Epidermal Necrolysis In Children Research Articles

Drugs associated with epidermal necrolysis in children: A World Health Organization pharmacovigilance database analysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare life-threatening mucocutaneous reactions most often induced by drugs. To date, no large pharmacovigilance study has been conducted in the paediatric population. To describe the spectrum of drugs associated with SJS-TEN in children through the analysis of cases reported in the WHO pharmacovigilance database (VigiBase). Disproportionality study using data from VigiBase. All paediatric (age under 18 years) cases reported between January 1, 1967, and July 6, 2022, were included. For each molecule, a case-non-case study was performed to assess a potential pharmacovigilance signal by computing the lower end of the 95% credibility interval for the information component (IC025). We performed sensitivity analyses, (i) taking into account only cases reported by physicians and (ii) taking into account only cases reported in the last 10 years. Among 31,376,783 adverse drug reactions reported in VigiBase, 2,248,727 were paediatric cases and 7342 were encoded as paediatric SJS-TEN. Significant statistical pharmacovigilance signals were observed for 165 drugs. The two most represented drug classes were antiepileptics and anti-infectious drugs. The five drugs with the highest IC025 were lamotrigine (IC025 4.99), carbamazepine (IC025 4.88), phenobarbital (IC025 4.67), phenytoin (IC025 4.52) and nimesulide (IC025 4.23). Acetaminophen was significantly associated with paediatric SJS-TEN (IC025 2.85) and we also described various new suspected drugs. Vaccines had no significant pharmacovigilance signal. These results were confirmed with the sensitivity analyses. This study updates the spectrum of drugs potentially associated with paediatric SJS-TEN.

Comparison of idiopathic and drug-induced epidermal necrolysis in children.

In this monocentric retrospective study in a French paediatric reference centre, children with idiopathic epidermal necrolysis (EN) had more severe disease than those with drug-induced EN during the acute stage. In our study, no EN was attributed to infection.

34016 A national analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis in children using the Kids’ Inpatient Database

Objectives: Toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome (SJS) are potentially life-threatening mucocutaneous vesiculobullous disorders typically arising due to medication. While suggested to have high mortality and slightly different presentation in children versus adults, pediatric TEN/SJS is yet to be characterized on a national level in terms of both epidemiology and associated costs.

Nursing Care of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children

Nursing Care of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children

ТОКСИЧЕСКИЙ ЭПИДЕРМОЛИЗ ПРИ ПРИЕМЕ ПРОТИВОЭПИЛЕПТИЧЕСКИХ ПРЕПАРАТОВ: КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ

Toxic epidermal necrolysis (Lyell's syndrome) and Stevens–Johnson syndrome are severe types of toxicodermia and require emergency medical care. The pathophysiology of toxicoderma is associated with an adverse drug reaction. The article provides data from Russian and international literature on the role of antiepileptic drugs in these diseases occurrence. It also presents clinical cases of toxic epidermal necrolysis in children caused by anticonvulsants intake.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma.

The aim of this study is to evaluate the clinical and laboratory findings of pediatric patients with non-Hodgkin lymphoma (NHL) who developed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Between 2006 and 2018, the medical records of child patients with NHL who developed SJS and TEN were reviewed retrospectively. SJS/TEN developed in 7 of 70 patients with NHL (10%). The pathologic subgroups of the patients with SJS/TEN were ALK-negative anaplastic large cell lymphoma (n: 3), Burkitt lymphoma (n: 2), lymphoblastic lymphoma (n: 1), and primary mediastinal B-cell lymphoma (n: 1). Five patients had TEN, 1 patient had SJS/TEN, and 1 patient developed only SJS. In 5 patients, both steroids and intravenous immunoglobulin were administered for treatment, and clinical improvement was achieved in 3 of these patients. Only steroid treatment was used for 1 patient, whereas for the other patient, intravenous immunoglobin was preferred. In addition, N-acetylcysteine treatment was administered for these 2 patients. Four patients with acute renal failure died, and it was found that SJS/TEN is observed more frequently in patients with NHL in which intensive treatment protocols with high-dose methotrexate are used more than with other childhood malignant diseases. Early diagnosis and administration of appropriate and supportive treatment approaches may improve the prognosis.

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.

How different is Stevens-Johnson syndrome/toxic epidermal necrolysis in children?

How different is Stevens-Johnson syndrome/toxic epidermal necrolysis in children?

British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018.

British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018.

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.

Stevens-Johnson syndrome and toxic epidermal necrolysis in children

SJS and TEN are diseases characterised by epidermal detachment and necrolysis predominantly of drug induced etiology. SJS/TEN begins with a prodrome of fever, malaise, anorexia, pharyngitis, and headache lasting for 2-3 days, at times, extending to 10-11 days. Mucosal lesions usually precede skin lesion. Usually, two mucosal membranes are involved, most commonly conjunctiva and oral mucosa. Oral mucosal involvement is seen in 90% of SJS and almost all patients diagnosed with TEN. In SJS, mucosal involvement is widespread and confl uent in contrast to erythema multiforme where it is focal and seen in only 25%-60% of cases. The exact pathogenesis of SJS and TEN is not fully elucidated. In view of the paucity of T-cell infiltrate,keratinocyte apoptosis could be the result of autocrine or paracrine interaction between Fas, a death receptoron keratinocyte and Fas Ligand (FasL) produced by the keratinocytes along with the substantial contributionof soluble FasL from peripheral mononuclear cells. FasL upregulation in keratinocytes is nitric oxide-dependent anddriven by T-cell derived tumor necrosis factor (TNF) alpha and Interferon-gamma.The management revplves around immediate stoppage of drug and.,supportive care .IVIG,corticosteroids and cyclosporine are all effective drugs but no RCT is available for any of them

Stevens-Johnson Syndrome and toxic epidermal necrolysis in children: a retrospective study at Srinagarind Hospital, Khon Kaen, Thailand 1992–2012

Abstract Background Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening skin conditions with an etiology of drug exposure or infections. Objectives To determine the cause, treatments, complications, and outcomes of SJS/TEN in children admitted to Srinagrind Hospital during 1992–2012. Methods Retrospective chart review. A diagnosis of SJS and TEN was confirmed by pediatric dermatologists. Results A total of 38 patients was recorded. They consisted 31 (82%) SJS patients and 7 (18%) TEN patients. Mean age 6.6 years (range 1 to 14 years). Male to female was 1.1:1. Most cases (30 or 79%) were caused by drug exposure. Three cases (8%) by infection, and 5 cases (13%) were of unknown cause. The antiepileptic drug group was the most common cause. Systemic corticosteroids were used in 33 cases (87%). Intravenous immunoglobulin was used in one TEN patient (3%). There were 18 cases (47%) with acute complications. Ocular complications (7 cases, 39%), septicemia (4 cases, 22%), and secondary skin infections (3 cases, 17%) were the most common. Mean difference in length of hospital stay between those with and without acute complications was 12.3 days (P < 0.01, 95% CI 5.9–18.6). Ocular complications were the only long-term complications at 1-year follow up, and included symblepharon, corneal pannus, and dry eyes. Two patients (5%), both having cases of TEN, died. Conclusions Antiepileptic drugs were the most common causes of SJS/TEN in our study. Good ophthalmologic care of the prevalent acute eye complications in these patients is needed to prevent long-term ophthalmic complications.

Nationwide Survey of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children in the United States.

Although Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious, life-threatening reactions to drug therapies, no efforts have been made to investigate the comprehensive change in epidemiology with respect to age, sex, and race and ethnicity in children. The risk of death was 0.3% to 1.5%, and the highest hospitalization rates were in children 15 to 19 years of age, boys, and black children. The highest proportions of hospitalizations were children with very low household income, those with private insurance, and those treated at large urban teaching hospitals in the West. A significant winter-autumn predominance was observed.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children: A Literature Review of Current Treatments

Stevens-Johnson syndrome and toxic epidermal necrolysis are among the most concerning drug reactions affecting adults and children. Although the overall mortality has reduced substantially after the introduction of several strategies, such as prompt withdrawal of the causal drug and management of the patients in an intensive care or burn unit, these conditions continue to be associated with severe complications and a mortality rate of 1–4%. Currently, several treatment options including systemic corticosteroids, intravenous immunoglobulins, cyclosporine, tumour necrosis factor-α inhibitors, and plasmapheresis among others, have shown inconclusive benefits regarding their efficacy and safety in patients with these conditions. This review analyses the most recent literature regarding treatment options for paediatric patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children: 20 years study in a tertiary care hospital.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe lifethreatening skin conditions. The most common cause of these manifestations is medications. Beside discontinued of the culprit drug, systemic corticosteroids were used as a primary treatment option among pediatric population. This study aimed to explore causative drugs (drug group/ latent period), treaments, complications, and treatment outcome (morbidity, mortality, length of hospital stay) of SJS and TEN in children. A retrospective chart was reviewed during the period of 1992 to 2012 at Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. SJS and TEN were clinically diagnosed and confirmed by pediatric dermatologists. Other possible causes other than druginduced SJS and TEN were excluded. A total of 30 patients was recorded, including 24 (80%) SJS patients and 6 (20%) TEN patients. The mean age was 6.9 years (SD 4.4). Male to female ratio was 1.5:1. Antiepileptic drug group was the most common causative drug (n=18, 60%), followed by antibiotic drug group (n=8, 26.6%), and others (n=4, 13.3%) which included nonsteroidal antiinflammtory drugs (NSAIDs) and chemotherapy drugs. Systemic corticosteroids were used in 29 patients (96.6%). Intravenous immunoglobulin was used in one TEN patient (3.3%). There was a medium correlation between time to treatment (systemic corticosteroids) and the length of hospital stay (Spearman correlation coefficient=0.63, P=0.005). Two TEN patients (6.6%) died. Carbamazepine was the most common causative drug of SJS and TEN in our study. The severity of skin detachment is not correlated to severity of ocular findings. However, the persistent of ocular complications up to one year is suggested for promptly appropriate ocular treatment in all SJS and TEN patients. Our data suggested that early administration of systemic corticosteroid may reduce the length of hospital stay and should be considered for the treatment of pediatric druginduced SJS and TEN.

Stevens–Johnson syndrome and toxic epidermal necrolysis in children

Stevens–<scp>J</scp>ohnson syndrome and toxic epidermal necrolysis in children

Recurrence and Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children

To report clinical course, etiology, management, and long-term outcomes of children suffering from Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). We conducted a study of all pediatric patients with SJS or TEN admitted between 2000 and 2007 to the Hospital for Sick Children and Children's Hospital Boston, and particular attention was paid to clinical manifestations, etiology, mortality, and long-term outcomes. We identified 55 cases of SJS (n = 47), TEN (n = 5), or SJS/TEN overlap syndrome (n = 3). Drugs were identified as the most likely etiologic agent in 29 children (53%); antiepileptic drugs were the most common agents (n = 16), followed by sulfonamide antibiotics (n = 7) and chemotherapy drugs (n = 2). Acute Mycoplasma pneumoniae infection was confirmed in 12 children (22%), and herpes simplex virus was confirmed in 5 children (9%). Treatment regimens differed significantly between participating sites and included systemic antimicrobial agents (67%), systemic corticosteroids (40%), and antiviral drugs (31%). Intravenous immunoglobulin was administered to 21 children (38%), of whom 8 received concomitant systemic corticosteroids. Ten children (18%) had recurrence of SJS up to 7 years after the index episode, and 3 experienced multiple recurrences. Twenty-six children (47%) suffered long-term sequelae that mostly involved the skin and eyes. Mortality rate in children was lower than that reported in adults, but half of affected children suffered long-term complications. The recurrence rate of SJS was high (1 in 5), which suggests vulnerability and potential genetic predisposition. In the absence of standardized management guidelines for these conditions, treatment regimens differed significantly between participating institutions.

Recurrence and Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children

Recurrence and Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children

Stevens‐Johnson syndrome and toxic epidermal necrolysis in children: a review of the experience with paediatric patients in a University Hospital

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening drug reactions considered to be part of the spectrum of a single pathological process. To describe the clinical and epidemiological characteristics of SJS/TEN in children attended at our hospital. Retrospective study of children diagnosed with SJS/TEN between 1999 and 2009 in a University Hospital provided with regional-level burn and paediatric intensive care units. We found 14 paediatric patients (eight SJS and six TEN). They presented an average of 60% of the body surface area affected and 31% of epidermal sloughing. The average of suspected drugs was 1.7 per patient, anticonvulsants (carbamazepine, phenytoin and lamotrigine) and antibiotics (penicillin and macrolides) being the most frequent ones. Silver sulfadiazine was the topical treatment most frequently used, 86% of patients received systemic steroids and 28.5% intravenous immunoglobulins. One patient died. The SJS/TEN complex is a true dermatological critical condition that also affects children. Any drug can be the causative agent, more frequently anticonvulsants and antibiotics. Depending on the extension of the affected body surface, patients should be rapidly admitted to a critical care area with experience in the care of burn patients. Discontinuation of the suspected offending drugs is mandatory. Optimal supportive care and management of denuded skin areas are still the mainstay of treatment. The use of specific therapies remains controversial. Compared with adults, the disease in children seems to be milder with lower mortality.

Medications as Risk Factors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children: A Pooled Analysis

Medications as Risk Factors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children: A Pooled Analysis