PDF(Pubmed)
Abstract：
Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly documented. High-dose intravenous immunoglobulin (IVIG), used either alone or in conjunction with systemic steroids and/or thalidomide, has been suggested as a first-line treatment. We report the case of a 45-year-old woman diagnosed with scleromyxedema with paraproteinemia that initially did not respond to systemic steroids, retinoids, and thalidomide but greatly improvement in terms of systemic and cutaneous symptoms after treatment with IVIG.
摘要：
巩膜粘液性水肿是一种罕见的特发性纤维粘液性疾病，其特征是全身性丘疹和硬皮样皮肤喷发。患者常有足原蛋白血症和皮外，甚至是致命的,表现。然而，硬粘菌水肿的预后和治疗特征却鲜有记载。大剂量静脉注射免疫球蛋白（IVIG），单独使用或与全身性类固醇和/或沙利度胺联合使用，已被建议作为一线治疗。我们报告了一例45岁女性，诊断为硬粘菌水肿伴副蛋白血症，最初对全身性类固醇无反应，类维生素A，和沙利度胺，但用IVIG治疗后在全身和皮肤症状方面有很大改善。
