Behcet Disease and the Emergence of Thalidomide

There may well be more articles about Behcet disease or syndrome in U.S. medical journals than there are patients with true Behcet disease in the United States. The international criteria for diagnosis [1] are recurrences of oral ulcerations plus two of the following: recurrent genital ulcers, skin lesions, eye lesions, and a positive result on a pathergy test. The seeming blandness and all-inclusiveness of these criteria lead to overdiagnosis because patients with other mucocutaneous diseases, such as ulcerative colitis, regional enteritis, the Reiter syndrome and other reactive arthropathies, the Stevens-Johnson syndrome, and systemic lupus erythematosus, might qualify [2].

Most true cases cluster along the ancient Silk Route: from Japan, where the disease is the leading cause of acquired blindness [3], to the Mediterranean littoral and areas of the former Ottoman empire [4]. Points in between (Iraq, Iran, China, and Korea) report additional thousands of cases (the Japanese and Turkish languages share Altaic roots, suggesting remote common ancestry of the people who speak these languages and perhaps explaining the genetic seeding along the Silk Route that connects the disparate areas of Asia and the Mediterranean coast) [5]. Western medical centers, even those with a special interest in the disease, accumulate only a handful of …