SAEM Clinical Images Series: Blue is Bad

66 year-old-male with a history of type 2 diabetes and hypertension presented as a transfer for rapid progression of lower extremity pain, swelling, and blue-purple discoloration of the entire limb with concern for a possible necrotizing infection. His symptoms began earlier in the day and progressed over just a few hours. He had no known thromboembolic risk factors.

MSK: Swollen, tender, and blue/purple-colored right lower extremity

Complete Blood Count (CBC): WBC 8.7; Hb 15; Hct 45; Plt 172

Glucose: 472

Severe venous thromboembolism (VTE), also known as Phlegmasia Cerulea Dolens, which means “painful blue inflammation”, is commonly seen with a unilateral exquisitely tender, swollen, and bluish/purple-colored lower extremity.

Phlegmasia Cerulea Dolens has a high amputation rate secondary to venous gangrene or compartment syndrome. If no pulse is noted on exam, a high suspicion for compartment syndrome must be maintained.

Take-Home Points

  • Phlegmasia Cerulea Dolens is a rare ischemic complication of massive venous thromboembolism with amputation and mortality rates as high as 50% and 40% respectively.
  • Phlegmasia Cerulea Dolens tends to affect the iliofemoral segment of the lower extremities and is commonly associated with malignancy.
  • The preferred imaging modality is doppler ultrasound. Management includes limb elevation, IV fluids, and either systemic anti-coagulation, catheter-directed thrombolysis, and/or thrombectomy.

  • Bazan HA, Reiner E, Sumpio B. Management of bilateral phlegmasia cerulea dolens in a patient with subacute splenic laceration. Ann Vasc Dis. 2008;1(1):45-8. doi: 10.3400/avd.AVDcr07002. Epub 2008 Feb 15. PMID: 23555338; PMCID: PMC3610218.
  • Chaochankit W, Akaraborworn O. Phlegmasia Cerulea Dolens with Compartment Syndrome. Ann Vasc Dis. 2018 Sep 25;11(3):355-357. doi: 10.3400/ PMID: 30402189; PMCID: PMC6200621.
  • Gardella L, Faulk J. Phlegmasia Alba And Cerulea Dolens. 2022 Oct 3. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 33085284.
  • Said A, Sahlieh A, Sayed L. A comparative analysis of the efficacy and safety of therapeutic interventions in phlegmasia cerulea dolens. Phlebology. 2021 Jun;36(5):392-400. doi: 10.1177/0268355520975581. Epub 2020 Nov 25. PMID: 33236674.
  • Schroeder M, Shorette A, Singh S, Budhram G. Phelgmasia Cerulea Dolens Diagnosed by Point-of-Care Ultrasound. Clin Pract Cases Emerg Med. 2017 Mar 13;1(2):104-107. doi: 10.5811/cpcem.2016.12.32716. PMID: 29849409; PMCID: PMC5965407.