Pemphigus foliaceus (PF) is a group of autoimmune skin blistering diseases 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 Cazenave first described by Cazenave is the classical form of PF. The elemental dermatological lesions are erythematous plaques with pastry puff squames predominantly affecting seborrheic 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 and immunosuppressive adjuvants. Rituximab and other anti-CD20 biologics targeting B lymphocytes have changed the prognosis of the disease from chronic relapsing to long-term remissions.
Endemic pemphigus foliaceus is a variant of PF that is endemic under peasants in rural Brazil, Indian tribes and gold miners in Colombia, and under women in Tunisia. The histopathology, immunology, and therapy are similar to PF Cazenave.
KeywordsDesmosome Immunoglobulin Autoimmune disease Vesiculobullous disease Pemphigus
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On the Web
- International Pemphigus & Pemphigoid Foundation http://www.pemphigus.org/.
- JAMA Dermatology Patient Page, Pemphigus http://archderm.jamanetwork.com/article.aspx?articleid=1879985.