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Stevens-Johnson Syndrome (SJS) is a rare but serious medical condition that primarily affects the skin and mucous membranes. While SJS can occur at any age, many cases commonly occur in children and younger adults. Although rare, the impact of SJS on a child’s health can be life-threatening, making awareness and early intervention critical. Causes and Triggers of SJS in Children One of the primary causes of SJS in children is an adverse reaction to medications. Studies have identified several drugs commonly linked to SJS, including: ●      Antibiotics (e.g., penicillins, sulfonamides, cephalosporins) ●      Anticonvulsants (e.g., carbamazepine, lamotrigine, phenytoin, phenobarbital, valproic acid) ●      Nonsteroidal anti-inflammatory drugs (NSAIDs) (e.g., ibuprofen, naproxen) In addition to medications, SJS can also be caused by infections, particularly Mycoplasma pneumoniae , a bacterial infection that affects the lungs. Viral infections such as herpes simplex and influenza have also been associated with cases of SJS in children. Symptoms and Diagnosis SJS typically presents with vague symptoms, making early diagnosis challenging. It often starts with flu-like symptoms and some early warning signs include: ●      Fever and body aches ●      Painful blisters in the mouth, nose, eyes, and genitals ●      Red or purplish skin rashes that spread quickly ●      Peeling or sloughing of the skin ●      Eye redness and irritation, which can lead to long-term vision problems Since these symptoms can be mistaken for other illnesses, doctors may use a combination of clinical evaluation and skin biopsy to confirm the diagnosis. Treatment Options for Children with SJS There is no single cure for SJS, so treatment focuses on stopping the progression, managing symptoms, and preventing complications. The key steps include: Discontinuing the Trigger  – If a medication is identified as the cause, it should be stopped immediately under medical supervision. Hospitalization  – Most children with SJS require admission to specialized units, such as burn centers or intensive care units, for close monitoring and treatment. Supportive Care  – Management includes pain control, wound care, intravenous fluids, and nutritional support to promote healing and prevent complications. Medication Therapy  – Cyclosporine has shown greater effectiveness in reducing disease severity compared to intravenous immunoglobulin (IVIG) and corticosteroids, which are not generally recommended. Specialist Care  – Given the multisystem involvement of SJS, early consultation with ophthalmologists, urologists, and gynecologists is essential to prevent complications such as lacrimal duct scarring, urethral and vaginal strictures, and long-term functional impairments. Complications and Long-Term Effects Even after recovery, many children experience long-term complications from SJS, including: ●      Chronic eye problems (dryness, scarring, vision loss) ●      Skin sensitivity and scarring ●      Psychological distress, such as anxiety or post-traumatic stress disorder (PTSD) Preventing SJS in Children Since SJS is mostly caused by drug reactions, careful medication use is crucial. If a child has had SJS in the past, avoiding the suspected medication and its drug class with a similar chemical structure is essential. Some genetic tests may help predict SJS risk in certain populations. Raising Awareness for a Rare but Serious Condition Although SJS is rare, its effects can be devastating. Awareness among parents, doctors, and caregivers can lead to earlier diagnosis and better outcomes. Understanding the signs, causes, and treatments is essential. Additionally, HLA genetic testing for drugs with strong associations can help identify at-risk individuals, enabling preventive strategies and reducing the incidence of SJS in children. References Del Pozzo-Magana BR, Lazo-Langner A, Carleton B, Castro-Pastrana LI, Rieder MJ. A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. J Popul Ther Clin Pharmacol. 2011 Mar 21;18(1):121-33. Ferrandiz-Pulido C, Garcia-Patos V. A review of causes of Stevens–Johnson syndrome and toxic epidermal necrolysis in children. Archives of disease in childhood. 2013 Dec 1;98(12):998-1003. Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years’ experience. Drug safety. 2002 Nov;25:965-72. Lucia L, Silvia C, Paolo B, Roberto B, Fabio C, Francesca S, Francesca M, Giuseppe C, Fabrizio F, Carlo C. Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis. Acta Bio Medica: Atenei Parmensis. 2019;90(Suppl 3):52. White KD, Abe R, Ardern-Jones M, Beachkofsky T, Bouchard C, Carleton B, et al., SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation. J Allergy Clin Immunol Pract. 2018 Jan-Feb;6(1):38-69. Chang WC, Abe R, Anderson P, Anderson W, Ardern-Jones MR, Beachkofsky TM, Bellón T, et al., SJS/TEN 2019: From science to translation. J Dermatol Sci. 2020 Apr;98(1):2-12.

Every February, Rare Disease Month brings attention to conditions that affect only a small percentage of the population. Although these diseases are rare, they still have a devastating impact on the lives of those affected. One such condition is Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)—two rare but severe skin reactions that require urgent medical care.  While SJS/TEN is life-threatening, it remains largely unknown to the public and even under-recognized in the medical community. This lack of awareness contributes to delayed diagnoses, mismanagement, and a struggle for adequate research funding. This Rare Disease Month, we’re shedding light on the importance of recognizing and supporting those affected by SJS/TEN.  How Rare is SJS/TEN?  SJS/TEN is one of the rarest life-threatening medical emergencies, affecting an estimated 1 to 2 people per million per year. Despite its rarity, the condition has a high mortality rate, with up to 35% of TEN cases being fatal, and patients often face lifelong complications.  Unlike many rare diseases that are present from birth, SJS/TEN can happen to anyone at any time. It is most often triggered by medications, including antibiotics, anticonvulsants, and allopurinol. Because SJS/TEN does not have a single genetic or hereditary cause, it is impossible to predict who will develop it, making prevention challenging.  Another alarming aspect of SJS/TEN’s rarity is the limited experience many doctors have in diagnosing it. Since the condition is so uncommon, many cases are initially misdiagnosed as common viral infections, allergies, or other skin conditions, leading to delays in stopping the triggering medication, which is a key factor in survival.  The Hidden Challenges of Rare Diseases  Being diagnosed with any rare disease can feel isolating, and SJS/TEN is no exception. The rarity of the condition creates several challenges for patients and healthcare providers, including:  1. Delayed Diagnosis and Treatment Since most doctors do not encounter cases of SJS/TEN often, early symptoms like fever, sore throat, and eye irritation are often mistaken for another condition. By the time the skin symptoms appear, the condition has already progressed, making early intervention difficult.  2. Limited Research and Awareness  Compared to other diseases, SJS/TEN research receives little funding. Since the disease is rare, clinical trials are limited, making it harder to find effective treatments. More research is needed to:  ● Identify better treatment options beyond supportive care.  ● Develop early warning tests for those at risk.  ● Improve long-term rehabilitation and mental health support for survivors. 3. Lifelong Health Complications  For those who survive SJS/TEN, the journey does not end with recovery. Many patients experience permanent complications, including:  ● Chronic eye problems, including severe dry eye, scarring, and even blindness. ● Skin sensitivity and scarring, sometimes leading to disfigurement.  ● Psychological distress, including PTSD and anxiety, due to the traumatic experience.  These long-term effects make post-recovery support essential, yet few resources exist to help survivors reintegrate into their daily lives.  Looking Ahead: Hope for the Future  While SJS/TEN remains rare, there is hope for better prevention, treatment, and awareness. Advances in pharmacogenetics have already identified genetic markers—such as HLA-B*1502—that can help predict a person’s risk of developing SJS/TEN when taking certain medications.  Additionally, increased research into drug safety and adverse reactions has the potential to reduce the number of new cases. By implementing stronger screening measures and improving early recognition, fewer people may develop SJS/TEN in the future.  However, more work still needs to be done. Advocacy efforts by patients, healthcare providers, and researchers are essential to ensuring that SJS/TEN is properly recognized, diagnosed early, and treated with urgency. Why Rare Disease Awareness Matters  SJS/TEN may be rare, but for those affected, it changes everything. This Rare Disease Month, we encourage people to take a moment to learn about SJS/TEN, support rare disease research, and help amplify the voices of those living with conditions that often go unnoticed.  The more we educate, advocate, and support those with rare diseases, the closer we come to a world where no one faces these conditions alone.  REFERENCES  Marks, M. E., Botta, R. K., Abe, R., Beachkofsky, T. M., Boothman, I., Carleton, B. C., ... & Phillips, E. J. (2023). Updates in SJS/TEN: collaboration, innovation, and community. Frontiers in Medicine, 10 , 1213889.  Harr, T., & French, L. E. (2010). Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet Journal of Rare Diseases, 5 (1), 39.  Desai, S., Yasmeen, N., Amgoth, N., Rama Rao, A., Vidhya, B., & Ragaswetha, J. (2022). A glance on medication triggered rare disease—Stevens Johnson syndrome as global challenge in healthcare system. Impending Inquisitions in Humanities and Sciences (ICIIHS-2022) , 484-486.  Cavero-Carbonell, C., Gimeno-Martos, S., Gras-Colomer, E., Páramo-Rodríguez, L., Alfaro, A., & Zurriaga, O. (2016). Stevens-Johnson Syndrome: identification of the risk factors in a rare disease. European Journal of Public Health, 26 (Suppl 1), 376.  Rzany, B., Mockenhaupt, M., Baur, S., Schröder, W., Stocker, U., Müller, J., & Schöpf, E. (1996). Epidemiology of Erythema Exsudativum Multiforme Majus, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis in Germany (1990-1992): Structure and Results of a Population-Based Registry. Journal of Clinical Epidemiology, 49 (7), 769-773.  Lee, H. Y., Walsh, S. A., & Creamer, D. (2017). Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up. British Journal of Dermatology, 177 (4), 924-935.

What Exactly is Stevens-Johnson Syndrome? Stevens-Johnson Syndrome (SJS) is a rare but serious disorder affecting the skin and mucous membranes, often triggered by a reaction to medication or infection. This condition causes the skin to blister and peel, forming painful lesions. If left untreated, SJS can lead to life-threatening complications, including infection and organ damage. You might wonder how SJS differs from other skin conditions. SJS is characterized by its sudden onset, typically beginning with flu-like symptoms before the skin reaction occurs. Often-affected sites include the mucous membrane surfaces of the mouth, eyes, and genitals, making it a medical emergency requiring immediate care. Who is Affected by Stevens-Johnson Syndrome? SJS is rare, affecting about 1 to 2 people per million each year. It can occur in individuals of all ages, but some people are at higher risk than others. Certain medications, such as antibiotics, anticonvulsants, and allopurinol are common triggers. People with HIV, a weakened immune system, or a genetic predisposition are also at higher risk. Recognizing the Signs and Symptoms of Stevens-Johnson Syndrome The early signs of SJS can easily be mistaken for common illnesses. Flu-like symptoms such as fever, cough, and sore throat often appear first. However, within days, a painful red or purplish rash develops, spreading across the body. This rash quickly turns into blisters, causing the skin to peel and leave raw areas exposed, which can lead to infection. Other symptoms include: ●            Red, sore eyes that may be sensitive to light ●            Swelling and crusting around the lips or mouth ●            Difficulty swallowing due to sores in the mouth and throat If you or a loved one experiences these symptoms, especially after starting a new medication, seek medical attention immediately. What Causes Stevens-Johnson Syndrome? SJS is often triggered by medications, with antibiotics, anticonvulsants, and pain relievers being the most common culprits. Infections like pneumonia or herpes can also cause SJS in some cases. Although rare, SJS may occur in individuals with genetic markers that make them more susceptible to adverse reactions. The body’s immune system plays a role in SJS by mistakenly attacking healthy skin cells, leading to the characteristic rash and blisters. How is Stevens-Johnson Syndrome Diagnosed? Diagnosing SJS can be challenging because its early symptoms mimic other conditions. A specialist, such as a dermatologist, will examine the skin and mucous membranes and may perform a skin biopsy to confirm the diagnosis. It's crucial to seek care from medical professionals experienced in managing SJS, as it requires urgent treatment. Does Stevens-Johnson Syndrome Mean I’ll Always Have Skin Reactions? Once someone has had SJS, they are at higher risk of developing it again if exposed to the same medication or trigger. For this reason, it's essential to avoid the suspected trigger and wear medical alert bracelets to inform healthcare providers of the condition. Living with SJS can be difficult, but recognizing the condition early and avoiding known triggers are key steps in managing it. Reviewed by: Dr. Bruce Carleton References Schwartz, R. A., & McDonough, P. H. (2013). Stevens-Johnson syndrome: A review of the literature. Journal of the American Academy of Dermatology , 69(1), 32-42. Mockenhaupt, M. (2011). Stevens-Johnson syndrome and toxic epidermal necrolysis: Clinical patterns, diagnostic considerations, etiology, and therapeutic management. Seminars in Cutaneous Medicine and Surgery , 30(4), 255-261. Harr, T., & French, L. E. (2010). Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet Journal of Rare Diseases , 5(1), 39. High, W. A., Roujeau, J. C., & Stern, R. S. (2008). Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and management.  Dermatologic Clinics , 26(4), 579-593.

During August, we have some challenges you can join to support us in our fight. Check it out. August 3rd Sunglasses Day Challenge: Support SJS survivors affected by photophobia by wearing dark glasses indoors, outdoors, and at night. Light sensitivity/photophobia is one of the long-term effects of SJS/TEN, as a result some survivors experience severe difficulty dealing with bright light both indoor and outdoor. Some describe this experience as temporary blindness. The next time you see an SJS/TEN survivor wearing dark glasses inside or during the night, think: this is not a fashion statement; it’s a survival statement. August 9th Visual Accessibility Day Challenge: Explore the visual accessibility function on your smartphone and try using these features! Also, be mindful of the e-mails you send today; think about the font size, colours, and spacing. How would these affect individuals with visual impairment? Visual impairment is a decreased ability to see that cannot be fixed with corrective devices such as glasses or contacts. Many SJS/TEN survivors have long-term complications to their eyes even after leaving the hospital. These complications often make it difficult for SJS/TEN survivors to read particular writing or see specific images. Did you know that nearly all smartphones have accessibility features to help overcome sensory impairment? August 17th Virtual Town Hall Challenge: Join us at our Virtual SJS Town Hall Please join us at the SJS Town Hall where we will be sharing updates, connecting with keynote speakers and listening to the harrowing stories of SJS / TEN survivors. ​ This town hall event offers public awareness, education, research updates as well as addressing some common issues and concerns on the subject. SJS/TEN patient representatives lovingly share their stories in an effort to help bring and spread awareness of the illness. August 17th-19th Virtual Move-A-Thon Challenge: Join SJS Canada from ANYWHERE as part of our Virtual “Move-a-Thon.” We are moving our bodies in support of Stevens-Johnson Syndrome Canada (SJSC) from August 17-19. August 18th SJS Awareness Day Challenge: Complete all checks for the day! Don't forget to Social media blast with informational facts about the illness Follow us on social media to hear survivors share their stories with us Encourage others to wear blue, SJS official color Be encouragement to tell someone about SJS/TEN throughout the day Join our virtual/in person SJS/TEN awareness Move-A-Thon Survivors can get together, within a virtual space, with a facilitator and share their experience and support each other. Please feel free to spread the word or distribute SJS awareness materials within the community August 23rd Hat Day Challenge: Support SJS/TEN survivors by wearing a broad rim hat! Check your closet – how many clothes can you identify with UPF? Wear your UPF clothing if you have any! And remember to wear sunscreen of at least SPF 30, reapplying every 80 minutes! Many survivors have to wear broad rim hats and other head covering to protect themselves from the sun as their skin has become very sensitive. Along with the dark glasses, the hat shades the eyes and help allow them to cope with the effect of the sun on their eyes. Some survivors also wear UPF (ultraviolet protection factor) clothing to protect their body from the sun’s UV rays. August 29th Eye Care Day Challenge: Remember to give your eyes a rest during your workday by using the 20-20-20 rule! Every 20 minutes, shift your eyes to look at an objective 20 feet away, for 20 seconds! (Have dry eyes? Use artificial lubricant or try a warm compress! Wet a clean washcloth with warm water and wring it out until somewhat dry. Place the washcloth over your closed eyes for at least 1 minute) The pain of SJS/TEN is so excruciating, sometimes just opening the eyes causes severe pain. As well some survivor’s eyes lids were sealed shut for days because numerous sores covered their eyes. When the eye lids were finally open again, some survivors sustain severe vision loss, and for some their eyelids never opens resulting in blindness. We kindly ask you to post your photo or video as you engage in these activities with #sjscantstopme

Meet Julie who miraculously survived SJS/TEN, not once but twice. With the help of her supportive mother Jean, Julie now uses her story to raise awareness, support and inspire other survivors. See video below. https://youtu.be/-Znz5lJTMzM?si=5Kjmvwj7BIbh0AWy&t=79 Her story begins at 1:19 and ends at 23:37.

https://youtu.be/_j3UWsEb5bM

https://youtu.be/rocX38HOUjE

Attention SJS survivors & care supporters Coming winter 2024 : to secure your spot Email: info@sjscanada.org Our FREE four weeks supportive counselling/educational session in collaboration with Elpizo Counselling Service is back.

The month of August is Stevens Johnson Syndrome Awareness Month in the USA. We wish to join our allies and combine our voices to spread awareness here in Canada. Join us and show your support by: Find and follow us on on Facebook. Like, repost, and share our weekly posts. Wear blue on August 18th, the official SJS awareness colour. Share your comments and photos wearing blue on our Facebook page in support of SJS Awareness Month. Tell us your SJS/TEN story

I had a bacterial infection in 2005. After 13 days of taking antibiotics, I developed a lot of bumps on my body, my face swelled up, my eyes and throat started to feel inflamed and irritated. I went to the doctor and was told I had an eye infection and was given sulfa (an eye drop) in addition to the oral sulfa antibiotics that I was already taking. Soon the skin around my face started peeling so I took a picture and sent it to my dad and brother. My brother’s pharmacist friend told him I was having a severe allergic reaction to the medication while my dad and I read up on the side effects of the antibiotics I was taking. By the time I was admitted to the hospital, my eyes were swelled shut, my skin was peeling, and raw from my scalp to torso. At the hospital, doctors stitched amniotic membrane to my eyes in the hopes that I would not lose my vision. I stayed in the hospital for 13 days. After one month they removed the stitches from my eyes but although I was able to see, I could hardly open them since they had become extremely photophobic. In 2012, I met Dr. Chiu who introduced me to PROSE contact lens which allowed me to open my eyes more and drastically reduced the photosensitivity. These lenses have also helped to decrease the irritation and redness of my eyes. Furthermore, I had cataract surgery for both my eyes. My skin has healed although my nailbeds are damaged and fragile. As part of my recovery, I started to hike and became a runner. I was able to train and run many organized races including two marathons. In June 2020, I joined the SJS Canada group and have been delighted by the support that the group provides for each other. Sonia’s care and attention to the members of this group is greatly appreciated, and her leadership has helped gain awareness and momentum for the SJS community. It has been a pleasure to be a part of this group.

Thank you to everyone who joined us for our AGM in May Below are some images from the event! Photos of our 2018 Annual General Meeting

SJS Founder, Sonia Whyte-Croasdaile was featured on Toronto’s news and entertainment show, The Scoop. Sonia took the liberty to help describe the complexities of SJS/TENS as a survivor and advocate to the shows audience. Sonia also highlighted a fund raiser and line up for the event.