Engelen J W, Kooistra M P, Canninga-van Dijk M R, Toonstra J, Sigurdsson V
Afd. Dermatologie, Universitair Medisch Centrum Utrecht, Postbus 85.500, 3508 GA Utrecht.
Ned Tijdschr Geneeskd. 2003 Dec 6;147(49):2435-8.
A 58-year-old man with renal insufficiency, who was being treated by haemodialysis, developed progressive skin lesions. He had thickening and hardening of the skin at the extremities and swelling of the toes and fingers with flexion contractures. His face was not affected. Laboratory evaluation was unremarkable and a skin biopsy [table: see text] showed an increase of collagen and mucin, without an inflammatory infiltrate. These clinical features resemble a recently reported new disorder: nephrogenic fibrosing dermopathy. This disorder manifests as scleromyxedema-like cutaneous skin lesions without associated paraproteinemia, occurring in the setting of renal disease. The histopathologic features of nephrogenic fibrosing dermopathy, i.e. thickened collagen and mucin deposition, are unique. The incidence, prevalence and cause of the disease are unknown and there is currently no effective treatment. The Centers for Disease Control and Prevention (CDC) in the USA are calling on physicians who have encountered patients suffering from this type of lesions to contact the CDC for an intended control study.
一名58岁的肾功能不全男性,正在接受血液透析治疗,出现了进行性皮肤病变。他的四肢皮肤增厚、变硬,脚趾和手指肿胀并伴有屈曲挛缩。他的面部未受影响。实验室检查无异常,皮肤活检显示胶原蛋白和黏蛋白增加,无炎症浸润。这些临床特征类似于最近报道的一种新疾病:肾源性纤维化皮肤病。这种疾病表现为类似硬化性黏液水肿的皮肤病变,无相关副蛋白血症,发生于肾脏疾病背景下。肾源性纤维化皮肤病的组织病理学特征,即胶原蛋白增厚和黏蛋白沉积,是独特的。该疾病的发病率、患病率和病因尚不清楚,目前尚无有效治疗方法。美国疾病控制与预防中心(CDC)呼吁遇到此类病变患者的医生与CDC联系,以进行一项意向性对照研究。