Frequent corneal abrasions precede scarring and vision loss in epidermolysis bullosa: An international patient survey

Validation and Repeatability of the Epidermolysis Bullosa Eye Disease Index in Dystrophic Epidermolysis Bullosa

“…Our previous survey of 37 participants found that the most frequent symptom affecting ADLs was the "inability to open eyes," reported by 21 of 37 participants (57%). 9 We found a higher rate of 73% in this study. Impact on ADLs was also higher in this study compared with the previous survey, specifically in "difficulty using screens" (77% vs. 57%), "inability to read" (83% vs. 51%), and "inability to attend school or work" (73% vs. 35%).…”

“…lww.com/ICO/B374) to better reflect the typical frequency of ocular symptoms in EB. 9 The EB-EDI baseline tool includes 23 questions, grouped into 3 domains, containing 5 to 10 questions each, and queries symptoms in the past 6 months. The EB-EDI interval tool includes 18 questions in 3 domains but asks only about symptoms within the past 1 month.…”

“…All questions were selected from previously validated surveys of patients with EB that were published by the author V.M.C. 9,12 In those surveys, Cronbach alpha scores were calculated for the questions that elicited the most responses. Questions regarding corneal abrasion symptoms and their impact on ADLs with Cronbach alpha scores .0.70 were included.…”

“…6 Corneal abrasions are severely painful and affect 24% to 53% of patients with DEB. 3,[7][8][9] There is no cure or disease-specific therapy for DEBrelated eye disease, but hope is on the horizon. Numerous protein and gene therapies are reaching phase III trials in the next 2 years, and we are optimistic that these interventions will alleviate ocular symptoms and sequelae.…”

“…The questions included in the tool were generated from our previously published, internally validated, international EB eye disease survey. 9 We also included questions from a 10-item survey that had been administered in person and was externally validated by vision testing and anterior segment optical coherence tomography for vision loss and corneal scarring, respectively. 9,12 Using the most internally consistent questions, we created an EB-specific assessment of ocular symptoms which we named the Epidermolysis Bullosa Eye Disease Index (EB-EDI).…”