RT Journal Article
SR Electronic
T1 Management of oesophageal stenosis in epidermolysis bullosa dystrophica.
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 551
OP 556
DO 10.1136/adc.64.4.551
VO 64
IS 4
A1 I B Kern
A1 M Eisenberg
A1 S Willis
YR 1989
UL http://adc.bmj.com/content/64/4/551.abstract
AB Seven patients with epidermolysis bullosa dystrophica and chronic and recurrent oesophageal lesions such as spasm, strictures, and complete occlusion were studied. Dysphagia could be cured with drugs if it was caused by bullae formation or spasm. If oesophageal strictures were present, endoscopy and bouginage with corticosteroid prophylaxis during the quiescent phase of the disease was a safe and useful procedure. We have also given corticosteroids, which reduced the oedema caused by bullae formation and oral phenytoin, which reduced epithelial detachment by inhibiting collagenase activity. Verapamil counteracted oesophageal spasm and pureed food during periods of dysphagia reduced blistering of the upper oesophagus.