Phlegmasia cerulea dolens (PCD) is a rare condition characterized by deep venous thrombosis with gangrene. It can result in critically severe edema that affects the blood supply to the limbs. PCD generally occurs in the lower rather than upper extremity. We herein present a case report of upper extremity PCD and discuss thrombophilia secondary to low protein S activity as the main cause. Catheter-directed thrombolysis via the occluded end of the artery may be one of the best treatment methods for PCD.
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