Epidemiological and Clinical Characteristics of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan: Finding from a Database of Patients Receiving Financial Aid for Treatment

Monday, 18 August 2014
Exhibit hall (Dena'ina Center)
Michiko Kurosawa, PhD , Juntendo University Faculty of Medicine, Tokyo, Japan
Yoko Kano, MD , Kyorin University School of Medicine, Tokyo, Japan
Tetsuo Shiohara, MD , Kyorin University School of Medicine, Tokyo, Japan
Kazuhito Yokoyama, PhD , Juntendo University Faculty of Medicine, Tokyo, Japan
INTRODUCTION: In 2003, the Ministry of Health, Labour and Welfare (MHLW) of Japan launched an online registration system for 45 intractable diseases. Stevens-Johnson syndrome (SJS) and Toxic epidermal necrolysis (TEN) were added to the system in 2009. The study aimed to explore the epidemiological and clinical characteristics of SJS and TEN in Japan using this clinical database. 

METHODS: We obtained clinical records for SJS and TEN from the MHLW between 2009 and 2012. Eighteen duplicate cases were excluded from the analysis and clinical data from 241 patients (SJS: 161 cases, TEN: 75 cases, Unknown disease type: 5 cases) including sex, age, activity in daily life, symptoms, and treatments were included in the final analysis.   

RESULTS: The sex ratio (male/female) of the subjects was 1.06 for SJS and 0.70 for TEN. TEN most frequently afflicted patients in the 60- 69 years age group, whereas SJS were widely distributed across the age groups. A total of 72.7% of patients with SJS and 53.3% of patients with TEN could perform daily activities almost independently, whereas 18.6% of patients with SJS and 37.3% of patients with TEN needed some assistance. A total of 92.5% of patients with SJS received corticosteroids (76.5% efficacy rate), 50.9% received steroid pulse therapy(75.6% efficacy rate), 17.8% received intravenous immunoglobulin (IVIG) therapy(58.8% efficacy rate), and 1.9% received plasma exchange therapy (66.7% efficacy rate). Of the patients with TEN, 88.0% received corticosteroids (60.6% efficacy rate), 82.7% received steroid pulse therapy (64.5% efficacy rate), 45.3% received IVIG therapy(58.8% efficacy rate), and 22.7% received plasma exchange therapy (64.7% efficacy rate).

CONCLUSIONS: Using a clinical database, we clarified the clinical and epidemiological features of SJS and TEN in Japan.

Acknowledgements: This work was partly supported by the Health and Labour Sciences Research Grants “Research on Intractable Diseases” from the MHLW of Japan.