Score For Toxic Epidermal Necrolysis Research Articles

Clinico- Therapeutic Study of Stevens Johnson Syndrome-Toxic Epidermal Necrolysis and Prognostic Significance of SCORTEN in Indian Patients.

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening dermatological emergencies. SCORTEN (SCORe of toxic epidermal necrolysis) is a validated score to predict mortality; however, there is a paucity of data to determine its usefulness in the Indian population. To evaluate the accuracy of SCORTEN as a prognostic marker in SJS-TEN. A prospective observational study was conducted at a tertiary care hospital for two years. SCORTEN was calculated on days one and three of admission. The actual death rates were compared to the predicted rates as estimated by the SCORTEN by standardised mortality ratio analysis (SMR). Of 40 cases included in the study, the mean age was 36.2 ± 14 years (range 11-65) with the male: female ratio being 1.67:1. Antibiotics (37.5%) were the most common group followed by anticonvulsants (22.5%). Comorbidities were observed in 60% of cases, with epilepsy (17.5%) and HIV (human immunodeficiency virus) infection (12.5%) being common. On univariate analysis, heart rate > 120/min, epidermal detachment > 10% BSA, and Se HCO3 (bicarbonate) <20 mmol/L were associated significantly with the death of the subjects (P < 0.05). The observed mortalities were 4.34%, 0, 0 and 80% for SCORTEN 0-1 (3.2%), 2 (12.1%), 3 (35.8%) and 4 (58.3%) respectively when compared to expected mortality. SMR of SJS was 0.69 and of TEN was 1.49. SCORTEN gave an overestimation of mortality in patients with lower scores and an underestimation of mortality in patients with higher scores in our study. Minor refinements based on the study population may increase the predictive accuracy of the original scale.

Emerging Insights into Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Induced by Immune Checkpoint Inhibitor and Tumor-Targeted Therapy.

Anticancer drugs have revolutionized tumor therapy, with cutaneous toxicities such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) being common immune-related adverse events. The debate over the efficacy of systemic corticosteroids in treating these conditions persists, while tumor necrosis factor (TNF)-alpha inhibitors show promise. This study aims to evaluate the effectiveness and safety of combination therapy involving the TNF-α inhibitor adalimumab for SJS/TEN induced by anticancer drugs. A literature review of SJS/TEN cases induced by anticancer drugs from 1992 to 2023 was conducted, alongside an analysis of patients admitted to the First Affiliated Hospital of Fujian Medical University during the same period. Clinical characteristics, skin healing time, mortality, and adverse events were evaluated in two treatment groups: SJS/TEN patients treated with targeted anticancer therapies and immunotherapies. Among the 27 patients studied (18 with SJS or SJS-TEN overlapping and 9 with TEN), combination therapy with adalimumab significantly reduced mucocutaneous reepithelization time and healing duration compared to corticosteroid monotherapy. Patients receiving adalimumab combined with corticosteroids had lower actual mortality rates than those on corticosteroid monotherapy. The combination therapy also showed a trend towards reducing standardized mortality rates based on the Score of Toxic Epidermal Necrolysis (SCORTEN). The findings suggest that adalimumab in combination with corticosteroids provides significant clinical benefits and is safer than corticosteroids alone for treating SJS/TEN induced by targeted anticancer therapies and immunotherapies. This study contributes valuable insights into potential treatment strategies for severe cutaneous adverse reactions to anticancer drugs, highlighting the importance of exploring alternative therapies such as TNF-α inhibitors in managing these conditions effectively.

Outcomes in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Patients Treated With a Medicine-Led Multidisciplinary Approach.

Patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) have traditionally been treated in burn centers. Our burn center's approach differs by admitting these patients to a medicine service, with support from the burn team. The aim of this study was to determine whether SJS/TEN patients cared for with our system, with burn involvement but not burn admission, demonstrate equivalent outcomes. We conducted a retrospective review of all SJS/TEN patients admitted to the medicine service at a single academic medical center from 2009 to 2021. Outcome measures such as mortality, length of ICU stay, and total length of hospitalization were collected. The Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) was used to calculate expected mortality rates within the cohort. The observed mortality rates were then compared to the expected mortality rates. One hundred and twenty-six patients who were admitted for SJS/TEN were included (70 SJS, 40 SJS/TEN overlap, 16 TEN). The mortality rate for the entire cohort was 10.32% as compared to a 22.33% expected mortality rate (P = .010). The observed and expected mortality rates for SJS, SJS/TEN overlap, and TEN subgroups were 1.43% observed versus 10.22% expected (P = .029), 20.00% observed versus 35.83% expected (P = .133), and 25.00% observed version 44.06% expected (P = .264), respectively. Mortality rates in SJS/TEN patients admitted to medicine units are equivalent or decreased as compared to SCORTEN-predicted mortality rates. Admission of SJS/TEN patients to a medicine unit is appropriate providing there is burn team involvement in their care.

002 National clinical audit on the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults

Abstract UK guidelines for the management of Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) in adults, published by the British Association of Dermatologists (BAD) in 2016, outline a set of audit standards (Creamer D, Walsh SA, Dziewulski P et al. U.K. guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol 2016; 174:1194–227). Our aim was to audit the current management of SJS/TEN in adults against audit standards in the BAD guidelines. BAD members were invited to submit data on five consecutive adults with SJS/TEN per department over an 8-week period in 2022. All data were obtained from respondents using a Microsoft Excel spreadsheet proforma returned to the BAD by email. No patient-identifiable data were requested. Initial data extraction was conducted using a novel and reproducible program written in the R statistical language (V3.7 and the readxl, ggplot2 and ggthemes packages). The data analysis was also performed using Microsoft Excel. Thirty-nine (29%) dermatology centres in the UK participated and data for 147 adults with SJS/TEN were collected. Within 24 h of a diagnosis being made or suspected, the following were documented: Severity-of-Illness Score for TEN (SCORTEN) for 52% (n = 76/147) of submitted cases, list of medications for 77% (n = 113) and timelines for commencement/alterations of medications for 71% (n = 104). Initial assessment was of the eyes (by an ophthalmologist) for 48% (n = 71) of cases, the mouth in 88% (n = 130), the genital skin in 70% (n = 103) and the urinary tract in 63% (n = 93). In the first 10 days after a suspected or confirmed diagnosis of SJS/TEN, daily assessments of the mouth were documented in 18% (n = 26) of cases, the eyes in 8% (n = 12) and the urinary tract and genital skin in 10% (n = 14). Documentation regarding advice on (i) avoidance of the culprit drug was present for 58% (n = 76/130) of cases and (ii) request for a medic alert bracelet/amulet in 6% (n = 9/147). In adults with SJS/TEN, drug causality assessment was documented adequately. Initial assessment of the mouth, eyes, urinary tract and genital skin was good to fair; however, daily assessments were poor. Other audit standards that require improving include documentation of SCORTEN and advice to avoid the culprit drug and request a medic alert bracelet/amulet. In adults with SJS/TEN, there are multiple points in the assessment pathway that require careful documentation and regular review. These are a challenge for many clinical inpatient environments. Care within a burns unit or similar may not be possible for all. This audit suggests that some form of assessment and clinical review checklist might enable colleagues to maintain standards outlined in the guideline. Among these, use of SCORTEN, advice to avoid the culprit drug and requests for a Medic Alert bracelet/amulet are key.

294 Outcomes in Stevens-johnson Syndrome/toxic Epidermal Necroylsis Patients Treated Without Admission to a Verified Burn Center

Abstract Introduction The care of patients with Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necroylsis (TEN) has traditionally been delivered within a burn center. Our burn center utilizes a multi-disciplinary approach which involves admission to the medicine service, with support from the burn team. The aim of this study is to determine whether SJS/TEN patients cared for with our system, including burn involvement but not burn admission, demonstrate equivalent outcomes. Methods A retrospective review of all SJS/TEN patients admitted to the medicine service at a single academic medical center from 2009 to 2021 was conducted. Demographic characteristics and outcome measures such as mortality, length of intensive care unit (ICU) stay and total length of hospitalization were collected. The Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) was used to calculate expected mortality rates within the cohort [2]. The observed mortality rates were then compared to the expected mortality rates. Results 126 patients who were admitted for SJS/TEN were included (70 SJS, 40 SJS/TEN overlap, 16 TEN). The mortality rate for the entire cohort was 10.32% as compared to a 22.33% expected mortality rate (p=0.010). The observed and expected mortality rates for SJS, SJS/TEN overlap, and TEN sub-groups were 1.43% observed versus 10.22% expected (p=0.029), 20.00% observed versus 35.83% expected (p=0.133), and 25.00% observed version 44.06% expected (p=0.264) respectively. The mean ICU length of stay was 3.58 days, and the mean length of total hospitalization was 11.69 days. Conclusions Mortality rates in SJS/TEN patients admitted to medicine units are equivalent or decreased as compared to SCORTEN predicted mortality rates. Applicability of Research to Practice Admission of SJS/TEN patients to a medicine unit is appropriate as long as there is burn team involvement in their care, which may be more suitable for patients who require management of complex medical comorbidities.

APOA4 as a novel predictor of prognosis in Stevens-Johnson syndrome/toxic epidermal necrolysis: A proteomics analysis from two prospective cohorts

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening adverse drug reactions. Conventional systemic therapies are of limited efficacy and often exhibit strong side effects. To assess the efficacy and safety of the combination treatment with a tumor necrosis factor-α antagonist adalimumab and delineate the underlying mechanisms. We evaluated the efficacy and safety of the combination therapy with adalimumab by comparing 2 treatment cohorts of SJS/TEN patients. Patient plasma samples were collected for proteomics analysis. The combination therapy with adalimumab significantly shortened the time to mucocutaneous re-epithelization and healing, with reduced side effects caused by corticosteroids. Plasma proteomic profiling showed that apolipoprotein A-IV (APOA4) was one of the most significant differentially expressed proteins. Multivariate regression analysis revealed that APOA4 level was significantly associated with prognosis parameter of SJS/TEN (P=.004), but not with disease severity score (severity-of-illness score for toxic epidermal necrolysis [SCORTEN]) (P=.118). Thus further research will be helpful to effectively incorporate APOA4 into current SCORTEN-driven protocols. The cohort size is relatively small. Both cohorts had low overall SCORTEN scores. Adalimumab in combination with corticosteroids demonstrates significant clinical benefits over corticosteroids alone in SJS/TEN patients. Moreover, APOA4 may serve as a novel prognostic marker of SJS/TEN.

Clinicoetiological Study of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Spectrum and the Correlation of SCORTEN with Prognosis.

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe cutaneous adverse reactions of major concern because of its high mortality. The prognosis of SJS and TEN is widely assessed with SCORTEN (SCORe of TEN). Although, it is a largely useful scale, the predictive ability is still variable. This study was conducted to assess the clinicoetiological profile and outcome of SJS and TEN and to evaluate the validity of SCORTEN in assessing the prognosis in South Indian population. This prospective observational study was conducted in the Department of Dermatology, Venereology and Leprosy in a Tertiary care hospital from January 2016 to June 2017. Detailed history, examination findings, treatment and SCORTEN scores were recorded. SCORTEN's accuracy in predicting the mortality was assessed on day 1, 3 and 5 of admission. The incidence of SJS/TEN among other drug reactions was 29.5%. The most common age group affected was 30-49years (41.1%), with male preponderance (76.5%). The age range of patients was 6 and 67 years. TEN (64.7%) was the predominant spectrum followed by SJS and SJS-TEN overlap in 17.6% each. Anticonvulsants (47%) were the commonest causative drug, followed by analgesics (35%) and antibiotics (11%). The validity of SCORTEN was the same on days 1, 3, and 5. There was good agreement between the actual and predicted mortality on all three days. A mortality of 17.6% (3 cases) was recorded in this study. Three patients (17.6%) died in our study. All survivors had a score of 4 or less. The predicted mortalities were 0.417, 1.836, and 2.574 and the observed mortalities were 0, 2, and 1 in SJS, SJS-TEN overlap, and TEN respectively. Analysis of SCORTEN on a single day, either day 1, 3, or 5 was found to be as useful as the serial analysis. SCORTEN gave a significant estimation of mortality in SJS-TEN overlap patients, whereas it overestimated mortality in TEN patients. An increase in individual scores for the elevation of blood urea nitrogen (BUN) in existing SCORTEN and the inclusion of new parameters like raised liver enzymes, thrombocytopenia, and pulmonary infiltrates aided in proposing a modified SCORTEN for the South Indian population. Further studies on a larger scale, are needed to validate the modified SCORTEN proposed by us.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Fresh Look at an Old Foe.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are some of the less common cutaneous adverse drug reactions with significant mortality. This study was undertaken with the objective of studying the demographics and clinical profile of SJS/TEN and identifying parameters associated with mortality. All patients with SJS/TEN over 10 years (2010-2020) were included in the study. Data obtained from in-patient and out-patient records were analysed. A total of 82 patients with SJS/TEN were admitted to our centre over a period of 10 years. Patients with SJS were significantly younger than those with TEN, with a male: female ratio >1 in SJS and <1 in TEN. The most commonly implicated drugs were antiepileptics (n = 29, 35.4%), antibiotics (n = 20, 24.4%). and Non-steroidal antiinflammatory drugs (NSAIDs) (n = 7, 8.5%). The mortality rate in the TEN group was 16% (n = 8). Certain factors such as cutaneous lesions preceding mucosal lesions at onset, high mean Body surface area (BSA) of denudation and a transfer to intensive care unit (ICU) more than 7 days after admission were significantly associated with higher mortality. There was no difference between survivors and deaths in terms of delay in hospitalisation, total disease duration, implicated drug, delay in initiation of therapy, the onset of re-epithelialisation, Severity-of-illness score for TEN (SCORTEN) and total duration of hospital stay. Factors significantly associated with increased mortality in TEN were cutaneous onset of lesions, mean BSA of involvement and transfer to the intensive care unit (ICU) beyond day 7 of admission.

Serum interleukin-21 level in patients with severe adverse cutaneous drug reactions and correlation with disease severity

IntroductionInterleukin-21 (IL-21) is accepted to play a pathogenic part in development of unfavorable cutaneous medicate responses. Adverse cutaneous drug reactions create a wide run of clinical signs such as pruritus, maculopapular ejections, urticaria, angioedema, fixed drug eruption, erythema multiforme, vesiculobullous responses (e.g. Stevens–Johnson syndrome and toxic epidermal necrolysis), and exfoliative dermatitis.Patients and methodsTwenty patients (drug-eruption group) and 14 healthy controls (control group) were recruited from Assiut University Hospitals' Dermatology Department and Outpatient Clinic. Data were collected in the period from October 2017 to December 2018. Patients were assessed clinically by percentage of surface area of the body involvement (body surface area %) and score of toxic epidermal necrolysis. At presentation and after 1 month of treatment, blood samples were taken to evaluate serum IL-21 levels using an enzyme-linked immunosorbent assay.ResultsInside the study, the cruel serum IL-21 level was considerably greater than in the control group, in drug-eruption group was 575.58 ± 94.67 ng/dl, and in the control group was 128.00 ± 73.94 ng/dl with P value of 0.000. The drug-eruption group had a significantly higher serum level of IL-21 before treatment than after treatment with P value of 0.000. Additionally, there was a critical relationship between IL-21 levels in the blood and the severity of the condition. Before and after therapy, there was a significant positive association between blood IL-21 levels and the proportion of body surface area involvement.ConclusionIL-21 levels in the blood were significantly higher in EM and SJS/TEN patients, suggesting that it may have a role in the etiology of EM and SJS/TEN and it could be employed as a marker for the severity of SJS/TEN and patient prognosis in the future.

A Literature Review on Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis and its Complications

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are delayed-type hypersensitivity reactions to medications. Early signs of identification and proper management are critical for patients’ survival. The distinct features initially resemble influenza-like conditions, malaise, and fever with gradual pain and lesions in the oral, ocular, and genital areas along with other systemic symptoms. SJS/TEN can be differentiated: SJS by the degree of skin involvement and TEN by the degree of exfoliation. In SJS, skin involvement is &lt; 10%, whereas in TEN, skin involvement is &gt; 30%; overlapping among the two develops with 10–30% skin involvement. For the identification of culprit drugs, oral provocation tests, patch testing, and in vitro assay tests are used. The estimation of mortality rates for SJS/TEN has been performed by using SCORTEN- severity-of-illness score for toxic epidermal necrolysis. Sulfonamides, anti-epileptics, NSAIDs (oxicams), and allopurinol are some of the medications with a high risk of SJS/TEN development. Drug-mediated SJS/TEN is facilitated by genetic involvement particularly HLA antigens along with CYP450 genetic variants. Most frequent clinical features include mucosal involvement of nasopharyngeal, esophageal, and genital regions accompanying blisters and erosions. A multidisciplinary approach is required for the handling and treatment of SJS/TEN. It is mandatory to withdraw the contributing medicine followed by supportive therapy. Clinical management involves the use of corticosteroids, cyclosporine, and immunoglobulins.

Clinical value of the prognostic nutritional index and red blood cell distribution width-to-albumin ratio for the prediction of severity of and mortality associated with Stevens-Johnson syndrome/toxic epidermal necrolysis.

The prognostic nutritional index (PNI) and red blood cell distribution width-to-albumin ratio (RAR) are considered to be related to the prognosis of disease severity. However, the role of these biomarkers in predicting Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) severity and mortality is unclear. The aim of the current study was to investigate the association of PNI and RAR with severity and mortality in individuals with SJS/TEN. Clinical data were retrospectively collected from 74 individuals with SJS/TEN and 74 healthy individuals, who were matched for age and sex during the same period. PNI, RAR, and other indicators were compared between individuals with SJS/TEN and healthy controls. The association of PNI and RAR with SJS/TEN severity was assessed using Spearman or Pearson correlation analyses. Individuals with SJS/TEN were categorized into two groups, either survivors or nonsurvivors. The correlation between PNI, RAR, and SJS/TEN mortality was analyzed using univariate and multivariate logistic regression. The predictive value of the previously mentioned indicators on the mortality of patients with SJS/TEN was assessed using receiver operating characteristic curve analysis. The RAR level of patients with SJS/TEN was greater than that of the control group (p < 0.05), whereas PNI was lower. In compliance with correlation analysis, RAR was positively correlated with SCORTEN (Score of Toxic Epidermal Necrolysis) and ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area) (p < 0.05), and PNI was negatively correlated (p < 0.05). RAR is a risk factor for death in patients with SJS/TEN, but an elevated PNI level is a protective factor for mortality. The best cutoff values of PNI and RAR for predicting death in patients with SJS/TEN were 31.375 (sensitivity, 84.7%; specificity, 80%) and 0.486 (sensitivity, 73.3%; specificity, 84.7%). These results underscore the potential clinical value of PNI and RAR as appropriate and meaningful biomarkers to assess the severity of SJS/TEN and the mortality associated with it.

Disease severity and status in Stevens-Johnson syndrome and toxic epidermal necrolysis: Key knowledge gaps and research needs.

Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are on a spectrum of cutaneous drug reactions characterized by pan-epidermal necrosis with SJS affecting < 10% of body surface area (BSA), TEN > 30%, and SJS/TEN overlap between 10 and 30%. Severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is a validated tool to predict mortality rates based on age, heart rate, BSA, malignancy and serum urea, bicarbonate, and glucose. Despite improved understanding, SJS/TEN mortality remains constant and therapeutic interventions are not universally accepted for a number of reasons, including rarity of SJS/TEN; inconsistent definition of cases, disease severity, and endpoints in studies; low efficacy of interventions; and variations in treatment protocols. Apart from mortality, none of the other endpoints used to evaluate interventions, including duration of hospitalization, is sufficiently standardized to be reproducible across cases and treatment centers. Some of the gaps in SJS/TEN research can be narrowed through international collaboration to harmonize research endpoints. A case is made for an urgent international collaborative effort to develop consensus on definitions of endpoints such as disease status, progression, cessation, and complete re-epithelialization in interventional studies. The deficiencies of using BSA as the sole determinant of SJS/TEN severity, excluding internal organ involvement and extension of skin necrosis beyond the epidermis, are discussed and the role these factors play on time to healing and mortality beyond the acute stage is highlighted. The potential role of artificial intelligence, biomarkers, and PET/CT scan with radiolabeled glucose as markers of disease status, activity, and therapeutic response is also discussed.

Modified Score of Toxic Epidermal Necrolysis to predict toxic epidermal necrolysis mortality

To the Editor: Score of Toxic Epidermal Necrolysis (SCORTEN) is used to evaluate mortality risk among toxic epidermal necrolysis (TEN) spectrum diseases. Some studies mention the need to enhance the efficacy of SCORTEN.1,2 This study aimed to evaluate the epidemiologic features of patients with TEN, characterize the validity and accuracy of SCORTEN, and improve the current scoring system in predicting mortality. Clinical data of 51 patients with TEN (>30% Total body surface area [TBSA]) who were admitted to a referral burn center were retrospectively extracted (2010-2020).

Characteristics associated with disease prevalence, SCORTEN, length of stay, and mortality in hospitalized SJS/TEN patients: A single-center, eleven-year experience

Stevens-Johnson syndrome (SJS), toxic-epidermal necrolysis (TEN), and SJS-TEN overlap syndrome form a continuum of rare, life-threatening disease that often warrants immediate and intensive treatment in a burn center. Our objective in this study was to determine factors associated with the prevalence, severity, and mortality in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) admitted to our center. We performed a retrospective analysis of 192 patients admitted to our burn center from 1 January 2009 to 31 December 2019 for SJS/TEN. Severity-of-illness Score for TEN (SCORTEN), length of stay, and inpatient mortality were analyzed. Of 192 patients, 104 (54%) were Black, 117 (61%) were female, and 39 (20%) had a pre-existing psychiatric illness. Overall, 43 (22%) patients died. Female patients had significantly longer lengths of stay, with a median length of stay of 21 days versus 11 days for males (P =.001). Among female patients, 31/117 (26%) died compared to 12/75 (16%) of male patients (P =.056). Females tended to have a higher SCORTEN, but the difference was not statistically significant (P =.061). Hypertension, chronic obstructive pulmonary disease, and diabetes mellitus were all associated with mortality (P <.001). Black and female patients each represented a seemingly disproportionate percentage of our patients and females had longer lengths of stay. Future studies looking at sex- and race-based differences are needed to better understand these potential disparities.

Evaluation of Combination Therapy With Etanercept and Systemic Corticosteroids for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Observational Study

Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) are fatal severe cutaneous adverse reactions, without consensus on the medical treatment. The use of systemic corticosteroids or intravenous immunoglobulin (IVIG) remains debatable. Tumor necrosis factor-alpha inhibitors are potentially effective. To evaluate the effectiveness and safety of combination therapy using etanercept combined with corticosteroids or IVIG combined with corticosteroids versus corticosteroid monotherapy for patients with SJS-TEN. We retrospectively enrolled SJS-TEN patients from Taiwan and the Chinese mainland, during 2014 to 2019. Patients enrolled were treated with corticosteroid monotherapy, or combinations with IVIG or etanercept. We analyzed the clinical characteristics, skin healing time, mortality, and adverse events among these treatment groups. Among the 242 patients (187 with SJS or SJS-TEN overlapping and 55 with TEN), patients who received combination therapy with etanercept and corticosteroids had lower actual mortality than those with corticosteroid monotherapy and those with IVIG combined with corticosteroids, respectively (0% vs 6.63% and 4.76%). There was a tendency of reducing standardized (observed/predicted) mortality rate (SMR) based on the Score of Toxic Epidermal Necrolysis in etanercept combined with corticosteroids compared with corticosteroid monotherapy and IVIG combined with corticosteroids therapy (SMR [95% CI] 0 [1.80-3.59], 0.71 [0.83-2.64], 0.30 [0.68-6.22]; P= .006). Etanercept combined with corticosteroids showed a reduced skin healing time (12.0 [8.5-14.0], median days [interquartile range]), compared with corticosteroid monotherapy (13.0 [10.0-18.0]) and IVIG combined with corticosteroids therapy (13.5 [10.0-19.5]); P= .004 and P= .012, respectively). Etanercept combined with corticosteroids also showed a lower incidence of adverse event with gastrointestinal hemorrhage than corticosteroid monotherapy, especially in patients with TEN (P= .001). The tumor necrosis factor-alpha inhibitors and corticosteroids combination therapy was effective and safer than corticosteroid monotherapy for SJS-TEN, and may be considered as an alternative therapy for SJS-TEN patients who responded poorly to conventional corticosteroid therapy.

Retrospective analysis of cases with Stevens-Johnson syndrome/toxic epidermal necrolysis: A case series of 20 patients

Background: Stevens-Johnson syndrome (SJS)/ toxic epidermal necrolysis (TEN) are rare, acute, severe cutaneous hypersensitivity reactions usually triggered by medications. They are classified by the extent of the detached skin surface area. Objective: We aimed to retrospectively evaluate the sociodemographic, clinical, therapeutic, and prognostic characteristics of SJS/TEN cases diagnosed between January 2015 and December 2020 in our centre. Materials and Methods: All the data regarding patient characteristics were obtained retrospectively. The SCORe of Toxic Epidermal Necrolysis (SCORTEN) was used to predict disease severity and mortality rates. Results: Out of 20 patients (14 females, 6 males), eight (40%) were evaluated as TEN, three (15%) as SJS/ TEN overlap, and nine (45%) as SJS. The mean age was 39.2 ± 27.92 years. A higher frequency of systemic antibiotic use was found in cases of SJS/TEN overlap or TEN compared to SJS cases during patients’ follow-up after the diagnosis (P = 0.006). The most common responsible drug was allopurinol (25%). While the estimated mortality in patients with SCORTEN values of 4 and 5 was 58.3% and 90.0%, the mortality observed in our cohort was 50% and 100%, respectively. In terms of complications, ocular problems were the most common ones. Ophthalmic sequelae were observed in 15 patients during the follow-up period, the most common belonging to the cornea. Conclusion: In conclusion, early diagnosis, immediate discontinuation of suspected drugs, and good clinical care are among the most crucial treatment steps in the treatment of SJS/TEN. In addition, multidisciplinary management of the disease is vital in preventing the development of long-term sequelae in survivors.

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap following sulfadoxine-pyrimethamine overdose: a case report

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap is a rare but severe delayed-type hypersensitivity reaction that presents with widespread blistering, ulceration and necrosis of the skin and mucosa in the genetically predisposed and leads to significant morbidity and mortality. Sulfadoxine-pyrimethamine has been reported to cause a spectrum of drug hypersensitivity reaction (Stevens-Johnson syndrome, toxic epidermal necrolysis or Stevens-Johnson syndrome/toxic epidermal necrolysis overlap), but in rare occasions, hence this report. We report a 5-year-old boy who presented with fever and extensive mucocutaneous blistering and ulcerations 10 days following the ingestion of an overdose of sulfadoxine-pyrimethamine administered by a community health extension worker. Skin involvement was about 19%. The Naranjo Adverse Drug Reaction Probability Scale score of 6 was assigned (implying probable association between the ingested drug and the hypersensitivity reaction). The severity-of-illness score of toxic epidermal necrolysis of 1 was also assigned to this patient (implying low severity). He was managed conservatively with fluid, antibiotics, nutritional support and wound care measures. He recovered completely and satisfactorily. The patient's sister and father had reacted similarly but less severely when exposed to sulfadoxine-pyrimethamine. Health workers should pay close attention to every patient's family drug allergy history before administering any medications. Also, basic pharmacogenetic testing should be introduced into health facilities, especially for drugs known to cause serious adverse drug reactions. Furthermore, health workers should keep abreast with updates on treatment guidelines and also dutifully report all cases of adverse drug reactions to relevant bodies.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: 11-year Demographic Clinical and Prognostic Characteristics.

Background:Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening, severe drug reactions. Randomized studies on these diseases are difficult to perform.Aims and Objectives:The purpose of this study was to summarize the demographic and clinical characteristics of patients with SJS and TEN in a tertiary hospital in Turkey.Materials and Methods:We evaluated the records of 33 patients with SJS and TEN who were followed in our clinic or examined between January 2008 and June 2019, retrospectively. Age, sex, time of admission to hospital, causative drug, presence of concomitant disease, skin findings, mucosal involvement, the severity-of-illness score for TEN, the medication used, antibiotic use, transfer to intensive care, development of complications, and death or discharge status were noted.Results:Of the 33 patients, 11 (33.3%) had SJS, 3 (9.1%) had SJS/TEN overlap, and 19 (57.6%) had TEN. The majority (60.6%) of the patients were female. Nineteen (57.6%) patients had one, and 13 (39.4%) had more than one suspected drug exposure in their history. The most commonly suspected drugs were antibiotics. Twelve (36.4%) patients had intensive care unit hospitalization. Ten (30.3%) patients died.Conclusion:The demographic data of our study were consistent with the literature. Similar to the literature, antibiotics were the most common reaction-causing drugs. However, antiepileptic drugs, which were more frequently reported in other studies, were identified as suspicious in only one patient. We believe that our study will contribute to the determination of characteristics of this rare disease with real-life data.

Improvement of Mortality Prognostication in Patients With Epidermal Necrolysis

Epidermal necrolysis is a severe cutaneous adverse reaction in which severe systemic inflammation results in extensive epithelial keratinocyte necrosis. The most commonly used prognostic score in epidermal necrolysis, the Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN), was recently found to overestimate mortality in contemporary cohorts. Identification of independent prognostic markers may help to stratify risk more accurately. This study evaluates the association between novel inflammatory markers and in-hospital mortality in patients with epidermal necrolysis to study the incremental prognostic value of these markers in combination with SCORTEN. A retrospective cohort study was conducted over a 17-year period from 2003 to 2019. Patients were enrolled from Singapore General Hospital, the national referral center for epidermal necrolysis. A total of 196 patients with epidermal necrolysis were recruited, 4 (2%) of whom were excluded owing to incomplete data. The main outcome assessed was the in-hospital mortality rate. Discrimination and calibration of risk scores were assessed using the area under the receiver operating characteristic curve (AUC) and calibration plot, respectively. Evaluation of the incremental prognostic value of these markers was done by comparing the AUC between the old and new risk score, and the use of net reclassification improvement (NRI) and integrated discrimination improvement (IDI). Among 192 total patients (median [IQR] age 56 [42-70] years; 114 [59.4%] women), there were 43 (22.4%) who did not survive to discharge. Of the novel inflammatory markers, only red cell distribution width to hemoglobin ratio was significant in predicting in-hospital mortality (odds ratio [OR] 3.55; 95% CI, 1.76-7.16; P < .001) after adjusting for SCORTEN. The RDW/Hb as applied in 4 risk groups showed similar discrimination to SCORTEN (AUC [95% CI]: RDW/Hb in 4 groups, 0.76 [0.69-0.84], vs SCORTEN, 0.78 [0.70-0.85], P = .89). When RDW/Hb was added to SCORTEN, the composite score Re-SCORTEN showed significantly better discrimination than SCORTEN alone (AUC [95% CI]: Re-SCORTEN, 0.83 [0.77-0.89], vs SCORTEN, 0.78 [0.70-0.85], P = .02). The overall NRI was 0.94 (95% CI, 0.68-1.20), P < .001. The IDI was 0.06 (95% CI 0.03-0.08), P < .001. Re-SCORTEN showed good calibration based on the calibration plot. In this cohort of patients, RDW/Hb, an inexpensive and readily available marker, showed similar predictive accuracy with SCORTEN. Furthermore, when used in combination with SCORTEN, it also helped augment prognostic ability.

Comparing the Accuracy of ABCD-10 and SCORTEN in Predicting the In-Hospital Mortality of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multi-Institutional Study from Central China

Background: The newly described ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area [BSA]) is a 5-item mortality prediction model for patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN). It was developed in the United States, has at present been externally tested only in the United States, Spain, and Singapore, and remains to be validated in resource-restricted settings. We sought to compare the accuracy of ABCD-10 and Score of Toxic Epidermal Necrolysis (SCORTEN) in predicting in-hospital mortality in a cohort from central China. Due to disease progression affecting the accuracy of the prediction model during hospitalization, for example, higher predictive accuracy of SCORTEN based on parameters collected on day 3 of hospitalization, we also assessed the overall predictive value of ABCD-10 on days 1 and 3, respectively. Methods: A retrospective study was performed over a 10-year period (2010–2020) from 3 medical institutions in Wuhan. The performance of predictive models was assessed by both discrimination and calibration. Receiver-operating characteristic (ROC) curves, Hosmer-Lemeshow goodness-of-fit tests and calibration plots were used to evaluate the model discrimination and calibration. Results: Of 84 included patients, 11 (13.1%) did not survive. The discrimination power of ABCD-10 was not significantly different from that of SCORTEN (area under the curve: day 1, p > 0.05; day 3, p > 0.05). Although the calibration of ABCD-10 was good, it was inferior to SCORTEN as it underestimated total mortality (Hosmer-Lemeshow goodness-of-fit test: day 1, p = 0.17 vs. p = 0.63; day 3, p = 0.35 vs. p = 0.93). Besides, the performance of ABCD-10 was slightly better on day 3 relative to day 1. During hospitalization, bacteremia developed in 21 (25.0%) patients, which was associated with a higher risk of death in our cohort (odds ratio, 22.88; 95% CI, 4.38–119.40; p < 0.001). Conclusion: ABCD-10 showed acceptable overall performance, but revealed mortality underestimation and was inferior to the performance of SCORTEN. In consistence with SCORTEN, ABCD-10 was a better model when using values collected at day 3 of hospitalization relative to day 1.