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Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are often associated with drug induction and can result in morbidity and mortality, even when managed in intensive care or burn units. Effective therapy has eluded us; the only randomized, controlled trial of therapy proved that thalidomide was not only ineffective but was associated with increased mortality. Since that time, there have been no controlled trials, and the literature is filled with open-label experiences that purport to show benefit from a variety of therapies. In 1998, Viard and colleagues (NEJM JW Dermatol Dec 1 1998) described decreased mortality with intravenous immunoglobulin (IVIG) therapy. However, reports have been conflicting.
These authors retrospectively analyzed outcomes of treatment with IVIG 1 gm/kg/day for 3 days or cyclosporine 3 to 5 mg/kg/day for up to 7 days. Among 64 patients with clinical and histologic evidence of SJS/TEN, 12 were treated conservatively, 35 were treated with IVIG, 15 with cyclosporine, and 2 with both IVIG and cyclosporine. Using calculations based on the patient's severity-of-illness score for TEN (SCORTEN), IVIG recipients had higher mortality than predicted, and cyclosporine recipients had lower than predicted mortality.
Kirchhof MG et al. Retrospective review of Stevens-Johnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol 2014 Jul 30; [e-pub ahead of print]. (http://dx.doi.org/10.1016/j.jaad.2014.07.016)
Comment
These findings coupled with other observations that corticosteroids offer a benefit have led us in my practice to change our protocol to utilization of cyclosporine. We also consider the possibility of using etanercept (NEJM JW Dermatol Jul 22 2014).