Phlegmasia cerulea dolens
Phlegmasia cerulea dolens (PCD) is a rare, limb‑threatening complication of extensive deep venous thrombosis (usually iliofemoral) in which thrombotic occlusion of major and collateral veins causes severe venous congestion, marked oedema, cyanosis and threatened arterial perfusion. Rapid recognition and urgent multidisciplinary escalation are essential to reduce the risk of tissue loss, amputation and systemic complications.
Clinical summary
- Presentation: sudden severe limb pain, massive tense swelling, dusky/blue skin, possible blistering or early necrosis; peripheral pulses may be reduced if arterial flow is compromised, but pulses can be preserved early despite severe venous disease.
- Key risk factors: most common in age 50-60 years; malignancy (≈50% of cases), pregnancy/postpartum, recent surgery or trauma, central venous catheters, inherited thrombophilia and other prothrombotic states.
- Significance: PCD is a vascular emergency requiring prompt anticoagulation, limb support and urgent discussion with vascular surgery and interventional radiology for definitive revascularisation (thrombolysis, mechanical thrombectomy) or surgical thrombectomy when indicated (see NICE NG158 and relevant IPG documents).
Pathophysiology (concise)
- Extensive proximal venous thrombosis causes venous outflow obstruction leading to marked venous hypertension, interstitial oedema and impaired microcirculation.
- Rising compartment/interstitial pressures can reduce arterial inflow, producing threatened or actual limb ischaemia and increasing risk of skin necrosis, compartment syndrome and gangrene.
- Reperfusion after intervention can also precipitate compartment syndrome and reperfusion injury.