Pemphigus, incidence, Etiology, classification, in detail

1. PEMPHIGUS
P CHANIKYA

2. DEFINATION
Pemphigus(Gk. pemphix = blister or bubble)
It is the term for a group of chronic autoimmune bullous dermatoses that are
characterized histologically by intraepidermal blister formation and
immunopathologically by the presence of bound and circulating autoantibodies
directed against the intercellular adhesion structures of the epithelial cells.

3. INCIDENCE
Pemphigus is an uncommon disease, varying in incidence from 0.5
to 3.2 cases per 100,000 population per year.
The disorder has been reported to occur from the age of 3 to 89
years

4. ETIOLOGY
• Idiopathic autoimmune phenomenon: presence of IgG autoantibodies
against desmogleins (Dsg) The antibodies bind to the Dsgs in the epidermis
and cause loss of cohesion of epidermal cells (acantholysis).
• Neoplasia-induced autoimmune phenomenon Thymoma and lymphoma.
• Drug-induced autoimmune phenomenon: Penicilla mine, rifampicin and
captopril.

5. CLASSIFICATION
Pemphigus is classified (based on level of split)
1. Pemphigus vulgaris
2. Pemphigus vegetans
3. Pemphigus foliaceus
4. Pemphigus erythematosus
5. Pemphigus variants, Including IgA pemphigus
6. paraneoplastic pemphigus

6. PEMPHIGUS VULGARIS
Characterized by cutaneous and
mucosal blisters.
Skin Lesions
Morphology of lesions: Flaccid bullae ,
rupture to form painful erosions,
spreads and take very long to re-
epithelialize.
Bedside Test : Nikolsky sign, Bulla
spread sign or Asboe Hansen sign

7. Mucosal lesions
50% of patients, Begins in oral mucosa
Sometimes sole manifestation of PV
Other mucosae like genital & eye.
Site of predilection - scalp, face,
flexures (axillae, Groin), trunk. Periungual
lesion, oral lesions
Complication – secondary infection
(Skin bacteria, candida in mucosa),
sepsis frequent. Water and electrolyte
Imbalance.

8. PEMPHIGUS FOLIACEUS
• Morphology:Transient superficial bullae,rupture rapidly
(so only rarely visible) to form areas of scaling and crusting .
Removal of scale-crust does not reveal an erosion, but only
a minimally moist skin.
• Sites of predilection: Initial seborrheic distribution (face
and trunk).
• Mucosal lesions: Infrequent (rare).
• Brazilian pemphigus: Also called fogo selvagen Variant of
PF (clinically,histologically and immunologically ).

9. PEMPHIGUS VEGETANS
Variant of PV (so bulla is suprabasal)
• Morphology: Characterized by
presence of heaped up , vegetating
lesions which extend centrifugally.
• Sites of predilection: Groins, axillae,
angles of mouth.
• Mucosal lesions: May be present.

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