Development and Content Validation of the Epidermolysis Bullosa Oral Health Assessment Form (EB-OHAF)

Epidermolysis Bullosa (EB) is a rare skin disorder with significant phenotypic variations according to the primary type and subtype of the condition. As a rare disease with uncommon oral features and high phenotypic variability, identifying all characteristics is important to define a patient-specific treatment strategy and monitor the impact of different strategies on the oral outcomes. This project aims to develop and validate an EB-specific oral health assessment tool that helps clinicians to identify every specific oral feature that can be presented in patients with different types of EB, and aids in monitoring these features in time.

Literature review and instrument development were conducted using the World Health Organization Oral Health Assessment Form as a reference. A first advisory panel provided preliminary feedback, and a second panel was used to validate content through an online form. Content Validity Index (CVI), Item CVI (I-CVI), Scale-CVI (S-CVI), Universal Agreement (UA), and a modified Kappa value (K*) were calculated.

The instrument: Epidermolysis Bullosa-Oral Health Assessment Form (EB-OHAF) and its Appendix 1: Orthodontic Assessment were developed. The first draft considered assessing 30 domains. The validation process revealed that 28 domains had an I-CVI = 1, while domain #XIX (Emergency intervention) had an I-CVI of 0.875, and domain #XXIII (History of oral ulcers) an I-CVI of 0.5; S-CVI/Ave = 0.979 and S-CVI/UA = 0.93. Universal Agreement was reached in 28 domains (K* = 1), while domain #XIX presented a K* = 0.87, and domain #XXIII showed a K* = 0.312. Therefore, domain #XXIII (History of oral ulcers) was removed from the instrument. The final version of the EB-OHAF includes a total of 29 domains. The final assessment form and a clinical example are provided.

The new Epidermolysis Bullosa-Oral Health Assessment Form and the Orthodontic Assessment appendix showed strong content validity and can be applied to patients with all types of EB.