Vafaei-Nodeh Saba, Masoudi Hamid
Department of Pathology and Laboratory Medicine, The University of British Columbia, Vancouver, BC, Canada.
St. Paul's Hospital, Vancouver, BC, Canada.
SAGE Open Med Case Rep. 2024 Dec 19;12:2050313X241307116. doi: 10.1177/2050313X241307116. eCollection 2024.
Light chain deposition disease is a rare condition associated with plasma cell dyscrasia and other lymphoproliferative disorders in which there is overproduction and deposition of non-amyloid light chains in various organs, leading to organ dysfunction. It is well-established that the majority of patients with light chain deposition disease exhibit renal involvement. Although awareness of extrarenal manifestations is increasing, cutaneous involvement has rarely been reported. Herein, we present a case of light chain deposition disease with cutaneous manifestations in the absence of any renal disease. A biopsy of the skin revealed amorphous eosinophilic material within the superficial dermis. Using special stains, immunohistochemistry, and direct immunofluorescence, the deposits were confirmed to be kappa light chains.
轻链沉积病是一种罕见疾病,与浆细胞发育异常及其他淋巴增殖性疾病相关,其特征为各种器官中出现非淀粉样轻链的过度产生和沉积,进而导致器官功能障碍。众所周知,大多数轻链沉积病患者会出现肾脏受累。尽管对肾外表现的认识在不断提高,但皮肤受累的报道却很少。在此,我们报告一例无任何肾脏疾病但有皮肤表现的轻链沉积病病例。皮肤活检显示真皮浅层有非晶形嗜酸性物质。通过特殊染色、免疫组织化学和直接免疫荧光检查,证实沉积物为κ轻链。