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亨特综合征的一种皮肤标志物:四例报告。

A cutaneous marker in the Hunter syndrome a report of four cases.

作者信息

Prystowsky S D, Maumenee I H, Freeman R G, Herndon J H, Harrod M J

出版信息

Arch Dermatol. 1977 May;113(5):602-5.

PMID:404968
Abstract

A report of four patients and review of the literature suggest that a cutaneous marker exists for the Hunter syndrome. All previously described patients with this eruption have been males with clear corneas. The distinctive lesions consist of firm ivory-white papules and nodules that may coalesce to form ridges or a reticular pattern. The papules are usually seen in symmetrical areas between the angles of the scapulas and posterior axillary lines, the pectoral regions, the nape of the neck and/or on the lateral aspects of the upper arms and thighs. These lesions are seen in both allelic forms and cannot be used to separate a benign from a rapid course.

摘要

一份关于4例患者的报告及文献综述表明,亨特综合征存在一种皮肤标志物。所有此前描述的有这种皮疹的患者均为男性且角膜清亮。特征性损害由坚实的象牙白色丘疹和结节组成,这些丘疹和结节可融合形成嵴或网状图案。丘疹通常见于肩胛角与腋窝后线之间的对称区域、胸部、颈后和/或上臂及大腿外侧。两种等位基因形式均可见到这些损害,且不能用于区分病情为良性还是进展迅速。

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