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[结节性皮肤淀粉样变性。临床、组织病理学及超微结构表现]

[Nodular cutaneous amyloidosis. Clinical, histopathological and ultrastructural findings].

作者信息

Goerttler E, Anton-Lamprecht I, Kotzur B

出版信息

Hautarzt. 1976 Jan;27(1):16-25.

PMID:1254472
Abstract

A case of nodular amyloidosis cutis of the face with minimal deposits of amyloid in the rectum is reported with respect to clinical, histopathological, and ultrastructural findings. A discussion of the problem of systematization in nodular amyloidoses is included. By electron microscopy, the principal similarity of the ultrastructure of amyloid is stressed again. Amyloid in this case of nodular amyloidosis cutis is thought to be synthesized by plasma cells which are found especially in the growing peripheral parts of the tumors. Fibroblasts with extremely dilated endoplasmic reticulum are almost totally surrounded by the fibrillar masses of amyloid within the tumor. Unusual spindle-formed cisternae of Golgi bodies containing oriented coarse fibrils are found in the fibroblasts. They are thought to represent a disturbance of the secretion of tropocollagen to the interstitium due to the surrounding amyloid masses.

摘要

本文报告了一例面部结节性皮肤淀粉样变病例,其直肠中淀粉样蛋白沉积极少,并阐述了临床、组织病理学及超微结构检查结果。文中还讨论了结节性淀粉样变的分类问题。通过电子显微镜观察,再次强调了淀粉样蛋白超微结构的主要相似性。该例结节性皮肤淀粉样变中的淀粉样蛋白被认为是由浆细胞合成的,这些浆细胞尤其见于肿瘤生长的周边部位。肿瘤内,内质网极度扩张的成纤维细胞几乎完全被淀粉样蛋白的纤维团所包围。在成纤维细胞中发现了含有定向粗纤维的异常纺锤形高尔基池。它们被认为是由于周围的淀粉样蛋白团块导致原胶原蛋白向间质分泌受到干扰所致。

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