Nephrogenic systemic fibrosis presenting as myopathy: A case report with histopathologic correlation

Eric Edgar, Randy Woltjer, Ruth Whitham, S. Humayun Gultekin, Suzanne Watnick, Edward J. Cupler

Research output: Contribution to journalArticle

4 Scopus citations

Abstract

Nephrogenic systemic fibrosis is primarily a skin disorder associated with renal insufficiency and exposure to gadolinium-containing (GAD+) contrast. We present the case of a 64-year-old man who was exposed to gadolinium while in acute renal failure, and months later developed limb stiffness, proximal weakness, and woody muscle texture. Muscle biopsy demonstrated chronic non-inflammatory fibrosing myopathy. CD34+ fibroblasts have previously been reported to be specific for nephrogenic systemic fibrosis dermopathy, and we found these in fibrotic areas of muscle and fascia. Nephrogenic systemic fibrosis is an emerging disorder, and our case highlights that it may present as a progressive myopathy with minimal skin findings.

Original languageEnglish (US)
Pages (from-to)411-413
Number of pages3
JournalNeuromuscular Disorders
Volume20
Issue number6
DOIs
StatePublished - Jun 1 2010

Keywords

  • CD34+ fibroblasts
  • Myopathy
  • Nephrogenic systemic fibrosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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