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  • Title: Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure.
    Author: Kucher C, Steere J, Elenitsas R, Siegel DL, Xu X.
    Journal: J Am Acad Dermatol; 2006 Feb; 54(2 Suppl):S31-4. PubMed ID: 16427988.
    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.
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