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.