Nephrogenic Fibrosing Dermopathy Treated with Extracorporeal Photopheresis
Nephrogenic Fibrosing Dermopathy Treated with Extracorporeal Photopheresis
复制标题
体外光采术治疗肾源性纤维化皮肤病
作者：
S. Läuchli;C. Zortea;F. Nestle;G. Burg;W. Kempf
肾源性纤维化皮肤病（NFD）是一种罕见的纤维化皮肤病，病因不明，发生于终末期肾病患者。它于 2000 年首次被描述。NFD 的组织学显示树突状细胞、成纤维细胞和增厚的胶原纤维数量增加，类似于硬化粘液水肿。其与硬化性粘液水肿的区别在于皮损分布模式不同，硬斑主要位于四肢，并且不存在副蛋白血症。迄今为止，还没有任何一种治疗 NFD 的方法被证明是一致有效的。我们描述了一位 40 岁肾功能不全患者的病例，他接受了血液透析治疗并接受了肾移植。移植两年后，她的四肢出现了硬皮样棕色斑块。 4个周期的体外光采术后硬结明显改善。
Nephrogenic fibrosing dermopathy (NFD) is a rare fibrosing skin disease of unknown etiology occurring in patients with terminal renal disease. It was first described in the year 2000. The histology of NFD shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It can be distinguished from scleromyxedema by a different distribution pattern of the skin lesions with indurated plaques mainly on the extremities and the absence of paraproteinemia. As yet, no treatment for NFD has been proven to be uniformly efficient. We describe the case of a 40-year old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years after transplantation, she developed sclerodermiform brownish plaques on her extremities. The induration improved significantly after 4 cycles of extracorporeal photopheresis.