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Volume 20, Issue 6, Pages 411-413 (June 2010

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9 of 14

Nephrogenic systemic fibrosis presenting as myopathy: A case report with histopathologic correlation
Eric Edgara, Randy Woltjerb, Ruth Whithama, S. Humayun Gultekinb, Suzanne Watnickc, Edward J. Cuplera

Received 15 December 2009; received in revised form 3 March 2010; accepted 1 April 2010.

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.
Keywords: Nephrogenic systemic fibrosis, CD34+ fibroblasts, Myopathy
a Department of Neurology, Oregon Health & Science University, Portland, OR, USA b Department of Neuropathology, Oregon Health & Science University, Portland, OR, USA c Department of Nephrology, Oregon Health & Science University, Portland, OR, USA Corresponding author. Address: Department of Neurology, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, CR120, Portland, OR 97239, USA. Tel.: +1 503 418 2125; fax: +1 503 494 0966. PII: S0960-8966(10)00166-5 doi:10.1016/j.nmd.2010.04.001 © 2010 Elsevier B.V. All rights reserved.