Clinical manifestation

Most commonly involves extremities or trunk, but may involve perineum (Fornier's

Nephrogenic fibrosing dermopathy 409

gangrene); often follows trauma, surgical wound or hematogenous seeding from another site; early, severe, local pain, out of proportion to visible findings; poorly marginated red plaque with subcutaneous edema, which progresses to dusky plaque with vesiculation and occasional crepitus; marked constitutional changes, including fever, prostration, decreased sensorium, and hypotension

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