Endemic pemphigus foliaceus (Fogo Selvagem)—1998

Abstract

The clinical, histological and immunological features of the endemic form of pemphigus foliaceus (PF), also known as fogo selvagem (FS), are similar to those patients suffering the nonendemic form of PF seen in North America, Europe, and other parts of the world as originally described by Cazenave.1–3 FS is an autoimmune disease that has remarkable features. The disease is characterized by superficial subcorneal blisters and pathogenic antiepidermal autoantibodies that recognize the desmosomal core glycoprotein known as desmoglein 1 (dsg1).4 In addition, FS possesses unique epidemiological features, such as a high prevalence of the disease in certain regions of Brazil, an increased frequency of familial cases, and an increased frequency of cases among young adults and children. Commonly, FS patients are poor farmers who live and work in certain rural areas of Brazil where the disease is endemic. Recently, other endemic foci of PF have been reported in other South American countries such as Columbia and Tunisia.5–8 Several etiological agents are thought to precipitate FS; however, there is no convincing experimental evidence supporting any of these hypotheses. The disease is treated with systemic steroids, and in certain cases, with immunosuppressive agents. The prognosis of FS has improved with the use of these new therapies.