Scleromyxoedema: treatment of cutaneous and systemic manifestations with high‐dose intravenous immunoglobulin
Scleromyxoedema: treatment of cutaneous and systemic manifestations with high‐dose intravenous immunoglobulin
复制标题
硬化粘液水肿：用高剂量静脉注射免疫球蛋白治疗皮肤和全身表现
DOI：
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发表时间：
2003
影响因子：
10.3
通讯作者：
M. Heffernan
中科院分区：
文献类型：
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作者：
A. Kulczycki;Matthew M. Nelson;A. Eisen;M. Heffernan
硬化黏水肿是一种罕见的疾病，其特征是皮肤硬化，粘蛋白沉积和副蛋白血症。内科疾病很常见，特别是肌肉骨骼、胃肠道和中枢神经系统受累。我们报告了一系列的三个连续的硬化粘液水肿患者治疗高剂量静脉注射免疫球蛋白（hdIVIg）。三名患者中的每一名都有相对较低水平的高碱性IgG-λ副蛋白，并且每一名患者都证明了他们的皮肤和皮外疾病对hdIVIg的持续应答。由于所有患者都有口周皮肤受累和小口畸形，皮肤改善的一个指标是增加切牙内距离。改善的硬化性粘液水肿的皮外表现包括输尿管狭窄、声音强度和吞咽困难。
Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high‐dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG‐λ paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.