Weidauer H, Feurle G E, Baldauf G, Anton-Lamprecht I
Laryngol Rhinol Otol (Stuttg). 1985 Oct;64(10):522-6.
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.
食管狭窄或完全闭塞是隐性营养不良性大疱性表皮松解症的潜在危及生命的并发症。其后果包括营养不良、生长发育迟缓、误吸或恶病质。对于此类患者，建议采用结肠代食管术进行食管全置换。我们报告了成功的保守治疗方法。我们应用了最近关于该疾病中缺陷性胶原酶和食管扩张的知识。已证明苯妥英可减少胶原酶的过度产生，从而减少皮肤和黏膜水疱形成以及食管狭窄的形成。采用食管狭窄逐步扩张而非探条扩张是恢复食管腔的一种创伤较小的方法。可通过柔软的鼻胃饲管维持管腔通畅，在扩张成功后可将其拔除。食管通道已维持达4年。隐性营养不良性大疱性表皮松解症这些严重并发症的治疗需要皮肤科医生、内科医生和耳鼻喉科医生的多学科协作。
