Anesthetic management of a patient with dystrophic ampullar epidermolysis

We report the clinical case of a 56 years old male suffering dystrophic ampullar epidermolysis. He underwent brachial plexus blockade to remove a right hand tumor. The patient presented bilateral pseudosyndactylia, flexion retraction of the left hand, and erosive lesions in the inferior extremities, forearm, and trunk interfered monitorization and venous catheterization. Cutaneous friction or trauma should be carefully avoided since in this patient might produce detachment of the epidermis and subsequent ampullar formation. Cutaneous electrodes without adhesive components, padded sphygmomanometers, ear pulsimeter, and venous catheter sutured to the skin were used during monitorization. Anesthetic management of patients with dystrophic ampullar epidermolysis should consider careful airway manipulation, reduction of mucocutaneous contacts, prevention of pressure or friction skin trauma, appropriate electrolyte and plasma volume reposition, and use of nonadhesive material. To avoid airway manipulation regional anesthesia should be considered in patients with ampullar epidermolysis. We recommend regional anesthesia with ketamine.