Flores G, Qian Y, Díaz L A
Department of Dermatology, University of North Carolina School of Medicine, Chapel Hill, North Carolina, United States of America.
Actas Dermosifiliogr. 2009 Dec;100 Suppl 2(Suppl 2):40-8. doi: 10.1016/s0001-7310(09)73377-0.
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.
地方性落叶型天疱疮在巴西被称为丛林火疱病（FS），是一种以角膜下水疱和IgG4抗桥粒芯糖蛋白1（Dsg1）自身抗体为特征的疾病。FS的流行病学研究强烈表明其病因与环境有关。对巴西利毛奥韦尔德美洲印第安人保留地进行的15年监测揭示了自身免疫反应从临床前期到疾病状态转变的相关信息。这种潜伏期可能会持续数年。FS临床前期的血清学标志物是IgM抗Dsg1、IgE以及针对Dsg1的非IgG4自身抗体。FS的疾病阶段以致病性IgG4抗Dsg1自身抗体的升高为特征。在这篇综述中，作者回顾了关于FS体液自身免疫反应的相关性以及抗Dsg1自身抗体形成可能的环境触发因素的文献。基于流行病学观察，作者推测FS中的致病性IgG4反应可能由吸血昆虫叮咬触发。
