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获得性皮肤松弛症与单克隆丙种球蛋白血症之间关系的超微结构显示。

Ultrastructural demonstration of a relationship between acquired cutis laxa and monoclonal gammopathy.

机构信息

Department of Dermatology, Université François Rabelais, Tours, France.

出版信息

Acta Derm Venereol. 2010 Jul;90(4):406-8. doi: 10.2340/00015555-0887.

Abstract

Acquired cutis laxa is an uncommon disorder sometimes associated with monoclonal gammopathy and multiple myeloma, although the mechanism of this link is unclear. We report here a case of a 34-year-old man with generalized acquired cutis laxa and monoclonal light chain disease with renal and neurological involvement. Electron microscopy examination of a skin sample revealed shortened and fragmented elastic fibres in the reticular dermis and normal collagen bundles. Immunogold labelling revealed anti-lambda antibodies closely bound to the microfibrillar component of elastic fibres, thus supporting a causal relationship between monoclonal gammopathy and the changes in skin elasticity.

摘要

获得性皮肤松弛症是一种不常见的疾病,有时与单克隆丙种球蛋白病和多发性骨髓瘤有关,尽管其机制尚不清楚。我们在此报告一例 34 岁男性,患有全身性获得性皮肤松弛症和单发性轻链病,伴有肾和神经受累。皮肤样本的电子显微镜检查显示网状真皮中的弹性纤维缩短和碎片化,以及正常的胶原束。免疫金标记显示抗 lambda 抗体与弹性纤维的微纤维成分紧密结合,从而支持单克隆丙种球蛋白病与皮肤弹性变化之间的因果关系。

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