029 DRB1*0804 is associated with PV in patients of African descent

Pemphigus Vulgaris (PV) has one of the strongest known HLA disease associations. The majority PV patients studied carry either DRB1*0402 or DQB1*0503. However, studies on associations in have largely focused populations with specific ethnic backgrounds, often Ashkenazi Jewish (AJ) and non-Jewish Caucasians there are limited African descent. We investigated status 11 American (AA) comparison to 282 non-African descent identify HLA-associations potential relevance this understudied population. find that 64% our AA class II allele DRB1*0804. These had highest levels anti-desmoglein (Dsg) 3 antibodies compared other alleles, but unlike associated HLAs, no skewing towards mucosal only, muco-cutaneous cutaneous only lesions. DRB1*0804 is believed be origin, most frequent Northwestern Africa near modern Burkina Faso. This previously been reported association from Egypt Brazil, however, a not established. Given similar phenotype we see DRB1*0804-carrying population main carrying at DNA protein level. For allele, it shown P4 pocket plays critical role MHC-linked susceptibility PV. Interestingly, 92.9% residues overlap those DRB1*0402, crucial pocket, DRB 70 71, identical. comparison, non-relevant such as DRB1*01:01, -10:01, -07:01 slightly lower similarities less sequence homology surrounding pocket. Our study indicates different alleles may predispose presentation recognition Dsg3-associated peptides leading initiation phenotypic expression.