Wambier Carlos Gustavo, Hoekstra Thaís Angélica, Wambier Sarah Perillo de Farias, Bueno Filho Roberto, Vilar Fernando Crivelenti, Paschoal Renato Soriani, Roselino Ana Maria, Frade Marco Andrey Cipriani, Foss Norma Tiraboschi
Department of Medicine, Universidade Estadual de Ponta Grossa, Ponta Grossa (PR), Brazil.
Division of Dermatology, Department of Internal Medicine, Universidade Estadual de Ponta Grossa, Ponta Grossa (PR), Brazil.
An Bras Dermatol. 2019 Jan-Feb;94(1):17-23. doi: 10.1590/abd1806-4841.20196864.
Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term 'Epidermal Necrolysis' has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for 'Epidermal Necrolysis' cases that still needed validation in acquired immunodeficiency syndrome.
To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts.
Retrospective cohort study of AIDS and non-AIDS 'Epidermal Necrolysis' cases admitted to a Brazilian reference center from 1990-2014.
Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02.
Heterogeneity of cases, wide variation of systemic corticosteroid doses when used.
SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.
史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症是危及生命的水疱性药物反应，眼部后遗症发生率高。术语“表皮坏死松解症”最近被用于更好地描述该疾病的全谱，包括两端的史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症，它们因表皮脱落的体表面积范围不同而有所差异。SCORTEN是一种针对“表皮坏死松解症”病例的死亡率预后评分，在获得性免疫缺陷综合征中仍需验证。
评估SCORTEN在获得性免疫缺陷综合征中的表现，以及获得性免疫缺陷综合征队列与非获得性免疫缺陷综合征队列在结局上的差异。
对1990年至2014年入住巴西一家参考中心的艾滋病和非艾滋病“表皮坏死松解症”病例进行回顾性队列研究。
30例艾滋病患者中有5例（16.7%）因表皮坏死松解症死亡，39例非艾滋病患者中有7例（17.9%）死亡，相对风险（RR）为0.92（p = 1.0）。SCORTEN表现出色，受试者操作特征曲线（ROC）下面积（AUC）为0.90，95%置信区间为0.81至0.99。SCORTEN在非艾滋病患者中的表现优于艾滋病患者：非获得性免疫缺陷综合征AUC = 0.99（95%CI 0.96 - 1.00），获得性免疫缺陷综合征AUC = 0.74（95%CI 0.53 - 0.95），p = 0.02。
病例的异质性，使用系统性皮质类固醇剂量的广泛差异。
SCORTEN对巴西人群有效，包括获得性免疫缺陷综合征患者，因此，建议在该亚组患者中使用它来辅助治疗选择。
