Epidermal Necrolysis Overlap Research Articles

Profile of Drug Hypersensitivity Patients Hospitalized in Dr. Soetomo Hospital, Surabaya, Indonesia: Preliminary Data of 6 Months Observation

Drug hypersensitivity is defined as an untoward response to medication which is noxious and unintended, and which occurs at doses normally used in human either for the prophylaxis, diagnosis, or therapy of disease or for the modification of physiological function. Drug hypersensitivity is common and may cause emergency condition until death. The incidence of drug hypersensitivity-related hospitalizations has usually been assessed within hospitals. The aim of this study is to determine the profile of drug hypersensitivity patients hospitalized at Dr. Soetomo Hospital in 6 months period from January to June 2016. This study was a descriptive retrospective study on medical records of drug hypersensitivity patients hospitalized in Dr. Soetomo Hospital in 6 months period. The patient’s demographic data, the type of hypersensitivity reaction, and the final outcome of the hospitalization were collected. Within the 6 months period, there were 16 drug hypersensitivity patients hospitalized in Dr. Soetomo Hospital. Most of them are female (56.25%), and aged between 46-55 years (25%). There were 4 patients (25%) with type I hypersensitivity: urticaria, angioedema and anaphylaxis; while type IV hypersensitivity occured in 12 patients (75%): Stevens-Johnson syndrome, Stevens-Johnson syndrome-Toxic Epidermal Necrolysis overlap, erythroderma, maculopapular drug eruptions, and DRESS. Most of the patients (87.5%) had favorable outcome after hospitalization. There were 16 patients with drug hypersensitivity reaction hospitalized in Dr. Soetomo Hospital, Surabaya in 6 months period. Most of them were female and had type IV hypersensitivity reactions.

Allopurinol-Induced Severe Cutaneous Adverse Drug Reactions: An Analysis of Spontaneous Reports in Malaysia (2000-2018).

Allopurinol-induced severe cutaneous adverse drug reactions (SCARs) are potentially debilitating and life-threatening reactions, which can cause a financial burden to the healthcare system. We aimed to identify risk factors for allopurinol-induced SCARs and to assess their impact on fatality. Adverse drug reaction (ADR) reports with allopurinol as suspected drug were extracted from the Malaysian pharmacovigilance database from year 2000 to 2018. Multiple logistic regression analysis was used to identify significant predictors of allopurinol-induced SCARs. We further analysed the association between covariates and SCARs-related fatality in a separate model. Level of significance was set at p value < 0.05. Out of 1747 allopurinol ADR reports, 612 involved SCARs (35%). The strongest predictors significantly associated with SCARs were underlying renal disease (odds ratio [OR] 2.02; 95% confidence interval [CI] 1.36, 3.00; p = 0.001), allopurinol-prescribed dose of 300mg/day or higher (OR 1.72; 95% CI 1.38, 2.15; p < 0.001), females (OR 1.54; 95% CI 1.24, 1.93; p < 0.001), age 65years and above (OR 1.31; 95% CI 1.04, 1.64; p = 0.020), and allopurinol-prescribed indication. SCARs cases were higher in patients who received allopurinol for unspecified hyperuricaemia (OR 1.87; 95% CI 1.29, 2.70; p = 0.001) and off-label indications (OR 3.45; 95% CI 2.20, 5.42; p < 0.001) compared to registered indications. Fatality was associated with older age and a diagnosis of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) overlap or TEN. Malaysian pharmacovigilance data show that predictors of allopurinol-induced SCARs were elderly females, patients with underlying renal disease and high allopurinol doses. These patients need close monitoring and must be educated to stop allopurinol at the first signs of rash.

A multicentric, prospective and retrospective analysis of Stevens–Johnson syndrome, toxic epidermal necrolysis and Stevens–Johnson syndrome/toxic epidermal necrolysis overlap: An Indian perspective

Background: Stevens Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) represent adverse drug reactions characterized by generalized rash, blisters, epidermal detachment, mucosal and systemic involvement presenting as dermatological emergencies. Drugs are the most common culprits implicated in the pathogenesis of SJS /TEN. The primary step in the management of SJS/TEN is identification and stopping the offending drug. Materials and methods: This was a multi-centric retrospective as well as prospective observational study conducted in 7 tertiary care hospitals including Father Muller Medical College Hospital , Mangalore, India. A detailed evaluation of the hospital in patient record for 10 years retrospective from September 2015 and all new cases of SJS/TEN as well as SJS-TEN overlap presenting to these centres for 1 year prospective was performed. Detailed history was taken and clinical examination was recorded and different modalities of treatment was noted and compared. Results: A total of 152 patients with clinical diagnosis of SJS ,TEN and SJS-TEN overlap were analysed. Males were more commonly affected than females and history of drug intake was present in 118 patients (77.6%). The most common drugs which caused SJS/ TEN in our study were antibiotics in 40 patients (26.3%) followed by anti epileptics in 35 patients (23%) and nevirapine in 10 patients (6.5%). In our study the most common finding on cutaneous examination was maculopapular rash (19.3%). Conclusion: SJS and TEN form part of a spectrum of severe cutaneous drug reactions that can lead to high morbidity which can be reduced by early withdrawal of the offending drug and timely intervention. Individuals with known drug allergies need to be educated and drug allergy card has to be carried.

Oral mucosal Myiasis: A rare association with pediatric SJS-TEN overlap

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) overlap is a severe cutaneous adverse reaction with predominantly drug-related etiology which is an immune complex mediated hypersensitivity disorder. Oral myiasis is defined as an intra-oral infestation of the living tissues of animals or humans by dipteral larvae. Herein, we report a rare case of an eight-year-old female child who presented to us with SJS-TEN overlap and was accidentally diagnosed with oral myiasis during her course of hospitalisation. This case report gives an overview of oral mucosal myiasis in childhood as a rare association with SJS-TEN overlap.

Enzalutamide Associated with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap: A Case Report

Enzalutamide Associated with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap: A Case Report

Stevens–Johnson syndrome/toxic epidermal necrolysis overlap syndrome due to oral phenytoin

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but lethal manifestation of hypersensitivity reaction precipitated by certain drugs and viral infections. Among the drugs, it is more common with antibiotics, antiepileptics and nonsteroidal anti-inflammatory drugs. We report the case of a 14-year-old male who presented to the medicine out-patient department with a history of fever since the previous day. Four days before this, he had developed an episode of generalised tonic–clonic seizures and was initiated on oral phenytoin since then. Following admission to the medical intensive care unit, fever persisted on the 2nd day as well. The patient developed a maculopapular rash over face and trunk, ulceration over lips and skin peeling involving >10% <30% body surface area suggestive of SJS/TEN overlap syndrome. Oral phenytoin was stopped, and he was started on oral levetiracetam. He was treated with intravenous corticosteroids, topical antibiotics and symptomatic management. The patient recovered, skin lesions subsided and he discharged in a stable condition after 2 weeks of in-hospital stay. The present case documents the rare occurrence of SJS/TEN overlap syndrome as an adverse drug reaction caused by phenytoin. Early identification and stopping of offending drug will aid in better management of the patient.

Stevens–Johnson/Toxic epidermal necrolysis overlap syndrome

Stevens–Johnson/Toxic epidermal necrolysis overlap syndrome

Stevens-Johnson/Toxic epidermal necrolysis overlap syndrome.

Stevens-Johnson/Toxic epidermal necrolysis overlap syndrome.

Case Report: Suspected Case of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis Overlap Due to Ursodeoxycholic Acid

Stevens–Johnson syndrome and toxic epidermal necrolysis are well-known severe cutaneous adverse reactions, with &gt;100 medications previously implicated, most frequently sulfonamide antibiotics. Ursodeoxycholic acid (UDCA), normally present in human bile at a low concentration, is used for the treatment of various cholestatic disorders. Reports of UDCA causing cutaneous complications are, however, rare. The present report describes a suspected case of UDCA-induced Stevens–Johnson syndrome–toxic epidermal necrolysis overlap in a 24-year-old female, admitted with a whole-body maculopapular rash with oromucocutaneous ulceration and skin desquamation. The patient was managed with supportive care, including fluid and electrolyte replacement, corticosteroids, antibiotics, antihistamines, and intravenous Ig. Early identification, prompt intervention with effective care, and support are the key action points in these severe cutaneous adverse reactions.

Stevens-Johnson Syndrome (SJS)-Toxic Epidermal Necrolysis (TEN) overlap in a 61-year old woman: a case report

Background: Epidermal necrolysis (EN) is a rare life-threatening reaction. The exact cause of this disease is unknown. However, the drug is one of the main factors as a cause. EN differentiated based on the extent of skin detachment limited to less than 10% of the body surface area (BSA) in SJS, 10%-30% BSA in SJS/TEN overlap, and greater than 30% of the BSA in TEN. SJS and TEN can occur at various ages, but more often occur at the age of 40 years. Mortality was almost 30% in SJS/TEN overlap patients. Management for SJS and TEN is the cessation of drugs that are thought to be the cause and other supportive therapies.Case presentation: A 61-year-old female with SJS-TEN overlap, who presented epidermal detachment, is about 10-30% with a positive Nikolsky sign. She had a history of taking medication, such as paracetamol, amoxicillin, and buscopan, before lesion appeared. There were multiple lesions widespread, macula hyperpigmentation multiple, erosion from the bullae that has broken, there also erosion covered by black crust, and various erosions covered with black, brown crusts on lip mucosa and also secret on both eyes. The management of this patient was a cessation of suspected drug and supportive therapy. The patient’s condition was improved within a few weeks, and no sequelae found.Conclusion: SJS-TEN overlap is one of the epidermal necrolysis in which skin detachment 10-30% BSA. The management was a cessation of all suspected drugs, administering high doses of steroids and other supportive therapies.

Retrospective review of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis cases at a pediatric tertiary care institution.

Stevens-Johnson syndrome and toxic epidermal necrolysis represent important sources of potential mortality and morbidity in children. There is a need for more clinical data in this population to determine whether specific treatments preferentially improve outcomes. This was a single-center retrospective review of children admitted with drug-induced Stevens-Johnson syndrome, toxic epidermal necrolysis or Stevens-Johnson syndrome/toxic epidermal necrolysis overlap at a tertiary care pediatric institution in North America from 2008 to 2018. Patients without a dermatology assessment and diagnosis were excluded. Demographic, clinical, and treatment information were abstracted and reviewed for all included patients. Sixteen patients were identified, 43% female (7/16), with a mean age at presentation of 10.4±5.2years. Antibiotics were implicated in 56.3% of patients (9/16) and anticonvulsants in 31.3% (5/16). Sulfamethoxazole-trimethoprim was the triggering antibiotic in 31.3% of patients. The majority of patients were treated with intravenous immunoglobulin alone (50%, 8/16) or intravenous immunoglobulin with steroids (25%, 4/16). Etanercept was added to intravenous immunoglobulin and corticosteroid in a 2-year-old patient, resulting in clinical stabilization and halting of epidermolysis. No patients died. Clinical sequelae were noted in five patients, including ocular complications (n=4), labial adhesions (n=1), and persistent skin dyspigmentation (n=3). Our results highlight that sulfamethoxazole-trimethoprim is an important cause of Stevens-Johnson syndrome-toxic epidermal necrolysis in children. Mortality was reassuringly low, but ocular sequelae were an important cause of morbidity. More data are needed to help determine whether specific treatments including etanercept may provide mortality or morbidity benefit in pediatric populations.

Phenytoin Induced Steven Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap.

Phenytoin Induced Steven Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap.

Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

Epidermal necrolysis (EN) is a rare and life-threatening condition. To assess whether admitting hospital characteristics and interhospital transfer are associated with mortality due to EN. We studied the French nationwide hospital discharge database (retrospective national cohort). All patients admitted during 2012-2016 with a code for EN in the International Classification of Diseases, 10th Revision, were eligible. We extracted data on the patients (age, sex, intensive care unit admission, comorbidities) and hospitals (private proprietary vs. public, nonteaching or teaching; and number of admissions for EN as a proxy for experience). Multivariable analysis was used to identify independent predictors of in-hospital mortality with mixed logistic regression. We identified 991 patients (467 male; mean age 52·7 ± 23 years). They were admitted to 300 different hospitals, including teaching hospitals (25% of hospitals) for around half of the patients. Overall, 597 patients (60%) had a diagnosis of Stevens-Johnson syndrome (SJS), 171 (17%) had SJS/toxic epidermal necrolysis (TEN) overlap and 223 (23%) had TEN. In total, 109 (11%) patients died: nine (2%) with SJS, 26 (15%) with SJS/TEN overlap and 74 (33%) with TEN. The in-hospital mortality rate was lower in centres with vs. without substantial EN experience - odds ratio for one supplemental EN admission in a department 0·5 (95% confidence interval 0·3-1·0); P = 0·05 - even after adjusting for potentially relevant individual risk factors. We found no significant association between mortality and interhospital transfer. Our findings highlight increased survival of patients with EN in centres with a high volume of EN procedures. If confirmed in other settings, these findings reinforce the importance of expertise in early diagnosis and management of this condition. What's already known about this topic? Epidermal necrolysis (EN) is a rare and life-threatening condition. At the individual level, risk factors for in-hospital mortality have been identified. Few studies have examined the association between hospital characteristics and EN mortality, with special attention to referral hospitals. What does this study add? Short-term mortality rates were lower for patients in centres with EN experience than in centres without EN experience, after adjusting for known risk factors. We found no association between interhospital transfer and survival. If confirmed in other settings, these findings support the early transfer of patients with suspected or diagnosed EN to centres with experience, where a multidisciplinary approach can be implemented by experienced healthcare professionals, to maximize short-term survival.

Stevens-Johnson syndrome and toxic epidermal necrolysis overlap associated with alternative medicine: A case report

Stevens-Johnson syndrome and toxic epidermal necrolysis overlap associated with alternative medicine: A case report - IJPCA- Print ISSN No: - 2394-2789 Online ISSN No:- 2394-2797 Article DOI No:- 10.18231/j.ijpca.2019.010, International Journal of Pharmaceutical Chemistry and Analysis-Int J Pharm Chem Anal

Profile of Drug Hypersensitivity Patients Hospitalized in Dr. Soetomo Hospital, Surabaya, Indonesia: Preliminary Data of 6 Months Observation

Drug hypersensitivity is defined as an untoward response to medication which is noxious and unintended, and which occurs at doses normally used in human either for the prophylaxis, diagnosis, or therapy of disease or for the modification of physiological function. Drug hypersensitivity is common and may cause emergency condition until death. The incidence of drug hypersensitivity-related hospitalizations has usually been assessed within hospitals. The aim of this study is to determine the profile of drug hypersensitivity patients hospitalized at Dr. Soetomo Hospital in 6 months period from January to June 2016. This study was a descriptive retrospective study on medical records of drug hypersensitivity patients hospitalized in Dr. Soetomo Hospital in 6 months period. The patient’s demographic data, the type of hypersensitivity reaction, and the final outcome of the hospitalization were collected. Within the 6 months period, there were 16 drug hypersensitivity patients hospitalized in Dr. Soetomo Hospital. Most of them are female (56.25%), and aged between 46-55 years (25%). There were 4 patients (25%) with type I hypersensitivity: urticaria, angioedema and anaphylaxis; while type IV hypersensitivity occured in 12 patients (75%): Stevens-Johnson syndrome, Stevens-Johnson syndrome-Toxic Epidermal Necrolysis overlap, erythroderma, maculopapular drug eruptions, and DRESS. Most of the patients (87.5%) had favorable outcome after hospitalization. There were 16 patients with drug hypersensitivity reaction hospitalized in Dr. Soetomo Hospital, Surabaya in 6 months period. Most of them were female and had type IV hypersensitivity reactions.

Stevens–Johnson syndrome/toxic epidermal necrolysis overlap syndrome due to oral cefuroxime

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening and severe cutaneous adverse drug reactions characterised by epidermal detachment presenting as blisters and differ only by their extent of skin detachment. A 22-year-old male presented to our tertiary care teaching institute with fever for the preceding 5 days and macular rash over the back for the past 1 day. Before this, he had consulted a local physician for fever and was using oral cefuroxime (250 mg bid) for the past 5 days. General physical examination revealed fever (103oF), conjunctival congestion, generalised maculopapular rash and ulcers over the lips. He was admitted to medical intensive care unit. On the next day, fever persisted; rash had worsened, conjunctivitis developed and skin peeling became evident, involving >10% but

Case report of Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome caused by severe Mycoplasma pneumoniae pneumonia

Case report of Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome caused by severe Mycoplasma pneumoniae pneumonia

Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous reactions, typically to drugs or infection. The incidence and outcomes of these conditions in children are unknown. The objective of this study was to report the overall burden of Stevens-Johnson syndrome and toxic epidermal necrolysis in children in the United States. We performed a retrospective cohort analysis of children and adolescents younger than 18years of age using the 2009 and 2012 Kids' Inpatient Database. We identified 1486 children and adolescents hospitalized with a diagnosis of Stevens-Johnson syndrome or toxic epidermal necrolysis. The national incidence per 100000 was 6.3 for Stevens-Johnson syndrome, 0.7 for Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome, and 0.5 for toxic epidermal necrolysis. The highest incidence in children was in those aged 11-15years (38.4 per 100000). Toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome were associated with longer stay, greater mortality, and higher hospital charges than those with Stevens-Johnson syndrome. Hospital mortality was highest in children with toxic epidermal necrolysis and in children aged 0-5years. The incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis in children is higher than reported in adults, and there are significant age-based variations in incidence and outcomes across the pediatric population. Further study is needed to determine the most effective treatment strategies to reduce costs and improve outcomes in children hospitalized with severe cutaneous reactions.

Tolerance Induction to Antituberculosis Drugs in a Patient With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap.

Tolerance induction and desensitization in Stevens–Johnson syndrome (SJS) or in toxic epidermal necrolysis (TEN) have been described as an absolute contraindication by some authors, but there are cases where there is no treatment alternative. Tuberculosis (TB) remains a leading cause of morbidity and mortality in developing countries and ranks alongside HIV as a leading cause of death worldwide. Severe drug reactions, such as SJS and TEN, occurring in these individuals are lifethreatening. Since alternative therapies for TB are limited, the role of desensitization and reintroduction becomes essential. We describe a case of tolerance induction to anti-TB drugs in a patient with SJS/TEN overlap syndrome using a specifically designed premedication, comedication, and desensitization protocol.

Severe Stevens‐Johnson syndrome/toxic epidermal necrolysis overlap syndrome—beyond skin involvement

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious dermatologic diseases with many potential multisystem complications. We describe the case of an 8-year-old girl who developed severe SJS/TEN overlap syndrome (25% of her body surface area was affected) complicated by pancreatitis and bronchiolitis obliterans. These rare complications emphasize the need for careful, intensive monitoring of possible complications and an interdisciplinary team approach to provide optimal treatment and follow-up.