[Therapy of esophageal stenoses in recessive epidermolysis bullosa dystrophica].
Stenosis or complete occlusion of the oesophagus are potentially life-threatening complications of recessive dystrophic epidermolysis bullosa. Consequences are malnutrition, growth retardation, aspiration, or cachexia. Total replacement of the oesophagus by colon interposition has been recommended in such patients. We report on successful conservative management. We applied recently developed knowledge concerning the defective collagenase involved in this disorder and oesophageal dilatation. Phenytoin has been shown to reduce the excessive production of collagenase and thereby to diminish blistering of skin and mucous membranes and stricture formation of the oesophagus. Stepwise dilatation of oesophageal strictures instead of bouginage represents a less traumatic way to restore the oesophageal lumen. The lumen can be maintained by soft nasogastric feeding tubes which may be removed later on after successful dilatation. Oesophageal passage has been maintained for up to 4 years. The management of these severe complications of recessive dystrophic epidermolysis bullosa requires interdisciplinary efforts of dermatologists, internists and otorhinolaryngologists.
D002648:Child; D003131:Combined Modality Therapy; D004106:Dilatation / Q000295:instrumentation; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000235:genetics; D004940:Esophageal Stenosis / Q000235:genetics / Q000628:therapy*; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D010672:Phenytoin / Q000627:therapeutic use
D010672:Phenytoin
Zur Therapie von Osophagusstenosen bei rezessiver Epidermolysis bullosa dystrophica.