Nephrogenic systemic fibrosis presenting as myopathy: a case report with histopathologic correlation
| Autor: | Eric M. Edgar, Ruth H. Whitham, S. Humayun Gultekin, Randy Woltjer, Suzanne Watnick, Edward Cupler |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Male
Pathology medicine.medical_specialty Biopsy CD34 Neural Conduction Contrast Media Antigens CD34 Gadolinium Kidney Nephrogenic Fibrosing Dermopathy Polyneuropathies Fibrosis medicine Humans Myopathy Muscle Skeletal Genetics (clinical) Muscle biopsy Muscle Weakness medicine.diagnostic_test business.industry Magnetic resonance imaging Fascia Fibroblasts Middle Aged medicine.disease Magnetic Resonance Imaging medicine.anatomical_structure Neurology Nephrogenic systemic fibrosis Pediatrics Perinatology and Child Health Neurology (clinical) medicine.symptom business |
| Zdroj: | Neuromuscular disorders : NMD. 20(6) |
| ISSN: | 1873-2364 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect