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Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases.
Metadata
Journalorphanet journal of rare diseases3.523Date
2020 Jun 06
2 months ago
Publication Type
Review
Journal Article
Volume
2020-Jun-06 / 15 : 142
Author
Mellerio JE 1, El Hachem M 2, Bellon N 3, Zambruno G 4, Buckova H 5, Autrata R 6, Salavastru C 7, Caldaro T 8, Greco C 9, Has C 10, Bodemer C 11, 12
Affiliation
  • 2. Dermatology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
  • 3. Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
  • 4. Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
  • 5. Dermatology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
  • 6. Pediatric Ophthalmology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic.
  • 7. Paediatric Dermatology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
  • 8. Endoscopy and Digestive Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
  • 9. Department of Pain and Palliative Medicine, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France.
  • 10. Department of Dermatology, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Breisgau, Germany.
  • 11. Dermatology Department, reference Centre MAGEC, Necker- Enfants Malades Hospital, Paris-Centre University, Paris, France. [email protected]
  • 12. Service de Dermatologie, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015, Paris, France. [email protected]
Doi
PMIDMESH
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.
Keywords: Airway obstruction Blister Corneal erosion ERN-skin Epidermolysis bullosa Esophageal obstruction Pain Sepsis Urinary retention
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3.5
Orphanet J Rare Disorphanet journal of rare diseases
Metadata
LocationEngland
FromBMC

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