Behçet´s Syndrome - The Cerrahpasa Experience

Journal/Book: Z Rheumatol 1998; 57 Suppl. 1: 16 (V 64). 1998;

Abstract: University of Istanbul About 8 % of probands with Behçet´s Syndrome (BS) have a relative afflicted with the same condition. On the other hand isolated symptoms like aphtae genital ulcers etc do not seem to be increased among the family members when investigated in a controlled setting. It is interesting however that the pathergy reaction. when present in two successive generations is preferentially inherited.(?) from the mother suggesting a non-Mendelian mode of transmission. Recently we have shown rather striking genetic anticipation among our patients. Out of 18 families who had a member afflicted in two successive generations in 15 families the disease onset was markedly earlier however with no indication for increased severity in the second generation. BS definitely runs a more severe course among the male. Furthermore the acneiform skin lesions might represent ordinary acne perhaps indicative of an augmented end organ response to male hormones. Previous studies had shown that androgen levels were not increased in BS. We studied the skin androgen receptors among 11 patients with BS and compared these to those found in healthy controls. There were no differences. Also the histology of the pustular lesions occurring in arms legs (sites unusual for acne vulgaris) were compared to those seen in patients with acne vulgaris. There were no differences in histology implying that at least some of the acneiform lesions of BS might indeed be ordinary acne. The main pathology of BS seems to center around an increased tendency to inflammation. However no work had been done related to the healing process. We looked at wound heeling in 20 BS patients using 20 acne vulgaris patients as controls. ... le

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