• Clinical and Experimental Pediatrics
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• 제57권3호
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• pp.153-156
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• 2014

Toxic epidermal necrolysis is an unpredictable and severe adverse drug reaction. In toxic epidermal necrolysis, epidermal damage appears to result from keratinocyte apoptosis. This condition is triggered by many factors, principally drugs such as antiepileptic medications, antibiotics (particularly sulfonamide), nonsteroidal anti-inflammatory drugs, allopurinol, and nevirapine. Lamotrigine has been reported potentially cause serious cutaneous reactions, and concomitant use of valproic acid with lamotrigine significantly increases this risk. We describe a case of an 11-year-old girl with tic and major depressive disorders who developed toxic epidermal necrolysis after treatment with lamotrigine, and who was diagnosed both clinically and pathologically. Children are more susceptible to lamotrigine-induced rash than adults, and risk of serious rash can be lessened by strict adherence to dosing guidelines. Unfortunately, in our case, the patient was administered a higher dose than the required regimen. Therefore, clinicians should strictly adhere to the dose regimen when using lamotrigine, especially in children.

• 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.

• 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.

• 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.

• Journal of Interdisciplinary Genomics
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• 제3권1호
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• pp.7-12
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• 2021

Adverse drug reactions (ADRs) is a hypersensitivity reactions to specific medications, and remain a common and major problem in healthcare. ADRs suchc as drug-induced liver injury and life-threatening severe cutaneous adverse drug reactions including Stevens-Johnson syndrome, toxic epidermal necrolysis, and drug rash with eosinophilia and systemic symptoms can be occurred by uncontrolled expansion of oligoclonal T cells according to genetically predisposing HLA. In this review, I summarized the alleles of HLA genes which have been proposed to have association with ADRs caused by different drugs.

• 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.

• 대한소아치과학회지
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• 제44권4호
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• pp.455-460
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• 2017

스티븐 존슨 증후군(Stevens-Johnson syndrome, SJS)은 피부와 점막에 광범위한 괴사를 초래하는 매우 심각한 급성 과민반응이다. SJS은 모든 연령대에서 발생할 수 있는 질환으로 원인은 명확하지는 않지만 대부분 약물 알레르기에 의한 것으로 알려져 있으며 그 외에도 세균감염에 의해 유발되기도 한다고 알려져 있다. 전체 표피면적의 10% 이하에서 병변이 발생한 경우를 SJS라 하며 30% 이상에서 발생한 경우를 독성 표피 괴사융해라 정의한다. SJS는 1년에 100만명 인구 당 1 - 2명 미만에서 드물게 발생하나, 성장기 어린아이에게 발생할 경우 치아발육 등에 미치는 영향을 미치게 된다. 6 - 7세경 상하악 제1대구치와 하악중절치의 맹출이 시작되고 이 시기에 치근의 발육이 함께 일어난다. 이 케이스의 환아의 경우 6세경 SJS이 발병하였다. 현재 완치된 상태이나 몇몇 후유증이 남아있는 상태이다. 환아는 이 시기에 발육이 완성되는 치아에 국한되어 발육이상이 관찰되었다. 구강내 특징으로 이러한 전신질환을 진단하고 이로 인한 치아 발육에 대한 영향에 대한 문제점을 인식하고 해결하고자 이 보고서를 쓰는 바이다.