How sickle cell disease can complicate venous leg ulcers in patients of colour

Dr. Brian Kunimoto on how venous insufficiency can create a vicious circle

In his presentation at Skin Spectrum Summit 2019 in Vancouver, Dr. Brian Kunimoto spoke about sickle cell disease and its complicating effects on venous that cells pile up. “Clinically, in dermatology, we see patients who have extremely painful venous ulcers,” he said. 

These ulcers can lead to a drastic decrease in quality of life. A person with venous insufficiency can develop hypoxia around the ankles, and this can trigger a sickle cell crisis, he said. 

“Which really is a vicious circle because you get the hypoxia, you get the ulceration and then you get the sickle cell crisis, which causes more hypoxia, and it just goes around and around,” he said. 

Dr. Kunimoto explained the difference between sickle cell trait and sickle cell disease. 

“Sickle cell trait is when the situation is heterozygous, and it is an autosomal recessive inheritance of a single amino acid substitution in the beta-globin protein as part of hemoglobin,” he said. 

Patients who are heterozygous have sickle cell trait. Patients who are homozygous have sickle cell disease, he said. 

Approximately 300,000 infants worldwide are born with sickle cell disease each year, he said, the majority coming from central Africa. 

“This is truly a genetic disorder that affects people of colour,” he said. 

According to Dr. Kunimoto, one of the effects of sickle cell disease is that cells pile up.

“They get very sticky, he said. “Red blood cells stick together, they stick to platelets, they stick to white cells and they stick to the walls of the vessels.” 

This causes an increase in blood viscosity, which can trigger thrombotic events. 

“Thrombosis in the vessels becomes a real problem with plugging and potentially something known as vasculopathy,” he said. 

Because of all this, ischemic injury can occur, which can lead to reperfusion injury and a severe stimulation of inflammation, he said. 

A patient with ischemic changes may develop livedoid vasculopathy, which Dr. Kunimoto said is less common. 

Livedoid vasculopathy looks very different, he said. The ulcers can look irregular in shape. When they heal, they heal with atrophie blanche, which is associated with dilated blood vessels and pigmentation, he said. 

In managing a venous leg ulcer, he said to first rule out arterial disease by doing an arterial Doppler ultrasound. Then concentrate on local wound care, which involves periwound protection.

“A lot of them drain a lot of fluid, so you have to select a good moisture balance dressing,” he said. He also emphasized the use of compression bandaging. 

The takeaway: “If your patient has sickle cell disease, make sure you consult with a hematologist because all of the conditions are comorbidities that a hematologist can really help with,” he said. 

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FROM THE LITERATURE ON SICKLE CELL LEG ULCERS

Leg ulcers in sickle-cell disease: Treatment update

  • Though treating sickle cell leg ulcers still remains a challenge, according to researchers, a recent study evaluated the critical issues, recent advances, and future direction of treating sickle cell leg ulcers (SCLU). 

    From “Advances in Wound Care”

Sickle cell disease induces resistance to cutaneous carcinogenesis

  • In a cross-sectional study of 1543 patients with leg ulcers, researchers found that skin carcinogenesis did not occur in patients who had sickle cell disease or were treated with hydroxyurea (HU) for myeloproliferative neoplasms.

From "Orphanet Journal of Rare Diseases"

Self-care deficits in women with leg ulcers and sickle cell disease

  • For women with leg ulcers and sickle cell disease, investigators have noted that self-care requirements are under met. These requirements include rest and proper nutrition, social interaction, and environmental stability. Researchers recommended planned actions for comprehensive care.

    From "Brazilian Journal of Nursing"

VIDEO: Model Helps Understand How Electricity Promotes Healing of Wounds

AT THE INTERSECTION OF SKIN & SOCIETY

  • In a story published by National Geographic, Black actors responsible for accurately portraying historical figures and acting out historical events talk about the challenges and goals that come along with their roles. “We still haven’t found the sweet spot to make sure that the full story is told, but we’re further than we’ve ever been,” says Stephen Seals (pictured below), who portrays enslaved American Revolution spy James Armistead Lafayette at Colonial Williamsburg. Read the full story here.

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