Are existing validated severity scores for epidermolysis bullosa reflective of the burden of disease in patients with epidermolysis bullosa simplex?

Our study demonstrates that severity scores do not correlate well with the impact that localized epidermolysis bullosa simplex (EBS) has on quality of life. There is a paucity of literature exploring correlations between disease severity and patient-reported outcome measures in EB. Both the Birmingham EB Severity (BEBS) score and EB Disease Activity and Severity Index (EBDASI) consist of weighted factors including body surface area, scalp, mucous membrane, nail and systemic involvement, as well as complications of longstanding disease such as cutaneous squamous cell carcinoma, which is more commonly seen in recessive dystrophic EB. Current scoring systems fail to account for the palmoplantar blistering and keratoderma which is the most significant source of morbidity in the EBS cohort.