High frequency of histocompatibility antigens HLA-DR3 and DR4 in Herpes gestationis

Herpes gestationis (HG) is a rare, autoimmune, vesiculobullous disease of pregnancy or the puerperium characterized by the deposition of complement (and occasionally immunoglobulin) within the lamina lucida of the cutaneous basement membrane zone. We have studied 23 patients with a history of HG, 20 of whom had typical immunofluorescence findings during the active phase of their disease. HLA typing showed HLA-DR3 in 61% of patients (controls 22%, Pc < 0.005) and the combination of DR3, DR4 in 43% (controls 3%, Pc< 0.00001). The most striking finding of this study was that the greatest risk of HG is associated with the concurrent presence of two specific histocompatibility leukocyte antigen (HLA)-DR antigens.