Skin Lesions in Dialysis - Part 3

Nephrogenic Systemic Fibrosis (NSF)

No post or summary on skin disorders in renal failure can omit NSF. This has been mentioned in multiple previous posts on RFN - although never summarized:

The first cases of this disorder were noted between 1997 and 2000. Nearly all have been in patients with gadolinium exposure. Most of these patients had severe CKD or were on dialysis. The onset of NSF post gadolinium exposure ranges from 2 months to 15 years. The risk of NSF in dialysis patients has been reported as between 2.5 and 5%. Most centers have stopped using gadolinium if the GFR is less than 30ml/min/1.73m2. Starting or increasing EPO dose has also been associated with NSF. This disorder is a systemic disease. Skin disease typically presents with symmetrical, bilateral fibrotic, indurated papules, plaques or subcutaneous nodules. Lesions start in the hands, feet and ankles and move proximally. Rarely are lesions found on the abdomen and the head is spared. Lesions are edematous and may look like cellulitits initially. Sharp pain, pruritis and burning are also a feature. Joints lose flexibility due to fibrosis. Histological findings are variable: Increased dermal cellularity, CD34+ cells with tram tracking, collagen bundles, septal involvement and osseous metaplasia are typical findings. A comprehensive screen for other causes of severe skin disease is important. NSF has a poor prognosis and one review of the literature suggests a mortality of 28%. There is no treatment for NSF other than recovery of renal function. The preventative measure of avoiding gadolinium exposure in those at risk has significantly reduced the incidence of NSF. A good website by The International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR) can be found at http://www.icnfdr.org/.

Posted by Andrew Malone