Aires Nádia Barbosa, Sotto Mírian N, Nico Marcello M S
Department of Dermatology, University of São Paulo Medical School, São Paulo, Brazil.
Acta Derm Venereol. 2007;87(6):521-4. doi: 10.2340/00015555-0296.
Nephrogenic fibrosing dermopathy is a recently described fibrosing disorder that emerges in different settings of renal insufficiency. The degree and cause of the underlying renal impairment seem not to be related to the severity of the nephrogenic fibrosing dermopathy. Lesions are typically symmetrical and usually develop on the limbs and trunk. These are characterized by skin-coloured to erythematous papules that coalesce into plaques markedly thickened in texture. Flexion contractures of the joints may be a feature of the disease. There is no consistently effective therapy for nephrogenic fibrosing dermopathy. We report here 2 Brazilian patients in connection with a short review. Remission was recorded in one case after plasmapheresis.
肾源性纤维化皮肤病是一种最近被描述的纤维化疾病，出现在不同的肾功能不全情况下。潜在肾功能损害的程度和原因似乎与肾源性纤维化皮肤病的严重程度无关。病变通常是对称的，通常发生在四肢和躯干。其特征是肤色至红斑丘疹，融合成质地明显增厚的斑块。关节挛缩可能是该疾病的一个特征。目前尚无针对肾源性纤维化皮肤病的一致有效的治疗方法。我们在此报告2例巴西患者并进行简短回顾。1例患者在进行血浆置换后病情缓解。
