• Journal of Yeungnam Medical Science
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• 제1권1호
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• pp.185-190
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• 1984

심한 점막병변을 보인 TEN 1예를 경험하여 문헌고찰과 함께 보고한다.

• Childhood Kidney Diseases
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• 제20권2호
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• pp.97-100
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• 2016

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the $4^{th}$ and $5^{th}$ weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.

• 한국치위생학회지
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• 제8권4호
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• pp.31-41
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• 2008

Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis(TEN) are severe mucocutaneous reaction which are most frequently caused by drugs. Although the incidence of SJS and TEN is known to be relatively low, outcomes may be fatal. A systematic approach is required because morbidity rate is currently increasing and oral lesion is frequent. We investigated the clinical features and outcomes of 6 patients diagnosed as SJS and TEN and referred from the department of dermatology, Chonnam National University Hospital for oral care. Ketoconazol, Ofloxacin, Chlorphenesin, Amoxicillin, Pontal, Harnal, and Ciprofloxacin were suspected as the causative drugs. Average treatment period was 3.2 weeks, and two patients were referred to 'burn-patients' hospital. Most of oral lesion were cured be normal tissue, but scares with discoloration were observed. For intraoral management, antibiotic disinfection and steroid application were performed according to systemic treatment principles. Additionally, ingestion of zinc, antioxidants, and vitamin was recommended. The establishment of oral treatment principles is demanded because it has not been yet. Also, through investigation of drug side effect and careful prescription are required.

• Clinical and Experimental Pediatrics
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• 제49권3호
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• pp.332-336
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• 2006

독성 표피괴사 증후군(Toxic epidermal necrosis)은 매우 드문 수포성 피부 질환으로 표피 전층의 괴사와 박리를 특징으로 하는 질환이며, 적절한 치료에도 불구하고 높은 사망률을 보이는 질환이다. 대부분의 독성 표피괴사 증후군의 약물과 연관이 있으며, 대표적인 약물로는 설폰아마이드(Sulfonamide), 항경련제(Phenytoin, Phenobarbital), 페니실린계 항생제, 비스테로이드성 소염제 등이며, 드물게 부신 피질 호르몬제에 의한 발병도 보고되고 있다. 최근 독성 표피괴사 증후군에서 각질 세포의 세포고사(cell apoptosis)에 Fas 수용체가 중요한 역할을 한다고 알려진 후 치료에 면역 글로불린 사용이 점차 늘어나고 있으며, 생존율의 증가와 치료기간의 단축이 보고되고 있다. 국내에서는 아직 독성 표피괴사 증후군에서 면역 글로불린정주가 치료방법으로 확립되지 않았지만 몇몇의 경우에서 면역 글로불린 정주로 치료한 경험들이 보고되고 있다. 저자들은 막성 신염 환아에서 부신 피질 호르몬제와 안지오텐신 수용체 길항제 사용 후 발생한 독성 표피괴사 증후군에서 면역 글로불린을 사용하여 치료한 1례를 경험하였기에 문헌 고찰과 함께 보고하자 한다.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.437-441
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• 2010

독성표피괴사용해(TEN)와 스티븐-존슨 증후군(SJS)은 약물이나 감염에 의해 발생하는 피부점막을 침범하는 드물지만 치명적인 질병이다. 스테로이드는 TEN의 치료에 많이 이용되어왔지만, 아직까지도 논쟁중이다. 스테로이드에 의한 정신과적 영향은 두통, 불면증, 우울증, 심리질환 등이 있다. 스테로이드에 의한 정신질환에서 치료는 스테로이드의 감량 또는 중단이고, 항정신성 약물을 투여한다. 신증후군으로 진단된 11세 남아에서 스테로이드 치료 후 관해상태에서 유지치료를 시행하고 있던 중에 TEN이 발생하였다. 저자들은 이 환아에서 치료목적으로 투여한 고용량 스테로이드 정맥주사로 인해 정신질환이 동반되었고, 이후 스테로이드 감량과 항정신성 약물과 면역글로불린으로 증상이 호전되는 것을 경험하여 이를 보고하는 바이다.

• IMMUNE NETWORK
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• 제16권4호
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• pp.256-260
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• 2016

An association between drug treatment for viral infections and severe cutaneous adverse reactions has been noted. We investigated six patients diagnosed with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) after being prescribed acetaminophen for suspected viral illnesses. Multiplex analysis was performed to measure cytokine levels in sera before and after treatment. IL-2Ra levels significantly decreased during the convalescence phase. Although acetaminophen is relatively safe, the drug can trigger SJS/TEN in patients with suspected viral infections. T-cells and monocytes may be key components of the link between viral infection and acetaminophen-induced SJS/TEN.

• 한국임상약학회지
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• 제23권4호
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• pp.344-364
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• 2013

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immune-complex-mediated hypersensitivity reactions that predominantly involve skin and mucous membranes. Despite the low incidence, both are considered medical emergencies as the mortality rate has been estimated at 30-50%. Although as many as half of cases are idiopathic, several drugs have been implicated as main cause of SJS/TEN. This review therefore aimed to identify drugs that were potentially associated with SJS/TEN and compare the relative risk of the medications. Method: A comprehensive search was performed using MEDLINE, EMBASE and 5 Korean databases. We defined study drugs as non-steroidal anti-inflammatory drugs (NSAIDs), antibiotics, antiepileptics, and allopurinol. Only epidemiologic studies investigating associations between the above drugs and drug-induced SJS/TEN were included. Two reviewers independently selected and evaluated candidate papers and extracted odds ratios or incidence rates. Meta-analysis was performed only for drugs that were reported from 4 or more studies. Results: We found 8 case-control studies, 3 cohort studies and 1 RCT. The ranges of adjusted ORs were 0.6-34.0 for NSAIDs, 1.6-302.0 for antiepileptics, 0.3-10.0 for antibiotics and 1.0-187.0 for allopurinol. The drug with the highest incidence of SJS/TEN was carbamazepine (40 persons/1,000 DDD). Conclusion: Finally, the risk was highest in first 8 weeks after onset of treatment in all drugs.

• BMB Reports
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• 제51권10호
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• pp.484-485
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• 2018

Receptor-interacting protein kinase-3 (RIP3 or RIPK3) is a serine-threonine kinase largely essential for necroptotic cell death; it also plays a role in some inflammatory diseases. High levels of RIP3 are likely sufficient to activate necroptotic and inflammatory pathways downstream of RIP3 in the absence of an upstream stimulus. For example, we have previously detected high levels or RIP3 in the skin of Toxic Epidermal Necrolysis patients; this correlates with increased phosphorylation of MLKL found in these patients. We have long surmised that there are molecular mechanisms to prevent anomalous activity of the RIP3 protein, and so prevent undesirable cell death and inflammatory effects when inappropriately activated. Recent discovery that Carboxyl terminus of Hsp 70-Interacting Protein (CHIP) could mediate ubiquitylation- and lysosome-dependent RIP3 degradation provides a potential protein that has this capacity. However, while screening for RIP3-binding proteins, we discovered that pellino E3 ubiquitin protein ligase 1 (PELI1) also interacts directly with RIP3 protein; further investigation in this study revealed that PELI1 also targets RIP3 for proteasome-dependent degradation. Interestingly, unlike CHIP, which targets RIP3 more generally, PELI1 preferentially targets kinase active RIP3 that has been phosphorylated on T182, subsequently leading to RIP3 degradation.

• Translational and Clinical Pharmacology
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• 제25권2호
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• pp.63-66
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• 2017

Allopurinol-induced severe cutaneous adverse reactions (SCARs) such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome are reportedly associated with the $HLA-B^{\star}58:01$ genotype. Three patients who developed SCARs after allopurinol administration were subjected to HLA-B genotyping and lymphocyte activation test (LAT) to evaluate genetic risk and to detect the causative agent, respectively. All three patients given allopurinol to treat gout were diagnosed with DRESS syndrome. Symptom onset commenced 7-24 days after drug exposure; the patients took allopurinol (100-200 mg/d) for 2-30 days. HLA-B genotyping was performed using a polymerase chain reaction (PCR)-sequence-based typing (SBT) method. All patients had a single $HLA-B^{\star}58:01$ allele: $HLA-B^{\star}13:02/^{\star}58:01$ (a 63-year-old male), $HLA-B^{\star}48:01/^{\star}58:01$ (a 71-year-old female), and $HLA-B^{\star}44:03/^{\star}58:01$ (a 22-year-old male). Only the last patient yielded a positive LAT result, confirming that allopurinol was the causative agent. These findings suggest that patients with $HLA-B^{\star}58:01$ may develop SCARs upon allopurinol administration. Therefore, HLA-B genotyping could be helpful in preventing serious problems attributable to allopurinol treatment, although PCR-SBT HLA-B genotyping is time consuming. A simple genotyping test is required in practice. LAT may help to identify a causative agent.