Stevens-johnson syndrome and toxic epidermal necrolysis standard reporting and evaluation guidelines (original) (raw)
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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Retrospective Analysis of 23 Patients
Acta Medica, 2021
Objective: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are dermatologic emergencies. There is a lack of consensus regarding appropriate management of SJS/TEN. The aim of this study was to evaluate demographic and clinical features, management and outcomes of SJS/TEN patients. Materials and Methods: The data of patients who were ≥18 years old and hospitalized with the diagnosis of SJS, SJS-TEN overlap and TEN at Hacettepe University, between 1992 and 2018 were analyzed retrospectively. Patient demographics, medications, time between the first causative drug intake and the onset of symptoms, mucous membrane involvement, treatment modalities including supportive measures, intravenous immunoglobulin, cyclosporine and systemic corticosteroids, duration of hospitalization, and mortality outcomes were recorded from patient charts. Results: A total of 23 patients (11 men; 12 women) with a mean age of 46.4 ± 19.5 years were included in the study. Twelve patients (52.2...
Faculty Reviews are written by members of the prestigious. They are Faculty Opinions Faculty commissioned and are peer reviewed before publication to ensure that the final, published version is comprehensive and accessible. The reviewers who approved the final version are listed with their names and affiliations. Abstract Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by detachment of the epidermis and mucous membrane. SJS/TEN are considered to be on the same spectrum of diseases with different severities. They are classified by the percentage of skin detachment area. SJS/TEN can also cause several complications in the liver, kidneys, and respiratory tract. The pathogenesis of SJS/TEN is still unclear. Although it is difficult to diagnose early stage SJS/TEN, biomarkers for diagnosis or severity prediction have not been well established. Furthermore, optimal therapeutic options for SJS/TEN are still controversial. Several drugs, such as carbamazepine and allopurinol, are reported to have a strong relationship with a specific human leukocyte antigen (HLA) type. This relationship differs between different ethnicities. Recently, the usefulness of HLA screening before administering specific drugs to decrease the incidence of SJS/TEN has been investigated. Skin detachment in SJS/TEN skin lesions is caused by extensive epidermal cell death, which has been considered to be apoptosis via the Fas-FasL pathway or perforin/granzyme pathway. We reported that necroptosis, i.e. programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, and its interaction with the formyl peptide receptor 1 induce necroptosis. Several diagnostic or prognostic biomarkers for SJS/TEN have been reported, such as CCL-27, IL-15, galectin-7, and RIP3. Supportive care is recommended for the treatment of SJS/TEN. However, optimal therapeutic options such as systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and TNF-α antagonists are still controversial. Recently, the beneficial effects of cyclosporine and TNF-α antagonists have been explored. In this review, we discuss recent advances in the pathophysiology and management of SJS/TEN. Switzerland 2 16 Jun 2020, (F1000 Faculty Rev):612 First published: 9 https://doi.
Therapeutic advances in drug safety, 2011
Epidermal necrolysis, whether involving limited, that is, Stevens-Johnson syndrome (SJS), or large, that is, toxic epidermal necrolysis (TEN), areas of the skin surface, is associated with high mortality. A specific prognosis score (SCORTEN) accurately predicts mortality. Management of SJS and TEN mainly relies on supportive care but several treatments have been proposed, particularly corticosteroids and intravenous immunoglobulins (IVIG), to block the progression of the disease. The aim of this study was a pooled analysis of published series addressing the question of treatment efficacy by comparing the mortality observed to the mortality predicted by the SCORTEN score. A literature search was carried out through PubMed from January 2001 to December 2009. It found 47 original series including at least 10 patients. Among these, 13 containing a clear description of treatment(s) and an evaluation of SCORTEN score were analyzed allowing the calculation of a mortality ratio (MR) for eac...
Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature
Annals of Allergy, Asthma & Immunology, 2005
Objective: To perform a comprehensive review of Stevens-Johnson syndrome and toxic epidermal necrolysis. Data Sources: A MEDLINE search was performed for the years 1975 to 2003 using the keywords Stevens-Johnson syndrome and toxic epidermal necrolysis to identify relevant articles published in English in peer-reviewed journals. Study Selection: All clinical studies that reported on 4 or more patients, review articles, and experimental studies that concerned disease mechanisms were selected and further analyzed. Clinical reports that included fewer than 4 patients were selected only if they were believed to carry a significant message about disease mechanism or therapy. Results: Stevens-Johnson syndrome and toxic epidermal necrolysis seem to be variants of the same disease with differing severities. A widely accepted consensus regarding diagnostic criteria and therapy does not exist at present. Despite the recent experimental studies, the pathogenic mechanisms of these diseases remain unknown. Although progress in survival through early hospitalization in specialized burn units has been made, the prevalence of lifelong disability from the ocular morbidity of Stevens-Johnson syndrome and toxic epidermal necrolysis has remained unchanged for the past 35 years. Further progress depends on modification of the acute phase of the disease rather than continuation of supportive care. The available published evidence indicates that a principal problem in the pathogenesis is immunologic and that immunomodulatory intervention with short-term, high-dose intravenous steroids or intravenous immunoglobulin holds the most promise for effective change in survival and long-term morbidity. Conclusions: The results of this review call for a widely accepted consensus on diagnostic criteria for Stevens-Johnson and toxic epidermal necrolysis and multicenter collaboration in experimental studies and clinical trials that investigate disease mechanisms and novel therapeutic interventions, respectively.
Journal of Investigative Dermatology, 2013
for the RegiSCAR study group Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous adverse reactions that are of major concern because of high mortality rates. On the basis of data collected in the RegiSCAR study, the aim was to assess risk factors (including modalities of patient management) for mortality, regardless of the cause, up to 1 year after the reaction. Within this cohort, the mortality rate was 23% (95% confidence interval (CI) 19-27%) at 6 weeks and 34% (95% CI 30-39%) at 1 year. Severity of reaction was a risk factor for mortality only in the first 90 days after onset, whereas serious comorbidities and age influenced mortality beyond 90 days and up to 1 year after onset of reaction. The risk of death for patients with identified drug cause was borderline lower than for patients with a reaction of unknown cause (hazard ratio 0.66, 95% CI 0.45-0.96). The study could not provide conclusive evidence regarding patient management. This large-scale population-based follow-up study of such patients confirmed high in-hospital mortality and revealed a remarkable number of deaths after discharge, which could mainly be attributed to severe comorbidities and older age, whereas the impact of severity of reaction on the risk of death was limited to the first few weeks.
Berkala Ilmu Kesehatan Kulit dan Kelamin, 2021
Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe eruptive skin reactions that can cause death. The incidence of SJS and TEN cases in the United States is 1.5–9.6 per 1,000,000 per year. Drugs are the primary etiology of SJS and TEN. Some drugs are at high risk and used frequently. The SJS and TEN mortality rates were relatively high, with SJS 4.8%, SJS / TEN overlap 19.8%, and TEN 14.8%. In Indonesia, there are lack of studies on the SJS and TEN. This study is needed to determine the epidemiological profile of SJS and TEN. Purpose: This study aimed to describe SJS and TEN patients' profiles. Methods: Drug-induced SJS and TEN cases from January 2016 to December 2019 were evaluated from the medical records patients' profile, incidence, suspected drugs, risk factors, and comorbidities of SJS and TEN were described. Result: There were 28 SJS and TEN patients, comprising of 24 SJS patients (85.7%), 3 TEN patients (10.7%), and 1 SJS overla...
Journal of The American Academy of Dermatology, 2020
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists (SDH) with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. 9-point Likert scale questionnaires regarding 135 statements were administered. The RAND/UCLA appropriateness method was employed to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5-9 and disagreement index ≤1 were included in the guideline. For the final round, the guidelines were appraised by all the participants. An evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN are included.
Seminars in cutaneous medicine and surgery, 2014
Severe cutaneous adverse reactions are associated with significant morbidity and mortality. They may be life-threatening for the affected patient and difficult to treat. Such conditions include toxic epidermal necrolysis (TEN), Stevens-Johnson syndrome (SJS), acute generalized exanthematous pustulosis and drug reaction with eosinophilia and systemic symptoms. Due to the fact that prognosis, etiology and treatment of the various reactions differ, a clear diagnosis based on the specific clinical pattern is important. This review will focus on the clinical presentations, pathogenesis, and important diagnostic and therapeutic considerations in the management of SJS and TEN.
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN): an update
South African Family Practice, 2016
Stevens-Johnson syndrome (SJS) is a form of toxic epidermal necrolysis (TEN) a rare but life-threatening hypersensitivity reactions that affect the skin and mucous membranes. The most common triggers are drugs, but they can also be triggered by infections. Granulysin has been recently identified as the major molecule responsible for the widespread keratinocyte necrosis. Early identification and removal of causative agent is crucial in preventing progression of condition and reducing patient mortality. Supportive care is often recommended over immunomodulating treatments as it helps improve patient outcome.
STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS: A SYSTEMATIC LITERATURE REVIEW (Atena Editora), 2022
Introduction: Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions, caused by late drug reactions in most cases. In general, the clinical picture includes fever, cough, eye irritation, erythematous or maculopurpuric skin reactions, which may progress to skin detachment, forming blisters. Goal: To address management and treatment possibilities for patients with SJS and TEN. Methodology: Systematic literature review performed by searching for eligible articles in the PubMed database and the following descriptors combined with Boolean operators: “Stevens-Johnson Syndrome AND toxic epidermal necrolysis AND treatment”. Results: After reading the selected articles, it was seen that the treatment for TEN and SJS includes treatment of the affected areas, supportive care and pain management. The most important initial step in management is the identification and discontinuation of all possible offending drugs. Discussion: In addition to dermatological disorders, SJS and TEN can also cause complications in various organs. In the case of non-pharmacological treatment, supportive care is important, which is the mainstay of treatment for patients with SJS/TEN and includes discontinuation of the causative drug, fluid and electrolyte management, nutritional assessment, supplemental oxygen, and treatment. of the wounds. Pharmacological therapy involves corticosteroids, immunoglobulins, cyclosporine, tumor necrosis factor inhibitors, among others. Conclusion: Finally, it became clear, in several studies, the importance of supportive care, both in the treatment of the disease and in the management of patients' pain, in addition to the best pharmacological choices.