Phlegmasia cerulea dolens: case report on a HIV-AIDS patient in a sub-saharian semi-urban practice

Venous thromboembolism has also become a major health concern in sub-saharian Africa. Studies addressing at this issue are rare in Cameroon. Thus, the case reported here presents singular characteristics: its clinical form, phlegmasia cerulea dolens, a severe but uncommon complication of venous thromboembolism; and its infrequent recorded triggering factor, HIV-AIDS.

All two are severe forms of VTE resulting from acute massive thrombosis and obstruction of venous drainage of the extremity. In PCD, venous occlusion extends to collateral veins, resulting in massive edema simulating arterial embolism; that differs to PAD (an early stage of PCD) in which ischemia is not described [3]. When capillaries are involved, irreversible gangrene installs in the skin, subcutaneous tissue or the muscle. Of the reported triggering factors, cancer is the most common; others include hypercoagulation syndrome, trauma, surgery, chronic colitis, heart failure, mitral valve stenosis, vena caval filter insertion and May-Thurner syndrome. Pregnancy is another risk condition, especially during the third trimester when the uterus compresses more the left common iliac vein. We have not found a literature on PCD describing implication of HIV-AIDS, even though it is now wellestablished that the pandemic constitutes an emerging VTE risk factor.

Patient and observation
It concerns a 47 years male who was admitted at Bamenda Military Hospital. In the past history, he declared smoking (about 10 packetyears) and alcohol consumption; but he has always been well in the exception of occasional fatigue attributed to hard work. Two months previous the hospitalization, he felt pains on the right ankle; the joint also showed edema which extended upward to the knee. For the patient and his family, these manifestations were due to witchcraft; he was then treated by a traditional practitioner. As the situation worsened, with pains and edema progressing, he was forced to consult in our service. At presentation, general signs showed a temperature of 38°4 C, weight 67 kg (BMI 23.4 kg/m2), pulse 102, and respiratory rate 18. He was unable to walk; presenting a warm shining, painful, discolored and swollen right calf and leg. A gangrenous area was formed on the foreleg (Figure 1).
The pain was more severe on pressure over the dilated and hardened popliteal and femoral vessels. Peripheral pulses were nonpalpable. There was no history of trauma. This presentation was very suggestive of phlegmasia cerulea dolens.

Discussion
Phlegmasia dolens (PD) is a rare complication of VTE. It occurs at all age but is more common in the fifth and sixth decades [2].
Gender predominance is not established; left-sided involvement is more frequent (3 to 4: 1 ratio). Upper limbs are involved in less than 5% [2]. PD results of acute and massive thrombosis in the lower limb veins with significant compromised vein outflow. It (+) cell count, protein S deficiency, protein C deficiency and circulating antibodies (antiphospholipid, anticardiolipid, etc...) [4]. As the same, diabetes is not known as a set off factor for PD, even though one case reported in India concerned a diabetic patient [3].
Literature does not mention whether atherosclerosis (potentially induced, in our case, by smoking and diabetes), can play a role in the onset of PD by pre-fragilizing arteries.
In the end, HIV-AIDS was the main VTE risk factors present in our patient. He did not have any of the reported triggering factors of PD. Clinically, PD presents as a triad of edema, agonizing pain and cyanosis. Conservative treatment of PD concerns PAD and mild PCD, and includes steep leg elevation, anticoagulant and fluid resuscitation. It aims to prevent venous gangrene. Steep leg elevation helps to decrease edema, while anticoagulant purposes to decrease proximal clot propagation. Paquet proposed to use thrombolysis for the treatment of PCD [5]. Other management approaches consist of catheter-directed thrombolysis associated to high doses of urokinase or tissue plasminogen activator (t-PA), or intra-arterial low-dose thrombolysis. The latest seems to be more efficient when venous gangrene is installing as the agent is delivered to arterial capillaries and, then after, to venules. Surgical thrombectomy allows rapid decrease of the hydrostatic pressure.
Fasciotomy alone or associated to thrombectomy, or thrombolysis reduces compartimental pressure; though, it significantly increases morbidity because of the prolonged wound healing and the risk of infection. Conservative management is associated to a high incidence of post-phlebitic syndrome (94% among survivors) [2].
Despite all the therapeutic modalities mentioned above, PCD and venous gangrene still remain life-threatening and limb-threatening conditions with a very high mortality (20-40%) [2]. Pulmonary embolism is responsible of 30% of the deaths related to PCD.

Conclusion
This case of PD appears important to be made aware with regards of its association with the HIV pandemia, and the history of its management out lighting the confrontation that still exist in subsaharian Africa between biomedical knowledge and socio-cultural believes.

Competing interests
The authors declare no conflict of interest.

Authors' contributions
All authors have read and agreed to the final version of this manuscript and have equally contributed to its content and to the management of the case.