Abstract：
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.
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.
摘要：
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。
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。
