A Rare Case of Epidermolysis Bullosa With Sudden Airway Obstruction

Abstract

Epidermolysis bullosa (EB) is a rare condition which may present at birth or early infancy. These patients have a poor prognosis with high mortality rate. Tracheolaryngeal stenosis or stricture is the most significant ENT complication which may result in death from airway obstruction. We present a case of EB presenting with sudden onset airway obstruction, requiring immediate airway management. The epidemiology, pathogenesis and clinical presentation of this disease are discussed as well as the diagnosis and management. ENT surgeons as well as other health care providers must be aware of the potential risks to the airway in these patients.