The nationwide epidemiological survey of Stevens-Johnson syndrome and toxic epidermal necrolysis in Japan, 2016-2018

Yuma Sunaga, Michiko Kurosawa, Hirotaka Ochiai, Hideaki Watanabe, Hirohiko Sueki, Hiroaki Azukizawa, Hideo Asada, Yuko Watanabe, Yukie Yamaguchi, Michiko Aihara, Yoshiko Mizukawa, Manabu Ohyama, Natsumi Hama, Riichiro Abe, Hideo Hashizume, Saeko Nakajima, Takashi Nomura, Kenji Kabashima, Mikiko Tohyama, Hayato TakahashiHiroki Mieno, Mayumi Ueta, Chie Sotozono, Hiroyuki Niihara, Eishin Morita, Akatsuki Kokaze

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)175-182
Number of pages8
JournalJournal of Dermatological Science
Volume100
Issue number3
DOIs
Publication statusPublished - 2020 Dec

Keywords

  • Epidemiology
  • Severity-of-illness score for toxic epidermal necrolysis (SCORTEN)
  • Stevens-Johnson syndrome (SJS)
  • Toxic epidermal necrolysis (TEN)

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Dermatology

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