EBDASI = EB Disease Activity and Scarring Index. Activity: body surface involvement, wound severity, ocular, oral, and other mucosal involvement. Damage: scarring, contractures, nail dystrophy, esophageal strictures. Range 0-552.

What gene therapy is available for EB?

Beremagene geperpavec (B-VEC, Krystal Biotech) is FDA-approved (2023) for dystrophic EB. It is a topically applied HSV-based gene therapy encoding COL7A1. Phase 3 GEM-3 trial showed significant wound closure.

What are the types of epidermolysis bullosa?

EB simplex (KRT5/14, superficial blistering), junctional EB (LAMB3/LAMC2/LAMA3, subepidermal, severe), dystrophic EB (COL7A1, dermolytic, most severe), Kindler syndrome (FERMT1). Severity varies from mild localized to lethal.

Epidermolysis Bullosa EB IQ Score

Assess epidermolysis bullosa severity using EBDASI (EB Disease Activity and Scarring Index) or EB-IQ for patient-reported outcomes: blistering, pain, pruritus, and functional impairment.

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Epidermolysis Bullosa EB IQ Score

Frequently Asked Questions

What is the EBDASI?

What gene therapy is available for EB?

What are the types of epidermolysis bullosa?