Macfarlane A W, Davenport A, Verbov J L, Goldsmith H J
Department of Dermatology, Royal Liverpool Hospital, U.K.
Br J Dermatol. 1987 Nov;117(5):653-7. doi: 10.1111/j.1365-2133.1987.tb07499.x.
A 34-year-old woman with scleromyxoedema failed to respond to initial treatment with cyclophosphamide or psoralen-UVA photochemotherapy (PUVA). She developed neurological symptoms which resolved after five 4 l plasma exchanges performed on successive days, together with pulse methylprednisolone therapy. Subsequent treatment was with oral prednisolone and cyclophosphamide. She had had no further episodes of neurological dysfunction 8 months after plasma exchange, and the appearance of the skin had improved almost to normal. The complications and difficulty in management of scleromyxoedema are discussed.
一名患有硬化性黏液水肿的34岁女性对环磷酰胺或补骨脂素-紫外线A光化学疗法（PUVA）的初始治疗无反应。她出现了神经症状，在连续5天每天进行4升血浆置换并联合脉冲甲基强的松龙治疗后症状得到缓解。后续治疗采用口服强的松龙和环磷酰胺。血浆置换8个月后，她未再出现神经功能障碍发作，皮肤外观几乎恢复正常。本文讨论了硬化性黏液水肿的并发症及管理难点。
