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Nephrogenic fibrosing dermopathy in children

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Abstract

Nephrogenic fibrosing dermopathy (NFD) is an entity of unknown etiology that has only been reported in patients with impaired kidney function. It has characteristic diagnostic findings by skin biopsy and due to its systemic involvement in some reported cases, the term “nephrogenic systemic fibrosis” has been recently suggested as more appropriate for the nomenclature of this entity. There is no curative treatment currently available and very few cases have been reported in children. We hereby report two cases so as to alert pediatric health care providers about the existence of this disorder.

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References

  1. Cowper SE (2001–2006) Nephrogenic fibrosing dermopathy [NFD/NDS website]. Available at http://www.icnfdr.org

  2. Jain SM, Wesson S, Hassanein A, Canova E, Hoy M, Fennell RS, Dharnidharka VR (2004) Nephrogenic fibrosing dermopathy in pediatric patients. Pediatr Nephrol 19:467–470

    Article  PubMed  Google Scholar 

  3. Jan F, Segal JM, Dyer J, LeBoit P, Sigfried E, Frieden IJ (2003) Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr 143:678–681

    Article  PubMed  Google Scholar 

  4. Cowper SE, Robin HS, Steinber SM, Su DL, Gupta S, LeBoit PE (2000) Scleromyxedema-like cutaneous disease in renal-dialysis patients. Lancet 356:1000–1001

    Article  PubMed  CAS  Google Scholar 

  5. Cowper SE, Su LD, Bhawan J, Robin HS, LeBoit PE (2001) Nephrogenic fibrosing dermopathy. Am J Dermatopathol 23:383–393

    Article  PubMed  CAS  Google Scholar 

  6. Mackay-Wiggan JM, Cohen DJ, Hardy MA, Knobler EH, Grossman ME (2003) Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol 48:55–60

    Article  PubMed  Google Scholar 

  7. Swartz RD, Crofford LJ, Phan SH, Ike RW, Su LD (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 114:563–572

    Article  PubMed  Google Scholar 

  8. Cowper SE (2003) Nephrogenic fibrosing dermopathy: the first 6 years. Curr Opin Rheumatol 15:785–790

    Article  PubMed  Google Scholar 

  9. Moschella SL, Kay J, Mackool BT, Liu V (2004) Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 35-2004. A 68-year-old man with end-stage renal disease and thickening of the skin. N Engl J Med 351:2219–2227

    Article  PubMed  CAS  Google Scholar 

  10. Levine JM, Taylor RA, Elman LB, Bird SJ, Lavi E, Stolzenberg ED, McGarvey ML, Asbury AK, Jimenez SA (2004) Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy). Muscle Nerve 30:569–577

    Article  PubMed  Google Scholar 

  11. Kucher C, Xiaowel X, Pasha T, Elenitsas R (2005) Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J Cutan Pathol 32:484–490

    Article  PubMed  Google Scholar 

  12. Daram SR, Cortese CM, Bastani B (2005) Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: report of a new case with literature review. Am J Kidney Dis 46:754–759

    Article  PubMed  Google Scholar 

  13. Ruiz-Genao DP, Pascual-Lopez MP, Fraga S, Aragues M, Garcia-Diez A (2005) Osseus metaplasia in the setting of nephrogenic fibrosing dermopathy. J Cutan Pathol 32:172–175

    Article  PubMed  Google Scholar 

  14. Hubbard V, Davenport A, Jarmulowicz M, Rustin M (2003) Scleromyxoedema-like changes in four renal dialysis patients. Br J Dermatol 148:563–568

    Article  PubMed  CAS  Google Scholar 

  15. Gambichler T, Paech V, Kreuter A, Wilmert M, Altmeyer P, Stucker (2004) Nephrogenic fibrosing dermopathy. Clin Exp Dermatol 29:258–260

    Article  PubMed  CAS  Google Scholar 

  16. Ortonne N, Lipsker D, Chantrel F, Boehm N, Grosshans E, Cribier B (2004) Presence of CD45RO+ CD34+ cells with collagen synthesis activity in nephrogenic fibrosing dermopathy: a new pathogenic hypothesis. Br J Dermatol 150:1050–1052

    Article  PubMed  CAS  Google Scholar 

  17. Cowper SE, Bucala R (2003) Nephrogenic fibrosing dermopathy: suspect identified, motive unclear. Am J Dermatopathol 25:358

    Article  PubMed  Google Scholar 

  18. LeBoit P (2003) What nephrogenic fibrosing dermopathy might be. Arch Dermatol 139:928–930

    Article  PubMed  Google Scholar 

  19. Ting WW, Stone MS, Madison KC, Kurtz K (2003) Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 139:903–906

    Article  PubMed  Google Scholar 

  20. Lauchli S, Zortea-Caflisch C, Nestle FO, Burg G, Kempf W (2004) Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis. Dermatology 208:278–280

    Article  PubMed  Google Scholar 

  21. Baron PW, Cantos K, Hillebrand DJ, Hu KQ, Ojogho ON, Nehlsen-Cannarella S (2003) Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis. Am J Dermatopathol 25:204–209

    Article  PubMed  Google Scholar 

  22. Kafi R, Fisher GJ, Quan T, Shao Y, Wang R, Voorhees JJ, Kang S (2004) UV-A1 phototherapy improves nephrogenic fibrosing dermopathy. Arch Dermatol 140:1322–1324

    Article  PubMed  Google Scholar 

  23. Cowper SE (2005) Nephrogenic systemic fibrosis: the nosological and conceptual evolution of nephrogenic fibrosing dermopathy. Am J Kidney Dis 46:763–765

    Article  PubMed  Google Scholar 

  24. D’Amato RJ, Loughnan MS, Flynn E, Folkman J (1994) Thalidomide is an inhibitor of angiogenesis. Proc Natl Acad Sci USA 91:4082–4085

    Article  PubMed  CAS  Google Scholar 

  25. Sampaio EP, Sarno EN, Galilly R, Cohn ZA, Kaplan G (1991) Thalidomide selectively inhibits tumor necrosis factor alpha production by stimulated human monocytes. J Exp Med 173:699–703

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Section of Pediatric Nephrology, The Children’s Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas, MO, USA

    Ari Auron & Bradley A. Warady

  2. Section of Pathology, The Children’s Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas, MO, USA

    Lei Shao

Authors
  1. Ari Auron
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  2. Lei Shao
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  3. Bradley A. Warady
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Correspondence to Bradley A. Warady.

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Auron, A., Shao, L. & Warady, B.A. Nephrogenic fibrosing dermopathy in children. Pediatr Nephrol 21, 1307–1311 (2006). https://doi.org/10.1007/s00467-006-0174-7

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  • DOI: https://doi.org/10.1007/s00467-006-0174-7

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