Nephrogenic systemic fibrosis presenting as myopathy: a case report with histopathologic correlation.
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.
Edgar
Eric
E
Department of Neurology, Oregon Health & Science University, Portland, OR, USA.
Woltjer
Randy
R
Whitham
Ruth
R
Gultekin
S Humayun
SH
Watnick
Suzanne
S
Cupler
Edward J
EJ
D018952:Antigens, CD34 / Q000235:genetics; D001706:Biopsy; D003287:Contrast Media / Q000009:adverse effects; D005347:Fibroblasts / Q000473:pathology; D005355:Fibrosis; D005682:Gadolinium / Q000009:adverse effects; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology; D054989:Nephrogenic Fibrosing Dermopathy / Q000235:genetics / Q000473:pathology*; D009431:Neural Conduction / Q000502:physiology; D011115:Polyneuropathies / Q000473:pathology
D018952:Antigens, CD34; D003287:Contrast Media; D005682:Gadolinium