BehÃ§et's disease is a complex, multisystem, inflammatory disease classically characterized by recurrent oral ulceration, genital ulceration and ophthalmologic inflammation. Additional areas of involvement may include the vascular, neurologic, articular, respiratory, and gastrointestinal systems leading to a myriad of clinical presentations.
In 1937, Hulusi BehÃ§et, a Turkish dermatologist, described a syndrome characterized by recurrent oral ulcers, genital ulcers, and hypopyon uveitis of unknown cause. The diverse range of clinical findings seems to stem from an underlying vasculitis; however, the ultimate etiologic agent remains unknown.
Occurring most frequently in the Middle East, BehÃ§et's is endemic among populations clustered along the historic silk road, which extended from eastern Asia to the Mediterranean basin. Highest prevalence is in Iran, Turkey, and other Near Eastern or Mediterranean Basin countries. In the Middle East, Europe and the United States, young men are most often affected; however, in Japan and Korea there appears to be a slight female preponderance. Current prevalence is estimated to range from a low of 0.3 per 100,000 in Northern Europe to a peak of 16 to 100 per 100,000 in Iran.