Anesthetic Management in Patients with Epidermolysis Bullosa: Two Case Reports

Abstract

Epidermolysis bullosa (EB) is a genetic disorder characterized by spontaneous or posttraumatic bullae developed in the skin and mucous membranes. General characteristics of these patients: scars that limit the opening of the mouth, esophageal constriction, dehydration, malnutrition, anemia, hypoalbuminemia, electrolyte imbalance, thrombocytosis, and infections. Lesions that may spread throughout the body in cases with EB may lead to bleeding, scar tissues, contractures and edema, difficult airway, and difficult vascular access. The most important approach in these groups of patients who need anesthesia for treatment and therapeutic interventions is to protect the integrity of skin and mucous membranes, to secure a reliable airway, to prevent the loss of heat and liquid, and to provide efficient perioperative analgesia. In this report, the anesthetic approach for two cases with an EB diagnosis is presented accompanied by the literature.