Epidemiology of Adamantiades-Behcet's disease

Adamantiades-Behcet's disease is a universal disorder with varying prevalen ce, i.e. 80-370 patients per 100,000 inhabitants in Turkey, 2-30 patients p er 100,000 inhabitants in the Asian continent and 0.1-7.5 patients per 100, 000 inhabitants in Europe and the USA. Certain ethnic groups are mainly aff ected, while the prevalence of the disease seems to be strongly dependent o n the geographic area of their residence. These data indicate environmental triggering of a genetically determined disorder. The disease usually occur s around the third decade of life. however, early and late onsets (first ye ar of life to 72 years) have been reported. Juvenile onset disease rates fr om 7 to 44% in different ethnic groups; juvenile disease is less frequent, i.e. 2-21%. Both genders are equally affected. Familial occurrence has been reported in 1-18% of the patients, mostly of Turkish, Israeli and Korean o rigin, and is increased in patients with juvenile disease. Oral aphthous ul cers represent the onset sign in the majority of patients worldwide (47-86% ). Oral aphthous ulcers (92-100%), genital ulcerations (57-93%), skin lesio ns (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 encountered skin lesions. The positivity of p athergy test varies widely in different populations (6-71%). HLA-B51 is ass ociated with high relative risk for the disease in a small geographic area of the Mediterranean Sea countries and Southern Asia. Diagnosis can be esta blished 2 to 15 years after the onset of the disease. Male gender, early de velopment of the disease, and HLA-B51 positivity are markers of severe prog nosis (mortality rates of 0-6%).