Majeski Candace, Taher Muba, Grewal Parbeer, Dytoc Marlene, Lauzon Gilles
University Dermatology Centre, University of Alberta, Edmonton, AB, Canada.
J Cutan Med Surg. 2005 Jun;9(3):99-104. doi: 10.1007/s10227-005-0137-9.
Scleromyxedema is a clinical variant of the rare disease papular mucinosis that has both cutaneous and systemic manifestations. Treatment options are numerous and tend to be associated with serious potential side effects and frequent relapse.
We report a case of scleromyxedema treated with low-dose oral prednisone and intravenous immunoglobulin (IVIg). This is followed by a review of the literature.
IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.
硬化性黏液水肿是一种罕见疾病丘疹性黏蛋白病的临床变体，具有皮肤和全身表现。治疗选择众多，且往往伴有严重的潜在副作用和频繁复发。
我们报告一例用低剂量口服泼尼松和静脉注射免疫球蛋白（IVIg）治疗的硬化性黏液水肿病例。随后对文献进行综述。
IVIg正被用于越来越多的炎症和免疫性疾病。越来越多的报道称其是硬化性黏液水肿的成功治疗方法。尽管我们的患者最终死于该疾病，但泼尼松和IVIg联合治疗使病情在症状、实验室检查及临床方面得到了暂时改善。因此，优化这一治疗策略对硬化性黏液水肿的管理具有指导意义。
