Guideline Offers Management and Monitoring Recommendations for Epidermolysis Bullosa

Epidermolysis bullosa
Epidermolysis bullosa
The guideline explains the precautions that should be taken when treating patients with epidermolysis bullosa, as well as recommendations for podiatry treatment.

An expert committee has released an evidence-based clinical guideline for foot care in patients with epidermolysis bullosa (EB), including recommendations on footwear selection, management of EB dystrophic nails, and mobility assessment. The guideline was published in the British Journal of Dermatology.

The clinical guideline was based on a systematic literature review performed by members of the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) International. Only articles that related to the podiatric care of patients with EB, some identified as early as 1979, were included. The creation of the guideline focused on patients of all ages. Following development, the clinical guideline is set to be piloted in three different healthcare centers in the United Kingdom and Australia.

Based on the literature, the first general recommendation integrated into the DEBRA International clinical guideline addressed blistering and wound management. The first recommendation in this category (strength of recommendation grade B) encourages clinicians to offer podiatry education programs for patients with EB. Education may relate to foot biomechanics and how to select appropriate footwear, including appropriate selection of shock-absorbing insoles and socks.

In addition, the guideline recommends offering treatment for EB dystrophic nails (strength of recommendation grade B). Application of a topical keratolytic agent, advising patients to keep toenails trimmed straight across, and having an expert podiatrist reduce nail thickness are all recommended. To facilitate disease monitoring, the guideline recommends assessment of hyperkeratosis with a validated tool (strength of recommendation grade B). The guideline also suggests pressure redistribution and cushioning may be helpful for the prevention of hyperkeratosis in these patients.

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Longitudinal mobility assessment and monitoring, as well as implementation of various methods to improve mobility, are also recommended for all subtypes of EB (strength of recommendation grade C). Gait-analysis systems appear to be safe and effective for identifying pressure areas and walking patterns and for evaluating therapeutic efficacy. Botulinum toxin injections are mentioned as a potentially effective therapy for improving mobility in patients with EB; however, the writing committee notes that this option is not tolerated by all patients. Surgery is also suggested as a consideration for patients with pseudosyndactyly, or the fusion of toes and mitten deformities, as well as for contractures of the lower extremities.

A limitation of the clinical guideline was the inclusion of articles on syndactyly surgery that were mostly case studies rather than controlled trials.

EB requires specialized podiatric care but, the investigators wrote, because of its rarity many podiatrists have limited knowledge of the disorder. Due to limited evidence regarding podiatric care of EB, management decisions are usually based on experience and expert opinion. The committee concludes that “a podiatrist should routinely be included as part of the multidisciplinary management of EB.”

Disclosure: None of the study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

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Khan MT, O’Sullivan M, Faitli B, et al. Foot care in Epidermolysis bullosa: Evidence-based guideline [published online August 9, 2019]. Br J Dermatol. doi:10.1111/bjd.18381