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Symptoms of Stevens-Johnson Syndrome

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Stevens-Johnson Syndrome is a rare, but serious condition in which the skin and mucous membranes have a severe reaction to a medication or infection. The affected skin develops a rash and blisters, and eventually the top layer of the skin dies and sheds away.

Stevens-Johnson Syndrome usually requires hospitalization, and recovery can take weeks to months depending on the severity of the condition. If the doctor determines that your child’s case of Stevens-Johnson Syndrome was caused by a medication your child will need to permanently avoid that medication and all others related to it.

Medications are the most common cause of Stevens-Johnson syndrome, and the drugs most commonly associated with the disease are:

  • nonsteroidal anti-inflammatory drugs (NSAIDs)
  • sulfonomides and penicillins, which are used to treat infections
  • anticonvulsants, which are used to treat seizures

Less common causes of Stevens-Johnson syndrome are other infections, such as:

  • Herpes (herpes simplex or herpes zoster)
  • Influenza
  • HIV
  • Diptheria
  • Typhoid
  • Hepatitis

Stevens-Johnson Syndrome can also cause other complications, putting the patient’s overall health at risk. Possible complications include:

  • Secondary skin infection ¬– skin infections such as cellulitis can lead to life-threatening complications like meningitis – an infection of the membrane and fluid surrounding the brain and spinal cord – and sepsis.
  • Sepsis – sepsis occurs when infection enters the bloodstream and spreads throughout the body. Sepsis spreads rapidly and can cause shock, organ failure and even death.
  • Eye problems – the rash caused by Stevens-Johnson syndrome can lead to inflammation of the eyes. In mild cases this can lead to dry eyes and minor irritation. In severe cases it can lead to extensive tissue damage and scarring within the eyes that can lead to blindness.
  • Damage to internal organs – Stevens-Johnson Syndrome can cause lesions, or sores, to develop on the internal organs, resulting in pneumonia, or damage to the heart, kidneys and liver.
  • Permanent skin damage – when the skin grows back following infection with Stevens-Johnson it may have unusual bumps and coloring, as well as lasting scars. In addition, patients may have lasting skin problems that cause their hair to fall out, and the fingernails and toenails to grow abnormally.

Symptoms of Stevens-Johnson Syndrome

Stevens-Johnson Syndrome often begins with flu-like symptoms, before transitioning to a red or purplish rash that spreads. Early symptoms include:

  • fever
  • sore throat
  • cough
  • burning eyes

Several days after onset of the disease you may notice other signs and symptoms, including:

  • facial swelling
  • tongue swelling
  • hives
  • skin pain
  • a red or purple rash that spreads within hours to days
  • blisters on the skin and mucous membranes, especially the mouth, nose and eyes
  • sloughing or shedding of the skin

Diagnosing Stevens-Johnson Syndrome

Stevens-Johnson Syndrome can be diagnosed through a combination of a physical examination, medical history and the disease’s unique signs and symptoms. Your doctor may also take a biopsy, or tissue sample, of your skin to examine under a microscope.

Treatment for Stevens-Johnson Syndrome

Shriners Hospitals for Children® offers treatment for Stevens-Johnson syndrome, but the first line of defense is to stop taking any medications that may be causing it. In fact, your child’s doctor may recommend that you stop taking all non-essential medications.

If your child is hospitalized for Stevens-Johnson syndrome treatment may include:

  • Fluid replacement through a tube placed in the nose
  • Cool, wet compresses to help soothe blisters while they heal
  • Removal of dead skin, followed by a dressing with a topical anesthetic
  • pain medication to reduce discomfort
  • antihistamines to reduce itching
  • antibiotics to control infection
  • Topical steroids to reduce skin inflammation

If a large part of the body is infected skin grafting may be required to help the skin heal. This is a process in which skin from one area of the body is removed and re-attached in another location on the body.

If the cause of Stevens-Johnson can be eliminated and the skin reaction stopped, skin may begin to grow again within several days. In severe cases of the disease recovery may take several months.