SJS and TEN are considered rare diseases: reported estimated incidence rates range between 2 and 7 cases per million people per year.[4]Charlton OA, Harris V, Phan K, et al. Toxic epidermal necrolysis and Steven-Johnson syndrome: a comprehensive review. Adv Wound Care (New Rochelle). 2020 Jul;9(7):426-39.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307670
http://www.ncbi.nlm.nih.gov/pubmed/32520664?tool=bestpractice.com
[5]Hsu DY, Brieva J, Silverberg NB, et al. Pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States. J Am Acad Dermatol. 2017 May;76(5):811-7.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5502094
http://www.ncbi.nlm.nih.gov/pubmed/28285784?tool=bestpractice.com
[6]Frey N, Jossi J, Bodmer M, et al. The epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the UK. J Invest Dermatol. 2017 Jun;137(6):1240-7.
https://www.jidonline.org/article/S0022-202X(17)30176-8/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/28202399?tool=bestpractice.com
SJS and TEN are more common in women than in men, affecting all age groups.[7]Sekula P, Dunant A, Mockenhaupt M, et al. Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Invest Dermatol. 2013 May;133(5):1197-204.
https://www.jidonline.org/article/S0022-202X(15)36214-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/23389396?tool=bestpractice.com
Patients with active cancer are associated with an increased risk of SJS/TEN.[6]Frey N, Jossi J, Bodmer M, et al. The epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the UK. J Invest Dermatol. 2017 Jun;137(6):1240-7.
https://www.jidonline.org/article/S0022-202X(17)30176-8/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/28202399?tool=bestpractice.com
One retrospective cohort study of patients with active cancer reported incidence rates of 7.2 and 17.9 per 100,000 patient-years for confirmed and confirmed plus possible cases of SJS/TEN, respectively.[8]Gillis NK, Hicks JK, Bell GC, et al. Incidence and triggers of Stevens-Johnson syndrome and toxic epidermal necrolysis in a large cancer patient cohort. J Invest Dermatol. 2017 Sep;137(9):2021-3.
https://www.jidonline.org/article/S0022-202X(17)31549-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/28549953?tool=bestpractice.com
For subpopulations exposed to particular drugs, such as anticonvulsants in patients with seizures and anti-HIV medications in HIV-positive individuals, the incidence and prevalence rates are increased compared with the population not taking those medications. HIV-positive patients have a combined incidence of SJS and TEN of 1/1000 person-years.[9]Coopman SA, Johnson RA, Platt R, et al. Cutaneous disease and drug reactions in HIV infection. N Engl J Med. 1993 Jun 10;328(23):1670-4.
https://www.nejm.org/doi/10.1056/NEJM199306103282304
http://www.ncbi.nlm.nih.gov/pubmed/8487826?tool=bestpractice.com
[10]Mittmann N, Knowles SR, Koo M, et al. Incidence of toxic epidermal necrolysis and Stevens-Johnson Syndrome in an HIV cohort: an observational, retrospective case series study. Am J Clin Dermatol. 2012 Feb 1;13(1):49-54.
http://www.ncbi.nlm.nih.gov/pubmed/22145749?tool=bestpractice.com
Pharmacogenomic studies indicate that ethnicity and human leukocyte antigen (HLA) types may predispose patients to adverse drug reactions.[11]Yip VL, Alfirevic A, Pirmohamed M. Genetics of immune-mediated adverse drug reactions: a comprehensive and clinical review. Clin Rev Allergy Immunol. 2015 Jun;48(2-3):165-75.
http://www.ncbi.nlm.nih.gov/pubmed/24777842?tool=bestpractice.com
[12]Chang CC, Too CL, Murad S, et al. Association of HLA-B*1502 allele with carbamazepine-induced toxic epidermal necrolysis and Stevens-Johnson syndrome in the multi-ethnic Malaysian population. Int J Dermatol. 2011 Feb;50(2):221-4.
http://www.ncbi.nlm.nih.gov/pubmed/21244392?tool=bestpractice.com
[13]Chung WH, Hung SI. Genetic markers and danger signals in stevens-johnson syndrome and toxic epidermal necrolysis. Allergol Int. 2010 Dec;59(4):325-32.
https://www.sciencedirect.com/science/article/pii/S1323893015306432?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/20962567?tool=bestpractice.com
[14]Leckband SG, Kelsoe JR, Dunnenberger HM, et al. Clinical Pharmacogenetics Implementation Consortium guidelines for HLA-B genotype and carbamazepine dosing. Clin Pharmacol Ther. 2013 Sep;94(3):324-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3748365
http://www.ncbi.nlm.nih.gov/pubmed/23695185?tool=bestpractice.com
[15]Ueta M, Sawai H, Sotozono C, et al. IKZF1, a new susceptibility gene for cold medicine-related Stevens-Johnson syndrome/toxic epidermal necrolysis with severe mucosal involvement. J Allergy Clin Immunol. 2015 Jun;135(6):1538-45.
http://www.ncbi.nlm.nih.gov/pubmed/25672763?tool=bestpractice.com
HLA-B*1502 allele has a strong association with carbamazepine-induced SJS and TEN in the Han Chinese population; the Food and Drug Administration (FDA) recommends testing all Asian people prior to prescribing this medication.[12]Chang CC, Too CL, Murad S, et al. Association of HLA-B*1502 allele with carbamazepine-induced toxic epidermal necrolysis and Stevens-Johnson syndrome in the multi-ethnic Malaysian population. Int J Dermatol. 2011 Feb;50(2):221-4.
http://www.ncbi.nlm.nih.gov/pubmed/21244392?tool=bestpractice.com
HLA-A*0206 and HLA-B*4403 are associated with cold-medicine-related SJS and TEN.[15]Ueta M, Sawai H, Sotozono C, et al. IKZF1, a new susceptibility gene for cold medicine-related Stevens-Johnson syndrome/toxic epidermal necrolysis with severe mucosal involvement. J Allergy Clin Immunol. 2015 Jun;135(6):1538-45.
http://www.ncbi.nlm.nih.gov/pubmed/25672763?tool=bestpractice.com
HLA-A*3101 has a strong association with SJS and TEN ocular and other complications regardless of ethnicity.[11]Yip VL, Alfirevic A, Pirmohamed M. Genetics of immune-mediated adverse drug reactions: a comprehensive and clinical review. Clin Rev Allergy Immunol. 2015 Jun;48(2-3):165-75.
http://www.ncbi.nlm.nih.gov/pubmed/24777842?tool=bestpractice.com
All individuals with HLA-B*5801 are at risk for allopurinol-induced SJS/TENS.[16]Hershfield MS, Callaghan JT, Tassaneeyakul W, et al. Clinical Pharmacogenetics Implementation Consortium guidelines for human leukocyte antigen-B genotype and allopurinol dosing. Clin Pharmacol Ther. 2013 Feb;93(2):153-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3564416
http://www.ncbi.nlm.nih.gov/pubmed/23232549?tool=bestpractice.com
Presence of HLA-B*1502, HLA-C*0602, or HLA-C*0801 alleles has been associated with trimethoprim/sulfamethoxazole-induced SJS/TEN.[2]Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermatol. 2015 Dec;16(6):475-93.
http://www.ncbi.nlm.nih.gov/pubmed/26481651?tool=bestpractice.com
[17]Kongpan T, Mahasirimongkol S, Konyoung P, et al. Candidate HLA genes for prediction of co-trimoxazole-induced severe cutaneous reactions. Pharmacogenet Genomics. 2015 Aug;25(8):402-11.
http://www.ncbi.nlm.nih.gov/pubmed/26086150?tool=bestpractice.com
The FDA recommends that patients are screened for the HLA-B*5701 allele before starting abacavir for HIV infection.[18]Tangamornsuksan W, Lohitnavy O, Kongkaew C, et al. Association of HLA-B*5701 genotypes and abacavir-induced hypersensitivity reaction: a systematic review and meta-analysis. J Pharm Pharm Sci. 2015;18(1):68-76.
http://ejournals.library.ualberta.ca/index.php/JPPS/article/view/23356/18071
http://www.ncbi.nlm.nih.gov/pubmed/25877443?tool=bestpractice.com