Abstract Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a predominantly drug-induced disease, with a mortality rate of 15–20%, that engages the expertise of multiple disciplines: dermatology, allergy, immunology, clinical pharmacology, burn surgery, ophthalmology, urogynecology, and psychiatry. SJS/TEN has an incidence of 1–5/million persons per year in the United States, with even higher rates globally. One of the challenges of SJS/TEN has been developing the research infrastructure and coordination to answer questions capable of transforming clinical care and leading to improved patient outcomes. SJS/TEN 2021, the third research meeting of its kind, was held as a virtual meeting on August 28–29, 2021. The meeting brought together 428 international scientists, in addition to a community of 140 SJS/TEN survivors and family members. The goal of the meeting was to brainstorm strategies to support the continued growth of an international SJS/TEN research network, bridging science and the community. The community workshop section of the meeting focused on eight primary themes: mental health, eye care, SJS/TEN in children, non-drug induced SJS/TEN, long-term health complications, new advances in mechanisms and basic science, managing long-term scarring, considerations for skin of color, and COVID-19 vaccines. The meeting featured several important updates and identified areas of unmet research and clinical need that will be highlighted in this white paper. Keywords: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, severe adverse cutaneous drug reactions, HLA genotyping, pharmacogenomics, body surface area, electronic medical record, SCORTEN
SHOW FULL ABSTRACT Madeline E Marks, Ramya Krishna Botta, Riichiro Abe, Thomas M Beachkofsky, Isabelle Boothman, Bruce C Carleton, Wen-Hung Chung, Ricardo R Cibotti, Roni P Dodiuk-Gad, Christian Grimstein, Akito Hasegawa, Jay H Hoofnagle, Shuen-Iu Hung, Benjamin Kaffenberger, Daniela Kroshinsky, Rannakoe J Lehloenya, Michelle Martin-Pozo, Robert G Micheletti, Maja Mockenhaupt, Keisuke Nagao, Suman Pakala, Amy Palubinsky, Helena B Pasieka, Jonathan Peter, Munir Pirmohamed, Melissa Reyes, Hajirah N Saeed, Jeffery Shupp, Chonlaphat Sukasem, Jhih Yu Syu, Mayumi Ueta, Li Zhou, Wan-Chun Chang, Patrice Becker, Teresa Bellon, Kemberlee Bonnet, Gianpiero Cavalleri, James Chodosh, Anna K Dewan, Arturo Dominguez, Xinzhong Dong, Elena Ezhkova, Esther Fuchs, Jennifer Goldman, Sonia Himed, Simon Mallal, Alina Markova, Kerry McCawley, Allison E Norton, David Ostrov, Michael Phan, Arthur Sanford, David Schlundt, Daniel Schneider, Neil Shear, Kanade Shinkai, Eric Tkaczyk, Jason A Trubiano, Simona Volpi, Charles S Bouchard, Sherrie J Divito, Elizabeth J Phillips
1Center for Drug Interactions and Immunology, Division of Infectious Disease, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States 2Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan 3Departments of Dermatology and Medicine, Uniformed Services University, Bethesda, MD, United States 4The SFI Centre for Research Training in Genomics Data Science, Dublin, Ireland 5Division of Translational Therapeutics, Department of Pediatrics, Faculty of Medicine, University of British Columbia and the British Columbia Children’s Hospital Research Institute, Vancouver, BC, Canada 6Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan 7National Institute of Arthritis and Musculoskeletal and Skin (NIAMS), National Institutes of Health (NIH), Bethesda, MD, United States 8Department of Dermatology, Emek Medical Center, Afula, Israel 9Division of Dermatology, Department of Medicine, University of Toronto, Toronto, ON, Canada 10Department of Dermatology, Bruce Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa, Israel 11Office of Clinical Pharmacology, Office of Translational Sciences, Center for Drug Evaluation and Research, U.S. Food and Drug Administration, Silver Spring, MD, United States 12Liver Disease Research Branch, Division of Digestive Diseases and Nutrition of NIDDK, National Institutes of Health (NIH), Bethesda, MD, United States 13Cancer Vaccine and Immune Cell Therapy Core Laboratory, Department of Medical Research, Chang Gung Memorial Hospital, Taoyuan, Taiwan 14Department of Dermatology, Ohio State University Wexner Medical Center, Columbus, OH, United States 15Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, United States 16Division of Dermatology, Department of Medicine, University of Cape Town, Cape Town, South Africa 17Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States 18Dokumentationszentrum schwerer Hautreaktionen (dZh), Department of Dermatology, Medical Center and Medical Faculty, University of Freiburg, Freiburg, Germany 19The Burn Center, MedStar Washington Hospital Center, Washington, D.C., DC, United States 20Department of Dermatology, MedStar Health/Georgetown University, Washington, D.C., DC, United States 21Division of Allergy and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa 22Department of Pharmacology and Therapeutics, University of Liverpool, Liverpool, United Kingdom 23Center for Drug Evaluation and Research, United States Food and Drug Administration, Silver Spring, MD, United States 24Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, United States 25Department of Surgery, Plastic and Reconstructive Surgery, Biochemistry, and Molecular and Cellular Biology, MedStar Washington Hospital Center, Georgetown University School of Medicine, Washington, D.C., DC, United States 26Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand 27Department of Cell Biology and Anatomy, College of Medicine, National Cheng Kung University, Tainan, Taiwan 28Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan 29Division of General Internal Medicine and Primary Care, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, United States 30Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Disease, Bethesda, MD, United States 31Drug Hypersensitivity Laboratory, La Paz Health Research Institute (IdiPAZ), Madrid, Spain 32Department of Psychology, Vanderbilt University, Nashville, TN, United States 33University of New Mexico School of Medicine, Albuquerque, NM, United States 34Department of Dermatology, Vanderbilt University Medical Center, Nashville, TN, United States 35Department of Dermatology and Internal Medicine, UT Southwestern Medical Center, Dallas, TX, United States 36Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, United States 37Department of Cell, Developmental, and Regenerative Biology and Dermatology, Black Family Stem Cell Institute, Mount Sinai School of Medicine, New York, NY, United States 38Department of Obstetrics and Gynecology, University of Washington, Seattle, WA, United States 39Division of Pediatric Infectious Diseases and Clinical Pharmacology, Children’s Mercy, Kansas City, MO, United States 40College of Medicine, University of Cincinnati, Cincinnati, OH, United States 41Division of Infectious Diseases, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States 42Department of Dermatology, Memorial Sloan Kettering Cancer Center, Weill Cornell Medical College, New York, NY, United States 43Stevens-Johnson Syndrome Foundation, Westminster, CO, United States 44Division of Pediatric Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, United States 45Department of Pathology, Immunology and Laboratory Medicine, University of Florida, Gainesville, FL, United States 46Division of Pharmacovigilance-I, Center for Drug Evaluation and Research, U.S. Food and Drug Administration, Silver Spring, MD, United States 47Division of Trauma, Surgical Critical Care, and Burns, Loyola University Medical Center, Chicago, IL, United States 48Department of Psychiatry and Surgery, MedStar Washington Hospital Center, Georgetown University School of Medicine, Washington, D.C., DC, United States 49Department of Dermatology, University of California, San Francisco, San Francisco, CA, United States 50Department of Veterans Affairs, Vanderbilt Dermatology Translational Research Clinic (VDTRC.org), Nashville, TN, United States 51Department of Infectious Diseases and Medicine, Austin Health, University of Melbourne, Melbourne, VIC, Australia 52National Human Genome Research Institute (NHGRI), National Institutes of Health (NIH), Bethesda, MD, United States 53Department of Opthalmology, Loyola University Medical Center, Chicago, IL, United States 