopharynx, nasal cavity, paranasal sinuses
radiolucency, plasma cells, nuclear and cellular
and rarely in the oral cavity. It is also called plasma cell
pleomorphism. Russell bodies, Bence Jones proteins.
myeloma.
Clinical Features
Age and sex distribution: Mean age of occurrence is 51
years and males are affected more commonly than females.
Location: The most common site of occurrence are nares,
tonsil, palate tongue, gingivae and the floor of mouth. They
are also found in pleura, mediastinum, thyroid, ovary,
intestine, kidney and skin.
Signs and symptoms: There is pain and swellings are
the most common complaints. Pathologic fractures are
common. Regional metastasis may develop in small
number of cases.
Oral Manifestations
It is rare in jaws and can occur in mandible or maxilla. The
lesions are described as sessile or polypoid reddish masses Figure 28.27 Plasmacytoma showing Russell bodies
EXTRANODAL NK/T-CELL LYMPHOMA Management

It is also called angiocentric T-cell lymphoma, midline It is treated by radiotherapy and combination chemo-
728 lethal granuloma, idiopathic midline destructive disease, therapy.
polymorphic reticulosis, midline malignant reticulosis and
Points to Remember
angiocentric immunoproliferative lesion.
It is characterized by aggressive nonrelenting T-cell lymphoma, midline lethal granuloma, angiocentric
destruction of the midline structure of the palate and nasal immunoproliferative lesion, natural killer (NK) T-cell
fossa. The word midline lethal granuloma should be used lymphoma, necrotic ulceration of palate, mixed infiltrate
only when as descriptive designation of destructive midline inflammatory cells, necrosis, large angular lymphocytes
condition. cells, radiotherapy, combination chemotherapy.
When the other cause of midline destruction has been
eliminated the disorders should be classified as natural
killer (NK) T-cell lymphoma.
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1. All are the normochromic normocytic types of anemia 2. Which one of the following is the type of hypochromic,
except: microcytic anemia:
a. Iron deficiency anemia a. Iron deficiency anemia
b. Anemia of acute blood loss b. Thalassemia
c. Anemia associated c. Both
d. Aplastic anemia d. None of the above
3. Which one of the following is the type of normo- 10. Most common type of anemia seen in female:
chromic, macrocytic anemia: a. Iron deficiency anemia
a. Iron deficiency anemia b. Sickle cell anemia
730 b. Pernicious anemia c. Both
c. Folate and B12 deficiency anemia d. Cooley’s anemia
d. Both b and c
11. Enlargement of heart and murmur found in:
4. In iron deficiency anemia, initially there is a atrophy a. Sickle cell anemia
of: b. Megaloblastic anemia
a. Cirumvalate papilla b. Fungiform papilla c. Aplastic anemia
c. Filliform papilla d. None d. Thalassemia
5. Webs in esophagus or ‘spasm in throat’ is characteristic
12. ‘Rh hump’–a ring like defect of teeth seen in:
feature seen in:
a. Pyruvate kinase deficiency anemia
a. Sjögren’s syndrome
b. Erythroblastosis fetalis
b. Burning mouth syndrome
c. Post hemorrhagic anemia
c. Patau’s syndrome
d. Polycythemia vera
d. Plummer Vinson syndrome
6. ‘Primary anemia’ refers to: 13. A crystal violet appearance of tongue is the oral
a. Pernicious anemia b. Iron deficiency anemia manifestation of:
c. Aplastic anemia d. Megaloblastic anemia a. Cyclic neutropenia
b. Lazy leukocyte syndrome
7. Hunter’s glossitis or Moeller’s glossitis seen in:
c. Erythroblastosis fetalis
a. Iron deficiency anemia
b. Aplastic anemia d. Polycythemia vera
c. Pernicious anemia 14. Hemophilia A is cause due to deficiency of:
d. Both b and c a. Factor v
8. ‘Cooley’s anemia’ refers to: b. Factor viii
a. Polycythemia vera b. G 6 P deficiency c. Factor ix
c. Neutropenia d. Thalassemia d. Factor vi
9. Mongolid appearance and rodent facies seen in: 15. ‘Crush spider’ appearance seen in:
a. Iron deficiency anemia a. Osler- Rendu- Weber syndrome
b. Megaloblastic anemia b. Christmas disease
c. Thalassemia c. Bernard- Soulier syndrome
d. Pyruvate kinase deficiency anemia d. Costen’s syndrome
29 Skin Disorders
Chapter Outline
Â Erythema multiforme Â Hereditary mucoepithelial dysplasia

• Steven Johnson syndrome Â Pseudoxanthoma elasticum
• Toxic epidermal necrolysis Â Hyalinosis cutis et mucosa oris
Â Pemphigus Â White sponge nevus
Â Paraneoplastic pemphigus Â Hereditary benign intraepithelial dyskeratosis
Â Bullous pemphigoid Â Hereditary hemorrhagic telangiectasia
Â Benign mucous membrane pemphigoid Â Peutz-Jeghers syndrome
Â Familial benign chronic pemphigus Â Ephelis
Â Epidermolysis bullosa Â Actinic Lentigo
Â Dermatitis herpetiformis Â Sebaceous hyperplasia
Â Pityriasis rosea Â Lentigo simplex
Â Incontinentia pigmenti Â Xeroderma pigmentosum
Â Acanthosis nigricans Â Tuberous Sclerosis
Â Ehlers Danlos syndrome Â Ectodermal dysplasia
Â Psoriasis Â Cowden Syndrome
Â Pachyonychia congenita Â Graft versus host resistance
Â Porokeratosis Â CREST syndrome
Â Keratosis follicularis Â Scleroderma
Â Warty dyskeratoma Â Kawasaki disease
Â Seborrheic keratosis
Mucocutaneous lesions are a group of disease and

Types
conditions that affect the skin as well as the mucous
membrane. These lesions affecting the oral mucosa may be • Erythema multiforme minor
arising due to infections, allergies, autoimmune diseases. • Erythema multiforme major
Some of the lesions may be hereditary in origins which are – Steven Johnson syndrome
called genodermatosis. – Toxic epidermal necrolysis
• Herpes associated erythema multiforme.
ERYTHEMA MULTIFORME
It is acute inflammatory disease of the skin and mucous Types
membrane that causes a variety of skin lesions, hence the ∙ Erythema multiforme minor: This is self-limiting
term multiforme. form.
• Erythema multiforme major: This is severe form. It is

traditionally thought to be synonymous with Steven John-
son syndrome or toxic epidermal necrolysis. But now a
732 day some literature suggested that Steven Johnson syn-
drome and toxic epidermal necrolysis are different entity.
But this classification scheme remains very controversial.
• Herpes associated erythema multiforme: Now a
day sophisticated technique demonstrated that in some
patient of recurrent erythema multiforme there is
presence of herpes simplex DNA.
Etiology
Immune mediated disease that is indicated by the deposition
of immune complexes in the superficial microvasculature of
the skin and mucous membrane or cell mediated immunity. Figure 29.1 Erythema multiforme showing sloughing of lip
Drugs like sulfonamides, trimethoprin, nitrofurantion,
phenylbutazone, digitalis, birth control pills and penicillin.
Microorganisms like Mycoplasma pneumoniae and Lip is prominently involved followed by buccal mucosa,
herpes simplex virus. palate, tongue and face. Oral lesions start as bullae, on an
Vaccination, radiation therapy and occasionally erythematous base and break rapidly into irregular ulcers
other disease like Crohn’s disease, ulcerative colitis and (Fig. 29.1).
infectious mononucleosis. Patient cannot eat or swallow and drools blood tinged
saliva. The lesions are larger, irregular, and deeper and
Clinical Features often bleed very freely.
It is most frequently seen in children and young adults and is In full blown cases, lips are extensively involved
rare after the age of 50. It affects males more than females. and large portions of the oral mucosa are denuded of
epithelium. Sloughing of mucosa and diffuse redness with
Sites involved: Most common area, involved are hands, bright red raw surface is seen. Healing occurs in 2 weeks.
feet, extensor surfaces of elbow and knees.
Onset of lesion: It has got acute or explosive onset with Histopathological Features
generalized symptoms such as fever and malaise. It may be (Figs 29.2 and 29.3)
asymptomatic and in less than 24 hours, extensive lesions The cutaneous or mucosal lesions generally exhibit
of oral mucosa may appear. intracellular edema of the spinous layer of epithelium and
Symptoms: It is characterized by macule or papule, 0.5 edema of superficial connective tissue, which may produce
to 2 cm in diameter, appearing in segmental distribution. subepidermal vesicle.
Typical skin lesions, of erythema multiforme may be There is zone of liquefaction degeneration in the
nonspecific macule, papule and vesicle. upper layer of the epithelium with intraepithelial vesicle
formation and thinning, with frequent absence of the
Bull’s eye: Target or iris or bull’s eye lesion consists of basement membrane.
central bulla or pale clearing area, surrounded by edema Dilatation of the superficial capillaries and lymphatics
and band of erythema. This gives concentric ring like in the upper most layer of the connective tissue is prominent
appearance. Morbidity is high due to secondary infection, and mixed inflammatory infiltrate with numerous eosin-
fluid and electrolyte imbalance or involvement of lungs ophils also seen.
liver and kidneys. Vesicle formation is seen as intraepithelial or the
subepithelial region.
Oral Manifestations Necrotic eosinophilic keratinocytes are seen in the
Erythema multiforme occurs along with skin lesions in 45 blister area. Inflammatory cells are in abundance in the
percent of the cases. vesicle region.
Skin Disorders
Points to Remember
Immune mediated disease, drugs, microorganism,
Bull’s eye - target or iris or bulls eye lesion, mucosal 733
vesicles, bullae, erythematous base, lips involvement,
intracellular edema, liquefaction degeneration, intra-
epithelial vesicle, dilatation of the superficial capillaries,
mixed inflammatory infiltrate, necrotic eosinophilic
keratinocytes, prednisolone, prophylactic acyclovir.
Steven Johnson Syndrome

It is the severe form of erythema multiforme with
widespread involvement, typically involving skin, oral
cavity, eyes and genitalia.
Figure 29.2 Erythema multiforme showing vesicle formation It commences with abrupt occurrence of fever, malaise,
photophobia and eruptions on oral mucosa, genital mucosa
and skin.
Cutaneous lesions: Cutaneous lesions are similar to erythema
multiforme and are hemorrhagic, often vesicular or bullous.
Eye lesions: It consists of photophobia, conjunctivitis,
and corneal ulcerations. Keratoconjunctivitis sicca has
also been described and blindness may result, chiefly from
intermittent bacterial infection.
Genital lesions: Genital lesions are reported to consist of
nonspecific urethritis, balanitis and vaginal ulcers.
Complications: Other reported complications are related
to respiratory tract involvement such as tracheo-bronchial
ulcerations and pneumonia.
Oral mucous membrane lesions in Steven Johnson syn-
Figure 29.3 Vesicle formation seen in patient with erythema drome may be extremely painful and so, mastication becomes
multiforme impossible. Mucosal vesicles or bullae occur, rupture and
leave a surface covered with a thick, white or yellow exudate.
Management Erosions of the pharynx are also common. Lips may exhibit
ulcerations with bloody crusting and are often painful.
Mild cases: Soft liquid diet and topical anesthetic mouth
wash. If the causative organism is identified or suspected it Toxic Epidermal Necrolysis
should be discontinued.
It is also called Lyell’s disease. It usually results due
Moderate to severe erythema multiforme: 30 mg/day to increases apoptosis of the epithelial cells. It occur
prednisolone or methyl prednisolone for several days and secondary to drug reaction.
tapered after the symptoms subside. Prophylactic Acyclovir It usually occurs in the older individual and more seen
to prevent HSV related erythema multiforme. in female.
Scalded appearance: It results in sloughing of skin and

mucosa in large sheets. The appearance of the patient is
like that is badly scalded.
734
Management: It is managed in burn centers where necrotic
skin is removed under general anesthesia and healing takes
place under sheets of porcine xenografts. Corticosteroid
should be avoided in this case. Intravenous administration
pooled human immunoglobulin has been results in
remarkable resolution of the toxic epidermal necrolysis.
PEMPHIGUS
It is autoimmune disease involving the skin and mucosa and
characterized by intraepithelial vesicle or bulla formation.
Figure 29.4 Pemphigus present on the back showing vesicle
Types
and bulla formation
• Pemphigus vulgaris
• Pemphigus vegetans Nikolsky’s sign: Characteristic sign of the disease is
• Pemphigus foliaceous that pressure to an apparently normal area will result
• Pemphigus erythematosus. in formation of new lesion. This phenomenon is called
Nikolsky’s sign. It results from upper layer of skin pulling
Mechanism away from the basal layer. It is caused by perivascular
Binding of autoantibodies against the epidermal cells gly- edema which disrupts the dermal-epidermal junctions.
coprotein, desmoglein 3 and desmoglein 1 which are Asboe-Hansen sign: After giving application of pressure
component of desmosomes (structure that bone epithelial to an intact bulla, the bulla will enlarge by extension to
cells to each other). This will in turn inhibit the molecular apparently normal surfaces.
interaction that is responsible for adherence. This will result The course of pemphigus vulgaris is a variable one; the
in split within the epithelium and blister will for. Separation disease terminating in death or recovery within a few days
of cell takes place in lower layer of stratum spinosum. or weeks or it may get prolonged over a period of months
or even years.
Pemphigus Vulgaris
It is the most common types occur out of above four. Oral Manifestations
Vulgaris in Latin is known as common. In 90 percent, of the cases oral lesions develop and in 60
percent cases they occur first. Initial lesion most frequently
Clinical Features occurs on buccal mucosa because the epithelium demon-
It is seen in 5th to 6th decades of life and male to female strates less intercellular substance and fewer intercellular
ratio is 1:1, with whites more commonly affected. junctions making the area more susceptible to acantholysis.
Palate and gingiva are other common sites of involvement.
Symptoms: Thin walled bullae or vesicles varying in
diameter from few millimeter to several centimeters arise Symptoms and signs: Oral lesions begin as classic bullae
on normal skin or mucosa. on noninflamed base with formation of shallow ulcers as
bullae break rapidly. Thin layer of epithelium peels away
Signs: These lesions contain a thin, watery fluid shortly
in an irregular pattern leaving denuded base.
after the development, but this may soon become purulent
or sanguineous (Fig. 29.4). They rapidly break and continue Nikolsky’s phenomenon: The oral lesions may exhibit
to extend peripherally, eventually leaving large areas of Nikolsky’s phenomenon and may be denuded by peripheral
denuded skin. enlargement of the lesion.
Skin Disorders
Lesions bleed easily and are tender on palpation. The Histopathological Features (Figs 29.5 to 29.9)
pain may be so severe that the patient is unable to eat. Basic defect is intraepithelial and is demonstrated as
The lesion may have ragged borders and be covered with acantholysis (loss of cell to cell attachment) in stratum
white or blood tinged exudate. Edges of the lesion may 735
spinosum. The cellular outlines are round, rather than
extend peripherally. Diffuse erythematous involvement of polyhedral; intercellular bridges are lost (spongiosis) and
gingiva. the nuclei are large and hyperchromatic.
Pemphigus Vegetans Characteristic suprabasilar split intraepithelial separa-
tion, which occurs just above the basal layer of the epithe-
Types lium, is seen.
• N
eumann type: It is more common and early lesions Row of tombstones: Sometimes the entire suprabasal or
are similar to those seen in pemphigus vulgaris. superficial layers are striped off leaving only the basal
• Hallopeau type: In hallopeau type; pustules, not layers which is called the row of tombstones.
bullae, are the initial lesions which are followed by
verrucous hyperkeratotic vegetations.
Clinical Features
It occurs in 1 to 9 percent of the cases. The flaccid bullae
become eroded and forms vegetations on some of the
erosions. These fungoid masses become covered by
purulent exudate and exhibit inflamed borders, frequently
occur first on nose and in the mouth or axilla. The disease
usually terminates in pemphigus vulgaris.
Oral Manifestations
Gingival lesions may be lace like ulcers with purulent sur-
face on red base or have granular or cobblestone appearance.
Pemphigus Foliaceus Figure 29.5 Pemphigus vulgaris showing acantholysis

(Courtesy: Dr Sangamesh Halawar, Reader, Oral Pathology,
It is a relatively mild form of pemphigus, which is most CDCRI, Rajnandgaon, Chhattisgarh)
common in older adults. It is manifested by characteristic
early bullous lesions which rapidly rupture and dry to leave
masses of flakes on scales suggestive of an exfoliative
dermatitis or eczema.
Brazilian pemphigus: It is a mild endemic form of pem-
phigus foliaceous found in tropical regions, particularly in
Brazil. It is seen in children and frequently in family groups.
Pemphigus Erythematosus
It is also called Senear-Usher syndrome. It is form of
disease which is characterized by the occurrence of
bullae and vesicles concomitant with the appearance of
crusted patches resembling seborrheic dermatitis or even
lupus erythematosus. Most cases ultimately terminate in
pemphigus vulgaris or foliaceous. The skin manifestations
in any form of pemphigus may be accompanied by fever Figure 29.6 Pemphigus vulgaris showing suprabasilar vesicle
and malaise. formation
736
Figure 29.7 Tzanck smear showing presence of Tzanck cell Figure 29.8 Vesicle seen in pemphigus
A B
Figures 29.9A and B Tzanck cells in pemphigus
Acantholysis: The spinous cell layer lose their cell contacts phonuclear leukocytes and the surface may show
and the loose cells assume a rounded shape, forming suppuration.
characteristic cells—“the Tzanck cells.” Indirect immunofluorescent antibody test: Antibodies
The diagnostic test of the cytological study of the against intercellular substance can be seen in the serum of
contents of the vesicle or the lesion shows these cells are patient. The titers of antibody are directly related to the
termed as Tzanck test. level of the clinical disease.
Tzanck smear: It is done to demonstrate Tzanck cells which Direct test: Antibody will bind the immunoglobulin depo-
often are found lying freely within the vesicular space. sit in the intercellular substance and show positive fluore-
The underlying connective tissue shows mild to mod- scence under fluorescence microscope.
erate chronic inflammatory infiltrate.
The underlying connective tissue is densely filled Management
with chronic inflammatory cells, which may also enter Corticosteroids: Corticosteroids usually prednisolone
the vesicular fluid. As the vesicle and bulla rupture, given in combination with immunosuppressive drugs like
the ulcerative lesion becomes infiltrated with polymor- azathioprine.
Skin Disorders
Plasmapheresis and administration of 8- methoxy-

psoralen, followed by exposure of peripheral blood to
ultraviolet radiation.
737
Points to Remember
Thin walled bullae or vesicles, Nikolsky’s sign, Asboe-
Hansen sign, classic bullae on noninflamed base,
vegetations, brazilian pemphigus, intraepithelial defect,
acantholysis, suprabasilar split, row of tombstones,
chronic inflammatory cells, Tzanck test, Tzanck smear,
plasmapheresis corticosteroid.
PARANEOPLASTIC PEMPHIGUS
It is autoimmune disease which is associated with Figure 29.10 Paraneoplastic pemphigus
malignancy like lymphoma, leukemia, etc.
The mechanism behind this is that there are abnormal
levels of cytokine, interleukin 6 in response to patient tumor. the intercellular zone of epithelium or linear deposition at
This in turn stimulates abnormal production autoantibodies the basement membrane.
against antigen associated with desmosomes complex.
Recently also it is stated that cutaneous and mucosal Management
damage appear to be mediated by cytotoxic T lymphocytes It is serous disorders with high morbidity and mortality rate.
also can lead to paraneoplastic pemphigus. Treatment consists of systemic prednisolone combined
with immunosuppressive agents.
Clinical Features
There is history of tumors and in some cases it is developed Points to Remember
before malignancy identified. So this can be indicative to Associated with malignancy like lymphoma, multiple
presence of tumor in the body. vesiculobullous lesion of skin and oral mucosa,
scarring, conjunctivitis, positive deposition of immuno-
Signs and symptoms: They appear sudden and polymor-
reactants (IgG and complement), subepithelial clefting,
phous. There is appearance of multiple vesiculobullous
intraepithelial clefting, systemic prednisolone.
lesion of skin and oral mucosa. Palmar or planter bullae
also present.
There is also involvement of conjuctival mucosa BULLOUS PEMPHIGOID
causing scarring and conjunctivitis. In some cases vaginal
It is also called para-pemphigus, or aging pemphigus. In
mucosa and mucosa of respiratory tract can be affected.
this, the initial defect is subepithelial in the lamina lucida
Oral features: The lip shows hemorrhagic crusting region of the basement membrane. It is associated with anti-
similar to that of in erythema multiforme. The oral mucosa basement membrane antibodies which are detected in the
shows multiple area of diffuse and irregular ulceration. basement membrane. It usually resembles mucous membrane
Some patient develop only oro-pharyngeal lesion without pemphigoid but one major difference is that clinical course of
cutaneous lesion. bullous pemphigoid is limited and that of mucous membrane
pemphigoid is protracted and progressive.
In most of the cases lichenoid mucositis with subepithelial Clinical Features
clefting (like pemphigoid) or intraepithelial clefting (like It occurs chiefly in adults over the age of 60 is self-limiting
pemphigus) is seen. and rarely lasts over 5 years.
Direct immunofluorescence studies show positive Skin lesions begin as generalized nonspecific rash,
deposition of immunoreactants (IgG and complement) in commonly on the limbs, which appear as blisters on
inflamed skin; itching precedes. It may persist for several

weeks to several months before ultimate appearance of
vesiculobullous lesions.
738
Vesiculobullous lesion: These vesicle and bullae are
relatively thick walled and may remain intact for some
days. Rupture does not occur although it does leave raw
eroded areas which heal rapidly.
Oral Manifestations
Oral lesions are smaller, form more slowly and are less
painful. Gingival lesions consist of generalized edema,
inflammation, and desquamation and localized areas of Figure 29.12 Bullous pemphigoid showing separation of the
discrete vesicle formation. epithelium at the basement membrane zone
Vesicles and ultimately erosion may develop not only
on the gingival tissue but any other area such as the buccal
Direct immunofluorescence shows continuous linear
mucosa, palate, floor of the mouth and tongue (Fig. 29.11).
band of immunoreactants usually IgG and C3 localized to
Histopathological Features (Fig. 29.12) basement membrane.
Indirect immunofluorescence antibody test—lesions
The vesicle and bullae are subepidermal and nonspecific. will demonstrate circulating IgG antibodies against base-
Epithelium appears normal. The vesicle contain fibrinous ment membrane antigen.
exudate admixed with occasional inflammatory cells.
These cells are usually eosinophils. In severe inflamm- Management
ation the basal keratocytes of mesenchymal demonstrates
Systemic steroids in lower doses, for shorter period
spongiosis. It is called eosinophilic spongiosis.
combined with immunosuppressive drugs and Dapsone.
It also shows stratified squamous epithelium, thin
epithelium and subepithelial bulla formation is the Points to Remember
characteristic feature of bullous pemphigoid. The vesicle
Para-pemphigus, aging pemphigus, skin lesions,
shows moderate numbers of the acute inflammatory cells
generalized nonspecific rash, vesiculobullous lesion,
as eosinophils and chronic inflammatory cell infiltrate
gingival lesions, generalized edema, subepidermal
within it.
fibrinous exudate, occasional inflammatory cells,
eosinophilic spongiosis, systemic steroids.
BENIGN MUCOUS MEMBRANE

PEMPHIGOID
It is also called cicatricial pemphigoid mucous membrane
pemphigoid. It is a disease of unknown etiology but
probably is autoimmune in nature. The term pemphigoid is
used as it appears clinically similar to pemphigus.
Clinical Features
Age and sex distribution: It occurs more commonly in
patients over 50 years of age and female to male ratio is
2:1.
Sites: Typically, the vesiculobullous lesions occur on the
Figure 29.11 Bullous pemphigoid showing lesion oral mucous membrane, conjunctivae and skin. The other
Skin Disorders
mucous membranes involved are those of nose, larynx,

pharynx, esophagus, vulva, vagina and penis.
Eye lesion: Most striking features of this disease is 739
involvement of eye. Initially there is subconjuctival
fibrosis. After that conjunctiva may become inflamed and
eroded. When healing process start there is scarring occurs
between bulbar (lining of globe of eye) and palpebral
(lining of inner surface of the eyelid) conjunctivae. This
will results in adhesion called symblepharons. Scarring can
ultimately results in eyelid to turn inward called entropion.
These cause eyelashes to rub against cornea and globe
called trichiasis. Scarring will close opening of lacrimal
gland resulting in dry eyes. Eyes then produce keratin as
a protective mechanism but this is opaque material and
ultimately blindness occurs. There is formation of scar, Figure 29.13 Cicatricial pemphigoid
i.e. the reason it is called cicatricial pemphigoid (cicatrix
meaning scar).
Dyspareunia: Vaginal mucosal lesion may cause
severe pain during attempts of intercourse. This is called
dyspareunia.
Involvement of esophagus and trachea may cause
strictures leading to difficulty in swallowing or breathing.
Oral Manifestations
It occurs on gingiva, buccal mucosa and palate. The mouth
may be the only site involved.
The mucosal lesions are also vesiculobullous in
nature, but appear to be relatively thick walled and for this
reason may persist for 24 to 48 hours before rupturing and
desquamation. After rupture of vesicle surface epithelium
is lost leaving raw red bleeding surface (Fig. 29.13).
Figure 29.14 Cicatricial pemphigoid showing chronic
Gingiva is edematous and bright red, involvement
inflammatory infiltrated
is patchy and diffuse. This types of pattern is called
desquamative gingivitis. There may be formation of
ulcer, which surrounded by zone of erythema. There may eosinophils (Fig. 29.14). Lesion which is chronic in nature
be erosion on cheek and vesicles on palate and narrower shows granulation tissue and fibrosis.
peripheral extensions. The oral lesions rarely produce scars. Direct immunofluorescent study will show positive
fluorescence for immunoglobulin and complement in
Histopathological Features basement membrane zone, i.e. in intercellular substance
The vesicle and bullae are subepidermal rather suprabasilar of prickle cell layer of epithelium. Immune deposit mainly
and there is no evidence of acantholysis. consists of IgG and C3. In some cases IgA is also found and
The basement membrane structure appears to detach if IgG and IgA is found in same patient disease is usually of
with the epithelium from the underlying connective tissue. severe variety.
So mucosa will show split between surface epithelium and Indirect immunofluorescence studies revealed the
underlying connective tissue. presence of tissue bound basement membrane zone
There is nonspecific chronic inflammatory infiltrate of antibodies as well as circulating anti-basement membrane
connective tissue chiefly lymphocytes, plasma cells and zone antibodies in the serum of some patients.
Management Histopathological Features

It is similar to pemphigus vulgaris but acantholysis is
Topical therapy: Application of topical steroid should
740 more extensive. Characteristic feature of this disease in
be done several times per day. In case of gingival lesion
occasional intercellular bridges persist so that adjacent
flexible mouth guard should be applied for carrier of
epithelial cells still adhere to each other. This gives classic
corticosteroid medication.
description of the dilapidated brick wall effect.
Systemic therapy: Systemic steroid in combination with
immunosuppressive therapy are given. Immunosuppre- Points to Remember
ssive agent used should be cyclophosphamide. Recently Hailey-Hailey disease, small groups of vesicles, Nikol-
intravenous human immunoglobulin is effective in sky’s sign, raw eroded areas, acantholysis, intercellular
managing ocular lesion. Use of dapsone (sulfa derivative) bridges.
can also aid to relief of sign of the disease. Tetracycline
or minocycline and niacinamide given in doses of 0.5 to Epidermolysis Bullosa
2 g have been found to be effective in the management of
mucous membrane pemphigoid. It is a dermatological disorder in which bullae or vesicles
occur on skin or mucous membrane surface spontaneously,
Points to Remember shortly after minor trauma.
Cicatricial pemphigoid, eye lesion called symblepharons, Types
entropion, raw red bleeding surface, desquamative
gingivitis, zone of erythema, subepidermal vesicle • Epidermolysis bullosa simplex
bullae, chronic inflammatory infiltrate, immune deposit, • Epidermolysis bullosa dystrophic, dominant
systemic steroid, cyclophosphamide. • Epidermolysis bullosa dystrophic, recessive
• Junctional epidermolysis bullosa
• Hemidesmosomal
FAMILIAL BENIGN CHRONIC • Epidermolysis bullosa acquista (acquired).
PEMPHIGUS
It is also called Hailey-Hailey disease. It is uncommon
Types
disease transmitted by irregular dominant gene. Epidermolysis bullosa simplex
∙ Generalized form
Clinical Features ∙ Localized form (Weber-Cockayne syndrome, recurrent
It is usually manifested during adolescent or young life bullous eruption of hands and feet)
with no predilection for sex. The most common sites are Epidermolysis bullosa dystrophic, dominant.
flexure surfaces of the axillae and groin, the neck and the Epidermolysis bullosa dystrophic, recessive.
genital area. Heat and sweating amplify the outbreak of Junctional epidermolysis bullosa (Epidermolysis bull-
the lesion while spontaneous remission may occur in cold osa latalis, junctional bullous epidermatosis, Herlitz’s
weather. disease).
Epidermolysis bullosa acquista (acquired): It is
Signs and symptoms: The lesions develop as small groups similar to dystrophic form of the disease but usually with
of vesicles appearing on normal or erythematous skin, an adult onset.
which soon rupture to leave eroded, crusted areas. Hemidesmosomal: It is most recently discover types of
Nikolsky’s sign: They enlarge peripherally but heal in Epidermolysis bullosa. In this mutation of genes associated
center with Nikolsky’s sign positive. Tender and enlarged with hemodesmosomal attachment protein such as plectin
regional lymph nodes may also be present. is associated.
Oral Manifestations Clinical Features

Oral lesions are similar to those occurring on the skin and Epidermolysis Bullosa Simplex
it develops as crops of vesicle, which rapidly ruptures It is inherited as autosomal dominant trait and manifests at
leaving raw eroded areas. birth or shortly thereafter. It is characterized by formation
Skin Disorders
of bullae or vesicle on the hands and feet at site of friction Epidermolysis Bullosa Dystrophic Dominant
or trauma.
Bullae can occur in this type sometimes oral milia can
The knees, elbows and trunk are rarely involved and
be seen but teeth are unaffected. Gingival recession and 741
nails are occasionally affected. When the blister heals
reduction in the depth of buccal vestibule may be seen.
within 2 to 10 days there is no resultant scarring or perma-
nent pigmentation. Epidermolysis Bullosa Dystrophic Recessive
The disease appears to improve at puberty and prog-
They may be preceded by the appearance of white spots or
nosis is good for normal life span. The localized form is
patches on the oral mucous membrane or by development
limited to hands and feet only and tends to exacerbate in
of localized areas of inflammation. Lesion can occur on lip
hot weather.
as vesicle or bullae (Fig. 29.15).
Epidermolysis Bullosa Dystrophic Dominant These bullae are painful especially when they rupture or
when the epithelium desquamates. Scar formation results in
The onset is at infancy and it may delay until puberty. The
obliteration of sulci and restriction of the tongue movement.
blister commonly develops on the ankles, knees, elbows,
Hoarseness and dysphagia may occur as a result of
feet and head. Healing results in scarring which is some
bullae of larynx and pharynx. Esophageal involvement
times keloid in type.
produces serious strictures.
Hair may be sparse, while nails are usually dystrophic
Dental defects like rudimentary teeth, congenitally
or absent with milia present.
absent teeth, hypoplastic teeth and crowns denuded of
Palmar-planter keratoderma with hyperhidrosis also
enamel may be seen.
may occur with ichtyyosis and sometimes hypertrichosis.
Junctional Epidermolysis Bullosa
Epidermolysis Bullosa Dystrophic Recessive
Oral bullae are frequently very extensive and because of
It has onset at birth or very shortly thereafter. The typical
their extreme fragility produce serious feeding problems.
sites of involvement are the feet, buttock, scapula, elbows,
Severe disturbance in enamel and dentin formation of
finger and occiput.
deciduous teeth also occur.
It is characterized by formation of bullae spontaneously
or at sites of trauma, friction or pressure. Histopathological Features
Nikolsky’s sign is positive in this type of epidermolysis
bullosa. The bullae contain a clear, bacteriologically sterile
Epidermolysis Bullosa Simplex
or sometime blood tinged fluid. When these bullae rupture Vesicle and bullae are developed as a result of destruction
or are peeled off under trauma or pressure, they leave raw, of basal and suprabasal cells so that some nuclei may
painful surface. The bullae heal by scar and milia formation persist on the floor of the blister.
which may result in afunctional club-like fists. The hair
may be sparse, while the nails are usually dystrophic.

It is extremely sever form of the dystrophic recessive form,
which is incompatible with prolonged survival. It has
onset at birth, absence of scarring, milia, pigmentation and
death within three months of age. The bullae are similar to
recessive form but they develop simultaneously and sheets
of skin may actually be shed.
Oral Manifestations
Epidermolysis Bullosa Simplex
Bullae of the oral cavity are reported in occasional cases
of generalized epidermolysis bullosa, but teeth are not
affected. Figure 29.15 Child patient having lesion on upper lip
The individual cells become edematous and Management

show dissolution of organelles and tonofibrils with
Drainage of blister: Large blisters should be pricked and
displacement of the nucleus to the upper end of cells.
742 the blister fluid released. Dressing, to minimize reaction
In localized form, bullae are intraepidermal and supra-
may be helpful.
basal in location. There is intraepithelial clefting can be
seen. Antibiotics: Super infections should be treated with
Electron microscopy shows perinuclear edema and appropriate local or systemic antibiotics.
subnuclear cytolysis. Surgical approach: Deformities of hand should be corrected
with plastic surgery. In case of dysphagia due to esophageal
Epidermolysis Bullosa Dystrophic Dominant involvement gastrostomy tube can be place.
The bullae in this form develop as a result of separation Management of oral lesion: Atraumatic dental procedure,
through the very thin, irregular PAS positive basement minimum dental manipulation should be done. If the dental
membrane which becomes divided. The basal layer appears restoration is required.
normal, although flattened on the roof of the blister and the
underlying connective tissue shows absence of elastic and Points to Remember
oxytalan fibers.
Bullae or vesicle, no scarring, blister, palmar-planter
keratoderma, Nikolsky’s sign, milia, pigmentation,
Epidermolysis Bullosa Dystrophic Recessive bullae of the oral cavity, hoarseness, dysphagia, intra-
The separation and bulla formation occur immediately epidermal, supra-basal vesicle, perinuclear edema,
beneath the poorly defined PAS positive basement subnuclear cytolysis, PAS positive basement membrane,
membrane which remains attached to the roof of the pre-elastic and oxytalan fibers.
blister. Fragment of basement membrane may adhere to the
dermis but basal layer of cells is normal. The pre-elastic
DERMATITIS HERPETIFORMIS
and oxytalan fibers in the connective tissue are increase in
number (Fig. 29.16). It is also called Duhring-Brocq disease. It is a rare, benign,
chronic, recurrent dermatologic disease of unknown etiology.
It is similar and identical to those occurring in the dystrophic
It occurs between 20 and 55 years of age, with males
recessive disease.
affected at least twice as frequently as females. These
occur most frequently on buttocks, extremities as well as
on the face, scalp and sometimes, the oral cavity.
Symptoms: The first manifestation of the disease is usually
pruritus and severe burning followed by the development of
erythematous papules, vesicles, bullae or pustules. The patient
usually shows increased severity in summer months.
Oral Manifestations
Vesicles and bullae rupture rapidly to leave areas of superfi-
cial ulceration at any intraoral site the characteristic finding.
The lesion begins with accumulation of neutrophils and
eosinophils in the dermal; papillae producing a microab-
scess. The connective tissue becomes necrotic and
Figure 29.16 Epidermolysis bullosa the overlying epithelium separates, usually forming a
Skin Disorders
subepithelial vesicle with destruction of the basement mem-

brane. There is also perivascular infiltrate of lymphocytes.
There is deposition of IgA along the basement membrane.
Electron microscopic study show blister formation below 743
the lamina lucida.
Laboratory Findings
Direct immunofluorescence staining is positive at
the epidermal-dermal junction. Patient may develop
eosinophilia and sensitivity to halogens (chlorine, bromine,
iodine and fluorine), both by patch test and after ingestion.
Management
Use of dapsone can give relief to the patient.
Figure 29.17 Pityriasis rosea showing exanthematous lesion
Points to Remember
Duhring-Brocq disease, pruritus, severe burning,
buccal mucosa, although tongue and palatal lesions have
erythematous papules, superficial ulceration at intraoral
been reported.
site, neutrophils, eosinophils, subepithelial vesicle,
Oral lesion appears as erythematous macule, with or
microabscess, eosinophilia, dapsone.
without central area of grayish desquamation. The lesion
may be single or multiple, irregular in shape, occasionally
PITYRIASIS ROSEA showing raised borders and vary in size from few millimeter
to 1 to 2 cm in diameter.
It is an acute skin eruption of unknown etiology.
Clinical Features
It consists of slight acanthosis and focal parakeratosis
It is more common in spring and autumn and it involves
with microvesiculation or simply sprinkling of leukocytes
young adults chiefly, with no sex predilection.
within the epithelium. There is also edema, hyperemia and
Prodromal features: The generalized outbreak is fre- perivascular infiltration of lymphocytes, plasma cells and
quently preceded by the appearance of a primary lesion histiocytes.
or herald spot seven to ten days previously. It is chara-
cterized by the appearance of superficial, light red macules Management
or papules, generalized over most of the skin surface. It requires no treatment since the disease is self-limiting
Signs and symptoms: The spot is brighter red and larger and generally undergoes rapid spontaneous regression.
(3 to 4 cm in diameter) than the multiple eruptions which
follow its appearance. Points to Remember
The individual exanthematous lesion is commonly Primary lesion, herald spot, exanthematous lesion,
ovoid, with long axis parallel to the natural lines of cleavage erythematous macule, grayish desquamation, acanthosis,
of skin and are covered by a thin silvery scales (Fig. 29.17). focal parakeratosis, microvesiculation.
The lesion often manifests mild aching headache and low
grade fever and cervical lymphadenopathy.
INCONTINENTIA PIGMENTI
Oral Manifestations It is also called Bloch-Sulzberger syndrome. It is transmitted
The oral lesion occurs either concomitantly with, or as X-linked dominant trait. It involves skin, eyes and
subsequent to the skin manifestations. It can occur on central nervous system.
Clinical Features ACANTHOSIS NIGRICANS

It appears shortly after birth and exclusively seen in It is an acquired disease characterized by development of
744 females. velvety brownish alteration of the skin.
Vesicular stage: It is characterized by the appearance of
erythematous and vesiculobullous lesions on the trunk and Types
extremities which frequently disappear and reappear.
Benign: It may be present at birth or occur later in
Verrucous stage: Then, they is gradually replaced by childhood; appears to be genetic in origin inherited as a
white keratotic, lichenoid, papillary or verrucous lesions, dominant characteristic. It can occur with some syndrome
which then persist for some months. like Crouzon syndrome or due to drug ingestion.
Hyperpigmentation stage: Some of the lesions in infants Malignant: It is associated with internal malignancy like
are brownish gray macules in a streaked, patchy distribution adenocarcinoma of stomach and occurs in older age group.
over the trunk and extremities, occurring subsequent to
the verrucous keratotic lesions. There is heavy melanin Pseudoacanthosis nigricans: It is most common and is
pigmentation of epithelium, dropping down into cluster of associated with endocrinopathy.
chromatophores in the upper dermis (incontinence), which
Clinical Features
gives the disease its name.
The most common areas involved are axilla, palms and
Atrophy and depigmentation stage: This stage ultimately soles and face and neck.
occur in the patient. Signs: Skin lesions are symmetric with mild hyper-
Other defects can be seen like cataract, optic atrophy, pigmentation and mild papillary hypertrophy of only small
strabismus, retrolental fibroplasia, central nervous system patchy areas. It usually involve flexural areas of skin.
involvement and lesions of skeletal system. Patches are browns in color and hyperkeratotic.
Oral Manifestations In some cases, it is heavily pigmented, aggressively
verrucous lesion involving much of the skin. The verrucous
Both the deciduous and permanent dentitions may be lesions are often pigmented and generalized pruritus is also
affected. There is delayed tooth eruption, peg and cone a common finding.
shaped crowns, congenitally missing teeth, malformed
teeth and additional cusps. Oral Manifestations
Histopathological Features The tongue and lips are most commonly involved,
followed by buccal mucosa. There is hypertrophy of
In early stage there is cleft in the epithelium which consists the filiform papillae producing a shaggy, papillomatous
of eosinophils. During the verrucous stage hyperkeratosis, surface on the dorsal tongue. The lips may be enlarged
acanthosis, and papillomatosis is seen. There are also macro- and covered by papillomatous growths, particularly at the
phages in the connective tissue with hyperpigmentation. angle of mouth. The buccal mucosa may show a velvety
white appearance with occasional papillary lesions.
Management
No treatment is necessary for skin disease. Dental defects Histopathological Features
can be corrected with the assistance of orthodontics and There is marked acanthosis, coupled with peculiar
prosthetic dentistry. parakeratosis (Fig. 29.18). Upward finger like projection
of dermal papillae occur. There is also thinning of adjacent
Points to Remember
epidermis and presence of pseudo horn cyst.
Bloch-Sulzberger syndrome, vesicular stage, verrucous Keratinized stratified squamous epithelium shows
stage, hyperpigmentation stage, brownish gray mac- papillary projections. Hyperkeratosis is a prominent
ules, atrophy and depigmentation stage, delayed feature. Skin lesions have more deposition of melanin
tooth eruption, peg and cone shaped crowns, cleft, and less acanthosis. Oral lesions show more spinous cell
hyperkeratosis, acanthosis, papillomatosis. acanthosis and less melanin deposition.
Skin Disorders
Rubber man: In some patients, skin extensibility is

pronounced, the patient is known as ‘rubber man’.
Papyraceous scarring: This is an unusual healing response 745
that occur to minor injury to skin. It resembles crumpled
cigarette paper.
Hypertelorism, a wide nasal bridge, epicanthic folds,
protruding ears and frontal bossing are often present.
Freely movable subcutaneous nodules are frequently found,
which represent fibrosed lobules of fat. The scarring of skin
following wound healing in these patients is unusual, as the
scars tend to spread rather than contract in time.
In hypermobility types there is hyperextensibility of the
joint.
In vascular types there is fragility of skin and blood
Figure 29.18 Acanthosis nigricans showing like projection, vessels, resulting in excessive bruising as well as defective
parakeratosis and hyperpigmentation. (Courtesy: Dr Sangamesh healing of skin wounds. Rupture of large arteries as well
Halawar, Reader, Oral Pathology, CDCRI, Rajnandgaon, as of intestine often occurs, producing a life-threatening
Chhattisgarh)
situation.
Oral Manifestations
Management Oral mucosa is of normal color but is excessively fragile
As such there is no treatment for this disease. Patient and bruises easily. The gingival tissue appears fragile and
should be properly evaluated and if malignancy is present, bleeds after tooth brushing. Hypermobility of temporo-
it should be treated. mandibular joint resulting in repeated dislocations of the
jaw have been reported.
Points to Remember Gorlin sign: There is ability of the patient to touch
Brown patches, verrucous pigmented lesions, hyper- their nose with tongue.
trophy of the filiform papillae, enlarged lips, acanthosis, There may be lack of normal scalloping of the
pseudo horn cyst, finger like projection of dermal dentinoenamel junction, formation of irregular dentin and
papillae, parakeratosis. increased tendency to form pulp stones with hypoplastic
changes in enamel.
EHLERS DANLOS SYNDROME Lab Findings

It is also called cutis hyperelastica and it is a group Clotting time is normal, but capillary fragility test is usually
of hereditary disorders of connective tissue. There is positive.
production of abnormal collagen protein which is structural
Management
compound of connective tissue.
There is no specific treatment for this disease, but surgical
Types procedures should be carried out carefully as healing
There are mainly seven types of Ehler Danlos syndrome. problems can exist.
They are classical (mild and severe), hypermobility,
Points to Remember
vascular, kyphoscoliosis, arthrochalasis, and dermato-
sparaxis. Hyperelasticity of skin, rubber man, papyraceous
scarring, hypertelorism, frontal bossing, hypermobility
Clinical Features of joint, fragility of skin, fragile gingival tissue, hyper-
There is hyperelasticity of skin is a feature of this mobility of temporomandibular joint, Gorlin sign
disease. positive capillary fragility test.
PSORIASIS
It is common a dermatological disease characterized by
746 white, scaly papules and plaque on an erythematous base
that preferentially affects the extremities and scalp. Word
psoriasis comes from Greek word for itching.
Etiology and Precipitating Factors

It has been suggested a possible inheritance pattern,
possibly transmitted as simple dominant trait. b-hemolytic
streptococcal infection often precedes psoriasis. Metabolic
disturbances and endocrine dysfunction can also precede
psoriasis.
There is increase proliferative activity of cutaneous
keratinocytes. Activated T-lymphocytes is found to
causative mechanism for abnormal production.
Antimalarials, b-blocker and lithium may worsen Figure 29.19 Patient is having dusky appearance on leg in
psoriasis and the rash may rebound after stopping systemic patient of psoriasis
corticosteroids or potent local corticosteroid. Anxiety
precipitates some exacerbation. Mental anxiety and stress
can increase severity of the disease. In areas of skin damage Clinical Types
such as scratches or surgical wound. Stable plaque psoriasis: It is most common type. The
lesions are red with dry, silvery-white scaling, which may
Clinical Features be obvious only after scraping the surface.
Age and site distribution: It commonly affects adults and Guttate psoriasis: It is seen in children adolescents and
arises in 2nd and 3rd decades of life. It is commonly occur may follow streptococcal sore throat. Individual lesions are
on extremities, and scalp. droplet-shaped, small and scaly.
It is usually chronic with acute generalized exacerb-
Erythrodermic psoriasis: The skin becomes universally
ations. It is more severe in winter and less severe in the
red and scaly or more rarely just red with very little scale
summer as a result of increase exposure to ultraviolet light.
present.
Symptoms: It is characterized by occurrence of small
Pustular psoriasis: It is a severe form of psoriasis with
sharply defined, dry papules each covered by delicate
eruption of minute pustule with shedding of nails is common.
silvery scale which appear as resembling a thin layer of
mica (Fig. 29.19). Oral Manifestations
Auspitz’s sign: If the deep scale is removed one or more Site: Oral lesions are reported on lip, buccal mucosa,
bleeding points are seen. palate, gingiva and floor of mouth.
Papules are enlarged at periphery and may form large
Appearance: They appear as plaques, silvery, scaly
plaques which are roughly symmetrical. After removal of
lesions with an erythematous base. Sometime they are
scale the surface of skin is red and dusky in appearance.
multiple papular eruptions which may be ulcerated or as
In some cases involvement of joint can occur which is
small, papillary elevated lesions with scaly surface.
term as psoriatic arthritis. This can also involve TMJ.
Usually four types of oral manifestation occur in patient
Clinical Types with psoriasis:
The first type consists of minute well defined gray to
• Stable plaque psoriasis
yellowish white lesion that is round or oval.
• Guttate psoriasis
The second type is characteristic by white, elevated,
• Pustular psoriasis
lacy, circinate lesion on the oral mucosa including the
• Erythrodermic psoriasis.
tongue. These eruption parallel those of the skin.
Skin Disorders
The third type consists of a fiery red erythema of the Management

oral mucosa, including the tongue is seen in acute form.
Topical Agents
The fourth type of oral lesion described in psoriasis is a
geographic tongue that occurs most frequently among the 747
Emollients: It have modest effect in terms of reducing
patients with psoriasis than without. scale.
Histopathological Features Dithranol: It is gold standard therapy with dithranol. It
Intraepithelial micro-abscess formation (Munro’s abscess) normalized differentiation and inhibits proliferation when
occurs. It is characterized by uniform parakeratosis, applied to psoriatic plaques.
absence of stratum granulosum. Tar: Crude tar is used which is pro-inflammatory and
Test tube appearance: There is elongation and action same as dithranol.
clubbing of rete pegs. This appearance of rete pegs is
Calcipotriol: It is vitamin D agonist and it reduces the
termed as test tube appearance (Figs 29.20 and 29.21).
thickness of plaque.
The epithelium over the connective tissue is thin and
it is from this point bleeding occurs when the scales are Retinoid: It diminished the induration, scaling and redness
removed. Tortuous, dilated capillaries extending high in of plaque.
the papillae are prominent. Corticosteroids: Use of potent topical corticosteroid on
the face or hair margins should be under close and expert
medical supervision.
Ultraviolet light: It is mainstay of management of patient
with moderate to severe psoriasis. It is used 3 to 7 times in
a week.
PUVA therapy: Psoralens are natural photosensitizes
found in number of plants. It includes clearance to greater
degree than any other therapy.
Systemic treatment: Three main systemic agents are
using, i.e. methotrexate, oral retinoid and ciclosporin.
Points to Remember
Figure 29.20 Psoriasis showing test tube shaped rete pegs b-hemolytic streptococcal infection rebound rash, dry
papules covered by delicate silvery scale, Auspitz’s
sign, psoriatic arthritis, scaly lesions, erythematous
base, Munro’s abscess, uniform parakeratosis, absence
of stratum granulosum, Test tube appearance, Tortuous
dilated capillaries, Dithranol, Calcipotriol PUVA therapy.
PACHYONYCHIA CONGENITA
It is also called Jadassohn-Lewandowsky syndrome. It is
extremely uncommon disease, inherited as an autosomal
dominant characteristic with incomplete penetrance.
Clinical Features
It usually occurs shortly after birth with no sex predilection.
Figure 29.21 Psoriasis showing elongation of rete pegs, It consists of dystrophic changes in the fingernails and
hyperkeratosis and inflammation of the papillary connective toenails (Fig. 29.22), hyperkeratotic calluses of the palms
tissue (Courtesy: Dr Sangamesh Halawar, Reader, Oral and soles, follicular keratosis about the knees and elbows,
Pathology, CDCRI, Rajnandgaon, Chhattisgarh) hyperhidrosis or excessive sweating of the hands and feet.
Points to Remember
Dystrophic changes, follicular keratosis, hyperhidrosis,
748 excessive sweating of the hands and feet, sparse hair,
corneal dyskeratosis, painful blister after walking, focal
or generalized white, opaque thickening of the mucosa,
oral aphthous ulceration, Natal teeth, intra-cellular edema
or vacuolization of the spinous cell, perinuclear clearing.
POROKERATOSIS
It is also called Mibelli’s disease and it is autosomal
dominant. It is characteristic by faulty keratinization of the
skin followed by atrophy.
Figure 29.22 Pachyonychia congenita showing dystrophic Clinical Features
nails
Age sex and site predilection: The majority of begins in
There is marked thickening, increasing toward the free early childhood but progression of disease is extremely
border with nail bed becoming filled with yellowish keratotic slow. It appears to occur in males with greater frequency
debris, often causing the nail to project upward at the free than in female. It occurs most commonly in extremities
edge. It is associated sparse hair and corneal dyskeratosis particularly in hands and feet, as well as shoulder, face and
producing corneal opacities have been reported. neck and the genitalia.
Bullae formation occurs on the feet, and secondary Keratotic papules: It consists initially of crateriform
infection of these may lead to crippling deformity. The keratotic papules which gradually enlarged to form
striking features of this disease is that there is formation elevated plaques. In some cases there is ring like keratotic
of painful blister after few minutes of walking in the lesion of the skin with atrophic center.
warm weather. Thickening of the laryngeal commissures,
tympanic membrane and nasal mucosa and mental Signs: Keratotic papules size is ranging in size from
retardation are also reported. a few millimeters to several centimeters. The plaques
are surrounded by a distinct raised border of epidermal
Oral Manifestations proliferation.
The most common site of occurrence are buccal mucosa, Nails: The nails commonly become thickened and ridged.
tongue and lips. The central portion of the lesions ultimately becomes
They consist of focal or generalized white, opaque atrophic, leaving permanent scarring.
thickening of the mucosa. There is frequent oral aphthous
ulceration is seen. In some cases inflammation of angle of Oral Manifestations
mouth is seen. Natal teeth are also present. It is most commonly seen on upper lip and palate. There is
numerous small slightly opalescent ring and serpenginous
Histopathological Features and hyperemic border studded over the palate.
The mucous membrane exhibits and intracellular edema
or vacuolization of the spinous cell reminiscent of white Histopathological Features
sponge nevus. A nonspecific thickening of the parakeratin The elevated horny margin of the lesion exhibits
and spinous cell layer is seen. There is also acanthosis with hyperkeratosis and acanthosis with a deep groove filled
perinuclear clearing of the epithelial cells. with parakeratin and a characteristic absence of the usual
underlying granular layer (Fig. 29.23). It constitutes the
Management coronoid lamella which is characteristic of the lesion.
There is no treatment for this disease as lesion in oral cavity The connective tissue beneath the coronoid lamella
has no tendency for malignant transformation. may exhibits lymphocytes infiltrate. The central portion
Skin Disorders
Skin lesion: In the skin fold the lesion tend to be

coalesce and produce verrucous or vegetating macerated,
foul smelling masses. Foul odor is results of bacterial
degradation of the keratin. 749
Nail changes: Characteristic nail changes are consisting of

splintering, fissuring, longitudinal streaking and sublingual
keratosis.
Oral Manifestations
Keratotic papule occur on oral mucosa particularly on hard
and soft palate, gingiva, tongue have whitish appearance.
They are multiple whitish papules which feel rough
upon palpation. In some cases it has been described as
rough, pebbly areas with verrucous white plaque or as
Figure 29.23 Porokeratosis showing hyperkeratosis and having cobblestone appearance. Papule become confluent
acanthosis as disorder progress.
of the lesion shows epithelial atrophy and occasionally Histopathological Features

dyskeratosis. The characteristic finding in skin lesion is hyperkeratosis,
papillomatosis, acanthosis and peculiar benign dyskera-
Management tosis.
There is no treatment for the disease except for removal of Corps ronds and grain appearance: Dyskeratosis is
individual lesions. characterized by typical cells called corps ronds and grains
(cell within cells) (Fig. 29.24). The corps ronds (round
Points to Remember bodies) are larger than normal squamous cells and have
Keratotic papules, nails thickened ridged, opalescent a round, homogeneous, basophilic nucleus with a dark
ring, serpenginous hyperemic border studded over the eosinophilic cytoplasm and distinct cell membrane. Grains
palate, hyperkeratosis, lymphocytes infiltrate, para- (resembles cereal grain) are small, elongated parakeratotic
keratin, coronoid lamella, acanthosis. cells situated in the keratin layer.
KERATOSIS FOLLICULARIS
It is called Darier’s disease, Darier-White disease and
dyskeratosis follicularis. It is autosomal dominant trait
with high degree of penetrance and variable expressivity.
There is lack of cohesiveness among the surface epithelial
cells characterized the disease.
Clinical Features
Age, sex and site distribution: It is usually manifested
during childhood or adolescence and has equal sex
distribution. They are generally distributed above the
forehead, scalp, neck, and over the shoulders.
Appearance of lesion: The cutaneous lesion appears
small, firm papules. They are red when they first appear but
characteristically become grayish brown or even purple. It Figure 29.24 Keratosis follicularis showing corps and grain
can ulcerate and crust over. appearance
Acantholytic cells are commonly found floating in the Oral Manifestations

lacunae produced by this intraepithelial separation.
It is very rare and it is if present found most commonly on
750 Management the alveolar ridge and palate.
Patient should minimized unnecessary exposure for hot Appearance: It appears as small whitish or pink area of the
environment. Administration of large dose of retinoid is mucosa with a central depression.
given. Focal acantholytic dyskeratosis: It is variant of warty
dyskeratoma in which two or three discrete lesion arising
Points to Remember
adjacent to one another.
Darier’s disease, cutaneous lesion appears small, firm
papules, skin lesion verrucous vegetating macerated, nail Histopathological Features
splintering and fissuring, sub-lingual keratosis, rough, The microscopic finding of skin and mucosal lesion are
pebbly areas with verrucous white plaque, cobblestone identical except for the absence of a pilosebaceous structure
appearance, Corps ronds and grain appearance, in the oral lesion.
Acantholytic cells, retinoid. The intraoral lesion exhibits a central orthokeratin
or parakeratin core beneath which the epithelium shows
WARTY DYSKERATOMA a suprabasilar separation resulting in a cleft like space
containing acantholytic and benign dyskeratotic cells.
It is also called isolated Darier’s disease which bears The connective tissue papillae are covered usually
histological similarity to Darier’s disease but it present as by a single layer of basal cells while the underlying
single isolated focus connective tissue shows a non-specific chronic inflamm-
atory infiltrate. Spinous layer is thickened and contains
Clinical Features
individually keratinized cells.
Age and site distribution: It is usually occur in older age
group with male predominance. The skin lesion occurs on Management
face, scalp, neck and upper chest. It should be treated by surgical excision.
They appear as elevated nodules, umblicated, with
raised borders and varying in color from yellow or brown Points to Remember
to gray or black (Fig. 29.25). Elevated nodules, umblicated with raised borders,
They appear as invariably single lesion varying in size color yellow or brown to gray or black, whitish or pink
from 1 to 10 mm in diameter. Purulent drainage as well as area of the mucosa, Focal acantholytic dyskeratosis,
bleeding occurs in some cases. pilosebaceous, orthokeratin or parakeratin core, spinous
layer thickened, chronic inflammatory infiltrate.
SEBORRHEIC KERATOSIS
In this there is benign proliferation of epidermal basal cells.
It can cause by chronic sun exposure or it can be hereditary.
Clinical Features
Location: It is commonly seen on skin of the face, trunk,
and extremities.
Age: It is usually presented in during 4th decade of life.
Appearance: Multiple lesion which initially small tan to
brown macules which enlarge gradually and elevated. Indi-
vidual lesions are well demarcated plaque with fissured and
Figure 29.25 Warty dyskeratosis showing elevated lesion pitted surface. They have got stuck onto skin appearance.
Skin Disorders
Dermatosis papulosa nigra: This appear in black and Oral Manifestations

appear as multiple dark brown to black papules scatter
Oral lesion is fiery red, flat or micropapillary frequently
about zygomatic and periorbital region.
involving gingiva and hard palate. Red enlarged and 751
Laser-Trelat sign: Sudden appearance of numerous fissured tongue is common occurrences.
seborrhoeic keratosis with pruritus has been associated
with internal malignancy. Histopathological Features
The oral lesions show a lack of cornified and keratinized
Histopathological Features cells, thinning of the epithelium and dyskeratosis. There is
There are exophytic proliferation of basilar epithelial cells disorganized maturation pattern. The squamous epithelial
with surface keratinization, acanthosis and papillomatosis. cells have high nuclear/cytoplasmic ratio. Cytoplasmic
vacuoles appear as grayish inclusion.
Horn cyst or pseudo horn cyst: The lesion exhibits deep
keratin filled invagination which appear cystic on cross Management
section.
No specific treatment for the disease.
Squamous eddies: Squamous metaplasia of the lesion
cells results in whorled epithelial pattern.
Points to Remember
Histopathological Types of Seborrhoeic Keratosis • F ollicular keratosis of the skin, non-scarring alopecia,
• A canthotic form: It exhibits little papillomatosis and severe photophobia
marked acanthosis with keratinization • Red enlarged and fissured tongue, cornified epithe-
• Hyperkeratotic form: There is prominent papillo- lium, high nuclear/cytoplasmic ratio, dyskeratosis,
matosis and hyperkeratosis with minimal acanthosis keratinized cells.
• Adenoid form: It consists of anastomosing trabecular
or lesion cell with little hyperkeratosis or papillo-
matosis
PSEUDOXANTHOMA ELASTICUM
• Inverted follicular keratosis Helwig: It is irritated It is autosomal recessive and the basic defect involves the
seborrhoeic which occur chronic trauma. It show structure of elastin, making it susceptible of calcification.
mild proliferation into connective tissue and chronic It is rare hereditary connective tissue disorder, chara-
inflammatory cell infiltrate adjacent to lesion. cterized by generalized degeneration of the elastic
fibers with a broad phenotypic expression. The clinical
Management picture consists mainly of cutaneous, ocular, and vascular
Cryotherapy with liquid nitrogen is treatment of choice. manifestations.
Clinical Features
HEREDITARY MUCOEPITHELIAL
Age: Although widely variable, the age of onset averages
DYSPLASIA
13 years with no predilection for sex.
It is described by Witkop and associates in 1978. It is
Appearance: Raised yellowish papules develop on areas
autosomal dominant. Mucosal epithelial cells do not
of thickened, coarsely grained skin especially around the
develop in normal fashion so the name dysplasia is given.
mouth, neck, axilla, elbows.
Clinical Features Angiod steaks: Brownish gray streaks of the optic fundus
It affects all orofacial mucosa and is characterized by (angiod streaks), recurrent gastrointestinal hemorrhage,
follicular keratosis of the in skin, non-scarring alopecia. and weak pulse, and failing vision is common occurrence.
Eyelashes and eyebrows are frequently affected. The typical cutaneous lesions are small yellowish
Severe photophobia develops at early age resulting in papules or larger coalescent plaques with an appearance
cataracts, corneal vascularization. Repeated episodes of similar to plucked chicken skin. More severely affected
pneumonia, spontaneous pneumothorax. skin results in hanging, redundant folds.
Oral Manifestations There may be calcification of dorsum sellae. Cutaneous

lesions commences as vesicles, upon healing acneform
Mucous membranes, mainly of the inner aspect of the
scars develop. Most of the patient exhibits thickened
752 lower lip are affected.
furrowed appearance.
Hound dog appearance: Skin around mouth becomes
In some cases intracranial calcification may lead to
redundant, producing a hound dog appearance. Lower lip
seizure disorders.
exhibit yellowish intramucosal nodule.
Histopathological Features Oral Manifestations

Intramucosal nodules show large number of thickened and Most commonly affected area is lower lip. It can be seen on
twisted elastic and collagen fibers. Skin histopathology tongue, buccal mucosa. Affected tissue become infiltrated
involves swollen, irregularly clumped and multiply with yellowish white elevated pea sized plaque which
fragmented elastic fibers in the middle and deep reticular gradually increased in size in puberty.
dermis, with secondary calcium deposition. Symptoms: Recurrent painful parotitis with stenosis of
parotid duct opening occurs. Congenital absence of teeth
Management and severe enamel hypoplasia may occur.
At present, no specific treatment exists. The knowledge, Radiating tissue may appear at angle of mouth. Tongue
however, of the potential complications may lead become firm and large and bound to floor of mouth. The
physicians to take some necessary precautions. dorsum of tongue may losses its papilla and ulcer may
develop. Diffuse hyperplastic appearing gingival infiltr-
Points to Remember ation is present.
Angiod steaks, raised yellowish papules, Hound dog There is restriction of oral function such as saliva flow,
appearance, multiply fragmented elastic fibers swollen tooth eruption, and swallowing. There is hypodontia of
irregularly clumped, twisted elastic and collagen fibers. maxillary lateral incisors and premolars.
Lips become thickened and nodular white while tongue
becomes thickened, enlarged, very firm to palpation and
HYALINOSIS CUTIS ET MUCOSA ORIS sometimes bound to floor of mouth.
It is also called lipoid proteinosis or Urbach-Wiethe syn-
drome. It is autosomal recessive trait, characterized by sub- Histopathological Features
dermal and submucosal infiltration of a hyaline glycoprotein It shows diffuse hyalinization with prominent hyaline peri-
material. It occurs due to disturbance of mucopolysaccharide vascular cuffing. There is deposition of lamellar material
metabolism or alteration in formation of lipoprotein. around blood vessels nerves, hair follicles and sweat gland.
These deposits are PAS positive and show equivocal
Clinical Features reaction for amyloid.
Age and site distribution: It is mostly seen in young
adults with no sex predilection. It is common in mucosal Management
tissue, skin, vessel walls, larynx, and brain. It is also seen No known treatment for the disease. Gingivectomy
in face, eyelids and neck. is recommended when diffuse hyperplastic appearing
Symptoms: Laryngeal mucosa and vocal cords are first gingival infiltration is present.
sites to involve. So patient wills complaint of hoarse voice
during childhood. In infancy hoarse cry and inability of the Points to Remember
infant to make crying sound. Hoarse voice, clustered yellowish white waxy nodules,
Signs: Patient develops solitary or clustered yellowish yellowish white elevated pea sized plaque, recurrent
white waxy nodules. It is solid in consistency and fixed to painful parotitis, firm tongue, diffuse hyperplastic
underlying tissue. It varies in size from 1 mm to 0.5 cm in gingival infiltration, hypodontia, diffuse hyalinization,
diameter. It has nonulcerated surface. hyaline perivascular cuffing.
Skin Disorders
WHITE SPONGE NEVUS

It is oral genodermatose, which was described by cannon
in 1935, hence it is also known as Cannon’s disease. It 753
is also called white folded gingivostomatitis, congenital
leukokeratosis, pachyderma oralis or oral epithelial
nevi. It is autosomal dominant with irregular penetrance.
It appears to represent a defect in epithelial maturation
involving tonofilament formation with impaired normal
desquamation of the superficial strata of cells.
Clinical Features
Age and site distribution: It has no definite sex
predilection with children most commonly affected and Figure 29.26 White sponge nevus showing marked increase
in the spinous cell layer and vacuolated cells
may present at birth and may become intense at puberty.
The most common sites are cheek, palate, gingiva, floor of
mouth, portion of tongue. It may be widespread and may
involve entire mucosa. It can also occurs on the mucous
membranes of the nose, esophagus, genitalia, and rectum.
Signs and symptoms: Mucosa appears thickened and
folded or corrugated with soft or spongy texture and a
peculiar white opalescent line. It has got sodden, furrowed
or wrinkled appearance. The lesion varies in extent from a
small patch to involvement of a large area of mucosa.
Friction may strip superficial keratotic area leaving
zone of normal looking epithelium or raw area. Ragged
white area may be present which can be removed by gentle
rubbing without bleeding.
Figure 29.27 White sponge nevus showing inflammatory cell
Histopathological Features infiltration
(Figs 29.26 to 29.28)
Epithelium thickened showing hyperkeratosis, acanthosis
with basal layer being intact. In some cases there is
extensive keratosis showing basket-weave appearance.
The cells of the entire spinous layer exhibit intra-
cellular edema and show pyknotic nuclei. There is also
oraganophilic perinuclear cytoplasmic condensation.
In addition, parakeratin plugs running deep into the
spinous layer are typically found. The submucosa may
show a mild inflammatory cell infiltration.
The characteristic feature of white sponge nevus
is the perinuclear eosinophilic condensation which is
more appreciable in exfoliative cytology. This is proved
ultra structurally to be tangled masses of keratin tono- Figure 29.28 White sponge nevus showing acanthosis with
filament. clear cytoplasm
Management Histopathological Features

There is no specific treatment for this disease but prognosis Buccal mucosa exhibits thickening of the epithelium with
754 is very good. Palliative procedure to relive burning and pronounced hydropic degeneration. Numerous round;
tenderness. waxy-appearing eosinophilic cells resembling minute
epithelial pearls are present.
Points to Remember There is peculiar intraepithelial dyskeratosis in addition
Cannon’s disease, thickened corrugated mucosa, to acanthosis and vacuolization.
raw ragged white area, basket-weave’ appearance,
intracellular edema, parakeratin plugs, perinuclear Management
eosinophilic condensation. No treatment is necessary but its potential for blindness,
genetic counseling may be in order.
HEREDITARY BENIGN Points to Remember
INTRAEPITHELIAL DYSKERATOSIS Witkop-Von Sallman syndrome, temporary blindness,
It is also called Witkop-Von Sallman syndrome. It is photophobia, white, spongy, macerated lesions of the
superficially similar to the white sponge nevus in its buccal mucosa, pinpoint elevated plaque, hydropic
hereditary pattern but its clinical and microscopic features degeneration, round; waxy-appearing eosinophilic cells.
are different. It is inherited as autosomal dominant trait
and there is defect in keratinization characterized by
cytoplasmic accumulation of tonofilament with loss of HEREDITARY HEMORRHAGIC
cellular interdigitation and desmosomes. TELANGIECTASIA
Clinical Features It was first described by Osler in 1901 and it frequently bears
It is most commonly seen in children with no sex his name (Osler-Rendu-Weber syndrome). It is transmitted
predilection. It is commonly seen in eyes. Superficial as an autosomal dominant trait and characterized by
gelatinous looking plaques occur on a hyperemic bulbar bleeding from mucous membrane and telangiectatic lesions
conjunctiva. on skin and mucosa.
Eye is characterized by superficial, foamy, gelatinous
Clinical Features
white plaque overlying the cornea, sometimes producing
temporary blindness. Eye lesion show seasonal variation Age and site distribution: They are found most comm-
tending to appear or increase in severity in the springs only on the skin of the face, finger, toes and on the oral
and disappear. There may be photophobia and cause by mucous membrane. The lesion may be present in childhood
involvement of cornea by plaque formation and scarring. but more often appear in puberty and become progressively
worse with increasing age.
Oral Manifestations
Telangiectases: The disease is characterized by multiple
Site predilection: They are most commonly seen in buccal localized angiomatas or telangiectases on the skin. The
mucosa, floor of mouth, ventral and lateral surfaces of the lesion bleeds easily, even after slightest trauma. Bleeding is
tongue, the gingiva, and palate. not caused by clotting factor deficiency but as a result of
Appearance: It appears generally as white, spongy, rupture of the weak capillaries. The typical lesion is cherry-
macerated lesions of the buccal mucosa with or without red to purplish macule or small papule that resembles a
folds. crushed spider.
These lesions vary from delicate, opalescent white The lesion blanches on pressure and regains it’s
membranous areas to a rough, shaggy mucosa. Lesion color when the pressure removed. The telangiectatic
frequently involves the corners of the mouth and appears areas are not-pulsating. As the affected individual grows,
as soft plaques with pinpoint elevation when the mucosa is the bleeding episode tend to increase in frequency and
stretched. intensity. They often give rise to profuse hemorrhage, such
Skin Disorders
as episodes of epistaxis. Severe bleeding may also occur Clinical Features

from the gastrointestinal tract.
Age and sex distribution: There is no sex or racial
Oral Manifestations preponderance. It is seen in childhood. 755
Oral cavity, lips and tongue are most commonly affected. Site: It is seen in periorificial area like mouth, nose, anus
Cherry red, often slightly raised, pin point or slightly larger and genital region. Skin is affected in half of the patient.
lesions resembling a crushed spider is seen at any intraoral Pigmented macules show an increased amount of
site, especially at the lip. Severe oral hemorrhage may be melanin in the basal layer of the epidermis without any
experience several times a day for weeks. associated increase in the number of melanocytes. The oral
At times there is gush of blood when involved areas mucosa is always involved with affection of buccal mucosa,
are simply touched with cotton. Hemorrhage can be gums hard palate and lower lips. Lesions are irregularly
encountered during dental treatment which encroaches the distributed round, oral or irregularly patch brown or black
affected area. in color and 1 to 5 mm in diameters.
Gastrointestinal polyps are benign hamartomas with
Histopathological Features relatively less potential for malignant transformation. They
Intrinsic defect in the endothelial cells permitting their may occur throughout the gastrointestinal tract but are
detachment or defect in the perivascular supportive tissue commonly observed in the small intestine. The symptoms
bed, which weakens the vessels. of Gl Polyps are repeated abdominal colic, rectal, bleeding,
hematoma, anemia usually start between 10 and 30 years
Diagnostic Criteria of age. Apart from an increased risk of malignancy life
Diagnosis of hereditary hemorrhagic telangiectasia is made expectancy is normal.
if patient has three of the following criteria.
Recurrent Spontaneous Epistaxis Polyp represents overgrowth of intestinal glandular
• Telangiectasia of the mucosa and skin epithelium which are supported by core of smooth muscle.
• Arteriovenous malformation involving the lungs There is also slight acanthosis of the epithelium with
liver or CNS elongation of rete pegs. Dentritic process of melanocytes is
• Family history of HHT. elongated.
Management
Management
Genetic counseling should be done.
Sclerosing agent such as sodium morrhuate or sodium
tetradecyl sulfate injected into the lesion is useful. Points to Remember
Spontaneous hemorrhages may be controlled by
pressure packs, particularly nasal bleeding. Periorificial lentiginosis, pigmented macules, gastro-
intestinal polyps, slight acanthosis, elongated, dentritic
Points to Remember process of melanocytes.
Osler-Rendu-Weber syndrome, cherry-red to purplish
macule, crushed spider, sclerosing agent sodium EPHELIS
morrhuate or sodium tetradecyl sulfate.
It is also called freckle. It is hyperpigmented macule seen
on skin which occurs due to increase melanin pigmentation.
PEUTZ-JEGHERS SYNDROME It has got genetic predisposition. Lesions become more
It is also called periorificial lentiginosis. This is an pronounced after sun exposure.
autosomal dominant disorder showing pigmented macules
on the oral mucosa and skin associated with gastrointestinal Clinical Features
polyposis. A family history is fond in 60 percent of cases. Age: It is more commonly seen in teenage years.
Location: It is more commonly seen face, arms, and back

Points to Remember
of the patients. Macule may be multiple in numbers.
Lentigo solaris, uniformly pigmented brown to tan
756 Appearance: It is round to oval and remains less than macules, lesion may coalesce, elongated rete ridges, club
3 mm diameter with light brown color. It is well dem- shaped actinic lentigines, cryotherapy.
arcated from the surrounding skin.
Histopathological Features LENTIGO SIMPLEX

It is composed of stratified squamous epithelium with It is benign melanocytic cutaneous hyperplasia of unkn-
melanin in basal cell layer. own cause.
Management Clinical Features

The use of sunscreen lotion with help in preventing Age: It is more commonly seen in children
appearance of new lesion
Location: It occur in areas which is not exposed to sunlight.
It is seen skin of trunk and extremities
Points to Remember
Freckle, face, arms, and back of the patients, round to Appearance: It is tan to dark brown color macule with well
oval, stratified squamous epithelium with melanin in demarcated border which is less than 5 mm in diameter.
sunscreen lotion. Histopathological Features
Basal layer of melanocytes show increase number of
ACTINIC LENTIGO benign melanocytes. This are seen clustered at the tip of
It is also called lentigo solaris, solar lentigo, age spot, liver rete ridges.
spot, and senile lentigo. Melanin incontinence: Abundant melanin and basal
It is macule which occurs due to chronic ultraviolent keratinocytes seen in melanocytes and papillary dermis in
damage to the skin. association with melanophages.
Management
Clinical Features
Conservative surgical excision should be done for esthetic
Age: It is more commonly seen in people more than 70
purpose.
years of age.
Location: It is seen on dorsa of hand, face, and arms of Points to Remember
older white people. Benign melanocytic cutaneous hyperplasia, skin of trunk
and extremities, tan to dark brown color macule, increase
Appearance: Individual lesion appear as uniformly
number of benign melanocytes, melanin incontinence,
pigmented brown to tan macules with well demarcated
conservative surgical excision.
irregular border.
Sign: Lesion is less than 5 mm in diameter. Lesion may
coalesce. SEBACEOUS HYPERPLASIA
In this there is localized proliferation of sebaceous gland of
Histopathological Features the skin. It can be associated with cyclosporine, systemic
There are elongated rete ridges with club shaped actinic corticosteroid, and Muir-Torre syndrome.
lentigines. There is also thinning of epithelium above the
Clinical Features
connective tissue papillae.
Age: It is seen in older individual in 5th decade of life.
Management Location: It is seen on skin of face in area of nose, cheeks,
Lesion is treated by cryotherapy, laser therapy, and intense and forehead. It can also occur on genital area, chest, and
pulsed light therapy. areola.
Skin Disorders
Appearance: It is yellow white nontender papules which

are soft in consistency.
Sign: Lesion is umblicated with central depression which 757
occur due to ducts of sebaceous gland. Lesion is smaller
than 5 mm in diameter. Thick yellow white sebum
expressed in the central depressed area after compression
of lesion.
Oral sebaceous hyperplasia: It show cauliflower appe-
arance seen on buccal mucosa.
There is collection of enlarged sebaceous gland lobule
around centrally located sebaceous duct. Figure 29.29 Pigmentation seen on neck and face due
xeroderma pigmentosum
Management
Excisional biopsy, cryosurgery, electrodessication, laser
therapy, and photodynamic therapy can be done. It is only Oral feature: Orally squamous cell carcinoma can occur
done for esthetic reason. on lower lip and tip of the tongue.
Points to Remember
Localized proliferation of sebaceous gland of the skin, Malignancy which occurs in this disease has got same
yellow white nontender papules, lesion is umblicated, manifestation as that seen in other malignancy.
central depression, oral sebaceous hyperplasia, collection Management
of enlarged sebaceous gland lobule, excisional biopsy.
Protection: Patient should avoid sunlight and non- filtered
light. If they cannot avoid sunlight protective clothing and
XERODERMA PIGMENTOSUM sunscreen should be used.
It is very rare genodermatosis where cutaneous malignancy Topical chemotherapeutic agents: 5-fluorouracil can be
develops. It is inherited as autosomal recessive trait. used to treat actinic keratosis.
Cause Points to Remember

There is inability of the epithelial cells to repair ultraviolet Actinic keratosis, oral squamous cell carcinoma, freckled
induce damage. Due to this mutation in epithelial cells pigmentation on skin, topical chemotherapeutic agents –
occur resulting in development of skin cancer. 5-fluorouracil.
Clinical Features
TUBEROUS SCLEROSIS
Age: It usually occurs in first decade of life.
It is also called Pringle-Bourneville syndrome. Tuberous
Site: As this is related to sun exposure head and neck is the sclerosis (TS) is an autosomal dominant disorder with
most common site of involvement. marked variability of expression in a given family.
Skin changes: Skin changes like atrophy, freckled pigmen-
tation and patchy depigmentation occur (Fig. 29.29) Clinical Features
Ash-leaf macules: These are hypopigmented macules of
Actinic keratosis: This can occur in childhood. This can
ovoid shape seen on the skin.
ultimately lead to squamous cell carcinoma or basal cell
carcinoma. Shagreen patch: These are connective tissue hamartomas.
Multiple angiofibroma: These are multiple red brown Management

macules and papules are seen about the mouth in the
Anticonvulsant agent: It is given for the management of
nasolabial folds.
758 seizure.
Ungual or periungual fibromas: These are seen under the Maintenance of oral hygiene should be done periodi-
margin of nails. cally.
Central nervous system (CNS) findings include epilepsy Points to Remember
mental retardation. Hamartomatous proliferation in the
CNS develops potato like growth (tubers). That is reason it Pringle-Bourneville syndrome, Ash-leaf macules,
is called tuberous sclerosis. Shagreen patch, multiple angiofibroma, ungual or
periungual fibroma, fibromas or papillomas are found
Other features: Others rare features include are cardiac on the gingiva tiny pits on dentitions, a high palate,
rhabdomyoma and angiomyolipoma. macroglossia, cleft lip, nonspecific fibrous hyperplasia,
plump uniform spaced fibroblast, anticonvulsant agent,
Oral Features thin walled vascular channels.
The oral cavity frequently shows distinctive changes.
Angiofibroma, fibromas or papillomas are found on the
gingiva, hard and soft palate, buccal mucosa and tongue.
ECTODERMAL DYSPLASIA
They may be white or yellow although they lack any It is also called hereditary hypohidrotic (anhidrotic)
distinctive tint. ectodermal dysplasia. It is an X-linked, recessive
Both dentitions have tiny pits arising from enamel Mendelian character. It results from aberrant development
defects. The pits are often an early diagnostic clue. of ectodermal derivation in embryonic life.
A high palate, macroglossia, cleft lip and palate and
hemangioma have been described. Clinical Features
Sex distribution: Males are affected more frequently than
Diagnostic Criteria (One major and two minor females.
criteria should be present)
Appearance: It is characterized by hypotrichosis,
Major criteria
hypohidrosis and anhidrosis with saddle nose appearance
• Facial angiofibroma
(Fig. 29.30).
• Hypomelanotic macules
• Shagreen patch
• CNS hamartomas
• Cardiac rhabdomyoma
• Renal angiomyolipoma
Minor criteria
• Enamel pit
• Gingival fibromas
• Multiple renal cyst
• Hamartomatous rectal polyps.
It is nonspecific fibrous hyperplasia seen in biopsy taken
from gingival fibrous papules.
Angiofibroma shows delicate fibrous connective tissue
which consist of plump uniform spaced fibroblast with Figure 29.30 Patient having saddle nose appearance seen in
interspersed thin walled vascular channels. case of ectodermal dysplasia
Skin Disorders
The hair of scalp and eyebrows tend to be fine, scanty

and blond. Supraorbital and frontal bosses are pronounced
(Fig. 29.31).
Skin is often dry, soft, smooth and scaly with partial 759
or complete absence of sweat glands. Such patient cannot
perspire and they usually suffer from hyperpyrexia and
inability to endure warm temperature.
Facial appearance of these individual resemble to each
other, enough to be mistaken for siblings.
In infancy patient cannot regulate skin temperature due
to decrease number of sweat gland.
Figure 29.33 Hypodontia in ectodermal dysplasia affected

child
Oral Manifestations (Figs 29.32 and 29.33)

Patient with this abnormality invariably manifest oligodontia
or partial absence of teeth, with frequent malformation of
any present tooth in deciduous and permanent dentition.
Teeth which are present are usually cone shaped.
There is reduction of normal vertical dimension of
alveolar process as there is absence of teeth. There is also
protuberance of lip due above reason.
The palatal arch is frequently high and cleft palate may
be present. Along with all this, since the salivary gland,
including the intraoral accessory gland, are sometimes
hypoplastic, they result in xerostomia and dry cracked lips
Figure 29.31 Hereditary ectodermal dysplasia in a child
with fissuring at the corners of mouth.
showing deficiency of hairs, eyelashes, eyebrows
In some cases there may be hypoplasia of accessory
gland which will results in xerostomia. There is also
Hypoplasia of nasal and pharyngeal, mucous gland lead to
chronic rhinitis and pharyngitis.
Histopathological Finding
There is reduction in the number of sweat gland, hair
follicles.
Epidermis is thin and flattened. Mucous gland in upper
respiratory tract is reduced in number.
Management
In dental point of view partial and complete dentures
Figure 29.32 Missing teeth seen in patient with ectodermal should be constructed for both functional and cosmetic
dysplasia purpose.

Hereditary hypohidrotic (anhidrotic) ectodermal dysplasia Multiple hamartoma syndrome, multiple hamartomatous
760 hypotrichosis, hypohidrosis, anhidrosis, saddle nose ap- papules, acral keratosis, palmoplanter keratosis, Thyroid
pearance hair of scalp fine, scanty, skin dry, soft, smooth and breast anomalies, polyposis of the gastrointestinal
and scaly, hyperpyrexia, sibling appearance, decrease tract, fibroepithelial hyperplasia.
number of sweat gland, oligodontia, cone shaped teeth, re-
duction of normal vertical dimension of alveolar process,
xerostomia, hypoplasia of accessory gland, chronic rhini-
GRAFT VERSUS HOST RESISTANCE
tis pharyngitis, reduction in the number of sweat gland, It usually occurs in person who is receiving allogeneic
hair follicles epidermis is thin. bone marrow transplantation.
Cause
COWDEN SYNDROME After giving bone marrow transplantation of HLA match
It is also called multiple hamartoma syndrome, PTN individual, the engrafted cells find them in different
hamartoma-tumor syndrome. It is a rare inherited environment. After this these cells start attacking other
genodermatose. This syndrome was named after the first cells thinking that they are foreign body. This will results
patient reported. It is inherited as autosomal dominant trait in GVHD (graft versus host resistance).
with high degree of penetrance. The gene responsible for
this syndrome is mutation PTEN. Clinical Features
Acute condition: This occur within first few week after
Clinical Features giving bone marrow transplantation. Patient complaint
Multiple hamartomatous papules: These are present on of rash on the body which can become severe sloughing
the skin and oral mucosa. Lesion is smaller than 1 mm. resembling toxic epidermal necrolysis. Patient may having
Acral keratosis: This is present on the dorsal surface of diarrhea, nausea, vomiting and abdominal pain.
hand and appears as warty growth. Chronic condition: This occur after 100 days. This
Palmoplanter keratosis: These are prominent callus like lesion often resembles lesion of systemic lupus
lesion seen on palms and soles. erythematous, Sjogren’s syndrome and lichen planus.
Thyroid and breast anomalies: Patient may suffer from Oral lesion: There is fine reticular white striae that can
goiter, thyroid adenoma, adenocarinoma and fibrocystic resembles oral lichen planus. There may be burning
disease of breast. Later on breast cancer can also occur. sensation, atrophy of oral mucosa, xerostomia, ulcerative
lesion and oral epithelial dysplasia.
Polyposis of the gastrointestinal tract: In the gastroin-
testinal tract presence of polyposis is seen. Histopathological Features
Oral features: Multiple papular lesions can affect gingivae, There is hyperorthokeratosis, pointed rete pegs, and
dorsal tongue and buccal mucosa. There may be high arch degeneration of basal cell layer.
palate, periodontitis and extensive dental caries. In advance cases abnormal deposition of collagen,
periductal inflammation of minor salivary gland. There is
Histopathological Features also gradual aciner destruction and periductal fibrosis.
Oral lesions represent fibroepithelial hyperplasia.
Management
Management Prevention: Proper HLA matching should be done. Patient
It is not so specific and patient should be treated for tum- can be given immune-modulator like cyclosporine or
ors. tacrolimus in combination with prednisolone.
Skin Disorders
Topical corticosteroid: This may facilitate healing of oral Management

ulceration.
Prognosis is better and patient should be monitored
Topical tacrolimus and PUVA therapy: This can be regularly. 761
given for the management of oral ulceration.
Types of Scleroderma
Points to Remember • Diffuse systemic or progressive systemic sclerosis
HLA match individual, rash on the body, fine reticular • Localized form
white striae, hyperorthokeratosis, pointed rete pegs, – Circumscribed or morphea
degeneration of basal cell layer, deposition of collagen, – Linear scleroderma.
topical corticosteroid, topical tacrolimus and PUVA
therapy.
SCLERODERMA
CREST SYNDROME It is also called systemic sclerosis, or Hidebound disease. It is
It is also called acrosclerosis, limited scleroderma. This a disease which involves connective tissue, blood vessels and
may be mild variant of systemic sclerosis. lead to fibrosis. It is also called progressive systemic sclerosis.
It is called sclera (hard) derma (skin).
CREST Syndrome In this disease there is deposition of dense collagen in
C- Calcinosis cutis the body.
R- Raynauds phenomenon
E- Esophageal dysmotility Etiology
S- Sclerodactyly It is caused by an endocrine dysfunction, vascular disease,
T- Telangiectasia. basically an endarteritis obliterans, nervous disorder, toxic
or infectious agents such as shock or pneumonia, influenza,
Clinical Features diphtheria and exanthematous disease.
Age and sex distribution: It is more common seen in sixth
and seven decade of life with female predilection. Clinical Features
Calcinosis cutis: It is movable, multiple, nontender Progressive Systemic Sclerosis
subcutaneous nodule. Age and sex distribution: It generally begins in childhood
Raynauds phenomenon: It occur when patient hands or or young adult and greatest incidence is between 30 and 50
feet are exposed to cold temperature. There is dead white years of age. Females are more commonly affected with a
color blanching due to vasospasm. After some time it take ratio of 3:1. It usually begins on face, hand or trunk.
bluish color due to venous stasis which later on become Symptoms: There is development of indurated edema of
dusky red hue after warming. skin, neuralgia and paresthesia.
Esophageal dysmotility: There is abnormal collagen Signs: Initial sign of progressive systemic sclerosis
deposit in epithelial submucosa. (PSS) is frequently Raynaud’s phenomenon, a paroxysm
Sclerodactyly: Finger undergo permanent flexure resulting vasospasm of finger. In several months the edema is
in claw deformity. Skin takes shiny appearance. replaced by tightening and hardening of the skin, which
results in difficulty in movements of the affected parts.
Telangiectasia: In this facial skin and vermilion border Hyper pigmentation, telangiectasia and subcutaneous
of lip is involved. These are same as that of hereditary calcification may occur, leading to deformity and severe
hemorrhagic telangiectasia. cosmetic problems along with involvement of internal
organs. Skin area has thickened hide bound cavity with
Histopathological Features lack of mobility of skin, limited mouth opening and renal
They are same as that of systemic sclerosis. involvement.
Localized Form (Circumscribed or Morphea) muscle. Gingival hyperplasia may result from calcium
channel blocker.
It usually occurs on the sides of the chest and thighs. It
762 begins with violaceous patches on the skin. These lesions Radiological Features
enlarge; become indurated and eventually loose hair and
ability to sweat. They may be present for several months Diffuse widening of periodontal ligament space is present
to many years. throughout the dentition. There is also resorption of ramus
Progressively, these lesions turn into hypo or hyper- of the mandible, coronoid process, chin, and condyle.
pigmented areas depressed below the level of skin. They
may become stiff and hard and are usually asymptomatic.
There is thickening and hyalinization of the collagen fibers
Localized Form (Linear) in the skin, with loss of dermal appendages, particularly
A linear form of disease develops as a thin band of sclerosis the sweat glands. There is atrophy of the epithelium with
that may run the entire length of extremities involving loss of rete pegs and increased melanin pigmentation.
underlying muscle, bones and joints. Subcutaneous fat disappears and the walls of the blood
A band made up of furrow with an elevated ridge on one vessels become sclerotic. In the periodontal ligament, there
side is often termed as a coup de sabre since it resembles is an increase in collagen and oxytalan fibers, as well as
the mark produced by the blow of saber. appearance of hyalinization or sclerosis of collagen with
diminution in the number of connective tissue cells is
Oral Manifestations usually found.
The tongue, soft palate, lips and larynx are commonly Management
involved. These are characterized by mild edema, which
D-penicillamine, a drug has shown promise in the
is followed by atrophy and induration of mucosal and
management by decreasing both, the skin thickening and
muscular tissue.
organ involvement by interference with cross linking of
Microstomia: The lips become thin, rigid and partially collagen and immunosuppression.
fixed, producing microstomia and the oral aperture narrows
considerably. Skin folds are lost around the mouth. Points to Remember
Systemic sclerosis, Hidebound disease
Tobacco pouch mouth: It can be seen periorally where
• Progressive systemic sclerosis: Indurated edema
furrow rows radiate from the atrophic vermilion borders
of skin, neuralgia and paresthesia, Raynaud’s
(purse string appearance), creating the so called tobacco
phenomenon, Hyperpigmentation, telangiectasia,
pouch mouth.
subcutaneous calcification may
Mouse species: Nasal alae become atrophied resulting in • Localized form (circumscribed or morphea):
pinched appearance of the nose called mouse species. Violaceous patches on the skin, hypo or hyper
pigmented areas
Tongue: Tongue can become hard and rigid, losing its
• Localized form (linear): Thin band of sclerosis, coup
mobility and papillary pattern, making speaking and
de sabre since it resembles the mark
swallowing difficult. The color of tongue changes to a livid
• O ral manifestations: Mild edema, Microstomia,
appearance. In the end stages, the tongue lays as a stiff,
Tobacco pouch mouth purse string appearance,
reduced body in the floor of mouth. The lingual frenum,
Mouse species, tongue can become hard, pseudo-
which usually reflects the first oral change, shortens,
ankylosis, diffuse widening of periodontal ligament
becomes tendinous and finally disappears. Involvement of
space
esophagus causes dysphagia.
• Histopathological features: Thickening and hyalini-
Involvement of soft tissues around the TMJ leads
zation, loss of dermal appendage, atrophy of the
to restricted movement of mandible, causing a pseudo-
epithelium, loss of rete pegs and increased melanin
ankylosis. When the facial tissues and muscles of
pigmentation, increase in collagen and oxytalan fibers
mastication are involved the pressure exerted will cause
• Management: D-penicillamine.
resorption of mandible at the attachment of masseter
Skin Disorders
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KAWASAKI DISEASE
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dermoscopic criteria for the diagnosis of psoriasis,
45. Said S, Golitz L Vesiculobullous eruptions of the oral cavity:
dermatitis, lichen planus and pityriasis rosea. Br J Dermatol.
Otolaryngol Clin North Am. 2011;44(1):133-60.
2012;166(6):1198-205.
46. Serrano_Martinez MC, Bagan JV, Silvestre FJ, et al. Oral
30. Leao JC, Batista V, Guimaraes PB, et al. Cowden syndrome
affecting the mouth, gastrointestinal and central nervous lesion in recessive dystrophic epidermolysis bullosa. Oral
system, a case report and review of literature. Oral Surg Oral Dis. 2003;9:264-8.
Med Oral Pathol Oral Radiol Endod. 2005;99:569-72. 47. Siegal MA, Anhalt GJ. Direct immunofluroscence of
31. Martelli H, Mourao-Pereira S, Martinis-Rocha T, et al. White detached gingival epithelium for diagnosis of cicatricial
sponge nevus: a report of three generation family. Oral Surg pemphigoid report of five cases. Oral Surg Oral Med Oral
Oral Med Oral Pathol Oral Radiol Endod. 2007;103:43-47. Pathol. 1993;75:296-302.
32. Mignogna MD, Lo Muzio L, Mignogna, et al. Oral 48. Smith CH. Barker JNWN: Psoriasis and its management: Br
pemphigus long term behavior and clinical response to Med J. 2006;333:380-4.
treatment with deflazacort in sixteen cases. J Oral Pathol 49. Stabfird TW, Peterson J, Machen RL. CREST syndrome
Med. 2000;29:145-52. and periodontal surgery: a case report: J Periodontol.
33. Mognogna MD, LoMuzio L, Ruocco V, et al. Early diagnosis 1999;70:536-541.
of multiple hamartomas and neoplasia syndrome (Cowden 50. Westerman AM, Entius MM, de Baar E, et al. Peutz Jeghers
disease). The role of dentist: Oral Surg Oral Med Oral Pathol syndrome: 78 years follow up of the original family: Lancet:
Oral Radiol Endod. 1995;79:295-9. 1999;353:1211-5.
Skin Disorders
51. Williams DM. Vesiculobullous mucocutaneous disease: 53. Younai FS, PheLan JA. Oral mucositis with features of
benign mucous membrane and bullous pemphigoid. J oral psoriasis a report of case and review of literature. Oral Surg
Pathol Med. 1990;19:16-23. Oral Med Oral Pathol Oral Radiol Endod. 1997;84:61-7.
52. Williams PM, Conklin RJ. Erythema multiforme: a review 54. Zawar V Giant pityriasis rosea. Indian J Dermatol. 765
and contrast from Stevens-Johnson syndrome/toxic epider- 2010;55(2):192-4.
mal. Dent Clin North Am. 2005;49:67-76.
1. ‘Bull’s eye’–concentric ring like appearance seen in: 6. Hyperelasticity of skin , ‘rubber man’ refers to:
a. Psoriasis a. Ehlers-Danlos syndrome
b. Erythema multiforme b. Bloch-Sulzberger syndrome
c. Porokeratosis c. Duhring-Brocq disease
d. Ehlers-Danlos syndrome d. Hailey-Hailey syndrome
2. Nikolsky’s sign is the characteristic feature of: 7. Which one of the following drugs worsen the con dition
a. Pemphigus b. Psoriasis of psoriasis:
c. Porokeratosis d. None of the above a. Antimalarials b. Beta blocker
3. Tzanck cells found in: c. Lithium d. All of the above
a. Porokeratosis b. Erythema multiforme
8. Test tube appearance of rete pegs seen in:
c. Pemphigus d. Psoriasis
a. Pemphigus b. Psoriasis
4. Hailey-Hailey disease refers to: c. Porokeratosis d. Both b and c
a. Familial benign chronic pemphigus
9. ‘Basket-weave’ appearance histologic feature seen in:
b. Bullous pemphigoid
c. Epidermolysis bullosa a. White sponge nevus
d. Pityriasis rosea b. Warty dyskeratoma
c. Darier’s disease
5. ‘Herald spot’ is the prodormal features of:
d. Both a and b
a. Incontinentia pigmenti
b. Acanthosis nigricans 10. Auspitz’s sign seen in:
c. Dermatitis herpetiformis a. Psoriasis b. Pemphigus
d. Pityriasis rosea c. Darier’s disease d. Both a and b
30 Allergic and Immunologic
Diseases of Oral Cavity
Chapter Outline Chapter Outline
Â Introduction/Overview Â Apthous stomatitis (Recurrent aphthous ulcers (RAUs) or

Â Hypersensitivity reaction canker sores)
Â Wegner’s granulomatosis Â Behçet’s syndrome
Â Sarcoidosis Â Transient lingual papillitis
Â Drug allergy Â Perioral dermatitis
Â Allergic contact stomatitis Â Reiter’s syndrome
Â Secondary vaccinia Â Lichenoid contact stomatitis/Lichnoid tissue reaction
Â Angioedema Â Chronic ulcerative stomatitis
Â Crohn’s disease
INTRODUCTION/OVERVIEW Clinically, it is difficult to distinguish between the

four types of hypersensitivities as they do not necessarily
The word ‘allergy’ was first used by von Pirquet, to denote occur in isolation from each other. Types I, II, and III
both host-protective and potentially host-injurious immune hypersensitivities are mediated by Antigen-antibodies
responses. Word ‘hypersensitivity’ was first described reaction, whereas, Type IV is mediated cell mediated
as denoting the status of a mammalian organism after response (especially T cells and macrophages).
exposure to an infectious agent.
Allergic reactions are a very common and important set
Types of Immune Reactions
of symptoms for clinician to learn to recognize and treat.
Allergy is a complex disease and allergic patients present • Type I reactions (Immediate hypersensitivity)
symptoms of varying severity as well as symptoms from • Type II reactions (Complement-mediated cytotoxicity)
different organs, such as eyes, nose, lungs, skin and the • Type III reactions
gastrointestinal tract. • Type IV reactions (Delayed hypersensitivity).
HYPERSENSITIVITY REACTION Types of Immune Reactions (Gell and

Coombs’ Classification)
A hypersensitivity reaction refers to a state of altered
reactivity in which the body mounts an amplified immune Type I reactions (Immediate hypersensitivity)—these are
response to a substance. In 1963, Gell and Coombs’ mostly IgE-mediated and their rapid onset, typically within
classified hypersensitivity reactions into four different minutes of exposure to antigen, is characteristic.
groups (Types I, II, III, and IV) which depended upon the Type II reactions (Complement-mediated cytotoxicity)
severity and latency of a reaction (Table 30.1). result from antibody binding to membrane-bound Ag
Allergic and Immunologic Diseases of Oral Cavity
Table 30.1 Gell and Coombs’ hypersensitivity reactions

Gell and coombs classification of hypersensitivity reaction
Reaction Description Antibody involved Time of onset 767
Type I Immediate hypersensitivity (e.g. Allergy) IgE 1–20 min
Type II Antibody to soluble antigen IgG/IgM --
(e.g. Hemolytic anemia)
Type III Antibody to soluble antigen immune complexes IgG 7–10 hrs
(e.g. serum sickness)
Type IV Delayed-type hypersensitivity --- 1–3 days
Table 30.2 Various methods for detection of type of hypersensitivity reaction

Type Description Method of detection
I An immediate reaction that can result in an anaphylactic Provocation test. Skin test, IgE RAST
reaction
II Cytotoxic reaction mediated by IgM and IgG antibody IgG serum test
response to host tissue
III IgG and IgM antibodies form immune complexes with IgG serum test
antigens in the blood
IV Delayed reaction that is mediated by memory T cells Skin test
resulting in complement-mediated cytotoxicity or Type I hypersensitivity reactions are traditionally

opsonization/inflammation. This class of reactions may be recognized through provocation testing or immediate-type
typified by hemolytic anemia and certain drug reactions. skin testing (Table 30.2).
Type III reactions occur when antibody binds to soluble Provocation testing involves a masked topical challenge
allergen to form immune complexes, which can cause any of certain antigens followed by observation of the patient’s
of several kinds of immune complex disease. Type III dermal response. Clinically, the provocation testing can
reactions are the result of deposition of circulating antigen- pose a danger since some antigens may result in a severe
antibody complexes in tissues, as opposed to the binding of anaphylactic reaction.
antibodies to antigens which are an integral part of a target Skin testing is an alternative method which may include
cell membrane. skin pricks or patches to determine hypersensitivity.
Type IV reactions (Delayed hypersensitivity)— Both the prick test and scratch test involves pricking
delayed-type hypersensitivity (DTH) is a synonym for the skin with a needle or pin containing a small amount of
CMI, and signifies to the slower development of such the antigen.
reactions compared with antibody-mediated reactions. A A patch test is conducted by applying a patch that
reaction can typically take 12 or more hours to develop. contains known antigens to the skin. If there is a visual
Type IV responses are dependent on T-cell interactions, reddening or swelling at the prick or patch site, the patient
which recruit other cells to the site of exposure. is considered allergic to that antigen.
Methods to Identify Hypersensitivity WEGNER’S GRANULOMATOSIS

Identifying the allergy or hypersensitivity is very important It is a disease of unknown etiology which basically
to prevent untoward incident. There are various methods to involves the vascular, renal and respiratory systems. It is
detect the existence and types of hypersensitivity. a granulomatous involvement of blood vessels resulting in
necrosis of tissue. It is generally thought that the disease is

aberrant hypersensitivity reaction to an unknown antigen.
768 Types of Wegner’s Granulomatosis

• G eneralized: It involve supper respiratory tract,
pulmonary and renal lesions.
• Localized: It affects oral and nasal cavity and the
lungs.
Clinical Features
Age and sex distribution: It usually occurs in 4th or 5th
decade of life, with slight predilection for males.
Symptoms: The most common symptom of Wegner’s
Figure 30.1 Wegener’s granulomatosis showing ulceration in
granulomatosis is nasal stuffiness with chronic discharge,
the palate
which is sometimes bloody. Patient soon develops cough,
hemoptysis, fever and joint pains. There is also presence
Histopathological Findings
of rhinitis, sinusitis and otitis or ocular symptoms. There
are also nonspecific symptoms of malaise, arthralgias and It reveals acute and chronic inflammatory cells, with areas
weight loss. Hemorrhagic or vesicular skin lesions are also of multinucleated giant cells.
Demonstration of neutrophils, cytoplasmic auto
commonly present.
antibodies in serum has very recently showed great
Glomerulonephritis, which develops ultimately to
promise for the immuno-diagnosis of this disease. Involved
uremia and terminal renal failure.
vessels demonstrate transmural inflammation with heavy
Oral Manifestations neutrophilic infiltration, necrosis and nuclear dust.
The disease usually starts with tumor like vegetations in The oral epithelium may demonstrate pseudoepitheli-
mouth and nose. oumatous hyperplasia and subepithelial abscess.
Inflammatory process starts in the interdental papilla,
spreading rapidly in to the periodontium. The lesion
Diagnosis
undergoes necrosis with formation of large perforating It should be suspected on the basis of clinical symptom
ulceration. and signs. Definitive diagnosis is made by histological
Strawberry gingivitis—involvement of gingiva is the examination.
most common manifestation; which is characterized by
ulceration, friable granular lesions or simple enlargement Management
of gingiva. Inflamed, hyperplastic appearing and hemorr- First line of treatment is oral prednisolone and
hagic gingiva may be found. Strawberry gingivitis is the cyclophosphamide. Trimethoprim-sulfamethoxazole has
characteristic feature of it.
also been used successfully in Wegner granulomatosis.
Oral lesions typically include ulceration of the palate by
extension of nose lesions and destruction of nasal septum,
Points to Remember
poorly healing extraction sites or oroantral fistule. There
may be perforation of palate (Fig. 30.1). Involvement of blood vessels, is nasal stuffiness with
There may be loosening of teeth, spontaneous chronic discharge, hemoptysis, fever, nonspecific sym-
exfoliation of teeth diffuse ulcerative stomatitis, post ptoms, malaise, arthralgias, glomerulonephritis, which
extraction poor healing, cranial nerve palsies and parotid develops, necrosis with formation of large perforating
swelling. There are often signs of alveolar bone loss. ulceration, strawberry gingivitis, oroantral fistule,
anemia, leukocytosis, elevated sedimentation rate,
Laboratory Findings acute and chronic inflammatory cells, multinucleated
It includes anemia, leukocytosis, elevated sedimentation giant cells, pseudoepitheliomatous hyperplasia, oral
rate and hyperglobulinemia. Hematuria with finding of prednisolone and cyclophosphamide. Trimethoprim-
albumin, casts and leukocytes in urine. sulfamethoxazole.
SARCOIDOSIS
It is also called Boeck’s sarcoid, Besnier-Boeck-
Schaumann’s disease. This term is given by Boeck given 769
the name of sarcoidosis (meaning flesh like condition).
It is a disease of unknown etiology. It is a multisystem
granulomatous disease. It is characterized by depression of
delayed type of hypersensitivity, suggesting an impaired
cell-mediated immunity and raised or abnormal serum
immunoglobulin, suggesting lympho-proliferation.
There are two types of antigen which are involved,
i.e. infectious antigen (mycobacterium, propionibacteria,
Epstein-Barr virus, HHV virus) environmental factors like
wood dust, pollen, clay and mold.
Clinical Features Figure 30.2 Sarcoidosis showing circumscribed collection of

histiocytes, lymphocytes
Age and sex distribution: It has got slight predilection
of females with bimodal age incidence years of age and
second between 45 to 65 years of age. It shows more On the palate and buccal mucosa, it is described as bleb
prevalence in blacks. like, containing a clear yellowish fluid or as solid nodules.
Location: Lesions are most common in lungs, skin, lymph Sign and symptoms: There is increased incidence of
nodes, salivary glands, spleen and bones. dental caries. There is ulceration of buccal mucosa due to
Symptoms: Mild malaise, fever, weight loss, fatigue and salivary insufficiency. It also appears to produce diffuse
cough can be the chief features of the disease. destruction of the bone.
Signs: It presents most frequently with hilar lymphadeno- Histopathological Features

pathy, pulmonary infiltration and skin and eye lesions.
It resembles proliferative noncaseating nodules of tuber-
Lupus pernio: These are chronic violaceous indurated culosis. Nests of epithelioid cells with multinucleated giant
lesion seen on nose ears lips and face. cells are also present (Fig. 30.2).
Fibrinoid necrosis and hyalinization are occasionally
Erythema nodosum: Scattered, nonspecific tender
found in the center of granuloma.
erythematous nodules seen on lower leg called erythema
The giant cells may contain ‘Schaumann bodies
nodosum.
(degenerated lysomes)’, which are concentrically or
Ocular lesion: There is may be keratoconjunctivitis sicca eccentrically laminated, calcified bodies.
of lesion. ‘Asteroid bodies (entrapped fragment of collagen)’ are
delicate spider like radiating structures that are sometimes
Syndrome associated: It is associated with Lofgren’s
seen in the macrophages of sarcoid granuloma.
syndrome (erythema nodosum, bilateral hilar lympha-
Hamazaki-Wesenberg bodies (large lysosomes) are
denopathy and arthralgias) and Heerfordt’s syndrome
small yellow brown structures in seen in subcapsular sinus.
(parotid enlargement, anterior uveitis of eye, facial
paralysis and fever). Diagnosis
Oral Manifestations Kveim-Siltzbach test: It is an intracutaneous test for the
diagnosis of sarcoidosis ‘Kveim-Sitzbach test’ is positive
Location: It is rare in oral cavity, but cases are reported on
in some cases. In this test, intradermal injection of a saline
lip, palate and buccal mucosa.
suspension of known human sarcoid tissue used as an
Appearance: It appears as small papular nodules or antigen is given to a patient suspected to have sarcoidosis.
plaques or resembles herpetic lesions or fever blister. One month after the injection, any palpable nodule is
excised and examined histologically for evidence of a

Drugs Involved in IgE Mediated Allergies
sarcoid reaction, or epithelial tubercle.
• Foreign proteins
770 • Immunoglobulin in preparation
Management
• Beta-Lactam antibiotics
Asymptomatic patient requires no treatment but in cases • Pyrazolones
where symptoms are present, corticosteroids can be given. • Quinolones
Drugs Involved in Pseudoallergic Reactions
Points to Remember • Radioisotopes (contrast media)
Boeck’s sarcoid’, ‘Besnier-Boeck-Schaumann’s disease, • Plasma expanders
lungs, skin, lymph nodes, mild malaise, fever, weight loss, • NSAID: Acetylsation, diclofenac, mefenamic acid,
fatigue hilar lymphadenopathy, Lupus pernio, erythema ibuprofen
nodosum, Lofgren’s syndrome, keratoconjunctivitis • Vancomycin
sicca, palate and buccal mucosa, it is described as bleb • Quinolones.
like, incidence of dental caries. Proliferative noncaseating
nodules of tuberculosis, multinucleated giant cells, Risk Factors
fibrinoid necrosis, Schaumann bodies (degenerated
There are factors which can be responsible for an increased
lysomes), asteroid bodies (entrapped fragment of collagen)’
risk of developing a drug allergy. These include age,
Hamazaki-Wesenberg bodies (large lysosomes), Kveim-
gender, genetic polymorphisms, certain viral infections
Siltzbach test, corticosteroids.
and drug-related factors (e.g. frequency of exposure, route
of administration, molecular weight) (Table 30.3).
DRUG ALLERGY Clinical Features

It is also called drug idiosyncrasy, ‘drug sensitivity’ and Sign: It is characterized by inflammation, ulceration and
stomatitis or dermatitis medicamentosa. vesicle formation with arthralgia, fever and lymphad-
The term “drug hypersensitivity” refers to objectively enopathy.
reproducible symptoms or signs initiated by exposure to The skin lesion is often of erythematous type, as in
a drug at a dose normally tolerated by nonhypersensitive erythema multiforme or they may be urticarial in nature
persons. (Fig. 30.3).
Drug allergy refers to immunologically mediated Fixed drug reactions may occur in those who are
drug hypersensitivity reactions. These may be either administrated on repeated occasions, a drug to which they
immunoglobulin E (IgE)–mediated (immediate) or non– are sensitive. It consists of appearance of same reaction at
IgE-mediated (delayed) hypersensitivity reactions. They the same site each time.
may cause acute multiple ulcers and vesicles of oral mucosa
or lichenoid reaction. It includes a variety of sensitive Table 30.3 Risk factors for the development of drug
reactions. Some patients have greater susceptibility to allergy
drugs and manifest reactions more readily than others. Patient-related factors:
Drugs which can most commonly cause drug reactions • Age: Young/middle-aged adults > infants/elderly
are aminopyrine, barbiturates, gold, bromide, penicillin, • Gender: Women > men
streptomycin, etc. • Genetic polymorphisms
The term Pseudoallergic reactions is referred to • Viral infections: HIV, herpes viruses
• Previous reaction to the drug
the nonimmune-mediated hypersensitivities reactions
to drugs. These are as frequent as true Ig E-mediated Drug-related factors:
reactions, are a pathogenetically poorly defined problem. • H
igh molecular weight compounds and hapten-forming
Most of these reactions resemble the clinical features drugs are more immunogenic
of milder forms of immediate, Ig E-mediated reactions; • Route: Topical > intravenous/intramuscular > oral
• Dose: Frequent/prolonged > single dose
Pseudoallergic reactions can be elicited by many drugs.
appear and angioneurotic edema is seen. Ulceration and

necrosis of gingiva often resemble ANUG.

There is nonspecific sub acute mucositis which contain
lymphocytes with mixed inflammatory cellular infiltrate of
eosinophils and neutrophils.
Vacuolar changes of basal cell layer and necrotic epithelial
cells are seen.
Management
The signs and symptoms of drug allergy regress with
discontinuing of the causative drug. The acute signs may
be relived by administration of antihistaminic drugs or
Figure 30.3 Erythematous fixed drug reaction seen on the cortisone.
hand of patient (Courtesy: Dr Sanjay Pincha)
Points to Remember
Drug idiosyncrasy, drug sensitivity, stomatitis or
dermatitis medicamentosa, erythematous type, skin
lesion, fixed drug reactions, same reaction at the same
site each time, anaphylactic stomatitis, purpuric spots
appear and angioneurotic edema, lymphocytes with
mixed inflammatory cellular infiltrate of eosinophils
and neutrophils, necrotic epithelial, administration of
antihistaminic drugs.
ALLERGIC CONTACT STOMATITIS

It is caused by delayed type of hypersensitivity reaction
to topical antigen. On the skin, it is referred as ‘dermatitis
venenata’ and oral lesions are referred as ‘stomatitis
venenata’.
Figure 30.4 Extensive erosion of lip seen in the drug allergy
(Courtesy: Dr Amit Parate, Government Dental, College Causes
Nagpur, Maharashtra, India)
It is cause by poison ivy, leather, rubber, nickel, medication
or other chemicals. Contact allergy to dental amalgam is
Oral Manifestations caused by mercury, which is released during condensation.
∙ Anaphylactic stomatitis: The oral lesions are diffuse Dental and cosmetic preparation like dentifrices,
in distribution and vary in appearance from multiple mouthwashes, denture powder, lip stick, cough drop and
areas of erythema to extensive areas of erosion or chewing gums.
ulceration (Fig. 30.4). Dental materials like vulcanite, acrylic, metal alloy
∙ Location: It is most commonly seen in labial mucosa. base.
In the early stages of reaction, vesicle or even bullae Dental therapeutic agents like alcohol, antibiotics,
may be found on the mucosa. Occasionally purpuric spots chloroform, iodide, phenol, procaine and volatile oils.
Reason Why Oral Mucosa Less Sensitive to Contact

Allergy
772 • Brief period of contact
• Dilution of antigen by saliva
• Due to limited keratinization haptens binding is diffi-
cult and high vascular tendency remove antigen quickly
• Allergen may not be recognized.
Clinical Features
Sign and symptoms: There are typically itching erythe-
matous areas with superficial vesicle formation, directly at
the site where allergen contacts skin. The skin may become
thickened and dry. Figure 30.5 Stomatitis venenata showing hyperkeratotic
After the rupture of vesicle, erosion may become white lesion
extensive and if secondary infection occurs, the lesion may
be serious. Burning is a more common complaint rather The superficial lamina propria demonstrate heavy
than itching of skin. inflammatory cell infiltrate which consist of lymphocytes
Localized area of erythema, edema and vesiculation in which may be intermixed with plasma cells, histiocytes or
specific areas of skin or mucosa whenever specific allergen eosinophils.
is administered.
Management
Oral Manifestations It involves the removal of allergen and application of
Allergy to cinnamon oil or formalin present in tooth paste, topical corticosteroids.
ice-cream, candy, and soft drinks appears clinically as
swelling, cracking and fissuring of lips, perioral desquam- Points to Remember
ation and edema, angular cheilitis, swelling of gingiva and Dermatitis venenata, stomatitis venenata, dental
oral ulcerations. amalgam, itching erythematous areas, vesicle, erosion
Plasma cell gingivitis: Another oral manifestation may become extensive, localized area of erythema,
is plasma cell gingivitis, which is characterized by edema, allergy to cinnamon oil or formalin, plasma
erythematous edematous attached gingiva accompanied by cell gingivitis, acute contact stomatitis, chronic contact
cheilitis and glossitis. stomatitis, patch test, acanthotic with elongated rete peg,
Acute contact stomatitis: Burning with mild or barely thinning of suprapapillary plates, superficial lamina
visible redness to brilliantly erythematous lesion with or propria demonstrate heavy inflammatory cell infiltrate
without edema of lymphocytes, removal of allergen.
Chronic contact stomatitis: It may be erythematous white
and hyperkeratotic (Fig. 30.5). SECONDARY VACCINIA
Diagnosis It is also called vaccinia autonoculata.
Undesired skin or mucosal lesion, after small pox
Patch test—suspected allergen is placed on normal non-hairy vaccination, is caused by transfer of the contents of
skin, i.e. on upper portion of back. It remains in contact with vaccination pustule to other parts of the body. It is followed
skin for 48 hours. Then the patch is removed and after 2 to 4 by formation of secondary lesions usually with weaker
hours, the area is examined for persistent erythema. reaction than the one seen in case of primary inoculation.
Histopathological Features Secondary vaccinia may develop at the site of scratching
possibly in already existing epithelial defect.
Epithelium in contact stomatitis form cinnamon flavoring
is acanthotic with elongated rete pegs and thinning of Location: Eyes, ears and areas of lips and tongue are
suprapapillary plates. possible sites. In this area, a patch develops that becomes
vesicular than pustular. After the development of crust,

there is repair with scar formation. Other ulcerative lesions
such as primary lesion of syphilis, tuberculosis, aphthae
of the major type should be differentiated. The history is 773
important as secondary vaccinia appear about 5 to 7 days
after inoculation.
Points to Remember
Vaccinia autonoculata, site of scratching possibly,
undesired skin or mucosal lesion, after small pox
vaccination.
ANGIOEDEMA
It is also called Angioneurotic edema, Quincke’s edema, Figure 30.6 Angioedema involving lip
and giant urticaria. It is also called quincke’s edema after
the name of clinician who related to changes in alteration
in vascular permeability. The term angioneruotic edema is
given as patient complaint of chocking sensation and was cularly involving the face, around the lips, chin and eyes,
labeled neurotic. the tongue and sometimes, the hands and feet (Fig. 30.6).
It is common form of edema occurring in both Sign and symptoms: Parotid gland may be affected in
hereditary and nonhereditary forms. It is one form of some cases. The eyes may be swollen, shut and lips may
acute anaphylactic reaction representing response allied to be extremely puffy. Symptoms may appear suddenly
urticaria, allergic rhinitis and asthma. sometimes may present in morning. A feeling of tenderness
or an itching or prickly sensation sometimes precedes the
Mechanism urticarial swelling.
The mechanism of development of swelling is due to
Other feature: The skin is of normal color and/or slightly
vasodilation brought about by the release of histamine like
pink. The condition usually last for 24 to 36 hours, although
substances with subsequent transudation of plasma.
some cases persist for several days. The hereditary forms
Causes are more dangerous because there is visceral involvement.
Vomiting and abdominal pain may occur and especially,
It appears closely related to general urticaria. It occurs
dangerous edema of glottis can result in death through
most commonly due to food allergy. In some cases, it is
suffocation.
thought that some drugs, endocrine disturbances or focal
infection play an important etiologic role. Management
Clinical Features When etiological agent such as food can be discovered, its
elimination from diet will prevent recurrent attacks.
Age and sex distribution: It affects both sexes equally,
Antihistaminic drugs (50 mg to 75 mg diphenylhy-
but it is infrequent in children while some cases originate
dramine hydrochloride) can give prompt relief.
at puberty.
Location: Most often, the face and lips are involved, but Points to Remember
sometimes the tongue also becomes swollen. Angioneurotic edema’, Quincke’s edema, general
Appearance: Edema may develop gradually in a matter urticaria, the face and lips edema, smooth, diffuse
of hours, but can also progress in minutes. It typically edematous swelling, parotid gland may be affected, lips
manifests as a smooth, diffuse edematous swelling, parti- may be extremely puffy, antihistaminic drugs.
APHTHOUS STOMATITIS (RECURRENT pathogenesis of apthous ulcer, i.e. oral streptococci and
Helicobacter pylori.
APHTHOUS ULCERS (RAUs) OR
774 CANKER SORES)
Etiology
It is a common disease characterized by development of
• Bacterial infection
painful, recurrent, solitary or multiple ulcerations of the
• Immunological abnormalities
oral mucosa, with no other signs of any other disease.
• Iron deficiency or folic acid deficiency
• Hereditary
Classification
• Hematological deficiency.
• M
inor aphthae: It is also called ‘canker sores’ or
Precipitating Factors
‘mickulicz’s apthae’ in which the ulcers are less than
1 cm in diameter and heal without scar. • Trauma
• Major aphthae: It is called ‘Sutton’s disease’ or ‘peri- • Endocrine conditions
adenitis mucosa necrotica recurrent’ and the ulcers are • Psychic factors
over 1 cm in diameter and heal with scarring. • Cessation of smoking
• Herpetiform ulcers: Recurrent crops of dozens of • Allergic factor.
small ulcers throughout the oral mucosa.
• Recurrent ulcers associated with Behçet’s syndrome. Precipitating Factors
• Simple aphthous: These are lesion which occur in Trauma: Local trauma including self-inflicted bites, oral
patient with few lesion and heal in 1 to 2 weeks and surgical procedures, tooth brushing, needle injections and
recur infrequently dental trauma.
• Complex aphthous: These are multiple and constant
ulceration occur that often develop as older lesion Endocrine conditions: There is some relation between
resolve. occurrence of aphthous ulcer and pregnancy, menstruation
and menopause. There is remission of ulcers during
pregnancy. Incidences of aphthae are greatest during
Etiology
menstruation. Ulcerations are maximum during
Bacterial infection: A pleomorphic transitional L-form of postovulation period.
a-hemolytic Streptococcus and Streptococcus sanguis has
been implicated as the causative agent of the disease. Psychic factors: Acute psychological problems appear
many times, to precipitate the attacks of the disease.
Immunological abnormalities: IgG and IgM binding of Anxiety can also precipitate the attack.
the epithelial cells of the spinous layer of oral mucosa is
seen in patients suffering from recurrent apthous ulcer. Cessation of smoking increases the frequency and severity
of RAS.
Iron deficiency or folic acid deficiency: Small percentage
of patients with recurrent aphthae have certain nutritional Allergic factor: Patients may have a history of asthma,
deficiency. Presence of a deficiency allows the expression hay fever and food or drug allergy.
of an unrelated, underlying tendency to ulceration.
Clinical Features
Hereditary: Increased susceptibility to RAS is seen
Age and sex distribution: It usually occurs between
among the children of RAS positive parents. Specific
second and third decades of life. It is common in women
HLA antigen has been identified in RAS patients. There is
than men.
familial tendency for the occurrence of the disease.
Hematological deficiency, serum iron or Vitamin B12 Precipitating factor: It may be precipitated by minor
deficiency, secondary malabsorption syndrome such as trauma, menstruation, URTI or contact with certain foods.
celiac disease.
Location: It occurs most commonly on buccal and labial
Micro organisms associated with aphthous ulcers: mucosa, buccal and lingual sulci, tongue, soft palate,
Several micro organisms have been implicated in the pharynx and gingiva.
Appearance: It begins with prodormal burning for 24 to

48 hours, before the ulcer appears. It begins as a single or
multiple superficial erosion covered by grey membrane.
Localized areas of erythema develop and within hours 775
small white papules form, ulcerate and gradually enlarge
over next 48 to 72 hours.
Sign and symptom: Lesions are round, symmetric and
shallow but no tissue tags are present from the ruptured
vesicles. The lesion is typically very painful so, it
commonly interferes with eating for several days. Multiple
lesions are present but the number and size are frequently
varied. Most patients have between 2 to 6 lesions at each
episode and experience several episodes a year. The ulcers
themselves generally persist for 7 to 14 days. Figure 30.8 Major aphthous ulcer seen in soft palate region
(Courtesy: Dr Amit Parate, Lecturer, Department of Oral
Minor aphthae: Size is 0.3 to 1 cm, heal without scarring, Medicine, GDC, Nagpur, Maharashtra, India)
within 10 to 14 days (Fig. 30.7).
Major aphthae (Fig. 30.8): Develop deep lesions, larger
than 1 cm and may reach up to 5 cms in diameter. They
interfere with speech and eating. Large portions may be
covered with deep painful ulcers. The lesions heal slowly
and leave scars, which result in decreased mobility of uvula
and tongue and destruction of portions of oral mucosa.
Herpetiform ulcers: Multiple small shallow ulcers
often up to 100 in number. Found on any intraoral
mucosal surface. Begin as small pinhead size erosions
that gradually enlarge and coalesce. Lesions are more
painful than would be suspected by their size. Present
continuously for one to three years, with relatively short
remission. Patient gets relief immediately with 2 percent
tetracycline mouthwash. Figure 30.9 Aphthous ulcer showing antischkow cell (Courtesy:
Dr Sangamesh Halawar, Reader, Department of Oral Pathology,
Fibrinopurulent membrane covers the ulcerated area.
Occasional superficial colonies of microorganisms may
be present in this membrane. Intense inflammatory cell
infiltrate is present in connective tissue, beneath the ulcer,
with considerable necrosis of tissue near the surface of the
lesion.
Neutrophils are predominant immediately below the
ulcer but lymphocyte prevailing adjacent to this. Epithelial
proliferation is along the margins’ of the lesion.
Anitschkow cells—consists of cells with elongated
nuclei, containing a linear bar of chromatin with radiating
processes of chromatin extending towards the nuclear
Figure 30.7 Aphthous ulcer presented as well defined lesion membrane (Fig. 30.9).
The epithelium at margin of the lesion demonstrates Etiology

spongiosis and numerous mononuclear cells in the
It is caused by immune complexes that lead to vasculitis
basilar one third. A band of lymphocytes intermixed with
of small and medium sized blood vessels. There may
776 Histiocytes is also seen in superficial connective tissue.
be inflammation of the epithelium caused by immuno-
Management competent T-lymphocytes and plasma cells.
Mild cases: Topical protective emollient base (Orabase). Clinical Features (Fig. 30.10)
Topical tetracycline mouth wash (250 mg per ml) use
The clinical spectrum includes oral and genital ulcerations,
four times daily for 5 to 7 days produces good response
uveitis, and vascular, neurological, articular, renal and
in nearly 70 percent of the patients. Topical corticosteroid
gastrointestinal manifestations.
preparation—topical corticosteroid triamcinolone aceto-
nide 3 to 4 times daily should be prescribed. Age and sex distribution: It begins between 10 to 45 years
of age, with a mean age of occurrence of 30 years. It is five
Severe cases: Fluocinolone gel, clobetasol cream or
to ten times more common in males.
beclomethasone spray. Injection of corticosteroid directly
in lesion in, combination of systemic administration of Recurring oral ulcers: It may be mild or may be deep,
cortisone. Chlortetracycline as mouth rinse to be flushed large scarring lesions and may appear anywhere on the oral
over the affected region, for at least 2 minutes provides and pharyngeal mucosa. They are painful lesions. They may
relief from pain. In some cases, dapsone or thalidomide can range from several millimeters to a centimeter in diameter.
be used. Interferon alpha, nicotine tablets and colchicine These ulcers have erythematous borders and are covered by
can be used, but it is under investigation. gray or yellow exudate.
Genital lesions: It include ulcers of scrotum and penis in
Points to Remember males and ulcers of labia in females. The genital ulcers are
small and painful in females.
Prodormal burning for 24 to 48 hours, lesions are round,
symmetric and shallow, very painful, minor aphthae, major Eye lesions: Consist of uvetitis, retinal internal edema
aphthae, herpetiform ulcers, multiple small shallow ulcers and vascular occultation, optic atrophy, conjunctivitis and
often up to 100 in number, fibrinopurulent membrane, keratitis.
Intense inflammatory cell infiltrate, neutrophils are pre-
dominant, anitschkow cells, topical protective emollient
base, topical corticosteroid triamcinolone acetonide.
BEHÇET’S SYNDROME
It is a disease of uncertain etiology that may resemble
an infectious origin. It is triad of recurring oral ulcers,
recurring genital ulcers and eye lesions. Mucocutanoues
lesions are hallmark of the syndrome. It is discovered by
Hulusi Behçet in 1937.
Behçet disease (BD) is a chronic, relapsing, multisys-
temic disorder characterized by mucocutaneous, ocular,
vascular and central nervous system manifestations.
Classification
• Mucocutaneous: Oral, genital and skin lesions.
• Arthritic: Arthritis, in addition to mucocutaneous
lesions.
• Neuro-ocular: Neurologic, ocular and mucocutaneous
lesions. Figure 30.10 Signs of Behçet’s syndrome
Skin lesions are generally small pustules or papules on

Points to Remember
the trunk or limbs and around the genital.
Recurring oral ulcers, ulcers of scrotum and penis in
Pathergy test: Cutaneous hypertrophy to intracutaneous males and ulcers of labia in female, uvetitis, retinal 777
injection or needle sticks (pathergy) with the finding of internal edema, skin lesions are generally small pustules
pustule forming 24 hours after needle puncture. or papules on the trunk, Pathergy test, Arthritis, CNS
Arthritis: Arthritis is common. Affected joint is red and involvement, thrombophlebitis, endothelial proli-
swollen. feration with vasculitis, leukocytoclastic vasculitis,
immunosuppressive drugs and systemic corticosteroids.
CNS involvement: It may occur and it may include
brain stem involvement of cranial nerve and neurologic
degeneration. TRANSIENT LINGUAL PAPILLITIS
Others: Thrombophlebitis, intestinal ulceration, venous It is also called lie bump, tongue torches.
thrombosis, renal and pulmonary disease. It may cause by local irritation, stress, gastrointestinal
disease, hormonal fluctuation, upper respiratory infection,
and topical hypersensitivity. There is involvement of
Diagnostic Criteria fungiform papillae of the tongue.
Recurrent oral ulcerations at least 3 times in one 12
month period, plus at least two of the following four Clinical Features
manifestations Age and sex distribution: It is more commonly seen in
Any two of the following: female in middle age group.
• Recurrent genital lesions. Localized pattern: This is seen on anterior portion of
• Eye lesions including uveitis or retinal vasculitis. dorsum surface of tongue. There is mild to moderate pain.
• Skin lesions including erythema nodosum, pseudo- There is enlargement of one or more fungiform papillae
folliculitis and papulo-pustular lesions. which are red with yellow and ulcerated cap.
• Positive pathergy test.
Generalized pattern (Fig. 30.11): In this involvement
of papillae is more on the tip and lateral portion of dorsal
Laboratory Findings
Hypergammaglobulinemia, leukocytosis with eosinophilic
and elevated ESR.
It has got similar appearance as that of aphthous stomatitis.
Endothelial proliferation with vasculitis is seen which is
called leukocytoclastic vasculitis.
Small blood vessels demonstrate intramural invasion by
neutrophils, extravasation of red blood cells and fibronoid
necrosis of the vessels wall.
Management
Patients with life threatening or slight threatening vasculitis
are managed with combination of immunosuppressive Figure 30.11 Transient lingual papillitis showing
drugs and systemic corticosteroids. erythematous papillae
surface. Papillae are sensitive erythematous, enlarged, with

surface erosion.
778 Papulokeratotic variant: It has got diffuse involvement.

Papillae are asymptomatic which appear as white elevated
papule. There is thickened parakeratotic cap present which
bear the name papulokeratotic type.
There are focal areas of exocytosis or ulceration. There
is proliferation of numerous small vascular channels with
inflammatory cell infiltrate.
In papulo-keratotic variant there is hyperparakeratosis
with ragged surface and bacterial colonization. Chronic
lymphocytes infiltrate present in superficial lamina propria.
Figure 30.12 Perioral dermatitis showing inflammation
Management
Topical corticosteroid, anesthetics and coating agent used
to reduce the pain or duration of pain. Histopathological Features
There is chronic lymphohistiocytic dermatitis which shows
Points to Remember spongiosis of hair follicles.
Lie bump, tongue torches, anterior portion of dorsum In some patient rosacea like pattern in which there is
surface of tongue, generalized pattern, papulokeratotic perifollicular granulomatosis inflammation (Fig. 30.12).
variant, white elevated papule, focal areas of exocytosis
or ulceration, hyperparakeratosis with ragged surface, Management
bacterial colonization, topical corticosteroid, anesthetics. Discontinuation of topical corticosteroids will results in
regression of the lesion.
PERIORAL DERMATITIS Oral tetracycline, topical metronidiazole or erythromy-
cin has also been successful in many patients.
It is inflammatory skin disease which involves circumoral
area.
It is cause by idiosyncratic response to exogenous Points to Remember
substance. The agents used are topical corticosteroid, tartar Vermilion border of upper and lower lip, pruritis, zone
control toothpaste, bubble gum, moisturizers, night creams erythema, chronic lymphohistiocytic dermatitis, rosacea
and cosmetic products. like pattern, perifollicular granulomatosis inflammation,
oral tetracycline, topical metronidiazole or erythromycin.
Clinical Features
Sex distribution: There is female dominance as they use
cosmetic more as compare to male. REITER’S SYNDROME
Location: It is located skin surrounding the vermilion Reiter’s syndrome (RS) is a disease of unknown etiology
border of upper and lower lip. There is spared skin between and is considered as an important complication of non-
immediately to the vermilion border. gonococcal urethritis and is often acquired sexually. It is
also called reactive arthritis.
Symptoms: Patient may complaint of pruritus. Classic Reiter’s syndrome is characterized by aseptic
Appearance: There is zone erythema without papules or inflammatory arthritis, urethritis or cervicitis and
pustules. conjunctivitis.
Cutaneous manifestations consist of a palmoplantar Location: It is seen on the buccal mucosa, lips and gingiva.
keratoderma, circinate balanitis or vulvitis, psoriasis-like skin The lesions appear as painless, red, slightly elevated areas,
lesions, and buccal ulcerations. RS was first described by sometimes granular or vesicular with a white circinate
Hans Reiter during the First World War though it had been border. 779
recognized since 1818. Classically, RS is characterized by
Appearance: The palatal lesions appear as small, bright red,
the triad of urethritis or cervicitis, conjunctivitis, and arthritis.
purpuric spots which darken and coalesce. Lesions on the
Triad of Reiter’s Syndrome tongue closely resemble geographic tongue. They may be
mistaken as recurrent aphthous ulcers.
∙ Urethritis or Arthritis.
∙ Conjunctivitis. Laboratory Findings
∙ Mucocutaneous lesions.
Usually, the diagnosis of RS is based on clinical features.
Etiology There are no definite diagnostic laboratory tests or
radiographic findings.
Reiter’s Syndrome (RS) is a genetically determined disease The elevated erythrocyte sedimentation rate (ESR)
and its association with HLAB27 has been suggested. and neutrophilic leucocytosis that suggested a bacterial
The RS is triggered by bacterial infection that enters via infection. All the laboratory investigations was negative.
mucosal surfaces usually, (but not always) associated with
human leukocyte antigen (HLA)- B27. It is may be due to Histopathological Features
pleuropnemonia like organism, variety of infectious agents
It consists of parakeratosis, acanthosis and polymorpho-
like bedsonia, mycoplasma, chlymadia, virus, etc.
nuclear leukocyte infiltration of epithelium, sometimes
It can be associated with staphylococci and in that case,
with microabscess formation. Connective tissue shows
it is called staphylococcal scalded skin syndrome.
lymphocytes and plasma cell infiltration.
Clinical Features
Management
Age and sex distribution: The RS has male preponderance
Spontaneous remission. It is treated by antibiotics and
and age range of between 15 to 35 years.
corticosteroids.
Appearance: The disease begins abruptly with diffuse
erythema and fever. Large flaccid bullae are formed which Points to Remember
contain a clear yellowish fluid. The bullae rupture very
Arthritis, urethritis or cervicitis and conjunctivitis, diffuse
easily leaving large areas of skin devoid of superficial
erythema and fever, large flaccid bullae, circinate balanitis,
epidermis. The urethral discharge is usually associated
crusted erosions, circumcised penis, buccal mucosa,
with an itching and burning sensation.
lips and gingiva, painless, red, slightly elevated small,
Arthritis: Arthritis is often bilateral, symmetrical and bright red, purpuric spots which darken and coalesce,
usually poly-articular. parakeratosis, acanthosis, polymorphonuclear leukocyte.
Conjunctivitis: Conjunctivitis is often so mild as to be
overlooked.
LICHENOID CONTACT STOMATITIS/
Skin lesion: The skin lesions consist of red or yellow
keratotic macules which eventually desquamate.
LICHENOID TISSUE REACTION
Additionally mucocutaneous findings include circinate These types of allergic reaction occur due to dental
balanitis in the uncircumcised penis and crusted erosions amalgam, gold restoration, copper, palladium, silver, zinc,
on the circumcised penis and on the scrotum may be etc.
observed. On patch testing many patients react to this agents and
lesion resolve after offending agents is removed. There is
Oral Manifestations lack of migration in this lesion
Oral lesions occur in less than 5 percent to about 50 percent Lichenoid tissue reaction (LTR) is characterized by
of the patients with the disease. epidermal basal cell damage which takes the form of
liquefaction degeneration or cell death either apoptosis or Role of Plasmacytoid dendritic cell-mediated type I
necrosis with an associated cascade of histological events interferon signaling is also postulated.
in epidermis and dermis. The term ‘lichenoid’ refers to Recent studies have suggested that T-lymphocytes
780 papular lesion of certain skin disorders of which lichen with helper phenotype may play an important role in the
planus is the prototype. pathogenesis of lichenoid tissue reactions (LTR).
LTR form are described in Table 30.5.
Etiology
Various factors may produce induce lichenoid tissue Clinical Features
reaction lesions such as, mechanical trauma, systemic Location: This is most commonly seen in posterior
drugs, contact sensitivity, infective agents including some buccal mucosa, ventral surface of lateral border of tongue.
viruses through cell mediated reaction (Table 30.4). Gingival cuffs adjacent to filling can also be affected.
Appearance: The affected area may be white or
Table 30.4 Most common allergen present in dental ma- erythematous with or without peripheral striae.
terials for specific oral disease. (Adapted from J Am Acad
Dermatol 10.1016/j.jaad.2007.04.017. Article in press) Sign and symptoms: Most of the patient does not have
Disease Allergies
symptoms but in some cases erosion can be seen.
Burning mouth syndrome Potassium dicyanoaurate Histopathological Features
Nickel sulfate hexahydrate
Gold sodium thiosulfate They exhibits many features of lichen planus with surface
Palladium chloride epithelium showing hyperkeratotic, atrophic and ulcerated.
Fragrance mix
Lichenoid tissue reaction Potassium dicyanoaurate Table 30.5 Classification of LTR/IFD (Adapted from Jour-
Fragrance mix nal of Investigative Dermatology (2009); 129: 1088–1099)
Gold sodium thiosulfate Lymphocyte rich LTR/IFD
Nickel sulfate hexahydrate
• Autoimmune connective tissue disease
Balsam of Peru
– Discoid lupus erythermatosus (LE)
Cheilitis Fragrance mix • Fixed drug eruption
Gold sodium thiosulfate • Keratosis lichenoides chronica
Dodecyl gallate • Lichen planus
Caine mix III • Lichen striatus
Benzoic acid • Lichenoid drug reaction
Stomatitis Mercury • Lichenoid and granulomatous dermatitis
Balsam of Peru • Graft versus host disease
Gold sodium thiosulfate • Mycosis fungoides
Nickel sulfate hexahydrate Lymphocyte-poor LTR/IFD
Dodecyl gallate
• Acute graft versus host skin disease
Gingivitis Potassium dicyanoaurate • Autoimmune connective tissue skin disease
Nickel sulfate hexahydrate – LE-acute cutaneous LE/Subacute cutaneous LE
Palladium chloride – Dermatomyositis
Beryllium sulfate tetrahydrate – Mixed connective tissue disease
Gold sodium thiosulfate • Erythema multiforme-
Orofacial granulomatosis Nickel sulfate hexahydrate – Erythema multiforme major (Stevens-Johnsons syn-
Benzoyl peroxide drome) and minor
Dodecyl gallate – Interface dermatities of HIV infection
Gold sodium thiosulfate – Morbilliform exanthems
Perioral dermatitis Cobalt chloride – Virus induced
Gold sodium thiosulfate – Drug induced
Balsam of Peru – Paraneoplastic pemphigus
Nickel sulfate hexahydrate – Pityriasis lichenoides
Recurrent aphthous stomatitis Vanillin
There are also areas of hydropic degeneration of basal Table 30.6 Differential diagnosis of LTR
cell layer with dense bandlike chronic inflammatory
Lichen planus
cellular infiltrated in lamina propria. Deeper perivascular
• B asal cell liquefaction degeneration with large number of 781
oriented lymphocytes aggregates can be seen.
Civatte bodies and colloid bodies.
In the LTR, this characteristic pattern of epidermal • There were significant vasodilatations in upper dermis
basal cell injury/degeneration is intimately associated inside the massive band like infiltrate.
with a band-like array of mononuclear inflammatory cells • PAS positive basement membrane was disrupted in reaction
in the papillary and mid dermis consisting of activated area. Hypergranulosis was conspicuous.
T cells, macrophages, and dendritic cells. Histological
Chronic DLE
appearances of idiopathic lichen planus and lichenoid
drug eruption are very similar. An inflammatory infiltrate • S potty lichenoid reaction in the form of basal cell
liquefaction degeneration.
located deep to superficial infiltrate in some or all
• Civatte bodies and colloid bodies are infrequent.
areas; a focal perivascular infiltrate; plasma cells in the
• Infiltrate is more focal but could be band like.
connective tissue and neutrophils in the connective tissue • Epidermal atrophy and thickening of PAS positive basement
are suggested as distinguishing features between OLP and membrane may be important differentiating features.
oral LTR. However, these features can be present in other
Lichenoid melanodermatitis (LM) or melanodermatitis toxica
nonlichenoid lesion. Sehgal and co-workers has described
the histopathological features of other lichenoid tissue • F ocal mild to moderate liquefaction degeneration of basal
reactions like lesion in their research article which can help cells with atrophy of the epidermis.
in diagnosis. • The infiltrate although band like is less dense with marked
pigmentary incontinence in clumps and giant melanophages.
Lichenoid tissue reaction should be differentiated from
• Civatte bodies, colloid bodies were not found and vascular
lichen planus chronic DLE, lichenoid melanodermatitis
changes were less prominent.
(LM) or Melanodermatitis toxica, Lichen nitidus (LN)
which are discussed in Table 30.6. Lichen nitidus (LN)
• L ocalized basal cell damage with claw like rete ridges
Management clutching a dense infiltrate.
Improved oral hygiene and removal of amalgam restoration • The dermal infiltrate often showed multinucleated giant
should be replaced with yellow gold/white gold. cell.
• Civatte bodies and colloid bodies were not present.
Points to Remember
Dental amalgam, gold restoration, copper, palladium
with or without peripheral striae, affected area may be Appearance: It appear as desquamative gingivitis with
white or erythematous, posterior buccal mucosa, surface ulceration and erosion of the tongue or buccal mucosa.
epithelium showing hyperkeratotic, atrophic, ulcerated, Sign and symptoms: Ulcer is surrounded by zones of
hydropic degeneration of basal cell layer, band-like array erythema and streaky keratosis resembling lichen planus.
of mononuclear inflammatory cells, Improved oral hygiene. The ulcer heals without scarring. Migration of ulcer can
also be seen.
CHRONIC ULCERATIVE STOMATITIS Histopathological Features
It is immune mediated disorders affecting oral mucosa. Epithelium is more atrophic as compared to epithelium in
This patient develop autoantibodies agent 70-kD nuclear lichen planus. Inflammatory infiltrated contain significant
protein and cause epithelial growth and differentiation. numbers of plasma cells.

Age and sex distribution: It is seen in women in sixth Hydroxychoroquine therapy gives good results as com-
decade of life. pared to corticosteroid.
presentations and diagnosis of common allergens relevant

Points to Remember
to dental practice. Acta Clin Croat. 2011;50(4):553-61.
Streaky keratosis resembling lichen, ulcer is surrounded Review.
782 by zones of erythema, epithelium is more atrophic, 2. Black, CA. Delayed type hypersensitivity: current theories
inflammatory infiltrated, hydroxychoroquine. with an historic perspective dermatol. Online J. (May 1999)
5(1):7 at http://dermatology.cdlib.org DOJvol5num1/
reviews/black.htm
CROHN’S DISEASE 3. Buchanan JA, Zakrzewska JM. Burning mouth syndrome.
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4. Cohen DE, Brancaccio RR, Soter NA. Diagnostic tests for
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∙ Age: It is more commonly seen in patient more than 60 Immunol. 2000;15:287-305. [Medline].
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1. Undermined edge seen in: 4. Food allergy is the most common cause in:
a. Tropic ulcer b. Tuberculosis ulcer a. Angioedema b. Quincke’s edema
c. Malignant ulcer d. Healing ulcer c. Giant urticaria d. All of the above.
2. Rolled out (everted) edge is a characteristic feature of:
5. Sutton’s disease refers to:
a. Tropic ulcer b. Healing ulcer
a. Major aphthae b. Minor aphthae
c. Malignant ulcer d. Venous ulcer
c. Recurrent ulcers d. Herpetiform ulcers
3. Contact allergy of oral lesions is referred as:
a. Stomatitis medicamentosa 6. Triad of recurring oral ulcers, recurring genital ulcers
b. Canker sores and eye lesions is the feature of:
c. Dermatitis venenata a. Behçet’s syndrome b. Sutton’s disease
d. Stomatitis venenata c. Book syndrome d. Chemical burns
31 Endocrine Disorders
Chapter Outline
Â Anatomy and physiology Â Hypoparathyroidism

Â Hyperpituitarism Â Pseudohypoparathyroidism
Â Hypopituitarism or pituitary dwarfism Â Diabetes mellitus
Â Progeria Â Addison’s disease
Â Hyperthyroidism Â Adrenogenital syndrome
Â Hypothyroidism Â Melasma
Â Hyperparathyroidism Â Cushing’s syndrome
Hormones vary tremendously in chemical composition extent, the pancreas. These glands are connected with the
and biologic activity. Various disorders of components of autonomic nervous system, secreting their hormones in
the endocrine system have generalized adverse effects on response to electrical stimuli originating in higher centers
skeletal system due to altered metabolism. in the brain and reaching them by way of the nerve fibers
that link them to that system.
ANATOMY AND PHYSIOLOGY Pituitary gland: It lies within the sella turcica at
the base of brain and it is divided into three distinct lobes.
The endocrine system is specifically designed to integrate The anterior lobe also called as adenohypophysis originates
and control the human body’s innumerable metabolic from epithelium of Rathke’s pouch, the intermediated lobe
activities. Its functioning components are the endocrine from dorsal portion of Rathke’s pouch and posterior lobe
glands. These units can function individually, in series, or nuerohypophysis develops from base of third ventricles.
or in parallel, their activities being integrated closely. Hormone secreted by anterior lobe are growth hormone
Communication with each other and with the tissues (GH), adrenocorticotropic hormone (ACTH), thyroid
under their control is established by means of hormones, stimulating hormone (TSH), follicle stimulating hormone
which they produce, store, and release as required and (FSH), leutinizing hormone (LH) and prolactin. Hormone
which are distributed throughout the body by means of the secreted by intermediated lobe is melanocytes stimulating
circulating blood. In most instances, the agent stimulating hormone and by posterior lobe vasopressin and oxytocin.
or inhibiting their activity is the hormone produced by the The secretory activities of the pituitary gland are modulated
corresponding target gland. The exceptions are the adrenal by hypothalamus through a series of complex feedback
medulla, the posterior pituitary gland, and to a lesser interaction.
Endocrine Disorders
Thyroid gland: It is situated in midline of body in the Adrenal gland: The adrenals are triangular-shaped structure
neck, at the level of cricoids cartilage having two lateral that sits on the superior poles of the kidneys. It produce
lobes which are join by isthmus. Third pyramidal lobe also and secretes a number of compounds that are essential for
extends from the isthmus. Embryologically, the thyroid maintenance of life adaptation to stress. Each gland is divided 785
gland develops as a downgrowth from the portion of into adrenal medulla and a cortex.
four pharyngeal pouches. It regulates the basal metabolic
Adrenal medulla: It arises form ectodermal tissues and
rate, stimulates somatic and psychic growth and plays
function as a part of the sympathetic nervous system. It
an important role in calcium metabolism. Follicular cells
manufacture and secretes two catecholamine, i.e. epinephrine
lining the follicles of the gland secrete tri-idothyronin
and norepinephrine. Epinephrine supports blood pressure
and tetra-idothyronin (thyroxin) which stimulates basal
by increasing the heart rate. Epinephrine also increases
metabolic rate and somatic and psychic growth of the
oxygen consumption by the tissue and glucose release by the
individuals. Para follicular cells lie in between the
liver. Norepinephrine increases peripheral resistance by its
follicles and they secrete thyrocalcitonin which promotes
vasoconstrictor effect. Epinephrine and norepinephrine also
deposition of calcium salts in skeletal and other tissue
exert important metabolic effects by promoting lipodiysis;
and tends to produce hypocalcemia. Around 83 percent
increase blood sugar levels by stimulating glycogeneolysis,
of T3 is produced by monodeiodination of T4 in others
elevating body temperature and increases basal metabolic
tissue such as liver, muscle and kidney. T4 is probably
rate. These compounds aid the body in adapting to stress
not metabolically active until converted to T3 and may
which is important in the dental sitting because release of
be regarded as prohormone. T3 and T4 circulate in plasma
endogenous epinephrine during stressful dental procedure
protein almost entirely bound to transport plasma proteins
can produce significant changes in blood pressure and pulse
mainly thyroxine binding globulin. It is a minute fraction
rate.
of unbound or free hormone, which diffuse into tissues
and exerts its metabolic action. Production of T3 and T4 Adrenal cortex: It secretes three major classes of hormone:
in the thyroid is stimulated by thyrotropin, a glycoprotein glucocorticoids or cortisols, which affects the inflammatory
released form thyrotropic cells of the anterior pituitary process and carbohydrates and protein metabolism, the
in response to the hyperthalamic tripeptide, thyrotropic mineralocorticoid aldosterone, which affects water and
releasing hormone. There is negative feedback of thyroid electrolyte balance and sex hormone testosterone, estrogen
hormones on the thyrotrophs such that in hyperthyroidism, and progesterone.
when plasma concentration of T3 and T4 are raised, TSH
secretion is suppressed and in hypothyroidism due to DISEASES OF PITUITARY GLAND
disease of the thyroid gland low T3 and T4 are associated
with high TSH level.
Hyperpituitarism
It results from hyperfunction of anterior lobe of pituitary
Parathyroid glands: The four parathyroid glands lie
gland, most significantly with increased production of
behind the lobes of the thyroid. They are not regulated by
growth hormone. The usual cause of this condition is a
pituitary gland, but respond directly to changes in serum
benign, functioning tumor of the eosinophilic cells in the
ionized calcium concentration. Parathyroid hormone (PTH)
anterior lobe of the pituitary gland. The GH acts directly
is a single chain polypeptide of 84 amino acid which are
on some tissue but most its biological effects are accounted
synthesized by the chief cells and released in response to
by stimulation of secretion of insulin-like growth factor I
a fall in serum ionized calcium concentration. The PTH
(IGF-I) and its binding proteins from the lower lobe.
directly promotes reabsorption of calcium from renal tubules
and bones. The PTH also has indirect effect, mediated by Types
increasing conversion of 1, 25 hydroxycholecalciferol,
• G igantism: If the increase occurs before the epiphysis
which results in increase calcium absorption from the food
of the long bone are closed.
and enhanced mobilization of calcium from bone. The initial
• Acromegaly: If the increase occurs later in life after
effect of PTH on bone is to stimulate osteolysis, returning
epiphysis closure.
from bone to extracellular fluid.
Clinical Features There is temporal headache, photophobia and reduction

in vision. The terminal phalanges of the hands and feets
Gigantism become large and the ribs also increase in size.
786 Generalized overgrowth of most tissue in childhood also Hypertrophy of soft palatal tissue may cause or
occurs. Most of the soft tissue and bones respond to the accentuate sleep apnea.
excess hormone by enlarging. Excessive generalized
skeletal growth. Oral Manifestations
Symptoms: Patient may often have of height to 7 to 8 Teeth in gigantism are proportional to the size of jaw and
feet. Patients achieve monstrous size because of tumors the rest of the body and root may be longer than normal.
of the pituitary gland. Later in life it may show genital The teeth become spaced, partly because of enlargement
underdevelopment and excessive perspiration and they of the tongue and party because upper teeth are situated
complained of headache, lassitude, fatigue, muscle and on the inner aspect of the lower dental arch, due to
joints pain and hot flashes. There is increase in size of disproportionate enlargement of the two jaws.
calvarium which may lead to change in the hat size. Mandibular condylar growth is very prominent.
Overgrowth of mandible leading to prognathism.
Signs: Pituitary tumors may also induce deficiency of Mandible may be of extraordinary proportions creating a
other pituitary hormones causing signs of hypogonadism major discrepancy between the upper and lower jaws and a
including decreased libido and menstrual problems in pronounced class III malocclusion.
women. In some cases, the growth at the condyle exceeds that
of the alveolar processes, so that increased in vertical depth
Acromegaly (Fig. 31.1)
of the ramus is greater than that of the body of the jaw,
It is more common in males and occurs most frequently consequently the upper and lower teeth fail to come into
in 3rd decade. Bone overgrowth and thickening of the soft proper occlusion.
tissue cause a characteristic coarsening of facial features The palatal vault is usually flattened and the tongue
termed acromegaly. increase in size and may cause crenation on its lateral
Symptoms: Hand and feet become large, with clubbing of border. The lips become thick and Negroid. In edentulous
the toes and fingers due to enlargement of the tufts of the patients’, enlargement of the alveolus may prevent the
terminal phalanges. comfortable fit of complete dentures.
It shows enlarge sella as a results presence of pituitary
adenoma. MRI will diagnose pituitary adenoma.
Laboratory Findings
Measurement of serum growth hormone level should be
done. This should be done after giving patient a measured
quantity of glucose. Normally, this glucose challenge will
reduce the production of growth hormone.
Diagnosis
It can be made from the characteristic clinical and
radiographic findings. Growth hormone concentration can
be measured by radioimmunoassay technique.
Management
Trans-sphenoidal surgery may result in cure of GH excess
Figure 31.1 Acromegaly of patient showing large head especially in patients with macroadenoma.
Endocrine Disorders
Octreotide, a long-acting analog of somatostatin,

lowers GH. It is administered as subcutaneous insulin 2 to
3 times/day. Dopamine antagonists are also used.
Growth hormone receptor blocking agent pegvisomant 787
can be use in combination with somatostatin analog. It is
injected daily and act on peripheral tissue to inhibit the
action of growth hormone.
Radiotherapy should be used in some cases, but it is not
so effective.
Points to Remember
Excessive skeletal growth, monstrous size, hypo-
gonadism, headache, lassitude, fatigue, coarsening
of facial features, clubbing of the toes, temporal
headache, photophobia, accentuate sleep apnea, teeth
spaced, enlargement of tongue, prognathism, class III
malocclusion, crenation on its lateral border of tongue,
enlarge sella, trans-sphenoidal surgery, octreotide, peg-
visomant.
Figure 31.2 Short height of patient of dwarfism

Hypopituitarism or Pituitary Dwarfism
Pituitary is the master gland of the body. It results due to
reduced secretion of pituitary hormone, which may occur The hallmark of this condition is that the growth
due to pituitary adenoma that compresses the pituitary is retarded to a greater degree than is bone and dental
gland. It can also occur due to reduce capacity of growth development. It may occur because of growth hormone and
hormone to respond to growth hormone. It is often the cortisol deficiency. Lack of gonadotropin delays the onset
consequence of growth of pituitary gland or interference of puberty. The presence of diabetes insipidus associated
with stalk function. It results in pituitary dwarfism. with a deficient secretion of vasopressin is suggestive of
Pathologic changes can results from a variety of pituitary pituitary dysfunction.
gland malfunction. Total absence of all pituitary secretions Growth hormone secretion is lost resulting in lethargy,
is known as panhypopituitarism. Hypopituitarism that muscle weakness and increase fat mass in adults. After LH
commences after puberty is called as ‘Simmond’s disease’. secretion becomes impaired, in the male loss of libido and
impotence and in female oligomenorrhea or amenorrhea.
Causes The male produce gynecomastia and skin becomes fair
It is caused by congenital or due to destructive disease of and wrinkled. The skull and facial bone are small and there
pituitary gland such as infarct occurring before puberty, is delay in maturation of the skeleton, and epiphysis may
space occupying lesions involving the sella turcica like remain nonunited throughout the life.
craniopharyngioma, adenomas and sarcoidosis.
Sheehan’s syndrome is a form of hypopituitarism caused Oral Manifestations
by infarction of the pituitary associated with postpartum Marked failure of development of maxilla and mandible
hemorrhage. along lack of condylar growth with short ramus and this
can lead to severe malocclusion and crowding of the teeth.
Clinical Features The two important hormones excreted by this gland—
The underdevelopment is symmetrical, individual is very somatotrophic and thyrotropic are responsible for the
small and in some cases, there may be a disproportional normal eruption of teeth and the alveolar growth. Thus in
shortening of the long bones (Fig. 31.2). case of hypofunction of this gland, the tooth eruption is
hampered. The dental arch is smaller than normal and thus Oral Manifestations
cannot accommodate all the teeth resulting in crowding
Accelerated formation of irregular dentin. Delayed eruption
and subsequent malocclusion. The clinical crown appears
788 of teeth.
smaller than normal because even through eruption does
occur it is not complete. Eruption is delayed and so the Management
shedding of the deciduous teeth.
No treatment and patient usually die before the age of 27
Laboratory Findings years.
Radioimmunoassay for human growth hormone shows Points to Remember
below normal level of human growth hormone.
Alopecia, pigmented areas of the trunk, high pitched
Management squeaky voice, exophthalmos, delayed eruption of teeth.
It is usually directed towards removal of the cause or
replacement of the pituitary hormone or those of its target HYPERTHYROIDISM
glands.
It is also called as thyrotoxicosis and it is a syndrome in
Growth hormone can be produce with recombinant
which there is excessive production of thyroxin in thyroid
DNA technology.
gland. It is associated with diffuse toxic goiter and less
Points to Remember frequently with toxic nodular goiter or toxic adenoma.
Excessive thyroxin causes generalized increase in
Panhypopituitarism, Simmond’s disease, very small metabolic rate of all body tissues.
individual, diabetes insipidus, loss of libido, impotence In patient with thyrotoxicosis, dental treatment can
oligomenorrhea or amenorrhea, lack of condylar growth, precipitate an acute emergency like thyroid crisis or thyroid
severe malocclusion, crowding of the teeth, maloc- storm.
clusion, eruption is delayed, replacement of the pituitary
hormone. Definition
Hyperthyroidism is excessive functional activity of the
PROGERIA thyroid gland that can be caused by Graves’ disease,
excessive replacement of/or overdose of thyroid hormone.
It is transmitted as autosomal dominant trait. This con-
dition is rare and underlying cause is entirely dependent on Causes
pituitary dysfunction. It is regarded as premature senility in
It is caused by exophthalmic goiter, toxic adenoma, ectopic
an individual of infantile proportions.
thyroid tissue, Graves’ disease, multinodular goiter, thyroid
Clinical Features adenoma, a pituitary disease involving anterior portion of the
gland, choriocarcinoma, excess pituitary TSH, autonomous
Affected infants appear normal at birth, but the typical
struma ovarii, polyostotic fibrous dysplasia.
clinical features become manifested within the first few
This disease is triggered by autoantibodies that are act
years.
against receptors for thyroid stimulating hormone (TSH)
Patient exhibits alopecia, pigmented areas of the trunk,
on the surface of thyroid cells. This in turn will stimulate
atrophic skin, prominent veins and loss of subcutaneous
the thyroid cells to release inappropriate thyroid hormone.
fat. The individual have high pitched squeaky voice, beak-
like nose and hypoplastic mandible. Clinical Features
The face is pointed, with the nose resembling the beak
Age and sex predilection: It has predilection for females
of a bird. The head is large, while mandible is small.
five to ten times more as compared to male between 20 to
Exophthalmos and joint deformities may be present.
40 years of age.
The lip is thin. The intelligence of this patient is either
normal or above normal and even at early age patient Signs: Thyroid is diffusely enlarged, smooth, possible
behave like old person. asymmetrical and nodular, a thrill may be present, may be
Endocrine Disorders
Oral Manifestations
There is advanced rate of dental development and early
eruption with premature loss of primary teeth. Generalized 789
decrease in bone density or loss of some areas of edentulous
alveolar bone. Early jaw development and alveolar bone
atrophy.
Laboratory Investigation
Plasma levels of T3 and T4 are increased; free thyroxin index
is raised in this disorder. Thyroid stimulating hormone is
decreased.
Anemia may be of moderate-to-severe degree and is seen
in patient with prolonged duration of the disease. The anemia
is hypochromic and abnormal forms of RBC may be seen.
Figure 31.3 Tumor of thyroid gland
Diffuse enlargement and hypercellularity of the thyroid
tender. Abdomen, liver and spleen may be enlarged (Fig.
gland is seen. Lymphocytic infiltration of the glandular
31.3).
parenchyma is also observed. There is also hyperplastic
Symptoms: It includes nervousness, fine tremors, and thyroid epithelium and little colloid production.
muscle weakness, mood swings from depression to extreme
euphoria, emotional liability, hyper-reflexia, ill sustained Management
clonus, proximal myopathy, bulber myopathy and periodic Antithyroid drugs: It would be appropriated to give
paralysis. There is weight loss despite normal or increases antithyroid drugs for 12 to 18 months to those in whom
appetite, diarrhea, bowel alterations, anorexia, vomiting a single episode was anticipated. Carbimazole for 0 to 3
and hyerdefaecation. weeks in 40 to 60 mg daily divided doses; for 4 to 8 weeks
Cardiac features: There is also palpitation, excessive in 20 to 40 mg daily divided doses and for maintenance
perspiration and irregular heart beat. Increased metabolic phase to 5 to 20 mg daily.
activity leads to increased circulatory demands, Radioactive iodine: This is common therapy used for
tachycardia and increased pulse pressure and sometimes patient having Grave’s disease. Thyroid gland takes up
congestive cardiac failure. Exertional dyspnea and ankle iodine as this is critical component of thyroid hormone.
edema, blood pressure normal, systolic hypertension may So after administration of radioactive iodine, thyroid gland
be present. Angina and cardiomyopathy and exacerbation quickly takes up it from bloodstream and sequestrated
of asthma. radioactive material in the glandular tissue. This radioactive
In thyrotoxicosis, the patient may have bulging eye material then destroys hyperactive thyroid tissue.
and partial paralysis of the ocular muscles, retraction and Other treatment modalities include subtotal thyroi dec-
jerky movement, corneal ulceration, optic neuritis, ocular tomy, administration of propylthiouracil and meth im azole.
muscle weakness, papilledema, loss of visual activity,
exophthalmos. There is amenorrhea, oligomenorrhea, Points to Remember
infertility, spontaneous abortion and loss of libido, Thyrotoxicosis, thyroid crisis, Graves’ disease, hyper-
impotence. reflexia, euphoria, periodic paralysis, tachycardia,
Other symptoms: There is increases sweating, pruritis, increased pulse pressure, bulging eye, partial paralysis
oncholysis, pigmentation, vitiligo, digital clubbing and of the ocular muscles, optic neuritis, vitiligo, bilateral
pretibail myxedema (bilateral nonpitting edema). Heat nonpitting edema, premature loss of primary teeth,
intolerance, sweaty and warm extremities, thin shiny decrease in bone density, anemia, hypercellularity of
skin, pretibial myxedema, increased PR and early fatigue, the thyroid gland, hyperplastic thyroid epithelium,
lymphadenopathy, thirst and osteoporosis occurs. carbimazole, radioactive iodine.
HYPOTHYROIDISM menorrhagia, and anorexia. In late stages, there is slowing

of intellectual and motor activity, absence of sweating,
It is caused by insufficient secretion of thyroxin by the modest weight gain, constipation, peripheral edema,
790 thyroid gland. As a failure of thyrotropic function on the pallor, hoarseness, decreased sense of taste and smell,
part of the pituitary gland or an atrophy or destruction of muscle cramps, aches and pains, dyspnea, anginal pain and
the thyroid gland leads to an inability of the thyroid to deafness.
produce sufficient hormone to meet the requirement of the
body. Signs: Dull expressionless face, periorbital edema, sparse
hair and skin that feels doughy to touch.
Definition There is hypothermia (decrease body temperature)
and the patient may be disorientated which may indicate
Decreased or deficient secretion of thyroid hormones is
impending myxedematous coma, pulse decreased, blood
caused by thyroiditis, insufficient thyroid replacement,
pressure normal, diastolic hypertension may be present.
post-thyroidectomy, postradioactive iodine therapy.
Facial pallor, puffiness of face and eyelids (myxedema),
Types occasional purpura, thickened nose and lips in more
advanced cases, note scars in neck from thyroidectomy.
• Cretinism
Thyroid gland may be enlarged, thin brittle nails,
• Juvenile myxedema
coarse thin hair, dry rough skin; displaced apical beat may
• Myxedema
be present. Delayed return of deep tendon reflexes, pleural
• Primary
effusion may be present. There are also watery eyes, brittle
• Secondary.
hair and patchy alopecia.
Types Oral Manifestations

Cretinism: If failure of hormone occurs in infancy Cretinism and Juvenile Myxedema
Juvenile myxedema: If it occurs in childhood Dental development delayed and primary teeth slow
to exfoliate. Enamel hypoplasia can also be seen.
Myxedema: If it occur after the puberty. In it there is
Abnormalities of dentin formation lead to enlarge pulp
subcutaneous deposition of hydrophilic mucopolysaccrides
chamber.
Primary: In this thyroid gland is itself abnormal Maxilla is overdeveloped and mandible is underde-
veloped. Retarded condylar growth leads to characteristic
Secondary: In this pituitary gland does not produce an
micrognathia and open bite relationship.
adequate amount of thyroid stimulating hormone.
Tongue is enlarged by edema fluid and due to it tongue
Clinical Features may protruded continuously and such protrusion may lead
to malocclusion of teeth. The base of skull is shortened
Cretinism and Juvenile Myxedema leading to a retraction of the bridge of the nose with flaring.
It may be present at birth or become evidence within the Face is wide and fails to develop in longitudinal direction.
first few months after birth. Lips are puffy, thickened and protruding.
Symptoms: Hoarse cry, constipation, feeding problems in Myxedema
neonates, retarded mental and physical growth.
Macroglossia and enlarged lip as a result of the deposition
Signs: There is delayed fusion of all body epiphysis of water and protein. Facial swelling of nonpitting type
and delayed ossification of paranasal sinus, partially and mandible is underdevelop. There is a greater tendency
pneumatization. There is protuberant abdomen with to periodontal disease, with alveolar destruction and
umbilical hernia. The hairs are sparse and brittle, the finger loosening of the teeth.
nails are brittle and the sweat glands are atrophic.
Laboratory Findings
Myxedema Thyroid stimulating hormone increased and T3 and T4
Symptoms: It may include weakness, fatigue, cold decreased. In ECG, a classical sinus bradycardia with low
intolerance, and lethargy, dryness of skin, headache, voltage complexes and ST/T wave abnormalities. There is
Endocrine Disorders
raised cholesterol and triglycerides level and low serum in decrease in serum phosphorus level. At the same
sodium. time, it induces an increase in calcium reabsorption from
glomerular filtrate. Parathyroid hormone may also increase
Complications the absorption of calcium from the intestine but this is not 791
It is caused by coronary artery disease, congestive heart definitely established. Hence, in a healthy person injection
failure, increased susceptibility to infection and mental of parathyroid hormone produces an elevated plasma
disturbances including depression. calcium level, a decreased plasma phosphorus level and an
increased alkaline phosphatase level.
Diagnosis
Clinical signs and symptoms and radiographic features Types
along with laboratory test revealing reduction in serum T3 Primary: There is autonomous secretion of parathyroid
and T4 levels are confirmatory. hormone (PTH) by hyperplasia, benign and malignant
tumor of one or more of the four parathyroid glands.
Management
Secondary: Compensatory increase in output of PTH in
Thyroid preparation: Patients are managed by thyroid response to hypocalcemia. The underlying hypocalcemia
preparation. Mainly use is levothyroxine, which is available may result from an inadequate dietary intake or poor
as 25, 50 and 100 micrograms tablets. It is customary to absorption of vitamin D or from deficient metabolism of
start slowly and a dose of 50 mg/day should be given for vitamin D in the liver or kidney. It effects to restore serum
3 weeks and finally to 150 mg/day. In the elderly and in calcium level at the expense of the lots of calcium in bone.
patient with ischemic heart disease the initial dose should
be 25 mg/day. Tertiary: Occasionally parathyroid tumor after long-
standing secondary hyperparathyroidism develops this
Points to Remember condition known as tertiary hyperparathyroidism. The
increased parathyroid level produces increased bone
Hoarse cry, pneumatization of sinus, sparse hairs,
resorption and a resultant hypercalcemia.
weakness, anginal pain, dull expressionless face,
hypothermia, puffiness of face, enlarged thyroid gland, Ectopic: Due to excessive parathyroid hormone synthesized
pleural effusion, primary teeth slow to exfoliate, enamel in patient with malignant disease.
hypoplasia, maxilla is overdeveloped, retarded condylar
growth, enlarged tongue, malocclusion of teeth, wide Clinical Features
face, increased thyroid stimulating hormone, thyroid Age and sex distribution: Female to male ratio is 3:1.
preparation. Mainly in 30 to 60 years of age.
Classic triad: It has classic triad of stones, bones and
HYPERPARATHYROIDISM abdominal organs.
It is an endocrine disorder in which there is an excess of Stones: Renal calculi are common due to elevated level of
circulating parathyroid hormone. Excess PTH stimulates serum calcium. Metastatic calcification is seen.
osteoclast to mobilize calcium from skeleton leading to Bones: There are variety of osseous changes seen in bones.
hypercalcemia in addition to PTH increased renal tubular There is also bone and joint pain.
reabsorption of calcium. Following is the sequence of
Abdominal organs: There is tendency for the development
event which gives an idea of the reaction promoted by this
of duodenal ulcers.
hormone.
The bone and kidney are the target organs of parathyroid Other symptoms: There is also hematuria, back pain,
hormone which mediates the osteoclast to resorb bone urinary tract infection, hypertension are common. Peptic
actively. When the bone is resorbed, calcium is released ulcer, psychiatric effect like emotional instability, and
in the extracellular fluid and the serum calcium level is sometime pathologic fractures occurs. Gastrointestinal
elevated. The parathyroid hormone acts on the epithelium difficulties such as anorexia, nausea, vomiting and crampy
of kidney tubules causing diuresis of phosphorus resulting pain may be present.
Pigeon chest: Bone deformities occur such as bending of

long bone occasional fracture and collapse of vertebrae
and formation of pigeon chest. It is associated with muscle
792 weakness, fatigue, weight loss, insomnia, headache, poly-
dipsia and polyuria.
Oral Manifestations
There is gradual loosening drifting and loss of teeth,
malocclusion. There is pathological fracture of bone.
Cystic lesion involving jaws are seen over 10 percent of
cases.
Brown tumor is present intraorally (Fig. 31.4).
First signs of disease are subperiosteal resorption of the Figure 31.4 Brown tumor seen in oral cavity
phalanges of the index and middle fingers. There is also
generalized loss of lamina dura surrounding the root.
Ground glass appearance: There is blurring of the normal
trabecular pattern and decrease in trabecular density.
Brown tumor: This are called brown tumor as it resembles
brown color in specimen. It appear well demarcated
unilocular or multilocular radiolucency (Fig. 31.5).
Osteitis fibrosa cystica: There is central degeneration and
fibrosis of longstanding brown tumor.
There is osteoclastic resorption of the trabeculae of the
spongiosa and along the blood vessels in the haversian
system of the cortex. Fibrosis especially in the marrow
spaces is marked. Fibroblasts replace resorbed trabeculae in
Figure 31.5 Radiograph of brown tumor of oral cavity
the fibrotic islands there is recent and old hemorrhage with
much hemosiderin is evidence. Large tortuous blood filled
sinusoidal channels are lined by a flat endothelial layer. Diagnosis
The surrounding tissue is fibroblastic and hypercellular. Serum alkaline phosphatase and serum calcium level is
Multinucleated giant cells lie adjacent to the sinusoids increased. Decreased blood phosphorus level. Increase in
and osteoid trabeculae tend to orient themselves in close circulating hormone demonstrated by radioisotope studies.
proximity to the vascular spaces.
Management
Laboratory Findings It often regresses without surgery and the rarefaction
The serum calcium level is raised and serum phosphorus disappears. Surgical excision of adenoma. The oral
level is decreased and serum alkaline phosphatase level is administration of vitamin D in secondary type can prevent
elevated in primary hyperparathyroidism and in secondary skeletal demineralization in most of the cases.
hyperparathyroidism the serum calcium level is decreased Cinacalcet is now a day use in management of over-
whereas the serum phosphorus and alkaline phosphatase production of parathormone associated with secondary
level are elevated. hyperparathyroidism. This is calciminetic agents that
Endocrine Disorders
sensitize the calcium receptor of the parathyroid cells to Oral Manifestations

extracellular calcium causing the cell to reduce output of
Hypoplasia of enamel, delayed eruption, external root
parathormone.
resorption and root dilacerations. There is also blunting of 793
molar roots.
Points to Remember
Chvostek sign: A sharp tap over the facial in front of ear
Classic triad of stones, bones and abdominal organs,
causes twitching of facial muscle around the mouth which
renal calculi, joint pain, abdominal organs, hematuria,
is called as Chvostek sign. Chronic candidiasis is also some
peptic ulcer, pigeon chest, loss of teeth, malocclusion,
time present.
generalized loss of lamina dura, ground glass appearance,
brown tumor, osteitis fibrosa cystica, osteoclastic Laboratory Findings
resorption, fibrosis in the marrow, tortuous blood
filled sinusoidal channels, hypercellular surrounding The serum calcium level is decreased usually below
tissue, raised serum calcium level, surgical excision of 7 mg/dL. Serum phosphate level correspondingly ele-
adenoma, cinacalcet. vated. Urinary calcium is low or absent. The PTH can be
measured by radioimmunoassay.
HYPOPARATHYROIDISM Management
It is an uncommon condition in which there is insufficient Supplemental calcium and vitamin D depending on severity
secretion of parathyroid hormone. of the hypocalcemia should be administered. In severe
cases intravenous administration of calcium gluconate is
Etiology the treatment of choice.
It can cause due to inadvertent surgical damage to Teriparatide a recombinant form of the active
parathyroid gland and their vascular supply during thyroid component of human parathormone.
gland procedure.
Points to Remember
Other causes which can cause parathyroid damage
from radioactive iodine 131, autoimmune destruction of Tetany, paresthesia-of-hand feet, tingling in the circumor-
parathyroid gland, DiGeorge syndrome, and endocrine- al area, short stature, Trousseau’s sign, Chvostek sign,
candidiasis syndrome. decreased serum calcium level, supplemental calcium,
teriparatide.
Clinical Features
Tetany: It can lead to tetany in the form of carpopedal PSEUDOHYPOPARATHYROIDISM
spasm of the wrist and ankle joint. There is also stiffness in
hands, feet and lips. Pseudohypoparathyroidism is a condition in which there
defect in the response of tissue target cell to normal level of
Symptoms: There is also paresthesia of hands, feet and parathyroid hormone. It is also called as Albright hereditary
around the mouth. Tingling in the circumoral area, fingers osteodystrophy or acrodysostosis.
and toes. Patients may complaint of anxiety, depression,
epilepsy and chorea. There is reduction in intellectual Types
capacity due to calcification within the brain. Pseudohypoparathyroidism type I: There is three
Signs: Patient with hypoparathyroidism manifest short subcategories Ia (molecular defect of specific intracellular
stature due to early closure of certain bony epiphysis. binding protein Gsα), which prevent the formation of cyclic
The face is round and the hand shows shortening of the adenosine monophosphate a critical component in the
metacarpal bones, so that finger are short. activation of cell metabolism, Ib (defective receptor of the
PTH on the surface of target cells) Ic (defect in adenylate
Trousseau’s sign: It is elicited by occluding blood flow to
cyclase or subtle Gsα alteration.
the forearm for 3 minutes with sphygmomanometer cuff
applied to the arm and raising the pressure above systolic Pseudohypoparathyroidism type II: Induction of cAMP
level. This will induce carpopedal spasm. by PTH in the target cells.
Clinical Features Types

Symptoms: Mild mental retardation, obesity, round face, Primary
794 short neck and short stature. • Type I or insulin dependent diabetes mellitus (IDDM)
Midfacial hypoplasia: It is commonly seen in patient with • Type II or noninsulin dependent diabetes mellitus
pseudohypoparathyroidism. (NIDDM)
• Nonobese
Signs: Metacarpals and metatarsal are shortened. • Obese
Osteoma cutis: Subcutaneous calcification. • Maturity onset diabetes of the young (MODY)
Secondary
Oral Manifestations • Pancreatic disease
There is generalized enamel hypoplasia, widened pulp • Endocrine disease
chamber. Presence of intrapulpal calcification is also noted • Drug induced
which is also term as dagger shaped. • Genetic syndrome
Patient also complaint as oligodontia, delayed eruption Impaired glucose tolerance
and blunting of apices of the teeth. Gestational diabetes.
Laboratory Findings
Types
There is elevated level of PTH, hypocalcemia, hyper-
phosphatemia with normal renal function. Primary
Type I or insulin dependent diabetes mellitus–it occurs due
Management to deficiency.
Administration of vitamin D and calcium should be done.
Type II or Noninsulin dependent diabetes mellitus–it
Prognosis is good.
occurs due to insulin resistance.
– Nonobese
Points to Remember – Obese
Mental retardation, obesity, midfacial hypoplasia, – Maturity onset diabetes of the young (MODY)
osteoma cutis, dagger shaped intrapulpal calcification,
oligodontia, elevated level of PTH, vitamin D. Secondary
Pancreatic disease (pancreatitis, hemochromatosis, neo-
plastic disease, pancreatectomy, cystic fibrosis).
DIABETES MELLITUS
Endocrine disease—excess endogenous production of
It may cause by autoimmune response. Principal laboratory hormonal antagonists to insulin, e.g. growth hormone in
sign are hypercalcemia. It is common endocrine disorders acromegaly.
characterized by chronic hyperglycemia and abnormalities
in carbohydrate and lipid mechanism. Drug induced like corticosteroid, thiazide diuretics and
phenytoin.
Definition Genetic syndrome like Down syndrome, Klinefelter’s
Inappropriate hyperglycemia due to tissue resistance to syndrome and Turner’s syndrome.
Insulin action, reduced insulin secretion or both.
Impaired Glucose Tolerance
Etiology It is a prediabetic state of hyperglycemia that is
It is caused by disorders of carbohydrate mechanism associated with insulin resistance. Two-hour glucose
resulting from insulin deficiency or ineffectiveness, pro- levels of 140 to 199 mg per dL on the 75 g oral
ducing hyperglycemia and glycosuria. Genetic, autoi- glucose tolerance test. A patient is said to be under the
mmune and pancreatic dysfunction. condition of impaired glucose tolerance (IGI). When
Endocrine Disorders
he/she has an intermediately raised glucose level after

2 hours, but less than would quality for type 2 diabetes
millitus.
795
Gestational Diabetes
It is a condition in which woman without previously
diagnosed diabetes exhibit high blood glucose levels
during pregnancy (especially during third trimester).
Etiology
Type I Diabetes Mellitus
Viruses: Several viruses are been implicated including

infection with mumps coxsackie B4, retrovirus, rubella
and cytomegalovirus and Epstein-Barr virus. Virus particle Figure 31.6 Periodontitis in diabetes patients
known to cause cytopathic or autoimmune damage to beta
cells have been isolated from the pancrease.
Malnutrition: It is proposed that malnutrition in utero and
Diet: Bovine serum albumin (BSA) a major constituent the infancy may damage beta cell development at a critical
of cow’s milk has been implicated in triggering type I period predisposing to type II diabetes later in life.
diabetes. It has been shown that a child who has taken
cow’s milk early in infancy has been more prone to develop Age: Age is an important risk factors for type II diabetes as
type I diabetes mellitus as compared to other who has taken it is principally disease of middle aged and elderly affecting
breast milk. 10 percent of the population over the age of 65.
Stress: It may precipitate the development of type I Pregnancy: During normal pregnancy, insulin sensitivity
diabetes by stimulating the secretion of counter regulatory is reduced through the action of placental hormone and
hormones and possibly by modulating immune activity. this affect glucose tolerance. The term gestational diabetes
refers to hyperglycemia occuring for the first time during
Immunological factors: There is evidence that type I pregnancy.
diabetes is a T cell-mediated autoimmune disease. There Insulin resistance occurs in type II diabetes is due to
is also HLA linked genetic predisposition. Monocular an abnormal insulin molecule, an excessive amount of
cell infiltration of pancreatic islets restoration in selective circulating antagonists and target tissue defect.
destruction of insulin secreting cells and induction
of remission by immunosuppressive drugs such as Clinical Features
cyclosporine suggest it immunological etiology. Polydipsia: There is excessive intake of fluid.
Type II Diabetes Mellitus Polyuria: There is excessive urine passage.
Genetic: The majority of cause of type II diabetes are Polyphagia: There is excessive hunger.
multifactorial. Various types are associated with it like There is presence of acetone breath. Visual difficulty
hepatocyte nuclear factor, glucokinase, and mitochondrial ranging form progressive color blindness to total blindness
DNA and insulin receptors. that have disease more than 20 years. Coronary artery
disease and stroke are frequent complication.
Environmental Factors Diabetic neuropathy can cause marked irritability.
Lifestyle: Overeating, especially when combined with Recurrent vaginal (yeast) infections, recurrent urinary tract
obesity and underactivity is associated with developmental infections, recurrent skin infections (especially of feet) and
of type II diabetes. reversible paresthesia of fingers or toe.
Nocturia, weight loss, fatigue, obesity usually present in There is delay in healing of oral wound due to decreased
older age group, nausea, vomiting. Temperature, blood pres- polymorphonuclear chemotaxis. There is also angular
sure may be elevated and peripheral pulses may be reduced. cheilosis, altered taste sensation, oral lichen planus, and
796 Complication of diabetic mellitus is peripheral vascular diffuse enlargement of parotid gland.
disease which can results in kidney failure and gangrenous
involvement of the limb. Management
Treatment modalities includes diet control, oral hypo-
Oral Manifestations
glycemic drugs like sulfonylurea, biguanides, alfaglu-
It will influence the onset and course of periodontal cosidase inhibitors, insulin therapy.
disease. Patient with diabetes are more prone to develop
periodontal disease than are those with normal glucose Points to Remember
metabolism (Fig. 31.6). There is a greater tendency for Polydipsia, Polyuria, Polyphagia, diabetic neuropathy,
bleeding on probing. The patient may exhibit a fulminating peripheral vascular disease, periodontal disease, alveolar
periodontitis with periodontal abscess formation and bone resorption, median rhomboidal glossitis, Candida
inflamed painful abscess and even hemorrhagic gingival albicans infection, paresis, dysethesia, increased caries
papillae, this factor culminated and give rise to tooth activity, oral hypoglycemic drugs.
mobility, i.e. loose teeth.
It will show more severe and rapid alveolar bone
resorption and are more prone to develop periodontal ADDISON’S DISEASE
abscess. Insulin dependent diabetic children tend to have It is also called as chronic adrenal insufficiency or hypo-
more destruction around the first molars and incisors adrenocorticism. It was first described by Addison in 1855.
than elsewhere. As such diabetes mellitus does not cause
periodontal disease directly but it alters the response of Causes
the periodontal lesion to local irritants, hastening bone
It is caused by tuberculosis, metastatic carcinoma, intra-
loss and retarding postsurgical healing of the periodontal
dermal hemorrhage, amyloidosis, hemochromatosis,
lesions. Gingival fluid in the diabetes has more glucose
adrenal infarction and congenital adrenal hypoplasia.
level which favors the growth of microflora.
Drugs causing Addison’s disease are aminoglute-
Diabetes is considered to be factor for median
thimide, ketoconazole and etomidate.
rhomboidal glossitis as frequency of abnormal blood sugar
level in diabetes and predisposition of these subjects to Types
candidiasis. There is also impairment of blood supply to
Primary hypoadrenocorticism: Insufficient production
dorsum of tongue due to arteriosclerosis changes in the
of adrenal corticosteroid hormone cause by destruction of
blood vessels supplying the area. Impairment of local
adrenal cortex
immune mechanism which decrease the concentration of
Langerhans cells in the lesion. Secondary hypoadrenocorticism: It occur if adrenal
It is infection with Candida albicans which occur due to gland is not functioning properly.
encouragement of local multiplication of Candida albicans
due to impaired glucose level and immune mechanism. Clinical Features
Dry socket develops in diabetes hence they show delayed Age and sex distribution: It is more common in males
healing and impaired immunological balance. It is often and, found in all age groups, it is most frequently seen in
associated with variety of otherwise unexplained oral the 3rd and 4th decade.
symptoms such as burning sensation, atypical paresis,
Symptoms: Feeble heart action, general debility, vomi-
dysethesia and dysgeusia.
ting, and diarrhea and severe anemia. Patient complained
Diabetes neuropathy is recognized as polymorphic
of postural hypotension.
condition as when manifested as polyneuropathy on the
assumption the trigeminal nerve might be involved. Bronzing of skin: The disease is characterized by bron-
Increased caries activity occurs due to excessive fluid zing of skin, a pigmentation of the mucous membrane.
loss. Patient complained of xerostomia. There is also This Hyperpigmentation is more common on sun exposed
atrophy of lingual papillae with fissuring and dry tongue. surface of the skin and overpressure point.
Endocrine Disorders
Decrease cortisol level interferes with the manufacture to appearance of lesion. In female child, it produces
of carbohydrates from protein, causing hypoglycemia and pseudohermaphroditism, while in male child, it produces
diminished glycogen storage in the liver. Neuromuscular macrogenitosomia praecox. In the females it produces
function is inhibited, producing muscle weakness. There is masculinization and in males in produce sexual precocity. 797
also reduced resistance to infection, trauma, and stress. In females it produce virilism and in males it produces
feminization. If the disease begins early premature eruption
Oral Manifestations of the teeth may occur. Administration of corticosteroid or
The pale brown or deep chocolate pigmentation of the oral estrogen.
mucosa, spreading over the buccal mucosa form the angle
of the mouth and/or developing on the gingiva, tongue, lips Points to Remember
may be first evidence of disease. Pseudohermaphroditism, Macrogenitosomia praecox,
masculinization, sexual precocity.
Biopsy of oral lesion shows acanthosis with silver positive
granules in the cells of the stratum germinativum. MELASMA
It is also called as mask of pregnancy. It is symmetrical
hyperpigmentation of sun exposed skin of face and neck.
The associated anemia is normocytic and normochromic It is thought to be caused by exogenous estrogen and
associated with reticulocytosis. There is reduction in the progesterone.
red cell mass. There is high blood levels of potassium and
low concentration of sodium and chloride. Elevated blood Clinical Features
urea nitrogen. Location: It is seen on midface, forehead, upper lip, chin
The diagnosis is confirmed by rapid ACTH stimulation and arms.
test, measurement of serum cortisol level and plasma
ACTH level. If serum cortisol level are below 20 µg/dL Appearance: There is dark brown cutaneous macules
then the patient has adrenal insufficiency. which appear bilaterally in the adults women. The
pigmentation may remain faint or darken over time
Management
Glucocorticoids replacement: Cortisol is the drug of Histopathological Features
choice. In patient who are not critically ill hydrocortisone There is increase melanin deposition in epidermis.
15 mg on waking and 5 mg at 6 PM in evening.
Management
Supplement mineralocorticoid: Can be given.
Triple combination topical therapy—a combination of
Points to Remember hydroquinone, tretinoin and fluocinolone acetonide give
good results.
Feeble heart action postural hypotension, bronzing of
Other therapy includes glycolic acid chemical peel,
skin, muscle weakness, pale brown or deep chocolate
laser therapy, and dermabrasion.
pigmentation of oral mucosa, acanthosis, silver posi-
tive granules, normocytic anemia, glucocorticoids
replacement, supplement mineralocorticoid. CUSHING’S SYNDROME
Cushing’s syndrome arises from excess secretion of
glucocorticoids by the adrenal glands. It is described
ADRENOGENITAL SYNDROME by Harvey Cushing in 1932. It is also called as hyper-
It refers to any situation in which there is overproduction cortisolism.
of androgens. It results when hyperplasia or tumors of the
adrenal cortex occur. Etiology
It may appear at three different times of life, i.e. at birth, It is caused by adrenal adenoma, adrenal carcinoma, adrenal
in childhood and in adult. Clinical features vary according hyperplasia and basophilic adenoma of the anterior lobe
of pituitary gland. Other factors which are responsible for patient. In normal patient administration of dexamethasone
Cushing’s syndrome are exogenous corticosteroid, ACTH will suppress the normal level of ACTH.
secreting tumor of the anterior pituitary associated with
798 adrenal cortical hyperplasia, ectopically located adrenal Management
like tumor like in ovary, alcohol excess, major depressive If the lesion in the pituitary gland is the cause, therapy
illness, and primary obesity. usually consist of combination of surgery and radiotherapy.
Drugs used—metyrapone in dose of 2 to 6 g per day
Clinical Features
in divided doses by mouth. Other drug which is given is
Female to male ratio is 3:5, seen in 3rd and 4th decades. aminoglutethimide which is anticonvulsant, and it act by
Signs: Rapidly acquired obesity about upper portion of the blocking steroid synthesis.
body and rounded moon face (Fig. 31.7). There is truncal
obesity with prominent supraclavicular and dorsal cervical Points to Remember
fat pads giving rise to the buffalo hump appearance at
Hypercortisolism, obesity, moon face, truncal obesity,
the base of neck. The distal extremities are usually thin.
buffalo hump, dusky plethoric appearances, retarded
Weakness, hypertension, or concurrent diabetes is usually
dental age, metyrapone, aminoglutethimide.
present.
Symptoms: There is alternation in hair distribution. Dusky
plethoric appearances with formation of purple striae BIBLIOGRAPHY
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Case 44. Buffalo hump. Dent Update. 2007;34(4):252.
In children growth and development including skeletal and
3. Bergman SA. Perioperative management of the diabetic
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patient. Oral Surg Oral Med Oral Pathol Oral Radiol Endod.
be osteoporosis of the jaws.
2007;103(6):731-7.
4. Borghelli RF, Pettinari IL, Chuchurru JA, et al. Oral lichen
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Measurement of free cortisol in the urine and assay of Oral Surg Oral Med Oral Pathol. 1993;75(4):498-500.
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6. Cohen RB, Wilcox CW. A case of acromegaly identified
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8. Croft LK, Witkop CJ Jr, Glas JE. Pseudohypoparathyroidism:
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10. De Pablos PL, Ramos I, De La Calle H, Brown tumor in
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11. Elfenbaum A. The adrenogenital syndrome in pedodon-
tology. Dent Dig. 1971;77(9):531-4.
12. Farinazzo-Vitral RW, Motohiro-Tanaka. O, Reis Fraga M,
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Endocrine Disorders
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Pathol. 1969;28(6):877-84. tes, sulfite sensitivity: corticodependent asthma, and pheo-
15. Grrenberg MS, Brightman VJ, Lynch MA, et al. Idiopathic chromocytoma. Oral Surg Oral Med Oral Pathol. 1992;74:
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1. Gland which lies within the sella turcica is: 6. Increase production of growth hormone results in:
a. Pituitary gland b. Thyroid gland a. Gigantism b. Acromegaly
c. Parathyroid gland d. Adrenal gland c. None d. Both a and b
2. Hormones secreted by anterior lobe of pituitary gland 7. Marked failure of development of maxilla and
are: mandible with lack of condylar growth seen in:
a. Growth hormone b. TSH a. Hypothyroidism b. Hypopituitarism
c. Oxytocin d. Both a and b c. Cushing’s syndrome d. Addison’s disease
3. Hormones secreted by intermediate lobe of pituitary 8. Plasma levels of T3 and T4 increased in:
gland is:
a. Hyperthyroidism b. Hypothyroidism
a. Oxytocin b. Vasopressin
c. Addison’s disease d. All of the above
c. MSH d. FSH
4. Parafollicular cells of thyroid gland secrete: 9. Polydypsia, polyuria, polyphagia markedly seen in:
a. Thyrocalcitonin b. Thyroxin a. Diabetes mellitus b. Adrenal insufficiency
c. Oxytocin d. Vasopressin c. Addison’s disease d. Goiter
5. Catecholamine, i.e. epinephrine and nor-epinephrine 10. The pale brown or deep chocolate pigmentation of the
secreted by: oral mucosa seen in:
a. Parathyroid gland b. Adrenal gland a. Cushing’s syndrome b. Addison’s disease
c. Parotid gland d. Adrenal medulla c. Diabetes mellitus d. None of the above
32 Nutrition and Oral Cavity
Chapter Outline
Â Disturbances in protein metabolism Â Disorders of vitamins

• Protein deficiency Â Water soluble vitamins
• Amyloidosis • Beriberi
• Porphyria • Riboflavin (vitamin B2)
Â Disturbances in lipid metabolism • Niacin (vitamin B3) Pellagra
• Classification • Pantothenic acid (vitamin B5)
• Hand – Schuller – Christian disease • Pyridoxine (vitamin B6)
• Eosinophilic granuloma • Biotin (vitamin B8)
• Letterer Siwe disease • Folic acid (vitamin B9)
• Gaucher disease • Cyanocobalamin (vitamin B12)
• Niemann-Pick disease • Vitamin C scurvy
• Tay Sachs disease • Choline
Â Disturbances in carbohydrate metabolism Â Fat soluble vitamins
• Hurler’s syndrome • Vitamin A (Retinol) hypervitaminosis A
• Hereditary fructose intolerance • Vitamin D deficient rickets
• Hypoglycemia • Osteomalacia
• Mucopolysaccharidosis • Vitamin D resistant rickets (familial hypophosphatemia,
Â Disturbances in mineral metabolism refractory rickets)
• Acrodermatitis enteropathica • Vitamin E (Tocopherol)
• Hypophosphatasia • Vitamin K (Phylloquinone)
Â Miscellaneous disorders Â Disorders of bilirubin
• Malabsorption syndrome • Jaundice
Man is a complex biologic unit in a complex environment. DISTURBANCES IN PROTEIN

Duncan defined metabolisms as sum total of tissue activity
as considered in terms of physicochemical changes
METABOLISM
associated with and regulated by availability, utilization Protein Deficiency
and disposal of protein, fat, carbohydrate, vitamins, It is also called marasmus or kwashiorkor disease.
minerals, water and influence which the endocrine exerts Marasmus is an overall deficit of food intake, notably
on these processes. Alternation from this normal metabolic energy while the kwashiorkor is associated with primary
process constitutes the disturbances of metabolism. dietary protein deficiency.
Nutrition and Oral Cavity
Protein is the most important group of food stuff. In Oral Manifestations

addition to contributing to cells and intercellular material,
Bright reddening of tongue with loss of papillae occurs.
proteins also help in the formation of hormones, enzymes,
In kwashiorkor patient may have edema of tongue and 801
plasma protein antibodies and numerous physiologically
may develop scalloping around the lateral margins due to
important substances.
indentation of the teeth. Papillary atrophy may be present
Etiology and dorsum of the tongue assumes an erythematous and
smooth appearance.
Protein deficiency is occur when there is prolonged febrile There is bilateral angular cheilosis, fissuring of lip.
illness. It can also be seen in massive burns, large chronic Loss of circumoral pigmentation also occurs. Mouth
ulcer, persistent vomiting and diarrhea. becomes dry. However there is reduced carried activity
Stress, chronic infections like urinary tract infection, due to lack of substrate carbohydrate. Epithelium easily
tuberculosis and parasitic infection also leads to protein becomes detached from underlying tissue, leaving raw
deficiency. Disease like hyperthyroidism and hyper- bleeding surface.
metabolic state which interfere with utilization can also Decreased overall growth of jaws, delayed eruption,
leads to protein deficiency. retarded growth of incisors and molars. The gingiva and
periodontal ligament membrane exhibit varying degrees
Clinical Features
of degeneration. Deciduous teeth of children may exhibits
It occurs between the ages of 1 and 3 years in children. linear hypoplasia of the teeth.
Symptoms: There is loss of weight and subcutaneous
fats, wasting of muscles, pigmented changes in skin with
Laboratory Findings
hair loss, hypotension, weakness, anemia and edema (Fig. It may cause mild anemia which is normocytic and
32.1). normochromic. The reticulocyte count is normal and the
In severe cases, there is edema, episodes of diarrhea, bone marrow tends to become hypocellular.
skin pigmentation, liver enlargement and poor resistance
to infection. Management
Kwashiorkor is distinguished from marasmus by the A nutritious, well-balanced diet with lots of fresh fruits
presence of edema and the relatively less severe degree of and vegetables, grains, and protein will reduce the risk of
body wasting. malnutrition.
Points to Remember
Marasmus, kwashiorkor disease, prolonged febrile
illness, loss of weight, diarrhea, bright reddening of
tongue, papillary atrophy, bilateral angular cheilosis,
fissuring of lip, delayed eruption, mild anemia, nutritious
and well-balanced diet.
Amyloidosis
It is also called amyloid disease. It is deposition of
extracellular proteinaceous substance called amyloid. The
term amyloid comes as it resembles starch (amyl-starch,
oid-resembling).
Forms of Amyloid
Type A (secondary) amyloid is a fibrillar protein of
Figure 32.1 Sparse hair in patient with marasmus unknown origin that is seen in prolonged inflammatory
disease, genetic disease and syndromes such as familial Clinical Features

Mediterranean fever.
It commonly affected organs are kidneys, heart, GI tract,
Type B (primary) amyloid is thought to be
802 liver, respiratory tract, skin, eyes, adrenals, nerves and
immunologic in origin because of sequence homology
spleen. There may be primary localized collection of
with the NH2 terminal end of immunoglobulin light chain.
amyloid.
It is commonly seen in patient with multiple myeloma and
macroglobulinemia. Symptoms: The general symptoms are fatigue, weakness,
Type C: It includes amyloid of aging, localized ankle edema, dyspnea, paresthesia, orthostatic hypotension
nonspecific amyloid and pheochromocytomas. and weight loss.
Purpuric spots caused by hemorrhage resulting form
Types amyloid deposits in the blood vessels.
Localized or organ limited amyloidosis Sign: Superficial waxy lesion occurs on the eyelids,
Systemic amyloidosis nasolabial folds, neck, axilla or perineum and they may
• Primary amyloidosis bleed on pressure.
• Amyloidosis associated with multiple myeloma Congestive cardiac failure is a common problem due to
• Secondary amyloidosis amyloid deposits on myocardium. There is hepatomegaly,
• Familial or heredofamilial amyloidosis malabsorption or colitis may develop.
• Hormone related amyloid
• Hemodialysis associated amyloidosis. Oral Manifestations
Fibrous glycoproteins are deposited in submucosa as well
Types as in deeper muscular layer of tongue. Skin lesion may
Localized or organ limited amyloidosis: It is characterized diffusely involve the face or may present as small elevated
by small localized deposits of amyloid in the skin, bladder yellow nodules.
and respiratory tract. Symptoms: There are difficulties in chewing, swallowing
Systemic amyloidosis it can occur in various form. or talking. The speech difficulties is due paresis of the
They are as follows: vocal cord resulting from deposit of amyloid in the upper
∙ Primary amyloidosis: There is no evidence of third of larynx.
preceding or existing disease.
∙ Amyloidosis associated with multiple myeloma. Sign: Amyloid deposition of tongue will result in
∙ Secondary amyloidosis: Associated with variety of macroglossia of tongue. It is seen in both primary and
chronic inflammatory disease. secondary form. Tongue is enlarged and studded with
∙ Familial or heredofamilial amyloidosis: It is rare small garnet colored enlargements along with nodes of
condition such as familial Mediterranean form or cheeks and lips. Mobility of the tongue is decreased.
familial amyloidosis with polyneuropathy. Yellowish nodules are present along the lateral border
∙ Hormone related amyloid: It is associated with tumors of the tongue and impression from the teeth is also visible.
of endocrine cells which secretes peptide hormones. The gingiva may be infiltrated and may be bluish,
∙ Hemodialysis associated amyloidosis: Patient who spongy and hypertrophied. Xerostomia may result from
has undergo long term renal dialysis are susceptible to salivary gland involvement.
amyloidosis.
Etiology There is extracellular deposition in the submucosal
It is caused by collagen diseases like rheumatoid arthritis, connective tissue of amorphous eosinophilic material in the
chronic infections like tuberculosis, osteomyelitis, regional gingiva. This material present is arranged in perivascular
enteritis and ulcerative colitis and malignant diseases like orientation or diffusely. Congo red stain should be used for
multiple myeloma, Hodgkin’s lymphoma and renal cell staining which has got affinity for the abnormal protein. In
carcinoma. this staining amyloid occur red (Fig. 32.2).
Classification
∙ Erythropoietic porphyria—there is excessive abnormal
porphyrins formation in developing erythrocytes. It is 803
localized to bone marrow.
∙ Uroporphyria
∙ Protoporphyria
∙ Hepatic porphyria—in it liver is the site of excessive
porphyrin formation.
∙ Acute intermittent porphyria
∙ Porphyria variegate
∙ Porphyria cutaneous tarda
∙ Hereditary coproporphyria.
Clinical Features
It is transmitted as non-sex linked recessive character and
both sexes are equally affected.
Figure 32.2 Amyloid deposit seen in histopathological Symptoms: The first sign is excretion of red urine
section of tongue containing uroporphyrin which may be noted at birth or
apparent at first two years after birth. Excessive deposition
of the excess porphyrin in the skin lead to photosensitivity.
Diagnosis It is absent in the neonatal period but may become apparent
Scintiscanning with Tc99m to localized soft tissue depo- during first few years after it becomes exposed to sunlight.
sits. Congo red is used to diagnose amyloid which shows Signs: Vesicular and bullous eruption appears on face,
birefringence and dichroism. back and hand, i.e. exposed parts. Vesicle contains a
serous fluid which exhibits red fluorescence. Ruptured
Management
vesicle heals slowly and leaves depressed pigmented scars.
You can treat amyloidosis by alkylating agents like There is often coexisting anemia. In this disease there is
melphalan. Combination therapy using melphalan, abdominal crisis and psychological or metal symptoms.
prednisone and fluoxymesterone reported significant There is demyelination of nerve fibers.
improvement.
Renal transplantation may be indicated. Oral Manifestations
The porphyrin has an affinity for calcium phosphate and
Points to Remember
due to this, deposition of porphyrin occur in dentine.
Amyloid disease, caused by collagen diseases, fatigue, Deciduous and permanent teeth show red or brownish
ankle edema, purpuric spots, congestive cardiac failure, discoloration which under ultraviolet light exhibits red
superficial waxy lesion, macroglossia of tongue, yellowish fluorescence due to incorporation of porphyrins during
nodules, infiltrated bluish gingiva, xerostomia, amorphous development. Bullous, erosive lesions of oral mucosa
eosinophilic material, perivascular orientation, Congo red may be present. There is also atrophic cheilitis, advanced
stain, melphalan, prednisone, renal transplantation. periodontal diseases.
Porphyria Histopathological Features

It is a group of disorders in which there is defective There is subepidermal blister with minimal cell-poor
metabolism of pyrrole compound. There is excessive dermal inflammatory infiltrate.
concentrations of porhyrins exposed to day-light generate
Management
free radicals, leading to cell membrane damage and cell
death. About 1 percent of acute attacks of porphyria may be fatal.
For severe pain, opiates are safe—that is, non- so now this condition is designated as Langerhans cell
porphyrogenic. Pethidine, morphine, or diamorphine can histiocytes. Langerhans cells are dentritic mononuclear
be given. cells normally found epidermis, mucosa, lymph nodes and
804 Chlorpromazine may be helpful to promote relaxation bone marrow. It is of three types:
and sleep. – Hand-Schuller-Christian disease (disseminated
histiocytosis X): Disease involving bone, skin and
Points to Remember viscera.
Pyrrole compound, excretion of red urine, photosensi- – Eosinophilic granuloma of bone (chronic localized
tivity, vesicular bullous eruption on face, back, red histiocytosis X): Solitary or multiple bone lesion
fluorescence, demyelination of nerve fibers, red or without visceral involvement.
brownish discoloration of teeth, bullous, erosive lesions – Letterer Siwe disease (acute disseminated histocy-
of oral mucosa, atrophic cheilitis, cell-poor dermal tosis X): Disease with prominent cutaneous, viscer-
inflammatory infiltrate, opiates and chlorpromazine. al, bone marrow involvement seen in infants.
∙ Lipid reticuloendothelioses: It is disturbance in
sphingomyelin and glucosyl ceramide metabolism.
DISTURBANCES IN LIPID Affected person lack certain enzyme which is required
METABOLISM for processing of specific lipids, this in turn results in
accumulation of the lipids within the cells.
Lipids are a heterogeneous group of organic compounds
∙ Gaucher’s disease
which are relatively insoluble in water but, soluble in
∙ Niemann-Pick disease
solvent such as ether, chloroform and benzene.
∙ Tay Sachs disease.
Types of Lipid Hand-Schuller-Christian Disease
• Simple lipids It is also called multifocal eosinophilic granuloma
• Compound lipids ‘chronic disseminated histiocytosis X or xanthomatosis. It
• Derived lipids. is characterized by widespread skeletal and extraskeletal
lesions and chronic clinical course. It is result of error in
Types of Lipid the metabolism of cholesterol and its esters.
∙ Simple lipids: They are esters of fatty acids with Clinical Features
various alcohols, i.e. natural fats and waxes.
It is more common in boys than girls 2:1. Occur in early
∙ Compound lipids: They are esters of fatty acids
life usually before age of fifteen.
containing groups other than and in addition to an
alcohol and fatty acids. Classic Triad
∙ Derived lipids: They are derivatives obtained by
∙ Single or multiple areas of punched out bone destruction
hydrolysis of the simple and compound lipids, which
in skull.
still posses the general characteristics of lipids.
∙ Unilateral or bilateral exophthalmos.
∙ Miscellaneous: It includes carotenoids, vitamin E and
∙ Diabetes insipidus.
K.
Otitis media and skin may sometime exhibit papular or
nodular lesion. Course is chronic with numerous remissions
Classification
and exacerbation.
Histiocytosis X: It is also called non-lipid reticulo-
endothelioses, idiopathic histocytosis and Langerhans cell Oral Manifestations
disease. It is a inflammatory reticuloendothelioma condition There is involvement of facial bone which is commonly
with evidence suggesting that it may be reaction to some associated with soft tissue swelling and tenderness causing
type of infection. There is pathological accumulation of facial asymmetry.
histiocytes and eosinophilic leukocytes. Nowaday cells Sore mouth with or without ulcerative lesion, halitosis,
present in the lesion is identified as Langerhans cells, gingivitis and suppuration is present.
An unpleasant taste, loose and sore teeth with precocious

exfoliation and failure of healing of tooth socket following
extraction.
Loss of supporting alveolar bone mimics advanced 805
periodontal disease.
Proliferative histiocytic phase: Accumulation of
collection of leukocytes (eosinophilic) scattered throughout
the sheets of histiocytes.
Vascular granulomatous phase: Persistence of histiocytes
and eosinophils sometimes with aggregation of lipid laden
macrophages.
Diffuse xanthomatous phase: Abundance of foam cells. Figure 32.3 Histiocytosis X-ray showing radiolucent lesion in
Fibrous or healing phase: Langerhans cells contain rod the skull
shaped cytoplasmic structure called Birbeck granules.
Clinical Features
Laboratory Findings
It occurs primarily in older children and young adults and
Anemia and less frequently leukopenia and thrombocy- proportion of male to female is 2:1. Skull and mandible
topenia occur. Serum cholesterol level is normal but tissue are common site but femur, humerus, ribs may be affected.
cholesterol level is raised.
Symptom: There may be local pain which may be dull and
Management steady, swelling and tenderness. General malaise and fever
Prognosis of this disease is good and half of the patients may accompany the eosinophilic granuloma of bone.
undergo spontaneous remission over a period of a year. It Signs: Lesion is destructive and well demarcated, roughly
is usually treated by curettage or excision of lesion. The round or oval in shape (Fig. 32.3). The area destroyed is
lesions which are inaccessible are treated by irradiation. replaced by soft tissue. Tissue of early lesion is soft and
Some patients may be given drug treatment like brown and since there is no necrosis, it is not friable. Later
prednisolone, vinblastine and cyclophosphamide. it become fibrous and grayish.
Points to Remember Oral Manifestations

Multifocal eosinophilic granuloma, triad of single It may occur in jaws and overlying soft tissues. There is
or multiple areas of punched out bone destruction in loss of superficial alveolar bone often mimicking juvenile
skull, unilateral or bilateral exophthalmos, and diabetes periodontitis. Gingivitis and bleeding gingiva, pain or
insipidus, facial asymmetry, ulcerative lesion, halitosis, ulceration is present. Loosening and sloughing of teeth
precocious exfoliation, advanced periodontal disease, often occurs after destruction of alveolar bone. Sockets fail
proliferative histiocytic phase, vascular granulomatous to heal normally.
phase, diffuse xanthomatous phase, fibrous or healing
phase, Birbeck granules, anemia, curettage, irradiation. Histopathological Features
Primary cell is histocyte which grows in sheets and sheet
Eosinophilic Granuloma like structures. It may coalesce to form multinucleated giant
This term was introduced by Lichenstein and Jaffe in 1940. cells. When fibrosis occurs eosinophils may disappear.
It is also called unifocal eosinophilic granuloma. It is the
lesion of bone which is primarily histiocytes proliferation Management
with an abundance of eosinophilic leukocytes by no intra It is usually treated with curettage and X-ray therapy.
or extracellular lipid accumulation. Symptoms usually subside within 2 weeks after treatment.
Points to Remember Gaucher Disease

Local pain, general malaise, fever, destructive lesion, It is most common of lipid reticuloendothelioses. It
806 friable tissue, juvenile periodontitis, bleeding gingiva, has got familial tendency and is thought to be due to
loosening of teeth, histocyte, multinucleated giant cells, faulty metabolism of the lipoid, kerasin. The reticular
curettage, X-ray therapy. cells and histiocytes are increased in number and many
of them become infiltrated with kerasin. It is autosomal
recessive.
Letterer Siwe Disease
In this disease there is lack of glucocerebrosidase
It is an acute fulminating disease, which invariably occurs which results in accumulation of glycosylceramide within
in infants usually before the age of 3 years. the lysosomes of cells of macrophage and monocytes
lineage.
Clinical Features
It is fulminating condition that most often occurs in infants Types
younger than 1 year of age.
• Type I (chronic non-neuropathic)
Symptoms: Initial manifestation is skin rash involving • Type II (acute neuropathic)
trunk, scalp and extremities. Rash may be erythematous, • Type III (subacute neuropathic).
purpuric and ecchymotic, some time with ulceration.
Patient may have persistent low grade spiking fever with Types
malaise and irritability.
∙ Type I (chronic non-neuropathic): There is no
Signs: Splenomegaly, hepatomegaly and lymphadenopathy cerebral involvement.
is common. Nodular and diffuse involvement of visceral ∙ Type II (acute neuropathic): It is characterized
organs particularly in lung and GI tract. Soft tissue and bony by hepatosplenomegaly and central nervous system
granulomatous reaction, hemorrhage, anemia, failure to thrive. disorders.
∙ Type III (subacute neuropathic): It resembles type II
Oral Manifestations
but later onset and more protracted clinical course.
Enlargement of the gingival tissue occurs. There may be
presence of ulcer on the oral mucosa. Diffuse destruction Clinical Features
of bone of maxilla and mandible which may result in
loosening and premature loss of teeth. It is mostly seen in adults and progress is slow.
Signs: There is enlargement of the lymph nodes, spleen and
Histopathological Features to a lesser extent the liver. There is hepatosplenomegaly,
Same as others but without foam cells and no fibrosis CNS involvement.
occurs.
Symptoms: There may be bone pain due to changes in
Laboratory Findings bone marrow. There is often bleeding from the nose or
form the gums. Teeth extraction from the affected area may
Progressive anemia is often present as well as leukopenia
result in bleeding complication.
or thrombocytopenia.
Pingueculae: The skin is sometimes pigmented and
Management the conjunctival fibrous tissue may be thickened
It has got very poor prognosis and it usually terminates and of brownish discoloration, a condition known as
fatally in short period of time. ‘pingueculae’.
Erlenmeyer flask deformities are seen in long bone
Points to Remember which is characteristic features of disease.
Erythematous purpuric rash, spiking fever, splenomegaly, Oral features: There is ill defined radiolucency which
hepatomegaly, gingival enlargement, diffuse destruction can be seen in mandible without causing devitalization of
of bone of maxilla and mandible, progressive anemia, the teeth. Lamina dura is intact in this case. There is also
poor prognosis. decrease salivary flow.

Examination of biopsy of spleen and liver show typical It involves lungs, liver and the nervous system. There is
Gaucher cells (Fig. 32.4) which is round pale cell, hepatosplenomegaly, retarded physical and mental growth 807
containing a small eccentric nucleus and wrinkled or and severe neurological disturbance.
crumpled silk cytoplasm. When the bone is involved
the bone marrow shows numerous large foamy, slightly Histopathological Features
granular cells with small round pyknotic nuclei, which are There is presence of foam cells, usually an enlarged
Gaucher cells. reticuloendothelial cell whose cytoplasm contains
numerous droplets like inclusion of lipid. Characteristic
Management sea blue histiocytes in bone marrow aspiration.
The prognosis of malignant infantile form is very poor;
Management
the disease results in death usually within the first year.
Administration of purified macrophage targeted glucocere- It is only symptomatic to control infection. Death usually
brosidase, an enzyme replacement therapy can exhibits im- occurs within 3 years of age. Enzyme replacement therapy
provement in anemia, in plasma glucocerebrosidase level and organ transplantation is carried also carried out.
and decrease in hepatosplenomegaly.
Points to Remember
Points to Remember Sphingomyelin lipidosis, hepatosplenomegaly, severe
Lymph nodes enlargement, bone pain, Pingueculae, Er- neurological disturbance, foam cells, sea blue histiocytes,
lenmeyer flask deformities, ill defined radiolucency in enzyme replacement therapy, organ transplantation.
mandible, Gaucher cells, wrinkled or crumpled silk cy-
toplasm, purified macrophage targeted glucocerebrosi- Tay Sachs Disease
dase.
It is caused by lack of hexosaminidase A. It results in
accumulation of ganglioside within lysosomes of neurons.
Niemann-Pick Disease
It is also called sphingomyelin lipidosis. It is characterized Clinical Features
by an abnormal storage of phospholipids due to lack of It may be mild or severe in nature. In severe form neuronal
sphingomyelinase. It is present in four forms, i.e. type A, degeneration will occur. There is also blindness, intractable
B, C and D. Type A and B caused by deficiency of acid seizures.
sphingomyelinase and type C and D occur due to mutation
of NPC-1 gene involved with cholesterol processing. Histopathological Features
They are same as seen in other lipid reticuloendothelioses.
Management
Genetic counseling results in marked decrease in affected
patient in last 3 decades.
Points to Remember
Lack of hexosaminidase, neuronal degeneration,
blindness, genetic counseling.
DISTURBANCES IN CARBOHYDRATE
METABOLISM
Hurler’s Syndrome
It is disturbance of mucopolysaccharide metabolism,
Figure 32.4 Gaucher cells which is characterized by elevated mucopolysaccharide
excretion ratio and an excessive intracellular accumulation

of chondroitin sulfate B and heparin sulfate in those tissue
and organ where they are normally found. It is inherited as
808 an autosomal recessive trait.
Clinical Features
Age: It usually becomes apparent within first two years of
life, progresses during early child hood and adolescence
and terminates in death before puberty.
Facial features: Head is large, prominent forehead, broad
saddle nose and wide nostrils, hypertelorism and puffy
eyelids with coarse bushy eyebrows (Fig. 32.5). There is
also nasal congestion and noisy breathing.
There is progressive corneal clouding, hepatos- Figure 32.6 Claw hand in Hurler syndrome
plenomegaly resulting in protuberance of abdomen.
A short neck and spinal abnormalities are typical, while body including liver, spleen, reticuloendothelial system,
flexion contractures result in the claw hand (Fig. 32.6). nervous system, cartilage, bone and heart.
Oral Manifestations Abnormal deposits are found in many sites with
involved fibroblasts assuming the appearance of clear
Shortening and broadening of mandible with prominent (gargoyle) cells.
gonions and wide intergonial distance.
There is typical spacing of teeth. Teeth are small and Laboratory Finding
misshapen. There may be gingival hyperplasia, thick lip, There is elevated level of mucopolysaccharides in the
large tongue, and open mouth. urine.
Histopathological Features Management
There is excessive accumulation of intracellular muco- Death usually occurs before the age of ten due to pneumonia
polysaccharides in many tissues and organs throughout the and cardiac failure.
Points to Remember
Prominent forehead, broad saddle nose, corneal clouding,
hepatosplenomegaly, claw hand, spacing of teeth,
prominent gonions, intracellular mucopolysaccharides,
gargoyle cells, death due to pneumonia.
Lipoid Proteinosis
It is discussed in skin disorders.
Hereditary Fructose Intolerance

It is transmitted as autosomal recessive trait. It results from
a deficiency in fructose 1-phosphate aldolase.
Clinical Features
It is manifested by hypoglycemia and vomiting after
ingestion of fructose containing foods. Affected individuals
Figure 32.5 Hurler syndrome rapidly acquire an intense aversion to all sweets and fruits.
Oral Manifestations Different Types of Glycosaminoglycans

There are fewer incidences of caries in these individuals. • Heparan sulfate
• Dermatan sulfate 809
Points to Remember • Keratin sulfate
Deficiency in fructose 1-phosphate aldolase, hypogly- • Chondroitin sulfate.
cemia, vomiting, fewer caries.
Clinical Features
Hypoglycemia Sign and symptoms: Most types of mucopolysaccharidosis
It is a subnormal blood glucose level. syndrome show mental retardation, heavy brow ridges and
stiff joint.
Causes Cloudy degeneration of corneas which can lead to
It may be caused by delayed meal, inadequate total caloric blindness is also seen in many syndromes.
intake, unusual physical exertion, insulin overdose, and
Oral features: There is macroglossia, gingival hyperplasia.
oral hypoglycemic agents.
There is also thin enamel with pointed cusps on posterior
Clinical Features teeth.
Symptoms: Sudden onset of hunger, sweating, shakiness, Syndrome associated: There are various syndrome
tremor, anxiety, restlessness, faintness, weakness, nausea, associated with mucopolysaccharidosis. These are Hurler,
palpitations. Scheie, Hunter, Sanfilippo-A, Sanfilippo-B, Morquio-B,
Progression to mental confusion, bizarre behavior, Morquio-A, Maroteaux-Lamy.
personality change, reduced level of consciousness, loss of
consciousness, seizures. Management
Life of patient may be increase by allogenic bone marrow
Signs: Pulse rapid, blood pressure may be elevated and
transplantation.
moderate hypothermia may be present due to sweating,
Enzyme replacement therapy is also available nowaday.
hyperventilation and vasodilatation. Bizarre or aggressive
behavior may be present, staggering gait, may appear
drunk, cold, clammy skin.
DISTURBANCES IN MINERAL
METABOLISM
Laboratory Findings
Acrodermatitis Enteropathica
Blood glucose reduced.
It is also called zinc deficiency. Rare disease of infancy and
Management childhood, transmitted as autosomal recessive character.
Since supplements appear to be curative even pathogenesis
Glucagons consult physician, ensure that the airway is
of the characteristic lesions involves a number of etiologic
patent and protected and that ventilation is adequate in the
factors.
patient with a reduced level of consciousness.
Points to Remember
Age: It is usually occur in childhood after weaning.
Hunger, shakiness, faintness, rapid pulse, elevated blood
The primary signs of the disorder are skin lesion, hair
pressure, staggering gait, reduced blood glucose.
loss, nail changes and diarrhea.
Erythematous, pustular, moist erosions of the orofacial
Mucopolysaccharidosis areas occur as an early manifestation.
It is heterogeneous group of metabolism which are In fully developed conditions the buttocks, elbows,
inherited as autosomal recessive fashion. It is cause by lack fingers and toes are affected by vesiculo-bullous rash
normal enzymes needed to process important intercellular similar to that affecting the orofacial region.
substance called glycosaminoglycans which used to known Retarded body growth and mental changes also occur
as mucopolysaccharidosis. with some frequency.
Oral Manifestations Clinical Features

Site: The buccal mucosa, palate, gingiva and tonsils. Perinatal type: Death in this patient occur due to
810 Large number of children suffer from candidiasis. The respiratory failure. There is also marked hypocalcification
perioral area usually being affected resulting in weeping of the skeletal structure.
erosions, angular fissuring and spreading dermatitis.
Infantile form: Severe hypocalcemia, bone abnormalities
There may be numerous small whitish papillomas on
and failure to thrive manifest the infantile form. Patient may
the buccal mucosa and borders of the tongue.
complaint of vomiting and hypotonia. Most of the cases are
The oral changes some time described as stomatitis,
lethal. Deformities of rib lead to pneumonia. Skull defect
glossitis and stomatitis producing thrush like picture.
can leads to increase intracranial pressure.
Buccal mucosa is present with red and white spots,
erosions, ulcers and desquamation. Lesion on the tongue is Juvenile form: Hypophosphatasia of childhood is
some time papillated and halitosis is often severe. characterized by increased infection, growth retardation and
rochite like deformities including deformed extremities,
Management costochondral junction enlargement (rochite rosary) and
220 mg of zinc sulfate three times daily produces pulmonary gastrointestinal and renal disorders.
remarkable improvement. Adults form: The adult form includes fracture with a prior
history of rickets and osseous radiolucency.
Points to Remember
Zinc deficiency, hair loss, erythematous erosions Skull: Skull suture close early resulting in bulging suture
of orofacial areas, vesiculobullous rash, candidosis, and grayal marking on internal surface of skull and
weeping erosions in perioral area, stomatitis, glossitis, brachycephalic shape.
stomatitis producing thrush like picture, zinc sulfate.
Oral Manifestations
There is almost total lack of cementum and normally
Hypophosphatasia
attached periodontal fiber leading to poor support and
It is a hereditary disease transmitted as a recessive premature loss of teeth in deciduous teeth (Fig. 32.7).
autosomal characteristic. There is deficiency of serum There is also delayed eruption of permanent teeth. Teeth
alkaline phosphate. It resembles rickets. There is low may be hypoplastic. The pulp chamber and root canal are
level of serum alkaline phosphatase activity and elevated sometimes larger than normal. The roots either fails to
urinary excretion of phosphorylethanolamine which result develop fully or undergo early resorption of the apices.
in formation of defective bone matrix.
Types
• Perinatal
• Infantile type
• Juvenile or childhood type
• Adult type.
Types
∙ Perinatal: It is present at birth and infant rarely survives
for more than few hours.
∙ Infantile type: They appear normal until 6 months of
age then there is sign of failure to grow.
∙ Juvenile or childhood type: It appear late and there is
wide range of clinical manifestation.
∙ Adult type: It very mild type. Figure 32.7 Hypophosphatasia with intraoral manifestation
The alveolar bone which support the teeth fail to level and occasional tetany. This disturbance in calcium
develop normally which result in premature loss of primary metabolism may result in osteoporosis and other skeletal
teeth. There is inflammation of the gingiva. anomalies.
811
Radiographic Features Symptom: It usually begins with intestinal disturbances
including diarrhea, constipation and flatulence. Nervous
Radiographic features are seen in infantile forms are irritability, numbness and tingling of the extremities occur;
reduce degree of ossification with preponderance of malaise and generalized weakness are also common.
hypomineralized osteoid.
In case of juvenile form skull has got beaten copper Sign: The skin changes include irregular brownish
appearance which shows uniform, poorly defined small pigmentation particularly on the face, neck, arms and legs
radiolucency. This pattern occurs as there is thinning of and drying of skin with scaly eruptions.
inner cortical plate due to cerebral gyri.
Oral Manifestations
Histopathological Features There may be severe glossitis with atrophy of filiform
There is abundant production of poorly mineralized osteoid. papilla, although the fungiform papillae persist for some
There may be increase amount of woven bone, which is of time on the atrophic surface.
less mature in nature than a osseous tissue. Painful burning sensation of the tongue and oral
Exfoliated teeth show absence of cementum on the root. mucosa are common. There are small projections which
are pink or red in color and the erythematous swelling and
Management palatal lesions appear as multiple aphthous ulcers.
Treatment is usually symptomatic as root cause of this
condition cannot be treated. Prosthetic appliance are Management
indicated to replace the missing teeth. Administration of vitamin B12 and folic acid is done.
Diet must be carefully supervised and supplemented with
Points to Remember vitamins and minerals.
Hypocalcification of the skeletal structure, severe
hypocalcemia, hypotonia, rochite like deformities (rochite Points to Remember
rosary), skull suture close early, grayal marking, premature
Sprue, occasional tetany, osteoporosis, diarrhea,
loss of teeth in deciduous teeth, hypoplastic teeth,
constipation, brownish pigmentation of skin, severe
inflammation of the gingiva, reduce degree of ossification,
glossitis, atrophy of filiform papilla, burning sensation
beaten copper appearance, poorly mineralized osteoid,
of the tongue, aphthous ulcers, vitamin B12.
woven bone.
Disorders of Vitamins
MISCELLANEOUS DISORDERS
Vitamins are organic substances in food which are required
Malabsorption Syndrome in small amounts but which cannot be synthesized in
It is also called sprue, idiopathic steatorrhea, celiac adequate quantities in the body and which are soluble in
disease. It includes conditions causing poor digestion or either fat or water. Vitamins are needed in small quantities
absorption to a variable degree of a number of nutrients, to act as a cofactor in a variety of metabolic reactions. Your
fats, proteins, carbohydrates, vitamins, minerals and water. body needs only small amounts of vitamins. But because
The defective absorption may be due to defective digestive what the body manufacture is often not enough, these must
or defective intestinal absorption. be obtained from your diet and from supplements.
Vitamins occur in a natural and in a physiologically
Clinical Manifestations inactive form and are called provitamins. They become
Excessive amounts of fat are passed in stools, inducing activated only after conversion within the animals. For
a concomitant excessive loss of calcium which in turn example, vitamin A exists in plants in the form carotene,
causes calcium deficiency with ensuing low blood calcium which is activated in the liver.
Small amounts of vitamins can be synthesized is limited to about 5 mg per day. It is phosphorylated by
endogenously. For example vitamin D is synthesized from the liver and kidneys. In tissues, it is found as thiamin
a precursor steroid, niacin from tryptophan which is an pyrophosphate. Thiamin pyrophosphate is a coenzyme for
812 essential amino acid, vitamin K and biotin by intestinal decarboxylation of pyruvate to acetyl coenzyme A. Any
microflora. excess supply of thiamine is excreted in the urine.
Deficiency of vitamins may be primarily due to vitamin
deficient diet or secondarily because of disturbances in Functions in the Body
intestinal absorption, transport in blood, tissue storage or Growth: It promotes growth, protects heart muscle and
metabolic conversion. stimulates brain action.
Causes of Vitamin Deficiency Nervous system: It plays an important role in the normal
functioning of the entire nervous system.
∙ Decreased amount of intake of essential nutrients.
∙ Impaired absorption from the alimentary tract. Digestion: It aids in digestion especially that of
∙ Increased metabolism due to rapid growth. carbohydrates.
∙ Inadequate storage, fever and pregnancy. Diuretic: It is a mild diuretic and it increases urine
Water Soluble Vitamins formation.
Water-soluble vitamins are found in yeast, grain, GIT: It improves peristalsis and helps prevent constipation.
rice, vegetables, fish and meat. They are essential co- Blood cells: It maintain the normal blood count and
enzymes required in energy releasing mechanisms and in improves circulation.
hemopoiesis. They also act as co-enzymes for metabolism
of proteins, carbohydrates and fats. Others: It also reduces fatigue, increases stamina, prevent
premature aging and senility by increasing mental alertness
B-complex Vitamins and promotes a healthy skin.
Most of B-complex occurs in nature in the bound form Deficiency Symptoms
within the cells of vegetables or animal tissues. The
digestion for the liberation of vitamins and its absorption Nervous disorders: When cells cannot metabolize glucose
is a result of breakdown of cellular structures in the gut. aerobically, it affects the nervous system first since it
Vitamin B-complex is not stored in appreciable amounts in depends entirely on glucose for its energy requirements.
the body tissues except vitamin B12. Excretion of vitamins There is mental depression, nervous exhaustion and
occurs in the kidney. insomnia.
The oral signs of deficiency of vitamin-B occur in the Digestive symptoms: It occurs due to defective
oral tissues like tongue, mucous membrane and gingiva. It hydrochloric acid production in the stomach. Patient
may result in dermatitis, stomatitis and gastritis and blood complains of loss of appetite, poor digestion, chronic
and bone marrow disorders. Degenerative changes of brain constipation and loss of weight.
and nerves are also a characteristic feature of deficiency
since nerve tissue depends on glucose. In hemopoiesis, Heart: There is accumulation of pyruvic acid and lactic
vitamin B12 and folate are essential for maturation of red acid derived from it, which produces vasodilatation and
cell precursors. increases cardiac output. The heart muscle becomes lazy
and fatigued and the auricles or the upper chambers of the
Thiamine (Vitamin B1) heart lose their strength and it gradually enlarges. It may
lead to a condition known as hypertrophy of the heart.
It is a vitamin for calm nerves. It is also known as aneurin.
It was discovered by Eijkman in 1897. It is a colorless basic Beriberi: Prolonged gross deficiency can cause beriberi.
organic compound composed of a sulfated pyrimidine ring. There are three types of beriberi:
∙ Wet beriberi
Absorption and Excretion ∙ Dry beriberi
It is readily absorbed from both small and large intestine. ∙ Infantile beriberi
The capacity of the human intestine to absorb this vitamin – Other diseases, which can be associated are
∙ Wernicke’s encephalopathy Management

∙ Peripheral neuritis
Thiamine 50 mg IM for 3 days then 10 mg 3 times daily
∙ Korsakoff’s psychosis
by oral route. 813
Types of Deficiency of Thiamine Dry Beriberi
• Wet beriberi
Clinical Features
• Dry beriberi
• Infantile beriberi It is a peripheral neuropathy. In long standing cases, there
• Wernicke’s encephalopathy is degeneration and demyelination of both sensory and
• Peripheral neuritis motor nerve fibers resulting in severe wasting of muscles.
• Korsakoff’s psychosis. Blood pyruvate levels are normal.
Oral Manifestations
Beriberi There is hypersensitivity of oral mucosa. Pain in the tongue,
Wet Beriberi teeth, jaws and face.
It is marked by cardiac dilation with four chamber
Wernicke’s Encephalopathy
enlargement, pallor and flabbiness of myocardium.
It is commonly seen in alcoholics with persistent vomiting.
Etiology There is a classical triad of ocular abnormalities, ataxia
Diet: It is caused due to eating diets in which calories are and confusion. There are facial symmetrical areas of
derived from polished rice. grayish discoloration. There is also bilateral symmetrical
ophthalmoplegia and ataxia.
Alcoholics: It is commonly seen in chronic alcoholics due Histologically, there is hypertrophy and hyperplasia of
to their poor nutrition in general and also because alcohol small blood vessels.
interferes with intestinal absorption of thiamine. Injection of thiamine should be given. 50 mg by slow
Others: It is often precipitated by infection, pregnancy and intravenous injection followed by 50 mg daily by oral route
lactation. for a week.
Pathogenesis Korsakoff’s Psychosis

Deficiency of thiamine, incomplete metabolism of glucose, In it, there is a predominant abnormality in mental function
accumulation of pyruvic acid and lactic acid in tissue and which is memory defect. There is profound impairment of
body fluid, dilation of peripheral blood vessels, fluid may memory recall and new learning ability.
leak out through capillaries, producing edema, high cardiac
output, heart dilation. Points to Remember
Pain in legs, tachycardia, increased blood pressure,
Clinical Features swelling of the myofibrils, peripheral neuropathy,
Symptoms: Pain in legs after walking due to accumulation hypersensitivity of oral mucosa, ocular abnormalities,
of lactic acid. ataxia and confusion, impairment of memory recall,
Cardiac signs: There is tachycardia and increased blood hyperplasia of small blood vessels.
pressure, cardiomegaly, increased JVP and palpitations.
There is also presence of sinus tachycardia and inverted Riboflavin (Vitamin B2)
T waves. It is also called the beauty vitamin. It is a yellowish green
Skin is warm due to vasodilation. Edema may develop fluorescent compound soluble in water. The word riboflavin
rapidly to involve leg, face and trunk. is derived from two sources ribose, referring to ribose sugar
found in several vitamins and enzymes and flavin meaning
Histopathological Features yellow. It is an essential component of coenzyme flavin
There is interstitial myocardial edema, swelling of the mononucleotide and flavin adenine dinucleotide, involved
myofibrils. mainly in a wide variety of oxidation-reduction reactions.
It is stable to boiling in an acidic solution. It is decomposed Oral Manifestations

by heat. It is also destroyed by ultraviolet light.
Tongue: Glossitis which begins with soreness of lip and
814 Absorption and Excretion lateral margins of the tongue. Filiform papillae become
atrophic while fungiform papillae remain normal or
It is readily absorbed from the intestinal tract and is become engorged and mushroom shaped giving the tongue
phosphorylated in the wall of the intestine. It is carried a reddened coarsely granular appearance. In severe cases,
to the tissue of the body and incorporated into the cells the tongue becomes glazed and smooth due to complete
enzymes. It is stored in liver, kidneys and heart. Riboflavin atrophy of the papillae and exhibits a magenta color.
is excreted primarily in the urine and bile and sweat are
other minor routes of excretion. Lip: Lips become red and shiny because of desquamation
of epithelium. Paleness of lips and cheilitis which is seen as
Function in the Body maceration and fissuring at the angle of the mouth.
It is essential for growth and general health. There is maceration at angle of mouth with pain on the
Metabolism: It is involved in the metabolism of opening mouth, it again results in fissuring and cracking
carbohydrates, fats and proteins. It is essential for normal with ulceration. As the disease progresses, angular cheilitis
tissue maintenance. spread to the cheek, the tissues bleed easily and are painful
if secondarily infected (Fig. 32.8).
Nervous system: It helps in functioning of the nervous
system. Management
Digestion: It helps in digestion and prevents constipation. Riboflavin 25,000 to 50,000 mcg is given daily in divided
doses.
Eyes: It alleviates eye strain and it is helpful in counteracting
the tendency toward glaucoma. Points to Remember
Others: It promotes a healthy skin, nails hair and strengthens Beauty vitamin, scaly gray dermatitis, corneal
the mucous lining of the mouth, lips and tongue. vasodilatation, photophobia, dull or oily hair, glossitis,
lips become red, maceration at angle of mouth.
Causes of Deficiency
It occurs due to inadequate diet and also inadequacy of Niacin (Vitamin B3)
other essential nutrients including vitamins and proteins.
It is also known as nicotinic acid. Niacin is required for
Secondary deficiency: It may occur due to diseases
the formation of coenzyme NAD and NADP, which are
of the intestinal tract. Prolong use of psychological drug
important pyridine nucleotides which play an important
that interfere with production of flavin monophosphate.
Chronic alcoholism, burns and trauma.
Deficiency Symptoms
It affects the nasolabial fold and ala of the nose which
exhibits a scaly gray dermatitis and consists of enlarged
follicles around the side of the nose which is plugged with
dry sebaceous material.
Ocular changes: It consists of corneal vasodilatation,
photophobia and superficial interstitial keratitis. There
may be itching and burning of the eyes.
Skin and nails: It may also result in dull or oily hair, an
oily skin, premature wrinkles on the face and arms and split
nails.
There is also malfunctioning of adrenal glands, anemia,
vaginal itching and cataract. Figure 32.8 Angular cheilitis in riboflavin deficiency
role in redox reactions involving carbohydrate, protein and

lipid metabolism. Deficiency of niacin leads to a disease
called pellagra which means rough skin.
815
Function in the Body
Nervous system: It is important for proper blood circulation
and healthy functioning of the nervous system.
Gastrointestinal tract: It is essential for the proper
metabolism of proteins and carbohydrates.
Blood vessels: It dilates the blood vessels and increases the
flow of blood to the peripheral capillary system.
Hormone: It is essential for the synthesis of sex hormone,
estrogen, progesterone and testosterone as well as cortisone,
thyroxin and insulin. Figure 32.9 Dermatitis seen in patient with pellagra
Others: It helps to maintain a normal healthy skin.

extend through the gastrointestinal tract. Diarrhea is caused
Pellagra by atrophy of gastric epithelium followed by submucosal
Causes of Deficiency inflammation which is then followed by ulceration.
Tryptophan deficiency: If insufficient tryptophan is Nervous system: Delirium is the most common mental
available for synthesis of niacin. disturbance in the acute form and dementia in chronic
cases. There is also loss of appetite, irritability and burning
Diet: Dietary deficiency of niacin. High dietary levels of sensation in different areas of the body.
amino acid lucine antagonize the synthesis of NAD and
NADP. Oral Manifestations
Miscellaneous: Chronic alcoholism, diarrhea and carcinoid Entire oral mucosa becomes fiery red and painful and
syndrome. salivation is profuse.
Clinical Features Tongue: The epithelium of the entire tongue is

desquamated. The filiform papillae are most sensitive
It can be developed in 3 weeks with prodromal symptoms and disappear first; the fungiform papillae may become
of loss of appetite, vague gastrointestinal disturbances and enlarged. The tongue becomes red swollen and beefy and
numbness or burning in various locations. in animals the deficiency leads to black tongue. In early
It is called disease of 3-Ds stages, only the tip and margins of the tongue are swollen
Dermatitis and red. In advanced cases, the tongue losses all the
Diarrhea papillae and the reddening becomes intense. In this stage,
Dementia the tongue becomes so swollen that indentation from the
Skin: There is an erythema resembling severe sunburns teeth are found along the borders of the tongue. The mouth
appears symmetrically overall parts of the body exposed is sore and shows angular stomatitis, cheilitis.
to sunlight and especially on the neck (Fig. 32.9). Affected Symptoms: Tenderness, pain and ulceration begin at the
area is well demarcated from the normal. In acute cases, interdental papillae and spreads rapidly. Superimposed
skin lesions may produce vesiculation, cracking, exudation, ANUG or Vincent’s infection involving the gingiva,
crusting with ulceration and secondary infection. In chronic tongue and mucosa is common.
cases, dermatitis occurs as roughening and thickening of
skin with brown pigmentation. Management
Alimentary tract: Anorexia, nausea, dysphagia. Glossitis Niacin 10 mg or 10,000 mcg per day. Vitamin B complex.
precedes the skin lesions. Noninfective inflammation may Alcohol should be stopped.
Points to Remember Management

Nicotinic acid, disease of 3-Ds, dermatitis, diarrhea, It is given in the dose of 1000 mg daily for 6 weeks.
816 dementia, red swollen tongue, superimposed ANUG,
niacin. Points to Remember
Muscle cramps, mental depression, gastric distress, loss
Pantothenic Acid (Vitamin B5) of hair.
It is water soluble vitamin of the B complex group. It was
discovered by Roger Williams in 1933. Tissue extracts from
Pyridoxine (Vitamin B6)
a variety of biological materials provided a growth factor for It is an important coenzyme in the intermedullary
yeast. This growth factor is identified as pantothenic acid, metabolism of amino acids and complex glycolipids. It is
derived from Greek word pantos meaning everywhere. It is a white crystalline substance soluble in water and alcohol.
a pale yellow oily liquid which is not crystallized, but its
calcium crystallizes readily and this is the form in which it Deficiency Symptoms
is generally available. Nervous: Peripheral neuropathy, mental retardation,
irritability, mental confusion and nervousness.
Function in the Body
Blood: Anemia, albuminuria and leukopenia.
Metabolism: It is a part of enzyme system which play
a vital role in the metabolism of carbohydrates, fats and Skin: Dermatitis and eczema.
protein and in the synthesis of amino acids and fatty acids. Others: Kidney stones, inflammation of the colon,
It is also essential for the formation of porphyrins, the damage to the pancreas, loss of muscular control, migraine
pigment portion of the hemoglobin molecule. headache and premature senility.
Stimulation of adrenal gland: It stimulates the adrenal
glands and increases production of cortisone and other Oral Manifestation
adrenal hormones. Cheilosis: Cracking at the corner of the lip.
Anti-stress factor: It is primarily used as an anti-stress Glossitis: Inflammation of the tongue.
factor and protects against most physical and mental stress.
Others: Angular stomatitis, tooth decay and halitosis.
Combat infection: It increases vitality, wards off infections
and speeds recovery from ill health. Management
Central nervous system: It helps in maintaining the 10 to 50 mg daily in divided doses.
normal growth and development of the central nervous
system. Points to Remember
It prevents premature aging and provides protection Peripheral neuropathy, dermatitis, anemia, kidney
against any damage caused by excessive radiation. stones, cheilosis, glossitis.
Deficiency Symptoms
Biotin (Vitamin B8)
Muscle tissue: Chronic fatigue, muscle cramps, painful
and burning feet and muscular weakness. It functions as a coenzyme for four carbohydrates involved
in fatty acid and amino acid metabolism. Previously was
Nervous system: Mental depression, irritability, dizziness known as vitamin H.
and insomnia.
Gastrointestinal: It may lead to gastric distress and Function in the Body
constipation. Metabolism: It is involved in the metabolism of
Others: Increases tendency toward infection, graying and carbohydrates, proteins and fats.
loss of hair, skin disorders, low blood sugar, low blood Hair: It is essential for the growth and health of the hair. It
pressure and duodenal ulcer. prevents premature graying of the hair as well as hair loss.
Others: It helps maintain the skin and nervous system in Pregnancy: It is an important nutrient for the pregnant
a sound condition. It controls proper distribution of color women and her developing fetus. Folic acid also improves
pigments. lactation.
817
Deficiency Symptoms Others: It helps in building of antibodies which prevent and
heal infection. It also produces nucleic acids, RNA and DNA.
Skin: Scaly dermatitis, eczema, seborrhea and prickling of
the skin. Deficiency Causes
Hair: It can cause alopecia and dandruff. Decreased intake: Inadequate diet, impaired absorption,
malabsorption states and intrinsic intestinal diseases.
Nervous: There is confusion, mental depression and
drowsiness. Increased loss: Hemodialysis.
Muscle: There is muscular weakness, extreme fatigue and Increased requirement: The body demands exceed the
lassitude. intake like in pregnancy, infancy, leukemia, hemolytic
anemia.
Others: Anemia, lack of appetite, hearing abnormalities
and lung infections. Others: Impaired utilization, diseases of the upper small
bowel where folate is mainly absorbed and idiopathic.
Oral: The fleshy part of the tongue may waste away.
Clinical Features
Management
Anemia: Deficiency of folic acid cause anemia which
20 mcg of biotin taken daily for 10 days IM can heal skin
often occurs in pregnant women and also children.
lesions. Oral biotin to be taken in amount of 400 mcg daily
for 8 to 12 weeks. Shampoo coating 1 percent biotin can be Skin: Loss of hair, grayish brown skin pigmentation can
useful in controlling excessive hair loss. also occurs.
Reproductive disorders: Spontaneous abortions, difficulty
Points to Remember during labor and high infant death can also occur. Loss of
Muscular weakness, alopecia, mental depression, scaly libido occurs in males.
dermatitis.
Nervous: Dementia, mental depression and fatigue.
Folic Acid (Vitamin B9) Oral Manifestations
It is also known as folacin or folate. It is a water-soluble Filiform papillae disappear first and fungiform papillae
vitamin. It is a yellow crystalline substance sparingly remain prominent.
soluble in water and soluble in acid solution. It undergoes In severe cases, fungiform papillae are lost and tongue
fairly rapid destruction when heated in neutral or alkaline becomes thick, smooth and fiery red. Severe ulcerative
substances. stomatitis may be seen. Swelling and redness of lips and
lateral margin of the tongue.
Functions in the Body
Red blood cell (RBC): Folic acid in combination with Hematological Findings
vitamin B12 is essential for the formation, maturation and The blood and bone marrow in megaloblastic anemia due to
multiplication of red blood cells. folate deficiency are similar to those in vitamin B12 deficiency
except that the serum and red cell folate levels are low.
Nerve: It is necessary for the growth and division of all
body cells, including nerve cells and for manufacturing a Management
number of nerve transmitters.
A daily dose of 5,000 to 10,000 mcg of folic acid is
Hair and skin: It is essential for the health of skin and hair sufficient and a maintenance dose of 5000 mcg once in
and helps to prevent premature graying of hair. week is given in cases of megaloblastic anemia.
Fatigue, headache, dizziness, nausea, vomiting, diarrhea,

Points to Remember
loss of appetite, pallor and abdominal pain.
Anemia, grayish brown skin pigmentation, spontaneous
818 abortions, dementia, mental depression, filiform papillae Oral Manifestations
disappear, ulcerative stomatitis.
Tongue: There is sore painful tongue, glossitis and
glossodynia. Tongue is inflamed and is beefy red in color.
Cyanocobalamin (Vitamin B12) Painful and burning lingual sensation occur. Small shallow
Vitamin B12 is a complex organomatrix compound called ulcers resembling aphthous ulcers on the tongue with
cobalamine which is cobalt containing porphyrins. It is atrophy of papillae with a loss of normal muscle tone is
freely soluble in water. It is resistant to boiling in neutral called Hunter’s glossitis. Fiery red appearance of tongue
solution, but it is liable to destruction in the presence of due to inflammation and burning sensation.
alkalis and acids. Discomfort in wearing dentures is due to weakened
muscular tone.
Red blood cells: Like vitamin B6 it is essential in the
production and regeneration of red blood cells. There is epithelial atrophy, enlarged basal cell nuclei and
increased mitosis in basal epithelium. Epithelial dysplasia
Nervous: It improves concentration, memory and balance and nonspecific infiltration of lymphocytes, plasma cells
and relieves irritability. or polymorphonuclear neutrophils (PMNs) in the lamina
Metabolism: It is necessary for proper utilization of fats, propria.
carbohydrates and proteins for body building. It is also
used in metabolism of folic acid. Lab Findings
There is decrease in the count of red blood cells. Cells
Others: It promotes growth and increases apatite in
show macrocytosis and poikilocytosis. There is increase in
children.
hemoglobin content which is proportional to their increased
Causes of Deficiency size. Mean corpuscular hemoglobin concentration (MCHC)
is normal.
Congenital: Congenital deficiency without gastric atrophy.
In advanced cases, the red blood cell abnormalities
Systemic diseases: Diseases of terminal ileum, i.e. Crohn’s are detected like polychromatophilic cells, stippled cells,
disease. nucleated cells, Howell-jolly bodies and Cabot’s ring.
Achlorhydria due to absence of gastric hydrochloride
Defective absorption: There is defective absorption of
secretion and pH of gastric content is usually high.
vitamin B12. There is chronic atrophic gastritis with failure
of production of intrinsic factor. Bone Marrow Findings
Smoking: Studies show that smokers have lower levels of Increased no of immature red cells or megaloblasts with a
vitamin B12 and folic acid than non-smoker. few normoblast. Polymacrocytes or large PMNs with large
poly labeled nuclei are found. Megakaryocytes appear
Others: Inadequate diet and intrinsic factor deficiency.
normal.
Deficiency Symptoms
Management
Deficiency of vitamin B12 leads to megaloblastic anemia or
Oral: In a dose from 6 to 150 mcg. Taken in these doses
pernicious anemia. However, pernicious anemia is a result of
it helps in the treatment of lack of concentration, fatigue
deficiency of intrinsic factor, which is essential for absorption
depression, insomnia, anorexia, poor memory and loss of
of vitamin B12 and hence a deficiency of vitamin B12.
weight.
It occurs in 5th to 8th decades of life. It is more
common in men than in women. There is generalized Parenteral: 1000 mcg of vitamin given twice weekly in
weakness, numbness and tingling of the extremities. cases of anemia.
Points to Remember Pathogenesis

Megaloblastic anemia, pernicious anemia, glossitis, There is defective formation of collagen in connective
glossodynia, Hunter’s glossitis, epithelial atrophy, tissues because of failure of hydroxylation of proline to 819
increased mitosis, plasma cells, poikilocytosis, macro- hydroxyproline which is a characteristic amino acid of
cytosis, lymphocytes. collagen. There is also increase permeability of capillary
(hemorrhage), anemia due to erythropoiesis and defective
collagen formation.
Vitamin C
It is also called ascorbic acid and antibiotic vitamin. It Clinical Features
is a modified simple sugar. It is the most active reducing Infantile scurvy: Lassitude, anorexia, painful limbs and
agent. Its highest concentration is in the pituitary, adenoid, enlargement of costochondral junction.
eye and WBCs. Stress and corticotrophin leads to loss
of ascorbic acid from the adrenal cortex. It is a powerful Folliculosis: Hair follicle rises above skin and there are
antioxidant. It is necessary for normal maintenance of perifollicular hemorrhages, i.e. tiny points of bleeding
intercellular substances of connective tissue in bone and occurring around the orifice of hair follicles with heaping
other tissue of mesenchymal origin. of keratin like material.
Hemorrhage may occur in the joint, into nerve sheath
Functions of Vitamin C under the nails or conjunctiva. Petechial hemorrhage occurs
Synthesis: It is important in the formation of collagen, in buttocks, abdomen, legs, arms, ankle and nail beds.
chondroitin sulfate and neurotransmitter. Scorbutic child usually assumes a frog like position and
this may reflect as subperiosteal hemorrhage. There is also
Maintenance: It is useful for maintenance of folate pool epistaxis, anemia and delayed wound healing. Edema of
and mobility and phagocytic activity of neutrophils. It the limbs and face is a frequent finding in severe ascorbic
is also necessary for maintenance of bones and proper acid deficiency. It may lead to premature aging, thyroid
functioning of the adrenal and thyroid glands. insufficiency and lower resistance to all infections.
Absorption: It enhance the absorption of iron in the body.
Oral Manifestations
Metabolism: Tryptophan, nor-epinephrine and tyrosine It occurs chiefly in gingival and periodontal region.
metabolism require vitamin C. Interdental and marginal gingiva is bright red, swollen,
Others: It promotes healing and protects against all forms smooth, shiny surface producing an appearance known
of stress. as scurvy bud (Fig. 32.10). In fully developed scurvy, the
gingiva becomes boggy, ulcerated and bleeds easily. These
Deficiency Symptoms types of gingival lesion are termed as scorbutic gingivitis.
Mild deficiency may appear in the form of lassitude Color changes to violaceous red.
fatigue, anorexia, muscular pain and greater susceptibility Typical fetid breath of the patient with fusospirochetal
to infection. A prolonged deficiency may cause scurvy. stomatitis. In severe cases, hemorrhage and swelling of
periodontal ligament membrane occurs followed by loss of
Scurvy bone and loosening of teeth which are exfoliated.
Prolonged deficiency of vitamin C may result in scurvy. It
is characterized by:
Weakened blood vessels particularly microvessels Osteoblasts fail to form osteoid. Cartilage cells of
having least muscular supports. epiphyseal plate continue to proliferate in normal
Defective synthesis of osteoid which is derivative of fashion and salts are deposited in the matrix between the
collagen. columns of cartilage cells. The calcified matrix material
Impaired wound healing. is not destroyed so that wide zone of calcified but non-
make choline from methionine, an amino acid, with the aid

of vitamin B12 and folic acid.
820 Functions: It helps in the transportation of fats in the

body and prevents accumulation of fat in the liver. In
combination with fatty acid and phosphorus, it stimulates
the formation of lecithin, an important constituent of nerve
cells in the body. It goes directly into the brain cells to
produce a chemical that aids memory.
Deficiency symptoms: It may cause cirrhosis and
fatty degeneration of the liver, high blood pressure and
atherosclerosis.
Doses: It can be given in doses of 1000 to 2000 mg daily
in divided doses.
Figure 32.10 Showing oral manifestation in scurvy
Points to Remember
Cirrhosis, high blood pressure, fatty degeneration of the
ossified matrix, called the scorbutic lattice develops liver.
in the metaphysis. As the lattice increases in width a
more and more fragile zone develops, so that eventually Inositol
complete fracture of the spicule occurs with separation
It is a crystalline compound which has sweet taste. It is
and deformity of the cartilage shaft joint.
highly soluble in water and is not destroyed by heat in
Laboratory Features neutral, acid and alkaline media.
Anemia in scurvy is mild to moderate but may be severe. Functions in the Body
It is usually normocytic and normochromic and associated
Transportation: It is essential for transportation of fat in
with leukopenia and thrombocytopenia, reticulocytosis
the body.
and normoblastic hyperplasia of the bone marrow are other
changes. Nourishment: It is important in providing nourishment to
the brain cells.
Management
Lowering cholesterol level: It helps to lower cholesterol
Vitamin C 250 mg 3 times daily can be given. levels.
Points to Remember Growth of hair: It also promotes the growth of healthy

hair and helps to prevent its falling.
Infantile scurvy, folliculosis, hemorrhage in the joint,
frog like position, epistaxis, delayed wound healing, Prevent eczema: It helps in preventing eczema.
scurvy bud, boggy, ulcerated gingiva, scorbutic gingi- Deficiency symptoms: It can cause alopecia or patchy
vitis, scorbutic lattice, anemia, leukopenia, thrombo- baldness, gastritis hypertension, fatty infiltration in the
cytopenia. liver, hardening of the liver and eczema.
Choline Doses: It is given in the dose of 2 g a day for 6 to 10

weeks.
It is a colorless crystalline compound which absorbs water
quickly. It is highly soluble in water and alcohol. It is Points to Remember
member of vitamin B group. It is present in foods as well
Alopecia, patchy baldness, fatty infiltration in the liver.
as in the body in relatively large amount. The body can
FAT SOLUBLE VITAMINS can occur. Xerophthalmia due to decrease in lacrimal

secretion.
Common Properties
Keratinizing metaplasia: The epithelial cells fail to 821
∙ They are soluble in fat. differentiate. This means that the cells in the basal
∙ Bile salts are essential for their absorption. layer lose their specificity and tend to form a stratified
∙ They are generally stored in the liver. squamous epithelium with keratin production. Keratinizing
∙ They are not excreted in urine. metaplasia of epithelial cells is usually evident in several
organs such as bladder, vagina and skin and predisposes
Vitamin A (Retinol)
them to infection. Drying of skin and atrophy of sebaceous
Carotene is a yellow pigment found in vegetable foods. glands.
It is converted into vitamin A in the body. Vitamin A or
retinol is found in foods of animal origin, while carotene is Effect on reproductive organs: Degeneration of germinal
provided by foods of both plant and animal origin. Vitamin epithelium thus affecting reproduction causes sterility in
A is stored in liver. As a concentrated solution retinol is males and cornification of vaginal epithelium in females.
light yellow in color. It solidifies when cooled and has a Effects on bone: Imbalance between osteoblasts (bone
mild pleasant odor. forming cells) and osteoclasts (bone resorbing cells)
causing aberrations in the shape of bone.
Skin disorders: It may result in pimples, acne, boils and
Epithelial tissue: Vitamin A helps in maintaining the
premature wrinkles.
integrity of epithelial tissue such as epithelial layer of skin,
respiratory mucosa and esophagus and gastrourinary tract. Oral Manifestations
Due to this it builds up resistance to respiratory infection.
Teeth: Defective formation of enamel in teeth. Odontogenic
Structural integrity: Its function in the preservation of epithelium fails to undergo normal histodifferentiation and
the structural integrity and normal permeability of the cell morphodifferentiation, which results in increased rate of
membrane as well as that of membrane of intracellular cell proliferation. Therefore epithelial invasion of pulpal
particles such as lysosomes and mitochondria. tissue is characteristic of vitamin A deficiency. There is
Bone and teeth: It accelerates the normal formation of also distortion of shapes of the incisors and the molars.
bones and teeth. Hypoplasia of teeth: Since the enamel forming cells
Vision: Vitamin A also has a specific role on the are disturbed, enamel matrix is poorly defined so that
physiological mechanism of vision. calcification is disturbed and enamel hypoplasia results.
Oxygenation: It also increases permeability of blood Dentin: Dentine too is atypical in structure, lacking the
capillaries thereby contributing to better tissue oxygenation. normal tubular arrangement and containing vascular and
Aging and senility: It also prevents premature aging and cellular inclusions.
senility. Caries: There is increased caries susceptibility.
Synthesis: It is required for synthesis of glucocorticoids Eruption is delayed. In prolonged deficiency, eruption
and cholesterol. ceases completely. Alveolar bone is retarded in its rate
of formation. Gingival epithelium becomes hyperplastic;
Somatic growth: Vitamin A is required for somatic
in prolonged deficiency it shows keratinization. Tissue
growth.
is easily invaded by bacteria that may cause periodontal
Deficiency Symptoms disease and microabscess formation. Major and minor
salivary glands undergo typical keratinizing metaplasia.
Effect on growth: Failure of growth in young and
collagenous tissue is affected. Management of Deficiency Symptoms
Effect on eyes: Night blindness, dry conjunctiva, Bitot’s Depending upon deficiency symptoms it is given in the
spot, corneal xerosis, corneal ulceration or keratomalacia dose of 7,500 to 15,000 mcg per day for one month.
It is very important for the proper formation of teeth and

Points to Remember
bones. It play important role in prevention of dental caries.
Night blindness, Bitot’s spot, xerophthalmia, keratinizing It is necessary for the healthy functioning of parathyroid
822 metaplasia, sterility in males, cornification of vaginal gland, which regulates the calcium levels in the blood.
epithelium, aberrations in the shape of bone, pimples,
acne, defective formation of enamel, hypoplasia of teeth, Pathogenesis
increased caries susceptibility, delayed eruption of teeth,
Overgrowth: There is overgrowth of epiphyseal cartilage
hyperplastic gingival epithelium.
due to inadequate provisional calcification and failure of
cartilage cells to form a matrix and disintegrates.
Hypervitaminosis A
Formation of irregular masses: There is persistence of
If more than 30,000 mcg of vitamin A is taken daily, it
distorted, irregular masses of cartilage many of which
can produce toxic effect in adults if continued for many
projects into the marrow cavity.
months. In infants, toxic effect can be produced by the
intake of more than 5,550 mcg per day. Deposition: Deposition of osteoid matrix on inadequately
mineralized cartilaginous remnants.
Toxicity symptoms: Painful joints, thickening of long
bones, anorexia, low grade fever, loss of hair, hepatomegaly, Disruption: Disruption of the orderly replacement of
blurred vision, rashes, irregular menstruation, fatigue and cartilage by osteoid matrix with enlargement and lateral
headache. expansion of osteochondral junctions.
Acute toxicity: It results from a single massive dose and it Microfracture: There is abnormal growth of capil-
manifests as abdominal pain, nausea, vomiting, headache, laries and fibroblasts in the disorganized zone because of
dizziness and sluggishness. microfracture and stresses on inadequately mineralized,
weak, poorly formed bone.
Chronic toxicity: It may occur following ingestion of
12,000 mcg or more daily for prolonged periods. It is Deformity: Deforming of skeleton due to loss of structural
characterized by joints pain, hair loss, and dryness and rigidity of the developing bone.
fissuring of lips, loss of appetite, low grade fever and
weight loss. Vitamin D Deficient Rickets
The word ‘Rickets’ refers to any disorder in vitamin D
Vitamin D calcium phosphorous axis which results in hypomineralized
It is also called sunshine vitamin. Vitamin D bone matrix that is failure of endochondral calcification.
(1,25-dihydroxycholecalciferol) is one of the compound It develops in an area where sunlight is deficient. It
that are grouped together as the hydroxylated chole- results from inadequate extracellular level of calcium and
calciferol. If vitamin D deficiency occurs in children and inorganic phosphate, mineral necessary for new bone to
infants it is called rickets and if it occur in adults it is called calcify. Osteoid builds in excessive amounts because it
osteomalacia. Deficiency of vitamin D tends to cause fails to mineralize properly. Rickets occur in infants and
hypocalcemia. children and osteomalacia common in adults.
Forms Clinical Features

D3: It is present in fish liver oils and animal fats. It is called It occurs in infants and children. In the first six months of
cholecalciferol. life, tetany, convulsions are common manifestations due to
hypocalcemia. The wrist and ankles are typically swollen.
D2: It is obtained artificially by irradiation of ergosterol
The changes in bone are found in the epiphyseal plates,
and called ergocalciferol.
metaphysis and the shaft.
Function in the Body Craniotabes: Localized area of thinning are sometimes
The major function of vitamin D is the maintenance of present in the skull, so that a finger can produce indentation.
normal plasma levels of calcium and phosphorous. This condition is called craniotabes. There is softening of
posterior part of the parietal bone, which may be first sign of adequate calcium resulting in softening and distortion of
of the disease. Patients have a short stature and deformed the skeleton.
extremities. Children with rickets show bowing of legs.
Symptoms: The majority of patients has bone pain and 823
Excess of osteoid produces frontal bossing and squared
muscle weakness of varying severity.
appearance to the head.
Signs: There is increased tendency towards fracture,
Rickety rosary: Deformation of chest results from over
peculiar waddling or penguin gait, tetany and green stick
growth cartilage or osteoid tissue at the costochondrial
bone fractures.
junction producing rickety rosary.
Pigeon breast: The weakened metaphyseal areas of the Oral Manifestations
ribs are subject to pull of the respiratory muscles and thus There is incidence of severe periodontitis in some cases of
bend inwards creating anterior protrusion of the sternum osteomalacia.
resulting in a pigeon breast deformity.
Biochemical Changes
Harrison grooves: The inward pull at the margins of
Elevation of serum alkaline phosphatase to three or more
diaphragm creates Harrison’s grooves, girdling the thoracic
times its normal levels. Serum phosphorus is low due to
cavity at the lower margin of the rib cage.
increased phosphorus excretion in response to reduction of
Lumber lordosis: The pelvis may be deformed. When an serum calcium. Serum calcium levels are usually on the
ambulatory child develops rickets, deformities are likely lower side.
to affect the spine, pelvis and long bones causing lumbar
lordosis. Management of Rickets and Osteomalacia
Dietary enrichment of vitamin D in the form of milk. If
Oral Manifestations tetany is present, give calcium gluconate IV. Daily dose
Developmental abnormalities of dentine and enamel, between 1000 and 2000 IU of vitamin D combined with 500
delayed eruption and malalignment of teeth. to 1000 mg of calcium. Hormonal therapy like flucytosine.
There is higher caries index in rickets as compared to Curative treatment includes 2000 to 4000 IU of calcium
normal. There may be hypoplasia of enamel; enamel may daily for 6 to 12 weeks followed by a daily maintenance
be mottled, yellow gray in color. dose of 2000 to 4000 IU for a prolonged period. Patients
There are large pulp chamber, high pulp horns and with osteomalacia due to intestinal malabsorption require a
delayed closure of root apices. The osteoid is so soft that larger dose of vitamin D and calcium, i.e. 40,000 to 1,00,000
teeth are displaced leading to malocclusion of the teeth. IU of vitamin D and 15 to 20 gm of calcium lactate per day.

Craniotabes, rickety rosary, pigeon breast, Harrison Adult rickets, bone pain, muscle weakness, increased
grooves, lumbar lordosis, delayed eruption, malalign- tendency towards fracture, penguin gait, tetany, severe
ment of teeth, higher caries index, delayed closure of periodontitis, elevation of serum alkaline phosphatase.
root apices, large pulp chamber, high pulp horns.
Vitamin D Resistant Rickets (Familial
Osteomalacia Hypophosphatemia, Refractory Rickets)
It is also known as adult rickets and only flat bones and It is X-linked trait with some defect in reabsorption
diaphyses of long bones are affected. It is most commonly of metabolism. It has got clinical features which are
seen in postmenopause females with a history of low characteristic of rickets but it does not respond to
dietary calcium intake and little exposure to UV light. therapeutic dose of the vitamin.
Clinical Features Causes

It is seen in adults and pelvic deformities are commonly The disease is now recognized as a specific disorder
seen in females. Remodeling of bone occur in the absence characterized by hypophosphatemia associated with
decreased renal tubular reabsorption of inorganic tones of cartilage which extend down toward the shaft and
phosphates. are separated form one another by collection of capillaries.
Familial occurrence being inherited as X-linked This zone contains trabeculae made of uncalcified cartilage
824 dominant trait. Rickets and osteomalacia which does not matrix upon which osteoid is deposited on pre-existing
respond to usual doses of vitamin D. Diminished intestinal bony trabeculae.
calcium and phosphate absorption. Normal vitamin D There is deposition of globular dentin which often
metabolism and absence of other related abnormalities. exhibits clefting.

Age and sex distribution: It is first recognized in children 25-hydroxy cholecalciferol in lower doses is useful than
when they are about to walk. Males are usually affected conventional vitamin D. Patient should also be given
more severely than female. calcitriol and multiple daily dose of phosphate.
Sign: Slight decrease in height of the patient and reduced Points to Remember
growth and rickets like bone changes. The lower limb are
shortened and bowed. Refractory rickets, decrease in height of the patient,
vertebral fracture, bone pain, globular hypocalcified
Symptoms: There is enlarged epiphysis, bone pain, muscle dentin, wide root canal, large pulp chambers, periapical
weakness and vertebral fracture. Bony outgrowth at the site involvement, multiple gingival fistulae, rachitic
of muscular attachment and around joints may limit the metaphysis.
movement.
Oral Manifestations Vitamin E (Tocopherol)

It is also called anti-aging factor. The word tocopherol
Dentin changes: Widespread formation of globular
is derived from the word tocos meaning child birth and
hypocalcified dentin with clefts and tubular defects
pheros meaning to bear. It is yellow, oily liquid freely
occurring in the region of pulp horns. Gross reduction in
soluble in fat solvents.
amount and quantity of dentin which results in abnormality
Tocopherol alpha, beta, gamma, lambda have been
wide root canal and large pulp chambers with faulty
obtained from natural sources and their relationship with
calcification and marked interglobular space in dentin.
fertility and prevention of muscular dystrophy have been
Pulp horns are elongated and extend high often reaching
found. They are not destroyed by heat even at room
the DE junction.
temperature or above 100°C. They are destroyed by UV
Because of this defect there commonly is invasion of the
light.
pulp by microorganisms without demonstrable destruction
of the tubular matrix. Thus there is often periapical Functions in the Body
involvement of a grossly normal appearing deciduous or
permanent tooth. Reproductive function: Protective effect of vitamin E
There the development of multiple gingival fistulae. on reproduction and prevention of sterility. All the three
Tract is frequently present to dentinoenamel junction or layers of embryo ectoderm, mesoderm and endoderm are
even outer enamel surface. This tract remains patent and preserved by vitamin E.
may result in early pulpal infection developing in abscess or Blood flow and clotting mechanism: Vitamin E dilates
carious lesion. There is formation of abnormal cementum. the capillary and enables the blood to flow freely into the
blood deficient muscle tissue thus strengthening both the
Histopathological Features tissue and the nerves supplying them. It also dissolves the
Failure of bone salts to be deposited in the cartilage matrix blood clot and also prevents their formation.
between the rows of hypertrophic cells so that these cells
Electron transport system: It functions as a cofactor in
are not invaded and destroyed by capillaries.
the electron transport system.
The histological feature is characterized by a broad
zone between the multiplying cartilage cells and the shaft Healing: It prevents the formation of excessive scar tissue
the so called rachitic metaphysis which is composed of and in some instance even melts away unwanted scar tissue.
Prevention: It is required for prevention and storage of Clotting: It prevents hemorrhage only in cases when there
creatinine in muscles. It has ability to prevent hepatic is defective production of prothrombin.
necrosis in animals. Prevents vitamin A from destruction
Oxidative phosphorylation: It acts as a cofactor in 825
and helps in its storage in tissue.
oxidative phosphorylation associated with lipid.
Deficiency Symptoms Effects of Deficiency
Reproductive: Abortion of fetus in females and atrophy Prolongation of clotting time and a tendency to bleed
of spermatogenic structures in males leading to permanent profusely. There may be nasal bleeding.
sterility.
Muscles: It causes degenerative changes in muscles. There Oral Manifestations
is muscle fiber atrophy which is replaced by connective Gingival bleeding can also occur in cases of vitamin K
tissue. deficiency.
Heart: There is necrosis and fibrosis of heart muscles. Management
Blood capillaries: Deficiency may lead to degenerative It is given in dose of 10 to 20 mg daily.
changes in the blood capillaries which in turn lead to heart
and lung diseases, pulmonary embolism and brain stroke. Points to Remember
Anti-hemorrhagic vitamin, prolongation of clotting time,
Oral Manifestations tendency to bleed, gingival bleeding.
Loss of pigmentation, atrophic degenerative changes in
enamel of vitamin E deficient rats. Clinical and oral effects of some vitamins are depicted
in Table 32.1.
Management
Vitamin E is given in the doses of 100 to 400 mg daily. DISORDERS OF BILIRUBIN
Points to Remember Jaundice
Abortion of fetus, degenerative changes in muscles, It is condition which is characterized by excess bilirubin in
necrosis and fibrosis of heart muscles, pulmonary the bloodstream. Bilirubin results in yellowish discoloration
embolism, brain stroke, loss of pigmentation. of the skin and mucosa.
Causes
Vitamin K (Phylloquinone)
Hemolytic anemia or sickle cell anemia: There is increase
It is essential for the production of a type of protein called level bilirubin as red blood cells are being broken down at
prothrombin and other factors involve in the blood clotting rapid rate so that liver cannot keep space with processing.
mechanism. Hence it is known as anti-hemorrhagic
vitamin. It is not easily destroyed by light, heat or exposure Liver dysfunction: There is decrease uptake of bilirubin
to air. It is destroyed by strong, acids, alkalis and oxidizing from the circulation or decrease conjugation of bilirubin
agents. in liver cells.
Gilbert syndrome: Defects in enzyme system will also
Forms
lead to impaired processing of bilirubin.
∙ K1: It is the form which occurs in plants.
∙ K2: it is produced by most bacteria present in human Clinical Features
intestine if not supplied in the diet. Appearance: Patient exhibits diffuse, uniform, yellowish
discoloration for skin and mucosa.
Functions in the Body As bilirubin has got affinity for elastin fiber, the tissue
Synthesis: It is essential for the hepatic synthesis of like sclera, lingual frenum, and soft palate which contain
coagulation factors II, V, VII, IX and X. elastin fiber are prominently affected.
Table 32.1 Clinical and oral effects of some vitamins

Vitamin General manifestations of deficiency Oral manifestations of deficiency
826 Vitamin A Night blindness Keratinizing metaplasia of epithelium resulting in
Xerophthalmia characterized by dryness in increased keratin formation
conjunctiva and cornea Occlusion of salivary gland ducts with keratin
Bitot spots in forms of triangular plaques in Enamel hypoplasia, atypical dentin formation and
conjunctiva epithelial invasion of pulpal tissue are characteristic
If xerophthalmia persist, destruction of cornea features
occurs, causing total blindness. This condition is Enamel is more severely effected than dentine
known as keratomalacia
Vitamin D Rickets in children Delayed eruption of primary and permanent teeth
(antirachitic vitamin) Osteomalacia in adults Malalignment of the teeth in the jaws
Pigeon chest is one of important feature of rickets Developmental anomalies of dentine and enamel
The vitamin, which acts more like a hormone, is The teeth shows wide predentin zone with much
vitamin D interglobular dentine
Renal rickets or renal osteodystrophy is seen in The pulp horns are elongated and extend high,
patients with chronic renal failure. Renal rickets is reaching the dentinoenamel junction
mainly due to decreased synthesis of calcitriol in
kidney
In rickets the plasma calcitriol is decreased and
alkaline phosphatase activity is elevated
Vitamin E Decreased male fertility
(antisterility vitamin) Impaired fetal – maternal vascular relationships
Encephalomalacia --------------------
Nutritional muscular dystrophy
Vitamin K Deficiency is uncommon. Prothrombin levels below 35% result in gingival
(coagulation vitamin) Brings about post-translational modification bleeding after tooth brushing
of 2,7,9,10 blood clotting factors, particularly Spontaneous gingival hemorrhages occur, when
prothrombin. So deficiency results in prolong the prothrombin levels fail below 20%
clotting time
Vitamin C Deficiency may result in scurvy. It is characterized The pathogenic sign is the swollen and spongy
(Ascorbic acid ) by spongy and sore gums, loose teeth, anemia, gums, particularly the interdental papillae are
swollen joints, delayed wound healing, hemorrhage, involved producing the appearance of scurvy buds.
osteoporosis, etc. In severe cases, hemorrhages to periodontal
Defective collagen synthesis membranes followed by loss of blood and loosening
“Cork – screw” hair pattern, “Wody legs” with of teeth occur
large spontaneous bruises in lower extremities are
other features
“Trummer field zone” is the classic histologic
picture of bone in scurvy
Vitamin B1 B1 deficiency is seen in populations consuming ----------------------
(anti-beriberi or anti- polised rice as a staple food
neuritic vitamin) Dry beriberi or peripheral neuritis; wet beriberi or
cardiac manifestations, and cerebral or wernickes
encephalopathy with korsakoff’s psychosis are
features of B1 deficiency
Contd...
Contd...
Vitamin General manifestations of deficiency Oral manifestations of deficiency
Vitamin B2 (Riboflavin) Nasolabial seborrhea or dyssabacea. Glossitis: The filiform papillae become atrophic 827
Vascularization of cornea while the fungiform papillae becomes-gorged and
Scrotal dermatitis mushroom shaped, resulting in magenta colored
tongue.
Cheilosis, ocular lesions.
(Non-specific bilateral angular cheilosis may be seen
in association with faulty dentures or in patients with
reduced vertical dimension due attrition.)
Niacin Pellagra (the symptoms of pellagra are referred to Bald tongue of sandwith.
as three ‘D’s, i.e. dermatisis, diarrhea, dementia ‘Raw beefy’ tongue.
and if not treated may lead 4th, i.e. death) The mucosa becomes fiery red and painful.
Salivation is profuse.
Vitamin B5 Burning feet syndrome
Pathothenic acid or Pain and numbness in the toes, sleeplessness and
chick anti-dermatisis fatigue are features.
factor Pathothenic acid is one of the water-soluble
vitamins that is synthesized in the body.
Vitamin B6 Peripheral neuropathy (due to decreased synthesis
(Pyridoxine) of serotonin catecholamine) and demyelination of
neurons.
Isoniazid (drug use in treatment of TB) is a
antagonist of vitamin B6.
Biotin (Vitamin B7 or Biotin deficiency is uncommon since it is well
Vitamin H or anti-egg distributed to foods and also supplied by the
white injury factor) intestinal bacteria.
Folic acid Macrocytic anemia, glossitis Glossitis:
Aminopterin and methotrexate are structural The filiform papillae disappear first, but in
analogs of folic acid use din treatments of many advanced cases the fungiform papillae are lost and
cancers including leukemia. These drugs block the tongue becomes smooth and fiery red in color.
the formation of THF and hence DNA synthesis is
impaired.
Vitamin B12 Pernicious anemia Beefy and tongue with glossopyrosis, glossitis and
(antipernicious vitamin Neurological manifestations due to degeneration of glossodynia.
or extrinsic factor of posterior and lateral tracts of spinal cord. Hunter’s glossitis or Moeller’s glossitis, which
castle) Degeneration of myelin sheath and peripheral is similar to “bald tongue of sandwith” seen in
nerves also occurs. pellagra.
Sign and symptoms: Other sign and symptoms are BIBLIOGRAPHY

abdominal pain, anorexia, and fatigue.
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1. Marasmus and kwashiorkar is cause due to deficiency 6. A classic triad of bone destruction in skull, exopthalmos, 829
of: diabetes insipidus seen in:
a. Carbohydrate b. Protein a. Hand-Schuller-Christian disease
c. Vitamins d. Water b. Letterer Siwe disease
2. Bright reddening of tongue, papillary atrophy and c. Hypoglycemia
bilateral angular cheilosis seen in: d. Pellagra
a. Marasmus b. Kwashiorkar 7. Shortening of mandible and clear gargoyle cells seen
c. Amyloidosis d. Both a and b in:
3. Stain used for detection of amyloid: a. Niemann-Pick disease b. Hurler’s disease
a. Congo red b. Crystal violet c. Gaucher’s disease d. Both a and c
c. Alizarin d. Alician blue 8. Which one is also refer as ‘antibiotic vitamin’:
4. Excretion of red color urine seen in: a. Vitamin C b. Vitamin B
a. Amyloidosis c. Vitamin D d. Vitamin A
b. Letterer Siwe disease 9. ‘Scorbutic lattice’ is the histopathologic feature seen
c. Porphyria in:
d. Both a and b a. Retinol b. Beriberi
5. Red or brownish color of teeth seen in: c. Scurvy d. Zinc deficiency
a. Porphyria 10. Large pulp chamber, high pulp horns and delayed
b. Marasmus closure of root apices seen in:
c. Tay-Sachs disease a. Hypervitaminosis A b. Rickets
d. Gaucher’s disease c. Pellagra d. None.
33 Neuromuscular Disorders and
Orofacial Pain
Chapter Outline
Â Muscle disorders Â Facial pain

• Muscular dystrophy • Trigeminal neuralgia
• Myotonias • Glossopharyngeal neuralgia
• Myasthenia gravis • Geniculate neuralgia
• Dermatomyositis • Atypical facial pain
Â Neuromuscular disorders • Neuralgia inducing cavitational osteonecrosis
• Auriculotemporal syndrome • Cluster headache
• Bell’s palsy • Paroxysmal hemicranias
• Motor neuron disease • Migraine
• Multiple sclerosis • Temporal arteritis
• Myositis ossificans • Burning mouth syndrome
MUSCLE DISORDERS begins in childhood, usually before the age of 6 years and
rarely after 15 years.
Muscular Dystrophy
Symptoms: The earliest symptom is inability to walk or
There are mainly two types of muscular dystrophy: run change to which, the children fall readily and associate
1. Severe generalized familial muscular dystrophy with muscular enlargement and weakness.
2. Mild restricted muscular dystrophy.
Waddling gait: The muscular enlargement ultimately
Severe Generalized Muscular Dystrophy proceeds to atrophy and the limbs appear flaccid. It is
It is described as a rapidly progressive muscle disease, the atrophy which is responsible for the postural and
usually beginning in early childhood and presenting a ambulatory defects, such as waddling gait.
strong familial transmission.
Oral Manifestations
Clinical Features The muscles of mastication, facial, ocular, laryngeal and
Age and sex distribution: It is the most common form the pharyngeal muscles are usually involved, only late in
of muscular dystrophy and predominately affects males. It the course of disease.
Neuromuscular Disorders and Orofacial Pain
Due to lack of muscle tension, teeth can not be kept Management

properly aligned in the arch. Locking and clicking of the
Some patients undergo temporary periods of remission or
jaw occur.
even complete arrest. 831
Points to Remember
There is gradual disappearance of muscle fibers, as the disease
progresses until ultimately no fibers may be recognized. Inability to walk, waddling gait, locking and clicking of
Persistent fibers show variation in size in the earlier stages the jaw, disappearance of muscle fibers, cardiomegaly,
of disease, some being hypertrophic but others atrophic. tapirlips, individual fibers become atrophic, infiltration
of fiber bundles, elevated serum creatinine phosphokinase
Lab Findings levels.
Serum creatinine phosphokinase levels are elevated in all
males. Myotonias
Management Types
There is no treatment for this disease. Physical therapy ∙ Dystrophic
may help prolong the use of specific muscle group. ∙ Congenital
∙ Acquired.
Mild Restricted Muscular Dystrophy
It is a slowly progressive proximal myopathy which pri Dystrophic Myotonia
marily involves the muscles of shoulder and face and has a It is also called myotonic dystrophy or dystrophic myo-
weak familial incidence. It is transmitted as an autosomal tonica. It is inherited as an autosomal dominant trait.
dominant trait.
Clinical Features
Clinical Features
Age: It does not appear until the 3rd decade of life, but may
Age: The disease begins at any age from 2 years to 60
be seen earlier, i.e. even in childhood.
years, although onset is in the first two decades of life.
Site: Atrophy of the muscles is seen usually in the hands
Symptoms: The earliest symptoms are inability to raise the
and forearms. It can be seen in muscles of face, jaws, neck
arms above the head and inability to close the eyes, even
and levators of eyelids.
during sleep as a result of weakness of the facial muscles.
Sign and symptoms: There is associated weakness of the
Signs: Scapular muscles become atrophic and weak with
muscles. There is alteration in the facial muscles, which
subsequent alteration in the posture.
consist of ptosis of the eyelids and atrophy of the masseter
Cardiac abnormalities including cardiomegaly and
and sternocleidomastoid muscles.
tachycardia are often present and many patients die of
sudden cardiac failure. Myopathic facies and swan neck: The masseteric
atrophy produces a narrowing of the lower half of the face
Oral Manifestations which, with ptosis and generalized weakness of the facial
The lips develop a characteristic looseness and protrusion, musculature gives the patient a characteristic ‘myopathic
which have been described as tapir-lips. The patient is facies’ and ‘swan neck’.
unable to whistle or smile. Pharyngeal and laryngeal myotonia also exhibit
There may be severe open bite and development of weakness manifested by a weak, monotonous nasal type of
diastema. voice and subsequent dysphagia.
Recurrent dislocations of the jaw are also reported in
Histopathological Features this disease.
There is some variation in size of muscle fibers and Other features are testicular atrophy, cataract,
moderate infiltration of fiber bundles by connective tissue. hypothyroidism with cold extremities, slow pulse, loss of
Individual fibers ultimately become atrophic. hair and functional cardiac changes.
Histopathological Features Clonus: If these spasms are intermittent, it is called

‘clonus’ (myoclonic contraction).
There is enlargement of scattered muscle fibers and
the presence of centrally placed muscle nuclei in long Trismus: If spasms are constant, it is called ‘trismus’
832
rows. True hypertrophy in some fibers, is found, as well (myotonic contractions).
as in isolated fibers which show extreme degenerative
changes including nuclear proliferation, intense basophilic Myasthenia Gravis
cytoplasmic staining and phagocytosis. It is an autoimmune disease characterized by progressive
weakness of the skeletal muscles, particularly those
Points to Remember innervated by the cranial nerves.
Myotonic dystrophy, atrophy of the muscles, ptosis,
myopathic facies and swan neck, pharyngeal and Etiopathogenesis
laryngeal myotonia, recurrent dislocations of the jaw, Effect on AChR: This disease affects acetylcholine
scattered muscle fibers, centrally placed muscle nuclei, receptor of muscle fibers which results in fatigability
phagocytosis, basophilic cytoplasmic staining. of skeletal muscle. Motor end organs of acetycholine
mechanism is normal that is reason smooth and cardiac
muscle are not affected.
Congenital Myotonia
Defective neuromuscular transmission: Defect in the
It is transmitted as an autosomal dominant trait with
neuromuscular transmission occur secondary to coating of
incomplete penetrance in some families. It is also called
the AChR by circulating antibodies to receptors.
Thomson’s disease.
Thymus hyperplasia or thymoma: It may occur due to
Clinical Features thymus hyperplasia or tumors of the thymus.
Age: It commences early in the childhood and may be Other factors: In some cases myasthenia gravis can be
first noticed because of difficulties in learning to stand and related to pregnancy, menstruation and hyperthyroidism.
walk.
Clinical Features
Sites: The muscles of the thighs, forearm and shoulders are
especially affected as well as are the muscles of the neck, Age and sex predilection: It occurs in adults in the middle
masseter and facial muscles. age group with a predilection for women.
Muscular contraction induces a severe, painless muscular Symptoms: It is characterized by a rapidly developing
spasm, and an actually delay in relaxation. weakness in voluntary muscles, following even minute
activities.
Percussion contraction: Electrical and physical stimul
ation of a muscle produces the characteristic prolonged Signs: Repeated muscular contraction will results in
contraction or percussion contraction. Blinking with strong weaker contracting muscle. This will lead to loss of weight.
closure of the eyes will sometimes produce a prolonged Soon patients become exhausted, may eventually become
contraction of the lids. bedfast.
Eye: There is diplopia (double vision) and ptosis (drooping
Management eyelids) (Fig. 33.1). The neck muscles may be so weak that
There is no specific treatment for this disease. the head can not be held up without support.
Respiratory failure: Death frequently occurs from
Points to Remember
respiratory failure.
Muscular contraction, painless muscular spasm, Percus
sion contraction. Orofacial Manifestations
Mastication problems: The patient’s chief complains
Acquired Myotonia may be difficulty in mastication, deglutition. This occur
It refers to spasm of muscles which are more intense than due to weakness in muscle of mastication. In some cases
typical myotonia. regurgitation of food are common.
Thymectomy: In patient where there is evidence of

thymoma and elevated AChR antibody, thymectomy is
indicated.
833
Points to Remember
Rapidly developing weakness in voluntary muscles,
muscular contraction, diplopia, ptosis, respiratory failure,
difficulty in mastication, hang open jaw or dropping of
the jaw, sorrowful appearance, dysarthria, weakness of
the tongue and palatal muscles, Lymphorrhages, foci of
atrophy or necrosis of muscle, elevated serum AChR
antibody level, anticholinesterases.
Figure 33.1 Ptosis in patient of myasthenia gravis
Dermatomyositis
Dropping of jaw: In some cases muscle of mastication It is also called polymyositis. It is an acute or a chronic
becomes very weak. So after having meal of patient this disease of unknown etiology and is characterized by a
will results in hang open jaw or dropping of the jaw. gradual onset with vague and indefinite prodromata,
followed by edema, dermatitis, myositis and sometimes
Sorrowful appearance: Dropping of the face, leads a neuritis and mucositis.
sorrowful appearance of the patient with myasthenia gravis.
Speech: Speech is often slow and slurred (dysarthria). Clinical Features
Age: It may occur in patients of any age ranging from very
Tongue: There is disturbance in taste sensation. There may
young children to elderly, but majority occur in the 5th
be weakness of the tongue and palatal muscles. Protrusive
decade of life.
movements of the tongue may become weak leading,
at times to posterior collapse of the organ with airway Symptoms: It begins with erythematous skin eruptions,
obstruction. edema, tenderness, swelling and weakness of the proximal
muscles of limbs. Fever may be associated with it.
Signs: The weakness of muscle is progressive and
Lymphorrhages: Focal collection of small lymphocytes or
characteristically spread to the face, neck, larynx, pharynx
lymphorrhages is found surrounding small blood vessels in
and heart.
the interstitial tissue of the affected muscles.
Foci of atrophy or necrosis of muscle: Foci of atrophy Heliotrope: The skin becomes the seat of violaceous
or necrosis of muscle fibers have been described. There is erythema and edema with a predilection for the eyelids,
also loss of rounded crosssectional appearance. malar area and dorsa of hands. The typical skin lesions
include heliotrope (lilaccolored) changes around the face
Elevated serum AChR antibody level: As this antibody
and fingers.
is not found commonly in human being, it is diagnostic
The edema which gives the skin a puffy consistency
indicator of myasthenia gravis.
including the face, leaves a reticulated telangiectatic
Management erythema when it subsides.
Anticholinesterases: Cholinesterase inhibitor like edro Calcinosis cutis: The skin lesions frequently calcify
phonium, neostigmine, administered intramuscularly im and form calcium carbonate nodules with a foreign body
proves the strength of the affected muscles. reaction which is known as calcinosis cutis.
Combination therapy: In some cases anticholinesterase Calcinosis universalis: The term calcinosis universalis is
can be combined with intermittent corticosteroids therapy. applied when these calcified masses are found generalized
This will give good results in many cases. throughout the soft tissues.
Oral Manifestations Points to Remember

The oral lesions consist of diffuse stomatitis and pharyngitis Polymyositis, heliotrope, weakness of muscle, puffy
834 and are extremely common. Involvement of the muscles of consistency edema of skin, calcinosis cutis, calcinosis
jaw, tongue and pharynx may pose problems in eating and universalis, diffuse stomatitis and pharyngitis, show
phonation. The oral mucosa may show dark red or bluish dark red or bluish erythema on oral mucosa, rigid tongue,
erythema. purplish black intrinsic staining of teeth, widespread
In the early stages, tongue is swollen and later becomes degeneration, hyalinization, vacuolization, granulation
harder and gradually it becomes atrophic. The tongue may fragmentation with phagocytosis, mild anemia.
become rigid owing to severe calcinosis. Telangiectatic
lesions of vermilion border of lips and cheeks may also
occur. There is purplish black intrinsic staining of teeth. NEUROMUSCULAR DISORDERS
Histopathological Features Auriculotemporal Syndrome
The muscle fibers in dermatomyositis exhibit widespread It is also called Frey’s syndrome or gustatory sweating
degeneration and hyalinization (Fig. 33.2). and flushing. It is described in 1853 by Baillarger and is
In advanced cases, the muscle fibers disappear leaving characterized by facial flushing and sweating along the
only the fibrous stroma. Many fibers show vacuolization, distribution of auriculotemporal nerve.
granulation and fragmentation with phagocytosis of
Pathogenesis
disintegrating fibers.
It is an unusual phenomenon which arises as a result of
Lab Findings damage to the auriculotemporal nerve. Auriculotemporal
It manifests as a mild anemia or leukocytosis. In addition, nerve, in addition to sensory function, also supplies
creatinuria is a constant finding as well as elevated levels parasympathetic fibers to parotid gland. The fibers
of serum transaminase and aldolase. regenerate, become misdirected and follow the course of
sympathetic fibers to the skin and sweat gland.
Management Parasympathetic fibers would therefore induce sali
Prognosis: Muscle involvement may become severe vation; inadvertently stimulate the preauriculardermal
enough to confine the patient to bed or cause death owing sweat gland and arterioles, causing hydrosis and vasodi
to failure of respiratory muscle. lation.
Etiology
It follows surgical operations such as removal of a parotid
tumor or ramus of mandible. It may follow superficial
parotidectomy. It may occur due to birth trauma.
Clinical Features
Symptoms: The patient exhibits preauricular flushing and
sweating of the involved side of face, following ingestion
of food or visual stimulation by foods. Patient may
sometimes feel pain while eating. The severity of sweating
is increased by tart food. Profuse sweating may be evoked
by parenteral administration of pilocarpine or eliminated
by the administration of atropine.
Sign: Local skin temperature is raised without sweating.
Figure 33.2 Dermatomyositis histopathological features Temperature may rise to 100°F. Presence of cutaneous
hyperaesthesia in front and above the ear, area supplied by Surgical procedures, such as removal of parotid gland
the auriculotemporal nerve. tumor in which the facial nerve is sectioned can also cause
facial paralysis. It may cause by ischemia of the nerve near
Crocodile tears: In it patient exhibits profuse lacrimation 835
the stylomastoid foramen, resulting in edema of the nerve,
when food is eaten particularly hot and spicy food.
its compression in the bony canal and finally, paralysis.
Minor Starch-Iodine test: 1 percent iodine solution is Familial and hereditary occurrence is also reported
painted on affected area. After the solution is dried, area in cases of Bell’s palsy. Tumors of cranial base,
is coated with layer of starch. After patient having taken parapharyngeal space and infratemporal fossa often cause
food, moisture of sweat will mixed with iodine on the skin. 7th nerve palsy.
It will results in blue color. Others causes of Bell’s palsy are MelkerssonRosenthal
syndrome, acute otitis media and atmospheric pressure
Management changes.
Intracranial division of auriculotemporal nerve has been
reported to be successful. Clinical Features
To control gustatory sweating, may be maintained for Age and sex distribution: Women are more commonly
up to 3 days, by topical application to the affected skin by affected than men and usually, it occurs in the middle age
1 percent glycolpyrrolate lotion or cream. group.
Other treatment modalities which can be use are Onset: It arises more frequently in spring and fall, than at
injection of atropine, botulinum toxin. You can also go any other time of the year. It begins abruptly as paralysis of
for topical application of scopolamine cream and systemic the facial musculature, usually unilaterally.
use of oxybutynin chloride, an antimuscarinic agent.
Symptoms: In some cases, it is preceded by pain on the
Points to Remember side of the face which is ultimately involved, particularly
within the ear, temple, and mastoid area or at the angle of
Frey’s syndrome, preauricular flushing and sweating, the jaw. On the affected side, eye can not be closed and
raised local skin temperature, Crocodile tears, Minor wrinkles are absent on that side. There is watering of eye,
StarchIodine test, intracranial division of auriculotem which leads to infection.
poral nerve. It is associated with Melkersson-Rosenthal syndrome.
When the patient smiles, the paralysis becomes obvious
Bell’s Palsy since the corner of the mouth does not rise nor does the skin
of the forehead wrinkles or the eyebrows raise (Fig. 33.3).
It is also called 7th nerve paralysis or facial paralysis.
Pathogenesis
The cortical tract communicating with the motor nucleus
ambiguous of facial nerve crosses over to get innervated
into the lower face musculature. Upper face fibers are
ipsilateral proximal to the nucleus. A cortical lesion will
cause contralateral lower face palsy; lesions of brain stem,
main trunk or peripheral fibers will result in total hemifacial
paralysis.
Etiology
It usually occurs after exposure to cold. But many workers
believe that it is a chance finding. It may be a causative
factor as Bell’s palsy occurs after extraction of teeth and
after injection of local anesthetic. Extraction and injection
may cause damage to the nerve and subsequent paralysis. Figure 33.3 Patient having facial paralysis
Oral Manifestations Symptoms: It is characterized by progressive weakness

of limbs with associated muscular atrophy, reflex loss and
The muscular paralysis manifests itself by dropping of the
sensory disturbances. The initial symptoms usually consist
836 corner of mouth, from which saliva may dribble. The patient
of difficulty in walking with leg pain and paresthesia.
has a typical masklike or expressionless appearance.
Speech and eating is difficult and occasionally, taste Signs: Atrophy of foot, leg and hand muscles ultimately
sensation on the anterior portion of tongue is lost or altered. occurs with the appearance of a typical footdrop, steppage
Food is retained in the upper and lower buccal and labial gait and storklegs.
folds due to weakness of buccinator.
Amyotrophic lateral sclerosis
Management Synonym: It is also called Lou Gehrig disease (he is
The use of vasodilator drug like histamine has been proved famous baseball player who died of these disease)
beneficial in some cases.
Age and sex distribution: It generally occurs between the
Other treatment modalities which are used are systemic
ages of 40 to 50 years and affects males more frequently.
corticosteroid, hyperbaric oxygen therapy, surgical decom-
Precipitating factors include fatigue, alcohol intoxi
pression of intratemporal facial nerve can be given.
cation and trauma. Infections like syphilis, influenza, typhus
Points to Remember and epidemic encephalitis can also lead to amyotrophic
lateral sclerosis.
7th nerve paralysis, arises in spring, pain on the side
of the face, Melkersson Rosenthal syndrome, on the Symptoms: The initial symptoms consist of weakness and
affected side, eye cannot be closed, wrinkles are absent, spasticity of limbs, difficulty in swallowing and talking
masklike or expressionless appearance, lost taste with indistinct speech and hoarseness. Atrophy, flaccidity,
sensation, histamine. symmetric weakness, slowness of movements and
impairment or loss of palatal movements may also occur.
Motor Neuron Disease Fasciculation: Fasciculation of shoulder and thigh are
It is described by Charcot in 1870. It is characterized by early symptom.
weakness and wasting of muscles. Bulbar paralysis: Dysfunction of muscle controlled by
medulla oblongata can be present in the later stage of
Etiopathogenesis disease.
Degeneration: There is progressive degeneration of motor
neurons of cranial nerves, pyramidal tract and anterior horn Oral Manifestations
cells of spinal cords. Stage I: In earlier stages, the tongue is slightly weakened,
Genetic: Many cases seem that it occur due to genetic leaving articulation relatively unaffected.
defect. It can be transmitted as autosomal dominant trait. Stage II: In the middle stages, a gradual and generalized
weakening of tongue occurs, accompanied by spasticity
Types which results in reduced rate, range and force of articulatory
• Progressive muscular atrophy tongue movements.
• Amyotrophic lateral sclerosis
Stage III: In the later stages, there is virtually unintelligible
• Progressive bulbar palsy.
articulation.
Clinical Features Progressive bulbar palsy

Progressive muscular atrophy Age and sex distribution: It generally in children and
young adults. There is no sex predilection.
Age and sex distribution: It usually occurs in childhood
with some reported case at birth. As there incidence of Symptoms: It is characterized by difficulty in swallowing
occurrence is same in case male and females. and phonation, hoarseness.
Palatal paralysis: Paralysis of palatal muscle will results

in regurgitation of food in the nasophayrnx and nasal
sinuses.
837
Facial involvement: There is weakens of facial muscle
which ultimately results in mastication problems. Chewing
is difficult as facial muscles become weakened.
Signs: Atrophy of facial, masseter, temporal muscles and
tongue, with fasciculation of the face and tongue.
Management
Fatal course: Course of this disease is fatal. Death usually
occurs within 2 years in case of progressive muscular
atrophy and progressive bulbar palsy. In case of ALS it can
occur within 5 years.
Figure 33.4 Hand stiffness in patient with multiple sclerosis
Antiglutamate agent: Riluzole has shown some
improvement but cure is not possible with this drug also. towards friendliness and cheerfulness, variety of ocular
disturbances including visual impairment as a manife
Points to Remember station of retrobulbar neuritis, nystagmus and diplopia.
Types, degeneration of neuron, stork leg, progressive
Charcot’s triad: It consist of intentional tremors, nysta
weakness, tongue involvement, bulbar paralysis, palatal
gmus, dysarthria and scanning speech.
paralysis, fatal course.
Oral Manifestations
Multiple Sclerosis Facial and jaw weakness occur in some patients. Staccato
It is also called disseminated scleroses. (a series of short, detached sound or words) type of speech
is interesting feature of this disease.
Etiology In some cases both trigeminal neuralgia and Bell’s
The lesions are allergic hypersensitivity manifestations palsy have been reported.
of the nervous tissue due to antigenantibody reactions.
The lesions are due to scattered venous thrombosis in the Management
nervous system associated with altered coagulation of Patient should be referred to neurologist for further
blood. The lesions are due to repeated, transitory localized management.
vasoconstriction in various portions of the nervous system,
precipitated by emotional disturbances or fatigue. Myositis Ossificans
It is a condition in which fibrous tissue and heterotopic bone
Clinical Features form within the interstitial tissue or muscle, as well as in
Age and sex distribution: It occurs chiefly in younger associated tendons and ligaments. Secondary destruction
age group with an onset of symptoms between the ages of and atrophy of the muscle occurs, as this fibrous tissue and
20 and 40 years. There is slight female predilection with bone interdigitate and separate the muscle fibers.
familial occurrences.
Types
Symptoms: There is fatigability, weakness and stiffness of
the extremities with ataxia or gait difficulty, involving one • L
ocalized myositis ossificans or traumatic myositis
or both leg (Fig. 33.4). ossificans.
Other symptoms includes are area of superficial • Progressive myositis ossificans or generalized myositis
or deep paresthesia, personality and mood deviation ossificans.
Localized Myositis Ossificans resemble callus formation. The more mature tissue is
usually found on the periphery of the lesion.
It is also called post-traumatic myositis ossificans or
838 solitary myositis. Management
Sufficient rest should be given with limitation of use.
Etiology
Excision after process becomes stationary.
It is caused by acute or chronic trauma or heavy
muscular strains caused by certain occupation or sports.
Points to Remember
Traumatization of the periosteum of an adjacent bone
with the displacement of osteoblasts into the muscle and Solitary myositis, swollen tender painful at site of trauma,
subsequent formation of bone occurs. overlying skin is red, difficulty in opening of the mouth,
Activation of periosteal implants already present hemorrhage, degeneration of muscle, ossification,
in muscle by trauma or hemorrhage. Metaplasia of the chondrification and connective tissue hyperplasia.
pluripotential intermuscular connective tissue into the
bone and metaplasia of fibrocartilage can also be causative
factors. Progressive Myositis Ossificans
It is characterized by formation of bone in tendons and
Pathogenesis
fascia with subsequent replacement of adjacent muscle
Injury → hemorrhage into the muscle or associated tendon
by expanded bony mass. In some cases there is history of
or fascia → the hemorrhage organized and undergoes
hereditary and familial pattern.
scarring → during healing process cartilage is formed →
calcification of cartilage → ossification of cartilage. Clinical features
Age and sex distribution: It usually affects children
Clinical features
before 6 years of age. It is seen more in males as compared
Age and sex distribution: It can occur at any age, sex and
to females. It may advance rapidly or there may be long
more often in young persons.
period of relative inactivity with intermittent bursts of
Sites: The most commonly involved muscles are the activity.
masseter and sternocleidomastoid but in some cases lateral
Sites: Starts in muscles of neck and upper back and moves
pterygoid muscle can be involved.
to extremities.
Symptoms: Site of trauma remains swollen, tender and Soft tissue swelling that is tender and painful and may
painful much longer than expected. In some cases there is show redness and heat.
a mild discomfort associated with a progressive limitation
Signs: Gradual increase in stiffness and limitation of
of motion.
motion of neck, chest and back and extremities occurs.
Signs: The overlying skin may be red and inflamed. Ultimately entire groups of muscles become transformed
Intramuscular mass is palpated at 2 to 3 weeks. The lesion into bone resulting in limitation of movements.
may appear fixed or it may be freely movable on palpation. It is associated with congenital shortness of first meta
Oral Manifestations tarsal and metacarpal bones, shortness of little bone.
It involves the muscles of face particularly masseter and Interphalangeal joint may be fused. The masseter muscle
temporal following single traumatic injury. Some difficulty is frequently involved so that fixation of jaw occurs.
in opening of the mouth occurs. Petrified man: The patient becomes transformed into a
Histopathological features rigid organism called ‘petrified man’. Patient dies during
It exhibits varying stages from hemorrhage, degeneration of 3rd or 4th decades. Premature death is usually results from
muscle and connective tissue hyperplasia to chondrification respiratory embarrassment.
and ossification. Histopathological features
The osteoid and bone trabeculae formed often trap The muscle in this disease is gradually replaced by
viable muscle fibers but these may ultimately disappear. connective tissue which undergoes osteoid formation
The trabecular pattern is often extremely bizarre with and subsequently ossification. In some cases cartilage
the cartilage and myxomatous tissue present which may formation may become evident.
Management Multiple sclerosis is usually associated with trigeminal

Surgical approach can be considered for the management neuralgia.
of progressive myositis ossificans.
Clinical Features 839
Points to Remember
Age and sex distribution: It usually occurs in middle and
Tender soft tissue swelling, increase in stiffness of old age, the disease seldom occurs before 35 years of age.
muscles, petrified man, muscle replaced by connective Incidence increases with age due to degenerative changes
tissue, osteoid formation. of the nerve fibers. It most frequently occurs in women.
Site: It is more common on the right side and the lower
FACIAL PAIN portion of the face is more frequently affected. The pain is
Trigeminal Neuralgia confined to the trigeminal zone, nearly always unilateral
It is also called Tic Douloureux, trifacial neuralgia or and, if bilateral, is successive rather than concomitant. The
fothergill’s disease. Tic douloureux’ is only used when the mandibular and maxillary divisions are more commonly
patient suffers from spasmodic contractions of the facial involved than the ophthalmic; in some instances these two
muscles. divisions may be simultaneously affected. The pain never
Trigeminal neuralgia is an extremely painful condition crosses the midline.
as it is unique to humans. It is a syndrome in which Pretrigeminal neuralgia: There is dull, continuous, aching
symptoms are sufficiently distinctive to permit a reliable type of jaw pain which may persist for days prior to onset
diagnosis solely on the basis of history. of characteristic occurrence of paroxysmal pain in the same
region of the jaw. This is called pretrigeminal neuralgia.
Etiology This can show dramatic response to carbamazepine.
Idiopathic trigeminal neuralgia
Nature of pain: The pain is paroxysmal, lasting only a
Dental pathosis is believed by some investigators to be few seconds to a few minutes and is usually of extreme
involved with the onset of trigeminal neuralgia. It may occur intensity. It may be described by the patient as resembling
secondary to excessive traction on the various divisions of knife like stabs lightening, electric shock, stabbing or
the fifth nerve, being influenced by maxillomandibular lancinating type of pain. During the intervals between
relationship, allergic and hypersensitivity reaction causing these violent experiences, there is usually no pain or a mild
edema of the trigeminal nerve root. or dull ache.
Wolf thought that ischemia at various portions of the
trigeminal pathway might be responsible for the paroxysms Aggravating factors: The pain is provoked by obvious
of pain. stimuli to the face. A touch, a draft of air, any movement of
Jannetta and others have shown subtle changes of a the face as in talking, chewing, yawning or swallowing may
compression-distortion phenomenon which is usually caused evoke a lancinating attack. Later the pain may be so severe
by arterial loops of atherosclerotic vessels. Vessels become that the patient lives in constant fear of an attack. Often there
elongated with advancing age and with atherosclerotic is a transitory refractory period after the attack. As the attack
involvement gain abnormal positionsby wedging into the occurs, the patient may clutch his face as if in terror of the
space between the pons and trigeminal nerve. It is postulated dreaded pain.
that with progressive material elongation, fascicles of
Triggers zones: Trigger zones which precipitate an attack
adjacent nerves later suffer myelin injury and pain results.
when touched, are common on the vermilion border of
Secondary trigeminal neuralgia the lips, the ala of the nose, the cheeks, and around the
Conditions such as carcinoma of the maxillary antrum, eyes. The patient learns to avoid touching the skin over the
nasopharyngeal carcinoma and tumors of peripheral nerve trigger zones which frequently makes him go unwashed or
root can cause secondary trigeminal neuralgia. unshaven for days.
Benign tumors, as well as intracranial vascular The neurological examination findings are normal with
anomalies may present trigeminal pain. no objective sensory loss along the trigeminal nerve.
Histopathological Features Glossopharyngeal Neuralgia

Trigger point may show fibrosis and infiltration by chronic It is also called vagoglossopharyngeal neuralgia. It is a
840 inflammatory cells. In some cases, there is focal area of myelin variant of tic douloureux that can mimic oral pathologic
degeneration are seen along the course of cranial nerves. condition in which pain is confined to the distribution of
the ninth cranial nerve.
Diagnostic Criteria
• Abrupt onset of attack initiated by light touch Clinical Features
• Pain is paroxysmal, extreme and lancinating Age and sex distribution: This neuralgia occurs without
• Duration of spasm is less than 2 minutes any sex predilection in the middle aged or older persons.
• After attacks refractory periods occur
Nature of pain: It manifests as sharp excruciating, electric
• Pain is limited to known distribution of trigeminal
like, lancinating paroxysms of pain in the ear, pharynx,
nerve branch
nasopharynx, tonsils or the posterior portion of the tongue.
• Pain is diminished dramatically initially by the use of
The pain is generally unilateral. Pain often radiates to ear
carbamazepine.
due to involvement of tympanic branch of glossopharyngeal
nerve. Attacks have abrupt onset with short duration of 30
Management to 60 seconds which can be repeated.
Topical capsaicin cream (a nociceptive substanceP Glossopharyngeal neuralgia has a tendency towards
suppressor) over the skin may be effective. remissions and exacerbations. Pain free intervals of
Carbamazepine (tegretol) has a special effect on the seconds, minutes, hours, days, and years are common.
paroxysmal pain. The use of this drug causes paroxysms
Trigger zones: The patient usually has a trigger zone in the
to become separated by intervals of freedom for weeks,
posterior oropharynx or tonsillar fossa. An important and
months or even years. This is considered to be the best
frequent trigger is the initiation of the act of swallowing.
conservative treatment for trigeminal neuralgia. As an
Rarely patient may have syncope, seizure, hypotension,
initial dose, 100 mg is given twice daily until relief is
arrhythmia and excessive salivation.
established. At no time the daily dose should exceed
1200 mg. Side effects include dizziness, unsteady gait, Management
gastrointestinal distress, skin rashes and aplastic anemia.
Recently, baclofen an antispastic drug is also being used. Topical anesthetic agent: Maximum patient may get
Anticonvulsant like, phenytoin, gabapentin are effec instant relief when anesthetic agent applied to the tonsil
tive in pain control as they decrease conductance of Na+ and pharynx on the side of pain. But this is only last fo one
channels and inhibit ectopic discharges. hour so this is used as diagnostic test.
Nonsurgical procedure like microvascular decom- Drug treatment: Drugs which can be given in this case
pression, radiofrequency rhizotomy, and gamma knife are carabamazepine, oxcarbazepine, baclofen, phenytoin
radiosurgery of gasserian ganglion are also effective and lamotrigine. But these drugs are less effective in
in younger patient. After surgery patient can have glossopharyngeal neuralgia as compare to trigeminal
complication like facial dysesthesia (distorted sensation neuralgia.
of facial skin) and anesthesia dolorosa (combination of
anesthesia and spontaneous pain. Surgical management: Microvascular decompression
or surgical sectioning of glossopharyngeal nerve can be
Points to Remember carried out.
Tic Douloureux, common on the right side, pretrigeminal
Points to Remember
neuralgia, knife like stabs’ ‘lightening’, ‘electric shock’,
‘stabbing’ or ‘lancinating’, triggers zones, trigger point Vagoglossopharyngeal neuralgia, sharp excruciating,
may show fibrosis, infiltration by chronic inflammatory electric like, lancinating paroxysms of pain in the ear,
cells, topical capsaicin cream, carbamazepine, phenytoin, pharynx, trigger zone in the posterior oropharynx,
gabapentin, microvascular decompression, radiofrequency topical anesthetic agent, carabamazepine, microvascular
rhizotomy. decompression.
Geniculate Neuralgia does not have characteristic of the cranial neuralgias

and is not associated with physical signs or demonstrable
It involves the intermediate nerve of Wrisberg, an
organic causes.
important component of the facial (VII) nerve. It is caused 841
Diagnosis of atypical facial pain is not easy diagnosis
pathological involvement of the sensory intermediate nerve
for the doctor to make. Patient usually travels from one
root of the VIIth cranial nerve due to neuroma; vascular
doctor to another for the relief from the pain. To make
malformations etc are the cause.
diagnosis disease like cracked tooth syndrome, allergic
Causes sinus, referred pain to face, myofascial pain impingement
of bone on nerve and TMD disorders and tumors should be
Neuroma and vascular malformation: Pathological
rule out.
involvement of the sensory intermediate nerve root of the
VIIth cranial nerve due to neuroma; vascular malformations Clinical Features
etc are the cause.
Age and sex distribution: It is usually seen 4th to 6th
Zoster infection: Zoster infection of geniculate ganglion decade of life with more common in women as compare
canal also cause this type of neuralgia. This is also called to men.
Ramsay Hunt syndrome.
Location: It more commonly affects one quadrant and may
Clinical Features extend to temple, neck or occipital area.
When the triggering is caused by touching the ear, topical Nature of pain: Pain is continuous, deep, diffuse, gnawing
anesthesia of the external auditory canal may arrest it. and sharp. Patient may called it as drawing, aching or
pulling.
Age and sex: Females are affected more commonly than
males. It occurs more commonly in old aged persons. Signs: Area of pain may contain zone of increase
temperature, tenderness and increase bone marrow activity.
Location of pain: Ear, anterior tongue, soft palate.
Nature of pain: The pain may be felt in front and deeply
Management
in the ear with occasional pain in the palate and the tongue Gabapentin: This anticonvulsant drug can give dramatic
and deeply in the facial musculature. relief in many cases.
Trigger zone: Triggering is caused by touching the ear. Opioid analgesic: Opioid analgesic like codeine, fentanyl,
hydrocodone, morphine can give relief initially. But their
Management continuous use may lead to addiction.
Topical anesthesia: Topical anesthesia of the external Tricyclic antidepressant like amitriptyline, nortriptyline
auditory canal may arrest pain in some cases. can also be used.
Other therapy like topical capsaicin for area of localized
Steroid: Short course of high dose steroids therapy is useful. pain, psychotherapy, behavioral modification, TENS and
Acyclovir: This is helpful in case of geniculate neuralgia removal of affected trigeminal nerve gland can also be
associated with zoster infection. used.

Ramsay Hunt syndrome, pain in ear, anterior tongue, Idiopathic facial pain, continuous, deep, diffuse,
soft palate, trigger by touching the ear, topical anes gnawing and sharp, pain, zone of increase temperature,
thesia, steroid, acyclovir. gabapentin, opioid analgesic, tricyclic antidepressant.
Atypical Facial Pain Neuralgia-inducing Cavitational

It is also called atypical facial neuralgia, idiopathic Osteonecrosis
facial pain, atypical trigeminal neuralgia and trigeminal It is also called NICO, alveolar cavitational osteopathosis
neuropathic pain. The international headache society bone marrow edema. It is controversial topic in the
defines atypical facial pain as persistent facial pain that diagnosis of orofacial pain.
Ischemic osteonecrosis can be cause by degeneration Ischemic myelofibrosis: There is wispy fibrous streaming
and death of marrow which results in decrease in bone between fat cells.
marrow flow resulting in necrosis of bone. This type of Intramedullary fibrous scar: There are of dense fibrosis.
842 osteonecrosis typically results in neuropathic type of pain
and that is reason this is included in this chapter. Cavitations: These are extracellular cystic space which
then coalesces to form large space which extent from
Clinical Features cortex to cortex.
Age and sex distribution: It is usually seen in women and Microinfarction: There is focal areas of marrow hemorr
3rd to 6th decade of life. hage.
There is also calcific necrotic detritus which smudged
Site: Third molar are is most frequently affected. Other globular dark masses which represent destroyed trabe
site which are involved are walls of sinus and mandibular culae.
condyle.
Nature of pain: Pain is described as deep ache or sharp Management
bone pain. It begins as initially mild and vague which Antibiotics: This will combat infection and decrease pain.
increase in intensity over the time. Pain can be referred to
Decortications and curettage: This can be done to remove
some distance from affected bone (Fig. 33.5).
dead marrow and bone.
Points to Remember
It appears as area of regional osteoporosis with ill defined
NICO, deep ache or sharp bone pain, Bulls eye lesion, hot
radiolucency. There are also vertical remnants of lamina
spot on Technetium99m scan, Plasmostasis, ischemic
dura. In some cases there mixed sclerotic and radiolucent
myelofibrosis, intramedullary fibrous scar, cavitations,
area.
microinfarction, calcific necrotic detritus, antibiotics,
Bulls eye lesion: Faint centrals sclerotic oval surrounded decortications and curettage.
by thick radiolucent circle which again surrounded by thick
and faint sclerotic ring. This is called bull’s eye lesion.
Cluster Headache
Technetium-99m scan show hot spot with increase uptake. It is also called migrainous neuralgia, sphenopalatine
Histopathological Features neuralgia, histamine cephalgia and Horton syndrome.
This is not common and has been called ‘the most pain
Bone marrow edema show dilated marrow capillaries, syndrome known to human’.
sinusoids. This can be cause by abnormal hypothalamic
Plasmostasis: There is serous ooze around blood vessels. function and abnormal release histamine from mast cells.
Figure 33.5 Referred pain pattern of NICO

Precipitating factors of headache are alcohol, cocaine, Paroxysmal Hemicranias

smoking and nitroglycerine.
The clinical features of this disease are similar to cluster
Clinical Features headache. This can be differentiated from cluster headache 843
by shorter duration of attacks with high frequency. Paroxys
Age and sex distribution: Can occur at any age with male mal hemicranias also respond dramatically to indomethacin.
predilection in the ratio of 6:1.
Clinical Features
Location: It is unilateral pain which follows the course of
ophthalmic division of trigeminal nerve. Age and sex distribution: This is more common women.
Nature of pain: It is deep felt in or behind the orbit. Site: Pain is unilateral and present on ocular, maxillary,
It can radiate to temporal and upper cheek region. Pain temporal and frontal region.
is paroxysmal and interns with burning or lancinating Nature of pain: Pain last for 2 to 30 minutes. Pain is of
quality. Attacks last for 15 minutes to 3 hours (Fig. 33.6). boring nature. Attacks occur 2 to 40 in a day.
Alarm clock headache: The pain begins at the same time Other features: There is lacrimation, conjunctival infec
in given 24 hours period with attack occurring in midnight. tion and rhinorrhea.
Other features: Other features like nasal stuffness, tear Management

ing, facial flush congestion conjunctival blood vessel.
Indomethacin is drug used in this disease. There is
Management resolution of symptoms within 2 days. It should be given
25 mg TDS for 10 days. If dose is not sufficient it can be
Drugs which can be use in cluster headache are prednisone,
increase to 50 mg TDS.
ergotamine, lithium carbonate, indomethacin, methysergide
maleate, verapamil and sumatriptan shows some relief in Points to Remember
the patient.
Boring pain, indomethacin, lacrimation, conjucntival
Neurosurgical intervention also can give relief in some
infection and rhinorrhea.
patient.
Points to Remember Migraine

Migrainous neuralgia, deep felt pain in the orbit, Alarm It is also called migraine syndrome or migraine headache.
clock headache, neurosurgical intervention. It is a syndrome presenting manifestations of diffuse
disturbances in body functions occurring during or
after stress, characterized by severe periodic headache,
irritability and nausea.
Cause
The cause of the disease is unknown but has seen postulated
to be a discharge of autonomic centers in the forebrain leading
to constriction in portions of the cerebral arterial tree.
In susceptible patients this may become manifest
in the preheadache phenomenon. Then, as part of an
attempt to maintain cranial homeostasis there is decrease
in constrictor in certain other cranial arteries, particularly
branches of the external carotid. These secondary effects,
possibly hormonal as well as neurogenic in origin are the
source of the headache.
Reduce activity of serotonin lead to vasoconstriction
which leads to cerebral ischemia, which is followed by
Figure 33.6 Cluster headache showing alarm clock pattern compensating vasodilatation with pain and cerebral edema.
Trigger Temporal Arteritis

Oral contraceptive, stress, alcohol, chocolate, missing a It is also called giant cell arteritis, cranial arteritis. It is
844 meal, flashing light, odors, lack of sleep, head trauma, multifocal vasculitis of cranial arteries usually temporal
physical exertion, fatigue, weather changes, fluorescent artery. It is caused by autoimmunity to elastic lamina of
lights, aged cheese and menstruation. the artery.
Clinical Features
Clinical Features
Age and sex distribution: It is seen in old age above 50
Age and sex distribution: It is usually seen in third decade years of age. Women are affected more commonly than
of life. Women are more frequently affected than men. family.
Site: The headache pain consists of severe pain in the Nature of pain: There is unilateral throbbing headache
temporal, frontal and retro orbital areas although other sites which later on intense, aching, burning and lancinating
such as parietal, post auricular, occipital or sub occipital are pain. Pain coincides with heartbeat (Fig. 33.7).
also occasionally involved. The pain is usually unilateral
but may become bilateral and generalized. Signs: Superficial temporal artery is sensitive to palpation
and may become erythematous, swollen, and tortuous.
Nature of pain: The frequency of attacks in extremely
variable as they may occur at frequent intervals over period Jaw claudication: There is pain while mastication. This is
of year or on only a few occasions during the lifetime of called jaw claudication.
patient. The pain is described as a deep aching, throbbing Eye involvement: There is loss of vision and retroorbital
type. pain. There may be blindness which is cause by involvement
Other features: Other features like nausea vomiting, of posterior cilliary artery. This artery supplies optic disc
diarrhea, photophobia and phonophobia can occur. which results ischemic papillopathy.
A prodromal stage (preheadache phenomena is noted Polymyalgia rheumatica: There is generalized muscle
by some patients consisting of lethargy and dejection aching and stiffness.
several hours before the headache. Visual phenomena such
as scintillation (seeing sparks), hallucinations or scotoma Other features: There is fever, malaise, nausea, anorexia,
(partial or complete loss of light perception), aphasia vomiting sore throat and earache.
(loss of ability to express thought) and temporal or partial
blindness are often described.
Management
The treatment of migraine includes a wide variety of
drugs ranging from acetylsalicylic acid and codeine to
ergotamine, methyl salicylic and nor epinephrine.
Cognitive behavioral therapy—volitional modulation
of stress response should be done.
The prognosis of the disease is good, since the
condition is not dangerous, and may undergo complete and
permanent remission.
Points to Remember
Severe pain in the temporal, frontal and retro orbital
areas, deep aching, throbbing type pain, nauseas
vomiting, scintillation, scotoma, aphasia, acetylsalicylic
acid, cognitive behavioral therapy.
Figure 33.7 Temporal arteritis pattern of pain
Histopathological Features 0.7 and 4.5 percent. It is considerably more common in

women than men the peak prevalence in the fourth to sixth
There is chronic inflammation of tunica intima and tunica
decades of life. Burning mouth also often coexists with
media of the temporal artery. There is also narrowing of 845
other chronic pain disorders.
lumen from edema and proliferation of the tunica intima.
There is also multinucleated giant cells which are mixed Cause
with macrophages, plasma cells and lymphocytes.
There are various local and systemic factors which can
Management cause burning mouth syndrome.
Corticosteroid: This disease respond well systemic and Systemic factors: As it is found more common in
topical corticosteroid. postmenopausal women, estrogen deficiency is thought
to be causative factors. Other systemic factors which can
Points to Remember be associated are vitamin deficiency, diabetes mellitus,
Giant cell arteritis, unilateral throbbing headache, gastritis, hypothyroidism, mercurlism and Parkinson
aching, burning and lancinating pain, Jaw claudication, disease.
polymyalgia rheumatica, chronic inflammation of
tunica intima and tunica media of the temporal artery, Local factor: Local factors like temporomandibular
multinucleated giant cells, corticosteroid. dysfunction, OSMF, oral candidiasis, trigeminal neuralgia
and xerostomia can be responsible for burning mouth
syndrome.
Burning Mouth Syndrome
It is also called stomatopyrosis, stomatodynia, burning Autoimmune: Abnormal level of antinuclear antibodies
tongue syndrome and glossopyrosis. and rheumatoid factors are found in serum of patient of
Burning Mouth Syndrome (BMS), is condition burning mouth syndrome.
characterized by a sensation described by the patient as Psychological factors: It include anxiety, depression,
stinging, burning that affects the oral mucosa, in the absence compulsive disorders, psychological stress and cancero
of clinical or laboratory data to justify these symptoms. phobia.
There is burning and pain of the mouth or of the
Idiopathic factor: Most times there was no cause found
tongue, without any visible changes is common with
for BMS.
many causes. When the tongue is involved, it is called
glossopyrosis. When mucosal surface is involved, it is Classification and Subtypes
called stomatopyrosis.
It is a chronic orofacial pain, unaccompanied by mucosal Different classification types have been proposed based on
lesions or other evident clinical signs upon examination. the daily fluctuations of the symptoms (Table 33.1).
Careful clinical histories, a general physical examination,
a detailed examination of the oral cavity are mandatory to
avoid misdiagnosis and inaccuracies in treatment of BMS. Table 33.1 Classification of BMS subtype
Clinical findings Association
Definition Type 1 Daily pain, not present Nonpsychiatric
It is defined as “an intraoral burning sensation for which upon awakening,
no medical or dental cause can be found” (Headache worsens as the day
Classification Committee of the International Headache progresses
Society, 2004). Type 2 Constant pain Psychiatric, chronic
anxiety
Epidemiology Type 3 Intermittent pain in Allergic contact anxiety
The actual prevalence of BMS is difficult to ascertain unusual sites (floor of due to preserving agents
however, vary widely, with prevalence varying between mouth) and additives
Fundamental inclusion criteria for BMS (Scala et al) Usually the onset is spontaneous but at times there is
• Burning sensation located in some area of the oral mucosa;
a precipitating event such as trauma or dental treatment.
• Persistence of the manifestations for at least 4 to 6 months; There can be an associated xerostomia, dysesthesia and/or
846 dysgeusia.
• Continuous burning sensation throughout the day, or with
increased intensity towards the afternoonevening; Nature of pain: The patient complains of intense and
• Infrequent associated sleep disturbances; and
unbearable pain that interferes with eating, sleeping and
• Symptoms relief upon eating or drinking
virtually every other body function. Usually, there is
Additional inclusion criteria continuous and spontaneous with an intense burning
• Dysgeusia/or Xerostomia sensation reported by the patient as if the mouth or tongue
• Sensory or chemosensory alteration were scalded or burnt.
• Mood changes or psychopathological alterations.
Tongue: The tongue may appear as red or atrophic or
the mucosa may appear entirely normal. The complaints
are usually limited to tongue but may involve the entire
In 1994, Lamey et al, divided the syndrome into three
mucosa.
types based on different types of patients
• Type 1: Characterized by progressive pain, patients Psychological dysfunction: Patient who suffers from
wake up without pain, which then increases burning mouth syndrome may be having depression,
throughout the day, affects approximately 35 percent anxiety, or irritability.
of patients. This type may be associated with systemic
diseases, such as nutritional deficiencies. Diagnosis
• Type 2: The symptoms are constant throughout The diagnosis of burning mouth syndrome depends on ex
the day and patients find it difficult to get to sleep, clusion of a detectable organic basis for the complaint. The
represents 55 percent. These patients usually present diagnosis of BMS requires careful evaluation of the symp
associated psychological disorders. toms, with due consideration of a series of inclusion criteria.
• Type 3: Symptoms are intermittent, with atypical
location and pain. Constitutes 10 percent of patients. Management
It seems that contact with oral allergens could play Removal of causative factors: Removal of factor which is
an important etiologic role in this group. causing the burning mouth syndrome should be done.
Mood altering drug: Mood altering drug like chlordia
Types (Cerchiari et al classified BMS according to zepoxide may be beneficial in some patient.
the associated risk factors:) Other drug like clonazepam, amitriptyline, TENS,
vitamin B complex and psychological counseling should be
• Idiopathic
given.
• Psychogenic
• Local and Points to Remember
• Systemic.
Stomatopyrosis, intense and unbearable pain, red tongue,
Scala et al proposed two clinical forms of BMS as psychological dysfunction, clonazepam, Mood altering
• ‘ Primary’ or essential/idiopathic BMS, in which the drug.
causes cannot be identified.
• ‘Secondary’ BMS, resulting from local factors or
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1. Trigeminal neuralgia is also called: 7. Myopathic facies and swan neck seen in:
a. Tic douloureux b. Trifacial neuralgia a. Myasthenia gravis b. Bell’s palsy
c. Fothergill’s disease d. All c. Dystrophic myotonia d. Motor system disease
2. Following are the trigger zones for trigeminal neuralgia 8. Purplish black intrinsic staining of teeth seen in:
except: a. Dermatomyositis b. Congenital myotonia
a. Tongue b. Lips c. Both d. None
c. Ala of nose d. Cheeks
9. Minor Starch-Iodine test is done for:
3. Bell’s palsy affects: a. Bell’s palsy b. Frey’s syndrome
a. V cranial nerve b. VII cranial nerve
c. Both a and b d. None of the above
c. VIII cranial nerve d. X cranial nerve
10. Staccato type of speech is an interesting feature of:
4. Syndrome associated with cranial nerve is:
a. Multiple sclerosis b. Pretrigeminal neuralgia
a. Bowen syndrome
b. Book’s syndrome c. Fothergill’s disease d. Dental pathosis
c. MelkerssonRosenthal syndrome 11. Ramsay Hunt syndrome is associated with:
d. Mohr’s syndrome a. Vascular malformations
5. ‘Crocodile tears’ seen in: b. Overdose of phenytoin
a. Frey’s syndrome b. Trigeminal neuralgia c. Zoster infection of geniculate ganglion canal
c. Motor system disease d. Bell’s palsy d. Glossopharyngeal neuralgia
6. Minor starch iodine test is perform to diagnose: 12. Alarm clock headache is the characteristic feature of:
a. Bell’s palsy b. Frey’s syndrome a. Gustatory sweating b. Cluster headache
c. Trigeminal neuralgia d. Muscle dystrophy c. Tic douloureux d. Multiple sclerosis
34 Forensic Odontology
Anil Govindrao Ghom, Savita Ghom
Chapter Outline
Â Record management Â Physical anthropologic examination of bones and teeth

Â Identification Â Postmortem serology and DNA profiling
Â Dental evaluation Â Bite marks
Â Personal recognition Â Human abuse
Â Fingerprinting Â Dentist as expert witness
INTRODUCTION Treatment plan which is given to the patient should be

Role of Forensic Odontology
updated in the record. All the letter of reference, letter of
Forensic odontology is the subject concerned with the • Record
consent, preparation:
insurance The correct
and financial handling
statement and be
should exam-
store
application of medical and paramedical science knowledge ination and the proper
in the record of the patient. preparation and presentation
to certain branches of law, both civil and criminal. The of dental
The evidence
progress note inofboth
the civil and should
patient criminalcontain
legal
medicolegal information obtained from the examination of procedures.
information about the restorative and therapeutic procedure
teeth and jaws falls in the preview of forensic odontology. • Identification:
which is carried out Personal identification, either indi-
on to the patient.
Upon exposure to physical injury and putrefaction, the Summaries of the telephonic disasters.
vidually or in context of mass conversation with the
human dentition, the enamel of which is the hardest • Bite mark investigation: Investigation
patient, consultant, insurance company representative of criminal
and
substance in the body outlasts all other tissues. The material cases where bite marks are involved
legal authorities should be maintained in the record. and the
used in restoration is also extremely resistant to destruction interpretation
Record should beof bite
signedmarks.
by the personnel. Any change
by chemical and physical elements. The fundamental •
H uman abuse: Recognition
made in the record should not erase of domestic,
but a lineand childbe
should
principles of dental identification are those of comparison abuse.
crossed on it, so that it is readable. This will help to remove
(when antemortem records of the proposed deceased are • Legal aspect: Legal
any fraud intention aspect
to alter of dental traumatology.
record.
available) and exclusion (when antemortem records of • Others: Forensic odontology also deals with age as-
other persons are available). In the absence of the ante- sessment of patient and comparison and identifica-
mortem records attempt to elicit dental information is made tion of lip print.
by interrogating the relatives and friends of the deceased,
which may frequently prove unreliable.
RECORD MANAGEMENT
Definition
The dental record is a legal document of dentist which
Forensic odontology may be defined as the application contains information about subjective and objective
of dental science to the administration of the law and the finding of the patient. It also includes pathological report,
furtherance of justice. radiographs, and clinical photographs of the patient.
Treatment plan which is given to the patient should be

Method
updated in the record. All the letter of reference, letter of
consent, insurance and financial statement should be store • Dental evaluation
850 in the record of the patient. • Personal recognition
• Fingerprinting (friction ridge analysis)
The progress note of the patient should contain
• Physical anthropological examination of bones
information about the restorative and therapeutic procedure
• Serologic and genetic comparison technique.
which is carried out on to the patient.
Summaries of the telephonic conversation with the
patient, consultant, insurance company representative and Results
legal authorities should be maintained in the record. • P ositive identification: No major difference found
Record should be signed by the personnel. Any change between antemortem and postmortem data.
made in the record should not erase but a line should be • Presumptive (possible identification): Some infor-
crossed on it, so that it is readable. This will help to remove mation is missing form antemortem or postmortem
any fraud intention to alter record. records to have positive identification. There is lot of
It is common now a day that dental record to be similarities between ante and postmortem record
maintained electronically. New software programs • Insufficient identification evidence: There is less
are developed to maintain patient dental information. supportive evidence to come at the conclusion on
This is advantageous as it can be easily networked and scientific principle.
transferred. • Exclusion of identification evidence: There are dis-
Record should be kept minimum of 7 to 10 years. In the crepancies seen between antemortem and postmor-
case of pediatric patient record should be maintain until the tem data. Exclusion is as important as positive iden-
patient reaches the age to maturity. tification.
IDENTIFICATION DENTAL EVALUATION

Forensic odontology is concerned with the identification of Dental Comparison
both living and deceased person.
Dental comparison affords a potentially straightforward
and simple means of establishing identity. The method of
Issues of Death Investigation dental identification depends upon:
• C ause of death: The sequence which initiated the ∙ The relative resistance of the mineralized dental
death of person like physical, chemical injury or tissues and dental restoration to changes resulting from
disease of person. decomposition or harsh environment extremes such as
• Mechanism of death: The pathological process which conditions of temperature and violent physical forces.
results in death. ∙ The unique individual characteristic of the dentition
• Mode or manner of death: Whether it is natural, and dental restoration.
accidental, suicidal or homicide. ∙ The availability of documentation of the antemortem
• Undetermined death: The manner of death is not status of the dentition in the form of dental treatment
established due to decomposition, dismemberment records and diagnostic radiographs.
or postmortem destruction of remains by insect or Each individual has 32 teeth with 5 surfaces each with
feral animals. their own character of size, shape, position and spacing
with the result that no 2 sets of teeth are alike.
The death certificate is required for probation of will, Teeth extracted after death leave a completely different
life insurance claim, and resolution of affairs associated socket from those removed during life. When the tooth
with settlement of an estate. is removed or dental work of any sort is carried out the
Forensic Odontology
teeth pattern is changed and its record may exist with the Localized wear on certain teeth: A pipe smoker may
dentist. have localized wear of teeth either on incisors or at angle
In natural decomposition, teeth are practically of mouth due to position of pipe. Notched incisors from
indestructible. They are not easily destroyed by fire. Being holding thread, pins, nail, between teeth on day-to-day 851
sheltered in the oral cavity, they are generally not damaged. basis may suggest the occupation of tailor or hairdresser
Teeth as well as dentures made of acrylic resin are generally or cobbler.
resistant to the action of corrosive acids. The identification
Missing teeth: The missing tooth may have been lost
from data of authenticated teeth depends entirely upon the
ante mortem or postmortem. Antemortem loss of teeth
accuracy and completeness of authenticated records made
due to trauma at or near the time of death if frequently
during life.
associated with fracture of thin bony plate surrounding
the alveolus. In loose tooth which has fallen out, it is not
Composition of Dental Records
so. Extraction or tooth loss in living person is followed
• T he number and situation of teeth present and number by bleeding from its socket which stops in about 24 hr
and situation of teeth lost. or sometimes 2 to 3 days when the clot forms in the raw
• Arrangement, irregularities, erosion, caries, fillings, socket. By about 14 days, the clot is obliterated by fibrous
bridge crown work and dentures. tissue and the alveolar rim is smoothened by resorption of
• Exact shape of edentulous arch. bone. By about 5 to 6 months, gradual new bone formation
• Some of the common identifying features of teeth fills the socket but its outline is still visible on X-ray
pertain to faulty development, faulty alignment, examination. By about 6 months to 1 year, remodeling of
presence of stains, localized wear on certain teeth new bone completely obliterates the socket leaving a slight
and missing teeth. depression and the socket outline is not visible on X-ray
examination. In recently recovered remains, postmortem
Faulty development: Teeth may be undersized, oversized,
tooth loss discloses a clean socket devoid of blood clot. In
notched or present some other irregularity as a result of
skeleton in which postmortem loss of teeth is common, the
faulty development and malformation. Hutchinson’s teeth
bony rim of alveolus is sharp and feathered.
constitute a classical example of malformation of the incisor
in congenital syphilis. These changes are most conspicuous Procedure or Guideline for
in central incisors which are usually small, widely spaced,
Dental Identification
notched and less broad at the cutting edge than at the gum
margin giving them the appearance of tip of screw driver. It should include not only the oral cavity of the victim but
when applicable, the surrounding scene as well, especially
Faulty alignment: The defect in the alignment may be
in case of conflagration or when only the remnants of the
in the space between teeth, e.g. widely spaced teeth or
dental arches may remain scattered and rubble and debris.
overriding teeth. Between the teeth of the upper and lower
jaw when there is protrusion of upper incisors resulting Postmortem Examination
in overlap of lateral incisors the bite pattern is known as
Recovery of dental structure: The task of dental
overbite and the reverse pattern is known as cross-bite.
identification begins at the site of discovery of the body.
Stains: Pan (betel leaf, tobacco) chewing habit stains the When the gross postmortem changes affecting the teeth
teeth with dark brown or black deposits. Yellowish or dark have occurred, such as charring, disintegration and
brown stain on the back of incisor teeth is common in fragmentation in fires and high impact accident, meticulous
cigarette smokers. Chalky white or yellowish brown areas care in their recovery and conveyance to the mortuary are of
of discoloration are found in fluorosis. Metal poisoning utmost important. Displaced teeth in decomposed bodies or
may cause pigmentation of gums and there by suggest a skeletal remains should be saved, labeled and later secured
cause of death. Copper causes green and mercury and lead to the intraoral position using adhesive cement. Most open
a blue black line on the gums. Gum hyperplasia induced by alveoli are the result of postmortem tooth loss. In recently
phenytoin may aid in identification and suggest epileptic recovered remains, postmortem tooth loss disclose a clean
seizure as a cause of death. socket devoid of blood clots.
Instrument: Instruments used for dental examination Failure to recover significant material may result in failure
includes explorer, mirror, tissue forceps, heavy duty to identify a body.
autopsy scalpel, handle and blades, tissue clamps, irrigating
852 syringe, rubber autopsy gloves, polythene specimen Antemortem Record Examination
bags, gauze sponges (for tooth cleansing) and a source of Acquiring antemortem data: This data can be obtained
illumination (flash light or battery operated head lamps); from police, medical examiner. Forensic dentist should
and dental examination form should be used. determine that records indicate name of person to be
Photography: It should be taken of full head and face views. identified, and name and address of submitting dentist.
Images of occlusal plane of maxillary and mandibular arch Inadequate antemortem data: Errors in charting teeth
should be taken. treated and insufficient descriptive details about the
Reconstruction and examination: Examination should be treatment provided are common. Other difficulty arises
performed by two persons thoroughly familiar with dental when a dentist has retired and destroyed his records.
terminology, one actually performing the examination and Comparison of Antemortem and
one recording the data. The recorder should view the actual
Postmortem Record
teeth in order to record the basic morphological pattern of
the restoration or cavities. In some cases, it is necessary After all the records are collected, it is compared to
to remove the jaw from the body for more detailed similarities and discrepancies. Forensic dentist must rely
examination and future reference. on antemortem records given to him are truly of those
person that are purported to represent.
Radiography: The use of double pack intraoral radiograph
should be used. When placement of intraoral film is not Acceptable discrepancies: In some cases, acceptable
possible occlusal film or lateral oblique film should be discrepancies are allowed. For example, if an antemortem
used. record shows deciduous teeth and postmortem records
shows no deciduous teeth. In this case as tooth is exfoliated
Charting of dental records (odontogram): The point to this is allowed.
be considered while charting are missing teeth, unerupted
teeth, supernumerary teeth, restoration, prosthesis, dentures, Problems in comparison: Many time postmortem dental
decayed, broken teeth, mal position, overlapping, crowding materials is compromised due to fractured, avulsed, melting
and spacing, peculiar shape of teeth. at high fire. When antemortem records radiographs are of
poor quality then also there are problems in comparison.
Identification of edentulous bodies: Frequently dentures
are present in the mouth of unknown bodies or may be found Written conclusion: It should be based on objective
elsewhere. If the denture can be identified and it can be shown analysis of data presented and it should be supportable or
to fit the mouth of the deceased, a reliable identification defensible in court of law.
can be made. The most reliable means of identification of Role of Dentist in Multiple (Mass) Fatality
denture is for them to be permanently marked with the name
Incident Identification
of the patient or some code during manufacture.
Mass fatality incidence (MFI) can occur due to natural,
Problems in identification: It depends upon the circums- accidental and criminal (serial homicide, mass suicide and
tances surrounding the death and the care exists in its acts of terrorism).
collection and transport. Incineration produces damage
to teeth ranging from mild scorching of the surface to Natural: It include earthquakes, tornadoes, hurricanes,
severe charring of the enamel and dentine with crumbling volcanic eruption, fire storms, tsunamis and floods. Victims
of the crown. Sustained very high temperature will result in natural disaster are scattered throughout broad area.
in calcinations of the teeth with considerable overall Many of the victims are unknown to areas as they may be
shrinkage. Burnt teeth are usually very fragile and suffer tourist. Another problem occur in natural disaster, there is
separation of the enamel and often gross disintegration of difficulty in retrieval of information and as dental records
the crowns. In high impact accidents such as aircraft and may be destroyed in natural disaster.
high speed road crashes much mechanical damage can Accident: It is cause by transportation accident, fires,
occur and teeth and jaws may fracture and disintegrate. industrial and mining accidents. Many times in industrial
Forensic Odontology
and mining accidents as people wear same short of cloths, Radiography can provide information in relation to age,
it is very difficult to identify these bodies sex, race, and occupation, diagnosis of certain conditions
and identification and cause of death.
Criminal disasters: The rise in national and international 853
terrorism in 21st century has lead to more participation of UV rays: An ultraviolet lamp can be used to locate and
forensic dentist in identification. define tattoo marks and scars on burned and decomposed
remains, and to segregate bones in cases of mix-up. When
Age: Age can be established by radiography of bones
examined by UV light washed blood stains are readily seen
and teeth (for root calcification). Calcification of costal
and seminal stains give a bluish white fluorescence.
cartilage and osteoarthritic changes in large joints and the
spine also help. Digital photography: The camera used should be LSR
digital camera with interchangeable lenses.
Sex and race: May be deduced by radiography in some
cases. Direct digital radiography: This device creates direct image
of on the pixels of its CCD or CMOS. This will save time so
Occupation: This can sometimes be deduced from X-ray.
it is recommended for clinical and forensics casework.
The whole range of pulmonary occupational diseases such as
silicosis, asbestosis may show specific radiographic findings. Cone beam computed tomography CBCT: It pro vide 3D
The radial artery in laborer’s using pneumatic drill may show imaging modality to collect complete maxillamandibular
calcification; coal carriers and professional wrestlers are facial anatomic volume of data.
liable to calcified lesions of the ligamentum nuchae. Football
X-ray fluorescence (XRF) methodology: Analysis
players may show calcified hematoma of the thigh muscle.
of dental material in cremation and difficult forensic
Identification: To is possible by comparison of postmortem identification case may be facilitated by this technique.
and antemortem X-ray.
PERSONAL RECOGNITION
Cause of death: Fracture of bones seen on X-ray may
indicate their antemortem origin and these include It is least reliable method used to identify the individual.
depressed fracture of skull, fracture of hyoid, fracture This is done by visual identification by family member,
dislocation of cervical vertebrae, severe injury to bones friend or acquaintance. Many time there is misidentification
by cutting instrument or fracture of several ribs which of the body by this way.
are incompatible with life. Foreign bodies in the upper
respiratory tract provide valuable clue. Evidence of FINGERPRINTING
poisoning by heavy metals and signs of diseases such as
It is analysis of epidermal friction ridges of the finger,
malignant growth may be apparent.
palms and feet. This pattern is unique for each person.
Technologic aids in multiple fatality incident analysis: These fingerprint are genetically determined and even
These include X-ray, UV light, postmortem serology and fingerprint of twins are not identical.
DNA profiling.
X-ray: All bodies which are found under suspicious PHYSICAL ANTHROPOLOGIC
circumstances and which are rendered unrecognizable due EXAMINATION OF BONES AND TEETH
to prolonged immersion in water, burning by fire and acid It is analysis of calcified structure of body bone and teeth.
or by any other destructive means such as explosion should Chronological age assessment may be an important factor
be routinely X-rayed. A dental radiograph when available in establishing the identity of the living or deceased person.
constitutes one of the most valuable pieces of evidence for It is also important in legal proceedings when specific
identification purpose. Panoramic X-ray technique provides charge for particular offence may depend on whether the
excellent pictorial dental record. alleged offender is a juvenile. Stage of eruption of the teeth
Computer software technology: The CAPMI (compu- and evidence of changes due to function such as attrition
ter assisted postmortem identification) system compares can give an approximate estimate of age. Radiography can
dental record of victims of mass disaster and enables rapid provide a great detail, the gross stage of dental development
identification of air crash, flood and explosion victims. of the dentition.
Histological: It requires preparation of the tissue for

detailed microscopic examination, which can determine
more accurately the stage of development of the dentition.
854
Physical and chemical analysis: It is done to determine
alterations in ion levels with age have been proposed.
POSTMORTEM SEROLOGY AND

DNA PROFILING
A known postmortem grouping of an individual serves to
narrow the range of possible identities. Even in putrefied
bodies, blood group antigen may be detectable for
serological studies. The bone marrow in skeletal remains
may still retain serologically detectable antigens. This is Figure 34.2 Bite marks on inmate subject
useful if suitable tissue (blood, semen stored in bank) is
available. If such tissue is available, a DNA profiling or
autopsy derived tissue should be compared by single probe
analysis with that of parent, children, sibling and if be
necessary other relatives. This is now used worldwide in
aircraft and other major accidents. The techniques which
are used for DNA profiling are restriction fragment length
polymorphism (RFLP) and polymerase chain reaction
(PCR). The RFLP results in splitting source DNA into
thousands of fragment. The PCR can do evaluation of
denture DNA or minute quantity of DNA.
BITE MARKS (FIGS 34.1 TO 34.3)

One of the two major interests of the forensic odontologist
is one that has direct relevance to the pathologist, in that it
concerns the interpretation of trauma to the body surface. Figure 34.3 Sexual bite mark
Dental evidence is used to identify the perpetrators of a

crime who happened to have left their teeth marks in
some substances left at the scene. Generally, it is easier to
exclude a certain person than to identify one conclusively
from the bite mark.
Definition
A bite mark is a patterned injury produced by teeth on
animate or inanimate objects, is caused by small enamel
defects on the incisal edge of incisor teeth creating
individual characteristics during biting procedures. It can
be:
Tooth mark: Mark left by a tooth (human or non-human).
Arch mark: Mark produce by four or five adjacent teeth
Figure 34.1 Bite marks seen on animate subjects in the same arch.
Forensic Odontology
Causes of Bite Marks

• C hild abuse: It can be found anywhere on the body,
favorite sites being the arms, hands, shoulders, 855
cheeks, buttocks and trunk. Most of the time it is
inflicted by the mother.
• Sexual assaults: It usually occurs in cases of rape.
Most common site of it is breast and nipples but
neck, shoulders, thighs, abdomen, pubis and even
vulva may be attacked.
• It may also be inflicted on police officers when
attempting to arrest resisting offenders.
• Sports: Bites can occur in sporting events especially
football and some forms of wrestling. In this, it can
occur anywhere but hands, fingers, nose, forearms,
ears and even lips may be target. Figure 34.5 Human dentition consist of 32 teeth with 5 surface
• Self inflicted: Falls onto the face or a fit may cause
the tongue and lips to be badly bitten. Some persons
deliberately bite themselves, sometime to fabricate not necessarily due to break in the continuity of the skin but
injuries for a variety of motives ranging from gain to due to a small subdermal or thin deep hemorrhage.
psychiatric disorders. Bite marks are always contaminated by saliva and
therefore contain amylase, ptyalin and blood group which
can be determined by the cast of suspect of mouth is
Importance of Bite Marks (Figs 34.4 and 34.5) made and transparency of his bite compared to that of the
unknown bite. It should be remembered that bite marks
It can permit precise identification because the alignment
on skin are modified by its elasticity when the teeth are
of teeth is peculiar to each individual. The mark may be on
withdrawn.
the article of food found at the scene of crime or on human
Self-inflicted bite marks are seen in Lesch-Nyhan
being to realize that a bite on human flesh can have marks
syndrome. This syndrome is X-linked manifesting insensi-
tivity to pain and self-mutilation by chewing away of lip.
Method of Preservation of Bite Marks

The doctor should be asked how the bite mark may be best
preserved. When the substance is plastic such as butter,
cheese, lard, or chocolate, it should be stored in a refrigerator
to prevent melting or gradual flowing. It should not be deep
frozen as this may cause brittleness and cracking. Fruits
should be preserved in Campden solution, a metabisulphite
fluid used for fruit bottling. If it is not there then 5 percent
acetic acid in 40 percent aqueous formaldehyde solution
can be used.
Whatever preservation is recommended, the object
should be adequately photographed with film plane at right
angles to the bite and a scale placed in the focal plane. Death
freezes a bite mark but a subdermal hemorrhage in living
Figure 34.4 Bite mark is represented distorted image of person disappears within 20 minutes hence it is important
human dentition to take an immediate photograph of the bite mark.
tend to bruise less easily and thus are more likely to

Classification of Bite Marks
demonstrate a bite mark.
• Non human (animals)
• Human Age: Infants and elderly individual tend to bruise more
856
– In foodstuffs (in a part eaten foodstuff abandoned easily than other age groups marking the detection of bite
by offenders at the scene of crime). marks more difficult.
– On non biological object (on pencils, pipe stems Sex: Females tend to bruise easier than males. Once
and detonators) produced a bite mark will be evident for a longer period of
– In human skin time on females as compared to males.
– Non criminal (erotic bite) In human, bite mark can be sexual or assault type.
– Criminal—it can be offensive (upon victim by
assailant) and defensive (upon assailant by victim). Influencing Factor
Bite mark can be influenced by time elapsed between
Bite Marks on Foodstuff the actual biting and when the impression is made.
Depression of the skin as occurs in most bite marks will
Foodstuff bite marks can be classified in to 3 types:
recover within 10 to 20 minutes after the bite, although
Type I: Bites that are found in material such as chocolate, discoloration and swelling may be present 24 to 72 hours
which fracture readily with limited depth of tooth on a living object.
penetration.
The force exerted: The skin appearance of bite mark may
Type II: Bites formed in foods where teeth obtain a good vary from bruising, abrasion, and indentation to actual
grip and then bitten piece is removed by fracturing it from lacerations.
main material, e.g. apples. The number of teeth: In general, the more teeth marks
Type III: Bites in which teeth bite right through the bitten present in the bite marks, the better is the likehood of
material, e.g. cheese. These bite exhibits extensive scrape identification utilizing that bite mark.
marks and may give and indication of the relative position The type of teeth: In many cases, the bite mark is seen to
of upper and lower incisor teeth in centric occlusion. be comprised of the upper anterior teeth. The investigator
Depth of penetration of the teeth: Where there is only should take note of the width of teeth.
minimum penetration of teeth into the food, the record is The reaction of the surrounding tissue: Bite marks made
of biting edges and where there is greater penetration the hours or days before the event will show inflammatory
record is of the labial aspect of the teeth. changes and signs of healing microscopically.
Time and temperature: Bite mark in foodstuff may
produce the exact mesial-distal dimension of the teeth
Types of Bite Mark
provided the record is taken immediately after the bite ∙ Definite bite mark
was made. Dimensional and color changes are expected to ∙ Amorous bite mark
occur in the foodstuffs due to effect of temperature. ∙ Moderately aggressive bite mark
∙ Aggressive bite mark
Bite Marks in Human Skin ∙ Very aggressive bite mark.
Human tissue has been described as one of the least
dependable substances for the registration of bite marks Nature of Bite Marks
as the bite marks in the tissue is affected by several Though called bite marks, but some times it may not be
variables. from actual teeth. The lips can transiently mark the skin,
if forcibly nipped, especially on children. Suction can
Status of the Tissue produce a crop of punctate hemorrhages, either small
Site: Type of tissue/condition of skin. For example, loose petechiae or larger ecchymoses merging into a confluent
skin or excessive subcutaneous fat commonly demonstrated central bruise. It is caused by front teeth from canine to
easy and extensive brushing leading to a poor bite mark canine with a gap at either side representing the separation
definition. Areas of fibrous tissue or high muscle content of upper and lower jaw.
Forensic Odontology
A human bite is near circular or a shallow oval. A deep

parabolic arch or ‘U-shaped’ can only be animal in origin.
The teeth may cause clear, separate marks or they may
run into each other to form a continuous or intermittently 857
broken line. As the time progresses, original teeth marks
spread out and blur. Teeth marks may be abrasion, bruises
or laceration or a combination of any two of three. The
clarity of bite marks depends on a number of factors:
∙ If the contour of the part bitten is irregular or markedly
curved, then only part of dental arch is in contact with
the tissues.
∙ If the bite is forcible, then extensive subcutaneous
bruising may spread and blur the outline.
∙ If the bite is inflicted many days before, then healing of
abrasion and lacerations and absorption of bruising will Figure 34.7 Impression is taken of the bite and cast is made
leave progressively less detail.
∙ When the teeth are forcibly applied the typical
appearance is of two ‘bows’ with their concavities
facing each other and a gap at each end.
Love bites: They are caused by firm application of the
lips, which form an airtight seal against the skin, and then
sucking action reduces the air pressure over the centre.
This causes shower petechial hemorrhage to appear from
rupture of small venules in the superficial layer of the
subcutaneous tissue. If forcible the petechiae are confluent
and a frank bruise or even hematoma develops.
Investigations of a Bite Mark (Figs 34.6 to 34.12)

Firstly, the bite mark should be carefully and fully
photographed. The photograph should be taken from
different angles, but especially from directly perpendicular Figure 34.8 Study case is made
Figure 34.6 Sample bite taken on apple Figure 34.9 Bite mark of suspect is taken
viewpoint, with the plane of film at right angle to that of the

lesions. An accurate scale should always be held near the
lesion, as close as possible. The lesion should almost fill
858 the camera frame in some shots to capture as much detail
as possible. When photography is completed, swabs of the
bite should be taken to try to recover saliva. Plane cotton-
wool swab are gently rubbed onto the bite. They should
be then deep frozen unless send straight to the serology
laboratory.
After this, impression of the bite can be taken. It is
done by laying a plastic substance over the bite mark,
which then hardens, so as to produce negative cast of the
lesion. It is usually made with a rubber-or silicon based
medium containing catalytic hardeners. Less satisfactory
Figure 34.10 Positive replica of bite mark is made
substances are water based pastes, such as plaster of Paris,
which are put on wet and allowed to dry before removal.
But they have a disadvantage of potential damage to
the actual bite. After autopsy, it is also possible for the
whole area of the skin carrying the bite to be removed and
preserved in formalin for future examination.
Matching the Bite Mark with the

Suspect Dentition
The teeth of those who are either suspected by the police or
who had access to the victims should be examined. In most
jurisdictions, it is vital that fully informed consent should
be obtained from the persons beforehand.
Any refusal must be a bar to any further action. When
children are concerned, usually in the setting of child abuse,
the consent of fully informed parents or guardian must be
Figure 34.11 Cast of suspect is made obtained. Then dentition is examined for number of teeth,
missing teeth, complete or partial denture, occlusion,
broken teeth, irregular teeth and abnormalities of the teeth.
Photographs of dentition of the patient can be taken.
After that impression of bite of suspect should be
taken. Tracing can be made from the positive cast of a
bite impression, inking the cutting edge of front teeth and
transferring these to transparent sheets, which can then be
laid over the photograph to determine correspondence.
HUMAN ABUSE
Dental professional are likely to encountered more victims
of physical, neglective sexual and psychological abuse.
Child abuse is nonaccidental, physical, mental,
emotional and sexual trauma before 18 years of age. This
abuse is done by parents, teacher, babysitter and person is
Figure 34.12 Cast is matched with previous mark acting as parent.
Forensic Odontology
Elder abuse is done for the patients who are physically Personal injury: TMJ damage, dental trauma in vehicular,
or mentally ill. home, sports, recreational and work related accident.
Intimate partner violence: This abuse are differ from Dental fraud: Charging for material or procedure that
859
child and elder abuse as they have choice of circumstance were not used.
and place.
Identification of multiple fatality incident victims:
Sign and symptoms of human abuse: There is laceration dental expert is requested in identification of homicide
in labial or lingual frenum which can occur due to forceful victims and in bite mark and human abuse cases.
feeding or blow. There may be fracture of zygomatic bone,
nasal bone, and teeth. There may be bilateral periorbital BIBLIOGRAPHY
ecchymoses (raccoon mask), bilateral contusion of the lip
commissures. There may be traumatic alopecia secondary 1. Arany S, Ohtani S, Yosioka N, et al. Age estimation from
to grabbing of head hair of victim. aspartic racemization of root dentin by internal standard
method. Forensic Sci Int. 2004;141:127-30.
Dentist role in reporting human abuse: When the dentist 2. Austin-Smith D, Maples WR. The reliability of skull/
determines to report child or elder abuse, documentation of photographs superimposition individual identification. J
the physical evidence to support the charge is must. Forensic Sci. 1994;39:446-55.
3. Jakush J. Forensic dentistry: J AM Dent Assoc. 1989;119:
DENTIST AS EXPERT WITNESS 355-68.
4. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and
Dentist act as ‘witness of fact’ i.e. they only testify the facts maxillofacial pathology, 3rd edition, Saunder Elsevier,
that are known to them. Dentists are qualified to testify 2009.
by the judge, who bases his or her opinion on education 5. Pretty IA. A web based surface of odontologist opinion
background, dental and forensic expertise, publication and concerning bite mark analysis. J Forensic Sci. 2003;48:
other professional qualifications. Dentist is required to 1117-20.
testify in following situation. 6. Standish SM, Stimson PG. Forensic dentistry: legal
obligation and methods of identification for the practitioner.
Malpractice based on negligence: It includes battery
Dent Clinic North AM. 1977;21:1-196.
(extraction of the wrong tooth), misdiagnosis, and failure
to diagnose.
1. Copper causes discoloration of gums which can be seen 3. The techniques which are used for DNA profiling are:
as: a. RFLP b. PCR
a. Green line b. Blue line c. Both d. None
c. Black line d. Reddish line 4. Bite mark on fruits are preserved in:
2. Wasted blood stains and seminal stains can be easily a. Deep freezed b. Campden solution
seen by: c. Alcohol d. Water
a. Infrared lights 5. Bite mark which fracture readily with limited depth of
b. UV rays tooth penetration:
c. X-rays a. Type I b. Type II
d. Both a and b c. Type III d. None
35 Syndromes of the
Orofacial Region
Chapter Outline
Â Classification of syndrome Â Johanson-blizzard syndrome

Â Syndromes associated with craniofacial anomalies of Â Oculodentodigital dysplasia
genetic origin Â Fanconi’s syndrome
Â Cleidocranial dysplasia Â Lacrimo-auriculo-dento-digital syndrome
Â Apert’s syndrome Â Nevoid basal cell carcinoma syndrome
Â Ellis-van Creveld syndrome Â Ascher’s syndrome
Â Crouzon syndrome Â Melkersson-Rosenthal syndrome
Â Trisomy 21 syndrome Â van der Woude syndrome
Â Trisomy 18 syndrome Â Hereditary hemorrhagic telangiectasia
Â Trisomy 13 syndrome Â Cowden syndrome
Â Cerebrohepatorenal syndrome Â Zinsser-Engman-Cole syndrome
Â Cerebrocostomandibular syndrome Â Winchester syndrome
Â Hajdu–Cheney syndrome Â Anhydrotic ectodermal dysplasia/Christ-Siemens-
Â Anderson syndrome Touraine syndrome
Â Caffey–Silverman syndrome Â Zellweger syndrome
Â Median cleft face syndrome Â Beckwith-Wiedemann syndrome
Â Melnick Needles syndrome Â Orofacial digital (OFD) syndrome
Â Mohr syndrome (OFD II) Â Hurler syndrome
Â Treacher Collins syndrome Â Mobius syndrome
Â Pierre-Robin syndrome Â Aglossia-Adactylia syndrome
Â Marfan syndrome Â Burning mouth syndrome
Â Ehlers-Danlos syndrome Â Tuberous sclerosis
Â Hereditary hypohydrotic ectodermal dysplasia Â Zimmerman Laband syndrome
Â Stevens-Johnson syndrome Â Rutherford syndrome
Â Mucocutaneous lymph node syndrome Â Ramon syndrome
Â Incontinentia pigmenti Â Papillon-Lefevre syndrome
Â Broad groups of pigmentary disorders Â Klippel-Trenaunay-Weber syndrome
Â Peutz-Jeghers syndrome Â Auriculotemporal syndrome
Â Albright’s syndrome Â Paratrigeminal syndrome
Â Sjögren’s syndrome Â Horton’s syndrome
Â Heerfordt’s syndrome Â Migraine syndrome
Â Riley-Day syndrome Â Horner’s syndrome
Â Gardner’s syndrome Â Jaw-Winking syndrome
Â Trichodento-osseous syndrome Â Plummer-Vinson syndrome
Â Naegali-Franceschetti-Jadassohn syndrome Â Chediak-Higashi syndrome
Contd...
Syndromes of the Orofacial Region
Â Sweet’s syndrome Â General adaptation syndrome

Â Lazy leukocyte syndrome Â Progeria 861
Â Maffucci’s syndrome Â Waterhouse-Frederichsen syndrome
Â Blue rubber bleb naevus syndrome Â von Recklinghausen’s neurofibromatosis
Â Diffuse neonatal haemangiomatoses Â Basal cell nevus or nevoid basal cell carcinoma syndrome
Â Sturge-Weber syndrome Â Multiple endocrine neoplasia syndromes
Â Reiter’s syndrome Â Tuberous sclerosis
Â Behçet’s syndrome Â Klippel-Trenaunay syndrome
Â Cushing syndrome Â von Hippel-Lindau syndrome
Â Adrenogenital syndrome Â Parkes-Weber syndrome
INTRODUCTION Eponyms: Eponyms are used to describe syndromes and

diseases since the middle of the nineteenth century when
The heritage of the term syndrome is ancient. It was used they were employed by neurologists to separate groups of
by Hippocrates to denote a group of regularly concurrent similar clinical entities—one of the most obvious merits is
symptoms which resulted from several causes. The word the honor bestowed upon several keen observers who have
syndrome has its origin from the Greek literature which dotted the medical and dental history.
means, “running together” (syn—together and dromos— The etiological factors for the occurrence of the
running). The word syndrome has been used in English syndromes are not definite. Many times it occurs due
language since 1541. to genetic influence or genetic disposition. Racial
At this time there is neither complete agreement predominance is also seen as a causative factor. Offsprings
concerning what constitutes a syndrome nor there is a of an affected individual usually have chance of acquiring
uniform method of nomenclature. Syndrome is defined the disorder if inherited. The syndrome sometimes becomes
as “A group of signs and symptoms or a set of symptoms progressively more severe in succeeding generations.
usually three or more, which occur together characteristic Syndromes may also occur due to immunodysfunction
of a morbid condition”. Or “It is the aggregate of signs or an abnormal immune response to microbial antigen–
and symptoms associated with any morbid process and dysfunction of certain glands and vascular anomalies can
constituting together the picture of the disease.” also result in form of syndromes. Diagnosis of a syndrome
Syndromes being a multisystem disease are difficult to is problematic because of the extreme variability of its
classify in a specific pattern. It is a grouped together symptom clinical expression.
complex which is of varied etiology and clinical presentation. Although the prognosis in some of the conditions
is extremely poor, mortality is drastically reduced with
Classification of Syndromes early diagnosis and recent advanced treatment modalities
Many syndromes are classified according to: available. Treatment can be directed at correction or
∙ Eponym—For example, Morquio-Brailsford syndrome reconstruction of the existing deformities. During infancy
∙ Cause—For example, Crush syndrome and childhood the patient should be examined frequently
∙ Pathogenesis—For example, Dumping syndrome to rule out suspicious conditions. Growth and development
∙ Anatomic location—For example, oculo-dento-digital should be observed closely for evidence of mental
syndrome impairment or abnormalities of sexual development. This
∙ Main symptoms—For example, progressive hemifacial will facilitate early identification of potentially dangerous
atrophy syndromes.
Syndromes as already stated are difficult to classify in Single Gene Disorders

a specific pattern as it is a grouped together multisystem
The disorders in this group are due to defects in a single
complex of signs and symptoms. For sake of convenience,
862 gene which refers to the primary error in the DNA code.
orofacial syndromes are compiled together under the follo-
These disorders are inherited following Mendelian laws
wing headings:
and are subdivided according to the chromosome on which
∙ Syndromes associated with craniofacial anomalies of
the abnormal (mutant) gene is situated and by the nature
genetic origin
of the trait.
∙ Syndromes associated with skin and pigmentation
The inheritance is known as autosomal when the
∙ Syndromes associated with salivary and lacrimal glands
abnormal gene is situated on an autosome and the trait is
∙ Syndromes associated with teeth
said to be X-linked when such a gene is situated on the X
∙ Syndromes associated with oral mucous membrane,
chromosome.
lips and cheek, gingiva, tongue
These traits may either be “dominant” or “recessive”
∙ Syndromes associated with nerves
(autosomal dominant or recessive, and X-linked dominant
∙ Syndromes associated with blood and vascular
or recessive).
malformations
Autosomal dominant inheritance has the following
∙ Syndromes associated with immunodeficiency
characteristics:
∙ Syndromes associated with hormonal disturbances
∙ Every affected person has at least one affected parent.
∙ Syndromes with benign oral neoplastic or hamar-
∙ Both males and females are likely to be affected and are
tomatous components.
capable of transmitting the disease
∙ Transmission is generally seen (father to son or
SYNDROMES ASSOCIATED WITH daughter and mother to son/daughter) with no skipping
CRANIOFACIAL ANOMALIES of generation
OF GENETIC ORIGIN ∙ Affected persons typically transmit the trait to half their
offspring
Genetic Factors These general rules however are modified by:
Genetic information in humans is carried by 23 pairs ∙ Penetrance—carrier of abnormal genes may be so
(46) of chromosomes, known as autosomes which carry mildly affected to be entirely indistinguishable from
information regarding the structure and functions of normal persons. A gene is said to be “penetrant” if it is
the body one pair (2) is known as the sex chromosome, expressed in a given carrier
determines the sex of the individual (XY in male and XX ∙ Expressivity—a mildly affected parent may have
in female). severely affected child or vice versa. There is variation
Genetically derived-disorders can be considered under in the expression
three major groups: ∙ New mutation—an individual who does not carry a
1. Chromosomal disorders gene for a particular inherited condition may rarely
2. Single gene (monogenic) disorders have an affected infant.
3. Multifactorial disorders. Autosomal recessive inheritance
The characteristic are:
Chromosomal Disorders
∙ The trait usually appears only in sibs and not in their
The disorder in this group is due to errors in number parents
and/or structure of the chromosomes. For example, ∙ On average one in four of the sibs are affected
Down syndrome (Trisomy 21) results from an additional ∙ Consanguinity is generally seen among the parents of
autosomal chromosome, while Klinefelter syndrome is the affected individual
associated with an additional X chromosome in male, i.e. ∙ Males and females tend to be equally affected
XXY. Deletion of part of chromosome 5 results in the ∙ Affected individuals will generally have normal children
Cri–Du–Chat syndrome. but all of the children will be carrier of the condition.
Sex-linked inheritance X-linked Inheritance Syndromes

When the X chromosome carries the abnormal gene, the
∙ Albinism—Deafness syndrome
disorder is said to be X-linked and when the Y chromosome
∙ Amelogenesis imperfecta (two types) 863
carries it, the condition is said to be X-linked dominant
∙ Coffin—Lowry syndrome
disorders, e.g. (the hypoplastic form of amelogenesis, e.g.
∙ Aarskog syndrome
hypohidrotic ectodermal dysplasia).
∙ Focal dermal hypoplasia
Multifactorial Inheritance ∙ X-linked hydrocephaly
∙ Lowe syndrome
This involves interaction between both environmental ∙ Mucopolysaccharide II (Hunter)
and genetic factors. The environmental factors include: ∙ Orofacial-digital syndrome.
(1) trauma; (2) neoplastic disease; (3) toxic drugs; (4)
metabolic factors and (5) infections. Multifactorial (MFR)
∙ Sturge-Weber syndrome
Genetics of Craniofacial Malformation ∙ Mobius syndrome
Causes of congenital malformation ∙ Frontonasal dysplasia syndrome
∙ Genetic factors ∙ Goldenhar syndrome
– Chromosomal disorders ∙ Aglossia–adactylia syndrome
– Single gene disorders ∙ Cleft lip–cleft palate malformations
– Multifactorial disorders (polygenic and environ- ∙ Craniosynostosis.
mental) at birth and disorders of later life
∙ Nongenetic factors Cleidocranial Dysplasia
– Maternal infections Cleidocranial dysplasia is an autosomal dominant condition
– Maternal metabolic derangements and genetic although in less than half the cases it occurs spontaneously.
diseases. (Diabetes, hypertension, thyroid disorders) The condition is characterized by the defects in the
– Maternal exposure to radiation clavicles, cranial vault and occasionally with other selected
– Disturbances of embryonic differentiation and fetal and dental findings.
growth. As the name suggest, the frequent changes seen in the
Some craniofacial malformation syndromes with auto- syndrome are in the clavicles and the cranium. The clavicles
somal dominant inheritance: are unilaterally or bilaterally hypoplastic or aplastic. When
∙ Blepharophimosis syndrome hypoplastic the deficiency is seen at the acromial end
∙ Waardenburg’s syndrome allowing the individual to approximate their shoulders in
∙ Treacher Collins syndrome front of the chest. X-ray of the chest reveal the exact defect.
∙ Van der-Woudes syndrome The skull is large with frontal, parietal and occipital
∙ Popliteal pterygium syndrome bossing. Delayed mineralization of the sutures is a constant
∙ Oculo-dento-digital syndrome finding of the syndrome. There is usually a midfacial
∙ Ectrodactyly-ectodermal dysplasia clefting (EEC) hypoplasia present resulting in relative prognathism of the
∙ Craniosynostosis syndromes: mandible.
– Crouzon syndrome Essential oral features include high arched vault of
– Saethre-Chotzen syndrome the palate with or without clefts, delayed eruption of
– Pfeiffer’s syndrome teeth, malformed roots, supernumerary teeth and frequent
– Apert syndrome. impactions.
Some craniofacial syndromes with recessive inheritance: A skull X-ray reveals the features together with the
∙ Cryptophthalmos syndrome obtuse mandibular angle which is increasingly reported in
∙ Orofacial digital syndrome these patients.
∙ Roberts syndrome Impacted teeth in affected individuals are reported to be
∙ Carpenter’s syndrome. lacking cellular cementum.
Apert’s Syndrome (Acrocephalosyndactyly) Trisomy Syndromes

Acrocephalysyndactyly is believed to be transmitted by an Trisomy syndromes represent autosomal chromosome
864 autosomal dominant gene occurring sporadically in about disorders. Several disorders in this group have been
1 to 2000,000 of the general population. discovered. Only those with craniofacial and associated
Clinical features include ovoid skull, brachycephalic dental anomalies will be dealt with in the following
and often presents a horizontal supraorbital groove. discussion. They are:
Premature closure of the sutures occurs and often the ∙ Trisomy 21 syndrome (Down’s syndrome)
anterior fontanelle is open due to late closure. ∙ Trisomy 18 syndrome (Edward syndrome)
Features of hand and feet include syndactylism. ∙ Trisomy 13 syndrome (Patau syndrome).
Osseous or cutaneous fusion of fingers is usually present.
Oral features include a high palatal vault and the Trisomy 21
presence of posterior palatal and uvular clefts. Dental Synonym: Down’s syndrome
malocclusion is consistent. Trisomy 21 occurs in one in about 650 births. This
Other oral features include mandibular prognathism, syndrome is characterized by several features involving
malocclusion and retarded eruption. almost all organs of the body.
The affected individual is mentally retarded, short
Ellis-van Creveld Syndrome stature and exhibits brachycephaly with a relatively flat
Synonym: Chondroectodermal dysplasia occiput. Fontanelles close later and frontal sinuses are
Ellis-van Creveld syndrome consists of anomalies hypoplastic.
derived from the ectodermal mesodermal germ layers. The The ears are small and the iris shows speckling
disorder is believed to be inherited as an autosomal recessive (Brushfield’s spots). Disorders involving the pelvis, skin
trait. The disorder is congenital. Defects are seen in the hair and genital occur with variable frequency. Facial
extremities, teeth, nails and hair. Rarely cardiac anomalies features include a small head and an open mouth.
also have been noted. Person is of short stature from birth.
Oral manifestation—short mouth, large tongue and
Polydactyly with or without syndactyly is present.
tongue thrust beyond the lips. Maxillary lateral incisors
Nails are dystrophic and hairs are sparse.
show abnormality. They are either conical or missing
Oral manifestations—deciduous teeth show hypoplasia developmentally. Microdontia is often present. Delayed
occasionally and partial anodontia of permanent dentition eruption and malocclusion are other findings. The palate is
is seen. Prognathism of lower jaw is present. There high arched and narrow, the usual may be bifid. Necrotizing
is obliteration of labial suture due to gingival frenal ulcerative gingivitis is reported to be common in these
attachments. individuals.
In about 95 percent of the affected individual’s condition
Crouzon Syndrome arises as the result of the failure of paired chromosomes to
Synonym: Craniofacial dysostosis disjoin and enter separate daughter cells during meiosis.
Craniofacial dysostosis is an autosomal dominant Both chromosomes (number 21) from parental cell enter
condition, characterized by cranial synostosis, ocular the gamete destined for fertilization and at fertilization a
proptosis and hypoplastic maxilla. third chromosome (number 2) is added from the normal
The appearance is quite characteristic because of gamete of the other parent. The result is a zygote with three
exophthalmos, hypertelorism and the under development chromosomes (number 21) which is known as trisomy 21.
of the maxilla. Exophthalmos is due to shallow orbits.
Maxillary hypoplasia is responsible for the relative Trisomy 18 Syndrome
mandibular prognathism which is a consistent feature of Synonym: Edward syndrome
this syndrome. The upper lip is usually short and the nose Children with trisomy 18 are quite striking at birth with
is parrot like. The cranium is brachycephalic. intrauterine retardation of growth and characteristic facial
Oral features include, malocclusion, cleft lip and palate appearance. Trisomy 21 and trisomy 13, nondysfunction
with V shaped palate. Some of the occasional findings is the cause of this syndrome. Increased maternal age is an
include peg shaped teeth and partial anodontia. important factor in its causation.
The affected individuals are mentally retarded and The affected individual is generally short. A long nose,
show hypertonicity. The index finger overlapping the third low frontal hairline and flared ears are the characteristic
finger and the fifth over fourth are the striking features. facial features. The phalanges of fingers and toes exhibit
Oral features—small mandible high arched palate and acro-osteoses and clubbing of nails present. 865
occasional cleft palate. The skull is dolicocephalic. Sutures are usually open.
Oral features include premature loss of teeth.
Trisomy 13 Syndrome
Synonym: Patau syndrome Anderson Syndrome
In the trisomy 13 syndromes, affected individuals show Synonym: Familial osteodysplasia
46 normal chromosomes but a portion of chromosome The recently reported syndrome of familial osteody-
13 is attached to another chromosome or is present as a splasia is an autosomal recessive disorder, characterized
fragment. The majority of cases are due to nondisjunction. by craniofacial and skeletal anomalies, presence of
The physical features are characteristic. They include hyperuricemia and diastolic hypertension.
microcephaly, microphthalmia, ocular hypertelorism, Oral features include mandibular prognathism, hypoplastic
malformed ear, deafness, polydactyly and heart anomalies. maxilla and resultant malocclusion. The mandible without
Oral features include a small mandible, high arched wide angle, increased body length and reduced ramus and
palate and occasional cleft palate and bifid uvula. As with body height is an essential features of the syndrome.
all trisomy syndromes. Chromosomal counts confirm the
diagnosis. Caffey-Silverman Syndrome
Synonym: Infantile cortical hyperostosis
Cerebrohepatorenal Syndrome Infantile cortical hyperostosis is characterized by the
Synonym: Bowen syndrome bilateral mandibular swellings, hyperirritability and fever
The cerebrohepatorenal syndrome is believed to be occurring in infants.
having an autosomal recessive inheritance. The condition The bilaterally symmetrical swellings which occur
is characterized by craniofacial anomalies, renal cortical over the ramus, angle and the body of the mandible are
cysts, hypoprothrombinemia and several other occasional very striking. The soft tissue is tender and edematous,
findings involving genitals, spleen and pancreas. often undergoing remission and exacerbations. Similar
Oral features include micrognathia, protruding tongue bony and soft tissues changes may be seen in ribs, ulna,
and high arched palate. femur, clavicle, tibia and irritability are seen as preceding
symptoms in about 70 percent of the affected infants.
Cerebrocostomandibular Syndrome Hematological and biochemical findings include raised
The cerebrocostomandibular syndrome is an autosomal erythrocyte sedimentation rate, leukocytosis anemia and
recessive condition. This disorder is characterized by increased alkaline phosphatase levels.
mental retardation, thoracic deformities and mandibular This disorder is characteristically seen in the infants,
and facial anomalies. the onset being usually between the second to sixth months
Clinical features include microcephaly, long trunk, after birth, although a few cases have been reported in utero.
rib anomalies producing gaps and tracheal cartilage Disorders to be considered in differential diagnosis include
malformation producing a barking cough. osteomyelitis and parotid gland inflammation or tumors.
Oral features include micrognathia, palate defects and
absence of hard and often the soft palate. Median Cleft Face Syndrome
Synonym: Frontonasal dysplasia
Hajdu-Cheney Syndrome Frontonasal dysplasia is a disorder of unknown genetic
Synonym: Acro-osteolysis causes (possibly multifactorial) characterized by facial
Acro-osteolysis is a rare autosomal disorder disorders such as nasal clefts and notches, preauricular
characterized by short stature, disintegration of the terminal tags and ocular hypertelorism. Extracranial anomalies are
phalanges of fingers and toes, premature loss of teeth and usually not found in this syndrome.
abnormal shape of skull. Oral features may include cleft lip and malocclusion.
Melnick Needles Syndrome This disorder is relatively rare with an incidence

between 0.5 and 10.6 cases per 10,000 births. Stapedial
Synonym: Osteodysplasty
artery dysfunction results in the defects of the stapes and
866 Melnick Needles syndrome is an autosomal dominant
incus and first arch vessels supplying the maxilla.
condition characterized by the generalized bone dysplasia
The expression begins as early as the sixth to seventh
and abnormal faces. The facial appearance is quite typical
embryonic week. Mandibular abnormalities such as
with marked exophthalmos, full cheeks, large ears,
improper orientation and hypoplasia of the mandibular
micrognathia and a transversely long mouth.
elevater muscles result due to failure of developments of
Radiographically the skull exhibits delayed closure
ancillary vascular supply by inferior alveolar artery.
of the basic of the skull and mastoid process. There are
also skeletal deformities involving clavicle, vertebrae, Clinical Findings
radius, tibia, fibula and humerus, micrognathia and
malocclusion. Treacher Collins syndrome is a combined development
Facies are quite striking in that two unrelated patients anomaly of first and second branchial arch. Varying degrees
look like siblings. X-rays and blood chemistry help in the of hypoplasia of the mandible, maxilla, and zygoma is seen.
diagnosis as serum alkaline phosphatase is elevated. Abnormalities of medial pterygoid plates and
hypoplasia of lateral pterygoid plates are common. Notched
Mohr Syndrome (OFD II) or linear colobomas of the outer third of the lower eyelid
are found in 75 percent of patients. The facial appearance
Synonym: OFD orofacial digital syndrome II
is characteristic and described as bird like or as fish like.
The Mohr syndrome is an autosomal recessive
Microdontia or underdeveloped ear and a narrow
disorder characterized by oral, facial and digital defects.
extension of the hair over the preauricular region known as
The affected individual is moderately short, exhibiting
“hair like” are common in this syndrome.
digital deformities such as brachydactyly, syndactyly or
Oral findings include cleft palate in about 30 percent
polydactyly. Facial features include the midline cleft lip
of the patients and macrostomia in 15 percent of the
and the broad and bifid tip of the nose.
patients. Cleft palate and malocclusion is present. The
Oral features include the lobate tongue, high arched
underdeveloped zygomatico-maxillary complex leads to a
palate, hypoplastic body of mandible, and the absence of
clinically severe midface deficiency.
mandibular incisors.
Radiographic findings include downward sloping
The syndrome resembles the orofacial digital I
floors of the orbits, peaked long nasal contor, aplastic
syndrome, which is X-linked and occurs only in females.
or hypoplastic zygomatic process of the temporal bone
Treacher Collins Syndrome and obtuse mandibular angle. Lateral cephalograms
demonstrate antegonial notching and broad concave
Synonym: Mandibulofacial dysostosis
nature of the inferior border of the mandible helps to
Treacher Collins syndrome primarily affects structures
distinguish the condition from other syndromes involving
developing from the first branchial arch but it also involves
the mandible.
the second branchial arch, to a minor degree.
The persons affected have a convex facial profile with a
Treatment and Prognosis
prominent nose and retrusive chin. It is generally a bilateral
anomaly with a characteristic faces including downward It is directed at correction or reconstruction of the existing
sloping of the palpebral fissures, coloboma of the lower eyelid, deformities. Neutralization of conductive hearing loss
mandibular and midface hypoplasia and deformed pinnas. through surgery and hearing aids is helpful. Ophthalmic
surgery is done to correct eye defects.
Etiology and Pathogenesis
It is transmitted by an autosomal dominant mode of Pierre-Robin Syndrome
inheritance although about half the cases are due to The Pierre-Robin syndrome manifest clinically as micro-
spontaneous mutation. Affected siblings are remarkably ganthia, glossoptosis and high arched or cleft palate in the
similar and the syndrome becomes progressively more neonate. It can occur as an isolated finding or as a component
severe in succeeding generations. of various syndrome or development anomalies.
Primary findings are retrognathia and hypoplasia of characteristic abnormalities of the musculoskeletal ocular
mandible. Respiratory and feeding problems are prevalent and cardiovascular systems and a positive family history.
and may result in episodic airway obstruction, infant
hypoxia, malnutrition and failure to thrive. Clinical Features 867
Patients characteristically present with a tall slender stature
Etiology and Pathogenesis with relatively long legs and arms, large hands with long
Incidence is 5.3 to 22.7 percent per 100,000 births with 39 fingers and loose joints. The arms, legs and digits are
percent of the infants exhibiting no additional abnormalities disproportionately long compared to the patient trunk.
25 percent have known syndromes and 36 percent have one Chest deformities present with a protrusion or indentation
or more anomalies that are not a part of a known syndrome. of the breast bone. Even the normal thoracic kyphosis is
Fetal malposition and interposition of the tongue absent and the back is straight.
between the palatal shelves have long been considered the Oral findings include high arched palate with dental
etiologic catalysts for palatal deformity and micrognathia. crowding. The face appears long and narrow.
Recent evidence suggest that the primary defect may be due The cardiovascular system shows mitral valve prolapsed
to genetically influenced metabolic growth disturbances in 75 to 85 percent of the cases. Aortic dilatation is there of
of the maxilla and mandible rather than to mechanical the ascending aorta leading to aortic regurgitation.
obstruction by the tongue during embryogenesis. Ocular findings include dislocation of the lens (ectopic
lentis). Retinal detachment is infrequent but myopia (short
Clinical Features sightedness) is a common finding.
Infants present without severe micrognathia and
Treatment and Prognosis
mandibular hypoplasia. A ‘U’ shaped high arched palate
is usually present. Glossoptosis is the result of the retro Subluxation of the lens of the eye, chest cavity deformities
positional attachment of genioglossus muscle because potential for pneumothorax are serious prognostic indica-
of the retrognathic mandible. The geniohyoid muscle is tors. Treatment consists of annual medical examination
fore shortened so that support to the hyoid bone and strap with a cardiovascular, emphasis frequent ophthalmologic
muscles of the larynx is also compromised. examination, scoliosis screening and echocardiography.
Mortality has been drastically reduced with the use of com-
Treatment and Prognosis posite grafts to replace the aortic value and region contain-
Treatment is supportive to overcome feeding problems and ing the aortic aneurysm.
the patient should be monitored for airway obstruction.
Ehlers-Danlos Syndrome
Marfan’s Syndrome The Ehlers-Danlos syndrome is an uncommon inherited
It is a heritable disorder of connective tissue characterized disorder of connective tissue, clinically characterized by
by abnormalities of the skeletal, cardiovascular and ocular joint hypermobility and skull hyperextensibility. There
systems. Diagnosis is problematic because of the extreme are inherited defect in collagen metabolism. In addition to
variability of clinical expression. The syndrome is notable the skin and joint anomalies, severe gastrointestinal and
for a number of cases of sudden catastrophic death occurred cardiovascular complications may occur and coexist.
in affected athletes (undiagnosed). The condition has been classified into eight variants.
The periodontal form (EDS type VIII) is characteristic
Etiology and Pathogenesis by rapidly progressing periodontal disease resulting in
It is an autosomal dominant inherited in 1 in 100,000 complete tooth loss in second or third decade of life.
individuals. There are no ethnic, racial or gender
predictions. The Marfan’s gene is believed to produce a Etiology and Pathogenesis
change in one of the proteins that provide strength to a Various subtypes of Ehlers-Danlos syndrome are inherited
complete connective tissues probably collagen. The gene as autosomal dominant, autosomal recessive and X-linked
has been located on chromosomes 15 and will provide for traits. There is defect in the synthesis and structure of type
diagnostic testing in pairs at risk. Diagnosis is based on III collagen. A deficiency of the enzyme lysyl hydroxylase
resulting in decreased amounts of collagen hydroxylsine evaluated and monitored. Sudden death can occur. surgical
has also been reported. The conversion of precollagen to intervention must be tempered in light of connective tissue
collagen is prevented. fragility unsuccessful owing to suture failure. Wound
868 healing is delayed and prolong bleeding may occur
Clinical Features following injury.
There is marked hyper elasticity of skin and extreme
laxity of the joints. The skin can be stretched for several SYNDROMES ASSOCIATED WITH
centimeters and when released it resumes its contor. Skin SKIN AND PIGMENTATION
has velvety appearance with high degree of fragility and
bruisability. Minor trauma may produce ecchymosis, Hereditary Ectodermal Dysplasia
bleeding and large gaping wounds with poor healing In order to be considered an ectodermal dysplasia, the
tendencies and “cigarette paper” scar formation especially disorder should meet the following criteria suggested by
on the forehead and lower legs and over pressure points. Solomon et al.
Articular hypermobility is variable. Extreme joint laxity ∙ The disease must be congenital
leads to spontaneous dislocation of joints (back knee) flat ∙ The disease must be diffuse (not localized) and must
feet, etc. involve the epidermis as well as at least one of its
Cardiovascular anomalies include mitral valve pro- appendages (hair, sebaceous glands, nails, mucosa,
lapsed and rupture of major blood vessels. Pulmonary mucosal glands and teeth)
problems include spontaneous pneumothorax and ∙ The epidermal component may involve keratinocytes,
respiratory impairment secondary to chest wall deformities. melanocytes or Langerhans cells or any combination
Orofacial features include a narrow maxilla flattened there of
midface and wide nasal bridge. Fragility of gingival and ∙ The disease is not progressive, infact patients often
mucosal tissues may be problematic. Marked extensibility improve somewhat over a period of years
of the tongue enabling contact with the tip of the nose has ∙ Increased delineation of syndromes with ectodermal
been described. defects has led to considerable difficulty in developing
Dental findings include deep anatomic grooves and a coherent classification of this disorder
excessive cuspal height of the molars and premolars. ∙ The ectodermal dysplasia are classified by Freire-Maia
Maceration of roots and pulpal calcified structures have and Pinheiro for the purpose of delimiting the field as
been noted. conditions with at least one of the following four features:
1. Trichodysplasia
Treatment and Prognosis 2. Dental defects
Prognosis depends on severity of the systemic mani- 3. Onychoysplasia
festation. The cardiovascular status of all patients should be 4. Dyshidrosis.
Classification of the Ectodermal Dysplasis
Disorder Inheritance
Subgroup 1-2-3-4
Anhidrotic X-linked ED X-linked semidominant
Hypohidrotic ED (autosomal recessive) Autosomal recessive
Xeroderma, talipes, and enamel defect Autosomal dominant
Rosselli-Gulienetti syndrome Autosomal recessive
Rapp-Hodgkin hypohidrotic ED Autosomal dominant
Ectrodactyly-ED-clefting syndrome Autosomal dominant
Contd...
Contd...
Disorder Inheritance
Subgroup 1-2-3 869
Hidrotic ED(Clouston’s syndrome) Autosomal dominant
Trichodento-osseous syndrome Autosomal dominant
Trichorhinophalangeal syndrome Heterogeneous recessive
Ellis-van Creveld syndrome Autosomal recessive
Schöpf-Schulz Passarge syndrome Autosomal recessive
Dento-oculocutaneous syndrome Autosomal dominant ?
Odontotrichomelic dysplasia Autosomal recessive ?
Tooth and nail syndrome Autosomal recessive
Subgroup 1-3-4
Freire-Maia syndrome ?
Subgroup 2-3-4
Hypoplastic enamel - onycholysis Autosomal dominant
hypohidrosis
Tooth and Nail syndrome Autosomal recessive
Gorlin’s syndrome Autosomal recessive ?
Oculodentodigital syndrome ?
Monilethrix and anodontia Autosomal dominant
Oral-facial digital syndrome (type I) X- linked dominant ?
Subgroup 1-3
Curly hair-ankyloble pharon-nail dysplasia Autosomal dominant
Palmoplantar hyperkeratosis and alopecia Heterogeneous
Onychotrichodysplasia with neutropenia Autosomal recessive
Subgroup 1-4
Congenital ED of the face Heterogeneous ?
Subgroup 2-3
Nail dystrophy-deafness syndrome Autosomal dominant
Triphalangeal thumbs-hypoplastic distal
Phalanges-onychodystrophy Autosomal recessive
Subgroup 2-4
Marshall’s ED with ocular and hearing defects Autosomal dominant
Other Ectodermal Dysplasias

A large number of further ectodermal dysplasias are associated with alterations of the oral mucosa or of the teeth. They
870 are listed in the following:
Syndrome Oral manifestations Other features

Cranioectodermal dysplasia; Small decidous teeth; hypodontia; Hypoplastic enamel; shortened arms,
dolichocephaly taurodonitism finger toes; hair anomalies
Trichodento-osseous syndrome Amelogenesis imperfecta enamel Curly hair, sclerotic bone
hypoplasia, unerupted teeth; taurodontism
Tricho-onychodental syndrome Hypodontia: conical teeth Fine short hair; thining of the lateral ends
of the eyebrows
Tricho-onychodental syndrome Taurodontism, enamel defects Fine curly hair; thin dysplastic nails
Curry-Hall syndrome Small and conical deciduous teeth; Short limbs; polydactyly, nail dysplasia
retained incisors; small permanent teeth
Otodental dysplasia Globodontia; taurodontism; microdontia; Sensorineural hearing loss
hypodontia
GAPO: (growth retardation alopecia Failure of both dentitions to erupt Frontal bossing midface hypoplasia
pseudoanodontia and optic atrophy)
Johanson-Blizzard syndrome Hypodontia in both dentitions. Roots of Hearing loss panceratic dysfunction
deciduous teeth are short and deformed, mental retardation microcephaly;
crowns are conical hypoplastic ala nasi
Waardenburg’s syndrome Cleft lip/palate Deafness; hair depigmentation
LEOPARD syndrome (lentigines, No mucosal lentigines but there may be Triangular face with hypertelorism and
electrocardiographic anomalies, ocular grandular cell myoblastomas ptosis
hypertelorism, pulmonary stenosis,
abnormal genitalia, retarded growth,
deafness)
Congenital erythrokeratoderma Hyperkeratosis occasional carcinoma Deafness erythrokeratoderma
with sensorineural hearing loss
Hereditary Hypohydrotic (Anhydrotic) persons cannot perspire and they consequently suffer
Ectodermal Dysplasia from hyperpyrexia and an inability to endure warm
temperatures. The hair of the scalp, a eyebrow are fine
Hereditary hypohydrotic (anhydrotic) ectodermal dysplasia scanty and blond.
is a specific syndrome characterized by a congenital
dysplasia of one or more ectodermal structures and their Oral Manifestation
accessory appendages, manifested primarily by:
∙ Hypohydrosis They manifest anodontia or oligodontia. Complete or partial
∙ Hypotrichosis absence of teeth with frequent malformation of any teeth
∙ Hypodontia. present. Where some teeth are present, they are commonly
It affects skin, hair, nails, eyes, teeth, facies, truncated or cone shaped when complete anodontia exists,
sensorineural apparatus and adnexal glandular structures the growth of the jaw is not impaired.
in various combinations and of varying severity.
Stevens-Johnson Syndrome
Clinical Features At one time considered to be a separate disease, Stevens-
The patients exhibit a soft, smooth, thin, dry skin with Johnson syndrome is now recognized as simply a severe
partial or complete absence of sweat glands. Such bullous form of erythema multiforme with widespread
involvement typically including the skin, oral cavity, eyes retardation, seizures, microcephaly, and other CNS
and genitalia. disorders and ocular and skeletal anomalies occur in about
It commences with the abrupt occurrence of fever, 30 percent of the patients. In 90 percent of patients major
malaise, photophobia and eruption of the oral mucosa, dental anomalies such as partial or complete anodontia, peg- 871
genitalia and the skin. The cutaneous lesions in this shaped or conical deformities of the teeth, enamel disorders,
mucocutaneous-ocular disease are similar to those of and delayed eruption are found (Bjellerup, Carrney, Milam
erythema multiforme, although they are commonly et al; Vogte and Matheson). The oral mucosa is not involved.
hemorrhagic and are often vesicular or bullous.
BROAD GROUPS OF PIGMENTARY
Mucocutaneous Lymph Node Syndrome
DISORDERS
Synonym: Kawasaki disease
This syndrome was first described by Kawasaki in A multitude of factors, genetic and/or acquired related to
1967 in the Japanese children. The etiology of this disease melanin and/or some other histologic substrates needs to be
is still unknown but is suggested to be of viral disease or a considered in properly understanding the etiopatho-genesis
“collagen-vascular” disease. in a particular case and its management. Disease of hypo-
and hyperpigmentation can for all practical purposes be
Clinical Features grouped into two broad divisions:
The diseases have been reported to occur in children of age 1. Cause showing cutaneous pigmentary alteration as the
3 to 12 years. The most frequent symptoms as determined most significant and predominant sign if not the only
by the epidemiological study group of the Japanese MCLS exclusive clinical feature, representing in most instances
research committee are: a relative lack of excess of melanin pigmentation.
∙ Fever for 5 or more days with no response to antibiotics 2. Those cases showing these disorders as one of the
∙ Bilateral congestion of ocular conjunction clinical features in associations with other cutaneous
∙ Changes in the extremities peripherally including and/or extracutaneous manifestation which often
indurative edema, erythematous palms and soles and appear more significant.
membranous desquamation of fingers and toes That is the pigmentary disorders may be either:
∙ Lips and mouth show dryness, cracking and swelling of ∙ Depigmentary and hypopigmentary or
tongue papilla ∙ Hyperpigmentary of various shades.
∙ Polymorphous exanthema of torso without vesicles or
Peutz-Jeghers Syndrome
crust
∙ Acute purulent swelling of cervical lymph nodes. Synonym: Periorificial lentiginosis
Other findings include diarrhea, proteinuria leukocytosis This is an autosomal dominant disorder showing
and increased sedimentation rate. Cardiac abnormality may pigmented macules on the oral mucosa and skin associated
occur .While the vast majority of the cases are self-limiting with gastrointestinal polyposis.
and nonfatal, occasional deaths may occur as a result of There is no sex or racial preponderance. A family history
cardiac complications. is found in 60 percent of cases. Pigmented macules show
an increased amount of melanin in the basal layer of the
Incontinentia Pigmenti epidermis without any associated increase in the number
Incontinentia pigmenti, also known as Bloch-Sulzberger of melanocytes. Macules are present at birth, infancy or
syndrome, is an uncommon genetic disease with an X- early childhood. The oral mucosa is always involved with
linked dominant mode of inheritance. This distinctive affection of buccal mucosa, gums hard palate and lower lips.
multisystem disorder is characterized by abnormalities of Lesions are irregularly distributed round, oval or
skin pigmentation. irregularly patch brown or black in color and 1 to 5 mm
Linear rows of blisters on the extremities are seen at birth. in diameters. Also present on the face, hands, feet and
Occasionally the trunk is also involved. Later on the blisters nails. Gastrointestinal polyps are benign hamartoma with
are replaced by warty lesions that may persist until first relatively less potential for malignant transformation. They
year of age. In the final stage, there is hyperpigmentation, may occur throughout the gastrointestinal tract but are
caused by melanin deposition in the upper dermis. Mental commonly observed in the small intestine. The symptoms
of GI polyps are repeated abdominal colic, rectal bleeding, Etiology

hematoma, anemia usually start between 10 and 30 years
Various causes of this disease have been suggested, genetic,
of age. Apart from an increased risk of malignancy life
872 hormonal, infections and immunologic among others. It is
expectancy is normal.
generally assumed that the antigen-antibody reaction is not
Albrights Syndrome the sole cause of this disease. As majority of those affected
are women, hormonal deregulation plays a role.
Albrights syndrome consists of fibrous dysplasia, large Laboratory findings support the autoimmune etiologic
pigmented cutaneous macules endocrine dysfunction with role. Bertram has reported that 75 percent of a series of
precocious puberty and somatic over growth. Cutaneous 35 patients with Sjögren’s syndrome had in their sera
pigmented macules are usually present or appear soon antisalivary duct antibody. In addition the sicca complex
after birth and are usually light brown in color. They and Sjögren’s syndrome have been found to be associated
have irregular serrated margins and are asymmetrically with the HLA system, specifically HLA - DR3 and DR4.
distributed, (with in the midline).
Bony lesions usually first present during the first Clinical Features
decade with pain, pathological fractures, swellings and
The disease occurs predominantly in women over 40 years of
deformities. In females precocious puberty at the age of
age, although children or young adults may be affected. The
5 years may occur in the form of pubic hairs, vaginal
female:male ratio is 10:1. The typical features are dryness of
bleeding and breast enlargement. Biochemical raised
mouth and eyes as a result of hypofunction of the salivary
serum alkaline phosphatase level may be observed in the
and lacrimal glands. This often results in painful, burning
presence of widespread bone involvement.
sensations of the oral mucosa. In addition various secretory
Histopathologically hyperpigmented macules and
glands of the nose, larynx, pharynx and tracheobronchial
normal skin show a normal number of melanocytes
tree (buccopharyngolaryngitis sicca) as well as of the vagina
with absent or occasional macromelanosomes in the
are involved with this dryness. Sialochemistry studies have
melanocytes and keratinocytes. The disease is not usually
demonstrated significantly elevated levels of IgA, potassium
lethal. Pathological fractures normally unite easily.
and sodium in the patients of sicca complex. Rheumatoid
arthritis is an integral part of the secondary Sjögren’s
SYNDROMES ASSOCIATED WITH syndrome. It has been shown that patients with rheumatoid
SALIVARY AND LACRIMAL GLANDS arthritis have certain different clinical manifestations than
• Sjögren’s syndrome patients with sicca complex, despite similar histologic
• Heerford’s syndrome findings and some laboratory findings.
• Riley-Day syndrome In this regard patients without rheumatoid arthritis that
• Felty’s syndrome. is sicca complex more frequently manifest parotid gland
enlargements, lymphadenopathy, purpura, Raynaud’s
Sjögren’s Syndrome phenomenon, kidney involvement and myositis.
Synonyms: Sicca syndrome, Gougerat-Sjögren’s syndrome,
Histologic Features
rheumatoid sialadenitis.
Sjögren’s has described this syndrome in 1933. Three types of histologic alterations have been described:
Sjögren’s syndrome is a condition originally described as • In one case there may be intense lymphocytic infiltration
a triad consisting of keratoconjunctivitis sicca, xerostomia of the gland replacing all the acinar structures
and rheumatoid arthritis. Primary Sjögren’s syndrome is so although the lobular architecture is preserved, there
called when patients present only with dry eyes and dry may be proliferation of the ductal epithelium and
mouth (Sicca complex). The secondary Sjögren’s syndrome myoepithelium to form “epimyoepithelial islands”.
involves or develops systemic lupus erythematosus, Both of these histologic changes are identical with
polyarteritis nodosa , polymyositis or scleroderma as well those occurring in the benign lymphoepithelial lesion
as rheumatoid arthritis. (Mikulicz’s disease).
• The third alteration may be simply an atrophy of the Riley-Day Syndrome

glands sequential to the lymphocytic infiltration.
Riley-Day syndrome is characterized by sialorrhea, exces-
Laboratory Findings sive perspiration, defective lacrimation and erythematous 873
blotching of the skin.
Primary Sjögren’s syndrome have shown a polyclonal
hyperglobulinemia and may develop cryoglobulins. Other Features
Multiple organ or tissue specific antibodies are found,
Wide fluctuance in blood pressure, emotional instability
including antisalivary duct antibodies, rheumatoid factor
cold hands and feet and hyporeflexia. It is manifested first
and antinuclear antibodies.
in infancy by impaired swallowing and sucking and by
An increased sedimentation rate is present in 80 percent
absence of tears, growth is retarded and the ability to walk
of these cases. The antisalivary duct antibody present is
and sit and speech is delayed. There is marked absence of
three times more common in secondary Sjögren’s syndrome
fungi form papillae on the tongue. Occurs commonly or
than the sicca complex.
almost inclusively in Jews and is inherited as an autosomal
Treatment and prognosis: There is no satisfactory recessive trait. Sialorrhea is especially marked during
treatment for Sjögren’s syndrome. Patients are treated excitement. The syndrome is thought to result from an
symptomatically. Keratoconjunctivitis is treated with inborn error in catecholamine metabolism.
lubrication with artificial tears containing methyl cellulose.
Xerostomia is treated with the saliva substitutes. SYNDROMES AFFECTING TEETH
• Gardner’s syndrome
Heerfordt’s Syndrome
• Trichodento-osseous syndrome
Synonym: Uveoparotid fever. • Naegeli-Franceschetti-Jadassohn syndrome
Uveoparotitis: Uveoparotitis is considered to be a form • Johanson-Blizzard syndrome
of sarcoidosis in which characteristically there is firm, • Oculodentodigital dysplasia
painless usually bilateral enlargement of parotid glands, • Fanconi’s syndrome
accompanied by the inflammation of the uveal tracts of the • Lacrimo-auriculo-dento-digital syndrome
eye and cranial nerve involvement. • Cleidocranial dysplasia
The submaxillary and sublingual glands may be • Hereditary ectodermal dysplasia
similarly involved and even the lacrimal glands may be • Nevoid basal cell carcinoma syndrome.
swollen, all features suggestive of Mikulicz’s disease or Gardner’s Syndrome
Sjögren’s syndrome. A chronic low grade fever is often
present and the patient may complain of lassitude, malaise Gardner’s syndrome is an autosomal dominantly
and vague gastrointestinal disturbances or even nausea and inherited disorder with variable penetrance, characterized
vomiting. Xerostomia is common. The most common eye by multiple supernumerary teeth, multiple osteomas of
lesion in uveoparotitis and often the earliest symptoms bony skeleton, intestinal polyposis, and various skin
is uveitis, but conjunctivitis, keratitis and corneal herpes and soft tissue tumors. Multiple sebaceous cyst are also
among others also have been reported. associated.
Although the uveitis may begin unilaterally, it
Etiology
eventually becomes bilateral and in most cases result in
some permanent visual impairment. The most common Manifestation of Gardner’s syndrome is thought to be due
nerve paralysis is of seventh nerve which may be unilateral to abnormal growth of all three primordial germ layers.
or bilateral, which is said to occur in 1/3rd to 1/2 of the
cases. The signs and symptoms of this syndrome disappear Clinical Features
within time, although some swellings of the parotid glands Multiple polyps of the colon appear in the second decade
and visual disturbance may persists. of life. Although asymptomatic, malignant changes or
risk of colonic cancer is almost 100 percent by the age of Features

fifty. Skeletal abnormalities are localized cortical skeletal
A characteristic face in which the nose is thin and also
thickening of long tubular bones and osteomas of the
874 hypoplastic microphthalmia or microcornia. Enamel
mandibular rami are characteristic of this syndrome.
hypoplasia and syndactyly and camptodactyly of the fourth
Dental anomalies noted are supernumerary teeth,
and fifth fingers.
odontomas, multiple unerupted teeth and multiple caries.
Sebaceous or epidermoid cyst appear later in life over the Fanconi’s Syndrome
face, scalp and extremities. Lipoma are frequently noted in
Synonym: Familial pancytopenia.
the subcutaneous tissue. Fibromas are also reported.
Familial panmyelophthisis: It is inherited disorder of
Diagnosis childhood characterized by diffuse cutaneous pigmen-
Family history of colonic malignancy. Osteomas of the tation, skeletal and dental changes, hematological
mandible and cutaneous lesions are indicators of the anomalies, mental retardation and hypogonadism. There
possible symptomatic colonic polyps. is enamel hypoplasia reported with this syndrome.
Cutaneous pigmentation is present with 85 percent of the
Trichodento-osseous Syndrome cases characterized by generalized dusky or olive brown
pigmentation on lower trunk and neck.
This autosomal dominantly inherited familial syndrome
Hematological abnormalities include progressive hypo-
affects the hair, teeth and bones. Approximately 50 percent
plastic anemia, neutropenia and thrombocytopenia. Skeletal
of the affected patients complain of brittle nails that peel
anomalies are short and broad hands with tapering fingers,
frequently.
absence of thumbs, aplasia of the radius and microcephaly.
Dental manifestations are enamel hypoplasia, impacted
teeth, taurodont teeth and multiple dentoalveolar abscesses, Lacrimo-auriculo-dento-digital Syndrome
a thickened cortical bone enamel is thin with normal dentin.
Lacrimo-auriculo-dento-digital syndrome is an autosomal
Naegeli-Franceschetti-Jadassohn Syndrome dominant malformation complex that affects craniofacial
structures including teeth and salivary glands. The first
This is a rare autosomal dominant inherited syndrome case of lacrimo-auriculo-dento-digital syndrome was first
characterized by the development of reticulate brown or reported by Levey in 1967.
slate-gray pigmentation, yellowish discoloration of teeth
palmoplantar hyperkeratosis and hypohydrosis with heat The features are:
intolerance. Lacrimal—absence of the lacrimal puncta nasolacrimal
ducts and lacrimal sacs.
Johanson-Blizzard Syndrome Dental—dental dysplasia hypodontia, hypoplasia, amelo-
This syndrome is characterized by hypoplasia of the nasal genesis imperfecta, high narrow palate.
alae:
Digital—arachnodactyly, radioulnar synostosis, hypoplasia
• Oligodontia
of the first digit.
• Scalp and hair defects
• Sensory neural defects Ears—protuberant ears that are cup shaped. Sensory
• Short stature hearing loss.
• Psychomotor retardation and malabsorption.
The nose is beak like and the teeth are peg shaped and Nevoid Basal Cell Carcinoma Syndrome
hair is sparse. Height, weight and head circumference are (Bifid Rib Syndrome Or Gorlin-Goltz
all subnormal and osseous development is delayed. Syndrome)
The dental/oral manifestation
Oculodentodigital Dysplasia The developing teeth may get displaced because of the
Oculodentodigital dysplasia is an autosomal dominantly odontogenic keratocysts associated with this syndrome.
inherited syndrome that affects the eyes, teeth, digits, There is deformity of teeth and may not develop till middle
skeleton and the nervous system. age of life.
SYNDROMES ASSOCIATED WITH LIPS Hereditary Hemorrhagic Telangiectasia

AND CHEEK Synonym: Osler’s disease, Osler-Weber-Rendu disease.
This is a relatively common autosomal dominant
• Ascher’s syndrome 875
disorder. It was first described by Bird, Garland and Aiming.
• Melkersson-Rosenthal syndrome
HHT is characterized by widespread vascular involvement
• van der Woude syndrome
with recurrent episode of spontaneous bleeding with
• Hereditary hemorrhagic telangiectasia
multiple telangiectasia of the skin and mucous membranes.
• Cowden syndrome
• Zinsser-Engman-Cole syndrome. Clinical Features
The first and the most common clinical sign is persistent
Ascher’s Syndrome
nose bleeds which begins in childhood. Tiny red macules
Ascher’s syndrome, first described in 1920, is the and papules are seen around the mouth. The lesions blanch
combination of a progressive enlargement of the upper lip readily on diascopy. These are telangiectasia throughout
(double lip) lid laxity (blepharochalasis) and goiter. the gastrointestinal system and lungs. CNS manifestation is
The lip abnormality may be identical to the acquired migraines and epilepsy. Recurrent GIT bleeding may occur.
double lip or it may be the result of chronic recurrent then There may be associated liver spleen vascular anomalies.
persistent lip swelling. Thus, Ascher’s syndrome does have
some parallels with Melkersson-Rosenthal syndrome, but Oral Manifestations
only the upper lip is involved and the horizontal ford is The oral mucosa has telangiectasia on the vermilion border
almost always seen. The lips feel soft and lobulated. of the lip, the lip mucosa, tongue a buccal, mucosa and
Excessive salivation may be present—it is considered palate and less oftenly gingiva. Oral bleeding in observed
a localized disorder of elastic tissue because both the as a frequent complaint after tooth brushing. Multiple
eyelids and upper lip seem to be deficient in this structural telangiectasias have been reported in the CREST syndrome.
component. There may also be endocrine problems C — Calcinosis cutis
including thryoid goiter acromegaly and menstrual R — Raynauds phenomenon
irregularities. E — Esophageal dysfunction
S — Sclerodactyly
Melkersson-Rosenthal Syndrome T — Telangiectasia.
Synonym: Miescher’s disease
This condition is characterized by recurring spon- Cowden Syndrome
taneous swelling of one or both lips (which may become Synonym: Multiple hamartoma syndrome.
permanent) has no cause. The manifestation of this Cowden syndrome is a rare inherited genodermatose.
syndrome is facial swelling, facial neural paresis and The major features include:
plicated tongue. The syndrome generally appears in • Multiple hamartomatous papules and nodules of the
adults. Many cases reveal possible additional involvement skin and oral mucosa
of areas away from the face such as hands and chest. • Together with thyroid and breast anomalies
Additional symptoms include secretary disturbances of • Polyposis of the gastrointestinal tract.
the salivary and lacrimal glands. This syndrome was named after the first patient reported.
Almost all patients have mucocutaneous abnormalities,
van der Woude Syndrome typically facial papules or nodules (usually hair follicle
The van der Woude syndrome was first described by hamartoma with histologic features of trichilemmomas).
Demarquay Rischet. It is also called Demarquay-Rischet Or nodules on neck, hands and forearms and of the palms
syndrome. Its manifestations are: and soles.
• Congenital lip fistulas, especially of the lower lip
• Cleft lip and cleft palate Zinsser-Engman-Cole Syndrome
• Hypodontia Synonym: Dyskeratosis congenita
• Flattened nose and This extremely rare syndrome is characterized by
• Skeletal and cardiac abnormalities. keratinization of the oral mucosa, reticular cutaneous
hyperpigmentation, nail dystrophy and pancytopenia. pregnancy hydrominon develops because of difficulty in
There is sex linked recessive inheritance pattern. The swallowing caused by enlarged fetal tongue.
initial lesions may be noted as early as fourth or fifth year Many of the metabolic storage or lysosomes disorders
876 as a reticulated hyperpigmentation on the face neck and are characterized by macroglossia; the patients usually
trunk. Purpuric lesions can be seen as the hematology have so many other problems that the macroglossia almost
abnormalities develop. The most commonly observed never is the presenting complaint.
involvement of the oral mucous membrane (sites being Included in this group are:
tongue and buccal mucosa) is characterized by extensive • Mucopolysaccharidosis
keratinization, primary lesion that consists of a bulla • Hurler syndrome
that eventually ruptures apparently leads to eventual • Hunter syndrome
keratinization. The likelihood of squamous cell carcinoma • Sanfilippo’s syndrome
developing is well documented and all cases should be • Sulfatidoses
followed very carefully for such occurrence. • Austin syndrome
• Gangliosidoses
• GMI or landing syndrome
SYNDROMES ASSOCIATED WITH • Cornelia de Lange syndrome II with muscle and tongue
TONGUE hyperplasia, abnormal ears and CNS abnormalities
• Leroy syndrome
• Orofacial digital syndrome
• Mannosidoses
• Meckel syndrome
• Glycogen storage diseases mainly type II or Pompe’s
• Mobius syndrome
disease.
• Hurler syndrome
• Maroteaux-Lamy syndrome Orofacial Digital (OFD) Syndrome
• Beckwith hypoglycemic syndrome
• Aglossia-adactylia syndrome
OFD Type 1
• Melkersson-Rosenthal syndrome Orofacial digital syndrome has been subdivided into
• Burning mouth syndrome. several disorders but the classic or type 1 OFD is of
Many syndromes associated with tongue are primary interest. It is inherited as an X-linked dominant
characterized by macroglossia. They include the following: triat so that only females are affected because the gene is
lethal in males. The first case was described by Papillon -
Winchester Syndrome Leage and Psaume in 1954.
Winchester syndrome clinically resembles a storage Features of OFD Type 1
disorder but lacks the metabolic defects. Patients have joint
Clefts of the jaw and tongue in canine region. Others
deformities, corneal opacities, a coarse facies, thickened
features are syndacyly, brachydactyly and polydactyly),
skin and macroglossia.
small nostril, a lobulated tongue with hamartoma. Aberrant
hyperplastic oral frenula which appears to lead to the
Anhydrotic Ectodermal Dysplasia/Christ- clefting of jaws, tongue and upper lip.
Siemens-Touraine Syndrome
It is characterized by skin anomalies, alopecia, dental
Hurler Syndrome
abnormalities and in some cases by an enlarged tongue. Synonym: Mucopolysaccharidosis
Hurler syndrome is a disturbance of mucopolysaccharide
Zellweger Syndrome metabolism exhibiting a variety of classical clinical features.
It is characterized by an elevated mucopolysaccharide
Its features are dwarfism, cardiac defects, facial anomalies,
excretion level in the urine.
cutis laxa and macroglossia.
Clinical Features
Beckwith-Wiedemann Syndrome
The disease usually becomes apparent at the age of two,
Patients have an omphalocele, microglossia and hyper- progresses during early childhood and adolescence and
trophy of various organs or even gigantism. Often during terminates in death usually before puberty.
Facies—large head with prominent forehead, broad saddle Aglossia-Adactylia Syndrome

nose and wide nostrils, thick lips and large tongue, open
This syndrome in which absence of the tongue is associated
mouth and nasal congestion. Progressive corneal clouding
with failure of digits to develop is extremely rare. It was 877
is classic feature with hepatosplenomegaly. Flexion
first described by De Jussier.
contraction results in “claw hand”. The individuals are
dwarf and mentally retarded. Systemic Manifestations
Oral Manifestation Extremities involvement may be severe, from complete
peromelia to absence of single digit.
Short and broad mandible with prominent gonions. Delayed
eruption of teeth, localized areas of bone destruction in the Oral Manifestation
jaws may be found which appear to be representing hyper-
The tongue is absent in majority of the cases. The sublingual
plastic dental follicles with large pools of metachromatic muscular ridge may be enlarged. The mandible is usually
material, probably mucopolysaccharide. Gingival hyperpla- small and poorly developed.
sia is almost always present. Enlarged tongue is present.
Burning Mouth Syndrome
Histopathology
Clinical Features
Excessive accumulation of intracellular mucopoly-
saccharide in many tissues and organs and throughout the Burning mouth syndrome [burning and pain of the mouth
body. There is elevated level of mucopolysaccharide in the or of the tongue, painful tongue. Glossodynia] without any
urine. visible changes is common with many causes.
The patient complains of intense and unbearable pain
Mobius Syndrome that interferes with eating, sleeping and virtually every
other body function. The tongue may appear as red or
Synonym: Congenital facial diplegia
atrophic or the mucosa may appear entirely normal. The
Congenital facial diplegia is a nonfamilial deficient
complaints are usually limited to tongue but may involve
development of cranial muscles consisting of facial diplegia the entire mucosa.
with bilateral paralysis of the ocular muscles, particularly Burning mouth syndrome is most common in middle
the abducens. The cause of the disease is now recognized aged women especially around the time of menopause.
as degeneration of the 6th and 7th cranial nerve muscles Neurologic disorders may also present with mouth
although it was originally thought to be a primary nuclear or tongue symptoms, most often unilateral. If the pain
hypoplasia with secondary muscle atrophy. seems localized to one side of the tongue, a microbiologic
Clinical Features evaluation may be indicated.
It is manifested in the first few days of life by failure

to close the eyes during sleep. Because of the partial or
complete facial paralysis, the infant exhibits no change GINGIVA
in the facial expression even when crying or laughing. • Tuberous sclerosis
There is difficulty in mastication, saliva frequently • Zimmerman - Laband syndrome
drools from the corners of the mouth and speech is • Rutherford syndrome
severely impaired. • Ramon syndrome
The majority of patients have other associated • Cowden syndrome
congenital defects, including external ophthalmoplegia, • Papillon-Lefevre syndrome
deafness, paresis of the tongue, soft palate or jaw muscles, • Klippel trenaunay-Weber syndrome
clubfoot, mental defects and epilepsy. • Melkersson-Rosenthal syndrome.
Treatment and Prognosis Tuberous Sclerosis

There is no treatment for the disease but the prognosis (Described under syndromes with benign oral neoplastic and
appears to be good, barring complications. hamartomatous components). There is gingival fibromatosis
or there may be single or multiple fibromas of the gingiva, • Premature loss of deciduous teeth by the age of 4 or 5
oral mucosa and skin. years
• Deep periodontal abscess may be present
878 Zimmerman-Laband Syndrome • Permanent teeth are also lost in the same manner.
Synonym: Laband syndrome
Laband syndrome (LS) is a rare disorder characterized Klippel-Trenaunay-Weber Syndrome (KTW)
by gingival fibromatosis, abnormalities of the nose Klippel-Trenaunay-Weber syndrome was reported by
and /or ears and absence and /or hypoplasia of the nails Klippel and Trenaunay in 1900. It consists of a classical
or terminal phalanges of the hands and feet. Other triad of clinical features and angioma formation. The
more variable features include hyperextensibility of predominant features of the KTW syndrome are asymmetric
joints, hepatosplenomegaly, mild hirsutism and mental limb hypertrophy. Cutaneous hemangiomas and pigmenta.
retardation. Varicose veins located on the legs, abdomen and trunk.
The first case was described by Zimmerman in 1928; Other abnormalities include macrodactyly hyperpigmented
Lanband et al described the first familial occurrences in nevi on skin, telangiectasia, macrocephaly and enlarged
1964. genitalia.
Rutherford Syndrome Oral Manifestation

This syndrome has autosomal dominant inheritance: the Angiomatous involvement of lips, buccal mucosa,
manifestations are: tongue, palate, gingiva and oropharynx. In addition,
• Congenially enlarged gingiva enlarged maxilla, premature eruption of teeth and marked
• Delayed tooth eruption displacement of teeth on the affected side producing dental
• “Curtain like” corneal opacities malocclusion have also been reported.
• Associated features are: mental retardation and
dentigerous cysts. SYNDROMES ASSOCIATED WITH
Ramon Syndrome
NERVES
The characteristic features of this syndrome are: Gingival • Auriculotemporal syndrome (Frey’s syndrome)
fibromatosis. Hypertrichosis, cherubism, mental retardation • Reader’s syndrome (Paratrigeminal syndrome)
and epilepsy. There is characteristic perivascular fibrosis in • Horton’s syndrome (cluster headache)
gingival biopsies. • Migrainous syndrome (migraine)
Other associated features include juvenile rheumatoid • Horner’s syndrome
arthritis; the gingival fibromatosis usually always precedes • Jaw-Winking syndrome (Marcus-Gunn Phenomenon).
cherubism.
Auriculotemporal Syndrome
Papillon-Lefevre Syndrome Synonyms: Frey’s syndrome, Gustatory sweating
This is an inherited disorder transmitted as an autosomal The auriculotemporal syndrome is an unusual phenom-
recessive trait. The disease usually manifests during early enon which arises as a result of damage to the auriculotem-
childhood as circumscribed erythematous hyperkeratotic poral nerve and subsequent reinnervation of the sweat
plaques on the palms and soles. Hyperkeratosis may extend glands by parasympathetic salivary fibers.
to the sides of hands and feet, knees and elbows. Transverse
Etiology
grooves of nails occur.
Dental changes are: The syndrome follows some surgical operation such as
• Gingivitis removal of parotid tumor or the ramus of mandible or parotids
• Periodontitis of some type that has damaged the auriculotemporal nerve.
After a considerable amount of time following surgery Etiology

during which the damaged nerve develops, innervating
The suggested etiology is that it is caused by vasodilatation
sweat glands which then function after salivary, gustatory
involving the internal maxillary artery a branch supplying 879
or psychic stimulation.
the sphenopalatine region. The theory of deviation of nasal
Clinical Features septum being one of the causes is also questioned.
The patient typically exhibits flushing and sweating of Clinical Features

the involved side of the face, chiefly in the temporal area
This syndrome is characterized by unilateral paroxysm of
during eating. The profuse sweating may be evoked by the
intense pain in the region of the eyes, maxilla, ear and the
parenteral administration of pilocarpine or eliminated by
mastoid, base of the nose and beneath the zygoma.
the administration of atropine or a procaine block of the
Sometimes the pain extends into the occipital area as
auriculotemporal nerve.
well. The paroxysm of pain has a rapid onset, persists for
The auriculotemporal syndrome is not a common
about 15 minutes to several hours and then disappears as
condition. Nevertheless the possibility of its occurrence
rapidly as they began. There is no “trigger zone”. The pain
must always be considered.
occurs atleast once in a day regularly. This disappears after
Treatment some weeks or months and the period of freedom may be
for months or years.
Treatment of the auriculotemporal syndrome by the In addition to pain sensation, other complaints may be
intracranial division of the auriculotemporal nerve has sneezing, swelling of the nasal mucosa, severe nasal discharge
been reported to be successful. may be there after painful attacks. Also accompanied
are epiphora or watering of the eyes, bloodshot eyes and
Reader’s Syndrome (Paratrigeminal paresthesia. Men are more commonly affected than women.
Syndrome)
Treatment
The paratrigeminal syndrome is a disease characterized
by severe headache or pain in the area of trigeminal Cocainization of the sphenopalatine ganglion or alcohol
distribution with signs of ocular sympathetic paralysis. The injection of this structure is one of the widely used therapies.
sympathetic symptoms and homolateral pain in the head Surgical resection of the ganglion is done in some cases.
or eye occur without vasomotor or trophic disturbances.
These signs and symptoms usually appear suddenly. The
Migraine Syndrome (Migraine)
disease appears to be the most common in males, chiefly Migraine is a syndrome presenting manifestations of
those of middle age. diffuse disturbances in body functions occurring during
Paratrigeminal syndrome presents some of the signs of or after stress, characterized by severe periodic headache,
the Homer’s syndrome but can be differentiated from it by irritability and nausea.
the presence of pain and little or no change in sweating The cause of the disease is unknown but has been
activity on the affected side of face. The cause of this postulated to be a discharge of autonomic centers in the
disease is unknown but dramatic improvement has been forebrain leading to constriction in portions of the cerebral
found after elimination of dental infection. arterial tree. In susceptible patients it may become manifest
in the preheadache phenomenon. Then, as part of an
Horton’s Syndrome attempt to maintain cranial homeostasis there is decrease
Synonyms: Sphenopalatine neuralgia, cluster headache, in constrictor in certain other cranial arteries, particularly
periodic migrainous neuralgia. branches of the external carotid. These secondary effects,
Sphenopalatine neuralgia is a pain syndrome originally possibly harmonal as well as neurogenic in origin are the
described by sluder as a symptom complex referable to source of the headache.
the nasal ganglion. Vial described a similar syndrome but
believed it involved the vidian nerve and concluded that
Clinical Features
the condition reported by sluder should be termed “vidian Usually seen in second decade of life. The frequency
neuralgia”. It is considered to be a variant of migraine. of attacks is extremely variable as they may occur at
frequent intervals over period of year or on only a few The syndrome may be later in life but also is seen to
occasions during the lifetime of patient. A prodromal be as hereditary in some cases. Males are more commonly
stage (preheadache phenomena) is noted by some patients affected than females. The left upper eyelid is more
880 consisting of lethargy and dejection several hours before frequently involved than the right. It is also thought that
the headache. Visual phenomena such as scintillation about 2 percent of all cases of congenital ptosis are due
hallucinations or scotomas are often described. The to this condition. There are numerous theories concerning
headache pain consists of severe pain in the temporal, the etiology of this disease and these have been reviewed
frontal and retro-orbital areas although other sites such by Simpson. The most widely accepted is that the levators
as parietal, postauricular, occipital or suboccipital are palpebral muscle is connected not only with the third
also occasionally involved. The pain is usually unilateral nucleus, but also with the external pterygoid portion of the
but may become bilateral and generalized. The pain is fifth nucleus.
described as a deep aching, throbbing type. However, there is some evidence of supranuclear
involvement. An interesting condition Marin Amat
Treatment and Prognosis Syndrome or “inverted Marcus-Gunn phenomenon” is
The treatment of migraine includes a wide variety of usually seen after the peripheral facial paralysis. In this
drugs ranging from acetylsalicylic acid and codeine to condition, the eye closes automatically when the patient
ergotamine, methyl salicylic and nor epinephrine. opens his mouth forcefully and fully as in chewing and
The prognosis of the disease is good, since the tears may flow.
condition is not dangerous, and may undergo complete and
permanent remission. SYNDROMES ASSOCIATED WITH
BLOOD
Horner’s Syndrome
Synonym: Sympathetic ophthalmoplegia • Plummer-Vinson syndrome
Horner’s syndrome is a condition characterized by: • Chediak-Higashi syndrome
miosis or contraction of the pupil of the eye due to paresis • Sweets syndrome (Acute febrile neutrophilic derma-
of the dilator of the pupil. Ptosis: drooping of the eyelid tosis)
due to paresis of the smooth muscle elevator of the upper • Lazy leukocyte syndrome.
eyelid. Anhidrosis and vasodilatation over the face due to
interruption of pseudomotor and vasomotor control. Plummer-Vinson Syndrome
Its chief significance lies in the fact that it indicates Synonym: Iron deficiency anemia
the presence of a primary disease. The lesions in the The Plummer-Vinson syndrome is one manifestation of
brain-stem, chiefly tumors or infections in the cervical iron-deficiency anemia and was first described by Plummer
or high thoracic vertebrae will occasionally produce this in 1914 and by Vinson in 1922 under the term “hysterical
syndrome. Preganglionic fibers in the anterior spinal roots dysphagia” not until 1936; however was the full clinical
to the sympathetic chain in the low cervical and high significance of this condition was recognized. Ahlbom
thoracic area are rather commonly involved by infection, then defined it as a predisposition for the development of
trauma or pressure as by aneurysm or tumor to produce carcinoma in the upper alimentary tract. It is in fact one of
Horner’s syndrome. the few known predisposing factors in oral cancer.
Jaw-Winking Syndrome (Marcus-Gunn Clinical Features

Phenomenon; Pterygoid-Levators Synkinesis) While an iron deficiency anemia can occur at any age
This interesting condition consists of congenital unilateral Plummer-Vinson syndrome occurs chiefly in women and
ptosis with rapid elevation of ptotic eyelid occurring on in the fourth-fifth decade of life. Presenting symptoms of
movement of the mandible to the contralateral side. It is the anemia and the syndrome are cracks or fissures at the
commonly recognized in the infant by the mother, when corners of the mouth; a lemon tinted pallor of the skin, a
breast feeding her baby she notices one of its eyelids shoot smooth red, painful tongue with the atrophy of filiform
up. As in the case reported by Smith and Gans. and later fungiform papillae and resulting from esophageal
stricture of the web. Koilonychias or spoon shaped finger generalized lymphadenopathy and hepatosplenomegaly.
nails which are brittle and break easily have been reported The disease has been sometimes associated with the
in many patients. Splenomegaly has also been reported in malignant lymphomas.
20 to 30 percent of the cases. The depletion of iron stores in 881
the body, manifested as iron deficiency anemia may be the Oral Manifestations
direct cause of the mucous membrane atrophy, since the Ulcerations of the oral mucosa, severe gingivitis and
integrity of epithelium is dependant upon adequate serum glossitis are the commonly described oral lesion.
iron levels. The atrophy of the epithelium predisposes
to the development of carcinoma in these tissues. This Laboratory Findings
relationship was first noted by Albom in the patients Hematological studies show that the patients classically
suffering from carcinoma of pharynx and upper part of exhibit giant abnormal granules in the peripheral
esophagus. circulating leukocytes in their marrow precursors and in
many other cells of the body as well. These granules are
Laboratory Finding the hallmark of the syndrome and are invariably present.
Blood examination reveals a hypochromic microcytic They are thought to represent abnormal lysosomes and
anemia of varying degree while megaloblasts typical of bear resemblance to toxic granulations and Dohle bodies.
pernicious anemia. The red blood cell count is generally Pancytopenia is sometimes presents.
between 3,000,000 and 4,000,000 cells per cubic mm;
and the hemoglobin is invariably low. The anemia of Treatment and Prognosis
the iron deficiency type can be confirmed by lack of a There is no specific treatment for this disease and death
reticulocyte response following administration of vitamin usually occurs within first few years of life as a result of
B12. Serum iron is low and there is an absence of free secondary infection or hemorrhage.
hydrochloric acid in the stomach. The achlorhydria is
generally the cause of the faulty absorption of iron since Sweet’s Syndrome
the absence of hydrochloric acid prevents the conversion Synonym: Acute febrile neutrophilic dermatosis
of unabsorbable dietary ferric iron to the absorbable Sweet’s syndrome was first described by sweet in 1964.
ferrous state. The exfoliated squamous epithelial cells also He identified young women who had sudden development
showed changes such as deficiency of keratinized cells, a of erythematous nodules associated with fever and malaise.
reduced cytoplasmic diameter of cells with a paradoxical Typically the skin lesions consists of coleasing, plague
enlargement of the nucleus, an increase in the nucleoli, forming papules which at first sight give the illusion of
presence of double nucleus and karyohexis. vesiculation but are solid on palpation. Histopathologically
the lesion show papillary dermal edema and an intense
Chediak-Higashi Syndrome
neutrophilic infiltrate, but do not represent an infection or
Chediak-Higashi syndrome is an uncommon genetic leukemic infiltrate.
disease which is often fatal in early life as result of a In majority of the cases a febrile infection of the
lymphoma like terminal phase, hemorrhage or infection. It upper respiratory tract, tonsillitis or influenza like disease
is transmitted as an autosomal recessive trait. precedes the clinical symptoms by 1 to 3 weeks. Twenty
percent of the acute myelogenous leukemia patients have
Clinical Features documented this manifestation as an early sign.
The characteristic clinical features of this disease consist
of oculocutaneous albinism, photophobia, nystagmus Lazy Leukocyte Syndrome
and recurrent infections. The degree of albinism and the First described by Miller, Oski and Harris in 1971. Lazy
structures involved are quite variable as is pigmentary leukocyte syndrome is caused by loss of the chemotactic
dilutions of structures. functions of the neutrophils. The bone marrow contains
Recurrent infections usually involve the respiratory normal numbers of mature neutrophils, but the patients
tract and skin. Occasional other findings include neurologic have severe neutropenia because the cells are unable to
problems, a variety of gastrointestinal disturbances, migrate from the marrow to the peripheral blood.
Clinical Manifestations Diffuse Neonatal Hemangiomatoses

The infections complications are apparent at age 1 to 2 This term encompasses a benign form in which the child
882 years. The most common infections noted are gingivitis, may have hundreds of small cutaneous hemangiomas
stomatitis, otitis media and bronchitis. The total WBC count without visceral involvement and a disseminated form
is in the low normal range. But the absolute neutrophil in which organ involvement is present. The latter group
count is as low as 100/mm3 to 200 mm3. Erythrocytes and is prone to complications like hemorrhage, disseminated
platelets are normal. The diagnosis is based on neutrophil intravascular coagulation. Cardiac failure and compression
mobilization tests showing lack of normal response. of vital structures. Systemic corticosteroids are usually
indicated.
VASCULAR MALFORMATIONS Sturge-Weber Syndrome
Synonym: Encephalofacial angiomatoses
Syndromes associated with hemangiomas:
A facial portwine stain in the distribution of the
• Maffucci’s syndrome
first branch of the trigeminal nerve is associated with
• Blue-Rubber bleb nevus syndrome
homolateral leptomeningeal angiomatosis, usually over
• Diffuse neonatal hemangiomatoses
the posterior parietal and occipital lobes of the cerebral
Syndromes associated with Ports wine stain cortex. It is postulated to be a developmental defect of
• Sturge-Weber syndrome certain ectodermal and mesodermal elements closely
• Klippel-Trenaunay syndrome approximated in the brain and meninges at 4 to 8 weeks of
• Von Hippel-Lindau syndrome gestational age.
• Parkes-Weber syndrome The port wine stain is present at birth and is unilateral,
• Beckwith-Wiedemann syndrome. but may cross the midline and also involve mucosae.
Patients with additional involvement of in the distribution
Maffucci’s Syndrome of the second branch of the trigeminal nerve have a high risk
Cutaneous hemangiomas appearing in infancy are of ocular complications, some patients also have bilateral
associated with hard nodules of the bones, especially distal, facial lesions or involvement of the trunk or extremities.
which are enchondroma on histology. Convulsions of the grand mal type generally appear
Abnormal bone growth, interference with epiphyseal during the first year of life and behavioral problems,
cartilage and pathology fractures can cause gross deformity subnormal intelligence, contralateral hemiparesis and EEG
which is added to by enlargement of the vascular swellings abnormalities (usually unilateral or focal) may be seen.
into large masses. Ocular involvement (40%) is in the form of ipsilateral
Maffucci’s syndrome is associated with an increased glaucoma, congestion strabismus and loss of vision.
risk of malignancies like chondrosarcoma, fibrosarcoma, Characteristic S-shaped intracranial calcifications are
angiosarcoma, lymphangiosarcoma, osteosarcoma and found in the leptomeninges within the first few months of
ovarian tumors. life, on CT scans and on X-rays after the age of 2 years.
They increase in density until the end of second decade
Blue Rubber Bleb Nevus Syndrome when 50 to 60 percent of patients show this on radiography.
Synonym: Blans syndrome They are thought to be due to anoxic injury from vascular
Blue-Rubber bleb nevus syndrome is seen as an stasis and increased vascular permeability in the areas of
association of multiple cutaneous and gastrointestinal the angioma.
tract hemangiomas, the skin lesions are typically bluish,
rubbery, nipple like compressible angioma, along with SYNDROMES ASSOCIATED WITH
smaller blue marks and larger cavernous hemangiomas.
IMMUNODEFICIENCY
Gastrointestinal bleeding, hematuria, menorrhagia and
epistaxis may be the presenting features if other visceral • Acquired immunodeficiency syndrome
angioma are present. Cases may be sporadic or familial • Reiter’s syndrome
with autosomal dominant inheritance. • Behçet’s syndrome.
Reiter’s Syndrome SYNDROMES ASSOCIATED WITH

The underlying cause of this disease syndrome is unknown HORMONAL DISTURBANCES
although there appears some genetic influence in some
• Cushing syndrome 883
cases. An abnormal immune response to microbial antigens
• Adrenogenital syndrome
is now considered to be a likely mechanism for the multiple
• General adaptation syndrome
manifestations of this disease. (Syndrome)
• Hutchinson-Gilford syndrome
Clinical Features • Waterhouse-Friderichsen syndrome.
Arthritis, nongonococcal urethritis, and conjunctivitis
Cushing Syndrome
or uveitis. The urethritis and conjunctivitis or uveitis.
The urethritis generally precedes the appearance of other Cushing syndrome is a result of hormonal excess resulting
lesions. Mucocutaneous lesions may be seen in up to half from any of the following:
of the patients. • Hyperplastic adrenal cortices without any other
Orally the lesions have been described as relatively clinically evident endocrine lesion.
painless aphthous type lesions (ulcers) occurring almost • Adrenal cortical adenoma or carcinoma
anywhere in the mouth. Tongue lesions are like that of • Ectopically located adrenal like tumor for, e.g. of an
the geographic tongue. The duration of the disease varies ovary
from weeks to months and recurrences are not common. • ACTH secreting tumor of the anterior pituitary
Nonsteroidal anti-inflammatory drugs are used for the • Nonpituitary carcinoma for, e.g. of a lung or pancreas.
treatment of this disease. When this syndrome is associated with spontaneous
bilateral adrenal hyperplasia, it is referred to as Cushing
Behçet’s Syndrome disease. This syndrome is characterized by a rapidly
acquired adiposity about the upper portion of the body,
Exact etiology of this syndrome is unknown although the
moon like appearance of the face. A tendency to become
underlying disease mechanism may be very similar to the
round shouldered and develop a “buffalo-hump” at the
one associated the aphthous ulcers. Genetic predisposition
base of the neck. Other features include muscle weakness,
and viral etiology has also been suggested.
vascular hypertension, glycosuria not controlled by insulin
Clinical Features and albuminuria.
The oral pathologist’s primary concern with this
The lesions of this syndrome typically affect the oral cavity, peculiar disease state lies in the bone changes. In children
the eye, and the genitalia. Recurrent arthritis of the wrists, there may be osteoporosis and premature cessation of
ankles and knees may be seen. Cardiovascular manifestations the epiphyseal growth, while in adults there is a severe
are usually of thrombotic type and neurologic manifestations osteoporosis.
are in the form of headaches. Oral manifestations of this The pathogenesis suggested is based on the SFN (sugar-
syndrome appear identical to the ulcers of the aphthous fat-Nitrogen) hormone group of steroids. The ‘N’ hormone
stomatitis. The ulcers are usually of the minor aphthous type stimulates osteogenesis and closing of the epiphysis and
and are found in the typical aphthous distribution. Ocular ‘S’ hormone leads to a retardation of osteoblastic activity
changes are found in most of Bechetix patients. and reduction in matrix formation.
Uveitis, conjunctivitis and retinitis are more common
inflammatory processes. Genital lesions are ulcerative in Adrenogenital Syndrome
nature and may be the cause of significant pain and discomfort. This condition results when there is hyperplasia of the
Inflammatory bowel disease has also been reported. adrenal cortex. Depending on the age at onset and the
Histopathology—T lymphocytes are predominant. Neutro- sex of the person affected the clinical signs occur. These
philic infiltrates in which the cells appear within the vessel manifestations are pseudohermaphroditism, sexual preco-
walls have also been described. city and virilism in women or feminization in men. If the
disease begins early, premature eruption of the teeth may
Treatment—consist of systemic steroid therapy. occur.
General Adaptation Syndrome of the premature ageing process. Delayed eruption is also
reported.
General adaptation syndrome (GAS) represents the signs
and symptoms occurring due to prolonged “stress” as a part
884
of individual adaptation mechanism. The adrenal gland and
Waterhouse-Friderichsen Syndrome
stress has a close relationship. This theory was proposed by Acute adrenal cortical insufficiency is relatively rare and
Hans Selye. it occurs in connection with waterhouse-Friderickson
Adrenal changes result due to prolonged stress leading syndrome. This disease primarily occurs in children
to mobilization of lipids and ultimate exhaustion atrophy but also occurs in adults. It is characterized by a rapidly
of the cortical cells. The hormones of the adrenal cortex fulminating septic course, a pronounced purpose and death
are necessary for cellular enzymes to catalyze the energy within 48 to 72 hours—Meningococci, streptococci and
producing processes of cells. All ‘stresses’ agents stimulate pneumococci are the organisms most responsible for the
adrenal function through stimulation of pituitary to secrete disease. At autopsy, the conspicuous change is bilateral
ACTH. If excessive amounts of hormones are produced, adrenal hemorrhage.
eventually pathologic changes occur in those tissues which The use of antibiotics and cortisone has changed the
respond to the stimulation and the diseases of adaptation, course of the disease from its usual fatal termination to
(hypertension, periarteritis nodosa and others) result. recovery in some cases.
The stages are:
• Ist “Alarm reaction” which consists of a shock phase SYNDROMES WITH BENIGN ORAL
and then a counter shock phase NEOPLASTIC OR HAMARTOMATOUS
• IInd “Adaptation stage” in which a person’s resistance
to original stressor is greater but his resistance to other
COMPONENTS
stressor agents is lowered • von Recklinghausen’s neurofibromatosis
• IIIrd—if the stressor is continued. He eventually enters • Nevoid basal cell carcinoma syndrome
a stage of exhaustion and dies. • Multiple mucosal neuroma syndrome (multiple endo-
If the stressor is removed, he enters a stage of crine neoplasia type III)
convalescence and recovers. • Tuberous sclerosis
• Acanthosis nigricans
Progeria (Hutchinson-Gilford Syndrome) • Albright’s syndrome.
Progeria is a very rare disease originally described by
Hutchinson in 1886. It is of unknown etiology and is von Recklinghausen’s Neurofibromatosis
characterized by dwarfism and premature senility. It is Two distinct varieties of this classic syndrome are now
thought to be transmitted as an autosomal recessive trait. recognized:
• Neurofibromatosis 1: which is often associated with
Clinical Features
oral lesions.
Affected infants appear normal at birth, but the typical • Neurofibromatosis 2: (bilateral acoustic neurofibro-
clinical features become manifest within the first few years. matosis) which is caused by a gene on a different
The patients all have amazing resemblance to each other, chromosome is much less common, and while often
exhibiting alopecia, pigmented areas of the trunk, atrophic accompanied by other central nervous system tumors
veins and loss of subcutaneous fat. The individuals have is less frequently associated with obvious peripheral
a squeaky voice, a beak nose and a hypoplastic mandible. neurofibromatosis or oral lesions.
The intelligence these individuals is either normal or above Neurofibromatosis 1 is inherited as an autosomal
normal. Even at an early age person resembles a wizened dominant condition. But only half the cases exhibit a
little old person. family history. The syndrome is characterized by the
simultaneous occurrence usually on the trunk, axilla
Oral Manifestations and pelvic area of light brown pigmentation (cafe au lait
As described by Gardner’s, there is accelerated formation spot, light brown macules with a smooth outline “like the
of irregular secondary dentin, apparently a manifestation coast of California”). The finding of six or more macules
with a diameter of 1.5 cm or greater is diagnostic of Neurologic anomalies: Mental retardation, dural calci-
neurofibromatosis. fication, agenesis of corpus callosum.
Cafe au lait spots are also found in 10 percent of the Sexual abnormalities: Hypogonadism in males and
normal population, especially in fair skinned persons. 885
ovarian tumors.
Similar skin lesions with the same name occur in Albright’s
syndrome. Oral manifestations: The keratocyst may displace the
Other findings are axillary freckling (Crowe’s sign) developing teeth and result in their deformity.
and a wide variety of nerve and nerve sheath tumors in Multiple Endocrine Neoplasia Syndromes
both central and peripheral nervous systems.
(MEN Syndromes)
The peripheral lesions are often indistinguishable from
those called neurilemmoma (tumors of the nerve sheath: This is a group of syndromes characterized by tumors of
schwannoma). Both neurofibroma and neurilemmoma are various endocrine organs occurring in association with
encapsulated S-100 positive tumors that show patterns of a variety of other pathologic features. Steiner and his
whorled connective tissue elements histologically with associates have classified these syndromes into:
readily recognizable axons with or without myelin sheath. MEN I — type I
The central lesions because of their location within a MEN II — type II
bony cavity often in association with various nerve roots Khairi and associates have decribed type III (MEN III)
lead to neurologic symptoms, mental retardation and other workers have subdivided type II into II A (synonymous
vertebral anomalies large infiltrating lesions that occur with original type II) and II B (synonymous with type III).
both peripherally and centrally and lead to severe deformity
Clinical Features
and are referred to as ‘plexiform neuroma’. Pheochromo
cystomas (tumors of the adrenal medulla and paraganglia). MEN I: Consists of tumors of hyperplasia’s of the
Approximately 5 percent of the patients with neuro- pituitary, parathyroid, adrenal cortex and the pancreatic
fibromatosis have well developed oral lesions and islets occurring in association with peptic ulcers and gastric
macroglossia. The tongue being the most common oral hypersecretion.
location for neurofibroma both in this syndrome and in MEN II: (Sipples syndrome, II A) is characterized by
solitary oral neurofibroma. parathyroid hyperplasia or adenoma but no tumors of
pancreas. However, in addition these patients have
Basal Cell Nevus or Nevoid Basal Cell pheochromocytomas of the adrenal medulla and
Carcinoma Syndrome medullary carcinoma of the thyroid gland. There is no
Synonym: Gorlin-Goltz syndrome, Jaw-cyst-bifid rib peptic ulcer.
syndrome. MEN III: (II B) it is characterized by mucocutaneous
This syndrome was first described by Binkely and neuroma, pheochromocytomas of the adrenal medulla,
Johansson in 1951. This has been thoroughly reviewed by medullary carcinoma of the thyroid. Other abnormalities
Gorlin and his co-workers. include hypertrophied corneal nerves, other skeletal defects
and gastrointestinal difficulties, oral manifestations.
Clinical Features
MEN III: Is particularly having the constant component
This syndrome has the following variable manifestations: of multiple oral neuroma. The neuroma are most common
Cutaneous anomalies: Basal cell carcinoma, other on the lips tongue and buccal mucosa. They produce
benign cyst and tumors, palmar pitting, palmar and plantar “Bumpy Lips” since the neuroma present at birth or may
keratosis and dermal calcinosis develop later appear as small elevated sessile nodules on
the vermilion producing puffy lips. On the tongue they are
Dental osseous anomalies: Multiple odontogenic kerato- commonly present on the anterior third.
cyst, jaw prognathism, rib anomalies (often bifid) and verte-
bral anomalies. Tuberous Sclerosis
Ophthalmologic abnormalities: Hypertelorism with wide Synonyms: Pringle-Bourneville syndrome adenoma seb-
nasal bridges, congenital blindness and strabismus aceum.
Tuberous sclerosis (TS) is an autosomal dominant papillomatous lesions can be found on the lips, especially
disorder with marked variability of expression in a given at the commissures. The tongue shows elongated filiform
family. papillae, deeper folds and may appear enlarged. The buccal
886 mucosa may be also thickened and show increased folds.
Clinical Features there may also be gingival hyperplasia. In contrast to skin
The key skin findings are hypopigmented macules (ash- lesions, the oral lesions are usually not pigmented. The
leaf macules) connective tissue nevi, (shagreen patch) and papillomatous lip changes resembling angular cheilitis
multiple angiofibroma. Red brown macules and papules are limited almost exclusively to tumor, related AN. The
are seen about the mouth in the nasolabial folds. CNS course of the disease is chronic and there is no satisfactory
findings include epilepsy mental retardation. treatment for this disease.
Oral Features Klippel-Trenaunay Syndrome

The oral cavity frequently shows distinctive changes. Classically, a port wine stain usually of the lower limb
Angiofibroma, fibromas or papillomas are found on the is associated with limb hypertrophy, lymphoedema,
gingiva, hard and soft palate, buccal mucosa and tongue. venous varicosities and obstructed, absent or deeper
They may be white or yellow although they lack any vessels.
distinctive tint. Both dentitions have tiny pits arising from The cutaneous lesions be angioma or angiokeratomas.
enamel defects. The pits are often an early diagnostic The bone and soft tissue hypertrophy may be both linear
clue. A high palate, macroglossia, cleft lip and palate and (almost 100% of patients) and circumferential (75%) and
hemangiomas have been described. develops gradually. Varicose veins are present which may
be obscured clinically by edema.
Acanthosis Nigricans
Acanthosis nigricans (AN) is a misnomer. Patients develop Von Hippel-Lindau Syndrome
velvety dark patches particularly in flexural areas, but also
in the mouth. There is papillomatosis and hyperkeratosis but An autosomal dominant condition is classically present
no acanthosis and no increase in pigment, it appears that the as bilateral retinal angiomatosis leading to visceral
deranged epithelial growth is directed through epidermal impairment and blindness and cerebellar or medullary
growth factor receptors. Several type of AN can be identified. hemangioblastoma.
Renal angioma and/or renal cell carcinoma, hepatic
• Tumor-related: In many cases, when tumor is removed
angioma and pheochromocytoma may occur. Port wine
AN disappears.
stains and café au lait spots are seen in 5 percent of patients.
• Endocrine: In a variety of endocrine syndromes AN is
a frequent finding.
Parkes-Weber Syndrome
• Juvenile or familial AN: Reported in some families
• Drug induced: A variety of drug, primarily nicotinic Arteriovenous fistulas are seen with features of the
acid can produce presumably by acting on growth Klippel-Trenaunay syndrome, viz. Port wine stain,
factor receptors. venous varicosities and limb hypertrophy. They may
be suspected by a thrill or continuous bruit at the
Oral Features involved site. Their diagnosis should be confirmed by
The perioral lesions may be velvety brown patches arteriography for large fistulae or by diagnostic methods
particularly at the corners of the mouth. Multiple using isotopes.
1. Defects in clavicle is characteristics feature of: 8. A syndrome characterized by dwarfism and premature
887
a. Cleidocranial dysplasia senility:
b. Edward syndrome a. Progeria
c. Pierre-Robin syndrome b. Hutchinson-Gilford syndrome
d. Mobius syndrome c. Both
2. Trisomy 18 syndrome refers to: d. MEN syndrome
a. Down’s syndrome b. Patau syndrome 9. A lemon tinted pallor of the skin, esophageal stricture
c. Edward syndrome d. Crouzon syndrome of web, koilonychias is the clinical feature of:
a. Plummer-Vinson syndrome
3. An autosomal recessive disorder characterized by oral,
b. Horner’s syndrome
facial and digital defects is:
c. Parkes-Weber syndrome
a. Anderson syndrome b. Mohr syndrome
d. None
c. Heerfordt’s syndrome d. Sjögren’s syndrome
10. Multiple odontogenic keratocyst, jaw prognathism,
4. A connective tissue disorder characterized by abnor-
bifid rib and vertebral anomalies seen in:
malities of skeletal, cardiovascular and ocular system is:
a. MEN syndrome
a. Marfan’s syndrome b. Ehlers-Danlos syndrome b. Gorlin-Goltz syndrome
c. Albright’s syndrome d. Both a and b c. Progeria
5. A triad consisting of keratoconjunctivitis sicca, xeros- d. Both a and b
tomia and rheumatoid arthritis is: 11. Treacher Collins Syndrome primarily affects struc-
a. Heerfordt’s syndrome tures developing from:
b. Sjögren’s syndrome a. First brachial arch
c. Riley-Day syndrome b. Second brachial arch
d. Fanconi’s syndrome c. Both first and second brachial arch
6. Flushing and sweating of the involved side of face, d. None of the above
chiefly in the temporal area seen in: 12. Ehlers–Danlos syndrome is characterized by except:
a. Horton’s syndrome b. Rutherford syndrome a. Skull hyperextensibilty
c. Frey’s syndrome d. Ramon syndrome b. Joint hypermobility
7. Buffalo hump development at the base of neck refer to: c. Hyperelasticity of skin
a. Beçhet’s syndrome d. Ectopic lentis
b. Reiter’s syndrome 13 Multiple sebaceous cyst are often associated with:
c. Adrenogenital syndrome a. Gardner’s Syndrome b. Kawasaki’s Syndrome
d. Cushing’s syndrome c. Fanconi’s Syndrome d. All of the above
Appendices
890
APPENDIX I: DIFFERENTIAL DIAGNOSIS OF MOST COMMON LESIONS OF ORAL CAVITY
Aparna Thombre
Etiology Types Clinical/Radiological Pathological features Management
features
Microdontia Pituitary True generalized— Affected teeth are Crown and bridge
dwarfism, Down’s all the teeth are maxillary lateral should be given
syndrome, smaller than incisors and 3rd
congenital heart normal. molars. Peg shaped
disease, progeria Relative laterals
generalized— (mesial and distal
normal or slightly sides converges or
smaller than taper incisally).
normal teeth; are
present in jaws
that are somewhat
larger than normal.
Localized—it
involves only
single tooth.
Macrodontia Pituitary True generalized— Crowding, Orthodontic
(megadontia) gigantism, facial all the teeth are malocclusion, treatment
hemi-hypertrophy, larger than normal. impaction of teeth
angioma of face, Relative generalized
and genetic —normal teeth
component present in smaller
jaw
Localized—one or
more large teeth
exist
Fusion Hereditary Complete (before Esthetic problems, Esthetic recovery
calcification) periodontal
Incomplete (after complication
calcification)
Concrescence Traumatic injury, Union with
crowding of teeth, cementum,
hypercementosis extraction of teeth
should be done
carefully
Dilaceration Trauma during Sharp bend in root,
development may cause
problems during
extraction
features
Gemination Hereditary and Bifid crown on
familial tendency single root.
Common pulp
canals and either
single or partially
divided pulp
chambers. Crown
is wider. Enamel
or dentin of crown
hypoplastic or
hypocalcified
Taurodontism Failure of Hertwig Hypotaurodont Bull like teeth, No treatment is
root sheath to Mesotaurodont elongated pulp required
invaginate Hypertaurodont chamber,
associated with
Klinefelter
syndrome
Dens in dente Defect in Coronal type Mild type Restoration of
Appendices
(dens invaginatus) morphological (occur in crown) (accenuted lingual defect

development Radicular type pit area),
(occur in root) intermediated
form (small pear
shaped
invagination)
producing tooth in
tooth appearance
Extreme form (it
extends beyond
pulp chamber)
Talon cusp T shaped Preventive care
projection called taken by
eagle Talon. performing
Associated with endodontic
Rubinstein-Taybi treatment
syndrome
891
892
features
Amelogenesis Hereditary, Hypoplastic Chalky white color Hypoplastic (lack Composite veneer
imperfecta involves only Hypomaturation teeth, open contact of differentiation crown should be
ectodermal Hypocalcification point, severe of ameloblasts, no given
component abrasion, cheesy matrix formation),
enamel, snow Hypocalcification
capped teeth (defective matrix
formation and
subnormal mineral
deposition),
Hypomaturation
(alteration in
enamel rod and
rod sheath
structure)
Dentinogenesis Autosomal Type I (with Amber like Enamel is normal, Metal and ceramic
imperfecta dominant mode osteogenesis appearance, mantle dentin crown
imperfecta) translucent teeth, (narrow zone of
Type II (without multiple pulp dentin beneath
osteogenesis exposure, teeth not enamel), less
imperfecta) sensitive dentinal tubules,
Type III large area of a
(brandywine type) tubular dentin,
pulp chamber are

obliterated with a
tubular dentin.
Dentin dysplasia Autosomal Radicular Mobility of teeth, Coronal and
(root less teeth) dominant coronal bluish or brownish radicular dentin
translucency at comprised of
cervical area, tubular and
enamel does not osteodentin,
chip off appear as lava
flowing around the
boulders. Normal
and abnormal
dentin is well
demarcated
features
Regional Local ischemic Abnormal pulp, Dentin is thin and Extraction of teeth
odontodysplasia changes during teeth deformed, globular, irregular followed by
(ghost teeth) odontogenesis soft leathery tubules, and wide fabrication of
appearance and predentin layer, prosthesis
yellowish brown in tiny droplet
color calcification
Agnathia Congenital Absence of
(hypognathous) mandible or
maxilla,
rudimentary
tongue, absence of
ear
Agenesis of jaws Congenital Absence of some
part of mandible or
maxilla, absence of
condyle
Micrognathia Congenital heart Apparent type, Midfacial Surgical approach
disease, Pierre Robin True type deformity, to correct
Appendices
syndrome, in (congenital or interference in micrognathia

acquired mouth acquired) feeding, speech
breathing, problems,
ankylosis malocclusion
Macrognathia Paget’s disease, Gummy smile Resection followed
(megagnathia) fibrous dysplasia, Mandibular by orthodontic
pituitary gigantism prognathisms, treatment
prominent chin
button, steep
mandibular angle
Facial Hormonal Thick skin, No treatment
hemihypertrophy imbalance, hypertrichosis,
incomplete excessive secretion,
twinning and unilateral
lymphatic enlargement
abnormalities
893
894
features
Facial hemiatrophy Trauma, heredity, Vertical furrow or No effective
infection line in midline, treatment
peripheral nerve howling of cheek,
dysfunction delayed tooth
eruption
Cleft lip and cleft Hereditary, Unilateral Difficult Obturators,
palate nutritional factors, incomplete, breastfeeding, surgical correction
defective vascular unilateral upper anterior
supply, traumatic complete, bilateral misplaced, middle
stress, Streeter’s complete cleft ear infection,
fetal dysplasia difficulty in speech
Double lip Hereditary Congenital or Cupid bow, Excision for
acquired associated with cosmetic reason
Ascher’s syndrome
Peutz-Jeghers Autosomal Café au lait spot, Oral macular Surgical treatment
syndrome dominant trait intestinal lesion exhibits for intestinal
polyposis, and excessive polyps,
intussusceptions accumulation of steroid injection
melanin granules
in basal cell layer
Cheilitis granulomatosa Allergic origin. Diffuse, nodular, Granulomatous Surgical treatment

Hypersensitivity to painless, firm inflammation with
bacterial toxins growth of lip. chronic
Associated with inflammatory cells,
Melkersson- Langhans types of
Rosenthal multinucleated
syndrome giant cells
Fordyce granules Hereditary, normal Multiple, small, The peripheral No treatment is
variation discrete milia like cells are flat and required
yellowish lesion. darkly stained and
Confluent plaque inner cells are lipid
are also present rich and pale. The
duct may show
keratin plugging
features
Heck disease (focal HPV type 13 and Small Hyperparakeratosis Self-regressing
epithelial 32 pedunculated, with extensive lesion
hyperplasia) polypoid or acanthosis,
nodular soft tissue koilocytes, fusion
growth. Size 1 to 5 of rete pegs,
mm increase mitotic
activity
Degeneration of
the basal layer
White sponge Autosomal Bilateral, Marked thickening No treatment is
nevus dominant with asymptomatic, of epithelium with required
incomplete deeply folded hyperparakeratinization,
penetrance white or gray acanthosis and
lesion. Spongy spongiosis.
consistency, Intracellular
sometimes ragged edema, pyknotic
white area nuclei. Basket
wave appearance
Fissured tongue Congenital, genetic Small furrows or No treatment is
Appendices
defect, vitamin grooves on the required.

deficiency dorsal surface of Cleaning of tongue after
tongue. Discomfort consumption of food
due to food thoroughly
accumulation.
Associated with
Melkersson-
Rosenthal
syndrome
Median rhomboid Persistence of Elongated Mild to severe Antifungal agents,
glossitis tubercular impar, erythematous parakeratosis, antiseptic gargles.
infection due patch, diamond shaped thinning
Candida albicans area devoid of suprapapillary
papillae epithelium, acanthosis,
elongation of
rete pegs, neutrophilic
infiltration
895
896
features
Geographic tongue Family history, Multiple irregular, Hyperparakeratinization Heavy dose of vitamins
associated with well demarcated of epithelium, with should be given
emotional stress patchy erythematous loss of filiform
areas with papillae. Intercellular
desquamation of edema, spongiotic
filiform papillae. abscess (neutrophil
Periphery polymorphs
surrounded by
yellowish-white
serpiginous line
Lingual thyroid Endodermal down Nodular exophytic It is composed of normal Surgical excision
nodule growth at the site mass, smooth cystic mature thyroid tissue. should be done
of foramina cecum swelling, dysphonia, Colloid degeneration
dysphagia and dyspnea
Thyroglossal duct Remnant of Asymptomatic mobile Linear stratified Surgical excision
cyst embryonic swelling in midline of squamous epithelium or along with tract
thyroglossal tract neck, dome shaped, transitional epithelium
compressible
Aberrancy Lingual It exhibits normal
mandibular salivary salivary gland lobules
gland depression, and ducts
gingival salivary
gland choristoma
Papilloma HPV can be responsible Slow growing, Multiple finger like Surgical excision of
cauliflower like projection, vascular the lesion
growth with finger connective tissue,
like projection. Base is few inflammatory cells,
sessile. White color and hyperkeratosis and
firm in consistency, it acanthosis
can occur in Down’s
syndrome or Cowden’s
syndrome
Keratoacanthoma Sun exposed Well circumscribed, Thick hyperkeratinized Surgical excision
Superficial epithelium of elevated umblicated with parakeratin should be carried
the sebaceous ducts crater like lesion with plugging, pseudoepith- out
central depression, eliomatous
margin rolled and hyperplasia, spinous cell
sharply delineated layer is thick
features
Intradermal Raised flat area on Clusters or nests of Surgical excision
(intramucosal) skin, with dark brown nevus cells confined to should be done
nevus common color, connective tissue,
mole asymptomatic spindle shaped
cells, multinucleated
giant cells, some cells
are pigmented
Junctional nevus Asymptomatic brown or Focal areas of Surgical excision
black macule proliferating nevus cells, should be carried
cluster of cells present out
at apex of epithelial rete
pegs
Compound nevus Pigmented papule or Presence of nevus Surgical excision
macules over the hard cells distributed in basal should be carried
palate or the gingiva layer of epithelium out
Appendices
as well as adjacent
connective tissue
Blue nevus Dome shaped dark blue Cells are elongated Surgical excision with
papule, flat pigmented bipolar, and spindle histopathological
macule over the skin of shaped. Fusiform evaluation
mucous membrane dendritic cells
Fibroma Excessive Small, well Proliferation of Surgical excision
proliferation of circumscribed, slow fibroblasts cells, should be carried
fibroblast cells, growing, nodular cells are spindle out
with synthesis of growth, pedunculated, shaped, bundles of
collagen surface smooth, soft to collagen, thin
firm in consistency epithelium and
flattening of rete pegs
897
898
features
Ossifying fibroma Reactive Peripheral type Peripheral—small Peripheral Surgical excision
proliferation of Central type painless, lobulated diffuse sheets of should be carried
periodontal or swelling, sessile, hard to proliferating out
periosteal tissue firm on palpation fibroblast with plump
Central—bony monomorphic nuclei,
hard swelling, hypercellular reactive
expansion and tissue, osteoids may be
distortion of the cortical present
plate. Central—whorled
Disfigurement of pattern of fibroblastic
face stroma with presence of
collagen fiber. Irregular
calcified masses seen in
later stage
Peripheral giant Connective tissue Small, exophytic, Epithelium Surgical excision
cell granuloma of the periosteum well circumscribed ulcerated with areas should be done
of jaw bone or pedunculated lesion, of hemorrhage,
from periodontal painless, firm lobulated proliferating fibroblasts,
ligament tissue dark red color, bleeding multinucleated giant
from surface cells, spindle shape
cells, intercellular edema
with inflammatory cell
infiltrate
Central giant cell Reactive lesion Small bony hard Fibrovascular Surgical excision
granuloma swelling, expansion of connective tissue should be carried
cortical plate, vital stroma, proliferating out
teeth, some lesion may spindle shaped stromal
cause perforation of cells and interlacing
cortical plate collagen fibers, multiple
multinucleated giant
cells which contain
5 to 20 nuclei, small foci
of osteoids of oven
bone is present
Myxoma It is origin from Rare lesion and Loose textured tissue Radical surgery is
primitive mesenchyme firm and nodular growth containing delicate recommended as it
of varying size reticulin fiber and is aggressive lesion
mucoid material, stellate
shaped cells
features
Lipoma Origin from Well defined, soft Proliferating mature fat Surgical excision
adipose tissue movable lump, painless, cells with loose areolar should be carried
yellow in color and tissue stroma, round out
smooth cells, vacuolated with
overlying surface centrally placed nuclei,
lobules of fat cells
separated by fibrous
tissue
Hemangioma Vascular tissue Cavernous Raised, multinodular, Capillary – small Injection of sclerosing
origin Capillary Portwine red, blue or purple endothelial line agent should be given
stain central lesion, blanches capillaries in the lesion,
on compression, cells are single layered,
compressibility test cells spindle shaped,
positive plump
Central type – Cavernous – large
painful expansile jaw irregularly shaped
swelling, affected bone dilated endothelial
pulsatile, loosening sinuses contain
of teeth and large aggregates of
anesthesia of skin erythrocytes, single
Appendices
and mucous layer of flattened

membrane endothelial cells line,
area of hemorrhage
Lymphangioma Proliferation of Painless nodule, Proliferating, thin May regress
lymphocytes may be diffuse walled, lymphatic spontaneously,
with lighter color, vessels, lined by persistent lesion
crepitant sound, plump endothelial should undergo surgical
may produce cells, multiple excision
macroglossia papillomatous
nodule on the
surface
Osteoma Neoplasms of Compact osteoma Nodular, Dense cortical Surgical excision
osseous tissue cancellous osteoma exophytic, bony bone with distinct
hard growth, lamellar pattern,
expansion of cortical bone
cortical plate, sclerotic and
Gardners avascular, reduce
syndrome present marrow spaces
with osteoma
899
900
features
Osteoblastoma Painful, small in Small area of Surgical excision
size, expansion osteoblastic
and distortion of activity followed
cortical plates by mature osteoid
calcified, nidus
consist of
interlacing
meshwork of bony
trabeculae,
vascular
connective tissue
stroma, numerous
osteoblasts are
present
Chondroma Cartilaginous Slow enhancing Well defined Surgical excision
tissue origin pain bony hard lobules of hyaline
swelling, cartilage, mature
expansion and chondrocytes, cells
distortion of the are round or oval
cortical plate in shape
Leiomyoma Neoplasm of Slow growing, Spindle shaped Surgical excision
smooth muscle cells painless, submucosal smooth muscle cells, done with
nodules, surface is cells arranged in fascicle surrounding
smooth, yellowish in or stream line normal tissue
color, firm, encapsulated fashion, cigar
shaped appearance
Rhabdomyoma Neoplasm of Slow growing, Sharply outline, Surgical excision is
striated muscles Well circumscribed, Unencapsulated mass of done
painless mass, round or oval striated
deep seated muscle cells, multiple
vacuoles in the cell
neoplasm, irregular
cross striation, cell
nuclei are vesicular
features
Neurilemmoma Derived from Slow enlarging, Proliferating spindle Surgical excision
(Schwannoma) Schwann cells, Well circumscribed, shaped neoplastic
neuroectodermal painless nodule, smooth Schwann cells with
origin firm, exophytic, tender elongated nuclei,
to palpation, central Antoni A tissue
lesion present, well (parallel rows of
demarcated bony palisading nuclei of
hard lesion Schwann cells)
Antoni B tissue
(disorderly arranged
cells and fibers band)
Verocay bodies
Neurofibroma Autosomal Submucosal mass, Numerous proliferating Surgical excision
dominant Multilobulated surface, spindle shaped cells should be done
hereditary expansion, pain, resembling fibroblast,
condition paresthesia, haphazard
Appendices
soft tissue lesion arrangement, delicate

present as freely collagen fiber, café au
movable nodule, lait spot revels basilar
flabby masses, café melanosis
au lait spot
Neuroectodermal Derived from Swelling, expansion, Pigmented cells and Surgical excision
tumor of infancy primitive neural distortion, facial nonpigmented cells, with through
crest asymmetry, surface open nucleus, curettage
is brown or black flattened or cubical
pigmentation, slow in shape with pale
growing nuclei, unpigmented
cells occur in cluster,
and surrounded by
pigmented cells
901
902
features
Pleomorphic Slow growing, Proliferation of Surgical excision
adenoma well delineated glandular, basophilic, should be carried
exophytic growth, epithelial cells in the out
surface smooth, form of diffuse sheets
lobulated, painless, or clusters, polygonal
soft and rubbery shape glandular
consistency epithelium cells
arranged in clumps or
interlacing strand,
squamous metaplasia,
myxoid and chondroid
areas are present
Monomorphic Proliferation of Basal cell adenoma Basal cell— Basal type— Surgical excision of
adenoma single epithelial Canalicular encapsulated, granular epithelial cells the lesion
cell type adenoma movable lesion less in the forms of oval
than 3 cm lesion shaped nests, outer layer
are firm is cubiodal and inner
Canalicular—small layer is uniform
painless, movable Canalicular—
encapsulated. Lesion anatomizing network of
covered by smooth cuboidal and columnar
intact epithelium cells which give
impression of multiple
interconnecting canals,
connective tissue stroma
is myxomatous
and composed
of eosinophilic
hypocellular mucoid
matrix
Warthins tumor Consist of Slow enlarging, Multiple cystic Simple surgical
(oncocytoma) oncocytes Well circumscribed, soft spaces lined by excision is done
painless swelling, well pseudostratified
encapsulated columnar epithelial
movable lesion, cells, cells are arranged
compressible and in double layer pattern,
doughy feeling cystic lumen filled
with homogeneous
features
Sq cell carcinoma Tobacco, betel nut, White or red Well differentiated Surgical treatment
(epidermoid alcohol, actinic variegated path, (resembles the cells and radiotherapy
carcinoma) radiation, herpes exophytic invasive of squamous epithelium, in some cases
simplex, ulcer, induration keratin pearls), should be given
immunosuppressant around periphery, moderately
and genetic factors painful due to differentiated (more
secondary infection, dysplastic little or no
pathological fracture can keratin and greater
occur in extensive cases number of mitotic cell
division), poorly
differentiated (no
keratin, no resemblance
to cells of stratified
squamous epithelium,
mitotic division rate is
high)
Basal cell Exposure to Slow growing, elevated Neoplastic proliferation Surgical excision
Appendices
carcinoma (rodent sunlight lesion which develops of baseloid epithelial or electrocautery

ulcer) central crust, with rolled cells in solid island, along with
border, it invades and cells are columnar in radiotherapy
destroys the adjacent shape, palisaded
tissue arrangement,
intercellular bridge
absent,
Verrucous carcinoma Tobacco chewing Slow growing Papillary surface Laser therapy
and snuff dipping exophytic papillary covered by thick
habits growth with white layer of parakeratin,
pebbly surface. Multiple acanthotic rete ridges,
rugae like folds with parakeratin plugging,
deep clefts, regional pushing margin,
lymph nodes enlarged connective tissue intense
and tender inflammatory
infiltration
903
904
features
Malignant Arises from Hutchinson’s Macular pigmented Excessive proliferation Radical surgery
melanoma melanocytes freckle type focal lesion which grow of neoplastic with prophylactic
superficial rapidly to results in large melanocytes, neck dissection
spreading type painful diffuse mass, extensive cellular
Invasive type surface ulceration is pleomorphism, cells
common, small satellite are round, polyhedral
lesion and multinucleated,
numerous mitotic
activity
Spindle cell Pain, ulceration, Spindle shape Surgical excisions
carcinoma swelling, fleshy epithelial cells, nuclear is the most
and polypoid hyperchromatism, effective treatment
growth pattern Cellular pleomorphism,
increased mitosis, tumor
giant cells, little or no
keratin formation,
inflammatory cell
infiltration
Fibrosarcoma Neoplasm of Fast enlarging, large, Proliferation of spindle Radical surgical
fibroblast painful, bulky, lobulated shaped, malignant excision and
fleshy mass, surface fibroblast cells. It has chemotherapy

ulcerated due to trauma tadpole like appearance, should be given
streaming fashion
arrangement, abnormal
mitotic activity
Malignant fibrous Arises from Enlarging, exophytic Proliferating polyhedral Wide surgical
histiocytoma undifferentiated lobulated and ulcerated, or oval shaped, excision with
mesenchymal cells fleshy appearance, malignant histiocytes, radiotherapy and
pain, hemorrhage, cart wheel or storiform chemotherapy
paresthesia, gross facial pattern, increased
disfigurement mitotic activity, cellular
pleomorphism, giant
cells are present
features
Liposarcoma Cell along the Rapidly growing, Cellular lesion contain Surgery and
adipose tissue line painful, submucosal foamy, fat containing radiotherapy
masses, lobulated malignant lipoblasts
lesion, firm in cells, signet appearance
consistency (vacuolated
cytoplasm) cells poorly
differentiated round
cells, irregularly shaped
giant cells
Hemangioendothelioma Mesenchymal Fast enlarging, Neoplastic proliferation Surgical exicision
tissue origin localized, painful, of malignant endothelial and radiotherapy
nodular swelling, cells. The cells are should be done
surface ulceration, pleomorphic, large
paresthesia, anesthesia, polyhedral or
bleeding upon slight slightly flattened, nuclei
trauma, expansile lesion are round, increased
abnormal mitosis
Kaposi’s sarcoma Arises from endothelial It can be endemic It can be presented Patch stage Treated by
cells of the blood Rarely can be in patch, plaque, (dilated, irregular, radiotherapy and
capillaries, triggering epidemic nodular form. blood vessels, chemotherapy
Appendices
factors Nodule can be lined by normal

are HIV infection, multiple endothelial cells),
immunosuppressant plaque stage
and environmental (dilated jagged
factors vascular channels
lined by spindle
type cells) nodular
stage (slit like
spacing containing
RBCs)
Ewing’s sarcoma Lesion arise from Moderate fever, Proliferating, packed Radiotherapy,
endothelial cells or leukocytosis, rapid round cells which have chemotherapy and
undifferentiated swelling severe pain, monotonous round surgery is
reticuloendothelial paresthesia, surface or oval nuclei, recommended
cells ulceration hyperchromatic cells
arranged in diffuse
pattern, cells are round,
increased mitotic
activity
905
906
features
Chondrosarcoma Neoplasm of Primary Painless facial Can be well Wide surgical
cartilage tissue, Secondary asymmetry, later differentiated like excision should be
may be caused by pain, tenderness, benign or poorly done
Paget’s disease anesthesia or paresthesia differentiated,
in the region, extensive spindle shaped
local tissue destruction, malignant cells,
expansion of bone, binuclear cells
nasal obstruction
Osteosarcoma Arises from bone, Medullary Fast enlarging Actively proliferating Combination
may arise from osteosarcoma, painful swelling spindle shaped, oval therapy is given in
pre-existing Periosteal causing expansion, angular malignant the form of
Paget’s diease, osteosarcoma, and distortion, osteoblast cells with radiotherapy and
fibrous dysplasia, Parosteal facial deformity, cellular stroma, chemotherapy
osteochondroma osteosarcoma, displacement of cellular pleomorphism,
and chronic Soft tissue teeth, numbness of increased mitosis,
osteomyelitis osteosarcoma lip, overlying skin multiple area of osteoid
inflamed, ulceration, bone within fibrous
hemorrhage, stroma
pathological
fracture
Non-Hodgkins Neoplasm of cells Fever, night sweats, Proliferation of Chemotherapy

lymphoma of lymphoid tissue malaise, Malignant lymphocytes, should be given
anorexia, weight loss, Cellular pleomorphism,
generalized cells small in size,
lymphadenopathy, Nodular pattern—
abdominal pain, fast tend to aggregate in
enlarging large cluster
exophytic growth, Diffuse pattern—
lymph nodes firm, monotonous
rubbery, overlying proliferating tumor
surface is red and cells within connective
inflamed tissue
features
Burkitt’s Cause by Epstein- Rapid painless, Proliferation of Chemotherapy
lymphoma Barr virus loosening of teeth, fast lymphocytes, syncytial should be given
enlarging large appearance (nucleus
expansile swelling, surrounded by
overlying mucosa is cytoplasm), mitotic
ulcerated, paraplegia, activity is abundant,
perforation of cortical starry sky appearance
plate (macrophages with
clear cytoplasm scatter
uniformly throughout
the tumor)
Hodgkin lymphoma Neoplasm of Persistent generalized Malignant lymphoid Chemotherapy
lymphocytes lymphadenopathy, cells, non-neoplastic should be given
lymph nodes are firm inflammatory cells,
rubbery, generalized Reed-Sternberg giant
weakness, pain low cells (mirror image
grade fever, edema nuclei), Owl eye
Appendices
of extremities, appearance. It can be of

hepatosplenomegaly, lymphocyte prominent,
oral lesion present as mixed cellularity,
submucosal swelling lymphocytes depletion
with ulceration and nodular sclerosis
Multiple myeloma Neoplasm of Severe deep bone Sheets of closely Chemotherapy
Plasma cell pain, gradual bone packed, round or oval
loss, increased cells, resemble plasma
susceptibility to cells, cartwheel or
infection, nausea, checkerboard pattern
vomiting, anemia, jaw (eccentrically placed
lesion fast enlarging, nuclei exhibits
painful swelling, chromatin), mitotic
egg shell cracking, figure may be high,
perforation of cortex binucleated or
multinucleated cells
907
908
features
Rhabdomyosarcoma Striated muscle Embryonal Rapidly growing Embryonal (small Surgery,
origin Alveolar lesion, swelling, round cells with radiotherapy and
Pleomorphic pain, extensive monotonous chemotherapy
damage, indurated, fixed hyperchromatic should be given
and ulcerated nuclei) Alveolar (round
cells pattern similar to
lung alveoli)
Pleomorphic
(primitive muscle
formation, prominent
nuclei)
Adenoid cystic Arises from Slow enlarging Small darkly Wide surgical
carcinoma glandular growth, surface staining, polygonal excision should be
(cylindroma) epithelium of ulceration, mass or cuboidal cells of carried out
salivary gland below the ear, pain uniform size, Swiss
is seen fixation and cheese pattern (double
induration of tumor layer of tumor cells
paresthesia may be seen arranged in duct like
pattern, containing
eosinophilic coagulum
at center), cribriform
pattern, neurotrophism
(spread via perineural or
intranueral spaces)
Mucoepidermoid Slow growing, It contain mucus Surgical excision
tumor painless swelling having secreting, epidermoid
cystic feeling, in some and intermediate types
cases rapid growth, pain of cells, it can be well
hemorrhage, ulceration, differentiated (no
paresthesia occurs. Bony cellular pleomorphism)
expansion, facial nerve Poorly differentiated
paralysis (cellular pleomorphism,
pushing front)
features
Leukoplakia Tobacco, alcohol, Homogeneous Homogeneous (white Hyperorthoker- Stoppage of habit,
candidiasis, dietary Non-homogeneous patch having smooth or atinization, cryosurgery, vitamin A,
deficiency, syphilis, Cryptogenic corrugated surface hyperparakeratinization, antioxidant therapy
viral, hormonal Speckled or with irregular margin) acanthosis, nuclear
imbalance, chronic nodular ulcerative Ulcerative hyperchromatism,
irritation, actinic (mixed red and white cellular pleomorphism,
radiation, galvanism lesion with small poikilocarynosis,
keratotic nodules) dyskeratosis, enlarged
Nodular (maximum risk nucleoli, dropshaped
for malignant rete pegs, basoloid
transformation) appearance, presence of
candidial hyphae
Carcinoma in situ Severe stage of White plaque, ulcerated, Hyperkeratosis, on Surgical excision
epithelial dysplasia eroded, or reddened surface of lesion, keratin should be carried
area, resemble pearl formation, loss out
leukoplakia or of orientation, loss
Appendices
erythroplakia of polarity, basement

membrane intact
Erythroplakia Heavy use of Homogeneous Small, extensive, Features of invasive Deep and wide
tobacco, alcohol Erythroleukoplakia red velvety lesion with Epidermoid carcinoma, surgical excision
Speckled well defined margin chronic inflammatory
cell infiltration,
reduction in
keratin production and
increase in vascularity
Leukoedema Normal variant Diffuse translucent, Thickening of No treatment
grayish, white area, epithelium with mild
filmy appearance, parakeratosis,
wrinkled appearance, enlarged spinous cells
when stretched mucosal with pyknotic nuclei,
condition disappear rete peg are broad,
909
910
features
Stomatitis nicotina Tobacco Red palatal mucosa, few Hyperparakeratosis, Complete
red and dot like areas acanthosis, ductal stoppage of oral
surrounded by white epithelium exhibits habits
keratotic ring, ulceration squamous metaplasia,
can be seen atrophic changes,
inflammatory cell
infiltration
Oral submucous Betel nut, red chillies, Burning sensation, Hyperkeratanized Intralesional
fibrosis nutritional vesicle inflammatory Atrophic epithelium, injection of steroid,
deficiency, reaction. flattening and shortening enzyme, systemic
immunological Xerostomia or of rete pegs, cellular steroid, injection of
factors, genetic excessive salivation, atypia, nuclear placental extract
factors stiffening of oral pleomorphism, increases
mucosa, leathery mitosis, basilar
feeling, trismus, hyperplasia, blood
blanched mucosa, vessels dilated and
difficulty in deglutition congested, perivascular
fibrosis
Lichen planus Psychological Reticular Wickham striae Hyperorthoker- Steroids, dapsone
stress, autoimmune Erosive present atinization, therapy
reaction Plaque Reticular – raised thin hyperparakeratinization,
Atrophic white radiating line, thickening of granular
Bullous non-elevated, cell layer, saw tooth

burning sensation appearance, liquefaction
erosive – mixture of degeneration, civatte
erythematous ulcerated bodies (round ovoid
and white amorphous eosinophilic
pseudomembranous bodies) are seen
area, pain and
burning sensation
plaque – raises or
flattened white area,
atrophic – smooth
poorly defined
erythematous area.
Bullous – large
vesicles are seen
features
Sialolithiasis Unknown stimuli Submandibular Stone is acellular, Can be removed
gland more involved, amorphous, outer by digital
intermittent pain margin aggregates manipulation,
which can be severe. microbial colonies, lithotripsy
Affected gland enlarged, ductal lining changed
stone can be palpated into stratified squamous
epithelium
Necrotizing Unknown etiology, Deep seated punched Absence of epithelium, Lesion heal
sialometaplasia infarction of tissue out ulceration on hard necrotic debris, spontaneously
or soft palate, presence coagulation of necrosis,
of gray granular lobules, distended cells,
numbness or burning basophilic nuclei,
pain accumulation of mucin
in zone of necrosis
Bacterial sialadenitis Streptococcus Acute Acute—sudden onset of Chronic—acinar atrophy Antibiotics therapy
pyogenes, chronic painful swelling, fever, of salivary gland with
Staphylococcus redness of subsequent fibrosis,
aureus overlying skin, trismus, dilatation of the ductal
difficulty in swallowing, system, hyperplasia of
Appendices
parotid papilla inflamed the ductal epithelium,

Chronic—recurrent periductal fibrosis,
tendered swelling, chronic inflammatory
duct orifice inflamed, cell infiltration
decreased salivary
flow
Mumps (endemic Caused by Parotid gland is Antiviral drugs
parotitis) paramyxovirus affected, eversion of ear
lobe, acute pain during
salivation, recurrent
exudation
Mikulicz’s disease Autoimmune Unilateral or bilateral Benign infiltration Steroid should be
disease, defective diffuse swelling. Soft of lymphocytes, given
cell-mediated swelling, movable, myoepithelial islands.
immunity fever, URI, xerostomia Obliteration of lumen of
duct due to proliferating
epithelial cells,
911
912
features
Sjögren’s syndrome Immune mediated Primary (sicca Xerostomia, Infiltration of Steroids, antibiotics,
chronic inflammatory syndrome) – no Xerophthalmia and lymphocytes in antifungal
response, presence other disease arthralgia. Severe intralobular ducts of
of serum antinuclear Secondary – with tiredness, disturbed taste salivary gland, atrophy
antibodies arthritis sensation, dry of salivary gland acini,
mucosa, red and hyperplasia of
atrophic tongue ductal epithelium,
mucosa, cobble stone myoepithelial
appearance (fissuring islands
and lobulation of the
surface)
Sialosis Hormonal Parotid gland Hypertrophy of serous Elimination of
disturbance, involved little acinar cells, secretory causative organism
malnutrition, liver pain, discomfort granules in cytoplasm
cirrhosis, chronic and lipomatosis may
alcoholism, diabetes occur
Ameloblastoma Arises from Peripheral type Slow growing, painless, Plexiform—continuous Enucleation should be
odontogenic damantinoma of ovoid, fusiform bony anastomosing strands, carried out
epithelium. It may long bone hard swelling, expansion fish net pattern,
be predispose by mural and distortion of columnar cell.
trauma, infection, ameloblastoma cortex, gross facial Follicular—discrete

previous inflammation asymmetry, egg follicle, island of fibrous
shell cracking, continuous strand,
pathological fracture microcyst formation.
Acanthomatous—
keratin pearls, squamous
metaplasia.
Granular cell—stellate
reticulum like cells
swollen with coarse
eosinophilic granules.
Basal cell—cuboidal
shaped in narrow strand
features
Adenomatoid Reduce enamel Maxillary anterior Odontogenic cells Surgical
odontogenic epithelium region, slow enlarging, in duct like pattern enucleation should
tumors bony hard swelling, within stroma giving be done
elevation of upper lip, adenomatoid
expansion of jaw appearance, small
foci of calcification
is seen, solid nests or
rosette patterns
Calcifying Cells of stratum Mandible > maxilla, Closely packed, Surgical
epithelial odontogenic intermedium Slow enlarging painless polyhedral cells, enucleation should be
tumor (pindborg tumor) swelling, expansion, cribriform arrangement, done
distortion of cortical hyalinized stroma
plate, displacement of ,oval shaped nuclei,
teeth hyperchromatic nuclei,
prominent intercellular
bridges and distinct cell
boundaries, Lisegang
ring (calcified masses),
clear cells
Squamous Remnants of Maxillary incisor canine Irregularly shaped, Surgical
Appendices
odontogenic tumor dental lamina, cells area, painless swelling, islands of well enucleation should
rests of Malassez, mobility of teeth, local differentiated squamous be done
and basal layer of tenderness epithelium in fibrous
oral epithelium connective tissue
stroma, round or
oval shaped islands,
microcyst
formation, calcification
is seen
Ameloblastic Mandibular Epithelial and Surgical excision
fibroma premolar-molar area, mesenchymal cells are should be carried
slow growing, painless, present, multiple sharply out
distortion of defined strands or
cortical plate, islands bordered by
displacement of teeth, tall columnar cells, cell
facial asymmetry free zone of hyaline
connective tissue in
epithelial component
913
914
features
Odontoma Occur after Complex— Complex—anterior Presence of Surgical
histodifferentiation disorganized dental maxilla. encapsulated mass of enucleation should be
but before tissue. Compound—posterior denticles, complex done
morphodifferentiation Compound— mandible, small odontome presented as
Discrete tooth like asymptomatic lesion, irregularly
structure expansion of cortex, arranged dental tissue
displaced teeth
Odontogenic fibroma Derived from Peripheral type— Peripheral—slow Peripheral—mass of Surgical excision
connective tissue extra-osseous growing, exophytic, well dense connective tissue should be carried
of odontogenic Central—arises circumscribed with spindle shaped out
epithelium within the jawbone growth, firm in fibroblast, surface
consistency, painless, epithelium slender rete
interdental lesion cause pegs project in CT, clear
separation of teeth cells
Central—slow growing, Central—thin strands
painless swelling, odontogenic, clear cells,
displacement of teeth, areas of spherical or
cortical expansion diffuse calcification and
contain giant cells
Odontogenic myxoma Derived from dental Slow growing, Widely separated Surgical excision should
papilla or follicular painless swelling, undifferentiated spindle be carried out
mesenchymal displacement of or angular or stellate
regional teeth, shaped cells. Focal areas
expansion of bone of delicate immature

collagen fibrilar strands,
blood vessels exhibits
hyalinization at
periphery
Periapical cemental Mandibular Initial stage - cemental No treatment is
dysplasia anterior teeth, females tissue at apex of required
are affected, involved
asymptomatic small and and replaced by fibrous
multiple, teeth are vital, connective tissue,
radiologically Cementoblastic stage—
detected small amorphous masses
Immature cementum
Mature stage—entire
fibrous tissue replaced
by mature cemental
tissue
features
Familial gigantiform Hamartomatous Slow growing painless Loose vascular tissue Surgical osseous
cementoma malformation of expansile jaw swelling, stroma, delicate collagen recontouring
cementum forming multiple lesion seen, fiber, monomorphic
tissue autosomal facial asymmetry can fibroblasts, acellular
dominant trait also occur cementum, ovoid
calcification
Cementoblastoma Arises from Mandible > maxilla, Large mass of Surgical excision
cementoblast slow growing bony hard amorphous cemental should be carried
swelling, expansion tissue with presence of out
of cortical plate, reversel line, soft and
intermittent pain, dull vascular connective
sound when tooth tissue stroma,
percussed cementoblasts or
cementoclast are
present, multinucleated
cells are present
Odontogenic carcinoma Intraosseous lesion Large swelling Islands or strands of Radical surgical
Appendices
with expansion of clear cells, cells are excision should be

cortical plate, glycogen rich, non-clear carried out
mobility of teeth cells resembles dental
lamina, epithelial tissue
surrounded by zone of
myxomatous tissue
Odontogenic keratocyst Arises from Islands or strands Cystic cavity lined Surgical enucleation or
remnant of the of clear cells, cells by keratinized stratified marsupialization
dental lamina, are glycogen rich, non- epithelium, lining of the cyst can be
developing tooth clear cells resembles epithelium is flat, carried out
germ, basal layer dental lamina, epithelial para-keratinization,
of oral epithelium tissue surrounded by daughter cyst or satellite
zone of myxomatous cyst present (multiple
tissue small micro cyst)
present
915
916
features
Dentigerous cyst Cells of reduced Mandible > maxilla, Cystic cavity line of Marsupialization
enamel epithelium asymptomatic, slowly odontogenic epithelium, should be carried
enlarging bony hard stroma contain young out
swelling occur, fibroblast cells separated
expansion of by ground substance
bone, Crepitus rich in collagen bundles,
sensation, facial cystic epithelium is flat,
asymmetry, paresthesia low columnar,
and pathological fracture some cases bud like
may occur proliferation called
mural proliferation is
seen
Radicular cyst Inflammatory Non-vital tooth, Cystic cavity line by Root canal
origin asymptomatic and non-keratinized treatment with
detected on radiograph, Stratified squamous apical curettage
expansion occur in epithelium, localized can be done. In
larger lesion, pain if area of increased cell larger lesion
secondarily infected proliferation, cholesterol excision should be done
cleft (small ribbon
shaped cleft like space)
arcading pattern can be
seen. Inflammatory cell

infiltration
Eruption cyst Accumulation of Small fluctuant swelling Ghost cells within No treatment is
fluid in follicular on alveolar ridge, the lumen of cyst needed
space eruption hematoma
occur due to mastication
Lateral periodontal cyst Asymptomatic, small Small cystic cavity lined Surgical excision should
painless soft tissue by connective tissue be carried out
swelling can occur, wall, on inner aspect by
mucosa is pale in color, nonkeratinized stratified
vital tooth Squamous epithelium.
Thickening of lining
epithelium, cells have
pyknotic nuclei
features
Gingival cyst of Cyst of dental lamina Multiple asymptomatic, Small keratin fill cystic Not needed
newborn (along alveolar ridge, small discrete white cavity lined by flattened
odontogenic origin) nodule. It undergoes epithelium
Epstein pearls (along spontaneous regression
midpalatine raphe)
Bohn’s nodule (at
junction of hard and soft
palate)
Gingival cyst of adult Arises from gingival Firm compressible, Cystic cavity lined by a Surgical excision should
soft tissue, cell rest of fluid filled swelling, thin epithelium made up be carried out
dental lamina well circumscribed, of flat or cuboidal cells.
smooth surface and pyknotic nuclei with
bluish or normal in color perinuclear cytoplastic
vacuoles
Sialo-odontogenic cyst Remnant of dental Slow enlarging Lined by thin squamous Surgical enucleation
lamina asymptomatic growth epithelium with focal should be carried out
in mandibular anterior area of thickening,
region microcyst is also found,
Appendices
organization of
glandular elements may
results in
acinar like cluster
Botyroid Well defined, painless Multiple cystic Surgical excision should
odontogenic cyst expansile jaw lesion cavities separated from be carried out
one another by fibrous
septa. Lined by cuboidal
or squamous epithelium
Calcifying Bony hard extensive Cystic cavity lined by Surgical excisions
epithelial odontogenic swelling of the jaw, odontogenic keratinized should be carried out
cyst expansion and distortion epithelium, cells are
of cortical pates, tooth columnar or cuboidal,
vital, perforation of the ghost cells are
cortex eosinophilic, satellite
microcyst
917
918
features
Paradental cyst Inflammatory in Seen in mandibular third Cystic cavity lined by Surgical excision
origin, cell rests of molar and tooth has hyperplastic, non- should be carried
Malassez or reduced history of pericoronitis keratinized squamous out
enamel epithelium epithelium, intense
inflammatory reaction
Globulomaxillary cyst Proliferation of Asymptomatic, pain Cystic cavity lined by Surgical removal
epithelium along the when secondary stratified or pseudo- should be done
line of fusion between infected, small stratified ciliated
maxilla and premaxilla swelling in canine columnar epithelium
region and tooth is chronic inflammatory
vital cell infiltration
Nasolabial cyst Lower part of Small painless swelling Cystic lumen supported Surgical excision
(Kelstadt’s cyst) embryonic of upper lip, by connective tissue should be done
nasolacrimal duct obliteration of wall, lined by pseudo-
nasolabial fold, can stratified ciliated
project into floor of columnar epithelium,
nose infolding of cystic lining
Nasopalatine duct cyst Proliferation and Small painful swelling Cystic lined ciliated Surgical excision
cystic degeneration of in midline of anterior columnar or non-
epithelial remnants after part of hard palate, keratinized stratified
closure of embryonic pressure sensation Squamous epithelium,

nasopalatine duct on floor of the nose, mucus secretory cells,
displacement of root presence of pigment,
of central incisor, salty presence of
discharged, neurovascular bundles
fluctuation can be done
Traumatic bone cyst Pseudocyst Painful bony hard Cystic cavity surrounded Surgical exploration
swelling, paresthesia of by loose vascular should
lip, expansion of connective tissue wall, be done
cortical plate, no epithelial lining,
displacement of teeth, CT stroma is made of
vital teeth fibrous tissue
features
Aneurysmal bone cyst Enlarging diffuse, Multiple blood filled Surgical curettage
firm swelling, facial spaces lined by spindle should be done
asymmetry, swelling shaped cells or flat
pulsatile, egg shell endothelial cells,
cracking, pathological epithelial absent, cystic
fracture, profuse spaces separated by
bleeding, paresthesia, loose connective tissue
difficulty in opening wall, multiple
mouth multinucleated
giant cells
Mucocele Accumulation of saliva Mucous retention Lower lip involved, Mucous retention— Surgical excision should
due to obstruction cyst, mucous small raised lesion and small cystic cavity filled be carried out
extravasations cyst bluish swelling, with mucous lined by
round to oval shaped flattened epithelial cells
smooth fluctuant Mucous extravasation—
cystic cavity surrounded
by compressed
connective tissue wall.
Appendices
Mucous stroma
Ranula Obstruction of the Soft fluctuant Large mucous Surgical excision
duct, compression unilateral swelling in filled area surrounded should be carried
of duct, perforation of floor of mouth, bluish by connective tissue out
duct translucent wall, mucous filled area
appearance like frog
belly
Dermoid cyst Remnant of Painless swelling Cystic cavity lined Surgical excision
embryonic skin with doughy by orthrokeratinized should be carried
consistency, stratified squamous out
elevation of tongue, epithelium exhibiting
midline location hair follicle, sebaceous
gland, desquamated
keratin, cyst capsule
consist of narrow
zone of compressed
connective tissue
919
920
features
Focal reversible pulpitis Chronic carious lesion, Tooth sensitive to Acute inflammatory Elimination of
stimuli of short duration, thermal changes, reaction in odontoblastic causative factor,
chemical irritation, pain of short regions, dilatation of pulp capping
severe attrition or duration, vitality pulpal blood vessels, should be done
abrasion test is positive edema in pulp with
infiltration by
polymorphonuclear
leukocytes, thrombosis
of pulpal blood
vessels
Acute pulpitis Caries reaching Tooth is extremely Severe edema with Direct pulp
pulp, pulp exposure sensitive, lacinating vasodilatation, capping, drainages of
by cavity preparation, pain, percussion test dense infiltration of pus, root canal treatment
trauma to teeth, positive, history of polymorphonuclear should be done
chemical irritation, night pain, pain leukocytes, destruction
cracked tooth syndrome subsided after drainage of odontoblasts cells
established at pulp dentin border,
microabscess formation
Chronic pulpitis Same as acute Intermittent dull or Cellular infiltration by Extraction of tooth or
pulpitis throbbing pain, tooth lymphocytes, plasma root canal treatment
is less sensitive to pain cells and macrophages, should be done
as compared to acute blood capillaries are
pulpitis prominent, fibroblastic

activity, formation of
collagen bundles
Pulp polyp (chronic Intense proliferation of Small pinkish red, Numerous proliferating Root canal
hyperplastic pulpitis) pulpal connective lobulated mass fibroblasts and young treatment
tissue due to low grade protruding from pulp blood capillaries,
infection chamber. Large open inflammatory cell
carious cavity, lesion infiltration, by plasma
bleeds profusely upon cells and lymphocytes,
provocation, tooth is stratified squamous
painless epithelial lining
Acute apical Results of Moderate pain, Restoration of
periodontitis extension of pulpal sensitivity, extrusion of tooth with proper
inflammation tooth, severe pain, antibiotics coverage
tenderness positive
features
Periapical Response to infection, Percussion test Granulation tissue mass Root canal
granuloma occlusal trauma positive, mild pain, consist of proliferating treatment with
discomfort fibroblasts, endothelial apicoectomy
cells, immature blood
capillaries, chronic
inflammatory cells,
cholesterol cleft, foam
cells
Osteomyelitis Infection of dental Acute suppurative, Acute suppurative— Acute suppurative– Antibiotics,
pulp, infected Acute subperiosteal, severe pain, diffuse bone marrow undergo sequestrectomy,
granuloma, infected Chronic large swelling, liquefaction, thrombosis analgesic, hyperbaric
cyst, compound suppurative, excessive salivation, bad of blood vessels. oxygen therapy should
fracture, postradiation Chronic diffuse breath, multiple sinus, Acute inflammatory cell be given
secondary infection sclerosing, paresthesia of lip. infiltration, Brodie’s
Chronic focal Chronic suppurative— abscess can be seen.
sclerosing pain is mild and dull, Chronic suppurative –
jaw swelling, sinus accumulation of
formation. exudates and pus,
Appendices
Focal sclerosing— lymphocytes, plasma

asymptomatic, tooth cells, macrophages,
with large carious osteoblastic and
lesion. osteoclastic activity
Diffuse sclerosing— producing reversal line.
asymptomatic, vague Focal sclerosing—
pain, foul taste, acute presence of dense mass,
exacerbation may without marrow tissue,
occur producing fibrotic marrow.
mild pain and fistula Diffuse sclerosing—
tract formation formation of dense
irregular bone with
hypocellular fibrous
stroma, reversal line and
resting line is seen
921
922
features
Garre’s Low-grade Carious non-vital Multiple osteoid, Elimination of
osteomyelitis infection or trauma tooth (lower first primitive bony tissue, causative agents
molar), slight osteoblastic activity is with extraction of
tenderness or prominent, marrow involved tooth
vague pain can be spaces contain patchy
present area of chronic
inflammatory cell
infiltration
Cellulitis Virulent bacteria Large diffuse, painful Fibrin and serum Antibiotics and
like Streptococcus swelling over the face fluid in tissue, removal of
pyogenes and or neck with facial separation of periosteum primary factors
Bacteroides, osteomyelitis, and muscle, acute
osteomyelitis, infected postextraction inflammatory cell
infected postextraction wound infiltration
wound
Ludwig’s angina Causative organism Large diffuse board Acute High dose of
Hemolytic like swelling in floor inflammatory cell antibiotics
Streptococci periapical, with brawny induration, infiltration
pericoronal or elevation of tongue,
periodontal infection, no pitting on pressure,
gunshot injury, speech difficulty
osteomyelitis
Hand-Schuller- Replacement of Exophthalmos, Multiple large Surgery and
Christian disease marrow by diabetes insipidus, vacuolated foam cells, chemotherapy
macrophages skins rashes, otitis small nonvacuolated should be given
media, facial cells
asymmetry, ulceration
and necrosis of oral
mucosa, loosing of
teeth and halitosis
Eosinophilic Fever malaise, headache Numerous proliferating Surgical curettage
granuloma and anorexia, localized histiocytes, in diffuse
pain, tenderness, sheets, eosinophils is
gingival soft tissue seen, multinucleated
swelling giant cell present
features
Letterer-Siwe Hepatosplenomegaly, Marked proliferation of Poor prognosis
disease lymphadenopathy, non-lipidized histiocytes
ecchymosis of skin,
mucosal ulceration,
gingival hyperplasia
Hyperparathyroidism Adenoma Primary Fatigue, weakness, Osteoclastic resorption Excision of
hyperplasia of Secondary polyuria, thirst, of bony trabeculae, areas parathyroid tumor
parathyroid gland depression, loss of of excessive hemorrhage
memory, peptic ulcer, and hemosiderin
bone pain, pigmentation, brown
loosening of teeth and tumor (tissue takes
fracture of jaw bone brown color), multiple
multinucleated giant
cells
Paget’s disease Inflammatory Deep aching bone with Osteoclastic bone Administration of
Appendices
reaction, circulatory bilateral symmetrical resorption, bone calcitonin, surgery in

disturbance, defective swelling, deformity replaced by highly severe cases
connective tissue of bone in stress vascularized
mechanism, bearing area, headache, cellular connective
autoimmune disorders deafness, blindness tissue, bone marrow
occur due to narrowing replaced by fibrous
of skull stroma, reversal and
foramina, bowing of leg, resting line, mosaic
waddling gait, diastema, pattern seen in the bone,
loosening of teeth, chronic inflammatory
difficulty in lip closure, cells and dilated blood
pathological fracture of capillaries
bone
923
924
features
Fibrous dysplasia Can be developmental Monostotic Slow enlarging, Highly cellular, Growth ceases
or caused by liver Polyostotic (Jaffe’s painless, unilateral proliferating, well after puberty,
damage, glandular type and Albright’s swelling, facial vascularized fibrilar surgical
dysfunctions, trauma syndrome) asymmetry, expansion connective tissue, recontouring
and gradual distortion of spindle shaped
cortical plate, fibroblasts arranged
displacement of teeth, in whorled pattern.
malocclusion, in Chinese latter
Albright’s syndrome pattern seen, spheroidal
café-au lait spot, areas of calcification,
and other endocrine lesion blends with
abnormalities can be surrounding normal
seen bone. Remodeling of
woven bone to lamellar
bone
Cherubism It can occur due to Bilateral painless Whorled pattern cellular Self limiting
latent Symmetric swelling, connective tissue disease
hyperparathyroidism, giving rise to chubby stroma, proliferating
hormone dependent face, eyes raised to fibroblasts,
neoplasm, trauma, heaven look, increases multinucleated giant
familial pattern cheek fullness, widening cells, eosinophilic

of alveolar ridge, perivascular cuffing
lymphadenopathy, of collagen fibers,
premature exfoliation extravasated RBC,
of teeth hemosiderin pigments
Osteogenesis Defective matrix Neonatal lethal Multiple fracture Thinning of cortex, No treatment is
imperfecta formation, cross type of bone, generalized immature woven possible
linking of adjacent Sever non-lethal body deformity, dwarfed bone, short, thin,
molecule type moderate and stature, blue sclera, fragile bony trabeculae,
deforming type deafness, defective heart increased osteoblasts,
Mild and non- valves osteoclasts
deforming type
features
Cleidocranial dysplasia Hereditary autosomal Absence of clavicle, No treatment is
dominant trait shoulder can meet in possible
midline, nose is
flat, wide, hypoplastic
maxilla, delayed
closure of fontanels,
high and
narrow arched
palate
Osteopetrosis (marble Genetic defect Autosomal Increased tendency of Dense and sclerotic No treatment is
bone disease) dominant development bone with required
Autosomal severe osteomyelitis, compensatory
recessive anemia, remodeling. Medullary
thrombocytopenia, cavity is small and little
leukopenia, deafness, amount of marrow
blindness, facial tissue. Osteoblasts are
paralysis, frontal present
bossing, long bones
Appendices
shortened, spontaneous
hematoma
Infantile cortical Rapidly bilateral Edema and thickening No treatment is
hyperostosis symmetrical of periosteum with required
mandibular swelling, apposition of many thin
deep seated tendered bony trabeculae parallel
soft tissue swelling, to each other
dysphagia, pseudo-
paralysis anemia occurs.
Massive osteolysis Replacement of bone by Progress rapidly, pain Foci of resorption, bone Radiation therapy can
fibrous tissue in bone, pathological is replaced by fibro- be given
fracture occur, facial vascular connective
asymmetry tissue showing chronic
inflammatory cell
infiltration
925
926
features
Osteoarthritis Degenerative disease, Clicking sounds Vertical or horizontal Analgesics, anti-
aging process or trauma while opening and crack on the articular inflammatory drugs
closing movements. cartilage, cartilage less should be given
Limitation of elastic, elevation of disc
movement. Pain surface called lipping,
degeneration of
chondrocytes
Psoriasis Genetically Painless, dry white Atrophy with Not specific
determined scaly patches, patch are Hyperparakeratosis,
well circumscribed, absence of granular
erythematous, sterile cell layer, clubbing of
pustule, Auspitz’s sign rete pegs, intraepithelial
(tiny bleeding microabscess
point) oral cavity formation (monro
lesion are well abscess), increased
defined, grayish white mitotic activity, mild
or yellowish patches lymphocyte cell
infiltration
Erythema Can be precipitated by Rapidly developing Acanthosis, intra or Topical and
multiforme tuberculosis, herpes Erythematous macules, intercellular edema and systemic steroid
simplex, papules, bulls eye or necrosis of the therapy is given
infectious target lesion (concentric epithelium, sub-
mononucleosis, erythematous rings epithelial
hyperimmune reaction separated by ring of near connective tissue shows

normal color on edema and perivascular
skin), vesicle are eroded infiltration of
or ulcerated bleed lymphocytes and
profusely, foul macrophages
smell in mouth
White sponge Hereditary Asymptomatic white Mild to moderate Self regression
nevus folded areas in the hyperparakeratosis, Self regressing
mucosa, oral lesion are acanthosis,
soft and spongy and Intercellular edema,
peculiar opalescent hue, vacuolated cells in the
surface show area of spinous cell layer having
desquamation pyknotic nuclei, mild
inflammatory cell
infiltration
features
Pemphigus Autoimmune P. vulgaris P. vulgaris – rapidly Suprabasilar split Steroid
mechanism P. vegetans Developing vesicle, occur due to formation antibiotics to
P. foliaceous rupture bullae are of vesicle or bullae, control infection
P. erythematosus painful, bleeds profusely basal layer of row of
Nikolsky’s sign positive, tomb stones, loss of
orally bleb like blister intercellular bridges,
are seen disruption of prickle
P. vegetans – cells, Tzank cells are
papillomatous found, acanthosis in
hyperplasia following spinous cell layer
rupture, cerebriform
tongue
Cicatricial Mild erosion or Extracellular edema, Systemic steroid
pemphigoid desquamation gingival vacuolation in basement therapy
tissue, erythematous membrane zone,
Appendices
lesion, mucosal bullae formation of

are tense, tough, after subepithelial vesicles
rupture painful eroded or bullae, inflammatory
or ulcerated area cell infiltration present,
Nikolsky’s sign is blood vessels dilated
positive. Heal by
scar formation, corneal
ulceration,
scaring results in
adhesion in conjunctiva
Bullous Ulceration and heal Same as cicatricial Systemic steroid
pemphigoid without scarring, skin type therapy
lesion as red
eczematous plaque
927
Etiology Types Clinical/Radiological Pathological features Management 928
features
Epidermolysis Acquired type is EB simplex Multiple vesicle and Destruction of basal Systemic steroid
bullosa caused by multiple EB dystrophic bullae on pressure area, or suprabasal layers therapy
myeloma, diabetes Junctional EB after rupture raw painful of the oral epithelium,
mellitus, TB, EB acquista ulcers which heal with resulting in formation of
amyloidosis scarring, nail sheds, vesicle or bullae
decreased mouth
opening due to scarring,
delayed eruption and
increased periodontal
disease
Lupus Autoantibodies Systemic LE SLE—skin lesion in SLE—atrophy with Systemic steroid
erythematosus and immune Discoid LE butterfly configuration hyperkeratinization, therapy should be
complex over malar region, liquefactive given
itching, burning, degeneration, edema of
hyperpigmentation, subepithelial connective
loss of hair, xerostomia, tissue, lymphocyte
mucosal petechiae infiltration fibrinoid
DLE—elevated red degeneration

purple macule, DLE—
covered by yellow or hyperparakeratinization,
gray scale, butterfly Atrophic epithelium,
distribution, carpet keratin plugging,
track appearance, acanthosis,
enlarge at periphery pseudoepitheliomatous
pain and burning of oral hyperplasia, perivascular
mucosa lymphocytes infiltration
Tabular presentation of tooth tissue loss
Term Definition Clinical Appearance
Attrition Loss by wear of surface of tooth or restoration caused by tooth-to-tooth Matching wear on occluding surfaces.
contact during mastication or parafunction Shiny facets on amalgam contact.
Enamel and dentin wear at the same rate.
Possible fracture of cusps or restorations
Abrasion Loss by wear of dental tissue caused by abrasion by foreign substance Usually located at cervical areas of teeth.
(e.g. toothbrush, dentifrice) Lesions are more wide than deep.
Premolars and cuspids are commonly affected.
Broad concavities within smooth surface enamel.
Erosion Progressive loss of hard dental tissue by Cupping of occlusal surfaces, (incisal grooving) with dentin
chemical processes not involving exposure.
bacterial action Increased incisal translucency, wear on non-occluding
Appendices
surfaces and raised amalgam restorations.

Clean, non-tarnished appearance of amalgams.
Loss of surface characteristics of enamel in young children.
Preservation of enamel “cuff” in gingival crevice is common.
Hypersensitivity and pulp exposure in deciduous teeth.
Abfraction Loss of tooth surface at the cervical areas Affects buccal/labial cervical areas of teeth.
of teeth caused by tensile and • Deep, narrow V-shaped notch.
compressive forces during tooth flexure • Commonly affects single tooth with excursive interferences
or eccentric occlusal loads.
929
930
Important bacterial infections
Lesion and causative Clinical features Patognomic/Characteristic oral Diagnosis
organism Diagnosis features
Scarlet fever Tonsillitis, Pharyngitis, fever, vomiting, Stomatitis scarletina Culture
B-hemolytic skin rash, bright scarlet on 2nd or 3rd Tonsillitis with grayish exudates Dick test
streptococci day due to injury to endothelium and “Strawberry tongue” and “raspberry
dilatation of blood capillaries tongue”
Diphtheria Skin and mucous membrane lesions. Leathery, grayish white Staining by Alberts stain, Ponder’s stain
Corynebacterium Mucous membrane lesions are found on “pseudomembrane”. It is formed by dead and Neisser’s stain. Culture on Loeffler’s
diphtheriae larynx, pharynx and tonsils.,conjunctiva. epithelial cells, coagulated fibrin and serum. Tellurite agar is selective media.
Hoarseness of voice, respiratory purulent exudates. “Bull neck” Swelling Eleck’s test Schick test
stridor and dyspnea may lead to airway of neck due to striking cervical
obstruction in children lymphadenopathy.
Tuberculosis Persistent cough with or without Oral tuberculous ulcers, more commonly Staining by Ziehl-Neelsen stain Culture
Mycobacterium tuberculosis hemoptysis, gradual weight loss, found on tongue, are irregular superficial by Lowenstein-Jensen
Acid fast bacilli cervical, axillary and mediastinal or deep painful ulcers which tend to media, Loeffler’s serum slope.
lymphadenopathy. Tuberculous increase in size. Tuberculoma- Tuberculin test (Mantoux test)
lymphadenitis (Scrofula), painful Tuberculous periapical granuloma
lymph nodes, with perforations and
crustations, Cold abscess. Lupus vulgaris
is primary tuberculosis of skin especially
of face leading to formation of papular
nodules which may ulcerate. Miliary
tuberculosis occurs due to lymphatic
dissemination
Leprosy It affects skin, peripheral nerves upper Lepromatous nodules tongue lips and Slit skin smears stained with Z-N stain.
Mycobacterium leprae respiratory tract. Hypopigmented patches hard palate. Gingival hyperplasia with Lepromin test
with partial or complete loss of loosening of tooth
sensation. Two types tuberculoid
(Skin) and lepromatous (Nerves)
type-It produces disfigurement like
loss of fingers, toes, (claw toe) Nasal
depression. May cause sudden death.
Actinomycosis Cervicofacial is most common. It Involvement of maxilla and mandible Grams staining, culture
Actinomycosis israeli, involves face, neck, tongue and may lead to osteomyelitis. Pus contains of sulfur granules
naeslundi, mandible. Organisms may enter sulfur granules. When stained with gram
viscosus, odontolyticus and oral mucosa producing swelling and stain they show “sun ray appearance”.
proprionica induration of the tissues. It produces
abscess which opens onto skin surface.
Other areas involved are pulmonary and
abdominal cavity
Lesion and causative Clinical features Patognomic/Characteristic oral Diagnosis
organism Diagnosis features
Tetanus It enters the wound and produce Lock jaw-Spasm of masseter muscle Grams staining Culture
Clostridium tetani toxins. Tetanospasmin in anaerobic leads to trismus. Dysphagia, laryngeal in Robertsons’s cooked meat medium
conditions. It affects the synapse of spasms may lead to asphyxia. Risus and blood agar. Direct
motor interneurons causing sever muscle sardonicus or Grimace-sustained immunofluorescence
spasms. Opisthotonus-Arched back contraction of facial muscles.
due to contraction of back muscles.
Syphilis Primary- (3 day to 3 months) Chancre. A Primary-Chancre on lips, palate, Direct examination by dark field
Treponema pallidum lesion that develops at site of inoculation. gingival and tonsils. Secondary-Mucous microscopy. Culture not possible
Secondary- (6 weeks after primary) patches (Snail track ulcers)-Multiple Wasserman’s test, VDRL test, Kahn
Skin-Macular popular painless lesions grayish white plaques overlying over an test, Fluorescent treponemal antibody
Painless macules and papules Tertiary- ulcerated surface. Seen on tongue, absorption test and T. pallidum
Gumma is focal gingival and buccal mucosa. hemagglutination assay.
Appendices
granulomatous lesion with central Tertiary syphilis-Gumma of tongue

necrosis. CVS and CNS involvement is and palate and produce a deep painless
seen ulcer and palatal perforation. Syphilitic
Congenital-Transmission of glossitis
infection to child by mother Frontal Congenital: Short maxilla high arched
bossing, saddle nose, irregular thickening palate, Hutchinson’s triad-Hypoplasia of
of supraclavicular joint incisor and molar teeth (Mulberry molar,
(Hegoumenaki’s Sign) Saber shin Moon’s molar, Fournier teeth, Screw
driver shaped incisors) Eighth nerve
deafness, and interstitial keratitis of eye
931
932
Important viral infections
Infection Incubation Clinical features Oral manifestations
Herpes simplex 2–26 days Herpetic eczema, herpetic conjunctivits, herpetic Herpetic stomatitis herpes labialis, vesicular lesions
Primary and meningoencephalitis and disseminated herpes simplex of rupture and form shallow ragged extremely painful ulcer
secondary the newborn. Herpetic whitlow. covered by gray membrane surrounded by erythematous
Prodromal symptoms include fever headache. Bodyache halo. Heal spontaneously within 7–14 days without scar.
Herpangina 2–10 days Sore throat cough, low-grade fever, rhinorrhea Tonsillar small vesicles that rupture to form crops of
Coxsackie group A virus ulcers.
Causes dysphagia
Measles 8–12 days Acute contagious dermatropic infection affecting Koplick’s spots: These occur 2–3
children. Fever, cough, conjunctivitis, days of cutaneous rashes. They are small irregular spots
photophobia, lacrymation and eruptive lesions of skin bluish white specks surrounded by bright margins.
and mucous membranes. Lesions are tiny red macules or Palatal pharyngeal petechiae, gingival ulceration
papules which coalesce to form large lesions congestion of throat may occur.
Chickenpox 2 weeks It is a primary varicella zoster infection. It is acute, Small blister like lesions on buccal mucosa tongue
Varicella zoster extremely contagious disease occurring in children. It gingival and palate. Lesions rupture to form eroded
is characterized by exanthematous vesicular rash with ulcers.
headache, fever anorexia.
Lesions occur on trunk face and extremities. Lesion
rupture to form a superficial crust and heal by
desquamation.
Varicella Zoster Reactivation Extremely painful lesion due to inflammation of dorsal Extremely painful, unilateral
of root ganglia. Fever pain tenderness vesicles which form ulcers are
chickenpox along the course of involved sensory nerves. It found on buccal mucosa tongue
is called is characteristically dermatomic and unilateral in uvula and pharynx and larynx.
zoster distribution.
Mumps 14–18 days It is an acute contagious viral infection of children. Duct of parotid gland becomes puffy and red.
epidemic parotitis Unilateral and bilateral swellings of the salivary glands
usually parotid glands. Swellings are usually rubbery in
consistency producing pain on mastication. Fever, pain
below the ear.
Comparison of different forms of gingivitis
Form of gingivitis Clinical features Contributory factors Significance
Marginal Erythema, edema, tenderness None beyond the poor oral hygiene and Superficial, resolve quickly with plaque
gingivitis limited to the marginal gingiva relatively short duration of the process formation with no permanent defect
and interdental papillae, younger
patient
Hyperplastic Bulbous, edematous enlargement Caused by poor oral hygiene, hormonal or Deeper, hyperplastic response resolve
gingivitis of gingiva, may be focal or generalized; medication with plaque removal but enlargement
‘boggy’ and pocket persist
depth is increase
Chronic gingivitis Pale, fibrotic appearance of gingiva, loss of Chronic inflammation produce a Inflammation of the crevicular pockets
stippling, edema, recurring cycle of active inflammation and can be controlled by plaque removal if
exudate and hemorrhage from the sulcular reparative fibrosis pockets can be kept clean
surface on probing, increase pocket depth
Desquamative Erythematous and atrophic appearance Autoimmune conditions such as Topical corticosteroid application
gingivitis without enlargement; lichen planus and cicatrical pemphigoid during episode of increase severity
pain and sloughing of surface usually required to maintain symptomatic
epithelium are characteristic control
ANUG Severe pain, fetid odor, punched Compromise host resistance to Rapid response to improved
out papilla, pseudomembranous infection status of the host, antibiotics
ulceration, exudate and erythema; usually therapy and superficial
most sever in anterior region debridment of the tissue
Appendices
Pregnancy Indistinguishable from hyperplastic gingivitis, Pregnancy, puberty, hormonal Improved hygiene and oral
gingivitis except that then erythematous enlargement is fluctuation may be contributory prophylaxis usually yields
generalized and the patient history significant improvement by
minimizing the inflammatory
component of the process
Hemorrhagic Edematous enlargement and Associate with bleeding disorders, scurvy, Diagnostic sign of the causative
gingivitis erythema may be dramatic, but leukemia and hemopoietic suppression; may condition; improvement is usually
the consistent feature is dramatic become severe dramatic following resolution of systemic
hemorrhage after even slight condition
pressure on the tissue
Uremic gingivitis Painful, erythematous gingiva as Associated with renal failure Often persist to some degree
well as odor of ammonia and despite local plaque control
excessive salivation
Idiopathic Evidence of inflammation persist Failure to respond to normally Indicates the need for additional
gingivitis following eliminations of plaque effective treatment usually suggest diagnostic evaluation to identify the
and calculus, as well as improved diminished host resistance or other underlying contributory condition
hygiene contributory systemic disease that has not
been diagnosed
933
Enamel pathology
A. Developmental C. Enamel caries
• Amelogenesis imperfecta • Pit and fissure
934
• Dens invaginatus • Smooth surface
• Enameloma • Caries at cementoenamel junction
B. Environmental pathology D. Pigmentation
• Attrition • Endogenous
• Abrasion • Exogenous
• Erosion E. Enameloma
Dentin pathology
A. Developmental C. Neoplastic
• Dentinogenesis imperfecta • Dentinoma
• Dentinal dysplasia • Odontoma
• Regional odontodysplasia D. Regressive changes
• Dentin hypocalcification • Secondary dentin
B. Dentinal caries • Dentinal sclerosis
Leukopenia
I. Infections • Aleukemic leukemia
A. Bacterial • Agranulocytosis
• Typhoid III. Chemical agent
• Paratyphoid fever A. Agents commonly producing leukopenia in all patient
• Brucellosis if given in sufficient dose
• Tularemia (early) • Mustards (sulfur and nitrogen mustards)
B. Viral and rickettsial • Urethane
• Influenza • Busulfan
• Measles • Benzene
• Rubella • Antimetabolites
• Chickenpox B. Agents occasionally associated with leukopenia
• Infectious hepatitis apparently as result of individual sensitivity
• Colorado tick fever • Analgesics, sedative and anti-inflammatory
• Dengue • Antithyroid drug
• Yellow fever • Anticonvulsant
C. Protozoal • Sulfonamides
• Malaria • Antihistamine
• Relapsing fever • Antimicrobial agents
• Kala-azar • Tranquilizers
D. Any overwhelming infection IV. Physical agents
• Miliary tuberculosis • X-ray radiation and radioactive substance
• Septicemia V. Anaphylactic shock and early stages reaction of foreign
II. Hemopoietic disorders protein
• Gaucher’s disease VI. Disease of unknown etiology
• Pernicious anemia • Liver cirrhosis
• Aplastic anemia • Disseminated erythematosus
• Chronic hypochromic anemia • Cyclic neutropenia
Appendices
Basophilia
I. Blood disorders III. Infection
• Chronic myelocytic leukemia • Chronic inflammation of accessory tissue
935
• Chronic anemia • Smallpox
• Hodgkin’s disease • Chickenpox
II. Splenectomy IV. Myxedema
V. After injection of foreign proteins
VI. Some cases of nephrosis
Neutrophilia
A. Acute infection D. Acute hemorrhage
• Coccal E. Acute hemolysis
• Bacilli F. Malignant tumor of
• Fungi • Gastrointestinal tract
• Spirochetes • Liver
• Virus • Bone marrow
• Rheumatic fever G. Blood disorders
• Diphtheria • Myelocytic leukemia
• Small pox • Polycythemia
B. Inflammatory • Myelofibrosis
• Coronary thrombosis • Myeloid metaplasia
• Gout • Chronic idiopathic neutropenia
• Collagen vascular disease • Hereditary neutrophilia
• Burns H. Miscellaneous
• Hypersensitivity reaction • Physiologic in the newborn
C. Intoxication • During labor
• Uremia • After repeated vomiting
• Diabetes acidosis • Convulsion
• Poisoning by chemical and drugs like lead, mercury, • Paroxysmal tachycardia
digitalis, insect venoms, black widow spider • After epinephrine injection
Eosinophilia
A. Allergic • Hodgkin’s disease
• Bronchial asthma • Pernicious anemia
• Urticaria E. Infection
• Angioneurotic edema • Scarlet fever
• Hay fever • Chorea
• Allergic rhinitis • Erythema multiforme
• Drug sensitivity F. Malignant disease of any type
B. Skin disease G. Following irradiation
• Pemphigus H. Miscellaneous
• Demits herpetiformis • Pulmonary infiltration with eosinophilia
• Bullous pemphigoid • Tropical eosinophilia
C. Parasitic infection • Polyarteritis nodosa
• Trichinosis • Rheumatoid arthritis
• Echinococcosis disease • Sarcoidosis
D. Blood disorders • Certain poison
• Chronic myelocytic leukemia I. Inherited
• Polycythemia vera J. Idiopathic
Lymphocytosis
A. Acute infection C. Lymphocytic leukemia
• Infectious mononucleosis D. Lymphosarcoma
936
• Acute infectious lymphocytosis E. Heavy chain disease
• Infectious hepatitis F. Hemopoietic disorders
B. Chronic infection • Neutropenia
• Tuberculosis • Exanthema
• Secondary and congenital syphilis
• Undulant fever
Monocytosis
A. Bacterial infection • Hodgkin’s disease
• Tuberculosis • Multiple myeloma
• Subacute bacterial endocarditis D. Lipid storage disease
• Syphilis • Gaucher disease
• Brucellosis E. Malignant neoplasm
• Typhoid • Carcinoma of ovary, breast and stomach
B. Protozoan and rickettsial infection F. Collagen vascular disease
• Malaria • Lupus erythematosus
• Rocky Mountain spotted fever • Rheumatoid arthritis
• Typhus G. Granulomatous disease
• Kala azar • Sarcoidosis
• Trypanosomiasis • Ulcerative colitis
• Oriental sore • Regional arteritis
C. Blood disorders H. Chronic high dose steroid therapy
• Lymphoma
• Leukemia
Peripheral plasmocytosis
I. Infection B. Antitoxins
A. Viral • Equine tetanus
• Rubella • Equine diphtheria
• Rubeola III. Neoplasm
• Varicella A. Hematological
• Infectious mononucleosis • Plasma cell leukemia
B. Bacterial • Chronic lymphocytic leukemia
• Streptococcal B. Non-hematological
• Diplococcal • Breast
• Syphilis • Prostate
• Tuberculosis IV. Miscellaneous
C. Protozoal • Transfusion
• Malaria • Hyper-immunization
• Trichinosis • Trauma
II. Serum sickness
A. Drugs
• Penicillin
• Sulfisoxazole
Appendices
Causes and mechanism of vitamin D deficiency

Predisposing factors Mechanism
Dietary lack of meat and dairy product Low levels of vitamin D in the diet 937
Lack of adequate exposure to ultraviolet light Failure of vitamin D precursor synthesis in the skin
Gastric intestinal disease or chronic liver disease Malabsorption of vitamin D and calcium
Aluminum toxicity and biphosphonates Direct inhibition of bone mineralization
Administration of anticonvulsant drug like phenobarbitone These drug enhance liver enzyme activity which breakdown of
result in increase vitamin D to biological inert product
Chronic renal failure Reduced conversion of 25(OH)D3 to 1,25 (OH)2 D3
Hypophosphatemia rickets (X linked dominant) Inherited defect in renal tubular phosphate reabsorption leading
to hypophosphatemia
Hypophosphatasia (autosomal recessive) Defect mutation in bone alkaline phosphatase which
cause inhibition of bone mineralization at the calcification front
Differentiating features of ossifying fibroma of fibrous dysplasia

Features Ossifying fibroma Fibrous dysplasia
Age 3rd and 4th decade 1st and 2nd decade
Gender predilection Females Equal
Location Body of mandible Maxilla
Radiography Well defined margin Poorly defined margin
Lesion shape Roughly Nodular or spherical Fusiform or elliptical
APPENDIX II: GLOSSARY

Rashmi Ekka
Aberration: It is a variation from the normal form or Aglossostomia: It is the congenital absence of the tongue and
course . of mouth opemng .
Aberrancy: It is defined as that situation in which a tissue Agranulocytosis: A marked decrease in the number of
develops at a site where it is not normally found. granulocytes, particularly neutrophils .
Ablation : It is removal of a part by excision or amputation. Allelograft: A graft using material not derived from a donor
Abnormal: It is not normal , deviating in some from the usual or from animal sources, e . g . synthetic resins , stainless steel
structure, position or state . alloy .
Abrasion : It is the wearing away of a structure or substance Allergen: A substance capable of inducing hypersensitivity
by mechanical means such as scrubbing or grinding . or an allergic reaction.
Abrasive: It is a substance which contains an abrasive which Allergy: It is hypersensitivity to any normally harmless
tends to erode the surface . substance resulting in an exaggerated or abnormal reaction .
Abfraction : Loss of tooth surface at the cervical areas of Allograft: It is a graft derived from a donor of the same
teeth, caused by tensile and compressive forces during tooth species but genetically dissimilar .
flexure ; cervical erosive lesions that cannot be attributed to Amniocentesis : It is diagnostic procedure in which a small
any particular cause . amount of amniotic fluid is withdrawn from amniotic sac,
Abscess: An abscess is a localized collection of pus a membrane surrounding the fetus in uterus , to detect fetal
surrounded by an area of inflamed tissue in which hyperemia defects .
and infiltration of leukocytes is marked . Amalgam tattoo : Oral soft tissue discolorations due to
Actinic keratosis: It is a premahgnant squamous cell lesion amalgam ; most common pigmentation of the oral cavity .
resulting from long-term exposure to solar radiation and may Amelogenes is: The formation of the enamel portion of the
be found on the vermilion border of lip as well as other sun tooth .
exposed skin surfaces . Analgesia: It is relief from pain or insensitibilty to pain .
Actinic clastosis: It is a lesion on the labial mucosa exposed Analogous : Having similar properties .
to sun . A white area of atrophic epithelium develops with Anesthesia: It is the general loss of all sensations or feelings .
underlying scarring of the lamina propria . Anemia: It is an abnormal reduction in the number of
Actinic cheilitis: When this atrophic tissue abrades to ulcer, circulating red blood cells, the quantity of hemoglobin and
it is called actinic cheilitis . the volume of packed red cells in a given unit of blood .
Acanthosis: This condition is characterized by widening and Anaphylaxis : It is an antigen-antibody reaction produced by
thickening of stratum spmosum . the parenteral injection of an antigen causing hypersensitivity .
Acantholysis: It is the pathological separation of epidermal Anastomosis : It is a communication between two vessels .
or epithelial cells by breakdown of desmosomes in stratum Anachoresis: If the bacteria circulating in the bloodstream
spmosum (seen in pemphigus) . settle in areas of inflammation or of lowered resistance
Acquired : Relating to something not of genetic origin but in the pulp and produce pulpitis, abscess or necrosis, the
resulting from outside influence . phenomenon is referred to as anachoresis .
Acrocephalic : It is a highly arched or pointed skull . Anomaly: Deviation or irregularity as compared with the
Acute: Having severe symptoms and a short course . normal .
Adduction : Drawing in towards the center or to median line , Anorexia: It is the lack of appetite .
as opposed to abduction . Anomalad : It is a malformation together with its subsequently
Adenomatosis oris : It is the swelling of the mucous glands derived structural changes; the primary defect setting off
of the lips with no inflammation or secretion . a series of secondary or even tertiary events resulting in
Adrenodontia: It is a morphological indication of over multiple anomalies .
activity of adrenal glands characterized by large pointed Anosmia: It is the absence of sense of smell .
canines and teeth with occlusal surfaces showing brown Antagonist: It is any tissue that acts against or in opposition
discoloration . to another tissue .
Aerodontia: It is that branch of dentistry concerned with Anaplasia: It is the reversion of the same type of cells from a
the care and treatment of dental conditions caused by high more highly differentiated to a less highly differentiated type .
altitude flying . Antibody: It is any one of the class of substances produced
Afferent nerve : It refers to any nerve transmitting impulse in the body as a reaction to a specific antigen and with which,
from the periphery to the center . it reacts in some observable way to produce a specific effect
Ageusia: It is the loss or absence of sense of taste . such as inactivation, agglutination, and/or flocculation .
Appendices
∙ Antibiotics: These are substances produced by micro- ∙ Baelz’s disease: It is a disease characterized by the presence
organisms which suppress the growth or kill other micro- of painless papules on the labial mucous membrane. (Cheilitis
organisms at a very low concentration. glandularis-superficial suppurative type).
∙ Antidote: It is an agent used to counteract or prevent the ∙ Ballooning degeneration: It is characterized by the isolation 939
action of poisons. of a cell from its neighbors, especially in the lower layers
∙ Antigen: It is any substance that when introduced into the of the epidermis, the withdrawing of its prickles after intra-
body, excites the formation of specific antibodies. cytoplasmic edema and vacuolization and the amitotic
∙ Angioma:A tumor made up of blood or lymph vessels. division of its nucleus so as to form multinucleated giant cells.
∙ Ankyloglossia: Extensive adhesion of the tongue to the floor ∙ Bay cyst: Apical cyst which have a direct connection with
of the mouth or the lingual aspect of the anterior portion of apical foramen have been termed as ‘bay cyst’.
the mandible caused by a short lingual frenum. ∙ Bednar aphthae: Two ulcers appearing symmetrically one
∙ Apertognathia (open bite): A condition in which the anterior on either side of the midline of the hard palate in infants,
or the posterior teeth of the mandible cannot be brought into thought to be caused by the nipple or by thumb sucking or
occlusion with antagonist teeth of maxilla. sucking hard object.
∙ Aponeuroses: These are collagenous sheets or ribbons that ∙ Benign: Not malignant; favorable for recovery.
resemble flat, broad tendons. It may cover the surface of the ∙ Bicameral abscess: It is an abscess which contains two
muscle and assist in attaching superficial muscles or separate chambers.
the structures. ∙ Biopsy: It is the gross and microscopic examination of tissue
∙ Aplasia: Absence of an organ or organ’s part due to failure of or cells removed from living patients for the purpose of
development of the embryonic tissue of origin. diagnosis or prognosis of the disease or the confirmation of
∙ Arteriosclerosis: A condition characterized by loss of the normal condition.
elasticity and thickening of artery walls. ∙ Blanching: To take the color out of and make white.
∙ Atrophy: It is a reduction in size of tissue or of an organ due ∙ Bleb: It is a bulla or other skin blister filled with blood or
to decrease in the size or number of its constituent cells. serous fluid.
∙ Atresia: It is the congenital occlusion or absence of one or ∙ Blind abscess: It is the one having no fistulous tracts.
two major salivary gland ducts. ∙ Blister: It is a vesicle caused by localized accumulation of
∙ Atypical: Irregular, not conformable to the type. fluid beneath the skin.
∙ Attrition: It is the physiologic wearing away of tooth ∙ Blood: It is the red fluid in the vessels of the circulating
material as a result of tooth to tooth contact. system which conveys oxygen and nutritive materials to the
∙ Auscultation: Listening to the sound produced within the tissue and removes carbon dioxide and waste matter.
body with the help of a stethoscope. ∙ Blood pressure: It is the pressure exerted by the blood on
∙ Autogenous: It is produced within the body itself. It is self the artery walls and is dependent on the force of heart action,
generated. the elasticity of the vessel walls, capillary resistance and the
∙ Autograft: It is a graft taken from one of the patient’s body volume and viscosity of blood.
and transplanted to another part in the same individual. ∙ Blood transfusion: The intravenous administration of blood
∙ Autoantibody: An antibody that reacts against an antigenic to help replenish excess blood loss due to hemorrhage or
constituent of the person’s own tissues. other wise, is known as blood transfusion.
∙ Autoimmune disease: A disease characterized by tissue ∙ Boil: It is a localized skin abscess usually at the site of a hair
injury caused by a humoral or cell-mediated immune response follicle.
against constituents of the body’s own tissues. ∙ Bosselated: Having a knob like protrusion or bosses.
∙ Autoimmunity: Immune-mediated destruction of the body’s ∙ Bowen’s disease: It is a localized intraepidermoid carcinoma
own cells and tissues; immunity against self. that may progress to invasive carcinoma over many years.
∙ Autosomes: The non-sex chromosomes that are identical for ∙ Bradycardia: It is an abnormal slowness of the heart and
men and women. pulse rate.
∙ Autoinoculation: To inoculate with a pathogen such as a ∙ Bradyglossia: It is an abnormal slowness of speech, due to
virus from one’s own body. difficulty in tongue movements.
∙ Bacteremia: It refers to the circulation of bacteria in the ∙ Bradypnea: It is an abnormal slowness of respiration.
blood. ∙ Bruise: It is a superficial injury, caused by a blow with no
∙ Bacteria: These are microscopic unicellular vegetative laceration but with discoloration of the skin and subcutaneous
organisms having a single chromosome, no nuclear envelope tissue produced by an accumulation of blood.
and a rigid cell wall. They may be seen as rods, cocci or ∙ Bruxism: It can be defined as the involuntary, unconscious,
filaments and divide by binary fission. and excessive grinding, tapping or clenching of teeth or it is
∙ Bacteriostatic: It is any agent that inhibits the growth and defined as non-functional grinding or gnashing of the teeth,
multiplication of bacteria. usually during sleep.
∙ Buccal bifurcation cyst: A cyst of uncertain origin found ∙ Cariology: It is the scientific study of dental caries, its
primarily on the distal or facial aspect of a vital mandibular causes, prevention and treatment.
third molar, consisting of intensely inflamed connective ∙ Carrier: The individual who continues to harbor infectious
940 tissue and epithelial lining. agent either following recovery from the illness it induced.
∙ Bullae: It is an elevated blister like lesion containing clear ∙ Cartilage: It is a form of elastic, nonvascular connective
fluid and is bigger than 1 cm in diameter. tissue attached to articular bone surfaces and also forming
∙ Burrows: These are short, linear, straight or sinuous lines in some parts of the skeleton.
the skin. ∙ Catabolism: It is the process of breakdown of complex
∙ Burn: It is the injury resulting from the application of compounds by the body.
excessive heat, electric current, friction and caustics to skin ∙ Catarrh: It is the inflammation of the mucous membranes,
or mucous membrane. especially those of nose and throat, with discharge of mucus.
∙ Carcinogenesis: Carcinogenesis or oncogenesis or tumor- ∙ Causalgia: It is a burning sensation arising after trauma to a
ogenesis means induction of a tumor agent which can induce sensory nerve.
tumor. The tumor agents are called carcinogens. ∙ Cellulitis: Cellulitis may be defined as a non-suppurative
∙ Carabelli’s cusp: It is an accessory lingual cusp located on inflammation of the subcutaneous tissue extending along the
mesiopalatine cusp of maxillary second primary molars and connective tissue planes and across the intercellular spaces.
1st, 2nd and 3rd permanent molars. ∙ Cell: It is one of the minute masses of protoplasm, containing
∙ Capsule: Compressed fibrous connective tissue around a a nucleus which forms the basis of all animal and plant
benign neoplasm separating it from surrounding tissues. structure.
∙ Carcinoma: A malignant growth made up of epithelial cells ∙ Cementicle: It is a small calcareous body developing in the
that are capable of infiltration and metastasis. periodontal membrane.
∙ Caries: Demineralization of inorganic and dissolution of ∙ Cell mediated immunity: It is the type of immunity in which
organic part of the tooth surface caused by bacteria. the predominant role is played by T lymphocytes.
∙ Carcinoma in situ: It is a histopathological diagnosis ∙ Central: In oral pathology, it is the lesion occurring within
defined as a proliferation of basal epithelial cells from the bone.
basement membrane to the surface, with almost all of the ∙ Centromere: The constricted portion of the chromosome
cells manifesting cytologic atypia. Immediate maturation that divides the short arms from the long arms.
into a superficial keratin layer is possible, but no invasion ∙ Chief complaint: It is the patient’s response to the dentist’s
into the underlying connective tissues can be seen. question.
∙ Calcareous: Relating to or containing calcium or calcium ∙ Cheilitis: It is the inflammation of lip.
salts; chalky. ∙ Chemoprophylaxis: It is the use of chemical drugs in the
∙ Calcification: It is the deposition in organic tissue of calcium prevention of disease.
salts causing hardening. ∙ Chemotherapy: It is the treatment of a disease by chemicals
∙ Calcinosis: It is a condition characterized by either localized which affect pathogenic organisms without harming the
or generalized deposition of calcium salts in nodules in the patient or it is the treatment of malignant neoplasia by
soft tissues. chemical means.
∙ Callus: The mesh of fibrous bony tissue surrounding and ∙ Cheesy: Lesion’s texture is similar to curd of cheese.
uniting the bone ends after fracture. It is later replaced by ∙ Chemotaxis: Taxis or movement in response to chemical
hard bone. stimulation.
∙ Camper’s line: It is the line extending from the external ∙ Chromatin: A general term used to refer to the material
auditory meatus to a point below the nasal point and is also (DNA) that forms the chromosomes.
called facial line. ∙ Chronic: Persisting over a long time; when applied to a
∙ Cancellous: Having a lattice like spongy structure; applied disease, chronic means that there has been little change or
to bone tissue. extremely slow progression over a long period.
∙ Canker: It is an ulceration especially of the mouth and lips ∙ Chills: It is cold sensation with shivering, often characteristic
and it is also called aphthous stomatitis. of onset of fever.
∙ Capillary: These are one of the very fine thread like blood ∙ Chloroma: It is a condition characterized by multiple
vessels connecting the veins and arteries. myeloid tumors of greenish color, affecting particularly the
∙ Carbuncle: It is a staphylococcal infection of the sweat face and skull, and associated with blood picture of leukemia.
glands or hair follicles causing inflammation of the ∙ Choriostoma: It refers to excessive amount of normal tissue
surrounding subcutaneous tissue and discharging pus through that is present in abnormal location.
several openings, finally sloughing away. ∙ Chondromalacia: It is a condition characterized by abnormal
∙ Carcinosarcoma: It is a mixed tumor containing characteris- softness of the cartilage.
tics of both carcinoma and sarcoma. ∙ Ciliated: Having hair like processes or fringe of hair.
Appendices
∙ Circulation: It is the movement or flow in a circle, retracing ∙ Crust: Dry products of exudation from lesions occurring on
its course repeatedly, applied especially to the flow of blood skin and lips.
through the body. ∙ Crepitations: It refers to a crackling noise occurring in the
∙ Cleft lip: It is a birth defect that results in a unilateral or joint when affected by certain disease. 941
bilateral opening in the upper lip between the mouth and the ∙ Cryosurgery: It is the use of extreme cold for surgical
nose. destruction of tissue.
∙ Cleft palate: Cleft palate is a birth defect characterized by ∙ Cryotherapy: It is the treatment of disease with use of
an opening in the roof of the mouth caused by lack of tissue extreme cold.
development. ∙ Cryptogenic leukoplakia: In a small proportion of cases
∙ Coagulation: When blood is shed, it loses its fluidity in of leukoplakia, no underlying cause has been found. Such
few minutes and sets into a semisolid jelly. This is called lesions are termed as idiopathic or cryptogenic leukoplakia.
coagulation or clotting. ∙ Culture: It is the growth of microorganisms in an artificial
∙ Cold abscess: It is a slow developing tuberculous abscess medium.
generally about a bone or joint and with little inflammation. ∙ Curettage: It refers to the removal of foreign matter from the
∙ Collar stud abscess: It is a superficial abscess connected by walls of a bony cavity or from the root surface.
a sinus tract to a larger deep abscess. ∙ Cyst: Cyst is a pathological cavity which may or may not be
∙ Complement system: This consists of a group of serum lined by epithelium and consists of fluid, semi-fluid or gaseous
proteins which by series of reactions produce and release content (but not by pus) and surrounded by connective tissue
by products whose functions are to initiate an inflammatory capsule. True cyst is a pathologic cavity always lined by
reaction, to regulate and enhance phagocytic function and epithelium usually containing fluid or semi-solid material.
attack the bacterial cell membrane. ∙ Cytology: It is the scientific study of cell.
∙ Congenital: Present at or before birth but not necessarily ∙ Cytopathic: Pertaining to or characterized by pathologic
inherited. changes in cells.
∙ Coalesce: It is a term used to denote to fusion or union of ∙ Cyanosis: It is the bluish discoloration of the skin and
separated parts. mucous membranes, often due to deficient oxygenation of
∙ Consanguinity: Blood relationship. In genetics, the term is the blood.
generally used to describe marriages among close relatives. ∙ Dental kinesiology: It is the study of motion and function of
∙ Corrugated: Having a surface that appears wrinkled. jaws and oral musculature; the accompanying neurological,
∙ Cotton wool: Confluent radiopacities. vascular and other supporting system network and the impact
∙ Coarctation: It is narrowing or constriction, applied of those muscle functions and neurological dynamics have on
especially to blood vessels. dental and systemic health.
∙ Col: It is a depression in an interdental papilla between the ∙ Developmental anomalies: Malformation or defects result-
two peaks, one on each side of the contact area. ing from disturbance of growth and development are known
∙ Coma: It is a state of complete unconsciousness from which as developmental anomalies.
a patient cannot be aroused, even by determined external ∙ Dens in dente: It is also called dens invaginatus. Infolding of
stimulation. the outer surface of the tooth into interior. It is a developmental
∙ Commensal: It is an organism that lives on or within another variation which is thought to arise as a result of invagination
organism, to its own advantage and without being detrimental in the surface of tooth crown before calcification occurs.
to the host. ∙ Dens evaginatus: Dens evaginatus is a developmental
∙ Commissure: It is the point of union between similar parts condition that appears clinically as an accessory cusp or
or bodies. globules of enamel on occlusal surface between buccal and
∙ Concretion: It refers to any hardened or solidified mass in lingual cusp of premolars.
the tissue. ∙ Debridment: It is the removal of dead tissue and foreign
∙ Counter irritation: It refers to the deliberate production of matter from a wound.
superficial irritation in order to mask or relive an existing ∙ Degeneration: It refers to the gradual deterioration of tissue
irritation or pain. with loss of function and chemical changes within the tissue.
∙ Concrescence: It is a form of fusion that occurs after the ∙ Dentistry: It is a branch of medicine concerned with oral and
root and other major parts of the involved teeth are formed or dental diseases and their prevention and treatment and with
when the roots of two or more teeth are united by cementum, oral prosthesis.
below the cementoenamel junction. ∙ Desmosomes: The term desmosomes refers to the structures
∙ Craniomalacia: It refers to a condition characterized by forming the site of contact between adjacent cells, especially
softness of bones of the skull, usually seen in infants. epithelial cells.
∙ Crater: It is a localized depression, usually circular, with ∙ Desquamation: It refers to the peeling off of the outer layer
raised edge or rim. of epithelium.
∙ Dental fluorosis: A condition of enamel hypoplasia ∙ Discoid lupus erythematous (DLE): It is a circumscribed
characterized by white chalky spots or brown staining and slightly elevated white patch that may be surrounded by a red
pitting of teeth due to an increased level of fluoride; affecting telangiectic halo.
942 enamel matrix formation and calcification by impairment of ∙ Dose: It is one measured portion of any medicine which is to
ameloblastic function. be taken one at a time.
∙ Dentigerous cyst: An odontogenic cyst that surrounds the ∙ Dominant: In genetics, a trait or characteristic that is
crown of an impacted tooth; caused by fluid accumulation manifested when it is carried by only one of a pair of
between the reduced enamel epithelium and enamel surface, homologous chromosomes.
resulting in a cyst. ∙ Dorsal: Directed towards or situated on the back surface
∙ Deoxyribonucleic acid (DNA): A substance composed of a (opposite of ventral).
double chain of polynucleotide; both chains coiled around a ∙ Dry abscess: It is an abscess that disperses without bursting
central axis form a double helix. DNA is the basic genetic or coming to a head.
code or template for amino acid formation. ∙ Drainage: It is the gradual removal of fluid from a cavity or
∙ Dermoid cyst: A cyst of midline of the upper neck or the wound.
anterior floor of the mouth of young patients, derived from ∙ Dressing: It is a medicament used to promote wound healing
remnants of embryonic skin; consisting of a lumen lined by or as a covering for a wound, used for protection or to assist
a keratinizing stratified squamous epithelium and containing healing.
one or more skin appendages such as hair, sweat or sebaceous ∙ Drug: It is any medicinal substance.
glands. ∙ Dyskinesia: It is defined as an impairment of voluntary
∙ Diffuse: Used in the description of a lesion; when borders of motions, causing movements that are incomplete or only
the lesion are not well defined and it is not possible to detect partial.
the exact parameters of the lesion, then this term is used. ∙ Dysesthesia: It refers to the impairment of feeling or
∙ Diploid: Having two sets of chromosomes; the normal sensations; a condition in which a normal stimulus produces
constitution of somatic cells. disagreeable sensations.
∙ Diagnosis: It is the determination of the nature or cause of ∙ Dyskeratosis: This lesion shows abnormal orientation in
the disease. development of epithelial cells.
∙ Differential diagnosis: The list of similar clinical picture, ∙ Dysodontiasis: It refers to the painful, difficult or delayed
according to probable identity of condition at hand, is the eruption of the teeth.
differential diagnosis. ∙ Dysostosis: It refers to the congenital defective bone
∙ Dimorphic anemia: It is a condition in iron deficiency and formation.
folic acid deficiency anemia can occur concomitantly. ∙ Dysphagia: An experience of having great difficulty in
∙ Diploe: The spongy layer of bone position between the inner swallowing.
and outer layers of compact bone. ∙ Dystrophic calcification: Pathologic calcification that
∙ Diverticuli: They are small pouches or out pocket of the occurs in degenerating and dead tissue.
ductal system of one of the major salivary glands. ∙ Dysplasia: It refers to the abnormal formation or development.
∙ Disinfection: This is the process by which pathogenic ∙ Dyspnea: It is a shortness of breath.
microorganisms are removed from the surface, without ∙ Ecchymosis: It refers to the diffuse extravasation of blood into
removing bacterial spores. the tissues. Larger purpuric lesions are called ecchymoses.
∙ Dilacerations: It refers to angulations or sharp bends or ∙ Ectoderm: The outermost of the three primary germ layers
curves in the root and crown of the teeth. of the embryo, from which are developed the epidermis, the
∙ Disease: It is the departure from the average anatomical external sense organs and the oral and anal mucous mem-
structure or is an abnormal degree of failure of physiological branes.
function or some reduction in psychological efficiency, ∙ Edema: It is accumulation of excess fluid in the intercellular
due to either adversity in the genetic endowment of the tissue spaces or body cavities.
individual or misuse of his free will or to adverse factors in ∙ Electrocautery: It is cauterization by low voltage current
the environment in which he lives or some combination of producing burn like tissue repair, but with no control over the
these factors or it is defined as loss of ease. extent or quality of tissue destruction.
∙ Distomolar: Found in the molar region frequently located ∙ Electrodesiccation: It refers to the deeply penetrating tissue
distal to 3rd molar. dehydration produced by the insertion of electrodes into the
∙ Discrete: Separate. Composed of separate parts, not joined tissue.
or blended. ∙ Empirical therapy: With serious infections, it is often
∙ Dislocation: It is the displacement of any part from its normal necessary to begin antibiotic therapy before culture result is
position, especially in the cases of bone and joints. available, this is called empirical therapy which is directed
∙ Direct fracture: It refers to the fracture that occurs at the towards organisms which are most likely to have caused that
site of blow. infection.
Appendices
∙ Embedded teeth: Those teeth which are unerupted usually ∙ Erythroplastic: It is characterized by a reddish appearance.
because of lack of eruptive force. This term implies abnormal tissue proliferation in the reddish
∙ Embolism: It refers to the sudden blockage of blood vessels area.
by a clot or other obstruction within the blood stream, causing ∙ Erythrocyte: One of the red cells found in blood which 943
failure of circulation. carries oxygen and is produced by the bone marrow.
∙ Empyema: It is the accumulation of pus in a body cavity or ∙ Erythroplakia: The term is applied to any area of reddened
a hollow organ. velvety textured mucosa that cannot be identified on the basis
∙ Enamel pearls, nodules, or droplets: Pearls or droplets of clinical and histopathological examination as a cause of
described as small buttons or nodules of enamel usually inflammation or any other disease process.
about 1 mm or 2 mm in diameter that form on the root or at ∙ Erythrodontia: There is deposition of porphyrins in dentin
the bifurcation of multi-rooted teeth. and to a lesser extent in the enamel which imparts red or
∙ Embryonic: Pertaining to the earliest stage of development brown color to the deciduous and permanent teeth and known
of an organism. as erythrodontia.
∙ Emigration: The passage of white blood cells through the ∙ Erosion: It is a shallow crater in the epithelial surface that
endothelium and walls of small blood vessels. appears on clinical examination as a very shallow erythematous
∙ Endotoxin: They are heat stable phospholipid-polysaccha- area with only superficial changes. Or a moist red lesion often
ride-protein complex contained as a structural part of the cell caused by rupture in vesicles and bullae as well as trauma.
of many gram-negative bacterias and released by disintegra- ∙ Erosion (teeth): It is loss of tooth substance due to chemical
tion of the cells. process that does not involve bacterial activity.
∙ Enanthema: It is an eruption occurring on a mucous surface ∙ Erythroplasia: These are painless erythematous eruptions,
or on any surface within the body as opposed to exanthema. popular or macular in nature, affecting the mucous membrane.
∙ Endemic: Prevalent in a particular region. ∙ Eschar: It is a dry slough, the result of burning or due to
∙ Endosteal: It is within the bone. contact with a corrosive agent.
∙ Endothelium: It refers to the membrane lining the heart and ∙ Etiology: The study or theory of the factors that cause disease
blood vessels. and their introduction to the host.
∙ Engorgement: It refers to the excess of blood in any part of ∙ Eversion: A turning outward or a state being turned outwards.
the body or it is the localized congestion or distension. ∙ Excrescence: It refers to an abnormal growth protruding
∙ Enostosis: It is a localized morbid bone growth arising from body or plant.
within the bone cavity. ∙ Exacerbation: It refers to an increase in the severity of a
∙ Enucleate: The word enucleate means to remove an organ disease or any symptoms.
or part, or a circumscribed, space filling lesion entirely, i.e. ∙ Examination: It refers to investigations carried out for
from its outer sheath or covering. diagnostic purpose.
∙ Endodermal: Pertaining to the innermost of the three ∙ Exanthema: It is an eruptive fever.
primitive germ layers of an embryo. Endodermal structures ∙ Excoriation: It is the superficial loss of surface skin or a
include the epithelium pharynx, respiratory tract (except the graze.
nose) and digestive tract. ∙ Excursion: It refers to any movement of a movable part from
∙ Epidemic: Affecting large number of people within an area a resting position during the performance of some functions.
or region. ∙ Exfoliation: It is the peeling off in layers or in scales.
∙ Epidemiology: It is that branch of science concerned with ∙ Exophthalmos: It refers to the abnormal protrusion of the
the study of a disease or condition through its frequency and eyeball.
distribution. ∙ Exophytic: It refers to a word relating to something growing
∙ Epithelium: It is a thin cellular layer covering or lining the outwards, used for tumor projecting above the normal surface
organs and tissues of the body. contours or it refers to any pathological growth that project
∙ Eponym: The name of an organ, syndrome, disease, etc. that above the normal contours of the oral surface.
contains or is derived from a proper name. ∙ Expansile: Capable of being extended or expanded.
∙ Epulis: Any tumor of the gums; more especially either a ∙ Expressivity: In genetics, the degree of clinical manifestation
fibrous or a giant cell tumor. of a trait or characteristic.
∙ Eruption: The act of appearing, or pushing through, as ∙ Exostosis: It is a bony swelling developing on the bone
of teeth coming through the gums or a visible skin lesion surface or on a tooth root.
occurring in disease. ∙ Exotoxin: It refers to a toxic secretion of bacterial cells
∙ Erythema: It is the redness in the skin either diffuse or which cause damage in sites distant from the focus of
patchy, caused by congestion of the subcutaneous capillaries. infections or they are heat labile proteins which are secreted
∙ Erythematous: It characterized by a redness of the tissue by certain bacteria and diffuse readily into surrounding
due to engorgement of the capillaries in the region. tissue.
∙ Extravasation: It is the escape of fluid from vessels into the ∙ Foramen: A small hole in a bone through which passes
surrounding tissue. either blood vessels or nerves or both.
∙ Extrinsic: Having its origin outside and separated from a ∙ Focal osteitis: A condition sometimes occurring after tooth
944 body, organ or part. extraction, particularly after traumatic extraction, resulting in
∙ Exudate: The matter that passes out into adjacent tissues a dry appearance of the exposed bone in the socket, due to
through vessel walls in inflammation. disintegration or loss of the blood clot.
∙ Facies: The appearance of the face. ∙ Foreign body granuloma: A reaction to foreign materials
∙ Factitial injuries: These are accidentally self induced that are too large to be ingested by either microphages
injuries on the basis of habits with frequent psychological (PMNs) or macrophages.
backgrounds. ∙ Frenal tag: A redundant piece of mucosal tissue that projects
∙ Favorable fracture: If the fracture line runs in such a from the maxillary labial frenum.
manner that the associated muscle tends to hold the fragments ∙ Fusion: It is also called synodontia. It represents the
together, the fracture is described as favorable. embryonic union of normally separated tooth germs.
∙ Facet: It is a small abraded area on a bone or on tooth surface. ∙ Fulguration: It refers to the superficial tissue dehydration
∙ Familial: Relating to a family, or affecting several of its produced by a surgical electrode held slightly away from the
members. tissue, causing sparking.
∙ Fascia: It is the layer of areolar tissue beneath the skin or ∙ Galvanism: The production of an electric current caused
the layer of areolar tissue investing the muscles, nerves and when two dissimilar metals used as restorations in the mouth
other organs. come into contact, this can cause discomfort and even pain.
∙ Fenestrate: To pierce with one or more holes, sometimes ∙ Gangrene: It is the necrosis of tissue due to failure of the
used on the walls of bony defect in an attempt to stimulate arterial blood supply caused by injury or disease.
repair. ∙ Gelation: The process of change of a colloid from a sol to a gel.
∙ Fenestration: It refers to a surgical procedure by which one ∙ Gerodontia: It is that branch of dentistry which deals with
or more holes are pierced in hard tissue. the care of old people.
∙ Fever: It refers to an abnormal increase in body temperature. ∙ Gemination: It refers to the process whereby single tooth
∙ Final diagnosis: It is statement with which precise diagnosis germ invaginates resulting in incomplete formation of two
has been made on the basis of all required observation, teeth that may appear as a bifid crown on a single root.
identification of definitive symptoms and the pathological ∙ Genetic heterogeneity: Having more than one inheritance
report and patient response to therapy. pattern.
∙ Fibro-cemento-osseous lesions: It is a skeletal disorder in ∙ Ghost teeth: A developmental disturbance of several
which bone is replaced by fibrous tissue which in turn is adjacent teeth in which the enamel and dentin are thin and
replaced by mineralized tissue. irregular and fail to adequately mineralize; surrounding soft
∙ Fissure: It is a linear often crusted, tender, painful defect in tissue is hyperplastic and contains focal accumulations of
continuity of the skin, occurring usually at the mucocutaneous spherical calcifications and odontogenic rests.
junctions and at sites where there is considerable elasticity of ∙ Gingivosis: It refers to the any degenerative condition
the skin. affecting the gingiva.
∙ Fistula: It is communicating tract between two epithelial ∙ Gland: An organ that produces secretions.
surfaces which is lined by granulation tissue which is ∙ Glossodynia: It refers to the burning or painful condition of
subsequently epithelized. the tongue.
∙ Fibrosis: There is an abnormal formation of fibrous tissue. ∙ Gomphosis: It is the firm attachment of two bones without
∙ Fluctuant: A wavelike motion felt on palpating a cavity with a movable joint.
nonrigid walls, especially one containing fluid. ∙ Gorham’s disease: In this condition a large portion of bone
∙ Fluoride mottling: A condition of enamel hypoplasia disappears without any apparent cause.
characterized by white chalky spots or brown staining and ∙ Granuloma: A tumor composed of granulation tissue.
pitting of teeth due to an increased level of fluoride affecting ∙ Granulomatosis: It refers to the development of multiple
enamel matrix formation and calcification by impairment of granuloma.
ameloblastic function. ∙ Granulation tissue: It is the reparative tissue that is
∙ Focus of infection: It refers to a circumscribed area of tissue, formed on the surface of wound having pink, soft, granular
which is infected with exogenous pathogenic microorganisms appearance showing histologically new small blood vessel
and which is usually located near a mucous or cutaneous and fibroblast.
surface. ∙ Green stick bone fracture: It is a fracture in which one side
∙ Focal infection: It refers to metastasis from the focus of of bone is broken and the other side is bent but intact.
infection of organisms or their products that are capable of ∙ Ground glass: Fine radiopaque spots in radiolucent
injuring tissue. background.
Appendices
∙ Gustatory: The sense of taste or the act of tasting. ∙ Hypsodont: Having teeth with long crowns and short roots
∙ Hamartomas: It is a tumor like malformation of oral tissues, seen in herbivorous animals.
developmental in origin with tissue being native to the site. ∙ Hydropic degeneration: It refers to replacement of the
∙ Habit: It is a tendency toward an act or an act that has nuclei of stratum basal by clear space due to edema and 945
become a repeated performance, relatively fixed, constant, degeneration of cells.
easy to perform and almost automatic. ∙ Iatrogenic diseases: Theses are the diseases produced by the
∙ Hemoglobinopathies: These are a group of hereditary action of a doctor or due to medical treatment.
disorders characterized by the presence of structurally ∙ Idiopathic: It is any spontaneous or primary disease with no
abnormal hemoglobin. apparent external cause.
∙ Hereditary disease: They are apparent at birth but some may ∙ Idiosyncrasy: It refers to a reaction to a particular drug in
not become evident for years. therapeutic doses in a manner not necessarily related to its
∙ Hemoptysis: The presence of blood in the sputum caused by pharmacological properties.
bleeding in the upper respiratory tract or the lungs. ∙ Impacted teeth: They are those prevented from erupting by
∙ Hemorrhage: It refers to the internal or external loss of some physical barriers in the eruption path.
blood due to injury or other damage to blood vessels. ∙ Immunity: It is the resistance exhibited by the host towards
∙ Hemidesmosome: It refers to a structure found on the basal injury caused by microorganisms and their products.
surface of an epithelial cell, the attachment site between the ∙ Impermeable: Not permitting passage especially of fluids.
cell and the underlying membrane. ∙ Implant: The word implant means to insert into the body or
∙ Heredity: It refers to the transmission of a characteristic to graft as in plastic surgery.
from parent to child or to later generation. ∙ Infection: It is a clinicopathological entity-involving invas-
∙ Heterotrophic: It is a term used relating to organisms which ion of the body by pathologic microorganisms and the
require a complex source of carbon for nourishment and reaction of tissues to microorganism and their toxins.
growth. ∙ Inspection: It refers to an examination of the affected part
∙ Hematoma: It is large clot resulting from blood released into of the body.
the tissue from a ruptured or injured blood vessel. ∙ Internal derangement: It can be defined as mal-relationship
∙ Healing: It is repair and replacement of dead or damaged of the meniscus to the condylar head and articular eminence
cells by healthy cells. where an alteration of its attachment allows the meniscus to
∙ Histology: It refers to the study of the anatomy and assume an abnormal position.
physiology of tissue and cells using microscopic technique. ∙ Inflammatory collateral cyst: It is a cyst which arises in the
∙ Holistic: It refers to an approach to treatment that takes periodontium of an erupted tooth as a result of inflammatory
into consideration the whole person, not just the disease or process in the periodontal pocket.
condition. ∙ Involucrum: Small section of necrotic bone may be
∙ Homologous: Having the same or corresponding structure or completely lysed, while a large one may get localized, and
position but not necessary similar in function. get separated and form shell of new bone called involucrum
∙ Horner’s teeth: Incisor teeth with horizontal grooves caused by a bed of granulation tissue or a sheath particularly new
by enamel deficiency. bone sheath that forms about sequestration.
∙ Hypodontia: It refers to the absence of one or more teeth. ∙ Indirect fracture: Fracture site distant from where the actual
∙ Hypertrophy: It refers to the enlargement caused by an blow takes place, usually seen on contralateral side.
increase in size of cells. ∙ In vitro: Within glass referring to observations made in a test
∙ Hydrocyst: It refers to a cyst whose contents are watery in tube or culture dish as opposed to in vivo.
nature. ∙ In vivo: Within a living organism.
∙ Hyperplasia: It refers to the enlargement caused by increase ∙ Indentation: It refers to the condition of being serrated or
in number of cells. notched.
∙ Hypoplasia: It is the failure of full development of an organ ∙ Induced: Brought on by an outside agent or is artificially
or tissue. produced.
∙ Hydrostomia: It refers to a condition characterized by ∙ Induration: It refers to the state of being hard or the process
constant dribbling from the mouth. of becoming hard.
∙ Hygroma: It refers to a swelling caused by fluid surrounding ∙ Inflammation: It is the reaction of living tissue to injury.
an inflamed bursa, or distending a sac or cyst. ∙ Infarction: It is a localized area of ischemic necrosis in an
∙ Hypertension: Exceptionally high tension especially organ or tissue resulting from sudden reduction of either its
abnormally high blood pressure. arterial supply or venous drainage.
∙ Hypnosis: It refers to a sleep or a trance state, especially one ∙ Inflation: It refers to the distension with gas especially air.
induced artificially by verbal suggestions or concentration ∙ Inostosis: It refers to the process by which bony tissue is
upon some object. reformed to replace tissue that has been destroyed.
∙ Insidious: Unperceived coming on gradually and stealthily. ∙ Malocclusion: It refers to any deviation from the normal
∙ Intermittent: Occurring at intervals with periods of occlusion of the teeth resulting in impaired functions.
cessation. ∙ Marrow: It refers to the soft tissue canal and interstices of
946 ∙ Intubation: It refers to the introduction of a tube through bones.
the mouth or the nose to allow air, gas or vapor to pass into ∙ Marsupialization: It refers to an operation for the evacuation
the lungs. of a cyst and the suturing of its walls to the edges of the
∙ Iontophoresis: It refers to the therapeutic treatment by wound.
electrical introduction of ions into the body tissue. ∙ Metastasis: It is defined as spread of tumor by invasion in
∙ Ischemia: It refers to the deficiency in the blood supply to a such a way that discontinuous secondary tumor mass/masses
part or an organ which may be due to constriction, contraction arc formed at the site of lodgment.
or blocking of the arteries. ∙ Metaplasia: It is a reversible change in which one adult cell
∙ Isograft: It refers to a graft derived from one member of a type is replaced by another adult cell type.
pair of monozygotic twins and transplanted to the other. ∙ Mesiodens: It refers to supernumerary tooth located at or
∙ Jaw winking: It refers to a movement of the lower jaw near the midline in the incisal region of maxilla between the
causing an involuntary movement of the eyelids. central incisors.
∙ Joint: The place of connection between two bones, allowing ∙ Medicine: It refers to the study and treatment of diseases
of more or less movement an articulation. especially treatment without recourse to surgery or any drug
∙ Keloid: It refers to a fibrous hyperplastic scar growth on the used for the treatment of the disease.
skin. ∙ Metabolism: It refers to the physical and chemical changes
∙ Kernicterus: Staining of brain tissue cause by accumulation in the tissue by which a living body is maintained and energy
of unconjugated bilirubin in the brain. generated.
∙ Knitting: It refers to the process of repair of a bone fracture. ∙ Mitosis: It is the indirect division of cells, a typical method
∙ Lain’s disease: Burning of the tongue and the soft tissue of cell reproduction.
of the mouth due to electrogalvanism caused by the use of ∙ Mucocele: It is a term used to describe swelling caused by
dissimilar metals in dental restoration. pooling of saliva at the site of injured minor salivary gland.
∙ Lancinating: It is the term used to describe shooting, tearing ∙ Muscle: It is a contractile organ by means of which movement
or sharply cutting type of pain. is produced in an animal organism.
∙ Leukoplakia: A white patch or plaque that cannot be scraped ∙ Muscle spasm: It refers to a sudden involuntary contraction
off and cannot be characterized clinically or pathologically as of the muscle or group of muscles attended by pain and
any other disease, which is more than 5 mm. interference with function.
∙ Lesion: A wound or injury or a patch of disease on the skin. ∙ Mucus plug: These are incompletely mineralized sialoliths.
A morbid change in tissue function. ∙ Natal teeth: These are teeth which are observed in the oral
∙ Lichen planus: Relatively common dermatitis occurs on cavities at birth.
skin and oral mucous membrane and refers to a lace-like ∙ Narcosis: A state of profound unconsciousness or stupor
pattern produced by symbolic algal and fungal colonies on produced by drugs.
the surface of rocks in nature. ∙ Nausea: A feeling of sickness or a tendency to vomit.
∙ Lipomatosis: It refers to excessive localized accumulation of ∙ Necrosis: It the sum of the morphologic changes that follow
fats in the tissues. cell death in a living tissue or organs.
∙ Localized: Lesion or condition happening within one small ∙ Neonatal teeth: These are teeth which erupt during the first
area. 30 days of life.
∙ Lateral: Away from midline. ∙ Neoplasia: It is an abnormal mass of tissue, the growth of
∙ Ludwig’s angina: This condition may be defined as an which exceeds and is un-coordinate with that of normal tissue
overwhelming rapidly spreading septic cellulitis involving and persists in the same excessive manner after cessation of
submandibular, submental and sublingual space bilaterally. stimuli which evoke the changes.
∙ Lymph: It refers to the clear fluid found in the lymphatics ∙ Neurotropic: Attracted to or having an affinity for nervous
vessels. tissue.
∙ Lymphadenitis: It refers to the inflammation of the lymph ∙ Nevus: It is circumscribed new growth of skin or oral mucosa
nodes. of congenital origin, presenting as small, elevated, or flat
∙ Macule: Well circumscribed flat lesion that is noticeable due pigmented lesion.
to the change from the normal skin color to red may be due ∙ Nodules: This lesion is present deep in the dermis and
to inflammation or pigmented due to presence of melanin epidermis and can be moved easily over them.
hemosiderin or other drugs. ∙ Nociceptive: Relating to any pain producing stimulus, or to
∙ Maceration: It refers to the softening of a substance by pain receptor nerves.
soaking in a liquid. ∙ Nosology: It refers to the science of classification of disease.
Appendices
∙ Numbness: Partial or total loss of sensation which may be ∙ Paresthesia: The term refers to perverted sensation like
deliberately induced as in cases of local anesthesia or it may burning, prickling or crawling sensation of the skin.
be pathological. ∙ Parageusia: It refers to an unpleasant taste in the mouth.
∙ Nutrition: It refers to the process by which food is ∙ Parakeratosis: It refers to any abnormality of the stratum 947
assimilated. corneum of the epidermis, which may be associated with
∙ Ointment: It refers to a fatty semisolid substance used as a inflammation of the prickle cell layers causing defective
base for local medicaments for external application. formation of keratin and characterized by the persistence
∙ Oligodontia: It is agenesis of a few numbers of teeth. nuclei.
∙ Oncology: It refers to the study of neoplasm. ∙ Paralysis: It is the loss or impairment of muscle function or
∙ Operation: Anything performed, especially any procedure of sensation due to nerve injury or destruction of neurons.
by a surgeon, either with instruments or by hand. ∙ Pararhizoclasia: It is the inflammatory ulcerative destruction
∙ Oroantral opening: The accidental opening in the floor of of the deep layers of tissue and the alveolar process about the
maxillary sinus during dental extraction is called oroantral root of a tooth.
opening. ∙ Parenteral: Descriptive of methods of drug administration
∙ Oral submucus fibrosis: An insidious chronic disease other than by the alimentary canal.
affecting any part of the oral cavity and sometimes the ∙ Parodontal: Near or next to a tooth sometimes used as
pharynx, proceeded by and/or associated with vesicle synonymous with periodontal.
formation, it is always associated with juxtraepithelial ∙ Parrot tongue: A horny, dry tongue which cannot be
inflammatory reaction followed by fibroelastic change of the protruded, seen in typhus and low fever is called parrot
lamina propria, with epithelial atrophy leading to stiffness of tongue.
the oral mucosa and causing trismus and inability to eat. ∙ Pathogen: Any agent that produces or is able to produce
∙ Oral medicine: It is that area of dental practice which deals disease.
with diagnosis and treatment of oral disease by nonsurgical ∙ Parulis: It is mass of granulation tissue which covers the
means, which may be localized in the oral cavity or which opening of a sinus.
may be oral manifestation of systemic disease and those ∙ Pathogenesis: The development of disease from its inception
phases of dental practice concerned with diagnosis and to the appearance of characteristic symptoms or lesions.
treatment of medically compromised patients. ∙ Pathognomonic: Characteristic of one specific disease or
∙ Organ: It refers to any separate part of the body having a pathological condition as distinct from any others.
specific function. ∙ Pathology: That branch of medicine which is concerned with
∙ Organism: It refers to any individual plant or animal or an the structural and functional changes caused by disease.
organized body of living cells. ∙ Pedunculated: Describing the tumor or growth whose base
∙ Osteomyelitis: It is an inflammation of bone marrow that is narrower than the widest part of lesion.
produce clinically apparent pus and secondarily affect the ∙ Peridens: Supernumerary teeth that are erupted ectopically
calcified component Or It is an infection of bone that involves either buccally or lingually to the normal arch referred to as
all three components periosteum, cortex, and marrow Or It peridens.
may be defined as an inflammatory condition of the bone that ∙ Petechiae: Purpuric lesions 1 to 2 cm in diameter.
begins as an infection of medullary cavity and the Haversian ∙ Permeation: The spreading or extension through tissues or
system which extends to involve the periosteum of the organs, used especially of malignant tumors extending by
affected area. continuous growth through the lymphatics.
∙ Papillary: Describing the tumor or growth exhibiting ∙ Pericemental abscess: A parodental abscess not arising from
numerous surface projection. a diseased pulp or an extension of the periodontal pocket.
∙ Papules: These are solid lesions raised above the skin surface ∙ Percussion: Listening to the tapping note with a finger
that are smaller than 1 cm in diameter. placed on the affected part or in cases of teeth with the help
∙ Pathogenecity: It is the ability of microbial species to of handle of the probe.
produce disease. ∙ Periodontitis: It is name given to periodontal disease when
∙ Paramolar: It is a supernumerary molar usually small and the superficial inflammation in the gingival tissue extends into
rudimentary which is situated buccally or lingually to one of the underlying alveolar bone and there is loss of attachment.
maxillary molars or inter-proximally between 1st, 2nd and ∙ Periodontosis: Chronic noninflammatory destruction of
3rd maxillary molars. periodontal ligament and the associated alveolar bone.
∙ Palpation: It refers to feeling of the affected part by hand. ∙ Phoenix abscess: It an acute exacerbation of a chronic or
∙ Pain: It refers to the distressing or unpleasant sensation suppurative apical periodontitis.
transmitted by a sensory nerve usually indicative of injury ∙ Phlegmon: Acute inflammation of the subcutaneous
or of disease. connective tissue.
∙ Palliation: It refers to the act of alleviating or affording relief ∙ Pit: It is defined as hollow fovea or indent blind tracts lined
without curing. with epithelium.
∙ Pilation: It is a hair like fracture found in cranial bones. ∙ Psychosomatic: Relating to the mind and the body;
∙ Plasmapheresis: It is a method of increasing the number particularly relating to the interdependence of mental
of blood cells in the blood count. From the blood plasma is processes and bodily function.
948 skimmed out simply on standing and remaining concentrate ∙ Pustule: It refers to a raised lesion containing purulent
is reinfused into the patient. material.
∙ Plaque: It is a solid raised lesion that is over 1cm in diameter. ∙ Purpura: It refers to reddish to purple flat lesion caused by
∙ Pleomorphic: The word pleomorphic means occurring in blood extravasated from a vessel leaking into subcutaneous
several distinct shapes. tissue.
∙ Pleurodont: Having teeth attached to the side of a bony ∙ Pulsation: It is the rhythmic throb or beating as that of the
socket or to the side of the jaw. heart.
∙ Plexus: A plexus of nerves or a network of blood or lymphatic ∙ Pulse: The expansion and contraction of an artery due to
vessels. increased tension of its walls following contraction of the
∙ Pocket: It is an abnormal space developing between the tooth heart and subsequent relaxation.
root and the gums. ∙ Pus: It is a liquid usually yellowish in color formed in certain
∙ Poison: Any substance that when absorbed into the system infection and composed of tissue fluid containing bacteria
of a living body is liable to cause injury and to endanger life. and leukocytes.
∙ Poikiloderma: It refers to a combination of atrophy, ∙ Putrefaction: The decomposition of organic matter through the
telangiectasia and pigmentary changes. action of microorganisms, resulting in the production of various
∙ Polylophodont: These are teeth with multi-ridged crowns. solid and liquid compounds and gases giving off a foul odor.
∙ Pre-cancerous lesion: Morphologically altered tissue ∙ Pyemia: Generalized septicemia caused by pyogenic
in which cancer is more likely to occur than its normal microorganism in the blood stream and marked by the
counterpart. formation of multiple abscesses.
∙ Pre-cancerous condition: It is a generalized state associated ∙ Ranula: The term ranula is used for a mucocele occurring
with a significantly increased risk of cancer. in the floor of mouth in association with ducts of the
∙ Premedication: The administration of drugs or sedatives submandibular or sublingual glands.
before treatment, to help in patient management especially ∙ Radiology: It is that branch of health sciences dealing with
with nervous patient. radioactive substances and radiant energy and with the
∙ Prescribe: To write instruction for the preparation, diagnosis and treatment of disease by means of both ionizing
composition and administration of a medicine. (X-rays) and non-ionizing (ultrasound) radiations.
∙ Prevalence: The number of cases of a disease at any given ∙ Radiolucent: Offering little resistance to X-rays in
time in any given place. radiography; almost transparent.
∙ Priestley’s mass: A green or brown stain on the anterior teeth ∙ Rash: It refers to a temporary cutaneous eruption.
of the young or where reduced enamel epithelium remains ∙ Recrudescence: The return of symptoms or the recurrence of
over the enamel. the disease after a temporary remission.
∙ Procheilia: It is the condition in which one lip protrudes ∙ Recurrence: The return of symptoms or of a disease after a
forwards of its normal position. period of remission.
∙ Prognosis: It is the prediction of the course, duration, and ∙ Regurgitation: The return of undigested or partially digested
termination of the disease and the likelihood of its response food from the stomach or esophagus to the mouth or of fluid
to therapy. or semifluid to the nose.
∙ Prosthesis: The word prosthesis is used for a manufactured ∙ Reticular: Relating to net or net like structure.
appliance used to take the place of a natural part or to correct ∙ Regeneration: It is replacement of injured tissue by
a congenital abnormality or it may be defined as an appliance parenchymal cells of the same types.
which replaces lost or congenitally missing tissue. ∙ Retrogenia: It refers to a condition in which chin is set back
∙ Proteolysis: The process of digestion of proteins and its in relation to the rest of the facial skeleton.
conversion by enzymes into peptones, proteoses, etc. ∙ Rh hump: In the deciduous 1st molar crown a characteristic
∙ Protuberance: The word protuberances refers to a swelling, ring like defect may be seen which is called Rh hump.
eminence or knob of the tissue. ∙ Rhinorrhea: It refers to any discharge of fluid from the nose.
∙ Pseudomembrane: It refers to a false membrane, a skin like ∙ Rhizotomy: A surgical division of either a tooth root or a
layer formed by fibrinous exudates containing leukocytes nerve root.
and bacteria. ∙ Root dehiscence: It is a pathological condition in which
∙ Pseudoepitheliomatous hyperplasia: In this conditions the vestibular surface of the tooth root is exposed to the oral
the rete pegs extend far downward, usually accompanied cavity over some or all of the apical two-thirds of its length.
by acanthosis. The cells are normal in size, shape and ∙ Rudiment: It refers to an organ or part either imperfectly
chromaticity. developed or at an early stage of development.
Appendices
∙ Rubber jaw: In this condition it is possible to mold the shape ∙ Spongiosis: This term is used to signify intercellular edema
of the jaw with the fingers, but teeth will resume its position of the epithelium, in which intercellular bridges of the stratum
when the pressure is released. spinosum become more prominent.
∙ Satellite abscess: It is a secondary abscess arising from and ∙ Stagnation: It refers to the cessation of flow of any circulating 949
situated near a primary abscess. fluid in the body.
∙ Saburra: It refers to a foul condition of the mouth and teeth ∙ Sterilization: It is the process of destruction of the microbial
or of the stomach due to food debris. life from an article or surface inclusive of bacterial spores.
∙ Saucerization: It is the wide and shallow depression ∙ Sterile abscess: It refers to an abscess containing no
occurring about a wound or bone cavity as in osteomyelitis. microorganisms.
∙ Sclerosis: It refers to hardening of vessels or part applied ∙ Stenosis: It is the constriction or narrowing of an aperture
particularly to arteries and to proliferation of connective canal or duct.
tissue in the nervous system as a result of degeneration. ∙ Stimulus: It refers to any agent or impulse that excites or
∙ Scale: Loosened imperfectly cornified parakeratotic promotes a functional reaction.
superficial layer of skin that is shed as fine, brawny, dirty ∙ Stippled: Having a mottled or spotted appearance with light
white, yellowish keratinous dust or large pearly white flakes. and dark patches.
∙ Sequestra: Small pieces of necrotic bone which are avascular ∙ Stomatology: The medical speciality concerned with the
and which harbor microorganisms are known as sequestra. mouth and its diseases sometimes used synonymously with
∙ Serum: If blood is allowed to clot an amber colored liquid dentistry.
which remains after separation of the clot is known as serum. ∙ Striation: It is a stripe or streak or a series of stripes or
∙ Septicemia: The word septicemia implies a overwhelming streaks.
bacterial proliferation and release of toxins in the blood. ∙ Stricture: It is an abnormal contraction of any aperture or
∙ Sessile: It describe a tumor or growth whose base is the vessels.
widest part of the lesion. ∙ Stridor: It is a harsh whistling sound produced by the
∙ Shock: It is state of inadequate perfusion of all cells and respiratory system.
tissues, which at first leads to reversible hypoxic injury, but if ∙ Superficially invasive (micro invasive) squamous cell
sufficiently protracted or grave, to irreversible cell and organ carcinoma: A histopathological diagnosis of a routine
injury and sometimes to the death of the patient. squamous cell carcinoma, usually well differentiated, which
∙ Sickle cell anemia: In homozygous individuals the whole of has invaded only slightly into the underlying connective
HbA (hemoglobin A) is replaced by HbS (hemoglobin S, i.e. tissues.
an abnormal hemoglobin) and this is known as sickle cell ∙ Subluxation (hypermobility): It is the unilateral or
disease. bilateral positioning of the condyle anterior to the articular
∙ Sickle cell trait: In heterozygous individuals only 50% of eminence, with repositioning to normal to accomplish normal
HbA is replaced by HbS and this is known as sickle cell trait. physiologic activity.
∙ Sinus: It is a blind tract leading from the surface down to the ∙ Subscription: It is a part of a prescription containing direction
tissue which is lined by granulation tissue or which may be for the preparation and compounding of the ingredients of a
epithelized. medicine.
∙ Sialorrhea (ptyalism): An increased salivary secretion is ∙ Suzanne’s gland: An oral mucous gland found in the
termed as sialorrhea or ptyalism. alveolo-lingual sulcus near the midline.
∙ Sialolithiasis: It is the formation of calcific concretions ∙ Symbiosis: It is the intimate association of two organism of
within parenchyma or ductal system of major or minor different species.
salivary glands. ∙ Symptoms: Any indication of the presence or course of a
∙ Sinusitis: Inflammation of mucosa of paranasal sinuses is disease either by functional or other changes occurring in the
referred to as sinusitis. When maxillary sinus is involved it is patient.
called maxillary sinusitis. ∙ Syncope: It is a transient loss of consciousness caused by
∙ Sialoschesis: It is the suppression of the secretion of the cerebral hypoxia or changes in cerebral blood flow.
salivary glands. ∙ Syndesmosis: It is the joining of two bone surfaces by the
∙ Sign: It defined as any change in the body or its function interposition of connective tissue which forms an interosseous
which is perceptible to a trained observer and may indicate a membrane.
specific disease. ∙ Syndrome: A complex of symptoms, occurring together,
∙ SLE (systemic lupus erythematous): It is characterized which characterize one disease or lesion.
by the presence of abnormal serum antibodies and immune ∙ Talon’s cusp: It projects lingually from the cingulum area of
complexes. maxillary and mandibular teeth Or It is anomalous hyperplasia
∙ Slough: It refers to the necrotizing tissue that scales or peels of cingulum on the lingual of maxillary and mandibular
off in ulcerative conditions. incisor resulting in the formation of a supernumerary cusp.
∙ Taurodontism: Body of tooth is enlarge at the expense of ∙ Ulcer: Deep craters that extends through the entire thickness
root. It is characterized by clinical and anatomical crown of of the surface epithelium and involve the underlying
normal shape and size, an elongated body and short root with connective tissue Or Defect in epithelium, it is a well
950 a longitudinally enlarged pulp chamber. circumscribed depressed lesion over which epidermal layer
∙ Tablet: It is a small solid disk containing one dose of a drug. is lost.
∙ Tapir mouth: It is a condition characterized by loose thickened ∙ Unfavorable fracture: If the associated muscle tends to pull
lips and caused by atrophy of the orbicularis oris muscle. the fragment of the fracture, it is described an unfavorable.
∙ Taste: The perception of flavor, a sensation produced by ∙ Uvuloptosis: It is a relaxed dropped position of palatine
stimulation of the gustatory nerve endings in the tongue with uvula.
a soluble substance. ∙ Vaccine: It is any material used for preventive inoculation
∙ Telangiectasia: It is the dilatation of the capillaries and small against a specific disease.
arteries forming types of angiomas. ∙ Vallate: Having a surrounding wall or rim.
∙ Teratoma: It is true neoplasm made up of a number of ∙ Varicosity: It refers to a distended vein, superficial, bluish
different types of tissue which are not native to the area in and painless.
which the tumor occurs. ∙ Vasodilator: The term refers to a nerve or some external
∙ Thalassemia: It is an inherited impairment of hemoglobin agent which cause vascular dilatation.
synthesis in which there is partial or complete failure to ∙ Vesicle: These are elevated blisters containing clear fluid that
synthesize a specific type of globin chain. are under 1 cm in diameter.
∙ Therapy: It refers to the treatment of disease. ∙ Vertigo: It is a sensation of loss of equilibrium in which
∙ Thermocautery: It is the use of head points for cauterization. sufferers feel either that the world is revolving round them or
∙ Tic: A spasmodic twitching particularly of the facial muscles; that they are revolving in space.
a habit spasm. ∙ Verrucous: Describing the tumor or growth exhibiting a
∙ Tinnitus: The term refers to a ringing noise in the ears. rough and warty surface.
∙ Toxin: It is a poisonous substance produced by animal or ∙ Virulence: It is a term applied to the properties in a particular
vegetable cells more particularly by bacteria. strain of microorganism.
∙ Trabeculae: It refers to a septum extending from the outer ∙ Vicarious: Relating to a normal process occurring in an
capsule or envelop into an organ. abnormal position or under abnormal conditions.
∙ Trephone: These are substances prepared by leukocytes ∙ Virus: A complex organic particle of submicroscopic
from the plasma protein which is necessary for nourishment dimensions capable of growth and reproduction only within
of tissue cell. the cells of the host organism it infects.
∙ Transplantation: It is the transfer of tissue either from ∙ Wasting disease of teeth: It is defined as any gradual loss
another donor or from one site to another. of tooth substance characterized by the formation of smooth
∙ Transposition: It is the interchange in position of two polished surfaces without ragged to the possible mechanism
adjacent teeth. of this loss.
∙ Treatment: It is the means used to combat or cure a disease. ∙ Wandering teeth: It is movement of unerupted teeth for no
∙ Trignodent: A tooth having three cusps in the form of a apparent reasons (distal drift).
triangle. ∙ Wandering abscess: An abscess that tracks through the
∙ Trismus: It is the inability to open the mouth because of tissue and finally comes to a point some distance form the
tonic spasm of the jaw muscles. original site.
∙ Tropics: It is that portion of the surface of the globe where ∙ Weal: It is a reddish, raised and circumscribed lesion on the
the sun passes directly overhead. skin, generally caused by a blow or a bite.
∙ Tumor: It is an autonomous new growth of tissue or it is an ∙ Working diagnosis: Following reappraisal of diagnostic data
abnormal mass of tissue the growth of which exceeds and at hand including those of follow-up examination which may
is uncoordinated with that of normal tissue and persists in be seen necessary and which may provide new relevant finding
the same excessive manner even after the cessation of stimuli and indicating result from any additional diagnostic procedure.
which evoked changes. ∙ Wound: It is any injury to the tissues or organs cause by
∙ Tubercle: It is a rounded eminence on bone. cut, stab or tear, usually going deeper than the outer skin or
∙ Tumefaction: It is the state of being or becoming swollen. integument.
∙ Twining: It indicates the cleavage of tooth germ into two ∙ Wolf’s law: This law states that, the bone structure depends
complete resulting in formation of supernumerary teeth that is on the strain and stresses to which bone is subjected.
mirror image or near image of tooth from which it has developed. ∙ Xerostomia: It is a subjective clinical condition of less than
∙ Tyndallization: A method of sterilizing culture media by normal amount of saliva.
exposure to steam at 100 degree Celsius on three successive ∙ Xenograft: It is a type of graft derived from a donor of
days for: About 30 minutes each day. different species.
Appendices
APPENDIX III: NORMAL VALUES OF VARIOUS LABORATORY PARAMETERS

Smruti Nanda
HEMATOLOGY
Red Blood Cells
RBC (Male) 4.2-5.6 M/ pL
RBC (Female) 3.8-5.1 M/ pL
RBC (Child) 3.5-5.0 M/ pL
White Blood Cells
WBC (Male) 3.8-11.0 K/mm cubed
WBC (Female) 3.8-11.0 K /mm cubed
WBC (Child) 5.0-10.0 K /mm cubed
Hemoglobin
Hb (Male) 14-18 g/dL
Hb (Female) 12-16 g /dL
Hb (child) 10-14 g /dL
Hb (Newborn) 15-25 g /dL
Hematocrit
Hct (Male) 39-54%
Hct (Female) 34-47%
Hct (Child) 30-42%
MCV 78-98 fL
MCH 27-35 pg
MCHC 31-37%
Neutrophils 50-70 %
Bands neutrophils 0-6%
Lymphocytes 20-40 %
Monocytes 2-8%
Eosinophils 1-5%
Basophils 0-1%
GENERAL CHEMISTRY
Amylase 50-150 U/dL
Bilirubin, Direct 0.1-0.3 mg/dL
Bilirubin, Indirect 0.2-0.7 mg/dL
Bilirubin, Total 0.3-1.0 mg/dL
BUN 8-18 mg/dL
Calcium (Total) 8-11 mg /dL
Creatine (Male) 0.2-0.6 mg/dL
Creatine (Female) 0.6-1.0 mg/dL
Creatinine 0.6-1.5 mg/dL
Glucose 70-110 mg/dL (diuresis
greater than or equal to
180 mg/dL )
Iron 50-150 pg/dL
Iron binding capacity 250-370 pg/dL
Phosphorus 2.2-4.8 mg/dL
Potassium 3.5-5.5 mEq/L
Appendices
APPENDIX III: NORMAL VALUES OF VARIOUS LABORATORY PARAMETERS

Smruti Nanda
951
HEMATOLOGY
Red Blood Cells
RBC (Male) 4.2–5.6 M/µL
RBC (Female) 3.8–5.1 M/µL
RBC (Child) 3.5–5.0 M/µL
White Blood Cells
WBC (Male) 3.8–11.0 K/mm cubed
WBC (Female) 3.8–11.0 K/mm cubed
WBC (Child) 5.0–10.0 K/mm cubed
Hemoglobin
Hb (Male) 14–18 g/dL
Hb (Female) 12–16 g/dL
Hb (child) 10–14 g/dL
Hb (Newborn) 15–25 g/dL
Hematocrit
Hct (Male) 39–54%
Hct (Female) 34–47%
Hct (Child) 30–42%
MCV 78–98 fL
MCH 27–35 pg
MCHC 31–37%
Neutrophils 50–70%
Bands neutrophils 0–6%
Lymphocytes 20–40%
Monocytes 2–8%
Eosinophils 1–5%
Basophils 0–1%
GENERAL CHEMISTRY
Amylase 50–150 U/dL
Bilirubin, Direct 0.1–0.3 mg/dL
Bilirubin, Indirect 0.2–0.7 mg/dL
Bilirubin, Total 0.3–1.0 mg/dL
BUN 8–18 mg/dL
Calcium (Total) 8–11 mg/dL
Creatine (Male) 0.2–0.6 mg/dL
Creatine (Female) 0.6–1.0 mg/dL
Creatinine 0.6–1.5 mg/dL
Glucose 70–110 mg/dL (diuresis
greater than or equal to
180 mg/dL)
Iron 50–150 µg/dL
Iron binding capacity 250–370 µg/dL
Phosphorus 2.2–4.8 mg/dL
Potassium 3.5–5.5 mEq/L
Protein (Total) 5.5–8 gm/dL

SGPT 8–32 U/L
Sodium 135–148 mEq/L
952 T3 0.8–1.1 µg/dL
Thyroglobulin Less than 55 ng/mL
Thyroxine (T4) total 5–13 µg/dL
Urea nitrogen 8–25 mg/dL
Uric acid (Male) 3.5–7.7 mg/dL
Uric acid (Female) 6.6 mg/dL
LIPID PANEL (ADULT)
Cholesterol (Total) Less than 200 mg/dL desirable
Cholesterol (HDL) 30–75 mg/dL
Cholesterol (LDL) Less than 130 mg/dL desirable
Triglycerides (Male) Greater than 40–170 mg/dL
Triglycerides (Female) Greater than 35–135 mg/dL
URINE
Color Straw
Specific Gravity 1.003–1.040
pH 4.6–8.0
Na 10–40 mEq/L
K Less than 8 mEq/L
Cl Less than 8 mEq/L
Protein 1–15 mg/dL
Osmolality 80–1300 mOsm/L
COAGULATION
APTT 21–35 seconds
Platelets 150,000–450,000/mm3
Plasminogen 62–130%
PT 10–14 seconds
PTT 32–45 seconds
FSP Less than 10 µg/dL
Fibrinogen 200–400 mg/dL
Bleeding time 2–6 minutes
Thrombin time 9–13.5 seconds
Appendices
APPENDIX IV: CLASSIFICATION SYSTEMS OF ODONTOGENIC TUMOR

Satish Chhugani
i . Broca ' s Classification (1869)

ii . Malassez Classification (1885)
hi . Bland-Sutton Classification (1888)
lv . Gabell , James, and Payne Classification (1914 )
v . Thoma and Goldman Classification (1946)
vi . Pmdborg and Clausen Classification (1958)
vii . WHO Classification (1971)
viii . WHO Classification (1992)
IX . WHO Classification (2002) [ Revised Version]
x . WHO Classification (2005)
Broca’s Classification [1869 ]

• In 1869, the French Physician and Professor of pathology and clinical surgery , Pierre Paul Broca proposed a classification of
tumor originating from dental tissue .
• Coined the term “ Odontome ” .
• Classified the lesions according to the stage of development of the tooth when abnormal growth commenced.
Malassez Classification [1885]

• Louis Charles Malassez made minor modifications to Broca’s classification with no major changes in the classification.
• Introduced “ Epithelial Rests of Malassez ” .
Bland-Sutton Classification [1888 ]

• Classification based upon the type of the particular cells of the tooth germ from which the tumor arose .
• Included odontogenic cyst and fibrous osteogenic tumors in the classification .
• Subdivided the odontome into those arising from :
a . Aberrations of the enamel organ .
b . Aberrations of the follicle, papillae .
c . Aberrations of the whole tooth germ .
d . Anomalous odontomes .
Gabell , Janies and Payne Classification [1914]

• Modified Bland-Sutton classification .
• Used the term “ odontome ” for all odontogenic tumors .
• Included radicular cyst and dentigerous cyst as odontome .
• Recognized 3 mam groups of odontomes :
1. Epithelial odontomes: Included neoplasm known at the time as a ‘ multilocular cyst’ as well as ‘non- neoplastic cysts’ .
2 . Composite odontomes: Lesions derived from epithelium and connective tissue and formed either irregular calcified masses
or recognizable tooth - like structures .
3 . Connective tissue odontome: Fibrous and other connective tissue tumors thought to arise from dental mesenchyme only .

Thoma and Goldman Classification [1946]

• dontogenic cysts introduced by Bland-Sutton were excluded.
O
• Enamel Pearls being developmental malformations were considered tumors under the name of “Enamelomas”.
954
• Classification was amplified by American Academy of Oral Pathology, and ran as follows:
I. Epithelial tumors:
– Adamantoblastoma
– Enameloma
II. Mesenchymal tumors:
– Odontogenic fibroma
– Dentinoma
– Cementoma
III. Mixed tumors: (Odontomas)
– Soft odontoma
– Soft and calcified odontoma
– Completely formed odontoma with enamel, dentin, pulp, cementum, periodontal mem brane
- Compound (many small teeth)
- Complex (irregular tooth structure)
Pindborg and Clausen Classification [1958]

The classification was based on the characteristic reciprocal epithelial mesenchymal interaction occurring during normal tooth
development.
Tumors were classified into two main groups:
Epithelial tumors:
a. Epithelial tumors with no inductive changes in the connective tissue:
• Ameloblastoma
• Calcifying epithelial odontogenic tumor
b. Epithelial tumors that do show inductive changes in the mesenchyme:
• Ameloblastic fibroma
• Dentinoma
• Odontomas
Mesodermal tumors:
• Odontogenic fibroma
• Odontogenic myxoma
• Cementifying fibroma
Appendices
WHO (World Health Organization) CLASSIFICATION [ 1971]

In 1971, the first authoritative and useful guide to the classification of odontogenic tumors was published by WHO, authored
by Pindborg and Kramer.
Benign Malignant 955
• Ameloblastoma Odontogenic carcinomas:
• Calcifying epithelial odontogenic tumor • Malignant Ameloblastoma
• Ameloblastic fibroma • Primary intraosseous carcinoma
• Adenomatoid odontogenic tumor • Other carcinomas arising from odontogenic epithelium,
• Calcifying odontogenic cyst including those arising from odontogenic cysts.
• Dentinoma Odontogenic sarcomas:
• Ameloblastic fibro-odontoma • Ameloblastic fibrosarcoma
• Complex odontoma • Ameloblastic odontosarcoma
• Fibroma (odontogenic fibroma)
• Myxoma (myxofibroma)
• Cementomas:
– Benign cementoblastoma
– Cementifying fibroma
– Periapical cemental dysplasia
– Gigantiform cementoma
– Melanotic neuroectodermal tumor of infancy
WHO CLASSIFICATION [1992]

In 1992, a second edition entitled: “Histological Typing of Odontogenic Tumors” appeared. The authors of the first edition
were joined by a third author, Professor Mervyn Shear in this edition.
Benign Malignant
Odontogenic epithelium without odontogenic ectomesenchyme Odontogenic carcinomas:
• Ameloblastoma • Malignant ameloblastoma
• Squamous odontogenic tumor • Primary intraosseous carcinoma
• Calcifying epithelial odontogenic tumor • Malignant variants of other odontogenic epithelial tumors
• Clear cell odontogenic tumor • Malignant changes in odontogenic cysts
Odontogenic epithelium with odontogenic ectomesenchyme, Odontogenic sarcomas:
with or without dental hard tissue formation • Ameloblastic fibrosarcoma
• Ameloblastic fibroma • Ameloblastic fibrodentinosarcoma and ameloblastic fibro-
• Ameloblastic fibrodentinoma and ameloblastic fibro- odontosarcoma
odontoma. Odontogenic carcinosarcoma
• Odontoameloblastoma
• Adenomatoid odontogenic tumor
• Calcifying odontogenic cyst
• Complex odontoma
• Compound odontoma
Odontogenic ectomesenchyme with or without included
odontogenic epithelium
• Odontogenic fibroma
• Myxoma (odontogenic myxoma, myxofibroma)
• Benign cementoblastoma
WHO CLASSIFICATION [2002] (Revised Version By Philipsen and Reichart)

Revision and updating of the WHO classification was done by Philipsen and Reichart in 2002.
956 Benign Malignant
Odontogenic epithelium with mature, fibrous stroma; Odontogenic carcinomas:
odontogenic ectomesenchyme not present • Malignant (metastasizing) ameloblastoma
• Ameloblastomas • Ameloblastic carcinoma:
– Intraosseous (Infiltrative) – Primary
– Extraosseous (Peripheral) – Carcinoma in intraosseous ameloblastoma (dedifferen-
– Desmoplastic tiated)
– Unicystic – Carcinoma in peripheral ameloblastoma
• Squamous odontogenic tumor • Primary intraosseous squamous cell carcinoma:
• Calcifying epithelial odontogenic tumor – Solid
• Adenomatoid odontogenic tumor. – Cystogenic:
Odontogenic epithelium with odontogenic ectomesenchyme, - Non-keratinizing cyst
with or without dental hard tissue formation - Odontogenic keratocyst
• Ameloblastic fibroma and fibrodentinoma (neoplastic) • Calcifying ghost cell odontogenic carcinoma (malignant
• Ameloblastic fibrodentinoma (non-neoplastic) epithelial odontogenic ghost cell tumor)
• Ameloblastic fibro-odontoma • Clear cell odontogenic carcinoma
• Complex odontoma Odontogenic sarcomas :
• Compound odontoma • Ameloblastic fibrosarcoma
• Odontoameloblastoma • Ameloblastic fibrodentino- and fibro-odontosarcoma
• Calcifying ghost cell odontogenic tumor • Odontogenic carcinosarcoma
Mesenchyme and/or odontogenic ectomesenchyme with or
without included odontogenic epithelium
• Odontogenic fibroma (simple)
• Odontogenic fibroma (WHO-type)
• Odontogenic myxoma or myxofibroma
• Benign cementoblastoma
Appendices
WHO CLASSIFICATION [2005]

Classification was approved at the Editorial and Consensus Conference held in Lyon, France (WHO/ IARC), in July 2003 in
conjunction with the preparation of the new WHO Blue Book Volume “Pathology and Genetics of Tumors of the Head and
Neck”. This new WHO Blue Book encompasses both histopathological and genetic criteria for tumor classification. 957
Benign Malignant
Odontogenic epithelium with mature, fibrous stroma; odontogenic Odontogenic carcinomas:
ectomesenchyme not present • Metastasizing , malignant ameloblastoma
• Ameloblastomas: • Ameloblastic carcinoma:
– solid/multicystic – primary
– extraosseous/peripheral – secondary (dedifferentiated), intraosseous
– desmoplastic – secondary (dedifferentiated), extraosseous
– unicystic • Primary intraosseous squamous cell carcinoma (PIOSCC):
• Squamous odontogenic tumor – PIOSCC solid type
• Adenomatoid odontogenic tumor – PIOSCC derived from odontogenic cysts
• Calcifying epithelial odontogenic tumor – PIOSCC derived from keratinizing cystic odontogenic
• Keratinizing cystic odontogenic tumor tumor
• Clear cell odontogenic carcinoma
Odontogenic epithelium with odontogenic ectomesenchyme,
• Ghost cell odontogenic carcinoma
with or without dental hard tissue formation
• Ameloblastic fibroma Odontogenic sarcomas:
• Ameloblastic fibrodentinoma • Ameloblastic fibrosarcoma
• Ameloblastic fibro-odontoma • Ameloblastic fibrodentino and fibro-odontosarcoma
• Complex odontoma
• Compound odontoma
• Odontoameloblastoma
• Calcifying cystic odontogenic tumor
• Dentinogenic ghost cell tumor
Mesenchyme and/or odontogenic ectomesenchyme with or
without included odontogenic epithelium
• Odontogenic fibroma (epithelium–poor and epithelium–rich
types)
• Odontogenic myxoma or myxofibroma
• Cementoblastoma
CLASSIFICATION OF ODONTOGENIC TUMOR

Benign B] Mesenchymal
A] Epithelial Odontogenic myxoma
With inductive changes in the connective tissue Odontogenic fibroma
AOT Cementoma
Ameloblastofibroma Periapical cemental dysplasia
Dentinoma Cementifying fibroma
Calcifying odontogenic tumor Benign cementoblastoma
Odonto-ameloblastoma Malignant
Odontoma A] Epithelial
Without inductive changes in the connective tissue With inductive changes in connective tissue
ameloblastoma Ameloblastic fibrosarcoma
CEOT Ameloblastic odontosarcoma
Epithelial atypia Without inductive changes in connective tissue
Ameloblastic changes in odontogenic cyst Malignant ameloblastoma
Primary intraosseous carcinoma
Malignant changes in odontogenic cyst
NON-ODONTOGENIC NEOPLASMS OF THE JAWS
958 Benign Malignant

• Ossifying fibroma • Osteosarcoma
• Fibrous dysplasia • Chondrosarcoma
• Osteoblastoma • Mesenchymal chondrosarcoma
• Osteoid osteoma • Ewing sarcoma
• Chondroma • Burkitt lymphoma
• Myxoma • Multiple myeloma
• Chondroblastoma • Solitary plasmacytoma of bone
• Chondromyxoid fibroma • Metastatic carcinoma
• Osteoma • Neurogenic sarcoma
• Osteoid osteoma • Kaposi sarcoma
• Central giant cell granuloma • Malignant hemangioendothelioma
• Giant cell tumor • Primary lymphoma of bone
• Hemangioma of bone
• Idiopathic histiocytes (Langerhans cell disease)
• Tori and exostosis
• Coronoid hyperplasia
• Central Schwannoma
CLASSIFICATION OF TUMOR
Tissue of origin Benign Malignant
Epithelial Squamous epithelium Squamous cell papilloma Squamous cell carcinoma
Transitional epithelial Transitional cell papilloma Transitional cell carcinoma
Basal cell layer --------- Basal cell carcinoma
Glandular epithelium Adenoma Adenocarcinoma
Hepatocyte Liver cell adenoma Hepatocellular carcinoma
Neuroectodermal Nevus Melanoma
Mesen- Adipose tissue Lipoma Liposarcoma
chymal Adult fibrous tissue Fibroma Fibrosarcoma
Embryonic fibrous tissue Myxoma Myxomsarcoma
Cartilage Chondroma Chondrosarcoma
Bone Osteoma Osteosarcoma
Synovium Benign synovioma Synovial sarcoma
Skeletal muscle Rhabdomyoma Rhabdomyosarcoma
Smooth muscle Leiomyoma Leiomyosarcoma
Mesothelium ------------ Mesothelioma
Blood vessels Hemangioma Angiosarcoma
Lymph vessels Lymphangioma Lymphangiosarcoma
Glomus cell Glomus tumor ---------------
Meninges Meningioma Invasive meningioma
Hemopoietic cell ---------- Leukemia
Lymphoid tissue ------------- Malignant lymphoma
Nerve sheath Neurilemmoma Neurogenic sarcoma
Nerve cell Ganglioneuroma Neuroblastoma
Mixed Salivary gland Pleomorphic adenoma Malignant salivary gland tumor
Tumor of more than Totipotent cells in gonads or in Mature teratoma Immature teratoma
one germ cell layer embryonal rest
Appendices
PRECANCEROUS LESION AND CONDITION

Precancerous lesion Precancerous condition
• Leukoplakia • Oral submucous fibrosis 959
• Erythroplakia • Sideropenic anemia
• Palatal lesion associated with reverse smoking • Erosive lichen planus
• Verrucous hyperplasia • Discoid lupus erythematosus
• Carcinoma in situ • Dyskeratosis congenita
Histological Classification of Cancer and precancers of the Oral Mucosa (histological typing of cancer and precancer
(WHO 1997)
Carcinomas Precancerous lesions (clinical classification)
• Squamous cell carcinoma • Leukoplakia
• Verrucous carcinoma • Erythroplakia
• Basaloid squamous cell carcinoma • Palatal keratosis associated with reverse smoking.
• Adenoid squamous cell carcinoma Precancerous lesions (histological classification)
• Spindle cell carcinoma • Squamous epithelial dysplasia
• Adenosquamous carcinoma • Squamous cell carcinoma in situ
• Undifferentiated carcinoma. • Solar keratosis
Benign lesions capable of microscopically resembling oral Benign lesions capable of resembling oral precancerous lesions
squamous cell carcinoma and oral verrucous carcinoma • White lesions resembling leukoplakia
• Papillary hyperplasia • Red lesions resembling erythroplakia
• Granular cell tumor • Focal epithelial hyperplasia
• Discoid lupus erythematosus • Reactive and regenerative atypia.
• Median rhomboid glossitis
Precancerous conditions
• Keratoacanthoma
• Sideropenic dysphagia
• Necrotizing sialometaplasia
• Lichen planus
• Juxtra oral organ of chievitz
• Oral submucus fibrosis
• Chronic hyperplastic candidiasis
• Syphilis
• Verruciform xanthoma
• Discoid lupus erythematosus
• Verruca vulgaris
• Xeroderma pigmentosum
• Condyloma acuminata.
• Epidermolysis bullosa.
WHO classification of malignant tumor of oral cavity

Malignant epithelial tumor Hematolymphoid tumors
Squamous cell carcinoma Diffuse large B-cell lymphoma (DLBCL)
Verrucous carcinoma Mantle cell lymphoma
Basaloid squamous cell carcinoma Follicular lymphoma
Papillary squamous cell carcinoma Extranodal marginal zone B-cell lymphoma of MALT type
Spindle cell carcinoma Burkitt lymphoma
Acantholytic squamous cell carcinoma T-cell lymphoma (including anaplastic large cell lymphoma)
Adenosquamous carcinoma Extramedullary plasmacytoma
Carcinoma cuniculatum Langerhans cell histiocytosis
Lymphoepithelial carcinoma Extramedullary myeloid sarcoma
Epithelial precursor lesions Follicular dendritic cell sarcoma/tumor
Soft tissue tumors Mucosal malignant melanoma
Kaposi sarcoma Secondary tumors
Lymphangioma
Ectomesenchymal chondromyxoid tumor
Focal oral mucinosis
Congenital granular cell epulis
Classification of malignant tumor

Epithelial Neural tissue
960 • Squamous cell carcinoma • Neurosarcoma
• Metastatic carcinoma • Neurofibrosarcoma
• Basal cell carcinoma • Neuroblastoma
• Transitional cell carcinoma • Ganglioneuroma
• Malignant melanoma Muscle
• Verrucous carcinoma • Leiomyosarcoma
• Spindle cell carcinoma • Rhabdomyosarcoma
• Primary intra-alveolar carcinoma • Malignant granular cell myoblastoma
• Intraepidermoid carcinoma Lymphoid tissue
• Adenoid squamous cell carcinoma • Hodgkin’s lymphoma
Fibrous connective tissue • Non-Hodgkin’s lymphoma
• Fibrosarcoma • Primary reticular cell sarcoma
• Malignant fibrous histiocytomas • Burkitt’s lymphoma
Adipose tissue • Leukemia
• Liposarcoma Myeloma
Cartilage • Multiple myeloma
• Chondrosarcoma • Plasmacytoma
Bone Tumor of salivary gland
• Osteosarcoma • Mucoepidermoid carcinoma
• Osteochondrosarcoma • Adeno-cystic carcinoma
• Ewing sarcoma • Adeno-carcinoma
Vascular • Acinic cell carcinoma
• Hemangioendothelioma • Malignant changes in pleomorphic adenoma
• Hemangiopericytoma
• Angiosarcoma
Classifications of cyst
By Robinson (1945)
From odontogenic tissues – Dentigerous cyst
• Periodontal cyst – Primordial cyst
– Radicular or dental root apex type From non-dental tissues
– Lateral type • Median cyst
– Residual type • Incisive canal cyst
• Globulomaxillary cyst
By who
Developmental Non-odontogenic
Odontogenic cysts Nasopalatine duct (incisive canal) cyst
Primordial Globulomaxillary cyst
Gingival cyst of infants Nasolabial cyst
Eruption cyst Inflammatory cysts
Dentigerous cyst (follicular) Radicular
Gingival cyst of adults Apical and lateral
Lateral periodontal cyst Residual
Glandular odontogenic cyst , sialo-odontogenic cyst Paradental (inflammatory collateral, mandibular infected
buccal cyst)
Appendices
By Gorlin (1964)
Odontogenic cysts • Median mandibular cyst
• Dentigerous cyst • Anterior lingual cyst 961
• Eruption cyst • Dermoid and epidermoid cyst
• Gingival cyst of newborn infants • Palatal cyst of newborn infants
• Lateral periodontal and gingival cyst Cysts of neck and oral floor and salivary gland
• Keratinizing and calcifying cyst • Thyroglossal duct cyst
• Radicular cyst • Lymphoepithelial (bronchial cleft) cyst
• Primordial cyst • Oral cysts with gastric or intestinal epithelium
• Multiple cysts of jaws and multiple cutaneous nevoid basal • Salivary gland cyst
cell carcinoma and skeletal anomalies • Mucocele and ranula
Non-odontogenic and fissural cysts Pseudo-cyst
• Globulomaxillary cyst (premaxilla maxillary cyst) • Aneurysmal bone cyst
• Nasoalveolar (Nasolabial Klestadt’s) cyst • Static (developmental latent) bone cyst
• Nasopalatine (median anterior maxillary) cyst • Traumatic (hemorrhagic solitary) bone cyst
DEVELOPMENTAL DISTURBANCES OF ORAL AND PARAORAL STRUCTURES

Developmental disturbances of jaws Developmental disturbances of oral mucosa
• Agnathia • Fordyce’s granules
• Micrognathia • Focal epithelial hyperplasia (Heck’s disease)
• Macrognathia Developmental disturbances of gingiva
• Facial hemihypertrophy • Fibromatosis gingivae
• Facial hemiatrophy • Retrocuspid papilla
• Abnormalities of dental arch relations Developmental disturbances of oral lymphoid tissue
Developmental disturbances of lips and palate • Reactive lymphoid aggregates
• Congenital lips and commissural pits and fistula • Lymphoid hamartoma
• Double lip • Angiolymphoid hyperplasia
• Cleft lip and palate • Lymphoepithelial cyst
• Cheilitis glandularis Developmental disturbances of salivary glands
• Cheilitis granulomatosis • Aplasia
• Labial and oral melanotic macule • Xerostomia
Developmental disturbances of the tongue • Hyperplasia of palatal gland
• Microglossia • Atresia
• Macroglossia • Aberrancy
• Ankyloglossia • Developmental mandibular salivary depression
• Cleft tongue Fissural (inclusion, developmental) cyst of oral region
• Fissured tongue • Median anterior maximum cyst
• Medial rhomboidal glossitis • Median palatal cyst
• Benign migratory glossitis • Nasoalveolar cyst
• Hairy tongue • Palatal cyst of the neonate
• Lingual varices • Thyroglossal tract cyst
• Lingual thyroid nodule • Benign cervical lymphoepithelial cyst
• Dermoid and epidermoid cyst
ABNORMALITIES OF TEETH
Alteration in size Defect of enamel
962 • Microdontia • Dentinogenesis imperfecta
• Macrodontia • Dentin dysplasia
Alteration in shape • Dentine hypocalcification
• Germination Defect of enamel and dentin
• Fusion • Regional odontodysplasia
• Dilacerations Abnormalities of dental pulp
• Dens in dente • Pulp calcification
• Dens evaginatus • Internal resorption
• Taurodontism • External resorption
• Concrescence Alteration in color
• Supernumerary roots • Exogenous stains
• Enamel pearls • Endogenous stains
• Talon cusp Disturbances of growth (eruption) of teeth
• Attrition, abrasion, erosion • Premature eruption
Alteration in number • Delayed eruption
• Anodontia (total or partial) • Multiple unerupted teeth
• Supernumerary teeth • Embedded and impacted teeth
• Predeciduous dentition • Ankylosed deciduous teeth
• Postpermanent dentition
Defects of enamel
• Environmental defects of enamel
• Amelogenesis imperfecta
CLASSIFICATION OF CANDIDIASIS
Candidiasis of nails and skin Mucocutaneous candidiasis
• Candidal onychia Confined to mucocutaneous surface
• Candidal paronychia • In condition with major immunologic defect
Candidiasis confined to skin – Swiss-type agammaglobulinemia
• Interdigital candidiasis – Hereditary thymic dysplasia
• Intertriginous candidiasis – DiGeorge syndrome
• Moniliids – AIDS
Candidiasis confined to mucosae • In condition with minor immunological or other systemic
Oral mucosa defect
• Acute oral candidiasis • Chronic mucocutaneous candidiasis (CMC) syndromes
– Acute pseudomembranous candidiasis (thrush) – Familial mucocutaneous candidiasis
– Acute atrophic candidiasis (antibiotics sore mouth) – Candidiasis endocrinopathy syndrome
• Chronic oral candidiasis – Localized chronic mucocutaneous candidiasis
– Chronic atrophic candidiasis (denture sore mouth) – Diffuse chronic mucocutaneous candidiasis
– Chronic hyperplastic candidiasis – Chronic mucocutaneous candidiasis in association with
Gastrointestinal mucosa thymoma
• Pharyngeal candidiasis Confined to mucocutaneous junctions
• Esophageal candidiasis • Candidal angular cheilitis
• Intestinal candidiasis • Perianal candidiasis
Respiratory mucosa Systemic candidiasis
• Bronchial candidiasis • Candidal endocarditis
Genitourinary mucosae • Candidal septicemia
• Candidal vulvovaginitis • Candidal meningitis
Appendices
CLASSIFICATION OF SALIVARY GLAND DISORDERS

Developmental disorders Cyst
• Aberrancy • Mucocele 963
• Aplasia and hypoplasia • Ranula
• Hyperplasia Asymptomatic enlargement
• Atresia • Sialosis
• Accessory ducts • Uveoparotid fever
• Diverticuli • Recurrent non-specific parotitis
• Congenital fistula Neoplasms
Functional disorders Benign but seldom recurrent
• Sialorrhea • Warthin’s tumor
• Xerostomia • Oncocytoma
• Allergic • Monomorphic salivary adenomas
• Associated with malnutrition and alcoholism Benign but often recurrent
Infection • Pleomorphic adenoma
• Viral infection • Mucoepidermoid tumor (low grade)
• Bacterial infection • Acinic cell tumor (some)
• Mycotic infection Malignant
Autoimmune disorders • Carcinoma in pleomorphic adenoma
• Sjogren’s syndrome • Adenoid cystic carcinoma
• Mickulicz’s disease • Mucoepidermoid tumor (high grade)
Obstructive disorders • Acinic cell tumor (some)
• Sialolithiasis • Squamous carcinoma
• Mucus plug • Adenocarcinoma
• Stricture and stenosis • Undifferentiated carcinoma
• Foreign bodies
• Extra-ductal causes
SOFT TISSUE TUMORS OF MAJOR SALIVARY GLAND AND

PARAGLANDULAR TISSUES
Vascular Skeletal muscle
• Primary hemangioma of parotic gland • Rhabdomyosarcoma
• Lymphangioma • Rhabdomyoma
• Arteriovenous fistula • Infantile rhabdomyoma
• Angiosarcoma • Massetric hypertrophy
Lymphoreticular Neurogenous
• Lymphoma • Neurofibroma
• Atypical lymphoreticular hyperplasia • Neurofibrosarcoma
• Histiocytosis • Neurilemmoma
• Lymphoepithelial lesion • Traumatic neuroma
• Benign reactive hyperplasia • Neuroepithelial sarcoma
Smooth muscle • Granular cell tumor
• Leomyoma • Meningoma
• Leiomyosarcoma Fibroblastic and histiocytic
• Fibrous scar or Keloid
• Fibrosarcoma
• Fibromatosis
• Histiocytoma
OLD WHO CLASSIFICATION OF SALIVARY GLAND TUMOR (THACKERAY AND

SOBIN 1972)
964
1. Epithelial tumors • Adenocarcinoma
A. Adenoma • Epidermoid carcinoma
• Pleomorphic adenoma • Undifferentiated carcinoma
• Monomorphic adenoma • Carcinoma in pleomorphic adenoma (Malignant
• Adenolymphoma (Warthin’s tumor) pleomorphic adenoma)
• Oxyphilic adenoma (Oncocytoma) 2. Nonepithelial tumors
• Other types Unclassified lesions
– Basal cell adenoma Allied conditions
– Canalicular adenoma • Benign lymphoepithelial lesion
• Mucoepidermoid tumor • Sialosis
• Acinic cell tumor • Oncocytosis
B. Carcinoma
• Adenoid cystic carcinoma
FIBRO-OSSEOUS LESION OF JAW BONE

Fibro-osseous lesions of medullary bone origin Fibro-osseous lesions of periodontal origin
• Fibrous dysplasia • Periapical cemental dysplasia
• Fibro-osteoma • Florid osseous dysplasia
• Cherubism • Cemento-ossifying fibroma
• Juvenile ossifying fibroma • Cementifying fibroma
• Giant cell tumor • Ossifying fibroma
• Aneurysmal bone cyst
• Jaw lesions in hyperparathyroidism
• Paget’s disease
Appendices
CLASSIFICATION OF TONGUE DISORDERS

Congenital and developmental disorders • Dermatomyositis
• Aglossia and microglossia • Diabetes 965
• Macroglossia • Syphilis
• Ankyloglossia • Zoster infection
• Cleft tongue • Tuberculosis
• Ankyloglossum superius syndrome Neurological disease
• Lingual varices • Glossodynia
• Lingual thyroid nodule • Dyskinesia
• Variations in tongue movement • Paralysis
• Patent thyroglossal duct cyst • Oropharyngeal dysphagia
• Tongue thrusting Cyst
• Lingual polyp • Anterior median lingual cyst
• Reactive lymphoid aggregate • Bronchogenic cyst
• Lingual cyst • Epidermoid and dermoid cyst
Local tongue disorders • Gastric mucosal cyst
• Fissured tongue • Parasitic cyst
• Median rhomboidal glossitis • Thyroglossal duct cyst
• Benign migratory glossitis Benign tumor
• Hairy tongue • Fibroma
• Crenated tongue • Glomus tumor
• Foliate papillitis • Granular cell tumor
• Leukokeratosis nicotine glossi • Leiomyoma
Depapillation of tongue • Rhabdomyoma
Local disease • Plasmacytoma
• Eosinophilic granuloma Premalignant lesion and conditions
• Traumatic injuries • Leukoplakia
• Lesions due to automutilation • Lichen planus
• Allergic stomatitis • Oral submucus fibrosis
• Facial hemiatrophy Malignant tumor
• Cranial arteritis • Squamous cell carcinoma
• Chronic candidiasis • Malignant lymphoma
Systemic disease • Malignant melanoma
• Iron deficiency anemia • Metastatic tumor
• Plummer Vinson syndrome • Sarcoma
• Pernicious anemia Miscellaneous
• Niacin deficiency • Pigmentation of tongue
• Folic acid deficiency • Phlebectasia
• Peripheral vascular disease
WHITE LESION OF ORAL CAVITY

Scrapable lesion Non-scrapable lesion
966 White coated tongue Linea alba
Pseudomembranous candidiasis Leukoedema
Morsicatio Leukoplakia
Thermal burn Tobacco pouch keratosis
Sloughing traumatic lesion Actinic cheilosis
Toothpaste or mouth wash reaction Lichen planus
Chemical burn Nictoine stomatitis
Secondary syphilis Hairy leukoplakia
Diphtheria Hyperplastic candidiasis
Lupus erythematous
Skin graft
Submucous fibrosis
White sponge nevus
Hereditary benign intraepithelial dyskeratosis
Pachyonychia congenita
Tertiary syphilis
Uremic stomatitis
RED AND WHITE LESION

Erythema migrans Nicotine stomatitis
Candidiasis Erythroleukoplakia
Lichen planus Cinnamon reaction
Burns Lupus erythematosus
Actinic cheilosis Scarlet fever
Verruciform xanthoma
RED LESION OF ORAL CAVITY

Pharyngitis Thermal burns
Traumatic erythema Erythroplakia
Denture stomatitis Anemia
Erythematous candidiasis Hemangioma
Erythema migrans Lupus erythematous
Angular cheilitis Scarlet fever
Plasma cell gingivitis
Radiation mucositis
Appendices
BLUE/PURPLE/BROWN/GRAY/BLACK/YELLOW LESION
Blue/purple lesion Brown/gray/black lesion Yellow lesion
Varicosities Racial pigmentation Fordyce granules 967
Submucosal hemorrhage Amalgam tattoo Superficial abscess
Amalgam tattoo Black/brown hairy tongue Accessory lymphoid aggregates
Mucocele Melanotic macule Lymphoepithelial cyst
Eruption cyst Smoker’s melanosis Lipoma
Salivary duct cyst Non-amalgam tattoo Jaundice
Hemangioma Melanocytic nevus Verruciform xanthoma
Ranula Malignant melanoma Pyostomatitis vegetans
Kaposi’s sarcoma Oral melanoacanthoma
Nasopalatine duct cyst Drug ingestion
Salivary gland tumor Peutz-Jeghers syndrome
Gingival cyst of adults Addison’s disease
Blue nevus Neurofibromatosis
Malignant melanoma McCune-Albright syndrome
Heavy metal poisoning
Melanotic ectodermal tumor of infancy
VESICULOBULLOUS AND ULCERATIVE LESION

Acute (short duration) Chronic (long duration)
Traumatic ulcer Erosive lichen planus
Apthous stomatitis Squamous cell carcinoma
Recurrent herpes labialis Mucous membrane pemphigoid
Primary herpetic gingivostomamitis Traumatic granuloma
Necrotizing ulcerative gingivitis Lupus erythematous
Mucosal burns Pemphigus vulgaris
Recurrent intraoral herpes simplex Deep fungal infection
Allergic reaction Tuberculosis
Erythema multiforme Sarcoidosis
Herpangina Epidermolysis bullosa
Varicella Pyostomatitis vegetans
Herpes zoster Wegner’s granulomatosis
Hand foot and mouth disease Midline lethal granuloma
Necrotizing sialometaplasia Noma
Anesthetic necrosis Tertiary syphilis
Primary syphilis
Behçet syndrome
PAPILLARY LESION OF ORAL CAVITY

Hairy tongue Giant cell fibroma
968 Papilloma Verruciform xanthoma
Inflammatory papillary hyperplasia Verrucous carcinoma
Verruca vulgaris Condyloma acuminatum
Leukoplakia (some variant) Multifocal epithelial hyperplasia
Squamous cell carcinoma Darier’s disease
Hairy leukoplakia Acanthosis nigricans
CLASSIFICATION OF GIANT CELL

Physiological Inflammatory condition Neoplastic condition
• Osteoblast • Foreign body type • Osteoclastic
• Trophoblast • Langhans type • Reed Sternberg
• Megakaryocyte • Aschoff’s cells • Atypical
• Touton type • Giant cell fibroblast
CLASSIFICATION OF GIANT CELL LESION

Physiological Infection
• Root resorption of deciduous teeth • Syphilis
Traumatic • Yaws
• Traumatic granuloma • Bezel
• Peripheral and central giant cell granuloma • Tuberculosis
• Epulis fissuratum • Leprosy
• Pyogenic granuloma • Sarcoidosis
• Chronic occlusal trauma • Plasmocytosis
• Internal resorption causing periapical granuloma • Blastomycosis
• Root resorption from pressure trauma • Histoplasmosis
• Healing of sockets after extraction • Candidiasis
• Actinomycosis
• Aspergillosis
• Sporotrichosis
• Mucormycosis
• Criptococosis
• Rhinosporidiosis
• Coccidiomycosis
Appendices
CLASSIFICATION OF GIANT CELL LESIONS BASED ON CAUSES OR LOCATION

Physiological conditions Infections
• Resorption of deciduous teeth • Bacterial 969
• Healing of extraction teeth – Tuberculosis
• Orthodontic tooth movement – Syphilis
• Syncytiotrophoblasts of placenta – Leprosy
Non-neoplastic growth – Actinomycosis
• Soft tissue – Osteomyelitis
– PGCG • Viral
– Giant cell fibroma – Herpes simplex
– Epulis fissuratum – Herpes zoster
– Traumatic granuloma – Mumps
• Bone – CMV
– CGCG – Measles
– Browns tumor • Fungal
– Fibrous dysplasia – Histoplasmosis
– Pagets disease – Candidiasis
– Osteomalacia – Blastomycosis
– Cherubism – Coccidioidomycosis
Cysts and neoplasms – Rhinosporidosis
• Cysts • Protozoal
– ABC – Leishmoniasis
– CEOT • Chlamydial
– Solitary bone neoplasm – Lymphogranuloma venerum
• Benign neoplasms Non-infective granuloma
– Giant cell tumor • Midline lethal granuloma
– Osteoid osteoma • Wegener’s granulomatosis
– Osteoblastoma • Plasma cell granuloma
– Central hemangioma • Sarcoidosis
• Malignant neoplasms Periodontal conditions
– Osteosarcoma • Periodontitis
– Hodgkin’s lymphoma • Periapical cyst
– Reticular cell sarcoma • Periapical granuloma
– Lymphosarcoma • Chronic periapical abscess
– Oral squamous cell carcinoma Miscellaneous
• Internal resorption
• External resorption
• Massive osteolysis
• Spindle or epithelial cell nevus
PRIMARY AND SECONDARY SWELLINGS OF HARD AND SOFT PALATE

INCLUDING GINGIVAE
970 Traumatic Neoplastic
• Hematoma Odontogenic
• Postoperative injection swellings • Ameloblastoma
• Denture granuloma • Adenomatoid odontogenic tumor
• Fibrous epulis • Calcifying epithelial odontogenic tumor
• Peripheral giant cell granuloma • Odontogenic myxoma
• Traumatic bone cyst • Odontogenic fibroma
Inflammatory • Odontoameloblastoma
• Periapical infections • Ameloblastic fibro-odontoma
• Gingival abscess • Complex odontome
• Osteomyelitis • Compound odontome
• Quinsy (peritonsillar abscess) Non-odontogenic
• Tuberculoma • Torus palatinus
• Gumma • Osteoma
Cystic • Chondroma
Odontogenic • Rhabdomyoma
• Primordial (keratocyst) • Chondroma
• Dentigerous • Neurofibroma
• Periapical • Schwannoma
• Gingival cyst of adults and children • Hemangioma
Non-odontogenic • Lymphangioma
• Nasopalatine duct cyst • Pleomorphic adenoma
• Median palatine cyst • Fibroma
• Palatine papilla cyst • Ossifying fibroma
• Mucocele of palatal glands • Myxoma
Drugs • Papilloma
• Dilantin sodium ‘ • Melanoma
• Cyclosporine Malignant
Systemic • Osteogenic sarcoma/chondrosarcoma
• Vitamin deficiency • Multiple myeloma
• Leukemia • Rhabdomyosarcoma
• Pregnancy tumor • Malignant hemangioendothelioma
• Hyperparathyroidism • Kaposi’s sarcoma
• Fibrous dysplasia • Ewing’s tumor
• Paget disease • Transitional cell carcinoma
• Histiocytosis X • Hodgkin’s lymphoma
Allergic • Malignant pleomorphic adenoma
• Angioneurotic edema • Adenoid cystic cell carcinoma
Miscellaneous • Fibrosarcoma
• Impaction of misplaced teeth-ectopic eruption • Squamous cell carcinoma
• Osteoradionecrosis • Malignant melanoma
• Chemical necrosis • Metastatic carcinoma
• Noma
Appendices
CAUSES OF TRISMUS
Traumatic Neoplastic
Extra-articular Any tumor intra/extra-articular/exostosis of condyle and coronoid 971
• Blow or fall Osteoradionecrosis (both intra or extra-articular)
• Surgical extractions and fracture mandible Systemic
• Keloid following burns • Tetany
• Improper inf. Alveolar nerve block injection • Hypothyroidism
• Masseter muscle / sphenomandibular ligament calcification • Strychnine poisoning
Intra-articular • Epilepsy
• Forceps delivery and followed by ankylosis • Hysteria
• TMJ fracture • Physical, chemical, electrical or mental shock or extreme fear
• Upward and backward dislocation • Brain tumors and hemorrhage in medulla oblongata
• Acute forward dislocation Congenital
Infections • Ankylosis of maxilla to mandible
Extra-articular Miscellaneous
• Pericoronitis • Fusion of coronoid process to zygoma
• Osteomyelitis • Submucous fibrosis
• Cellulitis • Scleroderma
• Parotid infections
• Otitis media
• Mastoiditis
• Tonsillitis
• Oral ulcers
• Diphtheria
• Tetanus
• Infected granuloma of bone like (TB, syphilis, actinomycosis)
Intra-articular
• Osteomyelitis of TMJ (local cause or through blood)
• Arthritis (rheumatoid, osteoarthritis, rheumatoid fever)
CONDITIONS PRODUCING MACROCHELIA

(LIP ENLARGEMENT AND SWELLING)
972 Traumatic Inflammatory
• Acute blow or face, friction and surgical interference • Collateral edema from dental infections
• Insect bite • Acute furuncle in nose or lip
• Chronic mouth breathing • Carbuncle or abscess due to mucous gland or hair follicle
• Self inflicted chronic injuries like lip sucking and biting, • Infected traumatic lesions
glass blowing and pipe smoking, etc. • Chronic macrocheilia following repeated attacks of
Neoplastic inflammations
Benign • Tuberculoma
• Papilloma • Sarcoidosis
• Melanoma • Syphilis
• Monomorphic adenoma • Actinomycosis
• Canalicular adenoma • Molluscum contagiosum
• Pleomorphic adenoma • Radiation cheilitis
• Mucoepidermoid tumor • Mucocele
• Fibroma Allergic
• Neurofibroma • Giant urticaria
• Lipoma • Angioneurotic edema
• Hemangioma Congenital
• Lymphangioma • Congenital macrocheilia
• Granular cell myoblastoma • Congenital double lip
• Solitary myeloma Miscellaneous and syndromes
Malignant • Amyloidosis
• Squamous cell carcinoma • Keratoacanthoma
• Malignant melanoma • Cheilitis glandularis
• Basal cell carcinoma • Papillomatosis in acanthosis nigricans and adenocarcinoma
• Malignant pleomorphic adenoma • Tuberous sclerosis
• Mucoepidermoid tumor • Hereditary hemorrhagic telangiectasia
• Adenocarcinoma • Lipoidosis
• Fibrosarcoma • Idiopathic fibroedema
• Neurofibrosarcoma • Melkersson syndrome
• Lymphosarcoma • Cheilitis granulomatosis
• Angiosarcoma • Crohn’s disease (macrocheilia)
• Rhabdomyosarcoma • Polystomatitis vegetans
• Kaposi sarcoma in AIDS
Appendices
SWELLINGS OF FLOOR OF THE MOUTH

Neoplastic Traumatic
Benign • Hematoma 973
• Papilloma • Embedding of foreign body (foreign body granuloma)
• Melanoma • Denture granuloma
• Monomorphic adenoma Postradiation swellings
• Pleomorphic adenoma Cystic swellings
• Oxyphilic adenoma • Sublingual dermoid
• Hemangioma • Thyroglossal duct cyst
• Hemangiopericytoma • Branchial cyst
• Endothelioma • Ranula
• Lymphangioma • Plunging ranula
• Neurofibroma Inflammatory
• Neurilemmoma • Collateral edema from odontogenic infection
• Plasmacytoma • Swellings of Wharton’s duct
• Rhabdomyoma • Stone in Wharton’s duct
• Leiomyoma • Mikulicz disease
• Lipoma • Swelling of sublingual and submandibular salivary gland
• Fibroma • Acute infections following trauma
Malignant Allergic
• Squamous cell carcinoma • Angioneurotic edema
• Mucoepidermoid tumor Syndromes
• Adenocarcinoma • Hereditary hemorrhagic telangiectasia
• Malignant pleomorphic adenoma • Struge-Weber disease
• Fibrosarcoma
• Reticulum cell sarcoma
• Lymphosarcoma
• Rhabdomyosarcoma
• Adenolymphoma
• Hemangioendothelioma
• Kaposi’s sarcoma (AIDS)
ULCERATION OF ORAL CAVITY

Traumatic Infections
Physical Nonspecific (Strepto and Staph)
• Rough and badly fitting restoration • Nonspecific infected ulcers primarily traumatic strepto-
• Ulcers due to tooth present at birth and sharp edges stomatitis
• From splints Specific (bacilli and cocci)
• Force injection ulcers on palate • Aphthous ulcer
• Electric burn due to live wire • Tuberculosis
• Post filling ulcer on frenum of tongue • Diphtheria
• Epileptic ulcers • Tularemia (rabbit fever)
Thermal • Granuloma inguinale
• Hot liquid • Leprosy
• Refrigerant local anesthetics • Gonorrhea
Chemical • Pneumococcal
• Drugs like aspirin, arsenic, bismuth, and lead • Ulcers of oral cavity in infections with bacillus
• Actinic cheilosis on lips
• Radiation and laser radiation
METABOLIC AND GENETIC JAW DISEASES

Metabolic conditions Genetic abnormalities
974 • Paget’s disease • Cherubism
• Hyperthyroidism • Osteopetrosis
• Hypophosphatasia • Osteogenesis imperfecta
• Infantile cortical hyperostosis • Cleidocranial dysplasia
• Phantom bone disease • Crouzon syndrome
• Acromegaly • Treacher Collins syndrome (mandibulofacial dysostosis)
• Fibrous dysplasia • Pierre Robin syndrome
• Marfan syndrome
• Ehlers Danlos syndrome
VIRUSES CAUSING DISEASE IN ORAL CAVITY

Name of virus Disease cause
Herpes simplex virus I or Human herpes virus I Herpetic gingivostomatitis (primary and secondary)
Herpes simplex virus II or Human herpes virus II Genital infection
Varicella-zoster or Human herpes virus III Chickenpox, shingles
Epstein Barr virus or Human herpes virus IV Mononucleosis, Burkitt’s lymphoma, hairy leukoplakia,
nasopharyngeal carcinoma
Cytomegalovirus or Human herpes virus V Salivary gland enlargement
Human herpes virus VI Roseola infantum
Human herpes virus VIII Kaposi sarcoma
Papilloma viruses Oral papilloma, wart, heck disease, condyloma acuminatum
Coxsackieviruses Herpangina, hand, foot and mouth disease
Measles virus Measles
Mumps virus Mumps, parotitis
OSTEOLYTIC LESIONS
Congenital Granulomatous disease
• Cherubism • Central giant cell granuloma
Traumatic • Giant cell tumor
• Solitary bone cyst Odontogenic cyst
• Osteoporotic marrow defect • Dentigerous
• Fractures • Radicular
Radiation induced • Odontogenic keratocyst
• Osteoradionecrosis Odontogenic tumor
Inflammatory • Ameloblastoma
• Acute or chronic osteomyelitis • Odontogenic myxoma
Metabolic Malignant neoplasm
• Hyperparathyroidism • Squamous cell carcinoma
• Eosinophilic granuloma • Malignant melanoma
• Letterer-Siwe disease • Verrucous carcinoma
Others
• Aneurysmal bone cyst
• Traumatic bone cyst
Appendices
TEETH ANOMALIES AND THEIR CAUSE

Anomalies Cause
Hyperdontia Idiopathic supernumerary teeth 975
Cleft lip and palate
Gardner syndrome
Cleidocranial dysplasia
Hypodontia Idiopathic
Hereditary hypohidrotic ectodermal dysplasia
Incontinentia pigmenti
Radiotherapy during childhood
Macrodontia Fusion
Gemination
Idiopathic
Facial hemihyperplasia
Gigantism
Microdontia Supernumerary teeth

Peg shaped lateral incisor
Dens invaginatus
Idiopathic
Hereditary hypohidrotic ectodermal dysplasia
Congenital syphilis
Hypopituitarism
Malformed crown Mesiodens
Environmental enamel hypoplasia
Peg shaped lateral incisor
Dens invaginatus
Turner’s tooth
Fusion
Gemination
Talon cusp
Dens evaginatus
Amelogenesis imperfecta
Dentinogenesis imperfecta
Regional odontodysplasia
Congenital syphilis
Vitamin D resistant rickets
Renal osteodystrophy
Hypoparathyroidism
Pseudohypoparathyroidism
Epidermolysis bullosa
Contd...
Contd...
Anomalies Cause
976 Enamel loss Caries
Trauma
Attrition
Abrasion
Erosion
Extrinsic staining of teeth Tobacco
Coffee, tea, cold drinks
Chromogenic bacteria
Chlorhexidine
Intrinsic discoloration of teeth Aging
Death of pulp
Fluorosis
Tetracycline
Internal resorption
Calcific metamorphosis
Congenital erythropoietic porphyria
Erythroblastosis fetalis
Abnormal shaped root External root resorption
Dilacerations
Hypercementosis
Concrescence
Taurodontism
Enamel pearl
Benign cementoblastoma
Dentin dysplasia
Appendices
TYPICAL HISTOLOGICAL FEATURE

Typical features Name of disease
Starry sky Burkitt’s lymphoma, infective mononucleosis, polycythemia 977
Rushton bodies Dentigerous cyst
Reed Stenberg cell Hodgkin’s disease
Dropping off effect Junctional nevus
Saw tooth appearance Lichen planus
Antoni type A and type B tissue Neurilemmoma
Honey comb or Swiss cheese pattern Adenoid cystic carcinoma of salivary gland
Picket fence or tombstone Primordial cyst
Liesegang ring Calcifying epithelial odontogenic tumor
Safety pin appearance Granuloma inguinale
Lipschutz bodies Herpes simples infection
Anitschkow cell Aphthous ulcer, sickle cell anemia, megaloblastic anemia and iron deficiency
anemia
Henderson-Paterson inclusion Molluscum contagiosum
Dilapidated brick wall effect Familial benign chronic pemphigus
Cartwheel or checkerboard appearance Multiple myeloma
Tobacco cells or cell in cells Hereditary benign intraepithelial dyskeratosis
Lava flowing around boulder Dentin dysplasia (shield type I)
Signet ring appearance Liposarcoma
Storiform Malignant fibrous histiocytoma
Tadpole like Fibrosarcoma
Cigar like appearance Leiomyoma

APPENDIX V: HISTOLOGY DIAGRAMS OF ORAL TISSUES

NORMAL HISTOLOGICAL DIAGRAM
Sangamesh Halawar
Oral epithelium
Dental lamina
Dental lamina
Ectomesenchyme
Vestibular lamina
Dental follicle
Primitive
Condensed
ectomesenchyme
ectomesenchyme
Figure AP.1 Dental and vestibular lamina Figure AP.4 Bud stage
Oral epithelium
!_ Oral epithelium
w
Dental lamina
V \
Dental lamina
Stellate reticulum
Inner enamel
epithelium
' 1' •
Condensed
ectomesenchyme
Figure AP.2 Development of dental lamina Figure AP.5 Cap stage
K Oral epithelium
* «
* *• •*
%
.H# j
T
I
9
• -- %1
/
«
#
* > * » *» # 0 * .*
Primary
> .
» *
t
* '•
4
**
*i * %
" 4 •* * *. 0 * * * 9 * . *
M * *- *
epithelium band
Enamel knot
• ^' * * • t
*
« %
.
*
* #
VJ » «
* f
,
t
4
t
* * *
*
i k
Ik
t
4
r % *• I
* ' Primitive
*
% •.
«
%
.' ' '

*%
*
* <
•^ T ~
i / /
*
ectomesenchyme
*
.•
4 %
b
V >
* k * * I
«
0
'*
r
Figure AP.3 Early tooth development Figure AP.6 Enamel knot

Appendices
979
Figure AP.7 Enamel cord
Figure AP.10 Hertwig’s epithelial root sheath
Figure AP.8 Bell stage
Figure AP.9 Advanced bell stage Figure AP.11 Fordyce’s granules

980
Figure AP.12 Gingiva Figure AP.15 Histopathological diagram of the normal lip
Figure AP.13 Taste buds Figure AP.16 Normal pulp
Figure AP.14 Circumvallate, fungiform and filiform papillae Figure AP.17 Periodontal ligament
Appendices
981
Figure AP.18 Principal fibers of periodontal ligament. Figure AP.21 Mucous salivary gland
1. Alveolar crest fibers; 2. Horizontal fibers; 3. Oblique fibers;
4. Apical fibers; 5. Inter-radicular fibers
Figure AP.19 Serous salivary gland Figure AP.22 Mixed salivary gland
Figure AP.20 Ducts of salivary gland Figure AP.23 Enamel

982
Figure AP.24 Key hole pattern of enamel Figure AP.27 Interglobular dentin
Figure AP.25 Dentin Figure AP.28 Dentinoenamel junction
Figure AP.26 Intra and intertubular dentin Figure AP.29 Cellular cementum
Appendices
983
Figure AP.30 Acellular cementum Figure AP.33 Cementoenamel junction butt joint
Figure AP.31 Cementoenamel junction gap joint Figure AP.34 Dead tract
Figure AP.32 Cementoenamel junction overlapping joint Figure AP.35 Epithelial cell rests of Malassez
984
Figure AP.36 Enamel niche
Figure AP.39 Internal dental epithelium. 1. Morphogenic

stage; 2. Inductive stage; 3. Initial secretory stage (No Tomes
process); 4. Secretory stage (Tomes process); 5. Ruffle ended
ameloblast of maturative stage; 6. Smooth ended ameloblast
of maturative stage; 7. Protective stage
Figure AP.37 Enamel spindles tufts and lamellae
Figure AP.38 Epithelial cell rest Figure AP.40 Buccal mucosa

Appendices
985
A B
Figure AP.43 Non-keratinized epithelium
C D
Figure AP.41 Nerve ending in human periodontal ligament
Figure AP.44 Soft palate
Figure AP.42 Nerve innervations Figure AP.45 Orthokeratinized epithelium

986
Figure AP.46 Tomes’ granular layer Figure AP.47 Alveolar bone
+,6723$7+2/2*,&$/',$*5$062)25$//(6,216
Figure AP.48 Squamous papilloma Figure AP.50 Intradermal nevus
Figure AP.49 Keratoacanthoma Figure AP.51 Junctional nevus

Appendices
987
Figure AP.52 Compound nevi Figure AP.55 Lipoma
Figure AP.53 Fibroma Figure AP.56 Chondroma
Figure AP.54 Giant cell fibroma Figure AP.57 Cancellous osteoma

988
Figure AP.58 Osteoma Figure AP.61 Mild epithelial dysplasia
Figure AP.59 Osteoblastoma Figure AP.62 Moderate epithelial dysplasia
Figure AP.60 Leukoplakia Figure AP.63 Severe epithelial dysplasia

Appendices
989
Figure AP.64 Leukoedema Figure AP.67 Severe oral submucous fibrosis
Figure AP.65 Mild oral submucous fibrosis

Figure AP.68 Moderately differentiated sq cell carcinoma
Figure AP.66 Moderate oral submucous fibrosis Figure AP.69 Poorly differentiated sq cell carcinoma
990
Figure AP.70 Basal cell carcinoma Figure AP.73 Liposarcoma
Figure AP.71 Verrucous carcinoma Figure AP.74 Chondrosarcoma
Figure AP.72 Fibrosarcoma Figure AP.75 Mesenchymal chondrosarcoma

Appendices
991
Figure AP.76 Osteosarcoma Figure AP.79 Multiple myeloma
Figure AP.77 Neurofibrosarcoma Figure AP.80 Hodgkin’s lymphoma
Figure AP.78 Leiomyosarcoma Figure AP.81 Plexiform ameloblastoma

992
Figure AP.82 Acanthomatous ameloblastoma Figure AP.85 Peripheral ameloblastoma
Figure AP.83 Granular ameloblastoma Figure AP.86 Mural ameloblastoma
Figure AP.84 Baseloid ameloblastoma Figure AP.87 Unicystic ameloblastoma

Appendices
993
Figure AP.88 Ameloblastic fibroma Figure AP.91 Odontogenic fibroma WHO type
Figure AP.89 Complex odontome Figure AP.92 Odontogenic myxoma
Figure AP.90 Odontogenic fibroma simple type Figure AP.93 Benign cementoblastoma
994
Figure AP.94 Periapical cemental dysplasia Figure AP.97 Daughter cyst present in odontogenic
keratocyst aneurysmal bone cyst
Figure AP.95 Dentigerous cyst Figure AP.98 Orthokeratinization seen in OKC
Figure AP.96 Odontogenic keratocyst Figure AP.99 Lateral periodontal cyst

Appendices
995
Figure AP.100 Aneurysmal bone cyst Figure AP.103 Nasopalatine duct cyst
Figure AP.101 Radicular cyst Figure AP.104 Traumatic bone cyst
Figure AP.102 Cholesterol cleft Figure AP.105 Aneurysmal bone cyst

996
Figure AP.106 Epidermoid cyst Figure AP.109 Pit and fissure caries
Figure AP.107 Smooth surface caries Figure AP.110 Congenital epulis
Figure AP.108 Dentinal caries Figure AP.111 Extravasation mucocele

Appendices
997
Figure AP.112 Retention of mucocele Figure AP.113 Pleomorphic adenoma
Figure AP.114 Different pattern of pleomorphic adenoma

998
Figure AP.115 Canalicular adenoma Figure AP.118 Mucoepidermoid carcinoma
Figure AP.116 Warthin’s tumor Figure AP.119 Adenoid cystic carcinoma tubular type
Figure AP.117 Oncocytoma Figure AP.120 Adenoid cystic carcinoma cribriform type
Appendices
999
Figure AP.121 Adenoid cystic carcinoma solid types Figure AP.124 Adenocarcinoma showing cribriform areas
Figure AP.125 Adenocarcinoma showing strands of perineural

Figure AP.122 Acinic cell carcinoma
whorls
Figure AP.123 Adenocarcinoma showing solid lobular area Figure AP.126 Tubular and papillary cystic area
1000
Figure AP.127 Necrotizing sialometaplasia Figure AP.130 Tuberculer granuloma
Figure AP.128 Pulp stone Figure AP.131 Actinomycosis
Figure AP.129 Tubercular ulcer Figure AP.132 Candidiasis

Appendices
1001
Figure AP.133 Fibrous dysplasia Figure AP.136 Florid osseous dysplasia
Figure AP.134 Central giant cell granuloma Figure AP.137 Ossifying fibroma
Figure AP.135 Paget’s disease Figure AP.138 Peripheral ossifying fibroma

1002
Figure AP.139 Pemphigus Figure AP.140 Mucous membrane pemphigoid

Answers Key of MCQs
Chapter 1 Chapter 10
1. c, 2. a. 3 - b, 4 . b, 5 . d, 6. a. 1. c, 2. b . 3 - d, 4. d, 5 . a, 6. c,
7 . d, 8. c, 9. a, 10. c. 7. d . 8. c, 9. a, 10. b .
1. c, 2. b. 3 - b, 4. a, 5 - d, 6 . a, 1. c, 2. d . 3. b , 4. a. 5 . b, 6 . c,
7. a. 8. a, 9. b, 10. d, 11. b, 12 . b, 7. d , 8. a, 9. b, 10. d, 11. c, 12. d .
13. c, 14. b, 15 . c, 16. d . 17. b . 18 . c, 13. a, 14. a, 15 . b, 16. d, 17. c, 18 . a,
19. d. 20. b. 19. d,
25 . d,
20. a,
26. a,
21. c,
27. b,
22. b,
28. d ,
23. b,
29. b,
24 . a,
30 . a,
Chapter 3 31. a, 32. d, 33 . b .
1. c, 2. d, 3 - b, 4. a, 5 - b, 6. c,
Chapter 12
7. a. 8. b, 9. c, 10. a.
1. a, 2. d, 3 . c, 4. c, 5 . c, 6 . d,
Chapter 4 7. b, 8. a, 9 . c, 10. a.
1. c, 2. d, 3. a, 4 . b, 5 - d, 6. c, Chapter 13
7. a, 8. c, 9. d , 10. b .
l . b, 2. c, 3 - d, 4. c, 5 - d, 6. a,
Chapter 5 7. b, 8 . d, 9. a . 10. b, 11. c, 12. a,
. 13. d . 14. c, 15 . a. 16. a, 17. b, 18 . d,

1. a,
7. b.
2. b
8. c,
3 - d,
9. a,
4. c,
10. d .
5 . a, 6. b, 19. a . 20. a. 21. a, 22. a. 23. b, 24 . c,
25 . a.
l . d,
7. d ,
2. c,
8. a,
3 . c,
9. a,
4. c,
10. b.
5 - b,
11. c,
6. c,
12 . c,
1. a, 2. b . 3 . c, 4. b, 5. a. 6 . d,
7. a, 8. b. 9 . c, 10. a, 11. c, 12. a,
13. c, 14. a . 13 . d, 14. a, 15 . a, 16. c, 17. c, 18 . c,
19. d, 20. a, 21. a, 22. c, 23. c, 24 . d,
Chapter 7 25 . d .
1. a. 2. c, 3. a, 4. d . 5 - b, 6. c,
Chapter 15
7. a, 8 . d, 9. c, 10. a.
l . b, 2. c, 3 . d, 4. c, 5 . a, 6. b
Chapter 8 7. c, 8. a, 9. b, 10. a, 11. c, 12. c,
l . b, 2. b, 3 - d, 4. c, 5 . a, 6. a, 13. a, 14. c, 15 . d , 16. d, 17. d . 18 . c,
7. b. 8. c, 9. d , 10. c, 11. b, 12. a. 19. d, 20. c .
1. a. 2. d . 3. c, 4 . b, 5 - d, 6. d, 1. a . 2. c, 3. d , 4 . b, 5 . b, 6 . c,
7. a, 8. b. 9. a, 10. b . 7. c, 8 . b, 9. d , 10. c, 11. a, 12 . c,
13. d, 14. b, 15. d, 16. c, 17. b, 18. c, Chapter 26

19. b, 20. c.
1. c, 2. d, 3. a, 4. b, 5. c.
1004 Chapter 17
Chapter 27
1. b, 2. a, 3. c, 4. c, 5. b, 6. d,
7. a, 8. d, 9. a, 10. b, 11. c, 12. b, 1. a, 2. b, 3. d, 4. b, 5. c, 6. a.
13. c, 14. c, 15. b, 16. a, 17. a, 18. c,
19. d, 20. a. Chapter 28
1. a, 2. c, 3. d, 4. c, 5. d, 6. a,
Chapter 18 7. c, 8. d, 9. c, 10. c, 11. a, 12. b,
1. d, 2. b, 3. c, 4. a, 5. d, 6. a, 13. d, 14. b, 15. a.
7. b, 8. c, 9. c, 10. d.
Chapter 29
Chapter 19 1. b, 2. a, 3. c, 4. a, 5. d, 6. a,
1. b, 2. a, 3. c, 4. b, 5. b. 7. d, 8. b, 9. a, 10. a.
1. a, 2. b, 3. d, 4. d, 5. c, 6. a, 1. b, 2. c, 3. d, 4. d, 5. a, 6. a.
7. d, 8. a, 9. a, 10. d.
Chapter 31
Chapter 21
1. a, 2. d, 3. c, 4. a, 5. d, 6. d,
1. a, 2. a, 3. b, 4. d, 5. a, 6. a,
7. b, 8. a, 9. a, 10. b.
7. a, 8. d.
1. b, 2. d, 3. a, 4. c, 5. a, 6. a,
1. b, 2. c, 3. c, 4. a, 5. a, 6. b,
7. b, 8. a, 9. c, 10. b.
7. a, 8. c, 9. d, 10. c, 11. a, 12. d,
13. b, 14. a, 15. b.
Chapter 33
Chapter 23 1. d, 2. a, 3. b, 4. c, 5. a, 6. b,
1. c, 2. a, 3. b, 4. d, 5. a, 6. b, 7. c, 8. a, 9. b, 10. a, 11. c, 12. b.
7. d, 8. d, 9. a, 10. a.
Chapter 34
Chapter 24 1. a, 2. b, 3. c, 4. b, 5. a.
1. a, 2. b, 3. a, 4. c, 5. d.
Chapter 35
Chapter 25 1. a, 2. c, 3. b, 4. a, 5. b, 6. c,
1. c, 2. b, 3. a, 4. b, 5. a, 6. d, 7. d, 8. c, 9. a, 10. b, 11. c, 12. d,
7. c, 8. b, 9. a, 10. d. 13. a.
13 -ds-retinoic acid 229 Actinomycosis 468 Adenosquamous carcinoma 269
7th nerve paralysis 835 Acute atrophic candidiasis 488 Adenovirus 90
Acute blastomycosis 494 Adipose tissue 283
A Acute contact stomatitis 772 Adrenal cortex 785
Acute disseminated histocytosis X 804 Adrenal gland 785
Abbe condenser 4, 12
Acute exacerbation of a Adrenal medulla 785
ABODE criteria 275
Aberrancy 412 chronic lesion 552 Adrenogenital syndrome 797, 883
Abfraction 670 Acute febrile neutrophilic Adult osteopetrosis 594
Abrasion 668 dermatosis 881 Adult periodontitis 402
Abrikossoff myocytes 213 Acute gouty arthritis 651 Adult rhabdomyoma 212
Absolute pocket 401 Acute leukemia 721 Adult rickets 823
Abtrofung effect 176 Acute lymphoblastic leukemia 720 Afibrinogenemia 713
Acantholysis 736 Acute lymphonodular pharyngitis 515 African Burkitt’s lymphoma 719
Acanthomatous ameloblastoma 310 Acute non-lymphoblastic leukemia 720 African Kaposi’s sarcoma 531
Acanthosis 226 Acute periapical abscess 549 Age spot 756
Acanthosis nigricans 744, 886 Acute posthemorrhagic anemia 699 Agenesis 128
Accessory duct 414 Acute primary histoplasmosis 492 Aggressive osteoblastoma 191
Accidental myiasis 477 Acute sinusitis 481 Aggressive periodontitis 403
Achondroplasia 599 Acute suppurative osteomyelitis 557, 558 Aging pemphigus 737
Achromatic 7 Acute suppurative pulpitis 544 Aglossia 623
Achromatic condenser 5 Acute transforming viruses 89 Aglossia adactylia syndrome 877
Acid phosphatases 58 Acute ulcerative gingivitis 391 Agnathia 128
Acidophilic adenoma 437 Acute ulceromembranous gingivitis 391 AgNOR 351
Acmic cell adenocarcinoma 444 Acylatmg agents 87 Agranulocytic angina 705
Ackerman’s tumor 271 Adamantine epithelioma 303 Agranulocytopenia 705
Acquired macroglossia 624 Adamantmoblastoma 303 Agranulocytosis 705
Acquired myotonia 832 Adamantinoma 303 AIDS 524
Acquired nevi 174 Adamantinoma of long bones 315 AIDS classification 525
Acquired pellicle 149 Addison’s anemia 702 AIDS dementia complex 528
Acquired syphilis 456 Addison’s disease 796 AIDS related complex 526
Acral keratosis 760 Adenoacanthoma 277 Air nitrogen system 28
Acral lentiginous melanoma 276 Adenocystic carcinoma 445 Alarm clock headache 843
Acrocephalosyndactyly 137, 864 Adenoid adamantoblastoma 318 Alban’ s test 162
Acrodermatitis enteropathica 809 Adenoid basal cell carcinoma 268 Albers-Schonberg disease 594
Acrodyma 683 Adenoid cystic carcinoma 445 Albright’s syndrome 578, 872
Acromegaly 786 Adenoid squamous cell carcinoma 277 Alcohol 222
Acromelic 136 Adenolipoma 189 Aldrich syndrome 709
Acro -osteolysis 865 Adenolymphoma 435 Aleukemia 720
Actinic cheilitis 613 Adenoma sebaceum 885 Aleukemic leukemia 725
Actinic elastosis 617 Adenomatoid hyperplasia of minor Alkaline phosphatases 58
Actinic keratosis 613 salivary gland 413 Alkylating agents 87
Actinic lentigo 756 Adenomatoid odontogenic Allergic contact stomatitis 771
Actinic lichen planus 239 tumor /cyst 321 Allergic fungal sinusitis 500
Allergic sialadenitis 421 Angioleiomyoma 211 Apert’s syndrome 137, 864

Allergic stomatitis 635 Angiolipoma 188 Aphthous pharyngitis 515
Allergy 766 Angiomyolipoma 211 Apical periodontal cyst 361
1006 Alpha thalassemia 696, 697 Angiomyoma 211 Aplanatic condenser 5
Alum hematoxylin 41 Angioneurotic edema 773 Aplasia of gland 412
Alveolar cavitational osteopathosis bone Angioreticulo-endothelioma 530 Aplastic anemia 697
marrow edema 841 Angry appearance 704 Apochromat 7
Alveolar cyst of newborn 355 Angular cheilitis 530, 612, 701 Apparent macroglossia 624
Alveolar osteitis 573 Angular cheilosis 612 Apparent micrognathia 128
Alveolar rhabdomyosarcoma 295 Angular diaphragm 14 Aphthous stomatitis 774, 775
Alveolar sort-part sarcoma 296 Anhydrotic ectodermal dysplasia 876 Arachnodactyly 597
Amalgam tattoo 681 Anisocytosis 227 Arch mark 854
Amelanotic melanoma 274 Anisonucleosis 227 Arhinencephaly 136
Ameloblastic carcinoma 335 Anisotropic specimen 15 Architectural changes 227
Ameloblastic carcinosarcoma 339 Anitschkow cells 775 Argyll Robertson pupil 458
Ameloblastic dentinosarcoma 339 Ankyloglossia 625 Argyria 684
Ameloblastic fibrodentinoma 328 Ankyloglossum superius syndrome 626 Argyrosis 684
Ameloblastic fibroma 326, 327 Ankylosed of teeth 123 Aromatic amines 87
Ameloblastic fibro-odontoma 328 Ankylosis 648 Arrested caries 161
Ameloblastic fibro-odontosarcoma 339 ANN Arbor staging 715 Arsenic keratosis 685
Ameloblastic fibrosarcoma 338 Annealing 61 Arsenism 685
Ameloblastic odontomas 331 Annular diaphragm 13 Arteriovenous malformation 199
Ameloblastic sarcoma 338 Annular lichen planus 238, 239 Arteriovenous shunt 199
Ameloblastoma 303 Annulus migrans 632 Arthritis 572
Ameloblastoma classification 307 Anodontia 109 Artifacts in histological section 33
Ameloblastoma pathogenesis 306 Anomalous dysplasia 118 Asboe-Hansen sign 734
Amelogenesis imperfecta 114 Anorexia nervosa 669 Ascher’s syndrome 606, 875
American Burkitt’s lymphoma 719 Antemortem record examination 852 Ascorbic acid 819, 826
Ammonium compound 165 Anterior median lingual cyst 377 Ash-leaf macules 757
Amputation neuroma 203 Anterlike appearance 202 Aspergilloma 501
Amyloid disease 801 Anthropologic examination of Aspergillosis 500
Amyloid like material 319 bones and teeth 853 Asteroid bodies 769
Amyloidosis 801 Anti-aging factor 824 Atlantoaxial instability 599
Amyloidosis types 802 Anti-beriberi vitamin 826 Atresia 413
Amyotrophic lateral sclerosis 638, 836 Antibiotic vitamin 819 Atrophic degeneration 546
Anabolic steroids 89 Antibiotics sore mouth 488 Atrophic lichen planus 238, 239, 241
Analyzer 14 Anti-egg white injury factor 807 Attached cementicles 676
Anaphylactic stomatitis 771 Anti-hemorrhagic vitamin 825 Attrition 666, 667
Anderson syndrome 865 Anti-neuritic vitamin 826 Atypical facial neuralgia 841
Anemia 694 Antioxidant therapy 229 Atypical facial pain 841
Anesthetic necrosis 659 Antipernicious vitamin 807 Atypical gingivostomatitis 393
Aneuploidy 65 Antirachitic vitamin 826 Atypical trigeminal neuralgia 841
Aneurin 812 Antisterility vitamin 826 Auric stomatitis 685
Aneurysm varix 199 Antithyroid drugs 789 Auriculotemporal syndrome 834, 878
Aneurysmal bone cyst 371 Antiviral drug HPA-23 538 Auspitz’s sign 746
Angiocentric immunoproliferative Antoni A 204 Autoimmune theory 150
lesion 728 Antoni B 204, 205 Autoimmunity 236
Angioid streaks 751 Antral hematoma 664 Autosomal dominant inheritance 127
Angioedema 773 Antral pseudocyst 374 Autosomal recessive inheritance 127
Angiogenesis 73 ANUG 391 Auxochrome 36, 37
Angiography 192 Anvil shape joint 647 Azo dyes 87
Index
B Bird face appearance 135 Buccal bifurcation cyst 366

Birefringent specimen 15 Buccal caries 153
Bacterial meningitis 568
Bis-biguanides 165 Buccal exostosis 195
Bacterial sialadenitis 422
Bismuth grippe 682 Buccolingual masticatory syndrome 638 1007
Baelz’s disease 610
Bismuth line 682 Bud stage 301
Bag of marble appearance 666
Bismuthism 681 Bud-like uvula 247
Bag of worms 207
Bite marks 854, 855 Buffer capacity test 163
Bald tongue 488
Bite marks classification 854 Bulbar paralysis 836
Balloon cells 532
Bite marks types 854 Bulbous drop-shaped rete pegs 227
Ballooning degeneration 508
Bite test 549 Bull neck 473
Barbell 679
Black hairy tongue 633 Bull’s eye 732
Barrier filter 16
Blans syndrome 882 Bull’s eye lesion 842
Bartholin’s duct 412
Blast cell leukemia 720 Bullous impetigo 454
Basal cell adenoma 433
Blastomycosis 493 Bullous lichen planus 238, 239, 241
Basal cell ameloblastoma 311
Bleeder’s disease 710 Bullous pemphigoid 737
Basal cell carcinoma 267
Bloch-Sulzberger syndrome 743 Burkitt’s lymphoma 718, 719
Basal cell epithelioma 267
Block resection 312 Burning mouth syndrome 845, 846, 877
Basal cell hyperplasia 226
Blue domed cyst 346 Burning tongue syndrome 845
Basal cell nevus 885
Blue nevi 175, 177 Burtonian line 683
Basal cell papilloma 168
Blue nevus 175, 176 Butterfly distribution 251
Basaloid mixed tumor 445
Basaloid squamous carcinoma 269 Blue rubber bleb nevus syndrome 882
Blue sclera 596 C
Basaloid squamous cell carcinoma 269
Batsakis theory 430 Bluing 40 C banding 64
Batson’s plexus 266 Boat shaped head 133 Cabot rings 704
B-complex vitamins 812 Boeck’s sarcoid 769 Café-au-lait spot 207, 578
Beauty vitamin 813 Bohn’s nodules 359 Caffey’s diseases 593
Beckwith-Wiedemann syndrome 876 Bon-Bon sign 638 Caffey-Silverman syndrome 593,865
Bednar’s ulcer 657 Bone eburnation 600 Calcific degeneration 545
Behçet’s syndrome 776, 883 Bone impingement 649 Calcific metamorphosis 671
Bell stage 301 Bone marrow transplantation 538 Calcifying ameloblastoma 318
Bell’s palsy 835 Bone remodeling theory 120 Calcifying cystic odontogenic tumor 359
Benign lymphoepithelial lesion 427 Bone scar 600 Calcifying epithelial
Benign melanocytic nevi 174 Bone whorl 600 odontogenic cyst 359
Benign migratory glossitis 631, 632 Botryoid odontogenic cyst 358 Calcifying epithelial odontogenic
Benign mucous membrane Botryoid rhabdomyosarcoma 295 tumor 318
pemphigoid 738, 739 Bowen syndrome 865 Calcifying fibroblastic granuloma 182
Benign osteopetrosis 595 Bowen’s disease 232 Calcifying ghost cell
Beriberi 813 Brachial cyst of parotid 435 odontogenic cyst 359
Besnier-Boeck-Schaumann’s disease 769 Brachycephaly 133 Calcinosis cutis 761, 833
Beta thalassemia 696 Brain abscess 569 Calcinosis universalis 833
Bicellular theory 429 Branchial cleft cyst 376 Caliber persistent artery 140
Biermer’s anemia 702 Branchioma 430 Callus formation 76
Bifid condyle 645 Brandywine type 117 Canalicular adenoma 434
Bifid crown 100 Brawny indurations 567 Cancellous osteoma 189
Bifid rib syndrome 874 Brazilian pemphigus 735 Cancrum oris 471
Bifid tongue 626 Broder’s classification system 93 Candidal balanitis 492
Biologic carcinogenesis 89 Bronchial candidiasis 492 Candidal endocarditis 492
Biopsy 47 Bronzing of skin 796 Candidal leukoplakia 222, 488
Biotin 816, 827 Brown tumor 792 Candidal meningitis 492
Biphosphonates associated Brudzinski’s sign 568 Candidal onychia 491
osteonecrosis 689 Bruxism 676, 677 Candidal paronychia 491
Candidal septicemia 492 Cemento-ossifying fibroma 588, 589 Chondroblastoma 185

Candidal vulvovaginitis 492 Cementoblastoma 334 Chondrocytes 185
Candidiasis 222, 484, 529 Cementoma 334 Chondrodystrophia fetalis 599
1008 Candidiasis endocrinopathy Cement-osseous dysplasia 586 Chondroectodermal dysplasia 136, 864
syndrome 491 Cementum 77 Chondrogenic sarcoma 284
Candidosis 484 Cementum hyperplasia 675 Chondroma 184
Canker sores 774 Central chondroma 184 Chondromatous metaplasia 662, 686
Cannon’s disease 753 Central giant cell granuloma 582, 583 Chondrometaplasia 652
Cap stage 301 Central mandibular carcinoma 336 Chondromyxoid fibroma 185
Capdepont’s teeth 117 Central mucoepidermoid carcinoma 444 Chondrosarcoma 284-286
Capillary lymphangioma 200 Central osteoma 189 Chorea minor 638
Carabelli cusp 109, 460 Central papillary atrophy of tongue 630 Choriostoma 84, 192
Carcinogenesis 87 Central squamous cell carcinoma 336 Christ-Siemens-Touraine syndrome 876
Carcinoma ex odontogenic cyst 338 CEOT 320 Christmas disease 710
Carcinoma ex-mixed tumor 448 Cerebrocostomandibular syndrome 865 Chromatic aberration 7
Carcinoma ex-pleomorphic Cerebrohepatorenal syndrome 865 Chromogen 36, 37
adenoma 448 Cervical caries 153 Chromosomal banding 64
Carcinoma in situ 228, 231 Cervical enamel extension 108 Chromosomal disorders 862
Carcinoma of alveolar ridge 263 Cervical lymphadenopathy 722 Chromosome ideogram 65
Carcinoma of buccal mucosa 263 Cervical lymphoepithelial cyst 376 Chronic adrenal insufficiency 796
Carcinoma of floor of mouth 262 Cervical ranula 420 Chronic atrophic candidiasis 489
Carcinoma of labial mucosa 264 Cervicofacial actinomycosis 468 Chronic benign neutropenia 707
Carcinoma of lip 616 Cervicofacial emphysema 665 Chronic candidiasis 635
Carcinoma of maxillary sinus 265 Chancre 456 Chronic cavitary histoplasmosis 492
Carcinoma of palate 264 Chapping of lips 617 Chronic contact stomatitis 772
Carcinosarcoma 277, 449 Charcot’s triad 837 Chronic diffuse mucocutaneous
Cardinal signs of inflammation 542 Charm needles 679 candidiasis 491
Cardioid condenser 12 Charm pin 679 Chronic disseminated histiocytosis X 804
Cardiovascular syphilis 458 Chediak-Higashi syndrome 707, 881 Chronic familial mucocutaneous
Caries activity test 162 Cheilitis glandularis apostematosa 610 candidiasis 491
Carotid body tumor 206 Cheilitis granulomatosa 611 Chronic fatigue syndrome 521
Carpet track extension 251 Cheiloplasty 609 Chronic hyperplastic candidiasis 488
Cartilage cells 185 Chemical burns 659, 660 Chronic hyperplastic gingivitis 389
Cartilage hair hypoplasia 111 Chemical carcinogenesis 87 Chronic hyperplastic pulpitis 543, 545
Cartwheel pattern 180 Chemical measure of caries control 164 Chronic idiopathic neutropenia 707
Cascade of wound healing 72 Chemical oxidation 41 Chronic inflammation 86
Cat scratch disease 478 Chemical theory 38, 145 Chronic inflammatory enlargement 396
Cat scratch fever 478 Chemistry of stain 36 Chronic inflammatory periodontitis 402
Cavernous lymphangioma 200 Chemodectoma 206 Chronic localized histiocytosis X 804
Cavernous sinus thrombosis 569 Chemoparasitic theory 145 Chronic localized mucocutaneous
Celiac disease 811 Cherubism 580 candidiasis 491
Cell interaction 92 Chevron 180, 234 Chronic lymphatic leukemia 721, 724
Cell rest of malassez 301 Chick anti-dermatisis factor 807 Chronic mucocutaneous
Cell rests of serrae 301 Chicken wire arrangement 185 candidiasis 491
Cellular adenoma 432 Chickenpox 510 Chronic mucocutaneous candidiasis in
Cellular blue nevus 176 Child abuse 855 association with thymoma 491
Cellular changes 227 Chinese character shaped 579 Chronic myeloid leukemia 721, 723
Cellular nevus 174 Chip munk facies 697 Chronic neutropenia 707
Cellulitis 565 Chloroma 722 Chronic periapical abscess 549
Cemental caries 158 Chlorophyll 165 Chronic recurrent multifocal
Cementicles 676 Cholesterol cleft 364 osteomyelitis 563
Cementifying fibroma 588, 589 Choline 820 Chronic sclerosing sialadenitis 423
Index
Chronic sinusitis 481 Common mole 175 Cooley’s anemia 696

Chronic superficial interstitial Common wart 518 Coomb’s test 252
glossitis 458 Compact osteoma 189 Coronal dens invaginatus 104
Chronic suppurative osteomyelitis 559 Compensating eyepiece 8 Coronal dentin dysplasia 118 1009
Chronic tendoperiostitis 562 Complement-mediated cytotoxicity 766 Coronoid hyperplasia 643
Chronic tophaceous gout 651 Complex aphthous 774 Corps ronds and grain appearance 749
Chronic ulcerative stomatitis 781 Complex composite odontoma 330 Corticosteroid 241
Chvostek sign 793 Complex hemihyperplasia 129 Cotton roll burns 660
Cicatrical pemphigoid 738 Complicated fracture 680 Cotton roll stomatitis 660
Cigarette smoker’s lip lesion 235 Complicated ulcer 657 Counterstaining 40
CIN grading 93 Compound composite odontoma 330 Cowden syndrome 169, 760, 875
Circumvallate papillae 620 Compound microscope 3 Cracked tooth syndrome 549, 680
Cirsoid aneurysm 199 Compound nevi 175 Cranial arteritis 635, 844
Civatte bodies 240 Compound nevus 175, 176, 177 Craniocleido-dysostosis 132
Classification of caries 152 Compressibility test 197 Cranioectodermal dysplasia 870
Classification of stain 37 Compressible swelling 196 Craniofacial dysostosis 133, 864
Classification of syndrome 861 Compromised host 541 Craniofacial fibrous dysplasia 578
Claw hand 463 Concrescence 101 Craniopharyngioma 314
Cleaning of eyepiece 20 Condenser 4, 13, 16, 20 Craniosynostosis 133, 137
Clear cell ameloblastoma 311 Condensing osteitis 563 Craniotabes 822
Clear cell chondrosarcoma 286 Condylar hypoplasia 643, 644 Crank bugs 663
Clear cell odontogenic tumor or Condylar osteochondroma 193 Crenated tongue 634
carcinoma 337 Condyloma acuminatum 517 Crest syndrome 761
Clearing 26 Condyloma latum 457 Cretinism 790
Clearing agent 26 Confocal microscope 17 Cribriform honey comb pattern 446
Cleft lip 606, 607, 608 Congenita toxoplasmosis 502 Critical illumination 3
Cleft palate 121, 607, 608 Congenital agranulocytosis 705 Crocodile tears 835
Cleft tongue 626 Congenital epulis 208 Crohn’s disease 782
Cleidocranial dysostosis 132 Congenital epulis of newborn 208 Cross syndrome 386
Cleidocranial dysplasia 111, 132, 863 Congenital erythrokeratoderma 870 Crouzon’s disease 133
Clinical classification of fluorosis 113 Congenital facial diplegia 877 Crouzon’s syndrome 133, 864
Clinical staging of salivary Congenital fistulas 605 Crowe’s sign 207
gland tumor 429 Congenital granular cell epulis of Crown fracture 680
Cloacae 556 newborn 208 Cryptococcal meningitis 496
Clonus 632 Congenital granular cell tumor 208 Cryptococcosis 496
Clouding of sinus 591 Congenital leukokeratosis 753 Cryptogenic leukoplakia 223
Cluster headache 842, 879 Congenital lip pits 605 Cupid bow 606
Coagulation necrosis 548 Congenital macrogingivae 386 Cup-shaped depression 173
Coagulation vitamin 826 Congenital macroglossia 624 Curry-Hall syndrome 870
Coated tongue 633 Congenital myotonia 832 Cushing’s syndrome 592, 797, 883
Cobblestone’ appearance 687 Congenital nevi 174, 177 Cushion hammock ligament 120
Coccidioidal granuloma 497 Congenital nevus 175 Cutaneous blastomycosis 494
Coccidioidomycosis 497 Congenital rubella syndrome 514 Cutaneous horn 518
Codman’s tumor 185 Congenital syphilis 456 Cutaneous myiasis 477
Cold abscess 466 Congenital teeth 121 Cutaneous sporotrichosis 498
Cold sore 507 Congo red 44 Cutaneous T cell lymphoma 718
Colloidal gold label 58 Connective tissue tumor 449 Cutis hyperelastica 745
Colobomas 134 Contact cheilitis 613 Cutis rhomboidalis 617
Color codes 6 Continuum concept 326 Cutright lesion 662
Commissural 223 Contraceptive hormones 89 Cyanocobalamin 818
Commissural pits 605 Contraindication of biopsy 48 Cyclic hematopoiesis 706
Common blue nevus 176 Control of dental caries 164 Cyclic neutropenia 706
Cylindrical cell papilloma 170, 171 Dentinogenesis imperfecta 117 Discoid lupus erythematosus 250, 251
Cylindroma 445 Dentinogenic ghost cell tumor 359 Disease of hapsburgs 710
Cyst classification 343 Dentist as expert witness 859 Disease of kings 710
1010 Cyst definitions 343 Dentition proceox 121 Disorders of vitamins 811
Cyst lining 302 Dentoalveolar compensation 667 Disseminated aspergillosis 500
Cystic basal cell carcinoma 268 Denture epulis 685 Disseminated blastomycosis 494
Cystic complex odontoma 318 Denture injury tumor 685 Disseminated gonorrhea 461
Cystic hygroma 200, 201, 377 Denture stomatitis 489 Disseminated histiocytosis X 804
Cystic lymphangioma 200 Depapillation of tongue 635 Disseminated juvenile
Cysticercosis cellulose 379 Deparaffinization 40 fibrous dysplasia 580
Cysts of the maxillary sinus 372 Dercum’s disease 186 Disseminated scleroses 837
Cytogenetic 64 Dermal melanocytoma 175 Disseminated sporotrichosis 499
Cytokines 538 Dermal myiasis 477 Disseminated syphilis 457
Cytomegalovirus inclusion disease 422 Dermatitis 664 Distomolar 110
Cytomegalovirus infection 520 Dermatitis herpetiformis 742 Distorted taste 637
Dermatitis medicamentosa 770 Diverticuli 414
D Dermatitis venenata 771 DNA oncogenic virus 90
Dapsone therapy 242 Dermatofibroma 179 DNA profiling 854
Dardick’s theory 430 Dermatomyositis 636, 833 Dolichocephaly 870
Darier’s disease 749 Dermatopolyneuritis 683 Donovanosis 475
Darier-White disease 749 Dermatosis papulosa nigra 751 Double chin appearance 375
Dark field microscopy 11 Dermoid and epidermoid cyst 374 Double lip 606
Darling’s disease 492 Desert fever 497 Doubly refractive specimen 15
Dead tract 671 Desmoplastic ameloblastoma 313 Down’s syndrome 169, 598, 864
Decalcification method for hard Desmoplastic fibroma 180 Dressing 69
tissue 31 Desquamative gingivitis 392 Dried mud appearance 233
Decortication 560 Detergent 164 Driven snow appearance 319
Decubitus ulcer 657 Development of salivary gland 410 Dropping of jaw 833
Dedifferentiated chondrosarcoma 286 Developmental lingual mandibular Dropping-off effect 176
Deep subcutaneous hemangioma 196 salivary gland depression 412 Drug allergy 770
Degenerative arthritis 645 Dewar test 163 Drug idiosyncrasy 770
Dehydration 26, 40 Dewdrop on rose petal appearance 511 Drug induced discoloration 661
Delayed eruption 122 Diabetes 71, 636 Drug induced osteoporosis 592
Delayed hypersensitivity 767 Diabetes mellitus 794 Drug sensitivity 770
Deletion 65 Diabetes mellitus type I 795 Drug-induced lichenoid reactions 239
Denaturation 61 Diabetes mellitus type II 795 Dry beriberi 813
Dens evaginatus 105 Diabetic neuropathy 795 Dry socket 573
Dens in dente 103 Differentiation 40 Dubreuilh’s elastoma 617
Dens invaginatus 103 Diffuse hyperplastic oncocytosis 437 Ductal papillomas 438
Dense bone island 600 Diffuse infiltrative lymphocytosis Dukes ABC staging 93
Dental evaluation 850 syndrome 535 Dumpy 117
Dental floss 164 Diffuse linear calcification 547 During-Brocq disease 742
Dental fluorosis 113 Diffuse lipoma 186 Dysfibrinogenemia 713
Dental follicle 301, 302 Diffuse neonatal hemangiomatoses 882 Dysgeusia 637
Dental plaque 148 Diffuse sclerosing osteomyelitis 561 Dyskeratosis 227
Dental root end cyst 361 Dilaceration 102 Dyskeratosis congenita 249, 875
Dental root fracture 680 Dilated composite odontome 103 Dyskeratosis follicularis 749
Denticle 546 Diphtheria 473 Dyskinesia 638
Dentigerous cyst 345 Direct immunofluorescent technique 60 Dyspareunia 739
Dentin dysplasia 118 Direct staining 39 Dysplasia 82
Dentin hypocalcification 119 Direct-acting carcinogens 87 Dystrophic myotonia 831
Dentinal sclerosis 671 Disappearing bone 600 Dystrophic myotonica 831
Index
Endothelioma 430 Erythrodermic psoriasis 746

E
Enostosis 600 Erythrodontia 700
Eagle’s talon 102 Enterocystoma 629 Erythroleukoplakia 221, 230
Early syphilis 456 Enterovirus 514 Erythroplakia 229 1011
Earthy tongue 634 Enucleation 382 Erythroplasia 230
Ebbing tide 223, 234 Environmental enamel hypoplasia 111 Erythroplasia of queyrat 229
Ebnuration of dentin 161 Enzyme histochemistry 58 Erythropoietic porphyria 700
Ecchondroma 184 Eosin 42 Esophageal candidiasis 492
Ecchymosis 663 Eosinophilic granuloma 805 Esophageal dysmotility 761
Echinococcosis 378 Eosinophilic granuloma of bone 804 Esthesioneuroblastoma 293
Ecological plaque hypothesis 149 Eosinophilic ulceration 658 Estrogen 89
Ectodermal dysplasia 758, 759 Eosinophilic granuloma of tongue 635 Etiology of oral cancer 85
Ectopic enamel 108 Ephelis 755 Evaginated odontome 105
Ectopic eruption 124 Epidermolysis bullosa 740 Ewing’s sarcoma 289, 290
Ectopic geographic tongue 633 Epidermolysis bullosa Excisional biopsy 50
Ectopic salivary gland 412 acquista (acquired) 740 Exciter filter 16
Eczema herpeticum 508 Epidermolysis bullosa dystrophic Excrescences 233
Eczematous cheilitis 612 dominant 740, 741 Exfoliative cheilitis 615
Edward syndrome 864 Epidermolysis bullosa dystrophic Exfoliative cytology 51
Egg shell crackling 214, 307 recessive 740, 741 Exocrine glands 410
Ehlers Danlos syndrome 351, 745, 867 Epidermolysis bullosa latalis 740 Exophytic growth 264
Electrical burns 658 Epidermolysis bullosa simplex 740, 741 Exophytic osteoma 189
Electron gun 18 Epididymo-orchitis 421 Exostosis 195
Electron lenses 18 Epigenetic theory 91 Exostosis of root 675
Electron microscope 3, 17 Epithelial dysplasia 226 External callus 76
Elephantiasis gingiva 386 Epithelial odontome 303 External resorption 672
Elephantiasis neuromatosa 206 Epitheloid cell nevus 175 Extrafollicular AOT 322
ELISA 536 Epitheloid leiomyoma 211 Extranodal NK/T-cell lymphoma 728
Elliptical rima oris 246 Eponyms 861 Extraosseous ameloblastoma 314
Ellis Van Creveld Epstein’s pearls 359 Extraosseous osteosarcoma 287
syndrome 121, 136, 864 Epulis fissuratum 685 Extravasation cyst 370
Ely cyst 646 Epulis granulomatosum 687 Extrinsic factor of castle 807
Embedded cementicles 676 Erosion 669 Extropia 133
Embedded teeth 122 Erosion interdigitalis 491 Eye raised to heaven 581
Embedding 26 Erosive lichen planus 242 Eyepiece 16
Embedding center 26 Eruption cyst 349 Eyepieces 8
Embryonal rhabdomyosarcoma 295 Eruption hematoma 349
En coupe de sabre 131 Eruption of teeth 120
F
Enamel 77 Eruption sequestrum 124 Facial hemiatrophy 130, 635
Enamel agenesis 115 Erysipelas 454 Facial hemihypertrophy 129
Enamel hypoplasia 111 Erythema areata migrans 631 Facial pain 839
Enamel pearl 108 Erythema circinate migrans 632 Facial paralysis 835
Encapsulated lipoma 186 Erythema migrans 631 Facies leprosa 464
Encephalofacial angiomatosis 882 Erythema multiforme 731 Factor V deficiency 713
Enchondroma 184 Erythema multiforme major 732 Factors affecting fixation 25
Enclavoma 430 Erythema multiforme minor 731 Factors affecting staining 39
Encrusted tongue 634 Erythema nodosum leprosum 463 Factors affecting the wound healing 68
Endemic Burkitt’s lymphoma 719 Erythema nodosum 769 False gemination 101
Endemic parotitis 421 Erythematous candidiasis 488,530 False pocket 401
Endochondroma 430 Erythremia 704 Familial benign chronic pemphigus 740
Endosteal osteoma 189 Erythroblastic anemia 696 Familial fibrous dysplasia of the jaws 580
Endothelial myeloma 289 Erythroblastosis fetalis 699 Familial gigantiform cementoma 587
Familial hypophosphatemia 823 Fluorescent in situ Frontonasal dysplasia syndrome 865

Familial multilocular cystic disease of the hybridization (FISH) 63 Frozen section 32
jaws 580 Fluorescent microscope 3 Frozen section biopsy 56
1012 Familial neutropenia 707 Fluorine 164 Functions of tongue 622
Familial or heredofamilial Fluorite 7 Fungiform papillae 620
amyloidosis 802 Flushing 834 Fungiform papilloma 170, 171
Familial osteodysplasia 865 Fly-catcher’s sign 638 Fusion 100
Familial pancytopenia 874 Foam cells 172 Fusospirochetal gingivitis 391
Familial panmyelophthisis 874 Focal cement-osseous dysplasia 586
Familial thrombasthenia 710 Focal dermal hypoplasia syndrome 169 G
Fanconi’s anemia 698 Focal epithelial hyperplasia 518 G banding 64
Fanconi’s syndrome 874 Focal fibrous hyperplasia 178 Galvanism 222
Farmer’s lip 613 Focal length 5 Ganglioneuroma 208
Fat soluble vitamins 821 Focal melanosis 138 Gangrenous stomatitis 471
Fetal deciduous teeth 121 Focal osteoporosis bone GAPO 870
Fetal rhabdomyoma 212 marrow defect 135 Gardener’s syndrome 601, 111, 180,
Fever blister 507 Focal periapical osteosclerosis 600 190, 873
Fibrinolytic alveolitis 573 Focal point 11 Gastric cyst 629
Fibroadamanblastoma 326 Focal sclerosing osteomyelitis 563 Gaucher’s disease 804 , 806
Fibrolipoma 189 Folacin 817 Gemination 99
Fibroma 178, 181 Folate 817 General adaptation syndrome 884
Fibroma molluscum 206 Foliate papillae 620 Generalized aggressive periodontitis 404
Fibromatosis 180 Foliate papillitis 634 Generalized neurological disease 638
Fibromatosis gingiva 386 Folic acid 817 Genetic factors 862
Fibromyxomas 334 Folic acid deficiency 636 Genetic theory 91
Fibro-osseous lesions classification 576 Follicular ameloblastoma 308 Geniculate neuralgia 841
Fibrosarcoma 279 Follicular AOT 321 Genital wart 517
Fibrosarcoma variants 282 Follicular cyst 345 Geographic tongue 631
Fibrous adamantinoma 326 Follicular cysts of the skin 378 Geotrichosis 498
Fibrous degeneration 546 Follicular lichen planus 239 German measles 514
Fibrous dysplasia 577 Folliculosis 819 Gestant odontome 103
Fibrous dysplasia of dentin 119 Foot and mouth disease 516 Ghost cells 360
Fibrous histiocytoma 179 Forchheimer sign 514 Ghost teeth 118
Fibrous nodule 178 Fordyce granule 138 Giant cell arteritis 844
Fibroxanthoma 179 Fordyce’s granules 84 Giant cell epulis 214
Field cancerization 220 Foreign bodies 69 Giant cell fibroma 180, 181
Fiery red tongue 703 Foreign body gingivitis 394 Giant cell lesion 213
Filiform papillae 620 Forensic odontology 849 Giant cell tumors 213
Filter 15 Forked tongue 679 Giant cells 181
Fimbriated fold 620 Formaldehyde fixed tissue 29 Giant hairy nevus 174
Fine needle aspiration cytology 55 Formication 663 Giant osteoid osteoma 190
Fingerprinting 853 Fosdick calcium dissolution test 163 Giant urticaria 773
First week wound 75 Fothergill’s disease 839 Giemsa stain 46
Fissured tongue 629 Fournier’s molar 112 Gigantism 786
Fixation 25, 29 Fourth week wound 75 Gilbert syndrome 825
Fixation in histochemistry 57 Fracture of teeth 680 Gilles de la tourette’s syndrome 638
Flashlamp pulsed dye laser 199 Franceschetti syndrome 134 Gingival abscess 394
Flat torus 194 Freckle 755 Gingival cyst of adults 356
Florid cement-osseous dysplasia 586 Free cementicles 676 Gingival cyst of newborn 355
Flow cytometry 61 Frenal tag 179 Gingival enlargement due to drugs 397
Fluctuation test 375 Frey’s syndrome 834, 878 Gingival inflammation 388
Fluorescence microscopy 15 Friedreich’s disease 129 Gingival pocket 401
Index
Gingival salivary gland choristoma 412 Granuloma venereum 475 Hemangioma of infancy 196
Gingivitis 388 Granulomatous cheilitis 611 Hemangiopericytoma 202
Glandular cheilitis 610 Granulomatous gingivitis 394 Hemarthrosis 711
Glandular fever 521 Groove sign 477 Hematoma 663 1013
Glandular odontogenic cyst 358 Ground glass appearance 578, 792 Hematoxylin stain 40
Glanzmann’s disease 710 Ground section 30 Hemidesmosomal 740
Glassblower’s white patch 689 Gubernacular dentis 302 Hemifacial hyperplasia 129
Globodontia 108 Gum boil 550 Hemifacial microstomia 130
Globulomaxillary cyst 370 Gumma 458 Hemihyperplasia 129
Glomangioma 201 Gummy smile 129 Hemimaxillofacial dysplasia 131
Glomus jugulare tumor 206 Gustatory sweating 834, 878 Hemodialysis associated amyloidosis 802
Glomus tumor 201 Guttate lichen planus 239 Hemoglobin Bart disease 696
Glomus tympanicum tumor 206 Guttate psoriasis 746 Hemoglobin H disease 696
Glossitis areata exfoliativa 631 Hemophilia 710
Glossopharyngeal neuralgia 840 H Hemophilia A 710
Glossoplegia 638 Habitual abrasion 668 Hemophilia B 710
Glossopyrosis 845 Habitual cheek biting 656 Hemophilia C 711
Goblet cells 171 Habitual lip biting 656 Hemorrhagic bone cyst 370
Gonococcal ophthalmia Hailey-Hailey disease 740 Hemostasis 72
neonatorum 461 Haim-Munk syndrome 406 Henderson-Paterson inclusion 519
Gonococcal stomatitis 461 Hairy leukemia 725 Hepadnavirus 90
Gonorrhea 460 Hairy leukoplakia 532 Hereditary benign intraepithelial
Gonzalez-Crussi grading of Hairy tongue 633 dyskeratosis 754
teratoma 84 Hajadu-Cheney syndrome 865, 876 Hereditary brown enamel 114
Gordon and Sweet’s method for Hallmarks of cancer 87 Hereditary brown opalescent teeth 114
reticulin fiber 43 Hallopeau type pemphigus 735 Hereditary disease of newborn 699
Gorham syndrome 600 Halo nevus 175, 177 Hereditary ectodermal dysplasia 868
Gorlin-Goltz syndrome 351, 380, 874, 885 Hamartoma 83, 168 Hereditary elliptocytosis 699
Gorlin cyst 360 Hamman’s crunch 666 Hereditary enamel dysplasia 114
Gorlin sign 628, 745 Hand foot mouth disease 516 Hereditary fibrous dysplasia of
Gougerat-Sjögren’s syndrome 872 Hand-Schuller-Christian disease 804 the jaws 580
Gout 651 Hansen disease 462 Hereditary hemorrhagic
Grading and staging of tumors 93 Haptens 91 telangiectasia 754, 875
Grading of dysplasia 228 Hard fibroma 179 Hereditary hypohidrotic (anhidrotic)
Grading of malignant melanoma 276 Hard tissue microtome 31 ectodermal dysplasia 758
Graft versus host disease 243 Harrison grooves 823 Hereditary hypohydrotic ectodermal
Graft versus host resistance 760 Healing cyst 366 dysplasia 870
Graham little syndrome 237, 239 Healing of biopsy wounds 74 Hereditary mucoepithelial dysplasia 751
Granular cell ameloblastoma 310 Healing of extraction wounds 74 Hereditary multiple exostosis 193
Granular cell myoblastoma 212 Healing of fractures 75 Hereditary opalescent dentin 117
Granular cell neural fibroma 21 Healing of osseointegrated implants 76 Hereditary spherocytosis 699
Granular cell odontogenic fibroma 333 Healing of pulp 76 Herlitz’s disease 740
Granular cell odontogenic tumor 333 Healing of skin 77 Herpangina 515
Granular cell Schwannoma 212 Healing of wound of oral mucosa 77 Herpes associated erythema
Granular cell tumor 212 Healing response in oral mucosa 78 multiforme 732
Granular erythroplakia 230 Hebra nose 474 Herpes barbae 508
Granulation 73 Heck disease 518 Herpes gladiatorum 508
Granulation tissue 74 Heerfordt’s syndrome 428, 769, 873 Herpes simplex infection 505
Granulocytopenia 705 Heliotrope 833 Herpes zoster 512, 535
Granuloma gravidarum 398 Hemangioendothelial sarcoma 291 Herpes zoster ophthalmicus 513
Granuloma inguinale 475 Hemangio-lymphangioma 201 Herpetic paronychia 508
Granuloma pyogenicum 399 Hemangioma 196 Herpetic whitlow 508
Herpetiform ulcers 774 Hyalinosis cutis et mucosa oris 752 Idiopathic steatorrhea 811
Herring bone 180 Hybridization method 62 Idiopathic thrombocytopenic
Herring bone pattern 207, 280 Hybridoma technique 58 purpura (ITP) 535, 708
1014 Heterotrophic gastrointestinal cyst 84 Hydatid cyst 378 Illumination 3
Hibernoma 187 Hydatid disease 378 Image formation in microscope 10
Hidebound disease 761 Hydration 40 Immediate hypersensitivity 766
Higoumenakis’s sign 459 Hyperbaric oxygen therapy 560 Immune reactions 766
Histiocytosis X 804 Hypercementosis 675 Immune surveillance theory 91
Histiocytosis Y 171 Hypercortisolism 797 Immunodeficiency 236
Histochemical stain 38 Hyperdontia 110 Immunodeficiency associated Burkitt’s
Histology 22 Hypermobility 650 lymphoma 719
Histopathology 22 Hyperorthokeratinization 225 Immunofluorescent study 241
Histoplasmosis 492, 536 Hyperostosis 195 Immunofluorescent technique 59
History of microscope 1 Hyperparakeratinization 225 Immunofluorescent test 736
Histotechnique 22 Hyperparathyroidism 791 Impacted teeth 122
HIV 524 Hyperpigmentation 536 Impaired glucose tolerance 794
HIV associated salivary gland Hyperpituitarism 785 Impetiginized dermatitis 454
disease 535 Hyperplasia 82, 168, 194 Impetigo 453
HIV virus 526, 527 Hyperplasia of salivary gland 413 Impetigo vulgaris 453
HME 193 Hyperplastic neutropenia 707 In situ hybridization 63
Hodgkin’s lymphoma 714 Hypersensitivity reaction 766 Incipient carcinoma 230
Homogenous erythroplakia 230 Hypertaurodont 106 Incisional biopsy 48, 231
Homogenous leukoplakia 224 Hypertelorism 133 Incisive canal cyst 366
Homozygous β-thalassemia 696 Hyperthyroidism 788 Incontinentia pigmenti 743, 871
Honeycomb appearance 196, 308 Hypertrophic lichen planus form 238, 239 Indication of biopsy 47
Hormonal carcinogenesis 89 Hypertrophy 168 Indirect Immunofluorescent
Hormone dependant tumors 89 Hypervitaminosis A 822 technique 60
Hormone related amyloid 802 Hypoadrenocorticism 796 Indirect staining 39
Hormones 222 Hypodontia 109 Indirect-acting carcinogens 87
Horn cyst 751 Hypofibrinogenemia 713 Infantile osteopetrosis 594
Horner’s syndrome 880 Hypogeusia 637 Infantile cortical hyperostosis 593, 865
Horton’s syndrome 842, 879 Hypoglycemia 809 Infantile osteomyelitis 560
Hypognathous 128
Host defense 541 Infantile scurvy 819
Hypoparathyroidism 793
Hot start PCR 62 Infected periapical lesion 571
Hypophosphatasia 810
Hound dog appearance 752 Infected ulcer 658
Hypopituitarism 787
Hour-glass manner 215 Infectious mononucleosis 521
Hypoplasia of gland 412
Howell-Jolly bodies 704 Infinity corrected optics 8
Hypoplasminogenemia 712
HTLV-III virus 526 Inflammation 73
Hypotaurodont 106
Human abuse 858 Inflammatory collateral cyst 365
Hypotelorism 136
Human herpes virus 505 Inflammatory fibrous hyperplasia 685
Hypothyroidism 790
Human T-cell lymphotropic virus 89 Inflammatory papillary hyperplasia 686
Humoral theory 145 Inflammatory radicular cyst 361
I
Hunter’s glossitis 818 Infrabony pocket 401
Huntington’s chorea 638 Iceberg tumor 430 Infravital staining 39
Hurler syndrome 807, 876 Id reaction 489 Inositol 820
Hutchinson-Gilford syndrome 884 Identification 850 Integration 90
Hutchinson’s freckles 274 Idiopathic bone cavity 370 Interdigital candidiasis 491
Hutchinson’s incisor 109, 112 Idiopathic histocytosis 804 Interface dentin 671
Hutchinson’s triad 459 Idiopathic leukoplakia 223 Interferon 537
Hutchinson’s sign 513 Idiopathic midline destructive Intermediate osteopetrosis 594
Huygenian eyepieces 8 disease 728 Internal callus 76
Hyaline cartilage 184 Idiopathic osteosclerosis 600 Internal resorption 673
Index
Interproximal caries 153 Juvenile myxedema 790 Lacrimo-auriculo-dento-digital

Intertriginous candidiasis Juvenile ossifying fibroma 590 syndrome 413, 874
Intra-alveolar cyst 370 Juvenile periodontitis 403 Lactobacillus count test 162
Intra-alveolar pocket 401 Lacy white pattern 243 1015
Intrabony pocket 401 K LADD 413
Intradermal nevi 175, 176, 177 Kaposi’s sarcoma 530 Lane tumor 277
Intraductal papilloma 438, 439 Kaposi’s varicelliform eruption 508 Langerhans cell disease 804
Intraepidermoid carcinoma 232 Karyogram 65 Laser ablation 614
Intraepithelial carcinoma 231 Karyotype analysis 65 Laser capture microdissection 63
Intraepithelial edema 226 Karyotyping 64 Laser-Trelat sign 751
Intralingual cyst of foregut origin 377 Kawasaki syndrome 763, 871 Late syphilis 456
Intramuscular lipoma 189 Keloid 74 Latent bone cyst 137
Intra-nuclear inclusions 508 Keratin horn 168, 518 Lateral facial cleft
Intraosseous mucoepidermoid Lateral periodontal cyst 357
Keratin pearl 260
carcinoma 444 Lateral radicular cyst 362
Keratin pit 173
Inversion 65 Lateral soft tissue fistulas 141
Keratinizing and calcifying
Inverted ductal papilloma 438, 439 LAV virus 526
odontogenic cyst 359
Inverted follicular keratosis Helwig 751 Lazy leukocyte syndrome 706, 881
Keratinizing metaplasia 821
Inverted papilloma 170, 171 Leaf like denture fibroma 686
Keratoacanthoma 173
Inverted schneiderian papilloma 170 Legend of worm 145
Keratoameloblastoma 311, 312
Inverted tear drop 367 Leiomyofibrillogenic capacity 212
Keratocarcinoma 173
Investigation of bite mark 857 Leiomyoma 211
Keratoconjunctivitis sicca 426
Involucrum 556 Leiomyosarcoma 294
Keratocyst radiological types 352
Iris lesion 732 Leishmaniasis 502
Keratosis 225
Iron deficiency anemia 636, 700, 880 Lemmoma 204
Keratosis follicularis 749
Iron hematoxylin 41 Lens 4
Irregular dentin 671 Keratotic basal cell carcinoma 268 Lentigo maligna melanoma 274
Irreversible pulpitis 543 Kernicterus 700 Lentigo melanoma 276
Irritation fibroma 178 Kernig’s sign 568 Lentigo simplex 756
Ischemic myelofibrosis 842 Kinetics of tumor cell growth 90 Lentigo solaris 756
Isolated darier’s disease 750 Kissing disease 521 Leong’s premolar 105
Isotropic specimen 15 Kissing lesion 631 Leonine facies 463
Ivory osteoma 189 Klestadt’s cyst 369 Leontiasis ossea 584
Klinefelter syndrome 107 LEOPARD syndrome 870
J Klippel-Trenaunay syndrome 886 Leporma 463
Jadassohn-Lewandowsky syndrome 747 Klippel-Trenaunay-Weber Lepromatous leprosy 462
Jadassohn-Tieche nevus 175 syndrome (KTW) 878 Lepromin test 464
Jaffe’s fibrous dysplasia 578 Koebner phenomenon 237 Leprosy 462
Jaffe-Lichtenstein syndrome 577 Kohler illumination 3 Leptomeningeal angiomatosis 200
James Ramsay Hunt syndrome 510, 514 Koilocytes virus 169 Letterer-Siwe disease 804, 806
Jarisch-Herxheimer reaction 460 Koilocytosis 534 Leukemia 427, 720
Jaundice 825 Koilonychias 701 Leukemia classification 720
Jaw Winking syndrome 880 Koplik spots 510 Leukemia types 720
Jaw cyst-basal cell nevus-bifid Korsakoff’s psychosis 813 Leukemoid reaction 720
rib syndrome 380 Kuttner’s disease 423 Leukoedema 140
Johanson-Blizzard syndrome 870, 874 Kveim-Siltzbach test 769 Leukokeratosis nicotina glossi 634
Junctional bullous epidermatosis 740 Kwashiorkor disease 800 Leukoplakia 220
Junctional epidermolysis bullosa 740, 741 Leukoplakia erosiva 221, 224
Junctional nevi 177 L Leukoplakia simplex 221, 224
Junctional nevus 175 Laband syndrome 878 Leukoplakia verrucosa 221, 224
Juvenile aggressive fibromatosis 180 Labial melanotic macule 138 Leutic glossitis 458
Juvenile melanoma 175 Labret 679 Libman-Sacks endocarditis 251
Lichen planopilaris 239 Local infection 69 Malignant mixed tumor 448

Lichen planus 60, 235 Localized aggressive periodontitis 404 Malignant odontoma 318
Lichen planus pemphigoides 239 Localized myositis ossificans 838 Malignant osteopetrosis 595
1016 Lichen planus pigmentosus 239 Localized or organ limited Malignant peripheral nerve sheath
Lichen planus types 236 amyloidosis 802 tumors 293
Lichen planus-lupus erythematosus Location of wound 69 Malignant Schwannoma 293
overlap 240 Lofgren’s syndrome 769 Mallory’s phosphotungstic acid
Lichenoid contact dermatitis 240 Loose bodies 652 hematoxylin for muscle striation 43
Lichenoid contact stomatitis 779, 780 Loss of polarity 227 Mandibular buccal infected cyst 366
Lichenoid tissue reaction 779 Lou Gehrig disease 836 Mandibular tori 195
Lie bump 777 Love bites 857 Mandibulofacial dysostosis 134, 866
Liesegang rings 319 Low grade fibrosarcoma 281 Maran-Achard syndrome 597
Light microscope 3 Ludwig’s angina 566 Marasmus 800
Light source 15 Lues 455 Marble bone disease 594
Ligneous conjunctivitis 712 Lues maligna 457 Marcus gun phenomenon 880
Limb defect 137 Lumber lordosis 823 Marfan’s syndrome 351, 597, 867
Linea alba 655 Lupus cheilitis 252 Marginal acute gingivitis 507
Linear gingival erythema 533 Lupus erythematosus 61, 250 Marie and Sainton disease 132
Linear lichen planus 239 Lupus pernio 769 Marsupilization 381
Lingua dissecta 629 Lupus vulgaris 466 Mask of pregnancy 797
Lingua villosa 633 Lyell’s disease 733 Mass fatality index (MFI) 852
Lingual caries 153 Lymph nodes 259 Massive osteolysis 600
Lingual cortical mandibular defect 137 Lymphangioma 200 Masson trichrome 43
Lingual cyst 629 Lymphangioma simplex 200 Mast cells 240
Lingual mandibular salivary gland Lymphogranuloma venereum 476 Maxillary sinusitis 479
depression 137 Lymphoid hyperplasia 693 McCune-Albright’s syndrome 577
Lingual polyp 629 Lymphokines 538 Measles 509
Lingual thyroid nodule 84, 627 Lympho-proliferative malignancy 425 Mechanical measure for caries
Lingual tonsil 694 Lymphosarcoma 716 control 164
Lingual varicosities 627 Mechanical stress 70
Lip disorders classification 604 M Mechanical tube 7
Lip ulcers due to caliber persistent Macrodontia 99 Mechanical tube length 8
artery 617 Macroglobulinemia 714 Mechanism of action of chemical
Lipid metabolism 804 Macroglossia 200, 624 carcinogen 88
Lipid proteinosis 808 Macrognathia 129 Mechanism of distant metastases 90
Lipid reticuloendothelioses 804 Maffucci’s syndrome 882 Mechanism of DNA viral
Lipoblastoma 187 Magical charm 679 oncogenesis 90
Lipoblastomatosis 187 Magnification of microscope 6 Mechanism of local invasion 90
Lipoid proteinosis 752 Main’s theory 345 Mechanism of RNA viral
Lipoma 186 Maintenance of laboratory oncogenesis 89
Lipomatosis 186 microscope 19 Mechanistic theory 92
Liposarcoma 283 Major aphthae 774 Meckel’s cartilage 184
Lipschutz bodies 508 Major salivary gland 410 Median anterior maxillary cyst 366
Liquefaction foci 156 Malabsorption syndrome 811 Median cleft face syndrome 865
Liquefaction necrosis 548 Malignancy 71 Median cleft of lip 608
Liquid base cytology 55 Malignant ameloblastoma 335 Median mandibular cyst 370
Lisch bodies 207 Malignant fibrous histiocytoma 282 Median palatine cyst 368
Liver spot 756 Malignant fibroxanthoman 282 Median rhomboid glossitis 489, 630, 796
Ljubljana classification system 228 Malignant granular cell myoblastoma 296 Mediastinitis 570
Lobular carcinoma 447 Malignant hemangioendothelioma 291 Mediterranean anemia 696
Lobular pattern 188 Malignant hemangiopericytoma 291 Megadontia 99
Lobular torus 194 Malignant melanoma 273 Megagnathia 129
Index
Melanin incontinence 138, 756 Mikulicz’s disease 427 Mucoepidermoid cyst 358
Melanoameloblastoma 209 Mikulicz’s disease proper 427 Mucopolysaccharidosis 809, 876
Melanocytic nevus 174 Mild neutropenia 705 Mucormycosis 495
Melanotic ameloblastoma 209 Mild periodontitis 402 Mucositis 664 1017
Melanotic neuroectodermal tumor of Mild restricted muscular Mucous membrane pemphigoid 60
infancy 209 dystrophy 831 Mucous patches 457
Melanotic progonoma 209 Miliary tuberculosis 465 Mucous salivary gland 410
Melasma 797 Miliary tubercle 467 Mucus duct cyst 419
Melkerson Rosenthal Miller’s acidogenic theory145 Mucus escape phenomenon 417
syndrome 413, 630, 875 Minor aphthae 774 Mucus extravasation phenomenon 417
Melnick Needles syndrome 866 Minor salivary gland 410 Mucus retention cyst 419
Membranous basal cell adenoma 433 Minor starch-iodine test 835 Muir-Torre syndrome 173
MEN syndrome 205, 885 Mixed odontogenic tumors 325 Mulberry molar 108, 112
Mercurialism 683 Mixed tumor 430 Multifactorial inheritance 863
Merkel cell carcinoma 279 Mobius syndrome 877 Multifocal eosinophilic granuloma 804
Merkel cell tumor 279 Moderate neutropenia 705 Multifocal epithelial hyperplasia 518
Mesenchymal chondrosarcoma 287 Moderate periodontitis 402 Multifocal papilloma virus epithelial
Mesiodens 110 Moderately differentiated SCC 260 hyperplasia 518
Mesocrine glands 410 Mohr syndrome 866 Multihead demonstration eyepiece 9
Multilocular cyst 303
Mesomelic 136 Mohs micrognathic surgery 268
Multinucleated giant cells 185, 215
Mesotaurodont 106 Molar incisor hypomineralization 114
Multiple angiofibroma 758
Metachromatic stain 38 Molluscum contagiosum 536
Multiple carcinomas 265
Metachromatic staining 39 Molluscum contagiosum infection 519
Multiple endocrine neoplasia
Metallic impregnation 39 Molluscum sebaceum 173
syndromes 885
Metaplasia 82 Mongolism 598
Multiple hamartoma
Metastasis 92 Mongoloid appearance 697
syndrome 386, 760, 875
Metastasizing mixed tumor 449 Mono 521
Multiple myeloma 725
Metastatic 263 Monoclonal antibodies therapy 538
Multiple PCR 62
Metastatic carcinoma 266 Monoclonal hypothesis 91
Multiple sclerosis 837
Meth sores 663 Monomelic fibrous dysplasia 577 Multistep theory 92
Methamphetamine abuse lesion 663 Monosomic 65 Mumps 421
Mibelli’s disease 748 Monostotic fibrous dysplasia 577 Murray-Puretic-Drescher syndrome 386
Mickey mouse ears 501 Moon molars 460 Muscles of tongue 621
Mikulicz’s aphthae 774 Moon’s molar 109, 112 Muscular dystrophy 830
Microbial homeostasis 149 Morbilli 509 Myasthenia gravis 832
Microcyst 441 Mordant 41 Mycobacterium infection 535
Microdontia 98 Morphea 762 Mycosis fungoides 718
Microglossia 623 Morsicatio buccarum 656 Myeloid leukemia 720
Micrognathia 128 Morsicatio labiorum 656 Myelomatosis 725
Micrometry 9 Morsicatio lingurum 657 Myiasis 477
Microstomia 135 Moth eaten appearance 262, 290 Myoblastic myoma 212
Microtophi 652 Mother of pearl appearance 140 Myoepithelioma 432, 438
Microwave biopsy 29 Motor neuron disease 836 Myopathic facies 831
Microwave oven 28 Mottled enamel 113 Myositis ossificans 837
Microwave stimulated processing 28 Mounting 33 Myospherulosis 666
Microwave tissue processing 28 Mouse species 762 Myotonias 831
Midline lethal granuloma 728 Muciphage 418 Myotonic dystrophy 831
Midline malignant reticulosis 728 Mucocele 373, 417 Myxadenitis labialis 610
Miescher’s syndrome 611, 875 Mucocutaneous leishmaniasis 502 Myxedema 790
Migraine 843, 879 Mucocutaneous lymph node Myxofibroma 183, 333
Migraine syndrome 879 syndrome 763, 871 Myxoid chondrosarcoma 286
Migrainous neuralgia 842 Mucoepidermoid carcinoma 440 Myxoma 182
Niemann-Pick disease 804, 807 Odontogenic hamartoma 83

N
Nikolsky’s phenomenon 734 Odontogenic infection of orbit 570
Naegeli-Franceschetti-Jadassohn Nikolsky’s sign 734, 740 Odontogenic keratocyst 350
1018 syndrome 874 Nitrofurans 165 Odontogenic myxoma 333
Nasoalveolar cyst 369 Nodular elastoidosis 617 Odontogenic tumors classification 300
Nasolabial cyst 369 Nodular fasciitis 688 Odontogram 852
Nasopalatine cyst 366 Nodular leukoplakia 224 Odontoma 329
Nasopalatine duct cyst 366 Nodular melanoma 274, 276 OFD II 866
Nasopharyngeal angiofibroma 203 Nodular oncocytic hyperplasia 437 OFD orofacial digital syndrome II 866
Nasopharyngeal carcinoma 278 Nodular subepidermal fibrosis 179 Oil red O stain 44
Nasopharyngeal cyst 378 Nodular torus 194 Olfactory neuroblastoma 293
Natal teeth 121 Noma 471 Oligodontia 109
Natural oxidation 41 Noma neonatorum 471 Ollier’s syndrome 184
Necrosis of pulp 548 Nonodontogenic hamartoma 83 OLP staging 225
Necrotizing fasciitis 571 Nonspecific plaque hypothesis 149 Oncocytic schneiderian papilloma 170
Necrotizing sialometaplasia 450 Non-bullous impetigo 454 Oncocytoma 437
Necrotizing stomatitis 391, 471, 533 Nondisjunction 65 Oncocytosis 437
Necrotizing ulcerative gingivitis 391, 533 Non-Hodgkin lymphoma 534, 716 Oncogenes 91, 304
Necrotizing ulcerative Non-lipid reticuloendothelioses 804 Optical aberrations 6
mucositis 391, 471 Nonradiation carcinogenesis 88 Optical maintenance 19
Necrotizing ulcerative Noonan’s syndrome 351 Oral alimentary tract cyst 377
periodontitis 391, 533 Normal cell cycle 85 Oral amyloidosis 726
Neonatal teeth 121 Northern blot technique 62 Oral cancer 255
Neoplasm 85, 168 Nosepiece 7 Oral cancer classification 256
Nerve sheath myxoma 183 Nuclear beading 532 Oral cancer etiology 256
Nested PCR 62 Nuclear staining 40 Oral cancer risk factors 258
Neumann type pemphigus 735 Numb chin syndrome 267 Oral candidiasis 486
Neumann’s tumor 208 Numerical aperture 6 Oral cyst with gastric epithelium 377
Neuralgia inducing cavitational Nursing bottle caries 160 Oral cyst with intestinal epithelium 377
osteonecrosis 841 Nutritional deficiency 222 Oral disorders in HIV disease 528, 529
Neurilemmoma 204 Nutritional measure of caries Oral epithelial nevi 753
Neurinoma 204 control 164 Oral florid papillomatosis 225
Neuroblastoma 292, 293 Oral focal mucinosis 182
Neuroendocrine carcinoma of skin 279 O Oral foci of infection 571
Neurofibromatosis 1 884 Object stage 5 Oral human papilloma virus lesions 534
Neurofibromatosis 2 884 Objective 13, 16, 20 Oral lesion as complication to
Neurofibromatosis 206 Objective of microscope 5 anti-neoplastic therapy 664
Neurofibrosarcoma 293, 294 Obligatory myiasis 477 Oral lymphoepithelial cyst 376
Neurogenic sarcoma 293 Oblique facial cleft 608 Oral melanoacanthoma 174
Neuroma 203 Occlusal caries 156 Oral mucosal brush biopsy 54
Neurosarcomas 293 Occlusal enamel pearl 105 Oral physiotherapy 249
Neurosyphilis 458 Occupational abrasion 668 Oral piercing 679
Neutrons-tropic ulcer 657 Oculodentodigital dysplasia 874 Oral sebaceous hyperplasia 757
Nevoid basal cell carcinoma 380 Oculoglandular syndrome of Oral squamous cell carcinoma 258
Nevoid basal cell carcinoma parinuad 478 Oral submucous fibrosis 243
syndrome 874, 885 Odontoameloblastoma 331 Oral ulceration with bone
Nevus flammeus 200 Odontogenesis 301 sequestration 662
Nevus unius lateris 169 Odontogenesis imperfecta 117 Oral-facial-digital syndrome 626, 876
Niacin 814, 827 Odontogenic dysplasia 118 Orofacial gangrene 471
Niacin deficiency 636 Odontogenic fibroma 332 Oropharyngeal aspergillosis 500
NICO 841 Odontogenic fibromyxoma 333 Orthochromatic stain 38
Nicotine palatinus 233 Odontogenic fibrosarcoma 339 Osler’s disease 704, 875
Index
Osler-Rendu-Weber syndrome 754, 875 Palatal epithelial hyperplasia 686 Pattern of metastatic speed 92
OSMF clinical staging 248 Palatal fauces 247 PCNA 351
OSMF functional staging 248 Palatal papillomatosis 686 Pellagra 815
OSMF grading 248 Palatal tubercle 195 Pelvic inflammatory disease 461 1019
OSMF malignant potential 249 Palatine torus 194 Pemphigus 734
OSMF management 249 Pallor 701 Pemphigus erythematosus 735
OSMF pathogenesis 245, 246 Palmoplanter keratosis 760 Pemphigus foliaceus 735
Osseous metaplasia 686 Panhypopituitarism 787 Pemphigus vegetans 735
Ossifying fibroma 588, 589 Pansinusitis 479 Pemphigus vulgaris 60, 734
Ossifying fibrous epulis 182 Pantothenic acid 816 Penicillin 165
Osteitis deformans 584 Paper test 682 Peri-adenitis mucosa necrotica
Osteitis fibrosa cystic 792 Papillae 620 recurrent 774
Osteoarthritis 645 Papillae simplices 620 Periapical abscess 549
Osteoarthrosis 645 Papillary cystadenoma Periapical cement-osseous dysplasia 586
Osteoblastoma 190, 192 lymphomatosum 435 Periapical cyst 361
Osteochondroma 192, 194 Papillon-Lefevre syndrome 405, 878 Periapical granuloma 553
Osteoclastoma 214 Papovavirus 90 Periapical pocket cyst 362
Osteodysplasty 866 Papular lichen planus 241 Periapical scar 554
Osteogenesis imperfecta 596 Papyraceous scarring 745 Periapical true cyst 362
Osteogenesis imperfecta types 596 Para pemphigus 737 Pericoronal abscess 395
Osteogenic sarcoma 287 Paraboloid condenser 12 Pericoronal cyst 345
Osteoid osteoma 191, 192 Paracoccidioidomycosis 501 Pericoronitis 395
Osteoma 189 Paradental cyst 365 Peridens 110
Osteoma cutis 189 Paraganglioma 206 Perimolysis 670
Osteomalacia 823 Parahemophilia 713 Perineural fibroblastoma 204
Osteomatosis 190 Parakeratin plugging 172, 272 Periodic acid Schiff method 42
Osteomyelitis 555 Paralysis of tongue 638 Periodic migrainous neuralgia 879
Osteomyelitis classification 557 Paramedian lip pits 605 Periodic neutropenia 706
Osteomyelitis with proliferative Paramolar 110 Periodontal abscess 551
periostitis 563 Paraneoplastic pemphigus 737 Periodontal disease associated with
Osteopetrosis 594 Parasitic cyst 378 HIV 533
Osteophytes 646 Parasitosis 663 Periodontal ligament traction
Osteophytic lipping 646 Parathyroid gland 785 theory 121
Osteoporosis 591 Paratrigeminal syndrome 879 Periodontal pocket 401
Osteoporosis circumscripta 584 Parkes-Weber syndrome 886 Periodontosis 403
Osteoradionecrosis 665 Parosteal osteosarcoma 287, 289 Perioral dermatitis 778
Osteosarcoma 287 Parotid gland 410 Periorificial lentiginosis 755, 871
Osteosclerosis 600 Paroxysmal hemicranias 843 Periosteal chondroma 184
Osteosclerosis fragilis generalisata 594 Parrot beak 134 Periosteal osteoma 189
Otodental dysplasia 111, 870 Parry Romberg syndrome 130 Periosteal osteosarcoma 287, 289
Oxidative mechanism of Partsch’s operation 381 Periostitis ossificans 563
carcinogenesis 90 Parulis 550 Peripheral AOT 322
Oxygenation 69 PAS 42 Peripheral fibroma with calcification 182
Oxyphilic adenoma 437 Pastia’s line 472 Peripheral giant cell granuloma 214
Patau syndrome 865 Peripheral giant cell reparative
P Patent thyroglossal duct cyst 628 granuloma 214
p53 tumor 350 Paterson-Brown-Kelly syndrome 702 Peripheral giant cell tumor 214
Pachyderma oralis 753 Pathergy test 777 Peripheral neuroblastic
Pachyonychia congenita 747 Pathologic hyperplasia 82 tumors (PNTs) 292
Paget’s disease 584, 585 Pathological attrition 666 Peripheral ossifying fibroma 182
Palatal caries 153 Pathophysiology of infection 541 Peripheral osteochondroma 193
Palatal cyst of newborn 359 Pathothenic acid 807 Peripheral osteoma 189
Peripheral osteosarcoma 289 Plasma cell gingivitis 393, 772 Pott’s disease 465
Peripheral vascular disease 636 Plasma cell myeloma 727 Poxvirus 90
Periungual fibromas 758 Plasmacytoma 727 Pre-eruptive caries 161
1020 Perl’s Prussian blue reaction 45 Plasminogen deficiency 712 Precancerous condition 220
Perleche 612 Pleomorphic adenoma 430 Precancerous lesion 220
Pernicious anemia 636, 702 Pleomorphic lipomas 189 Predeciduous dentition 121
Persistent ulcer 658 Pleomorphic rhabdomyosarcoma 295 Pregnancy gingivitis 389
Petechiae 663 Plexiform ameloblastoma 309 Pregnancy tumor 398
Petrified man 838 Plexiform neuroma 207 Preheadache phenomenon 844
Peutz-Jeghers syndrome 755, 871 Plica fimbriata 620 Pre-leukoplakia 223
PHACE(S) syndrome 197 Plicated tongue 629 Premature exfoliation 125
Phantom bone 600 Plumbism 682 Preparation of tissue specimen 23
Phantom taste 637 Plummer-Vinson Pretrigeminal neuralgia 839
Pharyngeal gonorrhea 461 syndrome 636, 702, 880 Primary acquired erythrocytosis 704
Pharyngotonsillitis 507 Plump cells 179 Primary agranulocytosis 705
Phase contrast microscopy 13 Plump nuclei 197 Primary amyloidosis 802
Phase shifting ring 14 Plunging ranula 420 Primary anemia 702
Phleboliths 199 Pneumoparotid 666 Primary chronic osteomyelitis 561
Phlegmon 565 Pointer eyepiece 9 Primary cutaneous
Phoenix abscess 552 Polarized light microscopy 14 coccidioidomycosis 497
Phosphotungstic acid hematoxylin 41 Polarizers 14 Primary epithelial tumor of the jaw 336
Phycomycosis 495 Polychromatic stain 38 Primary healing 74
Phylloquinone 825 Polycyclic aromatic hydrocarbons 87 Primary herpes simplex infection 506
Physical carcinogen 88 Polycythemia rubra vera 704 Primary intra-alveolar epidermoid
Physical stain 38 Polymerase chain reaction 61, 537 carcinoma 336
Physical theory 38 Polymorphic reticulosis 728 Primary intraosseous carcinoma 336
Physiologic hyperplasia 82 Polymorphous low grade Primary lymphoma of bone 717
Physiological attrition 666 adenocarcinoma 447 Primary pulmonary blastomycosis 494
Picket fence 355 Polymyalgia rheumatica 844 Primary pulmonary
Picking the section 27 Polymyositis 833 coccidioidomycosis 497
Picric acid 29 Polyostotic fibrous dysplasia Primary reticular cell sarcoma 717
Pierre-Robin syndrome 597, 866 (Jaffe’s type) 577 Primary Sjögren’s syndrome 424, 427
Pigeon breast 823 Polypoid squamous cell carcinoma 277 Primary syphilis 456
Pigeon chest 792 Polystotic fibrous dysplasia 579 Primary thrombocytopenic purpura 708
Pigmentation of tongue 640 Poor circulation 69 Primary tuberculosis 466
Pigmented ameloblastoma 209 Poorly differentiated SCC 260 Primordial cyst 350, 355
Pigmented mole 174 Popcorn cells 715 Principal focus 11
Pindborg tumor 318 Popliteal pterygium syndrome 605 Principle of PAP smear 51
Pingueculae 806 Popular lichen planus 237 Principle of phase contrast
Pink disease 683 Porokeratosis 748 microscope 13
Pink tooth mummery 674 Porphyria 803 Principle of polarized light
Pit and fissure caries 156 Port-wine stain 198, 200 microscopy 14
Pit and fissure sealant 164 Post axial polydactyly 136 Pringle-Bourneville syndrome 757, 885
Pituitary ameloblastoma 314 Post-herpetic neuralgia 513 Procarcinogens 87
Pituitary dwarfism 787 Postmortem examination 851 Progeria 788, 884
Pituitary gland 784 Postmortem serology 854 Progressive bulbar palsy 836
Pityriasis rosea 743 Postoperative maxillary cyst 373 Progressive disseminated
Pizza burns 659 Postpermanent dentition 125 coccidioidomycosis 497
Placental extract 249 Postradiation osteosarcoma 287 Progressive disseminated
Plan achromat 7 Postrhagadic scarring 459 histoplasmosis 492
Plaque lichen planus 237, 241 Post-traumatic myositis ossificans 838 Progressive facial hemiatrophy 130
Plasma cell cheilitis 615 Potassium ferrocyanide 165 Progressive muscular atrophy 836
Index
Progressive myositis ossificans 838 Pushing margin 272 Refractory rickets 823
Progressive osteolysis 600 Pustular psoriasis 746 Regional enteritis 782
Progressive staining 39 PUVA therapy 242 Regional ileitis 782
Progressive systemic sclerosis 761 Pyogenic granuloma 399 Regional odontodysplasia 118 1021
Proliferation 73 Pyostomatitis vegetans 479 Regressive staining 39
Prolymphocytic leukemia 725 Pyridoxine 807, 816 Reiter’s syndrome 778, 883
Promoters of carcinogenesis 88 Relative macroglossia 624
Proptosis 133 Q Relative pocket 401
Protein deficiency 800, 801 Q banding 64 Remodeling 73
Proteolysis chelation theory 150 Quantitative PCR 62 Reparative dentin 77, 671
Proteolysis theory 149 Quaternary syphilis 456 Replication 90
Proteomics 63 Quincke’s edema 773 Requirement of biopsy tissue 48
Provitamins 811 Residual cyst 364
Prussian blue 45 R Resolution 6
Pseudocarcinoma 173 Resorption of teeth 672
Pseudo hairy leukoplakia 532 Rabbit syndrome 638 Respiratory disease 71
Pseudo hemophilia 712 Rachitic metaphysic 824 Retention cyst 374
Pseudo horn cyst 751 Radiation burns 658 Reticular lichen planus 237, 241
Pseudo lymphoma 425 Radiation carcinogenesis 88 Retinal anlage tumor 209
Pseudoacanthosis nigricans 744 Radiation osteomyelitis 564 Retinol 821
Pseudoallergic reactions 770 Radicular dens invaginatus 104 Retrocuspid papilla 388
Pseudoepitheliomatous hyperplasia 174 Radicular dentin dysplasia 118 Reverse smoker’s palate 233
Pseudohypoparathyroidism 793 Radioactive iodine 789 Reversible pulpitis 543
Pseudoleukemia 427 Ramon syndrome 878 Rhabdomyoma 212
Pseudolipoma 186 Rampant caries 161 Rhabdomyosarcoma 294
Pseudomembranous Rampant dental caries 663 Rheumatoid arthritis 646, 647
candidiasis 486, 530 Ramsden eyepiece 8 Rheumatoid sialadenitis 872
Pseudosarcomatous fasciitis 688 Ranula 419 Rhinoscleroma 474
Pseudotumor of hemophilia 711 Rapidly progressive periodontitis 402 Rhinosporidiosis 499
Pseudoxanthoma elasticum 751 Rathke’s pouch tumor 314 Riboflavin 807, 813
Psoriasiform hyperplasia 393 Raw beef appearance 684 Rice bodies 648
Psoriasis 746 Ray fungus appearance 469 Rickety rosary 823
Psychotherapy 242 Reynaud’s phenomenon 761 Riga-Fede disease 122
PTAH stain 43 Reactionary dentin 77, 671, 778 Riley-Day syndrome 873
Pterygoid-Levators synkinesis 880 Reactive lymphoid hyperplasia 693 Ripening of staining solution 40
PTN hamartoma tumor syndrome 760 Reactive osseous metaplasia 662 Risk factors of oral cancer 86
Puberty gingivitis 390 Reactive proliferation 167 Risky epithelium 228
Pulmonary sporotrichosis 499 Reactive subpontic exostosis 195 Ritual abrasion 668
Pulmonary tuberculosis 466 Reader’s syndrome 879 RNA oncogenic viruses 89
Pulp 542 Real image 10 Robin anomalad Pierre Robin
Pulp artifacts 546 Record management 949 sequence 597
Pulp calcifications 546 Recovery of dental structure 851 Rodent facies 697
Pulp degeneration 545 Recrudescent herpetic infection 507 Rodent ulcer 267, 268
Pulp hyperemia 543 Recurrent aphthous stomatitis 536 Role of forensic odontology 849
Pulp polyps 543 Recurrent aphthous ulcer 774 Romberg hemifacial atrophy 130
Pulp stone 546 Recurrent caries 160 Root caries 158
Pulpitis 542 Recurrent herpes labialis 507, 534 Root growth theory 120
Pumice appearance 233 Recurrent herpetic infection 507 Rose Bengal staining test 426
Pumping tooth syndrome 196 Recurrent intra oral herpes Rose gardener’s disease 498
Punch biopsy 49 infection 507 Rose Waller test 648
Purpura 663 Red raspberry tongue 472 Rosette pattern 324
Purpura hemorrhagic 708 Reductase test 163 Rothmund-Thomson syndrome 111
Round cell carcinoma 289 Secondary amyloidosis 802 Sickle cell anemia 695
Routes of metastasis 92 Secondary carcinoma 266 Sickle cell crisis 695
RT PCR 62 Secondary dentin 671 Sickle cell disease 695
1022 Rubber man 745 Secondary healing 74 Sickle cell trait 695
Rubella 514 Secondary herpetic infection 507 Sideropenic anemia 222
Rubeola 509 Secondary Sjögren’s Sideropenic dysphagia 702
Rubinstein Taybi syndrome 102 syndrome 424, 427 Silver nitrate 165
Rudimentary supernumerary teeth 110 Secondary syphilis 456, 457 Simmond’s disease 787
Rushton bodies 348, 363 Secondary tuberculosis 466 Simple aphthous 774
Russell’s bodies 554 Secondary vaccinia 772 Simple bone cyst 370
Rutherford syndrome 386, 878 Sectioning with microtome 27 Simple hemihyperplasia 129
Seed and soil hypothesis 92 Simple microscope 3
S Segmental odontomaxillary Simple odontogenic fibroma 332
Safranin O 45 dysplasia 131 Single gene disorders 862
Sailor’s lip 613 Self-healing carcinoma 173 Single refractive specimen 15
Saint Anthony’s fire 455 Self-inflicted bites 855 Sinonasal papilloma 170
Saliva 70, 150 Senear-Usher syndrome 735 Sinonasal undifferentiated carcinoma 270
Salivary duct cyst 419 Senile lentigo 756 Sinus mucocele 372
Salivary gland calculus 415 Senile osteoporosis 592 Sinusitis 479
Salivary gland stone 415 Sentinal tubercle 466 Sipple syndrome 205, 885
Salmon patch 198 Septal squamous exophytic Sistrunk operation 629
Sanguinaire 222 papilloma 170 Six P of lichen planus 237
SAPHO syndrome 562 Sequestra 556 Sjögren’s syndrome 424, 872
Sarcoidosis 769 Sequestrectomy 560 Skin sporotrichosis 498
Sarcoside 165 Seromucous salivary gland 410 Slip signs 187
Satellite cyst 351 Serous cell adenoma 444 Slow transforming tumor viruses 89
Saucerization 560 Serous salivary gland 410 Slowly progressive periodontitis 402
Saw microtome 31 Sessile cementicles 676 Small cell carcinoma of skin 279
Saw tooth appearance 240 Severe generalized muscular Smokeless tobacco forms 257
Scale of development of teeth 98 dystrophy 830 Smokeless tobacco keratosis 234
Scaly gray dermatitis 814 Severe neutropenia 705 Smoker’s melanosis 661
Scanning electron microscope 19 Severe periodontitis 402 Smoker’s palate 233
Scaphocephaly 133 Sex-linked inheritance 863 Smoker’s tongue 634
Scarlatina 472 Shagreen patch 757 Smoking 257
Scarlet fever 472 Sharp’s staging 224 Smooth erythroplakia 230
Schirmer test 426 Sheehan’s syndrome 787 Smooth surface caries 153
Schwann cells 203 Shell teeth appearance 117 Snail track ulcers 457
Schwannoma 204 Shield type I 117 Snow burns 659
Sclerodactyly 761 Shield type II 117 Snow capped teeth 116
Scleroderma 761 Shield type III 117 Snuff dipper lesion 234
Sclerosing hemangioma 179, 199 Shingles 510, 512 Snuff dipper’s cancer 271
Sclerosing technique 199 Shovel shaped incisor 109 Snuff pouch 234
Sclerotic dentin 77 Sialadenoma papilliferum 438, 439 Snyder test 162
Scrofula 466 Sialadenosis 420 Soap bubble appearance 196, 308, 333
Scrotal tongue 629 Sialo odontogenic cyst 358 Soft fibroma 179
Scrumpox 508 Sialochemistry 426 Soft mixed odontoma 326
Scurvy 819 Sialocyst 419 Soft odontoma 326
Sebaceous hyperplasia 756 Sialolithiasis 415 Soft papilloma 168
Seborrheic keratosis 750 Sialometry 426 Solar cheilosis 613
Seborrheic wart 168 Sialorrhea 414 Solar elastosis or senile elastosis 617
Second week wound 75 Sialosis 420 Solar lentigo 756
Secondary agranulocytosis 705 Sicca syndrome 424, 872 Solid leiomyoma 211
Index
Solid or primordial basal cell Stem cell leukemia 720 Superficial lipoma 189
carcinoma 268 Stenosis 417 Superficial lymphangioma 200
Solitary bone cyst 370 Stensen’s duct 411 Superficial ranula 420
Solitary labial lentigo 138 Steps of metastasis 92 Superficial vacuolated cells 169 1023
Solitary myositis 838 Steps of processing 25 Supernumerary roots 107
Sorrowful appearance 833 Steps of staining procedure 40 Supernumerary teeth 110
South American blastomycosis 501 Stereomicroscopy 11 Supplemental supernumerary teeth 110
Southern blot technique 62 Stevens Johnson syndrome 733, 870 Suppurating cyst 366
Specific plaque hypothesis 149 Stiff joint 648 Suppurative parotitis 422
Specimen accessioning 22 STNMP staging system 95 Supra vital stain 38
Specimen submission 48 Stomatitis areata migrans 631 Supra-alveolar pocket 401
Speckled leukoplakia 224 Stomatitis medicamentosa 770 Suprabony pocket 401
Speed bumps 663 Stomatitis nicotina 233 Supracrestal pocket 401
Sphenopalatine neuralgia 842, 879 Stomatitis venenata 771 Supravital staining 39
Spherical aberration 7 Stomatodynia 845 Surgical ciliated cyst 373
Sphingomyelin lipidosis 807 Stomatopyrisis 245 Susuk 679
Spider finger 598 Stomatopyrosis 845 Sutton’s disease 774
Spider web appearance 212 Storiform pattern 180, 207 Swab test 163
Spike formation of cementum 676 Strabismus 133 Swan neck 831
Spindle cell carcinoma 277 Stratum intermedium 301 Sweet’s syndrome 881
Spindle cell lipomas 189 Straw mat appearance 180 Swift’s disease 683
Spindle cell nevus 175, 176 Strawberry gingivitis 768 Swiss cheese pattern 279, 446
Spindle cells 277 Strawberry hemangioma 197 Sympathetic ophthalmoplegia 880
Spindle torus 194 Streptococcal pharyngitis 476 Synodontia 100
Spit tobacco keratosis 234 Streptococcal tonsillitis 476 Synovial chondromatosis 652
Spitz nevus 175, 177 Streptococcus mutans level in saliva 163 Synovial sarcoma 282
Splints 678 Stress lesion 670 Syphilis 222
Split papule 457 Strictures 417 Syphilis 455, 636
Spontaneous dyskinesia 638 Sturge-Weber angiomatosis 199 Syphilitic rhagades 459
Spontaneous sequestration 662 Sturge-Weber syndrome 199, 882 Systemic blastomycosis 494
Sporotrichosis 498 Study of hard tissue 30 Systemic lupus erythematosus 250, 251
Sprue 811 Subacute cutaneous erythematosus 250 Systemic sclerosis 761
Squamous acanthoma 174 Subacute necrotizing sialadenitis 424
Squamous cell carcinoma 639 Subchondral cyst 646 T
Squamous eddies 751 Subclinical fibrous dysplasia 577 T lymphocytes 526
Squamous odontogenic tumor 317 Subcrestal pocket 401 Tabes dorsalis 458
Squamous papilloma 168 Subcutaneous nodules 647 Talisman 679
Stable plaque psoriasis 746 Subleukemia 720 Talon’s cusp 102
Stafne bone cyst 137 Sublingual gland 410 Tardive dyskinesia 638
Stafne defect 137 Sublingual keratosis 223 Target lesion 732
Stafne’s cyst 412 Subluxation 650 Taste buds 620
Stage of chemical carcinogen 88 Submandibular gland 410 Taurodontism 106
Staghorn appearance 202 Submerged teeth 123 Tay Sachs disease 804, 807
Staging of leukoplakia 225 Submucosal hemorrhage 663 T-cell lymphoma 728
Staining of chemical production 39 Submucous palatal cleft 609 Teeth specimen 48
Staining of cut section 32 Sucrose chelation theory 150 Telangiectasia 197, 761
Staining of selective solubility 39 Sudan black B 44 Temporal arteritis 844
Staining procedure 40 Sulphur granule 469 Temporomandibular joint
Starch-iodine test 835 Sun ray appearance 288 dysfunction 652
Starry-sky appearance 719 Sunburst appearance 196 Tennis racket appearance 333
Static bone cyst 137 Sunshine vitamin 822 Teratoma 84
Stellate cells 182 Superficial hemangioma 196 Teratoma of tongue 84
Terminal duct carcinoma 447 Tongue nerve supply 621 Trisomy 18 syndrome 864
Tertiary dentin 671 Tongue tie 625 Trisomy 21 syndrome 598, 864
Tertiary syphilis 456 Tongue torches 777 Trisomy syndrome 864
1024 Test tube appearance 747 Tongue venous damage 621 Tropic ulcer 657
Tetany 793 Tonsillar hyperplasia 469 Trousseau’s sign 793
Thalassemia 696 Tonsillar concretion 476 True cementoma 334
Thalassemia intermedia 696 Tonsillolithiasis 476 True micrognathia 128
Thalassemia major 696 Tooth brush injury 668 True pocket 401
Thalassemia minor 696 Tooth germ 302 Tubercular ulcer 466
Thalassemia trait 696 Tooth march 854 Tuberculin skin test 467
Theories of carcinogenesis 91 Tooth pick injury 669 Tuberculoid leprosy 462
Theories of cariogenesis 145 Tophi 651 Tuberculosis 465, 637
Theories of cyst enlargement 343 Torulosis 496 Tuberous sclerosis 757, 877, 885
Theories of salivary gland tumor Torus mandibularis 195 Tularemia 474
histogenesis 429 Torus palatinus 194 Tumor droplets 324
Theories of staining 38 Toxic epidermal necrolysis 733 Tumor metastasis 546
Theories of tooth eruption 120 Toxoplasmosis 502 Tumorigenesis 304, 305
Theques 175 Trabecular carcinoma of skin 279 Turner’s hypoplasia 113
Thermal burns 658 Trans illumination test 187 Turner’s tooth 113
Thiamine 812 Transformation 90 Twining 100
Third week wound 75 Transient lingual papillitis 777 Types of carcinogenesis 87
Thistle tube appearance 118 Transient osteopetrosis 594 Types of objective 6
Thomson’s disease 832 Transitional cell carcinoma 273 Tzanck smear 736
Thrombocythemia 710 Translocation 65
Thrombocytosis 710 Transmission electron microscope 18 U
Thrombotic thrombocytopenic Transparent dentin 671 Ulcerated leukoplakia 224
purpura 709 Transposition 124 Ulcerative lichen planus 239
Thrush 486, 530 Trapezoid lip 137 Uncomplicated fracture 680
Thymic cyst 378 Traumatic bone cyst 370 Ungual fibroma 758
Thymic replacement therapy 537 Traumatic ciliated cyst 373 Unicameral cyst 370
Thyroglossal duct cyst 377, 628 Traumatic fibroma 178 Unicystic ameloblastoma 315, 316
Thyroid crisis 788 Traumatic keratosis 689 Unifocal eosinophilic granuloma 805
Thyroid gland 785 Traumatic lesion due to sexual habit 679 Urbach-Wiethe syndrome 752
Thyroid storm 788 Traumatic neuroma 203 Urea 165
Thyrotoxic osteoporosis 592 Traumatic sequestration 662 Uremic stomatitis 688
Thyrotoxicosis 788, 789 Traumatic ulcer 657 Uveitis 428
Tic douloureux 839 Treacher Collins syndrome 134, 866 Uveoparotid fever 428
Tingible bodies 694 Trefoil tongue 628 Uveoparotid syndrome 873
TNM staging 93 Triangular frontal defect 134
TNM staging of melanoma 276 Trichinosis 503 V
Tobacco 221 Tricho-dento-osseous Vaccinia autonoculata 772
Tobacco pouch keratosis 234 syndrome 107, 870, 874 Vacuolar degeneration 226
Tobacco pouch mouth 762 Tricho-onychodental syndrome 870 Vaginogingival syndrome 237
Tocopherol 824 Trichrome stain 42 Vagoglossopharyngeal neuralgia 840
Toluidine stain 45 Trifacial neuralgia 839 Valley fever 497
Tombstone appearance 355 Trigeminal neuralgia 839 Van der Woude’s syndrome 605, 875
Tongue anatomy 620 Trigeminal neuropathic pain 841 van Gieson’s method 43
Tongue arterial supply 621 Trigger zones 839, 840 Vanishing bone disease 600
Tongue disorders classification 623 Trigonocephaly 133 Vaquez’s disease 704
Tongue embryology 619 Trisomy 65 Varicella 510
Tongue lymphatic drainage 622 Trisomy 13 syndrome 864, 865 Varicella zoster infection 510
Index
Varicose aneurysm 199 Vitamin B6 827 Wet beriberi 813

Varicosity 627 Vitamin B7 807 Wharton’s duct 411
Varix 627 Vitamin B8 816 White folded gingivostomatitis 753
Vascular hemophilia 712 Vitamin B9 817 White sponge nevus 753 1025
Vascular leiomyoma 211 Vitamin C 819, 826 White strawberry tongue 472
Vascular malformation 196, 198 Vitamin D 826 WHO dysplasia system 228
Vascular nevi 196 Vitamin D deficient rickets 822 WHO type odontogenic fibroma 332
Vascular pressure theory 120 Vitamin D resistance rickets 823 Whorl pattern 180
Vascular purpura 712 Vitamin E 824, 826 Wickham’s striae 237, 240
Venereal wart 517 Vitamin H 807 Winchester syndrome 876
Venous malformation 198 Vitamin K 165, 825, 826 Wire loop 251
Vermilion zone 605 Vitamin therapy 229 Wiskott-Aldrich syndrome 709
Vermilionectomy 611 volkman’s cheilitis 610 Witkop-Von-Sallman syndrome 754
Verocay body 205 von Hippel-Lindau syndrome 886 Woody tongue 567
Verruca acuminate 517 von Recklinghausen’s disease of Wound fibroblast 73
Verruca vulgaris 518 skin 206 Wound temperature 69
Verruciform xanthoma 171 von Recklinghausen’s Woven bone 191
Verrucous carcinoma 271 neurofibromatosis 884
Verrucous leukoplakia 224 von Willebrand’s disease 712 X
Vertical root fracture 680 Xanthoma cells 172
Vesicular pharyngitis 515 W Xanthomatosis 804
Vestigial cyst 366 Waardenburg’s syndrome 870 Xeroderma pigmentosum 757
Vichow’s physeal theory 193 Waddling gait 830 Xerostomia 414, 665
Vincent’s infection 391 Wakeful EMG feedback 678 X-linked inheritance 127
Virtual image 10 Waldeyer’s ring 278 X-linked recessive 128
Virus theory 91 Wandering rash 631
Visceral leishmaniasis 502 Warthin’s tumor 435 Z
Vital stain 38 Warty dyskeratoma 750 Zellballen 206
Vital staining 39 Waterhouse-Friderichsen Zellweger syndrome 876
Vital theory 145 syndrome 884 Zimmerman Laband syndrome 386, 878
Vitamin A 821, 826 Water soluble vitamins 812 Zinc chloride 165
Vitamin A palmitate 229 Wax infiltration 26 Zinc deficiency 809
Vitamin B1 812, 826 Weber-Cockayne syndrome 740 Zinsser-Engman-Cole
Vitamin B12 827, Wegner’s granulomatosis 767, 768 syndrome 249, 875
Vitamin B2 813, 827 Well differentiated SCC 259 Zona 510, 512
Vitamin B3 814 Werlhof’s disease 708 Zoster-Infection 637
Vitamin B5 816 Wernicke’s encephalopathy 813 Zoster sine herpete 513
Vitamin B5 827 Western blot method 537 Zycomycosis 495
Vitamin B6 816 Western blot technique 63 Zymogen granules 421

Textbook of Oral Pathology-Anil G. Ghom, S. Mhaske (2013)

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S E C O N D l D I T I O N

Anil Govindrao Ghom Shubhangi Mhaske ( Jedhe )

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

Jaypee Brothers Medical Publishers (P) Ltd.

© 2013, Jaypee Brothers Medical Publishers

Inquiries for bulk sales may be solicited at: jaypee@jaypeebrothers.com

Textbook of Oral Pathology

First Edition: 2009

Second Edition: 2013

Amol Gadbail Monal Yuwanati

Anil Govindrao Ghom Pradnya Lele

Satish Chhugani Shubhangi Mhaske (Jedhe)

Smruti Nanda BDS

Seema Vaidya Vivek Thombre

Manisha Sanjay Tijare MDS (Oral Pathology)

Anil Govindrao Ghom

Nothing can be changed by changing the face

Anil Govindrao Ghom

Anil Govindrao Ghom

Shubhangi Mhaske (Jedhe)

2. TISSUE PROCESSING METHODS 22

3. HISTOLOGICAL STAINING METHODS 36

5. ADVANCED DIAGNOSTIC TECHNIQUES 57

Fractures 75; Healing of Osseointegrated Implants 76 ; Healing of Pulp 76 ;

7. HYPERPLASIA , HAMARTOMA AND NEOPLASM 82

9. CRANIOFACIAL ANOMALIES 111

10. DENTAL CARIES 144

11. BENIGN TUMORS 167

12. PREMALIGNANT LESIONS AND CONDITIONS 219

13. MALIGNANT TUMORS 255

14. ODONTOGENIC TUMORS 299

15. CYST OF OROFACIAL REGION 342

16. PERIODONTAL PATHOLOGY 386

17. SALIVARY GLAND PATHOLOGY 409

18. BACTERIAL INFECTION 453

19. FUNGAL OR MYOCOTIC INFECTION 484

Cryptococcosis 496 ; Coccidioidomycosis 497; Geotrichosis 498 ; Sporotrichosis 498 ;

20. VIRAL INFECTION 505

21. ACQUIRED IMMUNODEFICIENCY SYNDROME 524

22. ODONTOGENIC INFECTION AND PULP PATHOLOGY 540

23. BONE DISEASE MANIFESTED IN JAW 576

24. DISEASES OF LIP 604

25. TONGUE DISORDERS 619

26. TEMPOROMANDIBULAR JOINT PATHOLOGY 643

27. CHEMICAL AND PHYSICAL INJURIES 655

28. BLOOD PATHOLOGY 693

29. SKIN DISORDERS 731

30. ALLERGIC AND IMMUNOLOGIC DISEASES OF ORAL CAVITY 766

31. ENDOCRINE DISORDERS 784

32. NUTRITION AND ORAL CAVITY 800

33. NEUROMUSCULAR DISORDERS AND OROFACIAL PAIN 830

34. FORENSIC ODONTOLOGY 849

35. SYNDROMES OF THE OROFACIAL REGION 860

ANSWERS KEY OF MCQS 1003

Shubhangi Mhaske (Jedhe)

3 Definition • Mechanical tube

Table 1.1 Contributors in history of microscope

∙ Negative: It is concave shaped and diverge rays and

Figure 1.5 Types of lenses used in optical components of

focused in the object plane and the aperture diaphragm is

similar to the above condenser. But when its position is

Table 1.2 Color codes used for objectives

Numerical aperture depends primarily on the extreme

NA = Refractive index × sine angular aperture

Alum hematoxylin: Alum hematoxylin are most com

The mechanism by which twocolor staining is Congo Red