Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China.
Front Immunol. 2023 Jan 13;13:1099918. doi: 10.3389/fimmu.2022.1099918. eCollection 2022.
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.
硬肿性黏液水肿是一种罕见的特发性纤维黏蛋白病，其特征为全身性丘疹样和硬皮病样皮肤疹。患者常伴有副蛋白血症和皮肤外，甚至是致命的表现。然而，硬肿性黏液水肿的预后和治疗特征记录不佳。高剂量静脉注射免疫球蛋白（IVIG），无论是单独使用还是与全身性皮质类固醇和/或沙利度胺联合使用，都被建议作为一线治疗。我们报告了一例 45 岁女性，诊断为硬肿性黏液水肿伴副蛋白血症，最初对全身性皮质类固醇、类视黄醇和沙利度胺无反应，但在接受 IVIG 治疗后，全身和皮肤症状均显著改善。
