D'Incan M, Franck F, Kanold J, Bacin F, Achin R, Beyvin A J, Demeocq F, Souteyrand P
Service de Dermatologie, Hôtel-Dieu, Clermont-Ferrand.
Ann Dermatol Venereol. 2001 Jan;128(1):38-41.
Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance.
A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up).
There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.
硬化性黏液水肿是由黏蛋白浸润皮肤所致，通常与意义未明的单克隆丙种球蛋白病相关。
一名46岁女性因硬化性黏液水肿伴双侧黄斑水肿及限制性肺综合征入院。在进行12次体外光化学疗法疗程及4次泼尼松龙冲击治疗后（随访17个月），皮肤反应基本完全，眼部及肺部症状完全缓解。
对于硬化性黏液水肿的治疗指南尚无共识。通常使用类固醇和马法兰。然而，这些药物会引发严重的副作用。由于缺乏关于硬化性黏液水肿所用不同药物疗效的对照研究，光化学疗法的主要优势在于该治疗方法的安全性。
