Pemphigus and Bullous Pemphigoid

Pemphigus and bullous pemphigoid are organ-specific autoimmune disorders with an established immunological basis but unknown etiology. Pemphigus has three variants categorized by the presence/absence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous membranes. The diagnosis of pemphigus and bullous pemphigoid is based on the clinical picture and confirmed by distinct immunopathological findings. In general, the natural history of pemphigus is characterized by constant progression with a high mortality risk; the prognosis of bullous pemphigoid is more favorable. There are no prognostic indexes for the different variations of the diseases. Treatment consists of systemic corticosteroids, corticosteroid-sparing agents, and specific immunobiological agents. Bullous pemphigoid tends to be more responsive to treatment and may also respond to topical as well as anti-inflammatory agents.