PEMPHIGUS

·         Damage to intercellular cementing substance by autoantibodies directed against cellular adhesion proteins ‘Desmogleins’ causing Acantholysis.

·          Immunofluorescence studies are ‘Gold Standard’ studies in diagnosis of autoimmune blistering disorders.

	PEMPHIGUS VULGARIS	PEMPHIGUS FOLIACEOUS
DIFFERENTIATING FEATURES
INCIDENCE	80% (most common)	15-20%
AUTOANTIBODIES	IgG antibodies against Desmoglein-3	IgG antibodies against Desmoglein-1
SEVERITY	More severe	Less severe
PROGNOSIS	Poor	Good
MUCOSAL LESIONS	Common (50-60%)	Uncommon
SKIN LESIONS	Flaccid bullae on normal/erythematous skin showing no tendency to heal spontaneously	Crusted, moist, scaly lesions Bullae are usually transient & heal spontaneously
HISTOPATHOLOGY	Supra-basal split in epidermis ‘Row of Tombstones’	Sub-corneal split
VARIANT	Pemphigus vegetans	Pemphigus erythematosus
COMMON FEATURES
NIKOLSKY SIGN	Positive
TZANC SMEAR	Acantholysis
IMMUNOFLUORESCENCE	Deposition of intercellular IgG with ‘Fish-net’ pattern throughout the epidermis

  

   ·         Treatment-

o   Systemic steroids- Prednisolone

o   Adjuvant therapy- dapsone, cyclosporine, methotrexate, gold salts, azathioprine & plasmapheresis