RESUMEN
La fibrosis sistémica nefrogénica (FSN) es una entidad de origen desconocido caracterizada por un incremento de la fibrosis cutánea. La mayoría de los casos se han descrito en pacientes con fracaso renal agudo o crónico. Las alteraciones cutáneas incluyen pápulas y placas dérmicas engrosadas e induradas distribuidas por las extremidades y el tronco. El estudio histológico pone de manifiesto un incremento en la dermis del número de células tipo fibroblasto acompañado de depósito de mucina. En la mayoría de los casos existe el antecedente reciente de exposición a agentes de contraste tipo gadolinio. Describimos una paciente que presenta los hallazgos clínicos y patológicos característicos de la FSN después de un fracaso renal agudo en el contexto de una vasculitis asociada a anticuerpos anticitoplasma de los neutrófilos (AU)
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 (AU)
Asunto(s)
Humanos , Vasculitis/complicaciones , Fibrosis/complicaciones , Insuficiencia Renal/complicaciones , Fibrosis/fisiopatología , Anticuerpos Anticitoplasma de Neutrófilos/efectos adversos , Medios de Contraste/efectos adversosRESUMEN
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.