Epidermolysis bullosa letalis in the larynx causing acute respiratory failure: a case presentation and review of the literature.
The case history is presented of a 29-month-old boy with the characteristic skin lesions of epidermolysis bullosa letalis, requiring steroid therapy. After an episode of gastrointestinal haemorrhage, probably caused by the steroids, the child experienced progressive stridor and respiratory failure and died 10 days later. The postmortem findings in the larynx were of marked mucosal inflammation and cystic swelling of the serumucinous glands producing severe narrowing of the supraglottic airway. The laryngeal cysts were thus entirely different in nature to the cutaneous bullae.
D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000473:pathology; D006801:Humans; D007818:Laryngeal Diseases / Q000150:complications* / Q000473:pathology; D007829:Laryngostenosis / Q000150:complications / Q000209:etiology*; D008297:Male; D012131:Respiratory Insufficiency / Q000209:etiology*