Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:506784L51.2
Who is this for?
Show terms as
1Specialists8Treatment centers

Where are you in your journey?

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

Overview

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome (SJS/TEN overlap) is a rare and serious skin condition where the body's immune system attacks the skin and mucous membranes. It sits between two related conditions: Stevens-Johnson syndrome (SJS), which is less severe, and toxic epidermal necrolysis (TEN), which is the most severe form. In SJS/TEN overlap, between 10% and 30% of the body's skin surface blisters and peels away, leaving raw, painful areas similar to severe burns. The condition also affects the moist linings of the body, including the eyes, mouth, throat, and genitals. The condition is almost always triggered by a medication — most commonly antibiotics like sulfonamides, anti-seizure drugs like carbamazepine and lamotrigine, allopurinol (used for gout), and certain HIV medications. Infections, particularly Mycoplasma pneumoniae, can also trigger it, especially in children. Symptoms usually begin within one to four weeks of starting a new medication and include flu-like feelings, a painful rash that spreads quickly, and blistering of the skin and mouth. Treatment requires urgent hospitalization, ideally in a specialized burn unit or intensive care unit. The triggering medication must be stopped immediately. Supportive care — including wound care, fluids, nutrition, and pain management — is the cornerstone of treatment. Some hospitals use immune-based treatments like intravenous immunoglobulin (IVIG) or cyclosporine, though evidence is still evolving. Early recognition and stopping the offending drug are the most important steps to improve survival.

Also known as:

Key symptoms:

Painful, spreading skin rash that starts on the face and chestBlisters forming on the skin that burst and leave raw, open areasSkin peeling off in sheetsSores and blisters inside the mouth, making eating and swallowing very painfulRed, painful, or swollen eyes — sometimes with dischargeFever and flu-like symptoms before the rash appearsBurning or stinging sensation on the skinBlisters or sores on the genitals or anusDifficulty swallowing or breathing if the throat is affectedSensitivity to lightFatigue and feeling very unwellSkin that looks like it has been scalded or burned

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome.

View clinical trials →

No actively recruiting trials found for Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome community →

Specialists

1 foundView all specialists →

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/toxic epidermal necrolysis overlap syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndromeForum →

No community posts yet. Be the first to share your experience with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome.

Start the conversation →

Latest news about Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

No recent news articles for Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome.

Follow this condition to be notified when news becomes available.

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 reaction, and how do I make sure I never take it or anything similar again?,Should I have genetic testing to find out if I am at higher risk from other medications?,What long-term complications should I watch for, and which specialists should I see regularly?,What treatments are you recommending for me, and what is the evidence behind them?,How do I protect my eyes, and when should I see an ophthalmologist?,Are there support groups or counseling services for SJS/TEN survivors?,What should I tell future doctors and pharmacists about my history to keep me safe?

Common questions about Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

What is Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome?

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome (SJS/TEN overlap) is a rare and serious skin condition where the body's immune system attacks the skin and mucous membranes. It sits between two related conditions: Stevens-Johnson syndrome (SJS), which is less severe, and toxic epidermal necrolysis (TEN), which is the most severe form. In SJS/TEN overlap, between 10% and 30% of the body's skin surface blisters and peels away, leaving raw, painful areas similar to severe burns. The condition also affects the moist linings of the body, including the eyes, mouth, throat, and ge

How is Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome inherited?

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

Which specialists treat Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome?

1 specialists and care centers treating Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.