Patients with epidermolysis bullosa (EB) have exquisitely sensitive skin as a result of genetic variants of collagen, laminin, and keratin that provide the structural integrity of the dermal-epidermal junction. The inheritance pattern is varied and the severity can range from very mild to severe. The skin of an EB patient is fragile and just mere touch can cause the skin to slough or blister. The anesthetic management of EB patients is similar to that of severe burn patients with a few key differences. Importantly, no adhesives may be used in this patient population.

Epidermolysis bullosa
Anesthetic relevance

Critical

Anesthetic management

No adhesives for monitors or surgical dressings. No rubbing or sheer stress to the skin. Typically difficult airways and best to use fiber.

Specialty

Adult General

Signs and symptoms

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Diagnosis

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Treatment

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Extracutaneous Manifestations

Anesthetic implications

Preoperative optimization

Intraoperative management

Monitors

Postoperative management

Common surgical procedures

  • Esophageal Dilations
  • Syndactyly/Contracture Release
  • Dental Extractions
  • Gastrostomy Placement
  • Skin Grafting
  • Squamous Cell Carcinoma Removal
  • Amputations
  • Vascular Access
  • Cesarian Section

References