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[两兄弟患米贝利汗孔角化症]

[Mibelli porokeratosis in 2 brothers].

作者信息

Yedomon H G, Do Ango-Padonou F

机构信息

Service de Dermatologie, Centre National Hospitalier et Universitaire (CNHU), Cotonou Bénin.

出版信息

Ann Dermatol Venereol. 1996;123(3):171-3.

PMID:8761776
Abstract

INTRODUCTION

Mibelli's porokeratosis is uncommon in black persons. We report two brothers who had two different clinical presentations.

CASE REPORT

The brothers were seen at the ages of 16 and 19 years. Both had Mibelli's porokeratosis, one with a papulo-verruciform presentation located on the scrotum, the anus, the gluteal area and the back of the hand, and the other with a superficial disseminated eruption involving the face and the forearm.

DISCUSSION

The incidence of Mibelli's porokeratosis in the black population at Cotonou is approximately 0.3 per 10.000. The presence of the disease in two uterine brothers confirms the monogenic and familial nature of Mibelli's porokeratosis. Dominant transmission cannot be easily demonstrated when the parents of the patients are phenotypically healthy.

摘要

引言

米贝利氏汗孔角化症在黑人中并不常见。我们报告了两兄弟,他们有两种不同的临床表现。

病例报告

兄弟俩分别在16岁和19岁时前来就诊。两人均患有米贝利氏汗孔角化症,其中一人表现为丘疹疣状,位于阴囊、肛门、臀区和手背,另一人表现为浅表播散性皮疹,累及面部和前臂。

讨论

科托努黑人人群中米贝利氏汗孔角化症的发病率约为万分之0.3。两名亲兄弟均患此病,证实了米贝利氏汗孔角化症的单基因和家族性本质。当患者的父母表型健康时,显性遗传难以轻易证实。

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