Abstract
Deposition of immune complexes probably plays a role in the pathogenesis of dermatitis herpetiformis (DH) and systemic lupus erythematosus (SLE). Dermatitis herpetiformis and SLE were diagnosed in a 15 yr old girl with Marfan''s syndrome who died of cardiac tamponade secondary to cystic medial necrosis of the ascending aorta. The concurrence of these diseases suggests that predisposition to immune-mediated disorders may be associated with the expression of multiple clinical entities.