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Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis.
F1000Res. 2020; 9F

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by detachment of the epidermis and mucous membrane. SJS/TEN are considered to be on the same spectrum of diseases with different severities. They are classified by the percentage of skin detachment area. SJS/TEN can also cause several complications in the liver, kidneys, and respiratory tract. The pathogenesis of SJS/TEN is still unclear. Although it is difficult to diagnose early stage SJS/TEN, biomarkers for diagnosis or severity prediction have not been well established. Furthermore, optimal therapeutic options for SJS/TEN are still controversial. Several drugs, such as carbamazepine and allopurinol, are reported to have a strong relationship with a specific human leukocyte antigen (HLA) type. This relationship differs between different ethnicities. Recently, the usefulness of HLA screening before administering specific drugs to decrease the incidence of SJS/TEN has been investigated. Skin detachment in SJS/TEN skin lesions is caused by extensive epidermal cell death, which has been considered to be apoptosis via the Fas-FasL pathway or perforin/granzyme pathway. We reported that necroptosis, i.e. programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, and its interaction with the formyl peptide receptor 1 induce necroptosis. Several diagnostic or prognostic biomarkers for SJS/TEN have been reported, such as CCL-27, IL-15, galectin-7, and RIP3. Supportive care is recommended for the treatment of SJS/TEN. However, optimal therapeutic options such as systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and TNF-α antagonists are still controversial. Recently, the beneficial effects of cyclosporine and TNF-α antagonists have been explored. In this review, we discuss recent advances in the pathophysiology and management of SJS/TEN.

Authors+Show Affiliations

Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

32595945

Citation

Hasegawa, Akito, and Riichiro Abe. "Recent Advances in Managing and Understanding Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis." F1000Research, vol. 9, 2020.
Hasegawa A, Abe R. Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. F1000Res. 2020;9.
Hasegawa, A., & Abe, R. (2020). Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. F1000Research, 9. https://doi.org/10.12688/f1000research.24748.1
Hasegawa A, Abe R. Recent Advances in Managing and Understanding Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. F1000Res. 2020;9 PubMed PMID: 32595945.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. AU - Hasegawa,Akito, AU - Abe,Riichiro, Y1 - 2020/06/16/ PY - 2020/06/10/accepted PY - 2020/6/30/entrez KW - Stevens-Johnson syndrome KW - drug reaction KW - erythema multiforme KW - necroptosis KW - toxic epidermal necrolysis JF - F1000Research JO - F1000Res VL - 9 N2 - Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by detachment of the epidermis and mucous membrane. SJS/TEN are considered to be on the same spectrum of diseases with different severities. They are classified by the percentage of skin detachment area. SJS/TEN can also cause several complications in the liver, kidneys, and respiratory tract. The pathogenesis of SJS/TEN is still unclear. Although it is difficult to diagnose early stage SJS/TEN, biomarkers for diagnosis or severity prediction have not been well established. Furthermore, optimal therapeutic options for SJS/TEN are still controversial. Several drugs, such as carbamazepine and allopurinol, are reported to have a strong relationship with a specific human leukocyte antigen (HLA) type. This relationship differs between different ethnicities. Recently, the usefulness of HLA screening before administering specific drugs to decrease the incidence of SJS/TEN has been investigated. Skin detachment in SJS/TEN skin lesions is caused by extensive epidermal cell death, which has been considered to be apoptosis via the Fas-FasL pathway or perforin/granzyme pathway. We reported that necroptosis, i.e. programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, and its interaction with the formyl peptide receptor 1 induce necroptosis. Several diagnostic or prognostic biomarkers for SJS/TEN have been reported, such as CCL-27, IL-15, galectin-7, and RIP3. Supportive care is recommended for the treatment of SJS/TEN. However, optimal therapeutic options such as systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and TNF-α antagonists are still controversial. Recently, the beneficial effects of cyclosporine and TNF-α antagonists have been explored. In this review, we discuss recent advances in the pathophysiology and management of SJS/TEN. SN - 2046-1402 UR - https://www.unboundmedicine.com/medline/citation/32595945/Recent_advances_in_managing_and_understanding_Stevens-Johnson_syndrome_and_toxic_epidermal_necrolysis L2 - https://f1000research.com/articles/10.12688/f1000research.24748.1/doi DB - PRIME DP - Unbound Medicine ER -
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