Sheridan Robert L, Schulz John T, Ryan Colleen M, Schnitzer Jay J, Lawlor David, Driscoll Daniel N, Donelan Matthias B, Tompkins Ronald G
Shriners Burns Hospital, Boston, Massachusetts, USA.
Pediatrics. 2002 Jan;109(1):74-8. doi: 10.1542/peds.109.1.74.
Toxic epidermal necrolysis (TEN) is an acute inflammatory systemic condition that involves injury not just to the skin. Historically, it has been associated with a high mortality but few long-term consequences among survivors. With improved survival, long-term consequences may be becoming more apparent. The objective of this study was to define these long-term consequences and their frequency.
From July 1, 1991, to June 30, 2000, 11 children with severe TEN were referred to a regional pediatric burn facility. Wounds were managed with a strategy involving prevention of wound desiccation and superinfection, including the frequent use of biological wound coverings. All children survived and have been followed in the burn clinic. The records of all children were reviewed in detail.
Two boys and 9 girls with an average age of 7.2 +/- 1.8 years (range: 6 months-15 years) and sloughed surface area of 76 +/- 6% of the body surface (range: 50%-95%) were admitted to the burn unit for care. Antibiotics (3 children), anticonvulsants (4 children), nonsteroidals (2 children), and viral syndrome or unknown agents (2 children) were believed to have triggered the syndrome. Six (55%) children required intubation for an average of 9.7 +/- 1.8 days (range: 2-14 days). Mucosal involvement occurred in 10 (91%) and ocular involvement in 10 (91%). Lengths of stay averaged 19 +/- 3 days (range: 6-40 days). Overall follow-up averaged 14 +/- 13 months. Three children had no apparent long-term consequences of the disease and were referred to primary care follow-up after the 2-month burn clinic visit. The remaining children had follow-up averaging 23 +/- 13 months. The most common long-term morbidity involved eyes (3 children [27%]), nails (4 children [36%]), and variegated skin depigmentation (all children). One child developed vaginal stenosis from mucosal inflammation. No esophageal strictures or recurrent TEN has been diagnosed.
Survival has improved in children with TEN, but long-term sequelae are not infrequent. The most common long-term consequences involve the eyes, the skin, and the nails.
中毒性表皮坏死松解症（TEN）是一种急性炎症性全身性疾病，不仅累及皮肤。从历史上看，它与高死亡率相关，但幸存者中很少有长期后果。随着生存率的提高，长期后果可能变得更加明显。本研究的目的是确定这些长期后果及其发生率。
从1991年7月1日至2000年6月30日，11名患有严重TEN的儿童被转诊至一家地区性儿科烧伤机构。伤口采用预防伤口干燥和超级感染的策略进行处理，包括频繁使用生物伤口覆盖物。所有儿童均存活，并在烧伤诊所接受随访。详细查阅了所有儿童的记录。
11名儿童（2名男孩和9名女孩）被收治入烧伤科，平均年龄为7.2±1.8岁（范围：6个月至15岁），体表剥脱面积为体表面积的76±6%（范围：50%至95%）。据信抗生素（3名儿童）、抗惊厥药（4名儿童）、非甾体类药物（2名儿童）以及病毒综合征或不明药物（2名儿童）引发了该综合征。6名（55%）儿童需要插管，平均插管时间为9.7±1.8天（范围：2至14天）。10名（91%）儿童出现黏膜受累，10名（91%）儿童出现眼部受累。住院时间平均为19±3天（范围：6至40天）。总体随访时间平均为14±13个月。3名儿童没有该疾病的明显长期后果，在烧伤诊所就诊2个月后转诊至初级保健进行随访。其余儿童的随访时间平均为23±13个月。最常见的长期并发症涉及眼睛（3名儿童[27%]）、指甲（4名儿童[36%]）和皮肤色素斑驳（所有儿童）。1名儿童因黏膜炎症出现阴道狭窄。未诊断出食管狭窄或复发性TEN。
TEN患儿的生存率有所提高，但长期后遗症并不少见。最常见的长期后果涉及眼睛、皮肤和指甲。
