Larger wound size tied to severity, worse life quality in RDEB

12 Nov 2021
Gel from tree bark: First effective treatment for epidermolysis bullosa

In patients with recessive dystrophic epidermolysis bullosa (RDEB), larger wound size is predictive of worsening skin disease severity and quality of life (QOL), reports a recent study. Worsening skin disease severity is significantly associated with key clinical manifestations.

This cross-sectional study was conducted on patients with RDEB surveyed through the global EBCare Registry to characterize the patient-reported outcomes and QOL. Patient-reported outcomes included skin disease severity, wound characteristics, pain, itch, extracutaneous symptoms, and medications. The investigators measured QOL using the validated Quality of Life in Epidermolysis Bullosa instrument.

Eighty-five patients with RDEB were included in the analysis, reporting a total of 1,226 wounds (937 recurrent wounds and 289 chronic open wounds). Overall skin disease severity was self-reported as follows: mild (22/83, 26 percent), moderate (40/83, 48 percent), or severe (21/83, 25 percent).

The predictors of worsening skin disease severity were as follows: larger wounds, high opiate use, anaemia, gastrostomy tube use, infections, osteoporosis, and squamous cell carcinoma. Notably, larger wound size correlated with worse QOL scores.

“These results show that patients with RDEB are able to self-report their skin disease severity and wounds,” the investigators said.

A recent systematic literature review found that RDEB patients and their families experience substantial economic, humanistic, and clinical burden. [Orphanet J Rare Dis 2021;16:175]

The current study was limited by self-reported data from an online epidermolysis bullosa patient registry.

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