Severity-of-illness Score For Toxic Epidermal Necrolysis Research Articles

Systematic review and meta-analysis of non-SCORTEN predictors of mortality in Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, severe cutaneous adverse reactions that result in in-hospital death in 12-49% of cases. The severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is the most widely used mortality prognosis score; however, it has been shown to have critical limitations. Other mortality predictors not incorporated in SCORTEN or other predictor tools are being increasingly reported. This systematic review and meta-analysis aimed to synthesize and evaluate the predictors of mortality in adults with Stevens-Johnson syndrome and toxic epidermal necrolysis not included in SCORTEN. It was registered with the International Platform of Registered Systematic Review and Meta-Analysis Protocols (INPLASY) and conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) reporting guidelines. Potential bias was assessed using the National Institutes of Health (NIH) criteria. Forty articles describing results from 52,398 cases were included. Sixteen predictors were reported in five or more articles, and thirty-three were reported in two to four articles. Meta-analysis showed preexisting renal disease (odds ratio (OR): 3.14, 95% confidence interval (CI): 1.99-4.97, P < 0.0001, I2 = 21%), renal involvement (OR: 5.62, 95% CI: 2.29-13.77, P = 0.0002, I2 = 36%), respiratory involvement (OR: 3.14, 95% CI: 1.25-7.92, P = 0.015, I2 = 66%), diabetes mellitus (OR: 1.87, 95% CI: 1.21-2.89, P = 0.005, I2 = 19%), sepsis (OR: 5.64, 95% CI: 2.81-11.29, P < 0.0001, I2 = 63%), comorbidity (OR: 9.13, 95% CI: 4.60-18.12, P < 0.0001, I2 = 0%), and time to hospitalization (OR: 2.56, 95% CI: 1.15-5.65, P = 0.021, I2 = 93) increased risk of mortality. This systematic review and meta-analysis support several clinical and laboratory parameters not included in SCORTEN (preexisting renal disease, renal involvement, respiratory involvement, diabetes mellitus, sepsis, comorbidities, and time to hospitalization) as predictors of mortality in adults with SJS/TEN. The future utilization of these factors may improve mortality prognostication in adults with SJS/TEN.

Utility of the Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) in Pediatric Stevens-Johnson Syndrome Patients.

The Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) is a system that predicts in-hospital mortality for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN). The system is widely utilized in adults but not pediatrics. We aim to determine the accuracy of the SCORTEN in pediatrics. A retrospective review of pediatric patients admitted to a verified pediatric burn center with SJS/TEN from 2008 to 2022 was performed. Twenty-four patients were analyzed. Ten patients had 0-1 SCORTEN risk factor, thirteen had 2 risk factors, and one had three risk factors. There was no relationship between initial BUN, bicarbonate, glucose, or initial heart rate on the length of intensive care unit (ICU) stay or ventilator days. Hospital length of stay and feeding tube days were positively related (p<0.001) along with length of stay and maximum total body surface areas (TBSA) (p<0.05 Hospital length of stay, ICU length of stay, and ventilator days were not statistically significant between those having 0-1 and 2 risk factors. This study suggests that the SCORTEN system is not useful for pediatrics and a different scoring system is needed, as SCORTEN overestimates mortality and does not have a relationship to outcome measures.

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.

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.

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.

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.

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.

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.

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.

ORAL SECONDARY INFECTION IN STEVENS-JOHNSON SYNDROME PATIENT WITH ORAL INVOLVEMENT: A CASE REPORT

ABSTRACTBackground: Stevens-Johnson syndrome (SSJ) is a hypersensitivity reaction that is often triggered by drugs but this case is rare. These reactions result in uncontrolled keratinocyte damage to the skin and mucosa throughout the body, including the oral mucosa, and are often life-threatening. The use of high doses of corticosteroids is a treatment that is often given but it can trigger secondary infections of fungal and viral in the oral cavity. Purpose: This case report discusses the management of oral manifestations and secondary infections in SSJ patients, and becomes guidance for health professionals. Case: A-42-years-old male patient was consulted from the Department of Dermatology and Venereology (DV) due to oral pain and eating difficulties. The severity-of-illness-score for toxic-epidermal-necrolysis (SCORTEN) was 1. Erosive serosanguinous crusts, tend to bleed were found on the lips. Intraoral clinically presented wide erosive lesions and multiple ulcers, accompanied by a pseudomembranous plaque, and teeth decay. Hematologic examination showed an increase in leukocytes, neutrophil segments, monocytes, SGOT, urea, and creatinine as well as decreased hemoglobin, hematocrit, erythrocytes, MCHC, protein, and albumin. Anti-HSV1 IgG increased almost 6 times than normal values. The patient was diagnosed with SJS with oral involvement, secondary infections of pseudomembranous candidiasis, and herpetic stomatitis. Case Management: Systemic therapy given were intravenous dexamethasone, ranitidine, calcium, and cetirizine, from the DV Department, while hydrocortisone lip ointment, Chlorhexidine digluconate 0.12%, and Nystatin oral suspension for oral problems. The lesions progressed in 24 days. Conclusion: Oral secondary infections may occur in SJS patients due to high-dose corticosteroid therapy.Keywords: Herpetic Stomatitis, Oral Manifestation, Oral Secondary Infection, Pseudomembranous Candidiasis, Stevens-Johnson Syndrome.

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.

Stevens-Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered delayed-type hypersensitivity reaction to drugs.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 diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology.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.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. Key words: Stevens-Johnson syndrome; Toxic epidermal necrolysis; Drug reaction; Cyclosporine A

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.

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.

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.

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.