沙利度胺治疗3例顽固性硬化性黏液水肿

Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients.

作者信息

Sansbury Julia C, Cocuroccia Barbara, Jorizzo Joseph L, Gubinelli Emanuela, Gisondi Paolo, Girolomoni Giampiero

机构信息

Department of Dermatology and Pathology, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA.

出版信息

J Am Acad Dermatol. 2004 Jul;51(1):126-31. doi: 10.1016/j.jaad.2004.01.058.

DOI:10.1016/j.jaad.2004.01.058

PMID:15243538

Abstract

Scleromyxedema is a generalized, papular, and sclerodermoid form of lichen myxedematosus associated with monoclonal gammopathy and systemic changes. Despite anecdotal reports of success with various agents, no satisfactory treatments are currently available. We report 3 adult patients with recalcitrant scleromyxedema associated with paraproteinemia who were treated with thalidomide. All 3 patients had marked improvement of the skin lesions and joint mobility after the first 2 months of therapy, with further amelioration after 4 months, and reduction in paraprotein levels.

摘要

硬化性黏液水肿是黏液水肿性苔藓的一种全身性、丘疹性和硬皮病样形式，与单克隆丙种球蛋白病和全身改变相关。尽管有关于各种药物治疗成功的轶事报道，但目前尚无令人满意的治疗方法。我们报告了3例与副蛋白血症相关的顽固性硬化性黏液水肿成年患者，他们接受了沙利度胺治疗。所有3例患者在治疗的前2个月后皮肤病变和关节活动度均有明显改善，4个月后进一步改善，副蛋白水平降低。