Disease at a Glance

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.
Estimated Number of People with this Disease
In the U.S., this disease is estimated to be fewer than

5,000

What Information Does GARD Have For This Disease?

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

*Data may be currently unavailable to GARD at this time.
Categories
When do symptoms of this disease begin?
The most common ages for symptoms of a disease to begin is called age of onset. Age of onset can vary for different diseases and may be used by a doctor to determine the diagnosis. For some diseases, symptoms may begin in a single age range or several age ranges. For other diseases, symptoms may begin any time during a person's life.
Prenatal Selected
Before Birth
Newborn Selected
Birth-4 weeks
Infant Selected
1-23 months
Child Selected
2-11 years
Adolescent Selected
12-18 years
Adult Selected
19-65 years
Older Adult Selected
65+ years
The common ages for symptoms to begin in this disease are shown above by the colored icon(s).

Symptoms

These symptoms may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom.
This disease might cause these symptoms:
Skin System

37 Symptoms

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Skin System

The skin or integumentary system is made up of skin, hair, nails, sweat glands, and oil glands. Common symptoms of problems in the skin system include redness, swelling, burning, itching, rashes, and hives. Skin diseases are often diagnosed and treated by dermatologists. Other specialists may also be involved including rheumatologists, allergists, and infectious disease doctors.

Causes

This section is currently in development. 

Last Updated: Nov. 8, 2021