Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of treatment options

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous reactions that are medication-induced in most instances. While the clinical manifestations of SJS and TEN are well-defined, the optimal treatment for these disorders is not. Case reports have shown benefit with the use of a variety of agents including tumor necrosis factor-alpha inhibitors and cyclophosphamide, whereas thalidomide was associated with an increased mortality. Plasmapheresis and cyclosporine have also demonstrated efficacy anecdotally, albeit with an even smaller number of cases in the literature. Most of the reporting has focused on the use of systemic corticosteroids and intravenous immunoglobulin (IVIG) for these severe reactions. The majority of studies analyzing the use of IVIG in the treatment of SJS/TEN show a benefit, though more recent series cast doubt upon this conclusion. The results of these studies are summarized in this present review study.

Treatment of Toxic Epidermal Necrolysis with the Combination of Infliximab and High-Dose Intravenous Immunoglobulin

“…The aim of the present therapeutic scheme is to combine the advantages of the three modalities currently proposed for TEN [1,2,5]. Although the pathophysiology of TEN is still not fully understood, extensive keratinocyte apoptotic cell death through cytotoxic T cell activation, Fas-Fas ligand interaction and increased TNFα levels underlies skin destruction [2].…”

“…The rarity of TEN (incidence: 0.4–1.2 cases per million) [4] has made comparative therapeutic studies quite difficult to perform. Suggested treatment options include high-dose corticosteroids with overall contradicting results, high-dose intravenous immunoglobulin (IVIg), cyclosporine and plasmapheresis in addition to supportive best care in burn units or equivalent facilities [1,5,6,7]. More recently, based on pathophysiological considerations, tumor necrosis factor alpha (TNFα)-targeting biologicals have also been used in a limited number of patients with overall promising results [8,9,10,11,12,13].…”

Adult Staphylococcal Scalded Skin Syndrome Successfully Treated with Plasma Exchange

“…Systemic glucocorticoid treatment is commonly indicated for drug eruptions including TEN (14), but is contraindicated for SSSS (6). When treating an adult patient in which clinical differentiation of SSSS from TEN is difficult, we should not use systemic glucocorticoid before the results of histopathological and bacterial examination are revealed.…”

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

“…Useful articles [23][24][25] on wound care management in TEN patients have been published. But no clear guidelines or even case reports with practical recommendations for delivery of nursing care to patients undergoing plasmapheresis are available.…”