Skin Clinical Manifestations of Phlegmasia Cerulea Dolens
Phlegmasia cerulea dolens presents with a triad of severe skin manifestations: massive edema, cyanotic (blue) discoloration, and severe pain of the affected limb. 1, 2
Primary Cutaneous Features
The hallmark skin findings that distinguish phlegmasia cerulea dolens from other vascular emergencies include:
Cyanotic discoloration: The limb develops a characteristic blue or purple color due to total or near-total occlusion of both deep and superficial venous systems, causing severe venous congestion 1, 2
Massive edema: Profound swelling affects the entire extremity, resulting from fluid sequestration and severe venous hypertension 1, 3
Tense compartments: The skin becomes extremely tight and distended as compartment pressures can increase up to 16-fold within 6 hours 4
Progressive Skin Changes
As the condition advances, additional cutaneous manifestations emerge:
Venous gangrene: In severe cases, skin necrosis and gangrenous changes develop, occurring in a significant proportion of patients and often necessitating amputation 3, 5
Loss of pulses: Pulselessness accompanies the cyanotic skin changes due to arterial compromise from compartment syndrome 3
Skin tension: The affected limb exhibits marked skin tautness from the massive fluid accumulation and venous congestion 4, 6
Distinguishing Features from Other Conditions
The American College of Radiology specifically differentiates phlegmasia cerulea dolens from acute arterial ischemia by emphasizing that massive edema combined with cyanotic discoloration is pathognomonic for this venous emergency, rather than the pale, pulseless presentation of arterial occlusion 1, 7
Clinical Urgency
These skin manifestations represent a vascular emergency with mortality rates up to 40% and amputation rates up to 50% if not treated immediately 7, 3. The presence of cyanosis, worsening pain, and these cutaneous findings indicates the need for immediate escalation to catheter-directed thrombolysis or surgical intervention 2.