A review of scoring instruments for the assessment of disease severity in epidermolysis bullosa acquisita

Abstract Epidermolysis bullosa acquisita (EBA) is a rare, chronic autoimmune blistering disease (AIBD) characterized by mucocutaneous blistering. Typically acquired in adulthood, EBA manifests in a diverse array of clinical variants and significantly impacts quality of life (QOL). Globally, there is an absence of multicenter clinical trials and randomized controlled trials assessing treatment responsiveness in EBA, in part due to the absence of a validated, disease-specific scoring instrument to monitor disease severity and compare treatment outcomes. This literature review critically evaluates current scoring tools employed in EBA and proposes the need for the development of an Investigator Global Assessment (IGA) for EBA, which could provide a simple and practical measure of disease severity for use in clinical trials.