Stevens-Johnson syndrome

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ORPHA:36426OMIM:608579L51.1
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1FDA treatments12Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Stevens-Johnson syndrome (SJS) is a rare but serious skin and mucous membrane disorder. It is sometimes called SJS for short. The condition causes the skin to blister, peel, and die in large patches, similar to a severe burn. The mucous membranes — the moist linings inside the mouth, eyes, nose, throat, and genitals — are almost always affected too. SJS is considered a medical emergency and requires immediate hospital care. In most cases, SJS is triggered by a reaction to a medication. Common triggers include antibiotics like sulfonamides, anti-seizure drugs such as carbamazepine and lamotrigine, allopurinol (used for gout), and certain pain relievers. Infections, especially with Mycoplasma pneumoniae, can also trigger SJS, particularly in children. The immune system mistakenly attacks the skin and lining tissues, causing widespread damage. Key symptoms include a painful red or purple rash that spreads quickly, blistering of the skin and mouth, peeling skin, fever, and sore eyes. Treatment focuses on stopping the triggering medication, managing pain, preventing infection, and supporting the body while it heals — similar to burn care. Most people are treated in a hospital, often in a burn unit or intensive care unit. Recovery can take weeks to months, and some people are left with lasting complications such as scarring, vision problems, or skin discoloration.

Also known as:

Key symptoms:

Painful red, purple, or dark rash that spreads quickly across the bodyBlisters forming on the skin and inside the mouth, eyes, nose, or genitalsSkin peeling off in sheetsHigh feverSore, red, or swollen eyesPainful swallowingMouth sores and ulcersBurning or stinging sensation on the skinFatigue and feeling very unwellDifficulty eating or drinking due to mouth painSensitivity to lightSwollen lips or tongue

Clinical phenotype terms (39)— hover any for plain English
EntropionHP:0000621Abnormality of the urethraHP:0000795Abnormal myocardium morphologyHP:0001637Abnormality of neutrophilsHP:0001874Hypokalemic metabolic alkalosisHP:0001960
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

1 available

Dyural 80-Lm

METHYLPREDNISOLONE ACETATE, LIDOCAINE HYDROCHLORIDE, BUPIVACAINE HYDROCHLORIDE, POVIDINE IODINE, ISOPROPYL ALCOHOL· Advanced Rx Pharmacy of Tennessee, LLC■ Boxed Warning

severe erythema multiforme (Stevens-Johnson syndrome)

No actively recruiting trials found for Stevens-Johnson syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Stevens-Johnson syndrome community →

Specialists

12 foundView all specialists →
PM
Pinnita Prabhasawat, MD
Specialist
PI on 3 active trials
EM
Elizabeth J Phillips, MD
Specialist
PI on 1 active trial
JM
Joseph Ciolino, MD
BOSTON, MA
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Stevens-Johnson syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Stevens-Johnson syndrome

No recent news articles for Stevens-Johnson syndrome.

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which medication caused my SJS, and how do I make sure I never receive it again?,Should I be tested for HLA gene variants that increase my risk of reactions to other medications?,What long-term complications should I watch for, and how often should I be seen by specialists?,What treatments are you recommending for my case, and why?,How can I protect my eyes, and when should I see an eye specialist?,Are there support groups or counseling services for SJS survivors?,What should I tell other doctors, dentists, or pharmacists about my history of SJS?

Common questions about Stevens-Johnson syndrome

What is Stevens-Johnson syndrome?

Stevens-Johnson syndrome (SJS) is a rare but serious skin and mucous membrane disorder. It is sometimes called SJS for short. The condition causes the skin to blister, peel, and die in large patches, similar to a severe burn. The mucous membranes — the moist linings inside the mouth, eyes, nose, throat, and genitals — are almost always affected too. SJS is considered a medical emergency and requires immediate hospital care. In most cases, SJS is triggered by a reaction to a medication. Common triggers include antibiotics like sulfonamides, anti-seizure drugs such as carbamazepine and lamotrig

How is Stevens-Johnson syndrome inherited?

Stevens-Johnson syndrome follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Stevens-Johnson syndrome?

12 specialists and care centers treating Stevens-Johnson syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.