Pharyngoesophageal manifestations of epidermolysis bullosa

Two cases of a rare hereditary disease, epidermolysis bullosa, congenital and acquired, are presented. The disease manifests by vesicular eruptions over the skin and mucous membrane of the oropharynx and oesophagus, alternating with sympton-free periods. The recurrent crops of vesicles heal by scarring and progressive stenosis of the pharynx and oesophagus. Although mucosal involvement is a less common and a late manifestation of the disease, it may occur in relatively mild cases and relatively early in the course of the disease. It is important that the radiologist be familiar with the pharyngoesophageal manifestations since the patient may present with dysphagia during an interval free of cutaneous lesions. The barium swallow appearance of stricturing with obstruction is illustrated.