Epidermolysis bullosa of the oesophagus with oesophageal web formation

Absceso paraesofágico: una causa poco frecuente de disfagia en la epidermólisis ampollosa

“…La formación de seudodivertículos, dilataciones preestenóticas, ulceraciones y espasmos se incluyen dentro de los hallazgos menos frecuentes. En ocasiones, sobre todo en los casos en que se afecta el tercio inferior, las estenosis recuerdan a las producidas por ingesta de cáusticos o a las de etiología péptica 5,8 .…”

Epidermolysis bullosa: radiographic findings in 16 cases

“…Short segmental stenoses or webs were also seen [2,4,5] (Fig. 1) Musculoskeletal changes elsewhere include diffuseosteopenia relatedto chronic immobilizationand to the poor nutritionalstate.…”

“…Mucosal trauma results in formation of bullae in which the epithelial layer is separated from the lamina propria. When the lesions heal with scarring, segmental stenoses result.Esophageal strictureshave been reported [2,4,5] inthe upper thirdof the esophagus in 50% of patients and in the lower third in 25%; they are multiple in 25% of cases. Eight (50%) of the 16 esophageal strictures in our series occurred in the proximal third, and five (31%) had multiple strictures.…”

Symptomatic Esophageal Web: A Diagnostic Clue to Epidermolysis Bullosa- Case Series From Pakistan.

“…Perhaps if all patients with epidermolysis bullosa were subjected to a routine barium swallow examination then esophageal involvement might be found to be more common than has been suspected. [8] 2194…”

“…The distal third is the next most commonly involved, possibly because it is subject to both reflux gastric juice and pulsations of the heart and aorta. [8] Repeated blistering and healing probably results in fibrosis and stricture formation. As in our case blistering was improving with age but dysphagia caused by esophageal web was more troublesome for the patient.…”