Yuma Sunaga1, Michiko Kurosawa2, Hirotaka Ochiai3, Hideaki Watanabe4, Hirohiko Sueki4, Hiroaki Azukizawa5, Hideo Asada5, Yuko Watanabe6, Yukie Yamaguchi6, Michiko Aihara6, Yoshiko Mizukawa7, Manabu Ohyama8, Natsumi Hama9, Riichiro Abe9, Hideo Hashizume10, Saeko Nakajima11, Takashi Nomura11, Kenji Kabashima11, Mikiko Tohyama12, Hayato Takahashi13, Hiroki Mieno14, Mayumi Ueta14, Chie Sotozono14, Hiroyuki Niihara15, Eishin Morita15, Akatsuki Kokaze3. 1. Department of Dermatology, Showa University School of Medicine, Tokyo, Japan; Department of Hygiene, Public Health and Preventive Medicine, Showa University School of Medicine, Tokyo, Japan. Electronic address: sunaga725@med.showa-u.ac.jp. 2. Department of Epidemiology and Environmental Health, Juntendo University Faculty of Medicine, Tokyo, Japan. 3. Department of Hygiene, Public Health and Preventive Medicine, Showa University School of Medicine, Tokyo, Japan. 4. Department of Dermatology, Showa University School of Medicine, Tokyo, Japan. 5. Department of Dermatology, Nara Medical University, Nara, Japan. 6. Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, Kanagawa, Japan. 7. Department of Dermatology, Showa University School of Medicine, Tokyo, Japan; Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan. 8. Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan. 9. Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. 10. Department of Dermatology, Iwata City Hospital, Shizuoka, Japan. 11. Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan. 12. Department of Dermatology, National Hospital Organization Shikoku Cancer Center, Ehime, Japan. 13. Department of Dermatology, Keio University School of Medicine, Tokyo, Japan. 14. Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan. 15. Department of Dermatology, Shimane University Faculty of Medicine, Shimane, Japan.
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.
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.