M蛋白相关罕见皮肤病变四例

Four Cases of the Rare Skin Manifestation: Monoclonal-Proteinemia

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.