Overview
Erythema multiforme major (also sometimes called Stevens-Johnson syndrome overlap or severe erythema multiforme) is a serious skin and mucous membrane condition where the immune system reacts strongly, usually triggered by an infection or a medication. The name 'erythema multiforme' means 'redness in many forms,' which describes the variety of skin changes that appear. In the major form, the reaction is more severe than the mild version and affects not just the skin but also the moist linings of the body — including the mouth, eyes, and genitals. The condition causes painful, target-shaped or bull's-eye-looking sores on the skin, along with blistering and raw, open areas on the lips, inside the mouth, and sometimes the eyes. These sores can make eating, drinking, and seeing very difficult. The most common triggers are the herpes simplex virus (the virus that causes cold sores) and certain medications such as antibiotics or anti-seizure drugs. Treatment focuses on finding and stopping the trigger, managing pain, protecting the skin, and keeping the eyes and mouth moist and free from infection. Most people recover fully, but the condition can come back, especially if the herpes virus is the cause. In severe cases, hospitalization may be needed. With proper care, the outlook is generally good, though complications involving the eyes can sometimes cause lasting problems.
Key symptoms:
Target-shaped or bull's-eye skin sores, often on the hands, feet, and armsPainful blisters or raw areas on the lips and inside the mouthSores or redness in the eyes, causing pain and sensitivity to lightSores on the genitals or around the anusFever and feeling generally unwell before or during the rashSkin peeling or crusting around the mouthDifficulty eating or swallowing due to mouth soresSwollen or crusted eyelidsWidespread redness and tenderness of the skinBurning or itching sensation on the skin
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
5 eventsMercator MedSystems, Inc. — PHASE3
Assistance Publique - Hôpitaux de Paris — PHASE3
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Erythema multiforme major.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Erythema multiforme major at this time.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Erythema multiforme major.
Community
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Start the conversation →Latest news about Erythema multiforme major
Disease timeline:
New recruiting trial: Temsirolimus Adventitial Delivery to Improve ANGioplasty and/or Atherectomy Revascularization Outcomes Below the Knee
A new clinical trial is recruiting patients for Erythema multiforme major
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.What triggered my episode — was it an infection, a medication, or something else?,Do I need to avoid any specific medications permanently because of this reaction?,Should I take daily antiviral medication to prevent future episodes?,How do I protect my eyes, and when should I see an eye specialist?,What are the warning signs that I need to go to the emergency room?,How likely is it that this will come back, and what can I do to reduce that risk?,Are there any genetic tests I should have before taking certain medications in the future?
Common questions about Erythema multiforme major
What is Erythema multiforme major?
Erythema multiforme major (also sometimes called Stevens-Johnson syndrome overlap or severe erythema multiforme) is a serious skin and mucous membrane condition where the immune system reacts strongly, usually triggered by an infection or a medication. The name 'erythema multiforme' means 'redness in many forms,' which describes the variety of skin changes that appear. In the major form, the reaction is more severe than the mild version and affects not just the skin but also the moist linings of the body — including the mouth, eyes, and genitals. The condition causes painful, target-shaped or
How is Erythema multiforme major inherited?
Erythema multiforme major follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Erythema multiforme major?
18 specialists and care centers treating Erythema multiforme major are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.