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掌跖播散性汗孔角化症

[Porokeratosis plantaris, palmaris et disseminata].

作者信息

Brasch J, Scheuer B, Christophers E

出版信息

Hautarzt. 1985 Aug;36(8):459-61.

PMID:2931406
Abstract

A 74-year-old patient has been affected for about 40 years by hardly discomforting keratotic lesions of the trunk and extremities, especially on the palms and soles. Some of his children and grandchildren have shown similar lesions. Histologically the diagnosis of porokeratosis was established by demonstration of a cornoid lamella. Clinically it appeared to be the rare form of porokeratosis plantaris, palmaris et disseminata, in which mainly the palms and soles are affected by hyperkeratotic papules, but nearly the whole body may be involved. It is discussed how this genodermatosis can be differentiated from other forms of porokeratosis. The family tree of our patient is compatible with autosomal dominant inheritance.

摘要

一名74岁患者的躯干和四肢出现角化性损害,几乎没有不适感,病程约40年,尤其是手掌和脚底。他的一些子女和孙辈也有类似损害。组织学上通过观察到鸡眼样板层确诊为汗孔角化症。临床上表现为罕见的掌跖播散性汗孔角化症,主要是手掌和脚底出现角化性丘疹,但几乎累及全身。文中讨论了这种遗传性皮肤病如何与其他类型的汗孔角化症相鉴别。患者的家族谱系符合常染色体显性遗传。

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[Circumscribed acral hypokeratosis].[局限性肢端角化减退症]
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