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.

[1]  R. Boughton,et al.  Epidermolysis Bullosa — a Review of 15 Years’ Experience, including Experience with Combined General and Regional Anaesthetic Techniques , 1988, Anaesthesia and intensive care.

[2]  R. Touloukian,et al.  Gastrointestinal manifestations of epidermolysis bullosa in children. , 1988, Archives of dermatology.

[3]  N. Sandberg,et al.  Instrumental perforation of the oesophagus A ten year study from two ENT clinics , 1986, The Journal of Laryngology & Otology.

[4]  R. Harmel.  Esophageal replacement in two siblings with epidermolysis bullosa. , 1986, Journal of pediatric surgery.

[5]  H. Weidauer,et al.  Management of esophageal stenosis in recessive dystrophic epidermolysis bullosa. , 1984, Gastroenterology.

[6]  B. Becker,et al.  The effect of verapamil on the lower esophageal sphincter pressure in normal subjects and in achalasia. , 1983, The American journal of gastroenterology.

[7]  R. Eady,et al.  DIAGNOSING EPIDERMOLYSIS BULLOSA , 1983, The British journal of dermatology.

[8]  E. Bauer,et al.  Therapeutic considerations in recessive dystrophic epidermolysis bullosa. , 1981, Archives of dermatology.

[9]  E. Bauer,et al.  Phenytoin therapy of recessive dystrophic epidermolysis bullosa. Clinical trial and proposed mechanism of action on collagenase. , 1980, The New England journal of medicine.

[10]  P. Schofield,et al.  EPIDERMOLYSIS BULLOSA—NEW THERAPEUTIC APPROACHES , 1978, The Australasian journal of dermatology.

[11]  E. Bauer.  Recessive dystrophic epidermolysis bullosa: evidence for an altered collagenase in fibroblast cultures. , 1977, Proceedings of the National Academy of Sciences of the United States of America.

[12]  R. Briggaman,et al.  Epidermolysis bullosa: gastrointestinal manifestations. , 1974, Annals of internal medicine.

[13]  R. Deleon,et al.  Five year followup of colonic transplants in patients with epidermolysis bullosa producing esophageal obstruction. , 1974 .

[14]  K. Absolon,et al.  Five year followup of colonic transplants in patients with epidermolysis bullosa producing esophageal obstruction. , 1974, The Medical annals of the District of Columbia.

[15]  Fisk Gc,et al.  Anaesthesia for oesophagoscopy in a child with epidermolysis bullosa--a case report. , 1973 .

[16]  G. Fisk,et al.  Anaesthesia for oesophagoscopy in a child with epidermolysis bullosa--a case report. , 1973, Anaesthesia and intensive care.

[17]  H. Spiro,et al.  Dysphagia in children with epidermolysis bullosa. , 1967, Gastroenterology.