Eye Involvement and Management in Inherited Epidermolysis Bullosa

Drugs. 2022 Aug;82(12):1277-1285. doi: 10.1007/s40265-022-01770-8. Epub 2022 Sep 8.

Abstract

Inherited epidermolysis bullosa (EB) is a group of genetic rare diseases associated with skin fragility, which leads to the formation of blisters, erosions, and scars on the skin and mucous membranes. Epidermolysis bullosa includes four main types and some several clinical subtypes including EB simplex, junctional EB, dystrophic EB, and Kindler's EB. Ocular involvement ranged from 51 to 68% in EB and can cause irreversible damages if not properly managed. Corneal erosions are the most common finding among series, including our cohort. We review here clinical and pathological features of ocular involvement in EB and the main keys for management, with a focus on recent innovative therapies.

Publication types

  • Review

MeSH terms

  • Epidermolysis Bullosa Dystrophica* / complications
  • Epidermolysis Bullosa Dystrophica* / genetics
  • Epidermolysis Bullosa Dystrophica* / pathology
  • Epidermolysis Bullosa Simplex* / complications
  • Epidermolysis Bullosa Simplex* / pathology
  • Epidermolysis Bullosa* / complications
  • Epidermolysis Bullosa* / genetics
  • Epidermolysis Bullosa* / therapy
  • Epidermolysis Bullosa, Junctional* / complications
  • Epidermolysis Bullosa, Junctional* / pathology
  • Humans
  • Skin / pathology