译者：范秋玉 校对：乔鹏燕 审核：张莉芸

Abstract：Scleromyxedema is a rare progressive cutaneous mucinosis, usually associated with a systemic involvement and paraproteinemia. Its aetiology remains unknown. The therapeutic options include numerous treatment modalities, however, no standard treatment exists as the rarity of this disease prevents the execution of controlled therapeutic trials. This paper reports a case of a 38-year-old male with progressive scleromyxedema associated with gammopathy. Initially, the patient was treated with prednisolone and later etretinate was added to the therapeutic schedule with quite good clinical improvement. However, after 6 months of treatment, several adverse effects were observed: hypercholesterolemia, hypertriglyceridaemia and cataract of the right eye. The patient was consulted by dermatologists in Warsaw and Gdansk as well as by a haematologist. The patient was excluded from oncological treatment. Melphalan therapy was not recommended as it is associated with very toxic side effects. IVIG treatment (intravenous immunoglobulin) was not initiated because of financial issues. As the disease progressed, treatment with plasmapheresis was introduced. The patient received 4 cycles of the therapy. It was well-tolerated by the patient and gave satisfactory, but temporary results. In order to obtain long-lasting improvement the patient was treated with IVIG (21.0 g/dose for 5 consecutive days). This treatment modality seems to have resulted in a more stable improvement.

摘要：硬化性粘液性水肿是一种病因不明、罕见、进展性的皮肤粘蛋白病，常出现多系统受累和副蛋白血症。该病治疗方案多种多样，但是没有一种标准治疗方案能很好的控制本病的发展。本文报道的是1例38岁女性，患有进展性硬化性粘液性水肿合并丙种球蛋白病。该患者起初用泼尼松片和阿维A酯进行治疗，有很好的临床疗效。然而，在治疗6个月后，出现了高胆固醇血症、高甘油三酯血症和右眼白内障等不良症状。本例的诊断得到了皮肤病学专家和血液学专家的认证。且患者没有用抗肿瘤治疗药物。马兰法毒副作用太大，不推荐使用。因为经济原因，静脉注射丙种球蛋白并未在初期使用。随着疾病的进展，该患者接受了4个周期的血浆置换，患者的耐受性较好，达到了很好的治疗效果，但是病情并未得到长期的控制。为了长期改善患者病情，我们给予该患者静脉注射丙种球蛋白21g/日，连续5天。这种治疗方案似乎能使病情得到长期缓解。

引自：Koronowska SK1, Osmola-Mańkowska A, Jakubowicz O. Scleromyxedema: a rare disorder and its treatment difficulties.Postepy Dermatol Alergol. 2013 Apr;30(2):122-126.

此文章内容仅代表医生观点，仅供参考。涉及用药、治疗等问题请到当地医院就诊，谨遵医嘱！