HLA class II alleles of susceptibility and protection in Brazilian and Dutch pemphigus foliaceus

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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