Photopheresis Provides Significant Long-Lasting Benefit in Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a rare complication of exposure to gadolinium-based contrast agents in patients who have significantly decreased renal function. Manifestations include fibrosis of the skin and other tissues. Effective therapies are lacking. Photopheresis has been tried with variable rates of improvement, and small numbers of cases (20 as of 2016) have been reported of NSF patients treated with photopheresis. We report a case of patient with nephrogenic systemic fibrosis who was treated with photopheresis and demonstrated significant lasting improvements.

Skin Thickening in a Hemodialysis Patient: A Case of Nephrogenic Fibrosing Dermopathy

Background: Nephrogenic fibrosing dermopathy (NFD), a rare fibrosing condition of the skin, occurs in patients with renal failure. Worldwide, 215 cases have been reported, but none of them are from Canada. Case Report: A 71-year-old woman with hypertensive vasculopathy and renal failure, hypothyroidism, deep vein thrombosis, and a previous abdominal aneurysm repair presented with the rapid development of indurated skin over her arms and legs. These skin changes developed 5 months after starting hemodialysis. Biopsy Results: Skin biopsies demonstrated thickened collagen bundles in the dermis with extension into the subcutaneous tissue and minimal increased mucin production. There were increased fibroblastic cells that were immunohistochemically CD34 positive. Conclusions: This patient has the clinical and pathologic features of NFD. Interestingly, this patient had a history of vein thrombosis and vascular surgery, emphasizing the possible role these features may play as instigators of fibrosis in this disease.