Adamantiades-Behget' s disease is seen in virtually every country, but the region with the highest prevalence stretches from the Mediterranean across Asia to Japan—the so-called "Silk Route." Along this route, the disease is most common in Turkey, with prevalence rates approaching 370 cases per 100,000 (4). The disease becomes less prevalent as distance from the Silk Route increases, with prevalence rates in Europe and North America between 1 in 15,000 and 1 in 500,000 (4).
Typically, patients present with symptoms in their second or third decades, although a wide range of age-of-onset has been described, including childhood disease. In contrast to many other autoimmune diseases, Adamantiades-Behget' s tends to occur equally in males and females; however, disease in males seems to predominate in the Mediterranean area, while disease in females is more common in Europe and North America (5). Additionally, disease in males of Mediterranean and Asian ancestry tends to be more severe than that seen in patients of European descent.
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