Disease burden often age specific in epidermolysis bullosa simplex
Individuals with epidermolysis bullosa simplex (EBS) have extensive disease burden often specific to their age, according to a new survey.
Significant wounding, pain, itch, difficulty walking, and impact on quality of life (QOL) were all reported in this international cross-sectional survey of 214 patients with EBS.
The mean age of survey respondents was 32.8 years.
The majority of patients reported the following:
-Recurrent wounds (89%)
-Chronic wounds (59%)
There was a “moderate” impact on QOL, with 12% of respondents regularly using opiates.
Younger patients (aged 0-9) were more likely to self-report severe disease than those between the ages of 10-17 years and those older than 18 years (24% vs 19% vs 5%), failure to thrive (9% vs 15% vs 3%), and use of gastrostomy tubes (15% vs 12% vs 1%) and topical antibiotics (67% vs 69% vs 34%).
Older patients were more likely to be overweight or obese and have difficulty walking.
So JY, Fulchand S, Wong CY, et al. A global, cross-sectional survey of patient-reported outcomes, disease burden, and quality of life in epidermolysis bullosa simplex. Orphanet J Rare Dis. 2022;17(1):270. doi: 10.1186/s13023-022-02433-3. PMID: 35841105; PMCID: PMC9287948.