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

Eric Edgar, Randall (Randy) Woltjer, Ruth Whitham, Sakir Gultekin, Suzanne Watnick, Edward J. Cupler

Research output: Contribution to journalArticle

4 Citations (Scopus)

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 2010

Fingerprint

Nephrogenic Fibrosing Dermopathy
Muscular Diseases
Gadolinium
Muscles
Skin
Fascia
Muscle Weakness
Acute Kidney Injury
Renal Insufficiency
Extremities
Fibroblasts
Biopsy

Keywords

  • CD34+ fibroblasts
  • Myopathy
  • Nephrogenic systemic fibrosis

ASJC Scopus subject areas

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

Cite this

Nephrogenic systemic fibrosis presenting as myopathy : A case report with histopathologic correlation. / Edgar, Eric; Woltjer, Randall (Randy); Whitham, Ruth; Gultekin, Sakir; Watnick, Suzanne; Cupler, Edward J.

In: Neuromuscular Disorders, Vol. 20, No. 6, 06.2010, p. 411-413.

Research output: Contribution to journalArticle

Edgar, Eric ; Woltjer, Randall (Randy) ; Whitham, Ruth ; Gultekin, Sakir ; Watnick, Suzanne ; Cupler, Edward J. / Nephrogenic systemic fibrosis presenting as myopathy : A case report with histopathologic correlation. In: Neuromuscular Disorders. 2010 ; Vol. 20, No. 6. pp. 411-413.
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