Epidermal Necrolysis Research Articles

Anesthetic management of toxic epidermal necrolysis: a report of two cases

Toxic epidermal necrolysis (TEN), is an acute, life-threatening emergent disease involving the skin and mucous membranes with serious systemic complications. It is characterized by widespread epidermal sloughing. Drugs are the most common triggers of TEN, but infection, vaccination, radiation therapy and malignant neoplasms can all induce it in susceptible patients. We report two cases in whom a hair dye and a COVID-19 vaccine (BioNTech®, Pfizer) were believed to be the causative agents. These patients have to undergo repeated debridements of the necrotic tissue. In this manuscript the anesthetic management of TEN patients is discussed. Detailed preoperative evaluation, aggressive fluid and electrolyte replacement, avoidance of hypothermia during debridement, minimizing anesthetic agents and limiting traumatic procedures are key points in the management.
 Abbreviations: BUN: blood urea nitrogen; BSA: Body surface area; Cr: Creatinine; OR: Operating room; SJS: Stevens-Johnson Syndrome; TEN: Toxic epidermal necrolysis
 Key words: Anesthesia; COVID-19; Toxic epidermal necrolysis; Vaccine
 Citation: Bilge B, Erkilic E, İlhan G, Akin M, Kanbak O. Anesthetic management of toxic epidermal necrolysis: a report of two cases. Anaesth. pain intensive care 2022;27(1):135−138; DOI: 10.35975/apic.v27i1.2113
 Received: November 22, 2022; Reviewed: November 29, 2022; Accepted: December 02, 2022

Stevens-Johnson syndrome and toxic epidermal necrolysis: case series and brief literature review

Stevens-Johnson syndrome (SJS) and toxic epi- dermal necrolysis (TEN) are rare forms of delayed-type drug hypersensitivity reactions (DHRs) with extensive skin necrosis and exfoliation. Drugs are the main cause of SJS and TEN in both adults and children. The most common medications are allopurinol, aromatic antiepileptics and sulfonamides with seasonal, geographical and ethnic variations. The purpose of this paper is to present the cases of SJS/TEN which were successfully treated during the year of 2021 in our department and to provide a brief literature review.

Immune checkpoint inhibitor-induced epidermal necrolysis: A narrative review evaluating demographics, clinical features, and culprit medications.

Immune checkpoint inhibitors (ICIs) have transformed cancer treatment but can cause immune-related adverse events (irAEs). Severe cutaneous irAEs, including epidermal necrolysis, are rare but potentially life-threatening. There is limited understanding of the clinical features and management of ICI-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), so we aimed to analyze 95 cases of ICI-induced SJS/TEN (35 cases of SJS, 26 cases of TEN, two cases of SJS/TEN overlap, and 32 cases of unspecified) to increase knowledge of this condition among oncologists and dermatologists. We conducted a comprehensive search of PubMed for all relevant case reports published until the end of December 2022, and collected data on patient demographics, cancer type, ICI regimen, time to onset of SJS/TEN, clinical presentation, management strategies, and outcomes. PD-1 inhibitors were the most common ICIs associated with SJS/TEN (58.9%), followed by the combination of PD-1 and CTLA-4 inhibitors (11.6%), and PD-L1 inhibitors (6.3%). Lung cancer and melanoma were the most frequent malignancies treated (35.8% and 25.4%, respectively). SJS/TEN occurred most frequently within the first 4 weeks (51.7%), and corticosteroid monotherapy was the most commonly chosen systemic treatment (56.4%). The overall mortality rate of ICI-induced SJS/TEN was 30.8%. Our findings highlight the frequency and severity of ICI-induced SJS/TEN and the urgent need for predictive molecular biomarkers aimed at preventive measures and early intervention.

Case Report: Toxic epidermal necrolysis: a rare pediatric case report from Nepal

Toxic epidermal necrolysis (TEN) is a life-threatening cutaneous reaction to various medications like antibiotics and antiepileptics characterized by erythematous rashes, diffuse necrosis, and exfoliation of skin and mucous membranes, with a high mortality rate. Here, we present a case of six years old female child from Nepal who presented with fever and rashes, was diagnosed with measles and treated with cefixime, ibuprofen, and other drugs. Six hours after taking the drugs, the child developed generalized rashes, blisters formation, and peeling of the skin which progressed to cover most of the skin of her body within the next two days. She was then diagnosed with TEN and admitted to the Pediatric Intensive Care Unit (PICU) and treated by a multidisciplinary team with antibiotics, systemic steroids, antihistamines, wound care, and other medications. The child's fever subsided and her skin and oral lesions gradually regenerated. She was discharged after twelve days of hospitalization as she improved clinically and symptomatically. TEN is a dermatological emergency that should be diagnosed and treated promptly to minimize the fatal outcome of the disease.

Cefuroxime Axetil: An oral Prodrug of Cefuroxime Sodium

Cefuroxime axetil is the oral prodrug formulation of the injectable antibiotic cefuroxime sodium. Cefuroxime is a second generation cephalosporin antibiotic with a broad spectrum activity and a poor absorption profile from gastrointestinal tract. It led to the synthesis of the 1-acetoxyethyl ester of cefuroxime (cefuroxime axetil). Bactericidal activity in vivo is resultant of its binding to essential target proteins, termed the penicillin-binding proteins. Cefuroxime axetil is generally well tolerated. Stevens-Johnson syndrome, toxic epidermal necrolysis, headaches, hypersensitivity reactions and pseudomembranous colitis have also been observed occasionally during cefuroxime axetil treatment. It is not the drug of choice for any infection, particularly those encountered in the field of obstetrics and gynecology. It is safe to use in pregnancy and has a low adverse effect profile.

Quantifying the clinical and economic burden of desquamating dermatological conditions: Implications for a supraregional burns centre

IntroductionThis study sought to identify the spectrum of desquamating skin diseases referred for tertiary burns care and quantify the care requirements and expense associated with caring for these patients within the burns service. MethodsPatient records were identified with non-burn induced skin loss between 2016 and 2022. Data was extracted from inpatient records, operative notes, dressing clinic records. A cost analysis was conducted using figures from the National Schedule of National Health Service Costs and our own unit-specific costs. Results20 patients were identified, with a median age of 46.5 and median TBSA of 30%. The mean length of stay was 21.2 days with 8/20 patients requiring intensive care. Overall mortality was 30%, rising to 50% if patients required ITU admission. Patients had a mean of 1.5 procedures under general anaesthesia and a mean operative time of 169minutes per patient. Post-operatively, a mean of 8.3 dressing changes were required per patient (range 1-21). 75% of patients were referred as suspected toxic epidermal necrolysis syndrome (TENS) yet, only 32% of patients histologically had TENS (32%), with linear IgA disease, pemphigus vulgaris, and bullous lupus comprising the other diagnoses. Cost analysis predicted a total cost to the unit of £1,422,106. ConclusionDesquamating dermatological diseases are life-threatening conditions with exhaustive care requirements. Our experiences highlight the importance of awareness of the range of desquamating skin conditions beyond TENS to enable optimum management, and the need to ensure adequate financial provisions to accommodate the care requirements mandated by these patients.

Adverse Drug Reactions in HIV/AIDS Patients on Highly Active Antiretro Viral Therapy: A Review of Prevalence

Antiretroviral therapy (ART) is associated with a variety of side effects, ranging from mild intolerance to lifethreatening side effects. Short-term adverse reactions include nausea, vomiting, diarrhea, rash, hypersensitivity reactions, urticarial reactions, erythema multiforme, toxic epidermal necrolysis or Stevens-Johnson syndrome, infection liver toxicity, drowsiness and vivid dreams. Long-term side effects include lipodystrophy, lipoatrophy, dyslipidemia, diabetes, and skin, nail, and hair abnormalities. When starting ART, HIV-infected patients may frequently experience a variety of side effects such as rash, hair loss, hypersensitivity syndrome reactions, urticaria or erythema multiforme, Stevens-Johnson syndrome, known as manifested by a short-term course of side effects. A large number of patients have abnormal blood sugar and lipid profiles when using all groups of ARV drugs despite their relatively young age; Hyperlipidemia has been associated with HIV PI use and is more common and more severe than hyperlipidemia in HAART-naïve patients. Dyslipidemia, hyperglycemia, and lipodystrophy are cardiovascular risk factors in these patients. Hypertension is associated with established risk factors and is common in HIV-infected individuals. However, long-term use of HAART/ARV is necessary to control HIV infection. Keywords: prevalence, adverse drug reaction, HIV, AIDS, HAART

Risk of serious skin and subcutaneous tissue disorders for nimesulide among the pediatric population: a jeopardy identified through the analysis of global individual case safety reports

ABSTRACT Background The safety reports arising currently on nimesulide are divulging the jeopardy of skin and subcutaneous tissue disorders (SSTDs). Research Design and Methods The global individual case safety reports on nimesulide-induced SSTDs available at VigiBase® were analyzed up to 31 March 2023. Disproportionality analyses viz. Proportional Reporting Ratio (PRR), Reporting Odds Ratio (ROR), and Information Component (IC) were performed to identify the quantitative signals. Results Out of 33,983,649 de-duplicated cases available in the VigiBase®, 1,664,134 (4.9%) were in pediatrics below 12 years of age. Among these, cases attributed to nimesulide were 251, of which 126 (50.2%) were on SSTDs. Among all the SSTDs reported for nimesulide, the serious reactions like urticaria [PRR = 2.3; lower bound (LB) ROR = 1.7; IC025 = 0.6], Stevens-Johnson syndrome (SJS) [PRR = 28.3; LB ROR = 18.2; IC025 = 3.2], angioedema [PRR = 7.5; LB ROR = 4.5; IC025 = 1.7], and toxic epidermal necrolysis (TEN) [PRR = 27.4; LB ROR = 11.5; IC025 = 1.5] were identified as potential signals. In comparison with non-SSTDs, SSTDs reported for nimesulide were significantly higher among children (2–11 years, 90.5%), from India (38.9%), and by the physician (60.3%). Conclusions Identifying the giant quantitate association between nimesulide and serious & life-threatening reactions like SJS and TEN, precautionary measures need to be taken by the regulatory authorities to prevent nimesulide-induced SSTDs among the pediatric population.

Keynote-B68: Updated Efficacy and Safety of Pembrolizumab Every Six Weeks in Relapsed/Refractory Classical Hodgkin Lymphoma or Primary Mediastinal B-Cell Lymphoma

Background: Pembrolizumab 200 mg Q3W demonstrated robust antitumor activity and manageable safety in relapsed/refractory (R/R) classical Hodgkin lymphoma (cHL) and R/R primary mediastinal B-cell lymphoma (PMBCL), resulting in FDA approval. Recently, the FDA granted accelerated approval of pembrolizumab 400 mg Q6W in all approved indications based on data in solid tumors. The global Phase 2 KEYNOTE-B68 trial (NCT04875195) evaluates the efficacy and safety of pembrolizumab 400 mg Q6W in patients (pts) with R/R cHL or R/R PMBCL. We previously reported ORR of 65% for pts with R/R cHL, and 50% for pts with R/R PMBCL with approximately 9 months (mo) of follow up. Here we present data from 66 patients (pts) with an additional 6 mo of follow up. Methods: In this nonrandomized, open-label trial, pts aged ≥18 years with PD-1 inhibitor naïve R/R cHL or PMBCL received 400 mg pembrolizumab Q6W for ≤ 18 cycles, until progression, unacceptable toxicity, or withdrawal. Eligible pts with cHL must have relapsed or failed to respond after ≥1 prior lines of therapy or relapsed or failed to respond after ≥1 prior multiagent lines of therapy, or autologous stem cell transplant (ASCT). Eligible pts with PMBCL must have relapsed or failed to respond after ≥2 prior lines of therapy including rituximab and relapsed or failed to respond to or were ineligible for ASCT. The primary endpoint was ORR per Lugano criteria by investigator. Secondary endpoints were DOR per Lugano criteria by investigator and safety. Exploratory endpoints were PFS per Lugano criteria by investigator and OS. Data cut-off date was May 15, 2023. Results: At data cut-off, 66 pts (60 R/R cHL, 6 R/R PMBCL) were enrolled. Pts had a median age of 32.5 years (range, 19 to 85), and 35 (53%) were female. Overall, 47 (71%) pts discontinued treatment, 34 (52%) due to progressive disease, 8 (12%) due to ASCT, 2 (3%) due to adverse events (AE), 2 (3%) withdrawals and 1 (2%) due to physician decision. A total of 57 pts with cHL and 5 with PMBCL had ≥2 prior lines of therapy. The median follow-up was 15.7 mo (range, 7.8 -22.7) for pts with R/R cHL and 17.5 mo (range, 12.0 - 22.2) with R/R PMBCL. The ORR was 66.7% (95% CI, 53.3 -78.3 [35.0% CR; 31.7% PR]) for pts with R/R cHL, and 50% (95% CI, 11.8 -88.2 [33.3% CR; 16.7% PR]) for R/R PMBCL. The median DOR was 16.6 mo (range, 1.6 -17.0) for pts with R/R cHL and 9.7 mo (range, 2.6 -9.7) for R/R PMBCL. Treatment-related AEs occurred in 26 pts with R/R cHL and 2 with R/R PMBCL. The most common treatment-related AEs were hypothyroidism in 8 pts with R/R cHL and 1 with R/R PMBCL, and neutropenia in 3 pts with R/R cHL and 1 with R/R PMBCL. Grade ≥3 treatment-related AEs occurred in 3 (5%) pts with R/R cHL and 1 (17%) with R/R PMBCL. No grade 5 treatment-related AE occurred. Immune-mediated AEs occurred in 14 (23%) pts with R/R cHL and 1 (17%) with R/R PMBCL. Grade 3 infusion-related reactions and immune-mediated AE of toxic epidermal necrolysis occurred in 2 (3%) pts and 1 (2%) pt, respectively, with R/R cHL. No grade 4-5 immune-mediated AEs occurred in pts with R/R cHL and no grade ≥3 immune-mediated AEs occurred in pts with R/R PMBCL. Antitumor activity is summarized in the Table. Conclusions: Following 15 months of follow-up the ORR and PFS rates in the cHL patient population have increased, further highlighting the consistency to pembrolizumab 200 mg Q3W as observed in KN-087 and KN-204 patient populations. Additionally, no new safety concerns occurred in either cHL or PMBCL patient populations. This KEYNOTE-B68 trial further demonstrates the continued antitumor activity in patients and confirms the acceptability of Q6W dosing in heme indications.

Characteristics of adverse drug reactions due to nonsteroidal anti-inflammatory drugs: a cross-sectional study.

Nonsteroidal anti-inflammatory drugs (NSAIDs) are widely used for treating pain and inflammation. Spontaneous adverse drug reaction (ADR) reports represent a rich data source for the detection of unknown and rare ADRs. This cross-sectional study aimed to analyze the characteristics of ADRs due to NSAIDs in Thailand. All ADR reports of NSAIDs for systemic use from 2015 to 2019 were extracted from the national database in Thailand. Patient characteristics, drug use information, adverse event information, and source of senders in 32,857 reports were analyzed. The annual number of ADR reports due to NSAIDs decreased from 7,008 in 2015 to 5,922 in 2019. The most frequently reported drug was ibuprofen (n=12,645, 38.5%) followed by diclofenac (n=7,795, 23.7%), most patients were 40-59 years old, and the major adverse reaction was angioedema (n=7,513, 22.9%). Serious reactions were recorded in 20.7% (n=6,801) of the total ADRs. Most patients (n=20,593, 62.7%) recovered without sequelae, but there were 5,420 patients (16.5%) who could not recover and 3,109 patients (9.5%) who were recovering. Eight patients (0.02%) died of Stevens-Johnson syndrome (n=3), toxic epidermal necrolysis (n=4), and anaphylactic shock (n=1), which were possibly related to ADRs. The number of ADR reports due to NSAIDs decreased from 2015 to 2019 in Thailand. Serious ADRs and death cases accounted for 20.7% and 0.02%, respectively. Most fatal cases exhibited severe drug-induced skin reactions.

Cutaneous adverse drug reactions

Cutaneous adverse drug reactions (ADRs) represent a heterogeneous field including various clinical patterns without specific features suggesting drug causality. Maculopapular exanthema and urticaria are the most common types of cutaneous ADR. Serious cutaneous ADRs, which may cause permanent sequelae or have fatal outcome, may represent 2% of all cutaneous ADR and must be quickly identified to guide their management. These serious reactions include bullous manifestations (epidermal necrolysis i.e. Stevens-Johnson syndrome and toxic epidermal necrolysis), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Some risk factors for developing cutaneous ADRs have been identified, including immunosuppression, autoimmunity or genetic variants. All drugs can cause cutaneous ADRs, the most commonly implicated being antibiotics (especially aminopenicillins and sulfonamides), anticonvulsants, allopurinol, antineoplastic drugs, non-steroidal anti-inflammatory drugs and iodinated contrast media. Pathophysiology is related to immediate or delayed "idiosyncratic" immunologic mechanisms, i.e., usually not related to dose, and pharmacologic/toxic mechanisms, commonly dose-dependent and/or time-dependent. If an immuno-allergic mechanism is suspected, allergological explorations (including epicutaneous patch testing and/or intradermal test) are often possible to clarify drug causality, however these have a variable sensitivity according to the drug and to the ADR type. No in vivo or in vitro test can consistently confirm the drug causality. To determine the origin of a rash, a logical approach based on clinical characteristics, chronologic factors and elimination of differential diagnosis (especially infectious etiologies) is required, completed with a literature search. Reporting to pharmacovigilance system is therefore essential both to analyze drug causality at individual level, and to contribute to knowledge of the drug at population level, especially for serious cutaneous ADRs or in cases involving newly marketed drugs.

Osimertinib tolerance in a patient with Stevens Johnson syndrome during osimertinib therapy after treatment with pembrolizumab

BackgroundOsimertinib has emerged as an important tool in the treatment of non-small cell lung cancers (NSCLC) with certain activating mutations of epidermal growth factor receptor (EGFR). However, Osimertinib may cause adverse effects, including severe cutaneous adverse reactions (SCARs) such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The risk of certain adverse effects may be increased in the setting of recent use of immune checkpoint inhibitor (ICI) therapy, although it is unclear whether recent use of ICI therapy is a risk factor for Osimertinib-induced SJS specifically.Case presentationWe present a patient with EGFR L858R mutation-positive metastatic NSCLC who developed Osimertinib-induced SJS after recent administration of eight cycles of a pembrolizumab-containing chemotherapy regimen. Osimertinib, which was the best treatment targeting his lung cancer, was avoided due to history of SJS. Four years later, because of unresponsiveness or side effects of alternative treatments, he underwent Osimertinib challenge and tolerated it.ConclusionThis case highlights the importance of multi-disciplinary care and supports the hypothesis that the risk of SJS to Osimertinib is significantly higher in the context of recent administration of ICI therapy and, patients may tolerate Osimertinib after certain time has elapsed after the last dose of ICI.

Кератопротезы в современной офтальмохирургии

The shortage of donor corneas for keratoplasty in patients with severe disorders (persistent opacity, corneal leucoma of IV–V categories, including leucoma with vascularization, after burns, multiple transplant failures, with extensive symblepharon, Stevens – Johnson syndrome, Lyell's syndrome, ocular cicatricial pemphigoid, etc) is currently compensated by keratoprostheses, which partly or completely consist of synthetic materials, show good engraftment and high vision restoration efficacy. The article is a review of main scientific papers on keratoprostheses. The authors state characteristics of different types of keratoprosthesis, indications and outcomes of their use, complications, efficacy rate and postoperative visual acuity. Each of the presented types of keratoprosthesis has its own benefits and drawbacks, so their improvement to achieve better surgical outcomes remains relevant and needs further study. Key words: ophthalmic surgery, corneal opacity, keratoplasty, multiple transplant failures, keratoprostheses

Pharmacogenetics to prevent hypersensitivity reactions to antiepileptic drugs: is testing performed when indicated?

Extensive scientific evidence consistently demonstrates the clinical validity and utility of HLA-B*15:02 pre-screening in averting severe cutaneous adverse reactions (SCARs), namely Stevens-Johnson syndrome and toxic epidermal necrolysis, associated with carbamazepine or oxcarbazepine usage. Current practice guidelines and drug labeling actively advocate for pharmacogenetic pre-screening before initiating these antiepileptic drugs (AED), with particular emphasis on patients of Asian descent. However, there is a potential need to strengthen compliance with these recommendations. This retrospective study aimed to describe the pharmacogenetic pre-screening, documentation, and SCARs incidence for patients of Asian ancestry initiated on carbamazepine or oxcarbazepine at a large Northeastern USA healthcare system. Between 1 July 2016 and August 1, 2021, 27 patients with documented Asian heritage in the electronic health record (EHR) were included. The overall rate of HLA-B*15:02 pre-screening before carbamazepine or oxcarbazepine initiation was 4%. None who underwent pharmacogenetic pre-screening carried the associated HLA-B risk allele, and no SCARs were reported. Notably, pharmacogenetic results were not discretely entered into the EHR, and the results were only found as attached documents in the miscellaneous section of the EHR. There remains a significant opportunity for improving HLA-B*15:02 pre-screening for patients starting carbamazepine and oxcarbazepine to prevent SCARs in the USA.

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.

Validation of an Algorithm to Identify Epidermal Necrolysis on a Medico-Administrative Database

Validation of an Algorithm to Identify Epidermal Necrolysis on a Medico-Administrative Database

A Case of Japanese Spotted Fever Followed by Toxic Epidermal Necrolysis

A Case of Japanese Spotted Fever Followed by Toxic Epidermal Necrolysis

Tata Laksana Terintegrasi Sindrom Stevens-Johnson (SJS) dan Nekrolisis Epidermal Toksik (NET)

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening mucocutaneous reactions characterized by extensive epidermal necrosis and epidermal detachment. The incidence of SJS in Japan is 2.3 cases/100,000 people/per year. The mortality rate of SJS-TEN in Saudi Arabia is 1 per 10 cases (10%). SJS-TEN can be caused by drugs, idiopathic, infection, post-bone marrow transplant, immunization, radiotherapy, and genetics. The pathogenesis of SJS-TEN is suspected as a result of a cytotoxic reaction to keratinocyte cells which causes massive apoptosis. Clinical manifestation begins with prodromal symptoms followed by erythematous macular lesions and atypical target lesions that may confluence, then develop into bullous, oozing erosions with mucosal involvement. Vital sign observation is important to predict prognosis. A diagnostic procedure is necessary if another diagnosis is being considered. Management of SJS-TEN includes cessation of suspected drugs, early treatment such as fluid resuscitation, early nutrition, treatment and evaluation of eyes and skin lesions. Multiorgan involvement can occur in SJS-TEN so multidisciplinary management is needed for optimal therapy. Corticosteroids and cyclosporin A provide beneficial effects on SJS-TEN. SCORTEN is used to evaluate prognosis.

Development and Validation of a Novel Score to Predict Mortality in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: CRISTEN.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening, severe mucocutaneous adverse reactions. Severity prediction at early onset is urgently required for treatment. However, previous prediction scores have been based on data of blood tests. This study aimed to present a novel score that predicts mortality in patients with SJS/TEN in the early stages based on only clinical information. We retrospectively evaluated 382 patients with SJS/TEN in a development study. A clinical risk score for TEN (CRISTEN) was created according to the association of potential risk factors with death. We calculated the sum of these risk factors using CRISTEN, and this was validated in a multinational survey of 416 patients and was compared with previous scoring systems. The significant risk factors for death in SJS/TEN comprised 10 items, including patients' age of ≥65 years, ≥10% body surface area involvement, the use of antibiotics as culprit drugs, the use of systemic corticosteroid therapy before the onset, and mucosal damage affecting the ocular, buccal, and genital mucosa. Renal impairment, diabetes, cardiovascular disease, malignant neoplasm, and bacterial infection were included as underlying diseases. The CRISTEN model showed good discrimination (area under the curve [AUC]= 0.884) and calibration. In the validation study, the AUC was 0.827, which was statistically comparable to those of previous systems. A scoring system based on only clinical information was developed to predict mortality in SJS/TEN and was validated in an independent multinational study. CRISTEN may predict individual survival probabilities and direct the management and therapy of patients with SJS/TEN.

Cyclosporine Monotherapy in Pediatric Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: Preserving Sepsis Indicators While Promoting Recovery

Abstract Stevens–Johnson syndrome and toxic epidermal necrolysis are severe skin reactions seen commonly following exposure to drugs. The quest for the ideal drug to treat these conditions faces significant challenges. There is an absence of head-to-head, comparative, clinical, double-blinded trials comparing the various disease-modifying agents, owing to the rarity and severity of these conditions, along with ethical issues. In such a scenario, gathering data through clinical experiences can provide valuable insights for formulating future guidelines and improving the quality of care. The following case highlights the potential of cyclosporine A to preserve sepsis signs while demonstrating therapeutic efficacy, adding to the growing body of evidence in its favor.