Kulczycki A, Nelson M, Eisen A, Heffernan M
Division of Allergy and Immunology, Department of Medicine, Washington School of Medicine. St Louis, MO, U.S.A.
Br J Dermatol. 2003 Dec;149(6):1276-81. doi: 10.1111/j.1365-2133.2003.05682.x.
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-lambda 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.
硬化性黏液水肿是一种罕见疾病，其特征为皮肤硬化、黏蛋白沉积和副蛋白血症。常伴有内科疾病，尤其是肌肉骨骼、胃肠道和中枢神经系统受累。我们报告了连续3例接受大剂量静脉注射免疫球蛋白（hdIVIg）治疗的硬化性黏液水肿患者。这3例患者的高碱性IgG-λ副蛋白水平相对较低，且均表现出皮肤和皮肤外疾病对hdIVIg的持续反应。由于所有患者均有口周皮肤受累和小口畸形，皮肤改善的一个指标是切牙间距离增加。改善的皮肤外硬化性黏液水肿表现包括输尿管狭窄、发声强度和吞咽困难。
