• 作者：Teresa Vá ; zquez Rey^a ; ^{Maria.Teresa.Vazquez.Rey@sergas.es} ; Amalia Carmona Campos^a ; Jesú ; s Yá ; ñ ; ez L&#243 ; pez^a ; José ; Souto Ruzo^a ; Remedios Pardeiro Pé ; rtega^a ; Joaquí ; n Serrano Arreba^b ; Pedro A. Alonso Aguirre^a
• 关键词：Epidermolisis ampollosa ; Absceso paraesofá ; gico ; Disfagia
• 刊名：Gastroenterolog铆a y Hepatolog铆a
• 出版年：2011
• 期刊代码：pca167_02105705
• 类别：med
• 出版时间：November 2011
• 卷：34
• 期：9
• 页码：614-618
• 文件大小：259 K

lass="h4">Introduction

Epidermolysis bullosa encompasses a group of disorders characterized by the development of blisters on the skin and mucous membranes after minimal trauma. Gastrointestinal involvement is almost always present in the recessive dystrophic form, with the esophagus being one of the most frequent sites of extracutaneous manifestations. The most common symptom is dysphagia, which is usually secondary to esophageal blisters that evolve to scar tissue and stenosis.

lass="h4">Case report

We report the case of a 48-year-old woman with recessive dystrophic epidermolysis bullosa who was referred because of dysphagia, with suspected esophageal stenosis. Pediatric gastroscopy was abandoned due to the development of blistering of the hypopharynx caused by the instrument and the apparent presence of extrinsic esophageal compression. To continue the examination, cervical computed tomography was performed, showing an image compatible with a paraesophageal abscess. After evaluating the risk-benefit ratio of performing endoscopic biopsy-drainage, we decided on conservative treatment, achieving favorable results and complete symptom resolution.

lass="h4">Conclusions

We describe a case of paraesophageal abscess associated with epidermolysis bullosa, a rare cause of dysphagia in these patients, which was resolved with antibiotic and steroid treatment. In patients with this disease, invasive procedures, including endoscopy, have a high success rate. Despite the safety of these techniques, the utmost precautions should be taken, an appropriate technique should be used, and other diagnostic options should be considered.