Dinneen A M, Dicken C H
Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
J Am Acad Dermatol. 1995 Jul;33(1):37-43. doi: 10.1016/0190-9622(95)90007-1.
Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality.
Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents.
A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted.
Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients.
The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.
硬化性黏液水肿是一种罕见的纤维黏液性疾病，通常难以治疗，且与显著的发病率和死亡率相关。
我们的目的是研究该疾病的自然病程及其对烷化剂治疗的反应。
对26例硬化性黏液水肿患者进行临床病理回顾，并记录皮肤外表现及对烷化剂治疗的反应。
26例患者中有20例出现皮肤外表现，最常见于胃肠道。26例患者中有23例发现异常副蛋白，最常见的是IgG-λ（18例患者）。17例患者使用美法仑进行治疗。10例接受治疗的患者病情最终致命。
硬化性黏液水肿的总体预后较差。治疗困难。虽然烷化剂可能在短期内证明有益，但长期使用这些药物的显著毒性是明显的。
