Preventing Perioperative Complications of Epidermolysis Bullosa

  • Claire SoriaEmail author
  • Mark Greenberg


Epidermolysis bullosa is a rare genetic mechanobullous disorder characterized by excessive fragility of skin and mucous membranes. Shearing forces and friction lead to intradermal fluid accumulation and resultant bullae formation. Complications of epidermolysis bullosa include skin infection, malnutrition, failure to thrive, esophageal strictures, tracheal stenosis, chronic pain, and high opioid tolerance. Children with epidermolysis bullosa will undergo multiple surgeries throughout their lifetimes, most commonly for wound care, extensive dressing changes, correction of pseudosyndactyly and joint contractures, and esophageal dilation. Improper management of these patients intraoperatively can have devastating consequences, including new blister formation. Shearing and compressive forces should be minimized as much as possible to avoid creating new lesions. This disease poses several unique challenges to the anesthesiologist, including monitoring, positioning, airway management, intravenous access, and pain control. The goals of safe anesthetic management include preventing formation of new lesions, optimizing pain control, and avoiding airway trauma, especially at induction and emergence. Clear communication among all staff members regarding safety of patient positioning is crucial. Intraoperative and postoperative analgesia can also be challenging, as patients may be tolerant to narcotics or suffer side effects such as nausea, constipation, and pruritus. Multimodal anesthesia is recommended to minimize narcotic use and provide a smooth and safe emergence.


Epidermolysis bullosa, Monitoring, Positioning, Airway management, Intravenous access, Pain control Chronic pain Multimodal analgesia Non-opioid analgesia Dexmedetomidine 


  1. 1.
    Culpepper TL. Anesthetic implications in epidermolysis bullosa dystrophica. J Am Assoc Nurse Anesth. 2001;69(2):114–8.Google Scholar
  2. 2.
    Denyer JPE. Best practice guidelines for skin and wound care in epidermolysis bullosa. Int Consens DEBRA 2012.Google Scholar
  3. 3.
    Nandi R, Howard R. Anesthesia and epidermolysis bullosa. Dermatol Clin. 2010;28(2):319–24. Scholar
  4. 4.
    Fine J-D. Inherited epidermolysis bullosa. Orphanet J Rare Dis. 2010;5(1):12. CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Iohom G, Lyons B. Anaesthesia for children with epidermolysis bullosa: a review of 20 years’ experience. Eur J Anaesthesiol. 2001;18(11):745–54. Scholar
  6. 6.
    Saraf SV, Mandawade NJ, Gore SK, Padhye UD, Pereira CS. Epidermolysis bullosa: careful monitoring and no touch principle for anesthesia management. J Anaesthesiol Clin Pharmacol. 2013;29(3):390–3. Scholar
  7. 7.
    Buvanendran A. Multimodal analgesia for perioperative pain management. ASA Refresh Courses Anesthesiol. 2012;40(1):1–6. Scholar
  8. 8.
    Sivrikaya GU. Multimodal analgesia for postoperative pain management, pain management - current issues and opinions. Racz G, editors. InTech; 2012. ISBN: 978-953-307-813-7, Available from:

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of AnesthesiologyUniversity of CaliforniaSan DiegoUSA

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