TY - JOUR
T1 - Update on pathobiology in Stevens-Johnson syndrome and toxic epidermal necrolysis
AU - Su, Shih Chi
AU - Chung, Wen Hung
PY - 2013/12
Y1 - 2013/12
N2 - Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) are rare but life-threatening severe cutaneous adverse reactions (SCARs), which are mainly induced by a variety of drugs. Once considered to be unpredictable, significant progress has been achieved in understanding the pathological mechanisms underlying such reactions. Recent studies suggested that SJS/TEN is a specific immune reaction where human leukocyte antigen (HLA) alleles specific for certain drugs in defined populations are involved in the activation of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Upon the activation, various cytotoxic and immunological signals, including but not limited to Fas/Fas ligand, perforin/granzyme B, and granulysin are launched to mediate the disseminated keratinocyte death in SJS/TEN. This review provides an update on the pathobiology of SJS/TEN in both the genomic and immunologic perspectives. The knowledge gained from these cutting-edge studies will form the basis for better prevention and management of SJS/TEN.
AB - Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) are rare but life-threatening severe cutaneous adverse reactions (SCARs), which are mainly induced by a variety of drugs. Once considered to be unpredictable, significant progress has been achieved in understanding the pathological mechanisms underlying such reactions. Recent studies suggested that SJS/TEN is a specific immune reaction where human leukocyte antigen (HLA) alleles specific for certain drugs in defined populations are involved in the activation of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Upon the activation, various cytotoxic and immunological signals, including but not limited to Fas/Fas ligand, perforin/granzyme B, and granulysin are launched to mediate the disseminated keratinocyte death in SJS/TEN. This review provides an update on the pathobiology of SJS/TEN in both the genomic and immunologic perspectives. The knowledge gained from these cutting-edge studies will form the basis for better prevention and management of SJS/TEN.
KW - Stevens-Johnson syndrome
KW - drug hypersensitivity
KW - human leukocyte antigens
KW - toxic epidermal necrolysis
UR - http://www.scopus.com/inward/record.url?scp=84887321022&partnerID=8YFLogxK
U2 - 10.1016/j.dsi.2013.09.002
DO - 10.1016/j.dsi.2013.09.002
M3 - 文献综述
AN - SCOPUS:84887321022
SN - 1027-8117
VL - 31
SP - 175
EP - 180
JO - Dermatologica Sinica
JF - Dermatologica Sinica
IS - 4
ER -