Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis, A Review of Pathogenesis, Clinical Features, Diagnosis and Treatment
DOI:
https://doi.org/10.12775/JEHS.2022.12.09.060Keywords
Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, cutaneous adverse drug reactionsAbstract
Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, acute conditions, potentially life-threatening, immune-mediated and often unpredictable.
Characteristic for SJS and TEN is acute necrosis of the epidermis and mucous membranes, caused by the extensive death of keratinocytes. These syndromes are considered hypersensitivity reactions. They are most often caused by drugs. There have also been reports of SJS / TEN being caused by infection,
SJS/TEN disease is very rare and due to its rarity there is no specific pharmaceutical algorythm. Supportive care and treatment of symptoms are very important. The most crucial part of non-pharmacologic treatment of SJS/TEN is the detection and cessation of the pharmaceutical that caused the disease.
The aim of this literature review was to summarize current knowledge about the pathogenesis, clinical features, diagnosis and treatment of Stevens- Johnson Syndrom and Toxic Epidermal Necrolysis.
Standard criteria were used to review the literature data. The search of articles in the PubMed and Google Scholar database was carried out using the following keywords: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, cutaneous adverse drug reactions
References
Roujeau JC, Kelly JP, Naldi L, Rzany B et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med
Roujeau JC, Guillaume JC, Fabre JP, Penso D et al. Toxic epidermal necrolysis (Lyell syndrome). Incidence and drug etiology in France, 1981-1985. Arch Dermatol 1990; 126:37–42.
Rzany B, Mockenhaupt M, Stocker U, Hamouda O, Schopf E. Incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with the acquired immunodeficiency syndrome in Germany. Arch Dermatol 1993;129:1059.
Ueta M., Sotozono C ., Tokunaga K., et al.: Strong association between HLA-A*0206 and Stevens-Johnson syndrome in the Japanese. Am. J. Ophthalmol., 2007; 143 (2): 367–368
Lee HY, Chung WH. Toksyczna nekroliza naskórka: przegląd roku. Curr Opin Allergy Clin Immunol. 2013; 13(4):330-6.
Ludlam G.B., Bridges J.B.: Association of Stevens-Johnson syndrome with antibody for Mycoplasma pneumoniae. Lancet, 1964; 1 (7340): 958–959
Duong TA, Valeyrie-Allanore L., Wolkenstein P., Chosidow O. Ciężkie skórne reakcje niepożądane na leki. Lancet. 2017; 390 : 1996-2011. doi: 10.1016/S0140-6736(16)30378-6.
Patterson R., Miller M., Kaplan M., et al.: Effectiveness of early therapy with corticosteroids in Stevens-Johnson syndrome: experience with 41 cases and a hypothesis regarding pathogenesis. Ann. Allergy, 1994; 73 (1): 27–34
Frantz R, Huang S, Are A, Motaparthi K. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management. Medicina (Kaunas). 2021 Aug 28;57(9):895. doi: 10.3390/medicina57090895. PMID: 34577817; PMCID: PMC8472007.
Abe R. Odpowiedź immunologiczna w zespole Stevensa-Johnsona i toksycznej martwicy naskórka. J. Dermatol. 2015; 42 :42–48. doi: 10.1111/1346-8138.12674.
Su S.-C., Mockenhaupt M., Wolkenstein P., Dunant A., le Gouvello S., Chen C.-B., Chosidow O., Valeyrie-Allanore L., Bellon T., Sekula P. i in. Interleukina-15 jest powiązana z ciężkością i śmiertelnością w zespole Stevensa-Johnsona/toksycznej nekrolizie naskórka. J. Dochodzenie. Dermatol. 2017; 137 :1065-1073. doi: 10.1016/j.jid.2016.11.034.
W. Hussain, N. M Craven, Burnley General Hospital, Toxic epidermal necrolysis and Stevens-Johnson syndrome, CME Dermatology, Clin Med 2005;5:555–8
A. Wong, A. Augusto Malvestiti, M. de Figueiredo Silva Hafner; Síndrome de Stevens-Johnson e necrólise epidérmica tóxica: revisão
https://doi.org/10.1590/1806-9282.62.05.468
Shanbhag S., Chodosh J., Fathy C., Goverman J., Mitchell C., Saeed HN Opieka multidyscyplinarna w zespole Stevensa-Johnsona.Tam.Przysł.Przew.2020;11 doi: 10.1177/2040622319894469.
Coias J.L., Abbas L.F., Cardones A.R. Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Review and Update. Curr. Dermatol. Rep. 2019;8:219–233. doi: 10.1007/s13671-019-00275-0.
Schneider J.A., Cohen P.R. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Concise Review with a Compre-hensive Summary of Therapeutic Interventions Emphasizing Supportive Measures. Adv. Ther. 2017;34:1235–1244. doi: 10.1007/s12325-017-0530-y.
Coias J.L., Abbas L.F., Cardones A.R. Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Review and Update. Curr. Dermatol. Rep. 2019;8:219–233. doi: 10.1007/s13671-019-00275-0.
Coias J.L., Abbas L.F., Cardones A.R. Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Review and Update. Curr. Dermatol. Rep. 2019;8:219–233. doi: 10.1007/s13671-019-00275-0.
Palmieri T.L., Greenhalgh D.G., Saffle J.R., Spence R.J., Peck M.D., Jeng J.C., Mozingo D.W., Yowler C.J., Sheridan R.L., Ahrenholz D.H., et al. A multicenter review of toxic epidermal necroly-sis treated in U.S. burn centers at the end of the twentieth century. J. Burn Care Rehabil. 2002;23:87–96. doi: 10.1097/00004630-200203000-00004.
McCullough M., Burg M., Lin E., Peng D., Garner W. Steven Johnson Syndrome and Toxic Epidermal Necrolysis in a burn unit: A 15-year experience. Burn. 2017;43:200–205. doi: 10.1016/j.burns.2016.07.026.
Dorafshar A.H., Dickie S.R., Cohn A.B., Aycock J.K., O’Connor A., Tung A., Gottlieb L. Antishear therapy for Toxic Epidermal Necrolysis: An alternative treatment approach. Plast. Reconstr. Surg. 2008;122:154–160. doi: 10.1097/PRS.0b013e3181773d5d.
Zhang S., Tang S., Li S., Pan Y., Ding Y. Biologic TNF-alpha inhibitors in the treatment of Stevens-Johnson syndrome and Toxic Epidermal Necrolysis: A systemic review. J. Dermatol. Treat. 2020;31:66–73. doi: 10.1080/09546634.2019.1577548.
Wang C.-W., Yang L.-Y., Chen C.-B., Ho H.-C., Hung S.-I., Yang C.-H., Chang C.-J., Su S.-C., Hui R.C.-Y., Chin S.-W., et al. Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions. J. Clin. Investig. 2018;128:985–996. doi: 10.1172/JCI93349.
Zimmermann S., Sekula P., Venhoff M., Motschall E., Knaus J., Schumacher M., Mockenhaupt M. Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. 2017;153:514–522. doi: 10.1001/jamadermatol.2016.5668.
Sekula P., Dunant A., Mockenhaupt M., Naldi L., Bavinck J.N.B., Halevy S., Kardaun S., Sidoroff A., Liss Y., Schumacher M., et al. Comprehensive Survival Analysis of a Cohort of Patients with Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis. J. Investig. Dermatol. 2013;133:1197–1204. doi: 10.1038/jid.2012.510.
reamer D., Walsh S.A., Dziewulski P., Exton L.S., Lee H.Y., Dart J.K.G., Setterfield J., Bunker C.B., Ardern-Jones M.R., Watson K.M.T., et al. UK guidelines for the management of Ste-vens-Johnson syndrome/Toxic Epidermal Necrolysis in adults 2016. J. Plast. Reconstr. Aesthetic Surg. 2016;69:e119–e153. doi: 10.1016/j.bjps.2016.01.034.
Tsai T.Y., Huang I.H., Chao Y.C., Li H., Hsieh T.S., Wang H.H., Huang Y.T., Chen C.Y., Cheng Y.C., Kuo P.H., et al. Treating Toxic Epidermal Necrolysis with systemic immunomodulating therapies: A systematic review and network meta-analysis. J. Am. Acad. Dermatol. 2021;84:390–397. doi: 10.1016/j.jaad.2020.08.122.
Zimmermann S., Sekula P., Venhoff M., Motschall E., Knaus J., Schumacher M., Mockenhaupt M. Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. 2017;153:514–522. doi: 10.1001/jamadermatol.2016.5668.
Gilbert M., Scherrer L.A. Efficacy and safety of cyclosporine in Stevens-Johnson syndrome and Toxic Epidermal Necrolysis. Dermatol. Ther. 2019;32:e12758. doi: 10.1111/dth.12758.
Chen Y.-T., Hsu C.-Y., Chien Y.-N., Lee W.-R., Huang Y.-C. Efficacy of cyclosporine for the treatment of Stevens-Johnson syndrome and Toxic Epidermal Necrolysis: Systemic review and meta-analysis. Dermatol. Sin. 2017;35:131–137. doi: 10.1016/j.dsi.2017.04.004.
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