Score For Toxic Epidermal Necrolysis Research Articles

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.

Bacteremia in Stevens Johnson Syndrome and Toxic Epidermal Necrolysis: Main Pathogens and Risk Factors: A Mini Review

Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are hypersensitivity reaction, mainly to drugs, characterized by skin detachment. They are dermatological emergencies, and bacteremia is the main cause death, especially in patients with extensive cutaneous involvement. We reviewed and summarized the large retrospective studies that focused on the risk factors and main pathogens involved in patients with SJS and TEN who developed bacteremia. Our results showed that the risk factors include a Total Body Surface Area (TBSA) higher than 10%, higher Severity-of-Illness Score For Toxic Epidermal Necrolysis (SCORTEN), hypertension, previous opiate use, White Blood Cells (WBC) &gt; 10000/mL; C-Reactive Protein (CRP) &gt; 100mg/mL; procalcitonin (PCT) ≥ 1μg/L; and skin colonization with P. aeruginosa, S. aureus, and methicillin-resistant S. aureus (MRSA). The most frequently isolated pathogens from blood cultures were S. aureus, E. faecalis, P. aeruginosa, Enterobacter spp, and A. baumannii. The identification and consideration of these variables on each patient with SJS and TEN could result in an earlier diagnosis, proper management and even prevention of bacteremia in this population.

Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy. To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. A retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. Risk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. Of 212 patients (134 of 211 [63.7%] women; mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (≥30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49; 95% CI, 1.21-5.12; P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30; 95% CI, 3.82-27.78; P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44; 95% CI, 1.12-10.52; P = .03) and an increased risk of infections (adjusted OR, 7.16; 95% CI, 1.52-33.74; P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40; 95% CI, 0.18-0.88; P = .02). No significant difference in 6-week mortality was found between treatment groups. This cohort study noted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.

Analysis of the clinical features and prognostic influencing factors of toxic epidermal necrolysis

Objective: To investigate the clinical features and prognostic influencing factors of toxic epidermal necrolysis (TEN). Methods: A retrospective observational study was conducted. From January 2008 to March 2019, a total of 46 TEN patients who met the inclusion criteria were admitted to Guangdong Provincial People's Hospital. The gender, age, and admission diagnosis of the 46 patients, the category of department of patients complicated with sepsis, death ratio of the sepsis patients with or without treatment history in intensive care unit (ICU)/department of burns and wound repair, and the cause of death of the deceased patients were recorded. Depending on whether complicated with sepsis, the patients were divided into sepsis group (32 cases) and non-sepsis group (14 cases). According to whether died or not, the patients were divided into death group (9 cases) and survival group (37 cases). The specific conditions of suspected pathogenic agents and combined basic diseases, the transaminase/bilirubin abnormality and creatinine and platelet count levels in blood on admission, and the detection of pathogenic microorganisms and drug resistance during the course of disease of patients were recorded in both sepsis group and non-sepsis group. The gender, age, lesion area, severity of illness score for TEN (SCORTEN) system score, combined basic diseases, blood microbial culture positivity, hormone use, and gamma globulin use of patients between sepsis group and non-sepsis group, death group and survival group were compared respectively. Data were statistically analyzed with chi-square test, Fisher's exact probability test, and Mann-Whitney U test. The factors with statistical significant differences between sepsis group and non-sepsis group, death group and survival group were selected for binary multivariate logistic regression analysis, so as to screen the independent risk factors affecting sepsis and death in TEN patients. Results: Of the 46 TEN patients, 30 were male and 16 were female, aged from 8 months to 92 years, with 11 cases (23.91%) of epidermolysis bullosa, 9 cases (19.57%) of exanthemative dermatitis, 9 cases (19.57%) of TEN, 7 cases (15.22%) of epidermolysis bullosa, 6 cases (13.04%) of Stevenss-Johnson syndrome, and 4 cases (8.70%) of severe drug rash for admission diagnosis. The patients complicated with sepsis were admitted to 11 departments, and the death ratio of patients with treatment history in ICU/department of burns and wound repair was similar to that of patients without such department treatment history (P>0.05). All the dead patients were complicated with sepsis, and the main cause of death was sepsis. On admission, the suspected pathogenic agents of patients in sepsis group were mainly allopurinol (11 cases) and non-steroidal anti-inflammatory drugs (4 cases), while those in non-sepsis group were allopurinol (3 cases) and psychotropic drugs (3 cases). Patients in sepsis group combined as many as 10 kinds of basic diseases, while those in non-sepsis group combined only 4 kinds of basic diseases. The proportions of patients with increased creatinine (χ2=13.349, P 0.05). A wide variety of pathogens were detected in the blood, respiratory tract secretions, and skin secretions of 21 patients in sepsis group, and 14 patients were infected with drug-resistant bacteria; among the 9 strains cultured from the blood samples, 8 were drug-resistant bacteria and 6 were Gram-positive bacteria. In non-sepsis group, pathogens were detected in blood, respiratory tract secretions, and skin secretions of 8 patients, with fewer species, and 6 patients were infected with drug-resistant bacteria. The gender, age, lesion area, blood microbial culture positivity, hormone use, and gamma globulin use of patients in sepsis group were similar to those of non-sepsis group (P>0.05). The proportion of patients combined with basic diseases and SCORTEN system score of patients in sepsis group were significantly higher than those in non-sepsis group (χ2=4.493, 7.222, P 0.05). The age and SCORTEN system score of patients in death group were significantly higher than those in survival group (χ2=4.412, 11.627, P<0.05 or P<0.01). The SCORTEN system score was an independent risk factor affecting sepsis and death in TEN patients (odds ratio=3.025, 2.757, 95% confidence interval=1.352-6.769, 1.244-6.110, P<0.05 or P<0.01). Conclusions: The diagnosis of TEN is difficult on admission. Male population is susceptible to TEN, and allopurinol is the common pathogenic agent. The proportion of patients combined with basic diseases is high in TEN patients complicated with sepsis, with mainly drug-resistant bacteria and mostly Gram-positive bacteria in blood-borne infections. The died patients are older than the survival, and the main cause of death is sepsis. The SCORTEN system score is an independent risk factor affecting sepsis and death in TEN patients.

Neutrophil:lymphocyte ratio is associated with disease severity and mortality in patients with Stevens-Johnson syndrome/toxic epidermal necrolysis.

The neutrophil:lymphocyte ratio (NLR), platelet:lymphocyte ratio (PLR), C-reactive protein:albumin ratio (CAR), and albumin:fibrinogen ratio (AFR) have been considered as useful inflammatory biomarkers. However, their roles in Stevens-Johnson Syndrome (SJS)/toxic epidermal necrolysis (TEN) still remain unclear. This study aimed to test whether NLR, PLR, CAR, and AFR serve as predictive markers of disease severity and systemic inflammation in patients with SJS/TEN. This retrospective study included 40 patients with SJS/TEN and 60 healthy controls. The correlation between these markers and severity-of-illness score for toxic epidermal necrolysis (SCORTEN), ABCD-10, procalcitonin (PCT), C-reactive protein (CRP) were analyzed and compared. Univariable and multivariable analysis were used to assess associations of variables with mortality. The receiver-operator curves (ROC) were used to evaluate the predictive value of variables for mortality in SJS/TEN patients. The results demonstrated that the NLR and PLR of SJS/TEN patients were significantly higher and the AFR was significantly lower when compared with healthy controls (p<0.05). The NLR and CAR were positively correlated with SCORTEN, ABCD-10, PCT, and CRP. The NLR in SCORTEN of ≥3 group was significantly higher than that in SCORTEN <3 group (p<0.05) and there were no significant differences between PLR, CAR, and AFR between the two groups. The univariate analysis suggested that NLR of >5.79 was a risk factor for mortality (odds ratio, 10.5; p<0.05), but the association was no longer statistically significant in multivariable analysis. The ROC showed that NLR had a sensitivity of 85.7% and specificity of 63.6% for predicting death with a cut-off value of 5.79 (p<0.05) in SJS/TEN patients. In conclusion, among the four markers, NLR and CAR can partially reflect severity and inflammatory status in patients with SJS/TEN. NLR was also a predictor of death.

Performance of ABCD-10 and SCORTEN mortality prediction models in a cohort of patients with Stevens-Johnson syndrome/toxic epidermal necrolysis

Age, bicarbonate, cancer, dialysis, 10% body surface area risk model (ABCD-10) has recently been proposed as an alternative to the SCORe of toxic epidermal necrolysis (SCORTEN) model for predicting in-hospital mortality in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). In contrast to SCORTEN, ABCD-10 incorporates prior dialysis and upweights the impact of cancer. To determine the performance of ABCD-10 compared with that of SCORTEN in mortality prediction at a large, tertiary burn center. A retrospective analysis of 192 patients with SJS/TEN admitted to the North Carolina Jaycee Burn Center from January 1, 2009, to December 31, 2019, was conducted.Data on these patients were collected using the burn registry and a manual chart review. The performance of both the mortality prediction models was assessed using univariate logistic regression and the Hosmer-Lemeshow test. The overall mortality was 22% (n=43). Nine (5%) patients had cancer, and 7 (4%) had undergone prior dialysis; neither factor was associated with mortality (P=.11 and P=.62, respectively). SCORTEN was well calibrated to predict inpatient mortality (P=.82), whereas ABCD-10 appeared to have a poorer fit (P<.001) in these patients. Both the models showed good discrimination. Small sample size. SCORTEN was a better predictor of inpatient mortality than ABCD-10 in a North American cohort of patients treated at the tertiary burn center.

Cohort study of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in China: evaluation of risk models and new predictor of pulmonary consolidation on computed tomography.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe diseases. This study aimed to validate the predictive ability of risk models in patients with SJS/TEN and propose possible refinement in China. Patients in the Department of Dermatology of Huashan Hospital from January 2008 to January 2019 were included. Results showed that the severity-of-illness score for TEN (SCORTEN) had a good discrimination (area under the receiver operating characteristic curve (AUC), 0.78), and it was superior to auxiliary score (AS) and ABCD-10, which indicates age, bicarbonate level, cancer, dialysis, and 10% involved body surface area (AUC, 0.69 and 0.68, respectively). The calibration of SCORTEN (Hosmer-Lemeshow goodness-of-fit test, P = 0.69) was also better than that of AS (P = 0.25) and ABCD-10 (P = 0.55). SCORTEN and ABCD-10 were similar (Brier score (BS), 0.04 and 0.04) in terms of accuracy of predictions. In addition, the imaging appearance of pulmonary consolidation on computed tomography was associated with high mortality. Refined models were formed using the variables and this imaging appearance. The refined AS and ABCD-10 models were similar in discrimination compared with the original SCORTEN (0.74 vs. 0.78, P = 0.23; 0.74 vs. 0.78, P = 0.30, respectively). Therefore, SCORTEN showed good discrimination performance, calibration, and accuracy, and refined AS or ABCD-10 model may be an option when SCORTEN variables are not available.

Drug-induced erythroderma in patients with acquired immunodeficiency syndrome.

To explore the clinical manifestations, diagnosis, and treatment of patients with acquired immunodeficiency syndrome (AIDS) complicated with drug-induced erythroderma. The clinical data of 12 AIDS patients with drug-induced erythroderma in our hospital were retrospectively analyzed. The general information, offending medications, complications, modified severity-of-illness score for toxic epidermal necrolysis (SCORTEN) scores, and disease outcome spectrums were analyzed. Drug-induced erythroderma was mostly caused by antiviral drugs, antituberculosis drugs, antibiotics, traditional Chinese medicine, and immune checkpoint inhibitors. The spectrum of sensitizing drugs was broad, the clinical situation was complex, and infections were common. The affected areas were greater than 40% body surface area in all patients. The modified SCOTERN score averaged 3.01±0.99. All patients were treated with glucocorticoids, and nine patients were treated with intravenous immunoglobulin (IVIG) pulse therapy at the same time. The average time to effectiveness was 7.08±2.23 days, and the average hospital stay was 17.92±8.46 days. Eleven patients were cured, and one patient died of secondary multiple infections, who had a modified SCORTEN score of 5 points. The mortality rate in this study was 8.3%. The clinical situation of AIDS patients with drug-induced erythroderma in hospitalized patients is complex and the co-infection rate is high. The use of modified SCORTEN score may objectively and accurately assess the conditions, and the use of glucocorticoid combined with IVIG therapy may improve the prognosis.

Treating toxic epidermal necrolysis with systemic immunomodulating therapies: A systematic review and network meta-analysis

Varioussystemic immunomodulating therapies have been used to treat toxic epidermal necrolysis (TEN), but their efficacy remains unclear. To perform a systematic review and network meta-analysis (NMA) evaluating the effects of systemic immunomodulating therapies on mortality for Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. A literature search was performed in online databases (from inception to October 31, 2019). Outcomes were mortality rates and Score of Toxic Epidermal Necrolysis (SCORTEN)-based standardized mortality ratio (SMR). A frequentist random-effects model was adopted. Sixty-seven studies involving 2079 patients were included. An NMA of 10 treatments showed that none was superior to supportive care in reducing mortality rates and that thalidomide was associated with a significantly higher mortality rate (odds ratio, 11.67; 95% confidence interval [CI], 1.42-95.96). For SMR, an NMA of 11 treatment arms showed that corticosteroids and intravenous immunoglobulin combination therapy was the only treatment with significant survival benefits (SMR, 0.53; 95% CI, 0.31-0.93). Heterogeneity and a paucity of eligible randomized controlled trials. Combination therapy with corticosteroids and IVIg may reduce mortality risks in patients with SJS/TEN overlap and TEN. Cyclosporine and etanercept are promising therapies, but more studies are required to provide clearer evidence.

Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment.

Prior to the first international consensus classification published in 1993, the clinical distinction between erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) had been subject to uncertainty and controversy for more than a century. Based on this classification, the three conditions are defined by the morphology of the individual lesions and their pattern of distribution. Etiopathogenetically, the majority of EM cases is caused by infections (primarily herpes simplex virus and Mycoplasma pneumoniae), whereas SJS/TEN are predominantly triggered by drugs. The SCORTEN (score of toxic epidermal necrolysis) can and should be used to assess disease prognosis in patients with SJS/TEN. While supportive treatment is generally considered sufficient for EM, there is still uncertainty as to the type of systemic therapy required for SJS/TEN. Given the lack of high-quality therapeutic trials and (in some cases) conflicting results, it is currently impossible to issue definitive recommendations for any given immunomodulatory therapy. While there is always a trade-off between rapid onset of treatment-induced immunosuppression and an uptick in infection risk, there has been increasing evidence that cyclosporine in particular may be able to halt disease progression (i.e. skin detachment) and lower mortality rates. Assistance in diagnosis and management of the aforementioned conditions may be obtained from the Center for the Documentation of Severe Skin Reactions (dZh) at the Department of Dermatology, University Medical Center, Freiburg, Germany.

531 Sex-based Differences in the Skin: Is It Just a Female Problem?

Abstract Introduction Female gender is associated with poorer outcomes in burns, not necessarily related to co-morbid conditions. Our objective was to analyze the effect of gender in biopsy proven Stevens-Johnson syndrome (SJS) and Toxic epidermal necrolysis (TEN) spectrum disorders at our institution. Methods Patients were identified using Institutional Burn Center registry, and linked to the clinical and administrative data. All patients admitted with biopsy proven SJS, SJS/TEN overlap, and TEN between January 1, 2009 and December 31, 2018 were eligible for inclusion. Demographics, length of stay, and mortality were evaluated. Statistical analysis was performed with Students’ t-test, chi-squared, and Fischer’s exact test. Results One hundred and sixty-eight patients had biopsy proven SJS, SJS/TEN overlap, or TEN. Sixty-one percent of patients were female. The average age of female patients was 51 years while the average age of male patients was 44 years, p&amp;lt; 0.05. Fifty-sex percent of female patients were black. All American Indian patients were female. Female patients had significantly longer lengths of stay, 34 days versus 22 days, p&amp;lt; 0.05. Female patients had an average SCORTEN (a severity of illness score of TEN) compared to male patients. Thirty-three percent of female patients died compared to 18% of male patients, p&amp;lt; 0.05. Two out of three American Indian patients in this cohort died. Conclusions Female gender is associated with increased mortality in burn patients, as well as in our cohort of patients with SJS/TEN. Females represent 51% of our state’s population, but 61% of patients with biopsy proven SJS, SJS/TEN overlap, and TEN at our institution. Compared to male patients, female patients presented with more severe disease (higher SCORTEN at presentation). Genomic, and proteomic studies looking at sex-based differences may be helpful in elucidating the origin of these differences. Applicability of Research to Practice This study demonstrates sex-based differences related to skin desquamating disorders, and these outcomes, coupled with known disparities in burns, may indicate a need for hypervigilance in inpatient management for women, and a re-evaluation of prognostic scoring tools to include a variable for gender.

Development and Validation of a Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis—ABCD-10

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of severe mucocutaneous drug reaction associated with significant morbidity and mortality. A previously developed SJS/TEN-specific severity-of-illness model (Score of Toxic Epidermal Necrolysis [SCORTEN]) has been reported to overestimate and underestimate SJS/TEN-related in-hospital mortality in various populations. To derive a risk prediction model for in-hospital mortality among patients with SJS/TEN and to compare prognostic accuracy with the SCORTEN model in a multi-institutional cohort of patients in the United States. Data from a multicenter cohort of patients 18 years and older treated for SJS/TEN between January 1, 2000, and June 1, 2015, were obtained from inpatient consult databases and electronic medical record systems at 18 medical centers in the United States as part of the Society for Dermatology Hospitalists. A risk model was derived based on data from 370 of these patients. Model discrimination (calculated as area under the receiver operating characteristic curve [AUC]) and calibration (calculated as predicted vs observed mortality, and examined using the Hosmer-Lemeshow goodness-of-fit statistic) were assessed, and the predictive accuracy was compared with that of SCORTEN. All analysis took place between December 2016 and April 2018. In-hospital mortality. Among 370 patients (mean [SD] age 49.0 [19.1] years; 195 [52.7%] women), 54 (15.14%) did not survive to hospital discharge. Five covariates, measured at the time of admission, were independent predictors of in-hospital mortality: age in years (odds ratio [OR], 1.05; 95% CI, 1.02-1.07), body surface area (BSA) in percentage of epidermal detachment (OR, 1.02; 95% CI, 1.01-1.04), serum bicarbonate level below 20 mmol/L (OR, 2.90; 95% CI, 1.43-5.88), active cancer (OR, 4.40; 95% CI, 1.82-10.61), and dialysis prior to admission (OR, 15.94; 95% CI, 3.38-66.30). A severity-of-illness score was calculated by taking the sum of 1 point each for age 50 years or older, epidermal detachment greater than 10% of BSA, and serum bicarbonate level below 20 mmol/L; 2 points for the presence of active cancer; and 3 points for dialysis prior to admission. The score was named ABCD-10 (age, bicarbonate, cancer, dialysis, 10% BSA). The ABCD-10 model showed good discrimination (AUC, 0.816; 95% CI, 0.759-0.872) and calibration (Hosmer-Lemeshow goodness of fit test, P = .30). For SCORTEN, on admission, the AUC was 0.827 (95% CI, 0.774-0.879) and was not significantly different from that of the ABCD-10 model (P = .72). In this cohort of patients with SJS/TEN, ABCD-10 accurately predicted in-hospital mortality, with discrimination that was not significantly different from SCORTEN. Additional research is needed to validate ABCD-10 in other populations. Future use of a new mortality prediction model may provide improved prognostic information for contemporary patients, including those enrolled in observational studies and therapeutic trials.

Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a delayed-type hypersensitivity reaction to drugs. They represent true medical emergencies and an early recognition and appropriate management is decisive for the survival. SJS/TEN manifest with an "influenza-like" prodromal phase (malaise, fever), followed by painful cutaneous and mucous membrane (ocular, oral, and genital) lesions, and other systemic symptoms. The difference between SJS, SJS/TEN overlap, and TEN is defined by the degree of skin detachment: SJS is defined as skin involvement of <10%, TEN is defined as skin involvement of >30%, and SJS/TEN overlap as 10-30% skin involvement. The diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology. The mortality rates for SJS and TEN have decreased in the last decades. Today, the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is available for SJS/TEN severity assessment. Drugs with a high risk of causing SJS/TEN are anti-infective sulfonamides, anti-epileptic drugs, non-steroidal anti-inflammatory drugs of the oxicam type, allopurinol, nevirapine, and chlormezanone. Besides conventional drugs, herbal remedies and new biologicals should be considered as causative agents. The increased risk of hypersensitivity reactions to certain drugs may be linked to specific HLA antigens. Our understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytotoxicity mechanisms were clarified. However, many factors contributing to epidermal necrolysis still have to be identified, especially in virus-induced and autoimmune forms of epidermal necrolysis not related to drugs. In SJS/TEN, the most common complications are ocular, cutaneous, or renal. Nasopharyngeal, esophageal, and genital mucosal involvement with blisters, erosions as well as secondary development of strictures also play a role. However, in the acute phase, septicemia is a leading cause of morbidity and fatality. Pulmonary and hepatic involvement is frequent. The acute management of SJS/TEN requires a multidisciplinary approach. Immediate withdrawal of potentially causative drugs is mandatory. Prompt referral to an appropriate medical center for specific supportive treatment is of utmost importance. The most frequently used treatments for SJS/TEN are systemic corticosteroids, immunoglobulins, and cyclosporine A.

Successful treatment of toxic epidermal necrolysis using plasmapheresis: A prospective observational study

Toxic epidermal necrolysis (TEN) is a rare, severe, life-threatening skin disease and it requires urgent critical care, including admission to the intensive care unit (ICU). It is characterized by fatal sequelae and high mortality. Currently, insufficient evidence exists to support the use of any systemic adjuvant therapy, such as cyclophosphamide, intravenous immunoglobulin (IVIg), or corticosteroids. However, plasmapheresis has been increasingly valued by clinicians due to its significant efficacy and little adverse side effects. To assess the efficacy of such treatment, 28 patients who were diagnosed with TEN or SJS/TEN overlap were continuously recruited in the ICU from February 2009 to August 2016. These patients including both children and adults were randomly divided into two groups based on whether or not plasmapheresis therapy was performed after admission, which resulted in a plasmapheresis group (n=13) and a non-plasmapheresis group (n=15). Severity of the disease and the efficacy of treatments were evaluated by the severity-of-illness score for TEN. The results indicated that plasmapheresis may be superior to conventional therapies, such as IVIg or corticosteroids. Furthermore, plasmapheresis combined with other treatments might not be advantageous compared to the effect of plasmapheresis alone.

Cyclosporine Use in Epidermal Necrolysis Is Associated with an Important Mortality Reduction: Evidence from Three Different Approaches

Several immunomodulatory agents are used in the treatment of epidermal necrolysis, but evidence of their efficacy is limited. The Autonomous Community of Madrid has two reference burn units to which all patients with epidermal necrolysis are referred. One burn unit has mostly used cyclosporine (CsA), and the other has used non-CsA therapies (mainly high-dose intravenous immunoglobulin). The allocation of patients to one or the other burn unit was mainly based on proximity, resembling a random assignment. Thus, we took advantage of this "natural experiment" to estimate the mortality risk ratio (MRR) of CsA (n= 26) compared with non-CsA (n= 16) treatment using hospital as an instrumental variable over the period from 2001 to 2015. We also computed the observed versus expected (O/E) MRR in a case series of 49 CsA-treated patients (including 23 patients from other regions treated in Madrid), and using the Score for Toxic Epidermal Necrolysis (i.e., SCORTEN) scale to estimate the expected values. The instrumental variable-based MRR of CsA versus non-CsA was 0.09 (95% confidence interval= 0.00-0.49). The O/E analysis also showed a reduction in mortality risk (MRROE= 0.42; 95% confidence interval= 0.14-0.99). We identified five other case series of CsA-treated patients providing MRROE and meta-analyzed their results. The pooled MRROE (including from this study) was 0.41 (95% confidence interval= 0.21-0.80). All three approaches consistently show that CsA reduces the mortality in epidermal necrolysis patients.

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.

A clinicoetiological analysis of patients with Stevens–Johnson syndrome and toxic epidermal necrolysis treated at Sohag University Hospital, Egypt

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are characterized by widespread epidermal necrosis and mucosal involvement secondary to keratinocyte apoptosis induced mostly by drugs and associated with high mortality. To examine the prevalence, etiology, clinical presentations, and outcome of SJS/TEN in patients admitted in the Department of Dermatology, Venereology, and Andrology, Faculty of Medicine, Sohag University, Sohag, Egypt. This observational study included all patients with SJS/TEN admitted in the Department of Dermatology, Venereology, and Andrology, Faculty of Medicine, Sohag University, Sohag, Egypt, between September 2013 and September 2014. All patients with SJS/TEN were subjected to complete medical history and examinations to evaluate the extent of skin and mucous membrane involvement, complications, and other system affections. They also underwent complete laboratory investigations. The severity-of-illness score for toxic epidermal necrolysis was used to evaluate all patients within the first 24 h of admission. Of the 500 patients admitted, 21 (4.2%) were diagnosed with SJS/TEN (six men and 15 women; mean age 30.4 years). Fourteen patients were diagnosed with SJS (n=14; 66.7%), three with SJS/TEN overlap (n=3; 14.3%), and four with TEN (n=4; 19%). Nineteen patients (90.5%) had a history of drug intake. The most common causative drugs were carbamazepine (33.3%) and antibiotics+NSAIDs (28.57%). The main limitations of our study were the small sample size. SJS and TEN are uncommon diseases. Most of the cases of SJS/TEN were drug-induced. The most common offending drugs were carbamazepine, NSAIDs, and antibiotics.

Enfants porteurs d’anticorps anti-U1RNP : description et profils évolutifs. Une étude rétrospective nationale sur 69 cas pédiatriques

Toxic epidermal necrolysis (TEN) is a rare, severe, life-threatening skin disease and it requires urgent critical care, including admission to the intensive care unit (ICU). It is characterized by fatal sequelae and high mortality. Currently, insufficient evidence exists to support the use of any systemic adjuvant therapy, such as cyclophosphamide, intravenous immunoglobulin (IVIg), or corticosteroids. However, plasmapheresis has been increasingly valued by clinicians due to its significant efficacy and little adverse side effects. To assess the efficacy of such treatment, 28 patients who were diagnosed with TEN or SJS/TEN overlap were continuously recruited in the ICU from February 2009 to August 2016. These patients including both children and adults were randomly divided into two groups based on whether or not plasmapheresis therapy was performed after admission, which resulted in a plasmapheresis group (n = 13) and a non-plasmapheresis group (n = 15). Severity of the disease and the efficacy of treatments were evaluated by the severity-of-illness score for TEN. The results indicated that plasmapheresis may be superior to conventional therapies, such as IVIg or corticosteroids. Furthermore, plasmapheresis combined with other treatments might not be advantageous compared to the effect of plasmapheresis alone.