Esophagitis and almost complete esophageal occlusion in a girl with epidermolysis bullosa.

[dystrophic epidermolysis bullosa]

Epidermolysis bullosa is a genetically transmitted skin disorder that typically manifests with trauma-induced skin blistering , scarring and in some cases mucosal involvement . Esophageal webs , strictures or stenosis can be found in about a third of the patients with the recessive dystrophic type of this disease . We report a six -year -old girl with recessive dystrophic epidermolysis bullosa and progressive dysphagia . Almost complete esophageal occlusion was found on barium swallow and esophagoscopy (endoscopic signs of esophagitis were also seen ). She was treated successfully with fluoroscopically guided balloon dilatation and esomeprazole . Fluoroscopically guided balloon dilatation and postdilatation use of esomeprazole is a safe and effective therapy for recessive dystrophic epidermolysis bullosa patients with almost complete esophageal occlusion and esophagitis .