title: Efficacy of additional i.v. immunoglobulin to steroid therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis.
abstract: 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.
authors.affiliation: Department of Dermatology, Yokohama City University School of Medicine, Yokohama, Japan.
authors.forename: Michiko
authors.initials: M
authors.lastname: Aihara
authors.affiliation: Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Kano
authors.affiliation: Department of Dermatology, Yokohama City University School of Medicine, Yokohama, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Fujita
authors.affiliation: Department of Dermatology, Yokohama City University Medical Center, Yokohama, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kambara
authors.affiliation: Department of Dermatology, Yokohama City University Medical Center, Yokohama, Japan.
authors.forename: Setsuko
authors.initials: S
authors.lastname: Matsukura
authors.affiliation: Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Katayama
authors.affiliation: Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Azukizawa
authors.affiliation: Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Miyachi
authors.affiliation: Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Endo
authors.affiliation: Department of Dermatology, Nara Medical University, Kashihara, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Asada
authors.affiliation: Department of Dermatology, Nara Medical University, Kashihara, Japan.
authors.forename: Fumi
authors.initials: F
authors.lastname: Miyagawa
authors.affiliation: Department of Dermatology, Shimane University Faculty of Medicine, Izumo, Japan.
authors.forename: Eishin
authors.initials: E
authors.lastname: Morita
authors.affiliation: Department of Dermatology, Shimane University Faculty of Medicine, Izumo, Japan.
authors.forename: Sakae
authors.initials: S
authors.lastname: Kaneko
authors.affiliation: Department of Dermatology, Hokkaido University School of Medicine, Sapporo, Japan.
authors.forename: Riichiro
authors.initials: R
authors.lastname: Abe
authors.affiliation: Department of Dermatology, Nihon University School of Medicine, Tokyo, Japan.
authors.forename: Toyoko
authors.initials: T
authors.lastname: Ochiai
authors.affiliation: Department of Dermatology, Showa University School of Medicine, Tokyo, Japan.
authors.forename: Hirohiko
authors.initials: H
authors.lastname: Sueki
authors.affiliation: Department of Dermatology, Showa University School of Medicine, Tokyo, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Watanabe
authors.affiliation: Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Nagao
authors.affiliation: Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Yumi
authors.initials: Y
authors.lastname: Aoyama
authors.affiliation: Department of Dermatology, Ehime University School of Medicine, Ehime, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Sayama
authors.affiliation: Ehime Prefectural University of Health Sciences, Ehime, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Shiohara
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D013262:Stevens-Johnson Syndrome / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous
keywords: Stevens-Johnson syndrome; corticosteroid; i.v. immunoglobulin; therapy; toxic epidermal necrolysis