TY - JOUR
T1 - Nephrogenic systemic fibrosis presenting as myopathy
T2 - A case report with histopathologic correlation
AU - Edgar, Eric
AU - Woltjer, Randy
AU - Whitham, Ruth
AU - Gultekin, S. Humayun
AU - Watnick, Suzanne
AU - Cupler, Edward J.
PY - 2010/6
Y1 - 2010/6
N2 - 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.
AB - 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.
KW - CD34+ fibroblasts
KW - Myopathy
KW - Nephrogenic systemic fibrosis
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U2 - 10.1016/j.nmd.2010.04.001
DO - 10.1016/j.nmd.2010.04.001
M3 - Article
C2 - 20462761
AN - SCOPUS:77953123686
VL - 20
SP - 411
EP - 413
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
SN - 0960-8966
IS - 6
ER -