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The enigmatic autoimmune response in endemic pemphigus foliaceus.

Abstract
Endemic pemphigus foliaceus, known as Fogo Selvagem (FS) in Brazil, is a disease characterized by subcorneal blisters and IgG4 anti-dermoglein 1 (Dsg1) autoantibodies. Epidemiological studies of FS strongly an environmental etiology. A 15-year surveillance of the Limao Verde Amerindian reservation in Brazil has uncovered information on the transition of the autoimmune response from the pre-clinical stage to disease state. This incubation time may evolve over several years. The serological markers of the pre-clinical state of FS are IgM anti-Dsg1, IgE and non-IgG4 autoantibodies against Dsg1. The disease stage of FS is characterized by the rise of pathogenic IgG4 anti-Dsg1 autoantibodies. In this review, the authors reviewed the literature on the relevance of the humoral autoimmune response of FS as well as the possible environmental triggers of anti-Dsg1 autoantibody formation. Based on epidemiological observations, the authors hypothesize that the pathogenic IgG4 response in FS may be triggered by hematophagous insect bites.
AuthorsG Flores, Y Qian, L A Díaz
JournalActas dermo-sifiliograficas (Actas Dermosifiliogr) Vol. 100 Suppl 2 Pg. 40-8 (Dec 2009) ISSN: 0001-7310 [Print] Spain
PMID20096161 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Review)
Chemical References
  • Immunoglobulin G
Topics
  • Autoimmunity
  • Humans
  • Immunoglobulin G (immunology)
  • Pemphigus (immunology)

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