History:

Pemphigus vegetans is a variant of pemphigus vulgaris that occurs in 1-2% of pemphigus vulgaris cases. The median age of onset is 40-50 years. Two clinical subtypes of pemphigus vegetans have been described; Neuman subtype is characterized initially by flaccid bullae and erosions and Hallopeau subtype is typified by the presence of pustules. Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery. The lesions of pemphigus vegetans are typically found in the intertriginous areas and on the oral mucosa. Cerebriform tongue, characterized by a pattern of sulci and gyri on the dorsum of the tongue is another characteristic feature.

Deposition of IgG and C3 on the cell surface of keratinocytes is demonstrated by direct immunoflourescence while indirect immunofluorescence reveals circulating antiepithelial cell surface IgG.