Krasagakis K, Zouboulis C C, Owsianowski M, Ramaker J, Trautmann C, Tebbe B, Orfanos C E
Department of Dermatology, Free University of Berlin, Germany.
Br J Dermatol. 1996 Sep;135(3):463-6.
Scleromyxoedema, a disseminated papular and sclerotic variant of lichen myxoedematosus, is a rare disease with a chronic progressive course, and little tendency towards spontaneous remission. The treatment of scleromyxoedema has been largely ineffective. Aggressive chemotherapeutic agents have been used, often leading to therapy-related morbidity and mortality. We report a 41-year-old woman with scleromyxoedema, associated with a monoclonal gammopathy of IgG-kappa type, whose condition almost completely cleared with 12 monthly sessions of extracorporeal photopheresis. The patient had previously not responded to isotretinoin, and chlorambucil with prednisolone.
硬化性黏液水肿是黏液水肿性苔藓的一种播散性丘疹和硬化变体，是一种病程慢性进展、很少有自发缓解倾向的罕见疾病。硬化性黏液水肿的治疗大多无效。已使用过积极的化疗药物，常导致与治疗相关的发病率和死亡率。我们报告一名41岁患有硬化性黏液水肿的女性，伴有IgG-κ型单克隆丙种球蛋白病，其病情在每月进行12次体外光分离置换术后几乎完全缓解。该患者此前对异维A酸、苯丁酸氮芥联合泼尼松龙均无反应。
