CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Classical Complement Activation in Light and Heavy Chain Deposition Disease with Acquired Cutis Laxa and Bronchiolitis Obliterans: A Case Report of Monoclonal Gammopathy of Clinical Significance
Provisionally accepted
Yuxiong Chen
Yue FanShoudong Yang
Haoyuan CuiWei YeWenling YeYubing Wen
Junling Zhuang
Ping Wang
Dong-Lai MaKai FangPeng Xia
Limeng ChenHang Li*
Chao Li*- Peking Union Medical College Hospital (CAMS), Beijing, China
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Light and heavy chain deposition disease (LHCDD) is a clonal plasma cell or monoclonal B-cell dyscrasia characterized by deposition of monoclonal immunoglobulin light and heavy chains. LHCDD mainly belongs to monoclonal gammopathy of renal significance (MGRS), including a spectrum of kidney disorders caused by a monoclonal protein (M-protein) secreted by a small plasma cell clone or other B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma or other B-cell malignancies. It may also occur as a renal complication of overt multiple myeloma. We report a 27-year-old man who presented clinically with chronic nephritic syndrome and was diagnosed with LHCDD confirmed by renal biopsy, accompanied by hypocomplementemia and bronchiolitis obliterans (BO). Notably, he initially developed acquired cutis laxa (CL) four years Running Title before renal dysfunction. Progressive dermatologic manifestations prompted repeat skin biopsies, revealing deposition of γ1 heavy chains, restrictive lambda light chains and complement components (C3, C4 and C1q) along dermal elastic fibers, establishing monoclonal gammopathy of dermatologic significance (MGODS) before systemic involvement. This case illustrates a rare constellation of MGRS, MGODS, and BO in a young adult and provides unique histologic and serologic evidence of classical complement pathway activation. Our findings support a potential immune-mediated mechanism underlying tissue injury in both renal and extrarenal manifestations of monoclonal gammopathy, highlighting the diagnostic value of early tissue biopsy and the importance of complement assessment in such cases.
Keywords: case report, Light and heavy chain deposition disease, Acquired cutis laxa, Bronchiolitis Obliterans, classical complement activation
Received: 06 Oct 2025; Accepted: 20 Nov 2025.
Copyright: © 2025 Chen, Fan, Yang, Cui, Ye, Ye, Wen, Zhuang, Wang, Ma, Fang, Xia, Chen, Li and Li. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Hang Li, lihang9@hotmail.com
Chao Li, superchad099@hotmail.com
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