Diseases of skin 2/ oral surgery courses

DISEASES
OF
THE SKIN
VARIANTS OF PEMPHIGUS
INDIAN DENTAL ACADEMY
LEADER IN CONTINUING DENTAL
EDUCATION
www.indiandentalacademy.com

LEARNING OBJECTIVES
At the end of the lecture student should beAt the end of the lecture student should be
able toable to describe thethe
 Clinical features, Oral manifestations,&
Histopathological features of Variants of Pemphigus

PEMPHIGUS VEGETANS
 An uncommon variant of Pemphigus vulgaris
 Occurs in 1 to 2% of Pemphigus vulgaris cases.
 Median age is 40-50yrs.
 Two clinical subtypes of pemphigus vegetans exists.
Flaccid bullae Pustules
& erosions

 Both subtypes develop into hyperpigmented
vegetative plaques with pustules &
hypertrophic granulation tissue at periphery.
 A characteristic feature of Pemphigus
vegetans is the Cerebriform tongue
characterized by pattern of sulci & gyri on
dorsum of tongue.

Vegetating lesions on
the buccal mucosa &
commissure.

ORAL MANIFESTATIONS
 Involves mucosa in 50-70% of patients. Intact
bullae are rare in mouth.
 Ill-defined, irregularly shaped, gingival,
buccal or palatine erosions which are painful
& slow to heal.
 Erosions extend peripherally with shedding
of epithelium. Erosions seen on any part of
oral cavity.

 Erosions may involve larynx with subsequent
hoarseness.
 Patient is unable to eat or drink adequately
because the lesions are so uncomfortable.
 Other mucosal surfaces may be involved,
including conjunctiva, esophagus, vagina,
cervix, penis, urethra & anus.

•An intraepithelial cleft
(Suprabasilar split) is
located just above the
basal cell layer.
•Rounded, acantholytic
epithelial cells sitting
within the intraepithelial
cleft. (Tzanck cells)Tzanck cells)
H
ISTO
PATH
O
LO
G
Y

Hyperchromatic Tzanck cellsHyperchromatic Tzanck cells

 Marked inflammatory
cell infiltration.
 Positive TzanckPositive Tzanck
test-test- Cytological test.

IMMUNOFLUORESCENCE
 Demonstrate presence of immunoglobulins,
predominantly IgG but sometimes in
combination with C3, IgA, IgM.
 Direct immunofluorescence – Frozen section of
patient’s tissue
 Indirect immunofluorescence – Patients serum

PARANEOPLASTIC PEMPHIGUS
 First described by Anhalt et al in 1990.
Diagnostic Criteria
1. Painful mucosal erosions, sometimes with a skin eruptions
that eventually resulting in blisters & erosions in the setting
of confirmed or occult malignancy
2. Acantholysis & keratinocyte necrosis

1. DIF typically reveals IgG & complement C3
within epidermal intercellular spaces as well as
at the epidermal basement membrane.
2. IDF reveals circulating antibodies specific for
stratified Squamous or transitional epithelium
is found.
3. Immunoprecipitation of a complex of proteins
with typical molecular weight.

 These are autoimmune response to intercellular adhesins
(Plakins).
 Mortality rate is 90 %
 Causes of death include –
1. Sepsis
2. Multiorgan failure
3. Respiratory failure

CLINICAL FEATURES
 Age - 60yrs (7-76yrs)
 M=F
 With little exception all patients had tumor (malignant)
(Non-Hodgkin Lymphomas 84 %)
 Conjunctival ulceration is a frequent features.
 Existence of neoplasm is recognized prior to
eruptions of lesions only in about 2/3rd
of cases

Other associated malignancies include –
• Chronic lymphocytic leukemia (18 %)
• Castleman Tumor (18 %)
• Giant Cell Lymphoma (Reticulum Cell Sarcoma)
• Waldenstrom Macroglobulinemia (1.2 %)
• Thymoma (5.5 %)
• Poorly differentiated Sarcoma, SCC of Oral cavity
• Bronchogenic SCC
• Follicular dendritic Cell Sarcoma
•

ORAL MANIFESTATIONS
 Painful oral lesions are accompanied by
generalized cutaneous eruption.
 Eruptions assumes form of morbilliform,
urticarial, bullous, papulosquamous or erythema
multiforme like lesions
 Pruritis, pain
 Erosions can occur on buccal, labial, lingual
mucosa & gingival.
 Erosions & crusting are similar to SJ syndrome
 Nose, pharynx, tonsils can be affected
 Nasal ulcers may cause epistaxis

Ocular involvement.
These diffuse oral ulcerations are
quite painful
Crusted, hemorrhagic lip Polymorphous cutaneous lesions.

HISTOPATHOLOGY
 Epidermal necrosis, Suprabasal acantholysis, dyskeratotic
keratinocytes, & lymphocytic infiltration.
 A distinctive feature is Dyskeratosis
Treatment
 Topical antibiotic ointment.

Paraneoplastic pemphigus. This low-power photomicrograph shows both
intraepithelial & subepithelial clefting.

PEMPHIGUS FOLIACEOUS
 Cazenave in 1844.
 It is an autoimmune skin disorder characterized by
superficial blisters.
 Positive Nikolsky sign.
 Little or no involvement of mucous membrane.

It includes 6 subtypes
1. P. erythematosus
2. P. herpetiformis
3. Endemic Pemphigus foliaceus
4. Immunoglobulin A (IgA) Pemphigus foliaceus
5. Paraneoplastic Pemphigus foliaceus
6. Drug induced Pemphigus foliaceus.
Formation of autoantibodies directed
against a cell adhesion molecule,
desmoglein1 expressed mainly in the
granular layer of the epidermis.

CLINICAL FEATURES
 Early bullous lesion which rapidly rupture & dry to leave
masses of flakes or scales suggestive of exfoliative
dermatitis or eczema.
 It is relatively mild form of Pemphigus
 More common in older adults.
 Site – Rare in oral cavity
lower extremity,abdomen

Brazillian pemphigus (Brazillian wildfire) –
 Founds in tropical region particularly in Brazil, Colombia,
Bolivia, Venezuela, Tunisia. occur in children & frequently in
family group.
 Oral lesions - Rare.
 Farmers, road constructers are affected
 Due to Arthopod borne
infective organism.
•Very severe scaling.
•Blisters are not apparent.

Pemphigus Erythematosus
 Eruptions of seborrheic areas that consists of ‘ Malar
Lesions’-
Butterfly eruptions of skin.
 scaly plaque to papule to vesicle.

Superficial crusting on
butterfly areas of face
Symmetrical
disrtibution
of red,
eroded,cruste
d lesion
Thin roof of vesicle leaves eroded &
crusted lesion
Localized erosion on the dorsum of
the tongue

Histopathology
Acantholysis
AcanthosisHyperkeratosis
Diskeratotic cells
Dermal lymphocytic
infiltrate
Subcorneal vesicle

SUMMARY
Introduction, Clinical features, Oral
manifestations,& Histopathological
features of Variants of Pemphigus

BIBLIOGRAPHY
 Text book of oral pathology Shafer's, 5 & 6th
edition
 Oral & Maxillofacial Pathology A Rationale for
Diagnosis & Treatment. R E Marx 1st
edition
 Color Atlas of Oral Diseases Cawson, R. 2nd
& 5th
edition
 Oral and Maxillofacial Pathology Neville, Brad
W. 2nd
 Lucas’s Pathology Of Tumor’s of the Oral Tissues
 Robbins Basic Pathology, Kumar V, A Fausto, 8th
edition
 Lever’s Histopathology of the skin, David Elder
8th
edition

Diseases of skin 2/ oral surgery courses

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