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系统性透明变性(青少年透明纤维瘤病)。透明质的超微结构,特别提及横纹结构。

Systemic hyalinosis (juvenile hyaline fibromatosis). Ultrastructure of the hyaline with particular reference to the cross-banded structure.

作者信息

Ishikawa H, Maeda H, Takamatsu H, Saito Y

出版信息

Arch Dermatol Res. 1979 Jun 25;265(2):195-206. doi: 10.1007/BF00407885.

DOI:10.1007/BF00407885
PMID:88923
Abstract

Systematic hyalinosis (juvenile hyaline fibromatosis) is characterized by hyalinized skin lesions. Electron microscopic examination of a hyalinized skin tumor from a 19-year-old man with this syndrome revealed that the hyaline was composed of ruthenium red-positive ultrastructures (granules, filaments, and a kind of cross-banded structure), indicating the presence of glycosamino-glycan or glycoprotein, and a small number of thin collagen fibrils. Using a new ruthenium red staining method combined with an enzymatic digestion procedure, it was demonstrated that the cross-banded structure and granules consisted of chondroitin sulfate-proteoglycan and/or glycoprotein, and that the cross-banded structure is probably a noncollageneous aggregate of the granules attached to the parallel-arranged filaments of hyaluronic acid-like nature.

摘要

系统性透明变性(青少年透明纤维瘤病)的特征是皮肤病变呈透明样改变。对一名患有该综合征的19岁男性的透明样皮肤肿瘤进行电子显微镜检查发现,透明物质由钌红阳性超微结构(颗粒、细丝和一种横纹结构)组成,表明存在糖胺聚糖或糖蛋白,以及少量细胶原纤维。采用一种新的钌红染色方法并结合酶消化程序,证明横纹结构和颗粒由硫酸软骨素蛋白聚糖和/或糖蛋白组成,并且横纹结构可能是附着在类似透明质酸性质的平行排列细丝上的颗粒的非胶原聚集体。

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Br J Dermatol. 1962 Jan;74:8-19. doi: 10.1111/j.1365-2133.1962.tb13905.x.
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HYALINOSIS CUTIS ET MUCOSAE. (LIPOIDPROTEINOSIS URBACH-WIETHE).
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