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Essentials in Dermatology

(with Multiple Choice Questions)

Essentials in Dermatology
(with Multiple Choice Questions)

Second Edition

Devinder M Thappa

MD, DHA, MNAMS, FIMSA

Professor and Head


Department of Dermatology and STD
Jawaharlal Institute of Postgraduate Medical Education and Research
(JIPMER)
Pondicherry, India

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Essentials in Dermatology (with Multiple Choice Questions)


2009, Jaypee Brothers Medical Publishers
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legal matters are to be settled under Delhi jurisdiction only.

First Edition: 2003


Second Edition: 2009
ISBN 978-81-8448-558-5

Typeset at JPBMP typesetting unit


Printed at Ajanta

Preface to the Second Edition


The second edition of Essentials in Dermatology (with Multiple Choice Questions) is being published
6 years after the appearance of the first edition. The encouraging response to the first edition prompted
me to revise the book, keeping in view the comments received, and changing trends in the field of
dermatology.
This new edition incorporates differential diagnosis for each entity or group of entities to further
understand the subject critically. Three new chaptersSkin in Systemic Diseases, Skin Changes of
Pregnancy and Old Age, and Antiretroviral Therapy (ART) have been added. The existing chapters
have been updated and treatment guidelines revised. Newer entities have been included under
various chapters, but not at the cost of brevity and conciseness. For better understanding of the text,
better photographs and clinical illustrations have been incorporated.
The section on multiple choice questions has been considerably expanded, and this section has
been divided into twofor PG entrance examinations and for postgraduates in dermatology.
Additional mnemonics have been included in the useful medical mnemonics section.
The objective of this edition remains the sameto serve as an aid for beginners in dermatology
and those aspiring for PG entrance examinations.
The making of the revised edition of this book involved a number of people besides myself.
Many of the chapters were revised with inputs from my senior residents Dr Rashmi Kumari,
Dr Amiya Kumar Nath and Dr Abarna Devi and junior residents Dr Nidhi Singh, Dr Abhijit
Chougule, Dr Kishan Kumar Agarwal, Dr Balaji Adityan, Dr Sowmya Kaimal and Dr Sakthi Kandan.
The photographs utilized in the book have been possible due to the Medical Illustration Department
of the hospital, and the digital cameras of my postgraduates, making the new edition a colorful
experience. My laboratory technician Mr Samsudeen deserves a mention for his skill in the staining
and preparation of laboratory material for photomicrography. The final making of this edition has
involved the support and cooperation of all my esteemed colleagues, patients and the forgotten
names of residents (who worked for the first edition of the book).
As always, this edition is open to constructive criticism and suggestions for its further
improvement.
Devinder M Thappa

Preface to the First Edition


Dermatology, the science of the skin, was one of the many specialties, which evolved from general
internal medicine during the course of the nineteenth century. In India, recognition of dermatology
as a specialty distinct from internal medicine is recent; it has still not grown to its full stature in
practice and teaching. In spite of having some share in the curriculum, dermatology remains a
neglected subject because of its non-inclusion in the qualifying examination at MBBS level. There
has been an explosion of knowledgeeasily documented by the size of standard dermatology
textbooks, whose length has increased from an average of 1000 pages to the most recent editions of
Fitzpatrick and of Rook, which are more than 3000 and 3600 pages, respectively. Expansion has
been greater on the surgical and cosmetic side of the specialty, which barely existed 50 years ago.
Such vast knowledge is difficult to grasp in 3 years course of MD dermatology, venereology and
leprology, sometimes may be at the cost of another. So there was need for a short textbook for
postgraduates who have just joined the specialty to have the glimpse of the subject and understand
the basic dermatology before venturing for detailed standard textbooks. There is lack of simple but
up to date book for undergraduates who are preparing for Postgraduate Entrance Examination.
Though market is flooded with a number of books, many of them are not even framed by dermatology
specialty individuals and lack correct and appropriate information. This prompted me to write this
book to fulfill the needs of students aspiring for entering in postgraduate courses in reputed institutes
of India. The material in this book is based on the standard textbooks and latest information from
specialty journals. Introduction to MCQs is a unique section in this book to guide the students. The
multiple choice questions are taken from a number of sources to sensitize the student to know
certain subject areas in this specialty thoroughly and accordingly the book section gives relevant
points highlighted for quick revision of facts. The suggestions and healthy critical remarks will be
very much appreciated to improve this book.
Devinder M Thappa

Acknowledgements
I would like to thank those who helped me to update chapters
1. Dr Balaji Adityan for updating
Principles of Diagnosis in Dermatology
Bacterial Infections
Viral Infections
Fungal Infections
Skin Changes in Pregnancy and Old Age
2. Dr Sakthi Kandan for
Infestations
Disorders of Hair and Nails
Metabolic and Nutritional Disorders
Skin in Systemic Diseases
3. Dr Sowmya Kaimal for
Pediatric Dermatology
Human Immunodeficiency Virus Infection (HIV) and Acquired Immunodeficiency Syndrome
(AIDS)
4. Dr Amiya Kumar Nath for
Eczema
Connective Tissue Disorders
Genetics and Genodermatoses
5. Dr Abhijit Chougule for
Differential Diagnosis for Leprosy
Treatment of Leprosy
6. Dr Kishan Kumar Agarwal for
Urticaria, Angioedema and Pruritus
Disorders of Sebaceous, Eccrine and Apocrine Glands
7. Dr Nidhi Singh for
Cutaneous Tuberculosis and Atypical Mycobacterial Infections
Vesiculobullous Disorders
Pigmentary Disorders
Following residents helped in framing MCQs for postgraduates
1. Dermatology Basics
Dr Abhijit Chougule
Dr Kishan Kumar Agarwal
2. Clinical Dermatology Part -I
Dr Rashmi Kumari

Essentials in Dermatology
Dr Balaji Adityan
Dr Ajay Kumar Singh
Dr Anuradha Priyadarshini
Dr Tukaram Sori
3. Clinical Dermatology Part -II
Dr Malathi
Dr Sathyamoorthy
4. Sexually Transmitted Diseases
Dr Sowmya Kaimal
Dr Rajalakshmi
5. Leprosy
Dr Abarna Devi
Dr Sakthi Kandan

Reviews
Most of the dermatology textbooks are too much voluminous for undergraduate students already
overburdened with other heavy weight subjects. Not only undergraduates, beginners at the
postgraduate level also face problem to acquire basic conception from such large books. So there is
always a need for a concise book which can provide clear basic conception and up-to-date knowledge
to the students.will be of immense help to the postgraduate entrance examinees.should be
collected in all undergraduate medical college libraries for the benefit of the students
Indian J Dermatol 2003; 48(4): 248.
The stated aim of the book is to have a short textbook for new entrants to postgraduate
studies in dermatology which could glimpse of the subject and understand basic dermatology before
venturing for detailed standard textbooks. The second aim stated is to fulfill the needs of students
aspiring for entering in postgraduate courses in reputed institutes.well written and fulfill the
stated aimsAn approach to attempting MCQs appears to be a very useful chapter.strongly
recommend this book to the new entrants in specialty training and those preparing for admission to
postgraduate courses
Indian J Dermatol Venereol Leprol 2004; 70(6): 393.

Contents

SECTION 1: DERMATOLOGY
1. Ten Most Influential People in Medicine and Dermatology .................................................... 3
2. History of Dermatology in the World ............................................................................................ 5
3. Microanatomy of the Skin ................................................................................................................ 8
4. Physiology, Biochemistry and Immunology of the Skin ......................................................... 13
5. Principles of Diagnosis in Dermatology ..................................................................................... 16
6. Bacterial Infections .......................................................................................................................... 31
7. Viral Infections ................................................................................................................................. 43
8. Fungal Infections ............................................................................................................................. 57
9. Infestations ........................................................................................................................................ 72
10. Papulosquamous Disorders ........................................................................................................... 82
11. Eczema ................................................................................................................................................ 99
12. Vesiculobullous Disorders ........................................................................................................... 114
13. Cutaneous Tuberculosis and Atypical Mycobacterial Infections ........................................ 127
14. Connective Tissue Disorders (Collagen Vascular Disorders) ............................................... 134
15. Pigmentary Disorders ................................................................................................................... 148
16. Keratinization Disorders .............................................................................................................. 156
17. Urticaria, Angioedema and Pruritus .......................................................................................... 166
18. Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome
and Toxic Epidermal Necrolysis ................................................................................................. 172
19. Disorders of Sebaceous, Eccrine and Apocrine Glands ......................................................... 180
20. Disorders of Hair and Nails ......................................................................................................... 189
21. Metabolic and Nutritional Disorders ......................................................................................... 198
22. Genetics and Genodermatoses .................................................................................................... 210
23. Skin in Systemic Diseases ............................................................................................................ 222

xiv

Essentials in Dermatology
24. Skin Changes of Pregnancy and Old Age ................................................................................. 237
25. Pediatric Dermatology .................................................................................................................. 240
26. Benign, Pre-malignant and Malignant Tumors of the Skin .................................................. 246
27. Topical Formulary and Key Systemic Drugs ............................................................................ 257
28. Dermatosurgical Procedures ........................................................................................................ 272

SECTION 2: SEXUALLY TRANSMITTED DISEASES AND HIV INFECTION


29. Historical Milestones in Sexually Transmitted Diseases ...................................................... 279
30. History Taking and Examination in Sexually Transmitted Diseases (STDs) .................... 282
31. Sexually Transmitted Diseases ................................................................................................... 288
32. Human Immunodeficiency Virus Infection (HIV) and Acquired
Immunodeficiency Syndrome (AIDS) ....................................................................................... 309
33. Antiretroviral Therapy (ART) ..................................................................................................... 318

SECTION 3: LEPROSY
34. Historical Milestones in Leprosy ................................................................................................ 327
35. History Taking and Examination in Leprosy ........................................................................... 330
36. Clinical Leprosy ............................................................................................................................. 333
Multiple Choice Questions .................................................................................................................... 351
Some Useful Medical Mnemonics ....................................................................................................... 441
Terminology ............................................................................................................................................. 447
Answers ..................................................................................................................................................... 465
Index ........................................................................................................................................................... 469

Ten Most Influential People in Medicine and Dermatology

Ten Most Influential People in


Medicine and Dermatology

THE MILLENNIUM AND MEDICINE:


THE TEN MOST INFLUENTIAL
PERSONS
1. Louis Pasteur (1822-1895): Proposed the
germ theory. He first associated a specific
micro organism (bacillus) with a specific disease
(anthrax). He developed the method of
pasteurizationa heating process that kills
bacteria in milk, wine and other liquids. He was
also a pioneer in stereochemistry.
2. Robert Koch (1843-1910): The first to isolate
the anthrax bacillus (1876). In 1883, he published
a method of preventive inoculation against this
disease. In 1882, he announced the discovery of
tubercle bacillus and in 1883; he discovered the
cause of cholera. He was awarded the nobel prize
in physiology or medicine in 1905.
3. Rudolf Virchow (1821-1902): Founded
Cellular pathology. His concept that the basis
of disease is the cell, the essential functional and
structural unit of the body, was of monumental
importance as a basis for understanding the
cause, the process and the results of the disease.
4. Gregor Mendel (1822-1844): Formulated the
laws of heredity. Mendels work laid the
mathematical foundation of the science of
genetics.

5. Francis Crick (1916-) and James Watson


(1928): Accredited with determining the
molecular structure of DNA, the chemical
substrate of heredity, which is regarded as the
most important discovery of the 20th century in
medicine and science. They were awarded the
nobel prize in 1962 sharing it with Maurice
Wilkins (1916). Currently, Crick is associated
with the Salk Institute for biological studies in
San Diego while Watson is the director of the
Cold Spring Harbor lab in southeastern New
York.
6. Marie Curie (1867-1934): Discovered
radioactivity and was given the nobel prize in
1903. The discovery formed the basis of radiation
therapy. In 1911, she was again conferred the
Nobel Prize in chemistry for her discovery of
radium and polonium.
7. Edward Jenner (1749-1823): Introduced the
inoculation for smallpox at the end of the 18th
century, which is considered one of the greatest
triumphs in the history of medicine.
8. Karl Landsteiner (1868-1943): Called the
father of blood grouping - a concept without
which blood transfusion would not be possible.
In 1901, he showed that there are at least three
major types of blood. Landsteiner was awarded
the noble prize for his work in 1930.

Essentials in Dermatology
9. Wilhelm Rontgen (1845-1923): Discoverer of
X-rays in 1895 and nobel prize winner in Physics
in 1901. The value of X-rays in the diagnosis and
treatment was recognised and accepted almost
from the outset of their discovery.
10. Sigmund Freud (1856-1939): Considered the
founder of psychoanalysis, he believed that a
complex of repressed and forgotten expressions
underlies all abnormal mental states and that
infantile mental processes are important in later
development.

TEN MOST INFLUENTIAL PERSONS IN


DERMATOLOGY, VENEREOLOGY AND
LEPROSY: INDIA
1. Dr. JS Pasricha: Pioneer of pulse therapy in
pemphigus, contact dermatitis in India.
2. Dr. LK Bhutani: Clinical dermatology,
Bhutanis Colour Atlas of Dermatology,
photobiology.
3. Dr. Dharmendra: Father of leprosy in India.
4. Dr. RV Rajam and Dr. PN Rangaiah:
Monograph on donovanosis.
5. Dr. VN Sehgal: For his literary contribution
in dermatology, venereology and leprosy.
6. Dr. Patrick Yesudian: Clinician par excellence,
known for Patrick Yesudian sign for palmar
freckling in neurofibromatosis type 1.
7. Dr. KC Kandhari: Established department of
dermatology at AIIMS.
8. Dr. Gurmohan Singh: contribution to Indian
and community dermatology.
9. Dr. Surinder Kaur: Established department
of dermatology at PGIMER, Chandigarh.
10. Dr. Sardarilal: First editor of Indian Journal
of sexually transmitted diseases, and for
contributions in donovanosis.

TEN MOST INFLUENTIAL PERSONS IN


DERMATOLOGY, VENEREOLOGY AND
LEPROSY: WORLD
1. Dr. Ferdinand Ritter von Hebra Founder of
the new Vienna school of dermatology, which
set the basis for modern dermatology.
2. Dr. Robert Willan Founder of dermatology
as a medical specialty.
3. Dr. Josef Jadassohn Best remembered for his
handbook of skin and venereal disease
(41 volumes), pioneer in allergology, introduced
patch testing.
4. Dr. Johnathan Hunter Natural history of
syphilis, role of inflammation in healing.
5. Dr. Paul Ehrlich Developed salvarsan (magic
bullet) as a treatment for syphilis, was the first
to stain tubercle bacilli.
6. Dr. Thomas Bernard Fitzpatrick Proved that
melanin was produced in melanosomes, first
editor of Dermatology in General Medicine
(1965).
7. Dr. Arthur Rook (1918-1991), Dr. Darrell
Sheldon Wilkinson and zoologist John Ebling
(1918-1992): Produced their major work,
Textbook of Dermatology (alias The Rook Book)
in 1968.
8. Dr. Paul Gerson Unna, Dr. HKB Pinkus, Dr.
A Bernard Ackerman, and Walter F. Lever:
Contributions to dermatopathology.
9. Heinrich Koebner Koebner phenomenon,
founder of the dermatology clinic at the
University of Breslau.
10. GHA Hansen Identified M. leprae as the
causative agent of leprosy in 1873.

History of Dermatology in the World

History of Dermatology
in the World

HISTORY OF DERMATOLOGY IN THE


WORLD
In Greek and Roman era, Hippocrates
recognized and described many diseases.
Some of the medical facts he observed are as
true today as they were over 2000 years ago.
He rescued medicine from magic and
superstition, therefore rightly so called the
Father of Medicine.
Dermatology, the science of the skin, was
one of the many specialties, which evolved
from general internal medicine during the
course of the nineteenth century. Most
diseases of skin, as external diseases, had
for many centuries fallen within the province
of the surgeon or of the quack.
Until the eighteenth century was welladvanced, physicians with few exceptions
were little concerned with the skin, apart
from the exanthematic eruptions of acute
fevers. However, during the last decades of
that century, many of the great physicians
recorded their observations on diseases of the
skin. The solid contributions of some, such
as Heberden and Cullen, which have
received too little attention from the
historians of dermatology, laid the
foundations on which the pioneer specialist

dermatologists of the following century were


able to build.
Despite developments in 19th centuryvaccination against small pox, recognition
of cellular pathology, Louis Pasteurs germ
theory of infection, development of
anaesthesia and microscope, the treatment
of most skin diseases was at best
symptomatic and at worst dangerous.
Amongst the first to specialize in
dermatology was Ferdinand Hebra (18161880) in Vienna, who led the torch of
dermatology, others followed him. The last
half of 19th century saw dermatology and
venereology emerge as a specialty in its own
right.
The 20th century brought a wealth of new
scientific knowledge that can be used to help
the sick. Perhaps, the most important single
discovery was that of Sir Alexander
Fleming, the British bacteriologist who
found the first antibiotic, penicillin.
During 20th century, certain turning points
occurred in general sociocultural factors
(welfarepublic health, vaccines, hygiene,
clean water, sewerage, etc; war; communicationsbooks, photography, radio, films,
television, computers; transport), general
scientific developments (genetics- structure

Essentials in Dermatology
of DNA; inflammation-histamine, prostaglandins, cytokines, adhesion molecules;
immunology-cell mediated and humoral
immunity; tissue culture; pathogenic agentsspirochetes, viruses, prion; therapies- x-rays,
antibacterial, immunosuppressive; controlled clinical trials), and strictly
dermatological areas(books- Jadassohn,
Pillsbury, Rothman, Rook; biologykeratinocyte, melanocyte, Langerhans cell,
basement membrane; diseases- epidermolysis bullosa, pemphigus, toxic epidermal
necrolysis; people- from Unna to Katz;
therapies- local steroids, griseofulvin,
phototherapy, retinoids, Mohs surgery,
laser, cryotherapy).

EVOLUTION OF DERMATOLOGY
In India, recognition of dermatology as a
specialty distinct from internal medicine is
recent; it has still not grown to its full stature
in practice and teaching.
Therapeutics of dermatoses have been
known and practiced by our ancient
physicians for centuries. Charaka Samhita
contains one chapter on the subject.
Medical charlatans selling panaceas for
cutaneous ailments and faith healers were
commonly seen all over the country. With the
advent of scientific dermatology, their
number and importance has dwindled.
In the latter part of the 19th century, the
health authorities in then British India
became aware of the need to have data on
prevalence of dermatoses and venereal
diseases.
The first chair of dermatology was
established at Grant Medical College,
Jamshedji Jeejebhoy Hospital (JJ Hospital),
Bombay in 1895.
The second department, at the School of
Tropical Medicine in Calcutta, was started in
1923, after a gap of nearly 28 years, under

the patronage of Dr Ganpati Panja and


Colonel Acton.
During the period from 1956 to 1974, the
status of the specialty was further elevated
and steps were taken by state governments
to set up departments of dermatology and
venereology in medical institutions.
Dr UB Narayan Rao, a pioneer in the
specialty, gets the credit for the creation of
an association of dermatologists and
venereologists in Bombay (July 1, 1947), and
for Indian Journal of Venereology started in
1935, renamed as Indian Journal of Venereal
Diseases and Dermatology in 1940, and later
renamed as Indian Journal of Dermatology
and Venereology in 1955, the first issue of
which was edited by him.
In 1962, it was decided to affiliate the
association of dermatologists and
venereologists with Association of Physicians
of India (API).
This continued until 1974, after which this
affiliation was severed and association
became an independent body.
On January 28, 1973, the present association
the Indian Association of Dermatologists,
Venereologists and Leprologists (IADVL)
came into existence.
Since 1976 the bimonthly journal is being
published under the title Indian journal of
dermatology, venereology and leprology.

EVOLUTION OF VENEREOLOGY
Syphilis was first introduced into North India
nearly 500 years ago.
National STD Control Programme was
started in 1946. This programme continued
to operate till 1991 and with the arrival of HIV
infection in the country, the programme was

History of Dermatology in the World

brought under the purview of National


AIDS Control Organization (NACO) in the
year 1992.
The monograph by Rajam and Rangiah on
donovanosis (granuloma inguinale,
granuloma venereum) is testimony to the
teaching and research standards set by these
two giants at institute of venereology,
Chennai.
Dr. CN Sowmini founded the Indian
Association for the study of sexually
transmitted diseases (IASSTD) in the year
1975.
In the year 1980, this specialty, under the
banner of IASSTD, started its own exclusive
journal, the Indian Journal of Sexually
Transmitted Diseases.
The late Dr. Sardarilal was its founder editor
and guiding force, who had already made a
mark in the field of research, especially in
donovanosis.
Somehow venereology did not prosper as
much, even though it led in front of
dermatology and leprology in teaching and
in the starting of its own journal in early part
of 19th century.
Unlike in the West, venereology in India has
been combined with dermatology in most of
the universities.

EVOLUTION OF LEPROLOGY
There is a great deal of speculation about the
early history of leprosy. The earliest records,
which give accurate descriptions of the
disease, come from India and may have been
written as early as 600 BC.

In Sushrata Samhita (600 BC), one finds a


reasonably good account of the clinical
features and treatment of the disease.
Sushrata described the different forms of
leprosy, and these forms fit in fairly well with
the forms of the disease as recognized at the
present time.
Sushrata described the treatment of the
disease with Chaulmoogra oil (hydnocarpus
oil), which till 1940s was the mainstay in the
treatment of the disease.
The first known asylum for leprosy patients
was established in Calcutta early in the 19th
century, followed by another in Varanasi.
Leprosy in India, a journal specific for
leprosy, was started by Dr. Ernest Muir in
1929, initially in the form of quarterly notes,
and later on transformed itself to a fullfledged scientific journal. Consistent with its
contents and nature, Leprosy in India was
renamed as the Indian Journal of Leprosy in
1984.
Dr. Dharmendra straddled the scene of
leprosy in India like a giant and is known for
lepromin test, Indian classification of types
of leprosy and the journal Leprosy in India.
The National Leprosy Control Programme
(NLCP) was started in 1954-55. In view of
scientific advancement and availability of
highly effective treatment of leprosy, the
programme was redesignated as National
Leprosy Eradication Programme (NLEP) in
1983.
In 1991, the World Health Organization set a
target of elimination of leprosy as a public
health problem by the year 2000. India
achieved this target in 2005.

Essentials in Dermatology

Microanatomy of the Skin

Dermatology may be defined as the study of the


skin and its diseases or is a branch of medical
science, which deals with systematic study of
skin in health and disease. Since skin conditions
constitute 10 to 30% of outpatient attendance in
any hospital, they are often easily noticed by
others (hence a cause of great social concern to
the patient) and very often, skin diseases offer
diagnostic clue to many major systemic
disorders, makes this subject challenging and
important to study.

FUNCTIONS OF THE SKIN


The skin is the largest organ of the body,
accounting for 16-20% of total body weight. The
skin of an average adult covers an area just under
2 m2. It not only gives shape to the body but also
helps it in many ways the important functions
of the skin are:
1. Protection (Barrier function) from:
a. Physical injuries
b. Chemical injuries
c. Infections
2. Thermoregulation
3. Sense organ: A number of sensations
touch, pressure, warmth, cold and pain are
perceived by the skin.
4. Storage of electrolytes, carbohydrates,
water, fat, vitamins, proteins, etc.

5. Vitamin D formation: Vitamin D 3 is


essential for skeletal development.
6. Absorption: The skin surface also performs
absorptive function and is the basis of
topical therapy in dermatology.
7. Excretion: Some of the toxins may be
excreted through the skin.
8. Immune surveillance: This immunological
function is performed by Langerhans cells,
dendritic cells (intermediate) and keratinocytes.
9. Mechanical function: The mechanical
properties of the skin depend mainly on the
dermis.
10. Cosmetic function: Colour of the skin and
hair and nails are important for their
decorative value. Hair does not perform a
vital physiologic function but it does
provide a sexually attractive ornament.

DEVELOPMENT OF SKIN
Epidermis develops from ectoderm lateral to
neural crest, dermis from mesenchyme and
neural crest cell, subcutaneous fat from
mesenchyme and melanocytes from neural crest.
Foetal skin development occurs in three stagesspecification, morphogenesis and differentiation.
Its specification occurs from 0 to 60 days,
morphogenesis from 2 to 5 months, and
differentiation from 5 to 9 months.

Microanatomy of the Skin

STRUCTURE OF THE SKIN


Skin has 3 layers (Fig. 3.1)
1. Epidermis
2. Dermis
3. Subcutaneous fat (Hypodermis).

Epidermis
It is approximately 0.4 mm to 1.6 mm in
thickness. The majority of the cells in the
epidermis are the keratinocytes. These cells are
organized into five layers-stratum corneum,
stratum lucidum (present only in palmar and
plantar skin), stratum granulosum, stratum
spinosum, and stratum basale or stratum
germinatum (Fig. 3.2).
Stratum corneum is the outermost layer
containing flattened anucleated cells without cell
organelles. The thick epidermis of palms and
soles has an additional layer underneath the
stratum corneum that is electron lucent and is
called the stratum lucidum. The stratum
granulosum is so called due to the presence of

intracellular basophilic keratohyaline granules


and consists of 2-5 cells layer. The stratum
spinosum contains 8-10 layers of polyhedral cells
with round nuclei. The stratum basale or stratum
germinativum consists of single layer of cuboidal
or columnar cells.
Keratin filaments are a hallmark of the
keratinocytes and the process by which a
keratinocyte of the basal layer ultimately changes
into keratin is known as keratinization and it
usually takes 4 weeks for its completion. The
epidermal turnover time is about I month.
The other member cells found in the
epidermis are melanocytes (derived from neural
crest, produce melanin), Langerhans cells (origin
from bone marrow, play important role in
cutaneous immune mechanisms), and Merkel
cells (slow adapting type 1 mechanoreceptors).

The Dermal-epidermal Junction


The dermal-epidermal junction (DEJ) is a
basement membrane zone (BMZ) that welds the
epidermis to underlying dermis.

Fig. 3.1: Structure of the skin

10

Essentials in Dermatology

Fig. 3.2: Diagrammatic representation of shapes of various cells in the epidermis and changes in them as
they move up from basal cell layer to stratum corneum during the process of keratinization

The dermal-epidermal junction is undulated,


forming dermal papillae (upward projections of
the dermis into the epidermis) and rete ridges
(downward projections of epidermis into the
dermis). The DEJ under electron microscope
consists of four components- plasma membrane
of basal keratinocytes with hemidesmosomes,
lamina lucida (made up of anchoring filaments
and laminin 5), lamina densa ( has type IV
collagen and laminin 5), and lamina
fibroreticularis (containing anchoring fibrils,
dermal microfibrils, and collagen fibers). This
DEJ is weakest at lamina lucida.

Dermis
The dermis is formed by connective tissue
having fibres (collagen, elastic and reticulin) and

ground substance (made up of proteoglycans


and glycosaminoglycans). It varies in thickness
from about 1 mm on the face to 4 mm on the
back and thigh.
Collagen fibres are the major component of
the dermis, accounting for 75% of dry weight of
the skin. Approximately 80-90% of collagen
fibres in the dermis are of type I collagen. They
are responsible for the mechanical properties of
the dermis. Elastic fibers constitute approximately 4% of dry weight of dermal matrix
proteins. Due to their elasticity, they maintain
the normal configuration of the skin.
The dermis can be divided into an upperpapillary dermis that interdigitates between the
rete ridges and the deeper-reticular dermis
recognized by the thicker, aggregated bundles
of collagen.

Microanatomy of the Skin

Epidermal Appendages Like


a. Pilosebaceous unit
b. Sweat glands.
Pilosebaceous unit: It consists of a hair follicle
containing hair and sebaceous glands opening
into follicular canal of hair follicle.
Sebaceous glands are lipid secreting
holocrine glands. Their maximum density is in
seborrhoeic areas of the body, i.e. scalp, face,
upper chest, etc. They get activated at puberty
under the influence of androgen hormone.
Functions of sebum are:
1. Barrier function by preventing loss of water
from the skin
2. Emulsification of surface eccrine sweat
3. May have mosquito repellant action
4. Protection against sunburn
5. Has a vitamin D precursor.
Hair structurally consists of a cuticle, cortex
and medulla. These keratinous fibres are of two
types in adults-terminal hair and vellus hair.
Hair grows at the rate of 0.3 mm per day and
they undergo growth cycle. These recurring
cycles consist of anagen (active growth phase),
catagen (static growth phase) and telogen
(shedding phase) phases. On the scalp, 80% to
85% of hair are in anagen phase and 15% to 20%
in catagen and telogen phase. The anagen phase
lasts for two to five years, a short catagen of few
days and a telogen phase of three months.
Functions of hair are:
1. Cosmetic
2. Hair screens entry of irritants to nose
3. Protect scalp from sunrays
4. Shields the eyes
5. Helps in perception of tactile stimuli.
Sweat glands: Two types of sweat glands are
eccrine and apocrine sweat glands.
Eccrine sweat glands: They are tubular
structures, which open on to the skin directly and
have three segmentsthe secretory coil (consists
of single layer of secretory cellsclear and dark

cells) in the deep dermis, straight intradermal


(has two layers of cuboidal cells lined by
eosinophilic cuticle on luminal side) part and
coiled or spiral intraepidermal (consists of an
inner layer of luminal cells and two to three outer
layer of epithelial cells) part. Sweat glands are
most abundant on the palms, soles, forehead and
axillae. These glands are highly developed and
responsive part of the thermoregulatory
apparatus, innervated by cholinergic nerve
fibers.
Functions of sweat are:
1. Sweating in heat stress
2. Excretion of heavy metals and drugs.
Apocrine sweat glands: These tubular glands
consists of two main parts the coiled secretary
gland (consists of single layer of cuboidal or
columnar cells, surrounded by a layer of
myoepithelial cells) and the straight excretory
duct (consists of double layer of cuboidal cells
and inner eosinophilic cuticle) which opens into
follicular canal just above the openings of
sebaceous glands. They are distributed along the
mammary line, i.e. axillae, areolae, periumbilical
area, mons pubis, genital and perianal areas.
Apocrine gland secretion in man serves no
function. Pheromonesits role in humans is
debated.
Nail unit: It is yet another epidermal appendage.
It consists of nail matrix just underneath the
proximal nail fold which gives rise to nail plate
a keratinized structure. The distal portion of
the nail matrix is visible usually in thumbnail as
white crescent or half moon known as lunula.
The rectangular nail plate rests on a nail bed and
is bounded on two sides by lateral nail folds.
The cuticle seals the space between nail folds and
nail plate. The distal portion of nail juts out as a
free end. The space underneath the free end of
the nail plate is called subunguium. In contrast
to hair, nail is a continuously growing structure,
usually at a rate of 0.1 mm per day.

11

12

Essentials in Dermatology
Functions of nails are:
1. Protect terminal phalanges
2. Cosmetic function
3. Helps in appreciation of tactile stimuli
4. Scratching of skin
5. Helps in holding minute objects with finger
tips.
Besides the above elements, dermis contains
blood vessels which form two plexuses (other
than providing nutrition to the skin, blood
vessels regulate temperature and blood
pressure), lymphatics roughly parallel the major
vascular plexuses, nerves of the skin are part of
two major systems somatic sensory and
autonomic motor, smooth muscle occurs in the
skin as arrectores pilorum, as the tunica dartos
of the scrotum and in the areolar around the

nipples and cells mast cells, fibroblasts,


monocytes, macrophages, dendrocytes and
pericytes, etc.

Subcutaneous Fat (Hypodermis)


The subcutaneous fat layer is constituted by
adipocytes. It is abundant over the breasts,
buttocks, and abdomen, thinner over the nose
and sternum and absent over the eyelids and
male genitalia. It acts as shock absorber, helps
in heat production and hormone conversion,
facilitate mobility of skin over structures that
underlie and acts as an insulator for heat. A
cosmetic role is contributed by the accentuated
distribution of fat in some sites in the sexes. Most
importantly, it stores triglycerides, which serves
as fuel for energy.

Physiology, Biochemistry and Immunology of the Skin

Physiology, Biochemistry and


Immunology of the Skin

Main functions of the skin are protection,


thermoregulation, sensory, storage organ,
vitamin D formation, absorption, excretion,
immune surveillance, mechanical and cosmetic
function. Some important physiological,
biochemical, and immunological processes of the
skin are summarized below.

epidermal barrier is localized to the stratum


corneum.
The skin has two barriers to UV radiations:
a melanin barrier in the epidermis; and a protein
barrier concentrated in the stratum corneum.
Both function by absorbing radiation thereby
minimizing absorption by DNA and other
cellular constituents.

PROTECTIVE FUNCTIONS OF THE SKIN


The innermost region of human skin is the
subcutaneous fat layer. This layer insulates
reduces heat movement into or out of the body,
absorbs energy from blunt mechanical trauma
and is active in general energy metabolism.
Superficial to the fat layer lies the dermis,
composed of collgen-glycosaminoglycan
complexes which also protects the body from
blunt mechanical trauma. Overlying the dermis
is the epidermis which consists of several
stratifying layers of nucleated keratinocytes and
anucleated top layer, the stratum corneum which
performs the major barrier function.
The skin acts as a two way barrier to prevent
the inward or outward passage of water and
electrolytes. The physical barrier is largely
situated in the epidermis, isolated epidermis
being as impermeable as whole skin, whereas
once the epidermis is removed, the residual
dermis is almost completely permeable. The

PERCUTANEOUS ABSORPTION
The skin is considered to be a composite
membrane with three anatomically distinct
layers; the stratum corneum (10 m), the viable
epidermis (100 m), and the uppermost papillary
layer of the dermis (100-200 m), each having a
different diffusion constant. Even healthy adult
human skin allows some permeation of almost
every substance, and rates of penetration of
different materials may differ by 10,000 fold.
The efficiency of the barrier differs between
body sites. The scrotum is particularly permeable
and the face, forehead, and dorsa of the hands
may be more permeable to water than the trunk,
arms, and legs. The palms are particularly
impermeable to nearly all molecules except
water.
The barrier is affected by many other factors,
such as age, environmental conditions and
physical trauma, and permeability can be

13

14

Essentials in Dermatology
enhanced by various agents, permitting
increased access of topically applied drugs.

KERATINIZATION
Keratinization is a process of differentiation of
germinative cells in the basal cell layer into the
deceased cornified cells of stratum corneum.
It involves:
1. Synthesis of distinctive proteins (e.g. keratins,
filaggrins, and involucrin) and lamellar
granules, and
2. Alterations of nuclei, cytoplasmic organelles,
plasma membranes and desmosomes.
Keratin function is to provide mechanical
strength, cellular structure, and assistance in
adhesion molecule attachment. Soft keratin
desquamates as the result of enzymatic action
but the hard keratin of the hair and nails
does not, thus requiring periodic cutting.
The epidermis is the prototype of keratinizing
squamous epithelia, also present in the
oesophagus, vagina and oral mucosa.

MELANOCYTES AND MELANOGENESIS


Melanocytes are pigment forming cells in the
epidermis. Each melanocyte supplies pigment,
melanin to approximately 36 keratinocytes and
thus form epidermal melanin unit. Two types of
melanin are synthesized by melanocytes,
eumelanins and pheomelanins. Melanin is
synthesized from tyrosine under the influence
of enzyme tyrosinase through the formation of
various intermediates (dihydroxyphenylalanine
[dopa], dopaquinone, leucodopachrome,
dopachrome, 5 6 dihydroxyindole, indole
5 6-quinone, melanochrome).

FUNCTIONS OF THE MELANIN


1. Protect the skin from harmful effects of
sunlight by scattering and absorbing
ultraviolet light.

2. Melanin may also act as a biochemical


neutralizer of toxic free radical oxygen
derivatives, byproducts of various inflammatory processes.
3. Melanocytes situated in the matrix of anagen
follicles impart to hair various colours, e.g.
blond, brunette and red head.

THERMOREGULATION
The maintenance of a near constant body core
temperature of 37oC is a great advantage to
humans, allowing a constancy to biochemical
reactions which would otherwise fluctuate
widely with temperature changes.
The thermoreceptor cells of the skin are
distributed irregularly over the skin, there being
warm- and cold-sensitive thermoreceptors.
Information on changes in their stimulation in
response to changes in the temperature is sent
to the hypothalamus leading to either to
inhibition of sweating or stimulation of
shivering. Skin temperature has a greater role
in mediating the behavior, for example by
turning on the heating or putting on extra
clothing.
Thermoregulation depends on several
factors, including metabolism and exercise but
the skin plays an important part in control
through the evaporation of sweat and by direct
heat loss from the surface. Heat can be lost
through the skin surface in four ways:
1. Radiation
2. Convection
3. Conduction
4. Evaporation

SKIN FAILURE
Skin failure is defined as a loss of normal
temperature control with inability to maintain
the core temperature, failure to prevent
percutaneous loss of fluids, electrolytes and
proteins with resulting imbalance and failure of

Physiology, Biochemistry and Immunology of the Skin


mechanical barrier to penetration of foreign
materials. Apart from thermal burns, skin failure
can occur as a consequence of a number of
dermatological diseases including Stevens
Johnson syndrome, toxic epidermal necrolysis,
pustular psoriasis and erythroderma of various
causes.

COLLAGEN IN THE DERMIS


The closely related proteins of collagen family
are the main fibrillary components of the
connective tissues and the major extracellular
proteins of the human body. The physiological
role of collagen fibers in the skin is to provide
tensile properties that allow the skin to serve as
a protective organ against external trauma.
Collagen is the major structural protein
constituting 70% to 80% of dry weight of the
dermis. The main aminoacids in collagen are
glycine, proline and hydroxyproline.

Elastic Fibers in the Dermis


Elastic fibers of the connective tissue form a
network responsible for the resilient properties
of the skin. In sun protected human skin, elastin
content is about 1% to 2% of the total dry weight
of dermis.
Ground Substance in the Dermis
The ground substance of skin is largely made
up of glycosaminoglycans(GAG) and provide
viscosity and hydration in the dermis. Three

types of GAG are chondroitin sulphate,


dermatan sulphate and hyaluronic acid.

IMMUNOLOGICAL COMPONENTS OF
SKIN
The immunological functions of the skin depend
both upon cells in the epidermis and on dermal
cellular constituents. Antimicrobial peptides
(AMPs) are a diverse group of proteins that are
involved as first line of immune defense by many
living things. In human skin, AMPs provide a
chemical barrier to potentially pathogenic microorganisms. Sebaceous lipids have been reported
to possess antibacterial properties and
glycophospholipids and free fatty acids of
stratum corneum have bacteriostatic effect
selective for pathogenic organisms.
Skin associated lymphoid tissue (SALT) is
langerhans cells, T lymphocytes, mast cells and
keratinocytes. They are involved in various
hypersensitivity reactions of the skin. Hypersensitivity is defined as inappropriate or
exaggerated immune response to a foreign or self
antigen resulting in tissue damage. Main types
of hypersensitivity responses of skin are type I
(immediate), type II (antibody-dependent
cytotoxicity), type III (immune complex disease)
and type IV(cell mediated or delayed). Urticaria
and anaphylaxis is the example for type I
hypersensitivity, transfusion reactions for type
II hypersensitivity, leukocytoclastic vasculitis for
type III hypersensitivity and allergic contact
dermatitis for type IV hypersensitivity.

15

16

Essentials in Dermatology

Principles of Diagnosis in
Dermatology

Dermatologists often prefer to examine the


patient before obtaining the history and review
of systems. This approach is preferred because
diagnostic accuracy is higher when visual
examination is approached without preconceived ideas. Moreover, some dermatologic
lesions are so distinctive that history is not
required to make a diagnosis.
A practical and convenient way to arrive at
a diagnosis may be...

Chief complaints: Limit them to just three only


History of present illness: What is your skin
problem?
This allows patient to point out the lesions and
the area involved.
Three basic questions.
1. Onset and evolution.
2. Symptoms.
3. Treatment taken.
Onset and evolution: To determine the duration
of the disorder, how it evolved over time, initial
site of the disease, mode of spread.

PRELIMINARY GENERAL HISTORY

Symptoms: Itching is the most common and


most important symptom in dermatology.
Intense itching, e.g. scabies, atopic dermatitis,
lichen planus, dermatitis herpetiformis.
Pain may predominate in herpes zoster,
furuncles, etc. Loss of sensation points towards
leprosy, or follicular mucinosis. Allodynia
(production of pain by trivial stimuli) occurs in
postherpetic neuralgia. Reversal of hot and cold
sensation may be due to ciguatera fish poisoning.
They may be just asymptomatic as in molluscum
contagiosum, basal cell carcinoma, etc.

Biodata: Age, sex, income, occupation, address,


marital status.

Treatment history: Skin lesions are often selfmanipulated by home remedies, or over the

Principles of Diagnosis in Dermatology


counter medication, since they are easily
accessible, and since disease is of chronic nature.
Full detailed history of medication used
should be known because
1. Disease may be caused or aggravated by
medication- Fixed drug eruption, dermatitis
medicamentosa.
2. Patient may have already used the drug
without desired outcome, we planned to
give. Avoid potential embarrassment when
the patient says I have already tried that and it
didnt work.
Detailed follow-up history: This history is taken
after some diagnosis or conclusion was reached
by initial history and examination, and this
includes.
Past history.
Family history.
Review of systems.
Social history.
Females- menstrual/obstetric history.
Past history:
a. History of same disease before.
b. History of prolonged illness diabetes,
hypertension.
c. Drugs used for other problems (drug rash,
urticaria).
d. Drug allergies avoid prescribing those
drugs.
e. Atopic history asthma, hay fever, eczema.
Family history: It is important for diagnosis,
prognosis, treatment and genetic counseling.
Family history important in:
a. Infectious disorders scabies
b. Inherited disorders atopy, psoriasis.
c. Genodermatoses.
Review of other systems: It is required in
multisystem disorders like SLE, scleroderma, or
lepromatous leprosy.
Social history: Encounter with potentially
sensitizing materials e.g., in patients with

industrial dermatosis, contact dermatitis. Stress


and strain at work may lead to exacerbation of
psoriasis, neurodermatitis, etc.
Habits: Alcohol induces porphyria cutanea tarda
in predisposed, influences the severity and
therapeutic options in psoriasis. Smoking may
be aggravating factor in palmoplantar
pustulosis.

PHYSICAL EXAMINATION
It has been said by Goethe What is most difficult
of all? It is what appears most simple: To see with
your eyes what lies in front of your eyes .

Requirements for the Skin to be


Properly Examined
Three essential requirements
1. Preferably a completely undressed patient,
clothed only in an examination gown. If not
possible, at least, the affected part should be
properly exposed.
2. Adequate illumination: Preferably sunlight
or a bright overhead fluorescent lighting.
Penlight is used in side lighting- to determine
if a lesion is subtly elevated and for
examining the oral cavity.
3. An examining physician ready to see what
is before him.
A complete cutaneous examination should
be made, this includes examining
Skin from head to foot.
Mucous membrane in mouth and genitals
Hair and nails.
The examination includes inspection and
palpation, besides percussion and auscultation.
Palpation is useful in
Assessing the texture and consistency.
Evaluate whether a lesion is tender or not.
Reassure a patient that they do not have a
contagious disease.

17

18

Essentials in Dermatology
Hand lens useful on occasions like identifying:
a. Altered skin markings in tumors.
b. Nail fold telangiectasia.
c. Burrows in scabies.
d. Wickhams striae- for this place a drop of
mineral oil on the area, which makes the
stratum corneum transparent.
Subtle genital warts- aceto-whitening,
gauze soaked with 5% acetic acid applied in
suspected area for 3 minutes, warts turn white.
Actually individual skin lesions are
analogous to the letters of the alphabet, and
groups of lesions can be analogous to words or
phrases. Basis of morphological lesions is given
in the form of table for clear understanding.
Basis of morphological lesions in dermatology
1. Impalpable change- Macule
2. Palpable change
Solid change-Papule, plaque, nodule, wheal

Superficial visualized free fluid collectionVesicle, bulla

Superficial free pus collection-Pustule, abscess

Deep free fluid/semisolid material collection-Cyst


3. Loss of skin-Erosion, ulcer
4. Healing stage- Scale, crust
5. End stage- Atrophy, scar

Fig. 5.1: Maculedepigmented flat lesions of variable


size and shape of vitiligo vulgaris and lip tip type

Ultimately, diagnosis may rest on recognition


of lesions and their distribution and
arrangement, whether they are primary,
secondary or some special lesions. Describe their
shape, size, color and distribution. Take the
help of diagnostic tools for further details.

Primary Lesions
These are the lesions, which appear first in any
skin disease. They are the best clues to the
diagnosis. They are:
Macule: The macule is a discrete, flat,
circumscribed lesion that differs from
surrounding skin because of its color
(Fig. 5.1). It may be a small or a large macule.
Earlier used term patch is now obsolete.
Macule may be erythematous, hypo-

Fig. 5.2: Papulesolid elevated lesions of verruca


vulgaris of less than 0.5 cm

pigmented, hyperpigmented or of any other


color.
Papule: It is a discrete, circumscribed, solid
elevated lesion of less than 0.5 cm in size (Figs
5.2 to 5.5). So, it is a palpable lesion. A papule
may be dome shaped, verrucous, umbilicated, pedunculated, etc.

Principles of Diagnosis in Dermatology

Fig. 5.3: Papuledome shaped papule, a few of


them umbilicated of molluscum contagiosum

Fig. 5.5 Violaceous colored papules of lichen


planus over the genitalia and thigh

Fig. 5.4: Typical umbilicated papule of molluscum


contagiosum

Fig. 5.6: Plaqueflat elevated lesions covered with


silvery white micaceous scales of psoriasis vulgaris

Plaque: A plaque is a circumscribed solid


raised lesion with a flat top. It is formed due
to coalescence of papules (Figs 5.6 and 5.7).
It may be a lichenified plaque, eczematous
plaque, psoriasiform plaque, flat smooth
plaque, etc.
Nodule: A nodule is a discrete circumscribed
solid elevated lesion, which is more felt than

seen from the top (Figs 5.8 and 5.9). It may


develop from a papule.
Vesicles and bullae: Vesicle and bullae are two
terms used for circumscribed elevated lesions
containing free clear fluid, called blister. If it is
less than 0.5 cm, it is called vesicle (Fig. 5.10)
and if more than this, it is a bulla (Figs 5.11 and
5.12). They may be tense or flaccid.

19

20

Essentials in Dermatology

Fig. 5.7: Large well-defined erythematous plaques


of psoriasis vulgaris

Fig. 5.8: Nodulesolid deep-seated elevated


lesion due to secondaries in the skin

Fig. 5.9: Erythematous tender nodules of furuncle


over the face and neck

Fig. 5.10: Vesiclecluster of tiny blisters of herpes


labialis over the lips

Fig. 5.11: Bullasmall blisters on erythematous


bases of bullous pemphigoid

Fig. 5.12: Large tense bulla of bullous pemphigoid


on an erythematous base

Principles of Diagnosis in Dermatology


Pustule: A pustule is a circumscribed
elevated lesion containing visible pus
(Fig. 5.13). It results from an epidermal or
upper dermal accumulation of pus.
Cyst: A cyst is a sac that contains liquid or
semisolid material.
Wheal: Wheal is a pale or erythematous
edematous, transient, evanescent lesion.
Diffuse thickening of skin: It may result
from edema of dermis (pitting edema or
nonpitting edema) or infiltration of dermis
(e.g. myxoedema, lepromatous leprosy).

Secondary or Consecutive Lesions


They are due to the subsequent changes, which
takes place on the primary lesions, either as a
part of natural evolution or due to manipulation
of the patient.
Oozing: It is due to the rupture of vesicles or
bullae.
Crust: It is dried up exudate like serum, pus
or blood (Fig. 5.14). It may be thick or thin,
friable or adherent. It occurs in many
inflammatory and infectious diseases.
Scale: Scales are thin, dry plates of heaped
up desquamating epithelial cells formed as

Fig. 5.13: Pustulenumerous tiny pus filled lesions


on erythematous background in a case of pustular
psoriasis

a result of either increased or abnormal


keratinization (Fig. 5.15).
Excoriation: An excoriation is a superficial
erosion or ulcer caused by scratching. So,
it will be linear or have a geometric outline
(Fig. 5.16).
Erosion: It is a superficial ulceration
involving epidermis only which heals
without scarring (Fig. 5.17).
Ulcer: It is a break in continuity of epithelium,
which involves epidermis, and dermis of the
skin (Fig. 5.18). It has length, breadth as well
as depth. It heals with scar formation.
Fissure: Fissure is a linear crack in the skin,
which may be superficial or deep to the
dermis.
Lichenification: It is characterized by
thickening of the skin (becomes leather
like) with increased skin markings and
pigmentation. It is seen in chronic dermatitis.
Scar: A scar is an evidence of destruction of
the skin with fibrotic tissue replacement. It
occurs wherever ulceration has taken place
and reflects the pattern of healing in those
areas.

Fig. 5.14: Crustdried out oozed material over the


face in impetigo contagiosa

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22

Essentials in Dermatology

Fig. 5.15: Scaleface and trunk covered with moist


scales of pemphigus foliaceous

Fig. 5.16: Excoriationmultiple linear scratch


marks due to itching over psoriatic plaques

Fig. 5.17: Erosionmultiple superficial eroded


areas in herpes genitalis

Fig. 5.18: Ulcersingle, painless, indurated


ulceration of extragenital primary chancre

Pigmentation: Pigmentation may be hyper,


hypo- or depigmentation of the skin (varies
according to the quantity of melanin).
Atrophy: Atrophy refers to a diminution
in the size of a cell, tissue, organ or part of
the body. The skin becomes thin, shiny and

wrinkled. Atrophy may be of epidermal,


dermal or subcutaneous fat.
Sclerosis: Sclerosis means a circumscribed or
diffuse hardening or induration in the skin.
It occurs as a result of an increase in the
amount of dermal collagen, expansion of the

Principles of Diagnosis in Dermatology


collagen by ground substance material or
altered quality of collagen.

Special Lesions
These lesions are given below:
Comedone: It is a plug of keratin and sebum
formed in the follicular canal of
pilosebaceous unit. Comedones may be
closed or open.
Burrows: These are serpentine caves of
scabies mite at the level of stratum
granulosum. They are visible as S-shaped
brownish-black lesions, which at their distal
end have a papule housing the mite.
Alopecia means loss of hair.
Telangiectasia: It refers to individually
visible dilated vessels.
Poikiloderma: It is a combination of atrophy,
pigmentation and telangiectasia.
Purpura: It is visible extravasated blood
(Fig. 5.19). It may occur as tiny pinpoint spots

(petechiae) or larger spots (ecchymoses). The


term hematoma refers to an area of massive
bleeding into the skin and underlying tissues.
Pinch purpura hemorrhage induced by
mild often subclinical trauma is a characteristic presentation of primary systemic
amyloidosis of the skin. Similar periorbital
hemorrhage following proctoscopy or
pulmonary function testing, postproctoscopic
purpura also typifies the vascular fragility
induced by systemic amyloidosis.
Livedo: Blue red discoloration of the skin of
skin due to passive congestion of the vessels
often with net-like pattern
Exanthem: Abrupt appearance of diffuse or
generalized similar skin lesions (usually
represents viral infections or drug reactions)
Enanthem: Abrupt appearance of mucosal
lesions similar to exanthems.
Nits: They are glistening white ovoid bodies
attached to shafts of hair.

Fig. 5.19: Purpuramultiple, small, erythematous non-blanchable lesions of purpura

23

24

Essentials in Dermatology

DISTRIBUTION OF SKIN LESIONS IN SOME DERMATOLOGICAL DISORDERS


Disease
1. Acne vulgaris
2. Atopic dermatitis
3. Dermatitis herpetiformis
4. Lichen planus
5. Neurodermatitis
6. Pityriasis rosea
7. Psoriasis vulgaris
8. Scabies

9. Seborrhoeic dermatitis

10. Tinea versicolor

Classical sites of involvement


Face, upper trunk, proximal parts of upper extremities
Infants -face and extensor aspects of limbs (Figs 5.20A and B)
Children and adults-flexures (Figs 5.21A and B).
Scalp, extensor aspects of limbs, shoulder and buttocks (Figs
5.22A and B).
Flexor aspect of upper extremities (wrists), trunk (lumbosacral
area), shins, glans penis (Figs 5.23A and B).
Nape of neck, wrist, ankle, genitalia, and perianal area (Figs
5.24A and B).
Herald patch-trunk. Daughter patches-Christmas tree pattern
over the trunk (Figs 5.25A and B).
Extensor aspects of limbs, scalp, lumbosacral area (Figs 5.26A
and B).
Infants-Face, intertriginous area of fingers, palms and soles,
extensor aspect of limbs, around umbilicus, genitalia and
gluteal area (Figs 5.27A and B). Children and adults-finger
web spaces, wrist, elbows, axillary fold, around areola and
umbilicus, genitalia, and gluteal area (Figs 5.28A and B).
Infants-Cradle cap over scalp (Figs 5.29A and B). Adolescence
and adults- scalp, eyebrows, nasolabial folds, presternal and
interscapular area, axilla and groin (Figs 5.30A and B).
Upper trunk (Figs 5.31A and B).

Figs 5.20A and B: Distribution of skin lesions in


atopic dermatitis in infants

Figs 5.21A and B: Distribution of skin lesions in


atopic dermatitis in children and adults

Principles of Diagnosis in Dermatology

Figs 5.22A and B: Distribution of skin lesions in


dermatitis herpetiformis

Figs 5.23A and B: Distribution of skin lesions in


lichen planus

Figs 5.24A and B: Distribution of skin lesions in


neurodermatitis (lichen simplex chronicus)

Figs 5.25A and B: Distribution of skin lesions in


pityriasis rosea

Figs 5.26A and B: Distribution of skin lesions in


psoriasis vulgaris

Figs 5.27A and B: Distribution of skin lesions in


infants in scabies

25

26

Essentials in Dermatology

Figs 5.28A and B: Distribution of skin lesions in


children and adults in scabies

Figs 5.29A and B: Distribution of skin lesions in


seborrhoeic dermatitis in infants

Figs 5.30A and B: Distribution of skin lesions in


seborrhoeic dermatitis in adolescence and adults

Figs 5.31A and B: Distribution of skin lesions in


tinea versicolor

Certain phenomenon and signs are there to be


seen and observed; others need to be elicited by
tools.

of dermatological disorders such as psoriasis


(Fig. 5.32), lichen planus, vitiligo, eczema,
dermatitis herpetiformis, bullous pemphigoid,
warts (Figs. 33 and 34), molluscum contagiosum
(Fig. 5.35), etc.
Reverse Koebner phenomenon: Area of
psoriasis clears of following injury.
Remote reverse Koebner phenomenon is the
spontaneous repigmentation of vitiligo
patches distant from the autologous skin
graft sites.

The Koebner phenomenon: The Koebner


phenomenon is the development of
morphologically identical lesion/s in the
traumatized uninvolved skin of patients who
have cutaneous diseases. It is also known as
isomorphic phenomenon, a self-explanatory
term. This phenomenon is observed in a number

Principles of Diagnosis in Dermatology

Fig. 5.32: Koebner phenomenon in psoriasis


vulgaris over the trunk

Fig. 5.33:Koebner phenomenon in


plane warts over the wrist

Fig. 5.34: Koebner phenomenon


in verruca vulgaris

Fig. 5.35: Koebner phenomenon in molluscum


contagiosum

Dermographism: It can be elicited with the


help of blunt instrument like key. Firm
stroking of skin may result in exaggerated
triple response of Lewis, which persists for
more than 5 minutes. Stroking causes histamine to be released, leading to localised
redness and edema (Wheal). Dermographism
can occur in urticaria.

Dariers sign: When the above phenomenon


is limited to skin overlying a lesion (macule
or papule), it is called as Dariers sign and is
diagnostic of urticaria pigmentosa (Mast cell
disease).
Pseudo-Dariers sign: Here stroking of skin
produces transient induration with pilo-

27

Essentials in Dermatology

28

erection, seen in congenital smooth muscle


hamartoma
White dermographism: Stroking the skin of
atopic patients produces a characteristic
white line in the involved area.
Grattage test: It is done on a scaly lesion to
look for types of scales. Scraping of the lesion
is done with a glass slide. Fine powdery
scales of tinea versicolor can be made out if
you examine the glass slide against light after
scraping the lesion.
Candle sign and last cuticle sign: In
psoriasis, if the silvery-white scales are
scraped off, they detach from the lesions as
small flakes, similar to wax scraped from
candle. With continued scraping, one can
remove a coherent moist sheet from the lesion
corresponding to the lowest layers of
epidermis.
Auspitzs sign: This sign if present is
diagnostic of psoriasis. It has three
components.
1. On scraping with glass slide, initially
silvery white micaceous scales come out.
2. Removal of the scales is followed by a thin
membranous structure.
3. On its removal by glass slide, minute
pinpoint bleeding spots are seen.
Diascopy (Vitropression): It is based on the
principle that vascular lesions will blanch in
response to pressure with a glass slide
whereas purpuric lesions, in which blood and
blood pigments have leaked from the
cutaneous vessels, will not blanch. Diascopy
is most useful in detection of nonblanchableraised purpura, the clinical hallmark of
cutaneous vasculitis. It is useful in
differentiating nevus anemicus from nevus
depigmentosus. Nevus anemicus (a localised
area of vasoconstriction) on diascopy of
adjacent skin reveals an identical color to
depigmented area. By contrast diascopy of
skin adjacent to nevus depigmentosus or
vitiligo, the affected area still remains paler.

Apple jelly nodules in lupus vulgaris active


edge of the lesion, appear as translucent
brownish color granulomatous nodules, a
distinctive feature of the disease.
Ollendorfs sign: If touching of the papule
of secondary syphilis with the head of pin is
exquisitely tender, then Ollendorfs sign is
said to be present.
Nikolskys sign: A frictional force is applied
with a finger or thumb over the apparently
normal skin, usually overlying a bone like the
clavicle or perilesional skin in a patient with
vesiculo-bullous lesions. If epidermis or
surface of the skin breaks down or peels off
leaving raw moist erosion, it is called positive
Nikolskys sign. Various disorders, in which
Nikolskys sign is positive, are pemphigus,
staphylococcal scalded skin syndrome, toxic
epidermal necrolysis, etc. This test is based
on the fact that in certain diseases, even the
normal looking skin has a weak cohesion
between its different layers.
Bulla spread sign: It can be demonstrated by
marking the boundary of the bulla and then
applying pressure with a finger on the edge
of the bulla. In pemphigus, bulla spreads
beyond the marked line showing that active
process of acantholysis has weakened the
cohesion between keratinocytes.
Button holing sign: In neurofibromatosis, if
fingertip is pressed over neurofibroma, the
finger gapes in due to defect in the dermis.
Dimple sign: It distinguishes dermatofibroma from malignant melanoma.
Applying lateral pressure with thumb and
index finger results in formation of a dimple
in dermatofibroma, whereas melanoma
protrudes above its original plane.
Hess test or Capillary fragility test: A blood
pressure cuff is applied to upper arm
between systolic and diastolic pressure for
5 minutes. The number of petechiae in
predetermined area of 5 cm circle is counted.

Principles of Diagnosis in Dermatology


If number is more than 5, it is abnormal
capillary fragility.
Pathergy test: Inject 0.1 ml of physiologic
saline intradermally with fine needle over the
forearm. Read after 24-48 hours. Pustules or
papules suggest the diagnosis of Behcets
disease. Histology shows neutrophilic
infiltrate or vasculitis.
Testing sensation and palpation of
peripheral nerves may be required to fulfil
one of the cardinal features of leprosy and
thus help in its diagnosis.

LABORATORY AND SPECIAL TESTS


They may be needed to confirm the diagnosis:
1. Woods light examination: It is useful in
detecting fungal infections of the scalp
(bluish-green fluorescence in tinea capitis
caused by Microsporum speciesMicrosporum canis and Microsporum audouinii),
tinea versicolor (yellow fluorescence),
erythrasma (coral red fluorescence),
porphyrins in patients with porphyria
cutanea tarda (Urine will produce bright red
fluorescence), trichomycosis axillaris
(orange fluorescence), Pseudomonas infection (green fluorescence), etc. In scabies,
fluorescein solution fills burrows. In
pigmentary disorders, vitiligo, piebaldism,
and ash leaf macules of tuberous sclerosis,
the lesions become prominent.
2. KOH preparation: Indicated when
infection with fungi or yeast is suspected,
e.g. dermatophytosis, tinea versicolor,
candidiasis, etc.
3. Tzanck test: It is used in the diagnosis of
various skin disorders characterised by
vesicles, pustules, bullae and erosions and
in particular in viral infections like herpes
simplex, herpes zoster and varicella
infections.

4. Grams stained pus smear: Indicated for


pyogenic infections, vaginal and urethral
discharge.
5. Tissue smear: It is used for diagnosis of
donovanosis (granuloma inguinale).
6. Dark field (ground illumination) test:
Primary and secondary syphilis can be
easily diagnosed by demonstrating
treponemes.
7. Wet preparation: It is utilized for diagnosis
of trichomonal infestation of the genital
tract.
8. Slit skin smear: This test is performed on
leprosy patients to demonstrate acid-fast
bacilli in skin smears.
9. Lepromin test: It is useful for classification
of leprosy and is strongly positive in
tuberculoid leprosy and mildly positive in
borderline tuberculoid leprosy. It is negative
in borderline borderline, borderline
lepromatous and lepromatous leprosy.
10. Dermatoscopy (Epiluminescence microscopy, dermoscopy): Method of observing
superficial layers of skin using 10-100 X
magnification with oil immersion. Both
hand held and computer assisted
instruments are available. It is used for
differential diagnosis of pigmented skin
lesions, melanoma, for detailed
examination of nail fold capillaries,
Wickhams striae, scabies burrows (hang
glider sign), surface of verrucae and the
scalp surface (cadaver hairs and
exclamation point hairs suggest alopecia
areata, loss of follicular openings indicates
scarring alopecia and follicular
hyperkeratosis point towards lichen
planus).
11. Biopsy: Most frequently skin biopsy is taken
to confirm a clinical diagnosis or to aid in
the establishment of a diagnosis where
clinical diagnosis is not apparent.

29

30

Essentials in Dermatology
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.

VDRL
HIV antibody detection test
Culture and sensitivity test
Patch testing
Prick testing
Intradermal testing
LE cell phenomenon
Mouse foot pad inoculation
Immunofluorescence
Hair examination and counts
Trichogram- method for analyzing hair
bulbs to identify in what stage hairs are
being lost and thus to distinguish between
different types of hair loss.
23. Electron microscopy

Five Thoughts for the Students in the


Field Of Dermatology
1. Diagnosis is the art of recognition, not the
science of cognition
2. The best diagnosticians are the ones with the
best visual memories
3. The best history is taken by one who already
knows the diagnosis
4. If puzzled, limit yourself to three working
diagnoses.
5. A good colour atlas (a memory of 75 diseases
allows you to immediately recognize 95% of
all the skin lesions you will ever see) and a
good dermatopathologist are your best
friends.

Bacterial Infections

Bacterial Infections

Normal human skin is colonized soon after birth


by a large number of bacteria that live as
commensal on the epidermis and epidermal
appendages. Coagulase negative staphylococci
(S. epidermidis) are inoculated during vaginal
passage; coryneform bacteria take up residence
on neonatal skin shortly after birth; and within
several weeks after birth, the flora of neonatal
skin is similar to that of adults. Staphylococcus
aureus is persistent member of the microbial
flora in 10 to 20% of the population. As many as
84% of healthy individuals have occasional
carriage of S. aureus in their anterior nares.

PYODERMAS
Pyoderma is a common purulent infection of the
skin caused by staphylococcal or streptococcal
organisms. They can be classified as primary
pyodermas and secondary pyodermas (Table 6.1).

Primary pyodermas are further divided into


non-follicular and follicular for clinical
application (Table 6.2) and on the basis of
organism involved (Table 6.3).

Staphylococcal Pyodermas

Impetigo
Non-bullous impetigo (Impetigo contagiosa of
Tilbury Fox):
It is caused by S. aureus or group A
Streptococcus or both
Occurs in children of all ages, common
in preschool and young school children
Commonly over the face (especially
around nares) or extremities after trauma
The initial lesion is a transient vesicle or
pustule that quickly evolves into a honey
coloured crusted plaque (Fig. 6.1)
Surrounding erythema may be present

Table 6.1: Distinctive features of pyodermas

Primary pyodermas
1. Invasion of normal skin by bacteria
2. Single species of bacteria involved
3. Appearance of lesions is characteristic
e.g., impetigo, erysipelas, furuncle
4. Treatment is clear cut Drugs aimed
at the microorganism

Secondary pyodermas
1. Develop in areas of already damaged/compromised skin
2. Mixture of organisms involved
3. Not characteristic
4. Role of antibacterial treatment less defined.
Here, the aim is to treat the underlying process

31

32

Essentials in Dermatology
Table 6.2: Non-follicular and follicular pyodermas
A. Non follicular pyodermas include
1. Impetigo
2. Ecthyma
3. Cellulitis
4. Erysipelas
5. SSSS
B. Follicular pyodermas include
1. Folliculitis
2. Furuncle
3. Carbuncle

Regional lymphadenopathy in up to 90%


of patients with prolonged untreated
infection
In severe cases, there may be fever,
adenitis and constitutional symptoms
Differential diagnosis: 1.Tinea corporis
has dry, scaly, advancing edge with
central clearing. 2. Ecthyma- characterized by crusted ulcers (not erosions).

Bullous Impetigo
Table 6.3: Cutaneous diseases caused by
staphylococci and streptococci
1. Cutaneous diseases caused by
staphylococcus include:
A. Direct infection of skin:
Impetigo, ecthyma, folliculitis, furunculosis,
carbuncle, sycosis.
B. Due to effect of bacterial toxin:SSSS, TSS.
2. Cutaneous diseases caused by streptococcus:
A. Direct infection of skin:
Impetigo, ecthyma, erysipelas, cellulitis,
vulvovaginitis, perianal infection, blistering
dactylitis, necrotizing fasciitis.
B. Due to effect of bacterial toxin:
Scarlet fever, toxic shock like syndrome

Fig. 6.1: Impetigo contagiosahoney colored


crusted lesions over the face of a child

Earlier bullous impetigo in neonates was


popularly known as pemphigus
neonatorum.
S. aureus (phage group II) is the causative
agent.
Occurs commonly in the newborn and in
older infants.
Bullae (flaccid) rapidly evolve from
vesicles on areas of grossly normal skin
(Fig. 6.2) due to local production of
exfoliative toxin A and B.
Differential diagnosis: Pemphigus
vulgaris-Generally occurs in young
adults. Erosions show no tendency to
heal, Nikolskys sign and bulla spread
sign are positive. More importantly,
mucosal erosions are more commonly
associated feature.

Fig. 6.2: Bullous impetigolarge pus filled blisters


over the trunk of a child

Bacterial Infections
Complications of Impetigo
Post-streptococcal acute glomerulonephritis
S. pyogenes type M-49
Scarlet fever
Urticaria
Erythema multiforme
Rheumatic fever in not a complication of
streptococcal skin infection (but of streptococcal
sore throat).

Ecthyma
Consequence of neglected impetigo
S. aureus and/ or group A Streptococcus
are causative agents
Most commonly occurs on the lower
extremities of children or neglected
elderly patients
Poor hygiene and neglect are key
elements in pathogenesis.
Dirty grayish-yellow crust surmounts a
punched out ulcer (Fig. 6.3).

Fig. 6.3: Ecthymacrusted lesion over the leg with


ulcerated lesions

Folliculitis
Pyoderma affecting the hair follicles, classified
according to depth of invasion.
Superficial folliculitis
Also known as follicular or Bockharts
impetigo
A small fragile dome shaped pustule
occurs at the infundibulum of a hair
follicle, often on scalps of children and in
the beard area, axilla, extremities and
buttocks of adults.
Differential diagnois: Miliaria pustulosa
non follicular pustules are wide spread,
which occur in hot and humid conditions
Deep folliculitis
Sycosis barbae is a deep folliculitis with
perifollicular inflammation occurring in the
bearded areas of the face (Fig. 6.4) and upper
lip.
Differential diagnosis
1. Pseudofolliculitis Papules/pustules are
irregularly scattered at the site of ingrowing
beard hairs. Neck and angle of jaw (vs.
sycosis barbae upper lip and below angles
of jaw) are preferentially involved.
2. Tinea barbae Site involved is usually
submaxillary region or the chin. Hairs are

Fig. 6.4: Sycosis barbae grouped follicular based


crusted lesions in the beard area of the face

33

34

Essentials in Dermatology
broken or loosened (easy and painless
pluckablity) in the affected area. Loss of hair
is the norm. Suppurative or granulomatous
nodules rather than pustules characterize this
condition. Spores and hyphae can be demonstrated in the hair by KOH examination.
3. Herpetic sycosis is usually limited for a few
days and invariably shows vesicles even in
persistent lesions
4. Acne vulgaris is polymorphous condition
mainly of glabrous skin of the face where
comedones are the hallmark of that condition
Lupoid sycosis is deep, chronic form of
sycosis barbae associated with scarring usually
occurring as circinate lesion. Pustules and
papules surround a central scar (Fig. 6.5).
Differential diagnosis: Lupus vulgaris is
characterized by areas of scarring on one side
and progression on the other side. There is
absence of pustules in the lesions but it
demonstrates apple jelly nodules on diascopy.

Furuncles and Carbuncles


A furuncle or boil is a deep-seated
inflammatory nodule that develops about a
hair follicle, often evolving into an abscess
(Fig. 6.6). They arise in hair bearing areas,

Fig. 6.5: Lupoid sycosisscarring alopecia in the


beard area showing active pustular lesions at the
periphery

particularly in regions subject to friction,


occlusion, and perspiration.
Differential diagnosis: FolliculitisInflammatory change is confined within the
follicle without any surrounding inflammation hence presents as a pustule whereas
furuncle presents as a nodule. There is less
pain and it heals without scar formation.
Cystic acne- Associated with other lesions of
acne comedones, papules and pustules and
acne scars and it is confined to face and trunk.
A carbuncle is a cluster of furuncles, more
extensive, deeper, communicating, in
filtrated lesion that develops when
suppuration occurs in elastic skin. It usually
involves the nape of the neck, back or thighs
and usually occurs in the setting of underlying diabetes mellitus, alcoholism, malnutrition, blood dyscrasias, iatrogenic or other
immunosuppression including HIV
infection. Fever, malaise, prostration
accompany.
Differential diagnosis: Anthrax characterized by painless, hemorrhagic crusted
(blackish eschar) lesion with surrounding
gelatinous edema out of proportion to the
extent of the lesion.

Fig. 6.6: Furunclea red tender suppurated nodule


in a case of erythroderma

Bacterial Infections
compromise. Typically, the patient has fever
and is irritable. The changes usually begin
periorificially or in body folds (Figs 6.7 and
6.8). Then they spread rapidly. Nikolskys
sign is positive even on apparently normal
skin. One should culture the perineum, eyes,
ears, nose and throat, looking for S. aureus as
the focus of infection is located at a distant
site. The bacteria can not be cultured from
the skin. For differential diagnosis Table 6.4.

Staphylococcal paronychia: Clinically, skin and soft


tissue of proximal and lateral nail fold are red,
hot and tender, and if not treated, can evolve to
abscess formation. In contrast, chronic or
recurrent paronychia caused by Candida albicans
is an infection of the space underneath the nail
folds.

Toxin Mediated Syndromes


Staphylococcal scalded skin syndrome
(SSSS) (Ritters disease)
This is the most severe form of skin disease
due to the exfoliative exotoxins (A and B)
produced by S. aureus of group II, phage type
71 or 55 and is characterized by generalized
bulla formation and exfoliation. Most
commonly involves neonates and young
children, but also in adults with renal

Toxic Shock Syndrome


TSS is a multiorgan systemic illness due to
exotoxin (TSS-Toxin 1) producing strains of
S. aureus.
Case definition
1. Temperature of 38.9oC or higher
2. Erythematous eruption

Fig. 6.7

Fig. 6.8

Figs 6.7 and 6.8: SSSStypical periorificial and body fold involvement with peeling skin

Table 6.4: Differential diagnosis of SSSS from toxic epidermal necrolysis (TEN)

Staphylococcal scalded skin syndrome (SSSS)


1.
2.
3.
4.
5.
6.

Age less than 5 years


Skin shows marked tenderness
Distribution face, neck , axilla, groin
Mucoca not involved
Prognosis good
Histology subgranular split due to acantholysis

TEN
1.
2.
3.
4.
5.
6.

More than 40 years


Mild to moderate tenderness
No clear distribution
Involved
Poor
Necrosis of epidermis

35

36

Essentials in Dermatology
3. Desquamation of palms and soles 1 to 2
weeks after onset
4. Hypotension
5. Involvement of 3 or more other organ
systems
About 85 to 90 percent of cases of TSS have
occurred in women at the time of menstruation; almost all had been tampon users
(particularly of super absorbent types).
Differential diagnosis:
1. SSSS It has the presence of bullae,
Nikolskys sign is positive with skin
tenderness, but systemic organs are not
involved and patient appears wellpreserved.
2. Scarlet fever not usually associated with
hypotension and shock.
3. Kawasakis syndrome prolonged fever,
cardiac involvement, generalized
lymphadenopathy and absence of
peripheral shock.

of subcutaneous tissue whereas erysipelas is


a bacterial infection of the dermis and upper
subcutaneous tissue.
Erythema, warmth, swelling and tenderness
are constant features (Fig. 6.9). In erysipelas,
the edge of the lesion is well defined and
raised, but in cellulitis it is diffuse.
In erysipelas, blistering is common.
Except in mild cases, there are constitutional
symptoms.
The leg is the commonest site; the next most
frequent site for classical streptococcal
erysipelas is face.
Milians sign cellulitis of the face does not
involve pinna, unlike erysipelas (as there is
no subcutaneous tissue there).
Without effective treatment, complications
are common-fasciitis, myositis, subcutaneous
abscesses, septicaemia, and nephritis.
For presumed streptococcal infection,
penicillin is the treatment of choice.

Streptococcal Pyodermas
The major pathogen belongs to group A and is
referred to as Streptococcus pyogenes. Streptococci
colonise damaged skin, although less frequently
than staphylococci. Major complications
following streptococcal infection are rheumatic
fever (following Streptococcal pyogenes
pharyngitis), acute glomerulonephritis (both
throat and skin infection), erythema nodosum
and guttate psoriasis, and scleredema of Buschke
(following throat infection).
Impetigo and Ecthyma already discussed.
Crowding, poor hygiene, and neglected minor
skin trauma contribute to the spread of
streptococcal impetigo in families.

Cellulitis and Erysipelas


Predominantly streptococcal disease,
Staphylococcus aureus is occasionally
implicated.
Cellulitis is an acute, subacute or chronic
infection of loose connective tissue, mainly

Fig. 6.9: Cellulitislower leg showing shiny


erythema and edema

Bacterial Infections

Blistering Dactylitis
This is nearly always a group A streptococcal
infection in children or teenagers.
A large blister containing thin seropurulent
fluid forms on the distal phalanx, usually of
a finger, typically on a phalangeal pad.

Perianal Streptococcal Infection


It occurs in children aged 1-10 years and is
characterized by intense perianal erythema,
perianal soreness, pain on defecation, faecal
retention and blood-streaked stools.

Streptococcal Vulvovaginitis
Streptococcus pyogenes accounts for 10% of
cases of vulvovaginitis in prepubertal girls.

Toxin Mediated Streptococcal Disease


Scarlet fever and streptococcal toxic shock
like syndrome are due to toxins.
Scarlet fever is a diffuse erythematous
eruption resulting from the production and
subsequent circulation of pyrogenic
exotoxins A, B, C (erythrogenic toxin)
produced by group A streptococci usually
located in pharynx. Incubation period 2 to
5 days starts with an acute tonsillitis. Rash
appear on the 2nd day as finely punctuate
erythema Sunburn with goose-pimples.
Other features include Pastias lines
(transverse streaks in the skin folds due to
capillary fragility), pallor around mouth, red
strawberry tongue, and high fever.
Myocarditis may complicate this condition.
Group A streptococci cause an acute
multisystemic syndrome coined toxic-shock
like syndrome (TSLS) resembling that
caused by S. aureus.

It is not only caused by group A streptococci


but also due to other bacterial species
(mixture of anaerobes/facultative organisms).

Diagnosis
1. Grams stained smear from the purulent
material may demonstrate streptococci or
staphylococci or both.
2. Culture and sensitivity: Swabs taken from
infected sites may be sent for culture and
sensitivity, so that appropriate antibiotic may
be instituted to treat the condition.
3. Tzanck smear in SSSS shows acantholytic
cells.
4. Histopathology: Histopathological examination is hardly required for the diagnosis.
Treatment
1. General measures such as improved
hygiene, loose light weight porous clothing,
regular bathing, use of antiseptics in bath,
antibacterial soaps, etc.

Necrotizing Fasciitis (Streptococcal


gangrene)
It represents cellulitis that has progressed to
gangrene of subcutaneous tissue followed by
necrosis of overlying skin (Fig. 6.10).

Fig. 6.10: Necrotizing fascitiscellulitis progressing


to gangrene over the leg

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Essentials in Dermatology
2. Wet compresses: Condys compresses for
crusted lesions of pyoderma.
3. Incision and drainage is indicated when
furuncle has become localized and shows
definite fluctuation.
4. Topical antibacterial agents such as gentian
violet, neomycin, fusidic acid or mupirocin.
5. Systemic antibacterial agents: Systemic
antibiotic therapy is indicated
i. For extensive lesions of pyoderma
ii. For erysipelas, cellulitis, carbuncle,
furunculosis, SSSS, etc.
Penicillin is preferred for streptococcal
infection whereas penicillinase resistant
penicillin such as cloxacillin and cephalosporins
are required for staphylococcal infections. Oral
antibiotics (e.g., rifampicin 600 mg orally daily
for 10 days) have been effective in eradicating S.
aureus from most nasal carriers for periods of up
to 12 weeks in cases having recurrent
furunculosis. Intranasl application of 2%
mupirocin ointment for 5 days can eliminate S.
aureus nasal carriage in 70% of healthy
individuals for up to 3 months.

NON-PYODERMAS
Erythrasma
Causative agent-Corynebacterium minutissimum, diphtheroids bacillus, gram positive,
non spore forming rod shaped organism.
Bacterial infection of the intertriginous areas
like axilla, groin, gluteal cleft, inframammary
folds, umbilicus and toe web spaces.
Usually manifest with asymptomatic red
brown macules with sharp border (Fig. 6.11).
The best way to make the diagnosis is Woods
light examination for coral red fluorescence.
Differential diagnosis: Hyperpigmented
tinea versicolor (asymptomatic in nature)
may appear like erythrasma. It predominantly affects upper trunk, individual
lesions are small, but not erythematous and
satellite lesions are more commonly seen than

Fig. 6.11: Erythrasmaasymptomatic brownish


macular lesion with fine scaling in the axilla

erythrasma. KOH examination and culture


from the lesions may clinch the diagnosis.
Tinea cruris is characterized by pruritic well
defined annular plaques with peripheral rim
of papulopustules, and satellite lesions.
C. minutissimum can be cultured under
aerobic condition
A short course of systemic erythromycin is
the easiest method of treatment. Topical
imidazole creams are also effective.

Trichomycosis Axillaris
Causative agent- Corynebacterium tenuis.
Collections of this bacteria forms concretions
on hairs, usually in the axilla.
The axillary hairs are surrounded by whiteyellow (Fig. 6.12), red or black, difficult to
remove concretions that extend for several
centimetres.
Diagnosis is established by examining hair
under microscope (Fig. 6.13), if necessary to
culture the organism.

Bacterial Infections

Fig. 6.14: Pitted keratolysissoles demonstrating


multiple pits

Fig. 6.12: Trichomycosis axillarisyellow


discoloration of axillary hairs due to concretions

Fig. 6.13: Trichomycosis axillarissame patient's


axillary hair under light microscope showing
concretions over the hair shaft

Differential diagnosis: Phthiriasis pubis can


be differentiated by its associated pruritis and
crawling sensation. Piedra can be
differentiated by its gritty hard feeling and
by doing a KOH examination.
The easiest treatment is to shave the area and
treat the regrowing hairs with any topical
disinfectant.

Pitted Keratolysis
Causative agent- Corynebacterium species,
Streptomyces species, Dermatophilus congolensis and Micrococcus sedentarius.
Multiple pitted defects, 2-5 mm in size occur
in thick horny layer of soles (Fig. 6.14).
The key factor is maceration, usually arising
from hyperhidrosis, prolonged wearing of
shoes and improper hygiene.
Differential diagnosis: The lesions are easily
recognizable, but simple hyperhidrosis,
erythrasma and tinea pedis have to be
considered.
Topical erythromycin solution or benzoyl
peroxide gel can be applied once or twice
daily. Remove aggravating factors, if
possible.
Botryomycosis
Botryomycosis is a chronic suppurative,
granulomatous disorder of bacterial origin.
True non- filamentous aerobic and anaerobic
bacteria such as Staphylococcus aureus,
Pseudomonas species, Proteus vulgaris,
Escherichia coli or Micrococcus cause it.
Botryomycosis needs to be differentiated
from two other granulomatous diseases that

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Essentials in Dermatology

Fig. 6.15: Botryomycosisswelling of the foot with


multiple nodules over it

form granules - mycetoma and actinomycosis, since clinically it has similar features
(Fig. 6.15).
Effective treatment of botryomycosis
depends on various factors such as the
causative agent, location of the lesion and
immune status of the host. Various drugs,
mostly as single agents given for several
weeks, have been successfully used in
botryomycosis including trimethoprimsulfamethoxazole, minocycline, erythromycin and cefazolin. In addition to
antibiotics, surgical excision and drainage of
lesions may be useful in certain patients.

Actinomycosis
Actinomycosis is a chronic suppurative
infection caused by anaerobic Actinomyces
species. Actinomycetes are bacteria producing
filamentous and branching hyphae.
Pathogenic organisms of these genera,
namely Actinomyces israelii exists as a
commensal in the oral cavity, tonsillar crypts
and genital mucosa.
This organism gains entry when there is a
disruption of mucosal barrier in the form of
trauma or surgery. The resultant disease,
actinomycosis is characterized by an early

inflammatory phase which resembles


cellulitis and a more typical chronic phase,
which presents as single or multiple
indurated swellings (usually fibrosis). These
swellings become soft and fluctuant and later
suppurate, sometimes forming sinus tracts
discharging yellow colour granules. These
so called sulphur granules are lobulated
masses of intertwining filaments.
Human infections are categorized based on
anatomical sites, namely cervicofacial (lumpy
jaw), thoracic, abdominal, pelvic and primary
cutaneous.
Cervicofacial actinomycosis is the most
common clinical presentation. It commonly
follows dental extraction and presents as
painful, indurated soft tissue swelling located
at the angle of the jaw.
Thoracic infection occurs due to aspiration
and it involves lungs and pleura.
Actinomycosis of gastrointestinal tract most
commonly develops in ileocecal region and
presents as appendicitis or slow growing
mass.
Pelvic actinomycosis occurs in women and
is usually associated with the use of
intrauterine device.
Primary cutaneous actinomycosis is a rare
type and probably occurs due to direct
implantation of the organism. It usually
occurs on the exposed skin (Fig. 6.16).

Fig. 6.16: Actinomycosisscalp showing ulcerated


indurated nodules with sulphur granules

Bacterial Infections

Cutaneous Anthrax
Causative agent- Bacillus anthracis, gram
positive bacillus,
The most common form of infection with
Bacillus anthracis is an acute cutaneous lesion
called malignant pustule.
Anthrax is primarily a disease of domestic
and wild animals, but humans become
accidentally involved through exposure to
animals and their products.
Human anthrax occurs in three clinical forms:
1) cutaneous anthrax due to direct contact

with contaminated meat, carcasses, hides,


hair, wool or bone, 2) inhalational anthrax
(Woolsorters disease) due to inhalation of
spores and 3) gastrointestinal anthrax due to
ingestion of contaminated meat or milk.
Anthrax meningitis occurs secondary to skin
lesions, but it can complicate the other two
forms also.
Cutaneous anthrax usually begins as a
painless, pruritic papule within 3 to 10 days
of inoculation. It rapidly progresses into a
serous or serosanguineous vesicles, which
ulcerate with a central black eschar,
surrounded by a ring of vesicles within 36
hours (Figs 6.17 and 6.18). Perilesional
oedema can be extensive. Toxic features
occur in 50% of cases only and healing occurs
in 1 to 3 weeks with variable scarring.
The following clinical features are strongly
suggestive of cutaneous anthrax 1. The presence of edema out of proportion
to the size of the lesion.
2. Lack of pain during the initial phases of
the infection.
3. The rarity of polymorphonuclear
leucocytes from vesicular fluid on grams
stain and distinctive box car arrangement
of bacillus anthracis (Fig. 6.19).

Fig. 6.17

Fig. 6.18

A definite diagnosis of actinomycosis cannot


made be on clinical grounds alone.
Demonstration of sulphur granules, grams
stain and culture from the appropriately
obtained specimen is needed to confirm the
diagnosis. Histopathology reveals granular
colonies from which delicate mycelial
filaments radiate. These colonies are
surrounded by a chronic inflammatory
infiltrate.
Actinomycosis may resemble various chronic
inflammatory diseases such as tuberculosis,
syphilis, etc.
Dramatic response to penicillin therapy
occurs.

Figs 6.17 and 6.18: Cutaneous anthrax (Malignant pustule)typical painless lesion with central blackish
eschar surrounded by wreath of erythema, edema and vesiculation

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Essentials in Dermatology
Ciprofloxacin, erythromycin, tetracycline
and chloramphenicol are alternative drugs
for penicillin sensitive patients.

Fig. 6.19: Anthrax"Box car" arrangement of


bacillus anthracis in gram's stained smear

Differential diagnosis: Carbuncle Tenderness is prominent and there is


presence of multiple furuncles in a group.
Cow pox and sporotrichosis are other
differential diagnoses. The history, rapid
course, clinical appearance and lack of
lymphangitis should suggest the diagnosis
of anthrax which should be confirmed by
bacteriological examination.
Since 20% of untreated cases of cutaneous
anthrax develop bacteraemia, which leads to
rapid death, cutaneous anthrax should be
treated energetically with penicillin.

Bacillary Angiomatosis
Causative agent: Bartonella henselae (organism
also causes Cat Scratch disease); rarely
Bartonella quintana.
Infection is most common in HIV/AIDS
patients, causes endothelial proliferation and
produces vascular tumors.
Clinically, lesions are rapidly growing
pyogenic granuloma like papules and
nodules, which often ulcerate.
Purple, papular and nodular vascular lesions
may resemble Kaposis sarcoma.
Diagnosis is based on demonstration of
organism in the skin biopsy tissue section by
Warthin Starry staining. Blood cultures are
positive in half of the cases.
Differential diagnosis: Pyogenic granuloma
and Kaposi sarcoma are close differential
diagnosis, differentiation can be made by
histopathological examination and
demonstration of the organism by Warthin
starry staining.
The mainstay of treatment is erythromycin.
Alternatively, doxycycline or ciprofloxacin
can be used.

Viral Infections

Viral Infections

Many viral infections have cutaneous


manifestations. These infections may sometimes
be limited to the skin as in warts or molluscum
contagiosum. In systemic viral infections, the
frequency of skin lesions may range from almost
always, as in varicella, to infrequently, as in
cytomegalovirus.
Viral infections of skin are characterized by
a definite morphology and distribution. Most of
the infections can be clinically diagnosed fairly
and accurately without the need of sophisticated
laboratory diagnostic aids. Five groups of viruses
can affect the skin or adjoining mucous
membrane surfaces. All except two (picorna
virus group and retroviruses group) belong to
DNA viruses.
Viruses can be seen with the light microscope
only when aggregated into inclusion bodies.
Inclusion bodies are roughly spherical. Their
average size is about 7 m, the size of an
erythrocyte. They are seen in three groups of
viruses and are of two types.
1. Intranuclear inclusion bodies are seen in the
herpes virus group and papilloma virus
group.
2. Intracytoplasmic inclusion bodies are seen
in the poxvirus group.
In India, molluscum contagiosum is the
predominant viral skin infection followed by

warts, herpes simplex, herpes zoster and


chicken pox.

MOLLUSCUM CONTAGIOSUM
Molluscum contagiosum is caused by up to
four closely related types of poxviruses,
MCV-1 to - 4 and their variants.
The incubation period for the molluscum
contagiosum (MC) has been reported to be
between 14 and 50 days.
Skin to skin transmission is presumed to be
the method of spread, including autoinoculation (the Koebner phenomenon), as
well as contact with fomites.
It mainly affects children, sexually active
adults and persons with impaired cellular
immunity including HIV infection
In small children, virtually all infections are
caused by MCV-1 whereas in patients
infected with HIV, however, MCV-2 causes
the majority of infections (60%), suggesting
that HIV infection associated molluscum
does not represent recrudescence of childhood molluscum. In all forms of infection, the
lesions are relatively similar.
Individual lesions are smooth surfaced,
firm, dome shaped pearly papules
averaging 3 to 5 mm in diameter. Some

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Essentials in Dermatology

Fig. 7.1: Molluscum contagiosumtypical


umbilicated papule

Fig. 7.2: Molluscum contagiosumlesions


occurring over the face in a child

Fig. 7.3: Molluscum contagiosumlesions


occurring over genitalia in a child

giant lesions may be up to 1.5 cm in


diameter.
A central umbilication is characteristic (Fig.
7.1)
Children more typically develop lesions on
the face (Fig. 7.2), trunk and extremities,
although perineal, scrotal (Fig. 7.3), perianal
(Fig. 7.4), and groin lesions may be present
as part of a wider distribution.
Clinically, molluscum in HIV positive
persons appears to be transmitted both by
sexual and nonsexual routes. The lesions are

Fig. 7.4: Molluscum contagiosumlesions over the


gluteal area and thigh in a child

more common on the face and neck (Figs 7.5


and 7.6).

Diagnosis
It is made clinically when necessary,
histological examination is diagnostic.

Viral Infections

Fig. 7.5: Molluscum contagiosumlesions around


eye in a HIV patient

Fig. 7.6: Giant molluscum contagiosum just below


the eye in an adult HIV patient

Microscopic examination of a lesion crushed


on a slide and left stained or unstained with
Wrights, Giemsa, Grams, or Papanicolaou
stains demonstrates the inclusion bodies
(Henderson Paterson bodies).

Oral cimetidine or intravenous cidofovir can


be used in widespread lesions.

Differential Diagnosis
Solitary MC may resemble pyogenic
granuloma, keratoacanthoma or epithelioma.
Cutaneous cryptococcos is histoplasmosis,
Penicillium marneffei infection, and basal cell
carcinoma have been mistaken for
molluscum in patients with AIDS.

Human papillomavirus (HPV) has been


associated with a broad spectrum of disease
that ranges from asymptomatic latent
infection to warts to squamous cell
carcinoma.
This virus infects mucosal and cutaneous
sites of immunocompetent as well as
immunosuppressed patients.
It represents the most common mucocutaneous viral infection, and 3 to 5% of all
patients have clinically evident warts.
Warts, or verrucae, are benign proliferations
of the skin and mucosa that result from
infection with papillomavirus.
These viruses do not produce acute signs or
symptoms but induce slow-growing lesions
that can remain subclinical for long periods
of time.
These warts assume many clinical forms
common warts (verrucae vulgaris), filiform
warts (digitate warts), flat warts (verrucae
plana), plantar warts, genital warts
(condylomata acuminata), oral and laryngeal

Treatment
In children, they may be left alone
Curettage Individual lesions can be treated
with curettage
Trichloroacetic acid (30-100%) application
10% KOH application
Podophyllin 10% to 25%
Silver nitrate
5-FU topically
Cryosurgery
Electrodesiccation
Topical cantharidin, tretinoin (0.05% - 0.1%),
cidofovir (1-3%), and imiquimod (5%) are
other options

HUMAN PAPILLOMAVIRUS INFECTION


(WARTS)

45

Essentials in Dermatology

46

papillomas and epidermodysplasia verruciformis. HPV types 1,2,3,4,7 cause first four
forms of warts commonly. Genital warts are
usually due to HPV types 6, 11, 16 and 18.
Warts occur at any age, but are unusual in
infancy and early childhood, the incidence
of warts increases during the school years.
Incubation period has been estimated to
range between a few weeks to more than 1
year.
Warts are spread by direct or indirect
contact.
Impairment of epithelial barrier function by
moisture or trauma is predisposing factor.

Paring shows multiple bleeding points or


black dots representing thrombosed
capillaries in warts.
Corns have a hard, painful translucent
central core.
Lateral pressure on a wart causes pain, but
corn is painful on vertical pressure.
Plane warts: They are due mainly to HPV 3 and
10, and are smooth, flat or slightly elevated,
skin colored or pigmented. The face (Fig. 7.8)
and the backs of hand and shin are the sites of
predilection. In differential diagnosis, lichen

Common warts: They are due mainly to HPV-2


and are characterized by symptomless, firm
papules with a rough, horny surface, range in
size from less than 1 mm to over 1 cm in
diameter, and by confluence can form large
masses. They are most commonly situated on the
backs of hands and fingers (Fig. 7.7). They need
to be differentiated from calluses. Warts do not
have dermatoglyphics as opposed to calluses in
which these lines are accentuated.
Plantar warts: A plantar wart at first appears as
a small, shining, sago grain papule, but soon
assumes the typical appearance of a sharply
defined, rounded lesion, with a rough keratotic
surface surrounded by a smooth collar of
thickened horn. Most plantar warts are beneath
pressure points, the heel or the metatarsal heads.
Mosaic warts are so described from the
appearance presented by a plaque of closely
grouped warts. Pain is a common, but variable
symptom.

Differential diagnosis is corn/s.


On paring, epidermal ridges are seen to
continue without interruption in corns,
whereas in warts epidermal ridges are
interrupted on the surface of the wart.

Fig. 7.7: Common wartsrough surfaced keratotic


papules over the dorsa of hand

Fig. 7.8: Plane wartssmooth surfaced papules


over the face

Viral Infections
planus causes most difficulty. Lichen planus
favours the flexor aspects of the forearms, is
unusual on the face and is often itchy. The
mucous membranes may be involved. The flat
polygonal papules are lilac-pink and smooth and
may show Wickhams striae.
Filiform and digitate warts: Occur commonly
in the male, on the face and neck. They are
irregularly distributed, often clustered. Digitate
warts often in small groups also occur on the
scalp in both sexes where they are occasionally
confused with epidermal naevi.
Anogenital warts: It has been estimated that up
to 30 to 50% of sexually active adults are infected
with HPV, making it the most common viral STD
in some STD clinics. Most of them occur on the
penis, scrotum, urethral meatus and perianal
area in men and on the introitus, vulva,
perineum and perianal area in women. The four
morphological types are
1. Cauliflower like (condyloma acuminata)
(Fig. 7.9)
2. Papular
3. Keratotic
4. Flat topped.
Bowenoid papulosis: is a clinicopathologic
entity in which HPVs (HPV-16 is the most

Fig. 7.9: Genital wartscauliflower like lesion of


condyloma acuminata over the glans penis along with
herpes genitalis

common that has been linked to it) have been


identified. These appear as 2 to 3 mm papules
often multiple over the external genitalia.
Histologically, there is cellular atypia resembling
Bowens disease.
Buschke Lowenstein tumor (giant condyloma):
This lesion emerges from a preexisting benign
anogenital wart before developing into a
verrucous carcinoma. Usually, it is associated
with type 6 and 11. Clinically, it resembles a
large aggregate of condyloma acuminata over
the glans penis (Fig. 7.10), foreskin, vulva or anal
region.
Epidermodysplasia verruciformis: Usually
manifests in childhood with widespread warts.
It is an inherited disorder in which there is wide
spread and persistent infection with HPV giving
rise to a combination of plane warts, pityriasis
versicolor like lesions and reddish plaques
(Figs 7.11 and 7.12).
Multiple warts that do not resolve
spontaneously, recur after treatment, persist for
years, and have an unusual morphology may
suggest epidermodysplasia verruciformis.
Malignant change is very common but
metastasis is rare.

Fig. 7.10: Buschke Lowenstein tumora large


verrucous growth seen involving the glans penis

47

48

Essentials in Dermatology

Fig. 7.11: Epidermodysplasia verruciformis


pityriasis versicolor like lesions over the forearm

Fig. 7.12: Epidermodysplasia verruciformisverruca


vulgaris like lesions aggregating over the dorsa of the
foot

Diagnosis
Clinical diagnosis of warts is often sufficient
but atypical, subclinical or dysplastic lesions
may need laboratory confirmation of HPV
infection.
Other than histopathology of lesion
(Koilocytosis in the stratum granulosum and
stratum spinosum), detection of virus
particles by electron microscopy,
immunocytochemistry using type specific
antibodies, DNA hybridization on tissue
extracts and polymerase chain reaction may
be done.

Electrosurgery: Electrodesiccation or
electrofulguration may be done.
CO2 laser ablation.
Cryotherapy: Either using liquid nitrogen
using dipstick method or spray method, or
nitrous oxide cooled probes.
Keratolytic agents like 10 to 20% salicylic
acid topically may be used.
Formalin soaks with 2 to 3% formalin in
water every day for 15 minutes is useful for
plantar warts.
Curettage or surgical excision for warts
unresponsive to topical treatment.
Topical retinoic acid for flat warts.
Cantharidin causes blistering and focal
destruction of the epidermis.
Cidofovir and 5-FU topically.
Intralesional bleomycin.
Injection of 0.1 ml of 1:1000 candida antigen
in the base of each wart.
Topical sensitizer and systemic immunomodulators (Interferon, especially interferon

Treatment
Most common warts disappear spontaneously
within 2 year or with simple nonscarring
treatment. Commonly employed modalities of
treatment are:
Chemical cautery: Trichloroacetic acid is
used for common warts, filiform warts and
plantar warts.

Viral Infections
alpha intralesionally or intramuscularly) may
be employed depending upon the condition.
Extensive warts, even in hitherto untreatable
epidermodysplasia verruciformis, have
improved or cleared with oral isotretinoin or
etretinate.
Treatment of genital wart is discussed
separately in STD section but salient
treatment options are given below.
1. Podophyllotoxin 0.5% self administered
twice a day for 3 days in week for 4 weeks
or podophyllin 25% in tincture benzoin,
once or twice weekly.
2. Liquid nitrogen cryosurgery
3. Electrosurgery
4. Surgical excision
5. Chemical cautery
6. Topical imiquimod.

HERPES SIMPLEX INFECTION


The word herpes is derived from the Greek,
meaning, to creep.
The human herpes simplex virus consists of
two closely related viruses designated herpes
simplex virus type 1 (HSV-1) and herpes
simplex virus type 2 (HSV-2). The virus
causes a wide variety of mucocutaneous
infections and produce both primary and
recurrent infections.
HSV -1 is generally associated with oral
infection, and HSV-2 is associated with
genital infection. However, HSV-1 genital
infection and HSV-2 oral infection are
becoming common, as a result of oral-genital
sexual contact and as most these infections
are asymptomatic
Transmission of HSV infections most
frequently occurs through close contact with
a person who is shedding virus at a
peripheral site, mucosal surface, in genital or
oral secretions.
Neonates often have maternally acquired
HSV-1 antibodies, which decline to low levels
by 6 months of age.

Fig. 7.13: Primary herpetic gingivostomatitislips


also involved along with oral cavity with vesiculation,
erosions and hemorrhagic crusting

Primary herpetic infection of the mouth and


pharynx (Herpetic gingivostomatitis) is a
disease of children (Fig. 7.13) and young
adults. The peak years of incidence occur
between age 1 and 5. The usual onset is with
fever, sore throat, painful vesicles and
ulcerative erosions.
The clinical manifestations of genital herpes
should be differentiated into first clinical
episode and recurrent episodes. The first
clinical episode can further be sub-divided
into primary infection, occurring in a person
without prior HSV 1 or 2 antibodies (true
primary) or nonprimary infection, occurring
in a person with prior HSV 1 or 2 antibodies.
The patients with first episode non primary
have lower frequencies of systemic
symptoms, shorter duration of pain, fewer
lesions and a shorter healing time compared
with the true primary infections.
Primary genital HSV-2 and HSV-1
infections (Herpes genitalis) are
characterized by frequent and prolonged
systemic and local symptoms. Fever,
headache, malaise and myalgia are reported
in 40% of men and 70% of women with
primary HSV-2 infection. Widely spaced
pustular or ulcerative lesions on the external
genitalia are the most frequent presenting
signs. Ulcerative lesions persist for 4-15 days
until crusting or re-epithelization occurs.

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Essentials in Dermatology

Fig. 7.14: Recurrent herpes labialiserosions seen


over the angle of mouth on one side

Fig. 7.15: Recurrent herpes genitalisclosely


grouped vesicles over the shaft of the penis

Recurrences occur in 30-50% of cases of oral


herpes (Fig. 7.14) but are more frequent after
genital herpes infection (Fig. 7.15),
developing in 95% of those with type 2 HSV
compared with 50% in type 1 infection. They
are usually triggered by fever, respiratory
tract infection, ultraviolet radiation,
trigeminal neuralgia, dental surgery or
dermabrasion. Recurrent infections differ
from primary infection in the smaller size of
the vesicles, their close grouping and absence
of systemic symptoms.
Herpes simplex virus type 2 is now the most
common cause of genital ulceration among
the sexually transmitted diseases.
Complications seen are eczema herpeticum
or Kaposis varicelliform eruption
(Widespread vesicular HSV lesions that arise
from primary or recrudescent infection in
patients with several dermatoses, particularly
atopic eczema.), neurological sequelae
(Aseptic meningitis, autonomic dysfunction
especially of the sacral dermatomes, rarely,
transverse myelitis, Bells palsy, Guillain
Barre syndrome may follow orolabial HSV
infection), extra genital lesions (extra genital
lesions is a common complication of first

episode primary genital herpes and is seen


over buttock, groin, or thigh, although finger
and eye can also be involved.), and
disseminated infection.
Atypical HSV presentation occur relatively
often in HIV infected patients, particularly
severe lesions occur over back, buttocks or
perianal region and these may expand to over
20 cm in diameter. Chronic HSV-2 ulcers of
more than 1-month duration are an AIDS
defining illness. HSV may also cause life
threatening disseminated infection,
esophagitis, hepatitis and pneumonitis in
AIDS patients.

Fig. 7.16: Herpetic whitlowgrouped vesicles over


the great toe

Viral Infections

Fig. 7.18: Tzanck smear showing multinucleated


giant cell

Diagnosis

Fig. 7.17: Eczema herpeticumwidespread vesicular


eruption over the face in a patient with allergic contact
dermatitis

Clinical Entities Caused by HSV-1


1. Primary herpetic gingivo-stomatitis
2. Herpes labialis
3. Traumatic herpesherpetic whitlow
(Fig. 7.16), herpes gladiatorum
4. Eczema herpeticum (Fig. 7.17)
5. Atypical presentationsZosteriform herpes
simplex, varicella like eruption, neurologic
herpes simplex
6. Ocular herpes simplex (Herpetic
keratoconjunctivitis)
7. CNS involvement
8. Disseminated herpes.

The most reliable way to make a diagnosis is


by viral culture.
A valuable clinical approach for making a
rapid diagnosis is Tzanck smear. Smears of
the bases of deroofed HSV vesicles are
stained with Giemsa or Wrights stain and
examined by light microscopy for multinucleated giant cells (Fig. 7.18).
Primary infections can be diagnosed by
seroconversion or a rise in antibody titer.
Differential Diagnosis
1. For herpes gingivostomatitis- Diphtheria,
thrush, aphthosis, streptococcal infections,
and Stevens Johnson syndrome.
2. For recurrent herpes labialis-Aphthous
ulcers, erythema multiforme, and impetigo.

Treatment
Acyclovir has modified the treatment outcome.

Clinical Entities Caused by HSV-2

Primary Infection

1. Genital herpes
2. Recurrent lumbosacral herpes simplex
3. HSV infections in the newborn.

Herpetic gingivo-stomatitis
Acyclovir 5 mg/kg body weight 8 hourly for
7 to 10 days.

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Essentials in Dermatology
Others like eczema herpeticum and
disseminated herpes need to be treated
energetically on similar lines.
Herpetic whitlow and keratoconjunctivitis
may be treated with oral acyclovir 200 mg 5
times a day for 7 to 10 days.

Recurrent Infection
Acyclovir 200 mg 5 times a day for 5 days or
Acyclovir 400 mg tds 5 days or
Acyclovir 800 mg BD 5 days
Alternative antiviral drugs are famciclovir
(125 mg, 250 mg twice daily) and valacyclovir
(500 mg, 1 gm twice daily).
For suppression of recurrences- Acyclovir
400mg twice a day for at least 1 year.

Treatment of Acyclovir Resistant


Cases Include
1. Cidofovir: Unlike acyclovir, it is phosphorylated only by cellular enzymes, hence
it is active against HSV with deficient or
altered thymidine kinase enzyme.
2. Foscarnet: It is a phosphonate viral DNA
polymerase inhibitor. It has been the
preferred agent for patients with acyclovir
resistant HSV infection.

the sensory neurons following an earlier


varicella attack.
Both conditions are usually benign and selflimiting in patients with normal immunological status.
One attack of varicella in childhood gives life
long immunity to the patient.
The incubation period is usually 14-17 days.
Varicella occurs throughout the world, the
highest incidence occurs in children aged
2-10 years.
In tropical and semitropical countries,
infection is delayed and varicella is seen more
often in adults. Subclinical infection may
occur.
On the other hand, zoster is uncommon in
childhood and the incidence increases with
age.
In patients with impaired immunity, both the
incidence and severity of zoster are increased,
and it is frequently complicated by disseminated cutaneous disease and systemic
involvement, usually pneumonia, hepatitis,
or encephalitis.

Vaccines: The precise role of vaccines in future


management of HSV infection is yet to be
determined. Modification of frequency and
severity of recurrences may be achievable with
vaccines. The most promising candidate vaccines
are those based on HSV glycoproteins B and D.

VARICELLA-ZOSTER VIRUS INFECTION


Varicella (chicken pox) and herpes zoster are
two distinct clinical entities caused by the
same virus, varicella zoster virus (VZV).
Varicella is the primary exogenous VZV
infection, while herpes zoster represents
reactivation of an endogenous VZV
infection, which persists in a latent form in

Fig. 7.19: Chicken poxvesicular eruption over the


trunk in different stages of evolution in an adult patient

Viral Infections
usually by staphylococci or streptococci and
may be prolonged by scarring. Other notable
complications are primary varicella pneumonia, Reyes syndrome, acute cerebellar
ataxia, encephalitis, and disseminated
varicella in immunosuppressed.
Maternal varicella during the early months
of pregnancy can lead to congenital varicella
syndrome, which manifests as extensive
cutaneous scarring, muscular atrophy, limb
hypoplasia, rudimentary digits, chorioretinitis and cortical atrophy.
Fig. 7. 20: Chicken poxdew drops on rose petals
appearance

In patients infected with HIV, zoster is 10


times more common than in normal
population and may become disseminated
and chronic.

Varicella
In older children and adults, the rash is often
preceded by 2 to 3 days of fever, chills,
malaise, headache, anorexia, severe backache
and in some patients, sore throat and dry
cough.
The rash of varicella begins on the face and
scalp and spreads rapidly to the trunk (Fig.
7.19) with relative sparing of the extremities.
New lesions appear in successive crops but
their distribution remains centripetal.
The lesion starts as a 2 to 4 mm red papule,
which develops an irregular outline (rose
petal) as a thin-walled clear vesicle appears
on the surface (dew drop). This lesion, dew
drop on a rose petal is highly characteristic
(Fig. 7.20). The vesicles become umbilicated
and pustular, rupture to form crusted lesions.
A distinctive feature of varicella is the
simultaneous presence of skin lesions in all
stages of evolution in any one area of the
body.
The most common complication is the
secondary bacterial infection of skin lesions

Differential Diagnosis of Varicella


Disseminated herpes simplex: Has
concentration of lesions at and surrounding
the site of the primary infection and there is
associated marked toxicity and encephalitis.
Eczema herpeticum: Vesicles develop over
the areas of active or recently healed atopic
dermatitis. The lesions are particularly
distributed on the face. Patients commonly
have high fever and adenopathy.
Zoster (Shingles)
The word Shingles is derived from the Latin
singulus a girdle that refers to the segmental
arrangement of the eruption.

Fig. 7. 21: Herpes zostergrouped vesicles on


erythematous patches in thoracic dermatome

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Fig. 7. 22: Herpes zostergrouped vesicles on


genitalia in a male

Fig. 7. 23: Herpes zoster ophthalmicusunilateral


involvement of forehead and scalp by herpes zoster

Zoster afflicts 20% of the general population,


during their life times, especially the elderly.
More than two thirds of the reported cases
occur in individuals over fifty years of age
and less than ten percent occur in those
under the age of twenty years.
The first symptoms are usually pain and
paraesthesia in the involved dermatome. This
often precedes the eruption by several days
and varies from superficial itching, tingling,
burning or lancinating pain.
The rash is nearly always unilateral.
It begins as closely grouped maculopapules,
which rapidly become vesicular in 12-24
hours (Figs 7.21 and 7.22) and then pustular
in 2 to 3 days. The lymph nodes draining that
area become enlarged and tender. The lesions
dry up and crust in 7 to 10 days. Recovery
takes about 3 to 4 weeks or longer in older
patients.
The thoracic (53%), cervical (20%),
trigeminal, including ophthalmic (18%)
(Fig. 7.23) and lumbosacral (11%)

dermatomes are most commonly involved


at all ages, but the relative frequency of
ophthalmic zoster increases in old age.
Hutchinsons sign: Vesicles on the side or on
the tip of the nose that occur during episode
of ophthalmic zoster are usually associated
with serious ocular complications.
Complications of herpes zoster are
hypopigmented or hyperpigmented scars,
post herpetic neuralgia (usually defined as
chronic segmental pain appearing or
persisting 3 months after cutaneous zoster
healing), post herpetic anesthesia, recurrent
zoster, motor neuron involvement (motor
weakness usually follows the pain and
eruption by a few days to weeks), ocular
complications (superficial and deep keratitis,
corneal ulcerations, panophthalmitis, ocular
palsies, neuralgia, retinal vasculitis and optic
neuritis), and rare complications include
meningitis, pneumonitis, and myelitis.
Post herpetic neuralgia- Overall incidence
is 8 to 15% and risk factors are old age, severe

Viral Infections
Skin biopsy of lesions also shows histological
changes similar to those found in herpes
simplex.
Definitive diagnosis accomplished by the
isolation of virus in cell cultures and electron
microscopy.
Identification of VZV antigens in cutaneous
lesions and PCR for detection of nucleic acids
in clinical specimen are the alternative
methods of diagnosis.

Fig. 7.24: Herpes zosterzoster occurring in


multiple dermatomes

pain during acute phase, presence of


prodromal pain, greater rash severity, and
delay in starting treatment.
In HIV infected patients, herpes zoster
ophthalmicus (HZO) is associated with
several severe complications and may the
initial manifestation of AIDS. Cutaneous
lesions can be unidermatomal, multidermatomal (Fig. 7.24), disseminated or
chronic and ulcerative.
Differential diagnosis: Zosteriform herpes
simplex- Vesicles vary in size in zoster, in
contrast to uniformly sized vesicles in herper
simplex and a history of multiple recurrences at
the same site in herpes simplex.

Diagnosis
Tzanck smears of cutaneous lesions of
varicella and zoster show multinucleated
giant cells and epithelial cells with typical
acidophilic intra-nuclear inclusions similar
to those seen in herpes simplex.

Treatment
Symptomatic treatment is given in otherwise
healthy patients. Rest and analgesics are
advised.
Acyclovir is given in severe varicella and
zoster (ophthalmic herpes zoster, Ramsay
Hunt syndrome) and also in those patients
who are at high risk of disseminated infection
(more than 50 years of age, immunosuppressed patients). Acyclovir 800 mg 5
times a day for 7 to 10 days (in children dose
is 20 mg per kg body weight four times daily).
Alternative antiviral drugs are famciclovir
(500 mg three times daily), valacyclovir (1
gm three times daily) and foscarnet in
acyclovir resistant cases.
Valacyclovir is contraindicated in
immunosuppressed individuals. Thrombotic
thrombocytopenic purpura and hemolytic
uremic syndrome have occurred in patients
with AIDS, renal transplant and bone
marrow transplant.
For post herpetic neuralgia-topical therapy
(capsaicin, aspirin, lignocaine), nerve blocks,
and systemic therapy (systemic steroids,
carbamazepine, valproate, tricyclic
antidepressants) may be tried. Gabapentin is
the drug of choice now. Initial dose of 300
mg/day is increased over 4 weeks (up to 3600
mg/day divided into three divided doses)
until efficacy obtained or side effects
tolerable.

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56

Live attenuated viral vaccine for varicella can


be used in healthy children as a prophylaxis.
Its efficacy persists for 10 years.
Varicella zoster immunoglobin therapy is
indicated in those who are high risk patients,
to prevent or modify varicella in them.

KAPOSIS VARICELLIFORM ERUPTION


(ECZEMA HERPETICUM)

It is a special form of widespread cutaneous


viral infection occurring in a patient with preexisting skin disease.
Atopic eczema is the most common
predisposing condition.
Other susceptible dermatoses include
Dariers disease, pemphigus foliaceous,
ichthyosis vulgaris, mycosis fungoides and
allergic contact dermatitis (Fig. 7.17).
Majority of cases are due to infections with
herpes simplex virus called eczema
herpeticum.
Less commonly occur with other viruses like
Coxsackie A 16 and vaccinia (eczema
vaccinatum- no more seen).
Clinically presents with clusters of
umbilicated vesicles in areas where the skin
has been previously abnormal.
They may be associated with fever and other
constitutional features. The face is usually
severely affected and may be edematous.

The evolved pustules become crusted and


heal with permanent scarring in 2-6 weeks.
Multinucleated epithelial giant cells are seen
in Tzanck smear.
Treated with antiviral such as acyclovir
where infection is due to herpes simplex
virus.

KAWASAKI DISEASE (KD)


KD is an acute multisystem vasculitis of
unknown etiology. It was first described in Japan
by Kawasaki (1967).
CDC diagnostic criteria:
Fever lasting longer than 5 days plus at least four
of the following:
1. Bilateral conjunctival injection.
2. Mucous membrane changes (1 or more): Red
or fissured lips, red pharynx, strawberry
tongue.
3. Extremity changes (1 or more): Erythema of
palms or soles, edema of hands or feet,
desquamation (generalized or periungual)
4. Rash-erythematous exanthema.
5. Cervical lymphadenopathy (at least 1 node
larger than 1.5 cm).
Death in KD occurs due to cardiac
complications.
Treatment: aspirin, intravenous gammaglobulin.

Fungal Infections

Fungal Infections

Fungi are aerobic organisms that form a cell wall


and grow on or in organic material, forming a
colony and reproducing either sexually or
asexually. In contrast to plants, fungi do not
manufacture chlorophyll. Without photosynthetic
capabilities, they are dependent on other life forms
including human beings. Fungi are also essential
part of the ecologic cycle and also of considerable
benefit to man as a source of antibiotics, in baking
and in beer brewing.
Fungal infections of skin may be divided into
superficial and deep.

Superficial fungal infections


DERMATOPHYTE INFECTIONS (TINEA,
RINGWORM)
Causative agent: The dermatophyte (or
ringworm). Three species of dermatophytes
implicated are:
1. Trichophyton (affects skin, hair and nails)
2. Microsporum (affects skin and hair)
3. Epidermophyton (affects skin and nails)
Humans acquire infection from three sources
soil, animal or humans
Dermatophyte fungi grow only within keratin
layers, do not invade living epidermis

Predisposing factorsTropical climate of heat


and humidity, poor nutrition and hygiene,
debilitating diseases including HIV infection
It occurs at any age except tinea capitis which
occurs in children mainly.
Most common dermatophyte incriminated is
Trichophyton rubrum
Tinea corporisinfection of the non-hairy skin
of trunk and limbs
The typical lesions start as itchy erythematous
macule or papules that spread outward and
develop into annular (ring like) and arciform
lesions with sharp, scaling or papulovesicular
advancing margin and healing centers (Figs
8.1 and 8.2)
A variant of tinea corporis called tinea
imbricata (imbricata is Latin for tiled) caused
by Trichophyton concentricum, is
characterized by large concentric rings, one
inside another, which manifest commonly in
childhood.
Other variants.
1. Majocchis granulomacharacterized by
perifollicular granulomatous nodules on
the scalps of children, often without
pustulation
2. Agminate folliculitis- characterized by
well defined erythematous plaques

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Essentials in Dermatology

Fig. 8.1: Tinea corporistypical annular


erythematous ring like lesions of tinea corporis

studded with perifollicular pustules


caused by zoophilic organism.
Differential diagnosis: A. For annular lesions
are nummular eczema (does not have an
advancing border, lacks central clearing),
granuloma annulare (no scaling, presence of
skin colored intradermal papules) and
erythema annulare centrifugum (have scales
at the trailing edge of the advancing border
instead of over the entire border). B. For
papulosquamous lesions are psoriasis (well
defined erythematous plaques, scales are
silvery white micacaceous and reveal
bleeding points when removed positive
Auspitz sign, lesions typically involve
extensor extremities, scalp and sacrum),
pityriasis rosea (papular or annular, oval
multiple lesions have characteristic
distribution pattern over the trunk, individual
lesions have a collarette of scales, scale does
not reach the edge of the erythematous border,
this acute eruption, has self limited course).
Tinea cruris-ringworm of the groin (jocks itch
Dhobi itch)
Most common in the tropics (Fig. 8.3).
Differential diagnosis are candidiasis (more
often involves concavities of the flexures and
produces macerated, moist, glazed, erythematous plaques with satellite pustules and it

Fig. 8.2: Tinea corporismultiple ring like lesions


over the trunk

Fig. 8.3: Tinea crurismarked hyperpigmentation


with active border showing erythematous papules

more often involves the scrotum), inverse


psoriasis (tends to be asymptomatic, welldefined erythematous plaque), and erythrasma (lacks a scaling border and
inflammation, asymptomatic in nature,
fluoresces coral red under a wood light).

Fungal Infections

Fig. 8.4: Tinea facieierythematous scaly annular


lesion over the face

Tinea barbae-ringworm of the beard and


moustache.
Tinea faciei-ringworm of the face- most often
misdiagnosed (Fig. 8.4).
Tinea capitis- ringworm of the scalp,
transmitted from child to child, most
commonly caused by Trichophyton violaceum.
In the western literature, commonest causative
organism recorded is T. tonsurans. Both of them
cause endothrix infection (arthroconidia of
dermatophytes contained within the hair
shaft) of hair. Less frequently, ectothrix
infection (arthroconidia of dermatophytes
surrounding the hair shaft as a sheath) of hair
also occurs.
Patchy hair loss and broken hairs,
inflammation and scaling are characteristic
of (back dot (Fig. 8.5), grey patch (Fig. 8.6),
seborrheic dermatitis like) tinea capitis.
Kerion is caused by zoophilic fungi
Trichophyton verrucosum and Trichophyton
mentagrophyte. Typically, it produces a boggy
painful inflammatory swelling studded with
exudative follicular pustules, hair fallen off
or easily pluckable (Fig. 8.7).
Favus in India occurs mainly in Kashmir and
is caused by Trichophyton schonleinii. On the
scalp, concave sulfur-yellow crust (called
scutulae) form around loose wiry hairs. It
heals with thin smooth atrophic scarring.

Fig. 8.5: Tinea capitispatchy hair loss with black


dots Black dot type

Fig. 8.6: Tinea capitispartial loss of hair, remaining


hair in the alopecia patch area are luster less Grey
patch type

Differential diagnosis are alopecia areata


(patchy non-scarring alopecia, presence of
exclamation mark hair, non-scaly condition),
trichotillomania (irregular patchy hair loss from
an approachable site because of hair pull trauma,
so hairs are of different lengths), seborrheic
dermatitis (greasy scaling is more diffuse, with
erythema, changes confined to scalp and other

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Fig. 8.7: Tinea capitisboggy inflammatory swelling


over the scalp Kerion type

Fig. 8.8: Tinea pedisscaly hyperkeratotic lesion on


the sole of one foot, other normal for comparison

seborrheic areas of the body, generally no hair


loss), and bacterial pyodermas of scalp (loss of
hair is minimal, plucking of hair may be difficult
and painful)

keratodermas and hyperkeratotic eczema. Contact


dermatitis may also be considered although it
affects the dorsum of foot than plantar aspect
(tinea pedis).
The vesicobullous type may be confused with
pustular psoriasis, palmoplantar pustulosis, and
bacterial pyodermas.

Tinea pedis- ringworm of the feet (Athlete foot)


Interdigital type-interdigital scaling and
maceration with fissures is the most common
form (Fig. 8.8).
Chronic hyperkeratotic type or Moccasin footwidespread scaling extends onto the sides of
the feet.
Vesicular or bullous type due to some
zoophilic fungi
Acute ulceratic type
Differential diagnosis: For the interdigital type are
erythrasma (very well defined lesion, it has
whitish appearance usually asymptomatic and
rarely causes fissures) and candidiasis (increased
severity of maceration, denudation and pruritus).
The hyperkeratotic type must be differentiated
from palmoplantar psoriasis, palmoplantar

Tinea manuum-ringworm of the hands


Usually seen as mild erythema with
hyperkeratosis and scaling, mainly over the
palmar surfaces (Fig. 8.9).
Unilateral scaling with onychomycosis if
present should always be scraped for fungus
(Fig. 8.10).
Contact dermatitis, psoriasis, and hyperkeratotic eczema must be considered as
differential diagnosis.
Tinea unguium- fungal infections of the nails
Nail infection starts at the free margin or lateral
borders of the nails, as yellow discoloration
and progress proximally (distal and lateral
subungual onychomycosis).

Fungal Infections

Fig. 8.9: Tinea manuumunilateral scaly lesions of


the palm

Fig. 8.11: Tinea unguiumthickening and


dystrophy of involved nails

infection caused by dermatophytes that cause


endothrix scalp infection characterized by
scarred nail plate with pits and lamellar
splits).
Ringworm of the nails is rarely symmetrical
and it is common to find the nails of only one
hand affected)
Differential diagnosis is psoriasis (coarse
pitting of dorsal nail plate is never produced
by fungal infection, and strongly suggests
psoriasis, as does the oil drop sign from the
free edge), eczema (irregularly buckled nails)
and lichen planus (ridged or dystrophic nails)
Tinea incognito- ringworm infections
modified by corticosteroids, systemic or topical,
prescribed for some pre-existing pathology or
given mistakenly for the treatment of
misdiagnosed tinea (Fig. 8.12).
Fig. 8.10: Tinea manuum with tinea unguium

The nails become brittle, friable, and


thickened (Fig. 8.11).
Four distinct patterns of tinea unguium are
distal and lateral subungual onychomycosis
(most common pattern), superficial white
onychomycosis, proximal subungual
onychomycosis (associated with HIV/AIDS
cases), and endonyx onychomycosis (nail

Diagnosis
KOH mount definitive diagnosis is made by
the microscopic examination and identification of hyphae and spores in scales
(Fig. 8.13) or hair. In nails, the presence of
hyphae usually means dermatophyte
infection, rarely saprophytic fungi infection.
Cellophane tape examination and staining
with periodic acid Schiffs (PAS) reagent of

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Essentials in Dermatology

Fig. 8.12: Tinea incognitoerythematous papular


eruption of tinea due to immunosuppression

Fig. 8.14: Periodic Acid Schiffs (PAS) stained smear


showing septate branching hyphaedermatophytes

2. Imidazole derivatives miconazole (2%),


clotrimazole (1%), econazole (1%),
ketoconazole (2%), oxiconazole, sulconazole,
eberconazole
3. Ciclopirox olamine
4. Undecylenic acid
5. Tolnaftate 1%
6. Azelaic acid
7. Castellanis paint
8. Terbinafine (1%)
Fig. 8.13: KOH mountshowing branching
septate hyphae

the material taken from skin (Fig. 8.14), hair


and nails can be studied.
Confirmed by culture on Sabourauds media.
Histological examination of nails with special
stain-Periodic Acid Schiffs stain.

Therapy
Topical therapy- indicated for infections of
the body and groin and superficial
involvement of the beard region, palms, and
soles
Topical agents used are
1. Whitfield ointment (contains 6% benzoic acid
and 3% salicylic acid)

Duration of therapy is 4 to 6 weeks or 2 weeks


more after clearance of lesions.
Systemic therapy required in case of hair and
nail infection and in those cases where tinea
is extensive, widespread and non-responsive
to topical therapy. Griseofulvin (10-20 mg per
kg body weight or 250 mg twice a day),
ketoconazole (200 mg daily), itraconazole (200
mg daily or one week pulse of 400 mg daily
for 2 to 3 months), fluconazole (6 to 8 mg per
kg body weight daily or 150 mg, 300 mg or
450 mg per week for 3 to 6 months) and
terbinafine (250 mg daily or 250 mg twice a
day) are the drugs used systemically. The skin
and hair infection require shorter course of
therapy (6-8 weeks therapy with griseofulvin)
whereas nail infection require longer therapy
(9-12 months with griseofulvin).

Fungal Infections

TINEA VERSICOLOR (PITYRIASIS


VERSICOLOR, DERMATOMYCOSIS
FURFURACEA, CHROMOPHYTOSIS)
It was thought that a single polymorphic yeast
Pityrosporum ovale, or two species P. ovale
and P. orbiculare, were the causative agents,
but it is now recognized that this genus was
invalid. There are at least 7 separate species
of lipophilic yeast- Malassezia on the human
skin: M-sympodialis (most commonly found on
the normal skin), M.globosa (most frequently
associated with tinea versicolor), M. restricta,
M. dermatis, M.slooffiae, M.obtusa and M. furfur.
Predisposing factors- Warm and humid
climate of tropics, pregnancy, serious
underlying diseases, immunocompromised
host, Cushings syndrome, malnutrition.
The term versicolor refers to the presence of
both hyper-and hypopigmented lesions
A peculiar aspect of tinea versicolor is its
propensity to present as either hypopigmented
or hyperpigmented, finely scaling, round or
perifollicular coalescing macular patches
found primarily over the trunk (Figs 8.15 and
8.16). The hypopigmentation is explained on
the basis of dicarboxylic acids produced by
Malassezia species (e.g., azelaic acid) causing
competitive inhibition of tyrosinase and
perhaps a direct cytotoxic effect on

Fig. 8.15: Tinea versicolorhypopigmented


macules with fine powdery scales

Fig. 8.16: Tinea versicolorhyperpigmented


macules with fine scaling

hyperactive melanocytes. The hyperpigmentation may be due to abnormally large


melanosomes and thicker keratin layer. To
elicit fine branny powdery scales, candle
grease sign or coup dongle sign is elicited.
The eruption is almost always asymptomatic
and only of cosmetic significance
Other cutaneous disorders associated with
Malassezia yeasts are seborrhoeic dermatitis,
Malassezia folliculitis, Confluent and
reticulate papillomatosis, and sebopsoriasis.

Diagnosis
KOH preparation-reveals numerous short,
straight and angular hyphae and clusters of
thick walled round and budding yeasts
Spaghetti and meat ball appearance or
Banana and grapes appearance(Fig. 8.17).
Cellophane tape examination and staining
with periodic acid Schiffs (PAS) reagent and
India ink of the material taken from skin (Figs
8.18 and 8.19).

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Fig. 8.17: KOH mountclusters of spores with short


mycelial filaments. Spaghetti and meat ball
appearance

Fig. 8.18: Periodic Acid Schiffs (PAS) stained smear


showing cluster of spores with short stumpy mycelial
filaments- Malassezia

Culture- Sabourauds dextrose agar overlaid


with sterile olive oil or lanolin. Antibiotics
such as penicillin, streptomycin and
cycloheximide are incorporated to reduce
growth of contaminants.
Woods light examination-golden to orange
fluorescence.

Differential Diagnosis
1. Pityriasis alba characterized by poorly
marginated, hypopigmented, slightly scaly
patches on the cheeks of young children.

Fig. 8.19: Parker ink staining of hyphae and spores


of tinea versicolor

2. Indeterminate leprosy usually seen in


children with one or more macules,
hypopigmented to faintly erythematous with
an ill-defined to well defined edge. Lesions
may be on the face or the limbs. Slight
anaesthesia may be demonstrable.
3. Vitiligo characterized by depigmented
macules without any scaling
4. Erythrasma Hyperpigmented tinea versicolor can be confused with erythrasma, but
satellite lesions are less common in
erythrasma and there is coral red fluorescence
under Woods lamp.
5. Seborrhoeic dermatitis May occur in areas of
tinea versicolor distribution, but patches have
an erythematous yellowish tint and scales are
soft and greasy

Therapy
Topical Selenium sulfide suspension (2.5%),
zinc pyrithione, ketoconazole (2%) shampoo,
sodium hyposulfite (25%), propylene glycol
and imidazole derivatives are used topically.
Systemic Ketoconazole (200 mg daily for 5 to
10 days), itraconazole (200 mg daily for 5 to 7
days) or fluconazole (single oral dose of 400
mg) are effective systemically.

Fungal Infections

CANDIDIASIS

Causative agent Candida albicans. This


yeast like fungus is normally found on the
mucous membranes, skin, in the gastrointestinal tract and in the vagina. Under
certain circumstances, it changes from a
commensal organism to a pathogen. Apart
from Candida albicans, few other species of
Candida like C. tropicalis, C. dubliniensis, C.
parapsilosis, C. guilliermondii, C. krusei, C.
glabrata may be involved
Predisposing factors Local factors are
moisture, warmth, maceration, and/or
occlusion and systemic factors are antibiotics
therapy, corticosteroids, oral contraceptive
pills, pregnancy, diabetes, immunocompromised state including HIV infection.

can be confused with coated tongue due to


dehydration, mucositis due to chemotherapeutic drugs, aphthous ulcerations,
herpetic infections, oral hairy leukoplakia,
erythema multiforme, pemphigus, lichen
planus, discoid lupus erythematosus,
Pernicious anemia, histoplasmosis, leukoplakia, secondary syphilis.
Perlechesore angles of mouth.
Intertriginous type Well-defined peeling
border around moist red macerated lesions
and surrounded by satellite papules or
pustules- occur in body folds such as groin,
inframammary, axillary, perianal and
interdigital areas (Fig. 8.22). Differential

Clinical Manifestations
Oral thrush It presents as curdy, white, easily
detachable deposits on tongue or oral mucosa
with underlying bright red and moist surface
(Figs 8.20 and 8.21). This form of candidiasis
is also known as acute pseudomembranous
candidiasis. It is the most common form of oral
candidiasis. Other forms of oral candidias is
include acute and chronic atrophic
candidiasis, chronic hyperplastic candidiasis,
and median rhomboid glossitis. Oral thrush

Fig. 8.21: Oral candidiasis with angular cheilitis in


a HIV adult case

Fig. 8.20: Oral candidiasiscurdy white deposits on


the hard palate in a baby

Fig. 8.22: Intertriginous candidiasistoe cleft


showing moist macerated lesion

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Essentials in Dermatology
diagnosis includes tinea cruris, seborrhoeic
dermatitis, flexural psoriasis and bacterial
intertrigo.
Paronychia inflammatory boggy swelling of
posterior and lateral nail folds of digits of
fingers (Fig. 8.23), commonly seen in house
wives, maidservants, due to continuous wet
job
Vulvovaginitis-frequently associated with
itching and vaginal discharge. The vaginal
mucosa shows erythema, edema and curdy
white deposits (Fig. 8.24). Candidal balanoposthitis occurs as a counter part in males
(Fig. 8.25).

Fig. 8.25: Candidal balanoposthitisglazed


erythema with pustulation over glans and prepuce

Diagnosis
KOH preparation-reveal budding yeasts with
or without hyphae or pseudohyphae.
Grams stained smear-gram positive
organisms longer than bacteria.
Confirmed by culture on Sabourauds media.
Whitish mucoid colonies grow within 2 to 5
days.

Fig. 8.23: Chronic paronychiaswelling of proximal


and lateral folds of many fingers with nail changes

Fig. 8.24: Candidal vulvovaginitisvulval erythema


with white deposits

Therapy
Evaluation and treatment of underlying
medical conditions
Nystatin, imidazoles and broad-spectrum
triazoles are the agents of greatest use in the
treatment of candidal infection. Gentian violet
and Castellanis paint are older effective
topical remedies.
Potassium permanganate soaks are more
effective in moist Candidal intertrigo.
Recurrent or recalcitrant infections require
oral medication with nystatin (to rid the gut
and vagina of candida organisms),
fluconazole (50 mg daily for 14 days),
ketoconazole (200 mg daily) or itraconazole
(200 mg daily).
For vaginal candidiasis, 500 mg clotrimazole
vaginal tablet once or 200 mg miconazole
tablet at bed time for 3 days or single oral dose

Fungal Infections
of 150 mg of fluconazole or twice daily dose of
itraconazole (200 mg twice a day).

CHRONIC MUCOCUTANEOUS
CANDIDIASIS
It is a rare syndrome which is characterized
by recurrent and persistent candidal infection
of skin, nail and mucous membrane. It has
autosomal dominant or recessive inheritance.
Several endocrinopathies (e.g. hypothyroidism, hypoadrenatism, etc.) may be
associated.
Treatment: Treatment of this condition
depends critically on systemic antifungal
chemotherapy. Attempts have been made to
restore T cell function, by the use of transfer
factor, or thymosin, or grafting compatible T
lymphocytes and non specific measures like
the restoration of normal iron stores.
Prolonged and repeated use of systemic
antifungals like fluconazole, itraconazole and
ketoconazole may be necessary.

PIEDRA (PIEDRA = STONE)


(TRICHOSPOROSIS)
Causative agent White piedra-Trichosporon beigelii
(Trichosporon cutaneum), a yeast seen in
temperate region
Black piedra-Piedra hortae, a mould, occurs
mostly in the tropics
Pinhead sized, hard nodes occurs on the hairs
of the scalp, brows, lashes, or beard
KOH examination and culture clinch the
diagnosis
Differential diagnosis Nits, hair casts,
developmental hair defects and trichomycosis
axillaris may be differentiated by doing a
microscopic examination.
Treatment is by cutting hair. Oral terbinafine
250 mg daily for 6 weeks has been shown to
be effective against black piedra. For white
piedra, imidazoles, selenium sulfide, zinc
pyrithione, etc. are effective agents.

Deep fungal infections

TINEA NIGRA

MYCETOMA

Causative agent- Phaeoannellomyces werneckii


(syn. Exophiala werneckii), a mould which
produces a melanin like pigment.
This characteristic disorder manifests as one
or several brown or black spots that resemble
silver nitrate or India ink stains.
Most frequently lesions occur on the palms
but also on the soles.
The fungus can be easily demonstrated by
KOH examination and culture.
Differential diagnosis are junctional naevi,
melanoma, Addisons disease, and chemical
stains, however, tinea nigra can be easily
differentiated by doing a KOH examination
which shows brown coloured hyphae and
spindle shaped yeast cells.
Topical imidazoles such as clotrimazole,
miconazole, ketoconazole, etc. are effective.

Mycetoma is a deep fungal infection,


characterized by a clinical triad of swelling,
discharging sinuses and discharge
containing granules (Figs 8.26 and 8.27). It
commonly occurs on the foot, hence also
called as Madura foot.
Mycetoma-caused by species of fungi is
known as eumycetoma, and that caused by
aerobic actinomycetes or filamentous
bacteria as actinomycetoma.
Type of mycetoma Causative organisms
Eumycetoma

Actinomycetoma

Madurella mycetomatis
Exophila jeanselmei
Pyrenochaeta romeroi
Fusarium species
Actinomadura madurae
Nocardia brasilensis
Actinomadura pelletieri

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Fig. 8.26: Mycetomaa triad of features swelling,


discharging sinuses and discharge containing
granules seen commonly over the foot

Differential diagnosis
1. Chronic osteomyelitis of bacterial or
tuberculosis origin (characteristic grains
are not discharged)
2. Elephantiasis (no sinus tracts)
3. Primary cutaneous actinomycosis
(develops on the exposed sites, very rare
type of actinomycosis, caused by Actinomyces isralei, a normal inhabitant of
human mouth, thus it is an endogenous
infection).
Actinomycetomas generally respond to
antibiotics such as a combination of dapsone
with streptomycin or sulfamethoxazoletrimethoprim plus rifampin or streptomycin.
Amikacin may also be used in recalcitrant
Nocardia infections.
Of the fungal causes of mycetoma,
M. mycetomatis may respond to ketoconazole
(200 mg daily over several months). For the
others, a trial of therapy with griseofulvin or
itraconazole is worth attempting. Surgery,
usually amputation, is the definitive
procedure and may have to be used in
advanced cases.

CHROMOMYCOSIS
(CHROMOBLASTOMYCOSIS)

Fig. 8.27: Mycetoma over the upper back

The organisms are usually soil or plant


saprophytes that are only incidental human
pathogens.
KOH examination, Grams staining,
histopathology of lesion and culture helps in
confirmation of the diagnosis.

Chromoblastomycosis is primarily a disease


of tropical or subtropical regions.
At least five distinct organisms are well
known to cause chromoblastomycosis:
Fonsecaea pedrosoi, Fonsecaea compactum,
Cladosporium carrionii, Phialophora verrucosa,
and Rhinocladelia (Acrotheca) aquaspersa.
The characteristic feature of a well-developed
lesion is a pruritic warty plaque of the
extremities in an agricultural worker, which
drains purulent material (Fig. 8.28).
The pathology of chromoblastomycosis
consists of striking epidermal thickening
(pseudoepitheliomatous hyperplasia) over-

Fungal Infections

Fig. 8.28: Chromomycosisverrucous itchy plaque


lesion over the dorsa of the hand

Fig. 8.29: Rhinosporidiosispink friable polypoidal


growth projecting from the nares

lying a suppurative granulomatous


dermatitis. At least three appellations are
frequently used to designate the tissue form of
these fungi: Medlar bodies, sclerotic bodies,
muriform bodies or the descriptive copper
pennies.
Differential diagnosis:
1. Blastomycosis (presence of sharp border
with minute abscesses and by the presence
of pulmonary lesions)
2. Cutaneous tuberculosis and leishmaniasis (biopsy and culture will
establish the diagnosis)
3. Elephantiasis verrucosa nostras (mossy
foot).
The known treatments may be divided into
three groups: surgery, physical modalities
(heat, cryotherapy, electrosurgery, and
radiation therapy), and systemic antifungal
medications(amphotericin B, 5-fluorocytosine,
triazole derivatives especially itraconazole,
and thiabendazole).

It is seen most often in southern India and


Srilanka.
It is more common in adult males and is
possibly transmitted to man by direct contact
with spores through dust, infected clothing
or fingers and swimming in stagnant waters.
Rhinosporidiosis frequently involves the
nasopharynx (70%) presenting as a painless,
friable, polypoidal growth, which may hang,
anteriorly from the nares (Fig. 8.29) or
posteriorly into the pharynx. The lesions are
pink or purple red and studded with minute
white spots which are the sporangia
containing spores. Nasal obstruction and
bleeding are the most common symptoms. The
conjunctiva and lacrimal sac are involved in
15% of cases.
Occasionally, it affects the lips, palate, uvula,
maxillary antrum, epiglottis, larynx, trachea,
bronchus, ear, scalp, vulva, vagina, penis,
rectum or the skin (Figs 8.30 and 8.31).
Since the causative agent cannot be cultured,
the diagnosis can be confirmed by
demonstrating typical sporangia and spores
in histopathology and imprint smears.
Differential diagnosis is nasal polyps, warts,
and condylomas.

RHINOSPORIDIOSIS
Rhinosporidiosis is a chronic granulomatous
mycosis caused by Rhinosporidium seeberi.

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Essentials in Dermatology

Fig. 8.30: Rhinosporidiosispink friable polypoidal


growth over the root of the nose

Fig. 8.32: Subcutaneous phycomycosisshiny disk


like indurated lesion over the thigh, can be insinuated
at the margin with fingers

Fig. 8.31: Rhinosporidiosisnoduloplaque lesions


over the arms

Surgical removal and electro desiccation are


the treatments of choice.

SUBCUTANEOUS ZYGOMYCOSIS
Subcutaneous zygomycosis or subcutaneous
phycomycosis (SP) has two clinically and
mycologically distinctive entities termed as
basidiobolomycosis (etiological agent:
Basidiobolus ranarum) and conidiobolomycosis (etiological agent: Conidiobolus

coronatus). These organisms belonging to


Entomophthorales cause granulomatous
infection that usually affects healthy people.
SP due to Conidiobolus is uncommon.
Clinically, the disease is characterized by
nasal obstruction due to the inflammation of
the submucosa of the nostril, usually in the
vicinity of the inferior turbinate.
SP is also caused by Basidiobolus. The site of
infection is usually confined to the limb
girdles or proximal limbs. It occurs chiefly in
children. Characteristically, it manifests as
painless, well-circumscribed, firm to hard
subcutaneous masses, which grow slowly at
the periphery and may envelop parts of or a
whole limb (Fig. 8.32). The border is smooth,
rounded, clearly defined, and can be raised
up by inserting fingers underneath it. This is
thought to be an almost diagnostic clinical
feature of the disease. There is no involvement
of the regional lymph nodes.

Fungal Infections
Differential diagnosis:
1. Lymphatic edema (no distinctive edge)
2. Subcutaneous malignant lymphoma
(grows more rapidly)
3. Subcutaneous morphea
A therapeutic trial with potassium iodide in
a clinical setting may be considered as an
important criterion for diagnosis where
facilities to culture the organism do not exist.

SPOROTRICHOSIS
It is caused by Sporothrix schenckii and is
characterized by nodulo-ulcerative and
crusted lesions arranged in a linear fashion
over the extremities with intervening
lymphatics thickened like a cord.
The best sources of diagnostic material are
smears, exudates, and biopsies (to look for
Asteroid bodies). S. schenckii is very rarely
seen in direct microscopic examination
because yeasts are usually present only in
small numbers; the organism can be readily
isolated on Sabourauds agar.
Differential diagnosis:
1. Fish tank granuloma
2. Cutaneous leishmaniasis
Potassium iodide (saturated solution) is
effective in the cutaneous types of
sporotrichosis.
Other deep fungal infections: Cryptococcosis
and aspergillosis are ubiquitous throughout the
world. In south east Asia, penicillinosis is
common whereas coccidioidomycosis and
histoplasmosis are restricted to certain
geographic regions.

CRYPTOCOCCOSIS
Cryptococcosis is an opportunistic infection
caused by the encapsulated yeast Cryptococcus
neoformans.

Virtually all infections involve the central


nervous system, with meningitis the most
frequent manifestation.
Cutaneous dissemination occurs in 10% to
20% of patients, has a variable presentation
and may precede other signs of infection.
Initial signs of cryptococcosis include
cellulitis, genital or oral ulcerations, or
molluscum-, herpes simplex-, or Kaposis
sarcoma- like lesions.
Diagnosis can be made by performing
curettage on a lesion, by making a potassium
hydroxide preparation, India ink
preparation, isolation of fungus on culture or
by a biopsy of lesion. Cryptococcal antigen is
present in these patient sera and can be
detected by latex particle agglutination.
Intravenous amphotericin B alone or with
flucytosine and oral fluconazole is highly
effective in the treatment of cryptococcus
infection.

PENICILLIOSIS
Penicillium marneffei is the only penicillium
species that is dimorphic and can cause
systemic mycosis in human beings,
particularly those who are immunocompromised.
Features of the infection frequently include
fever, anemia, marked weight loss, cough and
diarrhea, but skin eruptions occur in the
majority.
Cutaneous manifestations usually consist of
a generalised papular eruption, in which the
papules may be umbilicated (due to central
necrosis), although necrotic papules, nodules,
folliculitis, macular rash and mouth ulcer
have also been reported.
Diagnosis depends on isolation of the
organism from blood or tissue.
Treatment includes systemic amphotericin B,
itraconazole or fluconazole.

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Infestations

Causative agent: Sarcoptes scabiei var. hominis


(Fig. 9.1)
Morphology: The mite has an ovoid body,
flattened dorsoventrally. The body is creamy
white marked with transverse corrugations,
and on its dorsal surface by bristles and
spines (denticles). The mite has four pairs of
short legs. The rear two pairs of legs of female
mites end in long bristles called setae. Adult
female mite measures about 0.4 0.3 mm
whereas adult male about 0.2 0.15 mm. The
mite prefers non-hairy skin and areas of low
sebum production.

Life cycle: Copulation occurs in burrows


excavated by female mite in stratum
corneum. After copulation, the pregnant
female enlarges the burrows and begins egg
laying. It travels 5 mm per day and lays 4050 eggs during its life span of 4-6 weeks (Figs
9.2 and 9.3). These eggs hatch in a week and
reach maturity (eggs-larvae-nymph-adult) in
about 3 weeks.
Most infected adults harbor 10 to 12 mites
Mode of spread-close personal contact, but
may be transmitted through clothing, or
towels.
Incubation period- when a human is
infested for the first time, symptoms usually

Fig. 9.1: Sarcoptes scabiei mite (300-400 microns)

Fig. 9.2: Eggs and fecal pellets of sarcoptes

SCABIES (THE ITCH,


SEVEN YEAR ITCH)

Infestations

Fig. 9.3: An egg of sarcoptes

Fig. 9.5: Scabies periumbilical papular lesions

Fig. 9.4: Scabies typical finger web spaces


involvement with papular, vesicular and crusted
lesions

Fig. 9.6: Scabies genital and thigh area involved


by papular and excoriated lesions

develop after 3-6 weeks while after


reinfestation, they occur within 1 or 2 days.
The classic symptom is intense pruritus
especially at night in bed
The sites of predilection are the interdigital
spaces (Fig. 9.4), wrists, points of elbows,
antecubital fossae, the anterior axillary folds,
the umbilicus (Fig. 9.5), and the genitalia (Fig.
9.6) especially the gluteal cleft (Fig. 9.7)
(circle of Hebra).
The most diagnostic or pathognomonic
finding is an intact S shaped or linear
burrow with a papule or vesicle at its end
housing the mite (Fig. 9.8). Most common

Fig. 9.7: Scabies papular lesions over the gluteal


area

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Essentials in Dermatology

74

Fig. 9.8: Scabiestypical burrows of scabies of the


shaft of the penis

sites are webspaces of the hands, wrists, and


lateral aspect of palms.
Generalized urticarial papules, excoriations
and eczematous changes are secondary
lesions caused by sensitization to the mite.
Tiny scaly papules on the nipple and male
genitalia (glans, shaft and scrotum) are
pathognomonic of scabies.
Infants and small children often have
vesicular lesions on the palms, soles, head
and neck (Figs. 9.9 and 9.10). Scabies in
babies is generally more extensive in
distribution of burrows, vesicular or
vesicopustular lesions on the hands and feet,
extensive eczematization, multiple crusted
nodules on the trunk and limbs.
Nodular scabies- in some cases, itching
nodules (520 mm in diameter, red, pink, tan
or brown in color) persist for several months.
They are found most commonly on the
scrotum (Fig. 9.11). Burrow may be seen on
the surface of early nodules.

Fig. 9.9: Scabies in an infantpapulovesicular


lesions in finger web spaces

Fig. 9.10: Scabies in an infant vesicular, pustular


and crusted lesions on the ankle and feet

Scabies incognito means modified clinical


picture of classical scabies which mimic other
dermatoses due to inappropriate use of
topical steroids.
Complications of scabies: Secondary
infection of skin lesions, eczematization,
nephritogenic strains of streptococci may
produce secondary sepsis, and glomerulonephritis particularly in tropics.

Infestations

Fig. 9.11: Nodular scabiesnodules seen


over the scrotum

Diagnosis: If a mite is demonstrated, one


needs no diagnostic criteria. Typical lesions
on the penis and nipple, the presence of
burrows even without a mite and interdigital
lesions are almost diagnostic. Severe pruritus,
especially at night, of short duration or in
family members is also very suggestive.
Burrow identification (Ink method): The
suspected burrow is smeared with blue or
black fountain pen ink and then wiped off
with an alcohol swab after some time. The
dye that enters the burrows is highlighted as
a dark line.
Microscopic examination: The burrow is
scraped with 15 no. blade and examine the
material with 10% KOH or mineral oil under
light micrscope. Presence of mite, egg or fecal
concretions (scybala) confirms diagnosis of
scabies.
Under dermoscope, mite in burrow
resembles jet with contrail.
Differential diagnosis:
A. For pruritic localized or generalized
rash: In infants: Papular urticaria,
infantile acropustulosis, In children:
Papular urticaria, insect bite reactions,

atopic dermatitis, animal scabies, In


adults: acute generalized lichen planus,
adverse drug reactions, contact
dermatitis, pediculosis pubis, pediculosis
corporis, different forms of prurigo, In
elderly: Dermatitis herpetiformis, senile
pruritis, delusional parasitosis.
B. For pruritic nodules: Urticaria
pigmentosa, papular urticaria (insect
bite), and pseudolymphoma.
Therapy: Permethrin (5%) cream is
treatment of choice (single overnight
application below neck all over the body with
a second application after an interval of a
week). It is the treatment of choice for infants
(application includes head and neck also).
Sulfur and crotamiton are safe in pregnancy.
Other agents used are gamma benzene
hexachloride lotion (1%), benzyl benzoate
lotion (12.5% for infants and children, 25%
for adults), esdepallethrine 0.63%, malathion
0.5% lotion, ivermectin 0.8% lotion, and
monosulfiram (25%) diluted with two or
three parts of water to form an emulsion.
Ivermectin 200 microgram per kg body
weight single oral dose is also effective in
many cases of ordinary scabies, but
presumably because of lack of ovicidal
activity, higher cure rates are obtained with
two doses separated by an interval of a week.
It is a useful modality of treatment for
institutional outbreaks of scabies as it is
cheap, effective and easy to administer.
Pruritus may persist for up to 1-2 weeks after
the end of effective treatment.
Treat infested individuals as well as close
physical contacts simultaneously.
Bedding and clothing should be washed in
hot cycle of washing machine.
Intralesional triamcinolone 5-10 mg / ml in
each lesion is used for nodular scabies besides
routine scabies treatment.

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CRUSTED SCABIES
(NORWEGIAN SCABIES)
Very uncommon variant of scabies.
Though the first report of crusted scabies was
in patients with leprosy, recent reports have
described an increasing incidence of this
form of scabies in patients with
immunosuppression due to immunosuppressive agents, malignancy or acquired
immunodeficiency syndrome (AIDS).
In this form of scabies, the hosts response to
the mites is modified, allowing them to
multiply. As a result of this, the mite
population becomes enormous and may
number millions.
The large adherent crusts are most often seen
over the knees and elbows as well as the
hands (Fig. 9.12) and feet. There may even
be an erythroderma.
Frequently, it can be confused with psoriasis,
chronic eczema (contact dermatitis),
Langerhans cell histiocytosis, or neurodermatitis (Fig. 9.13).
Ivermectin seems to be ideal drug for this
condition; otherwise prolonged therapy
with topical scabicidal agents is required.
Methotrexate is another option.

Fig. 9.12: Crusted scabies hyperkeratotic scaly


lesions over the palm and finger web space

Fig. 9.13: Crusted scabiesscaly cursted lesions


over the side of the buttock

ANIMAL SCABIES
Contracted from pet animals-cats, dogs, or
birds.
No burrows seen, only itching and papulovesicular lesions are seen over the site of
contact-abdomen, thighs and arms.
Treatment of pet animal with scabicidal agent
and antipruritic agents for controlling itching
are required.
Human skin lesions are self limiting; resolve
spontaneously if further contact with affected
animal is stopped.

Fig. 9.14: Pediculus humanus capitis

Infestations

Fig. 9.15: Phthirus pubis

are oval, 1 mm long and firmly attached to


the hair (Fig. 9.16) (or seems of clothing),
hatch in about 7-9 days and become mature
in another week.
Body louse is the vector for the following
organisms:
1. Rickettsia prowazekii (Endemic typhus),
2. Bartonella quintana (Trench fever),
3. Borrelia recurrentis (Relapsing fever).
Extreme pruritus is the primary characteristic of pediculosis.
Incubation periodIt takes 6 weeks
(approximately 30 days) for the pruritus to
develop in non-sensitized individual and
only 24 to 48 hours with repeat exposures.

Pediculosis Capitis
Most common in children especially girls
with long hairs.
Mode of spread- human to human contact
or sharing combs, brushes and towels.
Scalp-favorite site is area behind the ear
(Fig. 9.17) or occiput.
Nits may be found most easily on the hairs.
Adult lice often impossible to find.
In patients with scalp pruritus, secondary
pyoderma (with cervical lymphadenopathy)
and dermatitis, one should always search the

Fig. 9.16: Nits attached to the hair

PEDICULOSIS (LICE INFESTATION)


Pediculus humanus capitis (2 to 4 mm long)head louse (Fig. 9.14).
Pediculus humanus corporis-body louse.
Phthirus pubis-pubic louse (Fig. 9.15).
Life cycle these wingless six legged blood
sucking insects are obligate parasites specific
for humans, lays eggs. The ova (nits), which

Fig. 9.17: Pediculosis capitis nits on the hair

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Essentials in Dermatology

Fig. 9.18: Plica polonicamatting of the scalp hair

hairs behind the ears carefully for nits. The


hairs may become matted from repeated
scratching (plica polonica) (Fig. 9.18).
Differential diagnosis: Dandruff, here flakes
come off easily from hair on manipulation.
Hair casts look similar to nits but form an
encompassing cylinder whereas nits are
attached at an angle. Piedra is much less
common and consists of clumps of fungi.
Permethrin 1% cream rinse (10 minute
application) is the treatment of choice. Repeat
application after one week is required to kill
hatched out nits. Pyrethrins, lindane,
malathion and benzyl benzoate are other
agents, which can be used topically.
Systemically, ivermectin, 200 g/ kg body
weight, two doses at 7 days interval may be
used. Co-trimoxazole 1 tablet twice a day for
3 days, following that second course 7-10 day
later.
For nit removal: After treatment, hairs are
soaked with vinegar and water (50:50) and
then combed with fine toothed nit comb.

Itching is the main symptom , which is due


to sensitivity to salivary antigens.
Patients harbouring body lice for long time
develop hyperpigmentation of skin, develop
numerous excoriations (frequently most
noticeable on the side of trunk and back),
secondary infection and even lymphadenopathy-vagabonds skin, vagabonds
itch, or vagabond disease (morbus errorum).
For diagnosis: Closely examine the clothing
for lice and nits along the seams.
Differential diagnosis: All pruritic
dermatoses especially scabies, contact
dermatitis, atopic dermatitis.
Therapy: Clothes must be disinfected.
Topical antipruritics or corticosteroids are
given to the patient. A course of antibiotic
may be needed to take care of secondary
infection.

Pediculosis Pubis
Transmission in adults occurs mostly
during sexual intercourse.
The pubic or crab louse is smaller than the
head or body louse, has resemblance to a crab
(for prominent claws in second and third
pairs of legs).
Pruritus or crawling sensation is modest in
the pubic area.

Pediculosis Corporis
Infestation associated with poor personal
hygiene, a disease of homeless.
The body louse feeds on body and lays its
eggs on seems of clothing.

Fig. 9.19: Phthriasis pubis adult louse clung to


the hair

Infestations
of insects such as mosquitoes, fleas, and
bedbugs.
This condition is mainly seen in childhood.
Treatment involves use of insect repellants,
topical antipruritic agents and antibacterial
with or without steroids.

LARVA MIGRANS ERUPTION

Fig. 9.20: Phthriasis palpebrarum nits attached to


eyelashes

Adult lice may be difficult to find and are


usually located at the base of hairs
resembling small freckles, scabs or moles
(Fig. 9.19).
Other than pubic hair, body and axillary hair
as well as eyelashes (phthiriasis palpebrarum) (Fig. 9.20) and beard should be
examined for nits.
A classical clinical finding is the maculae
ceruleaflat, indistinct, blue grey or slate
colored macules in the infested area.
All the treatments suggested for pediculosis
capitis are effective. Shaving the area is
another option. Infestation of the eyelids is
treated with twice daily applications of
petrolatum for 7 to 10 days. Alternative
treatments include anticholinesterase
(physostigmine) eye ointments, yellow oxide
of mercury, or fluorescein. The simplest
technique for the treatment of eyelid lice is
direct removal of the lice and nits with a fine
forceps. Cryotherapy may provide a fast cure.
Application of 1% gamma benzene
hexachloride cream and pyrethrin ointment
are other options.

Larva migrans eruption is usually caused by


filariform larvae of the dog and cat
hookworms, mainly Ancylostoma braziliensis
and rarely A. caninum, Uncinaria stenochepala,
Bunostomum phlebotomun, or the human
larvae of Necator americanus and Ancylostoma
duodenale.
Adult hookworms live in the intestines of
dogs and cats and their ova are deposited in
the animals feces. Under favorable
conditions of humidity and temperature, the
ova hatch into infective larvae, which will
penetrate human skin. Sandy, warm, moist,

PAPULAR URTICARIA
Papular urticaria occurs as episodic,
symmetrically distributed, pruritic,
urticarial papules that are caused by bites

Fig. 9.21: Larva migrans eruption serpentine


erythematous tract of larva migrans over the thigh of
a child

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Essentials in Dermatology

80

shaded areas are particularly favorable and


humans acquire numerous infections.
It is commonly known as creeping eruption
for its distinctive feature that the lesion creep
or migrate and is due to the presence of
moving parasite in the skin. An irregularly
linear, thin, raised burrow, 2-3 mm wide,
manifests this exceedingly itchy eruption
(Fig. 9.21). The larva moves a few mm to a
few cm per day. The eruption is self-limited
because humans are abnormal hosts.
The feet and buttocks are the areas most
commonly involved.
Larva migrans can be accompanied by
Loefflers eosinophilia, particularly in severe
infestations.
The classic clinical picture of wandering,
serpentine and itchy lesion is easily
recognized.
Biopsy in larva migrans is of little value as
the larvae have advanced beyond the clinical
lesions.
Differential diagnosis is granuloma
annulare, migratory myiasis and larva
currens. Larva currens caused by filariform
larvae of Strongyloides stercoralis is
characterized by wheal and flare response
along the path of larvae, especially around
anus and buttocks. The linear or serpiginous
tracks of urticarial papules move at the rate
of several cm/ hour. Demonstration of larvae
of Strongyloides spp. in faeces confirms the
diagnosis.
The treatment of choice is the topical
application of 10% thiabendazole (not
available in India). Even the oral preparation
of thiabendazole or two tablets of 500 mg
thiabendazole crushed and formulated in
10 g petrolatum may be applied topically
twice daily. Systemic use of thiabendazole,
though effective, is contraindicated due to its
possible poorly tolerated side effects.
Ivermectin, in a single dose of 200 microgram

Fig. 9.22: Myiasismaggots in chromomycosis


lesion on the hand

per kg body weight seems best. Albendazole


400 mg daily by mouth for 3 days is safe and
often effective. Flubendazole is currently at
an experimental stage, appears to offer good
prospects.

MYIASIS
Myiasis is the presence of fly larvae
(maggots) in the tissue.
Usually they need an entry site, such as a
chronic ulcer (trophic ulcer of leprosy) or
wound.
Most maggots only eat necrotic debris,
although some attack healthy tissue.
Clinically, myiasis is classified according to
the part of the body affected-cutaneous
myiasis (Fig. 9.22) (wound, furuncular),
nasopharyngeal myiasis, ophthalmomyiasis,
intestinal and urogenital myiasis.
Therapy-remove the maggots either after
anesthetizing the area or suffocating them
with turpentine oil or petrolatum.

LEISHMANIASIS
Leishmaniasis is a parasitic infection caused by
many species of the protozoa Leishmania,
manifested clinically as four syndromes.

Infestations

Fig. 9.23: Cutaneous leishmaniasis


noduloulcerative lesion over the forearm

Old world typeCutaneous leishmaniasis.


New world typeMucocutaneous leishmaniasis.
Diffuse Cutaneous Leishmaniasis
Visceral leishmaniasisKala-azar.
Transmitted by infected female sand fly
Old world leishmaniasis by Phlebotomus
spp. and New world leishmaniasis by
Lutzomyia, Psychodopygus spp.
Life cycle: The parasite exists in two forms:
a. Amastigote form in vertebrate host in
cells of reticuloendothelial system or
dermis. It has no flagellum.
b. Promastigote form in gut of female sand
fly and in culture media. It is motile with
an anterior flagellum.
Oriental sore (Delhi boil, Baghdad boil) is
an old world cutaneous leishmaniasis caused
by Leishmania major, seen in Rajasthan and
adjoining areas in India.
Unclothed parts of the body such as face,
neck, and forearms are usually involved. It

begins as papule that enlarges, ulcerates and


becomes crusted nodule with surrounding
induration (Fig. 9.23). Satellite lesions may
occur around the main lesion. Most of the
lesions heal by 6 months.
Post kala-azar dermal leishmaniasis (PKDL)
occurs in 20% of the patients treated for
visceral leishmaniasis (Kala-azar) caused by
Leishmania donovani. It is mainly confined to
West Bengal, Orissa, Jharkhand and Bihar in
India. The lesions appear after a year of
therapy and manifest as hypopigmented
macules, papules and nodules over the trunk
and face. They closely resemble lepromatous
leprosy, secondary syphilis and yaws.
Espundia is a type of mucocutaneous
leishmaniasis caused by Leishmania
braziliensis.
Chiclero ulcers are caused by Leishmania
mexicana.
Diagnosis is by demonstration of amastigotes
inside macrophages from the lesion on a slit
skin smear and culture in NNN media.
Treatment of cutaneous leishmaniasis.
A. Local therapyHeating the sore
(40-42C), cryotherapy,
B. Local infiltration of 1-2 ml of sodium
stibogluconate or meglumine antimoniate,
C. Systemic therapy: AntimonialsSodium
stibogluconate or meglumine antimoniate
20 mg/kg/day for 14-21 days. Pentamidine isethionate- 4 mg salt / kg
weekly doses may be used.
D. Other drugs: Amphotericin B, ketoconazole, itraconazole, dapsone,
rifampicin, etc.

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10

Papulosquamous Disorders

Papulosquamous disorders are characterized by


raised, scaly papules and plaques, include
psoriasis, lichen planus, pityriasis rosea, lichen
nitidus, pityriasis rubra pilaris and other
conditions. These are discussed below.

PSORIASIS
Psoriasis is a common chronic disfiguring,
inflammatory and proliferative epidermal
skin disorder mediated by T cells that affects
approximately 1 to 3% of the world
population.
Multifactorial inheritance most likely, a
family history of psoriasis is found in 30% of
patients.
The histocompatibility antigen HLA Cw6 is
most strongly associated with psoriasis and
the coexistence of HLA B17 or B27 portends
more severe skin disease or associated
psoriatic arthritis.
The pathogenesis of psoriasis remains
unclear. The initial reaction is possibly an
intrinsic defect of keratinocytes with
increased cytokine production which leads
to expansion of CD45RO + T cells with
resultant production of type 1 cytokines.
Subsequently, it results in epidermal
proliferation, migration of neutrophils into

the epidermis, and proliferation of vessels in


the papillary dermis.
The time necessary for psoriatic epidermal
cell to travel from the basal cell layer to the
surface and be cast off is 3 to 4 days, in
marked contrast to the normal 26 to 28 days.
Precipitating factors Stress and anxiety,
alcohol and smoking, drugs (chloroquine,
lithium, betablockers, ACE inhibitors,
NSAIDs, etc), sudden withdrawal of
systemic steroids, irritants, infections.

Classification of Psoriasis
1. Based on Morphological Types
Chronic stable plaque psoriasis (psoriasis
vulgaris) (Figs 10.1 and 10.2)
Guttate psoriasis
Pustular psoriasis
Erythrodermic psoriasis
Rupioid, elephantine and ostraceous
psoriasis.
2.

Based on Site of Involvement


Scalp psoriasis
Flexural (inverse) psoriasis (Fig. 10.3)
Nail psoriasis
Palmoplantar psoriasis
Genital psoriasis
Psoriatic arthritis.

Papulosquamous Disorders
3.

Atypical Forms of Psoriasis


Linear and zonal lesions
Follicular
Photosensitive
Seborrheic psoriasis
Annular psoriasis
Circinate psoriasis
Nummular psoriasis
Serpiginous psoriasis
Geographic psoriasis
Mucosal lesions annular plaques, diffuse
areas of erythema, geographic tongue; very
rare
Ocular lesions blepharitis, conjunctivitis,
keratitis, xerosis, symblepharon, trichiasis,
uveitis; extremely rare.

size, sharply circumscribed, dry, and usually


covered with layers of silvery white,
micaceous scales (Figs 10.1 and 10.2).
Grattage test involves scrapping a scaly
lesion to look for type of scales. Silvery white

Clinical Features
Most lesions of psoriasis are asymptomatic.
Its typical age of onset is in the third decade,
though it may develop at any time from birth
onward.
Erythematous papules and plaques
characterize it. The lesions are of variable

Fig. 10.2: Psoriasis vulgarisplaque lesions over


the trunk

Fig. 10.1: Psoriasis vulgarisplaque lesions over


the extensor surface of limbs and trunk

Fig. 10.3: Flexural psoriasispsoriatic plaque


involving axilla

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Essentials in Dermatology

84

micaceous scales are found on doing grattage


test in psoriasis.
Auspitzs sign is typical of psoriasis. It
consists of three components:
1. Silvery white micaceous scales on
scrapping (due to parakeratosis)
2. Shiny membrane called Bulkleys
membrane on continued scrapping (due
to suprapapillary thinning of epidermis)
3. Bleeding points on removal of membrane
(due to dilated capillaries in papillary
dermis).
Extensor surfaces of the extremities
(Fig. 10.1), the scalp (Fig. 10.4) and lumbosacral region are commonly involved.
Lesions of active psoriasis often appear in
areas of epidermal injury-Koebner phenomenon.
An acute variant, guttate or eruptive
psoriasis is often seen in younger patients
and is characterized by an abrupt eruption
of small drop shaped lesions. It is associated
with acute group A beta hemolytic
streptococcal infection of pharynx in the
preceding 7 to 10 days.

Fig. 10.4: Psoriasis vulgarislesions extending


beyond hair line

Nail involvement is common (50% of


psoriasis patients). The most frequent
alteration of nail plate surface is the
presence of pits (Fig. 10.5). Nail matrix
changes in psoriasis are pitting, longitudinal
ridging, grooves, leukonychia, erythema of
lunula (mottled erythema on lunula due to
matrix inflammation), thickening of nails,
crumbling of nail plate and trachynonychia.
Nail bed changes include subungual
hyperkeratosis, distal onycholysis, salmon
(oily) patches, splinter hemorrhages (thin red
or black longitudinal lines in distal portion
of nail due to psoriatic involvement of nail
bed capillaries, which are longitudinally
oriented). Paronychia results from
involvement of periungual skin of proximal
nail fold with retention of scales between
ventral nail fold and nail plate.
Psoriatic arthritis is of various types Distal
interphalangeal joint arthritis, asymmetrical
oligoarthritis, polyarthritis (rheumatoid
arthritis like), arthritis mutilans and
predominantly axial arthritis (psoriatic
spondylitis and/ or sacroiliitis). Psoriatic

Fig. 10.5: Psoriasis vulgarisnail pitting

Papulosquamous Disorders
arthritis characteristically involves the
terminal interphalangeal joints (Fig. 10.6), but
frequently the large joints are also affected,
resembling rheumatoid arthritis. However,
the rheumatoid factor is absent.
In severe cases, the disease may affect the
entire skin and presents as psoriatic
erythroderma.
Psoriasis rarely presents as generalized
pustular psoriasis (von-Zumbusch typeacute exanthematic type (Fig. 10.7) or
generalized pustular psoriasis of pregnancyImpetigo herpetiformis). Localized pustular
psoriasis occurs as acrodermatitis continua
of Hallopeau (localized to the distal portions
of the hands and feet) (Fig. 10.8) or pustulosis
palmaris et plantaris (chronic, relapsing
disorder occurring on the palms, soles, or
both).

Diagnosis
Psoriasis may be confused with seborrheic
dermatitis, secondary syphilis, dermatophyte
infections, cutaneous lupus erythematosus,

Fig. 10.6: Psoriatic arthritischaracteristically


involving distal interphalangeal joint leading to swan
neck deformity

eczema, lichen planus, pityriasis rosea,


Bowens disease or lichen simplex chronicus.
Diagnosis is mostly clinical supplemented at
times of doubt by histopathology of skin
lesion.

Fig. 10.7: Generalized pustular psoriasisgeneralized pustular eruption in a child

Fig. 10.8: Acrodermatitis continuanail dystrophy


and finger tip involved by pustular psoriasis

85

86

Essentials in Dermatology
Histopathology: The fully developed lesion
of psoriasis shows:
1. Hyperkeratosis and parakeratosis.
2. Within parakeratotic areas of the horny
layer, accumulations of neutrophils
forms, which are called as Munro microabscesses.
3. Hypo or absent granular layer.
4. Regular acanthosis.
5. Spongiform pustule of Kogoj
neutrophils accumulation in Malpighian
layer
6. There is regular elongation of the rete
ridges with thickening in their lower
portion, looking like elephant feet.
7. There is thinning of the supra papillary
portion of the epidermis.
8. The dermal papillae contain enlarged and
tortuous capillaries that are very close to
the skin surface and impart a
characteristic erythematous hue to the
lesions.
9. Sparse lymphocytic infiltrate in the upper
dermis.

Differential Diagnosis of Psoriasis


Seborrhoeic dermatitis In the scalp,
lesions are lighter in color, less well defined,
covered with dull or branny or greasy scales,
with absence of corona.
Pityriasis rosea Papular or annular
eruption usually confined to upper arms,
trunk and thighs. Herald patch is followed
by disseminated eruption, which has
christmas tree pattern. Typically individual
lesion shows collarette of scaling. Duration
of disease is few weeks (6-8 weeks), and it
has self limiting course.
Pityriasis rubra pilaris resembles closely the
psoriasis especially in the erythrodermic
phase. The follicular accentuation, focal areas
of sparing (islands of normal skin),
sometimes more salmon coloration of
pityriasis rubra pilaris and the acquired

palmoplantar keratoderma with yellow


orange tinge help to differentiate the
condition clinically; biopsy sections of
pityriasis rubra pilaris may show follicular
plugging, parakeratotic shoulder and
perifollicular lymphohistiocytic infiltrate.
Dermatophyte infection (tinea corporis)
Itchy annular erythematous plaque/s with
peripheral scaling, KOH examination
demonstrates dermatophytes.
Discoid lupus erythematosus Discrete
erythmatous plaques on face and scalp
(usually on sun-exposed areas), associated
with atrophy, scaling and alopecia. Scales of
discoid lesions are grayish and adherent.
There is follicular plugging and carpet-tack
sign.
Subacute lupus erythematous Presents
with annular erythematous or psoriasiform
lesions associated with systemic component,
may have other components of systemic
lupus erythematosus.
Eczema at times develops a psoriasiform
appearance especially on the legs.
Hyperkeratotic eczema of palms is a common
cause of misdiagnosis. Color, scratch evoked
scaling and well defined margins are
suggestive of psoriasis and nail changes may
be diagnostic.
Lichen planus It involves flexor surface of
forearm and wrists, and shins and ankles.
Pruritic purple flat topped papules and
plaques with scanty and tightly adherent
scales. Scalp is much less frequently involved.
Nails are longitudinally ridged, thickened or
thinned out, with pterygium a characteristic
finding. No nail pitting. Linear lichen planus
may mimick linear psoriasis but the
individual lesion characterstics helps in
differentiating.
Secondary syphilis Asymptomatic
eruption, brownish sparse scales, generalized
lymphadenopathy, mucous patches, condyloma lata, positive serological test for syphilis.

Papulosquamous Disorders
Drug eruptions Always take a drug
history; many drug reactions are
psoriasiform, and in some instances lead to
true psoriasis in susceptible host.
Pityriasis lichenoides chronica It can
closely resemble guttate psoriasis but the
lesions are less evenly scattered, have
brownish red or orange brown color and are
capped by an opaque soft mica like scale.

Treatment
It is important to emphasize that psoriasis is
a treatable condition. The treatment pyramid
for psoriasis is shown in Fig. 10.9.
Despite virtual explosion in therapeutic
options, the management of psoriasis
remains a challenge to clinicians.

Topical Therapies
Emollients Such as coconut oil, vaseline,
or liquid paraffin.
Coal tar (2, 5, 10% ointment) It may be used
effectively as monotherapy or in combination
with other treatment modalities.
Goeckerman therapy consists of black crude
coal tar application all over the patients
body. This tar is left on for 24 hours per day.
UVB phototherapy is administered before the
tar is applied or after it is washed off. The
Goeckerman regimen remains an intensive
daily therapy that is usually conducted for a
period of several weeks.
Anthralin (0.1 to 10% cream or ointment)
It is moderately effective and quite safe in
plaque psoriasis. The major side effect is
staining of skin and clothing and occasional
irritation. Ingram regimen consists of a
combination of anthralin and UVB
phototherapy. Short contact treatment of 30
minutes or less is likely to reduce irritation.
Topical steroids/ intralesional steroidsTopical corticosteroids are used for treatment
over face and neck, flexures and genitalia,
where tar or anthralin would cause irritation
and cannot be used. Systemic steroids

generally not recommended due to the wellknown risk of rebound and pustular
conversion.
Vitamin D analogues are both naturally
occurring calcitriol (1, 25-dihydroxyvitamin D 3 ) and synthetic analoguescalcipotriol, tacalcitol, maxacalcitol. Vitamin
D molecule is dispensed as ointment, cream
or solution and has risk of producing
hypercalcemia in less than 1% of cases.
Tazarotene (0.1 to 0.05% gel) It is a
modified vitamin A molecule formulated as
a topical agent. It is recommended for use in
patients with total body surface involvement
of 20% or less.
Topical methotrexate gel-used for
palmoplantar psoriasis with limited efficacy.
Tacrolimus (0.03, 0.1%) effective for facial
plaques and inverse psoriasis.

Medications Used in Phototherapy


Concurrent use of topical or oral medications,
psoralen (5 methoxy psoralen, 8 methoxy
psoralen, trimethyl psoralen) along with
UVA or UVB phototherapy is done.
Psoralen along with UVA from sunlight
(PUVASOL).
Narrow band UVB.
Methotrexate and PUVA combination
(Me-PUVA).
Retinoids and PUVA combination (RePUVA).
Excimer LASER (308 nm).
Systemic Medications for Psoriasis
Methotrexate (0.4 to 0.6 mg per kg body
weight) Used for severe cases of psoriasis,
taken orally just once a week. It is an effective
and well tolerated drug in appropriately
selected patients who are receiving folate
supplementation, and who are adequately
monitored for potential toxicities.
Cyclosporine (3-5 mg per kg body weight).
Cyclosporine has dose-related nephrotoxicity
and hypertension that impede its use as a

87

Essentials in Dermatology

88

long-term agent for most patients.


Nevertheless, cyclosporine is an effective
drug for rapid clearing in most patients.
Cyclosporine, when used in the appropriate
patient at the appropriate dose (usually 2.5
to 5.5 mg/kg daily), is an excellent bridging
agent that can be used safely for periods of
212 months.
Etretinate and Acitretin (0.5-1 mg per kg
body weight) They are the treatment of
choice for generalized pustular psoriasis.
Acitretin is less effective when used as
monotherapy for plaque psoriasis.
Hydroxyurea (maximum up to 500 mg three
times a day).
Sulfasalazine (3-4 gm/day).
Mycophenolate mofetil ( 1-2 gm/day, can
increase maximum up to 4 gm/day).
Oral tacrolimus ( 0.05- 0.15 mg/kg body
weight per day).
Fumaric acid esters 30 mg per day up to 240
mg three times a day.
Many of these medications are used in
combination and rotated periodically.
HIV associated psoriasis may respond to
zidovudine based HAART regimen.

Biologic Agents
Biologics are agents that selectively block the
immunologic steps in the pathogenesis of
psoriasis. Alefacept, efalizumab, etanercept, and
infliximab are currently approved for the
treatment of adults with moderate to severe
plaque psoriasis, and phase 3 trials for
adalimumab are ongoing (Table 10.1).
All biologics approved to date for the
treatment of psoriasis are recommended for
patients who are candidates for systemic or
photo-therapy.
Table 10.1: Biologic agents and their
mechanism of action

Strategy

Biologic agents

Reduction of the number


of pathogenic T cells

Alefacept, denileukin
diftitox

Inhibition of T-cell
activation and migration

Efalizumab, daclizumab,
siplizumab, CTLA4Ig,
galiximab,

Modulation of the immune


system

Ilodecakin, oprelvekin

Blockage of the activity of


inflammatory cytokines

Etanercept, infliximab,
adalimumab, ABX-IL8

Fig. 10.9: Treatment pyramid of psoriasis

Papulosquamous Disorders
Efalizumab is favorable in patients with high
risk of latent tuberculosis or evidence of
demyelinating disease.
Infliximab is advantageous where rapid
disease control is required (e.g. unstable
erythroderma).
Etanercept is the biologic of choice in stable
psoriasis where a TNF--blocking strategy is
appropriate.
Some biologics are also approved for
treatment of psoriatic arthritis; thus, patients
with psoriatic arthritis may benefit from a
biologic for both the articular and dermatologic manifestations.

Cutaneous lesions occur in about half of


affected patients. The cutaneous eruption has
limpet like scales (Figs 10.10 and 10.11).
Lesions have a predilection for the glans
penis (balanitis circinata) (Fig. 10.12), the
palms and soles (keratoderma blennorrhagica) and the subungual areas.
HLA B 27 is present in about 80% of cases
Most cases of Reiters disease are probably
caused by infection with micro-organisms
that infect urogenital or gastrointestinal
systems such as Chlamydia trachomatis,

Course and Prognosis


The course of psoriasis is prolonged but
unpredictable.
REITERS DISEASE
Reiters disease predominantly affects young
men and consists of the triad of urethritis,
arthritis, and conjunctivitis. As few patients
present with classic triad, the American
College of Rheumatology recognizes criteria
for limited manifestations of this syndrome
including peripheral arthritis of more than
one month duration in association with
urethritis, cervicitis or bilateral conjunctivitis.

Fig. 10.10: Reiters diseasetypical limpet like scales

Fig. 10.11: Reiters diseasetypical lesions over legs

Fig. 10.12: Reiters disease-circinate balanitis

89

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Essentials in Dermatology
Ureaplasma urealyticum, Shigella, Salmonella,
etc.
Differential diagnosis includes rheumatoid
arthritis, ankylosing spondylitis, gout,
psoriatic arthritis, gonococcal arthritis, acute
rheumatic fever, etc.
Mucocutaneous lesions are generally self
limited and clear with topical steroids.
Refractory lesions are managed like psoriasis.
Joint disease may require NSAIDS,
methotrexate or biologics (infliximab).

LICHEN PLANUS
Lichen planus (Greek leichen, tree moss;
Latin planus, flat) is a subacute or a chronic
dermatosis that may involve skin, mucous
membranes, hair follicles and nails.
The cause of lichen planus is unknown, but
several etiologies have been proposed. It is
likely that both endogenous-genetic and
exogenous-environmental components such
as drugs or infection may interact to elicit
the disease.
The prevalence of chronic liver diseases,
including primary biliary cirrhosis, alcoholic
cirrhosis, hepatitis B, and especially hepatitis
C, is increased.
HLA-B8 is more common in patients with
oral lichen planus as the sole manifestation,

Fig. 10.13: Lichen planusviolaceous, flat-topped


papules

and HLA-Bw 35 is more strongly associated


with cutaneous lichen planus.
At least two-thirds of cases occur between
the ages of 30 and 60 years.
Cutaneous eruption is characterized by
small, flat topped, shiny, polygonal,
violaceous papules that may coalesce into
plaques (Fig. 10.13)
The papules often show a network of white
lines known as Wickhams striae.
Koebner phenomenon commonly seen (Fig.
10.14).
Itching is usually pronounced.
The four Ps purple, polygonal, pruritic,
papule- is the abbreviation used to recall the
constellation of symptoms and skin findings
that characterize lichen planus.
The disease has a predilection for the flexor
surfaces of the forearms (Fig. 10.15), legs
(Fig. 10.16), trunk (Fig. 10.17), and the
genitalia including the glans penis (Figs
10.18 to 10.20).

Fig. 10.14: Lichen planuswrist involved, Koebner


phenomenon also seen

Papulosquamous Disorders
The oral lesions of lichen planus are
frequently found, either as sole manifestation
of the disease or associated with cutaneous
involvement. Most often consist of a lacy,
reticular network of coalescent papules over
the buccal (Fig. 10.21) or glossal mucosa.
Besides this, it forms plaque like, atrophic,
papular, erosive and bullous lesions.
The nails are involved in about 10% of cases
and show roughening, longitudinal ridging,
thinning and dystrophy. Pterygium

Fig. 10.17: Lichen planustrunk involved by typical


violaceous papular lesions

Fig. 10.15: Lichen planusflexor aspect of arm


involved

Fig. 10.18: Lichen planusan annular lesion over


the glans penis

Fig. 10.16: Lichen planushypertrophic violaceous


papules and plaques over legs

Fig. 10.19: Lichen planusmale genitalia involved


by lichen planus

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Essentials in Dermatology

Fig. 10.20: Lichen planusfemale genitalia involved


by lichen planus

Fig. 10.21: Lichen planuslacy white network over


the buccal mucosa

Fig. 10.22: Lichen planuspterygium unguium


formation in some finger nails

Fig. 10.23: Lichen planustranseversely aligned


lichen planus along Blaschkos lines

formation is a frequent finding (Fig. 10.22).


Common variants of lichen planus (LP) are
classified on the basis of:
A. Configuration
(annular,
linear,
zosteriform/along Blaschkos lines (Fig.
10.23),
B. Morphology of lesions (hypertrophic,
atrophic, vesicobullous, erosive,
ulcerative, follicular [planopilaris], actinic
(Fig. 10.24), LP pigmentosus, perforating,
guttate),
C. Site of involvement (palms and soles,
mucous membranes, nails, scalp),

D. Special forms (drug induced, overlap


syndrome of lichen planus/lupus
erythematosus, LP pemphigoides).

Diagnosis
The appearance of the typical papule of
lichen planus is usually sufficient to make the
correct diagnosis.
Histopathology: Microscopic features, similar
to the gross morphology, are diagnostic. The
two major pathologic findings in lichen
planus are basal epidermal keratinocyte
damage and lichenoid-interface lympho-

Papulosquamous Disorders

Fig. 10.24: Lichen planus actinicustypical


hypopigmented halo around pigmented violaceous
lesions over the face

cytic reaction. The histologic features are


summarized here:
1. Hyperkeratosis
2. Wedge shaped hypergranuloses which is
responsible for the Wickhams striae seen
clinically
3. Irregular acanthosis
4. Damage to basal cell layer
5. The rete ridges are pointed at their lower
end and the papillae between rete ridges
are often dome shaped thus resembling
the old styled bridges on rivers having
tapering down pillars and domes in
between. This pattern is also called as
saw-tooth pattern.
6. A band-like dermal lymphocytic
infiltrate closely hugs the epidermis.
7. Max-Joseph spaces are seen in some cases
as a sub-epidermal clear zone.
Melanin incontinence and vascular
inflammatory reaction probably give purple
colour to LP lesions.

Differential Diagnosis
Classic lesions: Lichenoid drug eruption
(large scaly lesions in sun-exposed areas,
devoid of Wickham striae, residual
pigmentation common), lichen nitidus

(asymptomatic pinhead sized shiny papular


lesions, rarely involves mucous membranes),
secondary syphilis, pityriasis lichenoides et
varioliformis acuta, early pityriasis rubra
pilaris.
Hyperkeratotic lesions: Lichen simplex
chronicus, prurigo nodularis, lichen amyloidosis, warts.
Linear lesions: Lichen striatus, linear
epidermal naevus, linear psoriasis.
Annular lesions: Granuloma annulare,
secondary syphilis, psoriasis
Lichen planopilaris: For early lesionskeratosis pilaris, other follicular keratoses,
Dariers disease, early pityriasis rubra pilaris.
For advanced lesions-discoid lupus erythematosus, and other forms of scarring
alopecia.
Wide-spread erosive oral lesions must be
differentiated from those of candidiasis
(curdy white deposits which on removal
leaves an erythematous base. KOH scrapping
shows budding yeasts, pseudohyphae),
aphthous ulcers (well circumscribed shallow
ulcers with a necrotic gray centre and an
erythematous halo. It usually heals in around
six weeks) pemphigus (ill defined irregular
buccal or palatal non healing painful
erosions. Other mucosal sites may also be
involved. Nikolskys sign positive), cicatricial
pemphigoid (vesicles, persistent extensive
erosions, desquamative gingivitis with
eroded bleeding gums, adhesions between
buccal mucosa and alveolar process and
around uvula and tonsillar fossae,
involvement of other mucosal sites including
conjunctiva, genital mucosa, larynx and
esophagus, and decreased mouth opening
due to fibrosis), carcinoma (usually
malignant ulcers are painless unless
secondarily infected. On palpation
induration of base may be present), and
erythema multiforme (usually involves lips,

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Essentials in Dermatology
palate and gingival. On lips, target lesions
may be identified. Vesicles, erosions and
crusting are present over oral mucosa.
Associated skin lesions- target lesions may
be seen over acral extensor sun-exposed
areas).

Treatment
Topical therapy: Topical glucocorticoids are
typically used for mucosal and limited
cutaneous disease.
Intralesional therapy: Intralesional triamcinolone acetonide (5 to 10 mg/mL) is
effective in treating oral and cutaneous lichen
planus.
Systemic therapy:
1. Systemic glucocorticoids are often useful and
effective in doses greater than 20 mg/day
(e.g. 30 to 60 mg prednisolone) for 4 to 6
weeks with subsequent taper over 4 to 6
weeks.
2. PUVA photochemotherapy is usually
successful in generalized cutaneous lichen
planus.
3. The systemic retinoids demonstrate antiinflammatory activity and have been used in
the treatment of lichen planus.
4. Cyclosporine (500 mg) rinses in oral lichen
and systemic cyclosporine in recalcitrant
lichen planus has been used successfully.
5. Other agents used in lichen planus are
dapsone, griseofulvin, cyclophosphamide,
methotrexate, phenytoin and extracorporeal
photochemotherapy.
In mucosal lichen planus, topical corticosteroids, tetracycline, betamethasone mouthwashes 0.5 mg 3-4 times daily, topical tretinoin
gel, cyclosporine mouth rinses, tacrolimus, and
pimecrolimus have been used. Maintenance of
good oral hygiene and replacement of amalgam
or gold dental restorations with composite
material is frequently of considerable benefit.
Erosive oral lichen planus may respond to oral
dapsone.

Course
Lichen planus is a benign disease with
spontaneous remissions and exacerbations.
Apart from hypertrophic lichen planus, most of
LP lesions tend to involute after several months
to a year.
LICHEN NITIDUS
Lichen nitidus is a chronic dermatitis usually
asymptomatic which begins commonly in
childhood or early adulthood
It is characterized by minute, round, flat or
dome shaped, shiny, flesh colored papules
2 to 3 mm in diameter that may occur in
groups
Predominantly, the arms, trunk (Fig. 10.25)
or penis (Fig. 10.26)are involved
Koebner phenomenon may be observed (Fig.
10.27)
The histology of a typical papule is
characteristic. A circumscribed epithelioid
cell granuloma is situated immediately below
the epidermis. The rete ridges at the margin
of the infiltrate are elongated and tend to
encircle it claw clutching a ball
appearance.
Differential diagnosis:
1. Keratoses pilaris (horny follicular papules
on extensor surfaces),

Fig. 10.25: Lichen nitidusflat, shiny, flesh colored


papules on the trunk

Papulosquamous Disorders
2. Lichen scrofulosorum (grouped follicular
papules in small patches on trunk),
3. Lichen planus,
4. Sarcoidosis,
5. Disseminated granuloma annulare,
6. Eruptive xanthomas,

7. Plane warts (more variable in size, have


verrucous surface and present with fewer
lesions with few anatomic sites involved)
As the disease is often asymptomatic and
eventually self-limiting, no treatment is
required in most cases
Topical steroids may be recommended if
treatment is demanded.

PITYRIASIS ROSEA

Fig. 10.26: Lichen nitidusflat, shiny, flesh colored


papules on the glans penis

Fig. 10.27: Lichen nitidus- over the arm showing


Koebner phenomenon

Pityriasis rosea is a self limited dermatitis


lasting from 4 to 7 weeks.
The etiology is unknown, though a viral
origin has been suggested.
Predominantly occurs in adolescents and
young adults.
May be asymptomatic or pruritic (50% of
cases).
It frequently starts with herald patch (mother
patch, plaque primitive, primary medallion,
primary plaque) (most commonly on the
trunk) followed by a disseminated eruption
(the trunk and proximal extremities) within
several days to 2 weeks (Fig. 10.28).
Sometimes, herald patch may be missing, or
present as double or multiple lesions often
close together.

Fig. 10.28 Pityriasis roseaherald patch with a few


daughter lesions showing collarette of scaling

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Essentials in Dermatology
Round to oval salmon colored patches
following the lines of cleavage (Christmas
tree like pattern) and showing peripheral
attached thin cigarette paper like scales
(collarette of scaling) (Fig. 10.28). When
individual lesion is stretched along the long
axis, the scales tend to fold across the lines of
stretch, so called hanging curtain sign.
Variants of pityriasis rosea are vesicular,
inverse (Fig. 10.29), cervicocephalic, pityriasis
rosea gigantea (pityriasis circinata et
marginata of Vidal), pityriasis rosea urticata,
purpuric pityriasis rosea, pityriasis rosea
perstans, pustular pityriasis rosea, and
eczematous pityriasis rosea.

white micaceous scales), parapsoriasis, pityriasis


lichenoides chronica, lichen planus, and, most
importantly, secondary syphilis (maculopapular
lesions, no herald patch, genital and oral mucosal
involvement).

Treatment
Most patients require no treatment.
Topical corticosteroids or short course of oral
corticosteroid may be required in severe
cases.
Oral erythromycin may be helpful.
Itching alleviated with antipruritic lotions or
antihistamines.
PITYRIASIS RUBRA PILARIS (PRP)

Diagnosis
Usually easily diagnosed by its morphology
and distribution.
Differential Diagnosis
Pityriasis rosea must be differentiated from
tinea corporis, tinea versicolor (well defined
hypopigmented coalescing macules with branny
scales), drug eruptions (acute onset without
herald patch, pruritic protracted rash and a
tendency for lesions to become lichenoid),
psoriasis (papules and plaques with silvery

Pityriasis rubra pilaris is an erythematous


squamous disorder characterized by
seborrheic dermatitis like rash which
progresses cephalo-caudally, follicular
plugging and perifollicular erythema that
coalesces to form orange red scaly plaques
(Fig. 10.30) that frequently contain islands of
normal appearing skin (Fig. 10.31), proximal
phalanges showing typical follicular
plugging and palms and soles keratotic
sandal (palmoplantar keratoderma)
(Fig. 10.32).

Fig. 10.29: Inverse pityriasis rosea lesions over


the face

Fig. 10.30: Pityriasis rubra pilaris follicular papular


lesions coalescing to form plaques over the trunk

Papulosquamous Disorders

Fig. 10.31: Pityriasis rubra piliarisislands of


normal skin in a patient having erythroderma

Fig. 10.32: Pityriasis rubra pilarispalmoplantar


keratoderma (keratotic sandal)

It may progresses to erythroderma.


Both familial and acquired forms of the
disorder have been described.

Types of PRP
Type Iadult onset classical, most common
type.
Type IIadult onset atypical.
Type IIIjuvenile onset, classical.

Type IVjuvenile onset, circumscribed.


Type V juvenile onset, atypical.
Type VIHIV associated.
Histopathology reveals hyperkeratosis and
follicular plugging with shoulder of
parakeratosis. Parakeratosis may alternate
both vertically and horizontally producing a
checkerboard pattern.
Differential diagnosis: Closely resembles
psoriasis but prominent facial involvement
or follicular lesions speak against it. Early
scalp involvement is often mistaken for
atopic dermatitis or seborrhoeic dermatitis.
Early diffuse lesions are confused with lichen
planus and pityriasis lichenoides et
varioliformis acuta. Circumscribed pityriasis
rubra pilaris mimics phrynoderma or
keratosis pilaris.
Treatment: Topical therapy with keratolytic
agents, vitamin D analogues and systemic
therapy with vitamin A, acitretin, isotretinoin
(systemic retinoid), methotrexate and
retinoid plus psoralen and ultraviolet A (RePUVA) has been tried with variable efficacy.
Antiretroviral therapy is of value in pityriasis
rubra pilaris like eruption of HIV infection.
The inherited form of the disorder is
persistent throughout life while the acquired
disease usually shows remissions and
exacerbations.

ERYTHRODERMA (EXFOLIATIVE
DERMATITIS)
Erythroderma is a condition which is
characterized by erythema, infiltration and
scaling involving more than 90% of the body
surface area or near universal involvement
of the body (Fig. 10.33).
Erythroderma can be caused by various
dermatological disorders: eczema, psoriasis,
drugs, lymphoma, leukaemia, pemphigus
foliaceus, ichthyosiform erythroderma,
pityriasis rubra pilaris, lichen planus,

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Essentials in Dermatology
In erythroderma where the etiology is
unknown, called as idiopathic erythroderma
(10% of cases).
Chronic erythroderma of unknown origin
with prolonged course in the elderly is called
as red man syndrome.
Szary syndrome manifest with erythroderma, generalized lymphadenopathy and
atypical cells in peripheral smear (more than
10%).
It can be a manifestation of internal
malignancy.

Fig. 10.33: Erythroderma near universal


involvement by erythema, scaling and infiltration

dermatomyositis, dermatophytosis and


crusted scabies.
The drugs causing erythroderma are
phenytoin, carbamazepine, cimetidine,
lithium, gold, chloroquine, isoniazid,
mercury, thiazide, quinidine and pyrazolone
derivatives.

Complications
Erythroderma leads to hemodynamic and
metabolic disturbances such as high out put
cardiac failure, hypothermia, dehydration
and hypoalbuminemia.
Temperature regulation is affected and
patient behaves like a poikilothermic animal.
Death may occur due to cardiac failure,
pneumonia and septicemia.
Management
It includes maintenance of the homeostasis and
treatment of the primary disease.

Eczema

11

Eczema

The word eczema is derived from the Greek


word ekzein meaning to boil out or to
effervesce. Eczema is an inflammatory skin
reaction characterized histologically by
spongiosis with varying degrees of acanthosis,
and a superficial perivascular lympho-histiocytic
infiltrate. The clinical features of eczema may
include itching, redness, scaling and clustered
papulo-vesicles. The condition may be induced
by a wide range of external and internal factors
acting singly or in combination.
The terms dermatitis and eczema are
nowadays generally regarded as synonymous,
although some authors still use the term
dermatitis to include all types of cutaneous
inflammation, so that all eczema is dermatitis,
but not all dermatitis is eczema.

CLASSIFICATION OF ECZEMA
Exogenous Eczema
Irritant dermatitis
Allergic contact dermatitis
Photoallergic contact dermatitis
Eczematous polymorphic light eruption
Infective eczema
Dermatophytide
Post-traumatic eczema.

Endogenous Eczema
Atopic dermatitis
Seborrheic dermatitis
Asteatotic eczema
Discoid eczema
Pompholyx
Pityriasis alba
Stasis dermatitis
Hand eczema
Lichen simplex chronicus
Prurigo nodularis
Lichen striatus
Juvenile plantar dermatosis
Metabolic eczema or eczemas associated with
systemic disease
Eczematous drug eruptions.
It must be remembered that, endogenous and
exogenous factors may co-exist. For example,
hand eczema, which is an endogenous eczema,
is often aggravated by exogenous factors. Some
of the important endogenous (Atopic dermatitis,
seborrheic dermatitis, nummular eczema,
pompholyx, pityriasis alba, stasis dermatitis,
asteatotic eczema, lichen simplex chronicus,
prurigo nodularis, lichen striatus) and exogenous
eczema (Contact dermatitis, infective eczema/
dermatitis, polymorphous light eruption) are
discussed as follows.

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Essentials in Dermatology

ATOPIC DERMATITIS
Atopic dermatitis (AD) or eczema is a
common chronic or relapsing dermatitis
characterized by severe pruritus, occurring
primarily in infants and children.
The age of onset is between 2 and 6 months
in the majority of cases, but it may start at
any age, even before the age of 2 months in
some cases.
The disease arises as a result of a complex
interplay between various genetic,
immunological and environmental factors.
The environmental factors include (a)
physical factors like sweating, climate, warm
surroundings, detergents and soap, synthetic
or woollen fabrics, cigarette smoke,
(b) psychological factors, (c) food items
(including tomato, orange and citrus fruits
juices, meat, fish) (d) allergens such as house
dust mite, animal hair, pollen, plants and
others such as Staphylococcus aureus and
release of exotoxins (superantigens) and
saliva in small children.
Majority of cases are associated with a
sensitization to environmental allergens
and increased serum IgE (extrinsic AD), but
about 10-30% of all cases lack any link to
the classical atopic diathesis and are
labelled as intrinsic AD.
There is no laboratory gold standard for the
diagnosis of AD. A detailed history and a
characteristic clinical picture would
establish the diagnosis. Hanifin and Rajka
have laid down certain major and minor
criteria for making a diagnosis of AD.

Must have Three or More Major Findings


Pruritus
Typical morphology and distribution
(Flexural lichenification or linearity in adults;
Facial and extensor involvement in infants
and children).

Chronic or chronically relapsing dermatitis


Personal or family history of atopic dermatitis
(e.g. asthma, allergic rhinitis, atopic
dermatitis).

Plus Three or More Minor Findings


Xerosis
Ichthyosis
Palmar hyperlinearity
Keratosis pilaris
Immediate (type 1) skin test reactivity
Elevated serum IgE level
Early age of onset
Tendency towards extraneous infections
(especially with Staphylococcus aureus and
herpes simplex) or impaired cell mediated
immunity
Propensity towards nonspecific dermatitis of
the hand or foot.
Nipple eczema
Cheilitis
Recurrent conjunctivitis
Dennie -Morgan infraorbital fold
Keratoconus
Anterior subcapsular cataracts
Orbital darkening
Pityriasis alba
Facial pallor or facial erythema
Anterior neck folds
Itch when sweating
Intolerance to wool and lipid solvents
Food intolerance
White dermographism or delayed blanch.
Course of atopic dermatitis may be divided in
three phases:
1. Infantile phase
2. Childhood phase
3. Adult phase
The distribution of lesions varies with age: Face
(Fig. 11.1)and scalp involvement is common in
infants as well as the extensor surfaces of the
extremities and the trunk. In the childhood phase
of AD 18-24 months onwards, eczema is

Eczema

Fig. 11.1: Atopic dermatitisface

Fig. 11.2

Fig. 11.3

Figs 11.2 and 11.3: Atopic dermatitisdiscoid eczematous lesions also


appearing on flexor aspect of limbs in a child

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102

Essentials in Dermatology
observed on the flexural surfaces, including the
neck, antecubital or popliteal fossae, wrists and
ankles (Figs 11.2 and 11.3). There is an adult
phase, which occurs in adolescents and adults,
where lichenification of the flexures and the
hands commonly occurs.
Some important signs: Thinning of lateral
eyebrows, Hertoghes sign is sometimes present.
Hyperkeratosis and hyperpigmentation
producing a dirty neck appearance is also
frequent in atopic individuals. Atopic
individuals often exhibit perioral, perinasal and
periorbital pallor headlight sign.
Differential diagnosis: The diagnosis of atopic
dermatitis (AD) is rarely aided by investigations.
In the individual patient, one must consider a
number of other conditions. Scabies should
always be excluded, and can cause confusion
when superimposed on pre-existing AD. In the
first few months of life, the differentiation of
infantile seborrhoeic dermatitis from atopic
dermatitis can be difficult, although with time
the distinction becomes apparent. The most
useful distinguishing feature between atopic
dermatitis and seborrheic dermatitis is the
increased number of lesions on the forearms and
shins in the former and axillae in the latter. The
development of the lesions solely in the diaper
area favors a diagnosis of infantile seborrheic
dermatitis. Absent to mild pruritus is considered
a significant feature of infantile seborrheic
dermatitis. Immunodeficiency states should
also be considered in infants in whom the disease
is unusually severe, when there are recurrent
systemic or ear infections and if there is failure
to thrive, malabsorption or petechiae. Then
appropriate investigations should be performed,
for example, immunoglobulin levels and
subclasses, IgE levels, white cell count, platelets,
complement levels, and function, and T, B, and
phagocyte numbers and functions. If clinically
appropriate, one may also consider testing for

HTLV-I and human immunodeficiency virus


(HIV).
Treatment: No disease is more complicated to
treat than atopic dermatitis. It is absolutely
essential to counsel and educate the patient and
their parents. Avoidance of exacerbating factors
like wool clothes, house dust mites, detergents,
dietary or aero-allergens, stress, etc. is advised
wherever implicated. The treatment is aimed at
suppressing the symptoms and controlling or
preventing complications. Besides emollients for
routine skin care, topical corticosteroids (TC) are
the mainstay of treatment for AD to control
acute exacerbations and can be used safely if
certain precautions are taken. Topical calcineurin
inhibitors (TCIs: Tacrolimus 0.03% and 0.1% and
Pimecrolimus 1%) are recommended in patients
of AD who are unresponsive to or intolerant to
other conventional therapies, should be applied
over the affected areas twice daily. Tacrolimus
0.03% and 0.1% formulations are recommended
for the use in adults, whereas only 0.03%
formulation is recommended for use in children
aged 2-15 years, often first line for face,
intertriginous areas and genitalia, useful in early
flare, continue treatment till disease clears
(approximately 1 month), and is useful for
maintenance therapy. Systemic corticosteroids
have a limited role in tiding over occasional flares
of severe AD. Sedative antihistamines such as
hydroxyzine and promethazine are preferred to
non-sedative ones to control pruritus. Avoidance
of provoking factors is paramount to achieve
control.
Other treatment modalities include
phototherapy and photochemotherapy,
cyclosporin, evening primrose oil, azathioprine, Chinese herbal medicines, interferons,
thymopentin, human interferon gamma and
plasmapheresis.
Algorithmic approach to management
depending upon severity of AD.

Eczema

1.

Mild AD

Bland emollients and parents/patient counseling


Mild to moderate TCs, followed by TCI or
TCI as first line of therapy
Sedative anti-histamines

2.

Moderate AD

Bland emollients and parents/patient counseling


Initial control with TCs and at the earliest shift to TCIs
Anti-histamines
Narrow band UVB
Systemic corticosteroids for short period

3.

Severe AD

Bland emollients and parents/patient counseling


Anti-histamines
Systemic antibiotics
Oral corticosteroids/cyclosporine/azathioprine

Course and prognosis Atopic dermatitis


follows a highly variable course with
exacerbations and remissions. About 95% of
children with AD remit around puberty, but
relapses m ay occur and the disease may
persist well into adulthood.

SEBORRHEIC DERMATITIS (SD)


Seborrheic dermatitis is a common,
endogenous eczema that is usually easily
recognized.
It affects infants and adults and is often
associated with increased sebum production
(seborrhea) of the scalp and the sebaceous
folliclerich areas of the face and trunk.
Various genetic factors, hormonal factors and
increased colonization of Malassezia may play
a role in the causation and perpetuation.
Seborrheic dermatitis has two age peaks, one
in infancy within the first 3 months of life
and the second around the second to the third
decade of life. Men are affected more often
than women in all age groups.

Classification of Seborrhoeic Dermatitis


Infantile
Scalp (Cradle cap)
Trunk (including flexures and napkin areas)
Leiners disease.

Adult
Scalp:
Dandruff
Inflammatory
Face (may include blepharitis and
conjunctivitis)
Trunk:
Petaloid
Pityriasiform
Flexural
Eczematous plaques
Follicular.
Generalized (may be erythroderma)
In infants, the scalp (the frontal and parietal
scalp regions are covered with an oilylooking, thick, often fissured crust -crusta
lactea (milk crust, or cradle cap) (Fig. 11.4),
face and diaper areas are often involved.
Leiners disease is due to complement C5
deficiency, presents as erythroderma.
Clinically, adult patients develop erythema
and greasy scale on the scalp (with often
pruritus), paranasal areas, eyebrows,
nasolabial folds, central chest and
intertriginous folds (seborrheic areas of the
body). Rarely generalized lesions may occur.
Dandruff is usually the earliest manifestation
of seborrheic dermatitis.

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Essentials in Dermatology

Fig. 11.4: Seborrheic dermatitis-cradle


cap in an infant

Fig. 11.5: Seborrheic dermatitis in an adultgreasy


scaling with erythema involving eyebrows, nasolabial
folds and beard area in a HIV case

Corona seborrheica, when SD on the scalp


extends beyond the frontal hairline onto the
forehead.
Sebopsoriasis: Early stage of scalp psoriasis
may closely resemble seborrheic dermatitis
with similar clinical and histological features.
Some believe that seborrheic dermatitis may
be a precursor of scalp psoriasis.
SD occur more commonly in variety of
medical disorders like parkinsonism,
myocardial ischemia, malabsorption,
epilepsy, obesity and alcoholic pancreatitis.
SD can be a cutaneous marker of early HIV
infection (Fig. 11.5).
Patients with HIV infection often have severe
recalcitrant disease.
The disease is usually protracted over weeks
to months. The prognosis is good.
Differential diagnosis:
1. HIV must be ruled out as patients with
HIV tend to have extensive/severe SD.

2. Scalp psoriasis: The lesions are well


defined, palpably thickened, brighter
pink in colour with silvery scales.
3. Infective dermatitis complicating
pediculosis may mimic SD.
4. Truncal SD should be differentiated from
pityriaisis rosea, tinea versicolor.
5. Flexural SD should be differentiated from
tinea, candidiasis, erythrasma.
6. Follicular SD should be differentiated
from Dariers disease.
7. SD-like lesions can occur in acrodermatitis enteropathica.
8. Drugs producing SD-like rash: Methyl
dopa, chlorpromazine, cimetidine.
Treatment: In general, therapy is directed
towards loosening and removal of scales
(daily shampoo containing selenium sulfide,
zinc pyrithione, ketoconazole, cetavalon,
salicylic acid, coal tar; topical corticosteroids;
topical antifungals like imidazoles) and

Eczema
crusts, inhibition of yeast colonization,
control of secondary infection, and reduction
of erythema and itching. For unresponsive
cases, narrow band UVB, oral ketoconazole,
itraconazole or terbinafine may be used.
Other treatment modalities include topical
metronidazole (1%) gel, topical lithium
succinate, vitamin D 3 analogues, and oral
isotretinoin.
Patients should be informed about the
chronic nature of the disease and understand
that therapy works by controlling the disease
rather than by curing it.

NUMMULAR ECZEMA (NE) (DISCOID


ECZEMA)
The word nummular means (Latin
nummulus) coin like.
Etiology of this is unknown.
It mainly affects men aged 55-65 years.
The eruption is characterized by pruritic,
coin shaped, eczematous lesions (Fig. 11.6)
The lesion tends to develop on the extensor
surfaces of the extremities and trunk.

Differential diagnosis: NE may simulate


tinea corporis (where scaling of the edge is
more conspicuous, scrapings shows presence
of mycelia). Exogenous dermatitis and
irritant dermatitis occasionally presents with
discoid response. In psoriasis, the lesions are
dry, the scaling is more prominent and the
irritation is milder.
NE has a chronic course with frequent
relapses.
Treatment: open wet compresses, topical
steroids and antibiotics for secondary
infection.

POMPHOLYX (DYSHIDROSIS)
Pompholyx is characterized by itchy deepseated vesiculation on the palms and sides
of the fingers. Lesions appear sago-like.
It may involve the soles of the feet.
It occurs bilaterally symmetrically, has
relapsing course.
Differential diagnosis:
1. Id eruptions: Id eruptions are pompholyxlike eruptions due to distant focus of
infections (e.g. kerion or bullous tinea
pedis)
2. Pustular psoriasis of palms and soles:
Sterile pustules, absence of clear vesicles,
and characteristic residual brown marks
as the lesions subside
3. Pemphigoid lesions occurring on the
palms
4. Bullous tinea pedis
Treatment: open wet compresses, topical
steroids and antibiotics for secondary
infection.

PITYRIASIS ALBA

Fig. 11.6: Nummular eczemacoin shaped


eczematous lesions over the leg

Pityriasis alba is a common disorder in


children that is characterized by an
asymptomatic, hypopigmented, slightly
elevated, fine, scaling plaque with
indistinct borders.

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Essentials in Dermatology
It usually disappears by adulthood.
The condition commonly affects the face.
Differential diagnosis:
1. Indeterminate leprosy: Lesion is usually
solitary to a few, hypopigmented macule
with ill-defined margins and equivocal
sensory loss.
2. Tinea versicolor: Lesions are well-defined
hypopigmented macules with branny
scales. KOH examination shows
spaghetti and meatball appearance.
3. Vitiligo-early lesions may mimic but lack
scaling.
4. Mycosis fungoides although relatively
rare may present with lesions clinically
resembling pityrasis alba.
Treatment: Emollients, mild topical steroid
or topical pimecrolimus or tacrolimus
application may suffice.

STASIS DERMATITIS
Stasis dermatitis occurs as a result of venous
stasis on the lower portions of the legs.
The dermatitis may be acute, subacute or
chronic.
It differs from other forms of dermatitis,
firstly by showing brownish black
pigmentation and secondly, by resulting in
some instances in ulceration and atrophic
scarring.
The surrounding skin may show changes due
to venous stasishyperpigmentation,
induration of skin and lipodermatosclerosis
in late stage.
Differential diagnosis:
1. Allergic contact dermatitis usually caused
by topical medications. Patch testing is
helpful.
2. Infected ulcer with infective eczematoid
dermatitis around the ulcer responds to
appropriate antibiotic therapy.
3. Discoid eczema is common on lower legs
usually on the anterior or anterolateral
aspect.

4. Asteatotic eczema commonly affects the


legs of elderly.
5. Dermatophyte infection may present as
diffuse erythema and scaling and can be
difficult to recognize, particularly if it has
been treated with topical steroids.
6. Psoriasis may present as a single irritable
plaque on the leg but is usually more scaly
and clearly marginated
Treatment: The underlying venous
hypertension should be controlled. Well
fitted stockings can be helpful if worn
regularly. The legs should be elevated when
patient is recumbent. Topical steroids may
be used to relieve irritation.

ASTEATOTIC ECZEMA (ECZEMA


CRAQUELE, WINTER ECZEMA,
DERMATITIS SICCA)
It is an eczema associated with a decrease in
skin surface lipids.
The eczema occurs after excessive drying,
especially during the winter months and
among the elderly and those with atopic
dermatitis or ichthyosis vulgaris.
The skin of the limbs and trunk is
erythematous, dry and itchy and shows a fine
crazy-paving pattern of fissuring.
Differential diagnosis: Atopic dermatitis,
various forms of ichthyosis especially
acquired ichthyosis.
Treatment: Central heating should be
humidified where possible. Avoidance of
frequent bath, use of synthetic detergent
instead of soap, regular lubrication of the skin
especially after bathing helps. Weak topical
steroids are often prescribed.

LICHEN SIMPLEX CHRONICUS


(NEURODERMATITIS)
Lichen simplex chronicus is characterized by
lichenified plaque lesion or lesions due to

Eczema
repeated rubbing or scratching as a habit or
due to stress.
Lichenification of the skin follows chronic
scratching and/or rubbing. This may occur
without a predisposing dermatosis, or may
follow any pruriginous dermatosis. The term
lichen simplex is used when there is no
predisposing dermatosis.
It is characterized histologically by acanthosis
and hyperkeratosis and clinically by
thickened appearance of the skin, with
accentuation of skin markings so that the
affected skin surface resembles tree bark.

Fig. 11.7: Lichen simplex chronicusthickening,


and pigmentation of ankle

Fig. 11.9: Lichen simplex chronicus-involving


female external genitalia

The areas most commonly affected are those


that are conveniently reached.
These areas are ankles (Fig. 11.7) and wrists,
nape of neck (Fig. 11.8), genitalia (scrotum
or mons pubis) (Fig. 11.9), etc.
It is common in Indian subcontinent amongst
adults (30-50 years).
Differential diagnosis: The morphological
diagnosis of lichenification is not usually
difficult-lichen planus, lichen amyloidosis,
and psoriasis have to be excluded and typical
lesions sought in other sites.
Treatment: Patient should be given some
assistance in reducing their tension. Sedation
is often needed and sedative antihistaminics
may be helpful. In most cases, a steroid cream
is the treatment of choice. To prevent the
scratching and to improve the outcome of the
treatment, steroid may be applied under
polythene occlusion.

PRURIGO NODULARIS (PN, HYDES


PRURIGO)

Fig. 11.8: Lichen simplex chronicus


involving nape of neck

Prurigo is a Latin word for itching.


PN is an intensely pruritic disorder in which
persistent rubbing and scratching in
particular area leads to formation of
distinctive hemispherical nodules with raised
warty surface (Fig. 11.10).

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Essentials in Dermatology

Fig. 11.11: Lichen striatuslinearly arranged flat


topped papular lesions over the chest of a child

Fig. 11.10: Prurigo nodularisraised itchy nodular


lesions over the legs and dorsa of feet

Disease occur in 20-60 years of age, both sexes


are equally affected.
The cause is unknown. The initial trigger for
itching in PN may include local trauma,
insect bite reactions and atopic eczema, etc.
Differential diagnosis:
1. Hypertrophic lichen planus: Usually
violaceous, may be associated with
typical lichen planus lesions elsewhere.
2. Pemphigoid nodularis may present as
nodular prurigo for sometime before
typical urticated plaques and blisters
supervene.
3. Allergic contact dermatitis may result in
papulonodular eruptions.
4. Systemic causes of pruritus that can give
rise to lesions resembling nodular prurigo
include renal failure, liver disease,
lymphoma, and HIV infection.
Treatment: Oral antihistamines, emollients,
topical steroids with salicylic acid and

intralesional steroids, cryotherapy, and


systemic agents such as thalidomide,
cyclosporine and azathioprine.

LICHEN STRIATUS
It is an acquired self-limited inflammatory
linear skin disorder
Exact etiology is unknown
Children aged 5 to 15 years are commonly
affected
The most characteristic lesion is a linear band
of hypopigmented lichenoid papules that
follow Blaschkos lines (Fig. 11.11)
The lesions commonly occur on the arm or
leg
Course of the lesion may extend for 2 weeks
to 4 months.
Differential diagnosis: Epidermal naevi
(persist indefinitely), linear lichen planus or
psoriasis (easily differentiated clinically, even
in the absence of typical lesions in other sites
which should always be sought for)
Treatment: Usually none is necessary, topical
steroids, topical tacrolimus and intralesional
steroids in persistent cases.

Eczema

HAND ECZEMA
Hand eczema is not a single diagnostic entity.
It is rather a morphological presentation of
various types of eczemas largely confined to
the hands and can have a multitude of factors
responsible for causing it, singly or in
combination. Causes of hand eczema are
broadly grouped into two groups as shown
below:

Exogenous
1. Contact irritants: Chemicals (e.g. soaps,
detergents, solvents)
Physical (e.g. friction, minor trauma, cold
dry air)
2. Contact allergens: Delayed hypersensitivity (e.g. chromium, rubber) Immediate
hypersentivity (e.g. seafood)
3. Ingested allergens (e.g. drugs, nickel,
chromium)
4. Infection (e.g. bacterial infections of hand
wounds)
5. Secondary dissemination (e.g. dermatophytide reaction to tinea pedis).
Endogenous
1. Idiopathic (e.g. Discoid, hyperkeratotic
palmar eczema)
2. Immunological or metabolic defects (e.g.
atopics)
3. Psychosomatic: Stress aggravates, but
may not be causative
4. Dyshidrosis: Increased sweating aggravates, but may not be causative.
Morphological types of Hand Eczema
1. Pompholyx
2. Recurrent focal palmar peeling
3. Hyperkeratotic palmar eczema
4. Ring eczema
5. Wear and tear dermatitis

6. Fingertip eczema
7. Apron eczema
8. Discoid eczema
9. Chronic acral dermatitis
10. Gut eczema.
Differential diagnosis: The diagnosis of
hand eczema is usually self evident but
distinction from psoriasis is very difficult. In
most cases of psoriasis on hands, however,
the silvery nature of the scales, involvement
of knuckles, sharply demarcated scalloped
edges to the erythema along the borders of
the hands and fingers, and the relative
absence of pruritus are helpful pointers.
Family history of psoriasis and the presence
of nail pits in the absence of nail fold lesions
are also suggestive. Tinea can also be missed
especially when it is extensive and irritable
or secondarily infected. Unilateral scaling of
palm should always suggest tinea manuum.
Treatment of hand eczema: Avoidance of
irritants, frequent application of emollients,
and sparing use of topical steroids.

JUVENILE PLANTAR DERMATOSIS


(FOREFOOT ECZEMA)
Occurs mainly in children aged 3-14 years of
age.
The presenting features are redness and pain
on the plantar surface of the forefoot, which
assumes a glazed and cracked appearance.
The condition is most severe on the ball of
the foot and toe pads, and tends to spare the
non-weight-bearing instep.
The symmetry of the lesions is a striking
feature.
Differential diagnosis: The toe clefts are
normal, and this helps to distinguish the
condition from tinea pedis. Patch testing to
exclude foot wear allergy may be done.
Treatment: Most cases will clear
spontaneously during childhood or

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adolescence. Patients are advised to use
emollients, 100% cotton socks and leather
shoes or sandals.

CONTACT DERMATITIS
Contact dermatitis is an eczematous dermatitis
caused by exposure to substances in the
environment. Common types of contact
dermatitis are discussed here.

Irritant Contact Dermatitis (ICD)


ICD is a non-immunological eczematous
reaction which occurs without previous
sensitization in most exposed individuals.
Most common type of contact dermatitis.
Common irritants include alkalis (soaps,
detergents), dye and ammonia containing
compounds.
The clinical features vary from mild xerosis
to erythema/chapping or even severe
ulceration depending on the concentration of
the irritant, the skin area exposed, mode of
contact and the substance itself.
The reaction remains localized and does not
spread to become generalized and it does not
cause systemic symptoms.
Acute irritant dermatitis is characterized by
vesicles and bullae (e.g. cement dermatitis,
savlon induced reaction).
Chronic irritant dermatitis manifests as
hyperkeratosis, fissuring and scaling (e.g.
house wife dermatitis).
Healing usually occurs within 2 weeks of
removal of noxious stimulus.
In cases of chronic subcritical level of
irritation, some develop tolerance or
hardening.
Differential diagnosis: Same as for allergic
contact dermatitis
Treatment: avoidance of irritants, use of
bland barrier creams, protective gloves and
mild topical steroids.

Allergic Contact Dermatitis (ACD)


ACD results when an allergen comes in
contact with previously sensitized skin.
It is a delayed hypersensitivity reaction,
consisting of sensitization phase and when
rechallenged-elicitation phase.
Occurs in individual of all ages, especially
elderly.
Sensitization remains lifelong.
The allergens are extremely varied and may
be nonprotein in nature.
Common causes of ACD include plants,
nickel (Fig. 11.12), chromate, para phenylene
diamine (hair-dye) (Figs 11.13 and 11.14),

Fig. 11.12: Contact dermatitisdermatitis over


abdomen due to trouser metallic hook

Fig. 11.13: Contact dermatitiserythema and


edema due to acute dermatitis caused by hair dye

Eczema

Fig. 11.14: Contact dermatitislichenoid dermatitis


over the forehead due to hair dye

Fig. 11.16: Common agents involved in contact


dermatitis of the face

Fig. 11.15: Contact dermatitisdermatitis due to


rubber chappal

rubber compounds (Fig. 11.15), fragrances


and preservatives in compounds.
The eczematous reactions develops at the
sites of skin contact with the allergen (Fig.
11.16) but occasionally spreads outside these
limits and cause systemic symptoms.
The patch test is diagnostic and helps to
identify allergens involved.
Patch test consists of application of
substances suspected to be the cause of the
contact dermatitis to the intact uninflammed
skin, in nonirritating concentration.

Most common plant allergen in India is


Parthenium hysterophorus.
Most common metal causing ACD is nickel
Differential diagnosis: Main differential
diagnostic consideration is irritant contact
dermatitis. Others are erysipelas (rapidly
spreading, non-pruritic, well defined
erythematous area, and patient is sick), atopic
dermatitis, nummular eczema, tinea (KOH
examination), seborrhoeic dermatitis,
polymorphous light eruption, and
angioedema
Treatment: The most important step is
avoidance of allergens. Acute dermatitis of
any sort is best treated with moist compresses
and high potency corticosteroid creams. In
severe cases, a short burst of systemic
corticosteroids tapered over 7-10 days is
needed. More chronic cases can be treated
with lower potency corticosteroids in an
ointment base. Oral cyclosporine is indicated
for therapy resistant chronic disease.

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DIFFERENCES BETWEEN IRRITANT CONTACT AND ALLERGIC CONTACT


DERMATITIS
Irritant contact dermatitis

Allergic contact dermatitis

*People at risk
*Mechanism of response
*Prior exposure required
*Nature of substance

Everyone
Nonimmunologic
No
Organic solvents, soap

*Concentration of substance required


*Symptoms
*Lesions
*Involvement of non-exposed sites
*Type of inflammatory cells
*Patch test

Usually high
Severe, stinging or burning sensation
Large tense bullae with necrosis
No
Neutrophils
Not useful

Genetically predisposed
Delayed hypersensitivity
Yes
Low molecular weight hapten
(metal, formalin)
May be low
Pruritus
Papules or papulovesicles
Yes
Eosinophils and lymphocytes
Diagnostic

INFECTIVE DERMATITIS (INFECTIOUS


ECZEMATOID DERMATITIS, MICROBIAL
ECZEMA)
Infective eczema is caused by microorganisms or their products, clears when
organisms are eradicated.
It is seen predominantly around discharging
wounds or ulcers or moist skin lesions of
other types.
It presents as an area of advancing erythema,
sometimes with microvesicles (Fig. 11.17).
Treatment: Factors predisposing to infection
should be sought and when possible
eliminated. Wet compresses, topical
antibiotic in combination with steroid or
systemic antibiotic.

POLYMORPHIC LIGHT ERUPTION


(PMLE)
PMLE is the most common photodermatoses.
The disease may begin at any age and the
symptoms are severe in spring and summer
season.
It is an idiopathic eruption caused by UV
exposure; appears in hours to days with
varied morphology (papular variant most

Fig. 11.17: Infective dermatitisoozing dermatitic


lesions behind ear and trunk

common, papulovesicular, eczematous,


erythematous, plaque like). Clinically, it
presents as non scarring, erythematous, itchy
papules, plaques or vesicles on exposed skin
that usually heal without scarring.
The amount of light exposure needed to elicit
an eruption varies greatly from one patient
to another.

Eczema
Light sensitivity decreases with repeated sun
exposure, this phenomenon is referred to as
hardening.
Differential diagnosis: Lupus erythematosus, photosensitive drug eruption,
prurigo nodularis, and photoallergic contact
dermatitis need to be differentiated.

Treatment: Photoprotection by clothing and


sunscreens, phototherapy, and topical
steroids. In severe resistant cases, consider
the use of azathioprine 50-100 mg daily for 3
months. Use antioxidants, one week before
exposure may help in prevention.

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12

Vesiculobullous Disorders

Vesiculobullous disorders cause significant


morbidity and mortality in dermatology. While a
number of disorders of varied etiology can present
with vesicles or bullae, two distinct groups of
disorders are the genetic blistering diseases
(epidermolysis bullosa) and the acquired
autoimmune blistering disorders (pemphigus,
bullous pemphigoid, dermatitis herpetiformis,
chronic bullous dermatosis of childhood,
epidermolysis bullosa acquisita).
Vesiculobullous disorders are diseases where
the primary lesion is a vesicle or bulla. It occurs
due to pathology in the epidermis or dermoepidermal junction or dermis. In this chapter we
are not dealing with secondary causes of
vesiculation like to burns, suction blisters,
ischemia leading to blisters (vasculitis, diabetic
bulla), drug eruptions like bullous fixed drug
eruption, insect bite reaction, infections (varicella,
herpes simplex, herpes zoster), dermatitis,
miliaria, etc. This chapter would discuss primary
vesiculobullous disorders. In epidermal autoimmune vesiculobullous disoders, the
mechanism behind blister formation is primarily
acantholysis in most cases. Decreased stability of
basement membrane is responsible for a variety
of subepidermal vesiculobullous disoders which
could be either inherited or of acquired
autoimmune origin.

CLASSIFICATION OF
VESICULOBULLOUS DISORDERS
1. Inherited/genetic blistering disorders:
a. Epidermolysis bullosa simplex
b. Junctional epidermolysis bullosa
c. Dystrophic epidermolysis bullosa.
2. Acquired autoimmune blistering disorders:
a. Intraepidermal immunobullous diseases
1. Pemphigus vulgaris and its variant
pemphigus vegetans.
2. Pemphigus foliaceous and its variants
i. Endemic pemphigus foliaceus (Fogo
selvagem; wild fire).
ii. Pemphigus erythematosus (SenearUsher syndrome)
iii.Pemphigus herpetiformis.
3. Drug induced pemphigus.
4.. IgA pemphigus
i. Intraepidermal neutrophilic type
ii. Subcorneal pustular dermatosis type.
5. Paraneoplastic pemphigus.
6. Subcorneal pustular dermatosis (Sneddon
Wilkinson disease).
3. Subepidermal immunobullous diseases
a. Bullous pemphigoid and its variants
b. Mucous membrane pemphigoid (cicatricial pemphigoid)

Vesiculobullous Disorders
c. Pemphigoid gestationis (Herpes gestationis)
d. Linear IgA disease/Chronic bullous
dermatosis of childhood
e. Dermatitis herpetiformis
f. Epidermolysis bullosa acquisita
g. Bullous systemic lupus erythematosus.
The following discussion deals with these two
groups of disorders with special focus on the more
common entities.

EPIDERMOLYSIS BULLOSA
Epidermolysis bullosa (EB) comprises a group
of genetically determined skin disorders
characterized by blistering of the skin (Fig.
12.1) and mucosae at birth or soon afterwards,
following mild mechanical trauma (due to
increased fragility of skin). Thus an alternative
term for these disorders could be the
mechanobullous disorders.
There are three main types of EB:
EB simplex (intraepidermal split due to
disruption of basal keratinocytes)
Junctional EB (split through the basement
membrane zone)
Dystrophic EB (split in the subepidermal
level).

Fig. 12.1: Epidermolysis bullosababy showing


multiple erosions

EB simplex is the commonest and mildest


form of EB of autosomal dominant
inheritance. It is characterized by onset of
blistering over trauma prone sites at birth or
infancy. Lesions heal without scarring.
Mucosae, nails and hair are essentially
uninvolved.
Junctional EB are autosomal recessive
disorders and are broadly classified into two
main types, the lethal and non-lethal forms.
They present at birth or soon after with severe
fragility of the skin leading to extensive
blistering and denudation. Oropharyngeal
mucosae may be severely involved. Teeth may
be malformed and prematurely lost and nails
may be shed. This is the most fatal type of EB.
Dystrophic EB is characterized by skin
fragility, scarring with milia (Fig. 12.2), nail
changes (Fig. 12.3) and have either autosomal
recessive or dominant inheritance. The more
severe autosomal recessive form is
characterized by:

Fig. 12.2: Epidermolysis bullosa dystrophicaknee


showing hemorrhagic bullae with scarring, milia and
pigmentation

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Essentials in Dermatology
Gene therapy appears as a realistic goal in
the future.

PEMPHIGUS

Fig. 12.3: Epidermoloysis bullosa dystrophica


albopapuloid lesions over the dorsa of feet and some
of the toenails completely lost

Onset at birth or early infancy


Blistering of skin mainly over trauma prone
sites
Oral blisters and scarring leading to
ankyloglossia and microstomia
Esophageal lesions causing painful
dysphagia and later esophageal strictures.
Perianal blistering, erosions and scarring
causing stenosis and fecal retention.
Ocular complicationssymblepharon,
corneal erosions and opacity
Repeated blistering and progressive
scarringcontractures and deformities
(e.g. Mitten hands).
Diagnostic techniques include skin biopsy,
electron microscopy to ascertain the level of
split and structures involved, antigen
mapping and immunohistochemistry. No
autoantibodies are demonstrated in the sera.
Prenatal DNA testing can be advised to
couples at risk of having affected children.
No specific treatment is available for EB and
thus the mainstay of treatment is based on
avoidance of provoking factors. Management
of the neonate includes maintaining adequate
nutrition and hydration and prevention of
sepsis. Other aspects include care of the oral
cavity, teeth, eyes and management of contractures and deformities.

Pemphigus is derived from the Greek


pemphix meaning blister or bubble and is
characterized by intraepidermal blistering at
various levels in the epidermis.
The key pathogenic process in this group of
disorders is disruption of the intercellular
cementing substance due to an autoantibody
attack on the cellular adhesion proteins
(desmogleins) leading to acantholysis.
The pemphigus group of disorders includes
two major types (and their variants) and
several other minor types. The major types are:
Pemphigus vulgaris (variant Pemphigus
vegetans): level of split-suprabasal.
Pemphigus foliaceus (variant Pemphigus erythematosus): level of split
subcorneal.
The other minor types of pemphigus include
paraneoplastic pemphigus, drug induced
pemphigus, IgA pemphigus and neonatal
pemphigus.

Pemphigus Vulgaris
Most common form of pemphigus, accounting
for up to 80% of pemphigus cases. Occurs at
any age, most commonly between fourth- sixth
decades. In India, it occurs at younger age.
It is due to IgG antibodies directed against
epidermal cell adhesion molecules
(desmoglein 3)- disruption of intercellular
cementing substanceloss of adhesion
between epidermal cells (acantholysis)
intraepidermal blistering.
Clinical Features
Almost all patients have mucosal lesions,
5070% present with painful oral erosions.
Lesions may be limited to oral cavity for
months to one year (Fig. 12.4).

Vesiculobullous Disorders
Skin flaccid bullae on normal or erythematous skin, with a predilection for scalp,
face (Fig. 12.5), trunk (Figs 12.6 and 12.7),
axillae and groins and pressure sites.
Bullae rupture producing painful erosions
that show no tendency to heal spontaneously.
Pruritus is absent or negligible.
Nikolskys sign and Bulla spread sign
(Asboe-Hansen sign) are positive. (Other
disorders with positive Nikolskys sign are

staphylococcal scalded skin syndrome, toxic


epidermal necrolysis, etc.).
Other mucosae involved are conjunctiva,
pharynx, larynx, oesophagus, urethra, vulva
and cervix.

Fig. 12.4: Pemphigus vulgarispainful erosions


involving tongue and lips

Fig. 12.5: Pemphigus vulgarisextending


erosions without tendency to heal over the face

Fig. 12.6: Pemphigus vulgarisinvolving chest

Fig. 12.7: Pemphigus vulgarisinvolving back

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Essentials in Dermatology
Prognosis is poor without treatment but with
systemic steroids mortality has been reduced
to 515 %.
Pemphigus may be associated with other
autoimmune diseases such as thymoma,
myasthenia gravis and malignancies like
lymphomas and bronchogenic carcinoma.

Diagnosis
Tzanck smear from the floor of the blister
shows acantholytic cells. Acantholytic cell is
a large, rounded epidermal cell with a large
nucleus, perinuclear halo and peripheral
condensation of cytoplasm (Fig. 12.8).
Histopathological examination of a blister
shows a supra-basal cleft in the epidermis.
The basal keratinocytes remain attached to the
basement membrane but are separated from
each other and stand like a row of
tombstones.
Immunofluorescence studies are the gold
standard in diagnosis of the autoimmune
blistering disorders. In pemphigus vulgaris,
direct immunofluorescence done on the
lesional skin shows deposition of intercellular
IgG throughout the epidermis in a fish-net
pattern (Fig. 12.9). Indirect immunofluorescence done to determine levels of
pathogenic antibodies in the sera of the

Fig. 12.8: Tzanck smear showing large rounded


epidermal cells with large nuclei, perinuclear halo
and peripheral condensed cytoplasm (Acantholytic
cell)

Fig. 12.9: Direct immunofluorescence


showing fish net pattern

patients shows circulating intercellular IgG


antibodies in 8090% of the cases. Levels of
these antibodies correlate with disease
activity.

Pemphigus Vegetans
It is a clinical variant of pemphigus vulgaris
characterized by vegetating lesions primarily in
the flexures (Figs 12.10 and 12.11). Initial lesions
are bullae or pustules, which rupture and
progress to form vegetating plaques.
Pemphigus Foliaceous
This disorder, characterized by blistering at a
higher level in the epidermis is less common
than pemphigus vulgaris and accounts for
only 1520 % of pemphigus cases.
It is caused by IgG antibodies directed
against intercellular adhesion molecules
(desmoglein 1) found predominantly in the
upper epidermisdisruption of intercellular
cementing substance of upper epidermal cellssubcorneal blister formation.
Clinically, pemphigus foliaceous is less severe
than pemphigus vulgaris and is characterized
by crusted, moist, scaly lesions in a
seborrheic distribution (Fig. 12.12) involving
scalp, face, chest and upper back. Blistering
may not be obvious due to the superficial level
of the split (very transient nature of blisters).

Vesiculobullous Disorders

Fig. 12.10: Pemphigus vegetansvegetating moist


lesions occurring in the axilla

Fig. 12.12: Pemphigus foliaceousmoist scaly


lesions in seborrheic areas of the face

Fig. 12.11: Pemphigus vegetansvegetating moist


lesions in the retroauricular area

Oral lesions are uncommon.


Nikolskys sign is invariably positive
(Fig. 12.13).

Fig. 12.13: Nikolskys sign tangential pressure with


finger tip producing moist erosion due to peeling of
skin

Diagnosis
Tzanck smear from fresh erosion shows
acantholytic cells.
Histology shows a subcorneal cleft with
acantholysis.

Immunofluorescence findings are usually


indistinguishable from pemphigus vulgaris.
Prognosis of this disorder is better than
pemphigus vulgaris. This benign disorder
responds well to treatment.

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Pemphigus Erythematosus
It is a variant of pemphigus foliaceous characterized by immunological features of both
pemphigus and lupus erythematosus (LE), that
is, intercellular IgG and C3 in the epidermis (as
in pemphigus) and in the basement membrane
zone (as in LE) and antinuclear antibodies (as in
LE). Clinically, erythematous, scaly rash over the
nose and cheeks simulate LE while lesions on the
trunk are similar to those in pemphigus foliaceous.
Other Variants of Pemphigus
Endemic pemphigus foliaceous (Fogo
Selvagem) is a variant of pemphigus
foliaceous, endemic to certain parts of South
America and is postulated to be precipitated
by bites of the black fly (Simuliidae). The burnt
appearance and burning sensation gave the
disease its name, fogo selvagem, meaning
wild fire.
Drug induced pemphigus (penicillamine,
captopril, pyritinol, penicillin, rifampicin)
clinically commonly present as pemphigus
foliaceous.
Paraneoplastic pemphigusa polymorphous blistering eruption with mucocutaneous ulcerations having an underlying
neoplasm.
Pemphigus herpetiformissuperficial
vesicles and inflammatory papules occur in
herpetiform distribution.
IgA pemphigushas bound and circulating
IgA autoantibodies against intraepidermal
cell surface antigens and clinically may
resemble subcorneal pustular dermatosis.
Juvenile pemphigus pemphigus occurring
before 20 years of age
Neonatal pemphigus due to transplacental
transfer of maternal anti-intercellular cement
substance antibodies to the fetus. Blisters
resolve in 2 weeks.

Differential Diagnosis
When skin is involved, other autoimmune
vesiculobullous disorders need to be differentiated from pemphigus. On rare occasions, bullous
impetigo, dyskeratotic acantholytic disorders
(Dariers disease, Hailey Hailey disease, Grovers
disease) can cause a problem.
When only the oral mucosa is involved, the
following should be considered-aphthous
ulcerations, oral erosive lichen planus, herpetic
gingivostomatitis, erosive candidiasis, and
erythema multiforme.
When both skin and oral mucosa are involved,
it closely resembles erythema multiforme, Stevens
Johnson syndrome, toxic epidermal necrolysis,
bullous systemic lupus erythematosus, and
generalized bullous fixed drug eruption.
Treatment of Pemphigus
The mainstay of therapy in the pemphigus
group of disorders is with systemic steroids,
which can be given as conventional therapy
(oral prednisolone in the dose of 1 mg per kg
body weight) or as pulse therapy. An
upcoming mode of therapy is the dexamethasone cyclophosphamide pulse regimen
consisting of 100 mg of IV dexamethasone in
5% dextrose infusion on 3 consecutive days
of each month combined with IV cyclophosphamide 500 mg bolus dose on day 1 of the
pulse and oral cyclophosphamide 50 mg daily
on other days. This therapy reduces the
conventional side effects of steroids.
The other modalities of therapy include
adjuvant therapy with dapsone, azathioprine,
cyclosporine, methotrexate, gold salts,
mycophenolate mofetil, intravenous
immunoglobulin therapy and plasmapheresis
and are essentially to reduce the side effects of
steroids or to control the severe form of
pemphigus.

Vesiculobullous Disorders

BULLOUS PEMPHIGOID (BP)


Autoimmune subepidermal blistering
disorder of the elderly, with onset usually after
60 years of age.
Basic pathogenic process is due to IgG
antibodies against components of the
basement membrane zone (BMZ structure
which binds epidermis to the underlying
dermis and mesenchyme) loss of structural
integrity of the BMZ due to resultant
inflammation separation of the intact
epidermis from underlying dermissubepidermal cleft formation.

Clinical Features
Preceded by pruritus with or without
urticarial wheals lasting usually for 13
weeks.
Tense bullae on normal or erythematous
skin predominantly over flexural aspects of
the limbs (Fig. 12.14), lower abdomen
(Fig. 12.15), groins and axillae. Facial skin
and scalp relatively spared.
Lesions rupture to leave erosions that heal
spontaneously with postinflammatory
hyperpigmentation.
Nikolskys sign usually negative; Bulla
spread sign +/ Mucosal lesions rare and less severe than
in pemphigus vulgaris and almost never
the presenting feature.
Bullous pemphigoid may be associated with
an underlying malignancy (gastric carcinoma
commonly) and may be associated with
diabetes mellitus, rheumatoid arthritis and
psoriasis.
Diagnosis
Tzanck smear will show numerous
eosinophils with few neutrophils but no
acantholytic cell.

Fig. 12.14: Bullous pemphigoiditchy urticarial


lesions over the thigh developing into tense bullae

Fig. 12.15: Bullous pemphigoidinvolving


lower back

Histopathological examination reveals


subepidermal cleft with intact epidermis
forming the roof of the blister.
Direct immunofluorescence of perilesional
skin shows linear IgG (4590%) and C3
(80100 %) deposition in the basement
membrane zone. Indirect immunofluorescence studies done on the patients sera
show circulating IgG autoantibodies in most
cases but unlike in pemphigus, their titers do
not correlate with disease activity.

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Differential Diagnosis
Bullous pemphigoid can be easily differentiated
from most other blistering disorders such as linear
IgA disease, chronic bullous disease of childhood,
dermatitis herpetiformis, erythema multiforme
and pemphigus by histology and immunofluorescence. Most difficult diseases to
differentiate from bullous pemphigoid are
epidermolysis bullosa acquisita and cicatricial
pemphigoid.
Prognosis of bullous pemphigoid is better
than pemphigus and it runs a chronic selflimiting course. It may be fatal in the active
stage in the elderly or debilitated patients.

Fig. 12.16: Cicatricial pemphigoid


tense bulla over the tongue

Treatment
The mainstay of treatment is a topical or
systemic steroid depending on the severity
of the disease. Pulse dexamethasone
cyclophosphamide, methyl prednisolone or
cyclophosphamide pulse may be used. Other
modalities include cyclophosphamide,
azathioprine, dapsone, tetracycline with
nicotinamide, erythromycin, leflunomide,
sulphapyridine, methotrexate, mycophenolate mofetil, intravenous immunoglobulin
therapy and plasmapheresis.

Fig. 12.17: Cicatricial pemphigoidface showing


symblepharon

Clinical Variants of Bullous


Pemphigoid Include
Cicatricial pemphigoid is a rare blistering
disorder of the mucosa and skin that results
in permanent scarring of the affected area.
Mucosal lesions predominate and involve in
decreasing order the oropharynx (Fig. 12.16),
nasopharynx, conjunctiva, larynx, genitalia
and esophagus. Sequelae include oropharyngeal adhesions, esophageal strictures,
stridor, introital shrinkage, symblepharon
(Fig. 12.17) and statue eye. Unlike bullous
pemphigoid, this disorder is not self-limiting
and has a chronic debilitating course.

Herpes gestationis is a non-viral autoimmune


blistering disease of young women that
occurs in pregnancy (2128 weeks of
gestation) or within 1st week postnatally.
Clinically, the disease starts as severe pruritus
with urticarial wheals and plaques followed
by blistering predominantly in the
periumbilical area, lower abdomen and
thighs. Mucosal involvement is rare and
lesions improve postpartum. Recurrence may
occur in subsequent pregnancies, premenstrually or with oral contraceptive pills
(OCPs). Direct immunofluorescence shows
linear C3 deposits at the BMZ with IgG in some
cases.

Vesiculobullous Disorders

Characteristic

Bullous pemphigoid

Pemphigus vulgaris

Age
Pruritus
Blister
Sites
Erosion
Nikolskys sign
Oral lesions

> 60 years
++
Tense
Flexures, groins, axillae, abdomen
Spontaneously heal
Negative
40 % less severe, almost never a
presenting feature
Benign, self limiting

Middle age
Not present
Flaccid
Face, scalp, trunk, pressure points
Extend peripherally
Positive
8090 % severe commonly the
presenting symptom
Poor without treatment

Prognosis

DERMATITIS HERPETIFORMIS (DH)


DH is defined as an intensely pruritic,
chronic, recurrent, papulovesicular disease
with an underlying gluten-sensitive
enteropathy which may be asymptomatic.

Clinical Features
Onset at any age, usually between 2055 years
of age.
Males outnumber females.
Pruritus is the first and predominant symptom
followed by a symmetrical eruption of
erythematous papules and papulovesicles,
which are so rapidly excoriated that intact
vesicles are difficult to demonstrate
(Fig. 12.18).
Sites extensor aspects of limbs (elbows and
knees), buttocks, natal cleft, shoulders, upper
back (Fig. 12.19), face and scalp. Grouping of
lesions accounts for it being described as
herpetiformis (not associated with herpes
virus).
Oral lesions are common but asymptomatic.
Provocation of lesions occurs with iodides
orally or in iodide patch testing.
Histological examination best done on lesions
that have not blistered or ruptured and reveals
neutrophilic microabscesses at the tips of
dermal papillae.

Fig. 12.18: Dermatitis herpetiformisextremely itchy


tense grouped vesicular lesions over the shoulder
area

Fig. 12.19: Dermatitis herpetiformisbilateral


symmetrical distribution of itchy eroded lesions over
the extensor aspect of limb and trunk

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Essentials in Dermatology
Direct immunofluorescence is the most
reliable diagnostic criterion and should be
performed on clinically normal skin
(preferably of the buttocks). It reveals granular
IgA deposits in dermal papillae.
Indirect immunofluorescence is negative for
anti-BMZ or dermal autoantibodies but
antithyroid and antigliadin antibodies may
be seen.

chronic bullous disease/dermatosis of childhood


(CBDC), with onset in childhood and linear IgA
disease which presents in adults. There is lot of
overlap in the clinical presentation of the two
entities but they differ in their age at presentation
and few clinical signs.
In linear IgA disease, periorificial and annular
lesions are not as common as in CBDC.
Hemorrhagic bullae may be present.

Differential Diagnosis
Dermatitis herpetiformis can be confused with
numerous conditions because of its pleomorphic
manifestations and occasional lack of diagnostic
lesions. Scabies, bullous pemphigoid before the
development of blisters, and the prurigo group of
disoders are the main issues. It also needs to be
differentiated from chronic exudative eczema,
papular urticaria, neurotic excoriations, and
transient acantholytic dermatoses. A high index
of suspicion is very helpful, even in the absence
of primary lesions. Dermatitis herpetiformis can
be diagnosed based on the typical in vivo bound
granular IgA deposits in normal appearing skin.

Chronic Bullous Disease/dermatosis of


Childhood (CBDC, Linear IgA disease of
Childhood)
CBDC is defined as a chronic acquired
autoimmune subepidermal blistering disease
of children characterized by IgA BMZ
antibodies.
Onset is usually at around 5 years of age
(toddlers and preschool children).
Urticarial plaques with blistering at the
edges string of pearls appearance and
localization of lesions around orifices
(perioral, perigenital) (Figs 12.20 and 12.21).

Treatment
Dapsone 100200 mg/day (up to 400
mg /day) is the drug of choice. It is amazingly
effective; hours to days after the first dose, the
pruritus disappears and no new lesions erupt
after 1-2 days of treatment.
Strict adherence to a gluten-free diet for
prolonged periods (e.g. 6 to 12 months) may
control the disease in some patients, obviating
or reducing the requirement for drug therapy.
LINEAR IgA DISEASE
It is a chronic acquired subepidermal blistering
disorder of children and adults, with skin and
mucous membrane involvement and characterized by linear deposition of IgA at basement
membrane zone. It consists of two main entities-

Fig. 12.20: Chronic bullous dermatosis of childhood


clustering of tense bullae around the mouth

Vesiculobullous Disorders
combined with low dose steroids in refractory
cases.

Fig. 12.21: Chronic bullous dermatosis of childhood


lower limbs showing cluster of jewels appearance
at many sites

Spontaneous remission usually occurs with


age.
Direct immunofluorescence shows linear
IgA at BMZ.

Differential Diagnosis
In young infant, bullous impetigo may resemble
the initial lesions, but its response to antibiotics
differentiates it. Epidermolysis bullosa often
present at birth and family history further
differentiates it. Bullous papular urticaria rarely
affects the face or genital region and it is usually
of short duration. Childhood bullous pemphigoid
may give us similar clinical picture but the
deposition of IgG and C3 at the BMZ is diagnostic.
Treatment is the same. Dermatitis herpetiformis
only occurs in small children (usually occurs
between 20 and 55 years of age) who are
heterozygous for predisposing HLA genes.
Treatment
Dapsone is usually effective (response usually
occurs within 24-48 hours), and may be

Disorder

Site of split

Pathogenic
antibody

P. vulgaris
P. foliaceous/
erythematosus
Paraneoplastic
pemphigus
BP / HG / CP
EBA
Dermatitis
herpetiformis
CBDC

Suprabasal
Subcorneal

IgG
IgG

Suprabasal

IgG

Subepidermal
Subepidermal
Subepidermal

IgG and C3
IgG
IgA

Subepidermal

IgA

(Note: Epidermolysis bullosa are a group of


genetic disorders with structural defects but NO
autoantibodies.)

EPIDERMOLYSIS BULLOSA ACQUISITA


(EBA)
It is defined as a chronic, acquired
autoimmune blistering disorder characterized
either by trauma-induced subepidermal
blistering or with a clinical picture
indistinguishable from bullous pemphigoid.
IgG class of autoantibodies directed against
collagen VII causes it. Collagen VII is the major
component of the anchoring fibrils found in
the subepidermal zone; (differentiate from
hereditary epidermolysis bullosa dystrophica
in which there is a collagen VII structural
defect leading to reduced or absent collagen
VII in the anchoring fibrils).
The five clinical presentations of EBA are:
1. Classical presentation Noninflammatory
tense bullae over trauma prone areas which
heal with scarring and milia formation.
Hemorrhagic bulla may be seen. It is a
mechanobullous disease marked by skin
fragility.
2. Bullous pemphigoidLike presentation
widespread inflammatory vesiculobullous
eruption over trunk, flexures and extremities.

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Essentials in Dermatology
Tense bullae are present over inflamed or
urticarial skin. Hemorrhagic bulla may be
seen. Large areas of erythema and urticaria
without blistering can be seen. Patient
complains of pruritus and does not show
prominent skin fragility, scarring or milia
formation. The clinical picture is more like a
bullous pemphigoid than a mechanobullous
disorder.
3. Cicatricial pemphigoid-like presentation is
marked by widespread mucosal involvement.
Clinical appearance is similar to cicatricial
pemphigoid. Erosions and scars over mucosal
surfaces of mouth, upper esophagus,
conjunctiva, anus or vagina are present with
or without similar lesions over glabrous skin.
4. Brunsting-Perry pemphigoid-like presentation is a chronic recurrent vesiculobullous
eruption localized to head and neck. It is
characterized by bullae healing with scarring
and has minimal to no mucosal involvement.
5. IgA bullous dermatosis-like eruption is
characterized by tense veskcles arranged in
an annular pattern and mucous membrane
involvement. It is also differentiated from
other EBA types by its DIF findings of linear

deposition of IgA at basement membrane


zone.
Histology shows subepidermal cleft with
neutrophilic infiltrate in the BP type while
sparse inflammatory infiltrate in the
mechanobullous type.
Direct immunofluorescence (DIF) on
perilesional skin shows thick polyclonal band
of deposition of IgG and C3 and sometimes
IgA and IgM in the BMZ.
DIF on salt split skin shows deposits of IgG
and C3 on dermal side whereas in bullous
pemphigoid in 85% cases deposits are present
on epidermal side and in 15% cases, it may be
seen on both epidermal and dermal sides.
EBA is a very difficult disease to treat. Steroids
in combination with dapsone or adjuvant
immunosuppressives (azathioprine, methotrexate, cyclophosphamide, cyclosporine,
high doses of colchicine) are the usual line of
therapy. Photopheresis and intravenous
immunoglobulin have been used. The
mechanobullous type is resistant to most
modalities of treatment. Supportive therapy
is warranted in all patients with EBA. This
includes instructions in open wound care and
strategies in avoiding trauma.

Cutaneous Tuberculosis and Atypical Mycobacterial Infections

13

Cutaneous Tuberculosis and


Atypical Mycobacterial Infections

Tuberculosis of the skin constitutes about 10% of all extra-pulmonary tuberculosis which in turn
constitutes only a fraction of all cases of tuberculosis.

CLASSIFICATION OF CUTANEOUS TUBERCULOSIS (TB)


Types

Mode of infection

Bacilli

Immunity

Tuberculin

Primary TB
* TB chancre
*Miliary TB

Inoculation
Hematogenous

+++
++

Secondary TB
*Lupus vulgaris
*TBVC
*Scrofuloderma
*TB gumma
*Orificial TB

Inoculation
Inoculation
Contiguous
Hematogenous
Autoinoculation

+/+
++
++
++

+++
+++
+++
+
+

+++
+++
++
+
-

Tuberculids
*Papulonecrotic
*Lichen scrofulosorum
*Erythema induratum

Hematogenous

+++
+++
+++

+++
+++
+++

Cutaneous Tuberculosis can be


Classified as Given Blow
1. Inoculation tuberculosis (exogenous source):
a. Tuberculous chancre
b. Tuberculosis verrucosa cutis
c. Lupus vulgaris (some).
2. Secondary tuberculosis (endogenous source)
a. Contiguous spread scrofuloderma
b. Autoinoculation orificial tuberculosis.

3. Hematogenous tuberculosis acute miliary


tuberculosis, lupus vulgaris (some),
tuberculous gumma.
4. Eruptive tuberculosis (tuberculids)
a. Micropapular lichen scrofulosorum
b. Papular papular or papulonecrotic
tuberculids
c. Nodular erythema induratum of Bazin,
nodular tuberculid.

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TUBERCULOUS CHANCRE

LUPUS VULGARIS

It occurs due to inoculation of M. tuberculosis into


the skin of an individual without natural or
acquired immunity to tubercle bacilli.
Usually seen in face or limbs of children
Presents as a brownish red papule or nodule
that ulcerates to form a ragged ulcer with
undermined edge
Associated with regional lymphadenopathy
If untreated, chancre heals slowly over
months.

It is the most common type of progressive


cutaneous tuberculosis occurring in people with
moderate or high immunity.
Lupus vulgaris arises from normal skin but it
can arise in a scar of scrofuloderma.
The initial lesion is a reddish-brown, soft,
gelatinous plaque which increases in size and
extends peripherally.
Peripheral extension occurs with resultant
scarring (Figs 13.1 and 13.2).
Diascopy reveals apple jelly nodules in the
periphery.
The various morphological forms are plaque
type, ulcerative and mutilating form,
vegetative form and tumor like.
Nasal mucosa can be involved leading to
resultant destruction of nasal septum.
Rarely squamous cell carcinoma can occur.

MILIARY TUBERCULOSIS
Miliary TB occurs due to hematogenous
dissemination of tuberculosis in infants and
children or immunosuppressed
The skin lesions are varied may manifest as
crops of bluish papules, vesicles, pustules or
hemorrhagic lesions
Diagnosis established by skin biopsy which
shows acid fast bacilli
The primary source of TB should be identified
and treated.

Fig. 13.1: Lupus vulgarisplaque lesion with active


infiltrated border with central scarring over the thigh

Differential Diagnosis
Discoid lupus erythematosus Lesions begin
as dull red macules or indurated plaques that
develop an adherent scale (carpet tack sign),
mainly over head and neck and evolve with
atrophy, scarring and pigmentary changes.

Fig. 13.2: Lupus vulgarisverrucous plaque with


trailing scar and depigmentation in the groin

Cutaneous Tuberculosis and Atypical Mycobacterial Infections


Lupoid form of cutaneous leishmaniasis may
be impossible to distinguish clinically.
Histopathology distinguishes them.
Deep mycoses may resemble vegetating form
of lupus vulgaris. It can be differentiated by
histology and culture reports.
Leprosy nodules of leprosy are firmer
compared to those of lupus vulgaris. Other
signs of leprosy would help in distinguishing
it from lupus.
Sarcoidosis nodules of sarcoidosis resemble
grains of sand on palpation and the surface is
shiny and waxy.
Psoriasis - usually multiple lesions over the
extensor aspect of limbs and lumbosacral
areas, but are less infiltrated compared to
lupus vulgaris. They are associated with
silvery white micaceous scales and there is
no evidence of scarring.
Lichen simplex chronicus of perianal area
may mimic lupus vulgaris. There will be no
scarring and it will be itchy and hyperpigmented rather than reddish brown in
lupus vulgaris.

TUBERCULOSIS VERRUCOSA CUTIS


(WARTY TUBERCULOSIS, ANATOMISTS
WART)
Tuberculosis verrucosa cutis (TBVC) manifests as
a warty growth that occurs as a result of

Fig. 13.3: Tuberculosis verrucosa cutisverrucous


plaque with fissuring and foetid discharge over the
sole

inoculation of mycobacteria into the skin of a


previously infected individual with moderate to
high immunity.
Lesions occur in exposed areas such as foot
(Figs 13.3 and 13.4), hand, ankle and rarely
buttock.
The lesions start as warty papules that enlarge
to a verrucous plaque with serpiginous border.

Differential Diagnosis
TBVC should be differentiated from other
warty lesions such as verruca vulgaris, lupus
vulgaris (usually not hyperkeratotic, diascopy
demonstrates apple jelly nodules), chromoblastomycosis, hypertrophic lichen planus
(has multiple itchy lesions usually over lower
legs with evidence of lichen planus
elsewhere), leishmaniasis and tertiary
syphilis.
SCROFULODERMA
Scrofuloderma results from breakdown and
involvement of skin overlying a tuberculous focus
such as lymph node, bone, joint, epididymis or
lacrimal gland.
It manifests as bluish red nodules overlying
lymph nodes or joints that break down to form
undermined ulcers with a bluish edge (Figs
13.5 and 13.6).

Fig. 13.4: Tuberculosis verrucosa cutisverrucous


plaque with fissuring over the dorsum of great toe

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Essentials in Dermatology

Fig. 13.5: Scrofulodermasubcutaneous nodules


and ulcerated lesions along with scarring in the neck

Fig. 13.7: Scrofulodermasinuses and fistulae


healing with puckered scarring over the back

by multiple sinuses, puckered scarring and


formation of new nodules that lead to an
uneven indurated lumpy surface. Pus
containing sulphur granules discharging from
multiple sinuses is quite characterstic,
distinguishing it from scrofuloderma.

TUBERCULOUS GUMMA (METASTATIC


TUBERCULOUS ULCER)

Fig. 13.6: Scrofulodermaruptured bluish nodules


with undermined edges in the axilla

The resultant sinuses and fistulae heal with


puckered scarring (Fig. 13.7).

Differential Diagnosis
Scrofuloderma should be differentiated from
hidradenitis suppurativa, acne conglobata,
syphilitic gumma and actinomycosis.
Cervicofacial actinomycosis is characterized

It occurs due to hematogenous dissemination


during periods of bacillemia and lowered
resistance
Common in poorly nourished children
Presents as firm subcutaneous nodules that
soften and ulcerate to form undermined ulcers.

ORIFICIAL TUBERCULOSIS
Tuberculosis of mucosa or skin adjoining orifices
in a patient with advanced internal tuberculosis
is known as orificial TB (Fig. 13.8).
The affected patient is usually an adult of poor
general health

Cutaneous Tuberculosis and Atypical Mycobacterial Infections


Lesions occur in the mouth, tongue, or
genitalia as reddish nodules that break down
to form painful shallow ulcers with bluish
undermined edges.

TUBERCULIDS
Tuberculids are hypersensitivity eruptions which
arise in response to an internal focus of
tuberculosis and clear with antituberculous
therapy.

Papulonecrotic Tuberculid
It is an eruption of necrotizing papules,
particularly affecting extremities and
occurring in more or less symmetric crops.
The lesions are hard, dusky papules that crust
or ulcerate to heal with atrophic scars (Fig.
13.9).
Papulopustular secondary syphilis, pityriasis
lichenoides et varioliformis acuta, ChurgStrauss granuloma, lymphomatoid papulosis,
perforating granuloma annulare, perforating
collagenosis and necrotizing or septic
vasculitis share clinical and histologic
features with papulonecrotic tuberculid.

The lesions are localized to legs (calf region)


of middle aged women with erythrocyanotic
circulation.
It must be distinguished from erythema
nodosum (short duration, rapid development,
affects chiefly anterior aspect of the leg, more
painful tender nodules that do not ulcerate),
nodular vasculitis, polyarteritis nodosa,
tertiary syphilis (gumma is usually unilateral
and single, serology and histology helps), and
other infectious and inflammatory
panniculitis.

Diagnosis
Absolute Criteria
Positive culture for M. tuberculosis
Guinea pig inoculation
PCR for M. tuberculosis.

Others

Proven TB elsewhere in the body


Presence of AFB in the lesion
Histopathology
Positive tuberculin test (Fig. 13.12)
Clinical history and physical signs
Response to therapy.

Lichen Scrofulosorum
It presents as grouped, closely set minute
perifollicular papules over the trunk (Fig.
13.10) or extremities in children with
tuberculous disease
Lichen nitidus, lichen planus, secondary
syphilis and sarcoidosis should be considered
in the differential diagnosis.
Erythema induratum
Presents as persistent or recurrent
erythematous tender nodular lesions
(Fig, 13.11) (usually ulcerate in contrast to
erythema nodosum) that occur secondary to a
tuberculous focus elsewhere.

Fig. 13.8: Orificial tuberculosispainful, shallow


ulcers with undermined edges around the anus

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Essentials in Dermatology

Fig. 13.9: Papulonecrotic tuberculidatrophic scars


over the thigh following papulonecrotic tuberculid

Fig. 13.10: Lichen scrofulosorummicropapular


eruption over the trunk

Fig. 13.11: Erythema induratumerythematous


indurated tender noduloplaque lesion over the leg

Fig. 13.12: Positive Mantoux reaction

Treatment
Anti tubercular therapy with three or four drugs
is given for a period of 6 to 9 months based on the
type of tuberculosis. Most regimens contain
isoniazid, rifampin, ethambutol and pyrazinamide.

ATYPICAL MYCOBACTERIAL
INFECTIONS
Atypical mycobacteria can present with varied
cutaneous features in normal as well as
immunosuppressed patients. The main features
are summarized in the following table.

Cutaneous Tuberculosis and Atypical Mycobacterial Infections

SUMMARY OF ATYPICAL MYCOBACTERIAL INFECTIONS


Organism

Disease

Clinical Features

M.marinum

Swimming pool granuloma,


fish tank granuloma

M.ulcerans

Buruli ulcer

Warty plaque or sporotrichoid


lesions on knees, elbows and
feet
Subcutaneous nodules rupture
to form shallow ulcers with
necrotic fat in the floor
Nodules, leg ulcers and papules
Disseminated heterogenous
infection in HIV patients
Cellulitis and subcutaneous
nodules
Cutaneous abscesses, chronic
ulcerative and nodular skin
lesions, and cervical
lymphadenitis

M.avium complex

M.chelonae
Injection
M.fortuitum
abscess
M. scrofulaceum Cutaneous abscesses

Treatment
Rifampin and ethambutol or
tetracycline
Surgery is the treatment of choice
followed by rifampin or
cotrimoxazole
Combined therapy of isoniazid,
rifampin and streptomycin.
Surgical debridement and amikacin
or doxycycline or ciprofloxacin
Treatment- surgical treatment
of infected lymph node.
Widespread disease- treatment
same as in M avium complex

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Essentials in Dermatology

14

Connective Tissue Disorders

The term connective tissue disorders (Collagen


vascular disorders) encompasses a group of
multi-system disorders which have certain
features in commonthe existence of
autoimmunity in the form of antibody production
or disordered cell mediated immunity, vascular
abnormalities in the form of Raynauds
phenomenon, occlusive vascular diseases or
vasculitis, arthritis, arthralgia and skin disease.
Involvement of skin is significantly predominant
in this group of disorders. They are: systemic
lupus erythematosus, systemic sclerosis,
dermatomyositis, overlap-syndrome and mixed
connective tissue disease.

LUPUS ERYTHEMATOSUS
Lupus erythematosus (LE) is a multisystem
disease of unknown origin characterized by the
production of numerous diverse types of
autoantibodies. Lupus erythematosus have wide
spectrum of manifestations ranging from solitary
chronic skin lesions in chronic discoid lupus
erythematosus (DLE) to wide spread
polymorphous lesions in subacute lupus
erythematosus (SCLE) to multiple organ
involvement in systemic lupus erythematosus
(SLE).

DISCOID LUPUS ERYTHEMATOSUS


(DLE)
Most common form of cutaneous lupus
erythematosus.
Characterized by discrete, discoid, welldefined erythematous plaques covered with
adherent scales (Figs 14.1 and 14.2). The
lesions slowly expand with active
inflammation at the periphery, leaving
depressed scars, telangiectasias and
permanent depigmentation (Fig. 14.3).
The central scarring with atrophy is very
characteristic.
These lesions occur most often on the face,
scalp, ears or neck.
Peeling the scale reveals an undersurface that
looks like a carpet penetrated by several carpet
tacks called carpet tack sign.
Scarring alopecia occurs in 60% of the cases
(Fig. 14.4).

Types
Localized DLE: lesions occur only on the head
or neck.
Generalized DLE: lesions are seen both above
and below the neck (Figs 14.5 and 14.6).

Connective Tissue Disorders

Fig. 14.1: Discoid lupus erythematosuswell


defined, discoid, erythematous plaques over the
chest

Fig. 14.3: Discoid lupus erythematosusdiscoid


lesions evolving with central depigmentation and
atrophy

Fig. 14.2: Discoid lupus erythematosusdiscoid


lesions involving the face

Fig. 14.4: Discoid lupus erythematosusscarring


alopecia

Other types are hypertrophic, tumid, lupus


erythematosus profundus, mucosal DLE,
chilblain lupus, etc.

The risk is higher in patients with


disseminated DLE (22%).

Prognosis
DLE has a chronic progressive course.
Patients with hematological and serological
abnormalities have 6.5% risk of developing
overt SLE.

Diagnosis
Based on clinical features and histopathology.
Direct immunofluorescence (DIF)-deposits of
IgG and C3 along the basement membrane in
affected skin in up to 80%, but normal nonsun exposed skin always negative. Negative

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Essentials in Dermatology
brown initially, later becoming red brown in color,
have a characteristic translucence and woodgrains appearance, telangiectasia may be seen on
the surface of the lesions, no prominent follicles),
tinea faciei (KOH examination), granuloma faciale
(brown color, no scarring), psoriasis (silvery
scales), rosacea (pustules, ears spared) and
Jessners lymphocytic infiltrate. In each case,
histology is most helpful.
The hypertrophic type of DLE may be
confused with hypertrophic lichen planus,
prurigo nodularis. LE profundus should be
differentiated from other types of panniculitis.
Mucosal DLE should be differentiated from
mucosal lichen planus.

Fig. 14.5: Generalized discoid lupus


erythematosusdiscoid lesions involving the trunk

Fig. 14.6: Generalized discoid lupus


erythematosusdiscoid lesions involving the feet

or low titer ANA; sometimes higher titer in


disseminated type. Exclude SLE.

Differential Diagnosis
Lupus vulgaris (rarely symmetrical, has
progression on one side and healing with
scarring on the other side, apple-jelly nodules on
diascopy), sarcoidosis (lesions are yellowish

Histopathology
Characteristic histopathological changes are:
Hyperkeratosis with follicular plugging
Irregular atrophy of the stratum malphighi
Liquefactive degeneration of the basal cell
layer and
Patchy perivascular and periappendageal
lymphocytic infiltration.
Degenerative changes in the connective
tissueshyalinization, edema, fibrinoid
change in the upper dermis.
Valuable clues are thickened periodic acidSchiff (PAS) positive basement membrane and
deposition of mucin.
Treatment
Photoprotection and topical sunscreens
Topical high potency or intralesional steroids
Topical calicineurin inhibitors (pimecrolimus,
tacrolimus)
Systemic therapy for widespread recalcitrant
disease are antimalarials (Hydroxychloroquine 200-400 mg daily or chloroquine
250 mg daily, monitoring by an ophthalmologist required every 6-12 months), dapsone
(50-100 mg daily), thalidomide (50-200 mg
daily), corticosteroids (prednisolone 40 mg

Connective Tissue Disorders


daily short courses), oral auranofin, etretinate,
isotretinoin, clofazimine, methotrexate,
azathioprine, cyclophosphamide, etc.

SUBACUTE LUPUS ERYTHEMATOSUS


(SCLE)
Characterized by scaly papules on the
shoulders, extensor surfaces of the upper
extremities, upper chest, upper back and neck
in widespread and symmetric fashion.
Above lesions evolve into two morphological
types either into papulosquamous or annular
and polycyclic lesions. Rarely erythema
multiforme-like with blisters (Rowell
syndrome).
All patients with SCLE have mild systemic
complaints and 50% of the patients fulfill the
criteria for the diagnosis of SLE.
SCLE patients have antibodies to the cellular
antigens Ro/SS-A and La/SS-B.
Children born to mother with SCLE may have
congenital heart block (especially, those with
anti Ro/SS-A antibody).
Differential diagnosis: To be differentiated
from discoid lupus erythematosus, psoriasis,
tinea corporis, annular erythemas, tinea
versicolor and rarely erythema multiforme.

It has been postulated that four or more genes


are involved in predisposing an individual to
SLE.

Pathogenesis
LE is a multifactorial disease with genetic and
immunopathologic abnormalities. The release of
nuclear antigens because of enhanced apoptosis
is a key factor.
Important predisposing factors are genetic
predisposition (HLA-B8, DR2, DR3, DQwl,
DRB1), complement defects, exogenous factors
(UV radiation, and medications), and individual
factors (hormone status, altered immune status).
The 1982 Revised Criteria for
Diagnosis of SLE are:
1. Malar rash: Fixed erythema, flat or raised,
over the malar eminences, tending to spare
the nasolabial folds (Fig. 14.7).
2. Discoid rash: Erythematous raised patches
with adherent keratotic scaling and follicular
plugging; atrophic scarring may occur in
older lesions.

Treatment
Same as for discoid lupus erythematosus.
SYSTEMIC LUPUS ERYTHEMATOSUS
(SLE)
SLE a multisystem disorder, primarily affects
skin, joints and vascular system. The age of onset
is usually between 16 and 42 years occurring more
frequently in females (F: M:: 8:1).

Etiology
Exact cause is unknown but there is evidence
to suggest the role of genetic, immune and
various environmental factors.

Fig. 14.7: Systemic lupus erythematosusmalar


and photosensitive rash over the face

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Essentials in Dermatology
3. Photosensitivity: Skin rash as a result of
unusual reaction to sunlight, by patient
history or physician observation.
4. Oral ulcers: Oral or nasopharyngeal
ulceration, usually painless, observed by a
physician.
5. Arthritis: Nonerosive arthritis involving two
or more peripheral joints, characterized by
tenderness, swelling, or effusion. or
6. Serositis:
a. Pleuritisconvincing history of pleuritic
pain or rub heard by a physician or
evidence of pleural effusion.
b. Pericarditisdocumented by ECG or rub
or evidence of pericardial effusion.
7. Renal disorder:
a. Persistent proteinuria > 0.5 g/day or
greater than 3+ if quantitation not
performed or
b. Cellular castsmay be red cell,
hemoglobin, granular, tubular, or mixed.
8. Neurologic disorder:
a. Seizuresin the absence of offending
drugs or known metabolic derangements,
e.g. uremia, ketoacidosis, or electrolyte
imbalance or
b. Psychosisin the absence of offending
drugs or known metabolic derangements,
e.g. uremia, ketoacidosis, or electrolyte
imbalance.
9. Hematologic disorder:
a. Hemolytic anemiawith reticulocytosis
or
b. Leukopenia < 4000/mL on two or more
occasions or
c. Lymphopenia < 1500/mL on two or more
occasions or
d. Thrombocytopenia<100,000/mL in
the absence of offending drugs.
10. Immunologic disorder:
a. Anti-DNAantibody to native DNA in
abnormal titer or
b. Anti-Smpresence of antibody to Sm
nuclear antigen or

c. Positive finding of antiphospholipid


antibodies based on (1) an abnormal
serum level of IgG or IgM anticardiolipin
antibodies, (2) a positive test result for
lupus anticoagulant using a standard
method, or (3) a false-positive serologic
test for syphilis known to be positive for
at least 6 months and confirmed by
Treponema pallidum immobilization or
fluorescent treponemal antibody
absorption test.
11. Antinuclear antibody (ANA): An abnormal
titer of antinuclear antibody by immunofluorescence or an equivalent assay at any
point in time and in the absence of drugs
known to be associated with drug-induced
lupus syndrome.
Note: The proposed classification is based on 11
criteria. For the purpose of identifying patients in
clinical studies, a person shall be said to have
SLE if any 4 or more of the 11 criteria are present,
serially or simultaneously, during any interval of
observation.

Chief Cutaneous Features


Butterfly rash.
Photosensitivity.
Raynauds phenomenon.
Non-scarring alopecia-short hairs in the
frontal region are referred to as lupus hairs.
Urticarial vasculitis.
Mouth ulceration.
Bullous lesions.
Chronic discoid LE lesion.
Cutaneous vasculitis.
Other features are vasculopathy, periungual
telangiectasia, leg ulcers, erythema multiforme, thrombophlebitis, peripheral gangrene,
etc.
Investigations
Complete blood count, ESR.
Urine analysis for microscopic hematuria and
proteinuria.

Connective Tissue Disorders


Skin biopsy- There is no single diagnostic
pathological feature in the skin, but a
combination of features aids diagnosis. Some
changes are similar to DLE. The primary
pathological lesions of SLE are fibrinoid
necrosis, collagen sclerosis, necrosis and
basophilic body formation and vascular
endothelial thickening.
LE cell test to demonstrate LE cell (a neutrophil
containing engulfed nuclear material) or
rosette phenomenon (neutrophils surrounding nuclear debris, trying to engulf it).
Lupus band test- deposits of IgG and C3 along
the basement membrane on normal, non-sun
exposed skin suggest SLE but is no longer an
accepted criterion. It positively correlates with
the presence of anti-ds DNA antibodies and
with risk for developing LE nephritis.
C3, C4, CH50 levels.
Serum globulins are frequently raised
especially gamma globulin.
Rheumatoid factor occurs in approximate 40%
cases.
ANA (Commonest pattern is homogeneous;
peripheral pattern-predictor of renal
involvement).
ndsDNA.
Antibodies-SSA (Ro), SSB (La), Sm, nRNP.

Differential Diagnosis
SLE must be differentiated from dermatomyositis,
erythema multiforme, polyarteritis nodosa, acute
rheumatic fever, rheumatoid arthritis, pellagra,
pemphigus erythematosus, drug eruption,
hyperglobulinemic purpura, Sjogrens syndrome,
necrotizing angiitis and myasthenia gravis. In
SLE, there may be fever, arthralgia, weakness,
lassitude, diagnostic skin lesions, an increased
ESR, cytopenias, proteinuria, immunoglobulin
deposition at dermoepidermal junction and a
positive ANA test. Biopsies of skin lesions and
involved kidney may also be diagnostic.

Treatment
SLE with only cutaneous lesions and arthritis:
Photoprotection.
NSAIDs, antimalarials, prednisolone ( 1-2 mg
per kg body weight daily).
Dapsone most effective in urticarial vasculitis
and bullous SLE.
Severe Disease with
End-organ Damage
Steroid pulse therapy.
Immunosuppressants such as azathioprine
(100-150 mg daily), cyclophosphamide (50100 mg daily), methotrexate (7.5-20 mg
weekly), chlorambucil, mycophenolate mofetil
(2 gm daily) and cyclosporine (5 mg/kg body
weight daily).
Experimental Therapies
Intravenous immunoglobulins, plasmapheresis, anti CD20 (rituximab), -CD40 and
TNF alpha antibodies.
Course and Prognosis
The course of the SLE is very variable. Acute
fulminating cases are much less common than
subacute cases, which smoulder on for many
years.
SCLERODERMA
Scleroderma (Gr. Skleros hard, and derma skin)
is a connective tissue disorder characterized by
generalized or localized sclerosis of the skin. The
localized type is called morphea.

Classification
Morphea Localized (Circumscribed plaque,
morphea profundus, bullous, linear, en coup de
sabre) and generalized.

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Essentials in Dermatology
Systemic sclerosis LM (Limited cutaneous) SSc,
DC (Diffuse cutaneous) SSc.
Occupational scleroderma Polyvinylchloride,
perchlorethylene, trichloroethylene, organic
solvents, malathion, DDT, epoxy resins, silicosis.
Iatrogenic scleroderma Bleomycin, carbidopa,
pentazocine, cocaine, appetite suppressants,
silicone or paraffin implants, GVHD.
Pseudoscleroderma Scleroedema of Buschke,
Scleromyxoedema, prophyria cutanea tarda,
phenylketonuria.
Primary systemic amyloidosis, carcinoid
syndrome, hypothyroidism.
Miscellaneous Toxic oil syndrome, eosinophilic
fascitis.

MORPHEA
Localized Morphea
Most common form of morphea.
Occurs most commonly in females than males
and primarily in young adults.
Seen commonly on the trunk.
The lesion of morphea may begin as
erythematous macule, evolve into ivory-

Fig. 14.8

colored center and violaceous bordered


plaque.
Lesions slowly involute over 3 to 5 years
period leaving permanent atrophic skin or
normal appearing skin behind.

Generalized Morphea
Lesions are more numerous and larger
Often coalesce to involve extensive portions
of the body.
Muscle atrophy may be associated.
Pansclerotic Morphea (Morphea
Profunda)
Sclerosis involves dermis, panniculus, fascia,
muscle and bones.
There is disabling limitation of the joints.
En Coup De Sabre
It is a variant of linear scleroderma involving
scalp parasagitally on frontal scalp and
forehead (Figs 14.8 and 14.9).
Often has the configuration of the stroke of a
saber (en coup de sabre).
Differential Diagnosis
1. Morphea-like lesions can occur in sarcoidosis
and morpheic basal cell carcinoma.

Fig. 14.9

Figs 14.8 and 14.9: En coup de sabrelinear indurated depressed lesion in the midline over the forehead

Connective Tissue Disorders


Histopathology is required to differentiate
between them.
2. Lichen sclerosus atrophicus (ivory white
plaques with follicular delling and atrophy)
and subcutaneous zygomycosis may
resemble morphea. Fingers can be insinuated
below the plaques of subcutaneous
zygomycosis.
3. Pseudoscleroderma especially porphyria
cutanea tarda and graft versus host disease.
4. Drug reaction (bleomycin induced sclerosis,
atrophic morphoeic plaques from intramuscular injection of vitamin K or
subcutaneous corticosteroid injections).

Treatment
Natural history is towards spontaneous
resolution.
Topical steroid, intralesional steroids and oral
chloroquine.
Topical calcipotriol.
Bath PUVA or UVA1.
For widespread or rapidly advancing disease
consider therapy usually reserved for systemic
sclerosis (corticosteroids, d-penicillamine,
cyclopsporine, low dose methotrexate,
etretinate, phenytoin, plasmapheresis).
Physiotherapy may be helpful in preventing
joint deformities.

Endothelial cell injury.


Dysregulation of the immune system.

Classification
Diffuse disease: Characterized by extensive
proximal and truncal skin induration.
Limited disease: Induration is confined to
hands, forearm, face, and legs. Its variant is
called CREST syndrome (ThibiergeWeissenbach syndrome) (Calcinosis,
Raynauds phenomenon, Esophageal dysfunction, Sclerodactyly and Telangiectasia).
American Rheumatism
Association Criteria
Major
Scleroderma proximal to the digits, affecting
limbs, face, neck and trunk.
Minor
Sclerodactyly (Fig. 14.10).
Digital pitted scarring (Fig. 14.11).
Bilateral basal pulmonary fibrosis.
One major criterion or two or more minor
criteria suggest the diagnosis of systemic sclerosis.
Note: These criteria have 97% sensitivity and 98%
specificity.

SYSTEMIC SCLEROSIS
It is characterized by cutaneous and internal
organ fibrosis. Raynauds phenomenon is the
earliest feature and may precede the onset of
disease by months or years. The heart, lungs,
gastrointestinal, kidney and other organs may be
involved.

Pathogenesis
Exact cause of systemic sclerosis is unknown.
Important steps in its pathogenesis include:
Excessive synthesis of collagen and matrix
macromolecules.

Fig. 14.10: Progressive systemic sclerosis


sclerodactyly with calcification over the interphalangeal joints of fingers

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Essentials in Dermatology

Cutaneous Manifestations
Three phases of dermal involvement can be
distinguished:
1. Edematous phase (stiff, puffy, fingers)
2. Indurative phase (hard, tight, hide bound)
3. Atrophic phase (softened skin, burnt out).
Hands and Feet
Early: Raynauds phenomenon.
Swollen or tumid fingers and hands.
Round finger pad sign-fingers lose their
normal peaked contour but rather appear as
rounded hemisphere when viewed from the
side.
Painful ulcerations at fingertips (Rat bite ulcer)
with pitted scars.
Late: sclerodactyly (induration of skin over
the fingers) with tapering of fingers (Figs 14.10
and 14.11).
Skin is tightly bound down.
Leathery crepitations over joints and flexion
contractures.
Heuck-Gottron sign- loss of cuticle with
telangiectases.
Bony resorption.
Atrophy of the pulp of the fingers.
Gangrene of the fingers.
Pigmentation.
Calcinosis.

Fig. 14.12: Progressive systemic sclerosismask


like facies, thinning of lips and small sharp nose

Face
Periorbital edema is the early manifestation
Late manifestations include: Mask like facies
(difficulty in eversion of lower eyelids),
thinning of lips, microstomia, radial perioral
furrowing, small sharp nose (Fig. 14.12),
telangiectasia (mat-like) and diffuse
hyperpigmentation.
Forehead is smooth and shiny, and skin is
bound down and hard.
There is reduced wrinkling on the forehead
and mandibular atrophy.
Trunk

Fig. 14.11: Progressive systemic sclerosisfinger


tip pits due to scarring

Early: tense, stiff and waxy appearing skin


that cannot be pinched and folded.
Late: impairment of respiratory movement of
chest wall and of joint mobility.
Neck sign-ridging and tightening of the
neck on extension due to sclerosis.

Connective Tissue Disorders

Fig. 14.13: Progressive systemic sclerosissalt and


pepper pigmentationdepigmentation with
speckled hyperpigmented macules over the front of
the chest

Other Changes
Salt and pepper pigmentation (Fig. 14.13),
gangrene of fingers, mat like telangiectasia, leg
ulcers and livedo reticularis.

Organ Involvement in PSS


Esophageal fibrosis, pulmonary interstitial
fibrosis, myocardial fibrosis, small intestinal
fibrosis, large intestinal fibrosis, renal
involvement, skeletal muscle atrophy and
thyroid fibrosis.
Others are bone, eye, CNS, teeth, tendons.
Investigations
Routine tests such as complete hemogram,
liver function tests, sedimentation rate,
C-reactive protein.
Skin biopsy- Dermal sclerosis typically results
in rectangular punch biopsy specimen. As the
dermis replaces the subcutaneous tissue,
eccrine glands appear to be in the mid portion
of the thickened dermis. The subcutaneous fat
is quantitatively reduced and adventitial fat
is lost. On DIF testing of skin the nucleolus
may be stained in the keratinocytes if
antinucleolar circulating antibodies are
present and a pepper dot epidermal nuclear

pattern may be seen in CREST patients who


have anticentromere antibodies in their serum.
Nail fold capillary microscopy.
ANA (+ve in more than 90%), Ab-SSA, SSB,
Sm, nRNP, Scl-70 (specific for diffuse systemic
sclerosis), anticentromere antibody (specific
for CREST syndrome).
Rheumatoid factor positive in 30%
Organ workup: urine analysis, barium
swallow, esophageal manometry, barium
enema, chest x-ray and pulmonary function
test.

Differential Diagnosis
1. Generalized
morphea
(Raynauds
phenomenon is rare, systemic involvement is
unusual, no atrophic stage, no facial
telangiectasia or perioral furrowing, skin of
trunk and limbs are equally involved)
2. Pseudosclerodermas (Specific features of each
should be looked for)
3. Occupational and iatrogenic scleroderma
(History of specific exposure should arouse
the suspicion).
4. Other collagen vascular disorders- mixed
collagen vascular disorders, overlap
syndromes
5. Graft versus host disease
Prognosis and Cause of Death
Course of the disease is variable. Death occurs
from intercurrent infection, respiratory failure,
cardiac failure, renal failure, sometimes,
malignant hypertension and perforation of the
gastrointestinal tract.
Other Conditions Where Sclerodermoid
Changes are Seen
Phenylketonuria, progeria, Rothmund-Thomson
syndrome, Werners syndrome, porphyria
cutanea tarda, primary systemic amyloidosis,
Hashimotos disease, carcinoid syndrome,

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Essentials in Dermatology
childhood diabetes mellitus and drugs
(bleomycin, pentazocine, carbidopa, and
5-hydroxytryptophan).

Treatment
There is no specific treatment and no therapy is
known to alter the course of a disease. Treatment
is primarily directed towards complications.
Pharmacological agents used for systemic
sclerosis can be grouped into various categories.
Collagen modulators: D-penicillamine (125
mg alternate days, 750-1500 mg per day),
relaxins, and interferons.
Vasoactive agents: Captopril, nifedipine and
pentoxifylline.
Immunosuppressive agents: Systemic corticosteroids (prednisolone 0.5 mg mg per kg
body weight daily), azathioprine (1-2 mg mg
per kg body weight daily), cyclophosphamide
(2 mg per kg body weight daily), cyclosporine
(3-5 mg per kg body weight daily) and
methotrexate (20-30 mg weekly).
For internal organ involvement Angiotensin
converting enzyme inhibitors are treatment of
choice for renal hypertension, proton pump
inhibitors (omeprazole 20-40 mg daily) indicated
for esophageal dysfunction and for pulmonary
hypertension intravenous prostacyclin; interstitial lung disease may respond best to
cyclophosphamide. Physical therapy can help
avoid contractures and retain function.

Cutaneous Manifestations of
Dermatomyositis
Pathognomonic
Gottrons papules: Violaceous, flat topped
papules on interphalangeal joints and
knuckles. Similar lesions may occur over other
bony prominences such as knees, elbows and
medial malleoli.
Gottrons sign: Symmetric macular violaceous
erythema with or without edema over the
above mentioned sites.

Characteristic
Periorbital violaceous erythema with
associated edema of eyelids and periorbital
tissue (heliotrope rash) (Fig. 14.14).
Periungual telangiectasia with associated
dystrophic cuticles.
Macular violaceous erythema overlying the
dorsal hands, extensor forearms and arms,
deltoids, posterior shoulders, nape of neck, V
area of neck, upper chest and forehead.
Shawl sign-erythema and scale (with or
without poikiloderma) over the shoulder
regions.
Mechanics hands: Bilaterally symmetrical
confluent hyperkeratosis distributed along the

DERMATOMYOSITIS
It is a systemic, inflammatory disease involving
primarily skin and muscles. Symmetric, proximal
muscle weakness occurs especially in the hips,
thigh, and upper arm. Patients with only
muscular symptoms and signs but no cutaneous
findings are said to have polymyositis. These can
be associated with malignancies especially in
adulthood carcinoma of lung, breast, female
genital tract, stomach, kidney and testes.

Fig. 14.14: DermatomyositisHeliotrope rash


periorbital violaceous erythema with associated
edema of eyelids

Connective Tissue Disorders


ulnar aspects of the thumbs and radial aspects
of the index and middle fingers with
occasional extension to the palmar surface.

Compatible
Poikiloderma atrophicans vasculare
Calcinosis cutis.
Systemic features include arthritis (25%), oral
ulcers (20%), calcinosis (distinctive feature of
juvenile dermatomyositis), pulmonary fibrosis
(20%), gastrointestinal ulcerations and
hemorrhages, occasionally myocarditis or
myopathy.

Investigations
Skin biopsy, muscle biopsy, muscle enzymes,
electromyography, ANA, Ab-SSA, SSB, Sm,
nRNP, Jo-1and PM-1.
Diagnostic Criteria
1. Progressive symmetric proximal muscle
weakness.
2. Elevated muscle enzyme levels.
3. Abnormal electromyogram.
4. Abnormal muscle biopsy.
5. Characteristic cutaneous manifestations.
Definite: 5 plus three other criteria.
Probable: 5 plus two other criteria.
Differential Diagnosis
Includes other myopathies (inclusion body
myositis, muscular dystrophy, neuromuscular
atrophy, myasthenia gravis, thyrotoxic myopathy,
Cushings disease, sarcoidosis, alcoholism),
drugs (lipid lowering agents, hydroxyurea,
NSAIDs), overlap syndromes, vasculitis,
polymyalgia rheumatica and trichinosis.
Treatment
Skin disease: Topical steroids, hydroxychloroquine.
Muscle disease: Systemic corticosteroids are
first line therapy. Second line agents are

methotrexate (5-15 mg weekly) and


azathioprine (1-3 mg per kg body weight
daily).
Intravenous immunoglobulins are indicated
for resistant cases, children with vasculitic
component and those with steroid induced
diabetes mellitus.
Bed rest during flares, physical therapy when
stable.
Appropriate treatment of underlying
malignancy where it is associated.

OVERLAP SYNDROME
The term overlap syndrome may be used
when patients exhibit symptoms of more than
one connective tissue disease.
Such patients may meet diagnostic criteria for
one disease but also have atypical
manifestations or findings suggestive of
second diagnosis.
Systemic sclerosis combined with dermatomyositis is the most frequently seen overlap
syndrome.

MIXED CONNECTIVE TISSUE DISEASE


This entity was first described by Sharp and
colleagues in 1972.
These patients, predominantly female, show
features of SLE, systemic sclerosis, dermatomyositis and polymyositis.
All patients have high titer of antibody to U1
RNP (ribonucleoprotein).
Prominent clinical features include myositis,
pulmonary hypertension, Raynauds phenomenon, oesophageal hypomotility, swollen
hands and sclerodactyly.

SJGRENS SYNDROME (SS)


Sjgren in 1933 described a triad of
keratoconjunctivitis sicca, xerostomia and
rheumatoid arthritis.

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Essentials in Dermatology
Dry eyes and dry mouth occur in primary
Sjgrens syndrome or if associated with other
connective tissue disease then referred to as
secondary Sjgrens syndrome.
Most patients are aged 50 years or older and
are women.
Clinical features include xerostomia, rhinitis
sicca, vaginal dryness and dry eyes (Fig.
14.15).
Skin manifestations of SS include vasculitis,
xerosis and annular erythema.
Rheumatoid factor is usually positive.
80% of patients have anti Ro/SSA antibodies.
Increased risk of developing lymphoreticular
malignancies.
No specific treatments available, only
symptomatic management.

eight or hour glass appearance (Fig. 14.16)


LSA of the penis in adults (Balanitis xerotica
obliterans) presents with acquired phimosis
or recurrent balanitis (Fig. 14.17).
LSA of vulva (Kraurosis vulvae) usually seen
in postmenopausal women.
LSA of the genitalia have increased risk for
squamous cell carcinoma (Fig. 14.18).

LICHEN SCLEROSUS ET ATROPHICUS


(LSA)
It is a chronic disease of the skin and mucous
membrane of unknown origin predominantly
affecting the females.
Early lesions of LSA are ivory white,
polygonal, flat-topped papules or plaques
with follicular plugs (delling). The lesions may
coalesce into large atrophic plaques.
In women, involvement of the vulvar and
perianal areas leads to the typical figure of

Fig. 14.15: Sjgrens syndromeface showing


submandibular salivary gland enlargement

Fig. 14.16: Lichen sclerosus et atrophicus of the vulvatypical figure of eight or hour glass appearance

Fig. 14.17: Lichen sclerosus et atrophicusivory


white indurated ring like lesion involvin preputial orifice

Connective Tissue Disorders

Fig. 14.18: Lichen sclerosus et atrophicusmale penis with


squamous cell carcinoma

Differential Diagnosis
On trunk- morphea, annular atrophic plaque type
of DLE and vitiligo, female genitalia-vitiligo,
chronic dermatitis, erosive lichen planus,
autoimmune bullous diseases, male genitaliabalanitis in all its variants, idiopathic phimosis,
erosive lichen planus.

Treatment
MedicalTopical steroids or intralesional
steroids, topical pimecrolimus or tacrolimus,
topical testosterone, topical tretinoin, UVB and
bath PUVA therapy. Surgical correction of
adhesions may be required.

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Essentials in Dermatology

15

Pigmentary Disorders

Human race has a wide variation in normal skin


color (pale white, light brown, dark brown and
black). Various skin chromophores like brown
(melanin), red (oxyhemoglobin), blue (deoxyhemoglobin) and yellow-orange (carotene) are
responsible for skin color. Of course, melanin is
the major component of skin color. Skin color
depends on the genetic make-up of an individual
and environmental effects like ultraviolet
radiation exposure. Melanocyte is the sole site of
melanin synthesis.
Pigmentary disturbances include decrease or
absence or increase in pigmentation which could
be either epidermal or dermal. These pigmentary
changes are a result of changes in number of
melanocytes or amount of melanin.
Pigmentary disorders may be congenital or
acquired, circumscribed or generalized,
hypomelanotic (Vitiligo, albinism, piebaldism,
Waardenburgs syndrome) or hypermelanotic
(Melasma, freckles, lentigo, Peutz Jeghers
syndrome). In this chapter, only the common ones
are discussed.

VITILIGO
Vitiligo is defined as a common, dermatological
disorder characterized by well-circumscribed,
milky-white cutaneous macules devoid of

identifiable melanocytes. In India, the incidence


of vitiligo is estimated to be between 3 and 4 %.
The term vitiligo is probably derived from the
Latin word vitium(blemish). Many ancient
terms are applied to this condition
shwetakustha in the sacred Indian book, Atharva
Veda (1400BC), and switra in Manusmriti (200
BC). In South India where the old Dravidian
language of Tamil is spoken, the condition is
known as ven kushtamwhite leprosy.

Pathogenesis
The white macules of vitiligo are the result of a
loss of melanocytes. The mechanism(s) by which
the melanocytes are lost may be multiple but have
not been identified unequivocally. Seven hypotheses, not mutually exclusive, have been
proposed to explain the causation of vitiligo:
Autoimmune: Strengthened by the
demonstration of specific autoantibodies to
melanocyte cell surface antigens and the
association of vitiligo with a variety of
autoimmune disorders.
Autocytotoxic: Also called the self-destruction
theory, it proposes melanocyte destruction by
intracellular retention of various precursors
of melanin synthesis.
Neural: Especially proposed to explain the
segmental type of vitiligo.

Pigmentary Disorders
Biochemical: Accumulation of pteridines (6biopterin and 7-biopterin) in the vitiliginous
skin causes the depigmentation.
Antioxidant deficiency theory: There is an
increased level of norepinephrine and
catecholamine derivatives in vitiligo skin
which leads to tissue ischemia and increased
activity of monoamine oxidase enzyme, which
in turn causes excess production of stress
related hydrogen peroxide. Reduced catalase
activity in vitiligo skin leads to impaired
degradation of H2O2 and accumulation of
superoxide radicals causing depigmentation.
Melanocyte growth factor reduction hypothesis:
In vitiligo, there is reduced melanocyte growth
factors derived from keratinocytes, fibroblasts
and other tissues.
Intrinsic (genetic) theory: An underlying
genetic/intrinsic factor predisposes some
individuals to be more prone to develop
vitiligo.

Clinical Features
The diagnostic lesion of vitiligo is the typical
vitiligo macule, which is of variable size,
round/oval in shape, has a milky white color
and scalloped margins (Fig. 15.1).
May appear at any age, however, the peak age
at onset has been reported to be five to thirty
years.
Prevalence is the same in both sexes.
The natural course of the disease is of gradual
progression, the lesions increasing both in
number and size. In some cases there may be
a rapid downhill course of vitiligo and this
has been termed galloping vitiligo or vitiligo
fulminans.
Segmental vitiligo and vitiligo in children
have a better prognosis.
Mucous membrane involvement is also noted
in vitiligo and is commoner, or rather, easily
detectable in dark-skinned races.
Leukotrichia refers to depigmentation of the
hair and may occur in some patients.

Fig. 15.1: Zosteriform vitiligomilky white macules


in a zosteriform distribution over the back.

Classification of Vitiligo
Localized
Focal vitiligo: This consists of one or more
macules in one area but not clearly in a
segmental or zosteriform distribution .
Segmental vitiligo: Number of macules
involving a unilateral segment of the body.
The lesions stop abruptly at the midline of the
affected segment (Fig. 15.1).
Mucosal vitiligo: Vitiligo affecting mucous
membranes of the lips, oral cavity or the
genitalia (Fig. 15.2).

Fig. 15.2: Vulval vitiligofemale genitalia involved


by milky white lesions

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Fig. 15.3: Acral vitiligovitiligo involving


hands and feet

Fig. 15.5: Lip tip vitiligo-lesions affecting the tips of


the digits and the lips

Lip-tip vitiligo: Lesions affecting the tips of the


digits and the lips (Fig. 15.5)
Mixed: Any combination- of vitiligo vulgaris
and acrofacial vitiligo or of vitiligo vulgaris
with segmental vitiligo.
Universal Vitiligo: This is the term used to
describe complete or near complete depigmentation.

Fig. 15.4: Vitiligo vulgarismultiple depigmented and


hypopigmented macules over the chest and
abdomen (Trichrome vitiligo)

Generalized
Acrofacial vitiligo: Lesions on the acral areas
(hands and feet) (Fig. 15.3) and on the face,
very often the perioral areas.
Vitiligo vulgaris: Multiple macules of variable
sizes over widely scattered areas often tending
to bilateral symmetry (Fig. 15.4).

Special Signs in Vitiligo


Trichrome vitiligo: The trichrome sign, also
termed vitiligo gradata describes a tan
colored zone (intermediate colour) between the
normal skin and the depigmented macules
(Fig. 15.2). Quadrichrome vitiligo implies the
presence of a fourth color dark brown at
the sites of perifollicular repigmentation.
Pentachrome vitiligo: Has five colorswhite,
tan, brown hyperpigmented, blue-grey hyperpigmented, and normal skin color.
Inflammatory vitiligo: Here skin lesions of
vitiligo have erythematous raised margin. This
should be differentiated from the erythema of
vitiligo macule following exposure to sunlight.
Koebners sign: This phenomenon, a common
feature of vitiligo, is defined as the
development of lesions along the lines of
specific trauma such as a cut, burn or abrasion.
It may be a marker of disease activity.

Pigmentary Disorders

Association of Vitiligo with Other


Diseases
Vitiligo has been shown to be associated with
autoimmune thyroid disease, Addisons disease,
pernicious anemia, diabetes mellitus and various
dermatological disorders like alopecia areata,
scleroderma, psoriasis and collagen vascular
disorders. The Vogt Koyanagi Harada syndrome
is an apparently rare, multisystem disease
characterized by vitiligo, poliosis, uveitis,
dysacousia, and alopecia.

Differential Diagnosis of
Localized Vitiligo Includes
Naevus depigmentosus (localized hypomelanosis present since birth), naevus anemicus
(pale area due to vasoconsconstriction), leprosy
(suspect in an endemic area, shows hypoanesthetic or anesthetic hypopigmented patch/
es), pityriasis alba (hypopigmented macular
lesions have ill defined margins with fine scaling,
self limiting course), postinflammatory hypomelanosis (such cases have preceding history of
dermatoses, and the skin lesions are ill defined),
tinea versicolor (typical localization to upper
trunk, pigmented macular lesions have fine
powdery scales, KOH +vity conclusive), ash-leaf
macules and confetti depigmentation of tuberous
sclerosis, and idiopathic guttate hypomelanosis
(tiny porcelain white macules, 2-6 mm with
distinct margins, localized to limbs in older
individuals).

Differential Diagnosis of
Generalized Vitiligo Includes
Chemical leukoderma (there is history of exposure
to phenolic germicides; confetti macules; localized
to the site of contact), leprosy (suspect in an
endemic area, has anesthetic hypopigmented
patch/es), mycosis fungoides (unpatterned
lesions, diagnosis confirmed by biopsy),
postinflammatory hypomelanosis (hypopig-

mented macules with ill defined border, history


of preceding dermatoses like psoriasis, eczema,
pityriasis rosea, etc. in same areas with pattern
mimicking it), tinea versicolor (has fine powdery
scales over the lesions, in doubt do KOH
examination of skin scrapings), Waardenburgs
syndrome, albinism and piebaldism (see next
page for last three).

Treatment
Treatment of vitiligo can be broadly divided into
medical and surgical modalities.
Medical management
Topical therapy
1. Photoprotection: It prevents sunburn and
Koebner phenomenon, prevents tanning of
uninvolved skin and therefore lessens
contrast between normal and depigmented
skin.
2. Topical potent corticosteroids.
3. Intralesional corticosteroids: (Triamcinolone
acetonide) especially for leukotrichia on
scalp.
4. Human placental extract.
5. Topical immunomodulators: Such as
tacrolimus (0.1%, 0.03%), pimecrolimus or
tacrolimus (0.1%) combined with narrow
band UVB three times a week.
6. Calcipotriol: Can be used as monotherapy or
combined therapy (sunlight, PUVA, or
narrow band UVB, clobetasol).
7. Topical pseudocatalase + calcium + UVB
8. Vitix: Formulation containing superoxide
dismutase and catalase. It removes
hydrogen peroxide from skin, thereby helps
in repigmentation.
9. Phenytoin local application: It inhibits release
of norepinephrine and activity of monoamine
oxidase, inhibits the production of
superoxide anion and suppresses cytotoxic
T- lymphocyte activity and induces type 2
like cytokine profile.

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10. Dead sea climatotherapy in combination with
pseudocatalase: Here patients take bath in
dead sea for 15 minutes twice daily followed
by a shower to wash off salt followed by
application of pseudocatalase cream prior
to sun exposure.
11. Topical prostaglandin analogues (PGE2).
12. Cosmetic camouflage where nothing works
or when on treatment.

Phenylalanine + UVA (PAUVA). Phenyalanine both topically and systemically can


be used in combination with UVA. Phenyalanine can be used at a dose of 50-100 mg/
kg body weight 45 minutes prior to UVA
exposure.
UVB radiation especially narrow band UVB
Targeted light therapy and Excimer laser (308
nm).

Systemic Therapy

Surgical Management
Indications
Stable vitiligo (i.e. no new lesions for last two
years)
Refractory to medical management.

1. Low dose oral corticosteroids-prednisolone


0.3 mg/kg body weight daily.
2. Oral dexamethasone or betamethasone pulse
therapy- 0.5 mg/ every 5 kg body weight for
two consecutive days in a week.
3. High dose methylprednisolone pulse therapy8 mg/kg/day IV over 30 minutes for three
consecutive days every 4-8 weeks.
4. Multivitamin therapy (folic acid/vitamin B12/
vitamin C).
5. Antioxidants (-carotene, -tocopherol,
methionine, ubiquinone, vitamin C).
6. Immunomodulators- Levamisole 150 mg on
two consecutive days every week; cyclophosphamide 50 mg twice daily; cyclosporine
6 mg/kg/day; dapsone 100 mg/day; azathioprine.
7. Quinoline compounds- chloroquine 250 mg/
day and hydroxychloroquine 400 mg/day.
They can be combined with psoralen therapy.

Phototherapy
Psoralen (stimulates melanogenesis in
presence of ultra violet radiation)with
ultraviolet A therapy (PUVA)- topical and
systemic
Khellin + UVA (KUVA). Khellin is extracted
from seeds of the plant Ammi visnaga. It can
be given either topically or systemically.
Khellin is given orally 50-100 mg/day, 2.5
hours prior to sun or UVA exposure up to 15
J/cm2. But due to systemic toxicity, topical
preparation of khellin is recommended.

Modalities

Punch grafting
Split skin thickness graft
Blister grafting
Melanocyte culture and transplantation
Tattooing
Therapeutic spot and regional dermabrasion
Trypsinized autograft injection
Topical 5- fluorouracil combined with
epidermal abrasion.

For universal vitiligo options for depigmentation


are:
Depigmentation with 20% monobenzyl ether
of hydroquinone (for islands of residual
repigmentation in extensive vitiligo).
Imatinib mesilate 400 mg/day for 15 days,
then 300 mg once a day for 30 days has caused
vitiligo-like depigmentation. It is a selective
inhibitor of several tyrosine kinases.

ALBINISM
Autosomal recessive inherited disorder.
It is characterized by reduced melanin
synthesis in the melanocytes of the skin, hair,
and eyes, termed oculocutaneous albinism

Pigmentary Disorders
(OCA), and hypopigmentation primarily
involving the retinal pigment epithelium of
the eyes termed ocular albinism (OA).
Due to genetic abnormalities of melanin
synthesis associated with normal number
and structure of melanocytes (differentiate it
from vitiligo where melanocytes are reduced
or absent).
Tyrosinase-related OCA, the most common
type of albinism is produced by loss of
function of the melanocytic enzyme tyrosinase
resulting from mutations of the tyrosinase
gene.
Affected individuals are born with white or
blond hair and skin and blue eyes (Fig. 15.6).

PIEBALDISM
Piebaldism is an uncommon, autosomal
dominant, congenital, stable leukoderma
characterized by a white forelock and vitiligolike amelanotic macules, usually containing
a few normally pigmented or hyperpigmented macules (Fig. 15.7).

Fig. 15.7: Piebaldismtypical white forelock

Hyperpigmented macules within the


amelanotic macules and on normally
pigmented skin are characteristic of
piebaldism.

WAARDENBURGS SYNDROME
WS (Waardenburg syndromeHirschsprungs
disease or Shab-Waardenburg syndrome) is a rare
autosomal dominant disorder that is
characterized by:
Lateral displacement of the inner canthi and
of lacrimal puncta
Prominence of the nasal root and of the medial
eyebrows
Congenital deafness
Heterochromic irides
White forelock
Hypomelanotic macules.

MELASMA (CHLOASMA)

Fig. 15.6: AlbinismBlond hair and white skin with


actinic keratosis.

Melasma is a common acquired hypermelanosis that occurs exclusively in sunexposed areas; it is exacerbated by sun
exposure, pregnancy, oral contraceptives, and
certain anti-epilepsy drugs.
Melasma presents in one of three usually
symmetric facial patterns. The most common

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Fig. 15.8: Melasmahyperpigmented light brown


macules over the cheeks

is a centrofacial pattern involving the cheeks


(Fig. 15.8), forehead, upper lip, nose, and chin.
Less common are the malar pattern, involving
the cheeks and nose, and the mandibular
pattern.
Successful treatment of melasma involves the
triad of sunblocks, bleach and time.

FRECKLE (EPHELIDES)
It is an area of pale brown pigmentation usually
less than 3 mm with poorly defined lateral
margins
Freckle appears as result of functionally
overactive melanocytes (though they are
normal in number )
They are seen only in fair skinned people
They are stimulated by ultra violet radiation
and fade away during winter
Histology reveals excess of melanin pigment
in the basal layer.

LENTIGO (PLURAL: LENTIGINES)


It is a sharply demarcated brown pigmented
macule usually circular or polycyclic in shape.
Lentigo appears as a result of increased
number of melanocytes in the basal layer
They do not show seasonal colour variations
( not affected by ultra violet radiation).
Histology reveals linear increase of
melanocytes in the basal layer

Syndromes Associated with Lentigines


LEOPARD syndrome:
Lentigines (multiple),
ECG abnormalities,
Ocular abnormalities (hypertelorism),
Pulmonary stenosis,
Abnormalities of genitalia,
Retardation of growth and
Deafness (sensorineural).
NAME syndrome:
Nevi,
Atrial myxoma,
Myxoid neurofibromas and
Ephelides.
LAMB syndrome:
Lentigines,
Atrial myxoma,
Mucocutaneous myxomas and
Blue nevi.
PEUTZ JEGHERS SYNDROME
Peutz-Jeghers syndrome is an autosomal
dominant disorder characterized by pigmented macules (lentigines) of the buccal
mucosa, lips, fingers, and toes and by
gastrointestinal polyps.
It is caused by mutations in a novel serine
threonine kinase, and its gene has recently
been mapped to chromosome 19p.
The pigmented macules (dark brown or bluebrown) are most common on the buccal
mucosa and lips, but also seen over palate and
tongue. The macules on the skin are usually
found on the face (around the mouth and eyes),
dorsa of the hands and feet and periumbilically.
The diagnosis is particularly important
because of the presence of gastrointestinal
polyps, which are most frequent in the small
bowel, particularly the jejunum, which may
manifest with gastrointestinal bleeding.
Malignant change may occur in these polyps.

Pigmentary Disorders
Any child with recurrent, unexplained
abdominal pain should be examined for the
typical mucosal, and periorificial pigmented
lesions of Peutz-Jeghers syndrome.

DRUGS CAUSING HYPERPIGMENTATION


GeneralizedAddisonian-like occurs with
ACTH and generalized diffuse due to clofazimine, cyclophosphamide, minocycline, etc.

Localized:
Melasma- like- estrogen, progesterone,
phenytoin
Knuckle pigmentation- bleomycin
Palmoplantar- cyclophosphamide, doxorubicin
Linear bleomycin (flagellate), zidovudine.

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16

Keratinization Disorders

ICHTHYOSIS
Ichthyosis (from Greek ichthys fish) denotes a
group of hereditary and acquired disorders of
keratinization characterized by the development
of dry rectangular scales.

Hereditary (Inherited) Ichthyoses


Ichthyosis Vulgaris
It is a common autosomal dominant disorder
characterized by mild scaling on extensor aspect
of the limbs (Fig. 16.1), more than trunk and
spares the flexures. Increased palmar markings
or frank palmoplantar keratoderma may be
associated features. Onset is after 3 months of age.
Scaling gets worse in winter.

side of the face and trunk are affected. This form


of ichthyosis is associated with steroid
sulphatase deficiency.
Differential diagnosis: In contrast to ichthyosis
vulgaris, no hyperlinear palms, no keratosis
pilaris, flexural involvement and larger, darker
scales.

Differential diagnosis: Atopic xerosis, eczema


craquele, acquired ichthyosis, and Refsums
disease.

X-linked Recessive Ichthyosis


It appears in infancy and occurs in males. Females
may be heterozygotes and female carriers are
either totally spared or only mildly affected. Large
dirty brown scales characterize it. Extensor and
flexor aspects of the limbs are involved but spare
rhomboidal spaces in body folds (Figs. 16.2 and
16.3). Palms and soles are spared but the neck,

Fig. 16.1: Ichthyosis vulgarisscaling limited to


extensor aspects of limbs (shin)

Keratinization Disorders

Fig. 16.2

Fig. 16.3

Figs 16.2 and 16.3: X-linked recessive ichthyosislarge dirty brown scaling involving flexor and extensor
aspects of limbs and trunk but sparing rhomboidal spaces in body folds

Fig. 16.4: Lamellar ichthyosisface involvement by


erythema and scaling

Fig. 16.5: Lamellar ichthyosisbrownish plate like


scales over the trunk

Lamellar Ichthyosis

the body including flexures. Facial involvement


often results in ectropion and eclabium (Figs 16.4,
to 16.6).

Lamellar ichthyosis is a rare form of ichthyosis,


which presents at birth as Collodion baby; baby
is encased in a taut inelastic membrane. In this
autosomal recessive disorder, the baby later
develops large, thick plate like scales all over

Differential diagnosis: Includes X-linked


recessive ichthyosis and ichthyosiform
eythrodermas with collodion presentation.

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Fig. 16.6: Lamellar ichthyosislarge plate like


scales involving lower legs

Congenital Ichthyosiform Erythroderma


It is of two types:
1. Bullous congenital ichthyosiform erythroderma: It is inherited as an autosomal
dominant trait. At birth, the skin is
erythematous, moist and tender. Blisters
develop at or shortly after birth and later, thick
dark gray brown scales form warty-ridged
pattern (Figs 16.7 and 16.8). There is
accentuation in the areas of flexures and on
the palms and soles (Fig. 16.9). Normal
appearing skin within a hyperkeratotic area
is a valuable diagnostic sign. Skin colonization
by Staphylococcus brevibacterium and
possibly fungi produces a distinctive and
embarrassing body odor. Ectropion and
deformed ears are common.
Differential diagnosis: At birth, may be
confused with epidermolysis bullosa,
staphylococcal scalded skin syndrome,
herpetic infections and incontinentia
pigmenti. In older patients, ichthyosis hystrix,
ichthyosis bullosa of Siemens, and nonepidermolytic epidermal naevi may resemble
bullous
congenital
ichthyosiform
erythroderma.
2. Nonbullous congenital ichthyosiform
erythroderma: It is an autosomal recessive
disorder. Most infants with it are born as

Fig. 16.7

Fig. 16.8
Figs 16.7 and 16.8: Bullous congenital ichthyosiform
erythrodermano more blisters seen, body folds
show thick dark gray brown scales forming warty,
ridged pattern

Fig. 16.9: Bullous congenital ichthyosiform


erythrodermapalms involved by keratoderma

Keratinization Disorders
collodion baby. As the membrane is cast off in
10 to 14 days, generalized erythema and
scaling are apparent. Scales may be large and
plate like on the legs but are apt to be fine on
the trunk, face and scalp. It has a tendency to
improve at the time of puberty. Ectropion,
deformities of the ears and sparsity of the
scalp hair are common accompaniments.
Differential diagnosis: Congenital infections
such as candidiasis, congenital psoriasis,
Nethertons syndrome, immunodeficiency
disorders, trichothiodystrophy and neutral
lipid storage disease may mimic the cutaneous
signs of non-bullous ichthyosiform
erythroderma.
A collodion baby is the usual presentation of
congenital recessive ichthyosis. Kollodes is the
Greek word for glutinous or glue like. The child is
born encased in a transparent, parchment-like
membrane, which is taut and may impair
respiration and sucking. Collodion presentation
can develop into a wide spectrum of ichthyosis
phenotypes as the child grows. Differential
diagnosis: Harlequin fetus can be easily
differentiated from collodion baby. Restrictive
dermopathy or Stiff baby syndrome produces
a generalized taut thick, tethered and unyielding
skin at birth which does not desiccate in the
neonatal period. Infective causes of
desquamation such as staphylococcal scalded
skin syndrome should be included in differential
diagnosis.
Harlequin ichthyosis (fetus) is a dramatic,
severe, and usually fatal presentation of
ichthyosis. The child is often premature and born
with massive, shiny plates of stratum corneum
separated by deep, red fissures that tend to form
geometric patterns. There are poorly developed
or absent ears and marked ectropion and
eclabium. These children are at great risk during
the neonatal period and often die shortly after
birth.

Differential diagnosis: As given for collodion


baby. A variant of infantile systemic hyalinosis
and the stiff skin syndrome (congenital fascial
dystrophy) also present with tight skin.

Ichthyosiform Syndromes
1. Refsums syndrome (Heredopathia atactica
polyneuritiformis)
Refsums syndrome is a rare autosomal
recessive metabolic disorder in which
there are characteristic neurological and
cutaneous clinical features:
The underlying abnormality is deficiency
of enzyme phytanic acid oxidase.
As a consequence of this deficiency,
phytanic acid (found in green vegetables)
accumulates and displaces some of the
unsaturated fatty acids, such as linolenic
acid, from the lipids through out the
tissues.
It manifests usually in the second decade.
Skin is affected by an ichthyosis very
similar to ichthyosis vulgaris.
Neurological changes include a cerebellar degenerative disorder (cerebellar
ataxia), a progressive polyneuropathy,
retinitis pigmentosa, and a sensory
deafness. Rarely, cardiac abnormalities
have been described.
DiagnosisHistopathology of the skin
shows some of the epidermal cells
containing lipid vacuoles. No or very little
phytanic acid is present in the blood.
Treatment by a phytanic acid free diet, in
which green vegetables and dairy
products are excluded, has been used.
2. Sjgrens-Larsson syndrome:
This uncommon neuroectodermal
genodermatosis appears to be inherited as
an autosomal recessive disorder.
The underlying metabolic defect is
deficiency of enzyme fatty alcohol:
nicotinamide-adenine dinucleotide
oxidoreductase.

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The skin disorder becomes evident after
the first few months of life with scaling
over the body and hyperkeratosis of palms
and soles.
The neurological component usually
starts at 2-3 years of life and remains static
after puberty, consists of a spastic diplegia
(Fig. 16.10), or occasionally a tetraplegia
with mental retardation. Other features
are seizures and degeneration of retina.
DiagnosisHistopathological changes
are similar to lamellar ichthyosis.
TreatmentDiet lacking natural fat and
containing medium chain triglycerides has
been beneficial.
3. KID syndrome:
The acronym KIDKeratitis, Ichthyosis,
and Deafness describes the salient clinical
features of this syndrome.
4. Nethertons syndrome:
It is a rare autosomal recessive disorder
characterized by concurrence of ichthyosis
linearis circumflexa, trichorrhexis
invaginata (bamboo hair) and atopic
dermatitis.

5. CHILD syndrome:
The acronym CHILD describes a very rare
disorder comprising Congenital Hemidysplasia with Ichthyosiform erythroderma
and unilateral Limb Defects mainly
skeletal hypoplasia.
6. IBIDS syndrome (Tays syndrome):
The acronym IBIDS describes Ichthyosis,
Brittle hairs, Impaired intelligence,
Decreased fertility and short Stature.

Acquired Ichthyoses
The distinction between dry skin (xerosis) from
environmental causes and acquired ichthyoses
is sometimes difficult. The sudden onset of
generalized pronounced ichthyoses in an adult
could be due to:
1. Lymphomas especially Hodgkins lymphoma
2. Internal malignancy
3. Malabsorption syndromes and malnutrition
4. Certain drugs like clofazimine
5. Hypothyroidism
6. Lepromatous leprosy
7. HIV disease.
Treatment
Emollients for scaly skin especially after bath
Salicylic acid, urea, lactic acid in ointment
form or propylene glycol-glycerine lactic acid
mixture for topical application
Oral retinoids Etretinate or Acitretin. They
are really useful in lamellar and congenital
ichthyosiform erythrodermas.

DARIERS DISEASE

Fig. 16.10: Sjgren's-Larsson syndromechild


having spastic diplegia with ichthyosis

It is disorder of keratinization with an


autosomal dominant mode of inheritance
(chromosome 12).
Onset is during puberty when dirty, warty,
greasy papules appear in the seborrheic
distribution (Figs 16.11 to 16.13)
In due course of time, the lesions grow in size
and form malodorous, papillomatous and
vegetative growths

Keratinization Disorders

Fig. 16.11

Fig. 16.12

Figs 16.11 and 16.12: Darier's diseasedirty, warty greasy papules in seborrheic distribution over the trunk

Fig. 16.13: Darier's diseasedirty, warty greasy papules in


seborrheic distribution over the scalp and face

Other features include palmar pits (Fig. 16.14)


and V shaped nicking of nails.
Oral cavity shows cobble stone appearance
of the mucosa (Fig. 16.15).
Acral areas may demonstrate dome shaped
papular lesions known as acrokeratosis
verruciformis of Hopf (Figs 16.16 and 16.17)
Exacerbation occurs following sun exposure
Histopathology shows dyskeratosis (corps
ronds and grains) and acantholysis.

Differential diagnosis: Well developed cases


distinctive; early lesions confused with other
follicular keratoses. Same histologic picture can
be seen in Grovers disease as well as epidermal
naevi and acquired acanthomas, so clinico
pathologic correlation required.
Treatment: Acitretin 25-50 mg daily is probably
the best treatment; should be used until disease
brought under control and then stopped if sideeffects develop. Topical retinoids and keratolytics

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Fig. 16.14: Darier's diseasepalmar


keratoses with pits

Fig. 16.15: Darier's diseasecobblestone


appearance of hard palate mucosa

Fig. 16.16:

Fig. 16.17

Figs 16.16 and 16.17: Darier's diseaseacrokeratosis verruciformis of Hopf lesions over the dorsa of hands

are disappointing at best. Some patients improve


dramatically with antibiotics. Avoid sun exposure
and use sun screens.

POROKERATOSIS
It is a chronic progressive disease which
occurs due to abnormal proliferation of a clone
of keratinocytes.

Porokeratosis of Mibelli is the most common


prototype of porokeratosis
The lesions are slightly atrophic plaques
surrounded by elevated keratotic wall with a
furrow (seen sometimes) (Figs 16.18 and 16.19)
Various types of porokeratosis are
porokeratosis of Mibelli, disseminated
superficial actinic porokeratosis, palmo-

Keratinization Disorders
plantar porokeratosis, porokeratosis
palmoplantaris et disseminata and linear
porokeratosis (Fig. 16.20).
Histological hallmark is the column of
parakeratosis known as cornoid lamella.
Differential diagnosis: Multiple lesions can be
mistaken for psoriasis, lupus erythematosus,
pityriasis rubra pilaris or verrucous lichen planus.
Solitary lesions often misinterpreted as tinea,
warts, or actinic keratoses. Palmoplantar lesions
are hard to diagnose clinically; one can consider

Fig. 16.18: Porokeratosis of Mibellian atrophic


plaque surrounded by elevated keratotic wall with a
furrow

Fig. 16.19: Porokeratosis of Mibelliridge made


prominent by gentian violet paint

punctate palmoplantar keratoderma, arsenical


keratoses and warts; only biopsy provides the
answer.
Treatment: Cryotherapy, CO2 and pulsed dye
laser therapy or other destructive methods can be
used for limited lesions. Porokeratosis is usually
resistant to treatment but in some cases retinoids
(acetretin) or PUVA have been used with success.

PALMOPLANTAR KERATODERMA (PPK)


Palmoplantar keratoderma means thickening of
palms and soles. They can inherited as well as
acquired. Inherited PPK can be classified based
on the presence or absence of transgradiens
(spreading of keratoderma on to the extensor
surface).
Transgradiens is seen in Mal de Meleda,
Vohwinkels, Greithers and Papillon-Lefevre
syndrome.
Transgradiens is not seen in Thost-Unna
(most common diffuse palmoplantar
keratoderma), Vorner (diffuse PPK with
epidermolytic hyperkeratosis) and focal types.
The important features of various inherited
PPK are given in the following table.

Fig. 16.20: Linear porokeratosis


involving the arm

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Type of keratoderma

Important features

Thost Unna (AD)


Vorner type (AD)

Diffuse PPK with livid red border (Fig. 16.21) Marked hyperhidrosis
Diffuse PPK similar to Thost Unna type.Histology shows epidermolytic
hyperkeratosis
Honey combed palmoplantar thickening with constriction bands and mutilation, star
fish shaped keratosis on dorsa of hands and fingers (Fig. 16.22)
Palmoplantar keratoderma in a glove like distribution. Erythema is prominent with
hyperhidrosis and malodor
Diffuse PPK that is progressive. Extensor surfaces of hands, knees and elbows
shows psoriasiform plaque (Fig. 16.23)
Congenital PPK with perioral hyperkeratosis. Spontaneous amputation can occur
PPK associated with periodontosis and increased frequency of pyogenic infection

Vohwinkels type (AD)


Mal de Meleda (AR)
Greither (AD)
Olmsted syndrome (S)
Papillon-Lefevre (AR)

Note: ADAutosomal dominant, ARAutosomal recessive, Ssporadic occurrence

Fig. 16.21: Palmoplantar keratodermadiffuse


keratoderma localized to palms

Fig. 16.23: Palmoplantar keratodermaextending


onto dorsa of hands and feet (transgradien
palmoplantar keratoderma)

Other types of palmoplantar keratodermas are


focal kertodermas, striate keratodermas and
puntate keratodermas (Fig. 16.24).
Various causes of acquired keratoderma are
pityriasis rubra pilaris, malignancy (Tylosis),
myxedema, Dariers disease, keratoderma
climactericum, psoriasis, lichen planus, and
tinea pedis.
Fig. 16.22: Palmoplantar keratodermaleading on
to mutilation

Treatment: Topical keratolytics, vitamin D3


analogues and mechanical debridement useful.
Some cases respond to oral isotretinoin.

Keratinization Disorders

Fig. 16.24: Punctate keratodermapunctuate


keratotic lesions involving palmar creases

ACANTHOSIS NIGRICANS
Acanthosis nigricans is a nonspecific reaction
pattern involving major body folds and
mucocutaneous regions characterized by
hyperpigmented, velvety, soft, verrucous lesions
in a symmetric fashion (Fig. 16.25).
It is broadly divided into benign and
malignant form.
Benign acanthosis nigricans involves limited
body areas and is less severe than malignant
forms.
The various benign forms that are the most
common include benign familial acanthosis
nigricans, acanthosis nigricans associated
with various syndromes, endocrine disease
(especially insulin resistance diabetes
mellitus, HAIR-AN syndrome [Hyper
Androgenism, Insulin Resistance, and
Acanthosis nigricans]), obesity (pseudoacanthosis Nigricans) and drugs (Nicotinic
acidic, fusidic acid, stilbestrol, oral
contraceptives, triazinate).

Fig. 16.25: Acanthosis nigricanshyperpigmented,


velvety, soft, verrucous involvement of neck

Malignant acanthosis nigricans is associated


with extensive, widespread lesions and
mucosal involvement. They may precede,
follow or occur simultaneously with onset of
malignancy. Thickening of palms, especially
fingertips produces accentuated dermatoglyphics with deep sulci called as Tripe
palms.
Gastric carcinoma is the most commonly
associated tumor; other sites include
the bronchus, pancreas, ovary, bile duct,
gallbladder, endometrium, breast and thyroid.
Differential diagnosis: Confluent and reticulated
papillomatosis, tinea versicolor, X-linked
ichthyosis, retention hyperkeratosis, dirty neck
of atopic dermatitis.
Treatment: As it is a reaction pattern to some of
the underlying conditions, treatment need to be
directed at them. Topical retinoids may bring
modest improvement.

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17

Urticaria,
Angioedema and Pruritus

URTICARIA AND ANGIOEDEMA


Urticaria
Is a dermal vascular reaction of the skin
characterized by the appearance of itchy wheals,
which are elevated (edematous), pale or
erythematous, transient and evanescent plaque
lesions. These lesions usually do not last beyond
24 hours. They change in size and shape by

Fig. 17.1

peripheral extension or regression during few


hours (Figs 17.1 and 17.2). Wheals itch. The
intensity of itch varies.

Angioedema (Quinckes Edema)


Is also a vascular reaction, which involves
subcutaneous or submucosal tissues (rather than
dermis in urticaria) so skin overlying the swelling

Fig. 17.2

Figs 17.1 and 17.2: Urticariashowing itchy erythematous wheals of various shapes and sizes

Urticaria, Angioedema and Pruritus

Fig. 17.3: Angioedemaswelling of chin and lip of


sudden onset

is normal in color and margins of swelling are


indistinct (diffuse swelling). It commonly results
in asymptomatic swelling of the lips (Fig. 17.3),
eyelids, etc. Due to laryngeal involvement, patient
may develop respiratory distress. Gastrointestinal involvement may cause vomiting and
abdominal pain.
Angioedema may be classified into hereditary
angioedema [Type I (85%)-C1 esterase inhibitor
absent, Type II (15%)-C1 esterase inhibitor not
functional] and acquired angioedema (Acute
angioedema due to allergic IgE mediated, e.g. due
to drugs, food, insects, contact dyes, serum
sickness, cold urticaria or Chronic recurrent
angioedema-(Idiopathic in most cases; rarely due
to acquired C1 esterase inhibitor deficiency).
Urticaria may be acute or chronic

Acute Urticaria
Means urticaria of less than 6 to 8 weeks duration.
Common causes are drugs especially penicillin,
foods, especially shell fish, and rarely infections.
In India, insect bites are another important cause.
No apparent cause can be found in at least half of
acute attacks. Even if a cause can be identified
and withdrawn, it is often several days before the
urticaria subsides.

Chronic Urticaria
If urticaria lasts for more than 6 to 8 weeks
duration, it is called chronic urticaria. Culprit in
the great majority of cases remains obscure. Up to
55% of patients with chronic idiopathic urticaria
possess functional IgG antibodies directed
against the high affinity IgE receptors or less
commonly against IgE itself. These autoantibodies release histamine from human skin
mast cells and blood basophils and appear to be
the cause of the disease (autoimmune urticaria).
Immunoglobulin and complement components
may be deposited perivascularly, and can be
visualized by direct immunofluorescence
examination.
Basic pathogenic mechanisms of urticaria are
immune and nonimmune mechanisms. Immune
mechanism consists of type I hypersensitivity
mechanism (IgE mediated), through complement
activation, immune complex mechanism, or
autoimmune process. Nonimmune mechanism
works through direct release of histamine and
various mediators from mast cells, vasoactive
stimuli, aspirin, dietary pseudoallergens and due
to angiotensin converting enzyme inhibitors.
Etiological Factors(6 Is + others )
1. Ingestants
a. Foods cheese, eggs, nuts, fish, mushrooms, etc.
b. Food additives tartarzine dyes, etc.
c. Food preservatives
d. Drugs penicillin, salicylates, sulphonamides, etc.
2. Injectants
a. Insect bites
b. Injection drug, sera, blood, etc.
3. Inhalants
Pollens, animal dander, etc.
4. Infestation by parasites
Amoebiasis
Giardiasis

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Essentials in Dermatology

168

Fig. 17.4: Dermographismstroking the skin with


blunt metallic instrument resulted in an exaggerated
triple response forming wheals along them

5.
6.
7.

8.

Hookworm
Round worm.
Infection focus in teeth, tonsils, sinuses or
elsewhere.
Implantsprosthetic and hormonal
Physical causes/factors (Urticarial attacks are
brief lasting for 30 to 60 minutes):
a) Dermographism (write-on-skin) stroking
the skin with blunt metallic instrument
results in an exaggerated triple response
(Fig. 17.4).
b) Pressure urticariapressure
c) Cholinergic urticariaincrease in core
body temperature (manifest as small
intensely pruritic papules)
d) Cold urticariacold air or water (Fig. 17.5)
e) Heat urticariaheated object
f) Solar urticariasun exposure
g) Exercise inducedexercise
h) Aquagenic pruritus/urticariacontact
with water
i) Vibratory urticariahandling vibratory
instruments.
Contact urticaria Contact urticaria refers to
a wheal and flare reaction following external
contact with a substance, e.g. on coming in

Fig. 17.5: Cold urticariaice cube test producing


urticaria

contact with potato, onion, nitrogen mustard,


etc. develops urticaria at site of contact. It
usually appears within 30 minutes and clears
completely within hours without residual
signs of irritation.
9. Rule out underlying collagen vascular
disorders, malignancy or any psychogenic
cause.
10. Chronic idiopathic urticaria (CIU), defined as
the occurrence of daily, or almost daily, wheals
and itching for at least 6 weeks, with no
obvious cause. Idiopathic no cause found.

Fig. 17.6: Proceeding in a case of urticaria

Urticaria, Angioedema and Pruritus

Diagnosis
How to proceed in a case of urticaria?
1. Urticaria due to physical causes or drugs
excluded by history and examination (Fig.
17.6).
2. Complete food elimination followed by
gradual introduction of one dietary element
at a time helps in detection of food induced
urticaria.
3. Mask use/nasal filter use/change of place
may work for inhalants.
4. Stool examination by concentration method
on 3 consecutive days infestations are
detected and treated accordingly.
5. Look for a focus of infection. If not possible to
detect, give a course of antibiotics. Still no
response, change the antibiotic.
6. The major advance in our understanding of
chronic idiopathic urticaria (CIU) in recent
years has been the discovery that in 30-50% of
patients with so labeled chronic idiopathic
urticaria, the disease is due to an autoimmune
process, and therefore is not strictly
idiopathic.
7. The autologous serum skin test is a useful
screening test for autoimmune chronic
urticaria (AICU). In this test, 0.05 ml of the
patients serum, removed during a period of
disease activity, is injected intradermally into
the same patients uninvolved forearm skin,
along with equal volumes of saline and
histamine (10 g/ml) at adjacent sites. The
test is read 30 min later. A positive result is
recorded if the diameter of the wheal at the
serum-injected site is 1.5 mm greater than that
of the bleb at the saline-injected site. The
sensitivity and specificity of the test are
65-81% and 71-78%, respectively. Patients
with AICU are more treatment-resistant, and
their disease runs a more aggressive course,
than those with non-autoimmune CIU.
8. If still getting urticaria, then look for other
causes and treat symptomatically.

Differential Diagnosis of Urticaria


Includes all dermatologic conditions with an
urticarial component like cutaneous mastocytosis,
urticarial vasculitis, insect bite reactions (papular
urticaria), acute febrile neutrophilic dermatosis,
pre bullous pemphigoid, acute facial contact
dermatitis, erythema multiforme, collagen
vascular disease, porphyria, pityriasis rosea,
psoriasis and last but not least scabies.
Differential Diagnosis of Angioedema
Infections (cellulitis), trauma, superior vena cava
syndrome, subcutaneous emphysema,
Melkersson Rosenthal syndrome.
Treatment
Acute urticaria
Minimize heat and stress,
Avoid alcohol, NSAIDs and opiates.
Soothing lotions such as calamine for topical
application given during attack of urticaria.
Antihistaminics:
H1 antagonists or
H2 antagonists or
Both together
Old sedative antihistaminics are still better
during acute episodes.
Corticosteroids may be required to tide over a
crisis-tapering regime commencing with
30 mg prednisolone daily, with or without
concurrent H1 antagonist administration.
Adrenaline used in anaphylaxis.
Other measures like intravenous fluids,
oxygen use may be required.

Chronic Urticaria
Antihistamines still remain the mainstay of
drug treatment.
Doxepin 25 to 50 mg at night time may be
added.
Corticosteroids-occasionally as short tapering
courses given in autoimmune chronic

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Essentials in Dermatology
urticarias as they fail to respond to
antihistamines.
Cyclosporine-2.5 to 5 mg /kg body weight/
day is another option. Control of urticaria
usually occurs within 1 week of commencing
treatment.
Intravenous immunoglobulin.
Other drug treatments are ketotifen (1-2 mg
daily or at night), disodium cromoglycate, beta
agonist (terbutaline 2.5 mg three times daily),
calcium channel blockers (nifedipine),
leukotriene antagonists (montelukast,
zileuton), and anti IgE monoclonal antibody.

PRURITUS (ITCHING)
Pruritus may be defined simply as that
unpleasant sensation which produces the desire
to scratch. It may be localized or generalized. If
generalized, needs investigations for any systemic
cause. Certain areas of the skin, particularly
susceptible to pruritus are ear canals, perianal
and genital areas, eyelids, etc. Localized pruritus
may give clues to the etiology and is less likely to
be associated with widespread or systemic
disease. In the scalp, pruritus may be due to
psoriasis or an ongoing folliculitis, in the inguinal
or anal region due to pruritus ani, pruritus vulvae,
pruritus scroti, venereal diseases, ectoparasites
(scabies, pediculosis), in the hands and feet due
to psoriasis, pompholyx, contact dermatitis, in the
mid back due to notalgia paresthetica, macular
amyloidosis, and in the lower limbs in older
patients due to asteatosis.
Causes of pruritus can be classified as follows:
1. Dermatological disorders
2. Systemic diseases
3. Psychogenic pruritus
4. Iatrogenic pruritus.

Dermatological Disorders
Common dermatoses known to produce pruritus
are scabies, pediculosis, insect bites, eczema,

urticaria, prickly heat, lichen planus, dermatitis


herpetiformis, bullous pemphigoid, psoriasis,
seborrhoeic dermatitis, dermatophytosis (tinea),
urticaria pigmentosa, varicella and rubella,
papular and pruritic eruption seen in AIDS
patients, etc.

Systemic Disorders
Three important systemic disorders most likely
to play role in acquired acute pruritus are renal
disease, hepatic disease and abnormalities of the
hematopoietic system.
Renal chronic renal insufficiency
Hepatic intrahepatic and extrahepatic
biliary obstruction (cholestasis).
Abnormalities of the hematopoietic system
iron deficiency anemia, polycythemia vera
(is associated with bath pruritus), leukemia,
Hodgkins lymphoma, mycosis fungoides,
mast cell disorders.
Endocrine diabetes mellitus, myxoedema,
hyperthyroidism, hypoparathyroidism.
Intestinal parasites infestation with
hookworm, round worm, pin worm.
Collagen vascular disorders SLE, sicca
syndrome.
Neurological multiple sclerosis, brain tumor.
Pregnancy.

Psychogenic Pruritus
Psychosomatic disorders resulting in itch
commonly occur in the middle aged or older
individuals.
Iatrogenic Pruritus
Commonly induced by opium alkaloids, CNS
stimulants, antidepressants, and belladona
alkaloids.

Diagnosis
History and clinical examination is of paramount
importance. In addition, investigations outlined

Urticaria, Angioedema and Pruritus


below may be undertaken to find the underlying
cause.
1. Hemogram including ESR.
2. Liver function tests.
3. Kidney function tests.
4. Urine examination for albumin, sugar and
cells.
5. Blood sugar.
6. Stool examination for occult blood, worms
and ova.
7. Chest X-ray.
8. Thyroid function tests.
9. Hepatitis C screening.
10. HIV testing.
11. Uric acid estimation and also acid phosphatases.
12. Skin biopsy for mast cells and immunofluorescence.
13. Patch testing, photopatch testing, immunoglobulin levels.
14. Screening for underlying malignancy.
15. Serum protein electrophoresis.

Treatment
1. Eliminate causative disease if possible or treat
it appropriately.
2. Avoid provocative influences:
Friction from rough clothing.
Overheating and vasodilatation, e.g. alcohol,
hot drinks.
Keep nails short.
Soothing lotions, e.g. calamine in urticaria,
moisturisers for dry skin.
Occlusive bandaging may be done to retain
moisture in the skin.

Emollients of bland nature, e.g. coconut oil,


liquid paraffin.
1% menthol in 90% ethanol is of significant
value.
Steroid creams.
Topical antipruritic drugs, e.g. doxepin,
crotamiton.
Avoidance of strong soaps in elderly
individuals.
3. Systemic treatment:
Antihistamines:
H1 antagonists-especially sedative ones
may be preferred
Systemic steroids:
Sometimes required to control urticaria
Doxepin
4. Specific treatments:
For uremic pruritus- Naltrexone (50 mg
oral daily), activated charcoal, UVB
phototherapy, ondansetron (4 mg twice daily),
cholestyramine
For cholestatic pruritus- cholestyramine,
phenobarbitol, UVB phototherapy, rifampicin
(300-450 mg daily), naltrexone (50 mg oral
daily), and danazol.
For hematologic disease-cimetidine,
cyproheptadine, pizotifen (0.5 mg three
times daily), danazol, interferon alpha 2b,
UVB phototherapy, iron replacement
therapy.
Lidocaine and intralesional steroids (for
localized pruritus and trigger points) may be
tried as last resort.

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18

Drug Eruptions, Erythema Multiforme,


Stevens-Johnson Syndrome, and
Toxic Epidermal Necrolysis

DRUG ERUPTIONS
A drug may be defined as a chemical
substance, or combination of substances,
administered for the investigation,
prevention or treatment of diseases or
symptoms, real or imagined.
Drug eruptions are some of the most
common skin disorders.
They are seen in 2 to 3% of hospitalized
patients.
These eruptions may closely mimic other skin
disorders.

Characteristics of Drug Eruptions


1. There is a history of drug intake preceding
the eruption. The history of drug intake must
include all systemic drugs, nonprescription
drugs, home remedies and topical
medications. A previous history of allergic
reaction may increase the risk of
development of an allergic reaction.
2. Drug eruption is sudden in onset.
3. Generalized eruption is often pruritic.
4. Eruption is bilateral and symmetrical,
exception to this is fixed drug eruption.
5. Regression of eruption occurs on withdrawal
of drug.

6. Similar type of rash recurs on re-exposure to


the same or similar drug.
Undesirable cutaneous or mucocutaneous
reactions to systemically absorbed drugs occur
through two mechanisms.
1. Immune mechanisms: All the four
hypersensitivity mechanisms may be
involved.
Type I IgE dependent reactions cause
urticaria, pruritus, bronchospasm and
laryngeal edema within minutes, hours
or days.
Type II Cytotoxic reactions may cause
thrombocytopenia.
Type III Immune complex dependent
reactions result in serum sickness,
urticarial or leukocytoclastic vasculitis
within a week or so.
Type IV Cell mediated immune response
may lead to eczematous and other types
of eruptions in 3 to 4 weeks time.
2. Non-immune mechanisms: They include
drug induced hemolysis (G6PD deficiency),
mast cell degranulation (codeine,
radiocontrast media), exacerbation of disease
(psoriasis by lithium or beta blocker), drug
deposition in skin, alopecia, etc.

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome

General Rules
Drug allergy is most frequent in older
individuals and may be related to
development of immune response and
increased exposure to drugs.
Topical application of drugs has the greatest
propensity to induce allergy, followed by the
intravenous route and the oral route.
The drugs most often responsible for the
eruptions are antimicrobials and antipyretic/
anti-inflammatory analgesics.
The appearance of the eruption may provide
some clues to its cause (e.g. fixed drug
eruptions associated with sulphonamides).
Always keep in mind the fact that drug
eruptions are great imitators of other skin
diseases.
The most common morphologic patterns of
drug eruptions are exanthematous (40%),
along with urticaria and/or angioedema
(37%), fixed drug eruption (6%), erythema
multiforme and others.

4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.

Acneiform eruptions (Fig. 18.1).


Drug hypersensitivity syndrome.
Lupus like eruptions.
Lichenoid eruptions.
Pityriasis rosea like eruptions.
Phototoxic (Fig. 18.2)and photoallergic
eruptions.
Vesicobullous eruptions.
Eczematous eruptions.
Alopecia.
Skin pigmentation.
Vasculitic eruptions.
Fixed drug eruptions.
Erythroderma.
Erythema multiforme.
Stevens-Johnson syndrome.
Toxic epidermal necrolysis.

Types of Drug Eruptions


1. Exanthematous eruptions (Maculopapular
eruptions) (occur within 1 week).
2. Urticarial eruptions.
3. Serum sickness.

Exanthematous eruptions: These are the most


frequent of all cutaneous reactions to drugs, and
usually appear within 1 week of the causative
drug being started. A rash may also start within
4-7 days of the offending drug being stopped.
Lesions most often start first and clear first from
head and upper extremities. They may be
accompanied by fever, pruritus and eosinophilia.
Antibiotics especially semisynthetic penicillins
and sulfonamides are the most common causes
of this reaction pattern.

Fig. 18.1: Acneiform drug eruption


monomorphous papular eruption over the back

Fig. 18.2: Phototoxic drug eruptionexaggerated


sunburn reaction limited to exposed areas

173

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Essentials in Dermatology
Urticarial eruptions: It is the second most
common type of cutaneous drug eruption.
Angiotension converting enzyme inhibitors are
the frequent causes of angioedema often without
urticaria.
Serum sickness-like reaction: Serum sicknesslike eruption consists of fever, a rash with usually
urticarial features and arthralgias occurring
within 1 to 3 weeks of initiation of the drug.
Lymphadenopathy and eosinophilia may also be
found.
Drug hypersensitivity syndrome (DHS): Also
known as drug rash with eosinophilia and
systemic symptoms (DRESS) syndrome or as
drug
induced
delayed
multiorgan
hypersensitivity syndrome (DIDMOHS). It
consists of an exanthema, hepatitis and fever.
Eighty percent of the cases have an exanthem
type eruption whereas others will develop more
serious Stevens Johnson syndrome. DHS may be
life threatening and requires prompt
discontinuation of the drug and systemic
corticosteroids.

with burning or stinging sensation (Fig. 18.3).


These lesions sometime develop into bullous
lesions (Fig. 18.4) and when they heal leave
behind slate gray colored pigmentation (Fig.
18.5). Mucocutaneous junctions are commonly
affected.
Erythroderma: It is a generalised erythema,
infiltration and scaling of the skin (involving
more than 90% of the surface area of the body).
It may be a manifestation of a drug eruption but
usually occur due to dermatological disorders
like psoriasis, contact dermatitis, pityriasis rubra
pilaris, etc.

Fixed drug eruption: It differs from other


eruptions in that it occurs and then recurs at fixed
sites. Single or multiple circular or oval
erythematous macule/s or plaque/s develop

Fig. 18.4: Fixed drug eruptioncircular, slate gray


colored pigmented macular lesion developing bullae
over it

Fig. 18.3: Fixed drug eruptiontypical slate grey


oval macule with halo of erythema over the trunk

Fig. 18.5: Fixed drug eruptionmultiple fixed drug


eruptions over the chest

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome


Erythema multiforme: Erythema multiforme is
characterized by many types of lesions including
urticaria, target lesions (iris lesions or bulls-eye
lesions) (Figs 18.6 and 18.7) and vesicles and
bullae, bilaterally symmetrically over acral areas
of the body. They may also involve mucous
membranes.
Stevens-Johnson syndrome: It is a severe form
of erythema multiforme associated with
constitutional symptoms (fever) and visceral
organ involvement, e.g. kidneys. The skin as well
as mucous membranes are both involved (Figs
18.8 and 18.9).

Toxic epidermal necrolysis (TEN): The term


toxic referred to a presumed toxin responsible
for the prodrome and the eruption, epidermal
to the presence of significant epidermal damage
and necrolysis to the pathologic findings of
necrosis and clinical findings of epidermolysis.
It is a potentially life threatening, severe
mucocutaneous reaction pattern characterized
by fever, systemic toxicity, erythema and
tenderness of skin followed by flaccid bullae
formation resembling a burns case (Fig. 18.10).
Nikolskys sign is positive. Mortality from TEN
is 11 to 33%.

Fig. 18.6: Erythema multiformechest showing


typical target lesions

Fig. 18.7: Erythema multiformetarget lesions


showing three zones of color

Fig. 18.8: Stevens-Johnson syndromefacial


eruption with hemorrhagic crusting of lips

Fig. 18.9: Stevens-Johnson syndromeatypical


target lesions over the back

175

176

Essentials in Dermatology
serum creatinine, urinanalysis and TSH may be
indicated in patients with suspected drug
induced hypersensitivity syndrome.
Third step is the invitro testing which include
Radio Allergosorbent Assays (RAST) and
Enzyme Linked Immunosorbent Assays
(ELISA). Other tests include Lymphocyte
Transformation Test (LTT), Macrophage
migration Inhibition Factor (MIF), Lymphocyte
Toxicity Assay (LTA), Basophil Degranulation
Test and Histamine Release tests.
Fig. 18.10: Toxic epidermal necrolysissheets of
necrotic tender epidermis over the back

Diagnosis
Diagnosis is basically based on suspicion and
history of drug intake. A thorough and stepwise
approach is essential to proper diagnosis of a
drug- induced skin reaction. First step in patient
evaluation should include (1) a comprehensive
drug history, (2) awareness of various clinical
manifestations of drug allergy and cutaneous
reaction, (3) awareness of factors that favor
development of allergic reactions to drugs, and
(4) awareness of the immunologic and
nonimmunologic mechanisms involved in
cutaneous reaction to drugs. A general rule of
thumb is that drugs started within one week of
the onset of the eruption are the most likely
suspects.
Second step is skin biopsy and in vivo testing.
This includes patch testing, scratch/ prick
testing and dechallenge/ rechallenge tests.
Dechallenge/ rechallenge continues to be
regarded as the most definitive method for
ascertaining drug-induced reactions. However,
it is often not an option if the patient has
experienced a life threatening condition or if
suspected agent cannot be continued. Blood
work-up may also aid in the clinical diagnosis.
A CBC with differential count may show atypical
lymphocytosis, leukopenia, leukocytosis,
eosinophilia and so on. Liver function tests,

Differential Diagnosis
Consider differential diagnoses such as
infections, collagen vascular disease, primary
skin conditions and neoplasia. Culture of skin,
blood, tissue, erythrocyte sedimentation rate,
ANA, and other tests may be ordered to help
confirm or rule out other conditions. If palpable
purpuric lesions are present, a complete physical
and laboratory examination is required with an
eye towards ruling out vasculitic involvement
of other organ systems and other causes of
vasculitis such as infections or collagen vascular
disease.
Treatment
Withdrawal of all drugs
Antihistamine (H1 antagonists)
Soothing lotion for topical application
Corticosteroids topical steroids may
provide some relief. Systemic corticosteroids
are indicated if signs and symptoms are
severe
Adrenaline in case of anaphylaxis
Other measures like fluid and electrolyte
balance maintenance, wet compresses, etc.
ERYTHEMA MULTIFORME
It is a self limited, usually mild but relapsing
exanthematic reaction of skin probably triggered
by circulating immune complexes, most often
related to recurrent herpes simplex infection,

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome


characterized by skin lesions with varied
morphology (typically target lesions) in acral
areas, frequently associated with mucous
membrane lesions.
Herpes virus infection is the single most
important cause of erythema multiforme
(EMF).
Other important agents include Mycoplasma
pneumoniae, drugs and malignancies.
EMF is common in young adults.
Prodromal symptoms are minimal to absent.
The lesions seen are urticarial papules, target
lesions, vesicles and bullae.
The lesions occur in a symmetric fashion on
the extensor aspects of limbs, palms and
soles.
The target lesions /iris lesions have three
zones of color: The central dark cyanotic area,
pale edematous zone and the surrounding
erythema (Figs 18.6 and 18.7).
The iris lesions result from centrifugal
spread of a red maculopapule as the center
becomes cyanotic, purpuric or vesicular.

Differential Diagnosis
Urticaria, urticarial vasculitis, figurate erythemas
(slow evolving lesions, usually asymptomatic, no
mucosal involvement), acute febrile neutrophilic
dermatosis, disseminated lesions of contact
dermatitis, bullous pemphigoid, linear IgA
dermatosis, herpes gestationis, lupus
erythematosus, exanthematous or morbilliform
drug eruption (accompanied by fever, pruritus,
eosinophilia, involving trunk and extremities,
sparing the face and pressure areas, fade with
desquamation and postinflammatory hyperpigmentation), viral exanthem (start on face and
involve the trunk, maculopapular/ urticarial/
vesicular/petechial lesions associated with fever,
conjunctivitis, and lymphadenopathy, fades
without pigmentation and scaling), fixed drug
eruption (solitary first, later new lesions appear
with repeated attacks, common on limbs, hands
and feet, genitalia and perianal area, also

periorbital and perioral), Kawasakis disease


(lips are red, dry with crust, transient red
macules, conjunctivitis without exudates,
strawberry tongue, lymphadenopathy),
paraneoplastic pemphigus (severe necrosis of
lips, eyes, oral mucosa, with polymorphous skin
lesions, biopsy shows acantholysis and
immunofluorescence confirms the diagnosis),
Stevens Johnson syndrome (severe episodic
acute mucocutaneous reaction, most often
elicited by drugs and occasionally by infections,
characterized by rapidly expanding irregular
macules or atypical target lesions involving body
surface area less than 10%, involvement of more
than 2 mucosal sites), toxic epidermal necrolysis
(mucosal erosions and epidermal detachment
more than 30% of body surface area, skin
tenderness, Nikolskys sign positive,
histopathology shows full thickness epidermal
necrosis with subepidermal cleft formation),
acute graft versus host disease (also precipitating
factor for Stevens Johnson syndrome, similar
lesions, histopathology also cant differentiate,
lesions begin over the palms and soles first) and
acute hemorrhagic edema of infancy (is a
manifestation of leukocytoclastic vasculitis in
infants and small children, it is characterized by
symmetric erythematous and purpuric
concentric rings in acral locations similar to
target lesions that spontaneously resolve in about
a week).
Treatment is symptomatic. If the herpetic
lesions are clinically evident, acyclovir needs
to be given.

STEVENS-JOHNSON SYNDROME
TOXIC EPIDERMAL NECROLYSIS
SPECTRUM
Stevens-Johnson syndrome (SJS) and Toxic
epidermal necrolysis (TEN) are closely related
severe acute mucocutaneous intolerance
reactions most often elicited by drugs and less
so by infections (Table 18.1).

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Table 18.1: Differentiating features between erythema multiforme and SJS-TEN

Features
1.
2.
3.
4.

Etiology
Course
Prodrome
Eruption

5. Typical lesions
6. Mucosal involvement
7. Body surface area
8. Constitutional symptoms
9. Pathology

10. Internal organs


11. Duration
12. Complications

13. Mortality rate


14. Healing

Erythema multiforme

SJS-TEN

HSV (in majority)


Acute, self limited, recurrent
Absent to moderate
Disseminated, symmetric, acral, face

Drugs (80-95%)
Acute, self limited, episodic
Intense, skin tenderness
Disseminated, confluent, symmetric,
on face, neck, trunk
Fixed plaques, target lesions, blisters,
Macules, flat atypical target, central
Nikolskys sign ve
necrosis, Nikolskys sign +ve
Frequent, mild oral
Prominent, severe, 2-3 mucosal
involvement
<10%
<10% to >30%
Absent to moderate
Prominent to severe
Satellite cell necrosis, DEJ blister formation, Massive keratinocyte necrosis,
prominent mononuclear cell infiltrate, papillary sloughing of epidermis, dermal
dermis edematous
infiltrate slight to absent
Not involved
Not infrequent
1-3 weeks
> 2-6 weeks
None
Septicemia, pneumonia,
gastrointestinal hemorrhage, renal
and cardiac failure
0%
1-50%
Without scarring
Sequelae due to mucosal scars

They have been classified based on the area


involved as:
SJS mucosal erosions and epidermal
detachment below 10%.
SJS/TEN overlap mucosal erosions and
epidermal detachment between 10-30%.
TEN mucosal erosions and epidermal
detachment more than 30% .

STEVENS-JOHNSON SYNDROME (SJS)


The important drugs causing SJS are
phenytoin, phenobarbitone, sulfonamides,
penicillins and NSAIDs.
It is common in children and young adults.
The disease is preceded by a nonspecific
prodrome with fever, myalgia, rhinitis and
cough.
Skin lesions occur abruptly and are purpuric
macules, atypical target lesions and papules.

Bullous lesions may occur in oral, genital and


anal mucosa.
Ulcerative stomatitis with hemorrhagic
crusting is the most characteristic feature
(Fig. 18.8).
Corneal erosions may lead to symblepharon,
synechiae and opacities.
Constitutional symptoms may be severe
during active stages.
Differential diagnosis: As given for erythema
multiforme.
The treatment includes immediate withdrawal of all potential causative agents, skin
care, fluid and electrolyte balance, eye care,
supportive care with antibiotics, diet and
others. Patients have to be managed in an
intensive care set up. Specific medications
include steroids, intravenous immunoglobulins, plasmapheresis, hemodialysis,
cyclophosphamide, and cyclosporine.

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome

TOXIC EPIDERMAL NECROLYSIS


(LYELLS SYNDROME)
TEN is a rare life threatening mucocutaneous
reaction characterized by widespread sheets of
erythema, necrosis and bullous detachment of
epidermis.
Incidence: 1 to 3 cases per million.
Pathogenesis: In predisposed patients, the
drug metabolites may bind to epidermis and
trigger an immune response leading to
immunoallergic cutaneous adverse reaction.
Drugs causing TEN are antiepileptics
(phenytoin, phenobarbital, carbamazepine),
sulfonamides, ampicillin, allopurinol,
antituberculous drugs (thiacetazone,
isoniazid) and NSAIDs.

Clinical Features
TEN begins with sheets of erythema covering
wide areas.
In hours, the skin lesions become painful and
extremely tender and small vesicles and
bullae appear over the involved skin (Fig.
18.10).
The epidermis can be separated from dermis
by slight tangential pressure (Nikolskys
sign).

Mucosal erosions and conjunctival erosions


are a constant feature.
Septicemia and bronchopneumonia are the
important causes of death.

Differential Diagnosis
As given for erythema multiforme.
Staphylococcal scalded skin syndrome (SSSS)
can be differentiated from TEN by occurrence
in children, absence of mucosal lesions and
absence of systemic features. Moreover in
SSSS, the involved skin is dry and
parchment- like while in TEN it is
erythematous, purpuric and necrotic.
Physical and chemical injury such as scalding
and burns, solar erythema and chemical
burns are other differential diagnosis,
difficult in an unconscious patient.
Treatment
Patients have to be managed in an intensive
care set up with proper maintenance of fluid
and electrolytes like Stevens Johnson
syndrome.
Role of steroids is controversial.
Intravenous immunoglobulins have been
found to be useful.
Plasmapheresis, hemodialysis, cyclophosphamide, cyclosporine, N-acetylcysteine,
thalidomide, etc are other treatment options.

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19

Disorders of Sebaceous,
Eccrine and Apocrine Glands

DISORDERS OF THE SEBACEOUS


GLANDS
Acne Vulgaris
Acne vulgaris is a self-limited disease, seen
frequently in adolescents, primarily involves the
sebaceous follicles. Most cases of acne are
pleomorphic, presenting with a variety of lesionscomedones, papules, pustules, nodules, and as
sequelae to active lesions-pitted or hypertrophic
scars.
Pathogenesis
Four major factors are involved in the
pathogenesis:
1. Increased sebum production
2. Hypercornification of the pilosebaceous duct
3. Microbial flora
4. Inflammation.

Increased Sebum Production


Acne patients, male and female, excrete on average
more sebum than normal subjects and the level of
secretion correlates with the acne severity.

Ductal Hypercornification
Hypercornification of pilosebaceous ducts
presents histologically as microcomedones and

clinically as blackheads and whiteheads. Thus


comedones represent the retention of
hyperproliferating ductal keratinocytes in the
duct.

Bacteria
Acne is not infectious. However, three major
organisms isolated Propionibacteria (P. acnes,
P. granulosum, P. avidum), Staphylococcus
epidermidis and Malassezia furfur. Environment of
bacteria (i.e. low pH, reduced oxygen tension and
bacterial lipases, proteases, etc.) more important
than absolute numbers.

Inflammation
The dermal inflammation is not caused by bacteria
in the dermis but from inflammatory mediators
that diffuse from the follicle where they are
produced by P. acnes.

Natural History
Usually starts in adolescence and resolves by
mid-twenties.
At least some degree of acne affects 95% and
83% of adolescent boys and girls.
Acne develops earlier in females than in males.
At the age of 40 years, acne may persist in 1%
of males and 5% of females.

Disorders of Sebaceous, Eccrine and Apocrine Glands

Clinical Features
Occurs predominantly on the face (99%), back
(60%) and chest (15%). Infraorbital area
spared even in severe acne.
Two types of lesions:
i. Non-inflammatory (comedones)
ii. Inflammatory
Comedones are the pathognomonic lesions of
acne (Fig. 19.1). They are conical, raised lesions
with a broad base and a plugged apex. Two
types of comedones blackheads/open
comedones (black color due to oxidation of
melanin) and whiteheads/closed comedones.
25% of the whiteheads resolve within three
days while another 75% develop into inflamed
lesions.
Inflammatory lesions include papules,
pustules and nodules or nodulocystic lesions
(Figs 19.2 and 19.3).
Nodules occur more frequently in males and
may be interconnected with sinuses.
In its most severe variant, acne can present
with cysts and abscesses.
Some amount of scarring in 90%. These could
be hypertrophic scars, keloids, atrophic scars
or ice-pick scars.
Acne severity can be graded into mild (multiple
open and closed comedones are present, with

Fig. 19.1: Acne vulgarisface showing comedones,


papules, and pustules

few inflammatory papules), moderate (with


erythematous papules and pustules which are
the predominant lesions, and disease is
limited to the face), moderately severe (with
erythematous papules, pustules, and nodules
that are present on the face), and severe (with
multiple painful nodules that are present on
the back in spite of aggressive topical and oral
interventions, respond only to isotretinoin).

Differential Diagnosis
Acne is rarely misdiagnosed. The commonest
mistaken diagnosis is rosacea (has facial flushing,
typically pustules occurring over erythematous
background, localization of lesions to mid-face,

Fig. 19.2: Acne vulgarisface showing comedones,


papules, pustules and nodules

Fig. 19.3: Acne vulgarisnodulocystic lesions in


addition to other acne lesions over the cheek

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Essentials in Dermatology
absence of seborrhea, comedones, nodules, cyst
or scarring). Perioral eczema/dermatitis can be
confused with acne in females, but lesions itch,
the skin is dry and there are no comedones.
Whiteheads may be confused with milia (which
are predominantly infraorbital in distribution and
are whiter). Acneiform drug eruptions are sudden
onset, follicular monomorphous eruptions
characterized by papules and pustules
resembling acne, induced by skin exposure to
various industrial chemicals or medications.
Folliculitis due to Gram-negative organisms can
complicate acne therapy and rarely folliculitis due
to Candida may also present as multiple pustular
eruptions as may S. epidermidis folliculitis.
Demodex folliculitis can present as nonresponsive acne, which best responds to
metronidazole or topical permethrin. Rarely acne
vulgaris need to be differentiated from Pityrosporum folliculitis, acne agminata, adenoma
sebaceum lesions of tuberous sclerosis,
pseudofolliculitis barbae, plane warts,
acneiform eruption of Behcets disease.

Treatment
Treatment involves counseling, acne assessment
and appropriate and ethical prescribing based
on the history, acne severity, lesion type and the
psychological effects of the disease. The various
modalities of treatment can be classified into
topical and systemic therapies (Table 19.1).

Topical
Predominantly comedolytic:
Tretinoin (0.025%, 0.05%, 0.1% creams,
gels) (reduce number of existing
comedones and prevent formation of new
comedones by loosening ductal keratinocytes) - The best treatment for
comedones. Almost every patient benefits
from topical retinoids.
Adapalene (0.1% cream, gel, solution).
Azelaic acid (20% cream).

Predominantly antimicrobial:
Clindamycin (1% gel, solution).
Erythromycin (1.5-2% gel, solution,
ointment).
Benzoyl peroxide (2.5-10% gel, lotion,
cream).
Nadifloxacin.
Clarithromycin.
Tetracycline.
Sodium sulfacetamide- sulphur combination.
Predominantly anti-inflammatory
Adapalene.
Topical antibiotics.
Combination preparations.
Zinc and erythromycin.
Benzoyl peroxide and erythromycin.

Systemic
Oral antibiotics (oral tetracycline 250-500 mg
1-4 times a day, oral minocycline 50-100 mg
daily, doxycycline 50-100 mg daily,
erythromycin 250-500 mg 2-4 times a day,
trimethoprim 300 mg twice a day, pulse
dosing with azithromycin -250 mg daily for
three days in a week for 4-6 months or
roxithromycin).
Hormones (anti-androgens like 2 mg
cyproterone acetate with 35 microgram ethinyl
estradiol, flutamide-250 mg twice a day, oral
contraceptive pills, spironolactone 100-200
mg daily, finasteride 2.5-5 mg daily): Indicated
for women with acne located primarily on the
lower face and neck, those with PCOS and
late onset acne, late onset adrenal hyperplasia
or other identifiable endocrinologic conditions.
Isotretinoin: It is the only anti-acne agent that
affects all four of the known major etiologic
mechanisms: sebum production, comedogenesis, Propionobacterium acnes (P. acnes)
colonization of ductal and skin surface, and
monocyte chemotaxis-induced inflammation.
It is indicated for nodulocystic acne, acne

Disorders of Sebaceous, Eccrine and Apocrine Glands


conglobata, acne fulminans, moderate acne
relapse, significant psychosocial impairment,
marked concomitant seborrhea, Gramnegative folliculitis, and scarring or persistent
dyschromia. Acne therapy is usually initiated
at a dose of 0.5 mg/kg daily for the first
2-4 weeks and then increased to 1.0 mg/kg/
day for the remainder of the 20 weeks course.
The chance of a prolonged remission is greater
when a total dose of 120 to 150 mg per kilogram
of body weight is achieved.
Corticosteroids (co-prescribed with
isotretinoin in severe acne variants).

Miscellaneous

Oral zinc.
Dapsone (nodulocystic acne).
Clofazimine (acne fulminans).
NSAIDs (to reduce inflammation).
Chemical peels.
Cryotherapy.
Blue light therapy.

Surgical Procedures

Comedone extraction.
Aspiration of cysts.
Incision and drainage (large cysts).
Intralesional steroid (0.1 ml triamcinolone
injected into base of the cyst; reduces scarring).

Course and Prognosis


The course and prognosis of acne is highly
variable. All acne causes scars.
ACNE VARIANTS WITH MARKED
INFLAMMATION
Acne Conglobata
It is a rare type of acne, which is highly
inflammatory and presents with comedones,
nodules, abscesses and draining sinus tracts.
Healing occurs with severe scarring. It usually
starts in adult life. Oral isotretinoin is the best
treatment for patients in whom antibiotics are
unsuccessful or in patients with very severe deep
acne like this one.
Follicular Occlusion Tetrad (Acne tetrad)
It is a combination of acne conglobata, acne
inversa/hidradenitis suppurativa, dissecting
cellulitis of scalp and pilonidal sinus. It is more
common in men.
SAPHO Syndrome
It is characterized by Synovitis, Acne (conglobata,
fulminans), Pustulosis (pustular psoriasis,
palmoplantar pustulosis), Hyperostosis and
Osteitis.
Acne Fulminans
It is characterized by the sudden appearance of
severe acne with systemic signs and symptoms
(fever, leukocytosis, osteomyelitis, polyarthralgia,

Acne Scar Surgery


Dermabrasion.
Laser abrasion.
Chemical peels.

Table 19.1: Treatment guidelines for acne vulgaris

Grade 1(Mild)

Grade II-III(Moderate)

Grade IV(Severe)

Maintenance therapy

Topical retinoids
Benzoyl peroxide
(BPO) or topical
antibiotic (A/B)

Topical retinoids
BPO or topical A/B

Isotretinoin or
Topical retinoids,
oral A/B, hormone
therapy

Topical retinoids + BPO

+
Oral A/B
Hormone Rx

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Essentials in Dermatology
polymyalgia). The disease exclusively occurs in
teenage boys. The face is often not involved.
Treated initially with high dose corticosteroids
to control inflammation and then retinoids

SPECIAL TYPES OF ACNE


Acne from Drugs and Chemical Agents
(Acneiform Eruptions)
Due to topical or systemic glucocorticoids,
anabolic steroids, INH, cyclosporine, iodides and
bromides, lithium, etc.
Oil Acne
It is due to lubricating and cutting oil application
and is seen mainly on front of thighs and forearms,
predominantly as comedones.
Chloracne or Industrial Acne
It is due to topical or systemic exposure to toxic
chlorinated hydrocarbons and causes severe
comedonal acne.
Excoriated Acne
It is seen in young women. Small acne spots are
picked, squeezed and altered by manual
interference. Resulting papules are crusted and
inflamed.
ROSACEA
It is a centrofacial disease, which is characterized
by papules and papulopustules against a vivid
erythematous background with telangiectases,
preceded by episodes of flushing (Fig. 19.4). Later,
there may be diffuse hyperplasia of connective
tissue with enlarged sebaceous glands,
particularly of the nose (rhinophyma). Women
are more often affected than men in their third
and fourth decade.
Ophthalmological complications are
common, occurring in over 50% of the patients
with rosacea. These include sensation of grittiness

Fig. 19.4: Rosaceaface showing erythema


topped by erythematous papules and pustules

or irritability of the eyes, often accompanied by


visible reddening of the conjunctiva. Blepharitis,
keratitis, episcleritis, chalazion, and hordeolum
are also common.

Differential Diagnosis
Important differential diagnoses include acne
vulgaris (typical acne lacks the redness,
telangiectasia, and flushing of rosacea), lupus
erythematosus (DLEscarring, scaling and
follicular plugging are not features of rosacea,
SLE-butterfly erythema is not pustular and is
usually associated with systemic symptoms),
perioral dermatitis (lesions itch, the skin is dry),
seborrhoeic dermatitis (scaling in typical
seborrhoeic areas) and demodex folliculitis
(clearly mimics rosacea). Nasal sarcoidosis (lupus
pernio) is the differential diagnosis for
rhinophyma.
Treatment
Papulopustular rosacea responds well to
treatment. Topical treatment with metronidazole
(1% cream, 0.75% gel, cream, lotion) is effective.
Additional topical therapies reported as effective
include tetracycline, clindamycin, erythromycin,
azelaic acid 20% cream and 15% gel, 0.025%

Disorders of Sebaceous, Eccrine and Apocrine Glands


retinoic acid and 10% sulphur cream. Topical
imidazoles are also gaining popularity in the
treatment of rosacea.
Effective oral treatment includes tetracycline
or oxytetracycline 250 mg twice daily and
erythromycin 250 mg twice daily. More recently
developed tetracyclines such as minocycline,
lymecycline and doxycycline are often used. Oral
metronidazole (750-1500 mg in divided doses) is
also effective. Oral isotretinoin (10-60 mg/day) is
an alternative in resistant rosacea and can even
improve rhinophyma.
Flushing and burning are the most difficult
features of rosacea to treat. Non-cardioselective
beta-blockers such as propranolol 40 mg twice
daily or nadalol 40 mg daily, clonidine 50 mcg
twice daily, and rilmenidine 1 mg daily.
Advent of vascular laser and intense pulsed
light sources has provided a range of highly
effective treatments for ablation of telangiectasia.
Surgical treatment is a very successful
treatment in rhinophyma. Excellent cosmetic
results can be obtained by scalpel or razor
remodeling, electrocoagulation, cryosurgery,
excision and vaporization with argon, carbon
dioxide or Nd: Yag lasers.

PERIORAL DERMATITIS
It is facial dermatosis predominantly affecting
females of childbearing years. The dermatosis is
characterized by an erythematous, micropapular,
fine scaling eruption classically affecting the
nasolabial folds, chin, and upper lip, sparing a
rim of skin at the vermilion border. In nearly every
patient, there is striking dependency on topical
steroids. A frequently heard story is that the
patient presents with the earliest signs of rash
and is given a potent topical steroid. The
condition then shows improvement but any
attempt to stop the treatment dramatically
worsens the condition after a few days.

Differential Diagnosis
The important differential diagnoses include
rosacea (usually no telangiectasia or flushing in
perioral dermatitis), lip licking cheilitis (seen
predominantly in 7-15 years old age group, the
rash is caused by repeated licking of skin around
the mouth and is marked by a scaling, pink band
around the mouth and involvement of lips),
seborrhoeic dermatitis (not usually circumoral
and the scalp, ears and eyebrows are commonly
involved), contact allergic dermatitis (does not
spare the immediate perioral area), late-onset acne
vulgaris (evidence of comedones, large papules
and cysts in wider distribution and responds
more slowly to treatment), acne agminata (difficult
to distinguish if confined to perioral area but can
be differentiated histologically) and facial AfroCaribbean childhood eruption (FACE) (do not
spare the perilabial skin, pustules do not occur,
occur often in males).
Treatment
The most important measure is usually to
discontinue the application of the topical
corticosteroids. The patient should be warned that
an initial flare may develop after the withdrawal
of a topical corticosteroid. A four week course of
oral tetracycline or its analogues such as
minocycline, doxycycline or lymecycline is
usually all that is required. Topical metronidzole,
topical erythromycin, and topical tetracycline
have also been used.
DISORDERS OF ECCRINE SWEAT
GLANDS
Disorders of eccrine sweat glands may be broadly
classified into four-chromhidrosis, hyperhidrosis,
hypo- or anhidrosis and miliaria.

Chromhidrosis
It means secretion of colored sweat. It is an
exceedingly rare functional disorder of the

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Essentials in Dermatology
apocrine sweat glands. The colored sweat may
be yellow (most common), blue, green, or black.
Colored sweat fluoresces and is caused by
lipofuscin. Topical capsaicin satisfactorily
reduces facial and nipple chromhidrosis.

Hyperhidrosis
It means excessive sweating which could be
localized or generalized. It may occur as
consequence of a number of causes. Most
commonly excessive sweating of the palms and
soles occurs during mental stress, and may be
associated with tachycardia and vasomotor
instability.

Palmoplantar Hyperhidrosis
(Emotional Hyperhidrosis)
This type of hyperhidrosis is usually localized to
the palms, soles or/and axillae. Frequently there is
a family history and commonly begins in
childhood or around puberty. There is a tendency
of spontaneous improvement after the age of 25
years. Sweating may be intermittent (triggered by
anxiety, stress, or fear) or may be constant.
Apart from the embarrassing nature of the
disorder, complications include pompholyx,
contact dermatitis, and pitted keratolysis.

Gustatory Hyperhidrosis
Sweating on the lips, forehead and nose after
eating certain foods is known as gustatory
sweating. Gustatory sweating may be idiopathic,
post-peripheral nerve injury (parotid surgery,
auriculotemporal nerve syndrome or von Frey
syndrome, cervical sympathectomy), sensory
neuropathy (diabetes mellitus, post herpes zoster).

Treatment
Topical anticholinergics (0.5% glycopyrronium
bromide cream), formalin 1% soaks, glutaraldehyde 10% in a buffered solution, 20%
aluminium chloride have been used. Iontophoresis either using tap water or anticholinergic

drugs like glycopyrronium bromide is a


satisfactory method. Direct current is usually
used, with each palm or sole being treated for 30
min with 20 mA initially three times a week.
Botulinum toxin injection into 4 cm2 areas on the
palms, soles or axillae dramatically reduces
sweating. The hypohidrosis continues for an
average of 7 months. Axillary hyperhidrosis may
be effectively controlled by excision of the most
active sweating portion of the axillary skin,
alternatively liposuction removal may be used.

Anhidrosis (Hypohidrosis)
It can result from poral occlusion, congenital or
acquired absence of sweat glands, damage to
sweat gland function or dysfunction of
sympathetic nerves in neuropathies. It may be
localized or generalized.
Miliaria
It results from the obstruction of free flow of eccrine
sweat to the skin surface and retention of the sweat
within the skin. It manifests with a variety of signs
and symptoms. Miliaria crystallina (Sudamina)
consists of non-inflammatory, superficial
subcorneal translucent vesicles that easily
rupture when rubbed with a finger. Miliaria rubra
(prickly heat) causes pruritic inflammatory
papules around the sweat pores, follows repeated
episodes of sweating in a hot humid environment.
Some of the eruptions of miliaria rubra become
pustular resulting in miliaria pustulosa. Miliaria
profunda results when sweat leaks into the
dermis and presents as multiple discrete, flesh
colored papules.
The most common complications of miliaria
are secondary infection and disturbance of heat
regulation. Periporitis staphylogenes is the name
given to multiple staphylococcal abscesses
superimposed on miliaria rubra in young infants
(Fig. 19.5). Postmiliarial hypohidrosis invariably
occurs following miliaria and sweating may be
depressed to half the normal amount for as long

Disorders of Sebaceous, Eccrine and Apocrine Glands

Fig. 19.5: Miliaria complicated by secondary


infection of sweat glandsperiporitis

as 3 weeks following miliaria. Affected persons


may show decreasing efficiency, irritability,
anorexia, drowsiness, vertigo and headache.
Tropical anhidrotic asthenia is a rare form of
miliaria with long lasting poral occlusion, which
produces anhidrosis and heat retention.

Treatment
The most effective treatment is to place the patient
in a cool environment as this will avoid further
sweating. Avoidance of excessive clothing,
friction with clothing, excessive use of soap and
contact of the skin with irritants will reduce the
incidence. Calamine lotion is effective in the relief
of discomfort. Oral ascorbic acid 500 mg twice
daily has been found to diminish the severity of
miliaria.
DISORDERS OF APOCRINE GLAND
The capacity to produce an unpleasant odor is
best-known property of apocrine gland.
Physicians in the past relied heavily on detecting
special body odors in diagnosis of diseases, e.g.
fruity odour in diabetic coma, butcher shop odor
in yellow fever, freshly baked bread odor in
typhoid fever, etc.

Bromidrosis (Bromhidrosis or
Osmidrosis)
Excessive odor arising from apocrine sweat, also
known as fetid sweat or malodorous sweating. It
is chiefly encountered in axillae. Often the patient
complains of offensive axillary sweat but actually
have no offensive odor. The complaint represents
a phobia, delusion, paranoia or a lesion of the
central nervous system. True bromidrosis is
usually not recognized by the patient. Antibacterial soaps, deodoarants, frequent bathing,
changing of underclothes, shaving of the axillae
and application of aluminium chloride are all
helpful measures.
Fish Odor Syndrome
(Trimethylaminuria)
Affected individuals are unable to oxidize tertiary
trimethylamine produced by oxidation of choline
and carnitine in food. This results in formation of
excessive amounts of offensively smelling (rotting
fish) sweat. Defect in flavin containing
monooxygenase 3 (FMO3) gene causes the
disease. Diet low in carnitine and choline may
help.
Chromhidrosis
Coloured apocrine sweat.
Fox Fordyce Disease
It is a disorder of the apocrine sweat gland
comparable to prickly heat of the eccrine glands.
It is a chronic itchy papular eruption of apocrine
gland bearing areas-axillae (Fig. 19.6) and pubic
area, classically seen in women between the ages
of 15 and 35 years. Pruritus is often exacerbated
by emotional or physical stimuli of the apocrine
glands.
Differential Diagnosis
Includes lichen planus, lichen nitidus, infective
folliculitis, chronic dermatitis, and syringoma.

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Fig. 19.6: Fox Fordyce diseasechronic itchy


papular eruption in the axilla of a female

Fig. 19.7: Hidradenitis suppurativanodules,


sinuses and puckered scarring in the axilla

Treatment
Estrogen therapy in the form of oral contraceptive
pills is universally effective. Topical tretinoin,
clindamycin solution, UV phototherapy have been
tried. Treatment response with topical steroids or
intralesional steroids is not satisfactory.

epidermoid cyst. Other dermatoses that produce


fistulas and sinuses should be excluded, such as
scrofuloderma, actinomycosis, tularemia, and cat
scratch disease. In the inguinal region,
lymphogranuloma venerum, granuloma
inguinale, Crohns disease, and ulcerative colitis
should be excluded.

Hidradenitis Suppurativa (Apocrinitis,


Hidradenitis Axillaris, Acne Inversa)
It is a chronic and cicatricial disease of apocrine
gland bearing skin areas-axillae, anogenital skin
(Fig. 19.7). The disease occurs in both sexes,
usually in the second or third decade. It is rare
before puberty. Affected patients are often
overweight.
Differential Diagnosis
A solitary abscess in the early stage resembles a
carbuncle, lymphadenitis, or an infected

Treatment
Earliest lesions often heal quickly with
intralesional steroid therapy. Concomitant use of
antibiotics (minocycline, cephalosporins,
ciprofloxacin) is recommended. Isotretinoin (1
mg/kg/day) is effective in some patients. In severe
disease, removal of the affected area is often
required. Wide local excision and healing by
second intention is considered the surgical
treatment of choice.

Disorders of Hair and Nails

20

Disorders of Hair and Nails

DISORDERS OF HAIR
Alopecia
Alopecia is loss of hair. It may be diffuse or patchy,
scarring or non-scarring.
Diffuse Non-scarring Alopecia
1. Androgenetic alopecia: Terminal hair is
converted into vellus hair. In men, recession of
the frontal hairline near the temples and thinning
over the vertex occurs (Fig. 20.1). In women, the
scalp hair is generally diffuse but more so in the
vertex area.
Treatment includes in males- topical
minoxidil (2 and 5% solution) or oral
finasteride competitive inhibitor of type II 5
-reductase -1 mg/day and in females
topical minoxidil 2% and antiandrogens
(spironolactone, cyproterone acetate,
flutamide) and surgical treatment such as
scalp reduction and hair transplantation.

induces a synchronous growth of hair follicles


6-10 weeks later such that more synchronous hair
fall is seen. It commonly follows delivery of a baby,
high fever, surgery or other stress. Drugs such as
enalapril, beta blockers, retinoids, oral
contraceptive pills, lithium, levodopa may be
responsible. Treatment is reassurance, removal
of stress or drug responsible. Hair density usually
returns to normal in 6-12 months.

2. Endocrine and nutrition related: Endocrine


abnormalities of the thyroid, pituitary or adrenals
can cause diffuse alopecia. Malnutrition and zinc
deficiency or iron deficiency may also cause
diffuse alopecia.
3. Telogen effluvium: Certain events can induce
hair follicles to enter the telogen phase. This then

Fig. 20.1: Androgenetic alopeciascalp showing


receding frontal hairline

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Essentials in Dermatology

Fig. 20.2: Anagen effluvium diffuse non-scarring


alopecia following use of cytotoxic drugs

Fig. 20.3: Alopecia areata rounded bald smooth


patches over the scalp

4. Anagen effluvium: Abrupt cessation of hair


growth follows use of cytotoxic drugs
(cyclophosphamide) (Fig. 20.2), heparin, warfarin,
colchicine, vitamin A, etc.

Patchy non-scarring alopecia


Alopecia Areata
Alopecia areata is an unpredictable usually
patchy, non scarring hair loss condition. The
word alopecia is derived from the Greek,
Alopex, meaning Fox mange (baldness).
Any hair bearing surface may be affected.
It is a common condition, probably autoimmune in etiology. Associated autoimmune
diseases are Hashimotos disease, vitiligo and
myasthenia gravis.
It affects men and women equally.
It generally starts in the 2nd or 3rd decade.
Patients are frequently quite young, sixty
percent of them present before 20 years of age.
The characteristic initial lesion is commonly
a round or oval, totally bald, smooth patch

Fig. 20.4: Alopecia areata many bald patches


coalescing over the scalp

involving the scalp (Figs 20.3 and 20.4) or any


hair bearing area on the body.
A frequent feature in an alopecia areata patch
is Exclamation mark hairs that are broken

Disorders of Hair and Nails


short hairs tapering proximally. The pull test
may be positive at the margins of the patch
indicating very active disease.
Alopecia areata progresses as a wave of
follicles, enter telogen prematurely.
The scalp is the most common site but any site
can be involved. When the perimeter of the
scalp is involved, it is called ophiasis (so called
from its resemblance to a snake) (Fig. 20.5),
which is associated with poor prognosis.
Alopecia areata can occur as diffuse loss of
hair from the scalp but spares the grey hair
(Fig. 20.6).

If there are multiple patches on the scalp, they


may become confluent to result in total loss of
hair from the scalp, called as alopecia totalis
(Fig. 20.7). Total loss of hairs from the whole
body is called alopecia universalis.
Nail dystrophy is associated with alopecia
areata in 10 to 66% of cases. Nail pitting
Hammered brass (Fig. 20.8), trachyonychia
(longitudinal striations), Beaus line,
onychorrhexis, thinning or thickening,
onychomadesis, punctate or transverse

Fig. 20.7: Alopecia totalis

Fig. 20.5: Alopecia areataophiasis pattern

Fig. 20.6: Alopecia areatadiffuse hair loss


sparing grey hair

Fig. 20.8: Alopecia areata fine pitting of nail

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Essentials in Dermatology

192

leukonychia, red spotted lunula and nail


dystrophy may be seen in the nails.

Classification of Alopecia Areata


According to Pattern
1. Patchy alopecia areata-round or oval patches
of hair loss (most common) (Fig. 20.3).
2. Reticulated pattern of patchy hair loss (Fig.
20.4).
3. Ophiasis, a band-like alopecia areata, hair
loss occurs in the parietal temporo-occipital
scalp (Fig. 20.5).
4. Ophiasis inversus(sisaipho), a rare band like
pattern of hair loss in the frontal parieto
temporal scalp (the exact opposite of ophiasis)
5. Diffuse alopecia areata, a diffuse decrease in
hair density over the entire scalp.

According to the Extent of Involvement


1. Alopecia areata, partial loss of scalp hair
2. Alopecia totalis, 100% loss of scalp hair (Fig.
20.7).
3. Alopecia universalis, 100% loss of hairs on
the scalp and body.

Indicators of a Poor Prognosis


1.
2.
3.
4.
5.
6.
7.

Underlying atopy
The presence of other autoimmune diseases
Family history of alopecia areata
Young age at onset
Nail dystrophy
Extensive hair loss
Ophiasis.

Diagnosis
The characteristic non scarring patchy loss of
hairs, the presence of exclamation point hair on
the periphery, a hair pull test indicating telogen
hair excess and in atypical cases skin biopsy
showing specific findings (a peribulbar and at
the lower one third of the follicle, a lymphocytic
infiltrate, swarm of bees, with no scarring) all
points towards the diagnosis.

Differential Diagnosis
Alopecia areata needs to be differentiated from
tinea capitis, trichotillomania and secondary
syphilis (moth-eaten appearance in beard or
scalp).
Treatment
Various modalities of treatment are available,
the simplest of which are the use of counter
irritants like phenol, benzoyl, tincture iodine,
dithranol, etc. Physical modalities like UVB
in erythema doses, grenz rays and thorium
X-ray may be used with varied success.
Considering the autoimmune etiology of the
disease various immunosuppressive drugs
have been used. Among them, corticosteroids
are important which can be used topically,
intralesionally and systemically.
Photochemotherapy, contact immunotherapy
(using dinitrochlorobenzene [DNCB], squaric
acid dibutylester [SADBE], diphencyprone),
topical minoxidil or topical cyclosporine may
be used.
Course and Prognosis
Alopecia areata, however, a serious cosmetic
problem, has unpredictable course.
Tinea capitis: Discussed earlier under
dermatophytosis.
Trichotillomania
It is the term (Greek-hair pulling
madness) first used by the French
dermatologist Hallopeau to denote a
morbid impulse to pull ones own hair.
It manifests as one or a few small oval
patches of hair loss which may occur in
any hair area of the body in children or
adults.
The patches are unusually irregular,
asymmetric, and ill defined and have
many broken hairs.
Common sites of involvement include
scalp (Fig. 20.9), eyebrows, eyelashes, and
pubic area.

Disorders of Hair and Nails

Fig. 20.9: Trichotillomania unusually irregular patchy


loss of hair over the temple area of scalp due to
pulling of hairs

Fig. 20.10: Perifolliculitis decalvans-scarring


alopecia with folliculitis

Trichophagy is rarely reported with


trichotillomania, and when it occurs,
trichobezoars (obstructive hair balls in the
gastrointestinal tract) are the most severe
complication.
Clinical differentiation from scalp alopecia
areata is usually based upon the presence
of short, broken hairs within the patch,
and more irregular, often angular outlines
of the area. Differentiation from tinea
capitis may require Woods light
examination, potassium hydroxide, or
culture. The scalp in trichotillomania is
usually healthy with broken hairs rather
than scaly or erythematous in tinea capitis.
Any method of preventing grasping of
hairs for a period of time (restraint, heavy
gloves) will clarify the diagnosis and in
one way serve as treatment, since hairs
will grow normally in the area in the
absence of trauma. One form of treatment
consists of simply shaving the area.
Psychological intervention and drugs
such as clomipramine or fluoxetine may
be given.

Secondary syphilis: Moth-eaten alopecia may be


produced.

Traumatic alopecia: It is due to traction or


pressure.

A. Ovarian (Polycystic ovarian disease,


hyperthecosis),

Patchy/Diffuse Scarring Alopecia


1. Physical and chemical injury.
2. Infection Kerion, favus, herpes zoster,
folliculitis decalvans (Fig. 20.10).
3. Lichen planus and DLE.
4. Pseudopelade: Pseudopelade describes a
scarring alopecia which represents the end
stage of an idiopathic or unidentified
destructive inflammatory process in the scalp.
Characteristically, involves the vertex area of
the scalp with crab like extensions (Fig. 20.11).
5. Malignancy Basal cell carcinoma, squamous
cell carcinoma.
Excess hair
Hirsutism: It refers to hair growth in a woman in
areas of the body where hair growth is under
androgen control and in which normally only
post-pubescent males have terminal hair growth.
These areas include the moustache, beard, chest
and inner thigh.
Causes are

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Essentials in Dermatology

Fig. 20.11: Scarring alopecia of scalp-crab like


extensions over the scalp-pseudopelade

Fig. 20.12: Clubbing of nails

B. Drugs (androgens, oral contraceptive pills,


glucocorticoids, diazoxide, minoxidil,
phenytoin).

A. Shape Abnormalities

C. Endocrine Adrenal (Congenital adrenal


hyperplasia, 21 and 11 hydroxylase deficiency,
ACTH dependant Cushings syndrome).

Clubbing: It means increased transverse and


longitudinal curvature of nails with hypertrophy
of the soft tissue components of the digit pulp
(Fig. 20.12). To confirm it, one can oppose dorsal
aspects of two fingers from opposite hands,
between the two opposed nails normally a
window of light is seen which get obliterated in
clubbing(window closes).

Others- hypothyroidism, acromegaly and


D. Physiological puberty, pregnancy.
Treatment includes removal of precipitating
cause/s, drug therapy with oral contraceptive
pills, spironolactone, flutamide, finasteride,
cyproterone acetate, and glucocorticoids, physical
methods such as shaving, bleaching, waxing,
laser, or electrolysis and topical therapy with
eflornithine hydrochloride cream (slows hair
growth by blocking ornithine decarboxylase).

Anonychia: It means absence of nails. Usually a


developmental anomaly.

Clubbing Causes
CLUBBING

DISORDERS OF NAILS

Cyanotic Heart Disease


Lung disease (hypoxia, lung cancer,
bronchiectasis, cystic fibrosis)
UC/Crohns disease
Biliary cirrhosis
Birth defect (harmless)
Infective endocarditis
Neoplasm (esp. Hodgkins)
GI malabsorption.

Nails may show their abnormalitiesin shape,


surface and color.

Koilonychia: Reverse curvature in the


longitudinal and transverse axis of nail gives a

Hypertrichosis: It specifically refers to hair density


or length beyond the accepted limits of normal
for a particular age, race, or sex. The excess hair
may be generalized or localized and may consist
of lanugo, vellus, or terminal hair.

Disorders of Hair and Nails

Fig. 20.13: Koilonychiaspoon like deformity of


finger nails

Fig. 20.15: Onychogryphosis thickened great toe


nail growing upwards

Pterygium: When a central fibrotic tongue like


band from proximal nail fold joins the nail bed
dividing the nail proximally into two, it is known
as pterygium. For example, it occurs in lichen
planus.
Subungual hyperkeratosis: It means excessive
collection of squamous debris under the free edge
of the nail. For example, it occurs in psoriasis,
onychomycosis, etc.
Onychogryphosis: Here nail gets thickened and
grows upwards in a spiral manner attributed to
chronic trauma (Fig. 20.15).

Fig. 20.14: Onycholysis with pitting of finger nails in


psoriasis

Pachyonychia congenita: Here hypertrophy of


nails is associated with nail bed and hyponychial
hyperkeratosis (Figs 20.16 and 20.17).

B. Surface Abnormalities

concave dorsal aspect to the nail, such nail is


thought to have koilonychia (Fig. 20.13).
Commonly, it is due to iron deficiency anemia.

Beaus lines: They are transverse grooves on nails


which arise out of temporary interference with
nail formation (Fig. 20.18).

Macronychia and micronychia: Too large or too


small nail in comparison with other nails of digits.

Pitting: These are punctate erosions on the nail


surface (Fig. 20.14). Commonly seen in psoriasis,
and alopecia areata.

Onycholysis: Distal or lateral separation of the


nail from the nail bed (Fig. 20.14). May be seen in
psoriasis, onychomycosis, etc.

Median canaliform dystrophy: Nail shows


midline split with fir tree like appearance of ridges

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Essentials in Dermatology

Fig. 20.16

Fig. 20.17

Figs 20.16 and 20.17: Pachyonychia congenitathickening of nails with subungual hyperkeratosis

Onychoclasis: Breaking of the nail.


Onychomadesis: Separation of the proximal part
of nail plate from the matrix and bed with
subsequent shedding of nail.
Onychomalacia: Softening of nail.
Onychorrhexis: Longitudinal ridging and
fissuring of nail plate with brittleness and
breakage.
Onychotillomania: Compulsive picking or tearing
of nail.
Fig. 20.18: Beaus linestransverse depressions
equidistant from the nail folds

angled backwards. Commonly, it affects the thumb


nail and involvement is symmetrical.
Onychoschizia: It is characterized by transverse
splitting of nail into layers at or near the free edge
of the fingers and toes.
Onychauxis: Nail plate appears to be thickened
due to subungual hyperkeratosis of nail bed seen
in psoriasis, eczema, distal subungual
hyperkeratosis.

Trachyonychia: Nails are rough and often


thinned (20 nail dystrophy or sand paper nail)
seen in alopecia areata, lichen planus, psoriasis,
etc.

C. Color Abnormalities
Color changes in nail may be due to exogenous
causes or endogenous causes.
Leukonychia: This term is used for white
discoloration of nails attributable to nail matrix
dysfunction.
Melanonychia: Means streaky hyperpigmentation of nails.

Disorders of Hair and Nails

Fig. 20.19: Half and half nail proximal half white and distal half brownish

Terrys nail: Nail is white proximally but normal


distally. It occurs in congestive cardiac failure,
cirrhosis or adult onset diabetes mellitus.
Red lunulae: Nail shows erythema of all or part
of lunula. Mostly affects thumb nail. Dotted red
lunula occurs in psoriasis. Other causes are
congestive cardiac failure, cirrhosis of liver,
systemic lupus erythematosus, etc.
Splinter hemorrhages: They represent
longitudinal hemorrhages in the nail bed,
resembling the pattern of subungual vessels. They
may occur in dermatoses like psoriasis,
dermatitis, fungal infection or systemic diseases
like endocarditis, systemic lupus erythematosus,
mycosis fungoides, rheumatoid arthritis, etc.
Half and half nails: It is onychopathological
index of renal diseases and nail exhibits either
normal or whitish proximal half and distinctly
abnormal, brownish distal portion (Fig. 20.19).

Infections of Nails and Nail Folds


Acute paronychia: It presents with painful
swelling of the nail fold. Most commonly caused
by staphylococci.
Chronic paronychia: It presents with chronic
swelling of nail folds of one or more fingers, often
middle and index fingers, and is associated with
candida and/or pseudomonas infection.
Herpetic paronychia (Herpetic whitlow): It is due
to primary inoculation of herpes simplex virus
on to the finger and presents as single/grouped
vesicles close to the nail.
Tinea unguium: It is the fungal infection of
nail complex by a dermatophyte and may be
distal subungual infection, proximal
subungual infection, or superficial white
onychomycosis
Onychomycosis: It includes tinea unguium and
also infection by non-dermatophytic fungi
including yeasts.

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Essentials in Dermatology

21

Metabolic and
Nutritional Disorders

PORPHYRIA
Porphyrias are hereditary disturbances in the
synthesis of heme involving well-defined
enzymatic defects.
The term porphyria is derived from the
Greek word for purple and originally
referred to the red to purple color of the urine
of patients affected by acute intermittent
porphyria. Other forms of porphyria produce
urine that varies from pink to red to brown.
Unlike findings with hematuria and
pigmenturias (e.g., hemoglobinuria due to
hemolysis, myoglobinuria caused by
rhabdomyolysis), routine dipstick tests are
negative for the presence of heme in porphyria
patients.
Porphyrins also account for the fluorescence
of urine viewed with a Woods lamp.
Almost all are inherited in an autosomal
dominant pattern except congenital
erythropoietic porphyria which is inherited
in an autosomal recessive manner.
In addition, although the enzyme defects are
genetic and permanent, the symptoms are
often intermittent and do not appear until
puberty except in congenital erythropoietic
porphyria and hepatoerythropoietic
porphyria (both manifest in infancy or

childhood, resemble clinically each other).


This is true in porphyria cutanea tarda, acute
intermittent porphyria, and variegate
porphyria.
Photosensitivity in porphyria is due to
absorption of UV radiation in soret band
(400-410 nm).
Factors which precipitate are four ms:
medication (ethanol, oestrogens, iron,
hexachlorobenzene), menses, malnutrition
and medical illness.

Clinical Classification
These disorders can be classified into those
without vesicobullous lesions and those
associated with them.
The former includes delta aminolevulinic acid
dehydratase deficiency porphyria and acute
intermittent porphyria (have no cutaneous
manifestations), and erythropoietic protoporphyria (can cause photosensitivity, but
vesicobullous lesions are rare).
The latter includes congenital erythropoietic
porphyria (Gunthers disease), hepatoerythropoietic porphyria, hereditary
coproporphyria, variegate porphyria (most
prevalent in white population of South Africa)
and porphyria cutanea tarda (PCT).

Metabolic and Nutritional Disorders

Congenital Erythropoietic Porphyria


Congenital erythropoietic porphyria manifests
in infancy or early childhood.
It is the result of defective activity of the
enzyme uroporphyrinogen cosynthase.
Clinical manifestations include severe
bullous cutaneous photosensitivity (Fig. 21.1),
hypertrichosis (Figs 21.2 and 21.3),

erythrodontia (fluorescent teeth), hemolytic


anemia with splenomegaly, and bone
abnormalities. However, the first clue to the
diagnosis in infancy is generally not one of
these striking cutaneous changes, but rather
the pink or burgundy discoloration of urine
(staining of diapers) due to massive
porphyrinuria (Fig. 21.4).
Diagnosis
Markedly elevated levels of uroporphyrin I and
coproporphyrin I in urine, stool and RBCs.

Fig. 21.1: Congenital erythropoietic porphyria


blistering over the fingers

Fig. 21.2

Differential Diagnosis
Other childhood photosensitivity disorders
Treatment of this disorder is aimed at the
cutaneous photosensitivity or at the anemia
and its complications. Sun protection
(clothing better than sunscreens) and
ingestion of beta carotene may ameliorate some
portion of the photodamage. Other modalities,

Fig. 21.3

Figs 21.2 and 21.3: Congenital erythropoietic porphyriaa child having


photosensitivity and hypertrichosis of face and trunk

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Essentials in Dermatology

Fig. 21.4: Congenital erythropoietic porphyriapink


to red discoloration of urine in the test tube

Fig. 21.5: Porphyria cutanea tarda


darkening of the urine

chronic transfusion regimens and splenectomy, the risks must be weighed against the
severity of the disorder in each individual
case. Bone marrow transplantation successful
in some cases.

Porphyria Cutanea Tarda


PCT was first recognized by Waldenstrom in
the 1930s, who identified a group of patients
with excessive porphyrins in the urine, skin
lesions in light exposed areas and a late
(tarda) onset in adulthood (in contrast with
Gunthers disease), so he called the disease
porphyria cutanea tarda.
Most common type of porphyria due to
deficiency of enzyme coproporphyrinogen
decarboxylase.
Clinical signs include darkening of the urine
(Fig. 21.5) and cutaneous photosensitivity
manifested as fragility and bullae of sun
exposed skin, scarring (Fig. 21.6), hypertrichosis and pigmentary and sclerodermoid
changes. In severe cases, the clinical

Fig. 21.6: Porphyria cutanea tardascarring and


pigmentary changes following photosensitivity

appearance may be similar to that of


congenital erythropoietic porphyria.
Diagnosis
Woods lamp examination of urine shows orangered fluorescence. Total porphyrins and
uroporphyrins are raised in urine and plasma;
coproporphyrins raised in stool; porphobilinogen

Metabolic and Nutritional Disorders


and delta aminolevulinic acid are normal in urine.
Immunofluorescence of skin demonstrates IgG
and other immunoglobulins at dermo-epidermal
junction, and in and around blood vessels.
Differential Diagnosis
Some forms of epidermolysis bullosa acquisita
look similar as does drug induced pseudoporphyria (usually furosemide) in renal dialysis
patients
Avoid hepatotoxic agents. Unlike other
porphyrias, it is usually not inherited and
responds to different treatments (venesection
and antimalarials). If hepatitis C is
documented, treat it with interferon and
ribaverin

PRIMARY CUTANEOUS AMYLOIDOSIS


Primary cutaneous amyloidosis is defined as
cutaneous amyloidosis in the absence of other
systemic or dermatological disease.
The various localized forms of primary
cutaneous amyloidoses include the more

Fig. 21.7: Lichen amyloidosispruritic brownish


lichenoid papules affecting the extensor aspect of
lower leg

common papular (lichen amyloidosis) and


macular types and the rare nodular or
tumefactive form.
Single or multiple nodules involving the trunk
or limbs characterize the nodular form. It is
due to localized plasma cell dyscrasia.
Lichen amyloidosis is the most common form
characterized by numerous pruritic, brownish
lichenoid papules distributed over the
extensor surface of legs (Fig. 21.7) and
forearms and upper back.
Macular amyloidosis typically manifests as
brownish patches with a reticulate or rippled
pattern, involving the upper back (Fig. 21.8),
arms and lower extremities.
In some patients features of both lichen and
macular amyloidosis can coexist and the term
biphasic amyloidosis has been coined for
these. Extensive variants of both macular and
papular amyloidosis have also been
described.
On the other hand, amyloidosis cutis
dyschromica (ACD) is a rare distinct type of
primary cutaneous amyloidosis, which is
characterized by reticulate hyperpigmentation with hypopigmented spots seen
almost all over the body without any
papulation.

Fig. 21.8: Macular amyloidosisrippled


pigmentation over the upper back

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Essentials in Dermatology
Differential Diagnosis
For macular amyloidosis is postinflammatory
hyperpigmentation, atopic dermatitis, lichen
simplex chronicus and fixed drug eruption.
Lichen amyloidosis needs to be differentiated from
lichen planus and lichen simplex chronicus.
Nodular amyloidosis, clinically needs
differentiation from naevus lipomatosus, lipoma,
cysts, lymphoma and histologically from all forms
of amorphous deposits, such as gout and nodular
elastosis.
Treatment is symptomatic with topical
corticosteroids (under occlusion), dimethyl
sulfoxide (DMSO) and sometimes dermabrasion.

XANTHOMATOSES
Xanthomas are composed of masses of lipid
containing histiocytes forming papular, nodular,
and plaque like lesions in the skin, tendons and
sometime in the internal organs. Xanthomas are
important clinical finding as they often evolve in
the presence of elevated blood lipids and
lipoproteins. Lipoproteins are macromolecular
complexes that carry hydrophobic plasma lipids,
particularly cholesterol and triglycerides, in the
plasma. An elevation of serum lipid levels is
called hyperlipidemia, or hyperlipoproteinemia
while the term dyslipoproteinemia signifies

Fig. 21.9: Xanthelasma palpebrarum

abnormalities in serum lipoproteins, whether or


not serum lipid levels are categorically elevated
or not. Of clinical interest is the fact that different
species of lipoproteins typically produce different
types of xanthomas. Thus, the type of xanthoma
observed in a particular patient provides
important clues as to the type of hyperlipoproteinemia.

Xanthelasma Palpebrarum
Soft, velvety papules and plaques arranged
around eyelids.
Common sites upper eyelid, inner canthus
(Figs 21.9 and 21.10)
Signify a systemic hyperlipidemia usually
LDL elevations
Usually occur in normolipidemic individuals
but may be seen in familial hypercholesterolemia, dysbetalipoproteinemia, mixed
hyperlipidemia. Secondary causes are
obstructive liver disease, myxedema, and
diabetes mellitus.
Treatment options includes surgical excision,
electrofulguration, trichloroacetic acid
chemical cautery , and CO2 laser.

Tuberous Xanthomas
Firm, yellow-orange, often with an erythematous halo, small papules (0.5 cm in

Fig. 21.10: Arcus juvenilis

Metabolic and Nutritional Disorders

Fig. 21.11: Tuberous xanthomas over the buttocks

Fig. 21.12: Tendinous xanthoma near the


left 5th toe laterally

diameter) to lobulated tumors (2.5 cm or more)


(Fig. 21.11).
Usually painless but may be tender on
pressure.
Sites knees, elbows, buttocks and pressure
points, typically bilateral.
Seen with raised LDL levels Familial
hypercholesterolemia, dysbetalipoproteinemia and secondary hyperlipidemias
(hypothyroidism, chronic biliary disease)

Tendinous Xanthomas
Slowly enlarging subcutaneous nodules
attached to tendons, ligaments, fascia and
periosteum (sub-periosteal) (Fig. 21.12).
Overlying skin appears normal.
Sites extensor tendons of hands and feet,
Achilles tendon, sub-periosteal bony
prominences such as malleoli and elbows
Occur in severe hypercholesterolemia with
raised LDL Familial hypercholesterolemia,
less frequently in secondary hypercholesterolemia (cholestasis).

Eruptive Exanthomas
Pinhead sized asymptomatic yellow papules
with a reddish base, usually fleeting in nature
and appear in crops.

Fig. 21.13: Eruptive papular and tuberous xanthomas


over the elbows and knees

Sites buttocks, shoulders and extensor


surfaces of extremities (Fig. 21.13).
Occasionally, these papules may coalesce and
overlie a tuberous xanthoma tuberoeruptive
xanthomas.
Associated with pure or mixed hypertriglyceridemia and a high concentration of
VLDL or chylomicrons.

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May occur in secondary hyperlipidemia of
diabetes mellitus.

Plane Xanthomas
Yellow-orange macules or slightly palpable
plaques.
May occur at any site.
Plane xanthomas over palmar creases
xanthoma palmaris et striata pathognomonic of Type III dysbetalipoproteinemia.
Plane xanthomas may occur in secondary
hyperlipidemias (biliary cirrhosis, cholestasis,
gammopathy).

May be associated with myeloma, macroglobulinemia or lymphoma; rarely with


normal plasma lipids.
50% of patients may have hypolipidemia with
low LDL levels.

Intertriginous Xanthomas
They appear as flat to slightly raised yellow
dermal plaques with corrugated surface
within or adjacent to finger webs (Fig. 21.14),
axillae, buttocks and antecubital and popliteal
fossa.
They are pathognomonic of type II hypercholestrolemia.

Generalized Plane Xanthomas


These cover large areas of the face, neck and
thorax and also may involve flexures and
palms.

Fig. 21.14: Intertriginous xanthomas in the finger


web spaces

Table 21.1: Abnormalities of lipoprotein metabolism with their cutaneous features

Lipid phenotype

Lipoprotein phenotype
(Friedrickson)

Isolated Hypercholesterolemia
Familial hypercholesterolemia

IIa

Familial defective apo B100


Polygenic hypercholesterolemia

IIa
IIa

Isolated Hypertriglyceridemia
Familial hypertriglyceridemia

I, V

Familial lipoprotein lipase deficiency


Familial apo CII deficiency

I, V
I, V

Hypertriglyceridemia and Hypercholesterolemia


Combined hyperlipidemia
Dysbetalipoproteinemia

II b
III

Cutaneous features

Tuberous xanthomas, tendon


xanthomas subperiosteal xanthomas
Tendon xanthomas
Usually none
None, occasionally eruptive
xanthomas
Eruptive xanthomas, plaques
Eruptive xanthomas, plaques
Usually none;
Tuberous xanthomas, tuberoeruptive
xanthomas, xanthoma striata palmaris

Metabolic and Nutritional Disorders

Cerebrotendinous Xanthomas
Rare autosomal recessive disorder with
xanthomas in the tendons and the brain.
It is due to mutation in the sterol
27-hydroxylase (CYP27A) gene leading to
defective conversion of cholesterol to bile
acids with accumulation of cholestanol.
Severe neurologic disease and tendency to
coronary artery disease.
Tendon xanthomas.
Urinary gas chromatography is the specific
test for this disease.
Treated with oral deoxycholic acid to replace
the bile acid pool.

Fig. 21.15: Lipoid proteinosis infiltrated lip and


buccal mucosa

LIPOID PROTEINOSIS (URBACH


WIETHE DISEASE, HYALINOSIS CUTIS
ET MUCOSAE)
Lipoid proteinosis is a rare disorder
characterised by infiltration of hyaline
material into the skin, oral cavity and internal
organs.
It is inherited as a monogenetic autosomal
recessive disorder of normal chromosomal
pattern and is due to mutations in the extracellular matrix protein 1 gene.
The earliest manifestation is hoarseness that
develops in infancy, which can progress to
complete aphonia without breathing
difficulty. Examination of larynx frequently
reveals infiltration, thickening and nodularity
of cords. The mucosa of lip, pharynx and
tongue (Fig. 21.15) may also develop firm
yellow nodules giving it a cobblestone
appearance. The tongue is enlarged and firm
on palpation with decreased range of
movements. Ankyloglossia can occur due to
involvement of the lingual frenulum.
Recurrent parotitis can occur.
In early stages, the skin lesions are crops
of bullae, pustule, that leave behind acne
like scars. Later on, patients develop hyperkeratotic wart like or nodular skin lesions over

Fig. 21.16: Lipoid proteinosisbeaded eyelid margins

dorsal aspect of hands, finger, elbows and


knees, and patchy alopecia.
Deposition of hyaline material along the eyelid
margins results in typical beaded papules
moniliform blepharosis. This eyelid beading
is the single most typical clinical feature of
this disease (Fig. 21.16). In later stages, there
may be total loss of eyelashes. Further
infiltration of eyelids leads to its malfunction
and can cause corneal ulcer.
Neurological abnormalities include calcification in the hippocampus and falx cerebri
and temporal lobe epilepsy.

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Drusen of Bruchs membrane seen in fundi in
half the patients.
Sickle-shaped calcifications dorsal and lateral
to sella turcica in skull X-ray films are
pathognomonic.
The nature of hyaline material and underlying
metabolic defect is unknown. It may represent
an underlying lysosomal storage disorder
with single or multiple enzyme defects, others
have postulated it to be a disorder of collagen
metabolism or collagen synthesis.
Distinctive histologic features include extreme
dilation of blood vessels and thickening of
their walls, progressive hyalinization of sweat
glands, and infiltration of the dermis and
subcutaneous tissue with extracelluar hyaline
deposits and also demonstrable in walls of
vessels.
Differentiation from erythropoietic protoporphyria may be difficult histologically.
Xanthomatoses and amyloidosis can be
excluded histologically. In adults, differential
diagnosis is from lichen myxoedematosus and
myxedema with hoarseness have to be
considered.
There is presently no effective therapy for
lipoid proteinosis. Hoarseness may be
relieved temporarily by surgical removal of
vocal cord infiltrates. Facial lesions may be
treated by dermabrasion, chemical skin
peeling and blepharoplasty. Treatment by oral
dimethyl sulphoxide has been claimed to be
successful.

PHRYNODERMA
Phrynoderma is a type of follicular hyperkeratosis typically seen in vitamin A
deficiency.
This eruption has also been associated with
deficiencies of vitamin B complex, C, and E,
calories, and essential fatty acids.
The morphology of these lesions is variable
and may range from filiform papules to small

Fig. 21.17: Phrynoderma hyper keratotic follicular


papules over the extensor aspect of the forearm

conical papules to large papules with large


horny centers. They may be of the same color
as the surrounding skin or may be slightly
hyperpigmented. Elbows (Fig. 21.17), knees,
anterolateral thighs, posterolateral superior
forearms, extensor aspect of limbs, shoulders,
abdomen, back and buttocks are the sites of
involvement.
Differential diagnosis includes keratosis
pilaris and Dariers disease.
The treatment is oral vitamin A 100,000 IU/
day for 2-3 days followed by the recommended
dietary requirements.

PELLAGRA
Pellagra is a nutritional disorder that occurs
due to deficiency of niacin or tryptophan or
both. Therapy with isoniazid, 5-fluorouracil,
6-mercaptopurine may provoke pellagra. Rare
causes are carcinoid tumors, and Hartnup
disease.
The term pellagra is derived from the Italian
words pelle agra meaning rough skin.
It is still endemic in areas of Africa and Asia
due to poor nutrition and intake of certain
cereals such as maize and jowar (Indian
millet) as staple diet.

Metabolic and Nutritional Disorders


In the present day context, in western world,
pellagra is confined to individuals who have
improper food intake such as psychiatry
patients, alcoholics and recluses.
Pellagra is a clinical syndrome characterised
by (1) symmetrical photosensitive skin
eruption (2) gastrointestinal manifestations
(3) neurological and psychiatric disturbances.
These well-known group of symptoms are
traditionally remembered as pellagras four
Ds -dermatitis - diarrhea - dementia and
when untreated, death - is very seldom seen.
But most of the manifestations are borderline
and/ or less typical in nature.
The initial manifestation is an erythematous,
photosensitive pruritic rash that occurs on the
dorsa of hands.
The usual sites affected are the face, neck and
dorsal surfaces of hands, arms and feet.
The dorsa of the hands are the most frequent
site from where it may extend up to arm to

Fig. 21.18

form the glove or gauntlet of pellagra (Figs


21.18 and 21.19). The dermatosis is strikingly
symmetrical and clearly demarcated from the
normal skin.
The feet is commonly involved and it may also
affect the front and back of the leg to form a
boot (Fig. 21.20).
In the face, an erythematous rash extending
from the nose to the cheeks, chins, lips, may
resemble lupus erythematosus Butterfly
rash. Rarely eyelids and ears may be affected.
Facial rash usually occurs concurrently with
lesions elsewhere.
This eruption forms a broadband or collar
around the neck, known as Casals necklace
(Fig. 21.19). In many instances, the necklace
has an anterior continuation, also known as
`cravat.
Differential diagnosis includes drug eruption,
photodermatitis, lupus erythematosus, and
actinic reticuloid.
Classical pellagra responds dramatically to
oral administration of nicotinamide or niacin
(nicotinic acid) 100-300 mg/day in three
divided doses. The mental changes disappear
within 24-48 hours but skin lesions may take
3-4 weeks to disappear.

Fig. 21.19

Figs 21.18 and 21.19: Pellagrachest and upper limb shows Casals necklace, and glove of hyperpigmented flaking dermatosis

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Fig. 21.21: Ariboflavinosiserythema and scaling of


nasolabial folds

Fig. 21.20: Pellagraboot of pellagrus


dermatosis affecting lower legs

ARIBOFLAVINOSIS
(ORO-OCULO-GENITAL SYNDROME)
This syndrome occurs due to vitamin B2
(Riboflavin) deficiency and is characterized
by mucocutaneous lesions of angular
stomatitis, cheilosis, glossitis, seborrhoeic
dermatitis (especially around the nose) (Fig.
21.21), scrotal (Fig. 21.22) and vulvar
dermatitis, and increased corneal vascularity.
It usually responds promptly and
dramatically to riboflavin supplementation
with 5 to 15 mg of riboflavin daily for 2 weeks
and correction of dietary errors.

ACRODERMATITIS ENTEROPATHICA
Acrodermatitis enteropathica, an autosomal
recessive disorder which appears to be due
to defective absorption of zinc from the
gastrointestinal tract.
It may be a presenting sign in cystic fibrosis or
AIDS.

Fig. 21.22: Ariboflavinosisscrotal dermatitis

It manifests insidiously between the ages of


three weeks and 18 months (often when the
baby is switched from breast milk to cows
milk) with periorificial (mouth, nose, eyes, ears,
and perineum) (Figs 21.23 to 21.25) and acral
(extensor surfaces of the major joints, fingers,
and toes) dermatitis, alopecia and diarrhea
(Mnemonic DAD to remember its clinical
features).

Metabolic and Nutritional Disorders

Fig. 21.23: Acrodermatitis enteropathica


perioral dermatitis

The primary cutaneous eruption is


vesicobullous, which is symmetrical and
grouped. The lesions soon evolve into erosive
vesicobullous eruption or psoriasiform
patches. Secondary infection of skin lesions
with Staphylococcus aureus is common and
impairs wound healing.
At the same time or shortly afterward, diffuse
loss of hair and gastrointestinal disturbances
manifest chiefly by diarrhea, occur.
Mental depression, listlessness, loss of
appetite, perleche, photophobia, and
blepharitis may occur during exacerbations.
Differential diagnosis includes atopic
dermatitis, seborrheic dermatitis and
psoriasis.
Laboratory verification of deficient plasma
or serum zinc levels may be undertaken
where facilities exist, otherwise, all these
cases respond to zinc sulphate / gluconate
(1 to 2 mg/kg body weight/day) given once

Fig. 21.24

Fig. 21.25
Figs 21.24 and 21.25: Acrodermatitis
enteropathicaperianal psoriasiform dermatosis

or twice daily. Skin lesions heal within one


to two weeks. Diarrhea ceases and variability
with depression of mood improves within 24
hours.

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22

Genetics and Genodermatoses

GENETICS IN DERMATOLOGY
Human genetics is the study of the range of
biological variations in human beings with each
individual showing considerable variability in
his/her expression of disease.
Human genome, the genetic material is
packaged into units called chromosomes
(Chromo-colored, somes-bodies). Each somatic
cell contains 46 including 2 sex chromosomes.
The karyotype of an individual identifies the
number and structure of chromosomes. This is
usually derived from peripheral blood cells
(T lymphocytes) that are stimulated to divide. They
are classified by the position of the centromeres
and the proportion of long and short arms.
Phenotype is the bodily manifestation of
genotype.

Congenital is one that is present at/before birth,


not necessarily genetically determined.

Genetic Principles
Gene is the sequence of bases on DNA that code
for one polypeptide.
Locus is the precise position of the gene on
chromosome.
Alleles are genes at a single locus, which may be
heterozygous or homozygous.
Dominant means full effect in heterozygous state.
Expression means effects of a gene are variable.
Penetrance is the frequency with which a gene
produces an effect. If trait is non-penetrant,
expressivity is zero.

Genodermatoses are genetically determined skin


disorders, course of events altered little by
environmental agents with single gene disorders.

Genetic heterogeneity means similar/identical


phenotypic features associated with genes at
different loci, e.g. albinism, ichthyosis, cutis laxa,
etc.

Familial means a condition more common in


relatives of an affected individual than in general
population.

Phenocopy means environmental causes


mimicking genetic diseases.

Inherited means that disorder is transmitted from


one generation to next.

Pleiotropy means multiple phenotypic effects due


to primary action of an abnormal genotype, e.g.
pachyonychia congenita, neurofibromatosis.

Genetics and Genodermatoses


Mutation occurs due to point substitution or
deletion and may be somatic or gamete mutation.
Mosaicism relates to an individual with 2 or more
cell lines of different genotypes derived from a
single zygote.

Autosomal Dominant Inheritance


In this mode of inheritance, the gene locus is on
an autosome and the trait is transmitted from
generation to generation.
Both males and females are affected in equal
proportions
Affected individuals are heterozygous
Every affected person will have affected parent
50% of the children will be affected
Severity varies considerably in the family
Few show lack of penetrance.
Examples: Ichthyosis, tuberous sclerosis,
neurofibromatosis.
Autosomal Recessive Inheritance
In these pedigrees, the trait does not appear in
successive generations. Parents appear entirely
normal.
Both males and females are affected with equal
frequency
Affected individuals are homozygous (means
one copy received from each parent double
dose of the gene)
One fourth or 25% of children are affected
No family history may be there
High frequency of consanguinity can be
expected
Disorders are very severe.
Examples: Xeroderma pigmentosum, phenylketonuria, etc.
X-linked Recessive Inheritance
In these conditions, the trait does not appear in
successive generations.
Males are uniquely affected, whereas female
siblings are carriers of the trait. Affected males
are in different generations

Females are healthy carriers, pass on disease


to of their sons and of their daughters are
carriers
All of the daughters of an affected male are
obligate carriers of the trait
Mothers brothers are affected
Family history always not positive.
Examples: Fabrys disease, Menkes-Kinkys
disease.

X-linked Dominant Inheritance


Successive generations show the trait.
Females are predominantly affected
There is family history of recurrent losses of
pregnancy early on. Although the losses may
not be identified, it is often the case that the
male fetus is preferentially aborted.
Hemizygous are males and heterozygous
females. In most X-linked dominant traits the
hemizygous male is more severely affected
than the heterozygous affected female
Affected males transmit disorder to all his
daughters, but none of his sons
Affected females transmit disease to of their
sons and of their daughters.
Example: Incontinentia pigmenti.
Multifactorial Traits
In single gene traits, transmission follows
Mendels classical laws. In multifactorial
inheritance, often referred to as polygenic
inheritance, a definite familial tendency for
development of the trait exists but the proportion
of affected relatives is much less than expected
for a single gene trait. Multiple genes modified by
environmental factors influence the expression
of the characteristic.
Genetic Counseling
Genetic counseling is the process undertaken to
help patients to better understand the genetic
basis of a medical problem and to plan for the
future. By this definition, genetic counseling

211

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Essentials in Dermatology
includes helping the patients to comprehend the
medical facts, including the diagnosis, the
probable course of the disorder and available
management, the hereditary basis of the disorder,
and the risk of recurrence in specific relatives. The
counseling also includes a discussion of the course
of action that seems appropriate in view of the
risk and the family goals.

Symbols in Common use in Preparation


of a Family Pedigree

PHAKOMATOSES (NEUROCUTANEOUS
SYNDROMES)
Phakomatoses or neurocutaneous syndromes are
a rare group of disorders including neurofibromatosis, tuberous sclerosis, von HippelLindau syndrome, and Sturge-Weber syndrome.

Neurofibromatosis Type 1 (NF-1)


Neurofibromatosis type1 (NF-1), also known
as von Recklinghausens disease, classic
neurofibromatosis, or peripheral neurofibromatosis is the most common form of
neurofibromatosis.
The NF1 gene responsible codes for
neurofibromin on chromosome 17.
The clinical expression of NF-1 is highly
variable. Roughly, half of NF-1 cases arise via
spontaneous mutation and are not associated
with any family history.
NF-1 is a multisystem disorder characterized
by cafe-au-lait macules (CALMs), neurofibromas, Lisch nodules, optic gliomas, bony
dysplasia, intertriginous freckling and
autosomal dominant inheritance.
Neurofibromatosis is named after
neurofibroma tumors. There are three types
of neurofibromas: cutaneous, subcutaneous
and plexiform. A single plexiform
neurofibroma or two of any type are
considered diagnostic of NF-1. Cutaneous
neurofibromas (mollusca fibrosa) are soft
lilac pink tumors, sessile and dome shaped,
sometimes pedunculated, most numerous on
the trunk and limbs, hundreds may be
present, ranging from a few millimeters to
several centimeters in diameter. They can be
easily pushed into underlying dermal defect
with light digital pressure - button holing.
When pressed, the soft tumors tend to
invaginate through a small opening in the
subcutaneous tissue, giving the feeling of a
seedless raisin or a scrotum without a testicle
(Fig. 22.1). The subcutaneous type may feel
hard like a pencil eraser. They often cause
localised pain or tenderness. On the other
hand, plexiform neurofibromas are
congenital and are pathognomonic for NF-1
(Figs 22.2 and 22.3). They feel like a bag of
worms because of the many interdigitating

Genetics and Genodermatoses


elements and can be thought of as combination
of cutaneous and subcutaneous types. They
present as a diffuse elongated fibroma along
the course of the nerve, frequently involving
the trigeminal or upper cervical nerves.

Fig. 22.3: Neurofibromatosis type 1plexiform


neurofibroma affecting foot

Fig. 22.1: Neurofibromatosis type 1 multiple


neurofibromas over the trunk

Fig. 22.2: Neurofibromatosis type 1 plexiform


neurofibromas affecting both lower limbs

Neurofibromas of the female areola and


nipple are virtually pathognomonic for NF-1.
Cafe-au-lait macules (CALMs), another
manifestation of NF-1, are discrete, well
circumscribed, round or oval, uniformly
pigmented patches (may be seen in normal
population, segmental neurofibromatosis,
familial CALMs, tuberous sclerosis, Mc Cune
Albright syndrome, ataxia telangiectasia,
Blooms syndrome, Watsons syndrome,
Rubinstein-Taybi syndrome) (Figs 22.4 and
22.5). Solitary CALMs are a common finding,
affecting up to one third of normal children.
Multiple CALMs are rare, particularly in the
white population. A diagnosis of multiple
organ disorder, the most common being NF-1,
should be considered in this instance. The
minimal number of 6 lesions of CALMs was
established as a criterion for the diagnosis of
NF-1 (more than 1.5 cm in diameter in adults).
They are the first feature of the disease to
appear in all children.
Freckling is a useful and often overlooked
sign of NF-1. Freckling involving areas of
hyperpigmentation up to 2 or 3 mm in
diameter occurs frequently in the axilla as well
as other intertriginous regions. Basically,
there are two kinds of neurofibromatosis
frecklesthose that are basically very small
CALMs ordinarily present at birth or in the

213

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Essentials in Dermatology
Systemic features include:
1. Skeletal manifestations include bony
dysplasias especially tibia, pseudoarthrosis, cysts and scoliosis of thoracic
region.
2. CNS manifestationsApproximately 50%
of children with NF-1 exhibit a learning

Fig. 22.4: Neurofibromatosis type 1 circumscribed


pigmented patch of caf-au-lait macule over the trunk

Fig. 22.5: Neurofibromatosis type 1 back


showing neurofibromas and caf-au-lait macules

first year of life and distributed over the entire


body and those that develop later in
intertriginous regions (Crowes sign) (Fig.
22.6). Multiple melanotic macules of palms
with varying size from 2 to 4 mm may be noted
in 90% of Indian cases (Fig. 22.7). This sign
has been named as Patrick Yesudian sign
by various medical schools, in south India.
Lisch nodules are pigmented iris hamartomas
which appear as dome shaped lesions on the
surface of iris. They are one of the most
common manifestations of NF-1 and have
significant diagnostic value.

Fig. 22.6: Neurofibromatosis type 1axillary


freckling

Fig. 22.7: Neurofibromatosis type 1palmar


freckling Patrick Yesudian sign

Genetics and Genodermatoses


disability, as well as attention deficit
disorder. More severe developmental delay,
including mental retardation occurs in
about 5%. Other features of NF-1 include
macrocephaly, short stature, and
hypertension (due to renal artery stenosis
or rarely pheochromocytoma).
3. Endocrine disturbances of many types
may be associated.
Diagnosis: The spectrum of clinical findings
in NF-1 is broad, but only six features constitute
the seven established diagnostic criteria.
These criteria were described at a National
Institutes of Health (NIH)sponsored
consensus conference in 1987. These were
updated in 1990. In the absence of a single
test that can confirm the diagnosis,
physicians often rely on these criteria. Two
criteria are required for a definitive diagnosis
and one is required for a presumptive
diagnosis.
1. Six or more cafe-au-lait macules (CALMs)
measuring 5 mm or more before puberty or
15 mm or more after puberty;
2. Axillary or inguinal freckling (Crowes
sign),
3. Two or more neurofibromas or a plexiform
neurofibroma;
4. Two or more Lisch nodules;
5. Optic nerve glioma;
6. Characteristic skeletal dysplasias (tibial or
orbital dysplasia) and
7. Affected first degree relative.
Three of these criteria manifest themselves on
to the skin (A single plexiform neurofibroma
or two of any type, minimal number of
6 lesions of CALMs, and axillary frecklingCrowes sign). It is often the dermatologist who
suggests or confirms the diagnosis based upon
cutaneous manifestations.
Management consists of genetic counseling,
anticipatory guidance, and surveillance for
complications. Tests such as MRI or X-rays

should be done for clinical indications only.


Children should be monitored for learning
disabilities and provided cognitive and
educational assessments as needed. Physical
examinations should include measurement of
height, weight, head circumference, and blood
pressure. Ophthalmologic check up is a must
in all cases. Surgery is indicated in only select
cases.
Risk of malignancy: NF-1 is also associated
with increased risk of malignancy (malignant
peripheral nerve sheath tumors in second or
third decades).
Prognosis: Variable, dependent upon severity
of involvement and development of
malignancy. Cosmetic disfigurement is
progressive and worsens with time. Mild
course during childhood is not a guarantee
for mild disease in adulthood.

Tuberous Sclerosis (Bournevilles


Disease)
It is now frequently designated the tuberous
sclerosis complex (TSC). It is an autosomal
dominant human genetic disease characterized by widespread hamartomas, usually,
occurring in the brain, eyes, skin, kidneys,
liver, heart, and lungs.
This disorder derives its name from a
description of its cerebral lesions by
Bourneville in 1880. Its systemic nature was
described by Vogt in the clinical triad (Epiloi-a) of epilepsy (seizures),low intelligence
(mental retardation) and facial lesions termed
adenoma sebaceum (a misnomer for
angiofibromas). Recent studies have shown
that full triad was evident in only about one
third of patients.
Two third cases are sporadic and one third
are autosomal dominant.
Two genetic loci have been identified TSC1
(Hamartin) and TSC2 (Tuberin) on
chromosome 9 and 16 respectively. These are

215

Essentials in Dermatology

216

tumor suppressor genes, which when


deficient result in Mtor disinhibition and
abnormal proliferation of tissues resulting in
hamartomas.
Cutaneous features are the most frequent
finding in TSC and if overlooked, will lead to
a delay in diagnosis. Although, there is
considerable variation in the age of expression
of all the skin lesions, there is a trend towards
the earlier expression of hypomelanotic
macules and forehead plaques compared
with facial angiofibromas and ungulal
fibromas. Shagreen patches are usually
present by puberty.
The relatively vascular and fibrous
components of adenoma sebaceum
(angiofibromas) determines their clinical
appearance (Fig. 22.8). They range from white
or flesh colored to classical red pink papules,
1-10 mm in diameter, symmetrically
distributed over the nasolabial folds, cheeks
and chin, sparing the upper lips. They are
regarded by many as a primary pathognomonic feature of TSC.
Shagreen patches are not as diagnostically
useful as facial angiofibromas. They most
often appear as flat, slightly elevated areas of
the skin, soft and skin colored plaques of
variable size (1 to 10 cm) with a pig skin,
elephant skin or orange peel appearance.
Usually, these lesions are localized
asymmetrically over the dorsal body surfaces,
particularly over the lumbosacral area
(Fig. 22.9).
Periungual fibromas (Koenens tumors)
common in adult patients with TSC, are much
less frequent in children. They usually appear
around puberty as smooth, firm, flesh colored
excrescences and are usually 5-10 mm in
length. They are located around or under the
nail plate and arise from the bed under the
nail plate or from skin of nail groove (Fig.
22.10). Regarded as angiofibromas, they are

classified as primary or pathognomonic


feature of TSC.
Ash leaf macules/spots (hypomelanotic
macules) are the most frequent lesions in TSC
patients. Since, they resemble the leaf of
European mountain ash tree, they are called
as ash leaf spots. They are ovoid or leaf shaped
white macules varying in size from 1-3 cm (Fig.

Fig. 22.8: Tuberous sclerosis angiofibromas and


forehead plaque seen over the face

Fig. 22.9: Tuberous sclerosis orange peel


appearance of a circumscribed area in the
lumbosacral area Shagreen patch

Genetics and Genodermatoses

Fig. 22.10: Tuberous sclerosis periungual


fibromas, also known as Koenens tumors

22.8) and may be the only skin lesions in


infants and if associated with infantile
spasms, strongly suggest the diagnosis of TSC.
These ash leaf spots are predominantly
distributed on the trunk and buttocks or limbs.
Forehead fibrous plaque is classified
histologically as an angiofibroma, although
clinically it differs from the typical
papulonodular angiofibroma because of
fibromatous appearance (Fig. 22.5). It is one of
the secondary features of tuberous sclerosis.
Molluscum fibrosum pendulum (skin tags)
are commonly seen in normal elderly people
and are uncommon in adolescents and young
adults, and should alert to the possibility of
TSC. In individuals with TSC, molluscum
fibrosum pendulum is commonly seen on the
neck, groin, axillae and near flexures of limbs,
especially in adults.
Caf-au-lait macules are not regarded as
characteristic for TSC and are not included in
the diagnostic criteria (Fig. 22.8).
Systemic features include:
1. Neurological findings: Tuberosclerotic
nodules of glial proliferation occur in
cerebral cortex, basal ganglia, and
ventricle wall (60-70%) and are causes of
mental retardation. Once calcified, these
lesions are visible on skull radiographs as

brain stones. Epilepsy is common, usually


begins in infancy as infantile spasms or
salam attacks. Autism, attention deficit
disorder are other features.
2. Ocular: Retinal phakomas (lump of
mulberry appearance) represent proliferation of astrocytes. Hypopigmented
spots in iris may be seen.
3. Cardiac: Rhabdomyomas may be present
at birth and regress spontaneously in first
few years.
4. Renal: Polycystic kidney disease, isolated
renal cyst, angiomyolipomas, renal cell
carcinoma.
5. Pulmonary: Multifocal micronodular
pneumocyte hyperplasia, pulmonary cyst,
lymphangiomyomatosis.
6. Gastrointestinal: Hamartoma and
polyposis of stomach, intestine and colon.
Investigations include:
X-ray (skull, hands and feet, lungs);
CT scan brain;
MRI (brain parenchymatous lesions);
EEG (seizures);
Renal ultrasonogram;
ECHO heart (rhabdomyomas);
Neurodevelopmental testing;
Opthalmological examination.
Diagnositic Criteria (Tuberous Sclerosis
Alliance, Consensus Conference 1998)
Major features
1. Facial angiofibromas or forehead plaque
2. Non-traumatic ungual or periungual
fibroma
3. Hypomelanotic macules (more than three)
4. Shagreen patch (connective tissue nevus)
5. Multiple retinal nodular hamartomas
6. Cortical tuber
7. Subependymal nodule
8. Subependymal giant cell astrocytoma,
9. Cardiac rhabdomyoma, single or multiple
10. Lymphangiomyomatosis
11. Renal angiomyolipoma.

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Minor features:
1. Multiple randomly distributed pits in dental
enamel
2. Hamartomatous rectal polyps
3. Bone cysts
4. Cerebral white matter migration lines
5. Gingival fibromas
6. Non-renal hamartoma
7. Retinal achromic patch
8. Confetti skin lesions
9. Multiple renal cysts.
Definite TSC: Either 2 major features, or 1 major
feature with 2 minor features.
Probable TSC: 1 major feature and 1 minor feature.
Possible TSC: Either 1 major feature, or 2 or more
minor features.
Treatment:
1. Facial angiofibromas: Dermabrasion,
electrodessication, CO2, argon or pulsed
dye laser
2. Seizures: For infantile spasms: ACTH/
steroids are useful. Seizures in children:
vigabatrin is the drug of choice.
Prognosis is related to the extent of systemic
involvement. Cardiovascular complications
occur in 1st decade, brain tumor in 2nd
decade, renal and pulmonary lymphangiomatosis in 4th decade are cause of morbidity
and mortality.

Von Hippel-Lindau Disease (VHL)


VHL also referred to as CNS angiomatosis is
inherited in an autosomal dominant fashion
with incomplete penetrance with VHL gene
located on chromosome 3p25. Both sexes are
equally affected. Hippel first described retinal
angiomatosis in 1904. Lindau subsequently
recognized association with central nervous
system tumors and hence the eponym von
Hippel Lindau disease was coined.

Cutaneous findings are portwine stains and


caf-au-lait macules. Dermal capillary
malformation has predilection for the head
and neck. Other manifestations are vascular
malformations in the cerebellum and brain
stem. Retina is also commonly affected. There
may be cystic neoplasms or angiomatous
lesions in the kidneys, liver and pancreas.

Sturge-Weber Syndrome
(Encephalofacial angiomatosis)
Sturge -Weber syndrome (SWS) is defined as
facial portwine stain in association with
ipsilateral plial (i.e. leptomeningeal) vascular
anomalies (with one or more symptoms;
epilepsy early in life, hemiparesis or
hemiplegia, gyriform intracranial calcifications and cerebral atrophy) and inconstant
ipsilateral choroidal vascular lesions with
glaucoma.
Portwine stain is usually unilateral, roughly
involving the areas supplied by ophthalmic
and maxillary divisions of trigeminal nerve,
and bilateral in 50% of cases.
Portwine stains (naevus flammeus) are
congenital vascular birthmarks that are
present at birth and persist into adulthood.
At birth, they are often pale pink macular
lesions which with time, progress to become
dark red to purple (Fig. 22.11) and even
nodular. These changes occur as a result of
progressive ectasia of cutaneous superficial
vascular plexus.
Portwine stains can either occur as isolated
cutaneous or be associated with structural
abnormalities especially of those underlying
the birth mark such as the choroidal vessels
in the eye which produce glaucoma and
leptomeningeal vessels in the brain which
causes seizures, then is known as SWS.

Genetics and Genodermatoses

Fig. 22.11: Sturge Weber syndromeport wine nevus


seen along ophthalmic and maxillary division of
trigeminal nerve

Freckles appear over face and hands, and later


on neck, legs, lips and conjunctiva. Eventually
freckles become permanent and progressively
increase in number.
Continued sun exposure causes skin to
become dry and parchment-like with
pigmentation (hence the name xeroderma
pigmentosum).
Next is the poikilodermatous stage
characterized with atrophy and telangiectasia
superadded to the existing freckles and
hyperpigmentation (Fig. 22.12).
Superficial ulcers and atrophy may leave scars
and contractures.
Ocular features include photophobia with
conjunctival injection, symblepharon,
ectropion / entropion and loss of eye lashes
due to atrophy of eyelid skin, keratitis leading
to corneal opacity, pterygium and ocular
neoplasms.

Xeroderma Pigmentosum
(Pigmented Dry Skin)
It is an autosomal recessive disorder characterized
by photosensitivity, pigmentary changes,
premature skin aging, neoplasia and abnormal
DNA repair.
Eight different subtypes complementation
groups A to G and XP variants.
Main defect is in the DNA excision repair
process (this is a process whereby damaged
DNA is replaced with new DNA).
XP- variants have a normal nucleotide
excision repair but the defect here is of a
reduced molecular weight of newly
synthesized DNA in UV radiated cells.
Clinical Features
Skin normal at birth.
Earliest symptoms of dryness and freckling
appear between six months and three years of
age.

Fig. 22.12: Xeroderma pigmentosumfreckle like


pigmentation with atrophy, telangiectasia and actinic
keratoses

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Essentials in Dermatology

Fig. 22.13: Xeroderma pigmentosumfreckle like


pigmentation with fungating squamous cell tumor of
the face and scalp

Pre-malignant lesions like actinic keratosis


and keratoacanthomas may occur in most
cases.
Patients with XP under 20 years of age have
a greater than 1000-fold increased risk of
cutaneous basal cell or squamous cell
carcinoma or melanoma (Fig. 22.13). The
median age of onset of nonmelanoma skin
cancer reported in patients with XP is
8 years.
Overall, there is a ten to twenty-fold increase
in internal neoplasms (central nervous system,
lung, gastric, breast, renal, etc.) in XP.
20% have neurological complaints mental
retardation, areflexia, spasticity, ataxia, and
sensorineural deafness.
Disease is often fatal under 10 years of age
and two thirds of the cases die by 20 years of
age.
De Sanctis Cacchione syndrome consists of
xeroderma pigmentosum, with microcephaly,

mental deficiency, dwarfism, hypogonadism,


choreoathetosis, and ataxia.
Prenatal diagnosis by amniocentesis is
possible.
Differential diagnosis includes ordinary
freckling, other causes of photosensitivity and
premature ageing syndromes such as
Rothmund-Thomson syndrome, Bloom
syndrome, Cockayne syndrome, progeria, and
acrogeria.
Treatment: The mainstay of management is
by ensuring maximum photoprotection
(clothing, topical and systemic sunscreens, UV
filter glasses). Ocular symptoms should be
managed promptly to prevent complications.
Early and adequate excision of all tumors is
essential and topical 5-fluorouracil may be
used for pre-malignant lesions. Oral retinoids
have been found to decrease the occurrence of
skin cancers in this condition.

Incontinentia Pigmenti
(Bloch-Sulzberger Disease)
Incontinentia pigmenti is an uncommon
genodermatosis of the developing
neuroectoderm in which vesicular, verrucous
and pigmented lesions are associated with
developmental defects of eye, skeletal
system and central nervous system.
Incontinentia pigmenti is a complex
hereditary syndrome that principally affects
female infants. It is inherited as an X-linked
dominant disorder, caused by mutation of
NEMO gene on chromosome Xq28. The gene
is generally lethal in male fetuses.
This multisystem disorder has manifestations
of dermatological, neurological, skeletal,
ocular or dental origin.
It manifests at birth or during first weeks of
life.
In the skin, the disorder characteristically
progresses through four stages. The first stage

Genetics and Genodermatoses

Fig. 22.14: Incontinentia pigmenti linear whorled


vesicular eruption along Blashkos lines over the
lower limbs

(vesicular phase) is characterized by linear


whorled vesicular eruption along Blaschkos
lines (Fig. 22.14). The eruption typically favors
acral locations. Peripheral leucocytosis and

eosinophilia may occur during this stage. This


stage is followed 2 to 6 weeks later by
verrucous or lichenoid lesions on the sites of
the former vesicular eruption in 30% of
patients. The third stage (pigmentary phase)
starts between 12th and 20th week and is
characterized by hyperpigmented lesions. A
fourth stage, rarely seen in some adult females
is characterized by faint hypochromic or
atrophic linear macules over extremities. The
hair is usually normal, but in 25% of cases,
cicatricial alopecia may be seen.
Incontinentia pigmenti achromians
(hypomelanosis of Ito) suggest the negative
image of incontinentia pigmenti characterized
by unilateral or bilateral hypopigmentation
along the lines of Blaschko.
Usually, no treatment is necessary other than
the control of secondary infection.

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23

Skin in Systemic Diseases

Hormones secreted by endocrine glands have


physiological effects on pigmentation, hair
growth, sebaceous glands and connective tissue.
Endocrine disorders are associated with various
cutaneous manifestations which offer clue to the
diagnosis of the underlying endocrine disorder.

sarcoidosis, or iatrogenic) leading to multiple


hormone deficiency. Cutaneous manifestations
are non-specific and include xerotic skin, fine
wrinkling around eyes and mouth, pallor,
generalized hypopigmentation, increased sunburn tendency, thinning of scalp and body hair,
reduced sweat and sebaceous gland activity and
thin brittle nails.

SKIN IN PITUITARY DISORDERS

SKIN IN ADRENAL SYNDROMES

Acromegaly: It results from excessive secretion


of growth hormone by pituitary adenomas (98%
of cases) in adolescents or adults. Patients are
tall and have prognathism, frontal bossing,
elongated, blunt and thickened fingers.
Dermatological features include thickening of
skin, accentuation of facial, neck and scalp
creases imparting corrugated appearance (cutis
verticis gyrata), widened triangular shaped nose,
large ears, large and protruding lower lip,
edematous thick eyelids, macroglossia,
numerous skin tags (fibroma molluscum),
seborrhea,
acne,
hyperhidrosis,
hyperpigmentation, acanthosis nigricans, coarse
scalp and body hair, wide and thickened nails.

Cushings syndrome and Cushings disease:


Cushings disease refers to hypercortisolism
(glucocorticoid excess) from ACTH overproduction, most commonly by pituitary
adenoma, whereas Cushings syndrome refers
to hypercortisolism, resulting from any cause,
such as pituitary ACTH overproduction, ectopic
ACTH production, or from adrenal adenoma,
carcinoma or hyperplasia, or from glucocorticoid
therapy. Skin is atrophic, smooth and
transparent (paper thin), and there is facial
fullness and plethora (moon facies), buffalo
hump (Fig. 23.1), fullness of supraclavicular
fossa, truncal obesity and relative lack of fat in
lower extremities lemon on stick appearance,
poor wound healing, striae particularly over
abdomen, arms, and thighs, purpura, petechiae,
easy bruising and purplish mottling (cutis
marmorata), acneiform eruptions, hirsutism,

SKIN IN ENDOCRINE DISORDERS

Panhypopituitarism: It results from destruction


of pituitary gland from various causes (post
partum hemorrhage Sheehans syndrome,
adenoma, craniopharyngioma, tuberculosis,

Skin in Systemic Diseases

Fig. 23.1: Cushings syndromebuffalo hump

Fig. 23.2: Addisons diseasehyperpigmentation of


palmar creases

male pattern baldness in females, addisonian like


pigmentation, and increased incidence of fungal
infections.

Pheochromocytoma: It is a rare adrenal tumor


(mostly benign). Main clinical feature is
hypertension, but can lead to flushing episodes.

Addisons disease (primary adrenal insufficiency): It refers to insufficient secretion of


adrenocortical hormones mainly cortisol and
mineralocorticoids due to destruction of adrenal
glands. Various causes of adrenal gland damage
are autoimmune disorders in 70% of cases,
tuberculosis, fungal infections, sarcoidosis,
metastasis, and hemorrhage. Secondary adrenal
insufficiency results from hypothalamic or
pituitary diseases leading to decreased ACTH
secretion, or steroid withdrawal after long term
use.
General features of Addisons disease are
fatigue, dizziness, anorexia, nausea, vomiting,
diarrhea, abdominal pain and hypotension.
Cutaneous findings are hyperpigmentation of
skin due to elevated ACTH and MSH, mainly
over light exposed areas, elbow, knee, knuckles,
axillae, areolae, umbilicus, genitalia, palmar
creases (Fig. 23.2), tongue and mucous
membranes, scars, and nails. Fibrosis and
calcification of ear may occur. Hyperpigmentation is absent in secondary adrenal
insufficiency (referred to as white Addisons
disease).

SKIN IN THYROID DISEASES


Hyperthyroidism
It may be a manifestation of Graves disease
(most common cause), toxic multinodular goiter,
adenoma, Hashimotos thyroiditis (early stage)
and iatrogenic.
Graves disease: It is an autoimmune disease
characterized by antithyroid antibodies such as
long acting thyroid stimulator (LATS). Skin is
warm, moist and smooth, and there is facial
flushing, palmar erythema, generalized
hyperhidrosis more so on palms and soles,
pruritis, urticaria, addisonian hyperpigmentation, hyperpigmentation of eyelid (Jellineks
sign), and thyroid swelling in the neck. Scalp hair
become fine, soft and friable, telogen effluvium
can occur. Nails grow rapidly, become thin and
soft, develop distal onycholysis (Plummers
nails).
In addition to these features of hyperthyroidism, patients of Graves disease may have
pretibial myxoedema, thyroid acropachy and
exophthalmos (Diamonds triad).

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Fig. 23.3: Pretibial myxoedemabilateral firm hyperpigmented plaques with orange peel texture over shins

Pretibial myxoedema is characterized by


bilateral asymmetric, firm, hyperpigmented,
plaques or nodules, with orange peel texture
(peau dorange) mainly over shins and feet due
to cutaneous accumulations of glycosaminoglycans (Fig. 23.3). Localized hypertrichosis and
hyperhidrosis can occur over these lesions .
Thyroid acropachy is characterized by digital
clubbing, soft tissue swelling of hands and feet,
and diaphyseal proliferation of periosteum in
acral and distal long bones (tibia, fibula, ulna and
radius).
Exophthalmos is due to orbital deposits in orbital
fossa, always bilateral. Autoimmune diseases
such as vitiligo, alopecia areata can occur in
association with Graves disease.

Hypothyroidism (Myxoedema)
Deficiency of thyroid hormone may be caused
by Hashimotos thyroiditis, iodine deficiency,
iatrogenic following total thyroidectomy,

radioiodine therapy, drugs such as lithium,


congenital hypothyroidism and secondary
hypothyroidism due to reduced TSH from
pituitary. The dermal manifestations are mainly
due to accumulation of mucopolysaccharides
(chondroitin sulphate and hyaluronic acid) in the
skin. Skin appears rough, cool, dry, swollen,
waxy and pale with increased skin creases, and
there can be reduced sweating, asteatotic
eczema, pruritis, and palmoplantar keratoderma
(palms and soles, yellow-orange due to
carotenemia). Patient may have characteristic
expressionless facies with puffy and drooping
eyelids, broad nose, swollen lips, and
macroglossia. There is delayed wound healing,
purpura, ecchymosis and xanthomatosis may
occur.
Nails grow slowly, become thick and brittle
with longitudinal and transverse striations. Scalp
and body hairs are sparse, coarse, dry and brittle,
tend to fall out easily resulting in diffuse or
partial alopecia. Loss of lateral third of eyebrows
(madarosis) -Hertoghes sign
In juvenile hypothyroids, waxy yellowish
skin change is more prominent, but puffiness
may be less apparent.
In cretinism, infants will have periorbital
puffiness, macroglossia, swollen hands and feet,
cold and dry skin, cutis marmorata and umbilical
hernia (pathognomonic). Unlike in adult
hypothyroid, eyebrows tend to be confluent.

SKIN IN PARATHYROID DISEASES


Hyperparathyroidism
Primarily occurs due to adenoma, hyperplasia
or carcinoma of parathyroid glands and
secondary due to chronic renal failure.
Subcutaneous calcifications can present as
linearly arranged white papules or as infiltrating
plaques usually over large joints. Subcutaneous
calcification and calciphylaxis mainly occur in
secondary hyperparathyroidism.

Skin in Systemic Diseases

Hypoparathyroidism
Congenital absence of glands (Di Georges
syndrome), thyroid surgery, hemochromatosis,
metastatic cancer, or idiopathic in origin may be
its causes. Skin changes may be similar to that
of hypothyroidism. The skin becomes dry,
hyperkeratotic and puffy with sparse and coarse
hair. Chloasma and pellagra like pigmentary
changes can occur. Nails become opaque and
brittle with transverse ridges. Impetigo
herpetiformis, psoriatic flares and eczematous
dermatitis have been associated with
hypoparathyroidism. Normalization of serum
calcium levels with calcium and vitamin D
usually reverse skin abnormalities.
SKIN IN DIABETES MELLITUS
Diabetes mellitus (DM) is characterized by
relative or absolute deficiency of insulin, leading
to gross defects in glucose, fat and protein
metabolism.
Type I DM (Insulin dependant DM) is due to
insulin insufficiency from antibody
mediated destruction of beta cells of
pancreas.
Type II (Non-insulin dependant DM) results
from hyperglycemia mainly due to
peripheral insulin resistance.
Nearly all patients with DM have some skin
manifestations. For most manifestations the
pathogenesis remains unknown, and others
result from damage to vascular, neurologic or
immune systems. Macro and microangiopathy
contribute significantly to the cutaneous
complications of DM.

Cutaneous Manifestations of DM
1. Skin Infections
DM patients are at higher risk of contracting
bacterial and fungal infections when compared
to normal population. Staphylococcal and
streptococcal pyodermas (folliculitis, furuncle,

carbuncle), malignant otitis externa (caused by


Pseudomonas aeruginosa), necrotizing fasciitis, and
clostridial gangrene occur more frequently in
diabetics. Candida albicans is the most common
pathogen, causing intertrigo, vulvovaginitis,
balanitis, glossitis, angular cheilitis, paronychia
and onychomycosis. Tinea pedis and onychomycosis of toe nails are common. Rhinocerebral
mucormycosis, a fatal condition caused by
mucor and rhizopus species occur more
commonly in diabetics.

2. Markers of DM
Acanthosis nigricans (AN) presents as brown
to gray black papillomatous cutaneous
thickening of the flexural areas including
posterolateral neck (most common site), axillae,
groin and abdominal folds. Affected area will
have velvety appearance, and it may involve
mucous membranes also. AN can also occur in
association with obesity, internal malignancy
and with drugs such as corticosteroids and
estrogens.

Fig. 23.4: Diabetes mellitusgeneralized


granuloma annulare

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Essentials in Dermatology
Generalized granuloma annulare presents with
numerous small annular plaques formed by 1-2
mm flesh colored papules (Fig. 23.4). These
lesions are symmetric and appear more
commonly over the abdomen, chest, thighs and
extensor of elbows, most frequently in older
adults.
Necrobiosis lipoidica (NL) is a cutaneous
disorder, often but not always associated with
diabetes mellitus. Sixty to seventy percent of
patients have DM, so patients presenting with
NL should be investigated for DM. More
commonly affects middle aged women. The skin
lesions start as skin colored or brownish red
papule which evolve slowly into well
demarcated waxy plaque of variable size. They
have sharply defined, broad violet red or pink,
elevated border and depressed yellow-orange
centre with telangiectasias. Ulcerations
commonly occur within the plaques and heal
slowly resulting in depressed scars. Almost
always affects shins, but can involve thighs,
trunk, and face, scalp (scarring alopecia). Rarely
disseminated NL can occur called as
granulomatous disciformis chronica and
progressiva.
Bullosis diabeticorum is characterized by
abrupt onset of bullae, mainly over the lower
legs, feet, toes, and occasionally over dorsa of
hands and fingers. Bullae appear over non
inflamed skin, and measured from few
millimeter to 3-5 cm in size, non-pruritic and
painless. Healing occurs within 2 to 5 weeks and
rarely leaves scar. Pathogenesis of bulla
formation is unknown. Increased skin fragility
due to glycosylation end products may be
involved.
Scleredema diabeticorum is characterized by
insidious onset of painless, symmetric induration
of skin and subcutaneous tissue mainly over
upper back, neck, and rarely face, shoulder and
anterior torso. Skin has non pitting, woody,

peau d orange quality. There is decreased


sensation to pain and light touch over the
affected area, and decreased range of
movements in neck and upper extremities. Most
patients have type II diabetes. It is due to
thickening of collagen bundles and deposition
of glycosaminoglycans (mainly hyaluronic acid).

3. Complications of Diabetes
Macroangiopathy
Atherosclerosis induced by DM can lead to
ischemic gangrenous changes in legs and feet,
hypothermia, dry skin and nail dystrophy.

Microangiopathy
Microangiopathy causes many cutaneous
manifestations, which include:
1. Wet gangrene of footoccurs as a late
complication.
2. Erysipelas like erythemawell demarcated,
red areas occur on the legs or feet usually in
elderly diabetics.
3. Diabetic rubeosis-peculiar rosy reddening of
the face, rarely of hands and feet in long
standing diabetics.
4. Diabetic dermopathy (diabetic shin spots or
Binkley spots) Most common dermatosis
associated with diabetes. They start as crops
of asymptomatic, oval, dull red papules
0.5-1cm in diameter, which gradually evolve
into atrophic hyperpigmented lesions mainly
over shins, also over forearms, thighs and
bony prominences. If 4 or more are present,
the specificity is high for microvascular
disease in other tissues. An association seems
to exist between dermopathy and other
serious complications such as retinopathy,
nephropathy and neuropathy.

Diabetic Neuropathy And Diabetic Foot


Diabetic foot is mainly caused by peripheral
neuropathy but micro and macroangiopathy and
infections also contribute. Diabetic neuropathy

Skin in Systemic Diseases


is usually distal, polyneuritic, sensory and motor
neuropathy. Motor neuropathy causes weakness
and wasting of muscles, thereby uneven pressure
on feet while walking. Sensory neuropathy
predisposes to trauma and autonomic
involvement causes hypohidrosis. All these
factors combine to produce neurotrophic ulcers
mainly over bony prominences; most commonly
over the ball of great toe. The ulcer is usually
painless, circular and punched out surrounded
by a ring of callus. Some patients present with
burning sensation in the feet.

4. Skin Diseases Commonly Associated with


Diabetes
Eruptive xanthomas, skin tags, vitiligo, lichen
planus, acquired perforating disorders such as
Kyrles disease, reactive perforating collagenosis,
uremic pruritis occur commonly in association
with diabetics.

5. Complications of Diabetic Therapy


Oral hypoglycemic agents may produce allergic
reactions, and photosensivity. Insulin causes
erythema, urticaria and lipodystrophy at
injection site.

SKIN IN PRIMARY SYSTEMIC


AMYLOIDOSIS
Primary systemic amyloidosis involves
mesenchymal tissue, the tongue, heart,
gastrointestinal tract and skin. Cutaneous
manifestations occur in approximately 40% of
cases of primary systemic amyloidosis. The
cutaneous eruptions usually begin as shiny,
smooth, firm, flat topped or spherical papules
of waxy color and have the appearance of
transluscent vesicles because of their tenseness.
These lesions coalesce to form nodules and
plaques of various sizes. The regions about the
eyes, nose, mouth and mucocutaneous junctions
are commonly involved. Purpuric lesions and

Fig. 23.5: Systemic amyloidosismacroglossia


showing indentations of the tongue with gum
hyperplasia

ecchymoses resultant of infiltration of blood


vessels, chiefly affect the eyelids, limbs and oral
cavity. Purpura typically occurs after trauma
(pinch purpura). Purpuric lesions also classically
appear after actions or procedures that result in
increased pressure in the vessels of the face such
as after proctoscopic examination. Glossitis with
macroglossia may be an early symptom and can
lead to dysphagia. The tongue becomes greatly
enlarged and furrows develop. The lateral
aspects show indentations from the teeth
(Fig. 23.5). Diagnosis is confirmed by evaluation
of patients serum and urine for immunoglobulin
fragments and by demonstration of amyloid
deposits in the skin.

SKIN IN VASCULITIS
The term vasculitis refers to inflammation and
necrosis of blood vessels. The vasculitides are
best classified according to the size of the
involved vessels into large, medium and small
vessel vasculitis.

Classification of the Vasculitides


A. Large Vessel Vasculitis
1. Giant cell arteritis
2. Takayasu arteritis

227

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Essentials in Dermatology

B. Medium-sized Vessel Vasculitis


1. Kawasaki disease
2. Polyarterites nodosa (PAN)
3. Benign cutaneous PAN

C.
1.
2.
3.
4.
5.

Small Vessel Vasculitis


Henoch-Schnlein purpura
Cutaneous leukocytoclastic angiitis
Microscopic polyangiitis
Wegeners granulomatosis
Churg Strauss syndrome
Blood vessels have limited ways of
responding to vessel wall injury. They may
respond with increased permeability that leads
to edema and purpura, attenuation of the vessel
wall lead to aneurysm formation or hemorrhage,
and intimal proliferation or thrombosis may
cause stenosis or occlusion with tissue ischemia
or infarction. The definitive diagnosis of a
specific vasculitis remains dependent upon
histopathologic confirmation of vasculitis in
conjunction with the appropriate clinical picture
and the exclusion of diseases that can cause
secondary vascular inflammation such as
infection (e.g., meningococcemia, gonococcemia,
and endocarditis). The principal clinical clue to
diagnosis of a specific vasculitic disorder is the
pattern of organ involvement. Identification of
qualitative pathologic changes in affected organs
(pattern of necrosis, presence of granulomas,
eosinophilic infiltration) further delineates the
differential diagnosis.
The identification of a vasculitic disorder may
not always be easy because of the varied clinical
presentations. More than one system is often
involved and the manifestations can, therefore,
be very heterogenous. However, there are some
clinical pointers that may suggest the presence
of a vasculitic disorder. These are1. Prolonged fever of unknown origin.
2. Suggestive skin lesions e.g. palpable purpura
(Fig. 23.6), gangrene.

Fig. 23.6: Vasculitispalpable purpuric lesions


over the legs of hypersensitivity vasculitis

3. Unexplained peripheral neuropathy


especially mononeuritis multiplex.
4. Arthralgia/arthritis, myositis, serositis.
5. Obscure pulmonary, cardiovascular or renal
disease especially when there is multi-system
involvement.
6. Laboratory parameters indicative of ongoing
inflammation: e.g. leukocytosis, elevated
ESR/CRP, eosinophilia, hypocomplementemia, cryoglobulinemia, circulating immune
complexes.
Kawasaki disease and Hench-Schonlein
purpura are the commonest vasculitides seen in
children while giant cell arteritis, polyarteritis
nodosa and Wegeners granulomatosis are more
common in the adults.

Giant Cell Arteritis (GCA)


GCA generally affects individuals older than
50 years of age and is variably associated with
fever, headache, masticatory muscle
claudication, peripheral vascular disease,
inflammatory aortic aneurysms, and retinal

Skin in Systemic Diseases


ischemic syndromes. Cutaneous manifestations
include rare cases of scalp or tongue ischemia
and necrosis. The diagnosis is established by
biopsy of the superficial temporal artery, which
shows chronic mononuclear cell infiltrate, and
giant cells.

Takayasu Arteritis (TA)


TA is a chronic, idiopathic inflammatory disease
primarily affecting the large vessels, such as the
aorta and its branches. It is also known as
pulseless disease and reverse coarctation. TA
is more commonly associated with aortic and
aortic branch vessel stenoses and aneurysms
than GCA.
TA is the most common cause of giant cell
arteritis in young patients. It mainly affects
females in the age group of 10-30 years. The
precise etiology of TA is still unknown. The most
common complaints at the onset of the disease
include headache, dyspnea, palpitations,
arthralgia/ arthritis and myalgia. Constitutional
features can be in the form of fever, night sweats,
weight loss and anorexia which may be seen in
>50% of patients. The onset of hypertension and/
or absence of upper limb pulses are often the
complaints, which lead to a correct diagnosis.
Polyarteritis Nodosa (PAN)
This is rare in childhood, commonly occurs in
adults. Clinical presentation of PAN is extremely
variable. The initial symptoms can be rather
vague and ill defined with fever, malaise and
weight loss being prominent complaints.
Hypertension is present in more than
80% of patients and the ESR is usually elevated.
CNS involvement occurs in 50 to 70% of children
and may first bring the patient to the attention
of the physician. The other features that suggest
the possibility of PAN are the presence of a
typical livedo reticularis rash, abdominal pain,
arthritis, myalgia and peripheral gangrene.

Kawasaki Disease (KD)


KD is an acute systemic vasculitis of infancy and
childhood. This clinical entity is also known as
mucocutaneous lymph node syndrome. The
clinical diagnosis of KD is usually quite
straightforward, provided one keeps it in mind,
whenever one encounters a young child with a
febrile illness for which no other cause can be
found. KD is a syndrome in which there is a
constellation of clinical findings, which appear
sequentially, with none of the features taken
individually being of any significance.
Diagnostic Criteria for Kawasaki Disease
1. Fever lasting for at least 5 days.
2. Presence of four of the following five
conditions:
i. Bilateral conjunctival injection
ii. Changes of the mucosae of the oropharynx,
including injected and/or fissured lips,
strawberry tongue
iii. Changes of the peripheral extremities such
as edema and/or erythema of hands and/
or feet, desquamation usually beginning
periungually
iv. Rash, primarily truncal; polymorphous but
non-vesicular
v. Cervical lymphadenopathy
3. Illness not explained by other known disease
process.
Henoch-Schnlein Purpura (HSP)
HSP is one of the most common vasculitides of
childhood. HSP is a clinical diagnosis and is
characterized by the presence of nonthrombocytopenic palpable purpura, arthralgia
or arthritis, abdominal pain and gastrointestinal
hemorrhage. These symptoms may occur over
days to weeks. HSP is usually associated with
vascular and renal deposition of IgA-containing
immune complexes.

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Cutaneous Leukocytoclastic Vasculitis


(Hypersensitivity Vasculitis, Small Vessel
Vasculitis)
This is the most common form of vasculitis that
exclusively affects capillaries and venules,
almost invariably involves the skin. It is
frequently associated with immune complex
deposition (with underlying infections, drugs,
malignancy, collagen vascular disorders, etc).
This form of vasculitis has also been termed as
hypersensitivity vasculitis. This small vessel
vasculitis may be limited to the skin or may be
associated with visceral involvement including
pulmonary hemorrhage, intestinal ischemia or
hemorrhage, and glomerulonephritis.
Common manifestations include purpura
and/or urticaria, abdominal pain, gastrointestinal bleeding or intussusception,
arthralgias, arthritis or periarthritis, and
glomerulonephritis. Visceral symptoms may
precede the skin lesions, leading to diagnostic
confusion. Purpura tends to occur in crops of
lesions of similar age. Palpable purpuric lesions
are generally more pronounced in gravitydependent areas like legs (Fig. 23.6). Papules,
urticaria/angioedema, pustules, vesicles, ulcers,
necrosis, and livedo reticularis may be seen.
Paraneoplastic vasculitis is a term used to
describe cutaneous necrotizing vasculitis with
associated malignant conditions, including
Hodgkins disease, lymphosarcoma, adult T cell
leukemia, mycosis fungoides, myelofibrosis,
acute and chronic myelogenous forms of
leukemia, IgA myeloma, diffuse large cell
leukemia, hairy cell leukemia, squamous cell
bronchogenic carcinoma, prostatic carcinoma,
renal carcinoma, and colon carcinoma.
Urticarial vasculitis represents a peculiar subset
of small vessel vasculitis, characterized by typical
wheals or serpentine papules formation,
sometimes with angioedema. Individual lesions
are slower to resolve than typical urticaria and

often last for several days. There is frequently a


burning sensation or discomfort from the lesions.
These lesions often heal with skin discolorationhyperpigmentation or an ecchymotic
area. Most cases of urticarial vasculitis are
idiopathic; they may be associated with an
underlying autoimmune disorder such as SLE
or Sjgrens syndrome, IgM paraproteinemia
(Schnitzlers syndrome), viral infections
(hepatitis, acute Epstein-Barr), and HenochSchnellein Purpura.

Microscopic Polyangiitis
Microscopic polyangiitis (MPA) involves vessels
ranging in size from capillaries and venules to
medium-sized arteries. Glomerulonephritis,
especially rapidly progressive glomerulonephritis, and alveolar hemorrhage are particularly
common in MPA and uncommon in PAN.
Antibody to myeloperoxidase, a type of PANCA, is detected in sera from 60 percent of
patients with MPA.
Benign Cutaneous PAN (BC PAN)
BC PAN is a rare vasculitic entity mostly seen in
adults. The appearance of painful, violaceous,
palpable nodules or ridges of variable size along
the course of arterioles characterize this relatively
benign condition. Mild constitutional symptoms
and arthritis of the weight bearing joints may
occur. The exact etiology of this condition is not
known.
Wegeners Granulomatosis (WG)
WG is a potentially lethal, necrotizing,
granulomatous angiitis affecting small and
medium sized vessels with a predilection for the
sinuses, nasal passages, pharynx, lungs and
kidneys. In some instances, the lesions may be
widely scattered and may involve the skin, heart,
CNS, GI tract and joints. Constitutional
symptoms such as fever and weight loss are
usually quite prominent.

Skin in Systemic Diseases

Churg-Strauss Syndrome (CSS)


(Allergic Angiitis and Granulomatosis)
CSS, like WG, affects small- to medium-sized
arteries and veins. Clinically, CSS and WG have
similar patterns of organ involvement and
pathology, especially in regard to upper and
lower respiratory system disease and glomerulonephritis. CSS differs most strikingly from
WG by its usual occurrence in patients with a
history of atopy, asthma, or allergic rhinitis,
which is often ongoing. In the prevasculitic atopy
phase, as well as during the systemic phase of
the illness, eosinophilia is characteristic and often
of striking degree. Systemic features of CSS
include some combination of pulmonary
infiltrates, cardiomyopathy, coronary arteritis,
pericarditis, polyneuropathy (symmetric or
mononeuritis multiplex), ischemic bowel
disease, eosinophilic gastroenteritis, ocular
inflammation, nasal perforations, glomerulonephritis, and cutaneous nodules and/or
purpura.
Early diagnosis and prompt treatment (with
corticosteroids or immunosuppressive drugs)
can go a long way in decreasing the morbidity
and mortality associated with these disorders.
METASTATIC DEPOSITS OF
MALIGNANCIES
Five to ten percent of patients with cancer
develop skin metastases. Usually metastases
occur as numerous firm, hard or rubbery masses
with predilection for the chest, abdomen, or
scalp, in an adult over the age of 40 years who
has had a previously diagnosed carcinoma. They
are most commonly intradermal papules,
nodules or tumors that are firm, skin colored to
reddish, purplish, black or brown. Inflammatory
carcinoma (carcinoma erysipeloides) is
characterized by erythema, edema, warmth and
a well defined leading edge similar to erysipelas

Fig. 23.7: Metastatic deposits of malignancies


Sister Mary Joseph nodules

in appearance. This is usually caused by breast


carcinoma. The so-called Sister Mary Joseph
nodule (Fig. 23.7) is formed by localization of
metastatic tumors to the umbilicus. The most
common primary sites are the stomach, large
bowel, ovary and pancreas. Dissemination to the
skin is often a late finding and metastases to other
organs have usually occurred. A poor prognosis
is thus the rule.
Most cases of Hodgkins disease of the skin
usually originate in the lymph nodes from which
the extension to the skin is either retrograde
through the lymphatics or by direct extension.
Lesions present as papules or nodules with or
without ulceration. Cutaneous B-cell lymphoma
may present with solitary or multiple papules,
plaques or nodules (Figs 23.8 and 23.9). The most
common morphology of leukemic infiltration of
the skin in all forms of leukemia is multiple
papules or nodules or infiltrated plaques. They
are rubbery on palpation and ulceration is
uncommon. Extensive involvement of the face
may lead to leonine facies.

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Essentials in Dermatology

Fig. 23. 9: Metastatic deposits of malignancies


infiltrated nodules of lymphoma over the arm
Fig. 23.8: Metastatic deposits of malignancies
infiltrated nodules and plaques of lymphoma over the
abdomen

PARANEOPLASTIC DISORDERS
Paraneoplastic disorders refer to cutaneous
reaction patterns that have a statistical
association with neoplasia of various internal
organ systems.
Schnyder criteria for paraneoplastic disorders:
No other explanation for skin findings.
Skin changes improve with cancer treatment
and may reappear if cancer recurs.
Skin changes appear at about the same time
as the systemic cancer: They may precede any
clinical manifestation of the underlying
malignancy.
Statistical connection is shown.

Paraneoplastic Disorders can be


Categorized as
1. Disorders of Keratinization
Acanthosis nigricans (AN) is characterized by
velvety thickening and hyperpigmentation of the
neck and body folds giving the skin a dirty
appearance. It occurs in association with DM,
obesity, internal malignancy and with certain

Fig. 23.10: Paraneoplastic disordersrugose


thickening of palmstripe palms

drugs. Malignant acanthosis nigricans usually


occurs in adults over 40 years of age. If differs
from other forms of AN by its rapid onset and
spread, and there is marked velvety hyperkeratosis and hyperpigmentation. There is more
pronounced involvement of mucosa, perioral
area, palms (tripe palms or acanthosis palmaris)
and associated weight loss and wasting.
Adenocarcinoma of stomach is most
commonly associated with malignant AN.

Skin in Systemic Diseases

Acquired ichthyosis: Sudden onset of ichthyosis


in adults should arouse the suspicion of internal
malignancy. Associated neoplasms are nonHodgkins lymphoma, mycosis fungoides,
multiple myeloma, carcinoma of breast, lung,
cervix and liver.

Necrolytic migratory erythema (Glucagonoma


syndrome) is caused by excessive production of
glucagon by alpha cell tumor of pancreas. It is
more commonly seen in women of 45-65 years.
The skin lesions start as circinate, annular or
arcuate erythematous plaques that spread
peripherally with central clearing. Borders show
vesicles, bullae, crusting and scaling giving
polycyclic or geographical appearance. These
lesions mainly localize around periorificial, acral,
and intertriginous areas, closely resembling
acrodermatitis enteropathica. Other features are
glossitis, angular cheilitis, blepharitis, wasting,
anemia, glucose intolerance. Zinc replacement,
orctreotide, surgical excision of tumor gives cure
only in 30% of cases, because of persistent
metastasis.

Acrokeratosis of Bazex: This condition is


characterized by symmetric, erythematous
psoriasiform eruption mainly involving hands,
feet, ears and nose. Nails are involved early and
severely. It is commonly associated with
carcinoma of pharynx, esophagus, tongue and
lung.

Erythema annulare centrifugum usually


idiopathic rarely associated with malignancies
particularly myeloproliferative disorders. It is
characterized by slowly expanding annular
erythematous plaques with central clearing and
scaling at trailing edge, commonly involves
buttocks, thighs (Fig. 23.11), and upper arms.

Erythroderma is commonly associated with


leukemia, lymphoma especially cutaneous T cell
lymphoma (CTCL), and carcinoma of lung, liver,
stomach, colon, pancreas and prostate.
Erythroderma secondary to solid tumors may
resolve after resection of the tumor.

3. Bullous Eruptions

Associated features may be Leser Trelat sign,


florid cutaneous papillomatosis, and pachydermoperiostosis. Malignant AN typically improves
with treatment of underlying malignancy.
Tripe palms is characterized by rugose
thickening of the palms and accentuation of
dermatoglyphic ridges (Fig. 23.10). It may occur
alone or along with acanthosis nigricans. It is
commonly associated with carcinoma of
stomach, and lung.

Paraneoplastic pemphigus (PN) is a bullous


disease characterized by severe and resistant

2. Migratory Erythemas
Erythema gyratum repens is characterized by
mobile, concentric, often palpable, erythematous,
wave-like bands (migrating at the rate up to 1
cm per day), giving wood grain appearance
to skin. A peripheral scale or collarete may be
present, and frequently involves the trunk.
Tumors associated are lung cancers, myeloproliferative disorders and many other solid
cancers. Resection of the tumor may result in
resolution of lesions.

Fig. 23.11: Paraneoplastic disordererythema


annulare centrifugum over the thigh

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Essentials in Dermatology
polymorphous mucocutaneous lesions.
Pemphigus like, pemphigoid like, erythema
multiforme like, graft versus host disease like
and lichen planus like lesions occur. Associated
neoplasms are Hodgkins lymphoma, chronic
lymphocytic leukemia, Castlemans tumor, and
thymoma.
Pemphigus vulgaris has well defined
association with thymoma. Hodgkins
lymphoma and many solid tumors have also
been associated.
Bullous pemphigoid is a subepidermal autoimmune bullous disease, mainly affecting
elderly individuals characterized by large tense
blisters over normal or erythematous skin
distributed mainly over central abdomen and
flexural aspect of limbs. Significant association
with gastric carcinoma has been found.
Adult linear IgA disease is a subepidermal
bullous disease characterized by vesicles, bullae,
and urticarial plaques mainly over trunk and
extremities. An increased association with
lymphomas has been found.
Epidermolysis bullosa acquisita is a
subepidermal bullous disease characterized by
bullae, and vesicles at trauma prone sites.
Association with myeloma and leukemia has
been found.

mainly on the anterior surface of legs. It is usually


associated with streptococcal infections, and
sarcoidosis. Associated neoplasms are Hodgkins
and non-Hodgkins lymphoma, leukemia and
renal cell carcinoma.
Subcutaneous fat necrosis has been reported in
association with pancreatic carcinoma (mainly
acinar cell type).

5. Collagen Vascular Diseases


Dermatomyositis is an autoimmune disease
characterized by inflammation of skin and
skeletal muscles. Associated neoplasms are
carcinoma of lung, ovary, stomach, pancreas,
colon and lymphoma. Adults rather than
children with dermatomyositis should be
thoroughly investigated for underlying
malignancy.
Systemic lupus erythematosus is rarely
associated with lymphoma or thymoma.
Scleroderma was found to be associated with
carcinoma of lung, but this could be due to lung
tumor developing secondary to chronic
pulmonary fibrosis.

Dermatitis herpetiformis is occasionally


associated with intestinal lymphoma.
Herpes gestationis has been described in
association with hydatiform mole and germ cell
tumor.
Erythema multiforme like lesions occur in
association with carcinoma, lymphoma and
leukemia.

4. Panniculitides
Erythema nodosum is characterized by crops of
painful, erythematous nodules and plaques

Fig. 23.12: Paraneoplastic disorderpyoderma


gangrenosum

Skin in Systemic Diseases


cancer. Generalized telangiectasias can occur
with malignant angioendotheliomatosis and
carcinoid tumors.
Purpura: Lymphoma, acute leukemia, multiple
myeloma can cause purpura.
Vasculitis: Leukocytoclastic vasculitis is rarely
associated with squamous cell carcinoma of
bronchus, renal carcinoma, leukemia and
lymphoma.

Fig. 23.13: Paraneoplastic disorderSweets


syndrome lesions over the upper limb

6. Neutrophilic Dermatoses
Pyoderma gangrenosum (PG) particularly
bullous PG is associated with myeloproliferative
disorders. Solid tumors such as carcinoma of
colon, prostate, bladder breast, bronchus and
ovary have also been reported in association with
PG (Fig. 23.12).

Digital ischemia: Persistent digital ischemia


often progressing to gangrene has been
associated with carcinoma of pancreas, stomach,
small bowel, ovary and kidney as well as with
lymphoma.
Monders disease is thrombophlebitis of anterior
chest wall, which may be associated with breast
cancer.

Sweets syndrome is characterized by abrupt


onset of painful, tender, erythematous to
violaceous plaques and nodules (Fig. 23.13)
mainly over trunk and proximal extremities.
Associated malignancies are acute myeloid
leukemia (most common), other leukemias,
multiple myeloma, and lymphoma, less
commonly with carcinoma of testis, ovary,
stomach, breast, prostate and rectum.

7. Vascular and Blood Abnormalities


Flushing: Marked flushing of central face and
upper trunk can be a feature of carcinoid
syndrome.
Palmar erythema can occur in liver failure
secondary to either primary or metastatic tumor
of liver.
Telangiectasias: Localized, grouped telangiectasia of anterior chest wall may indicate breast

Fig. 23.14: Paraneoplastic disorderwide-spread


lesion of seborrheic keratoses over the trunk Sign of
Leser Trelat

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Essentials in Dermatology

8. Hormone Related Disorders


Cushings syndrome: Tumors of lung and
pancreas producing excessive ACTH can lead
to Cushings syndrome.
Hirsutism: Androgen secreting ovarian tumors
can produce hirsutism.
Gynecomastia: Estrogen secreting tumors of
testis, lung tumor can cause gynecomastia.

9. Others
Xanthomas: Diffuse plane xanthoma can be
caused by multipl e myeloma.

Hypertrichosis lanuginosa acquisita refers to


acquired excessive growth of lunugo (vellus)
hairs mainly over face and ears but eventually
over all hair bearing areas. It is usually associated
with painful glossitis. Associated tumors are
tumors of colon, rectum, bladder, lung, pancreas,
uterus, breast, and lymphoma.
Multiple eruptive seborrheic keratosis Sign
of Leser Trelat refers to sudden development
of numerous often pruritic seborrheic keratosis,
in association with internal malignancy (Fig.
23.14). Associated neoplasms are adenocarcinoma of GIT, breast, tumors of reticuloendothelial system, and transitional cell carcinoma of
bladder.

Skin Changes of Pregnancy and Old Age

24

Skin Changes of
Pregnancy and Old Age

SKIN CHANGES OF PREGNANCY


Skin changes occurring during pregnancy can be
grouped into physiological variations, changes
in pre-existing dermatoses, and pregnancy
specific dermatoses.

Physiological Variations
Physiological changes are most likely caused by
hormonal changes. These include pigmentation
of nipples, areolae (Fig. 24.1), and external
genitalia. The linea alba becomes the linea nigra.
Chloasma or melasma, a mask like hyper
pigmentation of the face occurs in more than 50%

Fig. 24.1: Hyperpigmentation of nipple and areola


with prominent Montgomery tubercles

of women. Pre-existing nevi or ephelides


frequently darken during pregnancy. Mild to
moderate hirsutism is frequently seen during
pregnancy. After delivery, the resulting telogen
effluvium may be severe, resulting in significant
hair loss from 1 to 5 months postpartum. Striae
distensae (Fig. 24.2) occurs in up to 90% of
pregnant women. It is commonly seen over the
abdomen, hips, buttocks, and sometimes breasts.
Up to two-third of women develop palmar
erythema and/or spider angiomas during
pregnancy. Edema and varicosities commonly
occur in hands and feet. Pregnancy epulis
(granuloma gravidarum/pregnancy tumor of

Fig. 24.2: Striae distensae over the abdomen

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Essentials in Dermatology
gingiva) is a pyogenic granuloma that may appear
in the second or third trimester and resolves
shortly after delivery.

Cholestyramine and ursodeoxycholic acid may


be effective. Vitamin K to diminish the risk of
postpartum hemorrhage can be given before
delivery.

Changes in Pre-existing Dermatoses


Acne, acne inverse, psoriasis and sarcoidosis tend
to improve with pregnancy. Autoimmune collagen
vascular disorders (lupus erythematosus,
dermatomyositis, systemic sclerosis), autoimmune bullous diseases, invasive or metatastic
melanoma, porphyria cutanea tarda, EhlersDanlos syndrome, pseudoxanthoma elasticum,
viral and fungal infections tend to worsen. Atopic
dermatitis and pustular psoriasis may improve
or worsen.

Pemphigoid (Herpes) gestationis: It is the most


specific dermatosis of pregnancy which is an
autoimmune inflammatory bullous disease with
onset during second trimester or during the
postpartum period. Typically, pruritic urticarial
papules and plaques develop around the
umbilicus and extremities, which evolve into tense
vesicles and bullae. Remit spontaneously usually
within 3 months of delivery.

Pregnancy Specific Dermatoses


Recurrent cholestasis of pregnancy (Prurigo
gravidarum): It is a hepatic condition occurring
late in pregnancy characterized by severe
generalized pruritus that is often followed by the
appearance of clinical jaundice. It has an
estimated incidence of up to 2% of pregnancies.
A slight increase in fetal mortality and
prematurity has been reported. Postpartum
hemorrhage is also more likely in these women.
The condition remits within few days after
delivery but tends to recur in subsequent
pregnancies. Treatment includes bland
emollients, topical antipruritic regimens,
antihistamines, and UVB phototherapy.

Fig. 24.3

Pruritic urticarial papules and plaques of


pregnancy (PUPPP): Also called as polymorphic
eruption of pregnancy. It is a very common,
intensely pruritic dermatosis that usually occurs
late in the third trimester. It typically affects
primigravidas. It is characterized by erythematous
papules that begin in periumbilical striae
distensae (Figs 24.3 and 24.4) and then spread to
involve the buttocks and thighs yet spares the
upper chest and face. Spontaneous resolution
occurs most commonly few days after delivery.

Fig. 24.4

Figs 24.3 and 24.4: Pruritic urticarial papules and plaques of pregnancy involving striae and arms

Skin Changes of Pregnancy and Old Age

Fig. 24.5: Senile comedones over the face

Fig. 24.6: Cherry angiomas over the back

Topical corticosteroids form the main stay of


management.

alone; photoaging (extrinsic aging), the


superimposition on intrinsic aging of changes
attributable to chronic sun exposure, which are
preventable. Intrinsic aging changes include
epidermal atrophy, reduced wound repair, few
Langerhans cells and generally impaired immune
response, decreased sensation and fewer hairs,
decreased sweating, drier skin (less sebum and
epidermal lipids) and less vascular reactivity.
Chronic exposure to the ultraviolet (UV)
component of solar radiation leads to photoaging
which is characterized clinically by fine and
coarse wrinkles, dyspigmentation (freckling and
lentigines), telangiectasia, laxity, roughness and
a sallow appearance. Light exposed skin may
develop periorbital comedones (Fig. 24.5)
(maladie de Favre et Racouchot), purpura, and
venous lakes. Over a period of time, these extrinsic
aging changes develop into solar elastosis,
accelerated cell death and vascular changes (Fig.
24.6), actinic keratoses, basal cell carcinoma,
squamous cell carcinoma and malignant
melanoma. Topical retinoids and hydroxyacids
are the most effective treatment in preventing and
partially reversing photodamage. A healthy
balanced diet, avoidance of smoking, a good skin
care regimen and regular exercise, coupled with
sun protection in those with sensitive skin and
outdoor lifestyles helps in preventing photoaging.

Prurigo gestationis (Besnier): It is a rare pruritic


dermatosis that may occur any time during the
fourth to ninth months of gestation. It is
characterized by small papules that are excoriated
on the proximal limbs and trunk. The eruptions
tend to resolve quickly after delivery and it is
uncommon to recur in subsequent pregnancies.
Therapy with topical glucocorticoids is helpful.
Impetigo herpetiformis: It is a form of pustular
psoriasis that occurs during pregnancy and may
be life-threatening. It tends to occur in third
trimester of pregnancy. It is characterized by
erythematous patches with peripheral pustules
and central scaling favoring the abdomen and
upper inner thighs. Systemic signs and symptoms
include fever, chills, nausea and vomiting. The
disease tends to remit promptly after delivery but
may recur in subsequent pregnancies. Systemic
glucocorticoids are the treatment of choice in
impetigo herpetiformis.

SKIN CHANGES OF OLD AGE


Cutaneous aging includes two distinct
phenomena: Intrinsic aging, a universal,
inevitable change attributable to passage of time

239

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Essentials in Dermatology

25

Pediatric Dermatology

Some skin disorders are transient in newborn but


make the parents nervous and anxious about their
outcome, while other skin disorders develop later
on during infancy or childhood. Some of these
disorders are exclusive to children (Cradle cap,
napkin dermatitis, acrodermatitis enteropathica,
primary herpetic gingivostomatitis) while others
are common in children (Pediculosis capitis,
scabies, molluscum contagiosum, warts, chicken
pox, tinea capitis, atopic dermatitis, oral thrush,
Henoch-Schnlein purpura, acute hemorrhagic
edema of infancy, and papular urticaria).

Transient Cutaneous Lesions in Newborn


Vernix caseosagolden yellow staining of
vernix caseosa occurs in hemolytic disease of
the newborn and post-maturity.
Acrocyanosiscyanosis seen in acral areas
in a neonate
Erythema neonatorumwithin a few hours
of birth- erythema fades spontaneously within
24-48 hours.
Harlequin color changeseen when the
infant is placed on one side-upper half of the
body becoming pale and the lower half a deep
red color, with a sharp midline demarcation
between the two, staying for half a minute to
20 minutes, in a full term or preterm newborn
seen during first week of life.

Cutis marmoratathis marbling change due


to reticulate blue vascular pattern seen in
infancy, occurs on exposure to decreased
temperature and disappears on rewarming.
Physiological scaling of the newbornseen
in up to 75% of the normal neonates.
Sebaceous gland hyperplasia characterized
by multiple, uniform, pin point yellowish
papules, most prominent on the nose, cheeks,
upper lip and forehead, but may be visible on
the upper trunk, especially the areolae,
genitalia and the limbs. This phenomenon is
associated with milia which represents minute
follicular epidermal cysts.
Linea alba becomes pigmented in about 8%
of babies.
Exaggerated pigmentation of the scrotum
occurs in about 30% of oriental neonates,
generally associated with Mongolian spots.
Epstein pearlsare one or more 1-2 mm
yellowish white keratinous cysts along the
alveolar ridges or in the midline at the junction
of the hard and soft palate.
Milia represent miniature epidermal inclusion
cysts that originate from sebaceous apparatus
of vellus hair (Fig. 25.1). Epstein pearls are
clinically and histologically the intra-oral
counterpart of facial milia.

Pediatric Dermatology

Fig. 25.1: Milia tiny pearly white keratinous


inclusion cysts over the face

Succulent gums (Sucking pads)a whitish


hue to the oral mucosa.
Sucking blistersblisters or erosions on the
upper limb/upper lip, present at birth, due to
intrauterine sucking.
Erythema toxicum neonatorum (Toxic
erythema of the new born, erythema
neonatorum)presents as an asymptomatic
macular erythema on the trunk within first 48
hours of birth. Subsequently, it may evolve
into urticarial papules or pustules. Recovery
occurs in 3 days. Smears of the pustule
contents demonstrate inflammatory cells,
more than 90% of which are eosinophils.
There is an associated blood eosinophilia in
50% of cases.
Mongolian spotsare blue gray pigmentation areas present at birth on the sacral area
of the normal infants in dark skinned races.
Edwin Baez, thinking this as a characteristic
of Mongolian race, named it as Mongolian
spot. The Chinese people believed that the
mark is the imprint of their God of birth. As
the child is born, the God gives it a spank or
kick to give it a start in life, whereas the

Japanese believed that the spot was the


consequence of coitus performed during
pregnancy. It usually disappears during the
first decade and in a small percentage (3-4%)
persist beyond this into adult life.
Acne neonatorum (Neonatal acne)
develops within the first 30 days of life.
Perianal dermatitis is an erythema centered
on the anus, occasionally accompanied by
erosion and bleeding at 4th to 7th day of life
Transient neonatal pustulosis (Transient
neonatal pustular melanosis) invariably
presents at birth. It is characterized by the
presence of fragile superficial pustules mainly
over the chin, neck, forehead, back, and
buttock. Hyperpigmented macules develop
subsequently. They may persist for 3 months
and affected infants are otherwise normal.
Pustules are formed due to subcorneal
collection of neutrophils with a few
eosinophils. Bacterial culture is negative.
Pigmented macules demonstrate basal and
supra-basal increase in pigmentation only,
apparently without pigmentary incontinence.

Skin Disorders in Children


(Pediatric Dermatoses)
Skin disorders are extremely common in our
population and approximately 6% of visits to all
physicians entail a problem of the skin, hair or
nails. However, dermatologists see only
approximately 40% of these patients.
Dermatological problems manifesting as primary
and secondary cutaneous complaints, constitute
at least 30% of all outpatient visits to a pediatrician
and 30% of all visits to dermatologists involve
patients of pediatric age group.
The vast majority of complaints related to the
skin in children can be easily recognized and
treated by non-specialist. These common diseases
have been classified in the Table 25.1.

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Table 25.1: Classification of common pediatric dermatoses
1. Infestations and infections (a) Parasitic infestationsPediculosis capitis, scabies (b) Bacterial infections
Pyodermas (c) Viral infectionsMolluscum contagiosum, warts, herpes simplex, chicken pox, herpes zoster
(d) Fungal infections-Tinea capitis, tinea corporis, pityriasis versicolor, candidiasis
2. Dermatitis and eczemaInfantile seborrheic dermatitis, diaper or napkin dermatitis, atopic dermatitis, infective
dermatitis
3. Urticaria
4. ExanthemsViral exanthems (Measles, rubella, roseola infantum, erythema infectiosum)
5. Drug eruptions
6. Pigmentary disordersPostinflammatory pigmentation, hypopigmentary disorders (Pityriasis alba, vitiligo, leprosy,
nevus achromicus, ash leaf macule, albinism), hyperpigmentary disorders (Mongolian spots, caf au lait macules)
7. Diseases of hair and nailsTinea capitis, alopecia areata, diffuse alopecia, twenty nail dystrophy
8. Genetic diseases of the skin-Ichthyoses, acrodermatitis enteropathica
9. Collagen vascular diseasesConnective tissue diseases (Lupus erythematosus, scleroderma, and dermatomyositis)
and vasculitic syndromes (Henoch-Schnlein purpura, acute hemorrhagic edema of infancy, and polyarteritis
nodosa)
10. Miscellaneous conditionsPapular urticaria, miliaria rubra, miliaria crystallina, psoriasis, hemangiomas, chilblains

SOME IMPORTANT VIRAL EXANTHEMS


IN CHILDREN
Measles (Rubeola, Morbilli)
The term measles is thought to come from
Latin misellus or misella, a diminutive
of Latin miser, meaning miserable.
It is caused by measles virus (a paramyxovirus, RNA virus).
Incubation period is 10-11 days.
Measles, is a universal highly contagious
disease of children. It has a characteristic
prodrome of 3-4 days that consists of high
fever, cough, coryza, a striking palpebral
conjunctivitis with photophobia, and Kopliks
spots, which precede the appearance of florid
generalized macular and papular rash.
The first lesion to appear on the soft palate is
blotchy erythema, but the most pathognomonic lesion of the prodrome, if present
are Kopliks spots, which appear as tiny
white lesions surrounded by an
erythematous ring (grain of sands).
Kopliks spots precede the onset of generalized
rash by 1-2 days, remain for two to three days

and are usually heavily clustered on the


buccal mucosa opposite the second molar.
The purplish red rash on the body appears
first behind the ears and over the forehead,
and then spreads slowly to involve the entire
body by third day. The eruption extends
downwards over the neck, shoulders and
trunk and then distally over the upper and
lower extremities. The rash fades with brown
pigmentation followed by desquamation.
Uncomplicated measles runs a self limited
course lasting about 10 days.
Complications of measles include encephalitis, thrombocytopenia, otitis media,
pneumonia, exacerbation of tuberculosis and
subacute sclerosing panencephalitis.
There is no specific therapy for measles.

Rubella (German Measles)


Rubella virus is a togavirus, commonly
recovered from pharynx.
Incubation period is 14-21 days.
Rubella is a common communicable infection
of children and young adults characterized
by a short prodromal period; enlargement of

Pediatric Dermatology
cervical, suboccipital and postauricular
glands and a rash of approximately 2 to 3
days duration.
An enanthem, Forschheimers sign, is present
in up to 20% of patients during the prodromal
period or on the first day of the rash. Dull-red
macules or petechiae are confined to the soft
palate.
The disease has rare sequelae apart from
devastating effect on the fetus.

Exanthem Subitum (Sixth Disease,


Roseola Infantum)
Caused by Human herpes virus type 6 (DNA
virus).
Incubation period is 10-15 days.
Most common exanthem with fever in children
under age group of 2 years.
Prodromal fever is usually high. Fever drops
on fourth day.
Convulsions and lymphadenopathy may
accompany it.
Clinically, a morbilliform erythema
consisting of rose colored discrete macules
appears on the neck, trunk, and buttocks.
Often there is a blanched halo around the
lesions.
The lesions resolve in 1 to 2 days.
Other common associated findings include
otitis media, diarrhea and meningoencephalitis.
In adults HHV-6 infection resembles acute
infections mononucleosis.
Treatment acyclovir, ganciclovir.
Erythema Infectiosum (Fifth Disease)
Exanthematous disease occurring in patients
with primary human parvo virus B19 infection
(DNA virus).
Incubation period is 4-14 days.
More commonly seen in school children.

Infection spreads by respiratory droplets


during the prodrome.
Constitutional symptoms are absent or very
minimal.

Three Stages of Rash are


1. Slapped cheek appearance (1 to 4 days).
2. Erythematous papular eruption over the
upper and lower extremities spreading to
trunk. Assumes a lace-like or reticulated
appearance as it fades.
3. Recurrent evanescent stage (for weeks or
months) is precipitated by a variety of skin
irritants such as sunlight, hot showers.
Papular purpuric gloves and socks
syndrome seen in older children and
adolescence.
Adults may present with atypical rash and
arthritis.
ComplicationHydrops foetalis (maternal infection) and aplastic crisis.
Treatmentsupportive.

Gianotti-Crosti Syndrome
Infantile papular acrodermatitis, or the
Gianotti-Crosti syndrome, presents
with symmetric erythematous lichenoid
papules on the face, extremities, and
buttocks, usually sparing the trunk.
The eruption is not pruritic and may be
accompanied by splenomegaly, hepatitis, and
lymphadenopathy.
The process often occurs in young children
after an upper respiratory tract illness.
Pathologic specimens show a perivascular
infiltration of lymphocytes and histiocytes in
the upper portion of the dermis.
While the syndrome has been associated with
hepatitis B and enterovirus infection, several
cases have been associated with acute EBV
infection.

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SOME OTHER IMPORTANT ENTITIES


Henoch Schnlein Purpura
Henoch Schnlein purpura is an IgA
mediated vasculitis syndrome affecting skin,
joints, GIT and kidneys.
It usually occurs below the age of 20 years.
The eruption may begin as crops of palpable
purpuric lesions or urticarial rash in the lower
legs and buttocks.
Abdominal symptoms such as colic, vomiting
and diarrhea are seen in two-third of the
patients.
Polyarthralgia is seen in most patients,
commonly affecting the knees and ankles.
Renal involvement is usually mild, consisting
of proteinuria/hematuria, but can rarely lead
to end stage renal disease.
Diagnosis: Histopathology shows leucocytoclastic vasculitis and immunoflorescence
demonstrates IgA deposition around blood
vessels.
Treatment: No specific treatment available.
Antibiotics and corticosteroids can be given
to alleviate symptoms.

Fig. 25.2

Urticaria Pigmentosa
Urticaria pigmentosa is the most common type of
cutaneous mastocytosis that occurs due to the
accumulation of mast cells in the skin.
The skin lesions are itchy small, yellow tan to
reddish brown maculopapules/plaques/
nodules scattered all over the body (Figs 25.2
and 25.3).
Mild trauma such as scratching or rubbing
the lesions may cause urtication and erythema
around macules and this is known as Dariers
sign.
Urticaria pigmentosa is associated with
pruritus which may be exacerbated by
temperature, friction, spicy foods, alcohol and
drugs.
Diagnosis: Histopathology shows mast cell
infiltration in the dermis
Treatment: Avoidance of precipitating factors
and antihistaminies.
Langerhans Cell Histiocytosis (LCH)
Langerhans cell histiocytosis is a disease that
results due to the proliferation of cells like
Langerhans cells, called as LCH cell (share the

Fig. 25.3

Figs 25.2 and 25.3: Urticaria pigmentosamultiple brownish pigmented infiltrated


lesions over trunk and extremities

Pediatric Dermatology

Fig. 25.4: Letterer-Siwe diseaseseborrheic


dermatitis like rash involving scalp extending on to
forehead and ears

Fig. 25.5: Letterer-Siwe diseasehypopigmented


macular as well as purpuric papular lesions seen
over the abdomen

ultrastructural features with Langerhans cellscontaining Birbeck granules) in any organ. This
LCH cell is about four to five times larger than
small lymphocytes; has an irregular and
vesiculated nucleus; is often reniform (kidney
shaped); and has abundant, slightly eosinophilic
cytoplasm.

Fig. 25.6: Letterer-Siwe diseasemarked


hepatosplenomegaly

It is broadly classified into entities such as


Letterer-Siwe disease, Hand-Schller
Christian disease and eosinophilic granuloma.
Eosinophilic granuloma occurs due to
localized proliferation of LCH cells in the
bones, skin, lymph nodes, lung, liver and
spleen .
Hand-Schuller-Christian syndrome is a
chronic multisystemic disease known by the
triad of exophthalmos, multiple skull lesions
and diabetes insipidus.
Letterer-Siwe disease is usually seen in
children less than 1 year. It is characterized
by seborrheic dermatitis like rash (Fig. 25.4)
with hemorrhagic papular (Fig. 25.5),
vesicular, pustular and ulcerated lesions in
the intertriginous regions and trunk.
Letterer-Siwe disease is often associated with
hepatosplenomegaly, dysfunction of liver,
lungs and hematopoietic system. Lytic lesions
may be seen in skull bones (Fig. 25.6).
Diagnosis: Histopathology shows LCH cell
infiltration of the lesions.
Treatment: Localized forms can be treated with
intralesional steroids or surgery. Multisystemic involvement needs chemotherapy.

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26

Benign, Pre-malignant and


Malignant Tumors of the Skin

Skin tumors can be broadly classified into


various types based on cell of origin. Broadly
they can be classified as benign, premalignant
and malignant tumors. Some of these common
cutaneous tumors are discussed in this chapter.

BENIGN SKIN TUMORS


Seborrhoeic Keratosis (Senile Wart,
Senile Keratosis, Seborrheic Verruca,
Basal Cell Papilloma)
A benign tumor, more common in the elderly
people.
Seborrheic keratosis (SK) occur on any body
site, most frequent on the face and the upper
trunk.
Seborrheic keratoses typically begin as flat,
sharply demarcated, brown macules.
Follicular prominence is one of the hallmarks
of seborrheic keratoses.
Later on, typical asymptomatic, slowly
increasing, verrucous plaque develops and
have a stuck-on appearance.
Surface of SK has loosely adherent greasy
keratinous scales on the surface.
Sometimes, sudden eruptive lesions appear
which may be due to underlying malignancy.
Then it is called Leser-Trelat sign.
Classical SK shows features of hyperkeratosis
and numerous horn cysts in histology.

Treatment curettage, cryotherapy or


electrodessication.

Naevi
The word naevus is derived from the Latin
term meaning spot or blemish, originally
used to describe the congenital lesion or birth
mark (Mothers mark).
In modern usage, it denotes a cutaneous
hamartoma or benign proliferation of cells.
Nevi can be broadly classified into various
types according to the predominant cell type.
The various types are keratinocyte nevi (Figs
26.1 and 26.2), follicular nevi, sebaceous
nevi, apocrine nevi, eccrine nevi, connective
tissue nevi, smooth muscle nevi, elastic
nevi, fat nevi (Fig. 26.3), and vascular nevi
(Figs 26.4 to 26.7).

Melanocytic Nevi
They are benign tumors derived from
melanocytes. They are broadly classified into
acquired or congenital.
Acquired melanocytic naevi are subdivided
into junctional, compound (Figs 26.8 and
26.9) and dermal.
They begin as proliferative naevus cells along
the dermal-epidermal junction (forming a
junctional naevus)

Benign, Pre-malignant and Malignant Tumors of the Skin

Fig. 26.1

Fig. 26.2

Figs 26.1 and 26.2: Nevusverrucous epidermal (keratinocyte) nevus occurring on one side of the body

Fig. 26.3: Nevus-nevus lipomatosus cutaneous


superficialis presenting as lobulated mass over the
thigh

With continued proliferation, they extend


from the dermal-epidermal junction into the
dermis (forming a compound naevus).
The junctional component of the melanocytic
naevus may resolve leaving only an
intradermal component (intradermal
naevus).
Acquired melanocytic naevi may resolve
spontaneously.

Fig. 26.4: NevusPortwine stain over the face

Congenital melanocytic nevi may be


defined as melanocytic naevi present at
birth (Figs 26.10 to 26.13).
Those measuring more than 20 cm in greatest
diameter are referred to as Giant congenital
melanocytic nevi or bathing trunk nevi.
Treatment: surgical excision.

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Essentials in Dermatology

Fig. 26.5

Fig. 26.6

Figs 26.5 and 26.6: NevusHemangioma of infancy over the nape of neck and face

Fig. 26.7: Angiokeratoma circumscriptum of the


tongue

Fig. 26.8: Melanocytic nevuscompound nevus


over the cheek

Skin Tags (soft warts; Acrochordons)


Common benign lesion, occurs on the neck,
axilla and groin
The lesions are round, soft, pedunculated
connected to the skin by a narrow pedicle.

They vary in size from 1 mm to 1 cm long


(Fig. 26.14).
The skin tags are of three types which
includemultiple small furrowed papules (12 mm long), single or multiple filiform

Benign, Pre-malignant and Malignant Tumors of the Skin

Fig. 26.9: Halo nevus over the cheek

Fig. 26.10: Congenital melanocytic nevus over the


face of a child

smooth growths (2-5 mm) and solitary


baglikepedunculated growth (1 cm).
Histopathologically skin tags consist of loose
fibrous tissue covered by folded skin.
They have been found in association with
colonic polyps, diabetes and acromegaly.
Simple snipping, electrocautery and
cryotherapy are effective.

Fig. 26. 11: Congenital melanocytic nevus with


hairiness over the back

Pyogenic Granuloma (Granuloma


pyogenicum)
They are smooth surfaced, bright red,
friable, sessile or pedunculated lesions of
exuberant granulation tissue with a pale
epidermal collarette around the lesion (Fig.
26.15)
Occurs following a minor injury or infection
of the skin
They most often affect children or young
adults
The hands, fingers and face especially the lips
and gums are the most common sites.
The lesion once developed may persist
indefinitely unless destroyed. It may occur
in pregnancy in gingivacalled as Epulis
Gravidarum or Pregnancy tumor.
Differential diagnosis includes Kaposis
sarcoma and bacillary angiomatosis
Treatment: excision and electrofulguration.
Milia
They are tiny, white, globoid cysts that
commonly occur on the face around eyes
(Fig. 26.16).
Primary milia represent a keratinizing benign
tumor. They arise spontaneously on the face
in the predisposed individuals.

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Essentials in Dermatology

Fig. 26.12

Fig. 26.13

Figs 26.12 and 26.13: Dermal melanocytosisNevus of Ota in the periorbital area with scleral pigmentation

Fig. 26.14: Skin tagspedunculated soft lesions in


the axilla

Fig. 26.15: Granuloma pyogenicumpink colored


nodule bleeds on manipulation

Secondary milia are type of retention cyst that


arises due to damage to the epithelium and
occurs in diseases like bullous pemphigoid,
porphyria cutanea tarda and others following
trauma.
Treatment: Deroofing with hypodermic
needle, trichloroacetic acid cautery or
electrocautery.

Trichoepithelioma
Trichoepithelioma is benign appendageal
tumor with follicular differentiation.
They appear at puberty as solitary or
multiple, skin colored, translucent, rounded
nodules on the face (Fig. 26.17).
The lesions are distributed predominantly in
the nasolabial folds and eyelids.

Benign, Pre-malignant and Malignant Tumors of the Skin

Fig. 26.16: Miliawhite keratinous cysts over the face

Fig. 26.18: Syringomasmultiple angulated


papular lesions in the periorbital area

Clinically lesions are multiple, skin colored,


1-3 mm sized, angular papules (due to,
numerous small cystic ducts, as well as solid
epithelial strands in the upper dermis and
mid-dermis) distributed bilateral symmetrically, most commonly on the lower eyelids
(Fig. 26.18), less commonly over the chest and
neck.
Treatment with diathermy produces good
results.

A few telangiectatic vessels are often present


on the surface of the large lesions, which
resemble basal cell carcinoma.
Treatment with diathermy produces good
results.

Hypertrophic Scar
Injury or surgery in a predisposed
individual can result in an abnormally large
scar called as hypertrophic scar
A hypertrophic scar represents excessive
collagen deposition at the site of wound
healing
Typically, hypertrophic scar starts as an
asymptomatic, erythematous, smooth, firm
scar seen at anatomic locations
characterized by high tensions. With time,
they flatten and become white in color.
Hypertrophic scars do not extend beyond the
limits of the original trauma and heal by
6 months.

Syringoma
Benign tumors of eccrine differentiation
More common in females

Keloids
Keloids represent exaggerated collagen
deposition at the site of wound healing.

Fig. 26.17: Trichoepitheliomamultiple skin colored


translucent rounded nodules in the nasolabial folds

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Essentials in Dermatology

Fig. 26.19: Keloidindurated skin lesion showing


peripheral extensions

Fig. 26.20: Dermatofibromasingle skin colored


tender nodule over the shoulder

They frequently proliferate beyond the


wound margins onto the normal adjacent
skin (Fig. 26.19).
A keloid appears as a firm, mildly tender,
bosselated tumor present at a site of previous
injury. They are commonly pruritic,
erythematous, indurated lesions that show
extensions like claws of a crab. They may
even be the source of significant discomfort
or pain.
They rarely undergo involution.
Treatment: Medicaltopical steroids,
intralesional steroids and silicone gel
dressings. Surgical-surgical excision,
cryosurgery and skin grafts.

Glomus Tumor
This arises within the glomus body, a
neuromyoarterial receptor (the SucquetHoyer canal) that is sensitive to temperature
variations and regulates arterial blood flow.
These tumors, more common on the fingers
and toes and beneath the nail plate.
Usually seen in young adults between the
third and fourth decade of life.
They can be solitary or multiple.
Classically presents as solitary, small, blue
red nodules on the hand (nail bed) that
are characteristically associated with paroxysmal pain often elicited by changes in
temperature.
Simple excision- treatment of choice.

Dermatofibroma
Most common benign dermal fibrous tumor.
Typical dermatofibromas appear as skin
coloured to red-brown, firm, tender
nodule(s), most commonly seen on the
extremities (Fig. 26.20)
Lateral compression of the lesion with fingers
results in the depression on the topcalled
dimple sign
Histologically the fibroblasts and collagen
bundles are arranged in a storiform or
cartwheel pattern
Treatment of choicesimple excision.

PREMALIGNANT EPIDERMAL
TUMOURS
Actinic Keratosis (Senile or Solar
Keratosis)
Most common premalignant skin tumor.
These lesions occur in sun-damaged skin of
elderly people having light complexion
Clinically, these lesions are round to
irregular keratotic papules surrounded by
erythema.

Benign, Pre-malignant and Malignant Tumors of the Skin

Fig. 26.22: Erythroplasia of Queyratsharply defined


brightly erythematous velvety plaque over the glans
penis

Fig. 26.21: Actinic keratosesAlbino showing a


number of actinic keratoses lesions along with two
basal cell carcinoma lesions over the face

The principal sites are the back of the hands,


forearms and face (Fig. 26.21).
Squamous cell carcinoma can develop from
actinic keratosis.
Histologically, there may be vacuolization of
keratinocytes with numerous mitotic figures
seen involving the lower layers of the
epidermis (carcinoma in situ).
Treatment: Medical topical tretinoin and
topical 5-fluorouracil and surgical- curettage,
electrodessication and cryotherapy.

Erythroplasia of Queyrat
This condition affects uncircumscised males.
Seen in the fifth and sixth decades of life.
The lesions present as sharply defined,
brightly erythematous and velvety plaque
with moist glistening or granular surface
over the glans penis (Fig. 26.22).
Differentiated from benign inflammatory
dermatoses such as psoriasis, lichen planus,
Zoons plasma cell balanitis and fixed drug
eruptions.

Treatment with topical 5-fluorouracil


produces satisfactory results.

Bowens Disease
Refers to cutaneous plaques of
intraepidermal squamous cell carcinoma.
Chronic sunlight exposure, inorganic
arsenicals are important etiologic factors.
Clinically lesions of Bowens disease
appear as solitary, sharply defined, round
or oval to irregular erythematous
psoriasiform or eczematous plaque.
Ulceration is a sign of development of
invasive carcinoma.
Histology shows proliferating atypical
squamous cells through the full thickness of
the epidermis (wind- blown appearance)
The most effective treatment for Bowens
disease is surgical excision.
Cutaneous Horn (cornu cutaneum)
Cutaneous horn (cornu cutaneum) is the term
coined for horny skin excrescence, which in
its form and consistency resembles an
animal horn in miniature (Fig. 26.23)
The paramount consideration while making
a clinical diagnosis is the height of the

253

Essentials in Dermatology

254

MALIGNANT TUMORS OF THE SKIN

Fig. 26.23: Cutaneous hornhorny excrescence


resembling an animal horn in miniature

keratotic mass (at least one half of its largest


diameter). It is a reaction pattern of exaggerated hyperkeratosis and is rarely, if ever
seen nowadays. Its association with various
dermatoses may be informative.
The important issue is not the horn itself
which is dead keratin, but rather the nature
of underlying condition, which may be
benign (seborrheic keratoses, viral warts,
histiocytoma, inverted follicular keratosis,
verrucous epidermal nevus, organoid nevus,
ichthyosis hystrix, palmoplantar keratoderma, papilloma, keratoacanthoma, open
epidermoid and trichilemmal cysts, lichen
planus, corn and calluses, scar, molluscum
contagiosum), premalignant (solar keratosis,
arsenical keratoses, Bowens disease) or
malignant (squamous cell carcinoma, rarely,
basal cell carcinoma, metastatic renal
carcinoma, granular cell tumor, sebaceous
carcinoma or Kaposis sarcoma).
In the majority of cases, these horns are
benign.
Most commonly, they are single and arise
from a seborrheic keratosis lesion.
They are encountered most frequently on the
face and scalp, but may occur on the hands,
penis and eyelids.

Basal Cell Carcinoma (BCC) (Rodent


Ulcer)
Most common malignant skin tumor that
rarely metastasizes.
It is a malignant tumor of the skin that is
believed to arise from the hair follicle.
Its cells show a morphologic resemblance to
the relatively undifferentiated cells of the
basal layer of the epidermis.
BCC is more common in men.
Persons with fair complexion, having
outdoor occupation and chronic sun
exposure are at a higher risk to develop BCC.
The five clinical forms of BCC includethe
noduloulcerative (most common), superficial
type, pigmented type, sclerosing type and
fibroepithelioma.
The most common clinical presentation of
BCC is the noduloulcerative type, which
appears as a dome-shaped papule with a
telangiectatic surface and a pearly
translucent border (Figs 26.24 and 26.25). It
begins as small, shiny, waxy nodule increases
in size, undergo central ulceration
It is locally invasive without any metastasis
to regional lymph nodes
Treatment- surgical excision, electrodesiccation, irradiation.
Kaposis Sarcoma
Kaposis sarcomas (KS) are multisystem
vascular neoplasms, characterized by
mucocutaneous violaceous lesions and
edema as well as involvement of nearly any
organ
Human herpes virus type 8 has been
implicated in the pathogenesis
KS have been classified into five typesClassical type, African-endemic type,
iatrogenic type, immunosuppression associated type and HIV associated type
(Epidemic type)

Benign, Pre-malignant and Malignant Tumors of the Skin

Fig. 26.24

Fig. 26.25

Figs 26.24 and 26.25: Basal cell carcinomaulcerated nodule over the eyelid showing
pearly translucent border with telangiectasia

Presents as ecchymotic macules, which


become purple and brownish with
hemosiderotic halo. The lesion evolves into
patches, plaques and nodules and are often
arranged parallel to skin tension lines.
Classical KS lesion occurs on the leg at the
mean age of 60 years.
Oral lesion can be the first manifestation
of KS in the majority of cases, especially in
those having underlying HIV infection.
Histologically, a characteristic sign of KS is
the presence of solid cords and fascicles of
oval or spindle shaped cells arranged
between the jagged vascular channels. These
structures dissect the collagen bundles of the
entire dermis, leaving a spongy network of
collagen. This biphasic tumor morphology,
exhibiting both angiomatous and solid tumor
patterns, changes to a clear-cut sarcomatous
morphology.
Modalities of management include
radiation and chemotherapy.

Mycosis Fungoides
Mycosis fungoides is a form of cutaneous T- Cell
lymphoma (CTCL).
It is a chronic, slowly progressive disease that
evolves from patches (patch stage) (Fig. 26.6)

to plaques (plaque stage) and subsequently


nodules (tumor stage).
Pruritus is a prominent symptom.
The lesions coalesce and may ulcerate,
leading to deep ulcers.
Prognosis depends upon the TNM staging.
Diagnosis: Clinically lesions can be
confused with eczema and psoriasis.
Histopathology reveals atypical lymphocytes
infiltrating the epidermis without spongiosis
(epidermotropism). The pathognomonic
feature is presence of Pautrier microabscess
(collection of atypical lymphocytes in the
epidermis). The dermis has dense
monomorphous lymphomononuclear infiltrate with grenz zone.
Treatment: It depends on the stage of the
disease. Chemotherapy, retinoids, electron beam
therapy, photochemotherapy, etc. have been
used.

Szary Syndrome
Szary syndrome is a rare special variant of
cutaneous T-cell lymphoma characterized by a
triad of featureserythroderma, peripheral
lymphadenopathy and infiltrates of atypical
cells in the skin and blood.

255

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Essentials in Dermatology

Fig. 26.26: Mycosis fungoidesmultiple hypopigmented patches over the chest and abdomen

Fig. 26.27: Szary syndromeface showing


erythematous infiltrated plaques, in addition to diffuse
erythema

It occurs in people older than 60 years and


more commonly in males.
There is generalized erythroderma (Fig.
26.27) with diffuse hyperkeratosis of palms
and soles and diffuse hair loss.

Peripheral smear shows more than 10%


atypical lymphocytic cells in the blood.
Treatment is chemotherapy combined with
electron beam therapy or photochemotherapy.

Topical Formulary and Key Systemic Drugs

27

Topical Formulary and


Key Systemic Drugs

The use of topical remedies in the treatment of


disease is as old as medicine itself. Because the
skin is accessible and visible, it is uniquely
amenable to external chemical and physical
remedies.

Acute Exudative Dermatoses


(Erythema, edema, vesiculation, oozing, crusting,
infection, pruritus)

KEY POINTS IN TOPICAL THERAPY


From treatment point of view, most of the common
dermatoses fall into one of the two categories.
1. Dry dermatoses
2. Wet dermatoses
The popular saying in treatment is If the skin
is dry, wet it, or if the skin is wet, dry it. The most
effective way to dry skin that is involved with
acute exudative dermatoses is to apply cool, wet
compresses with or without astringents, followed
by open air or absorbent powders. Hence, for this
use lotions (aqueous solutions) or gels are the
vehicles of choice rather than ointments.
Conversely, the effective way to wet the skin as
in case of xerosis or chronic inflammatory and
papulosquamous skin lesions is to apply greasy
ointments, water in oil ointments or occlusive
wrappings.

Chronic Inflammatory Dermatoses


(Erythema, scaling, lichenification, dryness,
pruritus)
The term vehicle is used for those substances
that bring specific drugs in contact with the skin.
An ideal vehicle
1. Easy to apply and to remove.
2. Non-toxic.
3. Non-irritant.
4. Non-allergenic.
5. Chemically stable.
6. Homogeneous.

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Essentials in Dermatology
7. Bacteriostatic.
8. Pharmacologically inert.
9. Cosmetically acceptable.
For a layman, it should not Sting, stale/
stench and stain For any drug to be effective
topically, it must be formulated at the proper
concentration and in the proper vehicle.
In general, the most important vehicles for
topical use may be divided into monophasic,
biphasic, and triphasic forms.
There are three basic constituents of a
formulation, which are present singly or in
combination to form a vehicle.
They are:
1. Powders.
2. Greases.
3. Liquids.
When only one of the basic constituent is
present in a formulation vehicle, then it is called
monophasic vehicle (Powders, liquids or greases/
oils). In case two of them are present, it is called
biphasic vehicle(The combination of powder
with water gives either a drying paste or a shake
lotion. Grease or oil and powder will form either
a liniment or a protective (fatty) paste. Water and
grease, however, will mix to form emulsions in
the presence of a surfactant or emulsifier. These
then form oil in water (O/W) or water in oil
(W/O) creams) and if all the three are present, it
is called triphasic vehicle (Cooling pastes and
cream pastes are triphasic vehicles that consist of
oil-water-powder mixtures in varying proportion).

TERMINOLOGY IN TOPICAL
FORMULATION
Wet dressings are water or saline soaked cotton
gauze or cloth compresses used in the treatment
of acute inflammatory dermatoses with oozing,
weeping and crusting, bullous diseases and
ulcers.

Baths/soaks are a mode of drug delivery wherein


a part (soak) or the whole body (bath) is immersed
in medicated water.
Lotions are liquid formulations where the active
ingredient is present as powder suspended in
aqueous solution by aid of a suspending agent
known as shake lotion (need to be shaken before
use) or active ingredients are dissolved to clarity
called as clear solutions.
Gels are soft semisolid preparations that are
translucent to transparent in appearance. They
liquefy on contact with the skin and dry to leave
behind a thin greaseless film and are appropriate
for use on hairy areas.
Tinctures are alcoholic solutions with low
concentration of active ingredients, e.g. tincture
iodine.
Paints are medicated solutions which leave a
colored film on drying of the aqueous phase, i.e.
they cause staining, e.g. gentian violet paint,
Castellanis paint.
Ointments are semisolid soft to very firm greasy
preparations and employ lipophilic bases.
Petrolatum and white petrolatum are the most
widely used ingredients in the ointments.
Creams are semisolid soft to moderately firm
washable emulsions and are generally of the oil
in water type.
Pastes are preparations of about 50% powdered
ingredients in a greasy base such as petrolatum.
Water based preparation of powder form drying
pastes.
One gram of cream covers an area approximately 10 by 10 cm. An ointment spreads up to 10
percent further. According to Arndt, the amount
needed for the single application of a cream or
ointment to the face or hands is 2 gm; to one arm
or the anterior or posterior trunk, 3 gm; to one leg,
4 gm; and to the entire body, 30 gm.

Topical Formulary and Key Systemic Drugs

KEY SYSTEMIC DRUGSPENICILLINS


Originally derived from Penicillium notatum are
Beta-lactam antibiotic, inhibits transpeptidases
by binding to penicillin binding proteins (PBPs)
and thereby interferes with bacterial cell wall
synthesis.
Natural penicillins are benzyl penicillin
(Penicillin G), sodium penicillin G (crystalline
penicillin), and repository penicillin G (given
deep IM) (such as procaine penicillin G, and
benzathine penicillin G).
Semisynthetic penicillins
1. Acid resistant alternative to penicillin G
phenoxymethyl penicillin (Penicillin V).
2. Penicillinase resistant penicillins methicillin, oxacillin, cloxacillin.
3. Extended spectrum penicillins.
Aminopenicillins ampicillin, amoxycillin
Carboxypenicillins carbenicillin, ticarcillin
Ureidopenicillins piperacillin, mezlocillin
-lactamase inhibitors Clavulanic acid and
sulbactam prevent inactivation by penicillinases
produced by various organisms.
Spectrum of activity is against gram positive
cocci (non penicillinase-producing strains of
Staph. aureus, Staph. epidermidis, Streptococci except group D or enterococci), gram negative cocci
(Neisseria gonorrheae, Neisseria meningitides), gram
positive bacilli (B. anthracis, C. diptheriae, Cl. tetani,
Listeria), and spirochetes (Treponema pallidum).
Majority of gram negative bacilli are totally
insensitive to penicillin.
Penicillin G is rapidly degraded by gastric
acid (so preferred for IV administration). Penicillin
V is preferred for oral administration. Benzathine
and procaine penicillins are used for slow release
(IM administration). Excretion mainly occurs via
tubular secretion (dose adjustment in renal
insufficiency).

Dosage
Sodium penicillin G (crystalline penicillin):
0.5-5 MU IM/IV 6-12 hourly.
Procaine penicillin G: 0.5-1 MU IM 12-24
hourly.
Benzathine penicillin G: 0.6-2.4 MU IM every
2-4 weeks.
Indications
Syphilis.
Streptococcal infections.
Tetanus.
Gas gangrene.
Adverse Effects/ Precautions
Hypersensitivity reactions ranging from mild
rash/fever/eosinophilia to fatal anaphylaxis.
Jarisch-Herxheimer reaction occurs within
2-12 hours of the first dose when using
penicillin for syphilis; presents as headache,
fever, chills, sweating, sore throat, myalgia/
arthralgia, tachycardia and increased blood
pressure. The condition does not recur and
does not need interruption of treatment.
Aspirin and sedation offer symptomatic relief.
Pregnancy: category B.
Drug Interactions
Probenecid inteferes with tubular secretion
of penicillin.
AMPICILLIN
Pharmacology
-lactam antibiotic, aminopenicillin group.
Interferes with bacterial cell wall synthesis.
Greater activity than natural penicillins
against enterococci and H.influenzae.
Maximal absorption when taken on empty
stomach.
Primarily excreted in urine (dose adjustment
in renal insufficiency).

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Dosage
Oral
Adults and children >20 kg 250-500 mg Q6h.
Children <20 kg 50-100 mg/kg/day in
divided doses Q6h.

Parenteral
Adults 500-1500 mg IM/ 500-3000 mg IV
Q4-6h (maximum 12 g/day).
Children 100-400 mg/kg/day IM/IV in
divided doses Q4-6h.

Indications
Skin and skin structure infections.
Adverse Effects/Precautions
Hypersensitivity reactions (eosinophilia or
rash).
Maculopapular rash (concomitant with viral
infections, e.g. infectious mononucleosis).
GIT nausea, vomiting, abdominal pain,
diarrhea.
Black hairy tongue, glossitis, stomatitis, sore
tongue.
Pregnancy: category B (FDA).
Drug Interactions
Probenecid- interferes with excretion of
ampicillin.
OCPs- Ampicillin may decrease the efficacy
of OCPs.
Allopurinol-Concomitant allopurinol
increases the risk of ampicillin rash.
CEPHALEXIN
Pharmacology
First generation cephalosporin; -lactam
antibiotic.
Same mechanism of action as penicillin but
binds to different proteins.
Well absorbed orally.
Attains high concentration in bile.
Excreted unchanged in urine.

Dosage
Adults: 250-500 mg Q6h (maximum: 4 g/day).
Children: 25-50 mg/kg/day in divided doses
Q6h.
Indications
Skin and skin structure infections (staphylococcal/streptococcal).
Genitourinary tract infections (E. coli/P.
mirabilis/K. pneumoniae).
Adverse Effects/Precautions
Generally well tolerated.
GIT- nausea, vomiting, diarrhea.
Hypersensitivity reactions (cross allergenicity
with penicillin).
Pregnancy: category B.
ERYTHROMYCIN
Pharmacology
Macrolide antibiotic; binds to the 50S ribosome
subunit and interferes with protein synthesis.
Activity against mostly gram positive, and few
gram negative organisms-Streptococcus
pyogenes, Streptococcus pneumoniae, some Staph.
aureus, N.gonorrheae, N.meningitides, B.pertussis,
Treponema pallidum, Mycoplasma, Legionella,
Chlamydia and Campylobacter.
Available as several different derivatives
estolate salt is the least susceptible to acid
degradation and the best absorbed salt,
compared to the base, stearate and ethylsuccinate formulations.
Maximum absorption when taken on empty
stomach (except estolate taken with food).
Extensively metabolized in the liver, and
excreted in bile.
Penetration of CSF is negligible.
Dosage
All derivatives except ethylsuccinate: 250-500 mg
every 6 hours (expressed in terms of base)
maximum 4g/day.

Topical Formulary and Key Systemic Drugs


Ethylsuccinate: 400 mg is considered equivalent
to 250 mg of base.

Indications
As an alternative to penicillin for
Skin and skin structure infections of mild
to moderate severity caused by Strept.
pyogenes or Staph. aureus.
Erythrasma.
Streptococcal respiratory tract infections.
Diphtheria.
Tetanus.
Syphilis and gonorrhea.
Chlamydial infections.
As a first choice drug for
Chancroid.

Adverse Effects/Precautions
GIT- nausea, vomiting, diarrhea.
Allergic reactions (more common with
estolate).
Reversible cholestatic hepatitis (with estolate).
QT prolongation.
Estolate derivative contraindicated in hepatic
dysfunction; all others used with caution.
Pregnancy: category B.

Drug Interactions
CYP3A4 inhibitor - Increased levels of
theophylline, warfarin, phenytoin, carbamazepine, disopyramide and cyclosporine.
Increased risk of myopathy when used with
HMG CoA reductase inhibitors (atorvastatin,
simvastatin, lovastatin).

Expanded spectrum of activity compared to


erythromycin: retains similar gram positive
cover, but has greater gram negative cover
(N. gonorrheae, N. meningitidis, H. ducreyi, H.
influenzae, M. catarrhalis, B. pertussis).
More acid stable than erythromycin.
Widely distributed; persists in pulmonary
macrophages, polymorphonuclear leukocytes
and genital/pelvic tissues for several days.
Negligible penetration of CSF.
Excreted primarily in feces.

Dosage
Adults: 500 mg on day 1, followed by 250 mg
once a day for 5 days.
Chlamydia and chancroid: 1g single dose.
Indications
Skin and skin structure infections (Staph.
aureus, Strep. pyogenes, Strep. agalactiae).
Nongonococcal urethritis and cervicitis
(Chlamydia).
Prevention of disseminated M. avium complex
disease in HIV infected patients.
Chancroid.
Adverse Effects/ Precautions
GIT- nausea, vomiting, diarrhea, abdominal
pain.
Rare allergic reactions.
Reversible elevation of LFT.
Pregnancy: category B.
Drug Interactions
Does not affect cytochrome P450 enzymes.
Warfarin increased PT and INR can occur;
close monitoring required.

AZITHROMYCIN
Pharmacology
Macrolide antibiotic; inhibits bacterial protein
synthesis by binding to the 50S ribosomal
subunit.

TETRACYCLINE
Pharmacology
Inhibits protein synthesis by binding to the
30S ribosomal subunit, thereby preventing

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Essentials in Dermatology
attachment of the aminoacyl t-RNA to the A
site of the mRNA-ribosome complex; primarily
bacteriostatic.
In acne inhibits growth of propionibacterium acnes and decreases free fatty acid
concentration in sebum, resulting in
decreased inflammation and microcomedo
formation.
Maximal absorption on empty stomach (food
decreases absorption by 50% or more).
Eliminated primarily unchanged in urine
(needs dose adjustment in renal insufficiency).

Dosage
Acne vulgaris 500-1000 mg/day in 4 divided
doses for 1-2 weeks or till clinical improvement, followed by 125-500 mg/day.
Syphilis.
primary or secondary: 500 mg QID for 2
weeks
latent: 500 mg QID for 2 weeks (if duration
of infection < 1 year) 500 mg QID for 4
weeks (duration of infection unknown or
>1 year)
Indications
Acne vulgaris.
Primary, secondary or latent syphilis in nonpregnant patients with documented penicillin
allergy.
Adverse Effects/ Precautions
GIT - nausea, vomiting, diarrhea, abdominal
pain, epigastric burning, anorexia.
Maculopapular or erythematous rashes.
Hypersensitivity reactions.
Hemolytic anemia, thrombocytopenia,
neutropenia and eosinophilia.
Photosensitivity.
Precipitation of renal failure in patients with
underlying renal function.
Discoloration of teeth (in children < 8 years,
or infants of pregnant mothers who ingested
tetracycline).

Superinfection with Candida (oral and


anogenital).
Use of discarded or outdated tetracycline has
resulted in an adverse event similar to
Fanconis syndrome (patients should be
instructed to discard any unused tetracycline
after therapy).
Pregnancy: category D.

Drug Interactions
Absorption of tetracycline is impaired by dairy
products, flouroquinolones, sucralfate, or any
product containing iron, zinc, calcium,
aluminium, magnesium or bismuth (separate
administration by 4 hours).
OCPs decreased efficacy of OCPs.
Warfarin elevated PT.
DOXYCYCLINE
Pharmacology
Inhibits protein synthesis by binding to the
30S ribosomal subunit, thereby preventing
attachment of the aminoacyl t-RNA to the A
site of the mRNA-ribosome complex; primarily
bacteriostatic.
In acne inhibits growth of propionibacterium acnes and decreases free fatty acid
concentration in sebum, resulting in
decreased inflammation and microcomedo
formation.
Spectrum of activity.
Cocci: all gram positive and gram negative
cocci were originally sensitive but now
many Strep. pyogenes, Strep. pneumoniae,
Staph. aureus and enterococci have become
resistant
Gram positive bacilli: Clostridia, Listeria,
Corynebacteria, Propionibacterium acnes,
Bacillus anthracis
Gram negative bacilli: H.ducreyi,
Calymmatobacterium granulomatis, Vibrio
cholerae, Yersinia pestis, Yersinia
enterocolitica, Campylobacter, H. pylori,
Brucella, Pasturella, Francisella

Topical Formulary and Key Systemic Drugs


Spirochetes: Treponema pallidum, Borrelia
Rickettsiae
Chlamydia
Mycoplasma, Ureaplasma
Maximum absorption on empty stomach (food
may decrease absorption by up to 20%);
should be taken with adequate fluids to
reduce esophageal irritation/ulceration; can
be given with food if gastric irritation occurs.
Equally excreted in feces and urine (unlike
other tetracyclines); dose adjustment not
needed in renal insufficiency.

Dosage
100 mg twice a day.
Indications
Acne vulgaris
1st choice treatment in:
Lymphogranuloma venereum
Granuloma inguinale
Typhus, Rocky mountain spotted fever, Q
fever (Rickettsiae)
2nd choice treatment in:
Tetanus, anthrax, actinomycosis, Listeria
(to penicillin/ampicillin)
Gonorrhea (to ciprofloxacin/ceftriaxone,
especially for penicillin-resistant, nonPPNG organisms and in penicillin allergic
cases)
Chlamydial infections non-gonococcal
urethritis, endocervicitis, conjunctivitis,
pneumonia (to azithromycin)
Chancroid (to cotrimoxazole)

Adverse Effects/Precautions
GIT- nausea, vomiting, diarrhea, abdominal
pain, epigastric burning, anorexia.
Pseudotumor cerebri (increased risk when
combined with isotretinoin).

Photosensitivity.
Discoloration of teeth (in children < 8 years
and infants of pregnant women taking the
drug).
Superinfection with Candida (oral/ anogenital), pseudomembranous enterocolitis.
Pregnancy: category D.

Drug Interactions
Absorption of doxycycline is impaired by dairy
products, flouroquinolones, sucralfate, or any
product containing iron, zinc, calcium,
aluminium, magnesium or bismuth (separate
administration by 1-2 hours).
OCPs decreased efficacy of OCPs.
Warfarin elevated PT.
MINOCYCLINE
Pharmacology
Inhibits protein synthesis by binding to the
30S ribosomal subunit, thereby preventing
attachment of the aminoacyl t-RNA to the A
site of the mRNA-ribosome complex; primarily
bacteriostatic.
In acne inhibits growth of Propionibacterium
acnes and decreases free fatty acid
concentration in sebum, resulting in
decreased inflammation and microcomedo
formation.
Good activity against rickettsia, chlamydia,
mycoplasma.
Also displays activity against Staph. aureus
resistant to other tetracyclines.
Maximum absorption on empty stomach (food
may decrease absorption by up to 20%);
should be taken with adequate fluids to
reduce esophageal irritation/ulceration; can
be given with food if gastric irritation occurs.
Eliminated primarily via the hepatobiliary
system.

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Dosage
Acne vulgaris: 50 mg OD TID.
Infections due to susceptible organisms: 200
mg initial dose (orally or IV), followed by 100
mg Q12h.
Indications
Moderate to severe inflammatory acne
vulgaris.
Can be used to treat rickettsial, chlamydial,
mycoplasmal infections (although doxycycline is the preferred drug).
Adverse Effects/ Precautions
GIT nausea, vomiting, diarrhea, abdominal
pain, epigastric burning, anorexia.
Vestibular side effects dizziness, ataxia,
vertigo (dose-related, more common in
women).
SLE rare, reversible.
Pseudotumor cerebri (increased risk when
combined with isotretinoin).
Reversible skin and mucous membrane
pigmentation occurs with long-term use
Photosensitivity may be less than other
tetracyclines.
Discoloration of teeth (in children < 8 years
and infants of pregnant women taking the
drug).
Superinfection with Candida (oral and
anogenital) less frequent than tetracycline and
doxycycline.
Pregnancy: category D.
Drug Interactions
Absorption of minocycline is impaired by
sucralfate, or any product containing iron,
zinc, calcium, aluminium, magnesium or
bismuth (separate administration by 1-2
hours).
OCPs decreased efficacy of OCPs.
Warfarin elevated PT.

CIPROFLOXACIN
Pharmacology
Fluoroquinolone antibiotic; inhibits bacterial
DNA gyrase.
Most reliable for treatment of aerobic gram
negative organisms.
Enterobacteriaceae
Neisseria gonorrheae, Neisseria meningitides
H. influenzae, H. ducreyi
Campylobacter jejuni
Yersinia enterocolitica
Moderately susceptible organisms include
some streptococci, staphylococci, Pseudomonas
aeruginosa, chlamydia, and mycobacteria.
Well absorbed after oral administration.
Widely distributed throughout the body; high
tissue penetrability (except CSF).
Eliminated primarily via the kidney (dose
adjustment needed in renal insufficiency).

Dosage
250-750 mg BD, depending on the indication.

Indications

Skin and skin structure infections.


Gonorrhea.
Bone and joint infections.
Urinary tract infections (cystitis, pyelonephritis).
Intra-abdominal infections, diarrhea (in
combination with metronidazole).
Typhoid.
Sinusitis, lower respiratory tract infections.

Adverse Effects/ Precautions


GIT nausea, vomiting, bad taste, anorexia.
CNS dizziness, headache, restlessness,
anxiety, insomnia, impairment of concentration and dexterity (caution while driving),
tremor, seizures.

Topical Formulary and Key Systemic Drugs


Hypersensitivity reactions rash, pruritus,
photosensitivity, urticaria, swelling of lips,
fever, hepatic necrosis, fatal anaphylaxis.
Tendonitis and tendon rupture.
Cartilage damage in weight bearing joints (in
animal studies) not recommended for
children.
Pregnancy: category C.

Not effective against Pseudomonas, enterococci,


and most anaerobes
Increased resistance being seen with
H. influenzae, E. coli, staphylococci and
streptococci
Well absorbed orally.
Excreted primarily in urine (dose adjustment
needed in renal insufficiency).

Drug Interactions
Products containing multivalent cations such
as iron, calcium, zinc, magnesium, aluminium
(e.g. antacids, multivitamins, sucralfate)
impair the absorption of ciprofloxacin
should be administered 6 hours before or 2
hours after ciprofloxacin.
Theophylline ciprofloxacin can increase
theophylline concentrations.
Warfarin increased PT.

Dosage
All preparations contain TMP and SMX in the
ratio of 1:5.
Oral one double strength TMP/SMX (160/
800 mg) tablet BD.
Intravenous 8-20 mg/kg/day of TMP in 2-4
divided doses.

TRIMETHOPRIMSULPHAMETHOXAZOLE (SEPTRAN)
Pharmacology
Trimethoprim is a diaminopyrimidine related
to the antimalarial drug pyrimethamine,
which selectively inhibits bacterial dihydrofolate reductase (DHFRase).
Sulphamethoxazole belongs to the class of
sulfonamides, which are structural analogues
of PABA, and thereby inhibit bacterial folate
synthetase.
The combination (TMP-SMX) results in a
synergistic bactericidal effect on gram positive
and gram negative organisms
Strep. pneumoniae
Strep. pyogenes
Staph. aureus
H. influenzae
Moraxella catarrhalis
Nocardia species
Stenotrophomonas maltophila
Enterobacteriaceae (most)
Pneumocystis jiroveci

Indications
Chancroid (1st choice agent).
Granuloma inguinale (2nd choice agent).
Toxoplasmosis.
Nocardia infections.
Urinary tract infections treatment and
prophylaxis.
Respiratory tract infections (acute otitis media,
acute exacerbations of chronic bronchitis,
sinusitis).
Diarrhea and dysentery.
PCP pneumonia.
Adverse Effects/ Precautions
GIT nausea, vomiting, anorexia.
Hypersensitivity rash, urticaria, StevensJohnson syndrome, TEN, erythema multiforme
and exfoliative dermatitis.
Agranulocytosis, aplastic or hemolytic
anemia (folate deficient patients at increased
risk).
Fulminant hepatic necrosis.
HIV infected patients are at much greater risk
of dermatologic and hematologic reactions,
and desensitization may be required.
Crystalluria (advised to drink plenty of water).
Pregnancy: category C.

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Drug Interactions
Warfarin antithrombotic effects can be
potentiated by TMP/SMX.
Sulfonylureas hypoglycemia as a result of
displacement from protein binding sites.
Methotrexate displacement from protein
binding sites, and competition for renal
tubular excretion, leading to bone marrow
suppression.
CORTICOSTEROIDS
The corticosteroids bind to specific cytoplasmic
receptors in the target cell and the steroid receptor
complex then undergoes modification and is
translocated to the nucleus where it causes
formation of specific mRNA which then mediates
the various biological effects. Their modes of
actions are 1) Anti-inflammatory, 2) Antiallergic
and 3) Immunosuppressive.

Dosage
The intermediate acting steroid namely
prednisolone is commonly used at a dose of 1
mg/kg body weight/day.
Indications
1. Autoimmune bullous dermatoses (pemphigus, bullous pemphigoid, epidermolysis
bullosa acquisita, etc.).
2. Drug eruptions (erythema multiforme, Stevens
Johnson syndrome, TEN).
3. Connective tissue disorders (SLE, polymyositis, dermatomyositis, progressive
systemic sclerosis).
4. Severe dermatitis (atopic dermatitis, contact
dermatitis, photodermatoses).
5. Neutrophilic dermatoses (pyoderma gangrenosum, Sweets syndrome).
6. Sarcoidosis.
7. Leprosy in severe reaction.
8. Urticaria, angioedema and capillary hemangiomas.

Contraindications
There are no absolute contraindications
The relative contraindications are: Peptic
ulcer disease, any focus of infection including
tuberculosis, hypertension, glaucoma, cataract,
hyperlipidemia, renal calculi, first trimester of
pregnancy, and children.
Side Effects
1. Metabolic disturbances: Diabetes, faciotruncal obesity, hyperlipidemia, protein
hypercatabolism, etc.
2. Bone disturbances: Osteoporosis, avascular
necrosis of the bone, growth retardation.
3. Muscular disorders: Steroid myopathy,
amyotrophy, tendon rupture.
4. Cutaneous effects: Acneiform eruptions,
atrophy, telangiectasia, easy bruising,
alopecia/hypertrichosis, cutaneous
infection, striae distense.
5. Electrolyte disturbances: Sodium retention
and potassium depletion.
6. Endocrine changes: Inhibition of the
hypothalamo-hypophyseal suprarenal axis.
7. Infections: Acute bacterial infection,
tuberculosis, viral infection, parasitic
disease.
8. Neuropsychiatric disorders: Neuropathy,
psychosis, pseudotumor cerebri.
9. Ocular effects: Posterior subcapsular
cataract, open angle glaucoma, aggravation
of an existing infection.
10. Gastric disturbances: Peptic ulcer disease.
11. Hematologic alteration: Leucocytosis,
eosinopenia and lymphopenia.
GRISEOFULVIN
Griseofulvin is the most frequently used systemic
drug in the treatment of dermatophytosis. It is a
fungistatic agent derived from Penicillium
griseofulvum with selective activity against
dermatophytes only. In addition to its antifungal

Topical Formulary and Key Systemic Drugs


activity, it is also a weak vasodilator and inhibits
leucocyte chemotaxis.

herpes zoster, the dose is 800 mg orally 5 times a


day for 7 to 10 days.

Dosage
The daily recommended dose is 10-20 mg/kg
body weight/day to a maximum of 1 gm daily,
given in a single dose or in two divided doses.
For ringworm of the trunk, hand or foot, it is given
for a period of 4 to 6 weeks. For scalp infection 8
to 12 weeks and in nail infection for 12 to 18
months.

Side Effects

Side Effects
The most commonly reported adverse effects are
headache and gastrointestinal side effects namely
dyspepsia, nausea and diarrhoea. Other side
effects are photosensitivity, urticaria, erythema
multiforme, maculopapular skin rash, serum
sickness, angioedema and vesicular eruption. The
other uncommon but serious adverse effects
include hepatic and renal insufficiency, severe
leukopenia, peripheral neuropathy, mental
confusion and blurred vision. It can also
precipitate SLE.
ACYCLOVIR
Acyclovir is a synthetic guanosine analogue
which is widely used for the treatment of herpes
simplex virus and varicella zoster virus infections.
Acyclovir, a prodrug, initially undergoes
monophosphorylation by herpes virus encoded
thymidine kinase and is activated to acyclovir
triphosphate (by cellular kinase) which is the
active antiviral moiety. Acyclovir is highly
selective because only virus infected cells are able
to phosphorylate acyclovir.

Dosage
The recommended dose for the treatment of HSV
infection is 200 mg 5 times a day or 400 mg three
times a day for 7 to 10 days. For varicella and

They are renal crystalluria, interstitial nephritis,


seizures, tremors, etc.

ANTIHISTAMINES
Classification
Class I: First generation H1 type antihistamines
These traditional antihistamines are competitive
receptor blockers (reversible) and can be displaced
by high levels of histamines and they dissociate
from the receptors easily. They are divided into 6
groups.
1. Alkylamine
Pheniramine maleate (25-50 mg 2-3 times
daily)
Chlorpheniramine maleate (4 mg thrice
daily)
2. Ethanolamine
Clemastine fumarate (1 mg twice daily)
Embramine hydrochloride (25-50 mg/
day)
Bromodiphenyl hydramine hydrochloride
Diphenhydramine hydrochloride(25-50
mg thrice daily), citrate
3. Ethylenediamine
Mepyramine maleate
Pyrilamine maleate
Tripelamine citrate, HCl
4. Phenothiazine
Promethazine HCl (25 mg twice daily)
Methdilazine HCl
5. Piperidine
Cyproheptadine HCl (4 mg thrice daily)
Azatidine maleate
6. Piperazine
Hydroxyzine hydrochloride (10 mg or 25
mg thrice daily).

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Mechanisms of action: The main effects are as
follows:
1. Anticholinergic activity
2. Anti-inflammatory effect
3. Antiallergic effect
4. Antimotion sickness effects
5. Antiemetic activity
6. Inhibit most responses of smooth muscle to
histamine.

Indications

Side Effects
1. Neurological side effects Sedation, dizziness
and tinnitus, blurred vision and diplopia,
insomnia, reduced or disturbed concentration,
irritability and tremors.
2. Gastrointestinal side effects Anorexia,
nausea, vomiting, epigastric distress, constipation (can be minimized by giving them along
with food).
3. Anticholinergic effects dry mucous
membranes, difficulty in micturition and
urinary retention, frequency and dysuria,
impotence.
4. Cutaneous side effects eczematous dermatitis, fixed drug eruption, photosensitivity,
urticaria, petechiae.

3. Mast cell disorders: Ketotifen, cimetidine,


azelastine and chlorpheniramine are very
effective.

Class II: Second generation H1 antihistamines.


They are non-competitive blockers and
dissociate slowly from the receptors and have a
longer duration of action. They are devoid of
sedative and anticholinergic effects. These drugs
are: acrivastine, astemizole, azelastine, cetirizine,
ebastine, fexofenadine, ketotifen, loratidine,
mizolastine, terfenadine.
The drugs commonly used are cetirizine (10
mg/day), loratidine (10 mg/day), terfenadine (60
mg BD) and astemizole (10 mg/day), with a
loading dose of 10 mg tds x 3 days).
Class III: H 2 antihistamines Cimetidine,
ranitidine, famotidine, nizatidine.
Class IV: Other drugs with antihistamine activity
Tricyclic antidepressants (doxepin), ketotifen,
oxafomide.

1. Urticaria and angioedema: Both traditional


and second generation antihistamines are found
to be useful. The combination of both H1 and H2
antihistamines are very effective in the treatment
of acute or chronic idiopathic urticaria and
physical urticarias.
2. Pruritus of various causes: viz. dermatitis,
papulosquamous disorders, insect bites, etc.

4. Pruritus due to: Myelofibrosis, polycythemia


vera and carcinoid flush. Cimetidine is also
effective.
5. The immunomodulatory effects of H 2
antihistamines may offer great therapeutic
potential.
6. Other uses include, their use in motion
sickness, vertigo, preanesthetic medication, as
sedative, hypnotic and anxiolytic.

DAPSONE
Dapsone is a 4,4-Diaminodiphenyl sulphone. It
is weakly bactericidal against M.leprae. It acts by
inhibiting folate metabolism in M.leprae. In
addition to its antibacterial action, it also has antiinflammatory activityinhibits lysosomal
enzymes, interferes with myeloperoxidase system.
During daily treatment with 100 mg dapsone, it
inhibits the generation of 5-lipoxygenase product
in neutrophils.

Dosage
Dose is 6 to 10 mg/kg body weight/week. The
average adult dosage is 100 mg/day. For children
of 10 to 14 years, it is 50 mg/day and of 6 to 9
years it is 25 mg/day.
Indications
Other than its antileprosy action, it is used in
dermatology in pemphigus, bullous pemphigoid,

Topical Formulary and Key Systemic Drugs


dermatitis herpetiformis, subcorneal pustular
dermatosis, acne conglobata, systemic lupus
erythematosus, vasculitis, lichen planus etc.

Side Effects
1. Hemolytic anemia especially in G6PD
deficient individuals.
2. Agranulocytosis.
3. Hepatitis.
4. Allergic rashes including FDE and exfoliative
dermatitis.
5. Dapsone syndrome (fever, jaundice, lymphadenopathy, hepatomegaly and exfoliative
dermatitis).
6. Psychosis.
CLOFAZIMINE
Clofazimine is a riminophenazine dye. Like
dapsone, it is also weakly bactericidal against
M.leprae and virtually nontoxic in the usual
dosage used. Clofazimine has both antibacterial
and anti-inflammatory activities.

Indications
Apart from its use in multidrug therapy in leprosy,
it is used in dermatology for multidrug resistant
tuberculosis, atypical mycobacterial infection,
neutrophilic dermatoses (acne, pustular
psoriasis), rhinoscleroma, leishmaniasis, etc.

Dosage
The recommended dose is 50 mg daily.

Side Effects
1. Reversible dose related reddish to brownish
black discoloration of sweat, hair, sputum,
urine and feces.
2. Ichthyosis.
3. Phototoxicity, nonspecific skin rashes and
acneiform eruptions.

4. Eosinophilic enteropathy.
5. Conjunctival pigmentation.

METHOTREXATE
Methotrexate (MTX) is a folic acid antagonist and
is widely used for the treatment of severe
psoriasis.

Dosage
1. Weekly single oral or parenteral dosage
schedule.
2. Intermittent oral schedule of three divided
doses 12th hourly over a period of 36 hours
each week.
3. Oral dose is 0.4 to 0.6 mg/kg body weight/
week (maximum 30 mg).
4. Parenteral dose is 15-25 mg/week.
Indications
1. Psoriasis psoriatic erythroderma, psoriatic
arthritis, pustular psoriasis, extensive
psoriasis vulgaris.
2. Reiters syndrome and pityriasis rubra pilaris.
3. Sarcoidosis.
4. Polymyositis and systemic lupus erythematosus.
5. Pemphigus vulgaris and other bullous
disorders.
Side Effects
1. Gastrointestinal nausea, vomiting, stomatitis, diarrhoea, ulceration, etc.
2. Hepatitic abnormal liver enzymes, hepatic,
fibrosis and cirrhosis.
3. Hematopoietic anemia, thrombocytopenia,
leucopenia, pancytopenia.
4. Pulmonary acute hypersensitivity, fibrosis,
pneumonia.
Most common side effect is nausea and
vomiting. Serious late side effect is cirrhosis of
the liver.

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PSORALENS
Of the many psoralens isolated and synthesized,
three are in routine clinical use. These include 8
methoxypsoralen (8-MOP), 5 methoxypsoralen
(5-MOP) and trimethyl psoralen (TMP). They are
active only when combined with UVA irradiation.
It interferes with DNA synthesis and blocks
epidermal cell proliferation, has immunomodulatory effects and immunosuppressive effects
along with induction of epidermal thickening and
melanogenesis.

Dosage
Oral 8-MOP is given in a dose of 0.6 to 0.8 mg/kg
body weight/day. Two hours later, the patient is
exposed to ultraviolet radiation A (UVA) at a dose
of 1 joule/square cm to begin with, which is
gradually increased to minimal erythema dose
depending on the skin type. Therapy is usually
given two to three times per week. The number of
exposures required for achieving control are
usually between 15 and 25. Maintenance therapy
involves less frequent treatments often as little as
once every two to four weeks, with eventual
discontinuation of treatment.
Indications
1. Psoriasis.
2. Vitiligo.
3. Photodermatoses.
4. Cutaneous T-cell lymphomas.
Side Effects
1. Nausea, vomiting.
2. Cutaneous Acute sunburn and erythema,
irreversible hyperpigmentation of the skin,
PUVA lentigines, actinic keratoses, premature
ageing of the skin.
3. Ocular cataract. It can be prevented by using
sunglasses on the day of treatment.
4. Carcinogenesis.

Other than oral PUVA therapy, psoralen may


be used for topical PUVA therapy and bath PUVA
therapy.

RETINOIDS
Retinoids is a generic term that includes both
naturally occurring molecules and also synthetic
compounds showing specific biological activities
resembling those of vitamin A(retinol).
Synthetic retinoids are produced by chemical
modification of vitamin A. Three generations are
known today(nonaromatic, monoaromatic, and
polyaromatic).
First generation(Nonaromatic) includes retinal and
compounds that can be derived from it
metabolically e.g. retinyl palmitate(topical),
retinyl aldehyde(topical), tretinoin(all trans
retinoic acid)(topical), 9-cis-retinoic acid, a-14hydroxyretroretinol, fenretinide(N-[4-hydroxyphenyl]-retinamide), E 5166(polyprenoic acid).
Second generation(Monoaromatic) by addition of an
aromatic ring e.g. etretinate, isotretinoin, acitretin,
isoacitretin(13-cis-acitretin), motretinide.
Third generation(Arotinoids) e.g. temarotene,
arotenoid acid, arotenoid ethyl ester, arotenoid
ethyl sulphone, arotenoid methyl sulphone,
adapalene(topical antiacne agent), tazarotene
(topical antipsoriatic agent).
Intracellularly retinoids interact with cytosolic
proteins and then enter the nucleus. By changing
the expression of growth factors, oncogenes,
keratin or transglutaminases, retinoids could
exert wide spread changes in growth and
differentiation.

Indications of Systemic Retinoids


1. Psoriasis and related disorders.
2. Other disorders of keratinization.
3. Seborrhoea, acne and acneiform dermatoses.
4. Rosacea and other acne related dermatoses.

Topical Formulary and Key Systemic Drugs


5. Cancers-basal cell carcinoma, actinic
keratosis, cutaneous T-cell lymphomas, HIV
related Kaposis sarcoma.
6. Other dermatoses such as lichen planus,
lupus erythematosus, lichen sclerosus et
atrophicus, prurigo nodularis, photoageing
and ageing, pigmentary disorders, etc.

Side Effects of Systemic Retinoids


The adverse effect profile closely resembles those
of hypervitaminosis A.
Acute toxicity is dose dependent and reversible.
1. Cheilitis appears 2-3 weeks after initiation of
therapy in 100% of patients and is regarded
as a marker of sufficient absorption. Other
mucocutaneous adverse effects are skin and

mucosal dryness, skin fragility and/or


stickiness, retinoid dermatitis, palmoplantar
desquamation, pruritus, hair loss, paronychia, and photosensitivity.
2. Eye symptomatology and pseudotumor
cerebri.
3. Serum lipids and liver function abnormalities.
Chronic Toxicity
1. Bone changes include hyperostosis, periostosis, osteoporosis, thinning of the bones,
premature closure of epiphyses, DISH(diffuse
idiopathic skeletal hyperostosis), extraspinal
calcification, bone pain and acute arthritis.
2. Arthralgia and myalgias
3. Teratogenicity involving central nervous
system, skeletal system, cardiovascular
system, and other organs.

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28

Dermatosurgical Procedures

SKIN BIOPSY
Skin biopsy is the commonest investigation
performed by any dermatologist. The diseased
tissue obtained is subjected to microscopic and/
or other investigations.

Indications
1. Most frequently skin biopsy is taken to
confirm a clinical diagnosis or to aid in the
establishment of a diagnosis where clinical
diagnosis is not apparent.
2. Sometimes, biopsy is excisional for the
treatment of skin lesions particularly
malignant neoplasms and other lesions for
cosmetic reasons.
3. Skin biopsy may be used for a variety of
investigative procedures such as ultrastructural examination, immunofluorescence
studies, enzyme histochemistry and
immunohistochemistry, microbiological
studies, tissue culture, etc.
Site of biopsy: Ideally, the lesion biopsied should
be an early and untreated lesion and representative of the skin disorder as a whole. If lesions
are present at all stages of evolution, it may be
appropriate to biopsy more than one lesion.
Normal skin should be included with a
diagnostic biopsy wherever possible.

Written consent is normally obtained from


the patient and local anesthesia with 1% or 2%
lignocaine with or without adrenaline is injected
around the biopsy site.
Techniques of skin biopsy: Elliptical surgical
biopsy, punch biopsy, curettage or shave biopsy
may be undertaken.
Elliptical surgical biopsy: A reasonable size for
an elliptical biopsy is about 12 x 5 mm. It is
undertaken with a scalpel and the wound is
sutured.
Punch biopsy: The biopsy punch of at least 5
mm is required to obtain satisfactory specimen.
The punch is pushed into the skin with a
downward twisting movement and then
removed. The tissue specimen is lifted and cut.
The wound may be left to heal without suturing.
The biopsy specimen is put in 10% formalin
solution for histopathologic studies. It is
properly labeled and sent to pathology
department with full clinical details of the case.

ELECTROSURGICAL PROCEDURES IN
DERMATOLOGY
Electrical current of sufficiently high frequency
not to stimulate nerves or muscles can pass
through tissue with little effect other than the

Dermatosurgical Procedures
production of heat. Heat is produced from the
electrical resistance of the tissue as current travels
from one electrode to the other. When the
electrodes are both large (as in medical
diathermy), the heat is dispersed over a large
area, and no injury ensues. However, if the
emitting electrode is reduced to a small tip, the
heat produced at the point of contact is
sufficiently intense to cause localized tissue
injury.
As might be anticipated, variation in the
voltage, amperage, frequency, and method of
application gives each of the electrosurgical
modality its unique qualities. Equally important,
however, is the waveform of each current. In
practice, a spark-gap apparatus produces
damped waves, whereas a vacuum tube or
transistorized unit creates continuous waves.
Examples of the former include the Hyfrecator
(Birtcher Corp., El Monte, CA) commonly
employed in dermatology.

Electrodesiccation and
Electrofulguration
The difference between electrodesiccation and
electrofulguration lies in the placement of the
elec-trode tip. In electrodesiccation (Lat. Siccus
dry), the tip contacts the skin and causes a
radial spread of current . In electrofulguration
[Lat. fulgur lightning] the tip remains above
the skin and the charge leaps to the surface in
a diffuse, or defocused, pattern , causing
flatter and more superficial tissue destruction.
The histologic outcome of both modalities is one
of tissue desiccation.
As a result of the low heat production,
electrodesiccation and electrofulguration are best
suited for superficial and comparatively
avascular lesions such as verrucae and
seborrheic keratoses.
Electrocoagulation
Electrocoagulation [Lat. Coagulum clot or
curd] is produced by a high-frequency

alternating current of high amperage (2,5004,000 mA) and low voltage (<200 volts). This
current is generated by a spark-gap apparatus
applied biterminally through one active and one
dispersive electrode; the patient is an integral
part of the circuit.
The depth of penetration makes
electrocoagu-lation useful in the removal of thick
lesions. The greater production of heat and
conduction of cur-rent along vessels make
electrocoagulation supe-rior to electrodesiccation for hemostasis in a wet field and
for destruction of highly vascular lesions (e.g.
pyogenic granuloma).

Electrosection
Electrosection [Lat. secare to cut] may be
achieved by using either an undamped
continuous current or a mildly damped pulsed
current. The former is generated by a vacuum
tube or solid-state unit, whereas the latter is
produced by a spark-gap apparatus. Continuous
waves cause intense but sharply limited heat
production, resulting in localized tissue
destruction with little effect on the immediately
adjacent tissue. Microscopically, an incision
approximately 0. 1 mm wide results from
disintegration of cells along the cutting line.
Electrolysis
Electrolysis [Gr. Lysis dissolution] makes use
of low-voltage, low-amperage direct (galvanic)
current flowing unidirectionally between polarized electrodes. The resulting chemical
ionization causes release of acids and metallic
ions at the positive pole, with formation of
hydroxides and attraction of metallic ions at the
negative pole. The major electrosurgical
application of electrolysis has been in epilation.
Two disadvantages of electrolysis are that it is
difficult to master and is time consuming.
Although electrolysis is an inaccurate term
for the high-frequency method of hair removal,
it continues as the common expression solely by

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Essentials in Dermatology
virtue of its frequent misusage. Thermoepilation [Gr. Therme heat + e out + pilus hair]
is a more accurate and descriptive appellation.

Electrocautery
Although not a true form of electrosurgery (no
current actually enters the patient), electrocautery [Gr. Kauterion branding iron] is, by
convention, usually included among the other
modalities. Low-voltage, high-amperage, direct
or alternating current is passed through a needle
tip that is heated to red-hot temperatures by its
resistance to the flow of current. Application of
the heated needle tip to bleeding vessels causes
coagulation. As with electrosection, too little heat
causes coagulated tissue to adhere to the needle,
whereas too much heat causes unnecessary
destruction of tissue.
Safety Considerations
Potential interference of cardiac pacemakers
from electrosurgical current has been known.
Close attention to proper grounding is important to prevent unintentional shocks or burns.
Particularly with the mono-terminal currents
used for electrodesiccation and electrofulguration, care should be taken by the operator
to maintain a firm, broad-based, skin-to-skin
contact with the patient. Alcohol is commonly
used to cleanse skin pre-operatively. If sufficient
drying time is not allowed prior to electrosurgery, the alcohol can be ignited and the
resulting flame spectacular.

CRYOSURGERY IN DERMATOLOGY
The term cryosurgery is derived from a Greek
word Kryo meaning ice. This type of surgery is
carried out by using freezing agents. The freezing
agents are called as cryogens.
The anesthetic properties of hypothermia are
known since Hippocratic times. Salt and ice
mixtures were used in the 19th century to

relieve pain. Prolonged skin anaesthesia


follows even light freezing, but the main
purpose of cryotherapy is not to reduce pain
but to destroy tissue. Cold kills!
James Arndt of London pioneered refrigeration and he is often described as the
Father of modern cryosurgery.
Most practitioners now use liquid nitrogen
as the source of coldness, the cryogen.
The ability of cryosurgery to destroy both
benign and malignant lesions is now well
established.
The spot freeze method, using either cotton
buds or sprays are discussed below.
Cotton bud method: This is used for benign
lesions, in particular viral warts. A cotton
bud, slightly smaller than the wart, is dipped
into liquid nitrogen and applied firmly and
vertically onto the wart (achieve a
temperature of 20o C). A halo of 1 mm wide
should form around the base of the wart, this
may take up to 30 seconds for a large wart
and treatment must then be stopped.
Spray method: Here the delivery of nitrogen
can be controlled accurately. The portable
machine, which can be fitted with spray
nozzles of different apertures, is used
(achieve a temperature of 196 o C). To treat
a basal cell carcinoma, with clear-cut
margins, the nozzle is held 0.5-1 cm from the
skin. Spraying then leads to the formation of
a white ice field of slowly increasing
diameter. The frozen area should include a 5
mm ring of normal tissue. The area is allowed
to thaw slowly, to ensure maximum cell
death. Two freeze-thaw cycles are better than
one for the routine treatment of malignant
tumors.
To minimize inflammation, aspirin may be
taken before treatment and then later on for
5 days or so. An anti-inflammatory dressing
containing clobetasol propionate should also
be applied daily for 3 days.

Dermatosurgical Procedures
depends on the lasing medium, the molecules
of which have been excited (e.g. CO2, ruby,
argon, dyes, ND-YAG, etc.).

LASERS IN DERMATOLOGY
LASER:
Light
Amplified by
Stimulated
Emission of
Radiation.
(first pioneered by Leon Goldman).

Principle
Inside a laser, one photon stimulates the
emission of another identical photon from
molecules that are in an excited state. Two
mirrors are placed on each end of the laser in
exact parallel alignment so that the photons
bounce back and forth through the excited
medium and the light is amplified by stimulating
more and more photons. The result is an
impressively bright, monochromatic, highly
collimated, coherent, and controllable beam of
light. The wavelength of the light released

Laser Skin Interaction


When the light released by the laser beam comes
in contact with the skin, various molecules
known as chromophores absorb the energy.
These chromophores include various molecules
like water, melanin, hemoglobin, nucleic acids,
proteins and urocanic acid. Different chromophores have the ability to absorb lights of specific
wavelengths and thus the type of laser used
depends on the chromophore that needs to be
targeted. For example, hemoglobin specifically
absorbs blue, green and yellow light and thus
lasers emitting light in these wavelengths (480
600 nm) are used for treatment of hemangiomas.
After absorption of the light, the skin tissue is
destroyed or remodeled by the various
mechanisms of photothermal, photochemical
and photomechanical effects.

Type of laser

Wavelength, nm

Typical uses

Alexandrite

755

Epidermal/dermal pigmentation, tattoos (black, blue, green),hair


removal

Argon, argon dye

488630

Vascular lesions (Port wine stain, hemangiomas), light source for


photodynamic therapy

CO2

10,600

Vaporization/ablation of rhytides, verruca, seborrheic keratoses,


actinic cheilitis,scars, photodamage

Erbium:YAG

2940

Rhytides, scars, photodamage

Krypton

520, 568

520-pigmented lesions, 568-vascular lesions

Nd:YAG

532, 1064

532-epidermal pigmentation,red tattoos; 1064-Q-switched-nonablative


dermal remodeling, black tattoos, hair removal

Pulsed dye (yellow)

577600

Vascular lesions (children and adults)

Pulsed dye (green)

510

Epidermal pigment, red tattoos

Ruby

694

Epidermal/dermal pigmentation, tattoos (black, blue, green), and hair


removal

275

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Essentials in Dermatology

DERMABRASION
Dermabrasion means surgical abrasion in the
planes of the epidermis and dermis. It can be
achieved with the help of a manual metallic
dermabrader or a rapidly rotating wire brush or
diamond fraise.

How it works?
Lesions and defects of the epidermis, papillary
dermis, and upper reticular dermis can be
partially or completely removed by surgically
planing to the level of the reticular dermis.
During the maturation phase of wound healing,
fibroblasts replace and remodel collagen bundles
in the papillary and upper reticular dermis.
Indications Include:
Facial acne scars
Surgical and traumatic scars
Photoaging
Benign conditionssuch as rhinophyma,
adenoma sebaceum, epidermal nevi,
syringomas, small cysts, milia, and seborrheic
keratoses.
Superficial malignancies, including Bowens
disease and superficial basal cell carcinomas.
Pigmentary disorders such as melasma,
tattoos, and lentigines
Postoperative complications include scarring,
pigmentary changes, persistent erythema, and
infection. The deeper the dermabrasion, the
greater the risk of scarring.

CHEMICAL PEELS
Chemical peeling also called chemical
resurfacing or chemoexfoliation, involves the

application of one or more exfoliating agents to


the skin, resulting in the destruction of portions
of the epidermis and/or dermis with subsequent
regeneration.
Based on wound depth, peels can be
classified as superficial (epidermal injury),
medium depth (upper dermal injury) and deep
peels (mid-dermal injury).

Indications Include
Actinic keratoses
Pigmentary lesions in the form of melasma
or postinflammatory hyperpigmentation.
Acne vulgaris scars and other depressed
scars.
Photoaging

Chemicals which are Used for Chemical


Peeling Include
Trichloroacetic acid 10 to 35%
Resorcinol paste.
Jessners combination of resorcinol, salicylic
acid, and lactic acid in ethanol.
Salicylic acid.
Alpha-hydroxy acids (AHAs) are naturally
occurring carboxylic acids normally found in
many foods. The AHAs include glycolic,
lactic, malic, citric, and tartaric acids. Lactic
acid and glycolic acid are used most
commonly.
Topical tretinoin.
55% phenolic Baker-Gordon formula
penetrates into the deep dermis.
Complications include pigmentary changes,
scarring, infection (esp. herpes virus infection),
and prolonged erythema.

Historical Milestones in Sexually Transmitted Diseases

29

Historical Milestones in
Sexually Transmitted Diseases

Know syphilis in all its manifestations and


relations, and all other things clinical will be
added unto you.

Alexandre Donne (in 1836)


He identified a flagellate protozoon which was
subsequently named Trichomonas vaginalis.

Sir William Osler, 1897


Knowing AIDS is to Know medicine.

Albert Neisser (1855-1916)


Isolation of distinct causative organism for
gonorrhoea in 1879.

Late Dr. Jonathan M. Mann


(director, Global Programme for AIDS)
John Astruc, the French physician
(in 1736)
He described genital herpes in 1736, and the first
English translation appeared in his Treatus of
Venereal Disease in 1754.
Bell (in 1793)
He recognized genital warts as a distinct disease.
Philippe Ricord (1799-1889)
French Venereologist
1. Established the specificity of two infectionssyphilis and gonorrhoea. 2. Classified syphilis
into primary, secondary and tertiary stages.
Rudolf Virchow (1821-1902)
Documented the range of systemic manifestations
of syphilis and gonorrhea.

Fritz Schaudinn (1871-1906) Protozoologist and Erich Hoffman (1868-1959)


syphilogist
Provided final evidence for the cause of syphilis
Paul Ehrlich (1854-1915)
Nobel Laureate Immunologist
Announced the discovery of arsenic compounds
Salavarasan, a chemotherapeutic cure for
syphilis, Magic bullets.
Kenneth MacLeod (1844-1922AD)
He while working in Indian medical service, in
days of British Raj first described donovanosis in
1881.
Caesar Boeck, Norwegian Syphilologist
(1845-1930)
In order to determine more accurately the spectrum
of outcomes in syphilis infection, a prospective

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Essentials in Dermatology
study was setup by Norwegian syphilologist,
Caesar Boeck. He collected patients between 1891
and 1910 and his successor at the Oslo
Dermatological Centre, Bruusgaard reassessed
them in 1929. The data was further reanalyzed
by Gjestland in 1955. This study is usually known
as Oslo Study.

Brassereau (in 1852)


Chancroid, one of the five classic venereal
diseases, was first described in France.
Augusto Ducreyi (1860-1940 AD)
The causative organism of chancroid was
identified while he was working in Naples in
1889.
Diday and Doyon (in 1886)
They published the monograph Les Herpes
Genitaux in which they observed that herpes
often appeared after a venereal infection such as
syphilis, chancroid, or gonorrhea.
Caddy (in 1902)
He was the first to record cases of lymphogranuloma venereum in India under the title of
Climatic bubo.
Colonel Charles Donovan (in 1905)
He described the intracellular bodies Donovan
bodies, which bear his name, and was the codiscoverer of etiological agent in Kala-azar.
Halberstaedter and Prowazek (in 1907)
Microbiological investigations of chlamydial
infections began in 1907 when Halberstaedter
and Prowazek detected intracytoplasmic
chlamydial inclusions in the conjunctival
scrapings of non-human primates.
Lipshutz (in 1920s)
He inoculated material from genital herpetic
lesions into the skin of humans, eliciting clinical

infection within 48-72 hours in six persons and


24 days in one person.

HIV origin (1930)


Sometime around 1930, a strain of SIV was
transferred from chimpanzees to humans who
hunted and butchered chimps for bush meat
somewhere in what is now the Republic of
Cameroon, near the borders of the Gabonese
Republic and the Republic of Congo. The virus is
thought to have adapted to become HIV-1, the
most widespread form of HIV found today.
National STD Control Programme in
India (1946)
Started in 1946, continued to operate till 1991.
Gardner and Dukes (in 1955)
The syndrome of bacterial vaginosis was first
described as Haemophilus vaginalis vaginitis
in 1955 by Gardner and Dukes. They concluded
that it was an STI as the isolated etiological agent,
H. vaginalis (now renamed Gardnerella vaginalis),
was found in the male contacts of the female cases.
Genital Herpes as
Venereal Disease (1966)
Genital herpes was rarely considered in the
differential diagnosis of genital ulcers prior to
1965. It was only in 1966 that herpes genitalis
was recognized as a venereal disease.
Dr Robert Gallo (in January 26, 1984)
In the US National Cancer Institute (NCI), he
isolated the virus that causes AIDS which he
called as HTLV-III.
Evolution of Antiretroviral Therapy
Drugs and Regimens (1985 onwards)
Zidovudine was first tested on humans in 1985
and introduced for treatment in March 1987.
Between December 1995 and March 1996, three
protease inhibitors (PIs)-indinavir (IDV),

Historical Milestones in Sexually Transmitted Diseases


saquinavir (SQV), and ritonavir (RTV) got
approval for their use.

retroviral drugs, replaced the expression triple


combination therapy.

On June 21 1996, the US FDA approved the first


non-nucleoside reverse transcriptase inhibitor
(NNRTI) drug, nevirapine.

In November 1999, enfuvirtide, a drug from a new


class of drugs called a fusion inhibitor, began
clinical trials.

1996 onwards, combination therapy, highly active


antiretroviral therapy (HAART) began to spread
irreversibly.

National AIDS Control Programme (1992)

In 1995, David Ho came out with his slogan hit


hard and early.
In 1997, Highly Active Anti-Retroviral Therapy
(HAART), consisting of three or more anti-

The arrival/spread of HIV infection and because


of its strong relation with STD, the programme
National STD Control Programme in India was
brought under the purview of National AIDS
Control Organization in the year 1992.

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30

History Taking and


Examination in Sexually
Transmitted Diseases (STDs)

History taking and examination of an STD


patient is similar to that done in general
medicine. The examination should be preceded
by reassurance and evincing confidence in the
patient.
Most clinicians encounter no difficulty
during routine patient interviews but are uneasy
when faced with sexually related diseases. This
is unfortunate as detection of STDs and other
sexual problems is facilitated by a thorough
sexual history.
The brief explanation of the importance of
thorough sexual history assures the patient that
physician goal is to solve his or her problem.
Identification of sexual contact is best deferred
until a specific diagnosis is confirmed.
Name: Calling the patient by name will not only
help to make a doctor-patient relationship but
also reduce patient apprehension and anxiety.
Age: It is important to know the age of the
patient, as STDs are more prevalent in
reproductive age group.
Sex: It determines variations in manifestations
of STDs in males and females.
Occupation: It may give a hint of certain
occupations in which STDs are more frequent
e.g., commercial sex workers, truck drivers etc,
whether living at home or away from home.

Address: Address of the patient helps us in


follow up and contact tracing.
Marital status: Whether the person is staying
single, unmarried or married, divorced, widow
or widower, matters in sexual history.
Privacy: Whenever possible only the practitioner
and patient should be present during
consultation. Ideally it should be conducted in a
private room which is adequately sound proofed
and doors kept closed.

History of Present Illness


I. Chief complaints and associated symptomatology.
Patients usually present with complaint of either
discharge per urethra/vagina or genital sore.
For the discharge per urethra/vagina, we should
enquire about:
1. Amount of discharge- copious (gonorrhoea)
or scanty (chlamydial infection, balanoposthitis).
2. Colour of discharge- milky white (bacterial
vaginosis), yellowish (gonorrhoea, trichomonas infection), or blood stained
(malignancy)
3. Consistency of discharge- thin watery or
thick (gonorrhoea)

History Taking and Examination in Sexually Transmitted Diseases (STDs)


4. Whether had any relationship to micturition
or not
5. Present all the time or increased during any
particular time
6. Odour of discharge
7. In females-whether related to periods
8. Burning micturition
9. Pain during defecation, urethral discharge
during defecation.
For genital sore:
1. Number of lesions- single or multiple
2. Duration of sore(s)
3. Pain-painful (chancroid, herpes) or painless
(primary chancre)
4. Discharge from the sore- serous discharge
(primary chancre)
5. Associated swelling
6. Preceding erythema or burning sensation
7. History of recurrent vesicles
8. Any similar lesions before
9. Any local medication applied
10. History of phimosis after ulcer (chancroid).
For genital growth:
1. Duration
2. Progression-slow or fast
3. Whether bleeds to touch
4. Associated features.
For urinary symptoms:
1. Burning micturition- at onset of micturition
(urethritis) or after coming in contact with
vaginal walls (vaginitis, vulvitis).
2. Frequency of micturition- increased or
decreased.
3. History of associated hematuria.
4. History of urgency, loin pain, fever with
rigors to rule out pyelonephritis and urinary
tract infection.
5. Strangury-gonorrhoea.
Other symptoms like rash, pruritus, adenopathy,
abdominal pain, dyspareunia, diarrhoea,

hematochezia, anogenital pain, pain during


defaecation, pus stained stools (rectal
gonorrhoea) etc.
II. After asking for chief complaints, we should
enquire about onset and duration of
symptoms- whether started after sexual
exposure, duration-whether of long
duration or short duration
III. Then, we should enquire about recent dates
of sexual intercourse. It is important for
knowing the incubation period like in
gonorrhoea and chancroid-2-5 days,
herpes-4-5 days, non specific urethritis-814 days, syphilis-9-90 days, lymphogranuloma venereum-3days-3 weeks,
donovanosis-8-80 days, venereal warts-6-32
weeks, etc.
IV. Sexual orientation of the patient should be
found, whether heterosexual, homosexual,
or bisexual.
Approximate number of recent sexual
partners and known STDs if any among
sexual contacts.
Use of any barrier method for prevention of
STDs.
Previous STDs and therapy utilized.
Time of last urination because this might
affect the presence of discharge.

Past History
Past history of exposure if any, STD, medical or
surgical illness, hospital admission, blood
transfusion or medical illness complicating the
disease.
Treatment History
Present and recent medications, especially
antibiotics, topical and vaginal preparations.
History of drug sensitivity or any genitourinary
instrumentation e.g. cystoscopy, abortion,
dilatation, curettage etc.

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Personal History
Personal history regarding education, socioeconomic status, occupation, income, habits
(alcohol use, drug abuse, smoking), staying alone
or with family, relationship with spouse and
frequency of sexual intercourse may be essential.
Sexual History
It should be taken in detail. Exact dates of sexual
exposure, place of exposure, whether heterosexual, homosexual or bisexual, use of barrier
contraceptives during intercourse may be
enquired. Details of intercourse- genito-genital,
genito-anal, genito-oral may have relationship
to the diagnosis at hand. Further enquiry about
the sexual partner whether he/she was having
genital sore/discharge or any other related
problem, whether the concerned person has
single or multiple sexual partners need to be
known, and the frequency of sexual intercourse.
HIV Risk Assessment
Activities known to be associated with increased
risk of acquisition of HIV should be specifically
enquired about. These include involvement in
prostitution, male homosexuality or bisexuality,
having injected drugs, sexual partners from areas
of high endemicity, history of transfusions with
blood or blood products, sexual contact with
intravenous drug users, or (in the case of females)
hemophiliacs, or bisexual male partners.
Menstrual History
History regarding last date of previous
menstruation, whether menses are regular or
irregular, flow heavy or scanty, contains fresh
blood or clots, associated pain and history of post
coital bleeding or intermenstrual bleeding may
be helpful.
Obstetric History
Enquire about methods of contraception used if
any (barrier, intrauterine device, oral pills

intake), has ever become pregnant, number of


children if any and their health, death of children
if any and cause of their death, history of
miscarriage, abortion or stillbirth if any.

Family History
Health status of siblings, any family history of
similar problem or death if any and cause of
death may be ascertained. This is relevant in
cases of suspected congenital syphilis as it is
more common if the mother did not receive
adequate antenatal care.
General Physical Examination
Consideration should always be given to having
another member of health care worker present
when carrying out an intimate physical
examination on the patient, who will reassure
the patient, able to assist the practitioner in
carrying out the examination and provides a
witness as protection against allegation of
indecency or misconduct. Systematic approach
to physical examination is the best means of
reassuring a patient. Since the manifestations of
STDs are not limited to the anogenital region, a
general physical examination of each patient is
ideal. Relevant systemic examination may be
undertaken after thorough genital examination.
Genital Examination
It should be done in privacy, in the presence of
natural light if possible or good light source.
Examination of the Male Patient
Patient should be fully exposed and
undergarments inspected for staining with
the discharge.
External genitalia and inguinal skin are
examined visually and by palpation with
gloved hands.
Care should be taken to inspect and palpate
penis with attention to the urethral canal and
scrotal contents for evidence of pain,

History Taking and Examination in Sexually Transmitted Diseases (STDs)

inflammation, induration or edema, warts,


or rashes.
Hairy regions should be evaluated for the
presence of lice, rust coloured excreta or nits
attached to hair shafts.
In an uncircumcised male, the foreskin
should be retracted and concealed portion of
foreskin and glans examined.
Urethral meatus is then visualized for
evidence of discharge or other abnormalities.
With the penis held firmly at midshaft with
one hand, urethral contents stripped
anteriorly with free hand to elicit any
discharge.
Genital ulcers, vesicles, erosions should be
carefully examined for their characteristics
such as whether single or multiple, site of
involvement, shape (circular or irregular),
size, margin (clear cut, everted, undermined),
depth (superficial or deep), floor (clean,
covered with necrotic material, healthy
granulation tissue), tenderness, induration,
tendency to bleed on touch.
The inguinal, external iliac, axillary and
cervical group of lymph nodes are examined
in detail (unilateral or bilateral, single or
multiple, discrete or matted, consistency,
tenderness, mobility, erythema of overlying
skin, fixity to underlying tissue).
The spermatic cord on both sides should be
palpated between thumb and index finger for
tenderness, thickening and asymmetry.
Scrotum examined for erythema, asymmetry,
and suppurative lesions. Scrotal contents
palpated to know any abnormality.
Digital rectal, anoscopic and sigmoidoscopic
examination are recommended in
homosexual males especially in those
complaining of gastrointestinal and rectal
symptomatology.

Examination of the Female Patient


After a brief explanation of the necessity for
pelvic examination and steps involved, the

patient should assume the lithotomy position


on a table equipped with adjustable stirrups.
The examiner, seated at the foot end of the
table, should have both hands gloved and is
encouraged to maintain eye contact with the
patient. Patient may be draped to remove her
apprehension.
The external genitalia and pubic hair should
be carefully inspected. Then stepwise
examination of inguinal lymph nodes, vulvar
skin, urethral meatus, introitus, Bartholins
ducts and glands, and Skenes ducts is
completed. Evaluate for erythema,
excoriation, inflammation, vaginal discharge,
urethral discharge, induration, edema,
ulceration, erosion, discoloration, and areas
of abnormal appearing epithelium.
For palpation and stripping of the urethra, a
gloved index finger, lubricated with warm
water, is inserted into the vagina and the
urethra is then palpated through the anterior
vaginal wall. Feel for edema, inflammation,
induration or anomalies such as diverticuli.
Compressing the urethra anteriorly against
the pubic symphysis and milking it toward
the meatus may express exudate from the
urethra.
Prior to speculum insertion, the same-gloved
finger should be pressed gently but firmly
against the posterior vaginal wall for several
seconds. This allows for easy insertion of a
speculum (due to perineal relaxation),
lubricated with warm water. Proper
angulation of speculum on insertion results
in ready visualization of the cervix.
Cervix should be examined for discharge,
erosions, ectropion, ulceration and friability.
To test friability of cervix, cotton tipped swab
may be used.
The blades of speculum can be used to gently
compress the cervix in an attempt to exude
endocervical discharge. After specimen
collection is completed, the speculum is
slowly removed and the entire area of the
vaginal mucosa carefully examined.

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The vagina should be examined for rugal
folds, erythema, malodour, discharge,
purulence, ulcerations and other abnormalities.
It is important to ascertain the character and
source of discharge (physiological versus
vaginal versus cervical).
Following speculum examination, bimanual
examination including both abdominal
pelvic and genitorectal assessment is
important part of the pelvic examination.
With one hand on the abdomen and the index
and middle finger of the other hand inserted
in the vaginal canal, stepwise palpation of
the abdomen, vagina, cervix, uterus and
adnexal structures is completed. Uterine size
and position should be carefully evaluated
and superior displacement of the cervix
performed to check for the presence of
tenderness.
Prior to genitorectal examination, gloves
should be changed to avoid possibility of
iatrogenic rectal inoculation of pathogens.
Genitorectal examination is done for
evaluation of the rectum and cul-de-sac.
Anoscopic and sigmoidoscopic examination
is suggested for females with proctocolitic
symptomatology and those who have
engaged in rectal intercourse.

Oropharyngeal Examination
Routine examination of oropharynx is essential
and may give useful clues to diagnosis or helps
in detection of ulcerations or pharyngitis.
Bedside Procedures
1. Two or three glass test- is done to
differentiate anterior urethritis from
posterior urethritis or infection of the
bladder. In the two glass test, patient is
asked to void the urine. If the first glass is
hazy and second glass clear, suggests
anterior urethritis. In case, haziness is seen
in second glass only, it means there is

posterior urethritis. If both the glasses


contain hazy urine, it means there is cystitis.
In three glass test, anterior urethra is
irrigated till washings are clear (1:8000
oxycyanide of mercury solution) in the first
glass. If second sample of urine obtained
contains pus, it suggests posterior urethritis
and if third glass has pus, means cystitis.
2. Saline preparation for clue cells, lactobacilli,
leukocytes, Trichomonas vaginalis or Candida
albicans
3. Wet mount for Trichomonas vaginalis
4. KOH examination on vaginal discharge or
preputial sac scrapings
5. Whiff or Sniff test on vaginal discharge
6. Grams stain of urethral, vaginal, cervical
or other discharge
7. pH estimation of vaginal discharge
8. Dark ground illumination test- for
Treponema pallidum, Trichomonas vaginalis
9. Tissue smear for Donovan bodies in the
mononuclear cells seen in donovanosis.
10. Tzanck smear for giant cells in herpes
genitalis infection
11. Acetic acid test in case of genital warts
12. Scraping for mite if scabies is suspected
13. Microscopic examination for lice or nits
14. Bright field microscopic examination of
unstained specimens is useful for
examining uncentrifuged and centrifuged
urine for leukocytes and bacteria
15. Specimens obtained may be sent for culture
examination
16. Skin biopsy from skin lesions, genital sores
etc for histopathology
Based on clinical history and examination, a
provisional diagnosis is made. All patients with
history of premarital or extramarital sexual
contact, and those with STDs are screened for
syphilis and HIV infection. On the basis of
clinical diagnosis, treatment is instituted.
A proper history and examination should be
followed by counselling which should include
imparting knowledge about safe sex and creating
awareness about STDs.

History Taking and Examination in Sexually Transmitted Diseases (STDs)

Guidelines for Evaluating STDs


1. The presence of one STD signals the
possibility of a second pathogen (overt,
asymptomatic or in incubation phase).
2. All sexual contacts of an STD patient should
be evaluated and treated.
3. Complications of STDs may be averted by
early diagnosis, proper treatment, appropriate follow up, and detection of the
asymptomatic cases.
4. Site of infection should be specified in the
diagnosis.

5. Evaluation for pregnancy by history, physical


examination and laboratory tests is essential
6. STDs must be considered in all cases of child
abuse.
7. Geographic location and patient population
determine the prevalence of a specific disease
and affect the predictive values of laboratory
tests.
8. A single, non-reactive non-treponemal
serological test for syphilis (VDRL) does not
exclude syphilis.
9. Appropriate patient follow up and documentation of cure are critical.

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31

Sexually Transmitted Diseases

Sexually transmitted diseases (STDs) are a group


of communicable diseases that are transmitted
predominantly by sexual contact and caused by

a wide range of bacterial, viral, protozoal, fungal


pathogens and ectoparasites. Over 20 pathogens
have been found to be spread by sexual contact.

A classification of these agents and the diseases caused by them are


Pathogen

Disease or syndrome

Neisseria gonorrhoeae

Gonorrhoea, urethritis, cervicitis, epididymitis, salpingitis,


PID, neonatal conjunctivitis

Treponema pallidum

Syphilis

Haemophilus ducreyi

Chancroid

Chlamydia trachomatis

LGV, urethritis, proctitis, epididymitis, infantile pneumonia,


Reiters syndrome, PID, neonatal conjunctivitis

Calymmatobacterium granulomatis

Donovanosis

Hepatitis B

Acute and chronic hepatitis

Human papilloma viruses

Genital and oral warts

Herpes simplex virus

Herpes genitalis

Human immunodeficiency virus

AIDS

Molluscum contagiosum

Genital molluscum contagiosum

Candida albicans

Vaginitis

Trichomonas vaginalis

Vaginitis

Sexually Transmitted Diseases

GENITAL ULCER DISEASES


Syphilis
Causative organism- Treponema pallidum, a
spirochete, a motile, corkscrew-shaped, gramnegative, prokaryotic bacterium with a
flexible, helically coiled cell wall. It belongs to
the order Spirochaetales (coiled hair).
The majority of syphilis infections are
acquired through direct sexual contact with
an infected person in the early stages of the
disease (acquired syphilis).
The secondary stage of syphilis is most
contagious because of the greater amount and
variety of lesions present.
Acquired Syphilis
Primary Syphilis
After an incubation period of 9-90 days,
infection with treponema pallidum results in
a painless chancre (primary chancre,
Hunterian chancre, Hard chancre) at the
site of inoculation. Five percent of chancres
have extra-genital location.
Typical chancre of primary syphilis is a round
or slightly elongated ulcer, 1-2 cm across with
an indurated margin. The ulcer has a clear
base, without an exudate being present
(Fig. 31.1)

Fig. 31.1: Primary chancrepainless indurated


clean looking single ulcer over the glans penis

Modest enlargement of inguinal lymph nodes


(nontender, nonsuppurative, firm, shotty,
indolent bubo), frequently bilaterally is
observed in the majority of patients who have
genital lesions.
Without treatment, the chancre heals with
scarring in 3 to 6 weeks.
Relapses of primary syphilis, termed
monorecidive syphilis or chancre redux are
rare.

Secondary Syphilis
Lesions of secondary syphilis result from the
hematogenous dissemination of treponemes
during the evolution of the primary syphilitic
chancre and lesions appear 3-12 weeks after
the initial appearance of primary lesion.
Patients with secondary syphilis may be ill
with flu-like symptoms that include malaise,
appetite loss, fever, headache, stiff neck,
lacrimation, myalgias, arthralgias, nasal
discharge, and depression.
The skin manifestations of secondary syphilis
called syphilids are asymptomatic, polymorphous, variegate, bilateral and
symmetrical (Figs 31.2 to 31.6). The initial
finding in secondary syphilis is an evanescent
macular rash, a few days later symmetric
papular eruption appear involving the entire
trunk and the extremities, including palms
and soles. Papular lesions demonstrate
Ollendorf sign. Even nodules may appear.
They may be pustular, but never vesicular.
Because of the variety of clinical
manifestations, syphilis has been called the
the great imitator.
Lesions on mucous membranes appear as
raised gray white mucous patches.
Condylomata lata, the moist, smooth surfaced
warty intertriginous plaques are considered
to be most infectious lesion of syphilis because
of the eroded surface and large number of
spirochetes (Figs 31.7 and 31.8)

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Fig. 31.2: Secondary syphilispapular and nodular


syphilid over the face

Fig. 31.4: Secondary syphilispapular and nodular


syphilitic rash over the back

Fig. 31.3: Secondary syphilissplit papules at the


angles of mouth

Fig. 31.5: Secondary syphilissingle


noduloplaque over the back

Another hallmark of secondary syphilis is


generalized lymphadenopathy, which
frequently affect the inguinal, the posterior
cervical and the epitrochlear lymph nodes.
Their characteristics are similar to that seen
in primary syphilis.
Moth eaten alopecia may occur over the
scalp.

Rarely, central nervous system, eyes and other


visceral organs are affected.
Untreated secondary syphilis spontaneously
resolves after a period of 3 to 12 weeks leaving
the patient in asymptomatic latent phase.
Individuals may remain asymptomatic for life,
even though the T. pallidum organism continues
to multiply or enter late symptomatic syphilis.

Sexually Transmitted Diseases

Fig. 31.6: Secondary syphilisannular syphilid

Fig. 31.8: Secondary syphiliscondylomata lata


lesions in the perianal area

Fig. 31.7: Secondary syphiliscondylomata lata at


the root of penis and healed scar of primary chancre
seen over the glans penis

Fig. 31.9: Tertiary syphilisperforation of the palate

Tertiary Syphilis

The fetus is at greatest risk when maternal


syphilis is of less than 2 years duration. The
ability of the mother to infect the fetus
diminishes but never disappears in late latent
stages (Kassowitzs law).
Prenatal infection may also result in
miscarriage or stillbirths.
Congenital syphilis is divided into early (of
less than 2 years duration) and late (more than
2 years duration) congenital syphilis.

Tertiary syphilis may be gummatous


(nontender pink to dusky red nodules or
plaques that vary in size from millimeters to
many centimeters in diameter) (Fig. 31.9),
cardiovascular or neurosyphilis.

Congenital Syphilis
In congenital syphilis, the treponemes cross
the placenta and infect the fetus.

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Essentials in Dermatology
The early clinical signs of congenital syphilis
begin to appear in the third to eighth week of
life and in all cases by 3rd month of life.
Early congenital syphilis is known for
snuffles (a persistent nasal dischargesyphilitic rhinitis), myriad of cutaneous
lesions like secondary syphilis (scaling
papules, plaques, bullae, desquamation,
condylomata lata, mucous patches) (Figs 31.10
to 31.12), furuncle of Barlow, hepatosplenomegaly (Fig. 31.13), generalized
lymphadenopathy, pseudoparalysis of Parrot
(pain from osteochondritis of the long bones,

or epiphysitis, is exacerbated by movement,


so the child keeps the affected limb still),
osteochondritis with the sawtooth
metaphysis seen on radiographs, syphilitic
chorioretinitis (salt and pepper fundus)
and late congenital syphilis for Hot cross
bun deformity of skull, a saddle nose
deformity, notched molars (Moons molars or
Mulberry molars) and central incisors
(Hutchinsons teeth), Hutchinsons triad
(Hutchinsons teeth, interstitial keratitis,
eighth nerve deafness), rhagades, Cluttons
joints (painless swelling of joints- most
commonly both knees), sabre tibiae, palatal
perforation, neurosyphilis (tabes dorsalis,
general paresis and local gummata) and
paroxysmal cold hemoglobinuria.

Fig. 31.10: Congenital syphilispapular lesions of


early syphilis over the knee

Fig. 31.12: Congenital syphilisperianal


condylomata lata lesions in early syphilis

Fig. 31.11: Congenital syphiliserythema and


scaling of palms in early syphilis

Fig. 31.13: Congenital syphilis


hepatosplenomegaly in early syphilis

Sexually Transmitted Diseases

Stages of Acquired Syphilitic Infection


Primary syphilis

10-90 days (average 21 days)

Chancre single or multiple, on skin or mucous


membrane, regional lymphadenopathy

Secondary syphilis

6 weeks to 6 months

Multiple secondary lesions (skin or mucous membrane),


lymphadenopathy, fever, condylomata lata, alopecia,
asymptomatic CNS involvement

Latent syphilis

<1 year (early); >1 year (late)

Asymptomatic

Late syphilis

Months to years

Gummatous syphilis (monocytic infiltration, tissue


destruction of any organ); Cardiovascular syphilis
(Aortic aneurysms); Neurosyphilis (paresis, tabes
dorsalis, meningovascular syphilis)

Diagnosis
Acquired Syphilis
The most specific and sensitive method for
verifying the diagnosis of primary syphilis is
the finding of treponemes with characteristic
appearance by darkfield microscopic
examinations of fluid obtained from the
surface of the chancre. The dark field
examination is actually the only test that
specifically establishes the diagnosis of
primary syphilis.
The non-specific treponemal tests the
Venereal Disease Research Laboratory
(VDRL) and the rapid plasma reagin (RPR),
typically become reactive within 4 to 7 days of
chancre development. A titre of 1:8 or more is
said to be significant. These titre results
correspond with disease activity and should
reduce four fold within 6 to 12 months of
treatment and become undetectable several
years there after. False positive results may
occur with numerous conditions, such as
collagen vascular diseases, several chronic
infections such as HIV or tuberculosis,
advancing age, narcotic drug use, chronic
liver disease and several active infections such
as herpes.
Because of the decreased specificity of above
tests, positive results should be confirmed
with the more precise treponemal tests,
Treponema pallidum heme agglutination assay

(TPHA), microhaemagglutination assay for


T.pallidum (MHA-TP) and fluorescent
treponemal antibody absorptions test (FTAABS). In secondary syphilis all serologies
should be reactive, whereas in late syphilis,
non-treponemal tests may be negative or only
weakly positive whereas treponemal tests are
usually reactive.
CSF examination and chest X-ray should be
done in tertiary syphilis. Sometimes, skin
biopsy may be required to show characteristic
histopathological changes (Endarteritis
obliterans and predominantly perivascular
infiltrate of lymphoid cells and plasma cells
are basic pathologic changes of syphilis. Late
in the course of syphilis, granulomatous
changes also occur) and organisms in tissue
can be demonstrated by silver staining.

Congenital Syphilis
A diagnosis of congenital syphilis can be
made with confidence if the mother has
reactive non-treponemal and treponemal
serologies and the infant manifests classic
signs of disease.
Congenital syphilis is also highly likely when
the infants non-treponemal antibody titre is
four fold or greater than the mothers serum,
even in the absence of physical findings.
IgM FTA ABS test is specific for congenital
syphilis, particularly if the titre rises.

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Treatment
Acquired Syphilis
Stage

Treatment

Follow up

Primary, secondary, or
early latent syphilis

2.4 million IU
intramuscular benzathine
penicillin G once

Clinical and serologic exams at 3, 6, 9, 12,


and 24 months

Late latent syphilis or


syphilis of unknown
duration

2.4 million IU
intramuscular benzathine
penicillin G weekly for 3 weeks

Clinical and serologic exams at 6, 12, 18, and


24 months

Neurosyphilis or
ocular syphilis

3-4 million IU of intravenous aqueous


crystalline penicillin G every 4 hours
for 10-14 days or intramuscular procaine
penicillin 2.4 million IU daily and oral
probenecid 500 mg 4 times per day for
10-14 days

If CSF pleocytosis was initially present, CSF


exams every 6 months for up to 2 years or
until this parameter returns to normal.

If patient is allergic to penicillin in early syphilis,


he/she may be treated with
Doxycycline 100 mg bd PO 15 days or
Tetracycline HCl 500 mg qid PO 15 days
Alternative regimen for penicillin-allergic
pregnant patients in early syphilis is
Erythromycin, 500 mg orally, 4 times daily for 14
days.
Alternative regimen for penicillin-allergic nonpregnant patients in late syphilis.
Doxycycline, 100 mg orally, twice daily for 30
days.
Or
Tetracycline, 500 mg orally, 4 times daily for 30
days.
Alternative regimen for penicillin-allergic
pregnant patients in late syphilis.
Erythromycin, 500 mg orally, 4 times daily for 30
days.
Patients of early syphilis and late syphilis should
be followed up clinically and serologically at 3
monthly intervals.

Congenital Syphilis

Early Congenital Syphilis


Aqueous benzylpenicillin 100, 000150, 000 IU/
kg/day administered as 50, 000 IU/kg/dose IV
every 12 hours, during the first 7 days of life and
every 8 hours thereafter for a total of 10 days.
Or
Procaine benzylpenicillin, 50, 000 IU/kg by
intramuscular injection, as a single daily dose for
10 days.
Note
Some experts treat all infants with congenital
syphilis as if the CSF findings were abnormal.
Antimicrobials other than penicillin (e.g.
erythromycin) are not recommended for
congenital syphilis except in cases of allergy to
penicillin. Tetracyclines should not be used in
young children.

Late Congenital Syphilis


Aqueous benzylpenicillin, 200, 000300, 000 IU/
kg/day by intravenous or intramuscular
injection, administered as 50, 000 IU/kg/dose
every 46 hours for 1014 days.

Sexually Transmitted Diseases


Alternative Regimen for Penicillin-allergic
Patients, after the First Month of Life
Erythromycin, 7.512.5 mg/kg orally, 4 times
daily for 30 days.

Chancroid (Soft sore, soft chancre,


ulcus molle)
Three days to 2 weeks (1-5 days usually) after
exposure to Haemophilus ducreyi (gram
negative bacillus), a small inflammatory
papule or pustule arises at the site of
inoculation.
Within days, the lesion erodes to form an
extremely painful, deep ulceration.
The characteristic ulcer is soft, friable and
non-indurated, with ragged undermined
margins, a foul smelling, yellow-grey exudate
covering and surrounding erythema.
Within 1 to 2 weeks, painful inguinal
lymphadenitis (inflammatory bubo), most
often unilateral, develops in 30 to 60% of
patients (about half the cases). Twenty five
percent of patients have progression of the
lymphadenitis into a suppurative bubo
(unilocular abscess), which may
spontaneously rupture and develop
ulceration.

Diagnosis
H.ducreyi may be identified in the form of
chains (school of fish) of gram-negative
cocobacilli in the smear from the ulcer, or
preferably of pus from bubo.
Ideally it should be cultured on one of the
modern selective media at 33C in an
atmosphere of high humidity.
Ito-Reenstierna test- intradermal test with a
vaccine containing killed H.ducreyi in
suspension, producing an inflammatory
papule (0.5 to 1 cm in diameter) after 48 hours
Because of the current difficulty with
diagnosis, the CDC recommends that a
probable diagnosis can be determined by the

presence of the following: one or more painful


genital ulcers, a clinical presentation and
associated lymphadenopathy, a negative
laboratory evaluation for T.pallidum (serologic
testing or dark field examination) and a
negative testing for herpes simplex virus
(HSV).

Treatment
WHO 2003 and Center for Disease Control and
Prevention (CDC) in 2006 recommends
Azithromycin 1 g orally in a single dose, or
Ceftriaxone 250 mg intramuscularly (IM) in a
single dose, or Ciprofloxacin 500 mg orally twice
a day for 3 days (contraindicated for pregnant
and lactating women), or Erythromycin base 500
mg orally three times (CDC) or four times (WHO)
a day for 7 days.
NACO recommends erythromycin stearate /
erythromycin base, 500 mg orally 4 times a day
for 7 days or erythromycin ethyl succinate, 800
mg orally 4 times a day for 7 days (In case of
concomitant syphilis, treatment can be given for
15 days) or ciprofloxacin, 500 mg orally twice a
day for 3-5 days or till the clearance of lesions or
ceftriaxone, 250 mg IM as a single dose or
azithromycin, 1 g orally as a single dose or
doxycycline, 100 mg orally twice daily for 7 days
or trimethoprim (80 mg) + sulphamethoxazole
(400 mg), 2 tabs orally twice a day for 2 weeks.
Treatment should be given for the period
indicated, or until such time, the lesions heal.
Fluctuant bubo should be aspirated through
the surrounding healthy skin. Aspiration should
not be done from the dependent side. Incision and
drainage or excision of bubo delays healing and
is contraindicated.

Lymphogranuloma Venereum (LGV)


Causative organism-Chlamydia trachomatis
immunotypes L1, L2, and L3.
The course of disease in LGV consists of 3
separate stages.

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Essentials in Dermatology
After an incubation period of 3-30 days after
inoculation, the first stage begins with a small
painless papule or pustule that may erode to
form an asymptomatic herpetiform ulcer. This
typically heals without scarring in 1 week and
often goes unnoticed.
The second stage begins within 2-6 weeks after
the onset of primary lesion. It is referred to as
inguinal stage and consists of painful
inflammation and infection of inguinal and/
or femoral lymph nodes. Involvement is
limited to one groin in about two third of the
cases. Enlargement of tender lymph nodes
above and below the inguinal ligament may
give the bubo a grooved appearance the sign
of the groove (Fig. 31.14). Suppuration occurs
in them with the formation of multiple small
abscesses. These abscesses open on the skin
surface to form multiple sinuses, finally
healing with puckered scars. Constitutional
symptoms are very variable.
The third stage is called as genitorectal
syndrome. The interval between the early stage
and later manifestations may vary from a year
or two to many years. Patients may develop
genital elephantiasis (Esthiomene) or
anorectal syndrome. It more often develops in
women and homosexual men who engage in
receptive anal intercourse and includes
proctocolitis, peri-rectal abscess, fistula.

Diagnosis
A definitive diagnosis can be achieved with
isolation of the organism on culture and cell
typing of the isolates.
The Frei test-an intradermal test with 0.1ml of
Lygranum antigen, read at 48 to 72 hours- a
raised, red papule, at least 6 mm across.
With appropriate clinical presentation, a
complement fixation antibody titre of greater
than 1:64 is considered diagnostic of LGV.

Treatment
Center for Disease Control and Prevention (CDC)
in 2006 recommends.
Doxycycline 100 mg orally twice a day for 21
days
Alternative treatment is erythromycin base 500
mg orally four times a day for 21 days.
Some STD specialists believe that
azithromycin 1.0 g orally once weekly for 3
weeks is probably effective, although clinical
data are lacking.
WHO (2003) Recommends
Doxycycline, 100 mg orally, twice daily for 14
days.
Or
Erythromycin, 500 mg orally, 4 times daily for 14
days.
Alternative regimen
Tetracycline, 500 mg orally, 4 times daily for 14
days.

Fig. 31.14: Lymphogranuloma venereumsign of


the groove due to enlargement of lymph nodes above
and below inguinal ligament

Note
Tetracyclines are contraindicated in pregnancy.
NACO recommendations are same as WHO
except for an additional therapy- trimethoprim
(80 mg) + sulphamethoxazole (400 mg) 2 tablets
twice daily for 21 days.
Fluctuant lymph nodes should be aspirated
through healthy skin. Incision and drainage or
excision of nodes may delay healing. Some
patients with advanced disease may require

Sexually Transmitted Diseases


treatment for longer than 14 days, and sequelae
such as strictures and/or fistulae may require
surgery.

superficially in the area of the inguinal nodes


called pseudobuboes (not lymphadenitis).

Diagnosis

Granuloma inguinale (Donovanosis)


Causative organism- Klebsiella granulomatis
(formerly known as Calymmatobacterium
granulomatis), related to the Klebsiella species,
gram negative, obligate intracellular
bacillus.
After an incubation period of 8 days to 12
weeks (an average of 17 days), single or
multiple subcutaneous nodules or papules
develop at the site of inoculation (genitalia,
thigh, groin, or in the perineum). These lesions
enlarge and erode to form painless, soft, beefy
red, exuberant ulcerations with clean friable
bases and distinct raised rolled margins (Fig.
31.15)
The clinical presentation can also include
hypertrophic, necrotic, or sclerotic variants.
Inguinal enlargement may occur because of
subcutaneous granulomas that arise

The most reliable method of diagnosis involves


direct visualization of the bipolar staining
intracytoplasmic inclusion bodies. These
safety pin-shaped bodies also called as
Donovan bodies (Fig. 31.16) can be seen
within histiocytes (vacuolated monocytes) of
granulation tissue smears or biopsy
specimens. Wrights or Giemsa stains are both
satisfactorily used.
Biopsy and histopathological examination

Fig. 31.15: Donovanosispainless, soft, beefy red,


exuberant ulcerations with raised rolled margins over
the groin (pseudobubo) and genitalia

Fig. 31.16: Donovanosislarge mononuclear cell


demonstrating intracytoplasmic Donovan bodies
in tissue smear

Treatment
Center for Disease Control and Prevention (CDC)
in 2006 recommends
Doxycycline 100 mg orally twice a day for at least
3 weeks,
Alternative regimens
Azithromycin 1 g orally once per week for at least
3 weeks and until all lesions have completely
healed
Or
Ciprofloxacin 750 mg orally twice a day for at
least 3 weeks and until all lesions have completely
healed
Or

297

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Essentials in Dermatology
Erythromycin base 500 mg orally four times a
day for at least 3 weeks and until all lesions have
completely healed
Or
Trimethoprim-sulfamethoxazole one doublestrength (800mg/160mg) tablet orally twice a day
for at least 3 weeks and until all lesions have
completely healed
WHO (2003) Recommends
Azithromycin, 1 g orally on first day, then 500 mg
orally, once a day
Or
Doxycycline, 100 mg orally, twice daily
Alternative regimen
Erythromycin, 500 mg orally, 4 times daily
Or
Tetracycline, 500 mg orally, 4 times daily
Or
Trimethoprim 80 mg/sulfamethoxazole 400 mg,
2 tablets orally, twice daily for a minimum of 14
days
Treatment should be continued until all lesions
have completely epithelialized.
Note:
The addition of a parenteral aminoglycoside such
as gentamicin should be carefully considered for
treating HIV-infected patients.
NACO recommendations are same as WHO ones
except azithromycin
Other option used-Injection of streptomycin 1
gram given daily for 14 days.

Herpes Genitalis
Both HSV-1 and HSV-2 produce primary as
well as recurrent genital infections. Isolation
of HSV-2 can be successful in 80% of
instances.
HSV is the most common cause of genital
ulceration and accounts for 20% to 50% of
ulcerative lesions in patients attending
sexually transmitted disease clinics (STD
clinics).

The incubation period for both HSV-1 and


HSV-2 is between 3 to 14 days.
The outbreak begins with small grouped
vesicles, which break and progress to
ulcerative lesions in 2 to 4 days.
The first episode of genital herpes (primary or
non-primary) usually has multiple lesions,
which present bilaterally and coalesce to
involve a larger surface. Painful inguinal
lymphadenopathy is common. The dominant
local symptoms of primary genital herpes are
pain, itching, dysuria, and vaginal and
urethral discharge. The ulcers of herpes
genitalis are superficial and tiny. The severity
of these symptoms increases for the first 6-7
days of the illness and peaks during the first
week of illness in about 75% of patients. The
course of primary genital herpes may last for
18-21 days and the virus shedding is present
for about 11 days following the primary
infection.
About 50% of males will have a recurrence in
4 months but the severity of symptoms, and
duration of viral shedding are much shorter
in recurrent episodes than in primary disease
(Figs 31.17 and 31.18).

Diagnosis
Definitive diagnosis of genital herpes is made
by viral culture of the lesions, which can
distinguish between HSV-1 and HSV-2.
Biopsy and cytologic studies may be useful in
the diagnosis, but they are unable to
differentiate between HSV-1, HSV-2 and
varicella zoster virus.
The use of specific monoclonal antibodies
directed against HSV-1 and HSV-2 proteins
have proved to be sensitive and specific.
PCR can be used to detect HSV DNA.
And a valuable bedside clinical approach in
making a rapid diagnosis of herpes infection
relies on Tzanck preparation, which can be of
great value in resource poor setting.

Sexually Transmitted Diseases

Fig. 31.17

Fig. 31.18

Figs 31.17 and 31.18: Herpes genitalisgrouped vesicles and multiple,


superficial erosions involving genitalia

Treatment
WHO (2003) and Center for Disease Control and
Prevention (CDC) in 2006 recommends
First clinical episode of genital herpes
Acyclovir 400 mg orally three times a day for
710 days, or Acyclovir 200 mg orally five times a
day for 710 days, or Famciclovir 250 mg orally
three times a day for 710 days, or Valacyclovir
1 g orally twice a day for 710 days.
Recurrent episodes of genital herpes
Acyclovir 200 mg orally, 5 times daily for 5 days
(WHO) or 400 mg orally three times a day for 5
days, or Acyclovir 800 mg orally three times a
day for 2 days (CDC), or Acyclovir 800 mg orally
twice a day for 5 days, or Famciclovir 125 mg
orally twice a day for 5 days, or Valacyclovir 500
mg orally twice a day for 35 days, or Valacyclovir
1.0 g orally once a day for 5 days.
Suppressive therapy for recurrent genital herpes
Daily suppressive anti-viral therapy may be
employed in patients with frequent recurrences
of genital herpes (six or more recurrences per year).
Since daily antiviral suppressive therapy reduces
the recurrence rate of herpes genitalis by more
than 75%, option for daily suppressive therapy
may be discussed with all such patients suffering
from recurrent herpes genitalis.

Acyclovir 400 mg orally twice a day, or


Famciclovir 250 mg orally twice a day, or
Valacyclovir 500 mg orally once a day, or
Valacyclovir 1.0 gram orally once a day.
Periodically, once a year discontinuation of
suppressive therapy should be discussed.
NACO recommends only the use of acyclovir as
given above.
Sexual contact should be avoided as long as there
are active lesions.
Cervical cytology should be routinely done in
females with herpes genital infection.
Counseling of infected persons and their sex
partners is critical to the management of genital
herpes.

Differential Diagnosis of
Genital Ulcer Disease
Genital ulcers are defined as breach in the
continuity of genital mucosa and /or skin. Genital
ulcer disease (GUD) may be due to sexually
transmitted diseases (STD) like syphilis, herpes
genitalis, chancroid, lymphogranuloma
venereum, donovanosis or non-STD like traumatic
ulcers, Behcets disease, lichen planus, erythema
multiforme, lichen sclerosus et atrophicus,
bullous diseases, Fourniers gangrene, squamous
cell carcinoma (Fig. 31.19), etc.

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300

Essentials in Dermatology

Fig. 31.19: Squamous cell carcinoma presenting as


ulcerative destructive growth over the tip of penis

Differentiating features of the common sexually transmitted diseases are given below
Characteristics

Syphilis

Herpes genitalis

Chancroid

LGV

Donovanosis

1. Incubation
period

9-90 days
(2-4 weeks)
mean 21 days

2-7 days

1-5 days

3 days to
6 weeks
(avg. 7 days)

1 to 4 weeks
(8 to 80 days)

2. Causative
organism

Treponema
pallidum

Herpes simplex
virus (HSV 2
and 1)

Haemophilus
ducreyi

Chlamydia
trachomatis
sero-type L1-L3.

Calymmatobacterium
granulomatis

3. Primary
lesion

Papule

Vesicle

Pustule

Papule, pustule
or vesicle

Papule

4. Number of
lesions

Usually one

Multiple, may
coalesce

Usually multiple

Usually one

Variable

5. Pain

Painless

Painful

Usually very
tender

Variable

Uncommon

6. Diameter

5 to 15 mm

1 to 2 mm

2 to 20 mm

2 to 10 mm

variable

7. Edges/
margin

Sharply
demarcated,
elevated,
round/oval

Erythematous,
sharp

Sharply
circumscribed,
ragged
undermined edges

Elevated,
round or
oval

Elevated, irregular

8. Depth

Superficial/deep Superficial

Excavated and
deep

Superficial

Elevated

9. Floor

Smooth, clean

Variable

Beefy red
granulation tissue,
bleeds readily

Erythematous,
Yellow
covered with
necrotic purulent
serous exudates exudates

Contd...

Sexually Transmitted Diseases


Contd...
Characteristics

Syphilis

Herpes genitalis

Chancroid

LGV

Donovanosis

10. Base

Indurated

None

Nonindurated,
soft

Occasionally
firm

Firm

11. Inguinal
Bilateral, firm,
Bilateral firm
lymphadeno- non-tender,
and tender
pathy
discrete, shotty

30 to 60%,
unilateral, tender,
matted together,
suppuration with
formation of a
unilocular abscess
(bubo), may rupture

66% usually
None,
unilateral, less
pseudobubo
painful, Groove sign may be seen.
positive, multilocular
abcesses, rupture
to form multiple
sinuses

12. Spontaneous 3 to 8 weeks,


healing time
never exceeds
3 months

14 to 21 days

Self limiting
but may be
persistent.

2 to 5 days
(transient)

No tendency
to heal

13. Recurrence

Yes, in 80%

No

No

No

No.

URETHRITIS, CERVICITIS AND/OR


VAGINITIS
Gonorrhea
The term, gonorrhea is derived from Greek
flow of seed.
Causative organism- Neisseria gonorrhoeae.
The responsible organism, Neisseria
gonorrhoeae was demonstrated by Neisser in
1879 in stained smears of urethral, vaginal
and conjunctival exudates. It is a gramnegative coccus that is found in pairs
(diplococci) within polymorphonuclear
leukocytes in purulent material.
It infects noncornified epithelium most often
of the urogenital tract and secondarily of the
rectum, oropharynx and conjunctivae.
The incubation period ranges from 1-14 days
or even longer (on an average 2-5 days).
It is transmitted sexually or perinatally.
Acute anterior urethritis is the most common
manifestation of gonococcal infection in men
(Fig. 31.20) and as any asymptomatic or
minimally symptomatic endocervical
colonization in women.
Predominant symptoms are urethral
discharge or dysuria. Discharge initially is
scant and mucoid or mucopurulent. In most

males, the urethral exudate becomes frankly


purulent and relatively profuse within 24
hours of onset. On examination, mucoid or
mucopurulent discharge is seen from the
urethral meatus and the meatus is
erythematous and edematous. Epididymitis,
seminal vesiculitis and prostatitis may occur
but usually much later.

Fig. 31.20: Gonorrhea- mucopurulent discharge


per urethra

301

302

Essentials in Dermatology
In females, if manifest, it leads to increased
vaginal discharge, dysuria, intermenstrual
bleeding or menorrhagia. Examination reveals
mucopurulent cervical discharge, the cervix
has erythema and oedema, swabbing the
endocervix easily induces mucosal bleeding.
Salpingitis and Bartholin gland abscesses are
possible local complications. Ascending
infection and bacteremic dissemination
(disseminated gonococcal infection) are
responsible for most of the serious morbidity.
Rectal mucosa is a frequent site of infection in
homosexual men with symptoms ranging
from minimal anal pruritus, painless
mucopurulent discharge or scant rectal
bleeding to overt proctitis.
Pharyngeal infection occurs in 3-7 percent of
heterosexual men, 10 to 20 percent of
heterosexual women, and 10 to 25 percent of
homosexually active men.
Ophthalmia neonatorum may occur due to
perinatal transmission.

Diagnosis
Isolation of N.gonorrhoeae is the diagnostic
standard for gonococcal infections.
Urethral smear is sufficiently sensitive and
specific, that the culture may be considered
optional for routine care. Grams stain has
been the most extensively studied. A diagnosis
of urethritis is made if there are more than 5
pus cells present per oil immersion field. In
addition, these pus cells have intracellular
gram-negative diplococci, confirming the
diagnosis of gonorrhea (Fig. 31.21).

or Ciprofloxacin 500 mg orally in a single dose,


or Ofloxacin 400 mg orally in a single dose, or
Levofloxacin 250 mg orally in a single dose, plus
IF CHLAMYDIAL INFECTION IS NOT RULED
OUT Azithromycin 1 g orally in a single dose or
Doxycycline 100 mg orally twice a day for 7 days.

Alternative Regimens
Spectinomycin 2 g in a single IM dose or Singledose cephalosporin regimens- ceftizoxime (500
mg, administered IM), cefoxitin (2 g, administered
IM with probenecid 1 g orally), and cefotaxime
(500 mg, administered IM) or Single-dose
quinolone regimens - gatifloxacin 400 mg orally,
norfloxacin 800 mg orally, and lomefloxacin 400
mg orally.

Other Treatment Options Used in the Past


1. Aqueous procaine penicillin 4.8 million
units IM single dose with 1 gm probenecid
half an hour before the injection penicillin.
2. Spectinomycin 2 gm IM single dose.
3. Norfloxacin 800 mg single dose.
4. Ampicillin 3 gm or amoxicillin 3.5 gm with
1 gm probenecid.

Non-gonococcal Urethritis (NGU)


NGU is diagnosed if gram-negative
intracellular organisms cannot be identified
in the discharge on gram stain in a patient

Treatment
For uncomplicated gonococcal infections of the
cervix, urethra, and rectum, Center for Disease
Control and Prevention (CDC) in 2006
recommends
Ceftriaxone 125 mg IM (intramuscular) in a single
dose, or Cefixime 400 mg orally in a single dose,

Fig. 31.21: Grams stained urethral discharge


showing pus cell (polymorphonuclear cell)
containing intracellular gram-negative diplococci

Sexually Transmitted Diseases


with urethral discharge and burning
micturition.
Chlamydia trachomatis is responsible for 30 to
50%, Ureaplasma urealyticum for 10 to 40% and
the rest are due to Trichomonas vaginalis,
yeasts, Herpes simplex virus, Adenovirus,
Hemophilus spp., Bacteroides etc.
The incubation period is 1 to 5 weeks.
In males, urethritis begins with dysuria and
mucoid urethral discharge. In contrast to
gonococcal urethritis, symptoms are usually
mild. Most cases in females are asymptomatic.
Diagnosis
1. Grams stained urethral discharge shows
more than 5 pus cells per oil immersion field
but no gram-negative intracellular
diplococci are seen.
2. Culture: Urethral swabs are sent for
N.gonorrhoeae, mycoplasma, and anaerobes
culture. Chlamydial antigen detection may
be done on swab.
Differential diagnosis: Clinical features of
gonococcal and non-gonococcal urethritis are
tabulated below
Characteristics Gonorrhoea
urethritis

Non-gonococcal

1. Incubation
period

2-3 weeks

2-5 days

2. Constitutional Fever, malaise


symptoms

Absent

3. Discharge

Profuse, purulent
and yellowish

Scanty mucoid or
mucopurulent

4. Dysuria

Intense burning
sensation

Smarting feeling in
urethra on
passing urine

Treatment
Center for Disease Control and Prevention (CDC)
in 2006 recommends
Azithromycin 1 gm PO once or
Doxycycline 100 mg PO BD for 7 days

Alternative regimens:
Erythromycin base 500 mg PO qid for 7 days
or
Ofloxacin 300mg PO BD for 7 days or
Levofloxacin 500 mg orally for 7 days.

Pelvic Inflammatory Disease (PID)


PID comprises of a spectrum of inflammatory
disorders of the female upper genital tract,
including any combination of endometritis,
salpingitis, tubo-ovarian abscess and pelvic
peritonitis. It may be of the chronic type (caused
by M. tuberculosis) or acute PID (caused by sexual
pathogens). This section will be dealing with
acute PID.
Etiology
Cervical pathogens: N.gonorrhoeae, Chlamydia
trachomatis.
Vaginal microorganisms: Anaerobic bacteria
(e.g. Prevotella, Peptostreptococcus), H. influenzae,
Gardnerella vaginalis, Mycoplasma hominis, and
Ureaplasma urealyticum.
Symptoms and signs
Wide variations in symptoms in women
depending on the pathogen and the site of
pathology.
Malodorous, yellowish vaginal discharge
Midline abdominal pain, abnormal vaginal
bleeding (due to endometritis)
Dyspareunia
Lower abdominal and pelvic pain (salpingitis)
Fever, nausea vomiting, abdominal tenderness and rigidity (due to peritonitis).
(Fitz-Hugh-Curtis syndrome refers to perihepatitis
with acute PID caused by gonococcus or
chlamydia. Patient presents with pleuritic upper
abdominal pain and violin-string adhesions over
the liver seen on laparoscopy.)

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Essentials in Dermatology

304

Diagnostic Criteria

Minimum Criteria
Uterine/adnexal tenderness
Cervical motion tenderness
Additional Criteria

Oral temperature >101o F


Abnormal cervical or vaginal discharge
WBCs on saline mount of vaginal discharge
Elevated ESR and C-reactive protein
Laboratory documentation of cervical
infection with N.gonorrheae or C. trachomatis.

Specific Criteria (required only in


Certain Cases)
Endometrial biopsy showing endometritis
Transvaginal sonography or MRI scan
showing tubal abnormalities or tubo-ovarian
abscess.
Laparoscopic findings of PID.

Treatment
Treatment should be initiated with minimal
criteria and needs to cover all the etiological
pathogens. Early treatment prevents
sequelae.
Parenteral therapy is given for the initial
24 48 hours and thereafter oral therapy is
continued.

Bacterial vaginosis (Leukorrhea;


Haemophilus vaginalis vaginitis;
Gardnerella vaginalis vaginitis; Anaerobic
vaginosis; Vaginal bacteriosis)
Bacterial vaginosis (BV) is a common cause of
abnormal vaginal discharge in women of
reproductive age.
It represents a complex change in vaginal flora
characterized by a reduction in the prevalence
and concentration of hydrogen peroxide
producing lactobacilli and increase in the
prevalence and concentration of Gardnerella
vaginalis; Mobiluncus species; Mycoplasma
hominis; anaerobic gram-negative rods
belonging to the genera Prevotella, Porphyromonas, and Bacteroides; and Peptostreptococcus
species.
Amsel et al has proposed a set of practical
diagnostic criteria for the clinical diagnosis
of BV that is now often accepted as the gold
standard. Diagnosis requires three or more
of the following clinical/diagnostic features:
1. The presence of (excessive) homogeneous
vaginal discharge
2. Elevated vaginal pH >4.5
3. Positive amine test (Whiff test)
4. 20% clue cells (vaginal epithelial cells
adhered to by infective microbes) and
typical Gram stain appearance upon
microscopy of vaginal secretions including
absence of lactobacilli (Fig. 31.22).

Regimens include
IV Cefoxitin plus oral doxycycline (after 24 hours
IV drugs stopped and oral doxycycline
continued) or IV clindamycin and gentamicin
(24 hours) followed by oral doxycycline or IV
ofloxacin with metronidazole or IV ampicillin
plus doxycycline
Male sexual contacts (in the preceding 60 days)
should be treated.

Fig. 31.22: Grams stained vaginal discharge smear


showing a normal vaginal epithelial cell and another
coated with bacteria

Sexually Transmitted Diseases

Treatment
All women who have symptomatic BV disease require treatment.
Metronidazole 500 mg orally twice a day for 7 days, or Metronidazole gel 0.75%, one full applicator
(5 g) intravaginally, once a day for 5 days, or Clindamycin cream 2%, one full applicator (5 g)
intravaginally at bedtime for 7 days are recommended treatments.
Alternatively, patient may be treated with Metronidazole 2 g orally in a single dose, Clindamycin
300 mg orally twice a day for 7 days, or Clindamycin ovules 100 g intravaginally once at bedtime
for 3 days.
Differential diagnoses of vaginal discharge
Profile

Normal vaginal
discharge

Candidal
vulvovaginitis

Trichomonal
vaginitis

Bacterial
vaginosis

Etiology

Lactobacillus
predominant

Candida spp.
and other yeasts

Trichomonas
vaginalis

Associated with
G.vaginalis; anaerobic
bacteria, and
Mycoplasma hominis

Symptoms

None

Vulvar pruritus and/


or irritation; external
dysuria, increased
vaginal discharge

Profuse discharge,
often malodorous;
external dysuria and
genital irritation often
present

Malodorous, increased
discharge (commonly
present at the introitus)

Amount

Variable; usually Scant to moderate


scant
Clear or white
White

Profuse

Moderate to profuse

White, yellow or green

White or gray

Nonhomogenous, Clumped, Cheesy


flocculant
adherent exudative
plaques

Homogeneous, watery,
often frothy

Homogeneous,
uniformly coating
vaginal walls

Color
Consistency

pH

Usually <4.5

<4.5

Usually >5.0

Usually >4.5

Amine odor
with 10% KOH

None

None

Usually present

Present

Associated
inflammatory
signs

None

Erythema, edema and /


or erosions of vagina
or external genitalia;
vulvar dermatitis common

Erythema of vaginal
mucosa, introitus;
occasional cervical
petechiae; vulvar
dermatitis

None

Microscopy of
discharge

Normal epithelial
cells; lactobacilli
predominate

Leukocytes, epithelial
cells; yeast, mycelia,
pseudomycelia seen

Leukocytes; motile
trichomonads seen

Clue cells; rare


leukocytes; lactobacilli
outnumbered by mixed
flora

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MISCELLANEOUS STDs
Anogenital Warts
The etiologic agent of anogenital warts or
condyloma acuminata is the human
papilloma virus (HPV) a non-enveloped,
double standard DNA virus.
More than 80 different genotypes of HPV have
been identified. Forty-five of these are known
to affect the genital epithelium and are considered to be sexually transmitted infections.
It is roughly estimated that up to 30% to 50%
of sexually active adults are infected with
HPV. The highest rates of infection are found
in women, 19-22 years of age and in men, 2226 years of age.
HPV 6 and 11 are most commonly associated
with genital warts, but at least 20 different
HPV genotypes have been associated with
condyloma acuminata.
The infectivity of HPV between sexual partners
is estimated to be 60%.
The most common locations of primary
infection include the fourchette in women and

Fig. 31.23

the subpreputial region in men. Men have


been found to be at increased risk if they fail to
wear a condom and if they are smokers.
Most genital warts occur on the penis,
scrotum, urethral meatus, and perianal area
in men.
The clinical types of anogenital warts are
Condyloma acuminata (acuminate warts)
(Figs 31.23 and 31.24), papular warts, verruca
vulgaris type or keratotic warts, sessile warts,
flat warts, intraepithelial neoplasiaBowenoid papulosis and Bowens Disease,
and Giant condyloma (Buschke
Lowenstein tumor)
Subclinical HPV infection is one that is not
clinically visible with the unaided eye,
requiring colposcopy with or without acetowhitening or histopathology.
Latent HPV infection means the presence of
HPV can be confirmed only by the detection
of the HPV DNA
Atypical lesions occur in immunocompromized patients such as HIV infected
individuals

Fig. 31.24

Figs 31.23 and 31.24: Condyloma acuminatagenital outgrowths

Sexually Transmitted Diseases


Diagnosis
Diagnosis is typically made on clinical basis.
The most sensitive method for detection of
HPV DNA is polymerase chain reaction.
Acetowhitening is a diagnostic tool.
But the most commonly utilized and widely
available method is histopathologic
examination of a tissue biopsy.

Differential Diagnosis
Morphologically, various conditions, which need
to be differentiated from verrucous lesions of
genital warts, are condyloma lata of syphilis, nonvenereal treponematosis, hypertrophic verrucous
type of granuloma inguinale, tuberculosis
verrucosa cutis, skin tags, and malignancy. For
small genital warts, various differential diagnoses
need to be considered are pearly penile papules
(Fig. 31.25), vestibular papillomatosis, molluscum
contagiosum, seborrhoeic keratosis, Fordyces
spots, urethral caruncle, lichen planus, and
foreign body granuloma. For subclinical infection
(seldom appears reddish), candidial infection of
the vulva and repeated topical application of
antifungals need to be ruled out. Other benign
tumors like neurofibroma, lipoma, fibroepitheliomata, and normal physiological glands
like Tysons glands also need to be considered
before appropriate diagnosis is made. Around the
anus, prolapse and sentinel piles, and anal tags

Fig. 31.25: Pearly penile papules often


misdiagnosed as genital warts

may be confused with anogenital warts. In


unhygienic persons, dry smegma may appear like
warts and hence cleaning the sub-preputial
region is emphasized before the examination of
warts in male genitalia. For the pathological
specimen of genital warts, various differential
diagnoses considered are seborrheic keratosis,
molluscum contagiosum, psoriasis, lichen
planus, condylomata lata, Bowens disease and
squamous cell carcinoma.

Treatment
Treatment options may be categorized into
cytodestructive methods (surgical excision,
cryotherapy, laser therapy, bichloroacetic acid /
trichloroacetic acid, podophyllin and podophyllotoxin), antimetabolic therapy (5-fluorouracil), antiviral therapy (cidofovir and
interferons [IFNs]) and immunomodulation
(imiquimod)
1. Podophyllin 10-25% (applied to the warts
using cotton tipped swab once or twice a week
for up to six weeks) or podophyllotoxin 0.5%
(the solution or cream is applied with cotton
swab or finger respectively, over the
condylomas also on normal appearing skin
between the lesions twice daily for three days
followed by four days of no therapy) for local
use is first line therapy.
2. Imiquimod cream, supplied in single use
sachets, is applied to the warts with fingers
three times per week (every other night) and
the area washed with mild soap and water
the next morning. Treatment continued until
wart clearance, or for a maximum of 16 weeks
3. Cryotherapy treatment of choice for
condyloma acuminata in pregnancy.
4. CO2 laser
5. Surgery
6. Interferons (as adjuvant to surgery),
5-fluorouracil (urethral warts), cidofovir, or
retinoids are other treatment options
7. Since treatment is often unsatisfactory and is
not directed at elimination of the virus, some

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Essentials in Dermatology
progress has been made in the development
of the HPV vaccines
8. Patient counseling- Advise female patients
about regular participation in cervical
cytology screening programmes. Use barrier
protection with new sexual contacts until
successful treatment has been completed

Molluscum Contagiosum
Molluscum contagiosum is a benign papular
condition of the skin, which is often sexually
transmitted in adults.
Molluscum contagiosum virus is the final
remaining member of poxviridae family that
specifically infects humans.
The incidence of molluscum contagiosum in
sexually active adults has been increasing
significantly throughout the past few decades.
Mechanical trauma appears to facilitate the
transmission of molluscum contagiosum
virus. There is no information about the
efficiency of sexual transmission of MCV.
Lesions begin as tiny papules, which grow
over several weeks to a diameter of 3 to 5 mm.
The flesh coloured papules are smooth, firm
and dome shaped with a highly characteristic
central umbilication. In adults lesions most
commonly occur on the thighs, inguinal
region, buttocks and lower abdominal wall
(Fig. 31.26).
Scabies
Scabies can be sexually transmitted.
When scabies spreads through sexual
transmission, the characteristic lesions are
more likely to occur on the genitalia, lower
abdomen or thigh.
The penis and bra lines are other favourite
locations for mite.
Pediculosis Pubis
Pediculosis pubis is primarily a sexually
transmitted infection occurring predo-

Fig. 31.26: Molluscum contagiosum with herpes


genitalis

minantly in the young sexually active


population.
Phthirus pubis is the causative organism.
Main symptom is itching which leads to
scratching, erythema, irritation and
inflammation.

Chronic Viral Hepatitis


Sexual activity may profoundly influence the risk
for acquisition of both hepatitis A virus (HAV)
and hepatitis B virus (HBV) infection. To some
extent sexual behavior may also determine the
risk of infection with hepatitis C virus (HCV) and
HDV.
Prevention of STDs
The most reliable way to avoid transmission
of STDs is to abstain from sexual intercourse
(i.e., oral, vaginal, or anal sex) or to be in a
long-term, mutually monogamous relationship with an uninfected partner.
Both partners should get tested for STDs,
including HIV, before initiating sexual
intercourse.
If a person chooses to have sexual intercourse
with a partner whose infection status is
unknown or who is infected with HIV or
another STD, a new condom should be used
for each act of insertive intercourse.

Human Immunodeficiency Virus Infection (HIV) and AIDS

32

Human Immunodeficiency
Virus Infection (HIV) and Acquired
Immunodeficiency Syndrome (AIDS)

HISTORICAL BACKGROUND
AIDS was first recognized and described as a
clinical entity in mid 1981 when FriedmanKien and Gottlieb et al described groups of
gay (homosexual) men in the USA with
Kaposis sarcoma and pneumocystis carinii
pneumonia (PCP), both previously very rare
diseases and known to be linked to
immunosuppression.
In June 1983, Barr Sinoussi and Montagnier
of the Pasteur Institute in Paris reported the
discovery of a new virus isolated from a lymph
node of a gay (homosexual) man with
persistent lymphadenopathy. They called this
new virus lymphadenopathy associated
virus (LAV) and postulated that this was the
cause of AIDS.
In 1984, Gallo reported the isolation of a new
retrovirus that he called human T-cell
lymphotropic virus III (HTLV III). Serum
antibody testing for this virus was found to be
positive in almost 100% of AIDS patients.
In 1987, an international nomenclature
committee decided that the new virus should
be termed human immunodeficiency virus or
HIV rather than HTLV III or LAV.
In 1987, Clavel et al isolated a second
retrovirus from AIDS patients in West Africa,

which he termed it as HIV-2. HIV-2 causes


AIDS in an identical way to HIV-1 although
with a longer disease free period.
The first reported cases of HIV infections
diagnosed in India were among Madras
commercial sex workers (CSWs) in May 1986.
The epidemic of HIV in India is similar in
many ways to the experience in sub-Saharan
Africa and Thailand.
The estimated number of HIV infected adult
cases (15-49 years) in the country as on 2004
was 5.134 million (based on HIV sentinel
surveillance data of National AIDS Control
Organization).
Causative Organism
Human immunodeficiency virus (HIV) is an
RNA retrovirus. It is called retrovirus as it
utilizes a DNA polymerase enzyme reverse
transcriptase to convert viral RNA to DNA
and finally DNA is transcribed to RNA.
It mainly targets CD4+T lymphocytes and
thus causes a profound defect in cell mediated
immunity leading to opportunistic infections
and neoplastic processes.

MODE OF TRANSMISSION
HIV infection is primarily transmitted
through sexual route and less commonly
through blood transfusion, maternofetal

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Essentials in Dermatology
transmission, intravenous drug abuse, needle
stick injuries, etc.
In other words, HIV is transmitted through
semen and vaginal fluids, infected blood and
blood products, infected mother to her babybefore birth, during birth or through breast
milk.

SPECTRUM OF HIV INFECTION


Infection with HIV produces a spectrum of
disease that progresses from a clinically latent
or asymptomatic state to AIDS as a late

manifestation. The pace of disease progression varies.


In untreated patients, the time between
infection with HIV and the development of
AIDS ranges from a few months to as long as
17 years (median: 10 years).
Most adults and adolescents infected with
HIV remain symptom-free for extended
periods, but viral replication is active during
all stages of infection, increasing substantially
as the immune system deteriorates.
In the absence of treatment, AIDS eventually
develops in almost all HIV-infected persons.

Clinical Stages of HIV Infection


Stage of HIV infection

Clinical features

CD4 count
(cells/uL)

Duration
(days)

1. Primary HIV infection

Acute seroconversion illness: fever,


pharyngitis, fatigue, rash, mucocutaneous
ulcers, lymphadenopathy (occipital, axillary,
cervical), headache, meningitis, encephalitis,
neuropathies

150-800

3-14 days

2. Asymptomatic

Lymphadenopathy, headache

>300

2-10 years or
more

3. Early symptomatic

Recurrent varicella zoster, oral candidiasis,


oral hairy leukoplakia, seborrheic dermatitis,
psoriasis, skin and nail infections (impetigo,
folliculitis, fungal intertrigo, paronychia),
bacterial infections (pneumonia, bronchitis,
sinusitis), unexplained fatigue/fever /sweats/
weight loss, diarrhea, vaginal candidiasis,
cervical dysplasia, cervical carcinoma in situ,
recurrent pelvic inflammatory disease,
tuberculosis

150-500

1-5 years

4. Late symptomatic and


advanced

Kaposis sarcoma, lymphoma, PCP


pneumonia,toxoplasmosis, esophageal
candidiasis, cryptococcosis, cryptosporidiosis,
recurrent HSV infection, HIV-associated
dementia, progressive multifocal
leukoencephalopathy, tuberculosis, CMV retinitis,
primary CNS lymphoma, Mycobacterium avium
intracellulare (MAC) infection

Late symptomatic:
50-200
Advanced: <50

Late
symptomatic:
1-4 years
Advanced:
<2 years

Human Immunodeficiency Virus Infection (HIV) and AIDS


Because of the long asymptomatic phase of
most retroviral infections, a positive screening test
is often the first indication of a possible infection.
With the use of supplemented confirmatory tests,
the laboratory diagnosis of HIV infection can be
accurate, timely and sensitive. There are several
testing methodologies now available for detection
of retrovirus infection.

Revised CDC Classification for HIV


Infection and Expanded Case Definition
for AIDS in Adolescents and Adults
CD4 cell count

>500/mL (>29%)

A1

B1

C1

200 to 499/mL
(14% to 28%)

A2

B2

C2

<200/mL (<14%)

A3

B3

C3

Patients in categories A3, B3 and C1-C3 are


considered to have AIDS

Category A
Acute retroviral syndrome
Asymptomatic HIV infection
Persistent generalized lymphadenopathy
Category B
Symptomatic conditions occurring in a HIVinfected adolescent or adult that meet at least one
of the following criteria:
1. They are attributed to HIV infection or indicate
a defect in cell-mediated immunity
2. They are considered to have a clinical course
or management that is complicated by HIV
infection
Examples include, but are not limited to the
following:
Constitutional symptoms (fever of 38.5C
[101.3F], diarrhea >1 month)
Idiopathic thrombocytopenic purpura
Peripheral neuropathy Bartonella henselae,
B. quintana: bacillary angiomatosis

Pelvic inflammatory disease


Candidiasis: oropharyngeal or recurrent
vulvovaginal
Varicella-zoster virus infection: herpes
zoster (2 distinct episodes/ more than one
dermatomes)
Epstein-Barr virus infection: oral hairy
leukoplakia
Human papillomavirus infection: Cervical
squamous intraepithelial lesion
(dysplasia/carcinoma in situ)

Category C
HIV encephalopathy
Progressive multifocal leukoencephalopathy
Lymphoma: Burkitts, immunoblastic or
primary CNS lymphoma
Mycobacterium avium-intracellulare complex
or M. kansasii infection: disseminated/
extrapulmonary
M. tuberculosis: pulmonary/ extrapulmonary
Mycobacterium: other species/ unidentified species - disseminated/ extrapulmonary
Pneumonia (recurrent with >2 episodes in
12 months)
Salmonella septicemia, recurrent (nontyphoid)
Candidiasis: esophageal, pulmonary
Cryptococcosis: extrapulmonary
Coccidioidomycosis: disseminated/
extrapulmonary
Histoplasmosis: disseminated/ extrapulmonary
Pneumocystis jiroveci (formerly carinii)
pneumonia
Herpes simplex virus infection:
esophageal, pulmonary, mucocutaneous
ulcers of >1 month duration
Cytomegalovirus: other than liver, spleen
or nodes

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Essentials in Dermatology

Human herpesvirus-8 infection: Kaposis


sarcoma
Human papillomavirus infection: invasive
cervical cancer
Cryptosporidiosis: chronic intestinal (>1
month duration)
Isosporiasis: chronic intestinal (>1 month
duration)
Toxoplasmosis of brain
Kaposis sarcoma
Wasting syndrome due to HIV (involving
weight loss >10% of baseline body weight),
associated with either chronic diarrhea (>
2 loose stools per day for > 1 month) or
chronic weakness and documented fever
> 1 month

Note
AIDS in HIV-infected adolescents and adults
aged >13 years defined by CDC includes any
one of the following criteria: (a) <200 CD4+ cells/
mL; (b) a CD4+ cell percentage of total
lymphocytes of <14 percent; or (c) any of the
following three clinical conditions: pulmonary
tuberculosis, recurrent pneumonia, or invasive
cervical cancer.

WHO Criteria Used for Diagnosing HIV


Infection/AIDS
The WHO has proposed an alternative definition
for AIDS, in geographic areas where only clinical
parameters can be used. Provisional World Health
Organization (WHO) clinical case definition for
AIDS where diagnostic resources are limited.
WHO case definition for AIDS surveillance:

Adults
AIDS in an adult is defined by the existence of at
least two of the major signs associated with at
least one minor sign in the absence of known

causes of immunosuppression such as cancer


or severe malnutrition or other recognized
causes.
Major Signs
1. Weight loss greater than 10 percent of body
weight.
2. Chronic diarrhea for more than 1 month.
3. Prolonged fever for more than 1 month
(intermittent or consistent)
Minor Signs
1. Persistent cough for more than 1 month.
2. Generalized pruritic dermatitis.
3. Recurrent herpes zoster infection.
4. Oropharyngeal candidiasis.
5. Chronic progressive and disseminated herpes
simplex virus infections
6. Generalized lymphadenopathy
The presence of generalized Kaposis sarcoma
or cryptococcal meningitis itself is sufficient for
the diagnosis.

Children
Pediatric AIDS is suspected in an infant or child
presenting with at least two major signs associated
with at least two minor signs in the absence of
other known causes of immunosuppression.
Major Signs
1 Weight loss or abnormally slow growth
2. Chronic diarrhea for more than 1 month
3. Prolonged fever for more than 1 month
Minor Signs
1. Generalized lymphadenopathy
2. Oropharyngeal candidiasis
3. Repeated common infections (otitis,
pharyngitis etc.)
4. Persistent cough for more than 1 month
5. Generalized dermatitis.
6. Confirmed maternal HIV infection.

Human Immunodeficiency Virus Infection (HIV) and AIDS

WHO Case Definition for HIV Infection


Adults and Children 18 Months or Older
Positive HIV antibody testing (rapid/ ELISA)
confirmed by a second HIV antibody test (rapid /
ELISA) relying on different antigens or a different
operating technique AND/OR
Positive virological test for HIV or its
components (HIV RNA or HIV DNA or
ultrasensitive HIV p24 antigen) confirmed by a
second virological test obtained from a separate
determination.

Children Younger than 18 Months


A positive virological test for HIV or its
components (HIV RNA or HIV DNA or
ultrasensitive HIV p24 antigen) confirmed by a
second virological test obtained from a separate
determination, taken more than 4 weeks after birth.
Positive HIV antibody testing is not
recommended for definitive/ confirmatory
diagnosis of HIV infection in children until 18
months of age.
Case definition for AIDS in India: Case definition
for AIDS in India was revised in October, 1999.
The new case definition is as follows:
I. Case Definition of AIDS in Children (up to 12
years of age)
1. The positive tests for HIV infection by ERS
(ELISA/RAPID/SIMPLE) in children above
18 months or confirmed maternal HIV
infection for children less than 18 months.
AND
2. Presence of at least two major and two minor
signs in the absence of known causes of
immunosuppression.

Major Signs
a. Loss of weight or failure to thrive which is not
known to be due to medical causes other than
HIV infection.
b. Chronic diarrhea (intermittent or continuous)
> 1 month duration.
c. Prolonged fever (intermittent or continuous)
> 1 month duration.

Minor Signs
a. Repeat common infections (e.g. Pneumonitis,
otitis, pharyngitis etc.)
b. Generalized lymphadenopathy
c. Oropharyngeal candidiasis
d. Persistent cough for more than 1month
e. Disseminated maculo-papular dermatosis
II. Case Definition of AIDS in adults (for persons
above 12 years of age)
1. Two positive tests for HIV infection by ERS
test (ELISA/RAPID/SIMPLE) AND
2. Any one of the following criteria:
a. Significant weight loss (> 10% of body
weight) within last one month/Cachexia
(not known to be due to a condition other
than HIV infection). AND
Chronic diarrhea (intermittent or
continuous) > 1 month duration or
prolonged fever (intermittent or
continuous) > 1 month duration
b. Tuberculosis: Extensive pulmonary,
disseminated, miliary, extra-pulmonary
tuberculosis.
c. Neurological impairment preventing
independent daily activities, not known
to be due to the conditions unrelated to
HIV infection (e.g. trauma)
d. Candidiasis of the esophagus (diagnosable by oral candidiasis with odynophagia)
e. Clinically diagnosed life-threatening or
recurrent episodes of pneumonia, with or
without etiological confirmation
f. Kaposis Sarcoma
g. Other conditions: - Cryptococcal meningitis
- Neuro toxoplasmosis
- CMV retinitis
- Pencillium marneffei
- Recurrent herpes zoster or multidermatomal herpes infection
- Disseminated molluscum contagiosum

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Diagnosis of HIV infection


HIV infection is diagnosed on the basis of
blood tests using three different ELISA/
Rapid/simple tests using different antigen
preparation.
AIDS cases are diagnosed on the basis of two
different ELISA/Rapid tests on different
antigens and presence of AIDS related
opportunistic infections.
Western Blot test is used for confirmation of
diagnosis of indeterminate ELISA tests.

Mucocutaneous Manifestations of
HIV Infection
Mucocutaneous diseases are amongst the first
recognized clinical manifestations of AIDS. Over
the past decade, it has become increasingly clear
that cutaneous disorders are not only associated
with terminal immunodeficiency but occur
throughout the course of HIV infection. This may
be due to impairment of the skin immune system
as evidenced by the reduced epidermal
Langerhans cell population, which explains the
development of opportunistic infections and
neoplasms of the skin and mucous membranes
in these patients.
Mucocutaneous disorders remain one of the
most important clinical markers of the stages of
HIV infection from time of seroconversion. They
range from urticarial viral exanthema to a myriad
of cutaneous opportunistic infections or
malignancies. They function as visual markers
in assessing the progression of HIV disease.
Cutaneous disorders occur more frequently as
HIV infection advances and immune function
deteriorates. They affect between 80% and 95% of
HIV infected patients. Cutaneous disorders
during HIV infection are numerous. Some have
drawn attention because of their onset, defines
some of the Center for Disease Control and
Prevention (CDC), acquired immunodeficiency
syndrome (AIDS) clinical categories, e.g. oral
candidiasis, zoster, herpes simplex, oral hairy

leukoplakia and Kaposis sarcoma, but most have


been documented solely in case reports.
Mucocutaneous disorders are commonly the
indication for initial HIV serotesting and they are
listed in table.
Mucocutaneous disorders as indicators for HIV
serotesting
A. Disorders strongly associated with HIV
infection
1. Candidiasis-oropharyngeal (Figs. 32.1 and 32.2) or
recurrent vulvovaginal
2. Herpes zoster-necrotic (Fig. 32.3), multidermatomal
or disseminated
3. Chronic herpes simplex infection (Fig. 32.4)
4. Oral hairy leukoplakia (Fig. 32.5)
5. Papular eruption of HIV (Fig. 32.6)
6. Molluscum contagiosum-face involvement in an adult
or giant molluscum contagiosum (Fig. 32.7)
7. Proximal white subungual onychomycosis
8. Kaposis sarcoma
B. Disorders commonly associated with HIV
infection
1. Any sexually transmitted disease-indicative of
unsafe sexual behaviour
2. Signs of injected drug use
C.
1.
2.
3.

Disorders associated with HIV infection


Generalized lymphadenopathy
Psoriasis vulgaris-explosive onset
Seborrheic dermatitis-in an adult extensive and
refractory to therapy
4. Aphthous ulcers-recurrent, refractory to therapy
5. Crusted scabies

Xerosis/acquired ichthyosis and giant


molluscum contagiosum are characteristically
seen in group IV of HIV disease whereas oral
candidiasis, oral aphthae, papular dermatitis of
HIV and psoriasis are early warning signs.
Moreover, with progression of HIV disease, the
number of dermatoses goes on increasing.
Common dermatoses assume aggressive postures
(Figs 32.8 to 32.13) and some rare dermatoses (Figs
32.14 and 32.15) also present.

Treatment
There is no cure for AIDS at this time.
However, treatments are available that can

Human Immunodeficiency Virus Infection (HIV) and AIDS

Fig. 32.1: Oral candidiasiscurdy white deposits


over the tongue

Fig. 32.2: Hyperplastic candidiasis of the tongue

Fig. 32.3: Herpes zosternecrotic lesions of


herpes zoster along a thoracic dermatome

Fig. 32.4: Herpes genitalisnon-healing impetiginized ulcers over the glans penis and prepuce

Fig. 32.5: Oral hairy leukoplakiaun-removable white striations on the side and dorsa of tongue

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Essentials in Dermatology

Fig. 32.6: Papular eruption of HIV- itchy papular


eruption over the back

Fig. 32.7: Molluscum contagiosumgiant lesions


over the face

Fig. 32.8: Genital wartsmultiple verruca vulgaris


like genital warts

Fig. 32.9: Tinea versicolorextensive macular


lesions over the neck with cervical lymphadenopathy

Fig. 32.10

Fig. 32.11

Figs 32.10 and 32.11: Extensive tinea corporis and cruris lesions in a HIV patient

Human Immunodeficiency Virus Infection (HIV) and AIDS

Fig. 32.12: Secondary syphilispigmented lesions


in the palms

Fig. 32.14: Cryptococcosishemorrhagic crusted


papular lesions over the face

Fig. 32.13: Secondary syphilishyperkeratotic


scaly plaques over the lower legs

Fig. 32.15: Cryptococcosismolluscum


contagiosum like lesions over the chest

improve the quality of life of those suffering


the infection.
Use of condom during sexual acts should be
encouraged.
Patients need counselling at the first visit
apart from the general and specific treatments.

Antiretroviral therapy is discussed in next


chapter.
Opportunistic infections are treated with
appropriate therapeutic agents. With Highly
Active Retroviral Therapy (HAART), the
prevalence of mucocutaneous disorders in HIV
infected patients is coming down.

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33

Antiretroviral Therapy (ART)

Development of Antiretroviral Therapy


(ART)
Zidovudine was first tested on humans in
1985 and introduced for treatment in March
1987.
Zalcitabine (ddC), didanosine (ddI), and
stavudine (d4T) were introduced between
1991-1994.
Between December 1995 and March 1996,
three protease inhibitors (PIs)-indinavir
(IDV), saquinavir (SQV), and ritonavir (RTV)
got approval.
1996 onwards, combination therapy, highly
active antiretroviral therapy (HAART) began
to spread irreversibly.
In 1995, David HO came out with his slogan
hit hard and early.
By June 1996, first NNRTI (nevirapine), a
third drug class of antiretroviral drugs was
licensed. Soon after another PI, nelfinavir,
also arrived.
In 1996-97, some patients began to complain
of fat stomach and buffalo hump with PIs but
retained their thin facies and thin legs.
In 1998, a term lipodystrophy was used to
describe the characterstic fat distribution that
patients on HAART had developed.
In1999 Mitochondrial toxicity hypothesis
emerged as the reason behind lipodystrophy.

By year 2000, many strict recommendations


got revised. Hit HIV hard, but only when
necessary now heard more than hit hard and
early.
Despite all skepticism, HAART can often
achieve miracles e.g., cryptosporidiosis and
Kaposis sarcoma simply disappear. Once
daily regimens, even twice weekly regimens
are coming.
New classes of drugs are appearing- co
receptor antagonists, attachment-, integrase, and maturation inhibitors.
Since 1995, protease inhibitors (PIs) have
revolutionized the treatment of HIV
infection, but in recent years they have been
criticized for their high pill burden and side
effects. They remain an essential component
of HAART especially for treatment
experienced patients. With growing
knowledge of mitochondrial toxicity of
nucleoside analogs and the introduction of
easy to take PIs, boosted PIs, this class of
drugs is currently experiencing something of
renaissance. Boosting with small doses of
ritonavir is based on the fact that ritonavir a
very potent inhibitor of the isoenzyme 3A4,
a subunit of the cytochrome P450 hepatic
enzyme system, allows boosting of the most
important pharmacokinetic parameters of all

Antiretroviral Therapy (ART)


PIs (except nelfinavir), maximum concentration, trough levels, and half life. Boosting
is usually indicated by addition of /r after
the drug name.
Reduction in the cost of ART from INR
35000/month (in year 2000) to approx. INR
1000/month made ART affordable. There is
further chance of 20% price reduction.

Goals of Antiretroviral Therapy


Eradication of HIV infection cannot be achieved
with available antiretroviral regimens.
Therefore, once the decision is made to initiate
therapy, the primary goals of antiretroviral
therapy are:
1. Reduction of HIV-related morbidity and
mortality
2. Maximal and durable suppression of viral
replication
3. Preservation and/or restoration of
immunologic functions
4. Improvement of quality of life
Currently available antiretroviral drugs have
been classified in the Table 33.1.
Antiretroviral Drugs
1. Nucleoside reverse transcriptase inhibitors
(NRTIs): Their target is the HIV enzyme
reverse transcriptase.
2. Non-nucleoside reverse transcriptase
inhibitors (NNRTIs): The target enzyme is

reverse transcriptase. However, NNRTIs


bind directly and noncompetitively to the
enzyme at a position in close proximity to the
substrate binding site for nucleosides. The
resulting complex blocks the catalyst
activated binding site of the reverse
transcriptase. This, in turn, can bind fewer
nucleosides, slowing polymerization down
significantly. In contrast to NRTIs, NNRTIs
do not require activation within the cell. The
three available NNRTIs are nevirapine,
delavirdine and efavirenz.
3. Protease inhibitors (PIs): The HIV protease
cuts the viral gag-pol polyprotein into its
functional subunits. If the protease is
inhibited and proteolytic splicing prevented,
non-infectious virus particles will result.
4. Entry inhibitors: There are three crucial steps
for entry of HIV into the CD4+ T-cell.
a. Binding of HIV to the CD4 receptor
(target of attachment inhibitors),
b. Binding to coreceptors (target of coreceptor antagonists), and finally
c. Fusion of virus and cell (target of fusion
inhibitors)
5. Integrase inhibitors: Integrase, along with
reverse transcriptase and protease, is one of
the three key enzymes in the HIV replication
cycle. This enzyme is involved in the
integration of viral DNA into the host
genome, and is essential for the proliferation

Table 33.1: Currently available antiretroviral drugs

NRTI

NNRTI

PI

Azidothymidine(AZT)

Nevirapine (NVP)

Indinavir (IDV)

Stavudine (d4T)

Efavirenz (EFV)

Nelfinavir(NFV)

Didanosine (ddI)

Delaviridine(DLV)

Saquinavir(SQV)

Zalcitabine (ddC)

Ritonavir(RTV)

Abacavir (ABC)

Amprenavir(APV)

Tenofovir (TFV)

Lopinavir(LPV)

Emtricitabine(FTC)

Atazanavir(ATV)

Lamivudine (3TC)

Fosamprenavir(FPV)

* Fusion inhibitor: Enfuviritide

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Essentials in Dermatology
of HIV. Integrase inhibitors are occasionally
falsely classified as entry inhibitors.
However, unlike the latter, they do not
prevent entry of the virus into the cell.
6. Maturation inhibitors: The so-called
maturation inhibitors inhibit HIV replication
in a very late phase of the HIV reproduction
cycle, i.e., by the budding or maturation of
new virions. As is the case for integrase
inhibitors, 2005 can be counted as the
introductory year.

Highly Active Antiretroviral Therapy


(HAART)
It is a combination of different classes of
antiretroviral in order: to achieve maximal
(below level of detection) and most durable
suppression of viral replication; prevent
emergence of drug resistant mutants and to
improve survival and quality of life. HAART has
drawbacks of high cost, not being a cure, has
short and long term side effects, drug
interactions, need for stringent adherence, and
risk of viral resistance. The characteristics of an
ideal first line ART are that it should be
affordable, effective and well tolerated, potent
and robust, minimal drug inter-/contra-actions,
appropriate for use in TB and pregnancy, and
stable in tropical conditions
Indications for Antiretroviral Therapy
Antiretroviral therapy is recommended for
all patients with history of an AIDS-defining
illness or severe symptoms of HIV infection
regardless of CD4+ T cell count. (AI) (WHO
clinical stage IV).
Antiretroviral therapy is also recommended
for asymptomatic patients with <200 CD4+
T cells/mm3(AI) (WHO clinical stage I, II, III
with CD4 cell counts <200/mm3).
Asymptomatic patients with CD4+ T cell
counts of 201350 cells/mm 3 should be
offered treatment. (BII) (WHO clinical stage
III).

For asymptomatic patients with CD4+ T cell


of >350 cells/mm3 and plasma HIV RNA
>100,000 copies/mL most experienced
clinicians defer therapy but some clinicians
may consider initiating treatment. (CII).
Therapy should be deferred for patients with
CD4+ T cell counts of >350 cells /mm3 and
plasma HIV RNA <100,000 copies/mL. (DII).

Recommendations of HAART Regimens:


What to start with?
WHO recommended (2003) first and second line
regimens are given in Table 33.2.
DHHS panel recommended NNRTI based
regimen is efavirenz +zidovudine or tenofovir
+ lamivudine or emtricitabine and PI based
regimen is lopinavir/ritonavir+ zidovudine+
lamivudine or emtricitabine. Various possible
combinations with NRTI drugs (nukes) as
backbone are given in Table 33.3.
First Line Combination in India
Todays best available choice is combination of
stavudine/lamivudine/nevirapine. It has the
Table 33.2: WHO recommended 2003 ART regimens

First line regimen

Second line regimen

Stavudine (D4T) or
Azidothymidine (AZT)
+

Tenofovir (TFV)
or Abacavir (ABC)
+

Lamivudine (3TC)

Didanosine (ddI)

Nevirapine (NVP) or
Efavirenz (EFV)

Lopinavir(LPV)or
Saquinavir(SQV)

Table 33.3: HAART- Various possible combinations


with nukes as backbone
Column A
AZT + 3TC
d4T + 3TC
AZT + ddI

Column B

with

NVP
EFV
NFV
RTV + LPV
RTV + IDV
RTV + SQV

Antiretroviral Therapy (ART)


advantage of being well tolerated, affordable,
and easy to take. But it is ineffective against
HIV2, and has drug interactions with rifampicin.

Alternative First Line


Combinations in India
Stavudine/lamivudine/efavirenz (can be
used with rifampicin, teratogenic)
Zidovudine/lamivudine/nevirapine (can be
used in stavudine induced neuropathy, but
has risk of anemia, and thus requires Hb
monitoring).

3-NRTI combinations
Abacavir + tenofovir + lamivudine
Didanosine + tenofovir + lamivudine
Zidovudine + lamivudine + abacavir

Govt. Will Provision For Antiretroviral


(ARV) Drugs With Following Combination
Stavudine (30 or 40 mg) BID or Zidovudine
(300 mg) BID + Lamivudine (150 mg) BID+
Nevirapine (200 mg) BID or Efavirenz (600
mg) OD.

Pretreatment Evaluation
It is essential to confirm HIV results, complete
history and physical examination, treat OI (TB,
Toxoplasmosis, and cryptococcosis), complete
blood counts (CBC), chemistry profile, VDRL,
HBsAg. Assessment of readiness for treatment
and adherence is a must. It is desirable to get
CD4+ T-lymphocyte count and optional to get
plasma HIV RNA measurement if affordable.
For patients with pretreatment HIV RNA >1,000
copies/mL genotypic resistance testing prior to
initiation of therapy (BIII) should be considered;
if therapy is to be deferred, resistance testing may
still be considered (CIII).

ART Regimens Not Recommended


Monotherapy should not be used as it is less
potent and does not have sustained antiviral
activity.
Dual NRTI therapy has not demonstrated
potent and sustained antiviral activity as
compared to three-drug combination
regimens.
Didanosine + stavudine combination can
result in a high incidence of toxicities,
particularly peripheral neuropathy,
pancreatitis, and lactic acidosis.
Stavudine + zidovudine combination should
be avoided because of the demonstration of
antagonism in vitro and in vivo.
Didanosine + tenofovir combination has high
failure rate of therapy, higher toxicity
(pancreatitis).
Zalcitabine containing regimens are less
convenient (given three times daily) and
more toxic.

Follow-up of Patients on HAART


Two surrogate markers are routinely used to
determine indications for treatment and to
monitor the efficacy of therapy: CD4+ T cell
count and plasma HIV RNA (or viral load).
The CD4+ T cell count should be determined
every three to six months to (1)to determine
when to start ART; (2) to assess immunologic
response to ART; and (3) to assess the need for
initiating chemoprophylaxis for opportunistic
infections. Plasma HIV RNA (viral load) should
be monitored every 2-8 weeks at initiation or for
change in therapy and to confirm potency of the
new regimen. In patients on stable ART, viral
load should be monitored every 3-4 month or if
clinically indicated. Efficacy of HAART is
determined by clinical evaluation (weight gain,
resolution of OI), viral load reduction
(undetectable levels after 24 weeks), and CD4
cell counts (increase by 100-150 cells/mm3/
year).

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Adverse Effects of Antiretroviral Drugs


Gastrointestinal problems are the most common
side effects of almost all antiretroviral drugs nucleoside analogs, NNRTIs and particularly
protease inhibitors and occur especially during
the early stages of therapy. Typical symptoms
include abdominal discomfort, loss of appetite,
diarrhea, nausea and vomiting. Heartburn,
abdominal pain, meteorism and constipation
may also occur. Nausea is a common symptom
with zidovudine-containing regimens; diarrhea
occurs frequently with zidovudine, didanosine
and all PIs, particularly with ritonavir and
nelfinavir, as well as with saquinavir, lopinavir
atazanavir and tipranavir. Treatment with
zidovudine rarely leads to a severe form of
gastritic pain, nausea and vomiting in the early
phase of therapy, in which case it should be
discontinued.
Serious Adverse Effects of HAART
NRTI
1. Lactic acidosis mostly related to stavudine
and didanosine, less in patients on
zidovudine, abacavir and lamivudine. Risk
factors are obesity, female sex, pregnancy and
therapy with ribavirin or hydroxyurea, a
diminished creatinine clearance and a low
CD4+ T-cell nadir.
2. Hepatic steatosis, is probably caused by
mitochondrial toxicity and usually occurs
after more than 6 months on treatment
3. Pancreatitis - Up to 7 % of patients treated
with didanosine suffer from pancreatitis.
Occasionally, stavudine, lamivudine and
zalcitabine cause pancreatitis too.
4. Hypersensitivity reactions-especially in
patients on abacavir.
5. Anemia especially in patients on treatment
with zidovudine
6. Peripheral polyneuropathy mostly caused by
the zalcitabine, didanosine and stavudine. It
can present as paresthesia and pain in hands

and feet or distal symmetrical sensorimotor


paralysis
7. Myopathy,
8. Lipoatrophy

NNRTI
1. Rash-Nevirapine and delavirdine may cause
a slight rash in 15 to 20% of patients, 5 to 10%
of which discontinue treatment. The rash is
seen less frequently on efavirenz therapy,
where only 2 % of the patients discontinue
the drug. The NNRTI allergy is a reversible,
systemic reaction and typically presents as
an erythematous, maculopapular, pruritic
and confluent rash, distributed mainly over
the trunk and arms. Fever may precede the
rash. Further symptoms include myalgia
(sometimes severe), fatigue and mucosal
ulceration. The allergy usually begins in the
second or third week of treatment. Women
are more often and more severely affected. If
symptoms occur later than 8 weeks after
initiation of therapy, other drugs should be
suspected.
2. Stevens Johnsons Syndrome
3. TEN
4. Hepatitis-hypersensitive hepatitis often
occurs within the first 12 weeks. Liver toxicity
occurs more commonly in patients on
nevirapine than on other antiretroviral drugs.
5. Neuro-psychiatric-common in patients on
treatment with efavirenz. It includes
dizziness, insomnia, nightmares, mood
fluctuations, depression, depersonalization,
paranoid delusions, confusion and suicidal
ideation. These side effects are observed
mainly during the first days and weeks of
treatment. Discontinuation of therapy
becomes necessary in only 3 % of patients.

PI
1. Renal stones- occur particularly on indinavir
treatment,

Antiretroviral Therapy (ART)


2. Hepatitis, PIs can lead to hepatotoxicity at
any stage during the course of treatment.
Patients with chronic viral hepatitis are
particularly at risk. One possible cause is an
immune reconstitution syndrome on
HAART, with increased cytolytic activity
against the hepatitis viruses. Among the PIs,
toxic hepatitis is seen most frequently in
patients on boosted atazanavir, indinavir and
tipranavir, a novel non-petidic protease
inhibitor.
3. Rash
4. Dyslipidemia
5. Glucose intolerance
6. Lipodystrophy
7. Osteoporosis

Change of Therapy
Treatment Regimen Failure
Virologic failure can be defined as incomplete
or lack of HIV RNA response to antiretroviral
therapy:
Incomplete virologic response is defined as
repeated HIV RNA >400 copies/mL after 24
weeks or >50 copies/mL by 48 weeks in a
treatment-nave patient initiating therapy.
Virologic rebound is repeated detection of
HIV RNA after virologic suppression i.e.
repeated HIV RNA level >400 copies/mL
after prior suppression of viremia to <400
copies/mL.
Immunologic failure can be defined as failure
to increase the CD4 cell count by 25-50 cells/mm3
above the baseline count over the first year of
therapy, or a decrease to below the baseline CD4
cell count on therapy. Mean increases in CD4
cell counts in treatment-nave patients with
initial antiretroviral regimens are approximately
150 cells/mm3 over the first year.
Clinical progression can be defined as the
occurrence or recurrence of HIV-related events

(after at least 3 months on an antiretroviral


regimen), excluding immune reconstitution
syndromes.

Treatment regimen failure: Assessment


Review antiretroviral history
Assess adherence, tolerability, drug-drug
interactions, pharmacokinetic issues
Physical exam for signs of clinical progression
Resistance testing where feasible (while
patient is on therapy or within 4 weeks after
regimen discontinuation )
Identify other treatment options
If adherence is good, switch entire regimen.

Changing therapy: Toxicity


Change the offending drug in case of
Anemia on AZT, switch to d4T
Peripheral neuropathy on ddI/d4T, switch
to AZT/3TC
Nephrolithiasis on indinavir, switch to
another PI
Hepatitis or severe rash on nevirapine, switch
to efavirenz
Thus change of regimen is required in cases
of virologic failure, immunologic failure, clinical
failure, drug toxicity and drug resistance
Immune reconstitution inflammatory
syndrome: The immune reconstitution
inflammatory syndrome (IRIS) is characterized
by worsening clinical, laboratory, or radiologic
findings despite improvements in the HIV RNA
level and CD4 count after the introduction of
antiretroviral therapy and is due to the
restoration of pathogen-specific immune
responses. It is basically pathological
inflammatory response, where paradoxical
worsening occurs after HAART, in either
previously treated infections or subclinical
infections with recovery of immune responses.
It has been described with mycobacteria,
C. neoformans, herpes viruses, and Pneumocystis

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jerovecii pneumonia. IRIS may occur during or
shortly after the treatment of an opportunistic
infection or as a new clinical syndrome
resulting from a previously unrecognized occult
infection. Risk factors for IRIS include a low CD4
count (<50/ml), simultaneous (<30 days)
initiation of OI treatment and HAART, the
presence of latent infection(s), and a robust
virologic and immunologic response to HAART.
In addition to infectious pathogens, IRIS is

associated with autoimmune or malignancyrelated conditions. HAART should be continued.


Specific antimicrobial therapy, nonsteroidal antiinflammatory drugs, and/or steroids for
managing immune reconstitution syndrome
should be considered. The prognosis for most
IRIS cases is favorable because a robust
inflammatory response may predict an excellent
response to HAART in terms of immune
reconstitution and, perhaps, improved survival.

Historical Milestones in Leprosy

34

Historical Milestones in Leprosy

Leprosy has tormented humans throughout recorded history. The earliest possible account of a disease
that many scholars believe is leprosy appears in an Egyptian Papyrus document written around 1550
BC. Around 600 BC, Indian writings described a disease that resembles leprosy. In Europe, leprosy
first appeared in the records of ancient Greece after the army of Alexander the Great came back from
India and then in Rome in 62 BC coinciding with the return of Pompeiis troops from Asia Minor.
Danielssen and Boeck (1849)

Established leprosy as a clinical entity

G Armauer Hansen on 8 February 1873

Not only was the bacillus, the first mycobacterium,


discovered, it was the first etiologic agent of a chronic
disease of humans

Rafael Lucio (1819-1896) of Mexico

Distinguished leprologist after whom a type of leprosy


Lucio leprosy and the Lucio phenomenon are
named

First International Leprosy Congress

Held in Berlin in 1897, contagious nature of the disease


contributed to policy of segregation

Second International Leprosy Congress

Held in Bergen with Hansen as President, reaffirmed


the policy of isolation and segregation

Hayashi and Mitsuda (1919)

Established the lepromin reaction

Leprosy in India (1929)

The Indian Leprosy Association began


publication of its journal Leprosy in India (now
entitled the Indian Journal of Leprosy)

International Leprosy Association (ILA)

Was founded in Manila in1936, under the auspices of


the Leonard Wood Memorial and launch of
International Journal of Leprosy

First ILA Congress

Was held in Havana in 1948, the term leper in


designation of the patient with leprosy be abandoned
and the person suffering from the disease be designated
as leprosy patient

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Oil of the chaulmoogra nut

Until the late 1940s, leprosy doctors all over the world
treated patients by injecting them with oil from the
chaulmoogra nut.

Guy Faget (1943)

Soluble sulphone, Promin success in leprosy established


at Carville, Louisiana, USA

Robert Cochrane (1943)

Dapsone (DDS) was introduced for the treatment of


leprosy patient in Chingelput, India

Lowe and Smith (1949)

First to use dapsone orally.

Khanolkar in India, 1950s and 1960s

Leprosy became recognized as primarily an affliction


of peripheral nerves

National Leprosy Control Programme


(NLCP) in 1954-55

Started in 1954-55, the mainstay of NLCP was the early


detection and sustained, regular treatment of patients
with dapsone monotherapy

Chapman Binford (1956)

On the basis of Virchows observations in 1863 and on


his own clinical observations in Hawaii, proposed that
M.leprae grew selectively in the cooler areas of the hosti.e., ears, skin, eyes, peripheral nerves and testes. This
concept led Binford to infect hamsters successfully
(1958)

Shepard (1960)

Developed the mouse footpad model

Browne (1962)

Showed clofazimine to be an effective treatment for


leprosy

Ridley and Jopling (1962)

Codified a classification system based on clinical,


bacteriologic, immunologic and histopathologic criteria

Opromolla (1963)

First to use rifamycin SV (a potent antituberculosis drug) for the treatment of leprosy

Pettit and Rees (1964)

Detection of sulphone-resistant strains

Sheskin (1965)

First to use thalidomide as a sedative for severe erythema


nodosum leprosum(ENL)

Kirchheimer and Storrs (1969)

Nine banded armadillo used as an experimental animal,


inoculated animals developed a massive, disseminated
infection

International Federation of Antileprosy


Associations (ILEP) (1975)

Formed from earlier existing Coordinating Committee


of European Leprosy Agency (ELEP) founded in 1966

Working Group under the chairmanship of


Dr MS Swaminathan in 1980

In 1980 the Government of India declared its resolve to


eradicate leprosy by the year 2000 and the then Prime
minister Mrs. Indira Gandhi directed the constitution
of a working group

Historical Milestones in Leprosy


WHO Study Group on Chemotherapy
in 1981

Introduction of multidrug therapy (MDT) in leprosy

National Leprosy Eradication Programme


(NLEP) in 1983

Was launched in 1983 with the introduction of


multidrug therapy for leprosy following emergence of
dapsone resistance

WHO Study Group on Chemotherapy in


1993

Made important recommendation on fixed duration


MDT

Modified Leprosy Elimination Campaign


(MLEC) in 1997

A meeting of States Health Secretaries and State


Programme Officers was held from 31st July to 1st
August, 1997 to discuss the findings and
recommendations of mid term review and actions
required for strengthening the programme. Based on its
suggestions, Government of India decided to implement
MLEC in all states/UTs

Leprosy declared eliminated as a public


health problem in India

In 2006.

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35

History Taking and


Examination in Leprosy

The aim of history taking is to study the patient


as an individual, his environment and the reasons
for his illness. The patient should be allowed to
tell his/her history in his/her own words. It is
only then the clinician should ask the patient to
elaborate on particular aspects of the symptoms.
History may be obtained from relatives, friends
or onlookers in a depressed or confined patient.
Registration: It is required for follow up reference
of the patient.
Name: This will not only help to elicit the history
properly, but will also be of psychological benefit
to the patient.
Age: Leprosy is known to occur at all ages ranging
from early infancy to very old age, but it shows a
peak at ages of 10 to 14 years followed by a
depression, which in turn is followed by a rise
and plateau covering ages 30-60 years.
Sex: Males are more often affected by leprosy than
females.
Native place: Since leprosy is endemic in certain
areas of India, it is important to know the native
place of a person, more so the place of residence
in the past 10 years.
Marital status, occupation, religion, literacy
and income may be recorded.

PRESENTING COMPLAINTS
History regarding skin patches, loss of sensation
over the patches, loss of sweating, pain in nerves,
loss of eyebrows/eyelashes, nasal stuffiness/
epistaxis, edema of legs, hoarseness of voice,
tingling and numbness in limbs, weakness of
hands and legs, eye pain/blurring of vision/
photophobia, joint pain/fever/malaise/flaring of
patches may be recorded.

HISTORY OF PRESENT ILLNESS


Further details regarding present complaints
are explored e.g., age of onset of disease,
number of patches, site of onset of them, size,
shape, color and surface of patches, evolution
of them, any associated features.
In longstanding cases, enquiry may be made
for hoarseness of voice, tingling and numbness
in fingers and toes, blistering and deformities
of hands and feet. Factors responsible for acute
episodes in leprosy if any are found out.

PAST HISTORY
Whether, he had similar complaints in the past,
surgical correction of deformities, or lepra reaction
(type 1 or type 2).

History Taking and Examination in Leprosy

TREATMENT HISTORY

CUTANEOUS EXAMINATION

Whether the patient has taken any treatment


before and if so, the duration of treatment. If
discontinued, any reason for that, any adverse
effects noted

Following features are noted: Type of lesions-macule/papule/plaque/nodule


Number of lesions-1-3, 10, 20, numerous,
uncountable
Colour- erythematous/hypopigmented/
hyperpigmented
Margins-welldefined/illdefined
- regular/irregular
Surface- smooth/rough/shiny/dry
- scaly/non-scaly
- flat/raised
Induration-peripheral/central/whole
lesion/none
Sweating-present/absent/impaired
Hairs over the lesion (s) present or absent,
leprous alopecia
Distribution symmetrical/asymmetrical/
localized/others
Sensory functions of the affected area- normal/
impaired/ absent with regard to touch, pain
and temperature.
The most common sign of autonomic nerve
damage is loss of sweating which is shown
by dryness and may lead to fissuring. It may
be tested by pilocarpine test.

FAMILY HISTORY
If any one in the family is suffering from leprosy,
needs to be enquired. Health status of family
members should be ascertained.

Personal History
It should focus on accommodation facility,
number of rooms available and number of
persons staying together.
GENERAL PHYSICAL EXAMINATION
One may look for pallor in longstanding
cases.
Hair loss in the form of ciliary madarosis,
superciliary madarosis and alopecia of
axillary and pubic hair may be noted in
lepromatous leprosy cases. Besides this, such
cases may show xanthelasma over the eyelids,
collapse of bridge of the nose or perforation of
the nasal septum.
Examination of mouth and pharynx may
show papules on lips, nodules on the tongue/
palate or palatal perforation.
Nails may demonstrate longitudinal ridges
and become dry, lustreless and shrunken.
Hands and feet may show disabilities and
deformities like wrist drop, foot drop, claw
hand/toes, ape thumb etc. Digits may have
autoamputation.
Testes may get involved and finally may
become atrophic.
Gynaecomastia and sparse beard may be an
indication of testicular involvement in
lepromatous leprosy cases.

EXAMINATION OF PERIPHERAL NERVES


In leprosy cases, one may proceed with peripheral
nerve examination starting with inspection (any
visible deformity of limbs or face, wasting of the
muscles, trophic skin changes, scar or wound),
palpation (temperature of paralysed parts,
consistency of muscles, hyperesthesia of skin, scar
tenderness), assessing muscle power (gradation
of muscle power from 0-5) and palpation of
peripheral nerves for thickening and tenderness
(main nerve trunks as well as cutaneous nerves
especially those feeding the patch).
After the physical examination is complete,
make a slit skin smear for acid-fast bacilli. If the

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diagnosis is still in doubt take a biopsy from a
skin lesion or from an enlarged nerve that carries
only sensory fibers.

Decide
1. Does the patient have leprosy?
2. If so, where does his disease lie on the
spectrum?
3. What is the stage of activity?

4. Is the patient having a lepra reaction? What is


the immunologic response to M. leprae?
5. What do I need to teach or tell the patient?
6. Should I refer him for specialized care?
7. If not, what treatment should I give: specific
antileprosy treatment, anti-inflammatory
treatment, treatment for complications of
leprosy, or treatment for other illness?
8. What are the social implications of these
decisions?

Clinical Leprosy

36

Clinical Leprosy

Leprosy (Hansens disease) is a chronic infectious


disease caused by Mycobacterium leprae. It mainly
affects the peripheral nerves. It also affects the
skin, muscles, eyes, bones, testes and internal
organs.
There is no infectious disease in man in which
the clinical picture is as varied as that of leprosy.
Even then, leprosy can be easily diagnosed if we
remember the three cardinal features of leprosy.
1. Hypopigmented or erythematous hypoanesthetic patch (es).
2. Thickening of peripheral nerves with sensory
or motor deficit.
3. Demonstration of AFB from skin lesions.
Any one of them if present in a given case, you
can make a diagnosis of leprosy (provided he has
yet not completed full course of treatment).

Definition of a Case of Leprosy


The WHO in 1998 defined a case of leprosy as a
person having one or more of the following
features and who has yet to complete a full course
of treatment:
1. Hypopigmented or reddish skin lesion(s) with
definite loss of sensation.
2. Involvement of peripheral nerves as
demonstrated by a definite thickening with
loss of sensation.

3. Skin smear positive for acid fast bacilli (AFB)


This definition also includes treatment
defaulters and cases who relapse, but does not
include cured persons or persons with late
reactions or residual disabilities.

Agent
Mycobacterium leprae was discovered by
Gerhard Henrik Armauer Hansen in 1873.
It is an acid-fast bacillus measuring 1 to 8
microns in length by 0.3 microns in diameter.
The capsule of lepra bacilli contains a
phenolic glycolipid 1 (PGL1) that renders it
chemically unique and antigenically specific.
It is an obligate intracellular parasite that
divides every 12-13 days in histiocyte and
schwann cells
The optimal temperature for growth for lepra
bacilli is 30-33 centigrade.

Incubation Period
It can show considerable variation and in most
instances between 2 and 5 years.
Mode of Spread
The various modes of spread are by inhalation,
direct contact, ingestion and insects.

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Essentials in Dermatology

SPECTRUM OF LEPROSY
Ridley and Jopling classified leprosy taking into
account the immunological and histopathological
basis in addition to the clinical and bacteriological
findings. The classification divides leprosy into
Tuberculoid tuberculoid (TT)
Borderline tuberculoid (BT)
Borderline borderline /Mid borderline/
dimorphous leprosy (BB)
Borderline lepromatous (BL)
Lepromatous lepromatous (LL)
The two other types of leprosy, which does
not fall under Ridley and Jopling classification
but are included in Indian Leprologists
association classification are indeterminate and
polyneuritic leprosy.

Some General Facts


Cell mediated immune response of the host
determine the type of leprosy
TT and LL patients are stable
TT leprosy often self heals
LL leprosy remains heavily infected unless
given appropriate chemotherapy
BB leprosy is the most unstable, with most
patients down grading if not treated
Leprosy can affect all ages and both sexes
The limited growth of M. leprae in the mouse
foot pad provided a way to screen for
therapeutic agents and to identify drug
resistance in leprosy
The recognition of leprosy in the nine banded
armadillo provided a source of large
quantities of highly purified M. leprae for
biochemical and immunologic studies
including preparation of vaccine for leprosy
Rifampicin was the first drug to be identified
as bactericidal for M. leprae and is now the
cornerstone of most therapeutic regimens
Tuberculoid Leprosy
1-3 hypopigmented well defined macules or
plaques (Fig. 36.1).

Fig. 36.1: Tuberculoid leprosy- well defined oval


erythematous hypoanesthetic plaque lesion over the
trunk

The lesions are dry, hypoanesthetic or


anesthetic with loss of sweating and hair.
Occurs commonly in areas not covered by
clothes.
Peripheral nerve trunks are not involved, but
nerve feeding the patch may be thickened.

Borderline Tuberculoid Leprosy


3-10 or more hypopigmented macules or
plaques with irregular margins (Figs 36.2 and
36.3)
The lesions are large, hypoanesthetic with loss
of appendages
Satellite lesions are seen around the large
plaques.
Cutaneous nerves supplying the plaque may
be thickened (feeder nerves) (Fig. 36.4)
Peripheral nerve trunks are irregularly
enlarged and involved in an asymmetrical
pattern.
Prone to develop type 1 lepra reaction.

Clinical Leprosy

Fig. 36.2: Borderline tuberculoid leprosy-hypopigmented hypoanesthetic macule with irregular well
defined margin

Fig. 36.4: Borderline tuberculoid leprosy-hand


showing a thickened cutaneous nerve feeding an
erythematous hypoanesthetic plaque over the index
finger

Fig. 36.3: Borderline tuberculoid leprosy- a


hypoanesthetic ichthyotic plaque over the thigh with
a few satellite lesions

Borderline Lepromatous
Numerous but still countable lesions are seen
The copper colored macules, plaques and
nodules show a tendency towards symmetry
(Figs 36.5 and 36.6).
The lesions are shiny with minimal sensory
loss
Multiple peripheral nerves are thickened

Borderline Borderline Leprosy


Also known as dimorphous leprosy
More than 20 lesions are seen
The lesion are well to ill defined with irregular
borders presenting with polymorphic or
geographic appearance
Swiss cheese or punched out lesions are
typical of BB Hansen
Peripheral nerves are thickened.

Lepromatous Leprosy
Initially has cutaneous and mucosal lesions
Edema of lower legs and epistaxis are
considered early signs
The early skin lesions are macules which are
uncountable (Fig. 36.7)
The skin lesions are small, coppery colored,
numerous and symmetrically arranged
Over a period of time, the entire skin becomes
shiny and infiltrated, most often on the face,
especially forehead, ear lobes (Fig. 36.8),

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Essentials in Dermatology

Fig. 36.5: Borderline lepromatous leprosy-multiple,


countable, hypopigmented, slightly hypoanesthetic
macular lesions over the trunk

Fig. 36.6: Borderline lepromatous leprosy-multiple,


shiny, infiltrated plaques showing tendency to
symmetrical distribution over the back

Fig. 36.7: Lepromatous leprosy-coppery colored shiny


macules of uniformly small size over the upper back
in early lepromatous leprosy

Fig. 36.8: Lepromatous leprosy- infiltrated pinna


showing papules and nodules in a more advanced
case

eyebrows, nose, and malar eminences, finally


producing leonine facies(Fig. 36.9)
As the untreated lepromatous leprosy evolves,
papules and nodules appear (Figs 36.10 and
36.11)
Nasal involvement occurs in the form of
epistaxis, septal perforation and collapse of
nasal bridge

Neural changes occur later


Glove and stocking anesthesia, and
thickening of peripheral nerve trunks (Fig.
36.12), bilaterally and symmetrically is a
feature of advanced and progressive
lepromatous leprosy
Gynecomastia (Fig. 36.13)and testicular
atrophy (Fig. 36.14) occurs in late stages in
males

Clinical Leprosy

Fig. 36.9: Lepromatous leprosy-Leonine facies

Fig. 36.10: Lepromatous leprosy- infiltrated shiny


skin along with nodular gross lesions over the
knee in an advanced lepromatous leprosy case

Fig. 36.11: Lepromatous leprosy-shiny diffuse


infiltrated lesions on the back

Fig. 36.12: Lepromatous leprosy-thickening of


greater auricular nerve

Fig. 36.13: Lepromatous leprosy-gynaecomastia

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Essentials in Dermatology

Fig. 36.14: Lepromatous leprosy-sparse beard


hairs due to testicular atrophy

Multiple organs are involved due to bacillemia


in the absence of cell mediated immune
response against M leprae

Fig. 36.15: Histoid leprosy-waxy shiny nodules of


histoid leprosy over apparently normal skin over the
elbow and knee

Indeterminate Leprosy
Usually occurs as solitary, hypopigmented,
vaguely defined macules
Common in children
Sensory loss is equivocal
Most lesions heal spontaneously
Mitsuda negative patients are more likely to
develop lepromatous leprosy

It is characterized by the occurrence of well


demarcated cutaneous and subcutaneous
nodules and plaques over apparently normal
skin (Fig. 36.15)
Histoid leprosy lesions show the highest
loads of bacilli (the bacteriological index is
frequently 6+) and the majority are solid
staining, arranged in clumps like sheaves of
wheat
The histiocyte (macrophage) tissue reaction
is unusual in that the cells frequently become
spindle shaped and oriented in storiform
pattern, similar to those of fibrohistiocytoma
The presence of these spindle shaped
histiocytes in histopathological section has
given this entity the name of histoid leprosy

Polyneuritic/Pure Neuritic Leprosy


Manifests only with neural signs without any
clinical evidence of skin lesions
Peripheral nerves are thickened and
associated with sensory loss and motor
weakness
The deep reflexes are normal in contrast to
other peripheral neuropathies
Histoid Leprosy
A distinct, stable and unique variant of
lepromatous leprosy
It frequently follows incomplete chemotherapy
or acquired drug resistance

Lucio Leprosy
Occurs in Mexico ( in South America), also
called as lepra bonita /beautiful leprosy
Diffuse infiltration occurs without distorting
the body contour
May mimic myxedema

Clinical Leprosy

DIAGNOSIS
Slit Skin Smear (SSS)
Consists of obtaining tissue smear from the
skin and staining with modified Ziehl-Neelsen
stain
Smear should be taken from six sites: both ear
lobes and four representative active skin
lesions
In case of single patch, smear should be taken
from two sites diagonally opposite to each
other
A SSS is positive if at least 104 bacilli are
present per gram of tissue
Grading of SSS is based on Ridleys
logarithmic scale
_ 1 to 10 bacilli in 100 fields -1 +
_ 1 to 10 bacilli in 10 fields -2 +
_ 1 to 10 bacilli in an average field -3 +
_ 10 to 100 bacilli in an average field -4+
_ 100 to 1000 bacilli in an average field -5 +
_ Clumps and globi in an average field -6+
(Fig. 36.16)
Bacteriological index gives a quantitative
measure of M. leprae from the skin
BI falls by 1 unit every year after starting the
patient on MDT
Morphological index is given as the
percentage of regularly stained bacteria

Fig. 36.16: Slit skin smear showing red rods of acid


fast bacilli of M leprae packed as globi inside
macrophages (6+ on Ridleys logarithmic scale)

The MI falls to zero by 1 month after starting


the patient on multibacillary treatment.

Lepromin Skin Test


Lepromin is prepared from M.leprae and used
as skin test antigen
The two lepromin commonly used are
Mitsuda lepromin (commonly armadillo
derived) and Dharmendra lepromin (human
tissue derived)
Intradermal injection of lepromin evolves into
two types of responses, the early (Fernandez)
and delayed (Mitsuda) reactions
Fernandez reaction (within 48 hours) is a
delayed hypersensitivity response to bacillary
antigen
Mitsuda reaction (after 4 weeks) is a reliable
indicator of cell mediated immunity against
lepra bacilli
Both lepromin produce early and late reaction
but Fernandez reaction is prominent with
Dharmendra lepromin and Mitsuda reaction
is prominent with Mitsuda lepromin
Lepromin is strongly positive in TT Hansen
and gradually decreases across the spectrum
and becomes negative in LL Hansen.
Histopathology
Conclusive proof of leprosy is the demonstration
of an intraneural granuloma or the presence of
acid-fast bacilli in nerves.
TT Hansen- well defined epithelioid
granulomas with Langhan giant cells eroding
into the epidermis
BT Hansen- epithelioid granuloma is seen
with giant cells in the upper dermis
BB Hansen- few epithelioid granulomas with
absence of giant cells and scanty lymphocytes.
AFB are demonstrable
BL Hansen- granuloma composed of
histiocyte, lymphocytes and macrophages.
Nerves show schwann cell proliferation
(onion peel appearance)

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Essentials in Dermatology
LL Hansen- subepidermal zone is free (Grenz
zone) with diffuse infiltration of foamy
macrophages (Virchowcytes) in the dermis.
Abundant AFB, even globi may be seen.

DIFFERENTIAL DIAGNOSIS OF
LEPROSY
The first step towards diagnosing leprosy is to
think of the possibility of leprosy. The diagnosis
can easily be made if the case is classical i.e.,
having characteristic skin changes (hypopigmented patch with loss of sensation) and
nerve involvement, but this is not always the case.
Leprosy produce wide variety of clinical
situations depending upon the interaction
between M. leprae and hosts immune system such
as repeated epistaxes , paresthesias, mono or
unilateral neuralgia, muscle weakness, paralysis,
deformities, painless trauma or ulcer, chronic
asymptomatic skin lesions, madarosis, upgrading
and downgrading reactions, etc. Active leprosy
gives rise to either mono or polymorphous clinical
pictures where different skin lesions (macules,
papules, plaques, nodules, diffuse infiltration) are
variously assorted.
Before diagnosing a case of leprosy one should
be aware of following problems:
1. Bacteriological negative skin smear doesnt
rule out diagnosis.
2. AFB detection in nasal smear by itself is not
sufficient for diagnosis.
3. Lepromin test does not have any diagnostic
value.
4. Sometime, histopathological examination
may not show any evidence from suspected
leprosy lesions.
5. Leprosy presenting with only skin lesions or
nerve lesions
6. Contact with leprosy case can not be regarded
as diagnostic criteria.
Therefore to formulate a diagnosis of leprosy,
clinical, microbiological and histopathological
findings must be taken into account. The typical

association of skin lesions and symptoms of nerve


involvement is peculiar to leprosy even though
not constantly occurring.

Differential Diagnosis of Cutaneous


Manifestations
Macules in leprosy are fixed, round or oval in
shape, nonpruriginous, nonvesiculate, chronic in
evolution and resistant to topical therapies. They
may be erythematous, hypopigmented and rarely
hyperpigmented.
Differential diagnosis of erythematous macule
(seen in all forms of leprosy) are early fixed drug
eruption (initial lesion is an erythematous patch,
have a burning sensation, commonly seen in
perioral area, or genital areas, repeated episodes
occur at the same site, preceding history of drug
intake- sulfonamides, NSAIDs, tetracycline,
metronidazole, phenobarbitone etc), morphea
(one or few erythematous macules, gradually
loose their color, become centrally white, and
sclerotic), pityriasis rosea (self-limiting disorder,
starts with a herald patch 2-5 cm in diameter
predominantly on the trunk or proximal
extremities, daughter lesions follow Christmas
tree pattern, individual lesion shows collarette
scaling Hanging curtain sign), tinea corporis
(itchy annular scaly erythematous patches with
papulovesicular border, KOH examination
demonstrates dermatophytes), syphilitic roseola
(initially as erythematous macules called as
roseolar syphilide, in a few days it evolve into
papular form, nonscaly, non-itchy lesions follow
lines of cleavage on back, history of sexual
exposure, VDRL reactive), postkalazar dermal
leishmaniasis PKDL (occurs after recovery
from insufficiently treated visceral leishmaniasis,
characterized by hypopigmented and erythematous macules, usually located on the perioral
regions and cheeks in a butterfly distribution),
seborrheic dermatitis (hypopigmented or
erythematous papules and plaques with scaling,
in adolescent or adult age group, with sites of

Clinical Leprosy
predilection- scalp chest, back and face, presence
of pruritus helps in differentiating it from leprosy,
may have a history of cradle cap during infancy,
treatment with topical steroids and oral
antifungals like fluconazole helps in resolution),
gyrate erythemas-erythema chronicum migrans
(skin manifestation of Lymes disease, a
spirochete (Borrelia burgdorferi) infection, caused
by the bite of Ixodes ticks, starts as erythematous
macule at bite site, extends peripherally to become
annular lesion which may persist for months and
associated with fever, headache, myalgia and
arthralgia, titration of anti-Borrelia antibody helps
in diagnosis), erythema annulare centrifugum
(characterized by annular or polycyclic lesions
with raised erythematous border, cause is unclear
but in some cases hypersensitivity to tinea
infection or association with malignancy is
suspected, persists for weeks to months and tends
to recur over the years), erythema gyratum repens
(characterized by concentric raised erythematous
bands moving in waves over the body surface in
a wood-grain pattern at a speed of approximately 1 cm/day, always associated with
internal malignancy), erythema marginatum
(eruption has a serpiginous pink indurated
border, blanches on pressure and changes its
shape and is often hidden by the clothing, occurs
in association with rheumatic fever), erythema
multiforme (acute onset eruption in acral areas,
typical lesion is target lesion, recurrent form is
associated with herpes labialis)
Differential diagnoses of hypochromic macule
are nevus anemicus (a developmental anomaly,
presents as a circular patch of pale skin of normal
texture, does not flare on stroking, intradermal
injection of histamine produces a flare whereas
in leprosy the flare is delayed, feeble or entirely
absent), nevus depigmentosus (achromicus)
(birthmark, poorly demarcated hypopigmented
patches -resemble splashed paint in a dermatomal
pattern, diascopy does not show any change in
color at the edge), vitiligo (presents with

depigmented macules but can present with hypo


pigmented macule in previtiligo, also shows
leucotrichia, peripheral and perifollicular
hyperpigmentation, and normal histamine test,
no loss of sensation), tinea versicolor (hypo
pigmented usually on dark skin, well defined,
multiple, asymptomatic or mildly itchy macules
with fine branny scales, mainly on upper back,
shoulder and chest, KOH examination shows
banana and grape appearance, gold to orange
fluorescence on Woods light examination),
pityriasis alba (seen in children, on the face as
hypopigmented macules with powdery scaling,
has a seasonal variation and is more in summer
seasons, a manifestation of atopic diathesis, if the
lesion persists, it is often difficult to differentiate
from indeterminate leprosy as leprosy patch on
the face is not always anesthetic), postinflammatory lesions (occurs most commonly
with pityriasis rosea, the lesions show a
characteristic Christmas tree pattern on the
trunk), early PKDL ( as described earlier), yaws
and pinta (in secondary stage of yaws and late
stage of pinta, hypochromic macules may occur),
onchocerciasis (confluent achromic macules
occur on pretibial areas).
Differential diagnosis of hyperchromic macule
are residual lesions of fixed drug eruption,
Kaposis sarcoma (hyperpigmented macules are
seen in regressive stage), erythema dyschromicum perstans (eruption often begins with
erythematous macules that rapidly evolves into
blue-gray color with elevated edge, mainly seen
on trunk, limbs and face), tinea nigra (a fungal
infection produces brownish or black macules on
palms and soles), and hyperchromic form of tinea
versicolor.
Differential diagnoses of papular lesions are
sarcoidosis (a granulomatous multisystem
disease presents with variable skin manifestations such as macule, papule, nodule and
plaques, histopathologically shows characteristic
naked epithelioid granulomas), granuloma

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Essentials in Dermatology
annulare (presents typically with asymptomatic
papules arranged in a ring like fashion over the
hands and feet, disseminated lesions may
develop later on, these lesions may disappear after
a biopsy, the histology shows areas of necrosis
with collagen degeneration), granuloma
multiforme (can be confused with tuberculoid
type of leprosy, the lesions are seen on the
extremities-sun exposed areas as annular, with
well marked papular edges, evolving
simultaneously towards central healing,
coalescing of annular lesions gives rise to
circinate lesions, surroundings skin shows photo
damaging changes, the lesions persists
indefinitely or at least for several years), lichen
planus (a papulosquamous disorder characterized by itchy, violaceous flat topped, polygonal
papules especially on the flexor aspect of wrists,
shins and trunk), late secondary syphilis
(papules occur on the trunk and limbs in
secondary syphilis, associated with mucosal
erosions or snail track ulcers, Buschke
Ollendroff sign positive i.e. tenderness if lesion
is pressed with a blunt object in the center,
serological tests (VDRL) confirm the diagnosis,
histopathological examination shows endarteritis with plasma cell infiltrate, lesions
disappear in a few weeks if adequately treated
with penicillin or resolve in two months and pass
on to the early latent stage) and PKDL.
Differential diagnoses of nodular lesions are
Kaposis sarcoma (a vascular tumor characterized by the presence of bluish red or dark brown
plaques or nodules, on the distal portion of lower
extremities, may have lymph node involvement
and visceral lesions in 10 percent of patients, the
tumors may be associated with AIDS or human
herpes virus 8 (HHV8), histopathology shows
vascular formations with predominance of
endothelial cells and spindle cell formations
containing vascular slits), cutaneous
leishmaniasis (single or multiple nodules, seen
on the exposed parts i.e. at the site of insect bites.

Cutaneous leishmaniasis due to Leishmania


ethiopica presents with lesions on the face with a
few satellite papules, the lesion may ulcerate. In
diffuse cutaneous leishmaniasis, multiple lesions
are seen on the face and have to be differentiated
from lepromatous leprosy. Leprosy has to be
excluded by doing a slit skin smear or a biopsy.
In leishmaniasis, the organisms are seen inside
the macrophages and appear as a dot and a
dash), mycetoma (a deep fungal infection,
characterized by triad of nodules, discharging
sinuses and discharge containing granules,
commonly occurs on the foot), sarcoidosis
(systemic granulomatous disease of unknown
etiology, presents with various types of skin
lesions including nodules,histology is
characteristic, which show a naked epithelioid
cell granuloma), Histoid leprosy (nodules of
Histoid leprosy have to be differentiated from
molluscum contagiosum, keloid and dermatofibroma and steatocystoma multiplex),
neurofibromatosis (has to be differentiated from
leprosy as it produces not only nodules but also
thickened nerves like lepromatous leprosy, other
signs seen in neurofibromatosis helps in easy
differentiation such as Cafe-au-lait macules,
axillary freckling Crowes sign, or palmar
freckling Patrick-Yesudian sign, button-hole
sign and Lisch nodules or iris hamartomas on
slit lamp examination), dermatofibroma (a
benign tumor with hard consistency, appears as
small dome shaped nodule located on the
extremity), molluscum contagiosum (pearly
white or skin colored, asymptomatic nodules with
central umblication seen commonly on the face
and genitalia), cutaneous deposits in
lymphoproliferative deposits, onchocerciasis,
and PKDL.
Differential diagnoses of plaque lesions are
lupus vulgaris (a form of cutaneous tuberculosis,
presents typically as a plaque, heals on one side
and progresses on other side, shows apple jelly
nodules on diascopy, histology shows tuberculoid

Clinical Leprosy
granulomas with normal nerves), necrobiosis
lipoidica (observed with diabetes mellitus,
commonly over the legs and thighs as sharply
marginated, firm, depressed waxy, yellow brown
plaques with glistening surface and telangiectasia, heals with atrophy and scarring.
Histopathology shows palisading granuloma,
which is characteristic), late secondary syphilis,
mycosis fungoides (Cutaneous T cell lymphoma
(CTCL) affects the skin primarily, but ultimately,
lymph nodes and visceral organs are involved.
Skin lesions vary from erythematous patches to
plaques, tumors and erythroderma. Plaque stages
or tumor stages resemble lepromatous leprosy.
Skin biopsy shows lymphocytic infiltrate with
epidermotrophism. In the plaque stage, mycosis
cells may be seen along with a band like dermal
infiltrate), and Mycobacteria marinum infection
(Solitary erythematous nodule or plaque,
sometimes ulcerate and appear crusted, a skin
smear from the lesion may contain AFB similar in
appearance to M. leprae, but the organism can be
cultured on suitable media, a skin test using PPD
from M. marinum is positive). Other differential
diagnoses include parapsoriasis, sarcoidosis,
cutaneous leishmaniasis, erythema multiforme.
Differential diagnoses of diffuse infiltration of
the skin are lymphoma, actinic reticuloid (a
severe form of persistent photosensitivity
producing erythema, edema and thickness of the
skin on the face, neck and hands), systemic
sclerosis (patient develops a taut and thickened
skin which slowly becomes bound to
subcutaneous tissues, recurrent ulcerations
develop at the ends of fingers and terminal
phalanges become absorbed, polyarthritis of
small joints is common and finger contractures
may develop), myxedema (has many similarities
to lepromatous leprosy, thickening of the skin,
thinning of eyebrows, a hoarse voice, edematous
leg, normocytic normochromic anemia and carpal
tunnel syndrome as a complication), and
pachydermoperiostosis (a familial condition

predominantly affecting the males in which the


facial appearance with deepening of the lines of
face and forehead closely resembles lepromatous
leprosy, bone changes take the form of proliferative
periostitis, fingers become thickened and there is
clubbing of fingers and toes).

Differential Diagnosis of
Regional Manifestations
Differential diagnosis of madarosis in
lepromatous leprosy is lymphoma, follicular
mucinosis, hypothyroidism, secondary syphilis
and alopecia areata.
Differential diagnoses of earlobe infiltration/
nodules in lepromatous leprosy is lupus vulgaris,
coccidioidomycosis, chondrodermatitis nodularis
helices, lupus erythematosus, sarcoidosis.
Differential diagnoses of deformity of nose
(cartilage destruction) in lepromatous leprosy,
leishmaniasis (cartilage destruction), tertiary
yaws, relapsing polychondritis and congenital
syphilis (bone destruction).

Differential Diagnosis of Peripheral


Nerve Involvement
Differential diagnoses of only palpable nerve
thickening are excessive muscular development
(Localized - great auricular nerve thickening in
people carrying heavy weights on the head or
Generalized in professional wrestlers), and
pachydermoperiostosis (Generalized thickening
of skin, periosteum and bone, there is clubbing
and acromegalic features, furrowing due to the
thickened skin of forehead, can easily be mistaken
for the leonine facies of lepromatous leprosy).
Differential diagnoses of palpable nerve
thickening with regional anesthesia, with or
without muscle wasting are primary amyloidosis
of peripheral nerves (Inherited disorder, onset
in second or third decades, usually affects lower
limbs, with impaired sensations, muscle wasting

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Essentials in Dermatology
and dropped foot. Late effects are loss of tendon
reflexes and trophic ulceration of feet, associated
with autonomic neuropathy leading to orthostatic
hypotension, diarrhea, loss of bladder control or
impotence. Histological examination discloses
amyloid infiltrating the affected nerves), familial
hypertrophic interstitial neuritis (Djerine
Sottas neuropathy) (Hereditary motor and
sensory neuropathy type 3, autosomal recessive,
has onset in childhood or adolescence,
characterized by slowly progressive muscular
atrophy of limbs, commencing distally with claw
hands, dropped foot, anesthesia and loss of
tendon reflexes).
Differential diagnosis of regional anaesthesia
with or without muscle wasting but with
palpable nerve thickening in some cases are
recurrent or chronic progressive (endotoxic)
polyneuritis (an acquired disorder of nerves,
cause unknown, symptoms first appear in adult
life and tendon reflexes are diminished or absent),
and peroneal muscular atrophy (Charcot
MarieTooth type) (an inherited disorder, onset
in childhood, with muscle weakness in lower
limbs, pes cavus, hammers toes and callosities of
feet, tendon reflexes of lower limbs are diminished
or absent).
Differential diagnosis of regional anaesthesia
with or without muscle wasting but without
palpable nerve thickening are syringomyelia
(anesthesia and muscle wasting develop in upper
or lower limbs depending on the localization of
the cord lesion, there is dissociated anesthesia loss of pain and temperature sensation with
preservation of touch, tendon reflexes are
diminished or lost, and the histamine test is
positive), tabes dorsalis (characterized by
dysfunction of posterior nerve roots has triad of
symptoms-lightning pain, dysuria and ataxia and
triad of signs-ArgyllRobertson pupils, areflexia
and loss of proprioception), peripheral
neuropathy (a mononeuropathy can result from

compression of nerve or nerve plexus and may


simulate pure neural leprosy, e.g., cubital tunnel
compression syndrome, carpal tunnel syndrome,
cervical rib and meralgia paraesthetica causing
sensory changes in one or both thighs or multiple
neuropathy which has a large number of causes,
some of which like diabetes result in plantar
ulceration, depression of histamine flare in
anesthetic skin is similar to that in leprosy, but
there are no thickened nerves and tendon reflexes
are likely to be lost), hereditary sensory radicular
neuropathy (Loss of sensation and sweating is
most severe in lower limbs, but muscular
coordination is normal compared to tabes, chronic
painless plantar ulceration is classical, together
with high-tone deafness. Loss of ankle jerk is
usual and X- ray of feet reveals bone changes
similar to those in leprosy) and hysteria.

Differential Diagnosis of Trophic Ulcers


Many conditions, which produce sensory motor
neuropathy, may also produce trophic ulcers. The
following conditions are to be considered like
diabetes mellitus, syringomyelia, Moorvans
syndrome, tabes dorsalis, diastometamyelia,
Thevanard syndrome and congenital sensory
neuropathy.
Differential Diagnosis of Thickening of
Peripheral Nerves
The following disorders are associated with
thickening of peripheral nerves-Djrine-Sottas
disease, neurofibromatosis, schwannoma, lipofibromata, Tooth Charcot Hoffmanns syndrome,
Thevanards syndrome, syringomyelia,
amyloidosis, hereditary motor and sensory
neuropathy type 3 and Guillain-Barres
syndrome.
TREATMENT
The most important development in the leprosy
control in the last millennium has been the

Clinical Leprosy
introduction of multidrug therapy (MDT) instead
of dapsone monotherapy in the year 1982,
following the recommendations of the WHO
study group. There has been a dramatic
downward revision of estimated number of
leprosy patients in the world from 10 to 12 million
in the mid 1980s to 0.62 million (point prevalence)
in 2001. Although these figures are highly
encouraging, the number of new cases detected
annually has remained quite stable during the
last 15 years. India harbours 65% of the world
population of leprosy patients. The total number
of registered cases in India has shown a steep
decline over the years, from 3.4 million cases in
1986 to 0.266 million cases in 2004.
The standard schedule of MDT for leprosy has
not undergone any change after its introduction
in 1982, except for the temporary introduction of
ROM therapy for single skin lesionpaucibacillary (PB) leprosy. However there have
been significant changes in the duration of
therapy and in the criteria for allocating patients
into PB and multibacillary (MB) groups for
therapy. At the time of introduction of MDT, in
1982, this allocation was based on the grading of
smear positivity, with patients with a
bacteriological index (BI) < 2 classified as PB
leprosy. In 1988 the WHO expert committee on
leprosy recommended that only initially smearnegative patients should be classified as PB. With
time, a division based on skin smear examination
was not considered suitable in field conditions
and a division based on the number of skin lesions
gained favour. In the last 22 years, an increasing
number of patients who were previously
classified as PB for treatment purposes, were
allocated to the MB group for treatment purposes,
with skin smear examination no longer
considered necessary for gouping. Patients with
more than 5 skin lesions are now being classified
as MB patients, and wherever facilities are
available for a slit skin smear examination, all

smear positive patients are included in the MB


group.
The original recommendation of treating
patients till smear negativity meant that the
duration of treatment was for more than 2 years
for MB patients. This was reduced to a fixed
duration of 24 months in 1992, and further
shortened to 12 months in 1998.
WHO in 2003 has omitted any separate
mention of the single lesion PB group or ROM
therapy. At present, 12 months fixed duration
therapy for MB patients is being followed all over
India. From July 1997, the vertical NLEP
programme is being integrated into general health
services.
WHO (2003) presently classifies leprosy into
PB leprosy (patients with 1-5 skin lesions, slit skin
smear negative) and MB leprosy (patients with
more than 5 skin lesions, slit skin smear positive).
The Directorate General of Leprosy,
Government of India, had accepted the ROM
(single dose of ROM-Rifampin 600mg, Ofloxacin
400mg and Minocycline 100 mg) regimen for
single lesion PB leprosy and had incorporated it
in the National Leprosy Eradication Programme
(NLEP) in November 1997. However, they have
recommended that patients on ROM should be
under surveillance for 2 years after administration
of the therapy but this mode of therapy has come
under lot of criticism.

WHO/National Leprosy Eradication


Programme (NLEP) Recommendations
are:
Paucibacillary leprosy-6 months therapy
Monthly supervised rifampin (600 mg)
and dapsone (100mg) daily
Multibacillary leprosy-12 months therapy
Monthly-supervised rifampin (600 mg)
and clofazimine (300 mg), dapsone
(100 mg) daily and clofazimine (50 mg)
daily

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Laboratory monitoring for drugs used to treat leprosy

Drug

Laboratory studies

Initial studies for all drugs


Dapsone
Rifampicin
Clofazimine

CBC, platelets, urine analysis, blood biochemistry


Baseline
CBC, reticulocyte count, G6PD only if hemolysis recorded every 6 months
CBC, platelets, blood biochemistry
every 3 months
No recommended laboratory studies

Accompanied MDT (A-MDT) is designed to


address a frequent problem in field programs.
Patients often have to interrupt their treatment
because of a shortage of drugs at the health centre,
poor access to the health services or simply
because no one is at the health center when they
come to collect their treatment. A-MDT means
providing patient with full course of treatment
on their first visit to the leprosy clinic after
diagnosis. On this occasion, the patient and
accompanying person also receive information
about leprosy in the form of printed material about
the disease, its treatment and when and where to
come for follow-up or in the event of complications.
The term accompanied is adopted because
someone close to or important to the patient
assumes responsibility for helping the patient to
complete the treatment.

Alternative MDT Regime


Rifampicin resistant cases or those patients, who
developed toxicity to rifampicin- Stop rifampicin,
continue two other drugs in MB MDT. In addition,
add ofloxacin 400 mg + minocycline 100 mg
(or clarithromycin 500 mg) for 6 months, followed
by ofloxacin 400 mg or minocycline 100 mg for a
period of another 18 months
Dapsone resistant cases -Dapsone to be
continued with other drugs + add clofazimine in
PB cases.
Dapsone toxicity cases- In PB cases stop
dapsone and add clofazimine and in MB cases
stop dapsone and continue other two drugs.

Frequency

Clofazamine unacceptable cases(due to


pigmentation)- Omit clofazimine from regimen,
replace it with ofloxacin 400 mg or minocycline
100 mg can be used as substitute
MB patients who were on dapsone monotherapy
previously and negative smear-Give an additional course of MB MDT.
Leprosy with concomitant HIV infectionRifampicin is contraindicated with protease
inhibitors. Dose of rifabutine needs to be reduced
to half if indinavir or nelfinavir is given.
Leprosy and tuberculosis- Continue standard
antituberculous treatment. Rifampicin is given in
doses as for tuberculosis and other two antileprosy drugs are to be continued as per regime.
Relapse- Treat with minimum two new antileprosy drugs in addition to rifampicin.

New Regimes of Short Duration (Under


Trials)
1. MOR: minocycline(daily) + ofloxacin (daily)
+ rifampicin (monthly)
2. RMM: Rifapentine(monthly) + minocycline
(daily) + moxifloxacin(daily)
3. U-MDT: Regimens for paucibacillary (PB) and
multibacillary (MB) leprosy patients differ in
their duration and components. So Uniform
Multi Drug Therapy (U-MDT) with three drug
combination of MDT drugs as given for MB
cases has been recommended to overcome
difficulties in classification which usually
come in field conditions. Uniform triple drug

Clinical Leprosy
therapy is given with fixed duration of 6
months for PB and 12 months for MB cases.
4. U-MDT with fixed duration therapy of 6
months.
5. Monthly administered ROM for MB and PB
leprosy: Once a month ROM for 12 months in
MB and 6 months in PB leprosy cases.
6. Fully supervised regimes such as RMM
monthly regimes: Rifapentine + minocycline
+ moxifloxacin (monthly), RCM regimes:
Rifampicin + clarithromycin ( 1000mg) +
minocycline (200mg) monthly fully
supervised dose, Ampicilin/ sulbactum, or
Quadruple regime: Rifampicin (600mg) +
ofloxacin (400mg) + clofazamine (100mg) +
minocyclines (100mg) once weekly for 6
weeks. This regime is gaining importance.

Fig. 36.17: Type 1 lepra reaction- the previously


existing skin lesion over the face had become
erythematous and edematous

REACTIONS IN LEPROSY
Reactions are acute episodes of hypersensitivity
reactions due to fluctuations in the immune
status of a leprosy patient. There are three types
of reactions; type 1 reaction, type 2 reaction and
Lucio phenomenon.

Type 1 Lepra Reaction


(Reversal Reaction)
It is a type IV hypersensitivity reaction
(Coombs and Gell type)
Seen in borderline spectrum (BT, BB, BL)
May manifest in the skin or nerve lesions of
leprosy
Erythema, edema and hyperalgesia of existing
skin lesions occurs (Fig. 36.17)
Occurrence of new lesions
Rarely ulcerations of skin lesions
Excruciatingly tender and thickened nerves,
with sensory or motor deficit
Patient can develop nerve palsies and in severe
reactions oedema of the hands and feet (Fig.
36.18)

Fig. 36.18: Type 1 lepra reaction- swelling of hands


and arm in severe type 1 lepra reaction

No constitutional symptoms usually seen


The principles of management of reversal
reaction are the following:
1. Continued maintenance of ALT.
2. Prolonged anti-inflammatory therapy.
3. Adequate analgesia.
4. Physical support.

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Most important aspect of type 1 lepra reaction
is not the skin lesions but the condition of
peripheral nerves
Mild reaction can be managed with analgesics
Severe reaction and neuritis needs oral
steroids, commonly prednisolone is used in
the dose of 30 to 40 mg per day.
Other drugs used are cyclosporin,
azathioprine, clofazamine, intraneural
injections of lignocaine
Management of nerve abscess by aspiration
or neurolysis.

Type 2 Lepra Reaction


It is a type III or immune complex mediated
lepra reaction
Occurs in lepromatous spectrum (most
commonly in LL, occasionally in BL)
Triggered by infection, vaccination, drugs and
stress
Characterized by tender, erythematous,
evanescent nodules called as erythema
nodosum leprosum (ENL) (Figs 36.19 and
36.20)
ENL occurs over the face and extensors
ENL does not occur in legs in contrast to
erythema nodosum which occurs in legs
Constitutional symptoms such as fever,
arthralgia, anorexia and myalgia occur
May have systemic involvement in the form of
conjunctivitis, keratitis, iritis, iridocyclitis,
orchitis, hepatomegaly and lymphadenopathy
The principles of treatment include.
Identifying and treating precipitating
factors.
Continuation or start of ALT.
Treatment of reaction.
Identification and management of
complications.
Primary drug therapy is with analgesics and
corticosteroids. Thalidomide is very effective
for both neuritis and pain in type 2 lepra
reaction and works faster than corticosteroids;
however it should be given under supervision.

Fig. 36.19: Type 2 lepra reaction- back showing


erythematous papular and nodular, tender lesions
in a case of lepromatous leprosy

Fig. 36.20: Pustular erythema nodosum leprosum

Other drugs used are clofazimine (up to 300


mg per day), pentoxifylline, levamisole,
cyclosporin, azathioprine, cyclophosphamide
and zinc.

Lucio Phenomenon
Occurs in Lucio leprosy due to bacterial
endotoxins, precipitated by stress and strain
Characterized by painful, large, bizarre
ulceration of skin and is not accompanied by
systemic features (Fig. 36.21).

Clinical Leprosy
The lesions occur over legs, thighs and buttock
Starting of antileprosy treatment
Steroids and anti-inflammatory agents are
main stay of therapy.

COMPLICATIONS
Leprosy has been regarded as a dreaded and
stigmatizing disease, which can lead to gross
disabilities, deformities and mutilations.

Nerve palsies: due to silent neuritis or


reactions-ulnar claw hand (Fig. 36.22), median
nerve palsy, facial palsy (Fig. 36.23), foot drop
and claw toes.
Anesthetic complications: trophic ulcer (Fig.
36.24) leads to osteomyelitis, distortion of toes
and shortening of hands and feet (Figs 36.25
and 36.26), tarsal disintegration and
malignancy.

Fig. 36.21: Lucio phenomenon- angular


hemorrhagic infarcts over the lower limb

Fig. 36.23: Complications- left-sided facial nerve


palsy resulting in inability to close the eye

Fig. 36.22: Complications- ulnar claw hand due to


ulnar nerve damage in borderline tuberculoid leprosy

Fig. 36.24: Complications- trophic


ulcer on the sole

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Essentials in Dermatology

Fig. 36.25

Fig. 36.26

Figs. 36.25 and 36.26: Complications- shortening of digits of hands and feet in lepromatous leprosy

OCULAR LEPROSY
It is an important cause of preventable blindness
Due to direct leprous involvement: madarosis,
conjunctivitis, superficial punctate keratitis,

pannus, corneal ulcer, iris pearls, chronic


plastic iridocyclitis
Due to type 2 lepra reaction: iritis, iridocyclitis,
episcleritis
Trigeminal nerve and facial nerve involvement
also can cause ocular complications.

Multiple Choice Questions

Multiple Choice Questions


Introduction with Tips for MCQs
Entrance Examinations for
Post- graduation courses in Medical
Schools in India
All India Entrance Examinations for admissions
to Post-graduate Medical Studies (MS/MS/
Diploma) is conducted by All India Institute of
Medical Sciences (AIIMS), New Delhi
As per Supreme Court Directives, All India
Entrance Examinations for admissions to Post
Graduate Medical Studies (MS/MS/Diploma)
is conducted by All India Institute of Medical
Sciences (AIIMS) New Delhi for 25% seats of
all Government and Municipal Medical
Colleges all over India and recognized by
Medical Council of India. (MCI).
However, the examination does not cover the
states of Andhra Pradesh and Jammu and
Kashmir. The number of seats finalized
through the entrance is approximately 1,800.
Examination is held every year on first Sunday
of January.
There will be one paper of 3 and 1/2 hours
duration containing 300 Multiple Choice
Questions (MCQs) from pre-clinical, paraclinical and clinical subjects.
All questions will be of objective type. Each
answer with correct response shall be awarded

four marks. Negative marking will be adopted


for incorrect responses. One mark will be
deducted for each negative response. Zero
mark will be given for the question not
answered. More than one answer indicated
against a question will be deemed as incorrect
response and will be negatively marked.
The competitive entrance examination shall
be of the standard of MBBS examination and
shall cover all the subjects of the M.B.B.S
course.

At PGIMER Entrance Examination


250 MCQs with five alternatives each and single/
multiple correct response are to be solved in 3
hrs. Negative marks will be given for incorrect
responses marked correct.

At JIPMER Pre PG Entrance


Examination
There will be one paper of 3 hours duration
containing 250 Multiple Choice Questions
(MCQs). 100 from pre/para clinical and 150
from clinical subjects. Exam usually occurs
on 3rd Sunday of March every year.

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Essentials in Dermatology
All questions will be of objective type. Each
answer with correct response shall be
awarded one mark. There is negative marking.
More than one answer indicated against a
question will be deemed as incorrect response

MAHE Pre PG Entrance Examination


There will be one paper of 3 hours duration
containing 200 Multiple Choice Questions
(MCQs) of pre-clinical/para-clinical/clinical
subjects and is online examination.
All questions will be of objective type. Each
answer with correct response shall be
awarded three marks. Negative marking will
be adopted for incorrect responses. One mark
will be deducted for each negative response.
Zero mark will be given for the question not
answered. More than one answer indicated
against a question will be deemed as incorrect
response and will be negatively marked.
THE MULTIPLE-CHOICE
QUESTIONNAIRE (MCQ)
Why MCQs?
The multiple-choice questionnaire (MCQ) has
become one of the standard assessment methods
in most medical schools over the past 30 years.
MCQs were developed to address problems
associated with traditional essay style testing.
They are seen as a more objective test of students
knowledge than essays. MCQs have clear right
and wrong answers and so are free of any
interpretation bias. They are generally regarded
as being reliable and thus consistent and valid
tests of students knowledge and information
recall.
Advantage of MCQs
MCQs have many advantages for teachers. Once
designed they are easy to administer and mark.
Most MCQs are answered on a card, which can
be read by a computer coupled with an optical
card reader. So the whole process can be

automated leaving teachers free to do other things.


The scoring is done by the computer, which also
reports on the reliability and discriminatory power
of the MCQ questions. So it seems that the MCQ is
an assessment design that you are likely to
encounter many times during your undergraduate and postgraduate career.

Disadvantage of MCQs
It is important to note, however, what MCQs
cannot do. They cannot measure clinical performance or competence. They can occasionally be
used to test components of diagnostic reasoning
and problem solving. MCQs have also been
criticized for providing candidates with visual
clues that suggest a correct answer, one that
the candidate might not otherwise have recalled.
For some courses in JIPMER, Pondicherry,
they are combined with other test formats that
test different aspects of candidates performance
- for example, objective structured clinical
examinations, short answer questions, etc.
Marking Schemes for MCQs
There are two types of marking commonly
encountered in MCQs. These are negative
marking where marks are deducted for incorrect
answers and neutral marking where all
questions should be attempted - marks are given
for correct answers only and guessing is
encouraged. Negative marking was introduced
to reduce the amount of guessing by candidates
by punishing incorrect responses with a
negative mark.
STRATEGIES IN ATTEMPTING
MULTIPLE-CHOICE QUESTIONS
Strategy before the test
1. Try to gather as many examples as you can
of old papers and previous examples of
MCQs used by the department or school in
question in the past.

Multiple Choice Questions


2. Solve last year papers as 30-40% questions
are repeated. At the time of final revision by
solving mock test (like solve and send series)
helps a lot.
3. Do not, however, try to memorize hundreds
of responses to questions. The factual
knowledge you will gain will be superficial
and dissociated. It is better to look for the
topic areas that recur frequently and ensure
that you have a deeper knowledge of these
topics.
4. Revise with friends and colleagues. You
can share knowledge and techniques.
5. Practice makes a man perfect, is true for
any competitive exam. Learning by heart
helps very little, but if it is done with
understanding and clarity in concepts
followed by writing it out helps much better.
It prepares you to perform in exams
confidently and intelligently.
6. Practice is the key to success and attempting
sufficient number of mock tests before the
actual exams will help you improve your
presentation and scoring skills.
7. It helps you to improve your speed; thereby
you can complete your papers on time.
8. You never get a second chance to improve
yourself or rectify your mistakes. Once done
is final, something on which your whole
future is dependent. Mock tests will help you
realize your silly mistakes and thereby
eliminate them before the actual exams.
9. You will get thoroughly conversant with the
style and standard of entrance exam
questions papers and assessment of answer
papers.
10. Writing a number of mock tests shall relieve
you of examination fear and develop
tremendous confidence to score higher ranks.
11. You should know what type of MCQ is being
set for you. Will there be negative marking?
How much time will you have and how
many questions will there be?

Strategies During the Test


1. Check that your understanding of the MCQ
format is correct. It is negative marking,
there are 300 questions, and I have two hours
to complete this. Always read the stem for
each question carefully. Have you understood the question? Are there any ambiguities? If so ask an invigilator who will alert
an examiner. There are usually one or two in
the room. Allocate three quarters of the time
to answering the questions and a period at
the end to checking answers and accuracy.
2. Preview the test: Read through the paper
briefly to acquaint yourself with the task
ahead. Make sure your copy has no missing
or duplicate pages. Read the directions
carefully.
3. Start with questions that you can readily
answer: To build your confidence and to
save time for the harder ones. When you
identify a correct response carefully mark this
on the question paper. If you are unable to
make a choice and need to spend more time
with the question, or you answered the
question but are not at all sure that you made
the correct choice, put a big question mark
beside that question, and move on to the next.
Avoid getting bogged down on one question.
It is much better to move on and finish all
those questions that you can answer and
then come back later to process the
problematic questions. Sometimes the
answer will occur to you simply because you
are more relaxed after having answered
other questions.
4. Read each question carefully: Multiplechoice tests also examine your ability to read
carefully and thoughtfully, as much as they
test your ability to recall and reason.
5. Identify key words: Circle or underline key
words, such as except, true, all,
always, never, none, not, few,
many, some, and sometimes.

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6. Identify subject area: Identifying what
lecture, reading, or laboratory exercise the
question is from which might help you
narrow the choice of possible responses.
7. Identify what is being asked: Answer each
question as teacher intended, that is, within
the context of the course material that was
taught.
8. The cover up strategy: Some students find
it helpful to read the question and try to recall
the answer from memory before looking at
each of the four responses.
9. The true/false strategy: Identify if the
question is looking for a true or false
statement. Then label each of the four
responses as true or false and eliminate
those that do not correctly complete the
question.
10. Read each of the four responses: And do
not just stop when you come upon the one
that seems likely.
11. Do not select a response just because you
remember learning the information in the
course; it may be a true statement in its
own right, but not the best answer to the
question.
12. Do not dismiss a response because it
seems too obvious and simple an answer;
if you are well prepared for the test, some
of the questions may appear very straightforward.
13. Do not be persuaded by fancy terms in the
question; do not say to you, That sounds
impressive, so it must be the right answer.
As you read through the possible responses,
mark off the ones you know are wrong. This
will save time if you have to come back to the
question later.

14. Should I change an answer? : Change


answers only if you have a good reason for
doing so. (Changing your answer from
response b because you selected b to the
previous two questions is not a good
reason.). The origin of the myth that students
most often change correct answers to wrong
answers is probably that it is the wrong
answers that students remember most when
reviewing the test (for you are less likely to
remember the answers you changed from
wrong to right).
15. If two responses appear to be equally
correct: Eliminate the response that appears
to be least related to the question being asked.
Remember, you are looking for the best
answer, not only a correct one. Some
responses may be correct but are not directly
related to the question.
16. If you are not certain of an answer, do not
guess wildly: There is a stiff penalty for
wrong answers (negative marking).
Eliminate the responses you know are
incorrect. Narrow down your selection to two
responses and then compare them and
identify how they differ. Finally, make an
informed, intelligent guess. Do not make any
wild guess, as they will do you more harm
than good.
17. If the MCQ has a neutral marking design
you should answer all questions. You will
not be penalized for guessing, so have a go.
You cannot score if you leave answers blank.
18. It is generally advised that you do not guess
in situations where you have not a clue
and there is negative marking. The half and
half rule applies both ways. You may
improve your position; you could also
worsen it considerably.

Multiple Choice Questions

MCQs in Dermatology, Sexually Transmitted Diseases and Leprosy for


MBBS Students for PG Entrance Examination

Set I (1-400)
1. A patient is said to have AIDS when the
CD-4 count is
A. 200 to 499
B. <200
C. <100
D. <50
2. About autosomal dominant inheritance all
are true except
A. It is expressed in homozygotes
B. Always one parent is affected
C. 50% of children affected
D. Offspring of a non-diseased child of a
diseased parent will not have the mutant
gene.
3. Harlequin skin changes are associated
with
A. It is a normal phenomenon
B. Seen in ichthyosis
C. Septicemia
D. Atopic dermatitis

B. Systemic griseofulvin
C. Griseofulvin topically
D. Amphotericin
7. Grayish white vaginal discharge with
formation of bubbles is a feature of
A. Trichomoniasis
B. Bacterial vaginosis
C. Candida albicans
D. Gonorrhea
8. The most effective means of transfer of
HIV is
A. Sexual route
B. Perinatal route
C. Blood transfusion
D. Needle prick
9. Myiasis means
A. Infestation with Maggots
B. Infestation with fungi
C. Infestation with Bacillus anthracis
D. Infection with Bartonella hensalae

4. Norwegian scabies usually affects


A. Elderly
B. Children
C. Immunocompromised
D. Infants

10. Chancroid is caused by


A. Treponema pallidum
B. Hemophilus ducreyi
C. Calymmatobacterium granulomatis
D. Chlamydia trachomatis

5. A 67-year-old patient presents with


complaints of a pruritic red lesion on his
forearm, which is having silvery white
scales on a red base. The probable
diagnosis is
A. Psoriasis
B. Leprosy
C. Lichen planus
D. Pityriasis alba

11. Which of the following fungi is most


difficult to be cultured in vitro?
A. Microsporum canis
B. Rhinosporidium seeberi
C. Sporothrix schenkii
D. Coccidioides immitis

6. Which of the following is the treatment of


choice for tinea unguium?
A. Miconazole

12. Which of the following is NOT seen in


acute intermittent porphyria?
A. Abdominal pain
B. Neuropsychiatric manifestations
C. Dermatological manifestations
D. Passing dark coloured urine

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Essentials in Dermatology
13. All of the following are used in urticaria
except
A. Antihistamines
B. Topical glucocorticoids
C. Cyproheptadine
D. Hydroxyzine
14. Epstein Barr virus causes all of the
following diseases except
A. Burkitts lymphoma
B. Nasopharyngeal carcinoma
C. Hodgkins lymphoma
D. Kaposis sarcoma

C. Pigment deposition occurs


D. Blanches on pressure
20. Erysipelas is caused by
A. Streptococci
B. Staphylococcus aureus
C. Hemophilus influenzae
D. Bacillus anthracis
21. Tzanck test is done in infection with
A. Herpes simplex
B. Cytomegalovirus
C. Epstein Barr virus
D. Human papillomavirus

15. Oral candidiasis is seen in all of the


following diseases except
A. Pregnancy
B. Diabetes mellitus
C. Hypertension
D. Immunodeficiency

22. Which of the following is true about


rhinophyma
A. Seen in tuberculosis
B. Caused by K. scleromatis
C. Seen in Rhinosporidiosis
D. Seen in Rosacea

16. Topical preparation of Vitamin D


(Calcipotriol) is used in which of the
following skin conditions?
A. Psoriasis
B. Pityriasis rosea
C. Lichen planus
D. Lichen nitidus

23. Differentiation between multibacillary


and paucibacillary leprosy is based on
A. Morphological index
B. Bacteriological index and skin lesions
C. Lepromin test
D. Skin lesions and lepromin

17. Incubation period for measles is:


A. 2 7 days
B. 10 14 days
C. 14 28 days
D. 3 4 weeks
18. According to CDC/ WHO criteria for AIDS,
which of the following cannot be considered as a criterion?
A. Cryptococcosis
B. Oral candidiasis
C. Kaposis sarcoma
D. Disseminated Mycobacterium avium
complex infection
19. Which is NOT true about purpura?
A. Size more than 3 mm
B. Bleeding into the dermis

24. Most common cause of pelvic inflammatory disease is


A. Chlamydia trachomatis
B. Tuberculosis
C. Gonococci
D. Group B streptococci
25. Golden yellow crust is seen in
A. Impetigo
B. Ecthyma
C. Malignant pustule
D. Pseudomonas infection
26. A neonate suspected to have congenital
syphilis, diagnostic test to be done is
A. FTA-ABS IgM
B. FTA-ABS IgG
C. TPI
D. TPHA

Multiple Choice Questions


27. A child presents with seborrheic dermatitis, lytic skull lesions, ear discharge,
hepatosplenomegaly; likely diagnosis
A. Leukemia
B. Lymphoma
C. Histiocytosis X (Langerhans cell
histiocytosis)
D. Leiners disease
28. If urine sample darkens on standing, most
likely condition is
A. Phenylketonuria
B. Alkaptonuria
C. Mapple syrup urine disease
D. Homocystinuria

33. A girl aged 19 with arthritis, alopecia, photosensitive rash on the cheek; diagnosis
A. SLE
B. DLE
C. Dermatomyositis
D. Systemic sclerosis
34. A child with itchy lesions of scabies over
the groin and the genitalia. What will not
to be advised?
A. Bathe and apply scabicidal
B. Treat family
C. Burn clothes
D. Antibiotics

29. All of the following are true regarding


Ureaplasma urealyticum except
A. Causes non gonococcal urethritis
B. Associated with infertility
C. Bacterial vaginosis
D. Epididymitis

35. A man aged 19 years gets painless penile


ulcer 10 days after sexual intercourse with
a professional sex worker. Diagnosis is
A. Chancroid
B. Herpes genitalis
C. Primary chancre
D. Traumatic ulcer

30. A male patient, 20 years old, from Jaipur,


has erythematous lesion with central
crusting, on cheek; diagnosis
A. SLE
B. Lupus vulgaris
C. Chilblains
D. Cutaneous leishmaniasis

36. A boy aged 8 years from TN has a white


non anesthetic non-scaly hypopigmented
macule on his face
A. Pityriasis alba
B. Pityriasis versicolor
C. Indeterminate leprosy
D. Polymorphous light eruption

31. A child aged 3 years, has alopecia, boggy


scalp swelling; hair easily pulled out;
diagnostic test
A. KOH examination
B. Culture sensitivity
C. Biopsy
D. Grams staining

37. The most common organism causing Tinea


capitis
A. Trichophyton violaceum
B. Microsporum canis
C. Epidermophyton floccosum
D. Candida albicans

32. A girl aged 19 years has light brown


pigmentation over the malar eminences;
diagnosis
A. Chloasma
B. SLE
C. Photodermatitis
D. Tinea versicolor

38. Which of the following is not effective in


Tinea versicolor?
A. Clotrimazole
B. Ketoconazole
C. Griseofulvin
D. Selenium sulphide
39. The duration of therapy with Griseofulvin
for tinea unguium is
A. 6-9 months
B. 4 months
C. 2 months
D. 1 month

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40. Genital elephantiasis is seen in
A. Donovanosis
B. Lymphogranuloma venereum
C. Herpes simplex
D. Syphilis
41. A patient, Gopi, had lepromatous leprosy.
Which of the following is true regarding
globi in leprosy?
A. Consists of lipid laden macrophages
B. Consists of macrophages filled with
bacteria
C. Consists of neutrophils filled with
bacteria
D. Degenerated neural tissue
42. All the following are sexually transmitted
infections except
A. Candida
B. Group B streptococcus
C. Hepatitis B
D. Echinococcus
43. Exfoliative dermatitis is seen in all the
following except
A. Pityriasis rosea
B. Pityriasis rubra pilaris
C. Psoriasis
D. Drug reaction
44. In a patient, annular erythematous lesions
on the trunk were seen circumscribed by
collarettes scales. The diagnosis is most
likely to be
A. Pityriasis versicolor
B. Pityriasis rosea
C. Pityriasis rubra pilaris
D. Lichen planus
45. Wickhams striae are seen in
A. Lichen sclerosis et atrophicus
B. Lichen planus
C. Lichen aureus
D. Lichen nitidus
46. A patient with psoriasis was started on
systemic steroids. After stopping treatment,

the patient developed generalized


pustules all over his body. The cause is
most likely to be
A. Drug induced reaction
B. Pustular psoriasis
C. Bacterial infections
D. Septicemia
47. A 6-month-old infant develops high-grade
fever, which subsided after 3 days
following which there was appearance of
a generalized rash, which too subsided
after 48 hours without any residual
pigmentation. Which of the following is the
most likely diagnosis?
A. Fifth disease
B. Roseola infantum
C. Measles
D. Rubella
48. A patient, Bindu, presents with vaginal
discharge. Examination of the discharge
reveals the presence of Chlamydial
infection. The treatment of choice is
A. Azithromycin + contact tracing
B. Doxycycline + Metronidazole
C. Fluconazole + Doxycycline
D. Metronidazole
49. The most bactericidal drug in leprosy is
A. Rifampicin
B. Dapsone
C. Clofazimine
D. Ofloxacin
50. Steroids cause all the following except
A. Hypertrophy of muscle
B. Hypokalemia
C. Neuropsychiatric symptoms
D. Hyperglycemia
51. The treatment of pustular psoriasis is
A. Retinoids
B. Thalidomide
C. Steroids
D. Hydroxyurea
52. Pruritic ring-like lesion beneath the breast
is likely to be
A. Pityriasis rubra pilaris
B. Trichophytosis
C. Lichen planus
D. Bacterial infections

Multiple Choice Questions


53. Woods lamp examination is helpful in the
diagnosis of
A. Tinea versicolor B. Psoriasis
C. Lichen planus D. Varicella
54. A patient has scaly lesions on the elbow
and knee joint. Which of the following is a
bedside test for provisional diagnosis of
the illness
A. Auspitzs sign B. KOH preparation
C. Tzanck smear D. Slit skin smear
55. A male patient presents with multiple
nodulocystic lesions on the face with
multiple sinuses. The treatment of choice
is
A. Antibiotics
B. Retinoids
C. Antituberculous drugs
D. Steroids
56. Granules coming out of the multiple
discharging sinuses in mycetoma foot
contains
A. Fungal colonies
B. Pus cells
C. Inflammatory cells
D. Granuloma
57. Babloo, a 4-year-old boy, presents with
history of seizures. On examination there
is hypopigmented patches on his face.
There is mental retardation also. What is
the most likely diagnosis?
A. Neurofibromatosis
B. Tuberous sclerosis
C. Sturge Weber syndrome
D. Incontinentia pigmenti
58. ANCA is not associated with which of the
following diseases
A. Wegeners granulomatosis
B. Henoch-Schnlein purpura
C. Microscopic PAN
D. Churg Strauss syndrome

59. A child presents with a solitary white welldefined patch of his right thigh. What is
the diagnosis
A. Piebaldism
B. Nevus achromicus
C. Acral vitiligo D. Albinism
60. A patient gets recurrent urticaria while
doing exercising and on exposure to
sunlight. Which of the following is the most
likely cause
A. Chronic idiopathic urticaria
B. Universal dermographism
C. Cholinergic urticaria
D. Solar urticaria
61. Vasanti, a 28-year-old female, presents
with complaints of tightness of fingers.
There is also history of dysphagia. Which
of the following is the probable diagnosis?
A. Dermatomyositis
B. Scleroderma
C. Rheumatoid arthritis
D. Systemic lupus erythematosus
62. A lady approaches a physician for
contraceptive advice. On examination
there were two symmetrical painless ulcers
on vulva, which were well defined with a
firm base. Which of the following is the
most likely cause
A. Primary syphilis
B. Chancroid
C. Herpes genitalis
D. Malignancy
63. A girl presents with complaints of melena.
On examination there are pigmented
lesions involving her mouth and lips. Two
of her sisters also had similar complaints.
Which of the following is the most
probable diagnosis?
A. Cronkhite-Canada syndrome
B. Peutz Jeghers syndrome
C. Gardners syndrome
D. Behets syndrome

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Essentials in Dermatology
64. A 30-year-old Basanti presents with light
brown lesions involving both her cheeks.
The lesions had never been erythematous.
Which of the following is the most
probable diagnosis
A. SLE
B. Chloasma
C. Air borne Contact Dermatitis
D. Photodermatitis
65. Babloo, 5 years old male, presents with
small hypopigmented scaly macules on his
cheek. Some of his classmates also have
similar lesions. Which of the following is
the most probable diagnosis
A. Pityriasis alba
B. Pityriasis rosea
C. Pityriasis versicolor
D. Indeterminate leprosy
66. Kallu, a 30-year-old man, presented with
subcutaneous itchy nodules over the left
iliac crest. On examination, they are firm,
non-tender and mobile. Skin snips contain
microfilaria and adult worms of
A. Loa Loa
B. Onchocerca volvulus
C. Brugia malayi
D. Mansonella perstans
67. All are true about Neisseria gonorrhea
except
A. Gram positive cocci
B. Causes stricture urethra
C. Involves seminal vesicles and spreads
to epididymis
D. Drug of choice is Ceftriaxone
68. A 30-year-old male, Kallu, with a history
of sexual exposure comes with a painless
granulomatous ulcer over the penis with
everted margins. The diagnosis is
A. Primary chancre
B. Chancroid
C. Lymphogranuloma venereum
D. Donovanosis

69. All are features of peripheral neuritis in a


patient with Hansens disease except
A. Predominant sensory involvement
B. Decreased tendon reflexes
C. Mutilations
D. Muscle paralysis
70. Vitamin D is synthesized in
A. Stratum basale
B. Stratum spinosum
C. Stratum granulosum
D. Stratum corneum
71. Tram track appearance on CT scan of the
head is seen in
A. Sturge Weber syndrome
B. von Hippel Lindau disease
C. Tuberous sclerosis
D. Neurofibromatosis
72. A 40-year-old farmer with a history of
recurrent attacks of pain abdomen,
complains of itching when exposed to the
sun and maculopapular rash on sunexposed areas. His symptoms are exaggerated in the summer. The diagnosis is
A. Congenital erythropoietic porphyria
B. Acute intermittent porphyria
C. Variegate porphyria
D. Porphyria cutanea tarda
73. An 8-year-old boy presents with a welldefined annular lesion over the buttock
with central scarring that is gradually
progressing over the last 8 months. The
diagnosis is
A. Annular psoriasis
B. Lupus vulgaris
C. Tinea corporis
D. Annular lichen planus
74. An adult presents with oval scaly
hypopigmented macules over the chest
and the back. The diagnosis is
A. Leprosy
B. Lupus vulgaris
C. Pityriasis versicolor
D. Lichen planus

Multiple Choice Questions


75. The characteristic nail finding in lichen
planus is
A. Pitting
B. Pterygium
C. Beaus lines
D. Hyperpigmentation of the nails
76. In an 8-day-old child with no history of
consanguinity in the parents, the mother
reports blisters and peeling off of the skin
at the site of handling and pressure. There
was a similar history in the previous child,
which proved to be fatal. The diagnosis is
A. Bullous pemphigoid
B. Congenital syphilis
C. Epidermolysis bullosa
D. Letterer-Siwe disease
77. An 8-year-old female child following URTI
developed maculopapular rash on the face
spreading onto the trunk which cleared on
the 3rd day without desquamation and
tender post auricular and suboccipital
lymphadenopathy. The diagnosis is
A. Measles
B. Rubella
C. Erythema infectiosum
D. Kawasaki disease (Mucocutaneous LN
Syndrome)
78. A patient presented with scarring alopecia,
thinned nails, hyperpigmented macular
lesions over the trunk and oral mucosal
lesions. The diagnosis is most likely to be
A. Lichen planus
B. Psoriasis
C. Secondary syphilis
D. Scleroderma
79. A young boy presented with a lesion over
his right buttock, which had peripheral
scaling and central clearing with scarring.
The investigation of choice would be
A. Tzanck smear
B. KOH preparation
C. Biopsy
D. Culture on Sabourauds agar

80. Acne vulgaris is due to involvement of


A. Sebaceous glands
B. Eccrine glands
C. Hair follicles
D. Apocrine glands
81. The term tuberculids includes all except
A. Lichen scrofulosorum
B. Lichen nitidus
C. Erythema nodosum
D. Lupus miliaris
82. According to the WHO revised criteria, all
are true regarding the treatment of leprosy
except
A. All multibacillary cases should be
treated for 12 months with multi drug
therapy
B. Treatment of paucibacillary leprosy
involves Rifampicin 600 mg once a
month and Dapsone 100 mg daily for 6
months
C. Treatment of multibacillary leprosy
involves Rifampicin 600 mg once a
month, clofazimine 300 mg once a month
and 50 mg OD and Dapsone 100mg OD
for 6 months
D. Treatment of multibacillary leprosy
involves Rifampicin 600 mg once a
month, clofazimine 300mg once a month
and 50 mg OD and Dapsone 100mg OD
for 12 months
83. Regarding Neurofibromatosis Type I,
following are true except
A. Lisch nodules are seen
B. Multiple cafe-au-lait spots are seen
C. Plexiform neurofibroma is associated
D. Posterior capsular lenticular opacity is
seen
84. Eczema herpeticum is associated with
infection due to
A. Herpes simplex
B. Varicella
C. HHV 8
D. Parvo virus

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Essentials in Dermatology
85. Tinea capitis (Endothrix) is most
commonly caused by
A. T.mentagrophytes
B. E.floccosum
C. T.tonsurans
D. T.violaceum
86. Viable but not cultivable state is seen in
which of the following organisms
A. M leprae
B. M. tuberculosis
C. V. cholera
D. Staph. aureus
87. Regarding Henoch Schonlein purpura, all
are true except
A. Associated with glomerulonephritis
B. Palpable purpura
C. Decreased complement
D. Thrombocytopenia
88. Dyskeratosis is a feature of
A. Dariers disease
B. Pemphigus vulgaris
C. Psoriasis
D. Lichen planus
89. Regarding AIDS all are true except
A. Male to female transmission is more
common than vice versa
B. Condom provides 100% protection
C. Kissing is considered to be safe
D. Risk of transmission is more in the
presence of associated STDs
90. Staphylococcus aureus classically
causes
A. Impetigo contagiosa
B. Furuncle
C. Ecthyma
D. Blistering dactylitis
91. Which of the following is/are associated
with Swimming pool granuloma?
A. M. marinum
B. M. avium complex
C. M. ulcerans
D. M. Chelonae

92. All are AFB stain positive except


A. M. leprae
B. M. tuberculosis
C. Nocardia
D. M. mycetomatis
93. Lymes disease is caused by
A. Borrelia recurrentis
B. Fusobacteria
C. Spirochete
D. Borrelia burgdorferi
94. All are true about rhinophyma except
A. Fungal infection
B. Acne rosacea
C. Dermabrasion
D. Electrosurgery
95. A child presents with slapped cheek
appearance of face, diagnosis is
A. Pityriasis alba
B. Tinea versicolor
C. Pityriasis rosacea
D. Erythema infectiosum
96. Which of the following is not a feature of
rhinosporidiosis?
A. Bleeding polyp
B. Russell bodies are seen
C. Oral dapsone is useful in treatment
D. Chiefly involves eyes
97. Which of the following is the commonest
cause of urethritis with leukorrhea?
A. Chlamydia trachomatis
B. Gardnerella vaginalis
C. Trichomonas vaginalis
D. Candida albicans
98. Munros micro abscesses is seen in
A. Stratum corneum
B. Stratum basale
C. Dermis
D. Stratum spinosum
99. The drug of choice if available, for type 2
lepra reaction is
A. Clofazimine
B. Chloroquine
C. Thalidomide
D. Steroids

Multiple Choice Questions


100. A 6 months child, has developed
irritability, photophobia, diarrhoea and
hair loss of 2 months duration, following
weaning from breast milk. The child has
an eczematous rash around the perioral
and perianal area. His ALP is 500 IU/L, Hb
- 8.0 gm%, SGOT- 15 IU/L and SGPT- 20
IU/L. What is your diagnosis?
A. Acrodermatitis enteropathica
B. Selenium deficiency
C. Protein energy malnutrition
D. Biotin deficiency
101. Which of the following is not a pyoderma?
A. Furuncle
B. Ecthyma
C. Pyoderma gangrenosum
D. Carbuncle
102. Treatment of choice for disseminated
gonococcal infection is
A. Penicillin
B. Ceftriaxone
C. Doxycycline
D. Erythromycin
103. Scabies in children differs from that in
adults in that it affects
A. Finger web spaces
B. Face
C. Genitalia
D. Axilla
104. Swollen cherry red lip with strawberry
tongue is a feature of
A. Kawasaki disease
B. Scarlet fever
C. Toxic shock syndrome
D. Staphylococcal scalded skin syndrome

C. Trichomonas vaginalis
D. Leishmania brasiliensis
107. A smear from a genital lesion shows
Donovan bodies. The diagnosis is
A. Lymphogranuloma venereum
B. Chancroid
C. Granuloma inguinale
D. Primary chancre
108. The minimum period of treatment for
multi-bacillary leprosy is
A. 1 year
B. 2 years
C. 6 months
D. 9 months
109. Refsums disease is due to defect in
enzyme
A. Phytanic acid oxidase
B. Nicotinamide-adenine dinucleotide
oxidoreductase.
C. Homogentisic acid oxidase
D. Tyrosinase
110. Vesicular lesion is seen in
A. Primary syphilis
B. Secondary syphilis
C. Tertiary syphilis
D. Congenital syphilis
111. A patient comes with recurrent abdominal
pain, and urinary examination reveals
Ehrlich aldehyde test (+) for urobilinogen.
The diagnosis is
A. Porphyria
B. Renal calculi
C. Cholelithiasis
D. Angina pectoris

105. Maculae cerulea is seen in


A. Pediculosis corporis
B. Pediculosis capitis
C. Phthirus pubis
D. None of the above

112. In urticaria pigmentosa, the cell most


commonly seen in skin on microscopy is
A. Mast cell
B. Langerhans cell
C. Langhans giant cell
D. Fibroblasts

106. Which of the following is not sexually


transmitted?
A. Entamoeba histolytica
B. Giardia lamblia

113. An elderly male with dry skin and fissuring


scaly skin is likely to have
A. Nummular eczema
B. Venous eczema

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Essentials in Dermatology
C. Asteatotic eczema
D. Seborrheic dermatitis

C. Can cause arthritis


D. It commonly occurs in flexural areas

114. Thimble pitting in nails is seen in


A. Psoriasis
B. Tinea versicolor
C. Lichen planus
D. Tuberculosis

121. Koebner phenomenon is seen in


A. Erythema marginatum
B. Pemphigus
C. Lichen planus
D. Pityriasis Rosea

115. Ridley-Jopling classification includes all


except
A. Lepromatous leprosy
B. Borderline borderline leprosy
C. Tuberculoid leprosy
D. Neuritic leprosy

122. The following statements are true about


gonococci except
A. It causes vulvovaginitis in young girls
B. Causes interstitial pneumonia in new
born
C. Causes non specific urethritis
D. Causes endocervicitis

116. Which one of the following is due to type


IV hypersensitivity
A. Irritant dermatitis
B. House wife dermatitis
C. Air borne contact dermatitis
D. Neurodermatitis

123. In a patient 28 years old presenting with


infertility and recurrent history of PID with
lower pelvic pain must be investigated for
which of the following infections?
A. Gonococci
B. HIV
C. Tuberculosis D. Chlamydia

117. Hidradenitis suppurativa is due to


infection of
A. Apocrine sweat glands of axilla
B. Subcutaneous tissues
C. Sebaceous glands
D. Eccrine sweat glands

124. Tzanck test is used in the diagnosis of


A. Epidermolysis bullosa
B. Bullous impetigo
C. Pemphigus vulgaris
D. Tinea corporis

118. Podophyllin is used in the treatment of


A. Plantar wart
B. Plane wart
C. Condyloma lata
D. Condyloma acuminata

125. Pseudobubo is seen in


A. Granuloma inguinale
B. Lymphogranuloma venereum
C. Chancroid
D. Syphilis

119. Type 1 lepra reaction is seen in all except


A. Tuberculoid leprosy
B. Borderline tuberculoid leprosy
C. Borderline borderline leprosy
D. Borderline lepromatous leprosy

126. Apple-jelly nodules are seen


A. Tuberculosis verrucosa cutis
B. Scrofuloderma
C. Tuberculous gumma
D. Lupus vulgaris

120. All the following are true about psoriasis


except
A. Scalp is commonly involved
B. Nails are involved

127. A 3-year-old child was brought by his


mother with complaints of a rash on the
scalp, which was not responding to
therapy. Cutaneous examination showed

Multiple Choice Questions


few scaly purpuric papules over trunk and
greasy scaling over scalp. The child also
had loosening of teeth. Biopsy of the
papule showed large cells with reniform
nuclei. Skiagram of skull showed a few
lytic lesions. What is your diagnosis?
A. Histiocytosis
B. Neuroblastoma
C. Seborrheic dermatitis
D. Gauchers disease
128. Frei skin test is used in the diagnosis of
A. Lymphogranuloma venereum
B. Sarcoidosis
C. Leishmaniasis
D. Granuloma venereum
129. Strawberry vaginitis is seen in infection
with
A. Gonococcus
B. Trichomonas vaginalis
C. Hemophilus vaginalis
D. Candida albicans
130. Hemophilus ducreyi is the causative agent
of
A. Hard chancre
B. Urethritis
C. Soft sore
D. Granuloma inguinale
131. Commonest type of basal cell carcinoma
is
A. Noduloulcerative type
B. Superficial spreading type
C. Morphoeic type
D. Pigmented type

C. Staphylococci
D. Pneumococci
134. Endotoxins are implicated in all except
A. Psoriasis
B. Kawasaki disease
C. Atopic dermatitis
D. Seborrheic dermatitis
135. Skin lesions caused by Staphylococcus
aureus include all except:
A. Bullous impetigo
B. Staphylococcal scalded skin syndrome
C. Scarlet fever
D. Furuncle
136. Ecthyma gangrenosum is caused by
A. Staphylococcus aureus
B. Pseudomonas aeruginosa
C. Streptococcal infection
D. All of the above
137. The most common site of carbuncle
A. Nape of neck
B. Thigh
C. Face
D. Legs
138. Botryomycosis is due to
A. Bacteria
B. Fungi
C. Protozoa
D. Algae
139. Toxic shock syndrome; all are true except
A. Exotoxins producing strain of S.aureus
B. Seen in menstruating women
C. Fever, hypotension and rash are seen
D. Pastias lines are present

132. Chronic paronychia is caused by


A. Mycobacterium tuberculosis
B. Improper treatment of acute paronychia
C. Fungal infection
D. Repeated trauma

140. Blistering distal dactylitis is mainly caused


by
A. Group A streptococci
B. Group B streptococci
C. Staphylococci
D. E. coli

133. Schwartzmann phenomenon is seen in


A. Meningococcemia
B. Syphilis

141. Purpura fulminans


A. Follows group A streptococcal infection
B. Seen with deficiency of protein C

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Essentials in Dermatology
C. DIC occurs in this condition
D. All are true
142. Pitted keratolysis is caused by
A. Micrococcus sedentarius
B. Dermatophilus congolensis
C. Corynebacterium
D. All the above
143. Erythrasma, all are true except
A. Caused by corynebacterium minutissimum
B. It is a gram negative rod
C. Coralred fluorescence is seen with
woods lamp
D. It is treated using clotrimazole cream
144. All are true of trichomycosis axillaris
except
A. It is caused by fungus
B. Nodular thickening of hair shaft present
C. It involves axilla commonly
D. Treatment is shaving
145. Russell bodies are seen in
A. Rhinoscleroma
B. Leishmania
C. Syphilis
D. Leprosy
146. All are true about bacillary angiomatosis
except
A. Caused by B. henselae and B. quintana
B. Seen in AIDS patients
C. Diagnosed by Warthin Starry stain
D. Treatment is by ciprofloxacin
147. All are seen in anthrax except
A. Malignant pustule is seen
B. It is usually painful
C. Parenteral crystalline penicillin is the
drug of choice
D. Incision and debridement should be
avoided
148. Apple jelly nodules are seen in
A. Sarcoidosis
B. Lupus vulgaris

C. Leprosy
D. Cat-scratch disease
149. Scrofuloderma involves all except
A. Lymph nodes B. Bone and joints
C. Epididymis
D. Penis
150. Buruli ulcer is caused by
A. M. ulcerans
B. M. marinum
C. M. bovis
D. M. leprae
151. About staphylococcal scalded skin
syndrome all are true except
A. It is caused by phage II S. aureus
B. It is a exotoxin mediated condition
C. Can be seen in infants and adults
D. Diagnosis is by demonstration of
staphylococcus by gram stain
152. All are true about Orf except
A. Ecthyma contagiosum is a synonym
B. Caused by parapox virus
C. Treatment is acyclovir
D. Disease widespread in sheep and goats
153. All are true of molluscum contagiosum
except
A. May be seen in HIV infection
B. Umbilicated vesicles are characteristic
C. Caused by molluscipox virus
D. Treatment is by curettage
154. Papular purpuric gloves and sock
syndrome is caused by
A. EBV
B. Parvovirus B19
C. HSV
D. HPV
155. Gianotti Crosti syndrome is due to
A. Hepatitis B
B. Coxsackie virus
C. EBV
D. All of the above
156. Forscheimers sign is seen in
A. Polio
B. Rubella
C. Toxoplasma
D. Roseola infantum

Multiple Choice Questions


157. All are due to HPV infection except
A. Epidermodysplasia verruciformis
B. Buschke Lowenstein tumour
C. Epidermoid plantar cysts
D. Condylomata lata
158. EBV causes
A. Oral hairy leukoplakia
B. Gianotti Crosti syndrome
C. Lipshutz ulcer
D. All of the above
159. Roseola infantum is caused by
A. HHV-4
B. HHV-5
C. HHV-6
D. HHV-7

C. Pemphigoid
D. PLE
164. Drug induced pemphigus is seen in all
except
A. Penicillamine
B. Rifampicin
C. Chloroquine
D. Captopril
165. Acantholysis is seen in
A. Epidermis
B. Dermis
C. Dermal-epidermal junction
D. Subcutaneous tissue
166. Subepidermal bulla is seen in
A. Pemphigus vulgaris
B. Pemphigoid
C. Hailey Hailey disease
D. Transient acantholytic dermatoses

160. All are true about herpes zoster except


A. Occasionally pain is not followed by
eruption
B. Multidermatomal zoster is a clinical
marker for AIDS
C. Postherpetic neuralgia occurs 2 weeks
after eruption
D. Eruption can be bilateral

167. Drug of choice in dermatitis herpetiformis


A. Dapsone
B. Tetracycline
C. Psoralen
D. Steroids

161. Kaposis varicelliform eruption is caused


by
A. Herpes simplex virus
B. Coxsackie virus
C. Vaccinia
D. All of the above

168. Bullous disorder associated with gluten


sensitive enteropathy
A. Pemphigus
B. Bullous pemphigoid
C. Both A and B
D. None of the above

162. About herpes simplex virus infection all


are true except
A. It is a common cause of erythema
multiforme
B. Both HSV-1 and HSV-2 cause herpes
genitalis
C. Mucocutaneous herpes simplex
infection of 3 weeks is a CDC criteria for
AIDS
D. It does not cause herpes gestations

169. Row of tomb stone appearance seen in


A. Pemphigus
B. Pemphigoid
C. Epidermolysis bullosa
D. None of the above

163. Intraepithelial bulla is a feature of


A. Pemphigus
B. Dermatitis herpetiformis

171. Asboe-Hansen sign (Bulla spread sign)


elicited in
A. Bullous pemphigoid

170. Nikolskys sign is positive in


A. Pemphigus vulgaris
B. Bullous pemphigoid
C. Pemphigus follaceous
D. Both A and C

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Essentials in Dermatology
B. Dermatitis herpetiformis
C. Pemphigus vulgaris
D. Friction blister
172. Acantholysis is due to dissolution of
intercellular cement substance in the
A. Epidermis
B. Sub-epidermis
C. Dermis
D. Basement membrane
173. A 60 years old man presented with itchy
tense hemorrhagic blisters over thigh and
lower abdomen without any oral lesions
diagnosis
A. Bullous pemphigoid
B. Pemphigus
C. Both A and B
D. Dermatitis herpetiformis
174. Koebner phenomenon seen in
A. Lupus erythematosus
B. Syphilis
C. Lupus vulgaris
D. Psoriasis
175. Not transmitted sexually
A. Syphilis
B. Yaws
C. Gonorrhea
D. Chancroid
176. Diagnostic method of choice in contact
dermatitis
A. Tzanck test
B. Intradermal test
C. Skin biopsy
D. Patch test
177. Patch test read after
A. 12 hours
B. 24 hours
C. 48 hours
D. 72 hours
178. Tzanck cell is a
A. Keratinocyte
C. Neurtrophil

B. Lymphocyte
D. All of the above

179. Drug of choice for T.unguium


A. Amophotericin-B
B. Miconoazole
C. Griseofulvin
D. Nystatin

180. Test not used for diagnosis of syphilis


A. VDRL
B. Freis test
C. Reagin test
D. TPI
181. Most common precipitant of contact
dermatitis
A. Gold
B. Nickel
C. Copper
D. Silver
182. Mode of inheritance of ichthyosis vulgaris
A. Autosomal recessive
B. Autosomal dominant
C. X-linked dominant
D. X-linked recessive
183. Hyperpigmentation is not seen in
A. Addisons disease
B. Cushings disease
C. Hypothyroidism
D. Graves disease
184. Treatment of choice for nodulocystic
acne
A. Systemic steroids
B. Benzyl peroxide
C. Retinoids
D. Estrogens
185. Fordyce spots are
A. Ectopic sebaceous glands
B. Ectopic eccrine glands
C. Ectopic apocrine glands
D. Ectopic mucosal glands
186. Target lesions seen in
A. Urticaria
B. Lichen planus
C. Scabies
D. Erythema multiforme
187. Site not involved in psoriasis
A. Skin
B. CNS
C. Nail
D. Joints
188. Gonococcal infection does not affect
A. Testes
B. Ovary
C. Cervix
D. Fallopian tube

Multiple Choice Questions


189. Cicatrical alopecia seen in
A. Tinea capitis
B. Psoriasis
C. DLE
D. Alopecia areata
190. Erythema nodosum is seen in all except
A. Tuberculosis
B. Sulphonamide treatment
C. Sarcoidosis
D. Giant cell arteritis
191. Skin doubling time
A. 2 weeks
B. 4 weeks
C. 8 weeks
D. 12 weeks
192. Alopecia areata is due to
A. Androgenic stimulation
B. Estrogenic stimulation
C. Autoimmune
D. All of the above
193. Griseofulvin is used in all except
A. T. versicolor
B. T. Capitis
C. T. corporis
D. T. unguium
194. Palpable purpura is seen in all except
A. HS purpura
B. Vasculitis
C. ITP
D. Amyloidosis
195. Treatment of choice for exfoliative
dermatitis due to gold is
A. Steroids
B. Cephalosporins
C. Methotrexate D. Hemodialysis
196. Characteristic nail change in LP is
A. Pitting
B. Onycholysis
C. Pterygium
D. Subungual hyperkeratosis
197. Myrmecia warts are
A. Plantar warts B. Plane warts
C. Venereal warts D. Filiform warts
198. Fungi with no sexual stage
A. Basidiomyecetes
B. Phycomycetes
C. Ascomycetes
D. Fungi imperfecti

199. Rain drop pigmentation over the trunk is


seen in
A. Chronic lead poisoning
B. Chronic arsenic poisoning
C. Mercury poisoning
D. All of the above
200. Oil drop sign seen in
A.
B.
C.
D.

Psoriasis of nails
Lichen planus nails
Clubbing
Dermatophyte infection of nails

201. Dennie Morgan fold is seen in


A. Mastocytosis
B. Sarcoidosis
C. Atopic dermatitis
D. Seborrheic dermatitis
202. Black hairy tongue is due to intake of
A. Psoralen
B. Retinoids
C. Broad spectrum antibiotics
D. Steroid
203. All are seen in dermatomyositis except
A. Heliotrope rash
B. Gottrons sign
C. Gottrons papule
D. Sclerodactyly
204. Periungual telangiectasia seen in
A. Dermatomyositis
B. Scleroderma
C. SLE
D. All of the above
205. Drug induced lupus is seen with
antibody
A. Anti ds DNA B. Antihistone
C. Anti Sm
D. Anti ss DNA
206. Drug induced lupus occurs with all except
A. INH
B. Procainamide
C. Hydralazine
D. Aspirin

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Essentials in Dermatology
207. CREST syndrome contains all except
A. Calcinosis
B. Raynauds phenomenon
C. ECG changes
D. Sclerodactyly
208. Scl-70 is characteristic of
A. CREST syndrome
B. Diffuse systemic sclerosis
C. Diabetes mellitus
D. SLE
209. All are seen in mixed connective tissue
disease except
A. Sausage digits
B. Anti U1-RNP positive
C. Pulmonary involvement in common
D. Usually of infective origin
210. Bywaters lesion, a nail fold infarct, is seen
in
A. Rheumatoid arthritis
B. Psoriatic arthritis
C. Gonococcal arthritis
D. Osteoarthritis
211. Components of antiphospholipid
syndrome except
A. Recurrent abortions
B. Arterial and venous thrombosis
C. High VDRL
D. A-partial prothrombin time decrease
212. What is vagabonds disease
A. Pediculosis corporis
B. Pediculosis pubis
C. Scabies
D. Tinea cruris
213. Treatment of pediculosis includes
A. Permethrin
B. Cotrimoxazole
C. Physostigmine ointment
D. All of the above
214. Treatment of scabies includes all except
A. 5% Permethrin
B. 1% Lindane
C. 0.5% Malathion
D. 10% Sulphur

215. Nodular scabies is seen over


A. Palms
B. Scrotum
C. Trunk
D. Face
216. A 9-month infant presents to you with
complaints of itchy papules over the face,
and papulo-vesicles over palms and soles.
What is your diagnosis
A. Scabies
B. Pediculosis
C. Candidiasis
D. Acropustulosis
217. All are true about Norwegian scabies
except
A. Seen in Downs syndrome
B. Methotrexate is used as treatment for this
condition
C. The condition is often extremely pruritic
D. Patient should be isolated till cured
218. True about scabies incognito
A. It is a type of animal scabies
B. Classical burrows are seen in this
condition
C. Due to inappropriate use of topical
steroids
D. Does not need treatment
219. True about scabies
A. It is caused by the male sarcoptes mite
B. It is not an STD
C. Lindane is used in the treatment of
scabies in infants
D. Burrow is diagnostic of this condition
220. What is true about treatment of scabies
A. Ivermectin is used in outbreaks
B. Sulphur is contraindicated in pregnancy
C. Itching subsides immediately after
therapy
D. Lindane should be applied after hot bath
221. Wickhams striae represent a network of
fine white lines or puncta, are seen in many
papules of
A. Psoriasis
B. Pityriasis rosea

Multiple Choice Questions


C. Lichen planus
D. Pityriasis rubra pilaris
222. The herald patch is seen in 50 to 90 percent
of cases of
A. Pityriasis rosea
B. Pityriasis alba
C. Pityriasis rubra pilaris
D. Pityriasis lichenoides
223. A macule is a circumscribed, flat lesion that
differs from surrounding skin because of
its
A. Colour
B. Size
C. Shape
D. Texture
224. The Koebner reaction occurs in traumatized normal skin in
A. Vitiligo
B. Herpes zoster
C. Sporotrichosis
D. Linear scleroderma
225. Nikolskys sign refers to the sheet like
removal of epidermis by gentle traction that
can be observed in
A. Bullous pemphigoid
B. Dermatitis herpetiformis
C. Epidermolysis bullous acquisition
D. Pemphigus vulgaris
226. A rounded or flat-topped papule or plaque
that is characteristically evanescent,
disappearing within hours is known as
A. Nodule
B. Bulla
C. Cyst
D. Wheal
227. The appearance of pin point dots of blood
at the top of ruptured capillaries when scale
is forcibly removed from psoriatic plaques
refers to
A. Tzanck test
B. Bioscopy
C. Auspitzsign
D. Dariers sign

228. The classical triad of dermatitis, diarrhea


and dementia is seen in
A. Phrynoderma
B. Pellagra
C. Beri Beri
D. Scurvy
229. Probably the most frequent and the best
known nail deformity in psoriasis is
A. Thickening of nail
B. Pitting
C. Nail discoloration
D. Onycholysis
230. Exclamation mark hairs may be present
at the margin of smooth alopecia patch in
A. Tinea capitis
B. Androgenetic alopecia
C. Trichotillomania
D. Alopecia areata
231. Oral hairy leukoplakia is a lesion specific
to
A. Human papilloma virus infection
B. HIV disease
C. Varicella zoster virus infection
D. Cytomegalovirus infection
232. Most common form of oral candidiasis is
A. Chronic hyperplastic
B. Chronic atrophic
C. Acute atrophic
D. Acute pseudomembranous
233. Tinea capitis requires a systemic
antifungal to penetrate the hair follicle
such as
A. Potassium iodide
B. Griseofulvin
C. Amphotericin
D. Nystatin
234. The most common dermatophyte
producing favus is
A. T.verrucosum
B. T. violaceum

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372

Essentials in Dermatology
C. T. tonsurans
D. T. schoenleinii
235. Bullous impetigo is caused by
A. Staphylococcus aureus
B. Streptococcus pyogenes
C. Streptococcus epidermidis
D. Streptococcus haemolyticus
236. The mean incubation period of varicella is
A. 7 days
B. 14 days
C. 21 days
D. 28 days
237. A subcutaneous tuberculosis leading to
cold abscess formation and secondary
break down of the overlying skin is referred
to as
A. Lupus vulgaris
B. Scrofuloderma
C. Tuberculosis verrucosa cutis
D. Orificial tuberculosis

C. Seborrhoeic keratosis
D. Bowenoid keratosis
242. Vitiligo involving distal digits and
periorificial areas is named as
A. Vitiligo vulgaris
B. Acrofacial vitiligo
C. Focal vitiligo
D. Universal vitiligo
243. Acute vesicular eczema of hands and feet
is known as
A. House wife dermatitis
B. Nummular eczema
C. Atopic eczema
D. Pompholyx
244. Most common photodermatosis is
A. Polymorphic light eruption
B. Actinic prurigo
C. Hydroa vacciniformis
D. Chronic actinic dermatitis

238. Leprosy is a chronic granulomatous


infection primarily of
A. Skeletal muscles
B. Spinal cord
C. Skin and nerves
D. Central nervous system

245. Clue cells on wet mount are seen in


vaginal discharge of
A. Candidal vaginitis
B. Bacterial vaginosis
C. Trichomoniasis
D. Genital mycoplasma infection

239. Erythema Nodosum Leprosum occurs


most often in
A. Tuberculoid leprosy
B. Lepromatous leprosy
C. Borderline borderline leprosy
D. Borderline tuberculoid leprosy

246. Commonest form of cutaneous tuberculosis is


A. Lupus vulgaris
B. Scrofuloderma
C. Tuberculosis verrucosa cutis
D. Orificial tuberculosis

240. Syphilis is an infectious disease caused by


Treponema pallidum that is pathogenic to
A. Humans only
B. Humans and birds
C. Humans and amphibians
D. Terrestrial animals
241. Leser-Trelat sign basically consists of an
association of multiple internal malignancies with multiple eruptive
A. Actinic keratosis
B. Arsenical keratosis

247. Other than leprosy, dapsone is the drug of


choice in
A. Pemphigus vulgaris
B. Dermatitis herpetiformis
C. Bullous pemphigoid
D. Epidermolysis bullosa
248. Griseofulvin is the most frequently
systemically used drug in
A. Tinea versicolor
B. Dermatophytosis
C. Candidiasis
D. Tinea nigra

Multiple Choice Questions


249. Tinea versicolor lesions, under Woods light
demonstrate
A. Coral red fluorescence
B. Bright red fluorescence
C. Yellow fluorescence
D. Bluish green fluorescence
250. An antihelminthic agent given systemically found to be effective in scabies is
A. Mebendazole B. Thiabendazole
C. Albendazole
D. Ivermectin
251. KOH mount of skin scraping demonstrates
Spaghetti and meat balls in
A. Tinea versicolor
B. Tinea corporis
C. Tinea nigra
D. Tinea capitis
252. Tinea imbricata is caused by
A. Trichophyton rubrum
B. Trichophyton mentagrophytes
C. Trichophyton concentricum
D. Trichophyton verrucosum
253. Auspitzs sign occurs only in
A. Pemphigus
B. Psoriasis
C. Lichen planus
D. Lichen nitidus
254. Mucous patch is the best known and most
characteristic lesion of
A. Primary syphilis
B. Secondary syphilis
C. Latent syphilis
D. Tertiary syphilis
255. Herpes zoster is almost invariably
unilateral and most frequently affects
A. Cervical dermatome
B. Thoracic dermatome
C. Lumbar dermatome
D. Sacral dermatome
256. Oral hairy leukoplakia, a distinct
corrugated white plaque on one or both
sides of the tongue is an early clue to the
diagnosis of
A. HIV infection
B. Human papillomavirus infection

C. Herpes simplex infection


D. HTLV-I virus infection
257. Pellagra is due to deficiency of
A. Thiamine
B. Pyridoxine
C. Riboflavin
D. Niacin
258. Growth of the terminal hair in a male
pattern in a female is called as
A. Anagen effluvium
B. Telogen effluvium
C. Hypertrichosis
D. Hirsutism
259. On average, number of mites present at
the itching stage of scabies in an adult are
A. 6
B. 12
C. 24
D. 36
260. After first being infested, how many weeks
it takes for the hypersensitivity reaction to
scabies mite to occur and express it as
intense itching
A. 0-4 weeks
B. 4-6 weeks
C. 6-8 weeks
D. 8-12 weeks
261. Langerhans cells serves a primary role in
immune reactions of
A. Type I hypersensitivity
B. Type II hypersensitivity
C. Type III hypersensitivity
D. Type IV hypersensitivity
262. Heliotrope rash is characteristic of
A. Systemic lupus erythematosus
B. Scleroderma
C. Polymyositis
D. Dermatomyositis
263. Scrofuloderma is a form of
A. Syphilis
B. Sarcoidosis
C. Skin tuberculosis
D. Sporotrichosis
264. Finger nail growth is
A. 0.1 mm/day
B. 0.2 mm/day
C. 0.3 mm/day
D. 0.4 mm/day

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Essentials in Dermatology
265. The commonest type of basal cell
carcinoma is
A. Ulcerative
B. Pigmented
C. Morphoeic
D. Nodulo cystic/ Nodulo-ulcerative
266. Urticaria pigmentosa is characterized by
excessive accumulation in many tissues
A. Macrophages B. Fibroblasts
C. Mast cells
D. Langerhans cells
267. Increased sebum secretion and formation
of horny plugs in follicular lumens are the
earliest abnormalities in
A. Seborrheic dermatitis
B. Sebopsoriasis
C. Acne rosacea
D. Acne vulgaris
268. Wickhams striae on the flat topped
polygonal purple colored papule are
typical of
A. Lichen planus
B. Psoriasis
C. Pityriasis rubra pilaris
D. Pityriasis rosea
269. The nails are often affected and may show
the so called thimble pitting in
A. Lichen planus
B. Psoriasis
C. Pityriasis rubra pilaris
D. Pityriasis rosea
270. The definitive technique for diagnosing
allergic contact hypersensitivity is
A. Intradermal test
B. Patch test
C. RAST test
D. Lymphocyte transformation test
271. Raised level of serum IgE and its
relationship with severity of eczema is a
feature of
A. Nummular eczema
B. Asteatotic eczema

C. Atopic dermatitis
D. Seborrheic dermatitis
272. White dermographism is typically seen in
A. Angioedema
B. Urticaria
C. Contact dermatitis
D. Atopic dermatitis
273. Gluten enteropathy is a feature of the
following blistering disorder
A. Bullous pemphigoid
B. Epidermolysis bullosa
C. Pemphigus vulgaris
D. Dermatitis herpetiformis
274. Target like lesions are commonly
encountered in
A. Erythema annulare centrifugum
B. Erythema marginatum
C. Erythema nodosum
D. Erythema multiforme
275. Pediculosis capitis is recognized by louse
eggs stuck to hair called as
A. Hair casts
B. Hair beading
C. Piedra
D. Nits
276. The best sites on which to find scabies
burrows are
A. Interdigital areas of the fingers
B. Soles
C. Abdomen
D. Back
277. Herpes zoster caused by varicella zoster
virus is primarily due to
A. Primary infection of virus
B. Autoinoculation of virus
C. Reactivation of virus
D. Re-infection of virus
278. Common warts of hands and fingers are
also known as
A. Myrmecia warts
B. Plane warts
C. Mosaic warts
D. Verruca vulgaris

Multiple Choice Questions


279. Ritters disease (SSSS) is caused by
A. Pseudomonas aeruginosa
B. Bacillus anthracis
C. Streptococcus pyogenes
D. Staphylococcus aureus
280. Major skin disorder seen in AIDS patients
is
A. Dermatophytosis
B. Seborrheic dermatitis
C. Herpes simplex infection
D. Candidiasis
281. Hutchinsons triad consists of
Hutchinsons teeth, interstitial keratitis
and
A. Saddle nose
B. Cluttons joints
C. Sabre tibiae
D. VIII nerve deafness
282. Hardened deposits that result from drying
up of serum, blood or purulent exudate on
the skin surface is called
A. Scale
B. Crust
C. Calcinosis
D. Ossification
283. A group of autoimmune blistering diseases
of skin and mucous membrane from
intraepidermal blisters due to acantholysis
is
A. Pemphigus
B. Pemphigoid
C. Dermatitis herpetiformis
D. Epidermolysis bullosa

C. Miliaria pustulosa
D. Miliaria profunda
286. Tinea capitis caused by Microsporum
species, under Woods lamp demonstrates
A. Bluish green fluorescence
B. Yellow fluorescence
C. Coral red fluorescence
D. Pink fluorescence
287. Dendritic cells among keratinocytes of the
stratum spinosum of the epidermis,
having distinct folded nucleus and Birbeck
granules are
A. Langerhans cells
B. Melanocytes
C. Merkel cells
D. Fibroblasts
288. Deep seated vesicles resembling tapioca
on the sides of the fingers, palms and soles
characterize
A. Pompholyx
B. Nummular eczema
C. Atopic dermatitis
D. Xerotic eczema
289. Erythema nodosum leprosum occurs in
half of patients with
A. Indeterminate leprosy
B. Tuberculoid leprosy
C. Borderline tuberculoid leprosy
D. Borderline lepromatous or lepromatous
leprosy

284. Koenens periungual fibromas develop in


50% of cases of
A. Ataxia telangiectasia
B. Sturge Weber syndrome
C. Neurofibromatosis
D. Tuberous sclerosis

290. Safest as well as the most effective


medication for scabies is
A. Permethrin 5%
B. Precipitated sulphur 6-10%
C. Crotamiton 10%
D. Benzoyl benzoate 25%

285. A common condition resulting from


obstructed sweat, causing pruritic
inflammatory papules around the sweat
pores is known as
A. Miliaria crystalline
B. Miliaria rubra

291. Most effective sulfone in dermatitis


herpetiformis is
A. Dapsone
B. Sulfapyridine
C. Sulfasalazine
D. Sulphamethoxazole

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Essentials in Dermatology
292. Slit skin smear examination is used to
demonstrate organisms from the skin in
A. Pyoderma
B. Leishmaniasis
C. Donovanosis
D. Leprosy
293. A drug eruption which recurs at the same
site/s and heals with slate gray pigmentation is
A. Erythema multiforme
B. Stevens Johnson syndrome
C. Toxic epidermal necrolysis
D. Fixed drug eruption
294. A small solid elevated lesion smaller than
0.5 cm in diameter is called as
A. Macule
B. Papule
C. Vesicle
D. Nodule
295. If touching of the papule of sec