Score For Toxic Epidermal Necrolysis Research Articles

Prognostic accuracy of the SCORTEN scoring system in patients with toxic epidermal necrolysis or Stevens-Johnson syndrome

Objective To evaluate the prognostic accuracy of the score of toxic epidermal necrolysis (SCORTEN) scoring system in patients with toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS) . Methods Clinical data were collected from 39 patients with SJS/TEN hospitalized in Peking Union Medical College Hospital during April 1992 and March 2014, and retrospectively analyzed. Among the 39 patients, 13 had died, and the other 26 patients, who were matched to the dead patients in a ratio of 2∶1 for age, all had a definite diagnosis and were discharged with improved conditions. The SCORTEN scoring system was used to evaluate the 39 patients with SJS/TEN and calculate expected mortality. The expected mortality and actual mortality were compared between different groups stratified by age in the 39 patients. The receiver operating characteristic (ROC) curve was drawn to assess the prognostic accuracy of the SCORTEN scoring system. Results According to the SCORTEN scoring system, 15 out of the 39 patients scored 1 point, 14 scored 2 points, 6 scored 3 points, and 4 scored 4 points. The total number of expected deaths was 6.808, while that of actual deaths was 13. There was no significant difference between the expected mortality and actual mortality in every SCORTEN score-based group. The area under curve (AUC) was 0.832 8, indicating a good predictive ability of the SCORTEN scoring system. Conclusion The SCORTEN scoring system can predict mortality in TEN/SJS patients at early stage. Key words: Epidermal necrolysis, toxic; Drug eruptions; Stevens-Johnson syndrome; SCORTEN

Serum IFN-γ-inducible chemokines CXCL9 and CXCL10 are elevated in non-immediate drug hypersensitivity reactions.

The recruitment to the skin of drug-responsive T cells is responsible for the inflammatory profiles of non-immediate drug hypersensitivity reactions (niDHRs). Maculopapular exanthema (MPE) and Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have quite distinct T cell infiltrating patterns. To investigate serum levels of CXCL9, CXCL10 and IFN-γ in patients with niDHRs, including MPE and SJS/TEN, to evaluate correlations between the cytokines, and to determine whether the inflammatory factors correlate with clinical severity in patients with SJS/TEN. Twenty-four patients with SJS/TEN, 24 patients with MPE, and 24 healthy donors with good tolerance to the drugs involved in the drug reactions were recruited into the study. The modified severity-of-illness score for TEN (SCORTEN) and detachment of body surface area (dBSA) were used to assess the clinical severity of SJS/TEN. Serum levels of CXCL9, CXCL10 and IFN-? were determined by ELISA. The niDHRs group, SJS/TEN and MPE subgroups all exhibited significantly higher levels of CXCL9, CXCL10 and IFN-γ compared with the control group (P < 0.001). Serum IFN-γ levels were positively correlated with CXCL9 levels and CXCL10 levels in patients with niDHRs (rs = 0.576, rs = 0.449, P < 0.05). None of the levels of CXCL9, CXCL10 and IFN-γ had any correlation with modified SCOTEN index or dBSA in SJS/TEN group. The results suggest Th1 cytokine IFN-γ and chemokines CXCL9 and CXCL10 may play roles in the pathogenesis of niDHRs.

Clinical profiles and treatment outcomes of systemic corticosteroids for toxic epidermal necrolysis: A retrospective study.

Toxic epidermal necrolysis (TEN) is an uncommon severe cutaneous adverse reaction. Although controversies remain in the pathophysiology and management of this condition, improvements in survival and morbidity have been observed over the past decade. The aim of the present study was to demonstrate the clinical profiles of TEN in Thai patients and the treatment outcome with dexamethasone pulse therapy assessed by using the Severity of Illness Score for Toxic Epidermal Necrolysis (SCORTEN). Medical records of all patients with TEN were collected retrospectively from January 2002 to December 2012. Epidemiological features, etiologies, treatments and clinical outcomes were reviewed. Of 18 patients, the female to male ratio was 1:1 and the mean age was 49.7years. Cephalosporins (27.8%), phenytoin (16.7%), carbamazepine, sulfonamide drugs and allopurinol (11.1% each) were implicated as leading causes of TEN. Hepatitis was the most frequent complication (77.8%). Pulsed high doses of dexamethasone 1-1.5mg/kg per day for a short period were administrated in all cases. Two of the 18 patients receiving corticosteroids (SCORTEN 5 and 6) died. The mortality rate was 11% (2/18 patients), however, no patient receiving systemic corticosteroids died if the patients had less than 4points on SCORTEN. The clinical features of Thai patients with TEN were similar to other reports. In conclusion, in addition to withdrawal of the suspected agent and intensive supportive care, the administration of short-term dexamethasone pulse therapy, particularly during the initial phase, may be beneficial in reducing the mortality rate.

Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are erythematous skin lesions with blister formation accompanied by mucosal involvement. These conditions are considered to be life-threatening illnesses. Understanding the clinical presentation, risk factors, treatment options and results will be advantageous for physicians in the management of patients in the future. The aim of the present study was to review and analyze the clinical manifestations, drug implications, treatment and outcome of patients with SJS and/or TEN who had been hospitalized in a tertiary care center. All hospitalized patients with SJS and/or TEN during a 5-year period were retrospectively reviewed. The clinical severity was graded according to the score of toxic epidermal necrolysis (SCORTEN) scale. Clinical symptoms, diagnosis, possible precipitating factors, management and outcome data were collected for analysis. A total of 43 patients (mean age, 49.5 years) were hospitalized and classified into the SJS group (55.8%), SJS/TEN overlap group (20.9%) and TEN group (23.3%). The majority of the patients (90.7%) had mucocutaneous eruptions associated with oral drug administration. Allopurinol, anticonvulsants and antibiotics were the most common causative agents for the mucocutaneous eruption. Twenty-eight patients (65.1%) were treated with corticosteroids. The mortality rate was 6.9%. Comparison between the survival group and the non-survival group revealed that patient age >70 years (P=0.014) and body surface area involvement >20% (P<0.01) were the significant factors associated with mortality. The use of systemic steroids was higher in the survival group in comparison with the non-survival group (65.1 vs. 0%, respectively; P=0.014). The mucocutaneous eruptions in SJS and TEN are mostly caused by medication. With early recognition and treatment, the mortality rate in this study was lower than that in previous reports. Patient age and the area of mucocutaneous involvement were significant factors associated with mortality.

Pediatric toxic epidermal necrolysis: using SCORTEN and predictive models to predict morbidity when a focus on mortality is not enough.

Toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS) are rare yet severe exfoliative skin disorders. The authors examined the efficacy of predictive models for their use as prognostic indicators in pediatric SJS or TEN. Over a 15-year period, 41 patients ≤18 years of age were identified and reviewed. Predictive models compared retrospectively to the observed mortality in the population included: SCORTEN (Score of Toxic Epidermal Necrolysis), Pediatric Index of Mortality 2, Pediatric Risk of Mortality III-24, and Abbreviated Burn Severity Index. Correlation coefficients and 95% confidence intervals were calculated for the following: acute hospital length of stay, days until wound closure, days of mechanical ventilation, number of infectious complications, and number of acute operative procedures. When calculated within the first 24 hours of admission, the four models predicted low rates of mortality, approaching our 0% observed mortality, and also demonstrated a significant correlation between higher scores and the five chosen indicators of morbidity (P < .05). SCORTEN on day 3 of admission was statistically significant (P < .05) only when predicting the number of infectious complications and days of mechanical ventilation and was therefore felt to be less predictive than use of SCORTEN on admission. When calculated within the first day of admission of pediatric SJS or TEN patients, SCORTEN, Abbreviated Burn Severity Index, Pediatric Index of Mortality 2, and Pediatric Risk of Mortality III all significantly predict acute morbidity. This is the first time scoring systems have been assessed for their ability to predict mortality and morbidity in pediatric SJS or TEN despite its widely recognized, severe acute course of disease.

Toxic Epidermal Necrolysis: Treatment of Two Cases with Intravenous Immunoglobulin

Toxic epidermal necrolysis (TEN) is a severe allergic reaction, often to drugs, with an approximate 30-40% mortality. Discontinuation of the offending drug and burn unit supportive care are agreed upon as initial therapy. However, use of Intravenous Immunoglobulin (IVIG) has been shown effective but controversial. We report 2 cases of TEN treated with IVIG for 5 days and burn unit intensive care therapy. One case had early adjunctive systemic corticosteroid therapy; the other case had no systemic steroids. Both cases demonstrated a severity of illness score for TEN (SCORTEN) at day 1 of 35.3% and day 3 of 58.3%. Previous reports base their opinion regarding IVIG effectiveness on studies that do not uniformly specify these treatment details or wherein IVIG treatment protocol was for less than 5 days. Inclusion of this data and use of this treatment protocol may demonstrate significantly improved outcomes in TEN patients treated with IVIG.

A clinicotherapeutic analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis with an emphasis on the predictive value and accuracy of SCORe of Toxic Epidermal Necrolysis.

The SCORTEN scale (SCORe of Toxic Epidermal Necrolysis) is widely used to predict mortality in patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). Although it is largely a useful tool, the predictive ability of the scale is variable, and modifications to the existing scale have been suggested. In addition, there is controversy regarding the roles of active therapy and conservative management. This study was conducted to assess outcomes in SJS/TEN patients managed conservatively and to analyze the predictive performance of SCORTEN. Sixty patients were studied prospectively from October 2008 to September 2011. The accuracy of SCORTEN in predicting mortality was analyzed on days 1, 3, and 5 of admission. All patients were managed conservatively. The discriminative power of SCORTEN was acceptable, being best on day 5. The agreement between actual and predicted mortality was poor, as indicated by a low P-value of the Hosmer-Lemeshow statistic. Only three parameters (heart rate, blood urea, and serum bicarbonate) were found to be significant on multivariate analysis, and all of these represented components within the original SCORTEN scale. The mortality rate was 16.7%. All deaths occurred within the first twoweeks (8-12days). Serial analysis using SCORTEN is better than analysis performed only on day 1. Although the scale is largely useful, its performance is influenced by the demographic profile of the study population; minor modifications based on the population studied may increase the predictive accuracy of the original SCORTEN. In addition, conservative management is a valid therapeutic option, is preferable to treatment with steroids and immunosuppressants, and is highly recommended.

Toxic epidermal necrolysis (TEN): The Chelsea and Westminster Hospital wound management algorithm

Toxic epidermal necrolysis syndrome (TEN) is a potentially catastrophic exfoliative muco-cutaneous disorder first described by Lyell in 1956. It represents the most extensive form of Steven-Johnson syndrome. TEN is defined varyingly around the globe, but in the United Kingdom the consensus opinion describes the process as involving >30% of the total body surface area. It can rapidly become more extensive and threatens life. The estimated annual incidence is approximately 1-2 cases per million population. The risk of mortality increases with surface area involved and meta-analysis of the literature shows this risk to be between 16% and 55%. Over a six month period the Chelsea and Westminster Hospital Burns Service treated five consecutive patients with more than 80% total body surface area involvement or a more than 80% mortality risk, using the severity-of-illness score for toxic epidermal necrolysis (SCORTEN). All patients were treated according to the Chelsea and Westminster Hospital wound management algorithm with excellent outcome and no mortalities. The aim of this paper is to propose a generic TEN wound management algorithm according to the severity of skin lesions, using a simple wound grading system.

The role of intravenous immunoglobulin in toxic epidermal necrolysis: a retrospective analysis of 64 patients managed in a specialized centre

Toxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction with a mortality of 40%. Intravenous immunoglobulin (IVIg) is widely used as a specific treatment for this reaction, although evidence of its benefit is conflicting. We sought to evaluate whether the use of IVIg improved mortality in patients with Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. We retrospectively analysed data for 64 patients with SJS/TEN overlap and TEN who were treated with IVIg at a single referral centre. The primary outcome analysed was in-hospital mortality. Predicted mortality was calculated based on severity-of-illness score for TEN (SCORTEN) values. Secondary analyses of survival based on IVIg dosages and prior corticosteroid exposure were also performed. There were 28 cases of SJS/TEN overlap and 36 cases of TEN, with a mean SCORTEN value of 2·6. The mean dose of IVIg given was 2·4 g kg(-1) and the mean delay from the onset of epidermal detachment to administration of IVIg was 3·2 days. There were 20 deaths (31%) in our cohort. The standardized mortality rate was 1·10 (95% confidence interval 0·62-1·58). Subgroup analysis comparing survivors and nonsurvivors showed a higher SCORTEN in nonsurvivors (3·4 vs. 2·2). There were no differences with regard to the dosage, delay and duration of IVIg administration. When stratified according to dosage, there was no mortality difference between patients who receive high-dose (≥ 3 g kg(-1) ) vs. low-dose (< 3 g kg(-1) ) IVIg. This study shows that the use of IVIg does not yield survival benefits in SJS/TEN overlap and TEN, even when corrected for IVIg dosages.

Pediatric Toxic Epidermal Necrolysis

Stevens Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare debilitating mucocutaneuous diseases most commonly associated with severe drug reactions. A review of SJS/TEN cases was undertaken to describe the etiology, patient characteristics, treatment, and outcome in children affected at a large tertiary pediatric hospital. A retrospective chart review examined SJS/TEN cases admitted to a pediatric intensive care unit (PICU) between 2001 and 2011. Data concerning total body surface area (TBSA) involvement, causative agents, length of stay, and treatment were reviewed. PICU mortality predictors including Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN), Pediatric Index of Mortality 2 scores, and Pediatric Logistic Organ Dysfunction scores were also reviewed. Nutritional feeding information was collected and compared with calculated and measured (calorimetry) requirements. There were 10 SJS/TEN pediatric patients (mean age = 6.6 years) with significant skin involvement (mean TBSA = 42.2%) requiring PICU admission. Prescription drugs were the most common causative factor (n = 7). Intravenous immunoglobulin and corticosteroids were used for treatment in eight and two of the cases, respectively. The mean length of stay in the PICU was 10.2 days. There were no mortalities, and all mortality scores predicted high likelihood of survival. Actual feeds (n = 9) were uniformly lower than the calculated feeding requirements (mean = 1059 kcal/day vs mean = 2027 kcal/day). This data will help to provide insight into the management of SJS/TEN in an intensive care setting, and will help form the best treatment approach for future cases in a pediatric population.

Toxic Epidermal Necrolysis

Toxic epidermal necrolysis (TEN) represents the most severe drug-related skin condition that is potentially life-threatening with no well-established treatments. The application of corticosteroid therapy is controversial, whereas recently intravenous immunoglobulin (IVIG) therapy is emerging as a promising new method. A severity-of-illness score for TEN (SCORTEN) has gained acceptance in some western countries. In this study, our objectives were to assess the applicability of SCORTEN in Chinese patients with TEN and to evaluate the efficacy of the combination therapy of IVIG and corticosteroid in these patients. We performed a retrospective review of data from 61 patients with TEN treated at our intensive care unit from 2000 to 2010 to assess the performance of SCORTEN. In particular, 55 patients between 2002 and 2010 were grouped as a series to compare the therapeutic effects of corticosteroid therapy and IVIG combined therapy contemporaneously. During this period, 16 patients were administered with corticosteroid therapy and 39 were treated with the combination therapy. An initial dose of 1.5 mg/kg/day of methylprednisolone was given to all TEN patients. The combination therapy was combined with a total dose of 2 g/kg IVIG within 5 days. Areas under receiver operating characteristic curves and Hosmer-Lemeshow statistic were analyzed to illustrate the performance of SCORTEN. The comparison of the efficacy of the two therapies was conducted on the basis of clinical outcomes, standardized mortality ratio (SMR), and survival analysis. The overall actual mortality of patients between 2000 and 2010 was 16% (10/61), statistically insignificantly lower than predicted (24%, SMR = 67.98). Excellent discriminatory power (the areas under the receiver operating characteristic curves: 88.9, 88.2, 90.6%) and good calibration (P = .637, .833, .530) were found in all the groups. In patients admitted between 2002 and 2010, IVIG combined therapy showed a trend toward reducing the mortality rate (13%, SMR = 52.35), whereas corticosteroid monotherapy suggested no such difference (31%, SMR = 123.92). Besides, the cumulative survival rates of the combination therapy were higher at almost all the levels of SCORTEN (P = .002), especially at the score of 5 (P = 3.10 × 10⁻⁷). Compared with corticosteroid alone, the combination therapy arrested progression earlier (P = .013), although it did not significantly lead to a tapering of corticosteroid or a reduction of the time of hospitalization. We concluded that SCORTEN was generally applicable to Chinese patients with TEN. The comparison of the effect indicated that the combination therapy might achieve a better therapeutic effect than the administration of corticosteroid alone, especially in severe TEN patients.

In a Patient With Toxic Epidermal Necrolysis, Does Intravenous Immunoglobulin Improve Survival Compared With Supportive Care?

Clinical Question: A 67-year-old Chinese man presented with pain and widespread skin erythema and detachment. A clinical diagnosis of toxic epidermal necrolysis (TEN) was made, which was supported by skin biopsy findings. The TEN was thought to be due to recent initiation of lamotrigine for mild epilepsy, and the drug was stopped immediately. The severity of TEN, measured by the Severityof-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) scale, was 3 on admission owing to renal impairment, indicating a mortality risk of 35%. The patient was transferred to the burns unit for close monitoring of temperature, fluid balance, and appropriate dressings for his affected skin. A debate ensued among the dermatology, plastic surgery, and intensive care teams responsible for his care about whether intravenous immunoglobulin (IVIg) should be added to his treatment because improvement was slow with conservative treatment.

What Are the Fluid Requirements in Toxic Epidermal Necrolysis?

Acute fluid requirements in toxic epidermal necrolysis (TEN) have neither been quantified nor reported. The purpose of this study was to examine acute fluid administration in TEN patients. A consecutive series of criteria and biopsy-confirmed cases of TEN admitted to our burn centre were selected for retrospective analysis. Charts were reviewed for demographic and resuscitation variables for the first (D0), second (D1), and third (D2) 24-hour periods after burn center admission. Twenty-one TEN cases were available for study, with a mean epidermal detachment of 53 +/- 24% TBSA. Mortality was 29%, with all deaths occurring after the study period. Average crystalloid volumes decreased from D0 (2.2 +/- 1.5 ml/kg/%TBSA) through D1 (1.6 +/- 1.1 ml/kg/%TBSA) and D2 (1.4 +/- 1.0 ml/kg/%TBSA), whereas urine output increased from D0 (1.3 +/- 0.9 ml/kg/hr) through D1 (1.4 +/- 0.9 ml/kg/hr) and D2 (1.8 +/- 1.1 ml/kg/hr). Worst base deficit (BD) corrected significantly from D0 to D1 (P = .01) and from D1 to D2 (P = .002). There was no correlation between daily crystalloid volumes and %TBSA detachment. Nonsurvivors had significantly higher severity-of-illness score for TEN and 24-hour mean and worst BDs than survivors, but did not require significantly more crystalloid or display lower urine outputs. Initial provision of approximately 2 ml/kg/%TBSA epidermal detachment to patients with TEN resulted in more than adequate urine output and significant correction of the BD. We emphasize that these data do not represent a resuscitation formula but rather a guideline for initial fluid administration, which should then be titrated to the patient's response.

Antiretroviral-induced toxic epidermal necrolysis in a patient positive for human immunodeficiency virus

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two variants on a spectrum of severe systemic hypersensitivity characterized by blistering maculopapular lesions and desquamation of the skin and mucus membranes. Although several causative agents, including infections, have been reported for SJS/TEN, medications remain the most common cause. We report the case of a 42-year-old man with human immunodeficiency virus (HIV) who developed TEN 4 months after starting treatment with darunavir and abacavir. The patient presented with upper body lesions, oral mucosal ulcerations, and impending airway compromise. He was intubated and admitted to the burns unit. Score for Toxic Epidermal Necrolysis (SCORTEN) was 5, with > 90% predicted mortality. However, after intravenous immunoglobulin and supportive treatment, the patient made a remarkable recovery. Abacavir and darunavir may be associated with SJS/TEN. TEN should be considered a risk for patients with HIV and should be monitored for cutaneous eruptions for several months after changes in treatment regimen.

Antishear Therapy for Toxic Epidermal Necrolysis: An Alternative Treatment Approach

Toxic epidermal necrolysis syndrome is a devastating disease, with mortality rates ranging between 20 and 60 percent. This study evaluated an alternative treatment approach using antishear wound care and compared outcomes using the severity of illness score for toxic epidermal necrolysis syndrome (SCORTEN) system. Records of 48 patients with a histopathologic diagnosis of toxic epidermal necrolysis syndrome treated with burn equivalent critical care and antishear wound care from September of 1985 to April of 2004 were reviewed. Observed mortality data were compared with those expected using the SCORTEN, and the standardized mortality ratio was calculated. The overall mortality rate was 27 percent. Factors affecting mortality were advancing age, time to burn unit admission, multisystem organ failure, and presence of comorbidities (p = 0.02, p = 0.02, p < 0.001, and p = 0.003, respectively). Chronic renal insufficiency and malignancy were two independent risk factors for nonsurvival (p = 0.04 and p = 0.004, respectively). Patients with a SCORTEN score of 2 or less had no mortality rate in this series. Observed and predicted mortality rates were comparable for patients with SCORTEN scores of 3 or greater. Patients with combined scores of 3 or less had a standardized mortality ratio score of 0.58 (42 percent mortality reduction). Overall, the standardized mortality ratio was 0.89 (11 percent mortality reduction). Transfer to a burn intensive care unit and initiation of critical care and wound protocols similar to those used for burn patients are recommended for patients with toxic epidermal necrolysis syndrome. Antishear wound care provides an effective alternative wound care approach with equivalent mortality rates.

Toxic epidermal necrolysis: analysis of clinical course and SCORTEN-based comparison of mortality rate and treatment modalities in Korean patients

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, drug-induced cutaneous reaction. We herein report our experience regarding causes, clinical course, treatment and sequelae of TEN in Korean patients. In addition, we used the SCORTEN, a severity-of-illness score for TEN, to compare the predicted and actual mortality rates, and to evaluate the efficacy of treatment modalities. A retrospective study of 38 patients with TEN during a 13-year period (1990-2003) at the Asan Medical Center was performed. The mean involved body surface area was 49+/-17%. All except three cases were associated with medications, most commonly antibiotics, followed by non-steroidal anti-inflammatory drugs, acetaminophen and herbal remedies. Fourteen patients had a history of current infection, including upper respiratory infection, pneumonia and herpes simplex infection. The mean time from initial drug administration to the onset of TEN was 9.8+/-5.7 days. Twenty-one patients were treated with systemic corticosteroids. Fourteen received high dose intravenous immunoglobulin therapy. The actual mortality rate was 23.7% (9/38), not significantly different from the SCORTEN-predicted rate (25.5%, 9.699/38). Also based on SCORTEN, treatment with high dose intravenous immunoglobulin showed a trend to lower actual mortality than predicted mortality (standardized mortality ratio (SMR) = 0.425; 95% CI, 0.011-2.368), whereas corticosteroid therapy showed no such difference (SMR = 1.004; 95% CI, 0.369-2.187).