Inpatient Management of Epidermolysis Bullosa: Consensus-Based Hands-On Instructions for Neonates and Post-Neonates.
Challenges and progress related to gene editing in rare skin diseases
24 March 2024
Consensus-based Guidelines for the Provision of Palliative Care for People Living with Epidermolysis Bullosa
12 April 2024

J Am Acad Dermatol. 2024 Apr 9:S0190-9622(24)00583-8. doi: 10.1016/j.jaad.2024.04.014. Epub ahead of print. PMID: 38604489.

Inpatient Management of Epidermolysis Bullosa: Consensus-Based Hands-On Instructions for Neonates and Post-Neonates.

Authors: Brenda Abreu MolnarLaura LevinDuri YunKimberly MorelKaren WissJulie WieserChristopher WardHaley TriceMaria Teresa Garcia-RomeroAllison StephensonAdrienne ProvostHarper N PriceMarissa J PermanMorgan MoxonBeth MoevesCatherine C McCuaigChristine McCarthyAnne W LuckyMoise L LevyMichelle LeeIrene Lara-CorralesNatalia HennerNichole HalliburtonEmily GriffithEmily GorellSharon GlickLawrence EichenfieldClara CollinsAnna L BrucknerBrenda BoulriceSusan BaylissKellie BadgerAmy S Paller


Background: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America.

Objective: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and post-neonatal period.

Methods: A modified Delphi method (expert-based input via two surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium (EBCRC) centers were analyzed to determine areas of focus and formulate statements to be voted on by EBCRC members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected.

Results: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 non-healthcare caregivers. Consensus was established on 103/119 neonatal statements and 105/122 post-neonatal statements; no statements were rejected. Most recommendations applied to both age groups.

Limitations: Recommendations may require adjustment based on individual patient’s clinical context.

Conclusion: Using the Delphi method, a consensus-derived resource for hospital-based healthcare professionals who manage patients with EB has been developed to improve the quality of inpatient care.

Keywords: Delphi; epidermolysis bullosa; general dermatology; guidelines; hospital management; medical dermatology