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Adamantiades–Behçet Disease 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 most common signs.
  • Inflammatory disease representing a neutrophilic vascular reaction or vasculitis.
  • Chronic relapsing progressive course and potentially poor prognosis (especially in males with systemic presenting signs; mortality, 0–6%).

Adamantiades–Behçet disease is a multisystem inflammatory disease of unknown etiology, classified as systemic vasculitis involving all types and sizes of blood vessels and characterized clinically by recurrent oral aphthous and genital ulcers, skin lesions, and iridocyclitis/posterior uveitis, occasionally accompanied by arthritis and vascular, gastrointestinal, neurologic, or other manifestations1,2 (Fig. 166-1).

Figure 166-1

Adamantiades–Behçet disease: a multisystem disorder.

Hippocrates of Kos (460–377 bc) used the designation “στoματα 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 insisting for a single clinical entity.3 The first international multidisciplinary conference was organized by two dermatologists, Drs. M. Monacelli and P. Nazarro, 1964 in Rome, Italy.

Adamantiades–Behçet disease presents a worldwide occurrence 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, 7.3:100,000; Northern China, 14:100,000; Saudi Arabia, 20:100,000; Iran, 17:100,000) and 1.5–7.5:100,000 in Southern Europe. In Northern Europe (0.27–1.18:100,000) and the United States (0.12–0.33:100,000), the disease is rare.4 In countries with several ethnic populations, including of Turkmen and Mongol descents, the latter are mainly affected. The increasing prevalence of the disease is due to its chronic character. 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. In contrast to old Japanese and Turkish reports of male predominance, the male-to-female ratio drastically decreased in the last 20 years. Currently, both genders are equally affected; a male predominance is still observed in Arab populations, whereas female predominance is evident in Korea, China, ...

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