Toxic Epidermal Necrolysis In Children Research Articles

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.

Clinical features and outcomes in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous conditions. However, studies of pediatric SJS/TEN are limited. To investigate the causes, clinical course, outcomes and complications of SJS and TEN in children. This retrospective study included 47 pediatric patients (aged < 18 years) with SJS, SJS/TEN, or TEN treated at Chang Gung Memorial Hospital, Taiwan, between January 2009 and December 2019. ALDEN scores and serological tests were used to assess causes and SCORTEN scores were applied to evaluate disease severity. Forty-seven patients, including 30 with SJS, 6 with SJS/TEN, and 11 with TEN were included. Median age was 8 years (range 1-17 years); 51.1% were male. Thirty-three cases (70.2%) were caused by drugs and infectious pathogens were suspected in 14 cases (29.8%). Oxcarbazepine (5/47, 10.6%) and amoxicillin (5/47, 10.6%) were the most often-implicated drugs, and Mycoplasma infection (9/47, 19.1%) was the predominant infectious cause. Only one TENS patient died (mortality rate 1/47, 2.1%) due to septic shock with ARDS, acute renal failure and cardiopulmonary shock. Median hospital stay was 15.5 (3-42) days. Pulmonary involvement (2/39, 5.1%), including pneumonia and ARDS, was noted in acute stage. Long-term sequelae were ocular involvement (6/39, 15.4%), nail dystrophy (4/39, 10.3%) and post-inflammatory hypo-/hyperpigmentation (3/39, 7.7%). In the present study, pediatric patients with SJS, SJS/TEN, or TEN have good outcomes with few long-term complications and low mortality. Mycoplasma is the most common infectious cause in pediatric SJS/TEN. Ocular discomfort, nail dystrophy and skin dyschromia are common long-term sequelae requiring regular follow-up.

Clinical and Epidemiological Features of Patients with Drug-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Iran: Different Points of Children from Adults.

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.

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.

Toxic Epidermal Necrolysis Syndrome Following Single-Dose Diclofenac Suppository, A Case Report

Introduction: Toxic Epidermal Necrolysis (TEN) is a severe disease with high mortality, which can occur following the administration of some drugs such as antibiotics, anticonvulsants and non-steroidal anti-inflammatory drugs (NSAIDs). The condition is rare in children; therefore, providing a therapeutic experience and methods of care can be very helpful in similar cases. Case Presentation: A 32-month-old girl (2.8 years) was admitted at Mousavi Hospital of Zanjan (Iran) presenting weakness, illness, scattered macular lesions on the trunk and extremities, and several blisters on the trunk. He had a history of upper respiratory tract infection and had been administered a diclofenac suppository for fever. All the clinical manifestations appeared after diclofenac administration. The lesions rapidly progressed to extensive cutaneous necrosis with the involvement of more than 70% of the body surface. He was treated with high-dose intravenous immunoglobulin (IVIG), corticosteroid, and conservative management. Fortunately, the child was discharged in good general condition approximately one month after the onset of the illness. Conclusions: Diclofenac can cause TEN in children, even in single doses and in suppositories. The most important prognostic factors include supportive therapy, contact isolation, hand hygiene, and administration of high dose IVIG for at least three days.

Clinical features of Stevens-Johnson syndrome and toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but these conditions are associated with high mortality. There have been few reports of SJS and TEN in children. The aim of this study was to evaluate the clinical features and outcomes of SJS and TEN in a group of Japanese children. We retrospectively reviewed pediatric cases of SJS and TEN, from 2000 to 2015. We identified 12 pediatric cases of SJS and three of TEN. Six (all SJS) were caused by infection, and eight of the cases (SJS, n = 5; TEN, n = 3) were drug induced. Respiratory complications were the most common in terms of organ involvement, followed by hepatitis and gastrointestinal symptoms. Thirteen patients were treated with systemic corticosteroids, and two patients were treated with supportive therapy only. Concomitant with corticosteroid, four patients were given i.v. immunoglobulin. One patient with severe TEN was treated with systemic corticosteroids combined with plasmapheresis and cyclosporine. None of the present patients died. One patient with TEN had severe sequelae, with bronchiolitis obliterans and ocular involvement. SJS/TEN are rare, but are associated with severe complications. General pediatricians need to have up-to-date information regarding these conditions. The present study provides insights into the confirmation of the risk of SJS/TEN as well as the treatment of these diseases.

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

Toxic epidermal necrolysis: A study of clinical profile and treatment outcome in children

Objectives: The objective of this study is to study the clinical and laboratory profile, risk factors for the development of blood culture positive septicemia and treatment outcome of children admitted with toxic epidermal necrolysis (TEN). Methods: All children admitted with TEN from January 2012 to January 2017 were included in the study. Blood culture, organ functions, and serum electrolytes were sent at admission and repeated as clinically indicated. Children were managed with reverse isolation in workforce limited setting of a Government Medical College Hospital. Outcome measure analyzed were the duration of hospital stay, development of blood culture positive septicemia and death. Children were followed up for a variable period from 2-month to 1-year. Results: A total of 13 children were admitted with TEN in the 5-year study period, aged 7.9±3.2 years. Mean area of skin involvement was 69±19.9%. Drugs were found to be the most common cause of TEN accounting for 12 cases (92%). Evidence of renal injury was seen in 6 cases (46%). Disorders of sodium balance were the most common electrolyte abnormality observed in 11 cases (85%). There was no statistically significant difference in duration of progression of skin lesions in children who received ?2 g/kg of intravenous immunoglobulin (IVIG) and those who received &lt;2 g/kg of IVIG. The mortality rate was 7.6%. At follow-up, all patients had dyspigmentation and none had scarring. 6 children (46%) had ophthalmic complaints at follow-up. Conclusion: Non-steroidal anti-inflammatory drugs were found to be the most common cause of TEN in children. Furthermore, TEN was found to be a disease causing critical kidney disease in children. Multi-organ dysfunction, hyperglycemia, and need for invasive ventilation were found to be associated with increased risk of developing culture positive sepsis. Low-dose IVIG, parenteral steroids and skin care with normal saline washes and sterile liquid paraffin impregnated gauze are the effective and safe treatment options for children with TEN in resource-limited setting.

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 &lt; 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