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Fogo selvagem (FS) is an autoimmune blistering disease involving antibodies directed against desmoglein-1 (Dsg1) proteins present in the epidermis. It has clinical, histologic, and immunopathologic features identical to pemphigus foliaceus. FS has epidemiologic peculiarities: It is endemic in South America, especially in selected river valleys of rural Brazil, and has been associated with insect bites from sand flies, kissing bugs, and black flies, all of which are found in endemic regions. Exactly how these epidemiologic features relate to the immunopathologic findings has been obscure.
Investigators took sera containing anti-Dsg1 antibodies from 45 FS patients and two monoclonal anti-Dsg1 antibodies derived from FS patients. Both the sera …