TEN

  • 文章类型: Journal Article
    严重的皮肤不良反应(SCAR)是罕见的,但危及生命,抗生素是主要原因。这项来自单一中心的回顾性研究旨在分析罪魁祸首药物,抗生素诱导的SCAR的临床特征和治疗结果。
    我们分析了2013年1月至2024年1月间中国某三甲医院抗生素诱发的SCAR病例,包括史蒂文-约翰逊综合征(SJS)或史蒂文斯-约翰逊综合征-中毒性表皮坏死松解症(SJS-TEN)重叠,中毒性表皮坏死松解症(TEN),药物反应伴嗜酸性粒细胞增多和全身症状(DRESS)和急性全身性发疹性脓疱病(AGEP)。对人口特征的描述性分析,临床表现,进行治疗和预后。
    在354例SCAR中,纳入63例经过验证的抗生素相关病例。头孢菌素(31.7%),青霉素(25.4%),喹诺酮类药物(19.0%)是SCAR最常见的触发因素。总的来说,肝脏(50.8%),肺(31.7%),在SCAR病例中,肾脏(23.8%)是最常见的受累器官。SJS/SJS-TEN重叠组8例(28.6%)和TEN组8例(80.0%)接受糖皮质激素和IVIG联合治疗。由青霉素或头孢菌素引起的SCAR患者可以接受替代疗法,例如林可胺,喹诺酮类药物,还有四环素.TEN组死亡率最高,为20.0%,其次是SJS/SJS-TEN重叠组(7.1%),在DRESS和AGEP组中没有观察到死亡。
    识别罪魁祸首抗生素和应用替代抗生素疗法对于抗生素诱导的SCAR的管理至关重要。如果复杂的潜在疾病和并发症,如高龄,癌症和肺炎与SCAR并存,患者可能面临更大的死亡风险。
    UNASSIGNED: Severe cutaneous adverse reactions (SCARs) are rare but life-threatening, with antibiotics being the main cause. This retrospective study from a single center was designed to analyze the culprit drugs, clinical features and treatment outcomes of antibiotic-induced SCARs.
    UNASSIGNED: We analyzed cases of antibiotic-induced SCARs in a tertiary hospital in China between January 2013 and January 2024, including Steven-Johnson syndrome (SJS) or Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Descriptive analysis of the demographic characteristics, clinical manifestations, treatment and prognosis were carried out.
    UNASSIGNED: Among 354 cases of SCARs, 63 validated antibiotic-related cases were included. Cephalosporins (31.7%), penicillins (25.4%), and quinolones (19.0%) were the most common triggers for SCARs. Overall, liver (50.8%), lungs (31.7%), and kidneys (23.8%) were the most frequently affected organ in SCARs cases. Eight patients (28.6%) in the SJS/SJS-TEN overlap group and 8 patients (80.0%) in the TEN group received combination therapy of corticosteroids and IVIG. Patients with SCARs caused by penicillins or cephalosporins could receive alternative treatments such as lincomamides, quinolones, and tetracyclines. The mortality rate in the TEN group was the highest at 20.0%, followed by the SJS/SJS-TEN overlap group (7.1%), and no deaths were observed in the DRESS and AGEP groups.
    UNASSIGNED: The identification of the culprit antibiotics and the application of alternative antibiotic therapies are crucial for the management of antibiotic-induced SCARs. If complicated underlying conditions and complications like advanced age, cancer and pneumonia coexist with SCARs, patients might be more at risk for mortality.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    免疫检查点抑制剂(ICI)疗法具有重大免疫相关不良事件(irAE)的风险。最严重的irAE是可能在临床上模仿史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死(TEN)的表皮坏死。这项研究的目的是提供ICI相关表皮坏死的临床和组织学特征的总结,特别关注广泛疾病中与致命结局相关的因素。共98例,在PubMed和文献中报告了2例新病例和96例,评估ICI相关的表皮坏死。开始ICI治疗后1天至3年发生表皮坏死,有限(<30%BSA)的患者平均起病13.8周,广泛(≥30%BSA)的患者平均起病11.3周,中位发病时间分别为5.8周和4周。在52例中发现了先前的皮疹,在广泛的病例中更为常见。仅在65%的广泛病例中报告了粘膜受累,但与致命反应显着相关。细胞毒性化疗的共同给药与更广泛的疾病相关。分别在96%和65%的有限和广泛参与的患者中观察到恢复,并且没有特定的治疗与改善的生存率相关。年轻与广泛性疾病的不良预后显着相关,存活患者的平均年龄为64.5岁,而死亡患者的平均年龄为55.1岁,p<0.01。浅表血管周围和界面/苔藓样炎性浸润均常见。这些发现表明,ICI相关的表皮坏死应被视为与药物诱导的SJS/TEN不同的临床实体。
    Immune checkpoint inhibitor (ICI) therapies carry the risk of major immune-related adverse events (irAEs). Among the most severe irAEs is epidermal necrosis that may clinically mimic Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN). The aim of this study was to provide a summary of the clinical and histological features of ICI-associated epidermal necrosis, with a special focus on factors associated with fatal outcomes in cases of extensive disease. A total of 98 cases, 2 new cases and 96 reported on PubMed and in the literature, of ICI-associated epidermal necrosis were assessed. Development of epidermal necrosis occurred between 1 day and 3 years after starting ICI therapy, with an average onset of 13.8 weeks for patients with limited (< 30% BSA) and 11.3 weeks for those with extensive (≥ 30% BSA) involvement, and a median onset of 5.8 weeks and 4 weeks respectively. A preceding rash was seen in 52 cases and was more common in extensive cases. Mucosal involvement was only reported in 65% of extensive cases but was significantly associated with fatal reactions. Co-administration of cytotoxic chemotherapy was associated with more extensive disease. Recovery was observed in 96% and 65% of those with limited and extensive involvement respectively and no specific therapy was associated with improved survival. Young age was significantly associated with poor outcomes in extensive disease, the average age of surviving patients was 64.5 years old versus 55.1 years old for deceased patients, p < 0.01. Both superficial perivascular and interface/lichenoid inflammatory infiltrates were commonly seen. These findings suggest that ICI-associated epidermal necrosis should be considered a distinct clinical entity from drug-induced SJS/TEN.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    背景:史蒂文斯·约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是严重的疾病,具有很高的发病率和死亡率。
    目的:这篇综述突出了SJS/TEN的珍珠和陷阱,包括介绍,诊断,并根据当前证据对急诊科(ED)进行管理。
    结论:SJS/TEN是一种罕见的,导致皮肤和粘膜去上皮化的迟发性超敏反应。大多数病例与药物治疗或感染有关。临床医生应考虑SJS/TEN在任何出现皮肤皮肤起泡的患者。评估皮肤,粘膜,肺,肾,生殖器,和眼部系统在SJS/TEN的诊断中至关重要,以及在并发症的识别中(例如,脓毒症)。实验室和放射学测试无法确认ED设置中的诊断,但它们可能有助于识别并发症。ED管理包括稳定气道和呼吸,液体复苏,以及用广谱抗生素疗法治疗任何叠加感染。所有疑似SJS/TEN的患者都应转移到烧伤手术中心,重症监护,皮肤病学,和广泛的专家可用性。
    结论:了解SJS/TEN可以帮助急诊临床医生诊断和管理这种可能致命的疾病。
    BACKGROUND: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality.
    OBJECTIVE: This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence.
    CONCLUSIONS: SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes. The majority of cases are associated with medication or infection. Clinicians should consider SJS/TEN in any patient presenting with a blistering mucocutaneous eruption. Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis). Laboratory and radiological testing cannot confirm the diagnosis in the ED setting, but they may assist in the identification of complications. ED management includes stabilization of airway and breathing, fluid resuscitation, and treatment of any superimposed infections with broad-spectrum antibiotic therapy. All patients with suspected SJS/TEN should be transferred and admitted to a center with burn surgery, critical care, dermatology, and broad specialist availability.
    CONCLUSIONS: An understanding of SJS/TEN can assist emergency clinicians in diagnosing and managing this potentially deadly disease.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    一名40多岁的妇女出现在急诊科,患有与史蒂文斯-约翰逊综合征(SJS)一致的弥漫性皮疹。没有可识别的煽动因素。然而,在同一次住院期间,她被新诊断为人类免疫缺陷病毒(HIV)。鉴于她缺乏经典的促发因素,她为何开发SJS的主要理论是由于HIV引起的免疫失调。HIV患者中的大多数SJS/中毒性表皮坏死松解症(TEN)病例与高效抗逆转录病毒疗法和甲氧苄啶-磺胺甲恶唑的预防有关。缺乏关于SJS作为HIV的初始表现的文献,而没有已知的潜在病因或煽动因素。
    A woman in her 40s presented to the emergency department with a diffuse rash consistent with Stevens-Johnson syndrome (SJS). There was no identifiable inciting factor. However, she was newly diagnosed with human immunodeficiency virus (HIV) during that same hospital admission. The leading theory for why she developed SJS given her lack of classic precipitating factors is an immune dysregulation as a result of HIV. Most cases of SJS/toxic epidermal necrolysis (TEN) in patients with HIV are related to highly active antiretroviral therapy and prophylaxis with trimethoprim-sulfamethoxazole. There is a lack of literature regarding SJS as the initial presentation of HIV without known underlying etiology or inciting factors.
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  • 文章类型: Meta-Analysis
    毒性表皮坏死松解症(TEN)涉及广泛的粘膜皮肤损失,护理是支持性的。伤口护理的方法包括手术清创或使用敷料,同时保持表皮完整。缺乏任何一种方法的有力证据。我们比较了TEN成年患者的手术清创术和使用敷料,同时将表皮留在原位(在此称为敷料)。评估的主要结果是死亡率。次要结果是重新上皮化的时间。评估药物的影响。进行了个体患者数据(IPD)系统评价和荟萃分析。对IPD数据的随机效应荟萃分析和生存分析检查了死亡率,上皮再形成时间和全身药物的效果。根据建议评估等级对证据质量进行评级,开发和评估(等级)。PROSPERO:CRD4202126661154项研究纳入系统评价和荟萃分析,涉及227例患者。等级从非常低到中等。手术清创术或敷料的患者的生存率没有差异(单变量:p=0.91,多变量:p=0.31)。接受敷料的患者比接受清创的患者的上皮再生更快(多变量HR:1.96[1.09-3.51],p=0.023)。静脉免疫球蛋白(单变量HR:0.21[0.09-0.45],p<0.001;多变量HR:0.22[0.09-0.53],p<0.001)和环孢菌素显着降低死亡率(单变量HR:0.09[0.01-0.96],p=0.046;多变量HR:0.06[0.01-0.73],p=0.028),与伤口护理无关。这项研究支持皮肤科医院的专家共识,TEN患者的伤口护理应支持表皮保持完整并用敷料支撑,这导致更快的再上皮化。
    Toxic epidermal necrolysis (TEN) involves extensive mucocutaneous loss, and care is supportive. The approach to wound care includes surgical debridement or using dressings while leaving the epidermis intact. Robust evidence for either approach is lacking. We compared surgical debridement to the use of dressings while leaving the epidermis in situ (referred to hereon as dressings) in adult patients with TEN. The primary outcome assessed was mortality. The secondary outcome was time to re-epithelialisation. The impact of medications was evaluated. An individual patient data (IPD) systematic review and meta-analysis was undertaken. A random effects meta-analysis and survival analysis for IPD data examined mortality, re-epithelisation time and the effect of systemic medications. The quality of evidence was rated per the Grading of Recommendations Assessment, Development and Evaluation (GRADE). PROSPERO: CRD42021266611 Fifty-four studies involving 227 patients were included in the systematic review and meta-analysis, with a GRADE from very low to moderate. There was no difference in survival in patients who had surgical debridement or dressings (univariate: p = 0.91, multivariate: p = 0.31). Patients who received dressings re-epithelialised faster than patients who underwent debridement (multivariate HR: 1.96 [1.09-3.51], p = 0.023). Intravenous immunoglobulin (univariate HR: 0.21 [0.09-0.45], p < 0.001; multivariate HR: 0.22 [0.09-0.53], p < 0.001) and cyclosporin significantly reduced mortality (univariate HR: 0.09 [0.01-0.96], p = 0.046; multivariate HR: 0.06 [0.01-0.73], p = 0.028) irrespective of the wound care. This study supports the expert consensus of the dermatology hospitalists, that wound care in patients with TEN should be supportive with the epidermis left intact and supported with dressings, which leads to faster re-epithelialisation.
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