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[先天性皮肤松弛症]

[Congenital cutis laxa].

作者信息

De Anda G, Vignale R A

出版信息

Med Cutan Ibero Lat Am. 1984;12(1):1-5.

PMID:6234443
Abstract

A case of connatal flabby skin or generalized dermatochalasia without genetical familial background is presented. The cutaneous clinic is striking to which pulmonary emphysema with overcharge of the right heart is added. By optical and electronic microscopy the characters of elastic fibers are studied which show diminished with orceing with a normal microfibrilar component lacking the amorphous element or elastine with the electronic microscope. The formation of the dense normal element also appears compromized .

摘要

本文报告一例无遗传家族背景的先天性皮肤松弛或全身性皮肤松弛症病例。其皮肤临床表现显著,并伴有右心负荷过重的肺气肿。通过光学显微镜和电子显微镜研究了弹性纤维的特征,结果显示在光学显微镜下弹性纤维数量减少,在电子显微镜下微原纤维成分正常,但缺乏无定形成分或弹性蛋白,致密正常成分的形成似乎也受到影响。

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