Ⅰ型埃勒斯-当洛综合征中菜花状胶原原纤维的形态计量学研究。

Morphometric study of cauliflower collagen fibrils in Ehlers-Danlos syndrome type I.

作者信息

Piérard G E, Lê T, Piérard-Franchimont C, Lapière C M

机构信息

Dermatopathology Service, University of Liege, Belgium.

出版信息

Coll Relat Res. 1988 Sep;8(5):453-7. doi: 10.1016/s0174-173x(88)80019-0.

DOI:10.1016/s0174-173x(88)80019-0

PMID:3224502

Abstract

The abnormal cauliflower collagen fibrils present in skin of patients with Ehlers-Danlos type I were studied by electron microscopy and computerized image analysis. The size control of the few fibrils displaying a cauliflower shape is apparently lost, eventuating in an increased amount of collagen polymers constitutive of these fibrils. This situation is fundamentally different from that occurring in dermatosparaxis, an animal disease considered as a model for Ehlers-Danlos syndrome.

摘要

通过电子显微镜和计算机图像分析对Ⅰ型埃勒斯-当洛综合征患者皮肤中存在的异常菜花状胶原纤维进行了研究。少数呈现菜花状的纤维的尺寸控制显然丧失，最终导致构成这些纤维的胶原聚合物数量增加。这种情况与皮肤松弛症（一种被视为埃勒斯-当洛综合征模型的动物疾病）中发生的情况有根本不同。