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History
Behçet's disease is a chronic inflammatory disorder of unknown aetiology characterised by recurrent attacks. Although the triple symptom complex of oral and genital ulcerations with uveitis was reported by Hippocrates and other authors who attributed the symptom triad to major infections, Hulusi Behçet, a Turkish dermatologist, discarded the association with other illnesses and was the first to delineate the disease that now bears his name.1 Clinical manifestations additional to this triad were described later including involvement of the skin, joints, large vessels, lung, brain, gastrointestinal and genitourinary tracts (table 1).2-6It is now recognised as a multisystem disease with vasculitis as the main pathological finding.6-10
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Frequency of clinical manifestations in Behçet's disease8 ,9 ,12
Diagnosis
Since Behçet's disease does not have pathognomonic symptoms or laboratory findings, the diagnosis is made on the basis of the criteria proposed by the International Study Group for Behçet's disease in 1990 (table 2).11 According to the criteria, recurrent oral ulceration must be present and at least two of the following: recurrent genital ulceration, eye lesions, skin lesions, or a positive pathergy test (development of a papule or pustule following a needle prick to the skin, as in table 2).
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International Study Group criteria for the diagnosis of Behçet's disease11
Epidemiology
Although Behçet's disease has a worldwide distribution, most cases cluster along the ancient Silk Road which extends from far eastern Asia to the Mediterranean basin (table 3).12-18The highest prevalence rate was reported from Turkey as 80–370 per 100 000. The prevalence ranges from two to 30 cases per 100 000 in other Asian countries, with lower figures in Europe and the USA. The age of disease onset is usually in the second or third decade of life and the male to female ratio is …