Familial Behçet's syndrome with intestinal involvement--case reports and a review of the literature

Behçet's syndrome is a rare multisystem disorder. The occurrence in families although well recognised is uncommon. The mode of inheritance, however, has not been elucidated. Sixty-one cases of familial disease have been documented to date. There was only one previous report on familial Behçet's syndrome with gastrointestinal involvement. This paper reports two cases of Behçet's syndrome with gastrointestinal involvement in a Malaysian woman of Chinese ethnic origin and her child. The mother satisfied three major criteria (oral ulcers, genital ulcers and erythema nodosum) and one minor criterion (gastrointestinal involvement). Her son satisfied two major criteria (oral and genital ulcers) and three minor criteria (intestinal involvement, central nervous manifestation and a positive family history). The radiological and pathological features of the gastrointestinal lesions in both patients resembled those of Crohn's disease. The literature on Behçet's syndrome is reviewed.