Drug-induced Stevens-Johnson Syndrome Research Articles

Drug-induced Stevens-Johnson syndrome in Post traumatic facial injury.

Stevens-Johnson syndrome is a severe adverse drug reaction affecting the skin and mucous membrane. The causes include Sulfonamides, Anticonvulsants, etc. A patient developed ulcerations in the lips and oral cavity with difficulty in swallowing and rashes over the back, abdomen, and genitalia following administration of injection ceftriaxone 1 g intravenous (IV) b.i.d, injection pantoprazole 40 mg IV b.i.d, tablet aceclofenac + paracetamol 325 mg b.i.d, tablet cetirizine 10 mg b.i.d, chlorhexidine mouth wash, and injection metronidazole 500 mg IV t.i.d for the treatment of traumatic facial injury after 4 days of treatment. Injection ceftriaxone and tablet aceclofenac + paracetamol were suspected as the cause of this reaction. The two drugs were stopped. The patient was treated with corticosteroids, other antimicrobials, and oral topical anesthetics. Health-care providers should be careful about the possible adverse drug reactions even to commonly used drugs.

Drug-induced Stevens Johnson syndrome and toxic epidermal necrolysis: Interpreting the systematic reviews on immunomodulatory therapies.

Drug-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are non-immunoglobulin E-mediated severe cutaneous adverse reactions with a high risk of morbidity, mortality, and physical and mental health impact. These are associated with certain high-risk drugs, human leukocyte antigen (HLA)-specific genotypes and ethnicities. HLA class I-restricted oligoclonal CD8 cytotoxic T-cell responses occur at the tissue level in SJS/TEN. Cytotoxic T cells are the T effector cells that result in keratinocyte apoptosis (cell death) mediated by T effector molecules granzyme B, perforin, granulysin, gamma interferon, tumor necrosis factor-alpha, and lipocalin-2. The clinical hallmarks of SJS/TEN include fever, ≥2 mucosal involvements (ocular, oral, and genital), and positive Nikolsky sign with epidermal detachment. Systematic reviews on immunomodulatory treatments remain limited by the paucity of randomized controlled trials, heterogeneity of studies, and non-standardization of outcome measures. Preventive HLA genotype screening before the prescription of carbamazepine and allopurinol may further reduce the incidence of SJS/TEN. The role of immunomodulatory treatments in SJS/TEN is at present not supported by robust evidence from systematic reviews given the lack of randomized controlled trials. The evidence for improved survival with off-label use of corticosteroids plus intravenous immunoglobulins, ciclosporin plus intravenous immunoglobulins, and ciclosporin alone has not been demonstrated by network meta-analyses and meta-regression. In the real-world clinical setting, systemic corticosteroids (in SJS and overlap SJS/TEN), ciclosporin, and etanercept (in TEN) appear to be the off-label treatments currently most widely used.

A Stevens-Johnson syndrome due to rifampicin in a patient with acquired immune deficiency syndrome (AIDS) and pulmonary tuberculosis

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially fatal hypersensitivity reactions that can be drug-induced, especially in patients with AIDS. This report aims to highlight the diagnosis and management of a patient with acquired immunodeficiency syndrome (AIDS) that consumed multiple drugs including anti-tuberculosis drugs that develop SJS. Case presentation: A 30-year-old male patient with AIDS administered with chief complaints of burning sensation all over the bodies and erythematous macules scattered across the stomach and back, followed by blisters distributed across the chest, back, face, lips, hands and feet, as well as genitals for 3 days. Patient was diagnosed with AIDS and under ARV medication since about 5 years ago and dan cotrimoxazole for 9 months. Couple months before admission, patient was diagnosed with tuberculosis and under anti-tuberculosis treatment (isoniazid, rifampicin, pyrazinamide, and ethambutol (HRZE)) but stopped due to drug-induced hepatitis (DIH) which then changed to another regiment (streptomycin, levofloxacin, and ethambutol (SLE)) followed by another regiment (rifampicin, isoniazid and ethambutol (RHE)). The patient diagnosed with drug-induced SJS, however, in order to find out the SJS drugs inducer, an algorithm for assessment of drug causality in Stevens-Johnson Syndrome and toxic epidermal necrolysis (ALDEN) score as causality assessments tool was applied and rifampicin decided to be the precursor. The patient was managed for 13 days prior to discharge with improved condition. Conclusion: Diagnosing process for drug-induced in patient with AIDS, in addition to multiple drugs consumption, is a challenge. A multidisciplinary approach and appropriate causality assessments tool should be considered before administering further management and medications.

Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.

Drug-induced Stevens-Johnson syndrome: a disproportionality analysis from the pharmacovigilance database of the World Health Organization

ABSTRACT Background Stevens-Johnson syndrome is a rare but serious skin condition, which can lead to death. Stevens-Johnson syndrome is usually attributed to drug-induced reactions, thus making it vital for clinicians to prevent its occurrence by knowing the trigger drugs. The objective of this study was to comprehensively and systematically excavate the drugs that cause SJS to provide references for clinician. Research design and methods This is an observational, retrospective study, conducting a disproportionality analysis. Where the Information Component (IC) method and Reporting odds ratio (ROR) are used to mine the drugs that cause SJS. Results A total of 17,787,905 reports were extracted from VigiBase database, of which 25,051 reports were related to SJS. The 18–44 age group had the largest number of cases (N=7,973, 31.83%). A total of 295 drugs was detected as signals. Allopurinol (IC025/ROR025=5.86/69.84), phenytoin (IC025/ROR025=5.60/57.65) and carbamazepine (IC025/ROR025=5.25/43.88) were the top 3 strongest signals. Our study only considered the possibility of SJS caused by a single drug. Conclusions Allopurinol, phenytoin and carbamazepine were three strongest signals. Garenoxaci, carbocisteine and dimetindene were strong signals, but there are no relevant cases reported on PubMed or specific SJS in labels, which need further study to verify.

Clinical and Epidemiological Features of Patients with Drug-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Iran: Different Points of Children from Adults.

Background Different epidemiologic aspects of drug-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children are scarce. Aim To compare the clinical and epidemiological features of patients with drug-induced SJS and TEN in children and adults. Method This retrospective study was conducted at two academic referral centers (Isfahan, Iran) over 5 years. SJS and TEN were clinically diagnosed and confirmed by skin biopsy as needed. Results One hundred one patients (31 children and 70 adults) with a female to male ratio of 1.1 : 1 was identified in the present study. SJS was more commonly diagnosed in both pediatric and adult patients. The most frequent reason for drug administration identified was the infection (45.2%) and seizure (45.2%) in children and infection (34.3%) and psychiatry disorder (27.1%) in adults (P = 0.001). The most common culprit drugs in the pediatric were phenobarbital (9/31), cotrimoxazole (4/31), and amoxicillin (4/31); however, in the adult group, the most common drugs were carbamazepine (11/70) and lamotrigine (9/70). Fever was significantly more common in adults (44.3%) compared to pediatric patients (22.6%) (P = 0.03). Multiple logistic regression models showed that pediatric patients had significantly lower odds of hospitalization (OR [odds ratio]: 0.14; 95% CI 0.02, 0.67). In addition, patients with SCORTEN 1 had significantly higher odds of hospitalization (OR: 6.3; 95% CI: 1.68, 23.79) compared to patients with SCORTEN 0. Conclusions The present study showed several differences between the pediatric and adult patients with SJS and TEN, including the reason for drug administration, culprit drugs, length of hospital stay, presence of fever, and final diagnosis of disease.

Agreement Among Different Scales for Causality Assessment in Drug-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Identification of the offending drug is crucial and challenging in cases of severe cutaneous adverse drug reactions (CADR) like Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Poor reproducibility and varying levels of agreement have been observed among different causality assessment tools (CATs) in assessing severe CADRs. This study was conducted to examine the agreement among four different CATs in assessing cases of drug-induced SJS, TEN and SJS/TEN overlap. All cases of drug-induced SJS, TEN and SJS/TEN overlap, which were reported between January 2012 and January 2020, were identified from the ADR register at an ADR monitoring centre. Causality assessment was done in these reported cases using the following CATs: The World Health Organization-Uppsala Monitoring Centre (WHO-UMC) scale, Naranjo algorithm, Liverpool algorithm and Algorithm of drug causality for epidermal necrolysis (ALDEN). Weighted kappa (κw) test was used to evaluate the agreement among four CATs. A total of 30 cases of drug-induced SJS, TEN and SJS/TEN overlap were included in our analyses. The most common offending groups of drugs were anticonvulsants (46.7%), antimicrobials (40%) and nonsteroidal anti-inflammatory drugs (13.3%). Of the anticonvulsants, phenytoin (13.3%), carbamazepine (10%), and valproate (10%) were the commonly reported offending drugs. Poor agreement was observed among the four different causality assessment scales. Discrepancies were observed among four different CATs in assessing drug-induced SJS and TEN. A CAT, which is more specific to drug-induced SJS and TEN, simple, user-friendly with limited subjective interpretation, incorporating new immunological and pharmacogenetic markers, is necessary.

Clinical characteristics of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: A single-center study.

BackgroundStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse cutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis.ObjectiveWe investigated the differences in clinical characteristics of drug-induced SJS/TEN depending on the type of drug in a single center.MethodsThe relevance of sex, age, culprit drugs, clinical features, courses, treatment options, and follow-up results were retrospectively evaluated in patients diagnosed with drug-induced SJS/TEN at Pusan National University Hospital between 2008 and 2019.ResultsNinety-two patients with a mean age of 58.7 ± 20.2 years (range, 10–93 years) were included in the study. Those aged 60–80 years accounted for the largest number of patients (42.4%). Patients with drug-induced SJS/TEN comprised 40 women (43.5%) and 52 men (56.5%). We categorized drug-induced SJS/TEN cases by culprit drugs into 6 groups: antibiotics, allopurinol, antiepileptic (AED), nonsteroidal anti-inflammatory drugs (NSAIDs), acetaminophen, and other drugs. The rate of NSAID-induced disease significantly increased from SJS to TEN (p = 0.016). Among the patients in the NSAID group, the proportion of TEN (40%) was higher than that in the other groups (p = 0.021). The mean body surface area was significantly lower in the AED group than in the non-AED groups (7.1 ± 9.8 vs. 23.1 ± 27.3, p = 0.020) and higher in the NSAID group than in the non-NSAID groups (47.5 ± 39.5 vs. 15.7 ± 20.0, p = 0.010).ConclusionThis study showed that the clinical characteristics of each causative drug group may be different in drug-induced SJS/TEN. Our findings may help clinicians better understand drug-induced SJS/TEN.

When should you worry about bronchiolitis obliterans?

In this volume of The Journal, Carpenter et al describe a case series of 68 patients with Stevens Johnson Syndrome (SJS), reporting that 9% develop bronchiolitis obliterans. This is potentially an important observation, as it serves to underscore the need to have sufficient diagnostic suspicion to screen for bronchiolitis obliterans in at-risk patients. At the same time, questions remain. The first question centers on case definition of SJS. The authors include both Mycoplasma-induced SJS and drug-induced SJS. With the retrospective design covering January 2004-December 2016, it is not possible to determine whether many of these cases were actually Mycoplasma-induced rash and mucositis (MIRM), a newly described entity (J Am Acad Dermatol 2015;72:239-45). This is particularly important given the relatively high percentage of Mycoplasma-induced SJS and recurrent SJS in those with bronchiolitis obliterans. No patients with bronchiolitis obliterans had drug-induced SJS. We should also note that these cases are from Children's Hospital Colorado. As the mechanism is not yet clear, one speculation is that bronchiolitis obliterans may be more manifest related to altitude or relative hypoxemia. Despite these caveats the report serves to highlight one important complication of these entities and to propose some guidance around screening. As with any rare complication of an already-rare condition, the aggregate experience of multiple centers will need to be examined to clarify the clinical picture. Article page 307 ▸ Stevens-Johnson Syndrome in Children: Consider Monitoring for Bronchiolitis ObliteransThe Journal of PediatricsVol. 236PreviewWe reviewed patients with Stevens-Johnson syndrome (SJS) evaluated at Children's Hospital Colorado and investigated the occurrence of bronchiolitis obliterans (BO). Approximately 9% of patients with SJS developed BO. Pediatricians should consider monitoring patients with SJS for BO, especially those with recurrent SJS and patients treated with mechanical ventilation. Full-Text PDF

The Profiles of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) Patients in Tertiary Hospital

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe eruptive skin reactions that can cause death. The incidence of SJS and TEN cases in the United States is 1.5–9.6 per 1,000,000 per year. Drugs are the primary etiology of SJS and TEN. Some drugs are at high risk and used frequently. The SJS and TEN mortality rates were relatively high, with SJS 4.8%, SJS / TEN overlap 19.8%, and TEN 14.8%. In Indonesia, there are lack of studies on the SJS and TEN. This study is needed to determine the epidemiological profile of SJS and TEN. Purpose: This study aimed to describe SJS and TEN patients' profiles. Methods: Drug-induced SJS and TEN cases from January 2016 to December 2019 were evaluated from the medical records patients' profile, incidence, suspected drugs, risk factors, and comorbidities of SJS and TEN were described. Result: There were 28 SJS and TEN patients, comprising of 24 SJS patients (85.7%), 3 TEN patients (10.7%), and 1 SJS overlapping TEN patients (3.5%). The most common suspected drugs were paracetamol (22.2%), carbamazepine (20.4%), cefadroxil (8.8%), and ciprofloxacin (8.8%). Women (53.5%) experienced more severe drug eruptions than men (46.4%). The largest age group was 25–44 years (35.7%). Most comorbidities were epilepsy (21%), diabetes (15.7%), hypertension (15.7%), and stroke (15.7%). Conclusion: The most common manifestation was SJS with paracetamol as the most common suspected drug, followed by carbamazepine.

Retrospective review of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis cases at a pediatric tertiary care institution.

Stevens-Johnson syndrome and toxic epidermal necrolysis represent important sources of potential mortality and morbidity in children. There is a need for more clinical data in this population to determine whether specific treatments preferentially improve outcomes. This was a single-center retrospective review of children admitted with drug-induced Stevens-Johnson syndrome, toxic epidermal necrolysis or Stevens-Johnson syndrome/toxic epidermal necrolysis overlap at a tertiary care pediatric institution in North America from 2008 to 2018. Patients without a dermatology assessment and diagnosis were excluded. Demographic, clinical, and treatment information were abstracted and reviewed for all included patients. Sixteen patients were identified, 43% female (7/16), with a mean age at presentation of 10.4±5.2years. Antibiotics were implicated in 56.3% of patients (9/16) and anticonvulsants in 31.3% (5/16). Sulfamethoxazole-trimethoprim was the triggering antibiotic in 31.3% of patients. The majority of patients were treated with intravenous immunoglobulin alone (50%, 8/16) or intravenous immunoglobulin with steroids (25%, 4/16). Etanercept was added to intravenous immunoglobulin and corticosteroid in a 2-year-old patient, resulting in clinical stabilization and halting of epidermolysis. No patients died. Clinical sequelae were noted in five patients, including ocular complications (n=4), labial adhesions (n=1), and persistent skin dyspigmentation (n=3). Our results highlight that sulfamethoxazole-trimethoprim is an important cause of Stevens-Johnson syndrome-toxic epidermal necrolysis in children. Mortality was reassuringly low, but ocular sequelae were an important cause of morbidity. More data are needed to help determine whether specific treatments including etanercept may provide mortality or morbidity benefit in pediatric populations.

Correction to: Adverse drug reaction causality assessment tools for drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: room for improvement.

The correct name of the 9th Author is David J. Margolis. The original article was corrected.

Mining Sequential Patterns of Diseases Contracted and Medications Prescribed before the Development of Stevens-Johnson Syndrome in Taiwan

Medication is designed to cure diseases, but serious risks can arise from severe adverse drug reactions (ADRs). ADRs can lead to emergency room visits and hospitalization, straining healthcare resources and, thus, they have strong implications for public health. Stevens–Johnson Syndrome (SJS) is one ADR and comprises the highest proportion of all drug relief cases in Taiwan. Pharmacovigilance involves the collection, detection, assessment, monitoring, and prevention of ADRs, including SJS. Most medical specialists are not fully aware of the risk of drug-induced SJS. Consequently, various drugs may be prescribed to susceptible patients for a great variety of diseases and, in turn, cause SJS. In this research, medical records of SJS patients were retrieved from the Taiwan National Health Insurance Research Database, and the Generalized Sequential Patterns (GSP) algorithm was used to find the sequential patterns of diseases before SJS onset. Then we mined the sequential patterns of medications prescribed in each disease pattern. Afterwards, we detected significant associations of each pattern of diseases and medications prescribed among age groups with statistical analysis. We found that, first, most patients developed SJS after being prescribed the causative medications fewer than four times. Second, Respiratory System Diseases (RSDs) appeared in disease sequential patterns of all lengths. Patterns involving RSDs were more frequent than others. Third, NSAIDs, H2-antagonists for peptic ulcer, penicillin antibiotics, theophylline bronchodilators, and cephalosporin antibiotics were the most frequent medications prescribed. Fourth, we found that patients in certain age groups had higher risks of developing SJS. This study aimed to mine the sequential patterns of diseases contracted and medications prescribed before patients developed SJS in Taiwan. This useful information can be provided to physicians so that they can stop the administration of suspected drugs to avoid evolution towards more severe cases.

Causative drugs and HLA-B polymorphism in drug-induced Stevens-Johnson syndrome - toxic epidermal necrolysis: A study in five hospitals in Jakarta

Stevens-Johnson syndrome and toxic epidermal necrolysis is a very rare but lifethreatening form of cutaneous drug eruption. In recent years, several countries in Asia had succeded in preventing carbamazepine induced SJS/TEN by screening for HLA-B*15:02 before prescribing carbamazepine. This study aimed to acquire data regarding causative drugs and HLA-B allele polymorphism in SJS/TEN patient in Jakarta. We acquired data from 5 referral hospitals from March 2015 to March 2017. Subject fulfilling the inclusion and exclusion criteria was interviewed and blood sample was taken for DNA extraction. The DNA was examined with PCR SSOP and Luminex technology for high resolution HLA-B typing. We studied 22 subjects. The median age was 45,4 years old (14-74). The most common causative drug in this study is carbamazepine. HLA-B*15:02 and HLAB* 18:01 were the most common allele in all subjects. HLA-B*15:02 was found in five (72%) out of seven subjects whose condition was caused by carbamazepine. The most common causative drug of SJS/TEN in five hospitals in Jakarta is carbamazepine, with five (72%) out seven subjects had HLA-B*15:02 allele.

Antiepileptic drug-induced Stevens–Johnson syndrome: Which one to stop or continue?

Antiepileptic drug-induced Stevens–Johnson syndrome: Which one to stop or continue?

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: Proportion and determinants of underreporting to pharmacovigilance

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: Proportion and determinants of underreporting to pharmacovigilance

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in vietnamese spontaneous adverse drug reaction database: A subgroup approach to disproportionality analysis.

Despite the numerous studies investigating drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), the understanding and quantitative data in developing countries remain limited. The study aimed to describe and quantify the drug-related risk of SJS/TEN in a resource-limited context using the Vietnamese spontaneous reporting database (VSRD) of adverse drug reactions. Spontaneous reports relating to medium- and late-onset severe cutaneous adverse reactions (MLOSCAR) and SJS/TEN recorded in the VSRD from 2010 to 2015 were retrospectively analysed. The demographic characteristics and drug information were described and compared between SJS/TEN and other MLOSCAR reports. The drug-induced SJS/TEN signals were estimated using subgrouped disproportionality analysis with calculation of the reporting odds ratio (ROR) and the respective 95% confidence interval (CI). The VSRD received 2,849 MLOSCAR reports, 136 of which focus on SJS/TEN over a 6-year period. About 60% of SJS/TEN patients were male, and the majority of them were adults (mean age 42.5±22.9). Up to 91.8% of drugs induced SJS/TEN within 1-28days, and 45% SJS/TEN cases were evaluated as life-threatening. Positive signals were generated with carbamazepine (n=25, ROR [95% CI]=11.99 [7.07-19.92]), allopurinol (n=15, ROR [95% CI]=4.2 [2.20-7.59]), traditional/herbal medicines (n=7, ROR [95% CI]=2.76 [1.12-5.86]), colchicine (n=4, ROR [95% CI]=6.22 [1.69-18.72]), valproic acid (n=3, ROR [95% CI]=8.71 [1.89-30.19]) and meloxicam (n=3, ROR [95% CI]=7.09 [1.55-24.29]), which are well known for SJS/TEN. Cefixime (n=5, ROR [95% CI]=3.34 [1.13-8.00]) and paracetamol (n=22, ROR [95% CI]=5.23 [3.10-8.49]) also generated positive signals despite their popularity in Vietnam. This first Vietnamese population-based study has highlighted original characteristics and signals of drug-induced SJS/TEN, which are relatively consistent with other worldwide data and typical for a developing country.

Drug-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Call for Optimum Patient Stratification and Theranostics via Pharmacogenomics.

The Global Genomic Medicine Collaborative, a multinational coalition of genomic and policy experts working to implement genomics in clinical care, considers pharmacogenomics to be among the first areas in genomic medicine that can provide guidance in routine clinical practice, by linking genetic variation and drug response. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe life-threatening reactions to medications with a high incidence worldwide. Genomic screening prior to drug administration is a key opportunity and potential paradigm for using genomic medicine to reduce morbidity and mortality and ultimately eliminate one of the most devastating adverse drug reactions. This review focuses on the current understanding of the surveillance, pathogenesis, and treatment of SJS/TEN, including the role of genomics and pharmacogenomics in the etiology, treatment, and eradication of preventable causes of drug-induced SJS/TEN. Gaps, unmet needs, and priorities for future research have been identified for the optimal management of drug-induced SJS/TEN in various ethnic populations. Pharmacogenomics holds great promise for optimal patient stratification and theranostics, yet its clinical implementation needs to be cost-effective and sustainable.

Vancomycin-Induced Stevens-Johnson Syndrome in a Boy Under 2 Years Old: An Early Diagnosis by Granulysin Rapid Test

Stevens-Johnson syndrome (SJS) is a life-threatening disease, which is mainly ascribed to drugs, such as sulfonamides and psychoepileptics. In this article, we present a pediatric case of vancomycin-induced SJS and an alternative diagnostic algorithm. The patient presented with multiple target-like rashes and vesicles throughout the whole body after receiving vancomycin. Despite the fact that skin biopsy remains the gold standard for diagnosing SJS, the granulysin rapid test by immunochromatographic assay is a non-invasive option for children. In this article, we describe our use of the Algorithm of Drug causality for Epidermal Necrolysis and a modified T-cell activation assay for granzyme B and interferon gamma to screen for the culprit drug. Moreover, we applied the granulysin rapid test as an early diagnosis method for children with drug-induced SJS.

504 Mycoplasma pneumoniae-associated Stevens-Johnson syndrome; clinical and histological features

Mycoplasma pneumoniae (M. pneumoniae) induces various types of skin manifestations and potentially develop extensive mucous/eye lesion consistent with Stevens-Johnson syndrome(SJS). Specifically, in children cases with SJS, M.pneumoniae infection, as well as various drugs, is considered a major cause of SJS. M.pneumoniae-associated SJS cases often show only mucous/eye lesions without skin lesions, whereas typical drug-induced SJS cases show both skin lesions and mucosal lesions. In addition, palatal small vesicles are thought to be characteristic in M.pneumoniae-associated SJS. We aimed to investigate the clinical and histological findings of M.pneumoniae-associated SJS. Furthermore we performed M.pneumoniae staining in mucous lesions to detect M. pneumoniae-infected cells. The cases in our study (n=2) showed mucosal lesions and high fevers, which were suspected to be M.pneumonae-associated SJS. The patients had ocular lesions and severe mucous erosions including palatal small vesicles, however there were no skin lesions. Tests of serology and direct pathogen detection indicated M.pneumonae infection. Lip biopsies revealed sparse mucoepithelial necrotic cells in only basal cells and band-like inflammatory cell infiltrate in upper lamina propria mucosae. On the other hand, drug-induced SJS is widespread-epithelial cell necrosis through mucoepithelium. In M.pneumonae staining, there were positive cells in lamina propria mucosae, whereas there were no positive cells in mucoepithelium. From our study, M.pnumoniae-associated SJS is clearly distinguishable from drug-associated SJS in clinical manifestation and pathological findings. In addition, M.pneumonae infected cells in lamina propria mucosae might influence to basal layer cell death in M.pnumoniae-associated SJS.