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

2021 ◽  
Vol 8 ◽  
Author(s):  
Ming-Hsiu Hsieh ◽  
Tomoya Watanabe ◽  
Michiko Aihara

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.

2021 ◽  
Vol 30 (6) ◽  
pp. 492-496
Author(s):  
Khosrow S Houschyar ◽  
Christian Tapking ◽  
Mimi R Borrelli ◽  
Ina Nietzschmann ◽  
Behrus Puladi ◽  
...  

Objective: Stevens-Johnson syndrome (SJS) and its more severe counterpart, toxic epidermal necrolysis (TEN), are skin hypersensitivity reactions defined by epidermal blistering and necrosis. The exact pathophysiology of SJS/TEN is yet to be deciphered, but a number of risk factors have been identified including adverse drug reactions. The diagnosis of SJS/TEN is made on a clinical basis, and treatment consists of supportive care and occasionally immunosuppressants, such as cyclosporin, high-dose intravenous immunoglobulins and/or corticosteroids. Mortality rates can reach 20–25% in adults but are reduced with early intervention. To identify optimal treatment regimens, to better understand the patient cohort affected, and to help identify key risk factors for mortality, we report our experience with the treatment and management of SJS/TEN patients. Methods: A retrospective review of consecutive patients with SJS and/or TEN admitted to a single burns centre in Germany, between 2008 and 2018, was conducted. The primary outcomes of demographics, clinical course, treatment and patient-reported outcomes were recorded and compared with a control group of patients with burns without a diagnosis of SJS/TEN. Results: A total of 23 patients with SJS/TEN met the inclusion criteria: 17 (74%) with TEN; four (17%) with SJS/TEN overlap; and two (9%) with SJS. Of the patients, 14 (61%) were female and nine (39%) were male. Patient age ranged from 32–78 years (mean: 52 years). A matched cohort of 23 patients with burns served as the control group. All patients received standard of care with a multidisciplinary team. Compared with the control group, SJS/TEN patients had higher mortality rates (n=6, 26% versus n=8, 35%, respectively). The average age of death was 69 years in SJS/TEN patients versus 63 years in control group patients. Age and SCORTEN scores were significant predictors of mortality. Conclusions: SJS and TEN are rare but extreme reactions of the skin and mucosa, associated with high disease mortality rates. This 10-year single-centre retrospective review contributes to the bank of information for reviews evaluating the management of SJS/TEN patients.


2015 ◽  
Vol 64 (4) ◽  
pp. 379-381 ◽  
Author(s):  
Nahoko Kaniwa ◽  
Mayumi Ueta ◽  
Ryosuke Nakamura ◽  
Yoshimi Okamoto-Uchida ◽  
Emiko Sugiyama ◽  
...  

Epilepsia ◽  
2010 ◽  
Vol 51 (12) ◽  
pp. 2461-2465 ◽  
Author(s):  
Nahoko Kaniwa ◽  
Yoshiro Saito ◽  
Michiko Aihara ◽  
Kayoko Matsunaga ◽  
Masahiro Tohkin ◽  
...  

2016 ◽  
Vol 65 (1) ◽  
pp. 74-81 ◽  
Author(s):  
Yumiko Yamane ◽  
Setsuko Matsukura ◽  
Yuko Watanabe ◽  
Yukie Yamaguchi ◽  
Kazuko Nakamura ◽  
...  

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