Shalhout Sophia Z, Nahas Myrna R, Drews Reed E, Miller David M
Division of Hematology/Oncology and Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Division of Hematology/Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.
Case Rep Dermatol Med. 2020 Feb 12;2020:7480607. doi: 10.1155/2020/7480607. eCollection 2020.
Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis, systemic lupus erythematosus, and plasma-cell dyscrasias. . We report an unusual case of a 35-year-old male with progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory eruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His cutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by aortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone and high-dose prednisone, respectively, with no improvement to his cutis laxa.
The presence of monoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new term for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance, or MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including scleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that exemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of dermatological significance.
皮肤松弛症是一种罕见的皮肤病，可遗传或后天获得，临床特征为皮肤松弛、起皱且多余，弹性降低。这种异质性结缔组织疾病可局限或泛发，有或无内脏表现。泛发性皮肤松弛症通常呈头向尾进展，病因包括炎性皮肤疹、药物和感染。皮肤松弛症还与其他几种疾病相关，包括类风湿关节炎、系统性红斑狼疮和浆细胞发育异常。我们报告一例不寻常病例，一名35岁男性，泛发性获得性皮肤松弛症和血管炎在一年内进展。在其皮肤弹性降低之前或同时没有皮肤炎性疹，皮肤活检显示弹性组织离解。他的皮肤表现导致进行全身评估，最终诊断为冒烟型多发性骨髓瘤伴主动脉炎和贫血。他的骨髓瘤和血管炎分别成功用环磷酰胺、硼替佐米和地塞米松以及大剂量泼尼松治疗，但皮肤松弛症无改善。
单克隆丙种球蛋白病的存在与几种皮肤病实体密切相关，如获得性皮肤松弛症。我们提出一个新术语来描述由副蛋白血症引起的皮肤表现：具有皮肤学意义的单克隆丙种球蛋白病（MGODS），并强调在某些皮肤病情况下，包括硬化性黏液水肿和淀粉样变性，对潜在丙种球蛋白病的评估。我们展示一例继发于浆细胞发育异常的新获得性皮肤松弛症病例，该病例例证了MGODS，同时进行简要文献综述，并强调具有皮肤学意义的单克隆丙种球蛋白病的临床相关性。
