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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]

Tanaporn Anuntrangsee, Kumutnart Chanprapaph, Wimolsiri Iamsumang Division of Dermatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Wimolsiri Iamsumang, Division of Dermatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rajthevi, Bangkok, 10400, Thailand, Tel +662-201-1141, Fax +662-201-1211, Email i.wimolsiri@hotmail.comAbstract: D-penicillamine is used as the mainstay of chelation therapy for Wilson’s disease and for heavy metal intoxication. D-penicillamine itself has been noted to cause several systemic side effects as well as symptoms related to the skin. Common cutaneous side effects such as acute hypersensitivity reactions, elastic fiber abnormalities, and bullous diseases have been occasionally described. Herein, we report a case of a 23-year-old Thai female with gold intoxication who developed Stevens–Johnson syndrome (SJS) following the treatment of D-penicillamine. To our knowledge, D-penicillamine-induced SJS is exceptionally rare. To raise awareness of potentially fatal cutaneous adverse drug reaction triggered by D-penicillamine, published literature regarding SJS induced by this agent has also been reviewed. D-penicillamine should be regarded as a possible culprit in patients presenting with SJS following D-penicillamine administration and should be promptly discontinued.Keywords: chelating agent, cutaneous adverse drug reaction, cyanide, epidermal necrolysis, gold salt, penicillamine

Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), grouped together under the terminology of epidermal necrolysis (EN), are a spectrum of life-threatening dermatologic conditions. A lack of standardization and validation for existing endpoints has been identified as a key barrier to the comparison of these therapies and development of evidenced-based treatment. Following PRISMA guidelines, we conducted a systematic review of prospective studies involving systemic or topical treatments for EN, including dressing and ocular treatments. Outcomes were separated into mortality assessment, cutaneous outcomes, non-cutaneous clinical outcomes, and mucosal outcomes. The COSMIN Risk of Bias tool was used to assess the quality of studies on reliability and measurement error of outcome measurement instruments. Outcomes across studies assessing treatment in the acute phase of EN were varied. Most data came from prospective case reports and cohort studies representing the lack of available randomized clinical trial data available in EN. Our search did not reveal any EN-specific validated measures or scoring tools used to assess disease progression and outcomes. Less than half of included studies were considered “adequate” for COSMIN risk of bias in reliability and measurement error of outcome measurement instruments. With little consensus about management and treatment of EN, consistency and validation of measured outcomes is of the upmost importance for future studies to compare outcomes across treatments and identify the most effective means of combating the disease with the highest mortality managed by dermatologists. [ABSTRACT FROM AUTHOR]

BackgroundLong-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking.ObjectivesWe conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae.MethodsParticipants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method.ResultsFifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index

Epidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant institutional and geographical variations in treatment practices highlight the need for standardization of clinical assessment scores and prioritization of research outcome measures in epidermal necrolysis. At the present, clinical assessment is typically simplified to total body surface area (BSA) involvement, with little focus on morphology. Validated clinical scoring systems are used as mortality prognostication tools, with SCORTEN being the best-validated tool thus far, although the ABCD-10 has also been recently introduced. These tools are imperfect in that they tend to either overestimate or underestimate mortality in certain populations and are not designed to monitor disease progression. Although mortality is often used as a primary endpoint for epidermal necrolysis studies, this outcome fails to capture more nuanced changes in skin disease such as arrest of disease progression while also lacking a validated skin-directed inclusion criterion to stratify patients based on the severity of skin disease at study entry. In addition to mortality, many studies also use BSA stabilization or time to re-epithelialization as endpoints, although these are not clearly defined morphologically, and inter- and intra-rater reliability are unclear. More specific, validated cutaneous assessment scores are necessary in order advance therapeutic options for epidermal necrolysis. In this review, we summarize the strengths and weaknesses of current clinical assessment practices in epidermal necrolysis and highlight the need for standardized research tools to monitor cutaneous involvement throughout the hospitalization.

Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare spectrum of acute, mucocutaneous drug reactions characterized by epidermal necrosis of the skin and mucous membranes with progressive multiorgan failure. Cutaneous presentation of SJS/TEN is similar to that of acute graft-versus-host disease, creating a diagnostic dilemma in solid-organ transplant recipients presenting with diffuse, erythematous eruptions, skin sloughing, and systemic sequelae, reflective of both diseases. This case report details a 48-year-old woman post-orthotopic liver transplantation (OLT) who developed a diffuse, painful, morbilliform rash with progressive desquamation, along with corresponding pathological analysis indicative of SJS/TEN. There are few documented reports of SJS/TEN in solid-organ transplant recipients, and this case illustrates successful intervention and resolution of SJS/TEN in an OLT recipient while managing intraabdominal sepsis and an episode of acute rejection. Despite its rarity, prompt diagnosis of SJS/TEN and the implementation of tailored therapeutic strategies are crucial in the care of solid-organ transplant recipients. [ABSTRACT FROM AUTHOR]

Abstract Background Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index

Background: Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but potentially life‐threatening cutaneous adverse reactions. There is still no consensus on adjuvant treatments, and little is known about their effects on systemic inflammation in SJS/TEN. Our aim was to characterize the systemic and cutaneous immune profiles of SJS/TEN patients and to investigate whether/how intravenous immunoglobulins (IVIG), cyclosporine A (CSA), and best supportive care only (BSCO) affected the systemic immune signature and clinical outcome (6 week‐mortality, complications, hospitalization stay). Methods: We included 16 patients with SJS/TEN, treated with high‐dose IVIG (n = 8), CSA (n = 4) or BSCO (n = 4). Serial serum samples were obtained prior‐, 5–7 days, and 21 days after treatment onset. Serum levels of inflammation−/immune response‐associated proteins were measured by high‐throughput proteomics assay (OLINK) and cytotoxic molecules by ELISA. RNA extracted from skin biopsies collected prior treatment was analyzed by Nanostring. Results: Serum inflammatory profiles in SJS/TEN patients were notably characterized by massive upregulation of type 1 immune response and proinflammatory markers. Surprisingly, there was limited overlap between cutaneous and serum immune profiles. Serial serological measurements of immune response markers showed very diverse dynamics between the different treatment groups. IVIG‐treated patients showed completely different dynamics and most significant proteomic changes in an early phase (Day 5–7). In all treatment groups, type 1−/inflammatory response markers were dampened at day 21. Clinically, there were no outcome differences. Conclusion: Our study demonstrates that BSCO, CSA, and IVIG have very diverse biological effects on the systemic inflammatory response in SJS/TEN, which may not correlate with clinical outcome differences. [ABSTRACT FROM AUTHOR]

The treatment of epidermal necrolysis in pediatric patients remains a major challenge. Cyclosporine A has emerged as a promising therapy for epidermal necrolysis in adults; however, its efficacy in children is unclear. We present the case of a boy with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome who was initially resistant to methylprednisolone monotherapy but improved after receiving the combination of cyclosporine A and methylprednisolone. Published reports on the use of cyclosporine A for pediatric epidermal necrolysis are also briefly reviewed. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde 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.)

Generalized bullous fixed drug eruption (GBFDE) is the most severe form of fixed drug eruption and can be misdiagnosed as epidermal necrolysis (EN). We report the case of a 42-year-old male patient presenting with more than 50% skin detachment without defined areas of exanthema or erythema and a history of one prior event of EN caused by acetaminophen (paracetamol), allopurinol, or amoxicillin 1.5 years ago. The initial diagnosis was GBFDE or EN. The histology of a skin biopsy was unable to distinguish between the two diseases. The course of the disease, the later clinical presentation, and the medical and medication history, however, were in favor of a diagnosis of GBFDE with two potentially culprit drugs: metamizole and ibuprofen. Moxifloxacin, enoxaparin sodium, hydromorphone, and insulin human were administered concomitantly, which makes them suspicious as well. Unfortunately, the patient received an additional dose of metamizole, one of the possible causative drugs, and he developed another bullous reaction within 1 month. This led to the diagnosis of GBFDE due to metamizole. This report highlights the challenges of distinguishing two rare diseases and elucidates the importance of distinct clinical presentation and detailed medication history.

Epidermal necrolysis (EN) is a rare life-threatening condition, usually drug-induced and characterised by a diffuse epidermal and mucosal detachment. Calcinosis cutis is reported in various skin diseases, occurring preferentially with tissue damage, but has never been described in EN. Clinical, biological and histopathological characteristics of three patients were retrospectively obtained from medical charts. Immunohistochemistry of classical osteogenic markers was used to explore the pathogenesis of the calcifications; their chemical composition was determined by $\mu $Fourier transform infra-red ($\mu $FTIR) spectroscopy and their localization and morphology by field-emission scanning electron microscopy (FE-SEM). In a recent letter, part of the results of this investigation has been already presented. In this contribution, we have added original data to this previous letter. We have investigated a set of biopsies corresponding to patients who presented atypical healing retardation due to calcinosis cutis. Through FE-SEM observations at the nanometre scale, we describe different areas where are present voluminous calcifications at the surface, submicrometre spherical entities within the papillary dermis and then large “normal” fibres. FE-SEM observations show clearly that “large” calcifications are the result of an agglomeration of small spherical entities. Moreover, micrometre scale spherical entities are the results of an agglomeration of nanometer scale spherical entities. Finally, the last set of data seems to show that the starting point of the calcifications process is “distant” from the epidermis in part of the dermis which appears undamaged. Regarding the chemical composition of large calcifications, different $\mu $FTIR maps which underlined the presence of calcium-phosphate apatite have been gathered. Moreover, histopathology indicates that these pathological calcifications are not induced following a trans-differentiation of the skin cells into an osteochondrogenic phenotype. The association of caspofungin administration, known to induce in vitro intracellular calcium influx, and inflammation, induced by EN, known to favor dystrophic calcifications in various inflammatory skin diseases, could explain this never-before reported occurrence of calcinosis cutis.

The treatment of epidermal necrolysis in pediatric patients remains a major challenge. Cyclosporine A has emerged as a promising therapy for epidermal necrolysis in adults; however, its efficacy in children is unclear. We present the case of a boy with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome who was initially resistant to methylprednisolone monotherapy but improved after receiving the combination of cyclosporine A and methylprednisolone. Published reports on the use of cyclosporine A for pediatric epidermal necrolysis are also briefly reviewed.

Rare dermatological diseases cause great difficulties for sufferers. They impact their lives through visibility, pain, restrictive care and sometimes serious complications. This article describes the management of three rare dermatological diseases by caregivers trained and sensitized to these pathologies in centers of reference for rare dermatological diseases., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Stevens-Johnson syndrome (SJS) is a rare disease of the skin and mucous membrane. It develops as a result of a sudden skin reaction, which is most often triggered by the use of certain drugs. It is characterized by acute epidermal necrosis. The syndrome can lead to many complications and death. Currently, there are no guidelines and management schematics for this disease. Treatment has changed over the years due to the discovery of the possible pathomechanism of SJS. Nevertheless, because of the rarity of the disease, there is a lack of adequate prospective randomized studies that could provide valuable information on treatment. Objective: The purpose of this study was to present Stevens-Johnson syndrome, including clinical features, etiology, pathomechanism, complications and treatment. Methods: A literature search was performed in the PubMed medical publication database using the following keywords: Stevens-Johnson syndrome, acute drug-induced skin reaction, cutaneous adverse drug reactions. Results: Stevens-Johnson syndrome is an example of an acute cutaneous reaction to certain medicinal substances. The pathomechanism is not fully understood. There is an association of genetics with the risk of developing symptoms of the syndrome in selected populations. Treatment formerly was based on glucocorticosteroids, but now IVIG (intravenous immune globulin) is used. Dehydration, pneumonia or sepsis may develop as a complication of the syndrome. Conclusions: The discovery of a genetic predisposition to develop Stevens-Johnson syndrome offers the possibility of future effective disease prevention. It is necessary to create medical procedure schemes for the diagnosis and treatment of this disorder. The discovery of the possible pathomechanism has allowed the use of IVIG in the treatment of the syndrome. Effective and prompt diagnosis and treatment can prevent life-threatening complications and death from SJS.

Background: Possible biases in pharmacovigilance reporting may impact epidermal necrolysis (EN) and drugs associations. Objectives: To assess biases associated with EN‐reporting. Methods: Using VigiBase, the World Health Organization‐pharmacovigilance database, among drugs associated with EN between 2016 and 2020, we used an unsupervised clustering including reports characteristics, that is, reporter quality, time from drug intake to EN onset, and only one suspected drug in the report. Results: Among 152 drugs, three clusters were identified. Cluster 1 (n = 41) exhibited drugs frequently reported within a time from intake to onset longer than 4 days, in 57 ± 13% of reports. It corresponded to well‐reported drugs and was composed mainly of antivirals and antiepileptics. Cluster 2 (n = 42) exhibited drugs frequently reported within a time from drug intake to onset shorter than 4 days, in 31 ± 12% of reports. It corresponded to drugs with a high risk of protopathic bias and was composed of nonsteroidal anti‐inflammatory drugs (NSAIDs), analgesics, and antibiotics. Cluster 3 (n = 69) exhibited drugs frequently reported with an unavailable time from drug intake to reaction, in 66 ± 11% of reports, and reported by a high frequency of consumers (9 ± 9%). It corresponded to drugs reported with a high risk of classification bias, and was composed of anticancer therapies and cardiovascular drugs. Conclusion: Protopathic and classification biases impact EN‐reporting and should be considered regarding associations with antibiotics, NSAIDs, analgesics, anticancer therapies, and cardiovascular drugs. [ABSTRACT FROM AUTHOR]

Epidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant institutional and geographical variations in treatment practices highlight the need for standardization of clinical assessment scores and prioritization of research outcome measures in epidermal necrolysis. At the present, clinical assessment is typically simplified to total body surface area (BSA) involvement, with little focus on morphology. Validated clinical scoring systems are used as mortality prognostication tools, with SCORTEN being the best-validated tool thus far, although the ABCD-10 has also been recently introduced. These tools are imperfect in that they tend to either overestimate or underestimate mortality in certain populations and are not designed to monitor disease progression. Although mortality is often used as a primary endpoint for epidermal necrolysis studies, this outcome fails to capture more nuanced changes in skin disease such as arrest of disease progression while also lacking a validated skin-directed inclusion criterion to stratify patients based on the severity of skin disease at study entry. In addition to mortality, many studies also use BSA stabilization or time to re-epithelialization as endpoints, although these are not clearly defined morphologically, and inter- and intra-rater reliability are unclear. More specific, validated cutaneous assessment scores are necessary in order advance therapeutic options for epidermal necrolysis. In this review, we summarize the strengths and weaknesses of current clinical assessment practices in epidermal necrolysis and highlight the need for standardized research tools to monitor cutaneous involvement throughout the hospitalization.

Toxic epidermal necrolysis syndrome (TENS) is a rare medical emergency associated with high morbidity and mortality. At present, there is no agreement regarding specific treatments for this disease. The aim of this study is to determine the outcome of patients with TENS using a treatment protocol that did not include the use of intravenous immunoglobulin or systemic steroids. This is a retrospective study of all patients diagnosed with TENS admitted to a tertiary burns centre from 2015-2017. Twelve cases were identified with a mean age of 46±8 years. Mean total body surface area affected was 51±9%. The SCORTEN index was 3.4±0.3 (range 2-5) and predicted 3 deaths in this cohort. Overall mortality was 25%. This study casts no doubt on the prognostic validity of SCORTEN. No specific TENS treatment has been unequivocally proven to be effective. [ABSTRACT FROM AUTHOR]

Background: Epidermal necrolysis (SJS/TEN) is a rare but acute severe drug reaction associated with high morbidity and mortality rates. Aims: To describe the clinical, molecular, biochemical, and therapeutic profile of these patients. Methods: A total of 24 acute SJS/TEN patients were recruited during their hospital stay and detailed clinical history and treatment course recorded. Blood samples collected were subjected to DNA and serum separation for molecular and biochemical analysis. Results: Of 24 patients, 18 (75%) were females and six (25%) were males with six SJS, six SJS–TEN overlap, and 12 TEN cases. The inciting drugs were non-steroidal anti-inflammatory (87.50%; n = 21) followed by antibiotics (66.67%; n = 16), antiepileptics (37.50%; n = 9), and others (37.50%; n = 9). Seventeen patients (77.2%) showed skin eruptions within 7 days after drug intake. Different co-morbidities were observed in 22 (91.6%) and 20 (83.3%) patients showed ocular manifestations. Length of hospital stay ranged from 8 to 55 days, 20 (83.3%) patients were treated with corticosteroids, and four (16.6%) received antimicrobial therapy. Interleukin polymorphisms revealed significantly low frequency of IL-4 in the patients, HLA-A locus typing revealed higher frequency of HLA-A*3301 (20.8%), HLA-A*02 (25%), HLA-A*2402 (14.6%), and sera showed raised levels of granulysin and sFas L in the patients compared to controls. Conclusions: The preliminary study illustrates the clinical, molecular, and biochemical features of acute SJS/TEN and provides a better understanding that helps to improve patient care at an earlier stage. It also highlights the use of corticosteroids and antimicrobial therapy for effective treatment of patients.

Qin Xiang Ng,1,2 Michelle Lee Zhi Qing De Deyn,3 Nandini Venkatanarayanan,4 Collin Yih Xian Ho,2 Wee-Song Yeo,5 1Department of Medicine, National University Hospital, National University Health System, Singapore; 2MOH Holdings Pte Ltd, Singapore; 3Department of Medicine, James Cook University Hospital, Middlesbrough, UK; 4Department of Medicine, Nottingham University Hospitals NHS Trust, Queen’s Medical Centre, Nottingham, UK; 5Department of Paediatrics, National University Hospital, National University Health System, Singapore Background: Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are dermatologic emergencies with high morbidity and mortality risk. Cyclosporine, an immunomodulatory agent, is sometimes used off-label, and its role continues to be debated. This meta-analysis aimed to provide an update of current evidence and to clarify the role of cyclosporine in SJS/TEN treatment better.Methods: Using the keywords [cyclosporine OR cyclosporine OR ciclosporin OR CsA] AND [Steven-Johnson OR SJS OR toxic epidermal OR epidermal necrolysis OR TEN OR hypersensitivity OR dermatologic OR burns], a preliminary search on the PubMed, Ovid, Web of Science, and Google Scholar Database yielded 615 papers published in English between January 1, 1960 and July 1, 2017. The inclusion criteria for this review were: 1) published retrospective or prospective study (excluding single case reports); 2) patients with clinical diagnosis of SJS or TEN; 3) trial of cyclosporine treatment; and 4) available survival/mortality data.Results: A total of 12 studies, with a total of 358 SJS/TEN patients were reviewed. Two studies were excluded from the meta-analysis as they did not report SCORe of toxic epidermal necrosis/predicted mortality data; one was excluded because of possible data irregularities. Meta-analysis of nine studies revealed a significant reduction in mortality risk with cyclosporine therapy (standardized mortality ratio 0.320; 95% CI: 0.119–0.522; P=0.002). Cyclosporine was also generally well tolerated with little adverse effects or increased infection, albeit the patients tended to be critically ill. Publication bias was observed in the funnel plot and Egger test (P=0.0467).Conclusion: Currently available evidence are predominantly open trials and retrospective studies with a significant risk of bias, perhaps owing to the rarity and life-threatening nature of the condition. Given its immunomodulatory actions, cyclosporine could be a potential treatment option for SJS/TEN in addition to best supportive measures. Further confirmation with robust randomized, controlled trials or larger case series is necessary and should be encouraged. Keywords: SJS, TEN, epidermal necrolysis, cyclosporine, CsA, meta-analysis

Toxic epidermal necrolysis (TEN) is a potentially life-threatening, exfoliative disease. It is described as idiosyncratic, severe, skin reaction to drugs. With Stevens-Johnson's Syndrome, it presents as a continuum of a disease being categorized relating to the percentage of affected skin. Without any multicenter trials comparing TEN treatment modalities, there is dearth of strong evidence-based guidelines of care. Total plasma exchange with intravenous immunoglobulin (IVIG) is one among plethora of possible treatment strategies. In our 10-year experience, we have observed 21 patients admitted to our burns center due to TEN. All of them were placed under intensive care with daily plasmapheresis (TPE) and IVIG. We have observed 52% mortality, with observed severe concomitant diseases in every patient in nonsurvivor group (average Acute Physiology and Chronic Health Evaluation II score at admission: 31.5%). We consider that TPE with IVIG might be of use in selected group of patients with TEN without any severe comorbidities. However, further multicenter trials are needed because in some cases it may raise mortality. [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]

Copyright of Turkish Journal of Dermatology / Turk Dermatoloji Dergisis is the property of Galenos Yayinevi Tic. LTD. STI 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.)

The open abdomen is a well-known technique that is applied in a wide variety of clinical situations, including treatment of abdominal compartment syndrome, damage control laparotomy, and severe intraabdominal sepsis. Disease states such as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis or extensive deep partial and/or full-thickness torso burns involving the abdomen often result in a complete epidermal and partial dermal loss. When ABThera Open Abdomen Negative Pressure Wound Therapy is attempted in these patients, the exposed subcutaneous tissue rarely allows for an adequate seal between the adhesive barrier and the denuded skin. This is because successful placement of negative pressure wound therapy device necessitates having a dry epidermal surface allowing the adhesive tape to actually adhere. The ABThera Open Abdomen Negative Pressure Wound Therapy visceral protective layer was placed over the exposed bowel, followed by the blue perforated foam interface and then the adhesive barrier drape. Over the top of the "less-than-air-tight" adhesive barrier drape was placed a standard isolation transport bowel bag, which was stapled to the dermis, but outside of the previously placed adhesive barrier drape's diameter to "bolster" the adhesive barrier drape, only allowing the suction tube to extend inferiorly. This ABThera Open Abdomen Negative Pressure Wound Therapy ABNPWT held suction, even during multiple adjacent dressing changes, despite the exposed dermis. This case report reveals a unique approach that solves the issue of inadequate seal due to extensive burns to the torso and has significant benefit in burn and wound care practices. [ABSTRACT FROM AUTHOR]

Objectives: Nevirapine is used in developing countries for the treatment of HIV infection, but its use is associated with rare serious adverse reactions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Recently, an association between rs5010528 in the human leucocyte antigen (HLA)-C locus and SJS/TEN susceptibility has been described in sub-Saharan populations. Our aim was to verify this association in a population of nevirapine-treated patients from Mozambique.Methods: The rs5010528 SNP was analysed by direct sequencing in 27 patients who had developed SJS/TEN and 75 patients who did not develop adverse reactions after nevirapine treatment. A case-control association study was conducted. A multivariate analysis was performed in order to evaluate the role of HLA-C also in relation to other susceptibility genetic factors (CYP2B6, TRAF3IP2, HCP5, PSORS1C1 and GSTM1 genes).Results: rs5010528 was significantly associated with a higher risk of developing SJS/TEN; the variant allele was more frequent in cases than in controls, conferring a high risk of developing this adverse reaction in carriers (OR = 5.72 and P = 0.0002 at genotype level, OR = 3.51 and P = 0.0002 at allelic level). The multivariate analysis showed that the HLA-C SNP, CYP2B6 (rs28399499), TRAF3IP2 (rs76228616) and GSTM1 (null genotype) can explain 25% of the susceptibility to this reaction, with the HLA-C SNP as the most significant contributor (P = 0.02 and OR = 5.64).Conclusions: Our study confirmed the association of the rs5010528 SNP in the HLA-C region with susceptibility to developing SJS/TEN in a population from Mozambique, suggesting that it could be a good genomic biomarker for SJS/TEN susceptibility in different sub-Saharan populations. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, type IV hypersensitivity reactions of the skin and mucosa. These reactions (SJS/TEN) are frequently managed in burn units; however, no standardized guidelines exist for the treatment and management of SJS/TEN. To gain insight into current SJS/TEN management practices, a survey on admission, diagnosis, and management, was conducted across accredited burn units in the United States. A 28-item question survey on SJS/TEN management, diagnosis, and treatment practices was distributed among medical directors and co-directors of American Burn Association-verified burn centers. Responses were collected over a 6-week period. In total, 31 (48% response rate) burn unit medical directors/co-directors participated in the survey. The majority of responders indicate that acceptance to their burn unit is based on clinical suspicion of SJS/TEN (74%), and biopsy or dermatological evaluation is not required (67 and 87%, respectively). More than half (61%) of the burn units have their own SJS/TEN protocol in place. No consensus was observed on different treatment aspects, such as infection control, systemic treatment, and wound care. Most directors reported their burn units to consult ophthalmology (77%) and dermatology (54%) for the management of patients with SJS/TEN. Large variability in procedures of admission, treatment, and management of SJS/TEN was identified across burn centers. This study demonstrates the urgent need for SJS/TEN standardized guidelines in the United States. [ABSTRACT FROM AUTHOR]

Objective: To describe the history of toxic epidermal necrolysis, before and after the original report by the British dermatologist Alan Lyell in 1956. Methods: Subjective expert choice of key advances in the comprehension of the nosology, classification, causality, and mechanisms of epidermal necrolysis (EN) over more than a century. Results: Epidermolysis had been reported long before Lyell's paper, but most cases had likely been staphylococcal scalded skin syndrome in children. Concerning non-Staphylococcus EN, confusion with erythema multiforme dissipated and its relation to Stevens–Johnson syndrome was clarified. Tremendous advances were made in understanding the causes and mechanisms, with increased acceleration in the last 10 years. Conclusion: The next decade should be devoted to improve the prevention and management of a disease that is the most terrible form of drug hypersensitivity.

Severe cutaneous drug reactions also occur in children and range from reactions with blister forming of skin and mucosa to extensive exanthems with altered differential blood count and involvement of internal organs. The first group includes Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), which are considered as one disease entity with different degrees of severity and are also referred to as "epidermal" or "epithelial necrolysis" (EN). The group of drug reactions with primarily systemic alterations is represented by a condition known as drug reaction with eosinophilia and systemic symptoms (DRESS).Although EN is generally considered as a drug reaction, a causative agent can only be identified in about 50% of all pediatric cases. Once a clear diagnosis is established, specific treatment measures should be carried out, whereby discontinuation of the causative agent plays a decisive role in drug-induced cases. In order to be able to identify and discontinue the drug responsible, a detailed medication history must be obtained. Certain antiepileptic drugs, sulfonamides and sulfasalazine are among the most frequent triggers of EN and DRESS in children. Supportive therapy including appropriate topical treatment, pain management and ophthalmological consultations are of utmost importance in EN but a short-term immunomodulating treatment with cyclosporine A has been shown to be helpful. In contrast, in DRESS middle to long-term systemic treatment with glucocorticosteroids is recommended., (© The Author(s) 2023.)

Mycoplasma pneumoniae, one of the most frequent agents of community-acquired pneumonia worldwide, can affect different organs besides the lung, causing skin lesions in 25% of patients. The spectrum of dermatological features include macules, papules, purpura, target lesions and even well stablished conditions as erythema multiforme, Stevens- Johnson's Syndrome and toxical epidermal necrolysis. We present 3 pediatric patients with erythema multiforme and toxical epidermal necrolysis in which Mycoplasma pneumoniae was considered as the etiological agent. [ABSTRACT FROM AUTHOR]