Managing sickle-cell disease-driven leg ulcers
Wound care, managing blood perfusion and pain all key 1,294 words, 6 minutes 28 seconds
Sickle cell disease can lead to hypoxia in the skin and cause ulceration in areas already prone to low blood perfusions, such as the feet and legs.
This was the topic of Dr. Brian Kunimoto’s presentation at the 7th annual Skin Spectrum Summit on Nov. 4.
The sickle cell condition is quite common in some populations, he noted. “In Black Americans, sickle cell disease affects one in 365 births.” Rates of sickle cell are also higher in some Middle Eastern, Mediterranean, Central and South American and South Asian populations, he said.
Dr. Kunimoto is the founder and director of the Vancouver General Hospital/University of British Columbia (UBC) Wound Healing Clinic and is the Continuing Medical Education Director for the Department of Dermatology and Skin Science at UBC.
Sickle cell disease leads to hypoxia through several factors, Dr. Kunimoto said. The abnormal formation of red blood cells in sickle cell disease causes them to adhere to one another and white blood cells and the endothelium, causing clumping, he said. Also, the altered shape of the red blood cells reduces their ability to deform as they travel through blood vessels, effectively increasing blood viscosity. “The red blood cells that sickle become very fragile. And this results in both intravascular and extravascular hemolysis,” he said.
The condition also impairs nitric oxide-mediated vasodilation, resulting in vasoconstriction.
“And lastly, we will see the initiation of intravascular coagulation,” Dr. Kunimoto said.
Leg and foot ulcers, including those driven by sickle cell disease, tend to occur in areas that are very susceptible to low perfusion, Dr. Kunimoto said. These areas include the anterior shin, the medial malleolus, the lateral malleolus and the dorsal foot. “These are areas where pressure can reduce perfusion and initiate this whole [hypoxia and ulceration] process.”
Dr. Kunimoto made several recommendations for managing patients with these ulcers:
Management of the ulcer itself should encompass moisture management, debridement of biofilms, monitoring colonization, infection, and inflammation
Physicians should manage any chronic venous insufficiency. Compression bandages can lead to significant improvements
Patients with sickle cell disease and leg ulcers can experience significant pain, impacting their quality of life. This pain should be managed carefully
Good hydration can reduce the symptoms of sickle cell disease. Intravenous hydration may be of some use
Many patients with sickle cell disease are treated with hydroxyurea to improve blood oxygenation, but long-term, high-dose use of this medication in myeloproliferative disorders has been linked to the development of ulcers
Bottom line
Sickle cell disease is common in some populations with skin of colour, and it can induce tissue hypoxia that can lead to ulcers on the feet and legs. Early intervention may be beneficial in terms of pain relief and healing. Dermatologists should look for venous insufficiency, start treatment as soon as possible, and consult with experts in pain management because the pain in these patients is severe and management of that is critical.
From the literature on sickle cell disease and leg ulcers
Common, but neglected: A comprehensive review of leg ulcers in sickle cell disease
This literature review retrieved studies from the MEDLINE, PubMed, EMBASE, and Web of Science databases. It summarizes the current updated information on pathophysiology, diagnosis, and available treatment options related to leg ulcers and sickle cell disease.
The authors write that they hope this detailed discussion will lead to a better appreciation of t’ better appreciation of this clinical problem. This greater appreciation will have a long-term positive effect on the quality of life of patients with SCD.
Hydroxyurea-induced genital ulcers and erosions: Two case reports
The authors of this paper note that hydroxyurea often used to treat sickle cell anemia, is well known to cause painful mucocutaneous ulcers, typically involving the legs or mouth. They also note that ulcerations from this medication can also occur in the genital area. Ulcers in this area are more rare and may be underrecognized, leading to delays in diagnosis and management.
Two cases of genital ulceration in patients on hydroxyurea therapy are presented, both of which resolved completely within weeks of discontinuation of the therapy.
Insights into the skin microbiome of sickle cell disease leg ulcers
This study utilizes gene sequencing to describe sickle cell disease leg ulcer microbiome and its association with clinical factors. The authors recovered microbial profiles from an analysis of 42 ulcers. They identified profiles similar to other chronic wounds in the predominance of anaerobic bacteria and opportunistic pathogens, including Staphylococcus, Corynebacterium, and Finegoldia.
The investigators found that ulcers with a smaller surface area were defined by the predominance of Staphylococcus, while those with a larger area had a greater diversity of microorganisms. They also linked the relative abundance of certain types of microbes with disease severity, CD19+ B cell count, and other factors of the pathophysiology of sickle cell disease.
“Investigations of the skin microbiome in relation to SCD (sickle cell disease) ulcer pathophysiology can inform clinical guidelines for this poorly understood chronic wound, as well as enhance broader understanding about the role of the skin microbiome in delayed wound healing,” the authors write.
Hematological indices of sickle cell patients with chronic leg ulcers on compression therapy
This study in 18 patients with sickle cell disease and chronic leg ulcers evaluated the patients' hematological parameters while on high compression bandaging. The goal was to provide data for improved ulcer management and prevention. Another 18 age- and sex-matched sickle cell patients with no history of chronic leg ulcers served as controls.
Compared to the control group, hematological indices were more severe in leg ulcer patients at the start of treatment. The investigators isolated Pseudomonas aeruginosa, Staphylococcus aureus, Proteus sp., Escherichia coli and Klebsiella oxytoca from the ulcers.
They conclude that measures to improve hematological parameters during leg ulcer treatment in patients with sickle cell disease should be taken to aid wound healing.
VIDEO: Leg and foot ulcers in sickle cell disease—Doctor Simone says...
At the intersection of skin and society
Vancouver-based visual artist Sandeep Johal is presenting an exhibition titled “What if?” In the exhibition, (details here) Johal has used textiles, paintings, drawings, and animation to layer her personal history with the histories of South Asian women she wished she knew about as a first-generation South Asian youth. These are women she considers role models, pioneers, trailblazers, vigilantes, and rebels.
The exhibition can be seen in person at the Surrey Art Gallery in Surrey, B.C. until Dec. 11, 2021.
‘What if?’ takes the form of Johal’s own bedroom from her teen years, reimagined with the western influences replaced with images of these influential women. Examples include the bandit queen Phoolan Devi, suffragette Sophia Duleep Singh, and Laxmi Bai, leader of the Indian Rebellion of 1857. Images of these women and others replace the ‘90s music stars the Spice Girls on a poster.
Johal also highlights the stories of several murdered women through an art piece titled Rest in Power. This series of 12 goddesses is rendered in Johal’s black and white graphic line drawing style with colourful geometric patterns and shapes.
The museum’s page explains that through the What If? Exhibition “Johal gives South Asian girls a different story to grow up with, celebrating feminist resistance while creating space for alternative narratives. She returns to the past and invites the visitor to come along and imagine a different future—a better future. What If? is a quest for self-discovery and reconnection to past heritage.”
This week
November is Indigenous Disability Awareness Month in Canada
Nov. 14 is World Diabetes Day
Something to think about in the week ahead...
Next week
The 7th annual Skin Spectrum Summit delivered important information for attendees in its two sessions on Nov. 4 and 6. Next week Skin Spectrum Weekly will continue its coverage of the Summit talks with Dr. Renée Beach’s presentation on her top tips for managing Afro-textured hair.
Recordings of the Summit presentations will soon be available for registered delegates. Please watch this space for more details.