Dezoteux F, Bourges M, Lionet A, Carpentier O
Service de dermatologie, hôpital Claude-Huriez, CHRU de Lille, 2, avenue Oscar-Lambret, Lille, France.
Service d'imagerie médicale, hôpital Victor-Provo, 11, boulevard Lacordaire, 59170 Roubaix, France.
Rev Med Interne. 2018 May;39(5):360-363. doi: 10.1016/j.revmed.2018.02.018. Epub 2018 Mar 12.
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.
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.
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.
成人硬肿病（布施克硬肿病）是一种病因不明的皮肤黏蛋白沉积症，临床特征为皮肤弥漫性硬化，通常累及颈部、肩部和背部，这限制了患者的活动能力。
我们报告一例50岁女性，患有慢性硬皮病样综合征2年，伴有1型糖尿病。体格检查发现肩部、颈部和背部广泛皮肤硬化。组织学检查确诊为成人硬肿病。患者接受体外光化学疗法（EPP），每月两次，共两个月。随访时，两个月后活动能力得到显著改善。在长期维持治疗期间，EPP的有益效果得以保持，治疗间隔时间拉长。
EPP是一种治疗布施克硬肿病的非常规疗法。我们描述了一例成功用EPP治疗的布施克硬肿病病例，这可能代表了硬肿病治疗的一种选择。
