Abstract:A review of the current evidence reveals a need for prospective, randomized controlled studies to provide more definitive conclusions on steroid use in patients with SJS, TEN, and/or overlap.
“…Systemic corticosteroids, however, have shown variable success in reducing both ocular sequelae and patient mortality. 52 In comparative studies, the impact of high-dose pulsed systemic corticosteroids on ocular outcomes ranges from no benefit to possible benefit. 37,38,53 The systemic impact of corticosteroids is even more variable, with no benefit, increased complications including sepsis, and a mortality benefit all being reported.…”
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of T-cell mediated immune disorders. While the contributory mechanisms leading to the apoptosis of epidermal cells in SJS/TEN remain unproven, the keratinocyte apoptosis seen in SJS/TEN is thought to occur through the T-cell mediated Fas-Fas ligand (FasL), perforin/granzyme B, and other immune mediators. Most recently, emphasis has been placed on the granulysin pathway as being the primary mediator of apoptosis and widespread epidermal necrosis in SJS/TEN. This article aims to review the proposed mechanisms by which these pathways work and the immunomodulatory therapies that have been developed in an attempt to target them.
“…Systemic corticosteroids, however, have shown variable success in reducing both ocular sequelae and patient mortality. 52 In comparative studies, the impact of high-dose pulsed systemic corticosteroids on ocular outcomes ranges from no benefit to possible benefit. 37,38,53 The systemic impact of corticosteroids is even more variable, with no benefit, increased complications including sepsis, and a mortality benefit all being reported.…”
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of T-cell mediated immune disorders. While the contributory mechanisms leading to the apoptosis of epidermal cells in SJS/TEN remain unproven, the keratinocyte apoptosis seen in SJS/TEN is thought to occur through the T-cell mediated Fas-Fas ligand (FasL), perforin/granzyme B, and other immune mediators. Most recently, emphasis has been placed on the granulysin pathway as being the primary mediator of apoptosis and widespread epidermal necrosis in SJS/TEN. This article aims to review the proposed mechanisms by which these pathways work and the immunomodulatory therapies that have been developed in an attempt to target them.
“…Systemic corticosteroids (22), intravenous immunoglobulin (IVIG) (23), and cyclosporine (24) are often used to treat CTL-mediated SCARs; however, the clinical outcomes of these therapies remain controversial (25,26). Recently, several studies revealed that treatment with anti-TNF-α biologic agents appeared beneficial in patients with TEN and TEN-like acute cutaneous lupus erythematosus (17,(27)(28)(29)(30)(31)(32)(33)(34)(35)(36).…”
Section: Preclinical Testing Of Etanercept: Ex Vivo Testing For Potenmentioning
losing spondylitis, juvenile rheumatoid arthritis, plaque psoriasis, and psoriatic arthritis, its use in treating CTL-mediated SCARs has not yet been approved (37). We aimed to evaluate the efficacy and safety of etanercept for CTL-mediated SCARs treatment in this randomized trial and further investigate the therapeutic mechanism of etanercept in CTL-mediated SCARs.
“…For SJS/TEN, corticosteroids are the cornerstone of treatment albeit efficacy remains unclear. Despite improved knowledge of the immunopathogenesis of these conditions, immune-modulatory therapies currently used have not been definitively proved to be efficacious [49, 107], and new strategies are urgently needed.Fig. 3Management of patients with a suspected drug induced exfoliative dermatitis…”
Section: Discussionmentioning
confidence: 99%
“…In SJS, SJS/TEN and TEN the efficacy of corticosteroids is far from being demonstrated. Recently, a meta-analysis based on 6 retrospective studies evaluating the role of corticosteroids alone or together with IVIG has been published [107]. In this study, 965 patients were reviewed.…”
Drug induced exfoliative dermatitis (ED) are a group of rare and severe drug hypersensitivity reactions (DHR) involving skin and usually occurring from days to several weeks after drug exposure. Erythema multiforme (EM), Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Overall, T cells are the central player of these immune-mediated drug reactions. Here we provide a systematic review on frequency, risk factors, pathogenesis, clinical features and management of patients with drug induced ED.Electronic supplementary materialThe online version of this article (doi:10.1186/s12948-016-0045-0) contains supplementary material, which is available to authorized users.
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