Cases Of Stevens-Johnson Syndrome Research Articles

Medication Associations With Severe Cutaneous Adverse Reactions: A Case/Non-Case Analysis Using the FDA Adverse Event Reporting System.

Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS), and acute generalized exanthematous pustulosis (AGEP) are potentially life-threatening severe cutaneous adverse reactions (SCARs). Although the classical causal agents of SCARs (antibiotics, anticonvulsants, nonsteroidal anti-inflammatory drugs, and allopurinol) are well characterized, there has been little update to this list to account for newly marketed medications. To provide an updated and stratified list of medications with significant reporting odds ratios (RORs) of SCARs. A case/non-case analysis using the United States FDA Adverse Event Reporting System was performed. As expected, the prototypical medication classes made up the majority of reported cases of SJS, TEN, AGEP, and DRESS (77%, 64%, 75%, and 72%, respectively). In addition, several infrequently or previously undescribed classes/medications implicated in SCARs were identified to have significant ROR signals, including acetylcysteine, anticoagulants, diuretics, immunotherapies, proton pump inhibitors, antivirals, and antifungals. Among these reported for SJS were acetylcysteine (ROR: 64.38) and fluconazole (ROR: 17.13). For TEN, we identified furosemide (ROR: 26.32), spironolactone (ROR: 14.45), fluconazole (ROR: 30.21), amphotericin B (39.06), and acetylcysteine (ROR: 93.12). For AGEP, we identified acyclovir (ROR: 61.72), valacyclovir (ROR: 30.76), and enoxaparin (ROR: 27.37). For DRESS, we identified vemurafenib (ROR: 17.35), acyclovir (ROR: 30.63), abacavir (ROR: 26.62), raltegravir (ROR: 23.27), and valacyclovir (ROR: 21.77) to have strong reporting odds. Our analysis provides an updated tool for physicians to reference when identifying suspected SCARs and a basis for future studies to investigate atypical medication causality.

Immune checkpoint inhibitor-induced epidermal necrolysis: A narrative review evaluating demographics, clinical features, and culprit medications.

Immune checkpoint inhibitors (ICIs) have transformed cancer treatment but can cause immune-related adverse events (irAEs). Severe cutaneous irAEs, including epidermal necrolysis, are rare but potentially life-threatening. There is limited understanding of the clinical features and management of ICI-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), so we aimed to analyze 95 cases of ICI-induced SJS/TEN (35 cases of SJS, 26 cases of TEN, two cases of SJS/TEN overlap, and 32 cases of unspecified) to increase knowledge of this condition among oncologists and dermatologists. We conducted a comprehensive search of PubMed for all relevant case reports published until the end of December 2022, and collected data on patient demographics, cancer type, ICI regimen, time to onset of SJS/TEN, clinical presentation, management strategies, and outcomes. PD-1 inhibitors were the most common ICIs associated with SJS/TEN (58.9%), followed by the combination of PD-1 and CTLA-4 inhibitors (11.6%), and PD-L1 inhibitors (6.3%). Lung cancer and melanoma were the most frequent malignancies treated (35.8% and 25.4%, respectively). SJS/TEN occurred most frequently within the first 4 weeks (51.7%), and corticosteroid monotherapy was the most commonly chosen systemic treatment (56.4%). The overall mortality rate of ICI-induced SJS/TEN was 30.8%. Our findings highlight the frequency and severity of ICI-induced SJS/TEN and the urgent need for predictive molecular biomarkers aimed at preventive measures and early intervention.

P103 A retrospective observational study of toxic epidermal necrolysis at a tertiary referral centre from 2015 to 2022

Abstract Toxic epidermal necrolysis (TEN) is associated with high mortality and morbidity. Owing to a lack of robust evidence, treatment guidelines are ambiguous and clinicians must use discretion in management decisions, specifically with regard to the use and choice of systemic agents, including immunosuppressants. We present a retrospective study of cases referred to a tertiary referral centre between 2015 and 2022. Cases were identified using histopathology coding and a database compiled by clinicians. Forty cases were categorized as follows: TEN (n = 12); Stevens–Johnson syndrome (SJS)/TEN overlap (n = 5); SJS (n = 5); unclear (n = 9); insufficient information (n = 5) and others [n = 9 (lupus, n = 4; erythema multiforme major, n = 3; burn, n = 1; recurrent erythema multiforme, n = 1)]. The median age of patients with TEN was 66 [interquartile range (IQR) 53.3–74; 33% men, 67% women]. The median percentage body surface area of epidermal loss was 40% (n = 8; IQR 30–70). Forty-two per cent of patients had an underlying malignancy. Implicated medications included nivolumab/ipilimumab (n = 2), co-trimoxazole (n = 2), carbamazepine (n = 1) and proton pump inhibitors (n = 3). Sixty-seven per cent of patients with TEN were managed by the burns centre, with a further 17% admitted to the intensive care unit. One patient presented directly to the dermatology team and 58% were transferred from other hospitals. The median time from the documented onset of rash to transfer to specialist care was 5 days (IQR 2.8–11.5). Thirty-three per cent of patients were given immunosuppression (intravenous methylprednisolone). The median duration of admission was 14 days (IQR 7.5–21.5). Fifty-eight per cent of patients (n = 7) survived. None of the five patients who died had received immunosuppression. The median time from onset of rash to receiving specialist care was 3.5 days for those who survived (n = 4) vs. 10 days for those who did not (n = 3; P = 0.03). Thirty-three per cent of patients had documented chronic complications (severe ophthalmic sequelae, n = 2; circumcision for phimosis, n = 1). This study was limited by the retrospective collection of data and the method used to identify cases. The current evidence for the treatment of TEN is primarily derived from small case series that often combine heterogeneous cases of TEN, SJS/TEN overlap and SJS. This is highly problematic as the risk–benefit dynamic varies greatly between patients with SJS, who have an excellent prognosis without treatment, and patients with TEN with a high associated mortality, who potentially have more to gain from immunosuppression. Our data support the need for patients to access specialist care promptly, to allow for early consideration of immunosuppression. Future research should focus on effective treatments for reducing mortality in cases of TEN specifically.

Investigation of Four Cases of Stevens-Johnson Syndrome among Participants in a Mass Drug Administration Campaign with Sulfadoxine-Pyrimethamine and Primaquine in Haiti, 2020.

In 2018, a mass drug administration (MDA) campaign for malaria elimination was piloted in Haiti. The pilot treated 36,338 people with sulfadoxine-pyrimethamine (SP) and primaquine; no severe adverse events were detected. In 2020, another MDA campaign using the same medications was implemented to mitigate an upsurge in malaria cases during the COVID-19 pandemic. Four cases of Stevens-Johnson syndrome (SJS) were identified among the 42,249 people who took the medications. Three of these individuals required hospitalization; all survived. In addition to SP ingestion, an investigation of potential causes for increased SJS cases identified that all four cases had human leukocyte antigens A*29 and/or B*44:03, another known risk factor for SJS. Additionally, three of the four case individuals had antibodies to SARS-CoV-2, and the fourth may have been exposed around the same time. These findings raise the possibility that recent SARS-CoV-2 infection may have contributed to the increased risk for SJS associated with SP exposure during the 2020 campaign.

Toxic epidermal necrolysis and Stevens-Johnson syndrome after COVID-19 infection and vaccination.

Stevens-Johnson Syndrome (SJS) is a rare but severe skin reaction characterized by blistering and peeling of the skin and ulcerations of mucous membranes; toxic epidermal necrolysis (TEN) is a subset of SJS characterized by the involvement of >30% of the skin. Though previously associated with drugs and infections, discussions on the association between TEN/SJS and COVID-19 have been limited. We present a review of TEN/SJS after COVID-19 infection and vaccination. Literature searches were conducted on PubMed and Google Scholar from 2019 to 8/2022. Thirty-eight articles were selected based on subject relevance, and references within selected articles were also screened for relevance. As of 8/2022, there have been 34 published cases of TEN, SJS, and SJS-TEN overlap after COVID-19 infection and vaccination, including 12 cases after vaccination and 22 cases after infection. Multiple authors hypothesize that virotopes or excipients in COVID-19 vaccines can activate T-cells or cytokines to induce TEN/SJS. Meanwhile, some hypothesize that COVID-19 infection induces immune activation that can trigger TEN/SJS or increase susceptibility to drug-induced TEN/SJS. Treatments for post-infection and post-vaccination TEN/SJS vary significantly. We recommend remaining vigilant for this rare and severe potential complication.

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.

Chronic Analgesic (NSAID induced) SJS: A Case Report

In the contemporary era, use of drugs is the dominant paradigm of health care. The most quotidiandrug used for fever and pain is paracetamol. Although adverse reactions to paracetamol in India arerare, at times they can cause life-threatening situations. Stevens-Johnson syndrome (SJS) is one suchpotentially lethal adverse drug reaction. The most reported cases of analgesic-induced SJS were dueto oxicams or propionic acid derivatives. There are very few detailed reports of SJS due to the useof paracetamol. We report a case of SJS, which occurred due to the use of paracetamol. The clinicalfeatures of this condition and multidisciplinary management of the patient are described in brief.

Towards a better understanding of adult idiopathic epidermal necrolysis: a retrospective study of 19 cases.

Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are drug-induced. A small subset of cases remain with unknown aetiology (idiopathic epidermal necrolysis [IEN]). We sought to better describe adult IEN and understand the aetiology. This retrospective study was conducted in 4 centres of the French national reference centre for epidermal necrolysis. Clinical data were collected for the 19 adults hospitalized for IEN between January 2015 and December 2019. Wide toxicology analysis of blood samples was performed. Histology of IEN cases was compared with blinding to skin biopsies of drug-induced EN (DIEN, 'controls'). Available baseline skin biopsies were analysed by shotgun metagenomics and transcriptomics and compared to controls. IEN cases represented 15.6% of all EN cases in these centres. The median age of patients was 38 (range 16-51) years; 68.4% were women. Overall, 63.2% (n=12) of cases required intensive care unit admission and 15.8% (n=3) died at the acute phase. Histology showed the same patterns of early- to late-stage EN with no difference between DIEN and IEN cases. One toxicology analysis showed unexpected traces of carbamazepine; results for other cases were negative. Metagenomics analysis revealed no unexpected pathological microorganism. Transcriptomic analysis highlighted a different pro-apoptotic pathway in IEN compared to DIEN, with an overexpression of apoptosis effectors TWEAK/TRAIL. IEN affects young people and is a severe form of EN. A large toxicologic investigation is warranted. Different pathways seem involved in IEN and DIEN, leading to the same apoptotic effect, but the primary trigger remains unknown.

Rifampicin- and allopurinol-induced Stevens-Johnson syndrome: A case series

Stevens-Johnson syndrome (SJS) is a rare, serious disorder and may be life threatening affecting mainly mucocutaneous tissues. It is a type of generalised, multisystemic hypersensitivity reaction directly linked to the drug intake. It is one of the few serious adverse effects of drugs involving skin and mucous membranes which are characterised by rash, bullae and blisters spread on skin, mucous membranes, swelling with erosive lesions on lips and face and hyperpigmentation. Normally, SJS is a self-resolving condition but it has potential to be converted into life-threatening disease. Here, we describe and present a case series of SJS inflicted by rifampicin and allopurinol. First one is a 28-year-old-female and second case is a 50-year-old male, both received rifampicin for pulmonary tuberculosis. Third patient is a 22-year-old young male taken allopurinol for hyperuricemia. All these patients noticed a severe skin reaction which is a part of erythema multiforme spectrum. Causality assessment was done in these patients with the help of Naranjo’s algorithm and diagnosed as cases of SJS.

Drug-related Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review.

IntroductionStevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening, allergic reactions affecting the skin and mucous membranes. SJS is considered to be a milder form with less than 10% of body surface area (BSA) involvement. We report successful management of two cases of SJS and TEN. Firstly, a case of a 24-year-old female who presented with rashes over face, chest, and upper limbs after the oral intake of ciprofloxacin and local application of moxifloxacin eye drops. She developed high-grade fever and difficulty in breathing requiring intubation and lung-protective mechanical ventilation and was treated with high-dose methylprednisolone, azithromycin, soframycin skin dressings, and topical ocular antibiotics. Secondly, another case of a 16-year-old female who developed bullous eruptions over the trunk, arms, hands, face, and sole involving 60% of BSA, after oral intake of albendazole. She was diagnosed as TEN and successfully managed with sterile silver nitrate, soframycin dressings, and antibiotics.Key messageStevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening, allergic reactions affecting the skin and mucous membranes. Early identification, withdrawal of the suspected drug, and early transfer to a specialized center decrease mortality.How to cite this articleArora R, Pande RK, Panwar S, Gupta V. Drug-related Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review. Indian J Crit Care Med 2021;25(5):575–579.

Is there an association between Stevens-Johnson Syndrome and vaccination? A systematic review.

It is essential to make sure that vaccines are safe, effective, and of good quality. In the past years, there have been some reports of adverse effects regarding vaccination. One of these adverse effects is the development of Stevens-Johnson syndrome. Stevens-Johnson syndrome is a rare, severe, skin disorder, that usually occurs after medication. In Europe, its estimated incidence is of 2-3 cases/million population/year. Therefore, the aim of this study was to investigate, through a systematic review, the association between vaccination and the development of Stevens-Johnson syndrome. We performed a systematic review using PubMed, Scopus and Web of Science databases. We included studies dated between January 2000 and February 2018. The main selection criterion was the reporting of the disease, following vaccination. Ten studies were selected, from a total of 391 studies. Of these, 5 were case reports, 3 were cohort studies and 2 were case-control. All the studies were regarding cases of Stevens-Johnson syndrome after vaccination. The selected studies reported cases following vaccines such as influenza vaccine, smallpox, anthrax and tetanus vaccine, MMR vaccine, varicella vaccine, DTaP-IPV vaccine or rabies vaccine. None of the cohort studies reported statistically significant associations between vaccination and the syndrome. In the case-control studies, it was not observed significant increased risk for the Stevens-Johnson syndrome following the administration of vaccines. Regarding the case reports, there was not sufficient evidence to form a positive association between these two factors, and more studies are needed. In this review it was not possible to establish a positive relation between vaccination and the development of Stevens-Johnson syndrome.

Mechanisms of Severe Cutaneous Adverse Reactions and a New Treatment Strategy

Severe cutaneous adverse reactions (SCARs) are important in postmarketing drug safety because SCAR patients were highest in the adverse drug reaction relief system of Japan. The SCAR symptoms of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) include high fever, severe mucosal impairment, and epidermal necrosis-induced erosions and blisters. Approximately 600 cases of SJS and 300 cases of TEN are reported annually in Japan. Many suspected drugs such as acetaminophen, lamotrigine, allopurinol, and carbamazepine have been reported. Over the last 15 years, an association between human leukocyte antigen and SJS/TEN onset has been reported with several drugs. Pathophysiological examinations in those reports revealed marked CD8-positive T cell infiltration into epidermal lesions, and the presence of cytotoxic granulysin, soluble Fas ligand, and tumor necrosis factor (TNF)-α in blister fluid. Therefore, SJS and TEN are immunological disorders that lead to epidermal necrosis and are consequently treated with the systemic administration of corticosteroids and with high-dose intravenous immunoglobulin therapy and plasma exchange in severe cases. Additionally, because the epidermal necrosis has characteristics similar to those of organ rejection after transplantation, the administration of cyclosporine, an immunosuppressant that inhibits helper T cell activation, has been attempted. Further, the administration of the TNF-α inhibitor etanercept has also been reported. This review summarizes current knowledge on the mechanisms of onset of SJS/TEN and their treatments.

Radiation-induced dermatitis after administration of mogamulizumab for adult T-cell leukaemia/lymphoma: a multi-institutional retrospective study.

Cutaneous adverse reactions are frequently induced by mogamulizumab. Cases of Stevens-Johnson syndrome, toxic epidermal necrolysis and severe photosensitivity related to mogamulizumab have been reported. This study investigated whether severe radiation-induced dermatitis occurred in patients undergoing radiotherapy after the administration of mogamulizumab for adult T-cell leukaemia/lymphoma. We retrospectively reviewed 46 courses of radiotherapy administered to 15 consecutive patients with adult T-cell leukaemia/lymphoma (acute, n = 7; lymphoma, n = 7; smouldering, n = 1) who received mogamulizumab before or during radiotherapy at three institutions between 2012 and 2017. During 43 of the 46 radiotherapy courses, patients developed Grade ≤1 radiation-induced dermatitis. No patient developed Grade ≥3 radiation-induced dermatitis. No patient was prescribed ointments as prophylactic treatment for radiation-induced dermatitis. Development of radiation-induced dermatitis was not significantly associated with the number of days since the administration of mogamulizumab prior to radiotherapy (P = 0.85), frequency of administration of mogamulizumab before/during radiotherapy (P = 0.33), administration of mogamulizumab during radiotherapy (P = 0.41) or types of lesions in adult T-cell leukaemia/lymphoma cases (cutaneous vs. non-cutaneous, P = 0.74). Development of radiation-induced dermatitis was significantly related to the total cutaneous dose (mean, 31.9 Gy [95% confidence interval: 26.6-37.1 Gy] vs. 19.7 Gy [95% confidence interval: 16.2-23.2 Gy], P = 0.0004) and total prescribed dose (mean, 31.5 Gy [95% confidence interval: 26.2-36.8 Gy] vs. 18.5 Gy [95% confidence interval: 15.0-22.0 Gy], P = 0.0002). None of the 15 patients who received moderate-dose radiotherapy developed severe radiation-induced dermatitis during the 46 courses of radiotherapy after mogamulizumab administration.

Clinical features of Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but these conditions are associated with high mortality. There have been few reports of SJS and TEN in children. The aim of this study was to evaluate the clinical features and outcomes of SJS and TEN in a group of Japanese children. We retrospectively reviewed pediatric cases of SJS and TEN, from 2000 to 2015. We identified 12 pediatric cases of SJS and three of TEN. Six (all SJS) were caused by infection, and eight of the cases (SJS, n = 5; TEN, n = 3) were drug induced. Respiratory complications were the most common in terms of organ involvement, followed by hepatitis and gastrointestinal symptoms. Thirteen patients were treated with systemic corticosteroids, and two patients were treated with supportive therapy only. Concomitant with corticosteroid, four patients were given i.v. immunoglobulin. One patient with severe TEN was treated with systemic corticosteroids combined with plasmapheresis and cyclosporine. None of the present patients died. One patient with TEN had severe sequelae, with bronchiolitis obliterans and ocular involvement. SJS/TEN are rare, but are associated with severe complications. General pediatricians need to have up-to-date information regarding these conditions. The present study provides insights into the confirmation of the risk of SJS/TEN as well as the treatment of these diseases.

Corneal Ectasia in Stevens-Johnson Syndrome: A Sequela of Chronic Disease

To describe corneal ectasia in cases of chronic Stevens-Johnson syndrome (SJS). Prospective observational study. Setting: Institutional. Fifteen consecutive cases of chronic SJS. Best-corrected distance visual acuity (BCDVA), maximum corneal curvature (Kmax), anterior elevation, posterior elevation, thinnest pachymetry, and Sotozono severity score. Thirty eyes of 15 patients were included. Corneal tomography using Scheimpflug technology (Pentacam-HR, Oculus GmbH) was performed. Nine eyes were excluded owing to poor-quality scans. The median age was 26 years. The median time from onset of disease to assessment for corneal ectasia was 7 years. The median BCDVA was 0.8 logMAR units at presentation. The median Sotozono severity score was 11. Corneal ectasia (Kmax > 48 diopters [D]) was noted in 76.2% of eyes. The mean Kmax was 58.37 ± 14.89 D. On Belin/Ambrosio enhanced ectasia display the median front and back elevation was 42μm (10-176μm) and 267μm (15-2392μm), respectively. The mean pachymetry was 377.76 ± 165.05μm (133-448μm). The point of maximum ectasia was peripheral in 57.1%, both central and peripheral in19.1%, and central in 23.8% of eyes. On Spearmancorrelation analysis, deterioration in BCDVA (R= 0.759, P < .001) and increase in Kmax (R= 0.589, P= .005) was associated with higher disease severity (Sotozono grading). Corneal ectasia is a common but often missed entity in cases of chronic SJS that may be a cause for poor visual acuity in these cases. All cases of SJS must be evaluated for corneal ectasia, especially when the visual acuity is disproportionate to the disease severity.

Vaccine-induced toxic epidermal necrolysis: A case and systematic review

Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are cutaneous hypersensitivityreactions that develop in response to specific triggers such as medications and certain infections. Vaccines, which undergo rigorous safety testing prior to use in humans, are a rare cause of SJS/TEN and little is known about the frequency of such events and corresponding pathogenesis. Herein, we discuss a case of suspected TEN in a 19-year-old woman who received the meningococcal B vaccine (the first report of such an association) and conduct a systematic review of the associated literature. We also discuss management of this patient with a single dose of etanercept. Relevant literature was searched using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method. A total of 29 articles reporting EM, SJS, or TEN following vaccination were included from &gt;5 countries. Of the 29, 22 articles reported EM, 6/29 reported SJS, and 4/29 reported TEN (3 articlesreported cases of both EM and SJS/TEN). We suggest consideration of vaccines as an etiology for cases of SJS or TEN that begin with an EM-like presentation, and provide further evidence for the use of etanercept as a viable treatment for TEN.

Clinical Features and Treatment Outcomes among Children with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A 20-Year Study in a Tertiary Referral Hospital.

Aim To determine the probable causative factors, clinical features, and treatment outcomes of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap in children. Methods A 20-year database review of all children diagnosed with SJS/TEN/SJS-TEN overlap at the King Chulalongkorn Memorial Hospital, Thailand. Results 36 patients (M : F, 16 : 20) with the mean age of 9.2 ± 4.0 years were identified. There were 20 cases of SJS, 4 cases of SJS-TEN overlap, and 12 cases of TEN. Drugs were the leading cause for the diseases (72.3%); antiepileptics were the most common culprits (36.1%). Cutaneous morphology at presentation was morbilliform rash (83.3%), blister (38.9%), targetoid lesions (25.0%), and purpuric macules (2.8%). Oral mucosa (97.2%) and eye (83.3%) were the 2 most common mucosal involvements. Majority of the cases (77.8%) were treated with systemic corticosteroids, intravenous immunoglobulin, or both. Treatment outcomes between those who received systemic therapy and those who received only supportive care were comparable. Skin and eye were the principal sites of short-term and long-term complications. Conclusions SJS/TEN are not common but are serious diseases which lead to significant morbidities in children. Early withdrawal of suspicious causes and meticulous supportive care are very important. This study found that the systemic therapy was not superior to supportive care because the treatment outcomes for both groups were comparable.

Genitourinary involvement and management in children with Stevens–Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are devastating hypersensitivity disorders that cause epidermal cell death and can affect all epidermal surfaces, including the urethra, vagina, labial and scrotal skin. Despite the well-described ocular and orofacial manifestations of SJS/TEN, there is a paucity of reports on the genitourinary (GU) symptoms and their management. Specifically, consulting services often ask the pediatric urology team if it is safe to place a urethral catheter, but there is no data in the literature to help guide management. The present study sought to review all pediatric cases of SJS/TEN in a tertiary care hospital to determine the incidence and optimal management of GU manifestations, including the use of urethral catheters. With IRB approval, cases of SJS and TEN that were managed as an inpatient between January 2008 and June 2015 were retrospectively reviewed in order to identify the extent of GU involvement/manifestations, the treatment provided, use of urethral catheterization and long-term follow-up or complications. Thirty-one patients (15 female, 16 male; age range 2-18 years) presented with SJS or TEN over the study period. Etiologies for SJS/TEN included mycoplasma infection (48%) and medications (45%). Incidences of GU manifestations at presentation and their management are shown in Summary Table. Overall, 74% of patients had genital involvement of skin lesions. In 12 cases (39%), urology consultation was obtained. Twenty patients (61%) complained of dysuria and one child had gross hematuria in the setting of meatal lesion. Petroleum jelly was used in the majority of patients. A urethral catheter was placed in eight patients (25.8%, four female, four male) with a range of duration of 7-23 days. No patient developed hematuria or any other complications (i.e. strictures or urinary symptoms) after catheter removal. One boy required lysis of penile adhesions in the short-term. One of each gender developed penile and labial adhesions on long-term follow-up that self-resolved. GU involvement in SJS/TEN occurred in almost three-quarters of patients and was managed conservatively like other skin/mucosal manifestations. Long-term sequelae were rare and urethral catheterization appeared to be safe, without any short-term or long-term complications.

Effects of piroxicam on tissue distribution of sulfadimidine in West African Dwarf male and female goats.

Cases of Stevens-Johnson syndrome have been increasingly reported in Nigeria by individuals who consumed meat products of animals especially goats injected sulfonamides. Hence, tissue distribution and residues of intramuscular sulfadimidine were studied in West African Dwarf (WAD) goats. Twenty goats divided into two groups of 10 each (five males; five females) weighing 10.4 ± 1.63 kg were administered intramuscular sulfadimidine (100 mg/kg body weight), and the second group was coadministered 5 mg/kg of piroxicam via right and left thigh muscle, respectively. Samples of the liver, kidney, spleen, heart, lung, intestine, brain, and skeletal muscle were collected into sterile cellophane bags. Two untreated goats were killed and used for preparation of tissue standards. The tissue samples were stored frozen for analysis. High concentration of sulfadimidine residues was found in all the tissues of goats administered sulfadimidine as well as tissues of goats coadministered sulfadimidine/piroxicam for up to 30 days postdrug administration. Generally, residues of sulfadimidine were observed to be significantly higher than the acceptable limit (0.1 ppm). Hence, consumption of meats from WAD goats administered sulfadimidine may pose very high risk of Stevens-Johnson syndrome in sensitive humans. As such consumption of such meats should be avoided.

Drug-induced Stevens-Johnson syndrome in Indian population: A multicentric retrospective analysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening hypersensitivity reactions mainly caused by drugs. Data on incubation period, hospital stay, and outcome for HIV-positive patients are sparse. Role of corticosteroids in their management is still controversial. Indoor cases of SJS, SJS-TEN overlap, and TEN were analyzed for causative drugs, incubation period, a severity-of-illness score for toxic epidermal necrolysis (SCORTEN) score, HIV status, treatment, and outcome. Comparison of parameters between HIV and non-HIV cases was done. Utilization pattern of corticosteroids and their role in outcome were evaluated. Four SJS, 15 SJS-TEN overlap, and 21 TEN cases were evaluated. Antimicrobials (27.1%), antiviral (23%), antiseizure drugs (8.4%), and analgesics (8.4%) were commonly associated drugs. Among 12 (30%) HIV-reactive cases, nevirapine (97.6%) and cotrimoxazole (41.6%) were common causative drugs. Males (75%) were affected more than females (25%) among HIV-positive individuals. Incubation period was significantly higher in HIV-reactive patients. Total 30 (75%) patients were treated with corticosteroids. Dexamethasone (90%) and prednisolone (26.6%) were most commonly used. No significant difference was found among cases treated with or without corticosteroids. Antimicrobial drugs are common to cause SJS/TEN. Among HIV-reactive patients, male have more risk, incubation period is more and severity of reaction is less. Effectiveness of corticosteroids for treatment of SJS/TEN is inconclusive.