Phlegmasia cerulea dolens (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding phlegmasia alba dolens, is an uncommon severe form of lower extremity

deep venous thrombosis Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enla ...

(DVT) that obstructs blood outflow from a vein. Upper extremity PCD is less common, occurring in under 10% of all cases. PCD results from extensive

thrombotic Thrombosis (from Ancient Greek "clotting") is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel (a vein or an artery) is injured, the body uses platelets (thro ...

occlusion (blockage by a

thrombus A thrombus (plural thrombi), colloquially called a blood clot, is the final product of the blood coagulation step in hemostasis. There are two components to a thrombus: aggregated platelets and red blood cells that form a plug, and a mesh of ...

) of extremity veins, most commonly an "iliofemoral" DVT of the

iliac vein In human anatomy, iliac vein refers to several anatomical structures located in the pelvis: * Common iliac vein, formed by the external and internal iliac veins, drains into the inferior vena cava * Deep circumflex iliac vein, formed by the union ...

and/or

common femoral vein In the human body, the femoral vein is a blood vessel that accompanies the femoral artery in the femoral sheath. It begins at the adductor hiatus (an opening in the adductor magnus muscle) as the continuation of the popliteal vein. It ends at th ...

. It is a medical emergency requiring immediate evaluation and treatment.

Symptoms and signs

Primary symptoms

It is characterized by progressive lower extremity edema distal to the thigh, tight shiny skin, cyanosis (inadequate blood oxygenation), petechiae or

purpura Purpura () is a condition of red or purple discolored spots on the skin that do not blanch on applying pressure. The spots are caused by bleeding underneath the skin secondary to platelet disorders, vascular disorders, coagulation disorders, ...

, and sudden severe pain of the affected limb in proportion to the level of venous blockage. Patients often have difficulty walking. Blisters, bullae, paresthesias, and motor weakness may develop in severe cases, along with gangrene in ~50% of cases. Distal pulses are palpable early on but may diminish over time, and doppler signal can be usually heard throughout disease progression. The left limb is more commonly affected due to its vascular anatomy (the right internal iliac artery directly overlies the left iliac vein).

Associated diseases

PCD is associated with an underlying

malignancy Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

in 20-40% of cases. There is a high risk of massive

pulmonary embolism Pulmonary embolism (PE) is a blockage of an artery in the lungs by a substance that has moved from elsewhere in the body through the bloodstream ( embolism). Symptoms of a PE may include shortness of breath, chest pain particularly upon breathin ...

, even under

anticoagulation Anticoagulants, commonly known as blood thinners, are chemical substances that prevent or reduce coagulation of blood, prolonging the clotting time. Some of them occur naturally in blood-eating animals such as leeches and mosquitoes, where the ...

Etiology

Risk factors, present in around 50% of documented cases, include malignancy, hyper-coagulable states,

cardiac The heart is a muscular organ in most animals. This organ pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as carbon dioxide to t ...

disease, venous stasis, venous insufficiency, May-Thurner syndrome (right iliac artery compressing the left iliac vein that runs beneath it), surgery, trauma,

pregnancy Pregnancy is the time during which one or more offspring develops (gestation, gestates) inside a woman, woman's uterus (womb). A multiple birth, multiple pregnancy involves more than one offspring, such as with twins. Pregnancy usually occur ...

, inferior vena cava (IVC) filter, hormone therapy,

oral contraceptives Oral contraceptives, abbreviated OCPs, also known as birth control pills, are medications taken by mouth for the purpose of birth control. Female Two types of female oral contraceptive pill, taken once per day, are widely available: * The combin ...

, prolonged immobilization, inflammatory bowel disease, heart failure, and central venous catheters. Etiology is unknown in ~10% of PCD cases. BladderCADVTleftMark

Pathophysiology

When a

occludes an extremity

vein Veins are blood vessels in humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenat ...

, pressure backs up in the venous system leading plasma fluid to leak out into the interstitium of the affected limb. This increases the pressure of that limb compartment, which can collapse the arteries and lead to acute ischemia, gangrene, hypovolemia, and hemodynamic instability.

Diagnosis

PCD is best diagnosed with contrast venography, but venous duplex ultrasonography is used more commonly in clinical practice. Magnetic resonance and computed tomography

venography Venography (also called phlebography or ascending phlebography) is a procedure in which an x-ray of the veins, a venogram, is taken after a special dye is injected into the bone marrow or veins. The dye has to be injected constantly via a catheter ...

can also be used.

Differential Diagnosis

DDx is as follows: *

Cellulitis Cellulitis is usually a bacterial infection involving the inner layers of the skin. It specifically affects the dermis and subcutaneous fat. Signs and symptoms include an area of redness which increases in size over a few days. The borders of ...

* Venous insufficiency * Superficial thrombophlebitis *

DVT Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enla ...

* Arterial embolism *

Lymphedema Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fl ...

* Ruptured Bakers cyst

Treatment

Treatment for PCD includes immediate

, fluid resuscitation, bed rest, limb elevation above 60º, limb wrap to reduce pain and edema, and either catheter-based thrombolysis, percutaneous transluminal

angioplasty Angioplasty, is also known as balloon angioplasty and percutaneous transluminal angioplasty (PTA), is a minimally invasive endovascular procedure used to widen narrowed or obstructed arteries or veins, typically to treat arterial atheroscle ...

, or surgical venous

thrombectomy Mechanical thrombectomy, or simply thrombectomy, is the interventional procedure of removing a blood clot (thrombus) from a blood vessel. It is commonly performed in the cerebral arteries (interventional neuroradiology). The effectiveness of throm ...

+/-

fasciotomy Fasciotomy or fasciectomy is a surgical procedure where the fascia is cut to relieve tension or pressure in order to treat the resulting loss of circulation to an area of tissue or muscle. Fasciotomy is a limb-saving procedure when used to tr ...

to remove the

blood clot A thrombus (plural thrombi), colloquially called a blood clot, is the final product of the blood coagulation step in hemostasis. There are two components to a thrombus: aggregated platelets and red blood cells that form a plug, and a mesh of ...

. Some people also suggest an IVC filter before thrombolysis.

Prognosis

PCD is fully reversible if the causal venous thrombus is promptly removed. In the 40-60% of people who go on to develop venous gangrene, there is a 20-50% risk of amputation and 20-40% mortality rate. Following PCD resolution patients are more likely to develop venous insufficiency and

post-thrombotic syndrome Post-thrombotic syndrome (PTS), also called postphlebitic syndrome and venous stress disorder is a medical condition that may occur as a long-term complication of deep vein thrombosis (DVT). Signs and symptoms Signs and symptoms of PTS in the leg ...

A grading system has been established : * Grade I = non-complicated (no blistering, strong sensory-motor function and strong distal pulses) * Grade II = impending venous gangrene (blistering, weak sensory-motor function and weak distal pulses) * Grade III = venous gangrene

Epidemiology

PCD is most likely to occur in people in their 50s and 60s, but can occur as early as 6 months old. There is slight male predominance of around 1.5:1.

History

This phenomenon was first discovered by Fabricus Hildanius in the 16th century, and was officially termed "phlegmasia cerulea dolens" by Gregoire in 1938. ''Phlegmasia'' originates from the

Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...

root ''phlegma'' (

inflammation Inflammation (from la, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants, and is a protective response involving immune cells, blood vessels, and molec ...

), ''cerulea'' originates from

Latin Latin (, or , ) is a classical language belonging to the Italic branch of the Indo-European languages. Latin was originally a dialect spoken in the lower Tiber area (then known as Latium) around present-day Rome, but through the power of the ...

root ''caeruleus'' (dark blue), and ''dolens'' originates from Latin word ''dolens'' (suffering).

References

External links

{{DEFAULTSORT:Phlegmasia Cerulea Dolens Inflammations Vascular diseases