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%).
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
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.
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.
ETIOLOGY AND PATHOGENESIS