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AT-A-GLANCE
Rare disease with worldwide distribution but strongly varying prevalence; certain ethnic groups are mainly affected.
A genetically determined disorder with a probable environmental triggering factor.
Multisystem occurrence, with oral aphthous ulcers, genital ulcers, papulopustules, erythema nodosum–like lesions, uveitis, and arthropathy as the most common signs.
Inflammatory disease representing a neutrophilic vascular reaction or vasculitis.
Chronic, relapsing, and progressive course with a potentially poor prognosis (especially in males with systemic presenting signs; mortality, 0%-6%).
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Adamantiades–Behçet disease is a multisystem inflammatory disease of unknown etiology. It is classified as a systemic vasculitis involving all types and sizes of blood vessels and characterized clinically by recurrent oral aphthous and genital ulcers, skin lesions, iridocyclitis/posterior uveitis, and arthritis. These findings are occasionally accompanied by vascular, GI, neurologic, or other manifestations.1,2
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Hippocrates of Kos (460-377 BC) used the designation “στοµατα αφθωδεα, ελκωδεα” (oral aphthous ulcers) in a probable first description of a patient with the disease (Epidemion Book III, Case 7). The disease is named after Benediktos Adamantiades, a Greek ophthalmologist and HulÛsi Behçet, a Turkish dermatologist, who, in 1931 and 1937, respectively, described patients with the characteristic clinical complex.3 The first international multidisciplinary conference was organized by 2 dermatologists, Drs M. Monacelli and P. Nazarro, in 1964 in Rome, Italy.
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Adamantiades–Behçet disease occurs worldwide with varying prevalence, being endemic in the Eastern and Central Asian and the Eastern Mediterranean countries (along the so-called Silk Road) and rare in Northern European countries, Central and Southern Africa, the Americas, and Australia.4 A prevalence of 80 to 420 patients per 100,000 inhabitants has been reported in Turkey,5 7 to 30 patients per 100,000 inhabitants in the rest of the Asian continent (Japan, 14-31:100,000; Korea, 35:100,000; Northern China, 14:100,000; Saudi Arabia, 20:100,000; Iran, 17:100,000), and 1.5 to 7.5 per 100,000 in Southern Europe.4 In Northern Europe (0.27-1.18:100,000) and the United States (0.75:100,000), the disease is rare.6,7 The increasing prevalence of the disease is due to its chronic nature. Its annual incidence is low; 0.75 to 1.0 new cases per 100,000 inhabitants were assessed in Japan (1990) and Germany (2005).6 Adamantiades–Behçet disease most often affects patients in their 20s and 30s; however, early and late onsets (first year of life to 72 years) have been reported. Juvenile disease rates are 2% to 21% in different ethnic groups. Its prevalence was estimated to be 0.17:100,000 in France.8,9 In contrast to old Japanese and Turkish reports of male predominance, the male-to-female ratio drastically decreased in the last 20 years.10 Currently, both genders are equally affected overall; however, male predominance is still observed in Arab populations, and female predominance is evident in Korea, China, some Northern European countries, and the United States.
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ETIOLOGY AND PATHOGENESIS
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