What is Stevens-Johnson syndrome (SJS)?
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin conditions that cause your skin to develop rashes, blisters, and then peel. Your mucus membranes, including your eyes, genitalia and mouth, are also affected. If you get this condition, you’ll likely be admitted to a hospital.
Some people consider SJS and TEN to be different diseases, while others consider them to be the same disease but at different levels of severity. SJS is less intense than TEN. (For instance, skin peeling might affect less than 10% of the entire body in SJS, while skin peeling affects more than 30% of the body in TEN.) However, both conditions can be life-threatening.
Are there other names for Stevens-Johnson syndrome (SJS)?
Yes. It is also known as Lyell’s syndrome, Stevens-Johnson syndrome/toxic epidermal necrolysis, and Stevens-Johnson syndrome toxic epidermal necrolysis spectrum. It might be called drug-induced Stevens-Johnson syndrome or mycoplasma-induced Stevens-Johnson syndrome if it’s linked to a specific cause.
Who gets Stevens-Johnson syndrome (SJS)?
Many cases of SJS happen in children and adults younger than 30 years old, but also occur in others, especially the elderly. More cases of SJS occur in females than males. Infections, like pneumonia, are the most likely cause of SJS in children, whereas medications are the most likely cause of SJS/TEN in adults.
What are the risk factors for developing Stevens-Johnson syndrome?
A combination of factors is likely involved in developing these disorders, including a genetic bias. Environmental factors might cause the gene to be triggered. One of these genetic factors include specific human leukocyte antigens (HLAs) that may increase one’s risk of developing SJS or TEN.
What are the symptoms of Stevens-Johnson syndrome (SJS)?
Symptoms of Stevens-Johnson syndrome include:
- Skin pain.
- Body aches.
- Red rash or red blotches on your skin.
- Blisters and sores on the skin and on mucus membranes of the mouth, throat, eyes, genitals and anus.
- Peeling skin.
- Drooling (because closing the mouth is painful).
- Eyes sealed shut (due to blisters and swelling).
- Painful urination (due to blistered mucus membranes).
What causes Stevens-Johnson syndrome (SJS)?
Causes of Stevens-Johnson syndrome include:
- Allergic reaction to a medication (most cases of SJS and almost all cases of TEN).
- Infections, like mycoplasma pneumonia, herpes and hepatitis A.
- Graft-versus-host disease.
- No known cause.
If your case of SJS is caused by a drug, symptoms appear about one to three weeks after you start taking medication. The flu-like illness (fever, cough and headache, skin pain) is followed first by a rash and then peeling. In the case of TEN, some people even lose hair and nails.
What drugs are most likely to cause Stevens-Johnson syndrome?
Medications most likely to cause Stevens-Johnson syndrome include:
- Antibacterial sulfa drugs.
- Anti-epileptic drugs, including phenytoin (Dilantin®), carbamazepine (Tegretol®), lamotrigine (Lamictal®), and phenobarbital (Luminal®).
- Allopurinol (Aloprim®, Zyloprim®), a drug used to treat gout and kidney stones.
- Non-steroidal anti-inflammatory drugs (NSAIDs), including piroxicam (Feldene®), nevirapine (Viramune®), and diclofenac (Cambia®, Flector®).
Are there other factors that increase the risk of someone developing Stevens-Johnson syndrome (SJS)?
You are at greater risk of SJS if you have the following conditions:
- Bone marrow transplant.
- Systemic lupus erythematosus.
- Human immunodeficiency virus (HIV).
- Other chronic diseases of joints and connective tissue.
- Weakened immune system.
- Family history of SJS.
- Variation of a specific gene called human leukocyte antigen-B.