[Special aspects of anesthesia in patients with epidermolysis bullosa based on a case example]

Epidermolysis bullosa hereditaria dystrophica (Hallopeau-Siemens) is a rare autosomal recessive disease characterized by extreme bullae formation of skin and mucosa. Typical dystrophic nails and flexion contractures of the joints can lead to deformities. Carious teeth and microstomia caused by scarred contractures of the lips are characteristic of the clinical picture. Depending on the form and severity of epidermolysis bullosa, the anaesthetic and surgical management requires careful planning to avoid unnecessary complications as a result of positioning, anaesthesia or surgery. In cooperation with the patient, optimal positioning on the operating table without pressure or tangential friction of the skin needs to be achieved. Wherever possible, surgical tape and adhesive electrodes should be avoided. Artificial respiration, intubation and monitoring must be adapted to the skin conditions of the patient. In particular the skin below the blood pressure cuff must be protected by adequate padding, and maximum intervals between measurements should be chosen. Nasal, oral, laryngeal and tracheal manipulations should be kept to a minimum for protection of the upper airway. Fibre optic intubation is to be preferred because of the possibility of microstomia and the need for simultaneous airway diagnosis. In addition, oro- or nasopharyngeal tubes and catheters should be avoided where possible. Depending on the course of surgery and anaesthesia, postoperative therapy in an intensive care unit should be considered.