Stevens-Johnson syndrome and toxic epidermal necrolysis: a review

Rodrigo Banegas Ruiz, Alan I. Valderrama Treviño, Gómez Mendoza F. F., Baca Domínguez C. R., Campos Angulo G., Alejo Fuentes L. F., Jiménez Nateras C. N., Ramos-Lojero Víctor Manuel, Espejel Blancas J. A., Baltazar Barrera Mera


Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are considered a single entity with variability in the extent of the lesions, characterized by erythema multiforme that may involve mucosa. Severe cutaneous reactions secondary to medications are classified according to the area of epidermal detachment. The activation of cytotoxic T cells and macrophages is mediated mainly by IL-2 and interferon gamma secreted by Th1 lymphocytes, and the activation of eosinophils and B lymphocytes in IgE is mediated by secreted IL-4, IL-5, IL-10 and IL13 by B lymphocytes. The topography of SJS is predominantly central, affecting the trunk and sometimes a generalized dissemination is shown that affects a body surface area of less than 10%, characterized by irregular violaceous erythematous macules of target shooting, which can form confluent blisters. TEN is characterized by a skin detachment greater than 30% of the body surface, whose predominant lesion is diffuse erythema with individual macules, which give rise to detachment surfaces greater than 5 cm. The treatment is symptomatic, nonspecific, and aimed at avoiding complications, carried out in specialized intensive care units, due to ignorance of the pathogenesis. Integral management with different therapeutic alternatives can represent a crucial part in the multisystemic management of SJS and TEN.


Blisters, Complications, Drugs, Erythema, Mucous membranes, SJS, Skin rash, TEN

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Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia. Report of two cases in children. Am J Dis Child. 1922;24(6):526-33.

Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol. 1956;68(11):355-61.

Mockenhaupt, M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol. 2011;7(6):803-15.

Mustafa SS, Ostrov D, Yerly D. Severe Cutaneous Adverse Drug Reactions: Presentation, Risk Factors, and Management. Curr Allergy Asthma Rep. 2018;18(4):26.

Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis. J Am Academy Dermatol. 2013;69(2):173e1-13.

Heng YK, Lee HY, Roujeau JC. Epidermal necrolysis: 60 years of errors and advances. British J Dermatol. 2015;173(5):1250-4.

Sassolas B, Haddad C, Mockenhaupt M, Dunant A, Liss Y, Bork K, et al. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson syndrome and toxic epidermal necrolysis: comparison with case-control analysis. Clin Pharm Ther. 2010;88(1):60-8.

Frey N, Bodmer M, Bircher A, Jick SS, Meier CR, Spoendlin J. Stevens-Johnson Syndrome and toxic epidermal necrolysis in association with commonly prescribed drugs in outpatient care other than anti-epileptic drugs and antibiotics: a population-based case-control study. Drug Saf. 2019 Jan;42(1):55-66.

Chen CB, Abe R, Pan RY, Wang CW, Hung SI, Tsai YG, et al. An updated review of the molecular mechanisms in drug hypersensitivity. J Immunol Res. 2018;6431694.

Chen P, Lin JJ, Lu CS, Ong CT, Hsieh PF, Yang CC, et al. Carbamazepine-induced toxic effects and HLA-B*1502 screening in Taiwan. N Engl J Med. 2011 Mar 24;364(12):1126-33.

McCormack M, Alfirevic A, Bourgeois S, Farrell JJ, Kasperavičiūtė D, Carrington M, et al. HLA-A*3101 and carbamazepine-induced hypersensitivity reactions in Europeans. N Engl J Med. 2011;364(12):1134-43.

Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY, et al. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nature Med. 2008;14(12):1343-50.

Su SC, Chung WH. Cytotoxic Proteins and Therapeutic Targets in Severe Cutaneous Adverse Reactions. Toxins. 2014;6(1):194-210.

Hsu DY, Brieva J, Silverberg NB, Silverberg JI. Morbidity and mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis in united states adults. J Invest Dermatol. 2016;136(7):1387-97.

Garcia-Doval I, LeCleach L, Bocquet H, Otero X, Roujeau J. Toxic epidermal necrolysis and Stevens-Johnson syndrome does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol. 2000;136(3):323-7.

Tirado-Sánchez A, Ponce-Olivera RM, Montes de Oca-Sánchez G. Causas de mortalidad en un servicio de dermatología. Dermatología Rev Mex. 2007;51(1):1-3.

Karimkhani C, Dellavalle RP, Coffeng LE, Flohr C, Hay RJ, Langan SM, et al. Global skin disease morbidity and mortality an update from the global burden of disease study. JAMA Dermatol. 2013;153(5):406-12.

Bansal S, Garg VK, Sardana K, Sarkar R. A clinicotherapeutic analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis with an emphasis on the predictive value and accuracy of SCORe of Toxic Epidermal Necrolysis. Int J Dermatol. 2015;54(1):18-26.

Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, Shear NH. Stevens-Johnson Syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermat. 2015;16(6):475-93.

Ganapati S. Eponymous dermatological signs in bullous dermatoses. Indian J Dermatol. 2014;59(1):21-23.

Chantaphakul H, Sanon T, Klaewsongkram J. Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis. Experiment Therapeut Med. 2015;10(2):519-24.

Ziemer M, Mockenhaupt M. Severe drug-induced skin reactions: clinical pattern, diagnostics and therapy. InSkin Biopsy-Perspectives 2011 Nov 2. IntechOpen.

Lerch M, Mainetti C, Terziroli Beretta-Piccoli BT, Harr T. Current perspectives on Stevens-Johnson syndrome and toxic epidermal necrolysis. Clin Rev Allergy and Immunol. 2017;54(1):147-76.

Kinoshita Y, Saeki H. A review of the active treatments for toxic epidermal necrolysis. J Nippon Med School. 2017;84(3):110-7.

Chatproedprai S, Wutticharoenwong V, Tempark T, Wananukul S. Clinical features and treatment outcomes among children with Stevens-Johnson syndrome and toxic epidermal necrolysis: a 20-year study in a tertiary referral hospital. Dermatology Research and Practice. 2018;1-9.

Schneider JA, Cohen PR. Stevens-Johnson Syndrome and toxic epidermal necrolysis: a concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures. Adv Ther. 2017;34(6):1235-44.

Hajirah Saeed, Iason S. Mantagos, James Chodosh. Complications of Stevens-Jonhson Síndrome beyond the eye and skin. Burns. 2016;42(1):20-7.