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Nephrogenic fibrosing dermopathy.

S E Cowper1, L D Su, J Bhawan

  • 1Department of Pathology, University of California, San Francisco, California, USA. pathmax@yahoo.com

The American Journal of Dermatopathology
|January 22, 2002
PubMed
Summary
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A new fibrosing skin disorder, nephrogenic fibrosing dermopathy (NFD), affects patients with renal disease, particularly those on dialysis. Histopathology reveals increased fibroblasts, collagen, mucin, and CD-34 positive dermal dendrocytes.

Area of Science:

  • Dermatopathology
  • Nephrology
  • Fibrosing Disorders

Background:

  • A novel fibrosing disorder has emerged in patients with renal disease.
  • Cases were identified in renal transplant recipients and dialysis patients.
  • The condition presents with cutaneous indurated plaques and papules, resembling scleromyxedema but lacking systemic features.

Purpose of the Study:

  • To detail the histopathologic findings of this newly identified fibrosing disorder.
  • To characterize the cellular and extracellular matrix changes.
  • To propose a nomenclature for the condition.

Main Methods:

  • Routine microscopy of skin biopsies.
  • Special stains for mucin and elastic fibers.
  • Immunohistochemistry for CD-34, Factor XIIIa, and CD-68.

Related Experiment Videos

  • Electron microscopy.

    • Main Results:

    • Histopathology shows fibroblast and dendritic cell proliferation, thick collagen bundles, increased mucin, and elastic fibers.
    • CD-34 positive dermal dendrocytes are abundant, forming a dense network.
    • Findings extend into the subcutis, with some cases resembling sarcoma.
    • No infectious or toxic agent has been identified.

    Conclusions:

    • The disorder, termed nephrogenic fibrosing dermopathy (NFD), is characterized by significant dermal and subcutaneous fibrosis.
    • The etiology remains unknown, though an infectious or toxic agent was initially suspected.
    • Further investigation is needed to identify the cause of NFD.