title: Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure.
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.
authors.affiliation: Department of Pathology, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA. cynthiakucher@yahoo.com
authors.forename: Cynthia
authors.initials: C
authors.lastname: Kucher
authors.forename: Joanna
authors.initials: J
authors.lastname: Steere
authors.forename: Rosalie
authors.initials: R
authors.lastname: Elenitsas
authors.forename: Don L
authors.initials: DL
authors.lastname: Siegel
authors.forename: Xiaowei
authors.initials: X
authors.lastname: Xu
mesh_terms: D003964:Diaphragm / Q000473:pathology*; D017809:Fatal Outcome; D005355:Fibrosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D008297:Male; D008875:Middle Aged; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000473:pathology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology