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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.

DOI:10.2340/00015555-0887
PMID:20574607
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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Ultrastructural demonstration of a relationship between acquired cutis laxa and monoclonal gammopathy.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。
Acta Derm Venereol. 2010 Jul;90(4):406-8. doi: 10.2340/00015555-0887.
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Acquired cutis laxa with an interstitial granulomatous reaction associated with IgG lambda monoclonal gammopathy.获得性皮肤松弛症伴间质肉芽肿反应,与IgG λ单克隆丙种球蛋白病相关。
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引用本文的文献

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Neutrophil Extracellular Traps as a Possible Pathomechanism of Generalized Acquired Cutis Laxa Associated with IgA-lamda Monoclonal Gammopathy of Undetermined Significance.中性粒细胞胞外诱捕网作为意义未明的IgA-λ单克隆丙种球蛋白病相关的泛发性获得性皮肤松弛症的一种可能发病机制
Acta Derm Venereol. 2021 Sep 3;101(9):adv00536. doi: 10.2340/00015555-3894.
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Acquired Localized Cutis Laxa due to Increased Elastin Turnover.因弹性蛋白周转率增加导致的获得性局限性皮肤松弛症。
Case Rep Dermatol. 2016 Feb 13;8(1):42-51. doi: 10.1159/000443696. eCollection 2016 Jan-Apr.