Frederiks Aaron J, Kumarasinghe S Prasad, Wood Fiona, Rowe Sharon, Cunneen Tom, Raby Edward, Bourke Jack, Ricciardo Bernadette
Department of Dermatology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
Faculty of Health and Medical Sciences, School of Medicine, University of Western Australia, Perth, Western Australia, Australia.
Australas J Dermatol. 2022 Nov;63(4):437-451. doi: 10.1111/ajd.13903. Epub 2022 Jul 29.
Toxic epidermal necrolysis (TEN) is a rare and life-threatening mucocutaneous disease triggered by a reaction to a drug. Despite reported mortality of 30%, management differs between healthcare settings. Our hospital was established in February 2015 becoming the new state burns centre in Western Australia (WA). Following this, we collaborated on comprehensive multidisciplinary guidelines for the management of TEN. These guidelines are updated annually to reflect the weight of emerging evidence in managing TEN. Our aim was to review the management and outcomes of TEN patients presenting to our hospital between February 2015 and May 2021 (inclusive). We collected data for 10 patients on year, age, ethnicity, gender, medical history, culprit drug and exposure, SCORTEN, length of stay, maximum percentage of skin detachment, mucosal surface involvement, ophthalmic amniotic membrane transplant, burns unit input/admission, intensive care unit admission, weight, systemic treatment(s), complications and outcome. We excluded 7 out of 17 flagged patients who did not strictly meet the definition of TEN as greater than 30% epidermal detachment, with epidermal detachment defined as bullae, erosions, and/or positive Nikolsky. We found that the mortality rate in WA from TEN is improving compared with two previous WA studies, with a mortality rate in our study of 20% (2 deaths). Though limited by small sample size and retrospective design, our study suggests a shift towards at least one systemic therapy per patient (most commonly cyclosporine), the growing use of etanercept and the ophthalmic use of amniotic membrane transplants. It demonstrates the importance of burns unit input and the utility of comprehensive multidisciplinary guidelines. While the management and outcomes of TEN patients in WA are continuing to improve, we support calls for large registry data to facilitate evidence growth and collaboration for this rare life-threatening condition.
中毒性表皮坏死松解症（TEN）是一种由药物反应引发的罕见且危及生命的皮肤黏膜疾病。尽管报告的死亡率为30%，但不同医疗机构的治疗方法有所不同。我们医院于2015年2月成立，成为西澳大利亚州（WA）新的烧伤中心。此后，我们合作制定了TEN管理的综合多学科指南。这些指南每年更新，以反映TEN管理方面新出现证据的重要性。我们的目的是回顾2015年2月至2021年5月（含）期间在我院就诊的TEN患者的治疗情况和结局。我们收集了10例患者的以下数据：年份、年龄、种族、性别、病史、致病药物及接触情况、SCORTEN评分、住院时间、皮肤脱失的最大百分比、黏膜表面受累情况、眼科羊膜移植、烧伤科介入/入院情况、重症监护病房入院情况、体重、全身治疗、并发症及结局。我们从17例被标记的患者中排除了7例，这些患者未严格符合TEN的定义（表皮脱失大于30%），表皮脱失定义为水疱、糜烂和/或尼氏征阳性。我们发现，与西澳大利亚州之前的两项研究相比，该州TEN的死亡率正在下降，我们研究中的死亡率为20%（2例死亡）。尽管受到样本量小和回顾性设计的限制，但我们的研究表明，每名患者至少有一种全身治疗方法（最常见的是环孢素）的使用有所增加，依那西普的使用越来越多，以及眼科羊膜移植的使用。它证明了烧伤科介入的重要性以及综合多学科指南的实用性。虽然西澳大利亚州TEN患者的治疗情况和结局在持续改善，但我们支持呼吁建立大型登记数据，以促进针对这种罕见的危及生命疾病的证据积累和合作。
