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Clinical manifestations and diagnosis of Behcet's disease

INTRODUCTION

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

Most clinical manifestations of Behcet's disease are believed to be due to vasculitis. Among the systemic vasculitides, Behcet's disease 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.

Behcet's disease is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean [4]. It is most common in Turkey (80 to 370 cases per 100,000) while the prevalence ranges from 13.5 to 20 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia [4]. By comparison, the prevalence is from 1:15,000 to 1:500,000 in North American (Olmsted County, Minnesota) and Northern European countries. It is somewhat more common in men in the eastern Mediterranean area and in women in north European countries, and typically affects young adults 20 to 40 years of age. The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients [5-11].

Although there are limited data on children with Behçet's disease, clinical manifestations appear to be similar to those in adults [12-14]. The pathogenesis and treatment of this disorder are discussed separately. (See "Pathogenesis of Behcet's disease" and "Treatment of Behcet's disease".)

CLINICAL MANIFESTATIONS

The common clinical feature in patients with Behcet's disease is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients and populations. Men and women have different propensities to develop the disease, but this varies among populations. As an example, Behcet's is more common in men in the Middle East and in women in Japan and Korea [6].
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