Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa
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Orphanet J Rare Dis. 2023 Sep 4;18(1):268. doi: 10.1186/s13023-023-02870-8. PMID: 37667330.

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Authors: Mark P Popenhagen 1 2Paola Genovese 3Mo Blishen 4Dilini Rajapakse 5Anja Diem 6Alex King 7Jennifer Chan 8Eduard Pellicer Arasa 9Simone Baird 10 11Anna Carolina Ferreira da Rocha 12 13Gideon Stitt 14Kellie Badger 3Vlasta Zmazek 15 16Faiza Ambreen 17 18Caroline Mackenzie 19Harper Price 3Toni Roberts 20 21Zena Moore 22Declan Patton 22Paul Murphy 22Kattya Mayre-Chilton 23 24


  1. Department of Anesthesiology B090, Children’s Hospital Colorado, University of Colorado School of Medicine, Anschutz Medical Campus, 13123 E 16Th Ave, Aurora, CO, 80045, USA.
  2. Section of Pediatric Anesthesiology, Children’s Hospital Colorado, Aurora, CO, USA.
  3. Phoenix Children’s Hospital, Phoenix, AZ, USA.
  4. DEBRA New Zealand, Newtown, Wellington, New Zealand.
  5. Great Ormond Street Hospital Trust, London, UK.
  6. EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria.
  7. Human Sense, LLC, Phoenix, AZ, USA.
  8. Lucile Packard Children’s Hospital, Stanford, Menlo Park, CA, USA.
  9. Sant Joan de Déu Barcelona Hospital, Barcelona, Spain.
  10. DEBRA Australia, Pittsworth, QLD, Australia.
  11. Melbourne, Australia.
  12. DEBRA Brazil, Blumenau, Santa Catarina, Brazil.
  13. Santa Catarina, Brazil.
  14. Division of Clinical Pharmacology, University of Utah, Salt Lake City, UT, USA.
  15. DEBRA Croatia, Zagreb, Croatia.
  16. Zagreb, Croatia.
  17. DEBRA Pakistan, Lahore, Punjab, Pakistan.
  18. London, UK.
  19. Guys and St Thomas’ Foundation NHS Foundation Trust, EB Adult Service, East Hampshire, England, UK.
  20. DEBRA South Africa, Western Cape, Cape Town, South Africa.
  21. Cape Town, South Africa.
  22. Royal College of Surgeons in Ireland, University of Medicine and Health Sciences, Dublin, Ireland.
  23. DEBRA International, Vienna, Austria.
  24. Mildmay Mission Hospital, London, UK.


Background: Inherited epidermolysis bullosa (EB) is a cluster of rare, genetic skin and mucosal fragility disorders with multi-system and secondary effects, in which blistering and erosions occur in response to friction/mechanical trauma. Considering the incurable and potentially life-limiting nature of the condition and the challenges posed by its symptoms, a palliative approach to EB-related care is necessary. However, knowledge and experience related to the provision of EB palliative care is minimal. Evidence-based, best care guidelines are needed to establish a base of knowledge for practitioners to prevent or ease suffering while improving comfort at all stages of the illness, not just the end of life.

Methods: This consensus guideline (CG) was begun at the request of DEBRA International, an international organization dedicated to improvement of care, research, and dissemination of knowledge for EB patients, and represents the work of an international panel of medical experts in palliative care and EB, people living with EB, and people who provide care for individuals living with EB. Following a rigorous, evidence-based guideline development process, the author panel identified six clinical outcomes based on the results of a survey of people living with EB, carers, and medical experts in the field, as well as an exhaustive and systematic evaluation of literature. Recommendations for the best clinical provision of palliative care for people living with EB for each of the outcomes were reached through panel consensus of the available literature.

Results: This article presents evidence-based recommendations for the provision of palliative healthcare services that establishes a base of knowledge and practice for an interdisciplinary team approach to ease suffering and improve the quality of life for all people living with EB. Any specific differences in the provision of care between EB subtypes are noted.

Conclusions: Because there is yet no cure for EB, this evidence-based CG is a means of optimizing and standardizing the IDT care needed to reduce suffering while improving comfort and overall quality of life for people living with this rare and often devastating condition.

Keywords: Clinical practice guidelines; Consensus guidelines; End-of-life; Epidermolysis bullosa; Palliative care; Quality-of-life.