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

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

doi:10.1016/j.jdermsci.2020.09.009

Cited by 39 publications

(42 citation statements)

References 12 publications

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“…10 In our case, HLA-A*02:06 was detected, even if our patient did not present any obvious ocular complications, the incidence of which was 40-81% in recent SJS research. 2,17 In addition, the absence of ocular complications in our patient may be related to the treatment that she was undergoing. Indeed, steroid pulse therapy performed at early disease onset is strongly associated with the prevention of ocular complications in SJS/TEN.…”

Section: Discussionmentioning

confidence: 72%

“…18 Acetaminophen is believed to be a safe analgesic and antipyretic agent and is widely used as a prescription drug or over-the-counter medicine in all age groups. It has been reported that antipyretic anti-inflammatory agents, including acetaminophen, are one of the causative drugs of cutaneous adverse drug reactions 6,17 while analysis from the French pharmacovigilance database has shown that there is no SJS/TEN risk related to the use of acetaminophen. 19 Nevertheless, acetaminophen was the offending drug in two cases of SJS-related liver transplantation (Table 1).…”

Section: Discussionmentioning

confidence: 99%

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A pediatric case of Stevens‐Johnson syndrome with acute liver failure, resulting in liver transplantation

Totsuka

Watanabe

Takamura

et al. 2021

The Journal of Dermatology

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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous adverse reactions characterized by widespread epidermal necrolysis of the skin and mucosa. Skin involvement is greater in TEN, with more than 30% of total body surface area involvement. 1 SJS/TEN is occasionally complicated by various organ disorders, such as gastrointestinal disorders, respiratory disorders, liver dysfunction, and renal dysfunction, leading to irreversible damage and death in some patients. 2,3 Liver dysfunction is a relatively common complication in SJS/ TEN; however, this complication usually improves spontaneously or with SJS/TEN treatment and is rarely a serious clinical problem. 4 Indeed, several studies on the risk factors of SJS/TEN report that liver dysfunction did not affect the mortality rate of SJS/TEN. [5][6][7] However, very few cases of severe liver dysfunction require liver transplantation. We report the first case to be published on a

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

A pediatric case of Stevens‐Johnson syndrome with acute liver failure, resulting in liver transplantation

Totsuka

Watanabe

Takamura

et al. 2021

The Journal of Dermatology

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“…The nationwide epidemiological survey (2016–2018) in Japan revealed that mortality rate was 4.1% for SJS and 29.9% for TEN, respectively ( 32 ). High-dose systemic corticosteroid therapy followed by steroid pulse therapy contributed to the greatest reduction in mortality when comparing the ratio of expected mortality to actual mortality ( 32 ). Furthermore, the incidence of ocular sequelae of SJS/TEN was 7.6% for SJS and 8.0% for TEN, which were less than previously reported ( 40 ).…”

Section: Recent Treatments For Sjs/ten In Japanmentioning

confidence: 99%

Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

2021

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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions characterized by necrosis of the skin and mucus membranes, and are mainly caused by medication and infections. Although the exact pathomechanism of SJS/TEN remains unclear, keratinocyte death is thought to be triggered by immune reactions to these antigens. While there is no established therapy for SJS/TEN, corticosteroids and intravenous immunoglobulin (IVIG) have been utilized as immunomodulator. We previously conducted a study to evaluate the efficacy of IVIG therapy in Japanese patients with SJS/TEN. IVIG was administered at a dosage of 400 mg/kg/day for 5 consecutive days as an additional therapy with systemic steroids. Prompt amelioration was observed in seven of the eight patients. All patients survived without sequelae. Recently, we retrospectively analyzed 132 cases of SJS/TEN treated in our two hospitals. The mortality rates in the patients treated with methylprednisolone pulse were 0% (0/31) for SJS and 7.0% (3/43) for TEN, and 0% (0/10) in the TEN patients treated with methylprednisolone pulse in combination with IVIG. These results suggest that early treatment with high-dose steroids, including methylprednisolone pulse therapy, and IVIG together with corticosteroids are possible therapeutic options to improve the prognosis of SJS/TEN.

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“…Cutaneous reactions are most common manifestations seen in hypersensitivity reactions of drugs, named cutaneous adverse drug reactions, and among those with risk of life-threatening are classified into severe cutaneous adverse reactions (SCARs), mediated by drug-specific T lymphocytes, including drug rash with eosinophilia and systemic symptoms (DRESS)/druginduced hypersensitivity syndrome (DIHS), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). The mortality rates for SJS/TEN are ∼5-10% in SJS and 30-50% in TEN (1), and the common leading culprit drugs around the world are antibiotics, antiepileptics, allopurinol, and cold medicine (2)(3)(4). In this article, we reviewed the updated molecular mechanism and susceptible genes related to SCARs.…”

Section: Introductionmentioning

confidence: 99%

Genetics of Severe Cutaneous Adverse Reactions

et al. 2021

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Severe cutaneous adverse reactions (SCARs) including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug rash with eosinophilia and systemic symptoms (DRESS) are T cells-mediated life-threatening immune reactions, most commonly induced by drug. The last decade has seen significant progress in SCARs research. Recent studies have unveiled the pathogenesis of SCARs involved in susceptible genes, including human leukocyte antigens (HLA) and drugs-T cell receptor (TCR) interaction that may trigger T cell activation with downstream immune signaling of cytokines/chemokines and specific cytotoxic proteins releases. Advances in identification of multiple genetic alleles associated with specific drugs related SCARS in different populations is an important breakthrough in recent years for prevention of SCARs. This article summarized the findings on genetic factors related to SJS/TEN, especially for HLA.

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

Cited by 39 publications

References 12 publications

A pediatric case of Stevens‐Johnson syndrome with acute liver failure, resulting in liver transplantation

A pediatric case of Stevens‐Johnson syndrome with acute liver failure, resulting in liver transplantation

Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

Genetics of Severe Cutaneous Adverse Reactions

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