Pemphigus foliaceus

Pemphigus foliaceus (PF) is a group of autoimmune skin blistering disease comprising four subtypes: Cazenave, endemic, erythematosus and herpetiformis. The common characteristic is the frequent presence of pathogenic anti-desmoglein 1 antibodies leading to bullae in the skin. Other characteristics-clinical, histological, pathogenesis and therapy-vary and are specified in this chapter. Pemphigus foliaceus first described by Cazenave is the classical form of PF. The elemental dermatological lesions are erythematous plaques with pastry puff squames predominantly affecting seborrhoeic areas. The histopathological hallmark is acantholysis in the upper part of the epidermis (subcorneal or intraspinous). The disease is caused by autoantibodies to desmoglein 1 on the epithelial cell surface. Therapy is based on systemic corticosteroids, rituximab and immunosuppressive adjuvants. Rituximab an anti-CD20 biologics targeting B-lymphocytes have changed the prognosis of the disease from chronic relapsing to long-term remissions. Endemic pemphigus is a variant of PF. The disease is endemic in South-American countries especially in rural regions of Brazil, but also in Colombia, Paraguay, Peru, Venezuela and also in Africa in Tunisia. The histopathology, immunology and therapy are similar to classical form of PF.