Immunopathology of Beh9et's disease
نویسنده
چکیده
Behqet's disease (BD) is best considered as a systemic vasculitis in which recurrent orogenital ulceration is a prominent feature. In 1937 Hulusi Behqet described the syndrome that bears his name' but the term 'BehNet's disease' is now used in preference to 'Behqet's syndrome'. Behqet, himself, recognized the problems related to its diagnosis for which there remains no diagnostic test. BD is rare in Britain but it affects up to 1:1000 of the population of Japan. The increased incidence in Far Eastern countries2 and in the eastern Mediterranean,3 and an association with HLA B5'5 suggest that environmental and genetic factors are important influences in the aetiology of BD. Until recently, 5 sets of diagnostic clinical criteria for BD were variously used around the world, each with its own emphasis on symptoms and signs pertaining to a particular geographical region. In 1990 new criteria for the diagnosis of BD, proposed by an international study group, were published6 and these should facilitate comparisons between studies and hopefully foster collaborative work. Criteria:Recurrent oral minor aphthous, ulceration major aphthous, or herpetiform ulceration which has recurred > 3 times in a one year period. Plus 2 out of:Recurrent genital aphthous ulceration ulceration or scarring. Eye lesions Anterior/posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis observed by ophthalmologist. Skin lesions erythema nodosum, pseudo-folliculitis, or papulopustular lesions; or acneiform nodules observed by physician in postadolescent patients not on corticosteroid treatment. Positive pathergy test Read by physician at 24-48 h. (All findings applicable only in absence of other clinical explanations).
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