The Turkish Journal of Pediatrics
2012 , Vol 54 , Num 3
Esophagitis and Almost Complete Esophageal Occlusion in a Girl with Epidermolysis Bullosa
1Division of Gastroenterology and 3Division of Surgery and Orthopedics, Children’s Hospital, 2Clinic of Gastroenterology
and Hepatology, University of Niš School of Medicine, Niš, Serbia
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.
Keywords :
epidermolysis, esophageal occlusion, esophagitis.