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Epidermolysis Bullosa

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Epidermolysis bullosa is a heterogenous group of rare disorders causing severe bullous formation on the skin and mucosa membranes. Dystrophic epidermolysis bullosa is a hereditary form of this condition that also includes severe blister formation of the esophagus. These bullous lesions can occur in the proximal or distal esophagus and lead to scarring from minor trauma, including contact with food or acid reflux. Therefore, esophageal dilatation may worsen the preexisting damage already occurring in the esophagus. In this condition, mechanical dilatation is avoided since its shearing forces can lead to further strictures and perforation. If dilatation is necessary, hydrostatic balloons are preferred since only radial forces are applied. Patients may ultimately require esophageal resection in order to achieve sustained relief (McBride, 1989).