Epidermolysis Bullosa of the Esophagus: Report of Two Cases and Review of Literature

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Abstract
Because of an inherent fragile adherence between the layers, trauma causes separation of the mucosa from the submucosa, leading to the formation of blisters in the oropharynx and esophagus. Blisters heal with the development of scarring and resulting stenosis of the esophagus. This is analogous to the formation of blisters and scars on the skin in epidermolysis bullosa. Esophagoscopy may show friability of the mucosa, blisters, erosions and stenosis. Roentgenography with barium demonstrates the stenotic segment with smooth tapering conical margins at either end of the segment. Roentgenography has not been successful so far in demonstrating blisters in the esophagus. Esophageal involvement can cause severe disability and death. A review has been made of previously reported cases of epidermolysis bullosa exhibiting esophageal involvement.

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