Abstract

Behçet's disease is a multisystemic disease affecting most organs. Although a tendency towards an association with a certain genetic type and with HLA-B51 is suspected, the incidence of several siblings with Behçet's disease in a single family is rare. A family, in which three sisters were affected with Behçet's disease, uveitis being the most severe manifestation, was studied. In this family all siblings were B51 positive. Only the female siblings, however, with a positive identical HLA phenotype: A2, A11, B51, B44, Cw6, Cw5, DR4, DRw13, DRw53, DRw52, DQw7, DQw6, developed the disease symptoms, whereas none of the male siblings was affected.