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

Simon Warren, Mong Shang Lin, George J. Giudice, Raymond G. Hoffmann, Gunter Hans-Filho, Valeria Aoki, Evandro A. Rivitti, Vandir Dos Santos, Luis A. Diaz

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Abstract

Background: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence 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 pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected 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 samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. Conclusions: The prevalence of antibodies against desmoglein 1 is high among 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 findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent. (C) 2000, Massachusetts Medical Society.

Original languageEnglish (US)
Pages (from-to)23-30
Number of pages8
JournalNew England Journal of Medicine
Volume343
Issue number1
DOIs
StatePublished - Jul 4 2000
Externally publishedYes

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Desmoglein 1
Brazil
Antibodies
Pemphigus
Antibody Formation
Japan
Serum
Endemic Diseases
Medical Societies
Pemphigus and fogo selvagem
Skin Diseases
Autoantibodies
Autoimmune Diseases
Enzyme-Linked Immunosorbent Assay

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Warren, S., Lin, M. S., Giudice, G. J., Hoffmann, R. G., Hans-Filho, G., Aoki, V., ... Diaz, L. A. (2000). The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. New England Journal of Medicine, 343(1), 23-30. https://doi.org/10.1056/NEJM200007063430104

The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. / Warren, Simon; Lin, Mong Shang; Giudice, George J.; Hoffmann, Raymond G.; Hans-Filho, Gunter; Aoki, Valeria; Rivitti, Evandro A.; Dos Santos, Vandir; Diaz, Luis A.

In: New England Journal of Medicine, Vol. 343, No. 1, 04.07.2000, p. 23-30.

Research output: Contribution to journalArticle

Warren, S, Lin, MS, Giudice, GJ, Hoffmann, RG, Hans-Filho, G, Aoki, V, Rivitti, EA, Dos Santos, V & Diaz, LA 2000, 'The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil', New England Journal of Medicine, vol. 343, no. 1, pp. 23-30. https://doi.org/10.1056/NEJM200007063430104
Warren, Simon ; Lin, Mong Shang ; Giudice, George J. ; Hoffmann, Raymond G. ; Hans-Filho, Gunter ; Aoki, Valeria ; Rivitti, Evandro A. ; Dos Santos, Vandir ; Diaz, Luis A. / The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. In: New England Journal of Medicine. 2000 ; Vol. 343, No. 1. pp. 23-30.
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abstract = "Background: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence 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 pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected 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 samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. Conclusions: The prevalence of antibodies against desmoglein 1 is high among 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 findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent. (C) 2000, Massachusetts Medical Society.",
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T1 - The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil

AU - Warren, Simon

AU - Lin, Mong Shang

AU - Giudice, George J.

AU - Hoffmann, Raymond G.

AU - Hans-Filho, Gunter

AU - Aoki, Valeria

AU - Rivitti, Evandro A.

AU - Dos Santos, Vandir

AU - Diaz, Luis A.

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Y1 - 2000/7/4

N2 - Background: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence 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 pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected 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 samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. Conclusions: The prevalence of antibodies against desmoglein 1 is high among 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 findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent. (C) 2000, Massachusetts Medical Society.

AB - Background: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence 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 pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected 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 samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. Conclusions: The prevalence of antibodies against desmoglein 1 is high among 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 findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent. (C) 2000, Massachusetts Medical Society.

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