Tschen J A, Chang J R
St. Joseph's Hospital, Baylor College of Medicine, Houston, Texas, USA.
J Am Acad Dermatol. 1999 Feb;40(2 Pt 2):303-7. doi: 10.1016/s0190-9622(99)70471-8.
Scleromyxedema is a variant of papular mucinosis characterized by fibroblast proliferation and mucin deposition in the dermis. Historically, it has been very difficult to treat and can cause significant morbidity and mortality with systemic involvement. We describe a case of a woman with scleromyxedema and systemic manifestations treated with interferon alfa. Her skin responded very well to therapy within 3 months; however, her systemic manifestations showed little change. We conclude that interferon alfa may be a useful therapy for patients with scleromyxedema, particularly if the disease process is limited to the skin.
硬化性黏液水肿是丘疹性黏蛋白病的一种变体，其特征为成纤维细胞增殖和真皮内黏蛋白沉积。从历史上看，该病一直很难治疗，并且在出现全身受累时可导致显著的发病率和死亡率。我们描述了一例患有硬化性黏液水肿并有全身表现的女性患者，其接受了干扰素α治疗。她的皮肤在3个月内对治疗反应良好；然而，她的全身表现几乎没有变化。我们得出结论，干扰素α可能是治疗硬化性黏液水肿患者的一种有效疗法，特别是当疾病过程仅限于皮肤时。
