Steven-Johnson syndrome

史蒂文 - 约翰逊综合征
  • 文章类型: Case Reports
    以对药物的免疫反应为特征,中毒性表皮坏死松解症(TEN)和Steven-Johnson综合征(SJS)是潜在致命的皮肤粘膜反应,他们的管理仍然具有挑战性。考虑到关于使用激光治疗口面病变的有希望的研究,本研究旨在报告2例出现TEN和SJS的儿童在常规支持治疗的同时使用抗菌光动力疗法(aPDT)和光生物调节疗法(PBMT)治疗.本文提出的治疗方法在几天内显著改善了儿童的口腔状况,允许重新引入口服喂养。在研究的局限性内,报道的病例表明,PBMT和aPDT的辅助组合可能有利于改善TEN和SJS所致口腔损伤患儿的口腔状况.
    Characterized by an immune reaction to medications, toxic epidermal necrolysis (TEN) and Steven-Johnson Syndrome (SJS) are potentially fatal mucocutaneous reactions, and their management remains challenging. Considering the promising studies regarding the use of laser in managing orofacial lesions, this study aimed to report two cases in which children presenting with TEN and SJS were treated using a combination of antimicrobial photodynamic therapy (aPDT) and photobiomodulation therapy (PBMT) concurrently with conventional supportive care. The treatment proposed herein resulted in significant clinical improvement of the children\'s oral condition within a few days, enabling the reintroduction of oral feeding. Within the limitations of the study, the cases reported suggest that the adjuvant combination of PBMT and aPDT may be beneficial for improving the oral condition of children afflicted with oral injuries induced by TEN and SJS.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    目的:比较儿童和成人Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)的临床特征和视觉结果。
    方法:回顾性比较病例系列。
    方法:这项回顾性研究包括2010年至2020年间接受SJS/TEN治疗的140名患者(35名儿童和105名成人)的280只眼。主要结果指标是最终最佳矫正视力(BCVA)和干眼的严重程度。次要结果指标是使用的药物和手术治疗。
    结果:在这项研究中招募的64眼儿童中,急性眼部受累58眼(90.6%)。儿科患者的慢性评分明显高于成人患者(p=0.004)。使用抗生素/非甾体抗炎药(NSAIDs)和支原体感染是儿童中更常见的病因。在平均4.3年的随访时间内,75%的儿童眼睛保持20/40或更好的视力。儿童和成人组之间干燥的严重程度相当。慢性期儿童行羊膜和口腔黏膜移植的眼比例明显高于成人,反映出儿童表现出更严重的并发症。
    结论:尽管儿童SJS/TEN患者比成人有更严重的眼部并发症,大多数儿童保持长期良好的视力。早期干预和积极治疗有助于保护视力。
    To compare the clinical features and visual outcomes in children and adults with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).
    Retrospective comparative case series.
    This retrospective study included 280 eyes of 140 patients (35 children and 105 adults) with SJS/TEN treated between 2010 and 2020. The primary outcome measures were the final best-corrected visual acuity (BCVA) and severity of dry eye. The secondary outcome measure was the medical and surgical therapies used.
    Among 64 eyes of children recruited in the study, acute ocular involvement was found in 58 eyes (90.6%). The chronic score in pediatric patients was significantly higher than that in adult patients (P = .004). The use of antibiotics/nonsteroidal anti-inflammatory drugs (NSAIDs) and Mycoplasma infection were the more common etiologies in children. In all, 75% of eyes in children maintained a visual acuity of 20/40 or better at a mean follow-up time of 4.3 years. The severity of dryness was comparable between the child and adult groups. The proportion of eyes undergoing amniotic membrane and oral mucosa transplantation was significantly higher in children than in adults in the chronic stage, reflecting that children exhibit much more severe complications.
    Although pediatric SJS/TEN patients have more severe ocular complications than adults, most children maintain long-term good vision. Early intervention and aggressive treatment help to preserve vision.
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  • 文章类型: Case Reports
    COVID-19是由一种名为SARS-CoV-2的新型人畜共患RNA病毒引起的全球大流行。已经描述了与COVID-19相关的各种皮肤表现,包括荨麻疹,融合性红斑皮疹,丘疹囊泡放样,冻疮样的肢端模式,网状livedo,和紫癜性血管炎.这里,我们介绍一例45岁男性皮肤黏膜特征为Stevens-Johnson综合征.
    COVID-19 is a global pandemic caused by a novel zoonotic RNA virus named SARS-CoV-2. Various cutaneous manifestations associated with COVID-19 have been described, including urticarial rash, confluent erythematous rash, papulovesicular exanthem, chilblain-like acral pattern, livedo reticularis, and purpuric vasculitis pattern. Here, we are presenting a case of a 45-year-old male with mucocutaneous features of Stevens-Johnson syndrome.
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  • 文章类型: Case Reports
    SJS/TEN(Stevens-Johnson综合征/中毒性表皮松解性坏死)是一种T细胞介导的超敏反应综合征,其中细胞毒性CD8+细胞对角质形成细胞发生反应,导致广泛的细胞凋亡和坏死。这些病例中约有90%归因于药物反应,而10%是特发性的。该疾病根据体表面积(BSA)受累和表皮丢失的厚度进行分类。我们报告了一例女性患有边缘性人格障碍的抗精神病药物,服用环丙沙星治疗尿路感染(UTI)后出现SJS/TEN重叠。经过精心的管理,她的病情得到了改善,但是在她的抗生素从静脉注射克拉霉素改为口服利奈唑胺后,她又开发了SJS/TEN,这一次更严重的参与。她接受了涉及多学科方法的积极管理。她的病情慢慢好转,一个月后,她的病变开始愈合,她出院时建议今后不要同时使用这两种抗菌药物。
    SJS/TEN (Stevens-Johnson syndrome/toxic epidermolysis necrosis) is a T-cell mediated hypersensitivity syndrome in which cytotoxic CD8+ cells react against keratinocytes, resulting in widespread apoptosis and cell necrosis. About 90% of these cases are attributed to drug reactions, while 10% are idiopathic. The disease is classified according to body surface area (BSA) involvement and the thickness of epidermal loss. We report a case of a female with borderline personality disorder on antipsychotic medication, who developed SJS/TEN overlap after taking ciprofloxacin for her urinary tract infection (UTI). Her condition improved with meticulous management, but after switching her antibiotic from intravenous clarithromycin to oral linezolid, she developed SJS/TEN again, this time with more severe involvement. She received active management involving a multidisciplinary approach. Her condition improved slowly and, after one month, her lesions began to heal, and she was discharged with advice not to use both antimicrobial drugs in the future.
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  • 文章类型: Case Reports
    Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)是罕见但危及生命的药物引起的超敏反应,是基于皮肤脱离区域的疾病连续体。在使用多西他赛治疗三个周期后,一名患有早期人类表皮生长因子受体2(HER2)阳性乳腺癌的60岁女性,因流感样疾病和双侧轨道黑色结痂而被送往医院,肚脐,和肛周区域。Nikolsky信号是阳性的,患者随后被转移到专门的烧伤中心治疗SJS/TEN重叠综合征.在癌症患者中给予多西他赛后有少量记录SJS/TEN的病例。
    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening drug-induced hypersensitivity reactions existing as a disease continuum based on the area of skin detachment. Following three cycles of treatment with docetaxel, a 60-year-old female with early-stage human epidermal growth factor receptor 2 (HER2)-positive breast cancer presented to the hospital with a flu-like illness and black crusting of the bilateral orbits, navel, and perianal region. Nikolsky sign was positive, and the patient was subsequently transferred to a specialized burn center for treatment of SJS/TEN overlap syndrome. There are a small number of cases documenting SJS/TEN following docetaxel administration in cancer patients.
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  • 文章类型: Case Reports
    药物通常会引起严重的免疫介导的皮肤粘膜反应,包括Steven-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)。在SJS和TEN的急性期,发热性疾病后出现皮肤红斑并形成水疱,皮肤和粘膜坏死,皮肤和粘膜的分离。病人很快就有死亡的危险,需要立即就医。在这个案例报告中,我们描述了一例Steven-Johnson综合征患者,该患者为一名102岁女性,正在接受姑息治疗,患有5期慢性肾病.虽然在这名患者中导致SJS的病原体未知,患者接受了局部药物治疗,用绷带包扎病灶,和口服抗组胺药.皮肤活检,腹部超声,建议对患者进行sezary细胞测试。在那个年龄的这种演讲没有,根据我们的知识,以前被记录在案。
    Medicines often cause serious immune-mediated mucocutaneous reactions including Steven-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN). In the acute phase of SJS and TEN, a febrile illness is followed by cutaneous erythema with blister formation, skin and mucous membrane necrosis, and separation of the skin and mucous membranes. The patient swiftly becomes in danger of dying, necessitating immediate medical attention. In this case report, we described a case of Steven-Johnson Syndrome in a 102-year-old female who was receiving palliative care and had stage 5 chronic renal disease. Although the agent that caused SJS in this patient is unknown, the patient was managed with topical medication, bandages for the lesions, and oral antihistamines. Skin biopsy, abdomen ultrasound, and sezary cell test were advised for the patient. Such presentations at that age have not, to our knowledge, been documented before.
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  • 文章类型: Case Reports
    Nivolumab是一种人源化单克隆抗程序性细胞死亡受体-1(PD-1)抗体,已被授权用于治疗晚期恶性肿瘤。皮肤反应是抗PD-1药物报告的最常见的免疫相关不良事件,它们的范围广泛,从轻微的局部反应到很少严重或危及生命的全身性皮肤病。使用nivolumab的史蒂文-约翰逊综合征(SJS)或中毒性表皮坏死松解症(TEN)的发生是一种极其罕见的现象,仅在当前文献中的少数病例中记录。但由于SJS/TEN可能与致命结局相关,因此值得关注.我们在一名患有转移性胃腺癌的中年女性患者中介绍了一例nivolumab诱导的SJS/TEN,该患者已通过免疫抑制疗法和支持治疗成功治疗。当临床怀疑SJS/TEN时,有必要立即识别SJS/TEN并停用nivolumab。专业烧伤单位的多学科管理是改善SJS/TEN结果的关键。
    Nivolumab is a humanized monoclonal anti-programmed cell death receptor-1 (PD-1) antibody that has been authorized for use in the treatment of advanced malignancies. Cutaneous reactions are the most common immune-related adverse events reported with anti-PD-1 agents, and they range broadly from mild localized reactions to rarely severe or life-threatening systemic dermatoses. The occurrence of Steven-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) with nivolumab use is an exceedingly rare phenomenon that was only documented in a handful of cases in the current literature, but it deserves careful attention as SJS/TEN may be associated with fatal outcomes. We present a case of nivolumab-induced SJS/TEN in a middle-aged female patient with metastatic gastric adenocarcinoma that was successfully treated with immunosuppressive therapy and supportive care. Prompt recognition of SJS/TEN with discontinuation of nivolumab is warranted when SJS/TEN is suspected clinically. Multidisciplinary management in a specialized burn unit is the key to improving outcomes of SJS/TEN.
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  • 文章类型: Case Reports
    背景:我们报道了一名43岁的中国男性患有痛风,他在意大利生活了几年。
    方法:以前使用别嘌呤醇治疗会诱发StevenJohnson综合征,决定改用非布索坦每天80毫克。在用非布索坦治疗两年后,他出现弥漫性斑丘疹伴严重瘙痒。风湿病学家停止了非布索坦;然而,尽管接受了钾盐和秋水仙碱治疗,但痛风在接下来的几年中恶化。因此,要求进行过敏咨询。开始使用非布索坦进行缓慢脱敏方案,低口服剂量方案增加到80毫克/天。非布索坦是由咨询药剂师以固体制剂形式制成的药丸,而不是更常用的液体悬浮液。
    结论:患者目前正在接受非布索坦80毫克,到目前为止他还没有表现出副作用,虽然他的痛风有所改善。这是第一个报道的例子,到目前为止他还没有显示出副作用,而他的痛风改善了使用稀释的非布索坦作为药丸的固体制剂的成功脱敏方案。
    BACKGROUND: We report the case of a 43-year-old Chinese male with Tophaceous gout who had been living in Italy for some years.
    METHODS: Previous treatments with allopurinol had induced Steven Johnson syndrome, dictating a switch to febuxostat 80 mg daily. After two years of treatment with febuxostat, he developed a diffuse maculopapular rash with severe itching. Rheumatologists stopped febuxostat; however, gout worsened over the following years despite treatment with kalnicitrate and colchicine. Therefore, an allergy consultation was called for. A slow desensitization protocol with febuxostat was started, with a low oral dosage scheme to be increased up to 80 mg/day. Febuxostat was prepared in a solid formulation by the consultation pharmacist as pills instead of the more frequently used liquid suspension.
    CONCLUSIONS: The patient is currently receiving febuxostat 80 mg, and he has shown no side effects as of now, while his gout has improved. This is the first reported example and he has shown no side effect till now, while his gout improved of a successful desensitization protocol using a solid preparation of diluted febuxostat given as pills.
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  • 文章类型: Case Reports
    Stevens-Johnson综合征(SJS)是一种罕见的疾病,在药物治疗后会发生广泛的中毒性表皮溶解。大约90%的SJS病例涉及皮肤和粘膜,当它们受到影响时,可能会出现嘴角的织带(微口腔)。在SJS中,很少有关于微口腔的报告,关于治疗方法尚未达成共识,计时,或结果。我们在一名22岁的妇女中,在氧氟沙星药物治疗后,遇到了一例SJS后的微小口炎病例,该妇女使用菱形切除术进行了连缝成形术。我们提出了一种适当的矫正方法和手术时机,以治疗SJS后的小口。
    Stevens-Johnson syndrome (SJS) is a rare disease in which extensive toxic epidermolysis occurs after medication. Skin and mucous membranes are involved in about 90% of SJS cases, and webbing of mouth corners (microstomia) may occur when they are affected. Few reports have been issued on microstomia in SJS, and no consensus has been reached regarding treatment methods, timings, or results. We encountered a case of microstomia following SJS after ofloxacin medication in a 22-year-old woman treated by commissuroplasty using a lozenge-shaped excision. We present an appropriate correction method and surgical timing for microstomia following SJS.
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