Pemphigus vulgaris

Alternative names:

pemphigus

Definition:

An autoimmune skin disorder characterized by blistering of the skin and mucous membrane.

Causes, incidence, and risk factors:

Pemphigus involves blistering of the outer (epidermal) layer of the skin and mucous membrane. It is an autoimmune disorder where the immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies produce a reaction that leads to a separation of epidermal cells (acantholysis). The exact cause of the development of antibodies against the body's own tissues (autoantibodies) is unknown. A few cases have occurred from reactions to medications, including penicillamine and captopril. More than 95% of people with pemphigus have specific HLA antigens.

Pemphigus is uncommon. It occurs almost exclusively in middle-aged or older people, of all races and ethnic groups. About one-half of the cases of pemphigus vulgaris begin with blisters in the mouth, followed by skin blisters. The blisters (bullae) are relatively asymptomatic, but the lesions become widespread and complications develop rapidly and may be debilitating or fatal.