Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy).

摘要: Nephrogenic fibrosing dermopathy (NFD), a newly recognized scleroderma-like disease, was originally described as purely cutaneous disorder. More widespread involvement, including fibrosis of pulmonary and cardiac tissues, has been documented only recently, it suggested that more appropriate designation is dialysis-associated systemic fibrosis. We report five cases this novel disorder, spanning spectrum primarily skin to muscle involvement. Clinical, radiological, electrophysiological, pathological studies revealed moderate severe striated muscles. All patients had end-stage renal failure on chronic dialysis, subacute hardening the muscles, restriction limb movements with joint contractures, but normal mildly weak strength. Limitation caused predominantly by tightness induration, contractures rather than weakness. Computerized tomography showed fascia muscles in most severely affected patients, electromyography mild myopathic changes. Histopathology fibrosis, degenerating fibers, inflammatory cells. These results further support contention NFD not part larger fibrotic process may involve