Clinical Edge

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Esophageal stricture signals urgent treatment in kids with butterfly skin

Key clinical point: Esophageal strictures are a significant problem for children with dystrophic epidermolysis bullosa.

Major findings: A quarter of urgent contacts with a health care professional for generalized-severe recessive dystrophic epidermolysis bullosa are tied to esophageal narrowing, and location and number of strictures at presentation are predictive of restenosis.

Studies’ details: A review of electronic patient records of 20 patients with EB; a retrospective chart review of 24 EB patients with esophageal strictures; and a retrospective multicenter cohort study of 125 patients with EB.

Disclosures: Dr. Prodinger, Dr. Pope, and colleagues did not provide disclosure information. Dr. Bruckner is the principal investigator for the Epidermolysis Bullosa Clinical Characterization and Outcomes Database. She disclosed receiving grants or research funding, honoraria, or consultation fees from a number of drug companies, as well as other support from the EB Research Partnership and the EB Medical Research Foundation..

Citation:

Prodinger et al. EB 2020. Poster 3; Bruckner A et al. Pediatr Gastroenterol Nutr. 2018;67(6):701-5; Pope et al. EB 2020. Poster 8.