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  • Stevens-Johnson syndrome

Stevens-Johnson syndrome

Other Names: 
Dermatostomatitis, Stevens Johnson type; Stevens Johnson syndromeDermatostomatitis, Stevens Johnson type; Stevens Johnson syndrome

Disease Information

Summary
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have "SJS/TEN overlap." All forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms (such as general ill feeling, body aches, and cough). Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to "raw" areas of skin that are painful. This often starts on the face and then spreads to other parts of the body. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis (when the eyes are affected), trouble swallowing and breathing (when the mouth and airway are affected), and difficulty urinating and genital pain (when the genitals are affected). SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified. People that may be at increased risk to develop SJS/TEN include those with HIV, a weakened immune system, a personal or family history of the condition, and certain variations of a gene called HLA-B. There are no universal diagnostic criteria for SJS/TEN. Currently the diagnosis is based on the person's medical history and symptoms. People suspected of having SJS/TEN should be admitted to the hospital to confirm the diagnosis and assess severity.Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have "SJS/TEN overlap." All forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms (such as general ill feeling, body aches, and cough). Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to "raw" areas of skin that are painful. This often starts on the face and then spreads to other parts of the body. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis (when the eyes are affected), trouble swallowing and breathing (when the mouth and airway are affected), and difficulty urinating and genital pain (when the genitals are affected). SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified. People that may be at increased risk to develop SJS/TEN include those with HIV, a weakened immune system, a personal or family history of the condition, and certain variations of a gene called HLA-B. There are no universal diagnostic criteria for SJS/TEN. Currently the diagnosis is based on the person's medical history and symptoms. People suspected of having SJS/TEN should be admitted to the hospital to confirm the diagnosis and assess severity.
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Summary
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have "SJS/TEN overlap." All forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms (such as general ill feeling, body aches, and cough). Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to "raw" areas of skin that are painful. This often starts on the face and then spreads to other parts of the body. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis (when the eyes are affected), trouble swallowing and breathing (when the mouth and airway are affected), and difficulty urinating and genital pain (when the genitals are affected). SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified. People that may be at increased risk to develop SJS/TEN include those with HIV, a weakened immune system, a personal or family history of the condition, and certain variations of a gene called HLA-B. There are no universal diagnostic criteria for SJS/TEN. Currently the diagnosis is based on the person's medical history and symptoms. People suspected of having SJS/TEN should be admitted to the hospital to confirm the diagnosis and assess severity.Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have "SJS/TEN overlap." All forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms (such as general ill feeling, body aches, and cough). Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to "raw" areas of skin that are painful. This often starts on the face and then spreads to other parts of the body. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis (when the eyes are affected), trouble swallowing and breathing (when the mouth and airway are affected), and difficulty urinating and genital pain (when the genitals are affected). SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified. People that may be at increased risk to develop SJS/TEN include those with HIV, a weakened immune system, a personal or family history of the condition, and certain variations of a gene called HLA-B. There are no universal diagnostic criteria for SJS/TEN. Currently the diagnosis is based on the person's medical history and symptoms. People suspected of having SJS/TEN should be admitted to the hospital to confirm the diagnosis and assess severity.
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Resource(s) for Medical Professionals and Scientists on This Disease:
  • RARe-SOURCE™ offers rare disease gene variant annotations and links to rare disease gene literature.

About Stevens-Johnson syndrome

Many rare diseases have limited information. Currently, GARD aims to provide the following information for this disease:

  • Symptoms:May start to appear at any time in life.
  • Cause:GARD does not currently have information about the cause of this disease.
  • Organizations:Patient organizations dedicated to this rare disease are available on GARD, or you may contact a GARD Information Specialist for additional information.
  • Categories:Skindiseases

When Do Symptoms of Stevens-Johnson syndrome Begin?

Symptoms of this disease may start to appear at any time in life.

The age symptoms may begin to appear differs between diseases. Symptoms may begin in a single age range, or during several age ranges. The symptoms of some diseases may begin at any age. Knowing when symptoms may have appeared can help medical providers find the correct diagnosis.
PrenatalSelected
Before Birth
NewbornSelected
Birth-4 weeks
InfantSelected
1-23 months
ChildSelected
2-11 years
AdolescentSelected
12-18 years
AdultSelected
19-65 years
Older AdultSelected
65+ years
Symptoms may start to appear at any time in life.
This information comes from theHuman Phenotype Ontology (HPO)

Navigating Health Care Decisions

On average, it can take more than six years to receive an accurate diagnosis. Many primary care providers (PCPs) may not be familiar with rare diseases, and patients often need to visit multiple specialists or seek second opinions to get answers.

If a diagnosis remains unclear, visiting a multidisciplinary care center or university hospital may help. These centers bring together teams of specialists who can work together to evaluate symptoms and coordinate a diagnosis. This team-based approach is also helpful after a diagnosis, when managing care for rare diseases.

Because only about 5% of rare diseases have FDA-approved treatments, finding the right healthcare team to manage your symptoms and overall health is essential. People living with rare diseases often face challenges such as delayed diagnosis, limited treatment options, and difficulty accessing knowledgeable providers. Building a care team that understands your needs can make a significant difference in your quality of life.

Your Health Care Team

Why is building the right health care team important?

Building the right health care team is key to the diagnosis, treatment, and management of your long-term health journey living with a rare disease. Start by choosing a primary care provider (PCP). Your PCP will be your main point of contact and help coordinate care with other medical professionals. Your PCP may order tests or refer you to specialists. To find a PCP near you, use the Medicare provider search tool and enter your location and “Primary Care Provider.”

 

Seeing multiple specialists is important for people with rare diseases because these conditions often affect many parts of the body and require care from doctors with different expertise. Most primary care providers may not be familiar with rare diseases, so involving specialists can lead to a more accurate diagnosis and better care. A coordinated team approach ensures that all symptoms are addressed and that care is well-managed. It can also connect patients with the latest research or treatment options.

A PCP that specializes in the care of children is called a pediatrician. Use this tool by the American Academy of Pediatrics to find a pediatrician in your area by inputting your location.
These specialists may help in the diagnosis, management, and treatment of Stevens-Johnson syndrome:
 

Multidisciplinary Care Centers

Is It Time to Get a Second Opinion or Specialized Evaluation?

If you've visited your PCP, met with specialists, and undergone the recommended tests, but are still searching for a diagnosis, it may be time to visit an academic medical center or, for pediatric patients, a children's hospital. Academic medical centers and children's hospitals, often called multidisciplinary care centers, typically bring together specialists from different fields to work together on complex cases like rare diseases. 

 

Multidisciplinary care centers may offer more coordinated care and be involved in clinical research, which may help reduce the time to diagnosis and provide access to emerging diagnostic tools. Specialists at these centers may have a deeper understanding of rare diseases and serve as a resource when you'd like a second opinion, particularly when test results or treatment plans are not delivering expected results. 

 

Use this tool to find hospitals that may partner with medical schools and programs in your area.

Children’s hospitals and large teaching hospitals may also offer dedicated specialists and programs for pediatric patients with undiagnosed or rare diseases. These programs bring pediatric experts together in one place and may provide more coordinated care for your child.

Use this tool to search for children’s or university hospitals in your area.

Multidisciplinary Care Centers

Is It Time to Get a Second Opinion or Specialized Evaluation?

If you've visited your PCP, met with specialists, and undergone the recommended tests, but are still searching for a diagnosis, it may be time to visit an academic medical center or, for pediatric patients, a children's hospital. Academic medical centers and children's hospitals, often called multidisciplinary care centers, typically bring together specialists from different fields to work together on complex cases like rare diseases. 

 

Multidisciplinary care centers may offer more coordinated care and be involved in clinical research, which may help reduce the time to diagnosis and provide access to emerging diagnostic tools. Specialists at these centers may have a deeper understanding of rare diseases and serve as a resource when you'd like a second opinion, particularly when test results or treatment plans are not delivering expected results. 

 

Use this tool to find hospitals that may partner with medical schools and programs in your area.

Children’s hospitals and large teaching hospitals may also offer dedicated specialists and programs for pediatric patients with undiagnosed or rare diseases. These programs bring pediatric experts together in one place and may provide more coordinated care for your child.

Use this tool to search for children’s or university hospitals in your area.

Rare Disease Experts

How can you find a rare disease expert?

If a diagnosis, care management, or treatment plan remains unclear despite extensive efforts by your PCP and specialists, it may be time to find a rare disease expert for your disease, if available. A rare disease expert is a medical provider that has knowledge or training on specific rare disease(s), but there may only be a few experts in your state, region, or country. Rare disease experts may work at large research or teaching hospitals, sometimes called centers of excellence. Centers of Excellence commit to sharing knowledge and best practices that can lead to improved care and treatment for individuals living with a rare disease. 

You can alsocontact a GARD Information Specialist for help finding experts, centers of excellence, or clinics that focus on your disease.

You can ask your care providers for help finding an expert or use directory tools to search for experts near you. The National Organization for Rare Disorders (NORD) maintains a list of rare disease centers with experts that work together to find treatments and cures for a broad range of rare diseases. Search NORD's Center Directory to find experts near you.

Rare Disease Experts

How can you find a rare disease expert?

You can ask your care providers for help finding an expert or use directory tools to search for experts near you. The National Organization for Rare Disorders (NORD) maintains a list of rare disease centers with experts that work together to find treatments and cures for a broad range of rare diseases. Search NORD's Center Directory to find experts near you.
 

Find Your Community

How can patient organizations help?

Patient organizations can help patients and families connect. They build public awareness of the disease and are a driving force behind research to improve patients' lives. They may offer online and in-person resources to help people live well with their disease. Many collaborate with medical experts and researchers.

Services of patient organizations differ, but may include:

  • Ways to connect to others and share personal stories
  • Easy-to-read information
  • Up-to-date treatment and research information
  • Patient registries
  • Lists of specialists or specialty centers
  • Financial aid and travel resources


Please note: GARD provides organizations for informational purposes only and not as an endorsement of their services.Contact a GARD Information Specialist for more information on organizations that may be dedicated to this disease. Please contact an organization directly if you have questions about the information or resources it provides.


View GARD's criteria for including patient organizations, which can be found under the FAQs on our About GARD page.Request an update or to have your organization added to GARD. 

Patient Organizations

5 Organizations

Organization Name

Who They Serve

Helpful Links

Country

People With

Skin diseases

Country

United States

People With

Rare Diseases

Country

United States

People With

Rare Diseases

Country

United States

People With

Rare Diseases

Country

United States

People With

Rare Diseases

Country

United States

Participate in Research

Clinical studies are a part of clinical research and play an important role in medical advances for rare diseases. Through clinical studies, researchers may ultimately uncover better ways to treat, prevent, diagnose, and understand human diseases.

What Are Clinical Studies?

Clinical studies are medical research involving people as participants. There are two main types of clinical studies:

  1. Clinical trials determine if a new test or treatment for a disease is effective and safe by comparing groups receiving different tests/treatments.
  2. Observational studies involve recording changes over time among a specific group of people in their natural settings.

Learn more about clinical trials from this National Institutes of Health webpage.

Why Participate in Clinical Studies?

People participate in clinical trials for many reasons. People with a disease may participate to receive the newest possible treatment and additional care from clinical study staff as well as to help others living with the same or a similar disease. Healthy volunteers may participate to help others and to contribute to moving science forward.

To find the right clinical study we recommend you consult your doctors, other trusted medical professionals, and patient organizations. Additionally, you can useClinicalTrials.gov to search for clinical studies by disease, terms, or location.

What if There Are No Available Clinical Studies?

ResearchMatch helps connect people interested in research studies with researchers from top medical centers across the United States. Anyone from the U.S. can register with this free program funded by NIH. Researchers from participating institutions use the database to search for and invite patients or healthy volunteers who meet their study criteria to participate.

Why may you want to consider joining the All of Us Research Program?

TheAll of Us Research Program is inviting 1 million people from all backgrounds across the U.S. to help build one of the most diverse health databases in history. Researchers will use the data to learn how our biology, lifestyle, and environment affect health. This may one day help them find ways to treat and prevent diseases.

What Are Clinical Studies?

Clinical studies are medical research involving people as participants. There are two main types of clinical studies:

  1. Clinical trials determine if a new test or treatment for a disease is effective and safe by comparing groups receiving different tests/treatments.
  2. Observational studies involve recording changes over time among a specific group of people in their natural settings.

Learn more about clinical trials from this National Institutes of Health webpage.

Clinical studies are medical research involving people as participants. There are two main types of clinical studies:

  1. Clinical trials determine if a new test or treatment for a disease is effective and safe by comparing groups receiving different tests/treatments.
  2. Observational studies involve recording changes over time among a specific group of people in their natural settings.

Learn more about clinical trials from this National Institutes of Health webpage.

Read More

Why Participate in Clinical Studies?

People participate in clinical trials for many reasons. People with a disease may participate to receive the newest possible treatment and additional care from clinical study staff as well as to help others living with the same or a similar disease. Healthy volunteers may participate to help others and to contribute to moving science forward.

To find the right clinical study we recommend you consult your doctors, other trusted medical professionals, and patient organizations. Additionally, you can useClinicalTrials.gov to search for clinical studies by disease, terms, or location.People participate in clinical trials for many reasons. People with a disease may participate to receive the newest possible treatment and additional care from clinical study staff as well as to help others living with the same or a similar disease. Healthy volunteers may participate to help others and to contribute to moving science forward.

To find the right clinical study we recommend you consult your doctors, other trusted medical professionals, and patient organizations. Additionally, you can useClinicalTrials.gov to search for clinical studies by disease, terms, or location.
Read More

What if There Are No Available Clinical Studies?

ResearchMatch helps connect people interested in research studies with researchers from top medical centers across the United States. Anyone from the U.S. can register with this free program funded by NIH. Researchers from participating institutions use the database to search for and invite patients or healthy volunteers who meet their study criteria to participate.

Why may you want to consider joining the All of Us Research Program?

TheAll of Us Research Program is inviting 1 million people from all backgrounds across the U.S. to help build one of the most diverse health databases in history. Researchers will use the data to learn how our biology, lifestyle, and environment affect health. This may one day help them find ways to treat and prevent diseases.

ClinicalTrials.gov Logo
ClinicalTrials.gov, an affiliate of NIH, provides current information on clinical research studies in the United States and abroad. Talk to a trusted doctor before choosing to participate in any clinical study. We recommend checking this site often and searching for studies with related terms/synonyms to improve results.
GARDGenetic and Rare Diseases
Information Center
Contact a GARD Information Specialist if you need help finding more information on this rare disease or available clinical studies. Please note that GARD cannot enroll individuals in clinical studies.
Leave a detailed voice message and a GARD information specialist will respond to your inquiry.
Use the contact form to send your questions to a GARD Information Specialist.

Please allow 2 to 10 business days for us to respond.
ClinicalTrials.gov Logo
ClinicalTrials.gov, an affiliate of NIH, provides current information on clinical research studies in the United States and abroad. Talk to a trusted doctor before choosing to participate in any clinical study. We recommend checking this site often and searching for studies with related terms/synonyms to improve results.
GARDGenetic and Rare Diseases
Information Center
Contact a GARD Information Specialist if you need help finding more information on this rare disease or available clinical studies. Please note that GARD cannot enroll individuals in clinical studies.
Leave a detailed voice message and a GARD information specialist will respond to your inquiry.
Use the contact form to send your questions to a GARD Information Specialist.

Please allow 2 to 10 business days for us to respond.

Last Updated: February 2026

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