Epidermolysis bullosa and its E.N.T. manifestations. Two case reports.
The present paper describes two rare cases of epidermolysis bullosa dystrophica (recessive), one with oesophageal stricture and another with laryngeal stenosis, along with other usual features of the disease. Different views on the line of treatment of the oesophageal stricture as a complication of this dreadful disease are discussed. Replacement of the strictured oesophagus by coloplasty may be contemplated in the first case, if the patient develops increasing dysphagia. In the second case, with cicatrical stenosis of the larynx and hoarseness of voice which is a rare complication of the disease, not reported in the literature so far, a tracheostomy is contemplated when the child develops acute respiratory distress. These two cases are under observation.
D000328:Adult; D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000628:therapy; D004940:Esophageal Stenosis / Q000209:etiology*; D006801:Humans; D007829:Laryngostenosis / Q000209:etiology*; D008297:Male