blisters, severe rash, chest, upper arm
Stevens-Johnson syndrome (SJS)

What is Stevens-Johnson syndrome (SJS)?

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin conditions that cause skin to develop rashes, blisters, and then peel. Mucus membranes, including the eyes, vagina, and mouth, are also affected. People who get this condition are usually admitted to burn units in hospitals.

Some people consider SJS and TEN to be different diseases, while others consider them to be the same disease but in varying degrees. 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 fatal.

Are there other names for Stevens-Johnson syndrome?

Yes. It is also known as Lyell’s syndrome, Stevens-Johnson syndrome/toxic epidermal necrolysis, and Stevens-Johnson syndrome toxic epidermal necrolysis spectrum. Depending on the cause, it might be called drug-induced Stevens-Johnson syndrome or mycoplasma-induced Stevens-Johnson syndrome.

Who gets Stevens-Johnson syndrome?

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 have been reported in females than males. Infections like pneumonia are the most likely cause of SJS in children. People with the following conditions are at greater risk:

  • Bone marrow transplant
  • Systemic lupus erythematosus
  • Human immunodeficiency virus (HIV)
  • Other chronic diseases of joints and connective tissue

What are the risk factors for developing Stevens-Johnson syndrome?

There is most likely a combination of factors involved with developing these disorders, including a genetic predisposition. 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 causes Stevens-Johnson syndrome?

Many cases of SJS are caused by an allergic reaction to a medication (while almost all cases of toxic epidermal necrolysis or TEN involve medications.) However, infections (like mycoplasma pneumonia) can also cause SJS, as can vaccinations, and graft-versus-host disease. In some situations, the syndrome is said to be idiopathic (because no cause is known).

If the skin condition is caused by a drug, symptoms tend to start about one to three weeks after the person has begun taking a medication. The flu-like illness (fever, cough, and headache) 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?

  • Antibacterial sulfa drugs
  • Anti-epileptic drugs, including phenytoin, carbamazepine, lamotrigine, and phenobarbital
  • Allopurinol (used to treat gout and kidney stones)
  • Non-steroidal anti-inflammatory drugs (NSAIDs), including piroxicam, nevirapine, and diclofenac
  • Antibiotics

What are the symptoms of Stevens-Johnson syndrome?

  • Fever
  • Body aches
  • Red rash
  • Cough
  • 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)

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