Evaluation of the Factors Influencing Mortality in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Study of 166 Patients.

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. METHODS: Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. RESULTS: The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5-6 (odds ratio [95% confidence interval]: 13.902-507.537, p < 0.001) compared to SCORTEN 0-1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96-247.2, p = 0.012). CONCLUSION: Our study found that a high SCORTEN score within the first 24 h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.

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

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

RDW-SD and PCT Are Potential Prognostic Factors for In-hospital Death in Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

PURPOSE: Our study aimed to explore potential prognostic factors in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) patients from easily accessible laboratory data and to investigate whether the combination of these indicators with a score for toxic epidermal necrolysis (SCORTEN) can improve the predictive value. METHODS: Data from 85 SJS/TEN patients hospitalized from 2010 to 2021 were retrospectively analyzed. The primary outcome was in-hospital mortality. Univariate analysis was used to screen for laboratory indexes associated with death. Logistic regression was used to analyze significant risk factors for death. The differentiation and calibration of SCORTEN and modified score were assessed using receiver operating characteristic (ROC) curves and Hosmer-Lemeshow goodness-of-fit test. Net reclassification improvement (NRI) and integrated discrimination improvement (IDI) were used to evaluate the incremental prognostic value. RESULTS: Among the 85 patients (37 males, 48 females) aged 14-88 years, the mortality rate was 11.8% (n = 10). SCORTEN had good discrimination and calibration to predict mortality in this cohort of patients (area under the ROC curve [AUC] of 0.874, 95% confidence interval [CI], 0.758-0.990; Hosmer-Lemeshow goodness-of-fit test P = 0.994). Red cell distribution width-standard deviation index (RDW-SD) > 47.9 fL and procalcitonin (PCT) > 0.67 ng/mL were significant risk factors for death. When adding the 2 factors to SCORTEN, AUC was 0.915 (95% CI, 0.833-0.997), but not statistically different compared to SCORTEN alone (P = 0.091). The NRI was 1.2 (95% CI, 0.672-1.728; P < 0.001) and the IDI was 0.09 (95% CI, 0.011-0.173; P = 0.026), still suggesting that the modified score had better discriminatory and predictive power than SCORTEN alone. The modified score also showed good calibration (Hosmer-Lemeshow goodness-of-fit test, P = 0.915). CONCLUSIONS: SCORTEN is a good predictor of mortality in SJS/TEN patients in southwest China. Combining RDW-SD > 47.9 fL and PCT > 0.67 ng/mL with SCORTEN may enhance the ability to predict prognosis.

Updates in SJS/TEN: collaboration, innovation, and community.

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a predominantly drug-induced disease, with a mortality rate of 15-20%, that engages the expertise of multiple disciplines: dermatology, allergy, immunology, clinical pharmacology, burn surgery, ophthalmology, urogynecology, and psychiatry. SJS/TEN has an incidence of 1-5/million persons per year in the United States, with even higher rates globally. One of the challenges of SJS/TEN has been developing the research infrastructure and coordination to answer questions capable of transforming clinical care and leading to improved patient outcomes. SJS/TEN 2021, the third research meeting of its kind, was held as a virtual meeting on August 28-29, 2021. The meeting brought together 428 international scientists, in addition to a community of 140 SJS/TEN survivors and family members. The goal of the meeting was to brainstorm strategies to support the continued growth of an international SJS/TEN research network, bridging science and the community. The community workshop section of the meeting focused on eight primary themes: mental health, eye care, SJS/TEN in children, non-drug induced SJS/TEN, long-term health complications, new advances in mechanisms and basic science, managing long-term scarring, considerations for skin of color, and COVID-19 vaccines. The meeting featured several important updates and identified areas of unmet research and clinical need that will be highlighted in this white paper.

Linezolid and Ciprofloxacin-Induced SJS/TEN (Stevens-Johnson Syndrome/Toxic Epidermolysis Necrosis) Overlap in a Patient With Borderline Personality Disorder During a Single Hospital Stay: A Difficult Case to Manage.

SJS/TEN (Stevens-Johnson syndrome/toxic epidermolysis necrosis) is a T-cell mediated hypersensitivity syndrome in which cytotoxic CD8+ cells react against keratinocytes, resulting in widespread apoptosis and cell necrosis. About 90% of these cases are attributed to drug reactions, while 10% are idiopathic. The disease is classified according to body surface area (BSA) involvement and the thickness of epidermal loss. We report a case of a female with borderline personality disorder on antipsychotic medication, who developed SJS/TEN overlap after taking ciprofloxacin for her urinary tract infection (UTI). Her condition improved with meticulous management, but after switching her antibiotic from intravenous clarithromycin to oral linezolid, she developed SJS/TEN again, this time with more severe involvement. She received active management involving a multidisciplinary approach. Her condition improved slowly and, after one month, her lesions began to heal, and she was discharged with advice not to use both antimicrobial drugs in the future.

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

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

An Unusual Case of Stevens-Johnson/Toxic Epidermal Necrolysis Overlap Syndrome in HER2 (Human Epidermal Growth Factor Receptor 2)-Positive Breast Cancer Patient Treated With Docetaxel.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening drug-induced hypersensitivity reactions existing as a disease continuum based on the area of skin detachment. Following three cycles of treatment with docetaxel, a 60-year-old female with early-stage human epidermal growth factor receptor 2 (HER2)-positive breast cancer presented to the hospital with a flu-like illness and black crusting of the bilateral orbits, navel, and perianal region. Nikolsky sign was positive, and the patient was subsequently transferred to a specialized burn center for treatment of SJS/TEN overlap syndrome. There are a small number of cases documenting SJS/TEN following docetaxel administration in cancer patients.

An alternative model for assessing mortality risk in Stevens Johnson syndrome/toxic epidermal necrolysis using a random forests classifier: A pilot study.

Introduction: Mortality risk prediction is an important part of the clinical assessment in the Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) patient. The SCORTEN and ABCD-10 scoring systems have been used as predictive clinical tools for assessing this risk. However, some of the metrics required in calculating these scores, such as the total body surface area (TBSA) involvement, are difficult to calculate. In addition, TBSA involvement is calculated in a variety of ways and is observer dependent and subjective. The goal of this study was to develop an alternative method to predict mortality in patients with SJS/TEN. Methods: Data was split into training and test datasets and preprocessed. Models were trained using five-fold cross validation. Out of several possible candidates, a random forests model was evaluated as being the most robust in predictive power for this dataset. Upon feature selection, a final random forests model was developed which was used for comparison against SCORTEN. Results: The differences in both accuracy (p = 0.324) and area under the receiver operating characteristic curve (AUROC) (p = 0.318) between the final random forests model and the SCORTEN and ABCD-10 models were not statistically significant. As such, this alternative method performs similarly to SCORTEN while only requiring simple laboratory tests from the day of admission. Discussion: This new alternative can make the mortality prediction process more efficient, along with providing a seamless implementation of the patient laboratory tests directly into the model from existing electronic health record (EHR) systems. Once the model was developed, a web application was built to deploy the model which integrates with the Epic EHR system on the Fast Healthcare Interoperability Resources (FHIR) Application Programming Interface (API); this only requires the patient medical record number and a date of the lab tests as parameters. This model ultimately allows clinicians to calculate patient mortality risk with only a few clicks. Further studies are needed for validation of this tool.

Effectiveness and Safety of Early Short-Course, Moderate- to High-Dose Glucocorticoids for the Treatment of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Retrospective Study.

Objective: To summarise the clinical characteristics of patients with Stevens-Johnson syndrome/toxic epidermal necrolysis syndrome (SJS/TEN) and analyse the efficacy and safety of systemic glucocorticoid therapy. Methods: This study was a retrospective study of 56 patients with SJS/TEN who had been systematically treated with glucocorticoids in the dermatology ward of Peking University Third Hospital from 2010 to 2020. The clinical characteristics, treatment regimen, effects on underlying diseases, incidence and outcome of hormone-related adverse reactions and skin lesion prognosis were summarised and analysed for each patient. Results: ① The allergenic drugs were found to be antibiotics (31.51%), antipyretic and analgesics (21.92%), traditional Chinese medicines and health products (15.07%) and neuropsychiatric drugs (13.70%). ② Based on the 56 patients' scores of toxic epidermal necrosis at admission, the actual mortality rate was 1.8% (1/56), which was significantly lower than the average expected mortality rate of 15.0% (P = 0.032; standardised mortality ratio = 0.13; 95% confidence interval: 0.00-0.53). ③ A total of 33 patients (58.9%) had underlying diseases, of which 10 patients (30.3%) had underlying diseases that fluctuated during treatment but stabilised after symptomatic treatment. ④ During treatment, 73.2% (41/56) of patients had complications that may have been related to systemic glucocorticoids; 97.6% (40/41) had mild symptoms, and 92.7% (38/41) had improved/recovered complications at the time of discharge. Conclusion: ① Antibiotics are still the most common sensitising drugs, and traditional Chinese medicine and health products are also common sensitising drugs. ② Early systemic application of medium- to high-dose glucocorticoids is effective in the treatment of SJS/TEN, and it is beneficial in reducing mortality. ③ The short-term application of medium- to high-dose hormone therapy for SJS/TEN has little effect on underlying diseases. The related complications are mostly mild, and the treatment is safe.

Use of Intravenous Immunoglobulins and Systemic Corticosteroids in Patients with Toxic Epidermal Necrolysis: Experience of a Hospital in Mexico City. / Tratamiento con inmunoglobulina intravenosa y esteroides sistémicos en pacientes con necrólisis epidérmica tóxica: Experiencia en un hospital en Ciudad de México.

Toxic epidermal necrolysis is the most serious mucocutaneous adverse drug reaction. Multidisciplinary treatment and withdrawal of the causative drug are key to reducing mortality. Few studies have analyzed the use of systemic corticosteroids and intravenous immunoglobulins (IVIG) in patients with toxic epidermal necrolysis in Latin America. We describe our experience with 6 cases treated at a dermatology referral hospital in Mexico City. None of the patients died or developed complications in the short or medium term. The most widely used regimen was a combination of IVIG 1 g/kg for 3 to 5 days and methylprednisolone 1 g for 3 to 5 days. Mean hospital stay was 14.8 days. The combined use of systemic corticosteroids and IVIG seems to be a safe treatment option for patients with toxic epidermal necrolysis.

Combined use of cyclosporine in the treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis.

The exact efficacy of cyclosporine in the treatment of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) still needs evidence from more clinical data. This study was designed to compare the effectiveness and side-effects of combined use of cyclosporine in the treatment TEN with glucocorticoids (GC)/i.v. immunoglobulin G (IVIG). A total of 46 patients with SJS/TEN were enrolled and classified into two groups based on the therapeutic drugs used. Clinical characteristics, interventions, outcomes, and disease progressions were collected and compared between the two groups. In our cohort, seven patients eventually died and the overall fatality rate was 15.2%, but there was no difference between the two groups (p = 0.557). On discharge, the median SCORe of Toxic Epidermal Necrosis (SCORTEN) fell from 2.0 at admission to 1.0 and the median body surface area detached fell from 32.0% at admission to 9.5%. Patients in the cyclosporine group had a higher rate of re-epithelialized area than patients in the non-cyclosporine group (p < 0.05). Cyclosporine significantly reduced the length of stay (19.0 vs. 13.0 days, p = 0.019) and the rate of systemic infection (71.4% vs. 36.0%, p = 0.017) compared with the non-cyclosporine group. SCORTEN was the only significant risk factor for death and the risk ratio was 1.96 (1.17-3.31, p = 0.011). Conclusively, the combined use of cyclosporine could reduce the occurrence of systemic infection and accelerate the re-epithelialization.

Tratamiento con inmunoglobulina intravenosa y esteroides sistémicos en pacientes con necrólisis epidérmica tóxica: Experiencia en un hospital en Ciudad de México / Use of Intravenous Immunoglobulins and Systemic Corticosteroids in Patients with Toxic Epidermal Necrolysis: Experience of a Hospital in Mexico City

La necrólisis epidérmica tóxica es la reacción secundaria a medicamentos más grave dentro del espectro de las reacciones mucocutáneas. El tratamiento multidisciplinario es clave para disminuir la mortalidad de los pacientes, además de la suspensión del fármaco causal. Existen pocos estudios de tratamientos farmacológicos en pacientes con necrólisis epidérmica tóxica en Latinoamérica que combinen el uso de esteroides sistémicos e inmunoglobulina intravenosa (IgIV). Describimos 6 casos de pacientes con necrólisis epidérmica tóxica tratados con esteroides sistémicos e IgIV en un hospital de referencia dermatológica en Ciudad de México. Ningún paciente falleció ni presentó complicaciones a corto y mediano plazo de seguimiento. En la mayoría de los casos se empleó una dosis de IgIV de 1g/kg por 3-5 días y 1g de metilprednisolona por 3-5 días. El tiempo de ingreso hospitalario fue de 14,8 días. La combinación de esteroides sistémicos e IgIv parece ser una opción segura en pacientes con necrólisis epidérmica tóxica (AU)

Toxic epidermal necrolysis is the most serious mucocutaneous adverse drug reaction. Multidisciplinary treatment and withdrawal of the causative drug are key to reducing mortality. Few studies have analyzed the use of systemic corticosteroids and intravenous immunoglobulins (IVIG) in patients with toxic epidermal necrolysis in Latin America. We describe our experience with 6 cases treated at a dermatology referral hospital in Mexico City. None of the patients died or developed complications in the short or medium term. The most widely used regimen was a combination of IVIG 1 g/kg for 3 to 5 days and methylprednisolone 1 g for 3 to 5 days. Mean hospital stay was 14.8 days. The combined use of systemic corticosteroids and IVIG seems to be a safe treatment option for patients with toxic epidermal necrolysis (AU)

[Translated article] Use of Intravenous Immunoglobulins and Systemic Corticosteroids in Patients With Toxic Epidermal Necrolysis: Experience of a Hospital in Mexico City / Tratamiento con inmunoglobulina intravenosa y esteroides sistémicos en pacientes con necrólisis epidérmica tóxica: experiencia en un hospital en Ciudad de México

Toxic epidermal necrolysis is the most serious mucocutaneous adverse drug reaction. Multidisciplinary treatment and withdrawal of the causative drug are key to reducing mortality. Few studies have analyzed the use of systemic corticosteroids and intravenous immunoglobulins (IVIG) in patients with toxic epidermal necrolysis in Latin America. We describe our experience with 6 cases treated at a dermatology referral hospital in Mexico City. None of the patients died or developed complications in the short or medium term. The most widely used regimen was a combination of IVIG 1g/kg for 3–5 days and methylprednisolone 1g for 3–5 days. Mean hospital stay was 14.8 days. The combined use of systemic corticosteroids and IVIG seems to be a safe treatment option for patients with toxic epidermal necrolysis (AU)

La necrólisis epidérmica tóxica es la reacción secundaria a medicamentos más grave dentro del espectro de las reacciones mucocutáneas. El tratamiento multidisciplinario es clave para disminuir la mortalidad de los pacientes, además de la suspensión del fármaco causal. Existen pocos estudios de tratamientos farmacológicos en pacientes con necrólisis epidérmica tóxica en Latinoamérica que combinen el uso de esteroides sistémicos e inmunoglobulina intravenosa (IgIV). Describimos 6 casos de pacientes con necrólisis epidérmica tóxica tratados con esteroides sistémicos e IgIV en un hospital de referencia dermatológica en Ciudad de México. Ningún paciente falleció ni presentó complicaciones a corto y mediano plazo de seguimiento. En la mayoría de los casos se empleó una dosis de IgIV de 1g/kg por 3-5 días y 1g de metilprednisolona por 3-5 días. El tiempo de ingreso hospitalario fue de 14,8 días. La combinación de esteroides sistémicos e IgIv parece ser una opción segura en pacientes con necrólisis epidérmica tóxica (AU)

The Clinical Characteristics, Putative Drugs, and Optimal Management of 62 Patients With Stevens-Johnson Syndrome and/or Toxic Epidermal Necrolysis: A Retrospective Observational Study.

BACKGROUND: Case reviews of severe cutaneous adverse drug reactions (ADRs) such as SJS/TEN provide useful insights for clinical characteristics, putative drugs, and management protocols. PATIENTS AND METHODS: Medical charts of 62 (m:f- 20:42) patients with SJS/TEN hospitalized between 2010 and 2019 were analyzed retrospectively for clinical attributes, putative drugs and their indications, extracutaneous complications, and therapeutic outcome. The diagnosis was clinical based on established criteria. WHO-UMC scale for reporting ADR and ALDEN algorithm score were used for causality assessment. Therapies were customized based on in-house resources and affordability. RESULTS: Cases included were SJS (41.9%), SJS/TEN overlap (33.9%), and TEN (24.2%) aged 4-85 years. Complications included transaminitis (69.4%), lymphadenopathy (15.5%), septicemia (11.3%), and wound infections (4.8%). Aromatic anticonvulsants (37.1%), disease-modifying antirheumatic drugs (25.8%), antiretroviral drugs (12.9%), non-steroidal anti-inflammatory drugs (8.1%), antimicrobials (4.8%), and trihexyphenidyl (3.2%) were major putative drugs. The mean latent period was 16.6 days. The observed 8% mortality was because of primary comorbidities or multiorgan failure. Addition of fresh blood transfusion (BT, n = 11) or IVIg (n = 7) to systemic corticosteroids showed early relief in skin tenderness, improvement in general condition, and re-epithelialization. Only 16% of patients developed sequelae. CONCLUSION: Aromatic anticonvulsants, allopurinol, nevirapine, cotrimoxazole, paracetamol, and diclofenac remain the most implicated drugs. Sulfasalazine, leflunomide, ethambutol, and trihexyphenidyl were uncommon additions. A short course of high-dose dexamethasone in the early stage was useful. Addition of BT or IVIg provided rapid relief. Preexisting HIV disease, kidney disease, and sepsis remain important for in-hospital deaths. Retrospective study design and small number of cases remain major limitations.

Case Report: A successful case of toxic epidermal necrolysis treated with plasmapheresis therapy.

Toxic epidermal necrosis (TEN) is rare and can be life-threatening for patients. Appropriate management of TEN patients could give optimal results and prevent complications. One treatment modality for TEN is plasmapheresis, which is rarely available in most cases with severe TEN. Here we reported a successful treatment of severe TEN with plasmapheresis. A 40-year-old woman under tuberculosis therapy complained of shortness of breath that began four days prior to hospital admission and worsened ever since. The patient's skin was peeling with red spots and rashes all over the body for a week. During the examination, the patient was compos mentis, and the SCORTEN score was 2 with 12.1% risk of mortality rate. Dermatological examination of the face, trunk and extremities found extensive erosions, loose bullae filled with clear fluid, brown crusts, and generalized distribution with more than 30% epidermolysis. The patient was diagnosed with toxic epidermal necrolysis caused by antituberculosis therapy. We treated the patient by discontinuing the suspected drugs and administering the corticosteroids, but no improvement was observed. The patient underwent two cycle plasmaphereses with 5% albumin, resulting in 1.2 liter of plasma exchange. Re-epithelialization was observed after three days, and the patient was discharged on day 8. This case-report highlights the important role of plasmapheresis in treating the TEN patients. However, a study with larger sample sizes is warranted to validate the efficacy of plasmapheresis in TEN.

Mycoplasma-induced Stevens-Johnson syndrome/toxic epidermal necrolysis: Case-control analysis of a cohort managed in a specialized center.

BACKGROUND: Mycoplasma pneumoniae (MP) infection is associated with extrapulmonary complications such as Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN). OBJECTIVE: We evaluated the differences in epidemiology, clinical characteristics, and disease outcomes between drug-induced and Mycoplasma-related SJS/TEN. METHODS: All patients with SJS/TEN admitted to our center between 2003 and 2016 inclusive were treated under a standardized protocol. Comparative analysis was made between patients who tested positive for MP versus a control group with negative MP serology in the presence of high-notoriety drugs defined by an algorithm for assessment of drug causality in epidermal necrolysis >5. RESULTS: Of 180 cases of SJS/TEN patients treated in our institution, 6 had positive MP serologies and were compared to a control group of 71 cases of drug-induced SJS/TEN with an algorithm for assessment of drug causality in epidermal necrolysis score of >5. There were no significant differences in baseline characteristics, disease classification, body surface area involved, and extent of mucosal involvement. We found significant differences in mortality rates between the Mycoplasma and control groups on discharge (0% vs 22.5%, P < .001) and at 1-year follow up (0% vs 32.4%, P = .002), respectively. LIMITATIONS: Retrospective design, small sample size. CONCLUSION: Although recent studies have shown that MP-induced SJS/TEN is morphologically different and deserves a separate classification system, this would need to be borne out in larger prospective studies.

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.

Cephradine-Induced Toxic Epidermal Necrolysis: A Case Report of Treatment With Etanercept.

Toxic epidermal necrolysis (TEN) is a rare yet life-threatening severe cutaneous adverse reaction (SCAR) to various causative agents, including medications, vaccinations, infections, and malignancies, in addition to some other uncommon external stimuli. TEN is characterized by the sudden appearance of generalizeddusky erythematous targetoid macules with a purpuric center, which coalesces to form bullae and flaccid blisters, leading to an eventual sheet-like epidermal detachment of all necrotic areas. Extensive epidermal denudation in TEN is usually accompanied by thermoregulatory impairment, insensible fluid loss, and hemodynamic instability. The severity of presentation for TEN is calculated through the use of a "Severity-of-Illness Score for Toxic Epidermal Necrolysis" (SCORTEN) score. Certain medications, including antibiotics, anticonvulsants, corticosteroids, and nonsteroidal anti-inflammatory drugs, are considered the primary cause of this dermatosis. In this report, we describe a case of TEN caused by Cephradine, a first-generation cephalosporin antibiotic drug, in a 43-year-old South Asian male who presented to the emergency department one day after receiving Cephradine for the treatment of cellulitis. At presentation, this patient had a calculated SCORTEN score of 4 according to the SCORTEN criteria found in the literature, placing his mortality rate at 58%. His treatment plan consisted of a single 50mg dose of Etanercept (ENBREL), a soluble anti-tumor necrosis factor alpha inhibitor (TNF-α) monoclonal antibody, as an emergent intervention at presentation, along with cyclosporine and hydrocortisone in tapered doses. This is the first reported case of severe TEN in the Kingdom of Bahrain successfully treated with a TNF-α inhibitor, Etanercept in this case, achieving complete healing and remission within 20 days of presentation, after initially showing a poor prognosis and a high risk of fatality.

Toxic epidermal necrolysis as a rare and potentially fatal form of complication of systemic lupus erythematosus / Necrolisis tóxica epidérmica como forma infrecuente y potencialmente fatal de complicación del lupus eritematoso sistémico

Toxic epidermal necrolysis is an entity characterized by detachment of the epidermis. Its most described etiology is drug, it is also associated with other causes, mainly in patients who manifest an inadequate immune response. Systemic lupus erythematosus has been interpreted as a possible etiological cofactor, being an atypical presentation and difficult to manage. We present a case of a 54-year-old female with a history of systemic lupus erythematosus and abandonment of her treatment, was admitted due to acute diarrheal symptoms and 48 hours later she started with disseminated ulcerocostrous skin lesions with generalized epidermal detachment, severe eye damage and positive Nikolsky's sign. Despite the measures taken, she died 72 hours after being diagnosed due to septic shock. The case is presented because the epidermal toxic necrolysis that occurred as an infrequent and potentially fatal complication of systemic lupus erythematosus in a hospital with little experience in the care of these patients. The presence in this case of this dermatological disease, the absence of an obvious cause and a history of untreated lupus led us to consider that the patient manifested this severe dermatopathy as a complication of her underlying disease.      

La necrolisis tóxica epidérmica es una entidad caracterizada por desprendimiento de la epidermis. Su etiología más descrita es la medicamentosa, también está asociada a otras causas, fundamentalmente en pacientes que manifiesten una respuesta inmune inadecuada. El lupus eritematoso sistémico se ha interpretado como posible cofactor etiológico, siendo una presentación atípica y de difícil manejo. Se presenta un caso de una paciente femenina de 54 años de edad con antecedentes de lupus eritematoso sistémico y abandono de su tratamiento que ingresó por cuadro diarreico agudo y 48 horas después inició con lesiones en piel ulcerocostrosas diseminadas con despegamiento epidérmico generalizado, daño ocular grave y signo de Nikolsky positivo. A pesar de las medidas tomadas falleció a las 72 horas de diagnosticada debido a shock séptico. Se presenta el caso debido a que la necrolisis tóxica epidérmica ocurrió como una forma infrecuente y potencialmente fatal de complicación del lupus eritematoso sistémico en un centro hospitalario con poca experiencia en la atención a estos pacientes. La presencia de la enfermedad dermatológica, la ausencia de una causa evidente y el antecedente de lupus sin tratamiento llevaron a considerar que la paciente manifestó esta dermatopatía grave como complicación de su enfermedad de base.