Michiko Aihara 1 , Yoko Kano 2 , Hiroyuki Fujita 1 , Takeshi Kambara 3 , Setsuko Matsukura 3 , Ichiro Katayama 4 , Hiroaki Azukizawa 4 , Yoshiki Miyachi 5 , Yuichiro Endo 5 , Hideo Asada 6 , Fumi Miyagawa 6 , Eishin Morita 7 , Sakae Kaneko 7 , Riichiro Abe 8 , Toyoko Ochiai 9 , Hirohiko Sueki 10 , Hideaki Watanabe 10 , Keisuke Nagao 11 , Yumi Aoyama 12 , Koji Sayama 13 , Koji Hashimoto 14 , Tetsuo Shiohara 2
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and life-threatening cutaneous adverse drug reactions. While there is no established therapy for SJS/TEN, systemic corticosteroids, plasma exchange and i.v. immunoglobulin (IVIG) have been used as treatment. The efficacy of IVIG is still controversial because total doses of IVIG used vary greatly from one study to another. The aim of this study was to evaluate the efficacy of IVIG, administrated for 5 days consecutively, in an open-label, multicenter, single-arm study in patients with SJS or TEN. IVIG (400 mg/kg per day) administrated for 5 days consecutively was performed as an additional therapy to systemic steroids in adult patients with SJS or TEN. Efficacy on day 7 of IVIG was evaluated. Parameters to assess clinical outcome were enanthema including ophthalmic and oral lesions, cutaneous lesions and general condition. These parameters were scored and recorded before and after IVIG. We enrolled five patients with SJS and three patients with TEN who did not respond sufficiently to systemic steroids before IVIG administration. All of the patients survived and the efficacy on day 7 of the IVIG was 87.5% (7/8 patients). Prompt amelioration was observed in skin lesions and enanthema in the patients in whom IVIG therapy was effective. Serious side-effects from the use of IVIG were not observed. IVIG (400 mg/kg per day) administrated for 5 days consecutively seems to be effective in patients with SJS or TEN. IVIG administrated together with steroids should be considered as a treatment modality for patients with refractory SJS/TEN. Further studies are needed to define the therapeutic efficacy of IVIG.