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Clinical manifestations and diagnosis of Behçet's syndrome

Ellison L Smith, MD
Yusuf Yazici, MD
Section Editor
Peter A Merkel, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Behçet's syndrome is characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis. Behçet's may have been described by Hippocrates, but it was brought to the attention of the modern medical community by Hulusi Behçet in 1937 [1-3].

Most clinical manifestations of Behçet's syndrome are believed to be due to vasculitis. Among the systemic vasculitides, Behçet's syndrome is remarkable for its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation.

The clinical manifestations, diagnosis, and differential diagnosis of Behçet's syndrome are reviewed here. The pathogenesis and treatment of this disorder are discussed separately. (See "Pathogenesis of Behçet's syndrome" and "Treatment of Behçet’s syndrome".)


Behçet's syndrome is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean [4,5]. It is most common in Turkey (80 to 370 cases per 100,000) while the prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia [4]. By comparison, the prevalence ranges from 1 per 15,000 to 1 per 500,000 in North American (Olmsted County, Minnesota) and Northern European countries [6]. The prevalence is similar in men and women in the areas where it is more common, but women are affected more commonly in reports from the United States and northern Europe. It typically affects young adults 20 to 40 years of age but is infrequently also seen in children [7-9]. The disease appears to be more severe in young, male patients from Middle- or Far-Eastern Asia [10-16]. Most cases of Behçet's are sporadic, although families with multiple affected members, which is known as familial clustering, have been reported, and having a first-degree relative with Behçet's does increase risk for the disease [17,18]. Earlier onset of disease in successive generations, known as genetic anticipation, has been described [19].


The common clinical feature in patients with Behçet's syndrome is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients and populations.

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Literature review current through: Sep 2017. | This topic last updated: Sep 29, 2017.
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