Adamantiades-Behçet’s disease is a universal disorder with varying prevalence, i.e. 80–370 pat/100,000 inhabitants in Turkey, 2–30 in the Asian continent and 0.1–7.5 in Europe and the USA. Certain ethnic groups are mainly affected. The prevalence of the disease seems to be strongly dependent on the geographic area of their residence. These data indicate environmental triggering of a genetically determined disorder. The disease usually occurs around the 3rd decade of life, however, early and late onsets (1–72 y) have been reported. Juvenile onset disease rates 7–44% in different ethnic groups; juvenile disease is less frequent (2–21%). Both genders are equally affected.
Familial occurrence has been reported in 1–18% of patients, mostly Turks, Israeli and Koreans, and is increased in patients with juvenile disease. Oral aphthous ulcers represent the onset sign in the majority of patients worldwide (47–86%). Oral aphthous ulcers (92–100%), genital ulcerations (57–93%), skin lesions (38–99%), ocular lesions (29–100%) and arthropathy (16–84%) are the most frequent clinical features; sterile pustules (28–66%) and erythema nodosum (15–78%) are the most common skin lesions. The positivity of pathergy test varies widely in different populations (6–71%). HLA-B51 is associated with high relative risk for the disease in the Mediterranean Sea countries and Southern Asia. Diagnosis can be established 2–15 y after the onset of the disease. Male gender, early development of the disease in adults, and HLA-B51 positivity are markers of severe prognosis (mortality 0–6%).