Nieves D S, Bondi E E, Wallmark J, Raps E C, Seykora J T
University of Pennsylvania, Philadelphia, USA.
Cutis. 2000 Feb;65(2):89-92.
Scleromyxedema is a rare systemic disorder characterized by cutaneous sclerosis and papulosis, accompanied by deposition of mucin in the skin and other organs. We describe a case of scleromyxedema in a 62-year-old man. The cutaneous symptoms of the disorder were preceded by episodes of acute central nervous system dysfunction that included mental confusion, hemiparesis, tremor, and migraine. As the cutaneous symptoms progressed, the patient experienced persistent confusion and difficulty concentrating. Therapy with melphalan and plasmapheresis led to complete resolution of the cutaneous symptoms as well as near-resolution of the neurologic symptoms. This is the first report to describe the successful treatment of the cutaneous symptoms of scleromyxedema accompanied by reversal of chronic neurologic dysfunction.
硬化性黏液水肿是一种罕见的系统性疾病，其特征为皮肤硬化和丘疹病，同时伴有黏蛋白在皮肤及其他器官沉积。我们报告一例62岁男性硬化性黏液水肿病例。该疾病的皮肤症状出现之前，患者曾有急性中枢神经系统功能障碍发作，包括精神错乱、偏瘫、震颤和偏头痛。随着皮肤症状进展，患者出现持续精神错乱和注意力难以集中的情况。美法仑治疗和血浆置换使皮肤症状完全消退，神经症状也几乎完全缓解。这是首例描述成功治疗硬化性黏液水肿皮肤症状并逆转慢性神经功能障碍的报告。
