Association of DR4 with pemphigus

HLA typing for the A, B, C, and D locus antigens was performed on 65 patients with pemphigus vulgaris and on 558 controls living in the Los Angeles area. The patients were divided into several categories. These included Jewish and non-Jewish patients, patients with only mucous membrane involvement, only skin or both mucous membrane and skin involvement, and those with a single-episode or recurrent disease. Depending on the highest titer of anti-intercellular cement substance antibody titer, the patients were categorized into those whose titers were 0-80, 160-320, and 640 or greater. A statistically increased incidence of HLA-A25, HLA-B38, and HLA-DR4 antigens was observed in patients compared to controls. This incidence was significantly higher in Jewish compared to non-Jewish patients. The correlations were insignificant in the group with an antibody titer of 0-80, but significant in those with a titer of 160-320, and even more significant in those with titers greater than 640. No significant differences were present between patients who had a single-episode or recurrent disease or in those that had only mouth or only skin involvement. In all categories tested, the association was stronger with DR4 than with A26 or B38. DR4 was present equally in B38-positive and B38-negative patients. The primary association of pemphigus vulgaris may be with the DR4 antigen, and it may be a marker for the severity of the disease.