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一例先天性皮肤发育不全膜状型及其皮肤镜特征

A Case of Membranous Aplasia Cutis Congenita and Dermoscopic Features.

作者信息

Lozano-Masdemont Belén

机构信息

Department of Dermatology, Hospital General de Ciudad Real, Obispo Rafael Torija, 13005, Ciudad Real, Spain.

出版信息

Int J Trichology. 2017 Jan-Mar;9(1):33-34. doi: 10.4103/ijt.ijt_91_16.

Abstract

Membranous, bullous, or cystic aplasia cutis congenita is a clinical subtype of aplasia cutis, covered with a membranous or glistening surface. A male newborn presented at birth with two flat lesions on the left parietal scalp, surrounded by a rim of terminal hairs. Physical examination revealed two translucent papules. On dermoscopy, they showed a reddish background, thin, lineal vessels and, remarkably few hair bulbs could be seen because of the translucency of the lesion. No skull bone and brain defects were found. The diagnosis of membranous aplasia cutis congenita was established. Histologically, it is characterized by an atrophic epidermis with loose fibrovascular stroma and edematous dermal stroma. Dermoscopy may help to rule out other entities (herpes simplex, epidermolysis bullosa, trauma…) since the atrophic epidermis and fibrovascular stroma is evidenced by the hair bulbs and its characteristic translucency ("translucency's sign").

摘要

膜性、大疱性或囊性先天性皮肤发育不全是皮肤发育不全的一种临床亚型,其表面覆盖有膜性或有光泽的表皮。一名男婴出生时左顶头皮有两处扁平病变,周围有一圈终毛。体格检查发现两处半透明丘疹。皮肤镜检查显示为淡红色背景、细小的线性血管,由于病变的半透明性,可见到极少的毛囊。未发现颅骨和脑缺陷。确诊为膜性先天性皮肤发育不全。组织学上,其特征为表皮萎缩,纤维血管间质疏松,真皮间质水肿。由于毛囊及其特征性半透明性(“半透明征”)可证明表皮萎缩和纤维血管间质,皮肤镜检查有助于排除其他疾病(单纯疱疹、大疱性表皮松解症、外伤等)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5ad1/5514794/d4ec98ee897c/IJT-9-33-g001.jpg

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