Behçet's syndrome

Behçet's syndrome is a multi-system inflammatory disorder in which recurrent oral ulceration is associated with genital ulceration, pustules and/or uveitis. Other possible manifestations include superficial thrombophlebitis, deep vein thrombosis, arterial aneurysms, gastrointestinal ulceration and inflammatory lesions of the central nervous system. A hallmark of Behçet's syndrome is the pathergy response, which is the development of a papule/pustule 24–48 hours after insertion of a needle into the skin. The aetiology of Behçet's syndrome is not known and the diagnosis is made on clinical grounds; there is no established laboratory test. Treatment ranges from local application of corticosteroid to mucocutaneous lesions, to the use of systemic corticosteroids and immunosuppressant agents (e.g. azathioprine). The use of biological agents, such as those that target tumour necrosis factor, for patients with severe disease that is unresponsive to other agents is promising but not yet fully evaluated or licensed in the UK.