Immunogenetics of dermatitis herpetiformis.
Dermatitis herpetiformis (DH) is a pruritic, papulovesicular skin disease characterized in part by the presence of granular deposits of IgA at the dermal-epidermal junction, an associated gluten sensitive enteropathy (GSE), and a strong association with specific human histocompatibility leukocyte antigens (HLA). Initial investigations revealed that 60% to 70% of patients with DH expressed the HLA antigen B8 (normal subjects = 21%). Further investigation of the HLA associations seen in patients with DH has revealed an even higher frequency of the HLA class II antigens HLA-DR3 (DH = 95%; normal = 23%), HLA-DQw2 (DH = 100%; normal = 40%), and HLA-DPw1 (DH = 42%; normal = 11%). Analysis of the genetic linkage of HLA antigens has revealed that the strongest HLA association in patients with DH is with the HLA class II antigens DR3 and DQw2. This article will discuss current studies regarding the role of HLA associations and DH and the role this strong HLA association may play in the pathophysiology of DH.
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