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Summary: Stevens‐Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare, predominantly drug‐induced, acute life‐threatening diseases of skin and mucosae. SJS and TEN are nowadays considered as variants of one disease entity with varying degrees of severity called epidermal necrolysis (EN). EN is associated with high morbidity and mortality and constitutes a major disease burden for affected patients. The guideline "Diagnosis and treatment of epidermal necrolysis (Stevens‐Johnson syndrome and toxic epidermal necrolysis)" was developed under systematic consideration of existing scientific literature and in a formal consensus process according to regulations issued by the Association of Scientific Medical Societies in Germany (AWMF) to establish an evidence‐based framework to support clinical decision‐making. The interdisciplinary guideline commission consisted of representatives from various specialist societies and of patient representatives. The guideline is aimed at specialists in the fields of dermatology, ophthalmology, plastic surgery, intensive care, and pediatrics in hospitals and offices, as well as other medical specialties involved in the diagnosis and treatment of EN. The guideline is also aimed at patients, their relatives, insurance funds, and policymakers. The second part is concerned with the topics of supportive therapy in the acute phase of EN and outpatient follow‐up treatment. [ABSTRACT FROM AUTHOR]

Copyright of Journal der Deutschen Dermatologischen Gesellschaft is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Summary: Stevens‐Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare, predominantly drug‐induced, acute, life‐threatening diseases of skin and mucosae. SJS and TEN are nowadays considered variants of one disease entity with varying degrees of severity called epidermal necrolysis (EN). EN is associated with high morbidity and mortality and constitutes a major disease burden for affected patients. The guideline "Diagnosis and treatment of epidermal necrolysis (Stevens‐Johnson syndrome and toxic epidermal necrolysis)" was developed under systematic consideration of existing scientific literature and in a formal consensus process according to regulations issued by the Association of Scientific Medical Societies in Germany (AWMF) to establish an evidence‐based framework to support clinical decision‐making. The interdisciplinary guideline commission consisted of representatives from various specialist societies and patient representatives. The guideline is aimed at specialists in the fields of dermatology, ophthalmology, plastic surgery, intensive care, and pediatrics in hospitals and offices, as well as other medical speciallved in the diagnosis and treatment of EN. The guideline is also aimed at patients, their relatives, insurance funds, and policymakers. This first part focuses on the diagnostic aspects, the initial management as well as the immunomodulating systemic therapy. [ABSTRACT FROM AUTHOR]

The article describes a clinical case of a patient diagnosed with toxic epidermal necrosis - Lyell's syndrome, the mechanisms of disease occurrence, the treatment, and the provision of comprehensive health care. Aim of the study: To present the organization of nursing care aimed at alleviating the condition, safety and recovery of the patient in the treatment of Lyell syndrome in an intensive care clinic. Materials and Methods: A documentary method (desk-search) was used - scientific publications and documents dealing with the treatment of patients with Lyell's syndrome in the Clinic of Intensive Treatment of Acute Poisoning and Toxic Allergies at Naval Academy Hospital - Varna for the period 27/02/2023 – 12/03/2023 were studied. To collect relevant data on the topic, the author used a method of observation and description of a clinical case. Results and discussion: Analyzing scientific literature, documents and an observed clinical case, we found that the development of Lyell's syndrome starts from the moment the patient has taken the medication that caused it and ranges from a few hours to seven days. The dynamic processes that take place in these patients are associated with high fever, the appearance of erosions in the mucous membranes of the oral cavity, genital organs and the conjunctiva of the eyes. At the onset of primary, outward clinical signs, patients seek immediate medical attention and are hospitalized. The symptoms observed resemble signs of second-degree burns. The clinical case was from a hospitalized patient at the Naval Academy Hospital - Varna, and during the hospital stay clinical observation was performed by the nurse, laboratory and imaging tests were performed, their results were monitored and specific nursing care was applied. [ABSTRACT FROM AUTHOR]

Toxic epidermal necrolysis is a serious dermatological entity associated with multiple organ failure and high mortality. The most frequent etiology is related to the use of drugs. It begins with a prodromal picture, followed by a rash consisting of erythematous or erythematous-violaceous macules and plaques that progress to multiple blisters, leaving extensive denuded areas and mucosal involvement. It requires multidisciplinary management in the intensive care unit that includes the services of Dermatology, Internal Medicine, Critical Medicine, Plastic Surgery, Emergencies and Anesthesiology. The management of this disease is based on life support measures and targeted pharmacological treatment. Avoiding complications is a priority to improve survival. [ABSTRACT FROM AUTHOR]

Drug-induced toxidermia is an idiosyncratic adverse skin reaction that may become life-threatening in a small portion of patients, requiring intensive care unit (ICU) admission. The treatment recommendations are extrapolated from those of major burns, while prospective data remain sparse. The objective was to observe the application of these recommendations in patients treated in a burn ICU. Retrospective cohort study including patients requiring ICU between 2006 and 2020 in a tertiary university hospital. Inclusion criteria: Age >18 years. Patients were categorized as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN), or other toxidermia. Variables: severity scores, body surface area (BSA) involvement, nutritional and metabolic variables, trace element status, outcome variables. Descriptive statistics: median [IQR]. Altogether 35 patients were included (27 SJS/TEN and 8 "other") , aged 58 [48; 69] years. Skin involvement was 45% [30; 60] of body surface, 17 patients required mechanical ventilation, and length of ICU stay was 16 [6.5; 26] days. Hospital mortality was 23%. Fluid resuscitation requirements were moderate, despite intense inflammation (admission CRP (144 [89; 218] mg/L). The first 2 weeks' energy and protein intakes were below recommendations (p < 0.0001), lowest with oral feeding. Indirect calorimetry showed high energy expenditure in 11 patients (30.4 [23.9; 35.5] kcal/kg) resulting in negative energy balances (mean −245 kcal/day). Copper and zinc levels were below reference range during the first week, the low copper values being a novel finding. Trace elements should be monitored. The cohort was underfed with intakes lower than our ICU protocols, partly explained by short intubation times, and mucocutaneous involvement complicating the management and placement of feeding tubes. Oral feeding was least efficient and may become an indication for supplemental parenteral nutrition in the absence of an enteral feeding tube. ClinicalTrials.gov Identifier: NCT05320653 [ABSTRACT FROM AUTHOR]

Lyell syndrome or toxic epidermal necrolysis (TEN) is a severe mucocutaneous adverse drug reaction characterized by generalized erythema and widespread epidermal necrosis resulting in its detachment. If not appropriately intervened, it may result in sepsis, multiorgan failure, and death. TEN secondary to topical drugs is very rare and only a few cases have been documented so far. Here, we report a case of a 10-year-old child who presented with features of TEN following administration of ofloxacin eye drops, which is commonly prescribed in ophthalmic practice for conjunctival inflammation. She was treated in the pediatric intensive care unit with systemic corticosteroids, cyclosporine, intravenous immunoglobulin, and other supportive therapy including collagen dressing, and showed complete recovery. This case report highlights the need for awareness among physicians regarding serious adverse events caused by topical drugs and a multidisciplinary approach which paves the road for recovery.

Lyell syndrome or toxic epidermal necrolysis (TEN) is a severe mucocutaneous adverse drug reaction characterized by generalized erythema and widespread epidermal necrosis resulting in its detachment. If not appropriately intervened, it may result in sepsis, multiorgan failure, and death. TEN secondary to topical drugs is very rare and only a few cases have been documented so far. Here, we report a case of a 10-year-old child who presented with features of TEN following administration of ofloxacin eye drops, which is commonly prescribed in ophthalmic practice for conjunctival inflammation. She was treated in the pediatric intensive care unit with systemic corticosteroids, cyclosporine, intravenous immunoglobulin, and other supportive therapy including collagen dressing, and showed complete recovery. This case report highlights the need for awareness among physicians regarding serious adverse events caused by topical drugs and a multidisciplinary approach which paves the road for recovery. [ABSTRACT FROM AUTHOR]

Erythema multiforme (EM) also known as polymorph erythema is an acute skin disease of immunological nature with or without mucous membrane involvement, which may behave as chronic recurrent. It presents with distinctive targets like skin lesions, often together with ulcers or bullae in mucous membranes (oral, genital or ocular). Among its clinical forms are: a minor form characterized by a mild skin syndrome and its major form that manifests as a skin disease with marked mucosal damage. Among its main differential diagnoses are Stevens-Johnson Syndrome (SJS) and Lyell Syndrome (Toxic Epidermal Necrolysis (TEC)). It has an estimated incidence < 1%, with its major form being slightly more frequent than its minor form (0. 8-6 per million/year). It can occur at any age, presenting a peak incidence at the age between 20 and 30 years, with a slight predominance of males with a 3:2 ratio, without racial predilection. Its presentation in pediatric age is rare, even more so in early childhood. Minor recurrent EM is more common in this population. This paper reports a case of EM in the pediatric population as a rare form of exanthematic presentation, addressed at the Department of Pediatrics of the Complejo Asistencial Victor Rios Ruiz (CAVRR) in the city of Los Angeles, Chile this year. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome and Lyell syndrome are severe bullous drug reactions that can be life-threatening. The aim of this study is to describe the epidemiological, etiological, clinical, therapeutic and evolutionary data of patients hospitalized in our Dermatology Department. This is a retrospective descriptive study over a period of 10 years. All records of patients admitted to the Dermatology Department for these cutaneous adverse drug reactions were included. A total of 30 patients were recorded, with a male predominance. There were 18 cases of Lyell syndrome, 8 cases of Stevens-Johnson syndrome and 4 cases of overlap syndrome. The mean time to onset after drug administration was 7.5 days. The average skin area detached was 48%. Visceral involvement was frequently observed: pulmonary involvement, renal involvement, hepatic cytolysis and hematological involvement. The notion of medication was found in all our patients, with self-medication in 23% of cases. The reason for prescription was dominated by post-surgical anticonvulsant prophylaxis. All our patients received symptomatic treatment, and corticosteroid therapy was administered in only one patient for macrophagic activation syndrome. The mortality rate was of 17%. Skin area involved, presence of renal failure or respiratory distress were the main prognostic factors. [ABSTRACT FROM AUTHOR]

Abstract Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase. We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis. The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

Two cases of Lyell’s syndrome (toxic epidermal necrolysis) — a severe disease of the dermatovenereological profile of toxic-allergic nature with high lethality are considered. A favorable outcome of these clinical observations is due to the use of the so-called fluid bed in complex therapy. The development and introduction of new ways of treatment of such diseases is a critical direction in medical practice.

Cyclosporine 0.1% was used in a patient with Lyell syndrome, which had undergone a KeraKlear® keratoprosthesis implant due to the severe ocular involvement to avoid overuse of corticoid agents. To the best of our knowledge, this is the first reported case of cyclosporine 0.1% eye drops to use in Lyell syndrome previously treated with keratoprosthesis implant.

Abstract Epidermal necrolysis (EN) encompasses Stevens-Johnson syndrome (SJS,

Toxic epidermal necrolysis (TEN) is a devastating exfoliative disorder with life-changing complications. This study aimed to identify acute complications and long-term sequelae of TEN and to highlight the importance of a multidisciplinary management follow-up. A 19-year (1998–2016) retrospective review of all patients with TEN admitted to Cruces University Hospital´s Burns Unit was performed. Demographic and admission data were collected. Survivors were contacted for a follow-up multidisciplinary assessment involving dermatological, ocular, ENT, urological, gynecological, and psychological examination. Within the total cohort of patients analyzed (22), 6 survivors agreed to be interviewed. The patients presented with both physical and psychological sequelae, including dermatological (100%), oropharyngeal (50%), and ophthalmologic sequelae (50%), with corneal damage and severe dry eye as the most frequent. The only male patient underwent phimosis surgery due to mucosal adhesions. Psychometric scales captured symptoms of post-traumatic stress disorder (33.33%), and quality of life was affected in 66.67% of the patients by skin sequelae. Quality of life can be severely impaired by multiple long-term complications. It is important to emphasize the need for multidisciplinary awareness during their acute stay at Burns Unit. Furthermore, long-term follow-up might prevent or limit the progression of the chronic sequelae. [ABSTRACT FROM AUTHOR]

Background: Epidermal necrolysis (EN) involving ≥10% of the body surface area (BSA) is often complicated by bacterial infections.Objective: We sought to describe the epidemiology of bloodstream infections (BSIs) in EN involving a BSA ≥10% and the diagnostic performances of skin cultures for predicting the pathogen(s) isolated from BSIs.Methods: This retrospective single-center observational study was conducted between 2009 and 2017. All patients referred at the acute phase for EN involving a BSA ≥10% were included. All clinical and bacteriologically relevant data were collected (blood and skin cultures results, number, and severity and time of BSI). Sensitivity, specificity, and predictive values of skin cultures and impact of the bacterial inoculum were investigated.Results: Of 98 patients, 46 (46.9%) had ≥1 BSI episode during the hospital stay (BSIs were caused by Staphylococcus aureus [n = 17, 36.9%] and Pseudomonas aeruginosa [n = 17, 36.9%]). Skin cultures were concordant with blood cultures in 32 cases (71.1%). The positive and negative predictive values were 57.7% and 89.4% for S aureus and 50.0% and 80.9% for P aeruginosa, respectively. BSI increased with cutaneous inoculum of S aureus.Limitations: This was a retrospective single-center design with a low total number of BSIs.Conclusion: Skin cultures for S aureus and P aeruginosa may help predict the pathogens involved in BSIs. [ABSTRACT FROM AUTHOR]

Toxic epidermal necrolysis (TEN) or Lyell's syndrome is a rare, however, life-threatening mucocutaneous disorder with an epidermal detachment of a total body surface area (TBSA) of >30%. It is triggered by an idiosyncratic immune-allergic reaction to a drug, with many possible drugs implicated. Treatment success relies on early diagnosis and withdrawal of suspected/causative drug(s) and supportive care. Clinical evidence for specific therapies is still sparse. It is described a case of Lyell syndrome by sulfonamides for chemoprophylaxis of malaria. The patient presented with an extensive, rapidly evolving skin detachment, which progressed, despite supportive therapy, involving about 80% of TBSA. This led us to initiate a course of immunoglobulin with good clinical response. The aim of this work is to provide a discussion of the case and simultaneously make a practical literature review of TEN.

Cyclosporine 0.1% was used in a patient with Lyell syndrome, which had undergone a KeraKlear® keratoprosthesis implant due to the severe ocular involvement to avoid overuse of corticoid agents. To the best of our knowledge, this is the first reported case of cyclosporine 0.1% eye drops to use in Lyell syndrome previously treated with keratoprosthesis implant. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome and toxic epidermal necrolysis (Lyell syndrome) are rare diseases characterized by rapid blistering followed by extensive skin and mucosal exfoliation and constitutional symptoms. In most cases, drugs are the main triggers, but the etiopathogenesis of the diseases is not fully understood. Lyell syndrome is associated with a high mortality rate, reported to be around 35%. Therefore, early diagnosis requiring close interdisciplinary cooperation is essential. The diagnosis based on the clinical picture and a detailed pharmacological history should be confirmed by histopathological examination of the skin specimen, including analysis by direct immunofluorescence.

Le syndrome de Stevens Johnson et le syndrome de Lyell sont des réactions d'hypersensibilité médicamenteuses rares et potentiellement graves. Le but de ce travail est de décrire les aspects épidémiologiques, pharmacologiques, cliniques, thérapeutiques et évolutifs du spectre SSJ-SL. Il s'agit d'une étude rétrospective descriptive allant de 2011 à 2021 des données des patients hospitalisés au service de dermatologie du CHU Ibn Rochd. Nous avons recensé 27 cas (18 de SSJ 7 de SL et 2 Syndrome de Chevauchement). L'âge moyen était de 47 ans, avec une prédominance féminine (74 %). Les médicaments incriminés étaient l'allopurinol chez 37 % des cas, les AINS les antibiotiques (18 % chacun). Le délai moyen était de 21 jours. Tous nos patients avaient une atteinte de la muqueuse buccale, 74 % présentaient une atteinte conjonctivale. Les désordres biologiques étaient : une cytolyse hépatique dans 29 %, hypoprotidémie dans 29 %, une insuffisance rénale fonctionnelle dans 18 %. La prise en charge consistait à un arrêt immédiat de toute prise médicamenteuse, des soins locaux cutanés et muqueux, une correction des troubles hydro électrolytiques. Les principales complications étaient : passage à la forme Lyell chez 3 patients SSJ, dysphagie chez 5 patients, pneumonie chez 4 patients. Avec un taux de mortalité de 15 %. Au Maroc, le spectre de SSJ- SL présente les mêmes caractéristiques que les données des autres séries du monde : rareté, gravité, prédominance de l'âge adulte et du sexe féminin. L'imputabilité à l'allopurinol est la plus fréquente d'où l'intérêt de rationaliser la prescription de cette molécule. Stevens Johnson syndrome and Lyell syndrome are rare and potentially serious drug hypersensitivity reactions. The aim of this work is to describe the epidemiological, pharmacological, clinical, therapeutic and evolutionary aspects of the SSJ-SL spectrum. This is a retrospective analytical study from 2011 to 2021 of data from patients hospitalized at the dermatology department of CHU Ibn Rochd. Twenty-seven cases were identified (18 of SSJ 7 of SL and 2 SC). The average age was 47 years, with a female predominance (74%). The incriminating drugs were allopurinol in 37% of cases, NSAIDs and antibiotics (18% each). The average delay was 21 days. All our patients had oral mucosal involvement, 74% had conjunctival involvement. 29% had hepatic cytolysis. 29% had hypoprotidemia, 18% had functional renal failure. Management consisted of immediate cessation of all medications, a double antihistamine, local skin and mucous membrane care, and correction of electrolyte disorders. The main complications were: transition to the Lyell form in 3 SSJ patients, dysphagia in 5 patients, pneumonia in 4 patients. With a mortality rate of 15%. In Morocco, the spectrum of SSJ- SL presents the same characteristics as the data of other series in the world: rarity, severity, predominance of adult age and female sex. Allopurinol is the most frequent cause, hence the need to rationalize the prescription of this drug. [ABSTRACT FROM AUTHOR]

Toxic epidermal necrolysis (TEN) or Lyell's syndrome is a rare, however, life-threatening mucocutaneous disorder with an epidermal detachment of a total body surface area (TBSA) of >30%. It is triggered by an idiosyncratic immune-allergic reaction to a drug, with many possible drugs implicated. Treatment success relies on early diagnosis and withdrawal of suspected/causative drug(s) and supportive care. Clinical evidence for specific therapies is still sparse. It is described a case of Lyell syndrome by sulfonamides for chemoprophylaxis of malaria. The patient presented with an extensive, rapidly evolving skin detachment, which progressed, despite supportive therapy, involving about 80% of TBSA. This led us to initiate a course of immunoglobulin with good clinical response. The aim of this work is to provide a discussion of the case and simultaneously make a practical literature review of TEN. [ABSTRACT FROM AUTHOR]

The case report of a 60 years patient with a history of hypertension, reason why she had treatment with nifedipine, who went to the Emergency Room of "Orlando Pantoja Tamayo" Teaching General Hospital in Contramaestre, Santiago de Cuba, due to diarrheical stools, vomits, hyperthermia (38 °C) and generalized skin injuries in the type of erythemato-scabby pustules with flictenas and pain is described. The patient referred that she was accustomed to self-medication with co-trimoxazole due to repeated urinary infections and that she was consuming this medication for 3 days. The pathological study showed an epidermic toxic necrolysis (Lyell syndrome). In spite of the medical cares, she had an unfavorable clinical course and she complicated with an acute renal failure, leading to death. [ABSTRACT FROM AUTHOR]

A 16-year old girl with a 6 year history of valproate (Depakine Chrono) therapy for absence type epilepsy and 5 days history of Biseptol (Trimethoprim/Sulphamethoxazole) treatment for urinary tract infection (E. coli >106) developed an itchy papular exanthema of the neckline area with subsequent merging and spreading to the lower parts of the body. On the 7th day of Biseptol treatment, the girl was extremely restless, confused and complained of body pain, mainly of the head and the skin. A qualitative consciousness disorder kept progressing. Positive irritation signs of the meninges were present and hemorrhagic purpura developed on the abdomen and legs. A neurological examination confirmed the meningeal syndrome with a finding of strikingly generalized entire body painfulness and muscle weakness mainly of the lower limbs. The cerebrospinal fluid analysis revealed lymphocytic pleocytosis (CSF-Protein 0.96 g/l, reference range <0.2; Leucocytes 44/3, reference range <3/3) compatible with the diagnosis of aseptic meningitis. Due to negative culture, serologi-cal and molecular investigations, dermatomyositis or Guillain-Barré syndrome were considered. However, the clinical course of the disease and the results of skin biopsy with the focal desquamation of the upper layer of the epidermis with discrete lymphoplasmocytic infiltrates surrounding the blood vessels and lymphocytes in the interstitium led to the final diagnoses of drug induced aseptic meningitis and toxic epidermal necrolysis. Both diseases represent a rare complication of the adverse effects of pharmacotherapy in childhood and should be considered within the scope of the differential diagnostics in a child with aseptic meningitis and skin symptoms of unknown origin. [ABSTRACT FROM AUTHOR]

Objective: Toxic epidermal necrolysis is a rare disease that has very high mortality and morbidity. Despite the high mortality rate of the disease, treatment is still controversial today. In this study, we discussed the treatment of patients who were treated and followed up due to the toxic epidermal necrolysis in our hospital. Materials and Methods: This is a s tudy of children diagnosed as toxic epidermal necrolysis in our hospital between January 2011 and April 2013. We retrospectively analyzed response to the treatment. Results: We followed five patients in this period with a clinical diagnosis of toxic epidermal necrolysis. The median age at diagnosis was nine years (min: 2, max: 15), and three of them were male. Toxic epidermal necrolysis occured in three of the patients after the use of anticonvulsants, in one of the patients after use of antibiotic and in one of the patients after the use of antihypertensive. Three of the patients were given combination of intravenous immunoglobulin (IVIG) (1 g/kg) with corticosteroid (1 mg/kg/day), two of the patients only were given IVIG (1 g/kg/day). In two patients, corticosteroid therapy was given for five days. In one case who was given corticosteroid therapy for seven days and with lesions more severe according to SCORTEN developed gram-negative sepsis in clinical follow. Especially patients developed toxic epidermal necrolysis after anticonvulsant showed more severe clinic and longer recovery period. Conclusion: Improvement was observed in all patients. When only IVIG therapy compared to corticosteroid plus IVIG treatment, there were no superiority to each other. [ABSTRACT FROM AUTHOR]

Background: There are limited data on the use of skin testing, other than patch testing, and challenges in the evaluation of epidermal necrolysis (EN), including Stevens-Johnson syndrome and toxic epidermal necrolysis., Objective: To report a French multicenter experience in skin testing and challenges in EN, and investigate the factors associated with tests' positivity., Methods: All patients who were evaluated by patch tests (PTs), skin prick tests, intradermal tests (IDTs), or drug provocation tests (DPTs) for EN between 2010 and 2020 were retrospectively included through 2 French drug reaction networks., Results: In total, 113 patients were included from 8 centers. Median (interquartile range) time from EN to hypersensitivity workup was 7.9 months (5.1-15 months). All patients had PTs, 17 (15%) had skin prick tests or IDTs with delayed readings and 32 (28.3%) had DPTs. One mild reaction occurred after a DPT. Overall, 22 patients (19.5%) had positive PTs, and the only factors associated with positivity were Algorithm of Drug Causality for Epidermal Necrolysis (ALDEN) score and drug class. Only 1 IDT was positive but considered irrelevant. The DPTs were never performed to prove responsibility of a highly suspected drug but were used to confirm current tolerance of needed medications., Conclusions: Allergological workup in EN, performed by specialists involved in EN, seems safe. Skin tests, although of limited sensitivity, can be helpful for considering the reintroduction of essential drugs according to a benefit-to-risk decision. We propose an algorithm for approaching hypersensitivity testing in patients with EN, to be adapted to each patient., (Copyright © 2022 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Lyell's syndrome (toxic epidermal necrolysis) -- is a severe life-threatening disease of the skin and mucous membranes, demonstrating the extensive loss of epidermis, often developing after exposure to certain medications. Skin lesions are similar to those found in burn disease. A Nikolsky sign is easily demonstrated by applying lateral pressure to bullae. Lesions may include mucous membranes of the mouth, gastrointestinal tract, genitals, conjunctiva and epithelium of the respiratory tract. Mortality of Lyell Syndrome is 30-40%. Recent reports demonstrated the efficacy of treating a disease with cyclosporine A. We present the case of 32-year-old woman admitted to the Department of Skin Diseases of Provincial Hospital in Poznan with diagnosis of the Lyell Syndrome. On admission lesions of mucous membranes of the mouth, genitals and conjunctiva were present. Widespreading erythematous macules appeared over a period of hours. Skin lesions underwent dynamic progression. Epidermolysis was observed in large areas due to rupture of large blisters (Nikolsky sign positive). The intravenous treatment with high doses of corticosteroids and cyclosporine A was introduced. The patient has remained under the care of the Dermatology Clinic. The follow up revealed few hyperpigmented spots, patient's general condition was good, there was no evidence of side effects. Early implementation of immunosuppressive therapy supported by general multidirectional treatment resulted in complete withdrawal symptoms of the disease. [ABSTRACT FROM AUTHOR]

The overall immunopathogenesis relevant to a large series of disorders caused by a drug or its associated hyperimmune condition is discussed based upon examining the genetics of severe drug-induced bullous skin problems (sporadic idiosyncratic adverse events including Stevens--Johnson syndrome and Toxic epidermal necrolysis). New results from an exemplar study on shared precipitating and perpetuating inner causes with other related disease phenotypes including aphtous stomatitis, Behçets, erythema multiforme, Hashimoto's thyroiditis, pemphigus, periodic fevers, Sweet's syndrome and drug-induced multisystem hypersensitivity are presented. A call for a collaborative, wider demographic profiling and deeper immunotyping in suggested future work is made. [ABSTRACT FROM AUTHOR]

The overall immunopathogenesis relevant to a large series of disorders caused by a drug or its associated hyperimmune condition is discussed based upon the examination of the genetics of severe drug-induced bullous skin problems (sporadic idiosyncratic adverse events, including Stevens--Johnson syndrome and toxic epidermal necrolysis). An overarching pharmacogenetic schema is proposed. Immune cognition and early-effector processes are focused upon and a challenging synthesis around systems evolution is explained by a variety of projective analogies. Etiology, human leukocyte antigen-B, immune stability, dysregulation, pharmacomimicry, viruses and an aggressive ethnically differentiated 'karmic' response are discussed. [ABSTRACT FROM AUTHOR]

Résumé: Le léflunomide est un agent immunomodulateur qui a prouvé son efficacité dans le traitement de la polyarthrite rhumatoïde. Sa tolérance est globalement bonne. Quelques rares cas de syndrome de Lyell ont été rapportés. Observation: Mme L.A., âgée de 36ans, est suivie pour une polyarthrite rhumatoïde. Elle a été traitée successivement par la Salazopyrine®, le méthotrexate associé au traitement symptomatique. Devant la non-amélioration clinique et biologique le léflunomide a été prescrit. Deux semaines plus tard, la patiente a développé une éruption maculopapuleuse fébrile qui s’est rapidement étendue à l’ensemble des téguments avec des ulcérations de la muqueuse buccale et oculaire. Le léflunomide a été arrêté. La procédure du wash out associée à 60mg de prednisolone fut instaurée. Au bout d’un mois, les lésions cutanées ont cicatrisé mais la patiente a perdu la vue suite à une kératite ponctuée et la formation d’un pannus cornéen. Discussion: Les principaux effets indésirables du léflunomide comportent les diarrhées, les nausées, l’élévation des enzymes hépatiques, l’hypertension artérielle, l’alopécie et les réactions cutanées allergiques. Quelques rares cas d’effets secondaires cutanés sévères tels que le syndrome de Lyell ont été rapportés. Ces réactions cutanées imposent l’arrêt du médicament et son élimination immédiate par la procédure du wash out. Conclusion: En raison de la sévérité de ces réactions cutanées, il faut être vigilent lors de l’utilisation de ce médicament. [Copyright &y& Elsevier]

Background: Toxic epidermal necrolysis (TEN) is an uncommon but potentially life-threatening condition that involves sloughing of the skin at the dermoepidermal junction. TEN is a well-recognized syndrome and is part of a range of severe mucocutaneous intolerance reactions, mostly elicited by drugs and/or their metabolites. Reported mortality rates vary widely from 20 to 75%. Several systemic treatment protocols for TEN have been published; however, none has been formally standardized in a randomized controlled trial. The present study documents the current management principles and trends seen in 16 patients admitted with TEN or Stevens–Johnson syndrome over a 5-year period at the Concord Repatriation General Hospital Burns Unit. Method: Data were collected by retrospective chart review, and parameters included in the study were patient demographics, causative agents, percentage total body surface area and/or mucosal involvement, complications, treatment and outcome. In particular, dressing choice and documented healing of skin lesions were noted. Results: Sixteen patients were identified, with the beta-lactam antibiotics most commonly implicated as the cause. Complications tended to relate to degree of sepsis and/or mucous membrane involvement. Nanocrsytalline silver dressings, such as Acticoat™ (Smith & Nephew, Mount Waverley, Victoria, Australia), were used predominantly in more recent cases. Conclusion: The shift in dressing choice from traditional Vaseline-impregnated gauze coincided with a general trend towards the use of nanocrystalline silver dressings for superficial burns after 2003. The nanocrystalline silver dressings have shown considerable advantage over previously used dressings, with no adverse reactions noted and good healing of the skin lesions for all patients. [ABSTRACT FROM AUTHOR]

Background and Objectives: We reviewed our case-load of patients with Toxic Epidermal Necrolysis (TEN) and analysed this oncologic disease in order to define the prevalence of this comorbidity and find eventual clinical and prognostic differences, specific of this subgroup of patients. Materials and Methods: We reviewed charts from January 1995 to December 2005. Only those patients with a TEN diagnosis proved with an histologic examination were included. Causative drugs, symptoms, management and outcome were recorded and analysed. Results: We found 32 patients with TEN and 9 of them (28%) had also cancer. The comparison among oncologic vs. the rest of patients showed no significant differences in age, delay of referral, % surface area epidermal detachment, blood chemistry, immunoglobulins therapy and bacterial isolation of species throughout the recovery (p > 0.05). Conclusions: Oncologic diseases were the most frequent comorbidities in our series. There were no differences in the length of stay, duration of disease or mortality between patients with and without cancer. However, due to the small number of patients, future larger prospective studies are necessary to confirm these findings. [ABSTRACT FROM AUTHOR]

Abstract: Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are rare, drug-induced, severe acute exfoliative skin and mucosal disorders. Several treatments previously proposed have produced contradictory results in small series; in 1998 the use of intravenous immunoglobulins (IVIG) was introduced with excellent clinical findings. Our experience (1999–2005) using IVIG in the therapy of TEN/SJS, together with a local conservative approach, is reported and related to our previous treatments (1993–1998). The SCORTEN and the standardized mortality ratio (SMR) was used to evaluate the efficacy of our therapeutic modalities. Eight patients were treated before IVIG era and 23 patients have been treated with IVIG. There was no significant difference in SCORTEN between the two groups. Concerning the local approach, a conservative wound management in IVIG series replaced an extensive epidermal debridment and coverage with artificial skin substitutes of the pre-IVIG series. Overall mortality in patients treated before IVIG was 75% (6/8), in the IVIG group it decreased to 26% (6/23) with a cessation of further epidermal detachment after an average of 5 days (3–10 days) from the onset of the therapy. The SMR showed a trend to lower actual mortality (not significative) with IVIG treatment than the predicted mortality (SMR=0.728; 95% CI: 0.327–1.620). [Copyright &y& Elsevier]

Background and Objective: Toxic epidermal necrolysis and Steven Johnson syndrome are rare diseases that usually follow drug-exposures. The authors present one retrospective study with their management and focus their retrospective analysis on finding prognostic factors. Materials and Methods: We reviewed charts of admitted patients from January 1995 to December 2005. Only those with an histologic-proved diagnosis were included in the study. Causative drugs, symptoms, management and outcome were recorded and analysed. Results: We found 32 patients that met inclusion criteria. Mortality rate was 34.4% (11/32). Age, delay of referral, Total Burn Surface Area, white blood cells, creatinine, blood sodium, immunoglobulins therapy and more than two different types of blood bacterial species isolated were significantly correlated with death (p < 0.05). Conclusions: These data confirm prognostic factors already present in literature and find that the number of different bacterial species isolated from blood increase mortality. Further prospective studies are necessary to confirm these findings. [ABSTRACT FROM AUTHOR]

Abstract: A 51-year-old Caucasian woman developed severe drug-induced toxic epidermal necrolysis (TEN) due to allopurinol. The withdrawal of the culprit drug was unfortunately delayed, and dramatic retardation of reepithelialization was observed. At that stage of disease evolution, an inflammatory cell infiltrate was present in the dermis. Coverage of eroded lesions by frozen cultured keratinocyte allografts failed to hasten reepithelialization compared to ungrafted sites. This unusual protracted TEN evolution was followed by the development of extensive hypertrophic and keloid scars. Several biopsies were taken over 6 months. The histologic presentation of the grafted and ungrafted eroded scar tissues looked similar. Both the number and size of the Factor XIIIa-positive dermal dendrocytes, as well as the number of α-actin-positive myofibroblasts showed a marked increase between weeks 2 and 12 after grafting. They were reduced after 6 months when the scarring process was stabilized. α1 [IV] collagen was never expressed over the eroded scars. Similar to burn patients, delayed reepithelialization might be a risk factor for abnormal scarring in TEN. Cultured keratinocyte allograft apparently offered no improvement in reepithelialization and did not prevent abnormal scarring in this TEN patient. [Copyright &y& Elsevier]

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Copyright of Anaesthesist is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: The aim of this study was to describe the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children in dermatologyvenereology unit at National and Teaching Hospital of Cotonou., Methods: A retrospective and descriptive study was carried out for 10 years in dermatology-venereology unit at the National and Teaching Hospital of Cotonou to document the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children. It included all children aged from 0 to 18 years with clinical diagnosis of severe cutaneous adverse drug reactions. Drug imputability was based on the criteria of the French pharmacovigilance group., Results: Severe cutaneous adverse drug reactions accounted for 47.3% of paediatric cases (35/74 cases). The mean age was 9.3 years ± 5.2. The sex-ratio was 1.1. Self-medication was noted in 76.5% of children, on the initiative of parents in 66.7% of cases. There were 51.4% cases of Steven Johnson syndrome, 22.8% cases of Lyell syndrome, 8.5% cases of generalized and bullous fixed drug eruption, 2.9% cases of acute generalized exanthematous pustulosis and erythrodermic maculo-papular rash. Drug combinations was noted in 20% of cases. Penicillins (26.5%), paracetamol and sulfonamides (16.3%) were the drugs frequently incriminated., Conclusion: Steven Johnson syndrome and Lyell syndrome were the main severe cutaneous adverse drug reactions in children, mostly of school age. Penicillins, paracetamol and sulfonamides were the drugs frequently used and administered most often on self-medication., Competing Interests: The Authors declare that no competing interest exists., (Copyright © 2022 by West African Journal of Medicine.)