Immunologic Mediators in Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

Abstract

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.

Cefathiamidine-Induced Toxic Epidermal Necrolysis in a Child

“…The results of clinical and histopathological studies suggest that TEN is a specific hypersensitive immune response induced by drugs. Cytotoxic T lymphocyte is the main effector cells, cytotoxic proteins such as cell membrane receptor molecules Fas and its ligand Fasl, perforin and cytokine TNF-α play an important role [5][6]. Our patient had a high levels of IL-2R, IL-6, IL-10 and TNF-α, which supported above studies.…”

Multidisciplinary care in Stevens-Johnson syndrome

“…The use of systemic corticosteroids, intravenous immunoglobulins, and tumor necrosis factor (TNF)-alpha inhibitors have all been described in the acute phase, with mortality results varying from improved mortality, no benefit, to increased mortality. 5,20,24 Recent data on cyclosporine, however, includes a report that compared cyclosporine to other systemic therapies, and found that cyclosporine reduced mortality in SJS/TEN patients. 25 A recently published systematic review of treatment of SJS/TEN in the acute phase also reported a significant benefit of cyclosporine.…”

Rapid remission of Stevens-Johnson syndrome by combination therapy using etanercept and intravenous immunoglobulin and a review of the literature

“…Complete re-epithelialization was reached in a week (Figure 1d 3 | DISCUSSION SJS/TEN are a spectrum of rare, acute, and life-threatening delayedtype drug hypersensitivity reactions that are most commonly caused by drugs and, rarely, infections. The conditions are characterized by extensive skin necrosis, epidermal detachment, and involvement of mucous membrane, which typically occur within 1-3 weeks after the administration of the suspected drugs (Saeed & Chodosh, 2016). Antibiotics, anticonvulsants, and nonsteroidal anti-inflammatory drugs are the main triggers of this severe dermatosis (Gubinelli, Canzona, Tonanzi, Raskovic, & Didona, 2009).…”