患者男,35岁。出生后即发现面部出现水疱状皮疹,逐渐发展为近乎全身皮肤均出现水疱,水疱破裂后结痂形成瘢痕;双手指甲缺失,手指萎缩成棒状,功能缺失。患者长期进食呛咳、梗阻,8年前出现吞咽困难。家族未见类似疾病。临床诊断为大疱性表皮松解症。食管吞钡检查:喉咽、食管上段局 Patient male, 35 years old. Blister-like rashes were found on the face after birth, and gradually developed blisters on almost whole body skin. Scabs formed after the blisters ruptured and scars were formed. Finger nails were missing and their fingers atrophied into a rod shape with no function. Chronic cough, obstruction, 8 years ago, patients with dysphagia. No similar disease in the family. Clinical diagnosis of epidermolysis bullosa. Barium swallow esophageal examination: hypopharynx, esophageal upper bureau