Schubert K, Stein A, Meurer M
Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus der Technischen Universität Dresden, Germany.
Hautarzt. 2002 Jul;53(7):478-82. doi: 10.1007/s00105-001-0298-4.
Scleromyxedema is a rare mucinous disease without thyroid dysfunction, associated with a monoclonal gammopathy. The deposits of mucin in the skin and other organs such as the cardiovasculary system, determine the prognosis of the disease. A 74-year old patient with initial scleromyxedema is described, in whom a pulse therapy with melphalan was initiated already in the early stages of the disease. Four cycles of melphalan in combination with prednisolon led to a complete and sofar stable remission of the disease.
硬化性黏液水肿是一种罕见的无甲状腺功能障碍的黏液性疾病，与单克隆丙种球蛋白病相关。皮肤及其他器官（如心血管系统）中的黏蛋白沉积决定了该病的预后。本文描述了一名74岁的初发硬化性黏液水肿患者，在疾病早期即开始使用美法仑进行冲击治疗。四个周期的美法仑联合泼尼松龙治疗使疾病完全缓解且至今病情稳定。
