Endemic pemphigus foliaceus over a century: Part I

BACKGROUND: Endemic pemphigus foliaceus (EPF) is the only known autoimmune disease presenting in circumscribed geographic areas. AIM: We aim to provide information concerning the natural course of EPF, including systemic compromise in the presteroid era, which has been largely unavailable in the current medical literature. MATERIAL & METHODS: By a retrospective review of the literature we aim to compile and compare the focus of EPF and the current knowledge about them. The main aim of this review is to summarize our current knowledge of EPF, including data described almost one century ago; and, to include several unindexed reports, which may have not been available to many current scientists and health care personnel. RESULTS: Foci of EPF have been described in several Central American and South American countries, affecting predominately young people and Amerindians, with an additional female predilection. Although most cases have occurred in Brazil, some cases have been reported in Peru, Paraguay, El Salvador, and Venezuela. Another variant of EPF has been described in El Bagre, Colombia, affecting older men and a few post-menopausal females. Finally, another type of EPF was described in nomadic tribes affecting females of child bearing age in Tunisia, Africa. CONCLUSION: Our understanding of EPF has been hampered by a lack of government attention to these diseases, especially in some South and Central American countries. Other factors that have made past studies of EPF difficult include 1) that the disease foci are often located in rural areas bordering the rain forest of underdeveloped countries; and 2) military conflicts in some of these areas.