Emergency management in epidermolysis bullosa: Consensus clinical recommendations from the European reference network for rare skin diseases

Jemima E. Mellerio, Maya El Hachem, Nathalia Bellon, Giovanna Zambruno, Hana Buckova, Rudolf Autrata, Carmen Salavastru, Tamara Caldaro, Celine Greco, Cristina Has, Christine Bodemer, Christine Bodemer

Research output: Contribution to journalReview articlepeer-review

Abstract

Epidermolysis bullosa (EB) comprises a group of genetic disorders with the hallmark of fragility of the skin and mucosal surfaces. The severity of different types of EB varies markedly as does the occurrence of extra-cutaneous involvement and complications. A number of emergency situations may occur in the context of EB including obstruction to oral intake from oral or esophageal blisters or scarring, acute airway obstruction, acute urinary retention, sepsis and corneal erosions. Whilst general management principles apply in each of these settings, specific considerations are essential in managing EB to avoid undue trauma or damage to delicate tissues. These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.

Original languageEnglish
Article number142
JournalOrphanet Journal of Rare Diseases
Volume15
Issue number1
DOIs
Publication statusPublished - Jun 6 2020

Keywords

  • Airway obstruction
  • Blister
  • Corneal erosion
  • Epidermolysis bullosa
  • ERN-skin
  • Esophageal obstruction
  • Pain
  • Sepsis
  • Urinary retention

ASJC Scopus subject areas

  • Genetics(clinical)
  • Pharmacology (medical)

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