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Nephrogenic Systemic Fibrosis

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Scleroderma

Abstract

The earliest observations of the novel disorder, nephrogenic systemic fibrosis, were made in a renal transplantation unit of a medical center in Southern California in 1997 in patients with chronic renal disease undergoing renal dialysis. Many of these patients were recipients of kidney transplants, most of which had failed. The most apparent clinical manifestations were extensive and progressive cutaneous induration. Histopathological examination of skin biopsy specimens indicated that the disorder was distinct from scleroderma or morphea and resembled more closely scleromyxedema [1]. A detailed description of the first 15 cases was published in 2000 [2]. Nine of the fifteen patients had undergone a renal transplant and all had pre-existing chronic renal disease except for a single patient who had received dialysis for acute tubular necrosis. The manifestations consisted of extensive thickening, hardening and hyperpigmentation of the skin. Distinct papules and subcutaneous nodules were present in some of these patients as well as flexion contractures of the joints of the arms. These changes were observed predominantly on the lower extremities. Each patient initially received a dermatological diagnosis of fasciitis and myositis, scleroderma or calciphylaxis.

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Authors and Affiliations

  1. Jefferson Institute of Molecular Medicine, Philadelphia, PA, USA

    Peter J. Wermuth PhD

  2. Department of Dermatology and Cutaneous Biology, Jefferson Institute of Molecular Medicine, The Scleroderma Center and Division of Connective Tissue Diseases, Philadelphia, PA, USA

    Sergio A. Jimenez MD

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  1. Peter J. Wermuth PhD
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  2. Sergio A. Jimenez MD
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Correspondence to Peter J. Wermuth PhD .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Wermuth, P.J., Jimenez, S.A. (2012). Nephrogenic Systemic Fibrosis. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_13

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_13

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