Epidermolysis bullosa presents unique woundcare challenges

September 10, 2014

It’s advisable for clinicians to release the fluid in blisters that develop in children with epidermolysis bullosa (EB), but they should aim to keep the roof of the blister intact, an expert recommends.

Toronto - It’s advisable for clinicians to release the fluid in blisters that develop in children with epidermolysis bullosa (EB), but they should aim to keep the roof of the blister intact, an expert recommends.

“You want to release the fluid, but the roof of the blister serves as a biological membrane,” says Elena Pope, M.D., M.Sc., F.R.C.P.C., head of the section of dermatology, Division of Pediatric Medicine, Hospital for Sick Children in Toronto. She is also associate professor, department of paediatrics, University of Toronto. Dr. Pope spoke at a pediatric wound symposium organized through the Hospital for Sick Children.

Woundcare in EB - a cluster of genetic conditions causing the skin to be fragile and blister easily - is a complex aspect of the management of the condition, Dr. Pope notes. As with general woundcare, local and systemic factors have to be taken into account. Age is a factor that needs to be considered when developing a woundcare plan for a pediatric patient with EB, as each age presents its own challenges, she says. The severity and extent of the wounds depends on the type of EB.

Types of EB

EB simplex is characterized by blisters on the palms and soles and mild thickening of the soles, but there is no adverse impact on the life span of patients, Dr. Pope says. Another form of the condition Herlitz junctional EB, however, can shorten the lives of patients and carries a high-risk of complications. It is characterized by periorificial blistering, hypergranulation tissue and periungual involvement, as well as nail shedding. Patients can experience poor growth and there can be significant airway involvement, she says.

Recessive dystrophic EB is another form of the condition, and it presents with chronic blisters and wounds; scarring and contractures can develop, and it is associated with an increased risk of skin cancer.

“The majority of the patients die secondary to squamous cell carcinoma,” Dr. Pope says.

To minimize trauma in EB, physicians are urged to apply dressings that are described as “non-stick,” Dr. Pope says.

“We usually use silicone dressings,” she says. “You also want to avoid adhesives, such as tapes, stick probes at any cost.”

Moreover, the choice of a dressing is affected by parameters such as the presence of exudate, degree of exudate, and critical colonization, Dr. Pope explains. There are medical adhesive sprays that can be employed to remove dressings in an atraumatic fashion.

Promoting wound healing

When wounds present in patients with severe forms of EB, they can become “stuck,” or non-healing. In such instances, it may be a wise step to administer a therapy such as low-dose tetracycline.

“Such a treatment will reduce inflammation and promote wound healing in wounds that are stuck,” Dr. Pope says.

Aiming to decrease the total bioburden should be an objective in woundcare in EB. Bathing is a means of achieving that. Another consideration in woundcare for EB is to alternate therapies such as topical antimicrobials, to avoid possible sensitization and the potential for resistance to treatment, Dr. Pope says.

“Be mindful of your overall use (of therapies),” she says.

EB can be more challenging to treat on some sites of the body. The diaper area, for example, is complex to treat because of the presence of urine and feces. As in the management of diaper dermatitis, the generous application of zinc oxide in the diaper area is an effective management practice, particularly with junctional EB.

Disclosures: Dr. Pope reports no relevant financial interests.