Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure

J Am Acad Dermatol. 2006 Feb;54(2 Suppl):S31-4. doi: 10.1016/j.jaad.2005.04.024.

Abstract

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a disorder occurring exclusively in patients with renal disease. Until recently, it has been considered a fibrosing disorder essentially confined to the skin and underlying superficial soft tissue. Recent reports, however, have described patients with involvement of other organ systems, suggesting that this disorder is actually a systemic disease with preferential cutaneous manifestations. We describe a patient with end-stage renal disease with diagnosed NFD/NSF who subsequently developed respiratory failure leading to his death. Autopsy findings showed NFD/NSF involving the skin of all extremities, as well as diffuse diaphragm involvement.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Diaphragm / pathology*
  • Fatal Outcome
  • Fibrosis
  • Humans
  • Kidney Failure, Chronic / complications*
  • Male
  • Middle Aged
  • Respiratory Insufficiency / etiology*
  • Respiratory Insufficiency / pathology
  • Skin / pathology
  • Skin Diseases / etiology*
  • Skin Diseases / pathology