Nephrogenic systemic fibrosis in a patient with a p-ANCA systemic vasculitis

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• 作者：Eduardo Cuende^a ; ^{ecuende.hupa@salud.madrid.org} ; Mikel Aldamiz^b ; Joseba Portu^b ; Ramó ; n Ruiz de Gauna^c ; Rosa Zaballa^d ; Juan Carlos Vesga^a
• 关键词：Nephrogenic systemic fibrosis ; p-ANCA vasculitis ; Acute renal failure
• 刊名：Reumatolog铆a Cl铆nica
• 出版年：2009
• 期刊代码：pca303_1699258X
• 类别：med
• 出版时间：November-December 2009
• 卷：5
• 期：6
• 页码：264-267
• 文件大小：301 K

摘要

Nephrogenic systemic fibrosis (NSF) is a fibrosing skin condition of unknown origin. Most cases have been described in patients with acute or chronic renal failure. The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. Previous exposition to gadolinium-based contrast agents was closely associated with its onset. We described a patient with the clinical and pathologic picture of NSF presented after an acute renal failure in the course of a perinuclear antineutrophil cytoplasmic antibodies associated systemic vasculitis.