Victor Varun, Margareat Rashmi Maria
Department of General Medicine, St. John's Medical College, Bengaluru, Karnataka, India.
Indian Dermatol Online J. 2019 Jan-Feb;10(1):50-53. doi: 10.4103/idoj.IDOJ_135_18.
Scleromyxedema is a rare progressive cutaneous mucinosis of unknown etiology with equal prevalence in both men and women. It is usually associated with monoclonal gammopathy in most of the cases. Various treatment modalities have been tried for scleromyxedema including steroids, intravenous immunoglobulin (IVIg), autologous hematopoietic stem cell transplantation, and melphalan, but none has proved to be fully effective. This paper reports a case of scelromyxedema in a patient who was found to have multiple myeloma on further evaluation. The patient was treated with six cycles of bortezomib, thalidomide, and dexamethasone, following which he had complete resolution of his skin lesions. While recent case reports have mentioned treatment of patients of scleromyxedema with varying combinations of IVIg, thalidomide, bortezomib, and dexamethasone, we describe a patient who has been successfully treated with a combination of bortezomib, thalidomide, and dexamethasone with complete resolution of all skin lesions.
硬化性黏液水肿是一种病因不明的罕见进行性皮肤黏蛋白病，男女患病率相等。在大多数病例中，它通常与单克隆丙种球蛋白病相关。针对硬化性黏液水肿已经尝试了多种治疗方法，包括类固醇、静脉注射免疫球蛋白（IVIg）、自体造血干细胞移植和美法仑，但没有一种方法被证明是完全有效的。本文报告了一例硬化性黏液水肿患者，该患者在进一步评估中被发现患有多发性骨髓瘤。该患者接受了六个周期的硼替佐米、沙利度胺和地塞米松治疗，之后其皮肤病变完全消退。虽然最近的病例报告提到了用IVIg、沙利度胺、硼替佐米和地塞米松的不同组合治疗硬化性黏液水肿患者，但我们描述了一名成功接受硼替佐米、沙利度胺和地塞米松联合治疗且所有皮肤病变完全消退的患者。
