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A case of nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis

Nature Clinical Practice Nephrology volume 3, pages 111–115 (2007)Cite this article

Abstract

Background A 61-year-old female with end-stage renal disease who was undergoing hemodialysis presented with an 8-week history of upper and lower extremity weakness associated with skin tightness and contractures.

Investigations Physical examination, blood analysis, electromyogram and skin biopsy.

Diagnosis Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis.

Management Methylprednisolone, thalidomide and physical therapy.

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Figure 1: Skin thickening, tightness and contractures are hallmarks of the clinical findings in nephrogenic fibrosing dermopathy.
Figure 2: Punch biopsy slides of left thigh (hematoxylin and eosin stain; magnification ×100) showing widening of subcutaneous septae with thick collagen bundles in (A) the deep dermis and (B) the subcutaneum.
Figure 3: Punch biopsy slide of left thigh showing positivity in dendritic cells (CD34 stain, hematoxylin counterstain; magnification ×200).

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Author information

Authors and Affiliations

  1. AS Weiss is a Fellow in the Division of Renal Diseases and Hypertension, and I Teitelbaum is a Professor in the Division of Renal Diseases and Hypertension at the University of Colorado Health Sciences Center, MS Lucia is an Associate Professor of Pathology, Denver, CO, USA.,

    Andrew S Weiss, M Scott Lucia & Isaac Teitelbaum

Authors
  1. Andrew S Weiss
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  2. M Scott Lucia
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  3. Isaac Teitelbaum
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Correspondence to Isaac Teitelbaum.

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The authors declare no competing financial interests.

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Weiss, A., Lucia, M. & Teitelbaum, I. A case of nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis. Nat Rev Nephrol 3, 111–115 (2007). https://doi.org/10.1038/ncpneph0373

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