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大疱性表皮松解症是分娩时或分娩后短时间内出现的一种异型、先天性水疱疾患,并有不同的表现,严重程度和预后。根据松解损害的部位可分为:单纯型、致死型和营养不良型。单纯型损伤在表皮层未累及基底膜;营养不良型深达基底膜;致死型上皮基层的浆膜与基底膜分离并出现糜烂。细菌侵入可发生败血症,损伤很难痊愈。此型预后极差,常在出生后几周内死亡。本文报告一例致死型大疱性表皮松解症导致呼吸衰竭。患儿,男性,29月龄,生后皮肤即出现水疱,诊为致死型大疱性表皮松解症。用激素治疗。15个月时出现轻度喘鸣,X 线检查显有轻度气管狭窄。29个月时,患儿发生呕血和黑便;
Epidermolysis bullosa is a special or congenital vesicular disease that occurs during childbirth or shortly after childbirth and has different manifestations, severity and prognosis. According to the release of damage to the site can be divided into: simple type, lethal type and malnutrition. Simple injury in the epidermis does not involve the basement membrane; malnutrition deep basement membrane; lethal epithelial base serosa and basement membrane separation and erosion. Bacterial invasion can occur sepsis, damage is difficult to heal. This type of poor prognosis, often within a few weeks after birth, died. This article reports a case of lethal bullous epidermolysis causing respiratory failure. Children, men, 29 months old, after birth skin blisters, diagnosed as lethal bullous epidermolysis disease. Hormone therapy. Mild wheezing at 15 months and mild bronchoconstriction on X-ray. At 29 months, hematemesis and melena occurred in children.