Veraart J C, Neumann H A, Veraart C J, Engelen J
Academisch Ziekenhuis, afd. Dermatologie, Maastricht.
Ned Tijdschr Geneeskd. 1994 Jan 8;138(2):86-9.
Two patients, men of 38 and 32 years old, had Klinefelter's syndrome in combination with recurrent lower leg ulcers and hyperpigmentations. For the ulcers no venous insufficiency or other underlying cause could be found. Klinefelter's syndrome is characterised by the existence of at least one extra X-chromosome in phenotypical men. Clinical findings are eunochoid body proportions, scanty facial and body hair, gynaecomastia and small testicles. Plasma follicle-stimulating hormone is usually high and testosterone is reduced in about 50% of the patients. Leg ulcers, especially in combination with hyperpigmentation or atrophie blanche of the surrounding skin, can be a symptom of Klinefelter's syndrome and are not necessarily to be attributed to venous insufficiency.
两名患者,分别为38岁和32岁男性,患有克兰费尔特综合征,并伴有复发性小腿溃疡和色素沉着。对于溃疡,未发现静脉功能不全或其他潜在病因。克兰费尔特综合征的特征是表型男性中至少存在一条额外的X染色体。临床表现为类无睾体型、面部和身体毛发稀疏、男性乳房发育和睾丸小。血浆促卵泡生成素通常较高,约50%的患者睾酮降低。腿部溃疡,尤其是伴有周围皮肤色素沉着或白色萎缩时,可能是克兰费尔特综合征的症状,不一定归因于静脉功能不全。