Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature

Arch Pathol Lab Med. 2006 Feb;130(2):209-12. doi: 10.1043/1543-2165(2006)130[209:MIINFD]2.0.CO;2.

Abstract

Nephrogenic fibrosing dermopathy is a recently recognized, scleromyxedema-like fibrosing skin condition that occurs in individuals with acute or chronic renal failure. Although the early descriptions of this disorder describe a purely cutaneous disease process, 2 recent autopsy reports have identified apparent multiorgan fibrosis with involvement of skeletal muscle, myocardium, lungs, kidneys, and testes. We describe a 23-year-old man with nephrogenic fibrosing dermopathy and significant fibrosis of the atrial myocardium and dura mater, which was identified at autopsy. Dural fibrosis is a previously undescribed systemic manifestation of nephrogenic fibrosing dermopathy. The literature is reviewed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autopsy
  • Dura Mater / pathology
  • Fatal Outcome
  • Fibrosis
  • Heart Atria / pathology
  • Humans
  • Male
  • Myocardium / pathology
  • Myocytes, Smooth Muscle / pathology
  • Renal Dialysis
  • Renal Insufficiency / complications
  • Renal Insufficiency / pathology*
  • Skin Diseases / etiology
  • Skin Diseases / pathology*