Pediatric SJS/TEN Subdued by a Combination of Dexamethasone, Cyclosporine, and Etanercept

Coulombe, Jérôme; Belzile, Eugénie; Duhamel, Amélie; Rault, Pauline; Buteau, Chantal; Bruycker, Jean Jacques De; Bussières, Jean-François

doi:10.1177/1203475419861078

Cited by 23 publications

(15 citation statements)

References 12 publications

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“…As a result, there is no established standard of care pertaining to pharmacologic treatment. Due to the immunologic nature of the disease, it is believed that immunosuppressive therapies will aid in treatment, and many case reports have reported positive results with varying treatment regimens involving different combinations of corticosteroids, IVIg, cyclosporine, and TNF-alpha inhibitors [ 21 , 78 , 79 , 80 , 81 ]. However, it is difficult to determine if disease remission was due to specific treatment or simply the natural history of the disease.…”

Section: Clinical Updatesmentioning

confidence: 99%

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

et al. 2021

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Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease.

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Section: Clinical Updatesmentioning

confidence: 99%

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

et al. 2021

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“…Excitingly, TNF-α antagonists are translated into clinical therapeutics for SJS/TEN in recent years. Tons of evidence supports TNF antagonists halt SJS/TEN (Paradisi et al, 2014;Gavigan et al, 2018;Shear et al, 2018;Trujillo-Trujillo et al, 2018;Chafranska et al, 2019;Coulombe et al, 2019;Pham et al, 2019;Zhang et al, 2020). A randomized controlled trial compared etanercept (a TNF inhibitor) with systemic corticosteroid treatment in adult SJS/TEN patients and showed a decreased SCORTEN-predicted mortality rate, reduced skin healing time, and less gastrointestinal hemorrhage in the etanercept group.…”

Section: Tnf-α-related Apoptosismentioning

confidence: 99%

Current Pharmacogenetic Perspective on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Cheng

2021

Front. Pharmacol.

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Adverse drug reactions are a public health issue that draws widespread attention, especially for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) which have high mortality and lack of efficacious treatment. Though T-cell-mediated HLA-interacted immune response has been extensively studied, our understanding of the mechanism is far from satisfactory. This review summarizes infection (virus, bacterial, and mycoplasma infection), an environmental risk factor, as a trigger for SJS/TEN. The mutations or polymorphisms of drug metabolic enzymes, transporters, receptors, the immune system genes, and T-cell-mediated apoptosis signaling pathways that contribute to SJS/TEN are discussed and summarized. Epigenetics, metabolites, and mobilization of regulatory T cells and tolerogenic myeloid precursors are emerged directions to study SJS/TEN. Ex vivo lymphocyte transformation test has been exploited to aid in identifying the causative drugs. Critical questions on the pathogenesis of SJS/TEN underlying gene polymorphisms and T cell cytotoxicity remain: why some of the patients carrying the risky genes tolerate the drug and do not develop SJS/TEN? What makes the skin and mucous membrane so special to be targeted? Do they relate to skin/mucous expression of transporters? What is the common machinery underlying different HLA-B alleles associated with SJS/TEN and common metabolites?

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“…51 Reports of etanercept efficacy in paediatric medication-induced SJS and TEN suggest promise. [73][74][75] A recent meta-analysis in the respiratory literature showed that serum TNF-a levels are elevated in MP pneumonia. 76 This suggests that it is possible we may see a dichotomous response to anti-TNF agents in RIME caused by bacteria such as MP or C. pneumoniae compared with RIME caused by viruses such as influenza B.…”

Section: Treatmentmentioning

confidence: 99%

“…Infliximab was effective in one case that was also treated with meropenem 51 . Reports of etanercept efficacy in paediatric medication‐induced SJS and TEN suggest promise 73‐75 . A recent meta‐analysis in the respiratory literature showed that serum TNF‐α levels are elevated in MP pneumonia 76 .…”

Section: Treatmentmentioning

confidence: 99%

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Reactive infectious mucocutaneous eruption: Mycoplasma pneumoniae ‐induced rash and mucositis and other parainfectious eruptions

Ramien

2020

Clin Exp Dermatol

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Summary Mycoplasma pneumoniae‐induced rash and mucositis is the most accurate diagnosis for patients with blistering mucocutaneous disease provoked by an infection. Recent literature suggests expansion of the name is required, as other infections have caused a clinically similar presentation. This review provides a concise update on current understanding of M. pneumoniae‐induced rash and mucositis and other reactive infectious mucocutaneous eruptions.

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Pediatric SJS/TEN Subdued by a Combination of Dexamethasone, Cyclosporine, and Etanercept

Cited by 23 publications

References 12 publications

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

Current Pharmacogenetic Perspective on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Reactive infectious mucocutaneous eruption: Mycoplasma pneumoniae ‐induced rash and mucositis and other parainfectious eruptions

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