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Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpt...
Citations
... Logo, é importante que a biópsia da pele seja realizada em todos os casos, para que a confirmação diagnóstica seja realizada (Noe & Micheletti, 2020). A identificação das características da SSJ/NET é fundamental para elucidação do diagnóstico clínico, sendo importante avaliar o aspecto das lesões (máculas com aspecto de clareira central, dois componentes mal delimitados e grandes áreas de descamação dolorosa nessas lesões, podem passar a ser recobertas por crostas), progressão (geralmente de início em face e tronco, com progressão centrífuga, com couro cabeludo geralmente não afetado), uso de fármacos gatilhos, envolvimento de superfícies mucosas e com rara recorrência após a retirada do medicamento causativo (Frantz et al., 2021). ...
... Dentre outras características, a palma da mão e a planta dos pés pode ser afetada e a sensibilidade cutânea é proeminente na SSJ/NET e, portanto, deve-se suspeitar da afecção em pacientes com extensa erupção mucocutânea dolorosa. Logo, a fase aguda e progressiva tem um período médio de sete a nove dias, evoluindo para estabilização, que progride para reepitelização da pele em cerca de 07 a 21 dias após (Hosegawa & Abe, 2020;Frantz et al., 2021). ...
... Classicamente, a histopatologia evidencia apoptose de queratinócitos basais na fase inicial, evoluindo para necrólise de espessura total e separação da epiderme na junção dermoepidermica com a progressão da doença, o infiltrado inflamatório é leve com predomínio de linfócitos T e a imunofluorescência direta é sempre negativa (Noe & Micheletti, 2020). Associado a isso, considerações devem ser feitas para possíveis diagnósticos diferenciais, como pênfigo vulgar, dermatose por IgA linear (DAL), síndrome da pele escaldada estafilocócica e eritema multiforme maior, erupção fixa medicamentosa, além de outras condições (Frantz et al., 2021). ...
Introdução: O uso de medicamentos pode causar reações adversas, que incluem manifestações cutâneas em variados graus. Quando essas manifestações são graves, tem-se um espectro de condições que inclui as síndromes Stevens-Johnson (SSJ) e da necrólise epidérmica tóxica (NET), que são caracterizadas por uma reação exacerbada de hipersensibilidade com consequente apoptose de queratinócitos da pele. O objetivo do trabalho é elucidar a associação com fármacos e os aspectos clínicos e do manejo dessas condições. Metodologia: Trata-se de uma revisão integrativa acerca das associações farmacológicas com SSJ/NET. Utilizou-se dados das bases Biblioteca Virtual de Saúde (BVS), Scientific Eletronic Library of Medicine (SciELO) e National Library of Medicine (PubMed), a partir do cruzamento dos descritores “Síndrome de Stevens-Johnosn”, “antibióticos”, “anticonvulsivantes” e “efeitos adversos” para responder à questão formulada através da estratégia PICO. Resultados e Discussão: Dentre os fármacos associados ao desenvolvimento de SSJ/NET, destacam-se os antibióticos, anti-inflamatórios, antirretrovirais e anticonvulsivantes, principalmente as sulfonamidas e penicilinas do grupo de antimicrobianos e os antiepilépticos aromáticos. Ainda não se sabe ao certo se as manifestações diferem em relação ao medicamento causador. O diagnóstico geralmente é clínico, com auxílio da história do paciente. Ferramentas podem auxiliar a definição da conduta e da estimativa do prognóstico. O tratamento consiste na abordagem de suporte e de possíveis opções farmacológicas, como o uso de ciclosporinas. Conclusão: Uma vez que trata-se de uma condição rara e potencialmente fatal, mais estudos são necessários para definição de protocolos de conduta e tratamento mais eficazes para o atendimento dos pacientes com SSJ/NET.
... The nature of reactions underlying SJS and TEN development is considered to be T-cell-mediated [5], with the principal role of T-cell receptors (TCRs). For example, public αβTCR demonstrated a binding affinity for carbamazepine, which is a well-known cause of SJS/TEN [6]. ...
(1) Background: Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are extremely severe cutaneous adverse drug reactions which are relatively rare in routine clinical practice. An analysis of a national pharmacovigilance database may be the most effective method of obtaining information on SJS and TEN. (2) Methods: Design—a retrospective descriptive pharmacoepidemiologic study of spontaneous reports (SRs) with data on SJS and TEN retrieved from the Russian National Pharmacovigilance database for the period from 1 April 2019 to 31 December 2023. Descriptive statistics was used to assess the demographic data of patients and the structure of suspected drugs. (3) Results: A total of 170 SRs on SJS and TEN were identified, of which 32.9% were SJS and 67.1%—TEN. In total, 30% were pediatric SRs, 21.2%—SRs of the elderly. There were 12 lethal cases, and all cases were TEN. The leading culprit drugs were anti-infectives for systemic use and nervous system agents. The top 10 involved drugs are as follows: lamotrigine (23.5%), ibuprofen (12.9%), ceftriaxone (8.8%), amoxicillin and amoxicillin with beta-lactam inhibitors (8.8%), paracetamol (7.6%), carbamazepine (5.9%), azithromycin (4.1%), valproic acid (4.1%), omeprazole (3.5%), and levetiracetam (3.5%). (4) Conclusions: Our study was the first study in Russia aimed at the assessment of the structure of the drugs involved in SJS and TEN on the national level.
... An area Photobiomodulation can improve tissue repair and immune response in these patients [6]. Ocular involvement can manifest as conjunctival hyperemia or even complete epidermal peeling of the ocular surface [7]. ...
... The diagnosis of SJS/TEN is based on clinical findings and other possible conditions should be excluded through complementary exams [7]. The obtainment of the patient's medical history is necessary, investigating all medications used in the previous eight weeks using the algorithm of drug causality for epidermal necrolysis (ALDEN score) described by Sassolas [13] to identify suspected medications. ...
Objective
To report an unusual case of toxic epidermal necrolysis treated with adjuvant photobiomodulation.
Case report
The patient presented blistering lesions affecting 90% of the body surface, with blistering lesions on the face, involvement of the conjunctival mucosa, trismus, prostration, and reduced diuresis. Initially, the patient underwent systemic treatment with medical support. Twenty-one days later, a dental evaluation was requested as there was no regression of oral lesions. Laser therapy sessions were performed with 2 J/point, an energy density of 66.66 J/cm? at four points: labial commissures, midline of the upper lip, and midline of the lower lip. On the seventeenth assessment, clinical improvement of facial and oral cavity lesions was observed, with the patient accepting an oral diet.
Conclusion
Photobiomodulation can be clinically effective in controlling pain and healing oral lesions, which may be an important factor contributing to the clinical improvement of the patient.
... For ABCD-10, the interpretation may vary, but generally, a higher score indicates increased severity and potentially increased mortality risk. However, specific mortality risk estimates based solely on the ABCD-10 score might not be as established compared to SCORTEN [7]. Causality assessment essentially involves determining whether there's a cause-and-effect relationship between a drug and a drug reaction. ...
... One of the accepted hypotheses regarding how drugs lead to the immunological reaction in SJS/TEN is the hapten/prohapten concept. According to this concept, small molecular drugs covalently bind to proteins in the serum, are recognized by certain HLA molecules, and activate T cells to produce an immune response [8,9]. ...
... Supportive care encompasses monitoring renal functions, fluid and electrolyte levels, ensuring adequate nutrition, pain control, maintaining skin integrity, and preventing infections. Treatment may need to be continued in a burn unit or intensive care unit [8]. Ensuring and maintaining enteral nutrition is crucial. ...
... Ensuring and maintaining enteral nutrition is crucial. Prophylactic antibiotics are not recommended, but appropriate wound care is critical in preventing secondary infections [8]. There is currently no consensus on immunomodulatory treatment for SJS-TEN. ...
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN.
Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022.
The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients.
While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5–6 (odds ratio [95% confidence interval]: 13.902–507.537, p < 0.001) compared to SCORTEN 0–1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96–247.2, p = 0.012).
Our study found that a high SCORTEN score within the first 24 h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.
... 4 Globally, SJS/TEN cases were estimated to affect 2-7 people per one million population annually, 3 with reported incidences varying widely by location. 5 The incidence of SJS, SJS/TEN and TEN cases in the adult population in the United States was 9.2; 1.6; and 1.9 cases per one million population per year from 2009-2012. Although the incidence is considered quite rare, the mortality of SJS/TEN cases was high, with SSJ mortality at 4.8-9%, followed by SJS/TEN at 19.4-29% and TEN at 14.8-48%. ...
... Although the incidence is considered quite rare, the mortality of SJS/TEN cases was high, with SSJ mortality at 4.8-9%, followed by SJS/TEN at 19.4-29% and TEN at 14.8-48%. 5 Approximately 5-20% of SJS/TEN cases were idiopathic, but SJS/TEN is thought to result from a combination of immune predisposition and stimulus interactions such as drugs. 3,6 SJS and TEN have a genetic predisposition across age, gender and ethnicity but are more common in the elderly, women and individuals with Human Hmmunodeficiency Virus (HIV) infection. ...
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.
... The skin and mucous membranes are affected, typically presenting as reddish patches or unusual target-shaped lesions on the trunk. These lesions gradually merge and extend as areas of redness with darkened centers [3]. Oral manifestations are highly prevalent, with mucositis and ulceration observed in nearly all cases, affecting up to 100% of individuals [3,1b]. ...
... Ocular involvement is a common occurrence, with varying degrees of severity. It can range from conjunctival redness to the complete shedding of the outermost layer of the eye surface [3]. In the United States, it is estimated that approximately 1.9 adults per million experience TEN each year, while SJS affects approximately 9.3 individuals per million annually [4]. ...
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.
... Stevens-Johnson syndrome (SJS) is a rare and severe disease, with an estimated incidence of 0.4 to 1.2 cases per million inhabitants per year. 1 This syndrome primarily manifests as a reaction to drugs, characterized by the appearance of generalized skin rashes, vesiculobullous lesions on the skin and mucous membranes, and in severe cases, skin detachment. 2 Among the main triggers are anticonvulsants, such as lamotrigine, phenobarbital, and carbamazepine, responsible for approximately one-third of all cases. Clinical presentation can occur in the first days after starting a new drug treatment, and even up to 8 weeks later. ...
... However, the association with less-frequent pathogens, like cytomegalovirus or dengue, is exceptionally rare, with few cases reported to date. 1 Owing to the infrequency of this condition and the difficulty in identifying the etiological agent, we present a case of SJS associated with a dengue infection. ...
... Usually, when skin desquamation affects less than 10% of the total body surface, it is diagnosed as SJS; when it covers between 10% and 30%, the terms SJS and TEN overlap, and when it exceeds 30%, it is considered TEN. 8 Approximately 80% of SJS cases are related to medications, involving more than 200 drugs, while the remaining cases are attributed to infectious causes. 1 Anticonvulsants, antibiotics, NSAIDs, and allopurinol are among the medications most frequently associated with this adverse reaction. In addition, certain diuretics such as amiloride, methychlothiazide, and metolazone have been linked to SJS. 5 The underlying pathophysiology involves an immunomediated hypersensitivity type IV response, leading to keratinocyte apoptosis. ...
Stevens-Johnson syndrome is an infrequent condition affecting the skin and mucous membranes, it involves cutaneous detachment with high mortality without adequate treatment. We present the case of a 40-year-old male with a history of epilepsy treated with valproic acid and lamotrigine, previously diagnosed with dengue. Evaluation showed erythematous blisters on skin and mucosa with bleeding and desquamation, covering 10% of the body surface. The patient progressed favorably with the medical care received. Stevens-Johnson syndrome should be studied in association with arboviral diseases.
... Similar to the findings of our survey, all available previous reports revealed that medicines are the most common causative factors for SJS/TEN [5,14,[22][23][24]. In the present study, the most common causative drugs were NSAIDS (29.2%), antibiotics (20.8%) and antiepileptic drugs (14.6%). ...
This study analysed the causative factors and clinical characteristics of acute and chronic ocular sequelae of Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) treated at a large third-referral centre in a developed country (Hungary) over a 15-year period.
This was a retrospective review of patients with acute and/or chronic SJS/TEN who were managed between 2006 and 2020 at the Department of Ophthalmology of Semmelweis University in Budapest, Hungary. For each subject, clinical data, including patient demographics, clinical history, causative agents of SJS/TEN, and conservative and surgical treatment details, were reviewed.
Ninety-six eyes of 48 patients were included (28 female; 58.3%); the age at disease onset was 32.1 ± 22.4 years. The most common causative factors were medicines (n = 36; 75.0%). Among these drugs, 29.2% were nonsteroidal anti-inflammatory drugs (NSAIDs) (n = 14), 20.8% were antibiotics (n = 10) and 14.6% were antiepileptic drugs (n = 7). In patients with chronic SJS/TEN, the most commonly found ocular sequelae were conjunctival hyperaemia in 45 (56.3%) eyes, symblepharon in 38 (47.5%) eyes, trichiasis/distichiasis in 37 (46.3%) eyes, corneal neovascularization in 31 (38.8%) eyes and corneal scarring in 29 (36.3%) eyes. In patients with chronic SJS/TEN, the most frequently used topical conservative treatment included antibiotics in 53 (66.3%) eyes, preservative-free artificial tears in 50 (62.5%) eyes and topical corticosteroids in 42 (52.5%) eyes of 40 patients. The most frequently performed ocular surgeries for managing chronic ocular sequelae in patients with SJS/TEN were epilation for trichiasis (n = 27; 33.8%), cataract surgery (n = 14; 17.5%), entropion surgery (n = 12; 15.0%), penetrating keratoplasty (PK) (n = 11; 13.8%) and amniotic membrane transplantation (n = 4; 5.0%).
Our results suggest that NSAIDs, antibiotics and antiepileptic drugs are the most common causative factors for SJS/TEN in Hungary. Like in other countries, in Hungary, the ocular management of patients with acute and chronic SJS/TEN is heterogeneous, and most cases do not follow modern therapeutic guidelines.
... Stevens-Johnson syndrome/TEN is often confused with other skin conditions, including generalized fixed drug eruption, erythema multiforme major, and autoimmune bullous diseases such as bullous pemphigoid and pemphigus vulgaris [5]. Generalized fixed drug eruption is characterized by widespread red or brown spots or plaques with bullae, rare mucosal involvement; it usually resolves within one-to-two weeks after drug discontinuation [5]. ...
... Stevens-Johnson syndrome/TEN is often confused with other skin conditions, including generalized fixed drug eruption, erythema multiforme major, and autoimmune bullous diseases such as bullous pemphigoid and pemphigus vulgaris [5]. Generalized fixed drug eruption is characterized by widespread red or brown spots or plaques with bullae, rare mucosal involvement; it usually resolves within one-to-two weeks after drug discontinuation [5]. Erythema multiforme major typically presents with target or targetoid lesions, can involve mucosal membranes, but tends to involve less than 10 percent of the body surface area. ...
... This is favored to be caused by infection over medications. Bullous pemphigoid results in generalized, itchy, tense bullae on an erythematous base, clinically distinctive and usually in older patients [5]. Pemphigus vulgaris can be mucocutaneous in involvement with painful sloughing and may also be triggered by drugs. ...
