Anesthetic care of a patient with recessive-dystrophic epidermolysis bullosa: a clinical case and practical recommendations
Pochynok O.O. , Lisnyy I.I., Ostafiichuk V.
Summary. Epidermolysis bullosa (EB) is a rare inherited dermatological disorder characterized by disruption of the dermo-epidermal junction, resulting in blister formation following even minimal mechanical trauma. Anesthetic management of patients with severe forms of EB, particularly the recessive dystrophic subtype, presents a significant clinical challenge due to the high risk of skin and mucosal injury, as well as difficulties related to venous access, monitoring, intubation, and airway management. This article presents a clinical case of a 24-year-old patient with generalized recessive dystrophic EB who developed squamous cell carcinoma of the right hand in the setting of chronic dermatosis, necessitating surgical intervention in the form of right hand amputation. The report details the adapted preoperative preparation, modified monitoring techniques, rationale for the use of total intravenous anesthesia (TIVA), and postoperative care. The importance of a multidisciplinary approach, meticulous planning, and gentle technique are emphasized as key elements in achieving a safe and effective outcome without complications. This clinical example may serve as a model for developing anesthetic strategies in patients with severe forms of EB.
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