Review
Pharmacology & Pharmacy
Lin Cheng
Summary: Adverse drug reactions, particularly SJS and TEN, are a significant public health concern with high mortality rates and ineffective treatment options. Factors such as infections, mutations in drug metabolic enzymes, and immune responses contribute to SJS/TEN development. Future research directions include epigenetics, metabolites, and regulatory T cells.
FRONTIERS IN PHARMACOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Hua-Ching Chang, Tsung-Jen Wang, Ming-Hsiu Lin, Ting-Jui Chen
Summary: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases caused by drugs. The effectiveness of systemic immunomodulating treatments for SJS/TEN is still controversial, but cyclosporine and biologic anti-TNF-alpha have shown promising results. Combination treatments are also being explored. Large-scale randomized controlled trials are needed for more definitive evidence. Advances in understanding the pathogenesis of SJS/TEN may inspire the discovery of potential therapeutic targets.
Review
Medicine, General & Internal
Eri Tsukagoshi, Yoichi Tanaka, Yoshiro Saito
Summary: Drug-related Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare but severe adverse drug reactions, with predicting their onset remaining challenging. Pharmacogenomic information on drug labels may differ among countries, but CPIC guidelines provide a scientifically sound goal for future pharmacogenomic implementation.
FRONTIERS IN MEDICINE
(2021)
Article
Medicine, General & Internal
Ekaterina A. Nikitina, Daria S. Fomina, Ulyana A. Markina, Sergey S. Andreev, Yuri V. Streltsov, Tatiana S. Kruglova, Marina S. Lebedkina, Alexander V. Karaulov, Maryana A. Lysenko
Summary: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare syndromes characterized by necrotic skin and mucosal lesions. Drug-induced mechanisms are the most common cause, with certain drugs posing a higher risk. There is no gold standard treatment, but TNF-α inhibitors have shown promise in SJS/TEN cases.
FRONTIERS IN MEDICINE
(2023)
Article
Dermatology
Ting-Jung Hsu, Hsu-Hang Yeh, Chih-Hung Lee, Kwei-Lan Liu
Summary: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are potentially fatal adverse drug reactions. The most common culprit drug is carbamazepine, which has a strong association with HLA-B*15:02. There is no statistically significant correlation between in-hospital stay and the average daily dose of corticosteroids.
INTERNATIONAL JOURNAL OF DERMATOLOGY
(2021)
Article
Dermatology
Pan Lv, Jiangxia Huang, Qianru Yang, Ting Yang, Xianwei Cao, Ougen Liu, Zhibin Zhang
Summary: This study analyzed the expression profiles of circRNAs in peripheral blood mononuclear cells of SJS/TEN patients and found a correlation between circRNA expression and disease severity. Specifically, hsa_circ_0083619 was identified as a potential indicator for SJS/TEN severity and a circRNA-miRNA-mRNA network was constructed. The study suggests that the hsa_circ_0083619/miR-18a-5p/BCL2L10 axis may play a role in the pathogenesis of SJS/TEN.
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Dermatology
Yasmin Chia Chia Liew, Karen Jui Lin Choo, Choon Chiat Oh, Shiu Ming Pang, Yi Wei Yeo, Haur Yueh Lee
Summary: This study evaluated the differences between Mycoplasma-related and drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. The results showed no significant differences in baseline characteristics and extent of involvement, but significant differences in mortality rates between the two groups.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Dermatology
E. J. Roberts, V. Melchionda, G. Saldanha, S. Shaffu, J. Royle, K. E. Harman
Summary: Toxic epidermal necrosis (TEN)-like lupus is a rare condition that occurs in patients with acute severe flares of systemic lupus erythematosus, characterized by epidermal loss and mucosal ulceration. This condition may resemble drug-induced Stevens-Johnson syndrome/TEN, but differs in the absence of a suitable culprit drug and the context of acute lupus, requiring early input from Rheumatologists for appropriate management.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2021)
Letter
Dermatology
ShengYang Bertrand Lian, Choon Chiat Oh, Yi Wei Yeo, Haur Yueh Lee
Summary: This case series documents the incidence of various nail changes as a sequelae to Stevens-Johnson syndrome/toxic epidermal necrolysis.
Article
Dermatology
Rokea A. El-Azhary, Somaira Nowsheen, Lawrence E. Gibson, David J. DiCaudo
Summary: This study retrospectively assessed clinical characteristics and mortality rate of patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) who developed disseminated intravascular coagulation (DIC). The incidence of DIC in patients with SJS/TEN was low but associated with increased mortality. Common triggers of SJS/TEN included drugs and infections, and early clinician awareness and aggressive intervention are crucial for preventing death.
INTERNATIONAL JOURNAL OF DERMATOLOGY
(2021)
Review
Medicine, General & Internal
Qingzi Yan, Xiang Liu, Haibo Lei, Renzhu Liu, Yixiang Hu
Summary: This study investigated the clinical features of oxcarbazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis. The study found that males have a higher prevalence of these diseases and they usually occur within two weeks of medication. The main clinical manifestations include rashes, fever, mucosal lesions, conjunctivitis, and blisters. Stopping medication and other treatments can lead to good outcomes.
FRONTIERS IN MEDICINE
(2023)
Review
Pharmacology & Pharmacy
Bertrand Sheng-Yang Lian, Haur Yueh Lee
Summary: Prompt admissions to optimal care facilities, coordinated specialized care during the acute phase, as well as long-term follow-up to manage chronic sequelae are essential in the management of patients with epidermal necrolysis. Patients should be managed in specialized/reference centers experienced with the disease.
EXPERT OPINION ON DRUG SAFETY
(2022)
Review
Oncology
X. Rui, W. Meidan, W. Gongqiang, Z. Longyi, W. Xiaoxia, C. Wei, W. Chenhui
Summary: The patient in this case report developed toxic epidermal necrolysis (TEN) after receiving the Velcade-lenalidomide-dexamethasone (VRD) regimen for the treatment of multiple myeloma. The patient's concerns included the progression of the rash, pain, itching, and potential long-term complications. With the implemented interventions, the patient's condition gradually improved, and the rash resolved without any residual scarring.
FRONTIERS IN ONCOLOGY
(2023)
Review
Ophthalmology
Gabor Toth, Andrea Lukacs, Frank Schirra, Gabor L. Sandor, Petra Killik, Otto A. Maneschg, Zoltan Z. Nagy, Nora Szentmary
Summary: The aim of the review article was to summarize the current literature on Stevens-Johnson syndrome (SJS) and its severe form, toxic epidermal necrolysis (TEN). SJS/TEN is a serious multi-system, immune-mediated, mucocutaneous disease with a high mortality rate and potential for ocular complications. Early diagnosis, timely amniotic membrane transplantation, and aggressive topical management are important for preventing long-term eye problems. Ophthalmologists should regularly examine patients in both the acute and chronic phases for comprehensive ophthalmic care.
OPHTHALMOLOGY AND THERAPY
(2023)
Review
Medicine, General & Internal
Ming-Hsiu Hsieh, Tomoya Watanabe, Michiko Aihara
Summary: SJS/TEN are serious conditions caused by medication and infections, with keratinocyte death believed to be the main pathomechanism. Treatment options such as corticosteroids and IVIG as immunomodulators can potentially improve the prognosis of SJS/TEN.
FRONTIERS IN MEDICINE
(2021)
Letter
Allergy
Marcus Maurer, Aberer Werner, Rosana Agondi, Mona Al-Ahmad, Maryam Ali Al-Nesf, Ignacio Ansotegui, Rand Arnaout, Luisa Karla Arruda, Riccardo Asero, Emel Aygoeren-Puersue, Aleena Banerji, Andrea Bauer, Moshe Ben-Shoshan, Alejandro Berardi, Jonathan A. Bernstein, Stephen Betschel, Carsten Bindslev-Jensen, Mojca Bizjak, Isabelle Boccon-Gibod, Konrad Bork, Laurence Bouillet, Henrik Balle Boysen, Nicholas Brodszki, Sigurd Broesby-Olsen, Paula Busse, Thomas Buttgereit, Anette Bygum, Teresa Caballero, Regis A. Campos, Mauro Cancian, Ivan Cherrez-Ojeda, Danny M. Cohn, Celia Costa, Timothy Craig, Paulo Ricardo Criado, Roberta F. Criado, Dorottya Csuka, Joachim Dissemond, Aurelie Du-Thanh, Luis Felipe Ensina, Ragip Ertas, Jose E. Fabiani, Claudio Fantini, Henriette Farkas, Silvia Mariel Ferrucci, Ignasi Figueras-Nart, Natalia L. Fili, Daria Fomina, Atsushi Fukunaga, Asli Gelincik, Ana Gimenez-Arnau, Kiran Godse, Mark Gompels, Margarida Goncalo, Maia Gotua, Richard Gower, Anete S. Grumach, Guillermo Guidos-Fogelbach, Michihiro Hide, Natalia Ilina, Naoko Inomata, Thilo Jakob, Dario O. Josviack, Hye-Ryun Kang, Allen Kaplan, Alicja Kasperska-Zajac, Constance Katelaris, Aharon Kessel, Andreas Kleinheinz, Emek Kocaturk, Mitja Kosnik, Dorota Krasowska, Kanokvalai Kulthanan, M. Sendhil Kumaran, Jose Ignacio Larco Sousa, Hilary J. Longhurst, William Lumry, Andrew MacGinnitie, Markus Magerl, Michael P. Makris, Alejandro Malbran, Alexander Marsland, Inmaculada Martinez-Saguer, Iris V. Medina, Raisa Meshkova, Martin Metz, Iman Nasr, Jan Nicolay, Chikako Nishigori, Isao Ohsawa, Kemal Ozyurt, Nikolaos G. Papadopoulos, Claudio A. S. Parisi, Jonathan Grant Peter, Wolfgang Pfuetzner, Todor Popov, Nieves Prior, German D. Ramon, Adam Reich, Avner Reshef, Marc A. Riedl, Bruce Ritchie, Heike Rockmann-Helmbach, Michael Rudenko, Andac Salman, Mario Sanchez-Borges, Peter Schmid-Grendelmeier, Faradiba S. Serpa, Esther Serra-Baldrich, Farrukh R. Sheikh, William Smith, Angele Soria, Petra Staubach, Urs C. Steiner, Marcin Stobiecki, Gordon Sussman, Anna Tagka, Simon Francis Thomsen, Regina Treudler, Solange Valle, Martijn van Doorn, Lilian Varga, Daniel O. Vazquez, Nicola Wagner, Liangchun Wang, Christina Weber-Chrysochoou, Young-Min Ye, Anna Zalewska-Janowska, Andrea Zanichelli, Zuotao Zhao, Yuxiang Zhi, Torsten Zuberbier, Ricardo D. Zwiener, Anthony Castaldo
Correction
Allergy
Stephen Betschel, Jacquie Badiou, Karen Binkley, Rozita Borici-Mazi, Jacques Hebert, Amin Kanani, Paul Keith, Gina Lacuesta, Susan Waserman, Bill Yang, Emel Aygoeren-Puersuen, Jonathan Bernstein, Konrad Bork, Teresa Caballero, Marco Cicardi, Timothy Craig, Henriette Farkas, Anete Grumach, Connie Katelaris, Hilary Longhurst, Marc Riedl, Bruce Zuraw, Magdelena Berger, Jean-Nicolas Boursiquot, Henrik Boysen, Anthony Castaldo, Hugo Chapdelaine, Lori Connors, Lisa Fu, Dawn Goodyear, Alison Haynes, Palinder Kamra, Harold Kim, Kelly Lang-Robertson, Eric Leith, Christine McCusker, Bill Moote, Andrew O'Keefe, Ibraheem Othman, Man-Chiu Poon, Bruce Ritchie, Charles St-Pierre, Donald Stark, Ellie Tsai
ALLERGY ASTHMA AND CLINICAL IMMUNOLOGY
(2020)
Editorial Material
Allergy
J. Taya, C. L. Veronez, J. B. Pesquero, K. Bork, A. S. Grumach
JOURNAL OF INVESTIGATIONAL ALLERGOLOGY AND CLINICAL IMMUNOLOGY
(2021)
Article
Allergy
Volker Wahn, Werner Aberer, Emel Aygoeren-Puersuen, Konrad Bork, Wolfgang Eberl, Maria Fasshauer, Renate Krueger, Markus Magerl, Inmaculada Martinez-Saguer, Peter Spaeth, Petra Staubach-Renz, Christina Weber-Chrysochoou
PEDIATRIC ALLERGY AND IMMUNOLOGY
(2020)
Article
Medicine, General & Internal
Francois Marceau, Georges E. Rivard, Julie M. Gauthier, Karen E. Binkley, Arnaud Bonnefoy, Isabelle Boccon-Gibod, Laurence Bouillet, Matthieu Picard, Ghislain Levesque, Hannah Laure Elfassy, Helene Bachelard, Jacques Hebert, Konrad Bork
FRONTIERS IN MEDICINE
(2020)
Review
Genetics & Heredity
Konrad Bork, Thomas Machnig, Karin Wulff, Guenther Witzke, Subhransu Prusty, Jochen Hardt
ORPHANET JOURNAL OF RARE DISEASES
(2020)
Article
Allergy
Laure M. Fijen, Konrad Bork, Danny M. Cohn
Summary: Hereditary angioedema (HAE) is a rare disease characterized by episodic attacks of swelling, caused by uncontrolled activation of the kinin system. Recent advances in targeted therapies have provided more effective and convenient treatment options for HAE patients, with the potential to reduce the burden on patients.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2021)
Article
Allergy
Konrad Bork, Karin Wulff, Britta S. Moehl, Lars Steinmueller-Magin, Gunther Witzke, Jochen Hardt, Peter Meinke
Summary: The study identified a novel disease-linked mutation for HAEnCI using whole exome sequencing, which affects HS biosynthesis and likely disrupts cell surface interactions of key players in angioedema formation.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2021)
Review
Allergy
Camila Lopes Veronez, Dorottya Csuka, Farrukh R. Sheikh, Bruce L. Zuraw, Henriette Farkas, Konrad Bork
Summary: The knowledge of rare genetic diseases like HAE has evolved parallel to the development of new molecular tools. From the recognition of C1 inhibitor deficiency as the main cause of HAE in the 1960s to the discovery of a wide spectrum of mutations affecting the C1-INH gene in the late 1980s, and the involvement of other genes in HAE in 2006, recent advancements in next-generation sequencing have led to the identification of mutations in 5 new genes associated with HAE and normal C1-INH.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2021)
Review
Allergy
Konrad Bork, John T. Anderson, Teresa Caballero, Timothy Craig, Douglas T. Johnston, H. Henry Li, Hilary J. Longhurst, Cristine Radojicic, Marc A. Riedl
Summary: Individualized management plans should be considered for HAE patients, taking into account the frequency and severity of attacks, disease burden, and therapeutic control. Patient-reported outcomes and QoL assessments should be personalized and consider the impact of HAE on various aspects of a patient's life, beyond just attack frequency and severity.
ALLERGY ASTHMA AND CLINICAL IMMUNOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Blas Larrauri, C. Garren Hester, Haixiang Jiang, Vojislav D. Miletic, Alejandro Malbran, Konrad Bork, Allen Kaplan, Michael Frank
Summary: Hereditary angioedema (HAE) attacks are caused by excessive activation of the contact system. Understanding the activation of the contact system in HAE is crucial for effective treatment. Cold activation induced contact system activation in most HAEnCI patient samples, leading to fragmentation of proteins and increased enzymatic activity.
MOLECULAR IMMUNOLOGY
(2021)
Article
Allergy
Konrad Bork, Karin Wulff, Guenther Witzke, Jochen Hardt, Peter Meinke
Summary: This study analyzed the transmission of HAE-specific mutations to the next generations in families with various types of HAE. The results showed that females are more likely than males to carry disease-linked mutations in both HAE and HAEnCI. Additionally, it was found that there is a loss of male embryos carrying the HAE mutation specifically in HAEnCI.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2022)
Article
Allergy
Marcus Maurer, Markus Magerl, Emel Aygoeren-Puersuen, Konrad Bork, Henriette Farkas, Hilary Longhurst, Sorena Kiani-Alikhan, Laurence Bouillet, Isabelle Boccon-Gibod, Mauro Cancian, Andrea Zanichelli, David Launay
Summary: This article describes the challenges of discontinuing attenuated androgens in HAE treatment and its potential consequences. The study found that discontinuation of attenuated androgens may lead to increased frequency and severity of HAE attacks. Healthcare teams need to carefully plan and closely monitor patients after discontinuation, and modify treatment strategies if necessary.
ALLERGY ASTHMA AND CLINICAL IMMUNOLOGY
(2022)
Meeting Abstract
Allergy
D. Launay, M. Magerl, E. Aygoren-Pursun, K. Bork, H. Farkas, H. Longhurst, S. Kiani, L. Bouille, I Boccon-Gibod, M. Cancian, A. Zanichell, M. Maurer
Meeting Abstract
Allergy
K. Bork, K. Wulff, G. Witzke, T. Machnig, S. Prusty, J. Hardt