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Pictural representation of SJS, SJS-TEN overlap and TEN showing the surface of epidermal detachment (Adapted from Fig 21.9 Bolognia and Bastuji-Garin S. et al. Arch Derm 129: 92, 1993)
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Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mu...
Contexts in source publication
Context 1
... erosions) or detachable skin (Nikolsky positive) should be included in the evaluation of the extent of skin involvement. Bas- tuji-Garin et al. proposed classifying patients into three groups according to the degree of skin detachment (Table 1, Figure 1) [1]. ...Context 2
... including ocular, involvement develops shortly before or simultaneously with skin signs in almost all cases. To distinguish SJS, SJS-TEN and TEN the surface area of the detachment is the main discriminating factor ( Figure 1). Histological work up of immediate cryosections or conventional formalin-fixed sections of the skin reveal- ing wide spread necrotic epidermis involving all layers confirms the diagnosis. ...Similar publications
Toxic epidermal necrolysis is an idiosyncratic drug reaction which manifests with extensive epidermal detachment due to the massive keratinocyte apoptosis, mucous membrane involvement, and potentially lethal outcome. It is caused by adverse reactions to drugs, mostly idiosyncratic, unpredictable and independent of the applied dose, which develops 7...
Citations
... In the acute phase of SJS/TEN, patients may experience flu-like symptoms followed by a painful rash, blisters, and skin shedding, affecting mucous membranes and leading to complications such as sepsis and respiratory distress. 8 During the late phase with sequelae, patients can suffer from long-term complications such as skin hyperpigmentation or hypopigmentation, nail dystrophies, and serious ocular issues like severe dry eyes and vision loss. 8 In SJS/TEN, the lesions will initially appear as erythema to blackish red macules, purpuric macules, irregularly shaped which will progressively confluence and there are atypical lesions in the form of 'target' lesions. ...
... 8 During the late phase with sequelae, patients can suffer from long-term complications such as skin hyperpigmentation or hypopigmentation, nail dystrophies, and serious ocular issues like severe dry eyes and vision loss. 8 In SJS/TEN, the lesions will initially appear as erythema to blackish red macules, purpuric macules, irregularly shaped which will progressively confluence and there are atypical lesions in the form of 'target' lesions. The location of skin involvement is not limited to the body and face. ...
Background: Steven Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute and life-threatening skin diseases, commonly induced by medications. Study on SJS/TEN in Indonesia was still limited, hence knowledge about the profile and risk factors of SJS/TEN patients is still required to provide appropriate management and reduce patient mortality rate. This study aimed to determine the profile and risk factors of adult SJS/TEN patients in the inpatient installation of RSDM Surakarta. Methods: We conducted a cross sectional study using secondary data from medical records of SJS/TEN patients at the inpatient installation of Dr. Moewardi hospital, Surakarta from 2019 – 2022. Correlation tests on characteristics, comorbidity with length of stay (LoS) and discharge status were analyzed. Results: Of the total 147,531 inpatients, 35 (0.02%) of them were diagnosed with SJS/TEN, dominated by females (57.14%) with the mean of 45.74 years old. Most subjects were diagnosed with SJS (48.57%), followed by SJS/TEN (40.0%) and TEN (11.43%). The mean LoS was ± 8 days. Most subjects were discharged alive (85.71%). Paracetamol was the most common causative drug (25.71%), followed by cefadroxil (11.43%). Acute kidney injury (AKI) was the most common comorbidity (14.29%, p = 0.040). Spearman Rank test obtained no correlation between comorbidities and LoS (r = 0.028 ; p = 0.842) as well as discharge status (r = 0.063 ; p = 0.651). Conclusion: SJS/TEN is rare case with high mortality rate. Patients’ comorbidities have a very weak correlation with LoS and discharge status. Initial knowledge of the patient’s profile and risk factors including comorbidity and causative drugs can optimise comprehensive therapy for SJS/TEN patients.
... Estima-se que a incidência anual da SSJ varie de 1 a 6 casos por milhão de pessoas. Embora seja uma condição relativamente rara, a SSJ apresenta uma taxa de mortalidade significativa, que varia de 5% a 15%, e pode ser ainda maior em pacientes com complicações graves (Harr et al., 2020). ...
Introdução: A Síndrome de Stevens-Johnson (SSJ) é uma condição dermatológica rara, porém extremamente grave, caracterizada por lesões cutâneas bolhosas graves e envolvimento mucoso extenso. Esta revisão abrangeu diversas facetas dessa síndrome, desde suas manifestações clínicas até suas opções terapêuticas atuais, desafios diagnósticos e perspectivas futuras. Além disso, destacou a gravidade e a complexidade da SSJ, reconhecendo-a como um desafio significativo para a comunidade médica. A revisão abordou a tríade clássica de febre, lesões cutâneas bolhosas e envolvimento mucoso, destacando sua variabilidade clínica e o potencial de complicações graves. Metodologia: Envolveu uma abordagem sistemática de pesquisa, com identificação e revisão de artigos científicos relevantes sobre a SSJ em bases de dados acadêmicas. Termos de busca específicos foram utilizados para encontrar informações sobre manifestações clínicas, epidemiologia, fisiopatologia, opções terapêuticas, desafios diagnósticos e perspectivas futuras da SSJ. Discussão: Incluiu uma análise detalhada das manifestações clínicas da SSJ, sua epidemiologia, fisiopatologia e opções terapêuticas atuais. Foram discutidos os desafios emergentes no diagnóstico precoce, manejo clínico e prevenção da SSJ, juntamente com perspectivas futuras para melhorar os resultados clínicos e a qualidade de vida dos pacientes afetados. Conclusão: Enfatizou a importância da pesquisa contínua e da colaboração entre profissionais de saúde para enfrentar os desafios apresentados pela SSJ. Foi destacada a esperança de que avanços futuros na compreensão e no tratamento dessa síndrome possam melhorar significativamente os resultados clínicos e a qualidade de vida dos pacientes afetados.
... In SJS-TEN overlap, the detachment of the epidermis affects between 10% to 30% of the total BSA [8,1b]. The primary reason for the occurrence of most SJS-TEN cases is drug exposure, leading to a hypersensitivity reaction [9]. Roujeau et al. stated that certain medications pose a high risk of causing SJS-TEN when used for a short duration which includes trimethoprim-sulfamethoxazole and other sulfonamide antibiotics, aminopenicillins, cephalosporins, quinolones, and chlormezanone. ...
... The underlying mechanism behind the severe epidermal detachment observed in SJS, TEN is based on histopathological evidence of keratinocyte apoptosis followed by necrosis. During the early stage of the disease, blister fluid primarily contains CD8+ CTL, indicating an MHC class-I restricted drug presentation that leads to the clonal expansion of CD8+ CTLs [9]. Currently, the most compelling evidence indicates that the cytotoxic molecules FasL and granulysin play a significant role in the widespread apoptosis of keratinocytes observed in SJS and TEN [9]. ...
... During the early stage of the disease, blister fluid primarily contains CD8+ CTL, indicating an MHC class-I restricted drug presentation that leads to the clonal expansion of CD8+ CTLs [9]. Currently, the most compelling evidence indicates that the cytotoxic molecules FasL and granulysin play a significant role in the widespread apoptosis of keratinocytes observed in SJS and TEN [9]. Terbinafine is an antifungal medication that belongs to the allylamine class of antifungals. ...
The primary objective of this case report is to bring awareness among medical professionals regarding the potential cutaneous adverse reactions that may occur during the administration of terbinafine. SJS and TEN are uncommon, severe skin reactions characterized by extensive loss of skin and mucosal tissue, often accompanied by systemic symptoms. In more than 80% of cases, medications are identified as the causative agent for these reactions. SJS-TEN overlap syndrome is a very rare but severe cutaneous adverse drug reaction that is caused by terbinafine administration. We report a case of a 45-year-old female patient, with a documented history of uncontrolled diabetes and currently on medications, Metformin and Glimepiride, was admitted to a tertiary care hospital. The patient was suffering from vulvovaginal infection for which she was consuming terbinafine which led to the occurrence of cutaneous eruptions, across the right and left lower limbs, face, upper limbs, chest, back, arms, and abdomen, affecting almost the entire body covering 10- 30% of the body surface which makes it to fall under the category of SJS-TEN overlap syndrome. Even though terbinafine is thought to be safe, it can cause extremely serious adverse drug reactions, thus while prescribing it, appropriate safety measures must be performed. Patients having a history of drug reactions should not take the medication. The primary objective of this case report is to raise awareness among healthcare professionals about the potential for severe cutaneous drug responses induced by terbinafine and to instill vigilance in them regarding potential risks.
... 3 EM has a wide spectrum of clinical and histological manifestations, which has led to controversy over the distinction between EM, Steven Johnson syndrome, and toxic epidermal necrolysis. [4][5][6][7][8][9] Thus, oral lesions resulting from systemic diseases represent a very large clinical challenge in terms of therapy, mainly due to the relationship with autoimmune diseases that require treatment based on corticotherapy, which can cause several side effects to the patient. Low-level laser therapy (LLLT) was discovered in 1967 by Endre Mester at Semmelweis Medical University in Hungary. ...
Erythema multiforme is an autoimmune condition that can affect the skin and mucosa. Oral lesions initially present with edema and progress to superficial erosions with pseudomembrane formation. The most recommended treatment is the use of corticosteroids; however, low-level laser therapy can be effective in the treatment of erythema multiforme. We report a case of erythema multiforme in the oral mucosa treated with low-level laser therapy. A 73-year-old woman using alendronate for osteoporosis, losartan, and puran T4 with extensive ulcers on the upper and lower lips. The clinical diagnosis was erythema multiforme. The proposed treatment was 0.05% clobetasol propionate in gel, 3 times a day, and seven sessions of low-level laser therapy on alternate days. Low-level laser therapy significantly improved the erythema multiforme of the oral mucosa, offering the patient a non-invasive approach with no side effects.
... One hypothesis regarding the biological mechanism of SJS-TEN is that cytokines within blister fluid led to the recruitment of cytotoxic lymphocytes to the epidermis and the upregulation of Fas ligand (FasL), both responsible for cell apoptosis [13,14]. Analysis of blister fluid composition from patients with SJS-TEN has revealed high concentrations of secretory granulysin, FasL, TNF-α, perforin, and granzyme B [15][16][17] along with cytotoxic CD8+ T cells and natural killer-like cytotoxic T cells [16,18]. At the dermo-epidermal zone, high concentrations of CD14+ cells may increase CD8+ T cell proliferation and cytotoxicity [16,19]. ...
... Some argue that surgical management should be the standard treatment for SJS-TEN as this is a "burn-like" condition and, therefore, necessitates aggressive treatment to avoid burn-associated complications like infection [13]. Given that cytokines in blister fluid may recruit cytotoxic T cells to the epidermis and lead to further cell apoptosis [13,15,16,31], early removal of the blistered epidermis and blister fluid may help limit disease progression. ...
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immune-mediated skin reactions with high mortality as a result of severely compromised skin barrier function. Currently, there is no consensus on the topical management of these conditions. Some advocate for surgical debridement of affected skin as a means of preventing infection and facilitating reepithelialization with synthetic and biological wound coverage. Others prefer a conservative approach that relies on leaving the blistered skin in situ. A consensus is lacking, primarily due to the rarity of the disease and the lack of high-quality evidence supporting one particular form of management. The goal of this review is to explore and compare the two treatment approaches for SJS and TEN, namely conservative management and surgical debridement.
... Some drugs that have a high potential to trigger TEN are antibiotic drugs such as sulfonamide and cephalosporin groups, NSAID, and acetaminophen (paracetamol). 4,9,10 The patient received paracetamol for 3 days before admission to the hospital when the patient sought treatment at the primary health center. Then at the hospital, the patient received mefenamic acid on the first day of treatment. ...
Apart from allergy, TEN can be caused by infection, such as M. pneumoniae infection. Aim of this case report is to present clinical manifestations of TEN with pneumonia. A 37-year-old man, came to the emergency room with cough and fever since 5 days before hospitalized. The patient received intravenous ceftriaxone (with negative skin test), paracetamol, and n-acetylcysteine. Three days earlier, the patient had gone to primary health center and got paracetamol and n-acetylcysteine. On the first day of treatment, erythematous macules were seen on the anterior and posterior thoracic region, also the patient had sore throat and dysphagia, treated with intravenous dipenhydramine, mefenamic acid, and cetirizine. On the following day, the lesions expanded with multiple bullae on the anterior and posterior thoracic region, and erosion on the labia (BSA 28%). Intravenous methylprednisolone was administered, also Kloderma® and Ikagen® cream, and Kenalog®. Mefenamic acid and paracetamol were discontinued. On the third day of treatment, the lesions expanded (BSA 38%) and the next day, BSA reached 91.5%. The SCORTEN was 1. The patient was referred for treatment at the burn center and IVIg therapy. After the eleventh day of treatment at the referral hospital, the patient was fully recovered. The managements of TEN are stop suspected drugs, wound care, fluid therapy, systemic corticosteroids, and IVIg therapy. Appropriate management of TEN gives complete recovery to patient.
... SJS/TEN is a rare diagnosis associated with significant morbidity and mortality. Patients typically present with diffuse, erythematous eruptions, skin sloughing, and systemic sequelae, and diagnosis is confirmed by skin biopsy showing full-thickness dermal necrosis due to extensive keratinocyte apoptosis [5]. Limited cases hinder the establishment of clear SJS/TEN management protocols beyond discontinuing the offending drug, risking sepsis and death with delayed care. ...
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.
... Patients with 10% to 30% of their total body surface affected fall within the overlapping zone between SJS and TEN. A few of the most common medication classes known to cause SJS are antibiotics and antiepileptics [1][2][3][4]. An online search of the literature demonstrates many case reports of combined allopurinol and ACE inhibitors triggering severe hypersensitivity reactions, including SJS, and advises caution in using said medications concomitantly [5][6][7][8][9][10]. ...
Stevens-Johnson syndrome (SJS) is a severe and potentially debilitating skin reaction frequently related to medication use. Allopurinol and angiotensin-converting enzyme (ACE) inhibitors are commonly prescribed medications for prevalent health conditions worldwide, and their interaction associated with SJS warrants further investigation. A comprehensive literature search was performed to investigate cases as studies related to SJS occurring in patients with concomitant use of allopurinol and ACE inhibitors. We identified case reports and studies detailing hypersensitivity reactions, including SJS, attributed to a combination of allopurinol and ACE inhibitors. Despite the drug-drug interactions or lack thereof seen in patient populations, there is no definitive evidence of a pharmacokinetic interaction between allopurinol and ACE inhibitors. We were only able to find one case report specifically detailing SJS in a patient on combined ACE inhibitors and allopurinol. While the exact mechanism of the interaction is unclear, those reported cases of severe hypersensitivity reactions suggest a previous history of impaired renal function as a predisposing factor in the development of SJS. The potential risk of SJS with coadministration of ACE inhibitors and allopurinol is a drug-drug interaction that physicians should be aware of. This topic requires additional attention to determine if this drug combination should be avoided entirely in certain patients.
... Desquamating skin lesions can result from a number of conditions, including Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), pemphigus vulgaris, bullous pemphigoid, staphylococcal scalded skin syndrome, erythema multiforme major, and generalized fixed drug eruptions [1]. To differentiate such diseases in a patient presenting with symptoms such as conjunctivitis, erythematous macules, and mucosal erosions, urgent histopathology with immunofluorescence is often required due to the immediate danger of SJS/TEN [2]. Although a spectrum of drug toxicities exists and are mostly benign, SJS/TEN represents a life-threatening form of cutaneous drug reaction [3]. ...
Desquamating skin lesions are a non-specific finding that requires urgent evaluation given the life-threatening severity of one of the potential causes, Stevens-Johnson syndrome (SJS). Methotrexate toxicity, also known in its cutaneous form as methotrexate epidermal necrosis (MEN), is another entity that presents similarly to SJS and is described here in a patient with increased risk due to his age, chronic kidney disease, and increased dose of methotrexate. His diagnosis was complicated by other historical risk factors, including antibiotic use, but was eventually elucidated when he was noted to have bone marrow suppression. Given the pathophysiology of SJS, a T-cell mediated reaction, the patient’s leukopenia increased the likelihood of MEN as his ultimate diagnosis. However, in light of his aggressive treatment and non-specific histopathology, the clinical suspicion of MEN could not be confirmed.
... [1][2][3][4] Recent reports have shed light on various aspects of these syndromes, including their epidemiology, clinical manifestations, pathogenesis, and management. 1,[5][6][7][8] For instance, Kardaun indicated a higher incidence of SJS and TEN in older adults with a priority suggestion for the age-specific preventive measures. 1,7 Another study by Arici highlighted the importance of early recognition and prompt management of DRESS to avoid long-term complications. ...
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome and Stevens-Johnson Syndrome (SJS) are severe cutaneous adverse reactions to drugs. Those reactions which are rare in children can be especially severe and challenging to diagnose and manage. Herein we present a 59-month-old male who presented with a rash, fever, and multiple organ dysfunction initiation of Phenobarbital for epilepsy. Diagnosis of ovelaping SJS and DRESS syndrome had been made based on clinical manifestations accompanied with skin biopsy according to RegisSCAR diagnostic criteria. A therapy with intravenous immune globulin (IVIG), corticosteroids and supportive care was given successfully for the patient. This case underscored the significance of promptly and effectively recognizing and managing these intricate reactions.
