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Long-term efficacy of high doses of intravenous immunoglobulins in generalized scleromyxoedema: Case report

机译:大剂量静脉注射免疫球蛋白治疗广泛性巩膜粘膜浮肿的长期疗效:病例报告

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摘要

Scleromyxoedema is a rare disease with a progressive and disabling course involving dermal deposition of mucin and fibroblast proliferation; it is characterized clinically by a diffuse papular eruption, skin thickening, oedema and decreased skin flexibility, especially of the face and hands. Current therapy options are based on evidence from a limited number of case reports. The clinical manifestations and treatment of a 64-year-old man affected by scleromyxoedema with severe skin involvement of the face, arms and hands, decreased mouth opening and hypomotility of the fingers are reported. Dysphagia, asthenia and immunoglobulin G lambda monoclonal gammopathy were also present. Previous treatment with topical and systemic corticosteroids, psoralen plus ultraviolet A radiation therapy, plasmapheresis, extracorporeal photochemotherapy, hydroxychloroquine and cyclophosphamide had been unsuccessful. Treatment with intravenous immunoglobulins at a dosage of 2 g/kg monthly was started. Considerable improvements were observed after seven cycles of therapy, with recovery of skin elasticity, an increase in facial mimic movement, restoration of joint function and improvement in the modified Rodnan score. There were no observed side-effects. The patient remains in remission on monthly maintenance intravenous immunoglobulins, 2 years after initial treatment.
机译:巩膜水肿是一种罕见的疾病,其病情进展且致残,涉及粘蛋白的真皮沉积和成纤维细胞增殖。其临床特征是弥漫性丘疹喷发,皮肤增厚,浮肿和皮肤柔韧性下降,尤其是面部和手部。当前的治疗选择基于少数病例报告中的证据。据报道,一名患有巩膜水肿的64岁男子的临床表现和治疗方法是面部,手臂和手部皮肤严重受累,嘴巴张开和手指运动不足。还存在吞咽困难,乏力和免疫球蛋白Gλ单克隆丙种球蛋白病。先前使用局部和全身性皮质类固醇,补骨脂素加紫外线A的放射疗法,血浆置换术,体外光化学疗法,羟氯喹和环磷酰胺均未成功。开始以每月2 g / kg的剂量静脉注射免疫球蛋白治疗。经过七个疗程的治疗,皮肤弹性得到恢复,面部模仿运动增加,关节功能恢复,Rodnan评分提高,治疗效果得到显着改善。没有观察到副作用。初始治疗后两年,患者每月维持静脉注射免疫球蛋白仍可缓解。

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