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Abstract：
BACKGROUND: Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date.
METHODS: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud\'s phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient\'s diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition.
CONCLUSIONS: Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.
METHODS: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud\'s phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient\'s diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition.
CONCLUSIONS: Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.
摘要：
背景：Scleedema是一种罕见的硬皮样皮肤病，其特征是身体上部弥漫性对称增厚。最近描述了其与单克隆丙种球蛋白病和骨髓瘤的关联；迄今为止报道的病例很少。
方法：一名66岁的斯里兰卡妇女，在皮肤科接受随访34年，患有弥漫性系统性硬化症，出现皮肤病急性加重。没有雷诺现象；硬直；特征性肺，胃肠,和系统性硬化症的心脏受累；以及反复阴性的抗核抗体测试结果导致对硬肿症可能性的重新评估。来自四个身体部位的皮肤活检显示正常的表皮和增厚的网状真皮，肿胀的胶原蛋白束通过清晰的空间彼此分离，导致开窗。皮肤附件没有萎缩或束缚。Alcian蓝染色显示间质粘蛋白沉积。血清蛋白电泳显示β区的异常单克隆带，副蛋白水平为8.9g/dl。免疫固定在由免疫球蛋白A和κ组成的γ区显示异常条带。骨髓活检显示单克隆浆细胞异常（15％），多核。没有证据表明终末器官受损,全身磁共振成像没有发现任何骨受累的证据.患者的诊断被修改为与IgA-κ相关的2型硬肿症，她被转诊到血液肿瘤科医生那里接受化疗，这导致皮肤状况的显著改善。
结论：Scleedema是一种罕见的疾病，与单克隆丙种球蛋白病的罕见关联。皮肤科医生应该意识到这种罕见但重要的联系。
方法：一名66岁的斯里兰卡妇女，在皮肤科接受随访34年，患有弥漫性系统性硬化症，出现皮肤病急性加重。没有雷诺现象；硬直；特征性肺，胃肠,和系统性硬化症的心脏受累；以及反复阴性的抗核抗体测试结果导致对硬肿症可能性的重新评估。来自四个身体部位的皮肤活检显示正常的表皮和增厚的网状真皮，肿胀的胶原蛋白束通过清晰的空间彼此分离，导致开窗。皮肤附件没有萎缩或束缚。Alcian蓝染色显示间质粘蛋白沉积。血清蛋白电泳显示β区的异常单克隆带，副蛋白水平为8.9g/dl。免疫固定在由免疫球蛋白A和κ组成的γ区显示异常条带。骨髓活检显示单克隆浆细胞异常（15％），多核。没有证据表明终末器官受损,全身磁共振成像没有发现任何骨受累的证据.患者的诊断被修改为与IgA-κ相关的2型硬肿症，她被转诊到血液肿瘤科医生那里接受化疗，这导致皮肤状况的显著改善。
结论：Scleedema是一种罕见的疾病，与单克隆丙种球蛋白病的罕见关联。皮肤科医生应该意识到这种罕见但重要的联系。
