Behçet’s disease is a systemic inflammatory vasculitis, characterized by a course of remission and relapse of oral ulcers, genital ulcers, skin lesions and ocular lesions. It can affect the arteries and veins of almost any system and has been shown to involve the gastrointestinal and neurological systems. The cause of Behçet’s is unknown but it is believed to be triggered by an infectious or environmental agent in a genetically predisposed individual.

Clinical Utility

The HLA class I molecule HLA-B51 is the most strongly associated risk factor and has been shown to be associated with Behçet’s in Turkish and Asian patients, though the association in Caucasian patients is much weaker. Testing for HLA-B51 is a useful, though not diagnostic, tool for Behçet’s as the vast majority of HLA-B51 carriers do not develop Behçet’s. Diagnosis is instead based on the recurrence of symptoms including oral ulcers, genital ulcers, skin lesions and ocular lesions. Treatment options depend on organs involved.