Photophérèse extracorporelle

  • 文章类型: Case Reports
    背景:Scleedemaadultorum(Buschke\'sscleredema)是一种未知来源的皮肤粘液性病,临床特征为通常累及颈部的皮肤弥漫性硬化,肩膀和背部,这限制了患者的活动能力。
    方法:我们报告了一例50岁女性患者,该患者出现慢性硬皮病样综合征2年并伴有1型糖尿病。体格检查显示肩膀有广泛的皮肤硬结,脖子和背部。组织学检查证实了硬肿症的诊断。该患者每月接受两次体外光置换(EPP)治疗,持续两个月。在后续行动中,两个月后,流动性大大提高。EPP的有益效果长期维持,而会议间隔。
    结论:EPP是一种非常规治疗Buschke的硬肿症。我们描述了一例成功用EPP治疗的Buschke的硬肿症,这可能是治疗硬肿症的一种治疗选择。
    BACKGROUND: Scleredema adultorum (Buschke\'s scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients\' mobility.
    METHODS: We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Histologic examination confirmed the diagnosis of scleredema adultorum. The patient was treated with extracorporeal photopheresis (EPP) twice a month for two months. At follow-up, mobility was highly improved after two months. Beneficial effect of EPP was maintained on the long term while sessions were spaced.
    CONCLUSIONS: EPP is an unconventional treatment of Buschke\'s scleredema. We described a case of Buschke\'s scleredema successfully treated with EPP which may represent a therapeutic option for the treatment of scleredema.
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