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Vol. 100. Núm. S2.
Mirando hacia el futuro en Dermatología Extraordinario del Centenario. Parte II
Páginas 40-48 (diciembre 2009)
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Vol. 100. Núm. S2.
Mirando hacia el futuro en Dermatología Extraordinario del Centenario. Parte II
Páginas 40-48 (diciembre 2009)
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The Enigmatic Autoimmune Response in Endemic Pemphigus Foliaceus
La enigmática respuesta autoinmunitaria en el pénfigo foliáceo endémico
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G. Flores, Y. Qian, L.A. Díaz
Autor para correspondencia
ldiaz@med.unc.edu

Correspondence: Department of Dermatology. University of North Carolina. Chapel Hill 3100. Thurston Building, CB#7287 Chapel Hill, NC 27599.
Department of Dermatology. University of North Carolina School of Medicine. Chapel Hill. North Carolina. United States of America
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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.

Key words:
fogo selvagem
desmoglein
pemphigus foliaceus
pemphigus vulgaris
ELISA
Resumen

El pénfigo foliáceo endémico, conocido como fogo selvagem (FS) en Brasil, es una enfermedad caracterizada por ampollas subcórneas y autoanticuerpos IgG4 frente a la desmogleína 1 (Dsg1).

Los estudios epidemiológicos sobre el FS apoyan de manera sólida una etiología ambiental. Un seguimiento durante 15 años de la reserva amerindia de Limao Verde ha revelado información sobre la transición de la respuesta autoinmunitaria desde el estadio preclínico hasta la fase clínica florida. Este periodo de incubación puede desarrollarse a lo largo de varios años. Los marcadores serológicos del estadio preclínico del FS son autoan ticuerpos IgM, IgE y no IgG4 frente a la Dsg1. La fase florida del FS se caracteriza por el aumento de los autoanticuerpos patogénicos IgG4 frente a la Dsg1.

En este artículo los autores revisan la literatura sobre la relevancia de la respuesta humoral autoinmunitaria del FS, así como los posibles factores ambientales desencadenantes para la formación de autoanticuerpos frente a la Dgs-1. Los autores sugieren, basándose en los estudios epidemiológicos, que la respuesta patogénica mediada por IgG4 en el FS puede estar provocada por las picaduras de insectos hematófagos.

Palabras clave:
fogo selvagem
desmogleína
pénfigo foliáceo
pénfigo vulgar
ELISA
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