Management of oesophageal stenosis in epidermolysis bullosa dystrophica.
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.
Kern
I B
IB
Prince of Wales Children's Hospital, Australia.
Eisenberg
M
M
Willis
S
S
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000209:etiology / Q000628:therapy; D004106:Dilatation; D004820:Epidermolysis Bullosa / Q000150:complications*; D004940:Esophageal Stenosis / Q000209:etiology / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D010672:Phenytoin / Q000627:therapeutic use; D014700:Verapamil / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D010672:Phenytoin; D014700:Verapamil