Incidence of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Nationwide Population-Based Study Using National Health Insurance Database in Korea

Abstract

BackgroundStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases; however, it is hard to estimate their incidence due to the rarity of these diseases. We evaluated the incidence of SJS and TEN using a nationwide administrative database.MethodsWe used a national medical insurance review system (Health Insurance Review and Assessment) database which contained the claim data of the entire nation from 2009 to 2013 to estimate the accurate incidence of SJS and TEN in Korea. The diagnostic codes of L511 (SJS) or L512 (TEN) from the International Classification of Diseases-10th revision were used to define the target study population. We also retrospectively followed up a 2011 SJS and TEN cohort for 24 months in order to assess the in-hospital mortality, related complications and total claims cost due to SJS and TEN.ResultsA total of 1,167 (938 SJS and 229 TEN) cases were newly diagnosed from 2010 to 2013. The age- and sex-standardized annual incidences estimated in this study were 3.96 to 5.03 in SJS and 0.94 to 1.45 in TEN per million. There was no significant change in annual incidence throughout the study periods. When analyzed by 10-year age groups, the annual incidence was the lowest in group 20–29 years and the highest in group 70 for both SJS and TEN. Based on the 2011 cohort analysis, the in-hospital mortality were 5.7 and 15.1% for SJS and TEN, respectively. The mortality increased with age, particularly, after 40 years of age. Among the complications related with SJS or TEN, ocular sequelae was the most common (43.1 and 43.4% of SJS and TEN patients, respectively) followed by urethral sequelae (5.7 and 9.4% of SJS and TEN patients, respectively).ConclusionOverall, our data suggest that SJS, and TEN are infrequent but constantly arise throughout the years.

Clinical features of Stevens–Johnson syndrome and toxic epidermal necrolysis

“…Yang et al . noted that it was difficult to survey the real‐world incidences of SJS/TEN because of their rarity …”

“…Although both Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, they are potentially life‐threatening disorders characterized by widespread epidermal necrosis of the skin and mucosa . The incidences of SJS and TEN have been reported as 3.96–5.3 cases for SJS, and 0.4–1.45 cases for TEN, per million . Their classification is based on the body surface area (BSA) affected.…”

Incidence of and mortality from epidermal necrolysis (Stevens–Johnson syndrome/toxic epidermal necrolysis) in France during 2003–16: a four‐source capture–recapture estimate

“…4 Hospital-based studies and studies from large electronic databases have found an annual incidence of 1-13 cases per million individuals. [9][10][11][12][13][14] The overall mortality rate is usually reported to be between 10% and 20%. 4,9,[11][12][13] These wide estimate ranges may be explained by these studies using a unique source of data with their own inherent limitations or the selection of cases mostly based on disease codes with possible misclassification.…”

Stevens‐Johnson syndrome and toxic epidermal necrolysis: retrospective review of 10‐year experience

“…SJS/TEN is rare, with an annual incidence of 1–6 per million for SJS and 0.4–1.45 per million for TEN . Groups at increased risk of developing SJS/TEN include those with immunosuppression, especially those with human immunodeficiency virus, slow acetylator genotypes, and individuals with specific HLA alleles …”