Abstract
Pemphigus foliaceus (PF) is a worldwide chronic autoimmune bullous disease targeting desmoglein 1 (Dsg 1) on epithelial cell surface of skin and mucous membranes, causing lesions in skin but sparing mucosa, since the latter contain compensating desmoglein 31. Several environmental triggers for the autoimmune reaction have been imputed, such as drugs, ultraviolet radiation and mercurium. Human leucocyte antigens class II (HLA-DR4) alleles were linked to the disease in Ashkenazi Jews and other populations2. For endemic pemphigus foliaceus in Brazil, named fogo selvagem (FS), HLA-DRB1*04 was shown to have a relative risk >14 for the disease3.
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