BEHاET’S DISEASE

Behçet’s disease (BD) which is classified among vasculitides is a systemic disease with various manifestations. It progress by attacks and remissions. Till now, two nationwide surveys of BD from Iran and Japan and 4 major case series from Turkey, Korea, Morocco and England have been reported. Clinical picture of BD is dominated by mucous membrane manifestations, including oral aphthosis - seen in 96.8% of patients in Iran, 98.2% in Japan, 100% in Turkey, 97.5% in Korea, 100% in Morocco and 100% in England- and genital aphthosis which is seen less frequently- 65.3% in Iran, 73.2% in Japan, 88.2% in Turkey, 56.7% in Korea, 83.5% in Morocco and 89% in England. Skin aphthosis is not frequent but it is the most characteristic lesion of BD. Erythema nodosum is seen more frequently in China and Korea. Ocular manifestations include anterior uveitis, posterior uveitis and retinal vasculitis. Joint Manifestations include arthralgia, monoarthritis, oligo/poly arthritis, and ankylosing spondylitis. Other presentations include neurological, gastrointestinal and cardiopulmonary manifestations, vascular involvement, orchitis and epididymitis. Erythrocyte sedimentation rate is usually elevated. Urinary abnormalities are infrequent and transient. Pathergy test was positive in 57.4% of patients in Iran, 44% in Japan, 57% in Turkey, 40% in Korea, 68% in Morocco and 32% in England. Lesions usually heal without sequela, except for eyes, brain, and vascular system. The major cause of morbidity is the ocular lesion, which leads to severe loss of vision or blindness.