Background/Objectives: Epidermolysis bullosa (EB) comprises a group of inherited skin blistering diseases. There is currently no cure, and management includes skin protection and prevention of infection. To date, there has been no systematic investigation of home skin care practices among EB patients on a multicenter scale. Methods: This cross-sectional, observational study included data collected from patients with EB enrolled in the Epidermolysis Bullosa Characterization and Clinical Outcomes Database (EBCCOD) who provided answers to a patient-directed questionnaire between January 1, 2017, and December 31, 2017. Results: Of 202 respondents, 130 (64.4%) had dystrophic EB, 51 (25.2%) had EB simplex, 21 (7.4%) had junctional EB, 3 (1.5%) had Kindler syndrome, and 3 (1.5%) had an unspecified subtype. Seventy-eight patients reported cleansing in plain water only (39%). Of those who used an additive in their cleansing water, 75 (57%) added salt, 71 (54%) added bleach, 36 (27%) added vinegar, and 34 (26%) endorsed the use of an “other” additive (multiple additives possible). Reported concentrations of additives ranged widely from 0.002% sodium hypochlorite and 0.002% acetic acid solutions, which are thought to have negligible effects on microbes, to 0.09% sodium hypochlorite and 0.156% acetic acid, concentrations shown to be cytotoxic. One hundred eighty-eight patients answered questions regarding topical product use (93%). Of those, 131 reported topical antimicrobial use (70%). Mupirocin and bacitracin were the most commonly reported topical antibiotics (59, 58 [31.4%, 30.9%], respectively). Conclusions: These findings highlight the variety of skin care routines and frequent use of topical antimicrobials among EB patients and have potential implications for antibiotic resistance. The reported range of bleach and vinegar additives to cleansing water, including cytotoxic concentrations, emphasizes the need for clear and optimized skin cleansing recommendations.
Bibliographical noteFunding Information:
The Epidermolysis Bullosa Clinical Characterization and Outcomes Database is funded jointly by Epidermolysis Bullosa Research Partnership and Epidermolysis Bullosa Medical Research Foundation. Research Electronic Data Capture is supported by Colorado Clinical Translational Science Award grant UL1 TR002535 from the National Institutes of Health/National Center for Advancing Translational Sciences. The following individuals provided significant research support for this study: Kathleen Peoples, University of Colorado Denver; Bret Augsburger, Cincinnati Children's Hospital; Hannah Singer, MD, Columbia University Vagelos College of Physicians and Surgeons; Jenna Borok, MD, Allison Han, MD, and Nicola Natsis, BA, University of California San Diego; and Sarah H. Schwartz, Dell Children's Medical Center. We are grateful and indebted to the patients and their families who participated in this study. We would also like to thank the Pediatric Dermatology Research Alliance for facilitating collaborative multicenter research, as well as Yuan (Vivian) Zhang, MS, and Codruta Chiuzan, PhD, of the Biostatistics, Epidemiology, and Research Design program at Columbia University Irving Medical Center for their statistical support.
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- epidermolysis bullosa
- quality of life
- skin barrier
PubMed: MeSH publication types
- Journal Article
- Multicenter Study
- Observational Study