Epidemiology

SJS and TEN are considered rare diseases: reported estimated incidence rates range between 2 and 7 cases per million people per year.[4][5][6]

SJS and TEN are more common in women than in men, affecting all age groups.[7]

Patients with active cancer are associated with an increased risk of SJS/TEN.[6] 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]

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][10]

Pharmacogenomic studies indicate that ethnicity and human leukocyte antigen (HLA) types may predispose patients to adverse drug reactions.[11][12][13][14][15] 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] HLA-A*0206 and HLA-B*4403 are associated with cold-medicine-related SJS and TEN.[15] HLA-A*3101 has a strong association with SJS and TEN ocular and other complications regardless of ethnicity.[11] All individuals with HLA-B*5801 are at risk for allopurinol-induced SJS/TENS.[16]

Presence of HLA-B*1502, HLA-C*0602, or HLA-C*0801 alleles has been associated with trimethoprim/sulfamethoxazole-induced SJS/TEN.[2][17] The FDA recommends that patients are screened for the HLA-B*5701 allele before starting abacavir for HIV infection.[18]​​

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