DIGESTIVE-TRACT INVOLVEMENT IN DYSTROPHIC EPIDERMOLYSIS-BULLOSA

Congenital epidermolysis bullosa is an inherited disorder characterize d by an anomaly of joint structures between epidermis and dermis. This anomaly involves different malpighian epithelium and particularly in the digestive chorio-epithelial junction. Digestive and oropharyngeal manifestations include bullae, erosions and ulcerations that heal by f ibrosis, leading to retraction and stenosis. We report two cases of es ophageal stenosis, the first one in a young patient with a congenital epidermolysis bullosa of Hallopeau-Siemens and the second one, in a yo ung woman with a Pasini variant. In the first case, the esophageal ste nosis was treated by careful dilation. In the second case, the endosco py induced hemorragic bullae and septicemia, which were treated with a ntibiotics and total parenteral nutrition.