Vasculitis

Behçet's disease is a complex multisystem disease characterized by oral aphthae and other features.

Diagnosis is based on the criteria set forth by the International Study Group, including oral aphthae, genital aphthae, ocular lesions, cutaneous lesions, and a positive pathergy test.

Cutaneous lesions should display a neutrophilic vascular reaction on histopathologic examination.

Treatment is based on the degree of systemic involvement and ranges from topical corticosteroids to thalidomide to systemic immunosuppressive agents and tumor necrosis factor-α inhibitors.

Prognosis is variable, and patients typically have periods of exacerbations and remissions.

Behçet's disease is a chronic, complex multisystem disease characterized clinically by oral aphthae and at least two of the following: genital aphthae, cutaneous lesions, and ophthalmic, neurologic, or rheumatologic manifestations. The first description of Behçet's disease was probably by Hippocrates in the fifth century BC,[1] and the first modern account was presented in 1937 by the Turkish dermatologist Hulusi Behçet, who reported on a patient with recurrent oral and genital aphthae and uveitis.[2]

EPIDEMIOLOGY

Behçet's disease is seen worldwide, with the highest prevalence reported in Turkey (80 to 370 patients per 100,000 inhabitants)[3] and Japan (13.6 per 100,000).[4] Other regions with high prevalence include the Middle East and the Mediterranean (i.e., the “Silk Route”).[5] It is relatively uncommon in northern Europe and the United States (0.1 to 7.5 patients per 100,000 inhabitants). [3] [5] Patients commonly fulfill the diagnostic criteria in their mid-20s to 30s.[6] In the past, Behçet's disease was thought to predominantly affect males, but current epidemiologic data show a more equal male-to-female ratio.[7] Overall, males are more affected in the Middle East, whereas females predominate in northern Europe and the United States.[7]

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