Allopurinol‐Induced Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review of Case Reports and Case Series

Severe acute generalized exanthematous pustulosis with toxic epidermal necrolysis-like desquamation: A case series of 8 patients

Fever, Rash, and Systemic Symptoms: Understanding the Role of Virus and HLA in Severe Cutaneous Drug Allergy

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) associated with the use of nonsteroidal antiinflammatory drugs (NSAIDs) are described. A search of the English- language medical literature was conducted to identify studies and cases of SJS and TEN associated with NSAIDs and cyclooxygenase-2-selective NSAIDs available in the United States. Several epidemiologic studies, case reports, and case series involving SJS and TEN associated with NSAIDs were identified. Of the available NSAIDs, oxicam derivatives appeared to have the greatest association with SJS and TEN. The relative risks reported with other NSAIDs are much lower. The risk with cyclooxygenase-2-selective NSAIDs and meloxicam is less clear, since all were introduced after the completion of the epidemiologic studies. SJS or TEN from NSAIDs and cyclooxygenase-2-selective NSAIDs appears to affect the same patient population as other medications that cause SJS or TEN. The risk of SJS or TEN caused by NSAIDs is extremely low (less than 2 per 1 million users per week for oxicam derivatives, less than 1 per 1 million users per week for other NSAIDs, and 6 cases per 1 million person-years for celecoxib). Aspirin is not typically associated with SJS or TEN. Of the other salicylates, SJS or TEN has only been reported with diflunisal. The risk of SJS or TEN in patients receiving NSAIDs is extremely low; older patients, women, and patients within the first month of treatment initiation appear to have the greatest risk. Health care providers and patients should be aware of the signs and symptoms of SJS and TEN.

A pediatric case of Stevens-Johnson syndrome/toxic epidermal necrolysis with rapid response to intravenous cyclosporine

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.

Soluble FAS Ligand: A Discriminating Feature between Drug-Induced Skin Eruptions and Viral Exanthemas

Background. Stevens — Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe immune-mediated adverse skin reactions, most commonly caused by drug products or infections. Objective. To study the risk factors of SJS and TEN development in patients who were on inpatient treatment in the department of allergology of the City Clinical Hospital № 21 of the Republic of Bashkortostan in 2014—2023. Material and methods. A retrospective analysis of the case histories of 229 patients, considering the affected area of the body’s surface, was carried out. Patients were divided into 3 groups: 83 had SJS, 41 — SJS/TEN combination, 105 — TEN. The following parameters were analyzed with regard to the risk of developing SJS and TEN: age and sex, past medical history and concomitant diseases prior application of drug products, maximum detachment of the epidermis as a percentage of the body’s affected area. Results. Cardiovascular diseases (CVDs) (14.5%), gastrointestinal diseases (8.4%) and diabetes mellitus (6%) were risk factors for SJS development; CVDs (24.4%), peripheral vascular disease (14.6%) and diabetes mellitus (12.2%) — risk factors for SJS/TEN development; diabetes mellitus (16.2%), CVDs (12.4%), HIV (9.5%) and malignant tumors (9.5%) — risk factors for developing TEN. Drug products were the cause of SJS and SJS/TEN development (48.2 and 48.8%, respectively). Pharmaceuticals (63.8%) and viral infection (22.9%) were mainly the etiological factor of TEN development. Conclusion. The presence of HIV, malignant neoplasms and diabetes mellitus are more significant for patients with TEN. Peripheral vascular disease is more common in patients with SJS/TEN than with TEN. Drug products and viral infection were etiologically more significant for TEN than for SJS and SJS/TEN; in contrast, the cause of disease could not be identified more often in patients with SJS and SJS/TEN.

Toxic epidermal necrolysis (TEN) is a rare and potentially fatal skin disorder, precipitated by severe allergic drug reaction, and is one of a spectrum of conditions, which includes Stevens-Johnson syndrome (SJS). Mucosal involvement is common, resulting in extreme pain on swallowing and poor oral intake. The aim of this study was to describe swallow function in TEN and SJS and define the role of Speech Pathology in management. The Burns Unit database was reviewed for patients that presented over a five-year period with TEN and SJS. Diagnosis of TEN and SJS was confirmed by skin biopsy. Information specific to swallow function, treatment approaches, and adequacy of oral intake was collected. Fourteen patients' medical records were studied: eight TEN, two TEN/SJS spectrum, and four SJS. The majority had mucosal involvement causing odynophagia, poor oral intake, an ability to tolerate fluids more easily than solids, and increased aspiration risk. These symptoms were confirmed by Speech Pathology swallowing assessment. Severe mucosal involvement resulting in odynophagia, dysphagia, and poor oral intake is common in TEN and SJS. The speech pathologist is able to assess swallow function and provide recommendations to promote safe oral intake, minimize odynophagia, and facilitate nutritional input critical to optimizing recovery.

To review 10 years' experience in a tertiary care paediatric hospital of erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). In addition, to apply a recently described classification system for EM, SJS and TEN in children. Retrospective study of all children with a discharge diagnosis of EM, SJS or TEN over a 10-year period. A university tertiary care paediatric hospital. Sixty-one paediatric patients with a discharge diagnosis of EM, SJS or TEN. Epidemiology, laboratory features, causative factors, treatment methods, complications and mortality of EM, SJS and TEN in this group of patients. Comparison of correlation with aetiology of old and new classification systems in a paediatric population. Mucous membrane involvement was documented in 61% of patients. Ocular involvement was seen in 39%. Complications occurred in 21% cases, all of whom had SJS or TEN. Only one patient died as a result of their skin condition. Corticosteroids were used in 18% of cases; 95% of whom had a discharge diagnosis of SJS or TEN. The drugs most commonly identified as aetiological agents were sulphonamides and penicillins (26% each). The most frequently implicated infectious agent was herpes simplex virus (19.7%). Classification of study cases according to Bastuji-Garin et al. indicates a strong trend toward bullous EM cases being attributable to infection and SJS/TEN cases to drugs. There was no such clear trend with respect to aetiology when diagnosis was done without the classification system. EM, SJS and TEN rarely cause mortality but significant morbidity is seen. Infectious agents, particularly herpes simplex virus, and drugs, especially the sulphonamides and penicillins, are the most common aetiological agents. The classification system proposed by Bastuji-Garin et al. correlates better with aetiology than the practice that preceded it.

The incidence of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) has been reported to be between 0.95 and 1 per 1000 individuals with AIDS. Accessibility to a cohort of individuals with HIV with known drug exposure (including drug, dose, and time of exposure) and collection of adverse-event information may provide an opportunity to determine an incidence rate of SJS and TEN. The primary objective of this analysis was to determine the incidence of confirmed SJS and TEN in a cohort of Canadian HIV patients who were receiving HIV and HIV-related medications. This was a retrospective analysis of an HIV cohort. The Ontario HIV Treatment Network (OHTN) cohort population was eligible for this analysis. A search of the OHTN database was conducted to determine whether cases with a diagnosis of SJS or TEN were included. Search terms included 'TEN,' 'SJS,' 'epidermal necrolysis,' and 'erythema multiforme.' All SJS and TEN cases recorded in the OHTN database between January 1995 and August 2008 were obtained. Diagnostic criteria for SJS and TEN were established and two reviewers examined the medical records to confirm the SJS or TEN diagnosis. Drug exposure and utilization were documented. Incidence rates for the entire cohort were calculated. Seventeen cases over seven OHTN study sites were identified from an approximate cohort sample size of 3700. There were 15 men (88%). The mean ± SD age was 51.6 ± 11.3 years and time since HIV diagnosis was 16.1 ± 4.4 years. Only one patient reported experiencing a previous SJS or TEN episode. Of the 17 cases, clinical experts diagnosed five cases as true SJS and/or TEN, two cases were labeled as indeterminant, and the remaining cases were considered not SJS or TEN. Among the confirmed cases, drugs taken included nevirapine, trimethoprim/sulfamethoxazole (cotrimoxazole), stavudine (d4T), and clarithromycin. The incidence of SJS and/or TEN was 5-7 per 3710 or approximately 1-2 per 1000 individuals in this cohort with HIV. Careful diagnosis of this adverse event is required for an accurate measure of incidence and to avoid false inflation of the incidence.

Validation of two predictive models designed to aid clinicians in identifying Stevens-Johnson syndrome/toxic epidermal necrolysis: A single institution retrospective review

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare immune-mediated severe cutaneous adverse reactions with incidence rate of 0.05 to 2 persons per million populations per year. Drugs are the most commonly implicated in 95% of cases. To audit the causative drugs, clinical outcome, and cost of management in SJS, TEN, and SJS-TEN overlap. Tertiary care hospitals-based multicentric retrospective study (case series). Indoor case papers of SJS, TEN, and SJS-TEN overlap admitted between January 2006 and December 2009 in four tertiary care hospitals of Gujarat were scrutinized. Data were collected for demographic information, causative drugs, investigations, treatment given, duration of hospital stay, time interval between onset of symptoms and drug intake, clinical outcome, and complications. Data were analyzed to find out proportion of individual drugs responsible, major complications, and clinical outcome in SJS, TEN, and SJS-TEN overlap. Total cost of management was calculated by using cost of drugs, investigations, and consumables used during entire hospital stay. One-way Analysis of Variance followed by Tukey-Kramer multiple comparison test was used for comparison of incubation period, duration of hospital stay, and cost of management. Antimicrobials (50%), nonsteroidal anti-inflammatory drugs (22.41%), and antiseizure drugs (18.96%) were the most commonly associated groups. Nevirapine (28.12%) was the most common drug. Antiseizure drugs were more often associated with serious form of adverse reaction (TEN: 81.8%) than other drugs. Duration of hospital stay (20.6 vs 9.7 days) and cost of management (7,910/- vs 2,460/-) were significantly higher in TEN than SJS (P=0.020 and P<0.001, respectively). Time duration between drug intake and onset of symptoms (17.7 vs 27.5 days) was nonsignificantly lower in TEN as compared with SJS. Secondary infection (28.12%) was the most common complication noted. Mortality rate was 15.6% among all cases; 9% in SJS and 26.7% in TEN. Antimicrobial drugs are the most commonly implicated drugs and cost of managing these adverse drug reactions is higher than other serious ADRs.

In reply: Oral erythema multiforme: trends and clinical findings of a large retrospective: European case series

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as the severest end of spectrum of erythema multiforme. Various etiologies like infections, drugs and malignancies have been proposed. The aim of the present study was to know the incidence, common causes, clinical course of SJS and TEN and to estimate the morbidity and mortality&lt;span lang="EN-IN"&gt;.&lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; A 2 year study of patients presenting with SJS and TEN was carried out. A detailed examination to know the cutaneous and mucosal involvement was done. Biopsy was done in 3 patients.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; There were fifty patients of SJS-TEN spectrum. Of which 31 were SJS, 3 had SJS-TEN overlap and 16 had TEN. Anticonvulsants were implicated in causing these reactions in 24 patients (48%) with carbamazepine being the most common i.e. in 16 patients (32%). Sparing of pressure areas like the strap area of brassier and waist was noticed in two patients (4%). The most common complication was due to eye involvement seen in 20 patients (40%). 46 patients were treated with steroids and of the remaining, 3 were children and one was HIV positive. Only three patients with TEN (6%) died&lt;span lang="EN-IN"&gt;. &lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; To conclude, TEN was less common than SJS, had more sequelae and more mortality compared to SJS&lt;span lang="EN-IN"&gt;.&lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt; &lt;/p&gt;

BackgroundStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases; however, it is hard to estimate their incidence due to the rarity of these diseases. We evaluated the incidence of SJS and TEN using a nationwide administrative database.MethodsWe used a national medical insurance review system (Health Insurance Review and Assessment) database which contained the claim data of the entire nation from 2009 to 2013 to estimate the accurate incidence of SJS and TEN in Korea. The diagnostic codes of L511 (SJS) or L512 (TEN) from the International Classification of Diseases-10th revision were used to define the target study population. We also retrospectively followed up a 2011 SJS and TEN cohort for 24 months in order to assess the in-hospital mortality, related complications and total claims cost due to SJS and TEN.ResultsA total of 1,167 (938 SJS and 229 TEN) cases were newly diagnosed from 2010 to 2013. The age- and sex-standardized annual incidences estimated in this study were 3.96 to 5.03 in SJS and 0.94 to 1.45 in TEN per million. There was no significant change in annual incidence throughout the study periods. When analyzed by 10-year age groups, the annual incidence was the lowest in group 20–29 years and the highest in group 70 for both SJS and TEN. Based on the 2011 cohort analysis, the in-hospital mortality were 5.7 and 15.1% for SJS and TEN, respectively. The mortality increased with age, particularly, after 40 years of age. Among the complications related with SJS or TEN, ocular sequelae was the most common (43.1 and 43.4% of SJS and TEN patients, respectively) followed by urethral sequelae (5.7 and 9.4% of SJS and TEN patients, respectively).ConclusionOverall, our data suggest that SJS, and TEN are infrequent but constantly arise throughout the years.