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

Abstract

Background: Nephrogenic fibrosing dermopathy is a newly recognized cutaneous fibrosing disorder. NFD is an acquired, idiopathic hardening of the skin. Initially, Cowper, et al described it as “scleromyxedema-like illness of renal disease,” as it initially was seen in hemodialysis and renal transplant patients. In 2000 Cowper, et al renamed the entity “nephrogenic fibrosing dermopathy,” as subsequent reports of patients with acute renal failure and peritoneal dialysis were also being afflicted with this cutaneous fibrosing condition. Clinically patients present with sclerotic induration involving the extremities and the trunk. Thickening of the skin often leads to debilitating flexion contractures. To date the etiology, pathogenesis, and clinical course remains unknown. Swartz, et al found no correlating risk factors or laboratory findings other than renal failure in a review of 13 patients with NFD. The majority of cases have been in renal dialysis renal transplant patients. The vast minority (three patients) have had acute renal failure and never required dialysis. Currently, there is no effective treatment. Observation: A 65-year-old man was hospitalized for pneumonia. During hospitalization he developed acute renal failure secondary to acute tubular necrosis. He noticed hardening of his extremities and trunk. His comorbidities include hypertriglyceridemia, coronary artery disease, and diabetes mellitus. His medications include amiodarone, asparaginase, famotidine, isosorbide mononitrate, warfarin, losartan, furosemide, clonidine, humalog, insulin, and pioglitazone. He was noted to have tight, woody, indurated upper and lower extremities. Brown indurated plaques were on his trunk. He had significant flexion contractions that confined him to a wheelchair. Histopathologic examination revealed fibrosing dermatitis with septal panniculitis with a mild increase in dermal mucin. The patient’s fibrosing skin has slowly improved as his renal function improves. Conclusion: NFD is a novel fibrosing disorder that is diagnosed clinically and histopatholigically. Currently no risk factors other than renal failure have been identified. Our patient is a unique example of NFD seen in acute renal failure secondary to acute tubular necrosis, never requiring dialysis. It is imperative that dermatologists be familiar with this rare, but often debilitating disorder.