LARYNGEAL INVOLVEMENT IN EPIDERMOLYSIS-BULLOSA

Epidermolysis bullosa (EB) represents a group of rare hereditary mechanobullous disorders marked by blister formation following relatively minor trauma. There are three categories depending on the site of disruption within the skin: simplex (above the basement membrane), dystrophic (below the basement membrane), and junctional (at the lamina lucida). Laryngeal involvement is rare, but has been reported primarily in association with junctional EB. Presented is our experience with 5 children with EB who demonstrated laryngeal involvement. Three children required tracheostomies acutely and 1 died of airway obstruction. One patient, with the Dowling-Meara type of EB simplex, was treated conservatively with good results. Tracheostomy should be an early consideration in the management of these patients to protect the airway in the acute setting and to prevent any further laryngeal injury associated with endotracheal intubation. The rate of complications of tracheostomy appears to be low; however, the prognosis for decannulation is poor.