Nephrogenic fibrosing dermopathy in a patient with acute renal failure never requiring dialysis

Abstract

Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrosing disorder.To date the etiology, pathogenesis, and clinical course remains unknown. The majority of cases have been in renal dialysis or renal transplant patients. Only four cases have been reported in which patients had acute renal failure and never required dialysis. Currently, there is no effective treatment.A 65-year-old man was hospitalized for pneumonia. During hospitalization he developed acute renal failure secondary to acute tubular necrosis. The patient had woody indurated plaques on his upper extremities and trunk, and brown indurated plaques on his trunk.Histopathological examination revealed a spindle cell proliferation infiltrating through the dermis and subcutis with mild mucin deposition.Nephrogenic fibrosing dermopathy is a novel fibrosing disorder diagnosed clinically and histopatholigically. Currently no risk factors other than renal failure have been identified. Our patient is a unique example of NFD observed in acute renal failure secondary to acute tubular necrosis, never requiring dialysis. We also review the four other cases of acute NFD never requiring dialysis that have been reported in the literature.

Nephrogene systemische Fibrose

“…F Symmetrische, aszendierende, konfluierende, makulopapulöse Plaques mit kopfsteinpflasterartigem Relief (Peau-d' orange-Aspekt) und palpatorisch holzartiger Beschaffenheit. Sowohl chronische Dialysepatienten als auch Nierentransplantierte mit eingeschränkter Transplantatfunktion, aber auch Patienten mit einem akuten (nicht dialysepflichtigen) Nierenversagen sind betroffen [6,15,19,24,26,29,36]. Aufgrund der klinisch im Vordergrund stehenden Hautmanifestationen wurde das Krankheitsbild initial als nephrogene fibrosierende Dermatopathie bezeichnet.…”

Association of Gadolinium Based Magnetic Resonance Imaging Contrast Agents and Nephrogenic Systemic Fibrosis

“…However, subsequent case reports documented NSF in patients with severe CKD or AKI who did not receive dialytic therapy. 7–9 Although about 90% of cases to date have been reported in dialysis patients, NSF associates with renal dysfunction and not dialysis per se. 3, 10 No cases of NSF in patients with a glomerular filtration rate (GFR) > 30 mL/min have been described.…”

Nephrogenic systemic fibrosis following acute kidney injury and exposure to gadolinium

“…There are only few reported cases of NSF in the setting of acute renal failure and fewer reported cases where skin lesions developed after kidney function had returned to normal. [2,10,11] Our case is also unique in that NSF developed in the setting of prerenal azotemia. In our patient, renal insufficiency in addition to edema, vascular intervention and acidic milieu at the time of gadolinium administration probably led to deposition of gadolinium in the skin, which was not mobilized after recovery of renal function, and thus the development of NSF.…”

“…NSF has been associated with severe edema, venous thrombosis, anti-phospholipid antibody, NEPHROGENIC SYSTEMIC FIBROSIS vascular intervention, erythropoietin therapy and gadolinium exposure. [2][3][4][5] Metabolic acidosis may predispose patients exposed to gadolinium to develop NSF. [4] Of the above-mentioned conditions, our patient had edema, vascular intervention, gadolinium exposure, metabolic acidosis and history of a hypercoagulable state.…”