Pathogenesis of endemic pemphigus foliaceus

Dermatol Clin. 2011 Jul;29(3):413-8, viii. doi: 10.1016/j.det.2011.03.014.

Abstract

Pemphigus refers to a group of human autoimmune blistering diseases involving skin and/or mucous membranes. Endemic pemphigus foliaceus (EPF), or fogo selvagem is an organ-specific autoimmune blistering disease, first reported in the beginning of the 20th century in rural areas of Brazil. The disease follows the course of streams and creeks, and vanishes after urbanization of the endemic areas. The auto-antigen related to EPF is desmoglein 1, a 160 kDa glycoprotein of the desmossomal core, targeted by in situ and circulating IgG autoantibodies, mainly of the IgG4 subclass.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Endemic Diseases / statistics & numerical data*
  • Humans
  • Pemphigus* / epidemiology
  • Pemphigus* / etiology
  • Pemphigus* / pathology
  • Skin / pathology*