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Update on fogo selvagem, an endemic form of pemphigus foliaceus
Author(s) -
Aoki Valeria,
Rivitti Evandro A.,
Diaz Luis A.
Publication year - 2015
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.12675
Subject(s) - pemphigus foliaceus , autoantibody , desmoglein 1 , pemphigus , immunology , biology , autoimmune disease , epitope , antibody , pathogenesis , molecular mimicry , antigen
Pemphigus are organ‐specific autoimmune diseases, where autoantibodies (mainly immunoglobulin [ I g] G ) directed against epidermal targets (glycoproteins of the desmosomal core) are detected. Endemic pemphigus foliaceus or fogo selvagem ( FS ) is one of the variants of pemphigus foliaceus pemphigus foliaceus that shares the same clinical and immunopathological features of the classic non‐endemic pemphigus foliaceus form, including pathogenic I g G (mainly I g G 4) autoantibodies directed against the ectodomain of desmoglein 1 ( D sg1), that lead to acantholysis. Pathogenesis of FS is complex, involving genetic, environmental and immunological factors. Human leukocyte antigen ( HLA)‐DRB 1 alleles DRB 1*0404, *1402, *1406 or *0102 have been previously identified as risk factors for FS (relative risk, >14). Individuals exposed to hematophagous insects are more susceptible to develop the disease. Non‐pathogenic anti‐ D sg1 antibodies of the I g G 1 subclass, directed against the extracellular 5 domain of D sg1, are detected in patients in the preclinical stage of the disease, and also in healthy controls living in endemic areas. In counterpart, patients with FS show pathogenic anti‐ D sg1 I g G 4 autoantibodies that bind the pathogenic extracellular 1 and 2 domains of D sg1, emphasizing the intramolecular epitope‐spreading hypothesis. A possible explanation for the development of the autoimmune process would be antigenic mimicry, initiated by environmental stimuli in those genetically predisposed individuals. Characterization of the pathogenesis of FS will allow the development of specific therapeutic targets, and the elucidation of other autoimmune processes.