چکیده (انگلیسی):

Background: StevenseJohnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe
adverse drug reactions with high mortality.
Methods: To present the clinical characteristics of SJS and TEN in Japan and evaluate the efficacy of
treatments, we retrospectively analyzed cases of SJS and TEN treated in 2 university hospitals during
2000e2013.
Results: Fifty-two cases of SJS (21 males and 31 females; average age, 55.1 years) and 35 cases of TEN (17
males and 18 females; average age, 56.6 years) were included in this study. Twenty-eight cases of SJS
(53.8%) and all cases of TEN were caused by drugs. Hepatitis was the most common organ involvement in
both SJS and TEN. Renal dysfunction, intestinal disorder, and respiratory disorder were also involved in
some cases. The major complication was pneumonia and sepsis. All cases except for 3 cases were treated
systemically with corticosteroids. Steroid pulse therapy was performed in 88.6% of TEN. Plasmapheresis
and/or immunoglobulin therapy was combined with steroid therapy mainly in TEN after 2007. The
mortality rate was 6.9% and the rates for SJS and TEN were 1.9% and 14.3%, respectively. These were much
lower than predicted mortality according to a severity-of-illness scoring system for TEN prognosis
(SCORTEN) score. When comparing the mortality rate between 2000e2006 and 2007e2013, it was
decreased from 4.5% to 0.0% in SJS and from 22.2% to 5.3% in TEN.
Conclusions: Treatment with steroid pulse therapy in combination with plasmapheresis and/or immunoglobulin
therapy seems to have contributed to prognostic improvement in SJS/TEN.