施瓦赫曼综合征：一例病例报告。

Shwachman syndrome: a case report.

作者信息

Mortureux P, Taïeb A, Bazeille J E, Hehunstre J P, Maleville J

机构信息

Services de Dermatologie et Pédiatrie A, Hôpital des Enfants, Bordeaux, France.

出版信息

Pediatr Dermatol. 1992 Mar;9(1):57-61. doi: 10.1111/j.1525-1470.1992.tb00327.x.

DOI:10.1111/j.1525-1470.1992.tb00327.x

PMID:1374183

Abstract

Cutaneous involvement is frequent in Shwachman syndrome, and includes various degrees of dry skin, and eczematous and ichthyosiform lesions. A 12-year-old boy with Shwachman syndrome had cutaneous involvement characterized by dry skin, perioral dermatitis, and follicular keratosis. Polymorphonuclear motility was decreased. A nutrition work-up showed a decrease in liposoluble vitamins, and suggested a causative link with the skin lesions.

摘要

皮肤受累在施瓦茨曼综合征中很常见，包括不同程度的皮肤干燥、湿疹样和鱼鳞病样病变。一名患有施瓦茨曼综合征的12岁男孩出现皮肤受累，其特征为皮肤干燥、口周皮炎和毛囊角化病。多形核白细胞运动能力下降。营养检查显示脂溶性维生素减少，并提示与皮肤病变存在因果关系。