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Pemphigus

Oral Pathology
BDS
Pemphigus
A serious chronic skin disease
characterized by the
appearance of vesicles and
bullae, small or large fluid-filled
blisters that develop in cycles.
• Pemphigus – Greek word “Phemphix” meaning
bubble or blister.

• Three primary subsets :

-Pemphigus Vulgaris (70% cases)


-Pemphigus Foliaceus, (24% cases)
-Paraneoplastic pemphigus.

• Pemphigus Vegetans,1-2%(Variant of pemphigus vulgaris )


Pathogenesis Histopathology

Pemphigus
Vulgaris

Clinical Features,
Treatment
Oral manifestations
- An Autoimmune, intraepithelial, blistering disease
affecting skin and mucous membranes and is
mediated by circulating autoantibodies directed
against keratinocyte cell surfaces.
Pathogenesis:
• Blisters of pemphigus vulgaris is associated with the
binding of IgG autoantibodies to keratinocyte cell
surface molecules.
• Intercellular antibodies bind to keratinocyte
desmosomes and to desmosome free areas of
keratinocyte cell membrane.
• Binding of autoantibodies results in a loss of cell-cell
adhesion
• Pemphigus antibody binds to keratinocyte cell
surface molecules desmoglein 1 and desmoglein 3.
• Patients with active diseases have circulating and
tissue-bound autoantibodies of both Ig G1 and G4
subclasses.
• Pemphigus antibody fixes components of
complement to the surface of epidermal cells.
• Antibody binding may activate complement with
the release of inflammatory mediators and
recruitment of activated T-cells.
Clinical features
• All races affected, Equal gender distribution.
• Mean age of onset approx. 50-60yrs , children-rare
• Rapid appearance of vesicles and bullae with
varying diameter from mm to cm.
• Lesion - thin , watery fluid. May become purulent.
• Ruptured bullae leaves a raw eroded surface,
epithelium slide off either oblique pressure or
spontaneously w/o formation of vesicle or bulla.
• Characteristic feature: Nikolsky’s sign Loss of
epithelium occasioned by rubbing unaffected skin.
Clinical features
• Characteristic feature: Nikolsky’s sign Loss of
epithelium occasioned by rubbing unaffected skin.
Oral manifestations
• Oral lesions similar to skin lesions.
• Mucous membranes affected first. Mucosal lesions
may precede Cutaneous lesions by months.
• Lesions are commonly ill-defined, irregularly shaped,
gingival, buccal, palatine erosions which are painful
and slow to heal. Erosions may spread and involve
larynx.
• Patient is unable to eat or drink comfortably.
• Other mucosal surface involved – Conjunctiva,
esophagus, labia, vagina, cervix, penis, urethra
anus.
Histopathological Features
• Characteristic intraepithelial separation, which
occurs above the basal cell layer of epithelium.
• Formation of vesicle or bullae intraepithelially,
producing suprabasilar split.
• Superficial layers of epithelium are stripped away,
leaving only basal cells resembling a “row of
tombstones”.
Histopathological Features
• Acantholysis - Cells of spinous layer of surface
epithelium typically appear to fall apart.
• Presence of epithelial/rounded cells found within
vesicular spaces known as Tzanck cells
• Mild to moderate inflammatory chronic
inflammatory cell infiltrate is seen underlying
connective tissue.
• Diagnosis of pemphigus vulgaris should be
confirmed by Direct Immunofluorescence
examination of perilesional tissue.
Histopathology
Histopathology

Suprabasilary split
in the epithelum

Inflammatory cells
Differential Diagnosis

• Bullous Pemphigoid
• Erosive Lichen Planus
• Bullous erythema multiforme
Treatment and Prognosis
• Systemic corticosteroids (prednisone)

• Often combination with immunosuppressive drugs


(steroid sparing agents, azathioprine)

• Pemphigus vulgaris have the worst prognosis in


pemphigus group. Patients die in one year if not
treated.

• Mortality rate 25%-40%, mortality causes are the side


effects of high doses of Systemic corticosteroids.
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