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[先天性弥漫性黑变病]

[Congenital diffuse melanosis].

作者信息

Kint A, Oomen C, Geerts M L, Breuillard F

出版信息

Ann Dermatol Venereol. 1987;114(1):11-6.

PMID:3579125
Abstract

Two cases of diffuse congenital melanosis are presented. The hyperpigmentation appeared shortly after birth and invades progressively the trunk and the limbs. It is diffuse and most intensive on the abdomen and the back, and reticulated on the neck, the genitals and in the groins. The nails are thin and their surface is slightly irregular. Histologic examination reveals the presence of melanin in the deep and superficial layers of the epidermis. On electron microscopy the melanosomes are not grouped within the keratinocytes, but are dispersed throughout the cytoplasm of the epidermal cells. The disease can be considered as an autonomous entity. The mechanism of the hyperpigmentation is not known.

摘要

本文报告两例弥漫性先天性黑变病。色素沉着在出生后不久出现,并逐渐蔓延至躯干和四肢。色素沉着呈弥漫性,在腹部和背部最为明显,在颈部、生殖器和腹股沟处呈网状。指甲变薄,表面略有不规则。组织学检查显示表皮深层和浅层均有黑色素存在。电子显微镜检查发现黑素小体不在角质形成细胞内聚集,而是分散在表皮细胞的整个细胞质中。该病可被视为一种独立的疾病实体。色素沉着的机制尚不清楚。

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