Treatment Landscape for Epidermolysis Bullosa Simplex: A Review of Established and Emerging Therapies - PubMed
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- #Blistering Skin Disease
- #Genetic Skin Disorder
- #Epidermolysis Bullosa Simplex
- Epidermolysis bullosa simplex (EBS) is the most common subtype of EB, accounting for 70% of cases.
- No FDA-approved treatments exist for EBS, despite recent approvals for other EB subtypes.
- Supportive management for EBS focuses on wound care, sweating reduction, keratoderma management, and blister prevention.
- Environmental measures include friction and moisture control to prevent blistering.
- Wound care strategies involve draining blisters without unroofing, nonstick dressings, and dilute vinegar or bleach baths.
- Hyperhidrosis is managed with absorptive powders, glycopyrrolate, oxybutynin, and botulinum toxin.
- Keratoderma is treated with mechanical debridement, topical keratolytic agents, and investigational topical sirolimus.
- Emerging treatments target inflammatory pathways, including apremilast, dapsone, diacerein ointment, deucravacitinib, and topical broccoli sprout extract.
- Tetracycline antibiotics are used off-label for blister prevention.
- Gene-editing techniques like siRNA, TALEN, and CRISPR-Cas9 offer future therapeutic potential.