[Behçet's disease--clinical presentation, diagnostic and therapeutic approach]

Abstract

Behçet's disease is a complex, multisystem disease first described in 1937 by the Turkish dermatologist, Hulusi Behçet. In his original description, Behçet referred to a symptom complex of recurrent oral aphthous ulcers, genital aphthae, and iritis that could lead to blindness. Today we know that Behçet's disease is much more than a "triad syndrome" and it may also involve the gastrointestinal, central nervous system and large vessels. As yet, there are no existing diagnostic laboratory tests or curative treatments for Behçet's disease. Howver, genetic studies have identified those who are at risk and newer molecular biologic investigations further elucidate the pathogenesis of the disease. The treatment of Behçet's disease depends on clinical manifestations and the severity of the disease. Although various therapeutic modalities have been employed for Behçet's disease, treatment is still unsatisfactory. Treatment modalities include local, systemic, or surgical approaches. Only limited success has been achieved with classic agents such as: colchicine, indomethacin, chlorambucil, and systemic corticosteroids. New therapeutic approaches have been introduced for Behçet's disease using cyclosporine, thalidomide, interferon, high-dose corticosteroids or cyclophosphamide therapy. In this article we summarized the clinical and diagnostic approach for Behçet's disease and the current therapy indicated for different clinical presentations of the disease.