PMID- 32595945 OWN - NLM STAT- MEDLINE DCOM- 20201029 LR - 20201029 IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 9 DP - 2020 TI - Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. LID - F1000 Faculty Rev-612 [pii] LID - 10.12688/f1000research.24748.1 [doi] AB - 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-alpha antagonists are still controversial. Recently, the beneficial effects of cyclosporine and TNF-alpha antagonists have been explored. In this review, we discuss recent advances in the pathophysiology and management of SJS/TEN. CI - Copyright: (c) 2020 Hasegawa A and Abe R. FAU - Hasegawa, Akito AU - Hasegawa A AUID- ORCID: 0000-0002-7606-5551 AD - Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. FAU - Abe, Riichiro AU - Abe R AD - Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. LA - eng PT - Journal Article PT - Review DEP - 20200616 PL - England TA - F1000Res JT - F1000Research JID - 101594320 SB - IM MH - Apoptosis MH - Epidermis MH - Humans MH - Necrosis MH - *Stevens-Johnson Syndrome/diagnosis/drug therapy/etiology PMC - PMC7308994 OTO - NOTNLM OT - Stevens-Johnson syndrome OT - drug reaction OT - erythema multiforme OT - necroptosis OT - toxic epidermal necrolysis COIS- No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed. EDAT- 2020/07/01 06:00 MHDA- 2020/10/30 06:00 PMCR- 2020/06/16 CRDT- 2020/06/30 06:00 PHST- 2020/06/10 00:00 [accepted] PHST- 2020/06/30 06:00 [entrez] PHST- 2020/07/01 06:00 [pubmed] PHST- 2020/10/30 06:00 [medline] PHST- 2020/06/16 00:00 [pmc-release] AID - F1000 Faculty Rev-612 [pii] AID - 10.12688/f1000research.24748.1 [doi] PST - epublish SO - F1000Res. 2020 Jun 16;9:F1000 Faculty Rev-612. doi: 10.12688/f1000research.24748.1. eCollection 2020.