皮肤轻链沉积病：2例报告并文献复习
Cutaneous Light Chain Deposition Disease: A Report of 2 Cases and Review of the Literature.
作者信息
Hendricks Carlo, Fernández Figueras Maite T, Liersch Julia, Martin-Urdà Maria-Teresa, López Dolores, Brochhausen Christoph, Röcken Christoph, Schaller Jörg
机构信息
Dermatopathologie Duisburg, Duisburg, Germany.
Cap d'Area de Patologia Quirúrgica, Anatomia Patològica, Hospital Universitari General de Catalunya-Grupo Quirón Salud, Universitat Autònoma de Barcelona, Barcelona, Spain.
出版信息
Am J Dermatopathol. 2018 May;40(5):337-341. doi: 10.1097/DAD.0000000000000991.
Light chain deposition disease (LCDD) is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. Renal involvement with rapidly progressing renal failure presents the dominant manifestation of LCDD. Approximately 20%-30% of patients show symptomatic cardiac or liver involvement. Cutaneous manifestations are extremely rare with only a few published cases. We report 2 additional cases of cutaneous LCDD without detectable systemic disease.
轻链沉积病（LCDD）是一种罕见的系统性疾病，主要是单克隆无定形非淀粉样轻链沉积于多个器官。肾脏受累并迅速进展为肾衰竭是LCDD的主要表现。约20%-30%的患者出现有症状的心脏或肝脏受累。皮肤表现极为罕见，仅有少数病例报道。我们报告另外2例无可检测到的系统性疾病的皮肤LCDD病例。
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