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Anais Brasileiros de Dermatologia
On-line version ISSN 1806-4841
AOKI, Valéria et al. Historical profile of the immunopathogenesis of endemic Pemphigus Foliaceus (Fogo selvagem). An. Bras. Dermatol. [online]. 2005, vol.80, n.3, pp. 287-292. ISSN 1806-4841. http://dx.doi.org/10.1590/S0365-05962005000300010.
Fogo selvagem, or endemic pemphigus foliaceus, is an organ-specific disease, where IgG autoantibodies, especially from the IgG4 subclass are directed against the ectodomains of desmoglein 1, culminating in acantholysis. Genetic and environmental influences modulate this specific autoimmune response; individuals with a determined sequence of alleles (LLEQRRAA) in the positions 67-74 of the third hypervariable region of the HLA-DRB1, or those exposed to hematophagous insects, especially Simulium nigrimanum, are more susceptible to develop the disease. A possible explanation for the development of the autoimmune process would be antigenic mimicry, initiated by environmental stimuli in those genetically predisposed individuals.
Keywords : Autoimmunity; Fluorescent antibody technique; Epitope Mapping; Pemphigus.