eMedicine Specialties > Dermatology > Internal Medicine

Nephrogenic Systemic Fibrosis: Differential Diagnoses & Workup

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): Shawn Cowper, MD, Director, NFD Registry Project, Associate Professor, Departments of Dermatology and Pathology, Yale University
Contributor Information and Disclosures

Updated: Sep 16, 2009

Differential Diagnoses

Calciphylaxis
Lichen Myxedematosus
Cellulitis
Morphea
Dermatofibrosarcoma Protuberans
Porphyria Cutanea Tarda
Eosinophilia-Myalgia Syndrome
Systemic Sclerosis
Eosinophilic Fasciitis
Granuloma Annulare

Other Problems to Be Considered

Fibroblastic rheumatism
Scleromyxedema
Beta2-microglobulin amyloidosis
Panniculitis
Sclerodermatous chronic graft versus host disease

Workup

Laboratory Studies

The following has been noted:

  • Scleromyxedemalike skin lesions occur without associated paraproteinemia. Serum protein electrophoresis and immunoelectrophoresis results are negative.
  • Nephrogenic systemic fibrosis (NSF) occurs in the setting of renal disease. Thus, abnormal values of BUN and creatinine are typically present.
  • Thyroid studies produce normal findings in patients with nephrogenic systemic fibrosis.
  • In rare cases, peripheral eosinophilia has been noted.
  • In some cases, a positive antinuclear antibody test result has been reported.
  • Some patients with nephrogenic systemic fibrosis have anticardiolipin or antiphospholipid antibodies.
  • Rheumatoid factor and other autoantibodies are typically absent.
  • Many patients have an associated hypercoagulable state.
  • Some patients have hepatitis B or C.
In summary, no particular laboratory abnormality is consistently linked to nephrogenic systemic fibrosis.

Imaging Studies

No imaging study is indicated to assess nephrogenic systemic fibrosis. However, although no imaging study is indicated to assess nephrogenic systemic fibrosis, many authors have described soft tissue calcifications.

Other Tests

Abnormalities of nerve conduction have been described in some patients.

Thakral and Abraham38 developed an automated quantitative scanning electron microscopy and energy dispersive x-ray spectroscopy method for documenting gadolinium in tissue of patients with nephrogenic systemic fibrosis. They examined, under standardized conditions, freshly cut tissue in paraffin block using the variable pressure mode. This allowed them to perform a random search of the tissue area to conduct in situ detection and semiquantitative morphometric (volumetric) analysis of insoluble, higher–atomic number features using backscattered electron imaging.

Thakral and Abraham detected gadolinium ranging from 1-2270 cps/mm2 in 57 cutaneous biopsy specimens of nephrogenic systemic fibrosis. Gadolinium was associated with phosphate, calcium, and sodium in the tissues. This method reproducibly determines the elemental composition, relative concentration, and spatial distribution of detected features within the tissue; however, they could not detect features below their spatial resolution or concentrations below the detection limit of the scanning electron microscopy and energy dispersive x-ray spectroscopy system.

Procedures

A skin biopsy is indicated for this condition. A deep biopsy, such as an incisional biopsy or a deep punch biopsy, should be performed. A sample of dermis, subcutaneous fat, and fascia should be obtained, if possible.

Histologic Findings

In 2001, Cowper et al20 described the unique histopathologic features of nephrogenic systemic fibrosis, specifically, thickened collagen bundles with surrounding clefts, mucin deposition, and a proliferation of fibroblasts and elastic fibers.

Early lesions (within 20 wk of clinic onset) demonstrate reticular, dermal, large, and epithelioid or stellate spindle cells. These cells can extend into and widen the subcutaneous fat lobule septa. The spindle cells are diffusely arranged among thickened collagen bundles.

Clefts can encompass some spindle cells. Most of the spindle cells are CD34/procollagen dual-positive cells that form a dense interconnecting network.

Because this condition is not commonly reported, diagnostic errors abound. Spindle cell proliferation can be confused with dermatofibrosarcoma protuberans or even spindle cell melanoma. Alcian blue, mucicarmine, or Movat pentachrome stains can be used to detect variable amounts of mucin. Thick elastic fibers oriented parallel to collagen bundles are usually present.
Some cases of nephrogenic systemic fibrosis have manifested with a scant superficial and deep lymphocytic infiltrate, although inflammation is usually absent, and this finding should prompt consideration of an alternative diagnosis. Multinucleated cells positive for CD68 and factor XIIIa–positive cells are commonly present.

Late lesions (>20 wk of clinical onset) typically have less prominent clefting, less mucin, and fewer CD34/procollagen–positive cells. Calcification, which is described in some patients and which has been interpreted as dystrophic in nature, can be present.

More on Nephrogenic Systemic Fibrosis

Overview: Nephrogenic Systemic Fibrosis
Differential Diagnoses & Workup: Nephrogenic Systemic Fibrosis
Treatment & Medication: Nephrogenic Systemic Fibrosis
Follow-up: Nephrogenic Systemic Fibrosis
Multimedia: Nephrogenic Systemic Fibrosis
References
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References

  1. Wahba IM, Simpson EL, White K. Gadolinium is not the only trigger for nephrogenic systemic fibrosis: insights from two cases and review of the recent literature. Am J Transplant. Oct 2007;7(10):2425-32. [Medline].

  2. Marckmann P, Skov L, Rossen K, et al. Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging. J Am Soc Nephrol. Sep 2006;17(9):2359-62. [Medline].

  3. Cowper SE, Kuo PH, Bucala R. Nephrogenic systemic fibrosis and gadolinium exposure: association and lessons for idiopathic fibrosing disorders. Arthritis Rheum. Oct 2007;56(10):3173-5. [Medline].

  4. Thomsen HS, Marckmann P, Logager VB. Nephrogenic systemic fibrosis (NSF): a late adverse reaction to some of the gadolinium based contrast agents. Cancer Imaging. Sep 24 2007;7:130-7. [Medline].

  5. Bongartz G, Kucharczyk W. Nephrogenic systemic fibrosis: Summary of the special symposium. J Magn Reson Imaging. 2007;26:1179-81.

  6. Goveia M, Chan BP, Patel PR. Evaluating the role of recombinant erythropoietin in nephrogenic systemic fibrosis. J Am Acad Dermatol. Oct 2007;57(4):725-7. [Medline].

  7. Saab G. Epoetin and nephrogenic systemic fibrosis. J Am Acad Dermatol. Jan 2008;58(1):174-5. [Medline].

  8. Gibson SE, Farver CF, Prayson RA. Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature. Arch Pathol Lab Med. Feb 2006;130(2):209-12. [Medline].

  9. Brown H. Gadodiamide, NSF: Direct Link Identified. Skin and Allergy News. 2007 Dec;Vol. 38; Issue 12: 37.

  10. Parsons AC, Yosipovitch G, Sheehan DJ, Sangueza OP, Greenberg CS, Sane DC. Transglutaminases: the missing link in nephrogenic systemic fibrosis. Am J Dermatopathol. Oct 2007;29(5):433-6. [Medline].

  11. Edward M, Fitzgerald L, Thind C, Leman J, Burden AD. Cutaneous mucinosis associated with dermatomyositis and nephrogenic fibrosing dermopathy: fibroblast hyaluronan synthesis and the effect of patient serum. Br J Dermatol. Mar 2007;156(3):473-9. [Medline].

  12. Mackay-Wiggan JM, Cohen DJ, Hardy MA, Knobler EH, Grossman ME. Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol. Jan 2003;48(1):55-60. [Medline].

  13. McNeill AM, Barr RJ. Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis. Int J Dermatol. Jun 2002;41(6):364-7. [Medline].

  14. Jimenez SA, Artlett CM, Sandorfi N, et al. Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): study of inflammatory cells and transforming growth factor beta1 expression in affected skin. Arthritis Rheum. Aug 2004;50(8):2660-6. [Medline].

  15. Ortonne N, Lipsker D, Chantrel F, Boehm N, Grosshans E, Cribier B. Presence of CD45RO+ CD34+ cells with collagen synthesis activity in nephrogenic fibrosing dermopathy: a new pathogenic hypothesis. Br J Dermatol. May 2004;150(5):1050-2. [Medline].

  16. Kucher C, Xu X, Pasha T, Elenitsas R. Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J Cutan Pathol. Aug 2005;32(7):484-90. [Medline].

  17. Deo A, Fogel M, Cowper SE. Nephrogenic systemic fibrosis: a population study examining the relationship of disease development to gadolinium exposure. Clin J Am Soc Nephrol. Mar 2007;2(2):264-7. [Medline].

  18. Todd DJ, Kagan A, Chibnik LB, Kay J. Cutaneous changes of nephrogenic systemic fibrosis: predictor of early mortality and association with gadolinium exposure. Arthritis Rheum. Oct 2007;56(10):3433-41. [Medline].

  19. Hubbard V, Davenport A, Jarmulowicz M, Rustin M. Scleromyxoedema-like changes in four renal dialysis patients. Br J Dermatol. Mar 2003;148(3):563-8. [Medline].

  20. Cowper SE, Su LD, Bhawan J, Robin HS, LeBoit PE. Nephrogenic fibrosing dermopathy. Am J Dermatopathol. Oct 2001;23(5):383-93. [Medline].

  21. Jan F, Segal JM, Dyer J, LeBoit P, Siegfried E, Frieden IJ. Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr. Nov 2003;143(5):678-81. [Medline].

  22. Hancox JG, Mengesha YM, Sangueza OP, Yosipovitch G. Nephrogenic fibrosing dermopathy after five days of hemodialysis. J Drugs Dermatol. Oct 2003;2(5):550-3. [Medline].

  23. Ruiz-Genao DP, Pascual-Lopez MP, Fraga S, Aragues M, Garcia-Diez A. Osseous metaplasia in the setting of nephrogenic fibrosing dermopathy. J Cutan Pathol. Feb 2005;32(2):172-5. [Medline].

  24. Obermoser G, Emberger M, Wieser M, Zelger B. Nephrogenic fibrosing dermopathy in two patients with systemic lupus erythematosus. Lupus. 2004;13(8):609-12. [Medline].

  25. Levine JM, Taylor RA, Elman LB, et al. Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy). Muscle Nerve. Nov 2004;30(5):569-77. [Medline].

  26. Hauser C, Kaya G, Chizzolini C. Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis. Dermatology. 2004;209(1):50-2. [Medline].

  27. Glaich AS, Martinelli PT, Markus RF, Hsu S. Generalized elastolysis following nephrogenic fibrosing dermopathy. J Am Acad Dermatol. Jul 2005;53(1):174-6. [Medline].

  28. Kucher C, Steere J, Elenitsas R, Siegel DL, Xu X. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure. J Am Acad Dermatol. Feb 2006;54(2 Suppl):S31-4. [Medline].

  29. Edward M, Fitzgerald L, Thind C, Leman J, Burden AD. Cutaneous mucinosis associated with dermatomyositis and nephrogenic fibrosing dermopathy: fibroblast hyaluronan synthesis and the effect of patient serum. Br J Dermatol. Mar 2007;156(3):473-9. [Medline].

  30. Saenz A, Mandal R, Kradin R, Hedley-Whyte ET. Nephrogenic fibrosing dermopathy with involvement of the dura mater. Virchows Arch. Sep 2006;449(3):389-91. [Medline].

  31. Naylor E, Hu S, Robinson-Bostom L. Nephrogenic systemic fibrosis with septal panniculitis mimicking erythema nodosum. J Am Acad Dermatol. Jan 2008;58(1):149-50. [Medline].

  32. Keyrouz S, Rudnicki SA. Neuromuscular involvement in nephrogenic systemic fibrosis. J Clin Neuromuscul Dis. Dec 2007;9(2):297-302. [Medline].

  33. Sanchez-Ross M, Snyder R, Colome-Grimmer MI, Blumberg M, Huttenbach Y, Raimer S. Nephrogenic fibrosing dermopathy in a patient with systemic lupus erythematosus and acute lupus nephritis. Pediatr Dermatol. Sep-Oct 2007;24(5):E36-9. [Medline].

  34. Goddard DS, Magee CC, Lazar AJ, Miller DM. Nephrogenic fibrosing dermopathy with recurrence after allograft failure. J Am Acad Dermatol. May 2007;56(5 Suppl):S109-11. [Medline].

  35. High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol. Apr 2007;56(4):710-2. [Medline].

  36. Solomon GJ, Wu E, Rosen PP. Nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma. Arch Pathol Lab Med. Jan 2007;131(1):145-8. [Medline].

  37. Saab G, Cheng S. Nephrogenic systemic fibrosis: a nephrologist's perspective. Hemodial Int. Oct 2007;11 Suppl 3:S2-6. [Medline].

  38. Thakral C, Abraham JL. Automated scanning electron microscopy and x-ray microanalysis for in situ quantification of gadolinium deposits in skin. J Electron Microsc (Tokyo). Oct 2007;56(5):181-7. [Medline].

  39. Kintossou R, D'Incan M, Chauveau D, et al. [Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis: role of gadolinium?]. Ann Dermatol Venereol. Aug-Sep 2007;134(8-9):667-71. [Medline].

  40. Schmook T, Budde K, Ulrich C, Neumayer HH, Fritsche L, Stockfleth E. Successful treatment of nephrogenic fibrosing dermopathy in a kidney transplant recipient with photodynamic therapy. Nephrol Dial Transplant. Jan 2005;20(1):220-2. [Medline].

  41. Kafi R, Fisher GJ, Quan T, et al. UV-A1 phototherapy improves nephrogenic fibrosing dermopathy. Arch Dermatol. Nov 2004;140(11):1322-4. [Medline].

  42. Lauchli S, Zortea-Caflisch C, Nestle FO, Burg G, Kempf W. Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis. Dermatology. 2004;208(3):278-80. [Medline].

  43. Chung HJ, Chung KY. Nephrogenic fibrosing dermopathy: response to high-dose intravenous immunoglobulin. Br J Dermatol. Mar 2004;150(3):596-7. [Medline].

  44. Wahba IM, White K, Meyer M, Simpson EL. The case for ultraviolet light therapy in nephrogenic fibrosing dermopathy--report of two cases and review of the literature. Nephrol Dial Transplant. Feb 2007;22(2):631-6. [Medline].

  45. Richmond H, Zwerner J, Kim Y, Fiorentino D. Nephrogenic systemic fibrosis: relationship to gadolinium and response to photopheresis. Arch Dermatol. Aug 2007;143(8):1025-30. [Medline].

  46. Yerram P, Saab G, Karuparthi PR, Hayden MR, Khanna R. Nephrogenic systemic fibrosis: a mysterious disease in patients with renal failure--role of gadolinium-based contrast media in causation and the beneficial effect of intravenous sodium thiosulfate. Clin J Am Soc Nephrol. Mar 2007;2(2):258-63. [Medline].

  47. Kreuter A, Gambichler T, Weiner SM, Schieren G. Limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis. Arch Dermatol. Nov 2008;144(11):1527-9. [Medline].

  48. Marckmann P, Nielsen AH, Sloth JJ. Possibly enhanced Gd excretion in dialysate, but no major clinical benefit of 3-5 months of treatment with sodium thiosulfate in late stages of nephrogenic systemic fibrosis. Nephrol Dial Transplant. Oct 2008;23(10):3280-2. [Medline].

  49. Aires NB, Sotto MN, Nico MM. Nephrogenic fibrosing dermopathy: report of two cases. Acta Derm Venereol. 2007;87(6):521-4. [Medline].

  50. Bardin T. Musculoskeletal manifestations of chronic renal failure. Curr Opin Rheumatol. Jan 2003;15(1):48-54. [Medline].

  51. Bongartz G. Imaging in the time of NFD/NSF: do we have to change our routines concerning renal insufficiency?. MAGMA. Apr 2007;20(2):57-62. [Medline].

  52. Boyd AS, Zic JA, Abraham JL. Gadolinium deposition in nephrogenic fibrosing dermopathy. J Am Acad Dermatol. Jan 2007;56(1):27-30. [Medline].

  53. Broome DR, Cottrell AC, Kanal E. Response to "Will dialysis prevent the development of nephrogenic systemic fibrosis after gadolinium-based contrast administration?". AJR Am J Roentgenol. Oct 2007;189(4):W234-5. [Medline].

  54. Centers for Disease Control and Prevention. Fibrosing skin condition among patients with renal disease--United States and Europe, 1997-2002. MMWR Morb Mortal Wkly Rep. Jan 18 2002;51(2):25-6. [Medline].

  55. Centers for Disease Control and Prevention. Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St. Louis, Missouri, 2002-2006. MMWR Morb Mortal Wkly Rep. Feb 23 2007;56(7):137-41. [Medline].

  56. Chewning RH, Murphy KJ. Gadolinium-based contrast media and the development of nephrogenic systemic fibrosis in patients with renal insufficiency. J Vasc Interv Radiol. Mar 2007;18(3):331-3. [Medline].

  57. Clorius S, Technau K, Watter T, et al. Nephrogenic systemic fibrosis following exposure to gadolinium-containing contrast agent. Clin Nephrol. Oct 2007;68(4):249-52. [Medline].

  58. Collidge TA, Thomson PC, Mark PB, et al. Gadolinium-enhanced MR imaging and nephrogenic systemic fibrosis: retrospective study of a renal replacement therapy cohort. Radiology. Oct 2007;245(1):168-75. [Medline].

  59. Cowper SE. Nephrogenic systemic fibrosis: a review and exploration of the role of gadolinium. Adv Dermatol. 2007;23:131-54. [Medline].

  60. Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE. Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet. Sep 16 2000;356(9234):1000-1. [Medline].

  61. Edsall LC, English JC 3rd, Teague MW, Patterson JW. Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy. J Cutan Pathol. Mar 2004;31(3):247-53. [Medline].

  62. Endre ZH. Nephrogenic systemic fibrosis: is any contrast safe in renal failure?. Intern Med J. Jul 2007;37(7):429-31. [Medline].

  63. Fazeli A, Lio PA, Liu V. Nephrogenic fibrosing dermopathy: are ACE inhibitors the missing link?. Arch Dermatol. Nov 2004;140(11):1401. [Medline].

  64. Giersig C. [Nephrogenic systemic fibrosis as an adverse drug reaction of gadolinium-based contrast media for magnetic resonance imaging]. Radiologe. Sep 2007;47(9):794-9. [Medline].

  65. Grobner T. Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?. Nephrol Dial Transplant. Apr 2006;21(4):1104-8. [Medline].

  66. Grobner T, Prischl FC. Gadolinium and nephrogenic systemic fibrosis. Kidney Int. Aug 2007;72(3):260-4. [Medline].

  67. Hedley AJ, Molan MP, Hare DL, Anavekar NS, Ierino FL. Nephrogenic systemic fibrosis associated with gadolinium-containing contrast media administration in patients with reduced glomerular filtration rate. Australas Radiol. Jun 2007;51(3):300. [Medline].

  68. Hedley AJ, Molan MP, Hare DL, Anavekar NS, Ierino FL. Nephrogenic systemic fibrosis associated with gadolinium-containing contrast media administration in patients with reduced glomerular filtration rate. Nephrology (Carlton). Feb 2007;12(1):111. [Medline].

  69. Hellman RN. Erythropoietin, gadolinium, and nephrogenic fibrosing dermopathy. Ann Intern Med. Feb 6 2007;146(3):229-30; author reply 230. [Medline].

  70. Introcaso CE, Hivnor C, Cowper S, Werth VP. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: a case series of nine patients and review of the literature. Int J Dermatol. May 2007;46(5):447-52. [Medline].

  71. Jamboti J. Strong association between the use of gadolinium-based contrast agents with nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis. Intern Med J. Jul 2007;37(7):508-9; author reply 509. [Medline].

  72. Kanal E, Broome DR, Martin DR, Thomsen HS. Response to the FDA's May 23, 2007, nephrogenic systemic fibrosis update. Radiology. Jan 2008;246(1):11-4. [Medline].

  73. Karlik SJ. Gadodiamide-associated nephrogenic systemic fibrosis. AJR Am J Roentgenol. Jun 2007;188(6):W584; author reply W585. [Medline].

  74. Khurram M, Skov L, Rossen K, Thomsen HS, Marckmann P. Nephrogenic systemic fibrosis: a serious iatrogenic disease of renal failure patients. Scand J Urol Nephrol. 2007;41(6):565-6. [Medline].

  75. Krous HF, Breisch E, Chadwick AE, Pinckney L, Malicki DM, Benador N. Nephrogenic systemic fibrosis with multiorgan involvement in a teenage male after lymphoma, Ewing's sarcoma, end-stage renal disease, and hemodialysis. Pediatr Dev Pathol. Sep-Oct 2007;10(5):395-402. [Medline].

  76. Lauenstein TC, Salman K, Morreira R, et al. Nephrogenic systemic fibrosis: center case review. J Magn Reson Imaging. Nov 2007;26(5):1198-203. [Medline].

  77. Leiner T, Herborn CU, Goyen M. Nephrogenic systemic fibrosis is not exclusively associated with gadodiamide. Eur Radiol. Aug 2007;17(8):1921-3. [Medline].

  78. Lim YL, Lee HY, Low SC, Chan LP, Goh NS, Pang SM. Possible role of gadolinium in nephrogenic systemic fibrosis: report of two cases and review of the literature. Clin Exp Dermatol. Jul 2007;32(4):353-8. [Medline].

  79. Lin SP, Brown JJ. MR contrast agents: physical and pharmacologic basics. J Magn Reson Imaging. May 2007;25(5):884-99. [Medline].

  80. Markus JS, James AJ, Nunez-Gussman JK, Sheehan AM, Fegan L, Hsu S. Nephrogenic fibrosing dermopathy. J Am Acad Dermatol. Jan 2005;52(1):166-7. [Medline].

  81. Mendichovszky IA, Marks SD, Simcock CM, Olsen OE. Gadolinium and nephrogenic systemic fibrosis: time to tighten practice. Pediatr Radiol. May 2008;38(5):489-96; quiz 602-3. [Medline].

  82. Morcos SK. Nephrogenic systemic fibrosis following the administration of extracellular gadolinium based contrast agents: is the stability of the contrast agent molecule an important factor in the pathogenesis of this condition?. Br J Radiol. Feb 2007;80(950):73-6. [Medline].

  83. Ng YY, Lee RC, Shen SH, Kirk G. Gadolinium-associated nephrogenic systemic fibrosis: double dose, not single dose. AJR Am J Roentgenol. Jun 2007;188(6):W582; author reply W583. [Medline].

  84. Othersen JB, Maize JC, Woolson RF, Budisavljevic MN. Nephrogenic systemic fibrosis after exposure to gadolinium in patients with renal failure. Nephrol Dial Transplant. Nov 2007;22(11):3179-85. [Medline].

  85. Panda S, Bandyopadhyay D, Tarafder A. Nephrogenic fibrosing dermopathy: a series in a non-Western population. J Am Acad Dermatol. Jan 2006;54(1):155-9. [Medline].

  86. Partain CL. On the potential causal relationship between gadolinium-containing MRI agents and nephrogenic systemic fibrosis. J Magn Reson Imaging. May 2007;25(5):879-80. [Medline].

  87. Pedersen M. Safety update on the possible causal relationship between gadolinium-containing MRI agents and nephrogenic systemic fibrosis. J Magn Reson Imaging. May 2007;25(5):881-3. [Medline].

  88. Pinter I, Vagasi K, Wittmann I, Nagy J. [Nephrogenic systemic fibrosis]. Orv Hetil. Sep 23 2007;148(38):1801-4. [Medline].

  89. Pryor JG, Poggioli G, Galaria N, et al. Nephrogenic systemic fibrosis: a clinicopathologic study of six cases. J Am Acad Dermatol. Jul 2007;57(1):105-11. [Medline].

  90. Pryor JG, Scott GA. Nephrogenic systemic fibrosis: a clinicopathologic study of 6 cases. J Am Acad Dermatol. Nov 2007;57(5):902-3. [Medline].

  91. Rongioletti F, Rampini P, Parodi A, Rebora A. Papular mucinosis associated with generalized morphoea. Br J Dermatol. Nov 1999;141(5):905-8. [Medline].

  92. Saab G, Abu-Alfa A. Are patients with moderate renal failure at risk for developing nephrogenic systemic fibrosis?. Radiology. Sep 2007;244(3):930-1; author reply 931-2. [Medline].

  93. Saab G, Abu-Alfa A. Will dialysis prevent the development of nephrogenic systemic fibrosis after gadolinium-based contrast administration?. AJR Am J Roentgenol. Sep 2007;189(3):W169. [Medline].

  94. Samtleben W. [Nephrogenic systemic fibrosis]. Radiologe. Sep 2007;47(9):778-84. [Medline].

  95. Scheinfeld N. Nephrogenic fibrosing dermopathy: a comprehensive review for the dermatologist. Am J Clin Dermatol. 2006;7(4):237-47. [Medline].

  96. Solomon GJ, Rosen PP, Wu E. The role of gadolinium in triggering nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy. Arch Pathol Lab Med. Oct 2007;131(10):1515-6. [Medline].

  97. Streams BN, Liu V, Liegeois N, Moschella SM. Clinical and pathologic features of nephrogenic fibrosing dermopathy: a report of two cases. J Am Acad Dermatol. Jan 2003;48(1):42-7. [Medline].

  98. Swaminathan S, Shah SV. New insights into nephrogenic systemic fibrosis. J Am Soc Nephrol. Oct 2007;18(10):2636-43. [Medline].

  99. Swartz RD, Crofford LJ, Phan SH, Ike RW, Su LD. Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med. May 2003;114(7):563-72. [Medline].

  100. Thakral C, Alhariri J, Abraham JL. Long-term retention of gadolinium in tissues from nephrogenic systemic fibrosis patient after multiple gadolinium-enhanced MRI scans: case report and implications. Contrast Media Mol Imaging. Jul 2007;2(4):199-205. [Medline].

  101. Thomsen HS. ESUR guideline: gadolinium-based contrast media and nephrogenic systemic fibrosis. Eur Radiol. Oct 2007;17(10):2692-6. [Medline].

  102. Thomsen HS, Morcos SK. Nephrogenic systemic fibrosis and nonionic linear chelates. AJR Am J Roentgenol. Jun 2007;188(6):W580; author reply W581. [Medline].

  103. Tsai CW, Chao CC, Wu VC, Hsiao CH, Chen YM. Nephrogenic fibrosing dermopathy in a peritoneal dialysis patient. Kidney Int. Nov 2007;72(10):1294. [Medline].

  104. Widmark JM. Imaging-related medications: a class overview. Proc (Bayl Univ Med Cent). Oct 2007;20(4):408-17. [Medline].

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Further Reading

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Keywords

nephrogenic systemic fibrosis, NFS, nephrogenic fibrosing dermopathy, NFD, scleromyxedemalike illness of renal disease, scleromyxedemalike illness of hemodialysis, atypical lichen myxedematosus, scleroderma, scleromyxedema, dermatofibroma protuberans

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Shawn Cowper, MD, Director, NFD Registry Project, Associate Professor, Departments of Dermatology and Pathology, Yale University
Disclosure: Nothing to disclose.

Medical Editor

Carrie L Kovarik, MD, Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine
Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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