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

McPherson, Tess; Exton, L.S.; Biswas, Susmito; Creamer, Daniel; Dziewulski, Peter; Newell, Louise; Tabor, K.L.; Wali, G.N.; Walker, Greg; Walker, Rhonda; Walker, Stuart H.; Young, Amber; Mustapa, M.F. Mohd; Murphy, Ruth; Levell, N. J.; McHenry, P.; Leslie, Tabi A.; Wakelin, S. H.; Hunasehally, R.Y.P.; Cork, Michael J.; Johnston, Graham A.R.; Chiang, N.; Worsnop, Fiona; Rakvit, Pariyawan; Salim, Asad; McDonald, Bryan; Chua, Ser Ling; Buckley, David; Petrof, Gabriela; Hussain, Faezahtul Arbaeyah; Bardhan, Ajoy; Callachand, N.; Flavell, T.; Salad, A.A.

doi:10.1111/bjd.17841

Cited by 63 publications

(79 citation statements)

References 70 publications

(141 reference statements)

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“…Information on the management of children versus adults is also lacking given that a much higher proportion of cases in children are mediated by infectious and non-drug triggers. However, recent guidelines are useful for clinicians if such cases arise [12].…”

Section: Clinical Approaches and Managementmentioning

confidence: 99%

SJS/TEN 2019: From science to translation

Chang

Abe

Anderson

et al. 2020

Journal of Dermatological Science

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Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially lifethreatening, immune-mediated adverse reactions characterized by widespread erythema, epidermal necrosis, and detachment of skin and mucosa. Efforts to grow and develop functional international collaborations and a multidisciplinary interactive network focusing on SJS/TEN as an uncommon but high burden disease will be necessary to improve efforts in prevention, early diagnosis and improved acute and long-term management. SJS/TEN 2019: From Science to Translation was a 1.5-day scientific program held April 26-27, 2019, in Vancouver, Canada. The meeting successfully engaged clinicians, researchers, and patients and conducted many productive discussions on research and patient care needs.

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Section: Clinical Approaches and Managementmentioning

confidence: 99%

SJS/TEN 2019: From science to translation

Chang

Abe

Anderson

et al. 2020

Journal of Dermatological Science

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“…Dermatological status, facial, anterior and posterior thorax, abdominal, bilateral brachial, bilateral antebracial, perineum, bilateral femoral, there are erythematous macules, well-defined, multiple numular-plaque sizes, and also bullae, purpura, erosion, and epidermolysis; orbital: bilateral conjunctival hyperemia; labium oris: Erosion and black crust; genital: Erosion (total 86% blistered skin). Laboratory tests show, hemoglobin 14.2 g/dL, leukocytes 10.400/uL, platelets 403.000/mm 3 , Albumin 3.6 g/dL, ureum 48 mg/dL, cre-Introduction TEN (Toxic Epidermal Necrolysis) is an acute life-threatening drug reaction and is characterized by involvement of the mucous membrane and skin, where extensive necrosis can occur and erode the epidermis layer [1][2][3]. Most causes of this disease are due to drugs.…”

Section: Case Descriptionmentioning

confidence: 99%

“…This disease is a rare case, there are about 5-6 cases per year with a high mortality rate around more than 50% [1]. Severe TEN cases can be accompanied by systemic complications resulting in organ failure and death [3]. Her-erythematous macules, well-demarcated, multiple numular-plaque, and also bullae, purpura, erosion, crust, and epidermolysis; orbital: bilateral conjunctival hyperemia; labium oris: erosion and black crust; genital: erosion (total 86% blistered skin).…”

Section: Case Descriptionmentioning

confidence: 99%

“…Dermatological status, facial, anterior and posterior thorax, abdominal, bilateral brachial, bilateral antebracial, perineum, bilateral femoralis, bilateral cruris, there are skin layer will release serum so that it bleeds easily, leakage of albumin happens, and easily get infected [2,3,8]. Various complications can occur in TEN such as dehydration, sepsis, pneumonia, and also multiorgan dysfunction such as ulceration and gastrointestinal perforation, acute renal failure, thromboembolism, and DIC (Disseminated Intravascular Coagulopathy) [3,9,10]. In both of these patients fluid, albumin and protein loss occurred.…”

Section: Case Descriptionmentioning

confidence: 99%

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Albumin, Important Therapy & When to Use It in Ten Patients (Adult & Child): Case Report

Nathasia¹,

Tansil²

2020

J Dermatol Res Ther

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Toxic epidermal necrolysis (TEN) is a life-threatening drug reaction that requires immediate medical treatment. Mortality due to TEN cases is quite high until now and the main cause reported is multiple organ dysfunction syndrome. A 7-year-old child, was diagnosed TEN with 86% blistered skin, after receiving Metampiron. Given 20% albumin 50 ml from the first day treated until 20 days, also fluid resucitation, steroids, antibiotics, vitamin C, neurobion, paracetamol, antihistamines, oral hygiene, steroid and antibiotic ointment for body, eye ointment, woundcare, and high nutritions. The other case, 33-year-old woman, was diagnosed TEN with 100% blister skin, due to Phenytoin. Given 25% albumin 100 ml from the first day to 20 days. Both patients recover without complications. In TEN, hypoalbumin often occurs due to eroded skin. Hypoalbumin can reduce organ function and drug effectiveness. Drugs that are usually bound to protein become free in plasma and metabolism in hepatic become more rapid. Immidiate albumin therapy can prevent this and reduce patient mortality. The albumin also plays a role to accelerate skin regeneration.

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“…However, similarly to adults, increased mortality is noted with higher degrees of epidermal detachment: 60–70% BSA involvement was associated with 37.5% mortality in one study . Intravenous immunoglobulin (IVIG), ciclosporin and/or systemic corticosteroids have been used despite the relative lack of evidence, as summarized in recent guidelines . Bronchiolitis obliterans (BO) is a rarely reported complication, but when present, is associated with significant long‐term morbidity.…”

mentioning

confidence: 99%

Bronchiolitis obliterans as a long‐term sequela of Stevens–Johnson syndrome and toxic epidermal necrolysis in children

et al. 2019

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Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are characterized by widespread skin and mucosal blistering and necrosis. The triggers and long-term sequelae in children may differ from those reported for adults. Bronchiolitis obliterans (BO) is an uncommon complication, with only 15 previously reported cases, but can lead to significant long-term morbidity, requiring lung transplantation in some cases. We report three children with nondrug-related SJS (n = 1) and TEN (n = 2) who developed BO. Two were treated with intravenous immunoglobulin therapy (2-2.4 g/kg) and all three survived. We highlight salient learning points from our cases and potential pitfalls in diagnosis of BO, including delayed onset, and we also review the literature.Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, potentially life-threatening conditions, characterized by widespread skin and mucosal necrosis. SJS is defined as < 10% body surface area (BSA) detachment, SJS-TEN overlap as 10-30% BSA detachment and TEN as > 30% detachment. 1 The reported incidence is lower in children than adults: 5.3 vs. 9.3, 0.8 vs. 1.9 and 0.4 vs. 1.6 cases per million per year for SJS, SJS-TEN and TEN, respectively, in the USA. 2,3 Medications are usually culpable, although infections may be implicated, particularly in children. 4 Mortality in childhood is reportedly 0% (SJS), 4% (SJS-TEN) and 16% (TEN), 2 with deaths most frequently due to sepsis and organ failure. 2 However, similarly to adults, increased mortality is noted with higher degrees of epidermal detachment: 60-70% BSA involvement was associated with 37.5% mortality in one study. 5 Intravenous immunoglobulin (IVIG), ciclosporin and/or systemic corticosteroids have been used despite the relative lack of evidence, as summarized in recent guidelines. 6 Bronchiolitis obliterans (BO) is a rarely reported complication, but when present, is associated with significant long-term morbidity. We report three children with nondrug-related SJS/TEN who developed BO. ReportPatient 1 was a previously well 5-year-old boy who presented with a 2-day history of an erythematous maculopapular rash in association with fever and coryza. Overall, the skin changes involved 95% BSA, with 40% epidermal detachment. There were additional bilateral conjunctival defects with confluent superficial punctate epithelial erosions of the cornea. He had severe oral and upper airway ulceration with sloughing. Subsequent development of stridor necessitated intubation.Histological examination of a skin biopsy confirmed TEN, with negative direct immunofluorescence. Chest radiography was unremarkable. Bronchoscopy showed ulceration at the base of insertion of the endotracheal

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

Abstract: This summary relates to https://doi.

Cited by 63 publications

References 70 publications

SJS/TEN 2019: From science to translation

SJS/TEN 2019: From science to translation

Albumin, Important Therapy & When to Use It in Ten Patients (Adult & Child): Case Report

Bronchiolitis obliterans as a long‐term sequela of Stevens–Johnson syndrome and toxic epidermal necrolysis in children

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