Laryngotracheal involvement in epidermolysis bullosa.
Epidermolysis bullosa (EB) is a rare hereditary skin disease of infancy that can involve the mucous membranes of the oral cavity. Laryngotracheal involvement is rare. The disease is characterized by bullae formation in response to minor trauma. There are at least 18 described types of EB, however, there are 3 basic categories. These are simplex (with disruption above the basement membrane), dystrophic (in which disruption is below the basement membrane), and junctional (in which the split is within the lamina lucida). The prognosis of the different types ranges from early death usually secondary to overwhelming sepsis, to long term survivals with lack of growth retardation or significant dystrophic scarring. Presently, survival appears to be the only reliable criteria for distinguishing the benign and lethal forms of EB. Airway obstruction secondary to laryngotracheal involvement should be considered in any child with epidermolysis bullosa presented with symptoms of respiratory distress. Because there are few predictive prognostic indicators in the neonatal period, tracheotomy should be considered early in an effort to prevent further laryngeal injury from intubation, in those patients that will survive.
Otolaryngology Head and Neck Surgery Service, Walter Reed Army Medical Center, Washington, D.C. 20307.
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Gonzalez
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Roth
D004820:Epidermolysis Bullosa / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007818:Laryngeal Diseases / Q000473:pathology; D008297:Male; D012131:Respiratory Insufficiency / Q000209:etiology; D012135:Respiratory Sounds / Q000209:etiology; D014133:Tracheal Diseases / Q000473:pathology