title: Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature.
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.
authors.affiliation: Department of Pathology, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Gibson
authors.forename: Carol F
authors.initials: CF
authors.lastname: Farver
authors.forename: Richard A
authors.initials: RA
authors.lastname: Prayson
mesh_terms: D000328:Adult; D001344:Autopsy; D004388:Dura Mater / Q000473:pathology; D017809:Fatal Outcome; D005355:Fibrosis; D006325:Heart Atria / Q000473:pathology; D006801:Humans; D008297:Male; D009206:Myocardium / Q000473:pathology; D032389:Myocytes, Smooth Muscle / Q000473:pathology; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000150:complications / Q000473:pathology*; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology*