The multiple faces of Behçet's disease and its aetiological factors

Behçet's disease (BD) is a chronic, inflammatory multisystemic condition of unknown aetiology. It is clinically characterized by recurrent orogenital ulcerations and skin eruptions; ocular manifestations; arthritis; vasculitis and in some cases neurological and large vessel involvement. Aetiology has not been defined, but genetic, environmental, viral, bacterial and immunological factors have been proposed as causative agents. Treatment includes colchicine, thalidomide, steroids and immunosuppressive agents and it is based on the severity of systemic manifestations, such as central nervous system involvement, arterial aneurysms and thrombosis of the major veins. Mortality is related to major system involvement. In this article the different clinical features, the multiple faces of BD and a list of currently suspected aetiological factors of the disease are discussed, and treatment modalities summarized.