title: 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.
authors.affiliation: Department of Dermatology, University of North Carolina School of Medicine, Chapel Hill, North Carolina, United States of America.
authors.forename: G
authors.initials: G
authors.lastname: Flores
authors.forename: Y
authors.initials: Y
authors.lastname: Qian
authors.forename: L A
authors.initials: LA
authors.lastname: Díaz
mesh_terms: D015551:Autoimmunity; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D010392:Pemphigus / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G