Treatment Landscape for Epidermolysis Bullosa Simplex: A Review of Established and Emerging Therapies.

Pediatric dermatology 2026

Hanrahan GB, Zhu A, Reusch DB, Teng JMC

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Abstract

Epidermolysis bullosa (EB) is a group of rare genetic blistering skin diseases, with EB simplex (EBS) being the most common subtype, accounting for around 70% of cases. Although there is no cure for EB, recent advancements led to the historic FDA-approvals of two treatments for dystrophic and junctional EB in 2023 and a third for dystrophic EB in 2025. However, no approved treatments exist for EBS despite its relative prevalence. Here, we outline established and emerging therapies for EBS. Supportive EBS management focuses on five key areas: skin-directed wound care, sweating reduction, keratoderma management, EBS-severe specific considerations, and blister reduction/prevention. Environmental measures target friction and moisture control to prevent blistering with the proper socks, footwear, and ambulation assistive device use. Wound care strategies include draining blisters without unroofing, nonstick dressing protection, and dilute vinegar or bleach baths to prevent infection and mitigate itch. Absorptive powders, glycopyrrolate, oxybutynin, and botulinum toxin target hyperhidrosis reduction. Painful keratoderma management, while challenging, includes mechanical debridement and topical keratolytic agents; targeted treatments including topical sirolimus have been explored in early phase study. A number of emerging treatments targeting EBS inflammatory pathways under investigation include apremilast, dapsone, diacerein ointment, deucravacitinib, and topical broccoli sprout extract. Tetracycline antibiotics are commonly used off-label for blister prevention. Ongoing bench research and gene-editing techniques like siRNA, TALEN, and CRISPR-Cas9 offer hope for translational advancements.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
시술 botulinum toxin 보툴리눔독소 주사 dict 1
해부 skin scispacy 1
해부 junctional EB in 2023 scispacy 1
해부 blister scispacy 1
해부 sprout scispacy 1
합병증 EBS → EB simplex scispacy 1
합병증 wound scispacy 1
합병증 infection 감염 dict 1
약물 EB simplex C0079299
Epidermolysis Bullosa Simplex Kobner
scispacy 1
약물 glycopyrrolate C0017970
glycopyrrolate
scispacy 1
약물 oxybutynin C0069805
oxybutynin
scispacy 1
약물 sirolimus C0072980
sirolimus
scispacy 1
약물 apremilast C1678805
apremilast
scispacy 1
약물 dapsone C0010980
dapsone
scispacy 1
약물 diacerein ointment scispacy 1
약물 Tetracycline C0039644
tetracycline
scispacy 1
약물 diacerein scispacy 1
질환 Epidermolysis Bullosa C0014527
Epidermolysis Bullosa
scispacy 1
질환 sweating reduction scispacy 1
질환 keratoderma C0022579
Keratoderma
scispacy 1
질환 blisters C0005758
Bulla
scispacy 1
질환 itch C0033774
Pruritus
scispacy 1
질환 hyperhidrosis C0020458
Hyperhidrosis disorder
scispacy 1
기타 EBS → EB simplex scispacy 1
기타 TALEN scispacy 1

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