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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

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

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]

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.

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.

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

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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.

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]

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. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

Toxic epidermal necrolysis is an extremely severe drug reaction, manifesting itself by widespread apoptosis of keratinocytes, generally considered to result from Fas/CD95–FasLigand interaction, but of unknown primary mechanism. We looked at the role of cells present in the skin blisters as probable effectors of this immune reaction. In a patient suffering from cotrimoxazole-induced toxic epidermal necrolysis, blister fluid cells were phenotyped by FACS and tested without prior restimulation for cytotoxicity on autologous and allogeneic cells in the presence of the drug. Blister fluid lymphocytes were predominantly CD8+, DR+, CLA+, CD56+ T lymphocytes, perforin positive and expressing preferentially two Vβ chains of the T cell receptor repertoire. These lymphocytes were cytotoxic only in the presence of the drug towards autologous EBV transformed lymphocytes and towards allogeneic cells sharing HLA-Cw4. Cytotoxicity occurred in the presence of either cotrimoxazole, sulfamethoxazole, or the nitroso metabolite of sulfamethoxazole, but not with the hydroxylamine metabolite of sulfamethoxazole. The lysis was blocked by an anti-MHC class I monoclonal antibody. It was abolished by EGTA and CMA, but neither by anti-fas, brefeldin A, nor by anti-TRAIL receptor monoclonal antibodies, strongly suggesting perforin/granzyme-mediated cytotoxicity, without implication of Fas or TRAIL at this stage. This is direct evidence that T lymphocytes present within the lesions of toxic epidermal necrolysis may exhibit, without any re-stimulation, a drug-specific cytotoxicity against autologous cells. Harboring the markers of classical CTL and MHC class I restriction these lymphocytes reacted against the parent drug and one of its reactive metabolites. These results challenge several current concepts and could support new therapeutic approaches. [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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Dobry, Himed, Waters and Kaffenberger.)

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., (Copyright © 2021 Euro-Mediterranean Council for Burns and Fire Disasters.)