Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis
Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis
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DOI：
10.1097/00000372-200306000-00004
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发表时间：
2003-06-01
影响因子：
1.1
通讯作者：
Concepcion, W
中科院分区：
文献类型：
--
作者：
Baron, PW;Cantos, K;Concepcion, W
肾源性纤维化皮肤病（NFD）是最近描述的皮肤纤维化疾病与肾功能不全。它看起来类似于硬化性粘液水肿，但有一些值得注意的例外，包括组织学上没有累及面部和浆细胞，全身受累和副蛋白血症。患者可表现为皮肤增厚或水肿，伴有累及四肢和躯干的硬化丘疹和斑块。我们报告了前三例肝移植术后NFD成功地用血浆置换治疗。2例患者因丙型肝炎病毒诱导的肝硬化接受肝移植，1例患者因B型肝炎病毒诱导的肝硬化接受肝移植。所有患者在移植前都有脑病、难治性腹水和营养不良。像那些NFD患者一样，我们的三名患者在移植前后都有肾功能不全，需要血液透析。然而，两人在诊断时并不依赖透析。这些患者的移植肝功能良好，但另一名患者因丙型肝炎复发而发生移植失败。免疫抑制治疗包括巴利昔单抗、吗替麦考酚酯、钙调磷酸酶抑制剂和泼尼松。移植后1、2和120个月，患者出现四肢远端“木质”皮肤硬结、肿胀性丘疹和挛缩。皮肤活检类似于NFD。无明显副蛋白血症。一至三个为期5天的血浆置换疗程导致中度至显著的临床改善。我们的两名患者的肾功能改善似乎与皮肤病无关，因为在诊断NFD时肾功能正在改善。总之，我们报告了肝移植后的前三例NFD。血浆置换术在解决皮肤硬化丘疹、严重皮肤硬化和相关关节挛缩方面取得了中等程度的成功。初步研究（未发表的数据）表明，血浆置换术后转化生长因子-β 1的血浆水平降低似乎与这种临床状况的改善相关。
Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. It appears similar to scleromyxedema but with some notable exceptions, including the lack of involvement of the face and absence of plasma cells on histology, systemic involvement, and paraproteinemia. Patients can present with thickened or edematous skin with indurated papules and plaques involving the extremities and the trunk. We report the first three cases of NFD after liver transplantation successfully treated with plasmapheresis. Two patients underwent liver transplantation for hepatitis C virus-induced cirrhosis and one for hepatitis B virus-induced cirrhosis. All the patients had encephalopathy, refractory ascites, and malnutrition prior to transplantation. Like those patients with NFD, all three of our patients had renal dysfunction and required hemodialysis before and after transplantation. Two were not dependent on dialysis at the time of diagnosis, however. These patients had excellent liver allograft function, but the other patient had allograft failure secondary to recurrent hepatitis C. Immunosuppression therapy consisted of basiliximab, mycophenolate mofetil, calcineurin inhibitor, and prednisone. The patients developed "woody" skin induration of the distal extremities, erythematous papules, and contractures at 1, 2, and 120 months after transplantation. Skin biopsies resembled NFD. No paraproteinemia was evident. One to three 5-day courses of plasmapheresis resulted in moderate to marked clinical improvement. The improvement of the kidney function in two of our patients did not appear to correlate with that of the skin disorder, because the kidney function was improving at the time the diagnosis of NFD was made. In conclusion, we report the first three cases of NFD after liver transplantation. Plasmapheresis was moderately successful in resolving the skin-indurated papules, severe skin induration, and associated joint contractures Preliminary studies (unpublished data) show that decreasing plasma levels of transforming growth factor-beta1 after plasmapheresis appear to correlate with the amelioration of this clinical condition.