静脉注射免疫球蛋白治疗硬化性黏液水肿：伴双克隆丙种球蛋白病的硬化性黏液水肿急性加重
Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy.
作者信息
Manousaridis I, Loeser C, Goerdt S, Hassel J C
机构信息
Department of Dermatology, Venereology, and Allergology, Mannheim University Hospital, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, Mannheim, Germany.
出版信息
Acta Dermatovenerol Alp Pannonica Adriat. 2010 Dec;19(4):15-9.
Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy. The patient was successfully managed with intravenous immunoglobulin.
硬化性黏液水肿是一种罕见的慢性皮肤黏蛋白病，通常与单克隆丙种球蛋白病及潜在的全身性疾病相关。该病的病因尚不清楚。目前尚无标准治疗方法，对各种治疗方式的反应也各不相同。我们报告一例63岁男性难治性硬化性黏液水肿病例，其存在双克隆IgG和IgM λ型丙种球蛋白病。该患者通过静脉注射免疫球蛋白成功得到治疗。
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