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Mucocutaneus Diseases or
Disorders

Human Outer
Lining Tissues
Cutaneus

Genetik
disorders

Infective

Oral Mucous

Non
infective

Genetik
disorders

Muco-Cutaneus
Diseases or Disorders

Infective

Non
infective

Muco-cutaneus
disease or disorder

Geno
dermatosis

infective

Non infective

CLINICAL CLASSIFICATION OF ORAL SOFT TISSUE DISEASES


Regezi, et al. Oral Pathology 2003

ORAL MUCOSAL

MUCOSAL
SURFACE
LESIONS

Vesiculo-bulous Dis
Ulcerative Condition
White Lesions
Red-blue Lesions
Pigmentations of Oral
and Perioral
Verrucal-papillary
Lesions

SUB
MUCOSAL
SWELLING

Gingiva
Floor of Mouth
Lips and Buccal
Mucosal
Tongue
Palate
Neck

JAW LESIONS
DIFFERENTIAL
DIAGNOSIS
APPROACH TO

Cyst of the Oral Region


Odontogenic Tumors
Benign Non Odontogenic
Tumor
Inflamatory Jaw Lesions
Malignant Non-odont
Neoplasm
Metabolic and Genetic Jaw
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Diseases

CLINICAL CLASSIFICATION OF MUCOCUTANEUS DISEASES OR DISORDERS

ORAL MUCOCUTANEUS DISEASES

GENODERMA
TOSIS

White

Sponge Nevus

Darriers

Disease

Peutz-Jeghers
Dyskeratosis

Syndrome

Congenita

Hereditary

Benign
Interepithelial Dyskeratosis

Panchyionychia
Hyalinosis

Oris

Congenita

Cutis Et Mucosa

NON
INFECTIVE

INFECTIVE

Primary Herpetic Gingivo St


Secondary Herpetic Gingivo
Varicella (Chicken Pox)
Singles (Herpes Zoster)
Measles
Hand-foot-mouth Disease
Small Pox And Catscratch D
Infectious Mononucleosis
Herpangina
Acute Nodular Pharyngitis
Aids

A.

VESICULAR

B.

NON VESICULAR

C.

COLLAGEN DISORDER

D.

DEGENERATIVE AND
RELATIVE DISORDERS

E.

PIGMENTATION
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Genodermatosis : are inherited genetic skin


conditions often grouped into three categories:
chromosomal, single gene, and polygenetic.

A few genodermatoses

Epidermolysis Bullosa
Ichthyosis
Palmoplantar keratoderma
Neurofibromatosis
Xeroderma pigmentosum
Incontinentia pigmenti
Restrictive dermopathy
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Genodermatosis : are inherited genetic skin conditions


often grouped into three categories: chromosomal, single
gene, and polygenetic.

A few genodermatoses

Peutz-Jeghers syndrom
White sponge nevus
Darriers disease
Dyskeratosis congnita
Hereditary benign interepithelial dyskeratosis
Panchyonychia congenita
Hyalinosis cutis et mucosa oris
Pseudoxanthoma elasticum
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PeutzJeghers
syndrome

PeutzJeghers syndrome
Peutz-Jeghers

syndrome, also known as


hereditary intestinal polyposis syndrome
is an autosomal dominant genetic disease
characterized by the development of benign
hamartomatous polyps in the gastrointestinal
tract and hyperpigmented macules on the lips
and oral mucosa (melanosis).
PeutzJeghers syndrome has an incidence of
approximately 1 in 25,000 to 300,000 births.
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Diagnosis
The main criteria for clinical diagnosis are: Family history

Mucocutaneous lesions causing patches of


hyperpigmentation in the mouth and on the hands and feet.

The oral pigmentations are the first on the body to appear,


and thus play an important part in early diagnosis.
Intraorally, they are most frequently seen on the gingiva,
hard palate and inside of the cheek.

The mucosa of the lower lip is almost invariably involved as


well.

Hamartomatous polyps in the gastrointestinal tract are


benign polyps with an extraordinarily low potential for
malignancy.
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Diagnosis (cont...)

Having 2 of the 3 listed clinical criteria indicates a


positive diagnosis.

The oral findings are consistent with other conditions,


such as Addison's disease and
McCune-Albright syndrome, and these should be
included in the differential diagnosis.

90-100% of patients with a clinical diagnosis of PJS have


a mutation in the STK11/LKB1 gene. Molecular genetic
testing for this mutation is available clinically

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Peutz-Jegher syndrome

Pigmentation
on hand and
feet.

An important part in
early diagnosis.
Intraorally, they are
most frequently seen
on the gingiva, hard
The mucosa of the lower lip is
palate and inside of
almost invariably involved as well.
the cheek

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White sponge
nevus

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Skin manifestation White sponge nevus


Less commonly, sites outside the mouth are
affected, including the nasal, esophageal,
laryngeal, anal and genital mucosae.
It usually is present from birth, or develops
during childhood. Rarely, the lesions may
develop during adolescence.
Apart from the appearance of the affected areas,
there are usually no other signs or symptoms

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White sponge nevus

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Darier's disease

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Darier's disease

Darier's disease (DAR), also known as Darier disease,


DarierWhite disease,[Dyskeratosis follicularis and
Keratosis follicularis,:is an autosomal dominant disorder
discovered by French dermatologist
Ferdinand-Jean Darier.

Darier's is characterized by dark crusty patches on the


skin, sometimes containing pus. The crusty patches are
also known as keratotic papules, keratosis follicularis or
dyskeratosis follicularis.
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Hand-Foot and Mouth Disease HFM


Etiology and pathogenesis
HFM is an acute endemic viral infection
One of the subdivisions of the family of viruses known as
picornavirus is Coxackie group virus cause oral vesicular
eruptions: HFM disease and Herpangina
HFM is highly contagious caused by Coxackie type A5, A10,
A16.
Transmission through airborne spread or fecal-oral
contamination
Virus exhibits a predilection for mucous membranes of the
mouth and cutaneous regions of hands and feet.

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Hand-Foot and Mouth Disease

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Hand-Foot and Mouth Disease

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Hand-Foot and Mouth Disease


Clinical features
Affect children and younger than 5 years of age
Incubation: 1-2 weeks
Sign and symptom: mild to moderate in intensity and low
grade fever, malaise, lymhadenopathy and sore mouth
Oral vesicle quickly rupture to become ulcers covered by a
yellow fibrinous membrane surrounded erythematous halo.

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Hand-Foot and Mouth Disease


Clinical features
Oral lesion:
o

A few (5-20) small vesicles that soon rupture,


leaving slightly painful, shallow ulcers surrounded
by a red halo

Buccal mucosa, tongue and palate are most


frequently affected.

Skin

Small vesicles with a narrow red halo lateral and


dorsal surfaces f the fingers and toes , palms

soles and buttock are the sites of predilection


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Hand-Foot and Mouth Disease


Differential diagnosis
Aphthous ulcers
Herpetiform ulcers
Primary herpetic gingivostomatitis
Secondary herpes
Herpangina

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Varicella-Zoster Virus Infection


A cutaneous or mucosal vesiculoulcerative eruption
following reactivation of latent virus is also same typical.
Serious complications from chickenpox include bacterial
infections which can involve many sites of the body
including the skin, tissues

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Varicella - Zoster V
Primary disease ( varicella, chickenpox)
Self-limiting
Common in children, vesicular eruption of trunk and head and
neck occures in crops
Systemic signs symptoms , fever, malaise
Symptomatic treatment
Secondary disease (herpes zoster, shingles)
Self limiting
Adults
Rash, vesicles, ulcers unilateral along dermatome
Post herpetic pain can be severe
Immunocompromised and lymphoma patients, at risk
Treated with acyclovir
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Varicella (chickenpox)

Chickenpox which involve many sites of the body including the skin,
tissues and mucosa
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Varicella (chickenpox)
Pathogenesis
Transmission through the inhalation of contaminated
droplets.
Rapidly spread among child to child
Incubation in 2 weeks, virus proliferates within
macrophages
Viremia and dissemination to the skin and other organs
Systemic sign and symptom develop
Reside in a latent undetectable form in sensory ganglia

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Varicella (chickenpox)
Clinical features

Chills, malaise and headache may accompany rash that


involves primarily the trunk and head and neck

Rash vesicular eruption pustular and eventually


ulcerates

Crops of new lesion appear owing to repeated waves of


viremia

Self limiting and last several weeks.

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Varicella (chickenpox)
Clinical features

Oral mucous membranes my be


involved in primary disease and
usually demonstrate multiple
shalow ulcers that are preceded
by vesicles.

Healing on skin with scar


formation

Complication including
pneumonitis, encephalitis and
inflammation
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Measles, Rubeola
Etiology and Pathogenesis
Is a highly contagious viral infection cause by a member of
the paramyxovirus family
Related structurally and biologically to viruses of the
orthomyxovirus family which cause mumps and influenza.
The virus spread by airborne droplets through the respiratory
tract

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Measles, Rubeola

Kopliks spot : Pathognomonic


small erythematous macules
with blues white necrotic
centers appear in the buccal
mucosa opposite molar
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Measles, Rubeola
Clinical features
Is predominantly a disease of children, fever, malaise,
conjunctivitis, photophobia and cough develop
Pathognomonic small erythematous macules with
white necrotic centers appear in the buccal mucosa
the Kopliks spot
Complication is encephalitis and thromocytopenic
pupura. pneumonia.
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Target cell

An abnormal red blood cell characterized by a densely


stained center surrounded by a pale, unstained ring circled
by a dark, irregular band.

Target cells occur in the blood after splenectomy, in anemia,


in hemoglobin disease, and in thalassemia.

Also called leptocyte, codocytes, or Mexican hat cells, the


appearance of a bullseye.

In optical microscopy these cells appear to have a dark


center (a central, hemoglobinized area) surrounded by a
white ring (an area of relative pallor), followed by dark outer
(peripheral) second ring containing a band of hemoglobin.
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StevensJohnson syndrome,

Is a form of toxic epidermal necrolysis ,


(TEN) a life-threatening skin condition ,
in which cell death causes the
epidermis to separate from the dermis.

The syndrome is thought to be a


hypersensitivity complex that affects
the skin and the mucous membranes .

The best known causes are certain


medications, (lamotigrine) , but it can
also be due to infections, or more
rarely, cancers
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Other agents have been used, including

Cyclophosphamide and cyclosporin, but none has


exhibited much therapeutic success.

Intravenous immunoglobulin treatment has shown some


promise in reducing the length of the reaction and
improving symptoms.

Other common supportive measures include the use of


topical pain anaesthetics and antiseptics, maintaining a
warm environment, and intravenous analgesics.

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Pemphigus

Is a rare group of blistering autoimmune


diseases that affect the skin and mucous
membranes.

In pemphigus, autoantibodies form against


desmoglein. Desmoglein forms the "glue" that
attaches adjacent epidermal cells via attachment
points called desmosomes.

When autoantibodies attack desmogleins, the


cells become separated from each other and the
epidermis becomes "unglued", a phenomenon
called acantholysis.

Originally, the cause of this disease was


unknown, and "pemphigus" was used to refer to
any blistering disease of the skin and mucosa.
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Cutaneus pemphigus

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Oral manifestation of pemphigus

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Tzanck cells

(tsahnk), acantholytic epithelial cells seen in the Tzanck


test.
Acantholysis : disruption of the intercellular connections
between keratinocytes of the epidermis, caused by lysis
of intercellular cement substance.
Clumps of large hyperchromatic epithelial cells are seen
lying free within the vesicular space formed as a result of
acantholysis in the spinous layer of the epithelium.
These are seen in Herpes Zooster, Varicella, Herpes
Simplex virus, Pemphigus vulgaris, Cytomegalo virus.

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Cicatricial pemphigoid

It has been referred to by a variety of


designations based largely on its site of
involvements, with examples of such
terminology including "desquamative gingivitis,"
"ocular pemphigus," and "benign mucous
membrane pemphigoid."

(such designations are thought to be confusing or somewhat


misleadings)

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Signs and symptoms

The autoimmune reaction most commonly affects the


mouth, causing lesions in the gingiva or gums, but it can
also affect areas of mucous membrane elsewhere in the
body, such as the sinuses, genitals and anus.

When the cornea of the eye is affected, repeated


scarring may result in blindness.

Localized cicatricial pemphigoid (also known as


BrunstingPerry cicatricial pemphigoid. refers to a
localised variant of cutaneous cicatricial pemphigoid
involving the head and the neck without mucosal
involvement.
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Signs and symptoms

Nikolsky's sign (gentle lateral pressure) on unaffected


mucosa or skin raises a bulla. If no lesions are present
on examination it may be useful way of demonstrating
reduced epithelial adhesion.

In contrast, in Pemphigus, the epithelium tends to


disintegrate rather than form a bulla.

Nikolsky's sign is present in case of Pemphigus and


Cicatricial Pemphigoid, but not in the case of Bullous
Pemphigoid.

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Epidermolysis bullosa

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LICHEN PLANUS
Lichen

planus is a skin or mucous rash that is


triggered by the immune system.
Cell mediated immune response of unknown
origin
Skin lesions
Purple, pruritic, polygonal papules
Resolve in 6-18 mo
Associated with hepatitis C and other diseases
of impaired immunity

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LICHEN PLANUS
Additional

Sites
Mucous membranes
Nails
Scalp
Genitalia
Wickham striae

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OLP

may occur in up to 70-77% of patients with


cutaneous LP. Numbers range from 12-77%

OLP

occurs in 1-2 % of worlds population

OLP

makes up 9% of white lesions affecting the


oral cavity

2:1

Female: Male

Exact

mechanism unknown

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Exact mechanism unknown

T-cell mediated

Cytotoxic or CD8 T-cells


Against basilar keratinocytes, inducing apoptosis
Hypothesized to be interaction between
keratinocytes with endogenous or exogenous agent
as an inciting event i.e. virus, allergen, drug
Degranulation of mucosal mast cells and release of
TNF-

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Bilateral

Symmetrical
Multifocal
Most common sites
Buccal mucosa
Tongue
Gingiva

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Clinical

Presentation

Asymptomatic
Mucosal sensitivity, burning and pain
Relapsing and remitting periods
Inciting events
Xerostomia
Gingival recession

Reticular
Plaquelike
Erosive
Papular
Atrophic
Bullous
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Clinical

Subtypes
Reticular

Plaquelike
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Erosive

Desquamative

Gingivitis

Atrophic

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Desquamative

gingivits and atrophic gum


disease lead to inability to maintain good dental
hygiene

Gingiva tender and friable

Worsens underlying disease process

Oral

Lichenoid Reactions

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Oral Lichenoid Reactions

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Treatment Algorithm

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Treatments
Symptom management
Malignant transformation
Considered premalignant
Controversial
Risks

Dorsum of tongue
Atrophic and erosive lesions
3-6 years post diagnosis

OLP with dysplasia

Should be examined every 2-3 months


more likely for malignant changes
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Definitions: civatte bodies


eosinophilic hyaline spheric bodies seen in the epidermis, in
lichen planus and other skin disorders; formed by apoptosis
of individual basal cells.Synonyms: colloid bodiesbodies

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References
Nikolaos G Nikitakis. 2003 : Oral soft tissue lesion : A
Guide to differential dioagnosis
Scully & Felix DH, 2005 : Update for the dental
practitioner. British Dental Journal Vol 199 & 200.
Regezi , J.A. , Sciubba, J.J. and Jordan, R.C.K., 2003.
Oral pathology, clinical pathologic correlations
Other additional refferences

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Lorum Ipsum

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