Abstract
Purpose of Review
This review highlights current concepts in the management of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).
Recent Findings
A new scoring system, the ABCD-10, was recently developed to better estimate mortality among SJS/TEN patients. Supportive care remains the mainstay of treatment, and includes wound care, fluid and electrolyte management, management of medical co-morbidities, and infection control. The value of adjuvant therapy remains unclear, but new recent retrospective studies suggest that the combination therapies may be efficacious. Recent prospective studies investigating cyclosporine and etanercept have shown promise in the treatment of SJS/TEN.
Summary
SJS and TEN are severe mucocutaneous drug reactions associated with high morbidity and mortality. Supportive care is the most universally accepted therapy, although specific strategies may vary among institutions. Adjuvant therapies include corticosteroids, IVIG, cyclosporine, TNF alpha inhibitors, and plasmapheresis but prospective data is still lacking. Clinical trials that may better elucidate their efficacy are currently under way.
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References
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Paradisi A, Abeni D, Bergamo F, Ricci F, Didona D, Didona B. Etanercept therapy for toxic epidermal necrolysis. J Am Acad Dermatol. 2014;71(2):278–83. https://doi.org/10.1016/j.jaad.2014.04.044.
• Wang CW, Yang LY, Chen CB, Ho HC, Hung SI, Yang CH, et al. Randomized, controlled trial of TNF-alpha antagonist in CTL-mediated severe cutaneous adverse reactions. J Clin Invest. 2018;128(3):985–96. https://doi.org/10.1172/JCI93349This randomized, controlled trial of 96 patients with SJS/TEN found that Eetanercept decreased the SCORTEN-predicted mortality rate compared to with corticosteroids. Investigators also observed a significantly reduced skin-healing time in the moderate-to-severely affected patients (p = 0.010). Enteracept groups had reduced granulysin in blister fluids and plasma and an increased Treg cell population (p = 0.002) - —providing insights on the potential mechanism-of-action.
Shah R, Chen ST, Kroshinsky D. Use of cyclosporine for the treatment of Steven Johnson syndrome/ toxic epidermal necrolysis. J Am Acad Dermatol. 2019. https://doi.org/10.1016/j.jaad.2018.09.063.
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Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000;115(2):149–53. https://doi.org/10.1046/j.1523-1747.2000.00061.x.
• Morita K, Matsui H, Michihata N, Fushimi K, Yasunaga H. Association of early systemic corticosteroid therapy with mortality in patients with Stevens-Johnson syndrome or toxic epidermal necrolysis: a retrospective cohort study using a nationwide claims database. Am J Clin Dermatol. 2019;30:30 This large retrospective cohort study of 1846 patients evaluated the association of early systemic corticosteroid therapy with mortality in patients with SJS/TEN. Their results indicated that eary systemic corticosteroid therapy was not associated with lower in-hospital mortality.
• Liu W, Nie X, Zhang L. A retrospective analysis of Stevens-Johnson syndrome/toxic epidermal necrolysis treated with corticosteroids. Int J Dermatol. 2016;55(12):1408–13. https://doi.org/10.1111/ijd.13379This study retrospective analysis of 70 patients with SJS/TEN evaluated a response to treatment with corticosteroids. The study supports the use of corticosteroids in SJS/TEN as it was associated with lower mortality rate and serum albumin levels. Investigators conclude that corticosteroids should be administered in accordance with disease severity, age, underlying diseaes, serum albumin level, and concurrent antimicrobial therapy.
Roongpisuthipong W, Prompongsa S, Klangjareonchai T. Retrospective analysis of corticosteroid treatment in Stevens-Johnson syndrome and/or toxic epidermal necrolysis over a period of 10 years in Vajira Hospital, Navamindradhiraj University, Bangkok. Dermatol Res Pract. 2014;2014:237821. https://doi.org/10.1155/2014/237821.
• Lee HY, Fook-Chong S, Koh HY, Thirumoorthy T, Pang SM. Cyclosporine treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis: retrospective analysis of a cohort treated in a specialized referral center. J Am Acad Dermatol. 2017;76(1):106–13. https://doi.org/10.1016/j.jaad.2016.07.048This retrospective analysis of 44 patients sought to evaluate the impact of cyclosporine on hospital mortality in patients with SJS/TEN. 24 patients received cyclosporine and 20 were treated supportively. SCORTEN predicted 7.5 deaths in the cyclosporine group, and 3 were observed. SCORTEN predicted 5.9 deaths in the supportive group, and 6 were observed. Thus, the standardized mortality ratio of SJS/TEN treated with cyclosporine was 0.42. They concluded that the use of cyclosporine may improve mortality in SJS/TEN.
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Coias, J.L., Abbas, L.F. & Cardones, A.R. Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: a Review and Update. Curr Derm Rep 8, 219–233 (2019). https://doi.org/10.1007/s13671-019-00275-0
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DOI: https://doi.org/10.1007/s13671-019-00275-0