PMID- 35437717
OWN - NLM
STAT- MEDLINE
DCOM- 20220420
LR  - 20220719
IS  - 1940-6029 (Electronic)
IS  - 1064-3745 (Linking)
VI  - 2486
DP  - 2022
TI  - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the Era of Systems 
      Medicine.
PG  - 37-54
LID - 10.1007/978-1-0716-2265-0_3 [doi]
AB  - Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe 
      mucocutaneous bullous disorders characterized by widespread skin and mucosal 
      necrosis and detachment, which are most commonly triggered by medications. 
      Despite their rarity, these severe cutaneous adverse drug reactions will result 
      in high mortality and morbidity as well as long-term sequela. The 
      immunopathologic mechanisms is mainly cell-mediated cytotoxic reaction against 
      keratinocytes leading to massive skin necrolysis. Subsequent studies have 
      demonstrated that immune synapse composed of cytotoxic T cells with drug-specific 
      human leukocyte antigen (HLA) class I restriction and T cell receptors (TCR) 
      repertoire is the key pathogenic for SJS/TEN. Various cytotoxic proteins and 
      cytokines such as soluble granulysin, perforin, granzyme B, interleukin-15, Fas 
      ligand, interferon-gamma, tumor necrosis factor-alpha have been as mediators involved in 
      the pathogenesis of SJS/TEN. Early recognition and immediate withdrawal of 
      causative agents, and critical multidisciplinary supportive care are key 
      management of SJS/TEN. To date, there is yet to be a sufficient consensus or 
      recommendation for the immunomodulants of the treatment in SJS/TEN. Systemic 
      corticosteroids remain one of the most common treatment options for SJS/TEN, 
      though the efficacy remain uncertain. Currently, there is increasing evidence 
      showing that cyclosporine and TNF-alpha inhibitors decrease the mortality of SJS/TEN. 
      Further multicenter double-blinded, randomized, placebo-controlled trials are 
      required to confirm the efficacy and safety.
CI  - (c) 2022. The Author(s), under exclusive license to Springer Science+Business 
      Media, LLC, part of Springer Nature.
FAU - Chen, Chun-Bing
AU  - Chen CB
AD  - Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, 
      Chang Gung Memorial Hospital, Linkou, Taipei, and Keelung, Taiwan. 
      chunbing.chen@gmail.com.
AD  - Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial 
      Hospital, Linkou, Taiwan. chunbing.chen@gmail.com.
AD  - Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung 
      University, Taoyuan, Taiwan. chunbing.chen@gmail.com.
AD  - College of Medicine, Chang Gung University, Taoyuan, Taiwan. 
      chunbing.chen@gmail.com.
FAU - Wang, Chuang-Wei
AU  - Wang CW
AD  - Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, 
      Chang Gung Memorial Hospital, Linkou, Taipei, and Keelung, Taiwan.
AD  - Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial 
      Hospital, Linkou, Taiwan.
AD  - College of Medicine, Chang Gung University, Taoyuan, Taiwan.
FAU - Chung, Wen-Hung
AU  - Chung WH
AD  - Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, 
      Chang Gung Memorial Hospital, Linkou, Taipei, and Keelung, Taiwan. 
      wenhungchung@yahoo.com.
AD  - Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial 
      Hospital, Linkou, Taiwan. wenhungchung@yahoo.com.
AD  - Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung 
      University, Taoyuan, Taiwan. wenhungchung@yahoo.com.
AD  - College of Medicine, Chang Gung University, Taoyuan, Taiwan. 
      wenhungchung@yahoo.com.
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - Methods Mol Biol
JT  - Methods in molecular biology (Clifton, N.J.)
JID - 9214969
RN  - 0 (Tumor Necrosis Factor-alpha)
RN  - 83HN0GTJ6D (Cyclosporine)
SB  - IM
MH  - Cyclosporine/therapeutic use
MH  - Humans
MH  - Keratinocytes
MH  - Skin/pathology
MH  - *Stevens-Johnson Syndrome/etiology/therapy
MH  - Systems Analysis
MH  - Tumor Necrosis Factor-alpha
OTO - NOTNLM
OT  - Cytotoxic T lymphocyte
OT  - Granulysin
OT  - Human leukocyte antigen
OT  - Severe cutaneous adverse reaction
OT  - Stevens-Johnson syndrome
OT  - T cell receptors
OT  - Toxic epidermal necrolysis
EDAT- 2022/04/20 06:00
MHDA- 2022/04/21 06:00
CRDT- 2022/04/19 06:02
PHST- 2022/04/19 06:02 [entrez]
PHST- 2022/04/20 06:00 [pubmed]
PHST- 2022/04/21 06:00 [medline]
AID - 10.1007/978-1-0716-2265-0_3 [doi]
PST - ppublish
SO  - Methods Mol Biol. 2022;2486:37-54. doi: 10.1007/978-1-0716-2265-0_3.