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[浆细胞瘤相关的皮肤大疱性出血性淀粉样变]

[Plasmocytoma-associated bullous hemorrhagic amyloidosis of the skin].

作者信息

Hödl S, Turek T D, Kerl H

出版信息

Hautarzt. 1982 Oct;33(10):556-8.

PMID:6759462
Abstract

Case report of a 70-year-old woman with plasmocytoma-associated bullous "amyloid purpura" and a lambda light-chain IgA paraproteinemia. Cutaneous hemorrhage with blisters, erosions, and milia in the intertriginous areas were the main clinical features. Amyloid deposits in the walls of blood vessels, and the pericollagenous affinity of the amyloid in the dermis are likely to be responsible for the increased capillary fragility, i.e., the hemorrhagic tendency and the infrapapillary blisters. A differentiation from epidermolysis bullosa acquisita and porphyria cutanea tarda is possible by means of the histological level of the blisters.

摘要

一名70岁女性患有浆细胞瘤相关的大疱性“淀粉样紫癜”及λ轻链IgA型副蛋白血症的病例报告。皮肤褶皱部位出现伴有水疱、糜烂和粟丘疹的皮肤出血是主要临床特征。血管壁中的淀粉样沉积以及真皮中淀粉样物质对胶原周围的亲和力可能是导致毛细血管脆性增加的原因,即出血倾向和乳头下水疱形成。通过水疱的组织学层面可以与获得性大疱性表皮松解症和迟发性皮肤卟啉症相鉴别。

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