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Complications noted in SJS, TEN, and SJS-TEN overlap (SJS: Stevens-Johnson syndrome; TEN: Toxic epidermal necrolysis; LFT: Liver function test)
Source publication
Background : Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare immune-mediated severe cutaneous adverse reactions with incidence rate of 0.05 to 2 persons per million populations per year. Drugs are the most commonly implicated in 95% of cases. Aims : To audit the causative drugs, clinical outcome, and cost of management...
Context in source publication
Context 1
... complications noted in cases of SJS, TEN, and SJS- TEN overlap were secondary infection, septicemia, altered liver function test, electrolytes imbalance, leucocytosis, leucopoenia, thrombocytopenia, hyperglycemia, respiratory distress, and acute renal failure. Secondary infection was the most common complication (9 of 32 cases) noted [ Figure 1]. Mortality rate was 15.6% among all cases; 9% in SJS and 26.7% in TEN. ...
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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) form a spectrum of a rare and life-threatening cutaneous drug reaction. SJS/TEN in pregnancy poses largely unknown risk factors and outcomes for both the mother and foetus compared to the general population.
We conducted a study of consecutive pregnant women admitted to single tert...
Background:
Toxic epidermal necrolysis (TEN) is a rare life threatening dermatological disorder characterized by extensive epidermal detachment and erosion of mucous membranes. It is typically a side effect of some medications. Nevirapine, a nonnucleoside reverse transcriptase inhibitor (NNRTI) is one of the frequently used components of highly ac...
Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN)
are closely related severe, acute mucocutaneous reactions usually caused
by drugs. They are acute life‑threatening conditions and cause widespread
necrosis of the epithelium. There is persistence of a high risk of SJS or TEN
in relation to human immunodeficiency virus (HIV) infecti...
Background
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening adverse drug reactions (ADRs) that are commonly caused by medications. Apart from their contribution to morbidity and mortality, these diseases may also present substantial consequences on health care resources. In this study, we aimed to ident...
Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are closely related severe, acute mucocutaneous reactions usually caused by drugs. They are acute life-threatening conditions and cause widespread necrosis of the epithelium. There is persistence of a high risk of SJS or TEN in relation to human immunodeficiency virus (HIV) infecti...
Citations
... Adverse Drug Reactions (ADR) accounts 6% of the total hospital admission, increases economic burden on healthcare system, results into withdrawal of drugs from market and death. 1 Among various ADR, cutaneous drug reactions mainly Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but potentially fatal reactions that endangers patient's life. 1 It is nearly always drug-related. 2 Incidence of SJS and TEN is 0.05 to 2 persons per million population per year. 3 In India, Nimesulide is easily available as an over the counter drug (OTC), different cutaneous reactions associated with, Nimesulide including Angioedema, maculo-popular rash, severe urticaria, pityriasis rosea and worsening of preexisting psoriasis. 4 Indian government restricted its use in 2011 for paediatric purposes in the age group of less than 12 years 4 . ...
Introduction: Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare, acute and life-threatening mucocutaneous diseases that are nearly always drug-related. Incidence of SJS and TEN is 0.05 to 2 persons per million populations per year. In India, Nimesulide is easily available as an over the counter drug (OTC), different cutaneous reactions associated with, Nimesulide including angioedema, maculo-papular rash, severe urticaria, pityriasis rosea and worsening of preexisting psoriasis. Patient concern (Case summary): A 17 years old female patient complained about fever and she was prescribed with Tab.Nimesulide twice daily by private practitioner. On the 3rd day of intake of oral Nimesulide, patient developed skin lesions over face, trunk, back and all four limbs. This reaction was diagnosed as Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) overlap by Nimesulide based on clinical picture and drug history. Interventions: Tab. Nimesulide was stopped and patient was admitted in dermatology ward. Patient was treated with Inj.Hydrocortisone sodium succinate 100mg iv twice daily, Inj.Cefotaxime 1gm iv thrice a daily, Inj. Metronidazole 500mg once a day and Liquid Cyclosporine 50mg twice a day for 10days. Other treatments included Inj. Albumin 100ml i.v slowly for 3 days given. Patient recovered and was discharged after 10 days. Conclusion: We are presenting a case of SJS-TEN overlap caused by Nimesulide, which is commonly used as an over the counter drug (OTC). Our aims to minimize this type of serious ADR by making alert to physicians and other healthcare professionals, we can avoid hospital admissions because of ADR, reduce economic burden of the patients and health related quality of life of the patient can be improved.
... 18,[20][21][22][23] In Asia, reported overall mortality rates vary from 12.3% to 25%. [24][25][26][27] Sepsis leading to multiorgan failure is the most common cause of death. 21 Despite the substantial mortality, there currently is no therapeutic regimen with a clear benefit for patients with SJS/TEN. ...
Introduction:
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) [hereafter, SJS/TEN] are uncommon but severe mucocutaneous reactions. Although they have been described in many populations worldwide, data from Hong Kong are limited. Here, we explored the epidemiology, disease characteristics, aetiology, morbidity, and mortality of SJS/TEN in Hong Kong.
Methods:
This retrospective cohort study included all hospitalised patients who had been diagnosed with SJS/TEN in Prince of Wales Hospital from 1 January 2004 to 31 December 2020.
Results:
There were 125 cases of SJS/TEN during the 17-year study period. The annual incidence was 5.07 cases per million. The mean age at onset was 51.4 years. The mean maximal body surface area of epidermal detachment was 23%. Overall, patients in 32% of cases required burns unit or intensive care unit admission. Half of the cases involved concomitant sepsis, and 23.2% of cases resulted in multiorgan failure or disseminated intravascular coagulation. The mean length of stay was 23.9 days. The cause of SJS/TEN was attributed to a drug in 91.9% of cases, including 84.2% that involved anticonvulsants, allopurinol, antibiotics, or analgesics. In most cases, patients received treatment comprising either best supportive care alone (35.2%) or combined with intravenous immunoglobulin (43.2%). The in-hospital mortality rate was 21.6%. Major causes of death were multiorgan failure and/or fulminant sepsis (81.5%).
Conclusion:
This study showed that SJS/TEN are uncommon in Hong Kong but can cause substantial morbidity and mortality. Early recognition, prompt withdrawal of offending agents, and multidisciplinary supportive management are essential for improving clinical outcomes.
... SJS/TEN has a variety of possible skin presentations including decentralizing atypical targetoid macules resulting in dusky plaques and subsequent skin-sloughing, macular targetoid lesions, or flaccid bullous lesions . The percentage of body surface area (BSA) affected in SJS and TEN is what differentiates the two conditions; SJS affects < 10 % BSA, while TEN affects > 30 %BSA, and overlapping SJS/ TEN covers between 10 and 30 % BSA (Barvaliya et al., 2011). Medical education resources demonstrate the different presentations of SJS through images, which is a helpful tool to enable trainees and physicians to identify such a severe condition, allowing them to intervene and treat before the condition is exacerbated. ...
Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe dermatologic immune-related adverse events (irAEs) characterized by the separation of the epidermal and dermal layers of the skin. Less commonly documented, these adverse events have shown to be secondary to immune checkpoint inhibitors such as anti-PD-1 monoclonal antibody pembrolizumab. We present the case of a 33-year-old African American female with a pertinent past medical history of history of recurrent progressive metastatic squamous cell carcinoma cervical cancer treated with pembrolizumab. The patient presented with symptoms of SJS/TEN four weeks after treatment with pembrolizumab was initiated. Intervention was delayed because the definitive diagnosis of an irAE was difficult due to time from initiation of treatment and obfuscated by intervening urosepsis episode treated with meropenem, and lack of literature illustrating SJS/TEN in patients of darker skin. From this case, we can learn the importance of immediate intervention in cases of irAE secondary to immune complex inhibitors and demonstrate the presentation of such a severe-life threatening condition in a patient of a darker skin tone.
... Some of the identified causes of SJS/TEN include medications, mycoplasma pneumonia, herpes, hepatitis A, and vaccination [3]. The primary cause of this life-threatening disorder is drugs, with common examples being sulfonamides and anti-epileptic drugs such as phenytoin, carbamazepine, lamotrigine, phenobarbital, and allopurinol, as well as non-steroidal anti-inflammatory drugs like piroxicam, diclofenac, and nevirapine [4]. ...
Stevens-Johnson syndrome and toxic epidermal necrolysis overlap is a rare but severe cutaneous hypersensitivity reaction that can lead to death if not treated aggressively and adequately. Drug-induced hypersensitivity reactions are often related to drug exposure, with sulfonamides, anti-epileptics, fluoroquinolones, cephalosporins, and nonsteroidal anti-inflammatory drugs being the most common culprits. This case report describes a 10-year-old boy who was administered phenytoin at a local clinic to manage his seizures. This treatment led to the onset of SJS-TEN overlap, ultimately resulting in his demise.
... Have different pharmacokinetic or pharmacokinetic roles. Pharmacodynamics effect [8]. Associations between drug hypersensitivities and HLA haplotypes also suggest a key role for immune mechanisms. ...
Drug-induced skin disease or cutaneous adverse drug reaction (CADR) is a term encompassing clinical manifestations of the skin, induced by drugs or their metabolites. The skin is the organ most commonly affected by drug reactions, affecting up to 10% of hospitalized patients and can occur in 1–3% of her polypharmacy patients. Most CADRs are mild or self-resolving conditions. The most frequently reported are macular papular rash, urticaria/angioedema, fixed drug eruption and erythema multiforme. Less common but more severe patterns include, drug reactions with eosinophilia and systemic symptoms, and the Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum. Almost any drug can induce CADR, but antibiotics (especially sulfa drugs), nonsteroidal anti-inflammatory drugs, and antiepileptic drugs are most commonly implicated. Various mechanisms are involved in the pathogenesis of CADR, some of which are still unknown. Which may be immune mediated or non-immune mediated Recognition of a specific CADR depends primarily on the physician's ability to perform a detailed clinical examination, an accurate description of the skin lesion morphology, and corroboration of laboratory and/or skin biopsy findings.
... Although, cutaneous ADRs are frequently mild and benign, their early diagnosis with withdrawal of the causative drug at the earliest is crucial for avoiding a more serious problem. Angioedema, erythema multiforme, Stevens-Johnson syndrome (SJS), skin rashes, urticaria, itching, fixed drug eruption, and Toxic Epidermal Necrolysis (TEN) are some of the important cutaneous ADRs (Sharma et al., 2001;Barvaliya et al., 2011). SJS and TEN are uncommon but are severe forms of cutaneous ADRs that negatively impact the patient's quality of life (Sharma et al., 2001). ...
... TMs could cause rare but serious cutaneous ADRs like SJS/TEN. Drugs account for nine out of ten cases of SJS/TEN (Barvaliya et al., 2011;Patel et al., 2013). SJS/TEN are associated with a high incidence of mortality (Kumar et al., 2018). ...
Background: Data on traditional medicine-induced cutaneous adverse drug reactions (ADRs) is very scarce. The current secondary analysis based on the WHO database (VigiBase) of individual case safety reports (ICSRs) focuses on the suspected cutaneous ADRs linked to traditional medicines (TMs).
Methods: All the ICSRs reported between 1st January 2016 and 30th June 2021 from the UN Asia region in VigiBase where at least one TM was suspected to cause cutaneous ADRs were included in the study. Data regarding demographic details, suspected drug, adverse reaction as per MedDRA term, the seriousness of the reaction, de-challenge, re-challenge, and clinical outcome for suspected cutaneous ADRs associated with TM were obtained from VigiBase and analyzed for frequency of reported events and suspected medicines.
Findings: Total 3,523 ICSRs with 5,761 ADRs related to “skin and subcutaneous tissue disorders” were included in the analysis. Amongst these, 6.8% of ICSRs were reported as serious. Pruritus (29.6%), rash (20.3%), urticaria (18.9%), and hyperhidrosis (3.3%) were commonly reported ADRs. Artemisia argyi H.Lév. and Vaniot. (14.9%), Ginkgo biloba L. (5.1%), Vitis vinifera L. (4%), Vitex agnus-castus L. (3.8%), Silybum marianum (L.), Gaertn (3.5%), and Viscus album L. (2.7%) were some commonly suspected TMs for cutaneous ADRs. There were 46 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis reported with TMs during the study period. Death was reported in 5 ICSRs.
Interpretation: TMs are linked with various cutaneous ADRS ranging from pruritus to toxic epidermal necrolysis which may have serious consequences. TMs listed as suspected offending agents in this analysis, should be kept in mind while dealing with suspected cutaneous ADRs. Clinicians should be more vigilant in detecting and reporting events associated with TMs.
... HIV patients experience a higher incidence than the general population. [5] However, these may often go undetected and unreported. SJS is a severe lifethreatening mucocutaneous syndrome caused by drugs like antimicrobials, antiepileptics, and analgesics. ...
Stevens-Johnson syndrome and Toxic epidermal necrosis are acute, self-limited diseases, rare but life-threatening adverse drug reactions. Anti-epileptic drug-induced Steven-Johnson syndrome is a severe cutaneous adverse reaction, among anti-epileptic drugs carbamazepine and phenytoin are the major culprit. We report here a case of Steven-Johnson syndrome due to phenytoin. A 22-year-old female reported the chief complaint of fever and multiple rashes all over the body with skin peeling, gradual onset, and progressive in nature. The reaction was evoked after intake of T. Phenytoin for 1 ½ months. She started heavy skin eruption all over the body for 15 days. She was treated with corticosteroids, anti-microbial, and anti-fungal agents. Healthcare providers must carefully regard the adverse effect of the drug, especially the one that is Steven-Johnson syndrome which is a potentially fatal condition. The most commonly prescribed drug regimen should also be used and continuously monitored to prevent adverse drug reactions. Keywords: Steven-Johnson syndrome, Adverse drug reaction, Phenytoin, Skin eruption
... The cost of illness for SJS was found to be 119.49 USD per day in a study conducted in Indonesia whereas, another study in India showed 15.16 USD per day [3,4]. These differences in the treatment costs were due to different parameters they accounted like the cost of medication, diagnosis and consumables utilized during the hospital stay (see Figs. 1 and 2). ...
Introduction
Stevens Jonson syndrome, a type IV mediated hypersensitivity reaction is a rare mucocutaneous disorder accounting for <10% of TBSA. It affects skin, oral mucosa, eyes, esophagus, mouth, pharynx, larynx, skin and genitals. SJS is caused mainly due to drugs, infectious agents, immunization, and radiation therapy.
Presentation of case
We present a case of a 40 years old male who developed SJS after being administered cefixime for a short period. Given the patient's past profile, he was admitted due to RTA and was under treatment with cefixime. Irrespective of any symptoms of SJS in the past, he started developing symptoms soon after being treated with cefixime giving us clue about cefixime-induced SJS.
Discussion
Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are opposite ends of a spectrum of diseases arising usually from an adverse reaction to medications. The most common drug reactions include penicillin in antibiotics, carbamazepine in antiepileptics and allopurinol in gout treatment in the Asian community. In our case, the patient was under Cefixime for 6 days after which cutaneous manifestations were seen. SJS is a fatal condition, with a global mortality rate stretching between 10% to 34%. The first step in its management is to identify the culprit drug and stop its use. Other is symptomatic, with special attention to airway and hemodynamic stability, wound care, and pain alleviation measures. Among medical therapy include corticosteroids, cyclosporine, intravenous immunoglobulin (IVIG), and TNF- α inhibitors.
Conclusion
Cephalosporin group, like cefixime, is a commonly prescribed drug in developing countries due to its efficacy and cost-effectiveness. Therefore, physicians must beforehand be mindful of the consequences of its use and advice patients to visit the hospital with even the slightest cutaneous manifestation.
... Although the list of drug-induced SJS/TEN is open and, theoretically, each drug can cause SJS/TEN, a limited number of drugs is responsible for the majority of cases, especially in children. Thus, most cases of drug-induced SJS/TEN occur in association with a few families of medication included anticonvulsants, antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), and allopurinol [10][11][12][13]. ...
... Duration of hospital stay in TEN and TEN/SJS overlap syndrome is higher than SJS, which is in line with earlier studies [12,23]. This result is compatible with the fact that TEN and overlap syndrome patients have a more severe clinical presentation and a larger total body surface involvement than others. ...
Background:
Different epidemiologic aspects of drug-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children are scarce.
Aim:
To compare the clinical and epidemiological features of patients with drug-induced SJS and TEN in children and adults.
Method:
This retrospective study was conducted at two academic referral centers (Isfahan, Iran) over 5 years. SJS and TEN were clinically diagnosed and confirmed by skin biopsy as needed.
Results:
One hundred one patients (31 children and 70 adults) with a female to male ratio of 1.1 : 1 was identified in the present study. SJS was more commonly diagnosed in both pediatric and adult patients. The most frequent reason for drug administration identified was the infection (45.2%) and seizure (45.2%) in children and infection (34.3%) and psychiatry disorder (27.1%) in adults (P = 0.001). The most common culprit drugs in the pediatric were phenobarbital (9/31), cotrimoxazole (4/31), and amoxicillin (4/31); however, in the adult group, the most common drugs were carbamazepine (11/70) and lamotrigine (9/70). Fever was significantly more common in adults (44.3%) compared to pediatric patients (22.6%) (P = 0.03). Multiple logistic regression models showed that pediatric patients had significantly lower odds of hospitalization (OR [odds ratio]: 0.14; 95% CI 0.02, 0.67). In addition, patients with SCORTEN 1 had significantly higher odds of hospitalization (OR: 6.3; 95% CI: 1.68, 23.79) compared to patients with SCORTEN 0.
Conclusions:
The present study showed several differences between the pediatric and adult patients with SJS and TEN, including the reason for drug administration, culprit drugs, length of hospital stay, presence of fever, and final diagnosis of disease.
... Perioral dermatitis manifested in two cases due to application of halogenated topical corticosteroid with multiple erythematous papular rash extending to the vermillion border of the lips. Barvaliya M. et al. [9] , observed antimicrobials (50%), nonsteroidal antiinflammatory drugs (22.41%), and antiseizure drugs (18.96%) as the drug commonly resulting in SJS, TEN, SJS-TEN overlap in his study. These findings were not similar to our study. ...
