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[关于与副蛋白血症相关的皮肤变化问题。光镜和电镜研究(作者译)]

[On the question of cutaneous changes associated with paraproteinemia. Light- and electron-microscopic study (author's transl)].

作者信息

Rupec M, Kavemann K, Aust W, Wulf K

出版信息

Arch Dermatol Res. 1980;268(2):191-206. doi: 10.1007/BF00403803.

Abstract

This report deals with a case of paraproteinemia (IgG Kappa) associated with skin indurations and yellowish papules on the trunk, edema of the face, and crystals in the cornea. In addition to the absence of elastic fibers and reductions of collagen fibers, light microscopy revealed single and/or multinucleated dermal cells with slight streaky patterning of the cytoplasm. These cells contained some sudanophilic (the greatest quantities of sudanophilic substances were observed in the deep corium) and partly birefringent material. In particular after warming, numerous spherocrystals appeared, which give Maltese cross birefringence. The presence of cholesterol esters can be assumed. The light-microscopic localization of acid phosphatase (incubation in Gomori medium) coincides in part with the distribution of bifringent lipids. Electron microscopy showed the cells with streaky contents to be chiefly histiocytes with numerous cristalline bodies in their cytoplasm. The cristalloids with a periodic inner structure are always surrounded by a single membrane. Identical bodies can be detected in endothelial cells of capillaries and epidermal keratinocytes. After pronase P incubation they disappear completely. We assume that the deposits correspond to phagocytized paraprotein. In addition, some histiocytic cells contain numerous lipid droplets in their cytoplasm. There is disintegration of some cells with crystalline inclusion, particularly in the deep corium. These cells show not only diffuse intracytoplasmic but also extracellular distribution of acid phosphatase activities. Particularly in the pericellular space of such cells numerous "banded structures" appear in connection with collagen degradation probably initiated by lysosomal enzymes. Amyloid could not be detected either light- or electron-microscopically.

摘要

本报告涉及一例副蛋白血症(IgG κ型),伴有躯干皮肤硬结、黄色丘疹、面部水肿及角膜晶体。光镜检查显示,除缺乏弹性纤维和胶原纤维减少外,还可见单个和/或多核真皮细胞,其细胞质有轻微条纹状图案。这些细胞含有一些嗜苏丹物质(在真皮深层观察到最多的嗜苏丹物质)和部分双折射物质。特别是在加温后,出现大量呈马耳他十字双折射的球晶体。可以推测存在胆固醇酯。酸性磷酸酶的光镜定位(在Gomori培养基中孵育)部分与双折射脂质的分布一致。电子显微镜显示,含有条纹状内含物的细胞主要是组织细胞,其细胞质中有许多晶体小体。具有周期性内部结构的类晶体总是被一层膜包围。在毛细血管内皮细胞和表皮角质形成细胞中可检测到相同的小体。经链霉蛋白酶P孵育后,它们完全消失。我们认为这些沉积物对应于被吞噬的副蛋白。此外,一些组织细胞的细胞质中含有大量脂滴。一些含有晶体包涵体的细胞发生崩解,特别是在真皮深层。这些细胞不仅显示酸性磷酸酶活性在细胞质内弥漫分布,也显示在细胞外分布。特别是在这些细胞的细胞周间隙中,出现许多与可能由溶酶体酶引发的胶原降解相关的“带状结构”。在光镜或电镜下均未检测到淀粉样物质。

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