Epidermolysis Bullosa – Tread Carefully!!

Abstract

Patients with epidermolysis bullosa (EB) provide a unique and complex challenge for the anaesthesiologist. This is a rare inherited group of diseases characterized by excessive fragility of skin and mucous membranes. These patients tolerate compressive forces to the skin but barely tolerate friction and shearing forces. Major challenges to the anaesthetist include establishing adequate monitoring without damaging the skin, issues with positioning, high possibility of difficult airway, compromised immunity and nutritional deficiencies, and other comorbidities. We present a case of a 4-month-old baby boy with epidermolysis bullosa simplex presented with blisters and ulcers all over the body, posted for thoracoscopic plication for diaphragmatic eventration. We highlight that with meticulous preoperative planning and extreme attention to prevent any shearing or frictional forces, anaesthesia can be delivered safely.