Stevens Johnson Syndrome Research is ongoing to better understand the causes and triggers of SJS a deadly skin rash and to develop more effective treatments. A few active research areas include: Pinpointing genetic factors that increase Stevens Johnson Syndrome susceptibility.
Stevens Johnson Syndrome Research is ongoing to better understand the causes and triggers of SJS a deadly skin rash and to develop more effective treatments. A few active research areas include: Pinpointing genetic factors that increase Stevens Johnson Syndrome susceptibility.
Better genetic screening methods for predicting susceptibility in response to medication
Developing new treatments to stop the progression of Stevens Johnson Syndrome a deadly skin rash reaction
SJS Research Consortium is studying the long-term effects of SJS.
Steven-Johnson Syndrome (SJS) severely affects the skin and mucous membranes. SJS often results from an adverse reaction to medication causing detachment of the skin layers and the development of blisters and sores on the mucous membranes.
Investigating the role of the immune system in SJS Progress on Stevens Johnson Syndrome is slow and emphasizes the complexity of associated genetics and the skin disease reaction involved in causing the Stevens Johnson Syndrome condition.
New research suggests that older adults face an increased risk of severe skin reactions from antibiotics, regardless of the specific version of the oral medication they receive, potentially resulting in the need for hospitalization due to symptoms associated with Stevens-Johnson syndrome (SJS) or other conditions.
Stevens-Johnson syndrome (SJS) is a life-threatening skin rash condition that can be triggered by a reaction to medication, the most common drugs / prescriptions / RX that cause SJS include:
Anticonvulsants: Lamotrigine, carbamazepine, phenytoin, and phenobarbital
Anti-gout medications: Allopurinol, especially i
Stevens-Johnson syndrome (SJS) is a life-threatening skin rash condition that can be triggered by a reaction to medication, the most common drugs / prescriptions / RX that cause SJS include:
Anticonvulsants: Lamotrigine, carbamazepine, phenytoin, and phenobarbital
Anti-gout medications: Allopurinol, especially in doses of more than 100 mg per day
Sulfonamides: Cotrimoxazole and sulfasalazine
The impact of Stevens Johnson Syndrome on the eyes is particularly prominent, resulting in severe eye pain, light sensitivity, vision loss, and, in some cases, blindness.
However, advances in care and treatment options for Stevens Johnson Syndrome have vastly improved patient outcomes, including the ability to preserve eye health.
The purpose of the Stevens Johnson Syndrome Study is to connect individuals who have been diagnosed with SJS/TEN. This will be used to identify the nature and risk for long-term complications of SJS/TEN and genetic risk factors associated with drug-induced SJS/TEN.
Spotlight on Stevens Johnson Syndrome
Stevens-Johnson syndrome (SJS), also called erythema multiforme major, is a systemic disease mostly involving the skin and mucous membranes. The cutaneous lesions are those of erythema multiforme, which may be erythematous papules, vesicles, bullae, or iris lesions.
Mucosal lesions occur and include conjunctivitis as well as oral and genital ulcers. In addition, hepatitis, nephritis, gastrointestinal bleeding, pneumonia, arthritis, arthralgia, fever, and myalgia have all been reported. SJS is believed to be the result of a cell-mediated hypersensitivity reaction to a number of immunologic stimuli including drugs and infectious agents.
The differential diagnosis includes other diseases that can result in cutaneous and mucous membrane lesions. Vesiculo-bullous diseases such as pemphigus vulgaris, erosive lichen planus, and varicella zoster may mimic SJS. Behcet's and Reiter's syndrome may have ocular and genital lesions that can be confused with those seen in SJS. The diagnosis is made when the characteristic drug reaction rash appears 1 to 3 weeks after exposure to a known stimulus and can not be explained by some other diagnosis. The treatment depends, in part, on the suspected precipitating cause.
Implicated drugs, such as sulfa drugs, penicillin, or anticonvulsants, especially phenytoin, should be discontinued. Infectious agents such as herpes simplex virus 1 or 2, or Mycoplasma pneumoniae, should be identified and appropriately treated. The most commonly prescribed therapy for SJS is corticosteroids.
The most frequent complications are keratitis, uveitis, and perforation of the bulb--all of which may result in permanent visual impairment.
Our research is focused on improving patient outcomes and quality of life. We prioritize the needs and perspectives of patients in all aspects of our work.
We are conducting cutting-edge research in a variety of areas, including genetics, immunology, and regenerative medicine.
Jha N, Alexander E, Kanish B, Badyal DK. A Study of Cutaneous Adverse Drug Reactions in a Tertiary Care Center in Punjab. Indian Dermatol Online J. 2018 Sep-Oct;9(5):299-303. [PMC free article] [PubMed]
Auyeung J, Lee M. Successful Treatment of Stevens-Johnson Syndrome with Cyclosporine and Corticosteroid. Can J Hosp Pharm. 2018 Jul-Aug;71(4):272-275. [PMC free article] [PubMed]
KIyer G, Srinivasan B, Agarwal S, Ravindran R, Rishi E, Rishi P, Krishnamoorthy S. Boston Type 2 keratoprosthesis- mid term outcomes from a tertiary eye care centre in India. Ocul Surf. 2019 Jan;17(1):50-54. [PubMed]
Frey N, Bodmer M, Bircher A, Jick SS, Meier CR, Spoendlin J. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Association with Commonly Prescribed Drugs in Outpatient Care Other than Anti-Epileptic Drugs and Antibiotics: A Population-Based Case-Control Study. Drug Saf. 2019 Jan;42(1):55-66. [PubMed]
Sato S, Kanbe T, Tamaki Z, Furuichi M, Uejima Y, Suganuma E, Takano T, Kawano Y. Clinical features of Stevens-Johnson syndrome and toxic epidermal necrolysis. Pediatr Int. 2018 Aug;60(8):697-702. [PubMed]
Yang SC, Hu S, Zhang SZ, Huang JW, Zhang J, Ji C, Cheng B. Corrigendum to "The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in China". J Immunol Res. 2018;2018:4154507. [PMC free article] [PubMed]
Velasco-Tirado V, Alonso-Sardón M, Cosano-Quero A, Romero-Alegría Á, Sánchez-Los Arcos L, López-Bernus A, Pardo-Lledías J, Belhassen-García M. Life-threatening dermatoses: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Impact on the Spanish public health system (2010-2015). PLoS One. 2018;13(6):e0198582. [PMC free article] [PubMed]
Safiri S, Ashrafi-Asgarabad A. The risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptic drugs: Comment on data sparsity. Epilepsia. 2018 May;59(5):1083-1084. [PubMed]
Wolf R, Marinović B. Drug eruptions in the mature patient. Clin Dermatol. 2018 Mar-Apr;36(2):249-254. [PubMed]
Tangamornsuksan W, Lohitnavy M. Association Between HLA-B*1301 and Dapsone-Induced Cutaneous Adverse Drug Reactions: A Systematic Review and Meta-analysis. JAMA Dermatol. 2018 Apr 01;154(4):441-446. [PMC free article] [PubMed]
Richard EB, Hamer D, Musso MW, Short T, O'Neal HR. Variability in Management of Patients With SJS/TEN: A Survey of Burn Unit Directors. J Burn Care Res. 2018 Jun 13;39(4):585-592. [PubMed]
Ortonne N. [Histopathology of cutaneous drug reactions]. Ann Pathol. 2018 Feb;38(1):7-19. [PubMed]
Plachouri KM, Vryzaki E, Georgiou S. Cutaneous Adverse Events of Immune Checkpoint Inhibitors: A Summarized Overview. Curr Drug Saf. 2019;14(1):14-20. [PubMed]
Lerma V, Macías M, Toro R, Moscoso A, Alonso Y, Hernández O, de Abajo FJ. Care in patients with epidermal necrolysis in burn units. A nursing perspective. Burns. 2018 Dec;44(8):1962-1972. [PubMed]
Kumar R, Das A, Das S. Management of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: Looking Beyond Guidelines! Indian J Dermatol. 2018 Mar-Apr;63(2):117-124. [PMC free article] [PubMed]
Schneider JA, Cohen PR. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Concise Review with a Comprehensive Summary of Therapeutic Interventions Emphasizing Supportive Measures. Adv Ther. 2017 Jun;34(6):1235-1244. [PMC free article] [PubMed]
Zhang S, Tang S, Li S, Pan Y, Ding Y. Biologic TNF-alpha inhibitors in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis: a systemic review. J Dermatolog Treat. 2020 Feb;31(1):66-73. [PubMed]
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013 Aug;69(2):187.e1-16; quiz 203-4. [PubMed]
Hsu DY, Brieva J, Silverberg NB, Silverberg JI. Morbidity and Mortality of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in United States Adults. J Invest Dermatol. 2016 Jul;136(7):1387-1397. [PubMed]
Bettuzzi T, Penso L, de Prost N, Hemery F, Hua C, Colin A, Mekontso-Dessap A, Fardet L, Chosidow O, Wolkenstein P, Sbidian E, Ingen-Housz-Oro S. Trends in mortality rates for Stevens-Johnson syndrome and toxic epidermal necrolysis: experience of a single centre in France between 1997 and 2017. Br J Dermatol. 2020 Jan;182(1):247-248. [PubMed]
Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T. Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Clin Rev Allergy Immunol. 2018 Feb;54(1):147-176. [PubMed]
Papp A, Sikora S, Evans M, Song D, Kirchhof M, Miliszewski M, Dutz J. Treatment of toxic epidermal necrolysis by a multidisciplinary team. A review of literature and treatment results. Burns. 2018 Jun;44(4):807-815. [PubMed]
Antoon JW, Goldman JL, Shah SS, Lee B. A Retrospective Cohort Study of the Management and Outcomes of Children Hospitalized with Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. J Allergy Clin Immunol Pract. 2019 Jan;7(1):244-250.e1. [PMC free article] [PubMed]
Antoon JW, Goldman JL, Lee B, Schwartz A. Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis. Pediatr Dermatol. 2018 Mar;35(2):182-187. [PubMed]
Determining causality is critical for prompt discontinuation of the culprit drug and strict avoidance of the culprit drug and potentially cross-reactive drugs in the future.
Causality is primarily determined through careful history-taking and construction of a drug timeline, including prescription drugs, over-the-counter medications, and supplements. Determining when each drug was started, stopped, held, or had a dose change is critical.
Causes: SJS and TEN are typically drug-induced, with common triggers including antibiotics, anticonvulsants, and over-the-counter medications like ibuprofen.
Additional Resources
MedWatchLearn teaches health professionals, and consumers how to complete the forms necessary to report problems to FDA. Here, you have the opportunity to practice filling out FDA Form 3500 (for health professionals) or FDA Form 3500B (for consumers).
Each case study promotes active learning through exercises, instructor-led discussions, and quizzes. Designed as a fictional case study based on a real-world scenario, the curriculum follows a format similar to Harvard Business Review Case Studies and builds on previous case studies.
A national campaign to raise awareness of the dangers of buying prescription medicines from fake online pharmacies.
Original article announcing the implementation and rationale for the MedWatch program.
Culture test: Small samples of the skin or mouth are sent to the laboratory to check for infections and the type of organism causing the infection.
X-ray: Chest X-ray is taken to check for indications of pneumonia.
Blood test: To check for the infections or other causes.
Skin biopsy: A small sample of the skin is sent for microscopic examination to confirm Stevens-Johnson syndrome, and check for other causes, such as infections.
CJ Hsiao, JYY Lee, TW Wong… - British Journal of …, 2001 - academic.oup.com… To the best of our knowledge, this is the first report of FDE due to lamotrigine (Lamictal ® ), …
There was no specific diagnosis of Stevens–Johnson syndrome (SJS) or FDE made …Save Cite Cited by 19 Related articles All 8 versions[PDF] psu.edu
JL Rosselli, JP Karpinski - Annals of Pharmacotherapy, 2011 - journals.sagepub.com… The risk of Stevens-Johnson syndrome, a dose-related adverse effect, can be minimized …
Le risque du syndrome de Stevens-Johnson, un effet secondaire dose-apparenté, peut être …Save Cite Cited by 12 Related articles All 6 versions[PDF] springer.com
BJ McKinley, ME Allen, N Michels - European Journal of Medical …, 2023 - Springer… Ultraviolet radiation (UVR) exposure is commonly reported as a risk factor for Stevens–Johnson
syndrome (SJS) and toxic epidermal necrolysis (TEN). However, minimal evaluation of …Save Cite Cited by 6 Related articles All 11 versions
T Zeng, YS Long, FL Min, WP Liao… - International journal of …, 2015 - Wiley Online LibraryBackground Despite several studies investigating the association between the human
leukocyte antigen HLA ‐B*1502 allele and lamotrigine‐induced Stevens–Johnson syndrome ( …Save Cite Cited by 72 Related articles All 7 versions
BD Newell, M Moinfar, AJ Mancini… - Pediatric …, 2009 - Wiley Online Library… range from an exanthem to Stevens–Johnson syndrome (C-1, … skin findings consistent with
Stevens–Johnson syndrome/toxic … reoccurrence developed Stevens–Johnson syndrome and …Save Cite Cited by 83 Related articles All 9 versions[PDF] jmedicalcasereports.org
V Shah, P Patel, K Ullrich - Frontiers in Medical Case …, 2021 - jmedicalcasereports.org… When compared to other familiar cutaneous hypersensitivity reactions such as Steven-Johnson
Syndrome, toxic epidermal necrolysis, and acute generalized exanthematous pustulosis, …Save Cite Related articles All 4 versions
Misdiagnosis of Stevens-Johnson Syndrome (SJS) can occur due to several factors:
Rarity: SJS is a rare condition, affecting only a small number of people each year. This means many healthcare providers may have limited experience diagnosing or treating it.
Similar Symptoms: Early symptoms of SJS, such as fever, fatigue, and sore throat, can resemble more common conditions like the flu or allergic reactions. This can lead to initial misdiagnosis.
Lack of Awareness: Due to its rarity, there may be a lack of awareness about SJS among both healthcare providers and the general public. This can contribute to delays in recognizing and diagnosing the condition.
Complex Presentation: SJS can present with a wide range of symptoms affecting the skin and mucous membranes, making it challenging to distinguish from other conditions with similar presentations.
Delayed Diagnosis: Misdiagnosis or delayed diagnosis can lead to more severe symptoms and complications, as timely intervention is crucial for managing SJS effectively.
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