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迟发性对称性遗传性色素异常症?

Dyschromatosis symmetrica hereditaria of late onset?

作者信息

Gaiewski Caroline Balvedi, Zuneda Serafini Sergio, Werner Betina, Deonizio Janyana M D

机构信息

Dermatology Department, Federal University of Parana, 80530-905 Curitiba, PR, Brazil.

Pathology Department, Federal University of Parana, 80530-905 Curitiba, PR, Brazil.

出版信息

Case Rep Dermatol Med. 2014;2014:639537. doi: 10.1155/2014/639537. Epub 2014 Feb 4.

Abstract

Dyschromatosis symmetrica hereditaria (DSH), also known as reticulated acropigmentation of Dohi, is an autosomal dominant disease with high penetrance, characterized by hypo- and hyperpigmented macules of varying sizes on the dorsal of the extremities with reticulated pattern. This paper presents a female patient with typical dermatological lesions, but only diagnosed in adulthood. It is necessary to perform differential diagnosis with other pigmentary disorders. This entity is not very common in South America, and the vast majority of cases were described in Japanese population. Since it is a benign disease, it is important to be aware of this diagnosis in order to establish the correct conduct for these patients.

摘要

对称性进行性色素异常症(DSH),也称为土肥网状肢端色素沉着症,是一种具有高外显率的常染色体显性疾病,其特征为四肢背部出现大小不一的色素减退和色素沉着斑,并呈网状分布。本文介绍了一名有典型皮肤病变的女性患者,但直到成年才被诊断出来。有必要与其他色素性疾病进行鉴别诊断。这种疾病在南美洲并不常见,绝大多数病例是在日本人群中报道的。由于它是一种良性疾病,认识到这一诊断对于为这些患者确立正确的治疗方法很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59ea/4006555/777d6ea134b2/CRIDM2014-639537.001.jpg

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