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Related Experiment Videos

Nephrogenic fibrosing dermopathy with systemic involvement.

William W Ting1, Mary Seabury Stone, Kathi C Madison

  • 1Department of Dermatology, University of Iowa College of Medicine, Iowa City, USA.

Archives of Dermatology
|July 23, 2003
PubMed
Summary

Nephrogenic fibrosing dermopathy (NFD) can involve systemic calcification and fibrosis in end-stage renal disease patients. This rare complication can lead to catastrophic outcomes, highlighting the need for further investigation.

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Area of Science:

  • Dermatology
  • Nephrology
  • Pathology

Background:

  • Growing literature on cutaneous conditions in end-stage renal disease (ESRD).
  • Nephrogenic fibrosing dermopathy (NFD) characterized by sclerotic plaques and scleromyxedema-like histology in ESRD patients.
  • Soft tissue calcification is rare in NFD, and systemic involvement is unreported.

Observation:

  • A case of ESRD patient with diffuse indurated plaques consistent with NFD.
  • The patient presented with associated soft tissue calcification and severe complications.
  • Histopathology showed thickened collagen bundles, spindle cells, increased mucin, and focal dermal calcium deposition.

Findings:

  • Autopsy revealed extensive fibrosis and calcification in multiple organs, including diaphragm, psoas muscle, renal tubules, and rete testes.

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  • The patient experienced catastrophic sequelae and died 11 months after NFD onset.
  • This case demonstrates a rare association of NFD with significant soft tissue and systemic calcification.

    • Implications:

    • A subset of NFD patients may experience significant systemic involvement.
    • Highlights the potential for severe, widespread complications in NFD beyond cutaneous manifestations.
    • Suggests the need for increased vigilance and research into systemic manifestations of NFD in ESRD.