罕见病研究 (Oct 2022)

Four Cases of the Rare Skin Manifestation: Monoclonal-Proteinemia

  • JIN Xianghong,
  • XU Junqing,
  • YANG Chen,
  • ZHU Tienan,
  • ZHUANG Junling

DOI
https://doi.org/10.12376/j.issn.2097-0501.2022.04.013
Journal volume & issue
Vol. 1, no. 4
pp. 440 – 445

Abstract

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The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.

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