The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil.

Background: Pemphigus foliaceus is an autoimmune skin disease mediated by a utoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso d o Sul, Brazil, is a recently identified focus of the disease, with a preval ence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. Methods: We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic pemphig us foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazi l, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and s urrounding locations, and 126 normal subjects from the United States and Ja pan. Results: Antibodies against desmoglein 1 were detected in 59 of the 60 pati ents with fogo selvagem (98 percent) but in only 3 of the 126 normal subjec ts from the United States and Japan (2 percent). Antibodies were also detec ted in 51 of the 93 normal subjects from Limao Verde (55 percent) and in 54 of the 279 normal subjects from surrounding areas (19 percent). Serum samp les obtained one to four years before the onset of disease were available f or five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody val ues. Conclusions: The prevalence of antibodies against desmoglein 1 is high amon g normal subjects living in an area where fogo selvagem is endemic, and the onset of the disease is preceded by a sustained antibody response. These f indings support the concept that the production of antibodies against desmo glein 1 is initiated by exposure to an unknown environmental agent. (N Engl J Med 2000;343:23-30.) (C) 2000, Massachusetts Medical Society.