M蛋白相关罕见皮肤病变四例  

Four Cases of the Rare Skin Manifestation: Monoclonal-Proteinemia

作　　者：金相红 徐俊卿 杨辰[1] 朱铁楠[1] 庄俊玲[1] JIN Xianghong;XU Junqing;YANG Chen;ZHU Tienan;ZHUANG Junling(Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Hematology,Yantai Yuhuangding Hospital Affiliated to Qingdao University,Yantai 264000,China)

机构地区：[1]中国医学科学院北京协和医院血液内科,北京100730 [2]中国医学科学院北京协和医学院,北京100730 [3]青岛大学附属烟台毓璜顶医院血液内科,烟台264000

出　　处：《罕见病研究》2022年第4期440-445,共6页Journal of Rare Diseases

基　　金：中央高水平医院临床科研业务费(2022-PUMCH-B-048);首都卫生发展科研专项基金-自主创新类(2022-2-4013)。

摘　　要：M蛋白相关皮肤病变较为罕见,在意义未明的单克隆丙种球蛋白血症(MGUS)、冒烟性骨髓瘤(SMM)或多发性骨髓瘤(MM)患者中均可出现。本文报道了4例合并罕见皮肤异常的M蛋白血症患者,包括坏疽性脓皮病(PG)、持久性隆起性红斑(EED)、皮肤松弛症(CL)和黏液水肿性苔藓(LM)。各种皮损表现相对特异,机制主要是免疫介导的副瘤综合征,并非浆细胞直接浸润。抗浆细胞病治疗对皮肤病变有效。MM患者转归主要取决于肿瘤控制,而MGUS和SMM相关皮肤损害预后较好。皮肤病变在M蛋白相关临床表现中罕见,鲜被认知,同时严重影响生活质量,这4例患者的治疗经验值得借鉴。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, whereasthe 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.

关 键 词：M蛋白血症 皮肤病变 坏疽性脓皮病 持久性隆起性红斑 皮肤松弛症 黏液水肿性苔藓 

分 类 号：R758[医药卫生—皮肤病学与性病学] R55[医药卫生—临床医学]