Journal ArticleDOI
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update.
Roni P. Dodiuk-Gad,Roni P. Dodiuk-Gad,Wen-Hung Chung,Laurence Valeyrie-Allanore,Neil H. Shear,Neil H. Shear +5 more
TLDR
This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment, and it is hoped these suggested guidelines serve as a practical clinical tool in the management of SJS /TEN.Abstract:
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous reactions, predominantly drug induced. The mortality rates for SJS and TEN are as high as 30 %, and short- and long-term morbidities are very common. SJS/TEN is one of the few dermatological diseases that constitute a true medical emergency. Early recognition and prompt and appropriate management can be lifesaving. In recent years, our understanding of the pathogenesis, clinical presentation, and management of SJS/TEN has improved. Nevertheless, in 2015, there are still no internationally accepted management guidelines. This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment. We hope these suggested guidelines serve as a practical clinical tool in the management of SJS/TEN. The classic manifestation of SJS/TEN consists of initial "flu-like" symptoms (malaise, fever, anorexia) in the prodromal phase, followed by cutaneous and mucous membrane (ocular, oral, and genital) inflammation and pain, and other systemic involvement. Symptoms usually begin 4-28 days after the onset of drug intake. Treatment is multidisciplinary and includes identification and withdrawal of the culprit drug, transfer to a specialist unit, supportive care, medical treatment, communication, and provision of appropriate information and emotional support.read more
Citations
More filters
Journal ArticleDOI
Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
TL;DR: The understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytot toxicity mechanisms were clarified.
Journal ArticleDOI
SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation
Katie D. White,Riichiro Abe,Michael R. Ardern-Jones,Thomas M. Beachkofsky,Charles S. Bouchard,Bruce Carleton,Bruce Carleton,James Chodosh,Ricardo Cibotti,Robert Davis,Joshua C. Denny,Roni P. Dodiuk-Gad,Roni P. Dodiuk-Gad,Elizabeth N. Ergen,Jennifer L. Goldman,James H. Holmes,James H. Holmes,Shuen-Iu Hung,Mario E. Lacouture,Rannakoe J. Lehloenya,Simon Mallal,Simon Mallal,Teri A. Manolio,Teri A. Manolio,Robert G. Micheletti,Caroline M. Mitchell,Maja Mockenhaupt,David A. Ostrov,Rebecca Pavlos,Munir Pirmohamed,Elena Pope,Alec J. Redwood,Misha Rosenbach,Michael Rosenblum,Jean-Claude Roujeau,Arturo P. Saavedra,Hajirah N. Saeed,Jeffery P. Struewing,Hirohiko Sueki,Chonlaphat Sukasem,Cynthia Sung,Cynthia Sung,Jason A Trubiano,Jessica Weintraub,Lisa M. Wheatley,Kristina B. Williams,Brandon Worley,Wen-Hung Chung,Neil H Shear,Elizabeth J. Phillips,Elizabeth J. Phillips +50 more
TL;DR: The current state of knowledge and expert opinion related to SJS/TEN is summarized covering a broad spectrum of topics including epidemiology and pharmacogenomic networks; clinical management and complications; special populations such as pediatrics, the elderly, and pregnant women; regulatory issues and the electronic health record; new agents that cause SJS-TEN; pharmacogenomics and immunopathogenesis; and the patient perspective.
Carbamazepine-induced toxic effects and HLA-B*1502 screening in Taiwan
陳珮(Pei Chen),林瑞榮(Juei-Jueng Lin),陸清松(Chin-Song Lu),翁德(Cheung-Ter Ong),謝福源(Peiyuan F. Hsieh),楊智超(Chih-Chao Yang),戴志達(Chih-Ta Tai),巫錫霖(Shey-Lin Wu),盧成憲(Cheng-Hsien Lu),許永居(Yung-Chu Hsu),尤香玉(Hsiang-Yu Yu),羅榮昇(Long-Sun Ro),呂宗達(Chung-Ta Lu),朱俊哲,蔡景仁(Jing-Jane Tsai),蘇裕翔,藍聖星,宋昇峰,林書儀(Shu-Yi Lin),莊惠萍(Hui-Ping Chuang),黃麗真(Li-Chen Huang),陳盈如(Ying-Ju Chen),蔡佩蓉(Pei-Joung Tsai),廖虹婷(Hung-Ting Liao),林侑璇(Yu-Hsuan Lin),陳建勳(Chien-Hsiun Chen),鍾文宏(Wen-Hung Chung),洪舜郁(Shuen-Iu Hung),鄔哲源(Jer-Yuarn Wu),張琦芬(Chi-Feng Chang),陳亦聖(Luke Chen),陳垣崇(Yuan-Tsong Chen),沈志陽(Chen-Yang Shen),周宜卿(I-Ching Chou),郭煌宗(Haung-Tsung Kuo) +34 more
Journal ArticleDOI
Mechanisms of Severe Cutaneous Adverse Reactions: Recent Advances.
TL;DR: Intensive research in recent years has led to major progress in understanding of the contribution of certain cell types and soluble mediators to the variability of SCAR phenotypes.
Journal ArticleDOI
Major psychological complications and decreased health-related quality of life among survivors of Stevens-Johnson syndrome and toxic epidermal necrolysis.
Roni P. Dodiuk-Gad,Roni P. Dodiuk-Gad,Cristina Olteanu,A. Feinstein,Rena Hashimoto,Raed Alhusayen,S. Whyte-Croasdaile,Y. Finkelstein,Marjorie Burnett,Shachar Sade,Robert Cartotto,Marc G. Jeschke,Neil H. Shear +12 more
TL;DR: The long-term psychological complications and HRQOL of SJS/TEN survivors is characterized and 65% of participants had symptoms of post-traumatic stress and 29% even had total scores in keeping with clinical signs of possible PTSD.
References
More filters
Journal ArticleDOI
Interferon-γ: an overview of signals, mechanisms and functions
TL;DR: The current understanding of IFN‐γ ligand, receptor, ignal transduction, and cellular effects with a focus on macrophage responses and to a lesser extent, responses from other cell types that influence macrophages function during infection are reviewed.
Journal ArticleDOI
Medical genetics: A marker for Stevens-Johnson syndrome
Wen-Hung Chung,Shuen-Iu Hung,Hong-Shang Hong,Mo-Song Hsih,Li-Cheng Yang,Hsin-Chun Ho,Jer-Yuarn Wu,Jer-Yuarn Wu,Yuan-Tsong Chen,Yuan-Tsong Chen +9 more
TL;DR: This paper showed that there is a strong association in Han Chinese between the human leukocyte antigen HLA-B*1502 and Stevens-Johnson syndrome induced by carbamazepine, a drug commonly prescribed for the treatment of seizures.
Journal ArticleDOI
Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme
Sylvie Bastuji-Garin,Berthold Rzany,Robert S. Stern,Neil H. Shear,Luigi Naldi,Jean-Claude Roujeau +5 more
TL;DR: This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents.
Journal ArticleDOI
HLA-B*5801 allele as a genetic marker for severe cutaneous adverse reactions caused by allopurinol
Shuen Lu Hung,Wen-Hung Chung,Wen-Hung Chung,Wen-Hung Chung,Lieh Bang Liou,Chen Chung Chu,Marie Lin,Hsien Ping Huang,Yen Ling Lin,Joung Liang Lan,Li Cheng Yang,Hong Shang Hong,Ming Jing Chen,Ping Chin Lai,Mai Szu Wu,Chia-Yu Chu,Chia-Yu Chu,Kuo Hsien Wang,Chien-Hsiun Chen,Cathy S.J. Fann,Jer-Yuarn Wu,Jer-Yuarn Wu,Yuan-Tsong Chen,Yuan-Tsong Chen +23 more
TL;DR: The results indicated that allopurinol-SCAR is strongly associated with a genetic predisposition in Han Chinese, and in particular, HLA-B*5801 allele is an important genetic risk factor for this life-threatening condition.
Journal ArticleDOI
Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin.
Isabelle Viard,Philippe Wehrli,Roberto Bullani,Pascal Schneider,Nils Holler,Denis Salomon,Thomas Hunziker,Jean-Hilaire Saurat,Jürg Tschopp,Lars E. French +9 more
TL;DR: Antibodies present in pooled human intravenous immunoglobulins (IVIG) blocked Fas-mediated keratinocyte death in vitro and indicated that IVIG may be an effective treatment for toxic epidermal necrolysis of TEN.