Nephrogenic fibrosing dermopathy: a novel, disabling disorder in patients with renal failure.

Nephrogenic fibrosing dermopathy (NFD) is a rare, recently described cutaneous fibrosing disorder that primarily affects patients with a history of renal disease [1]. Since the first report in 2000 by Cowper et al., about 100 cases of NFD have been described worldwide, and clusters have been observed in dialysis units and medical centres in the US, the UK and Switzerland. The disorder is not limited to haemodialysis patients as was suggested by initial observations, but may also occur in patients receiving peritoneal dialysis and in patients suffering from renal insufficiency who never have been dialysed [1–4,7]. Patients typically develop acute, lumpy, plaque-like indurations involving the extremities, and occasionally the trunk and buttocks. The head is almost always spared. The diffuse skin thickening and hardening may be accompanied or preceded by isolated nodules and, rarely bullae. Pruritus and burning pain are common clinical complaints, and sometimes there is a palpable warmth. The lesions may be complicated by flexion contractures of the adjacent joints. The disease appears to have a chronic and unremitting course in most patients [1,2,7]. The aetiology and pathogenesis remain largely unknown. As a consequence, therapeutic measures with proven efficacy are non-existing to date. In the present paper we report two additional cases of NFD recently identified in our unit. Furthermore, an overview of the available literature data is presented. The main aim is to make the nephrology community aware about this disabling skin condition. Cases