Nephrogenic Systemic Sclerosis (Nephrogenic Fibrosing Dermopathy)

  • Seen in patients with ESRD; associated with exposure to gadoliniumbased contrast medium
  • Indurated fibrotic plaques on extremities and trunk with brawny hyperpigmentation, ± joint contractures
  • Histology: dermal sclerosis with ↑ # of CD34+ cells
  • Treatment: UVA1, restoration of renal function