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Epidermolysis Bullosa: A Review of Wound Care and Emerging Treatments

  • Narges Maskan Bermudez,
  • Peyton V. Warp,
  • Abby Hargis,
  • Marita Yaghi,
  • Lawrence Schachner

摘要

Purpose of Review

Epidermolysis bullosa (EB) is a heterogeneous group of rare inherited disorders that cause skin and mucous membrane fragility. Patients with EB have compromised wound healing due to the underlying genetic defect, which can be further complicated by related comorbidities.

Recent Findings

Wound healing in EB patients is challenging due to multiple wounds of varying duration and healing ability. In the absence of a cure, the goal of treatment is to minimize the risk of blister formation, optimize wound care, pain, and itch relief, and address specific complications. However, newly approved therapies like beremagene geperpavec (Vyjuvek) and birch triterpenes (Fisulvez) are paving the way for improved management and quality of life for these patients.

Summary

EB is a complex skin disorder that is associated with considerably impaired quality of life. In this review, we discuss wound healing challenges, wound care strategies stratified by EB subtypes, as well as investigational cell-based, protein replacement, and gene therapies. While there are many promising advances, novel and effective treatments are still urgently needed.