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

doi:10.1016/j.nmd.2010.04.001

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 journal › Article › peer-review

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 language	English (US)
Pages (from-to)	411-413
Number of pages	3
Journal	Neuromuscular Disorders
Volume	20
Issue number	6
DOIs	https://doi.org/10.1016/j.nmd.2010.04.001
State	Published - 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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10.1016/j.nmd.2010.04.001

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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.",

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AU - Cupler, Edward J.

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