The nationwide epidemiological survey of Stevens-Johnson syndrome and toxic epidermal necrolysis in Japan, 2016-2018. Issue 3 (December 2020)

• Record Type: Journal Article
• Authors: Sunaga, Yuma; Kurosawa, Michiko; Ochiai, Hirotaka; Watanabe, Hideaki; Sueki, Hirohiko; Azukizawa, Hiroaki; Asada, Hideo; Watanabe, Yuko; Yamaguchi, Yukie; Aihara, Michiko; Mizukawa, Yoshiko; Ohyama, Manabu; Hama, Natsumi; Abe, Riichiro; Hashizume, Hideo; Nakajima, Saeko; Nomura, Takashi; Kabashima, Kenji; Tohyama, Mikiko; Takahashi, Hayato; Mieno, Hiroki; Ueta, Mayumi; Sotozono, Chie; Niihara, Hiroyuki; Morita, Eishin; Kokaze, Akatsuki
• Is Part Of: Journal of dermatological science. Volume 100:Issue 3(2020)
• Page Start: 175
• Page End: 182
• Publication Date: 2020-12
• Subjects: SJS Stevens-Johnson syndrome -- TEN toxic epidermal necrolysis -- SCARs severe cutaneous adverse reactions -- BSA body surface area -- 95 %CI 95 % confidence intervals -- IVIg intravenous immunoglobulin -- IQR interquartile range; Stevens-Johnson syndrome (SJS) -- Toxic epidermal necrolysis (TEN) -- Epidemiology -- Severity-of-illness score for toxic epidermal necrolysis (SCORTEN); Dermatology -- Periodicals; Skin Diseases -- Periodicals; Dermatologie -- Périodiques; 616.5005
• DOI: 10.1016/j.jdermsci.2020.09.009
• Languages: English
• ISSNs: 0923-1811

Abstract

Highlights: Estimated Yearly prevalence per million was 2.5 for SJS and 1 for TEN. Mean age was 53.9 years for SJS, and 61.8 years for TEN. Mortality rate was 4.1 % for SJS, and 29.9 % for TEN. In TEN, mean age and mortality rates had increased from the previous survey. High-dose steroid therapy showed the greatest reduction in mortality. Abstract: Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening severe cutaneous adverse reactions (SCARs). The first national epidemiological survey of SJS/TEN was carried out in 2008. We conducted a new survey to identify changes from the previous survey. Objective: The present survey aimed to estimate the number of SJS/TEN patients in Japan between 2016 and 2018 (primary survey) and to clarify clinical epidemiological profiles (secondary survey). Methods: A primary survey asking for numbers of SJS/TEN patients during the study period was sent to 1205 institutions nationwide. A secondary survey was sent to institutions reporting SJS/TEN patients, seeking detailed information. Results: Yearly prevalence per million was 2.5 for SJS and 1 for TEN. The secondary survey allowed analysis of 315 SJS cases and 174 TEN cases from 160 institutions. Mean age was 53.9 years in SJS, and 61.8 years in TEN. Mortality rate was 4.1 % for SJS and 29.9 % for TEN. In TEN, mean age and mortality rates had increased from the previous survey. The ratio of expected to observed mortality calculated by SCORTEN score was lowest with high-dose steroid therapy (0.40), followed by steroid pulse therapy (0.52). Conclusion: The present findings suggest that the mortality rate of TEN has increased because of increases in mean ages of patients and patients with malignant neoplasm as underlying disease. When comparing the ratio of expected mortality to actual mortality, high-dose steroid therapy achieved the greatest reduction in mortality.