pidermolysis bullosa (EB) is a rare disease characterized by vesicu- lobullous lesions developed with minimal trauma to the skin and mucous membranes. It is observed at a frequency of 1/30 000. General characteristics of these patients: scars that limit the opening of the mouth, esophageal constriction, dehydration, malnutrition, anemia, hypoalbuminemia, electrolyte imbalance, thrombocytosis, and infec- tions. 1 In addition, temporomandibular joint involvement, which is very im- portant in terms of maintaining the airway opening, can be seen, and un- wanted airway problems may be encountered due to the bullae that may Turkiye Klinikleri J Anest Reanim 2018;16(2):51-6 51 Anesthetic Management in Patients with Epidermolysis Bullosa: Two Case Reports ABSTRACT Epidermolysis bullosa (EB) is a genetic disorder characterized by spontaneous or post- traumatic bullae developed in the skin and mucous membranes. General characteristics of these patients: scars that limit the opening of the mouth, esophageal constriction, dehydration, malnu- trition, anemia, hypoalbuminemia, electrolyte imbalance, thrombocytosis, and infections. Lesions that may spread throughout the body in cases with EB may lead to bleeding, scar tissues, contrac- tures 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 pro- tect 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 ap- proach for two cases with an EB diagnosis is presented accompanied by the literature. Keywords: Epidermolysis bullosa; general anesthesia; difficult airway; peroperative care ÖZET Epidermolizis bülloza (EB), cilt ve mukozalarda kendiliğinden veya travma sonrası gelişen büllerle karakterize genetik geçişli bir hastalıktır. Bu hastaların genel özellikleri; ağız açıklığını kısıtlayan skarlar, özofageal darlıklar, dehidratasyon, malnütrisyon, anemi, hipoalbüminemi, elektrolit dengesizliği, trombositoz ve enfeksiyonlardır. EB’li olgularda vücudun geneline yay- ılabilen lezyonlar, kanama, skar dokusu, kontraktürler ve ödem, zor havayolu ve zor damar yolu erişimine neden olabilir. Tanı ve tedavi edici girişimler için anestezi alması gereken bu grup hastalardaki en önemli yaklaşım, cilt ve müköz membranların bütünlüğünün korunması, güvenli bir havayolu sağlanması, ısı ve sıvı kaybının önlenmesi ve etkili bir perioperatif analjezi sağlan- masıdır. Bu yazıda EB tanısı konulmuş iki olguya uygulanan anestezik yaklaşım literatür eşliğinde sunulmuştur. Anahtar Kelimeler: Epidermolizis bülloza; genel anestezi; zor havayolu; perioperatif bakım Enes ÇELİK, a Zeynep BAYSAL YILDIRIM, a Feyzi ÇELİK, a Gönül ÖLMEZ KAVAK, a Mustafa BIÇAK a a Department of Anesthesiology and Reanimation, Dicle University Faculty of Medicine, Diyarbakır Received: 02.02.2018 Received in revised form: 19.05.2018 Accepted: 24.05.2018 Available online: 26.09.2018 Correspondence: Enes ÇELİK Dicle University Faculty of Medicine, Department of Anesthesiology and Reanimation, Diyarbakır, TURKEY/TURKİYE ec_1907@hotmail.com This study was carried out in TARK-2017 - Turkish Anesthesiology and Reanimation Association 51 st National Congress (25-29 October 2017, Antalya) presented as a paper. Copyright © 2018 by Türkiye Klinikleri OLGU SUNUMU DOI: 10.5336/anesthe.2018-59904