BEH ET＊S DISEASE

作者：F Davatchi; F Shahram; C Chams; H Chams A Nadji

来源：Acta Medica Iranica, 2005.

• clinical manifestations
• muco-cutaneous manifestations
• aphthous
• ulcers

Beh et＊s disease (BD) which is classified among vasculitides is a systemic disease with various manifestations. Its clinical course is characterized 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. Ocular manifestations include anterior uveitis, posterior uveitis and retinal vasculitis. Joint manifestations include arthralgia, monoarthritis, oligo/poly arthritis, and ankylosing spondylitis. Other manifestations include neurological, gastrointestinal and cardiopulmonary manifestations, vascular involvement, orchitis and epididymitis. Erythrocyte sedimentation rate is usually elevated. Urinary abnormalities are infrequent and transient. Positive pathergy test has been reported 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 lesions of eyes, brain and vascular system. The major cause of morbidity is the ocular lesion, which could lead to severe loss of vision or blindness.