Epidemiology | Autoantigens | Clinical features | Histopathology | Direct immunofluorescence | Indirect immunofluorescence | ELISA | Variants | |
Pemphigus vulgaris | Primarily middle-aged adults | Desmoglein 1 and desmoglein 3, others | Mucosal erosions; flaccid bullae and erosions on skin | Suprabasal acantholysis; "row of tombstones" pattern of basal keratinocytes | Intercellular deposition of IgG | Intercellular deposition of IgG; monkey esophagus is preferred substrate | Desmoglein 3 or both desmoglein 1 and desmoglein 3 autoantibodies | Pemphigus vegetans and pemphigus herpetiformis |
Pemphigus foliaceus | Primarily middle-aged adults; endemic form most common in children and young adults | Desmoglein 1 | Fragile blisters, shallow erosions, erythematous patches, crusts; mucosal involvement absent | Subcorneal or granular layer acantholysis | Intercellular deposition of IgG | Intercellular deposition of IgG; normal human skin or guinea pig esophagus is preferred substrate | Desmoglein 1 autoantibodies | Endemic pemphigus foliaceus (fogo sevalgem), pemphigus erythematosus, and pemphigus herpetiformis |
Paraneoplastic pemphigus | Primarily adults, but may occur at any age | Envoplakin, periplakin, others | Severe stomatitis, variable cutaneous findings (eg, blisters, erosions, lichenoid lesions), bronchiolitis obliterans | Variable findings; suprabasal acantholysis, keratinocyte necrosis, and lichenoid interface dermatitis are most common | Intercellular and/or basement membrane zone deposition of IgG and/or C3 | Intercellular deposition of IgG; rat bladder is preferred substrate | Envoplakin and periplakin autoantibodies | |
IgA pemphigus | ||||||||
Subcorneal pustular dermatosis-type IgA pemphigus | Any age | Desmocollin 1 | Vesicles, pustules, crusts on skin; annular, circinate, or herpetiform morphology; mucosal involvement usually absent | Subcorneal clefts and pustules; minimal acantholysis; mixed infiltrate in dermis | Intercellular deposition of IgA | Intercellular deposits of IgA on monkey esophagus* | Desmocollin 1 autoantibodies¶ | |
Intraepidermal neutrophilic dermatosis | Any age | Poorly defined | Vesicles, pustules, crusts on skin; annular, circinate, or herpetiform morphology; mucosal involvement usually absent | Intraepidermal pustules; minimal acantholysis; mixed infiltrate in dermis | Intercellular deposition of IgA | Intercellular deposits of IgA on monkey esophagus* | Desmoglein 1 and 3 autoantibodies have been reported in some patients |
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