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Stevens-Johnson Syndrome

Definition :
Stevens-Johnson syndrome — also called erythema multiforme major — is a rare, serious disorder of the skin and mucous membranes. Often, Stevens-Johnson syndrome begins with several days of flu-like symptoms, followed by inflammation of your mucous membranes and a painful red or purplish rash that spreads and blisters, eventually causing the top layer of your skin to die and shed.

Although the cause isn't always clear, Stevens-Johnson syndrome usually is a specific type of allergic reaction in response to medication or infection. An emergency medical condition, Stevens-Johnson syndrome requires hospitalization. Treatment focuses on eliminating the underlying cause, if possible, controlling symptoms and minimizing complications. Recovery after Stevens-Johnson syndrome can take several weeks to several months, depending on the severity of your condition.

If your doctor determines that your case of Stevens-Johnson syndrome was caused by medication, be sure to avoid that medication and all others related to it that may cause a similar reaction, because recurrences of Stevens-Johnson syndrome can be fatal.

Causes :
The exact cause of Stevens-Johnson syndrome can't always be identified. Usually, the condition is an allergic reaction in response to medication, infection or illness.

Medications are most often the cause of Stevens-Johnson syndrome. Drugs commonly associated with Stevens-Johnson syndrome include :

  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Sulfonamides and penicillins, which are used to treat infections
  • Anticonvulsants, which are used to fight seizures

Infections that can cause Stevens-Johnson syndrome include :

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

In some cases, Stevens-Johnson syndrome may be caused by physical stimuli, such as radiation therapy or ultraviolet light.

Risk Factor :
Stevens-Johnson syndrome is a rare and unpredictable reaction. No test is available to help predict who is at greater risk. Some factors, however, may increase your risk of developing Stevens-Johnson syndrome :

  • Existing medical conditions. Viral infections, diseases that decrease your immunity, human immunodeficiency virus (HIV), and systemic lupus erythematosus — a chronic inflammatory disease — increase your risk of developing Stevens-Johnson syndrome.
  • Genetics. Carrying a gene called HLA-B12 may make you more susceptible to Stevens-Johnson syndrome.

When to seek medical advice :
Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience any of the following signs or Symptoms:

  • Skin pain
  • Facial swelling
  • Blisters on your skin and mucous membranes
  • Hives
  • Tongue swelling
  • A red or purple skin rash that spreads
  • Shedding of your skin

Symptoms :
Signs and symptoms of Stevens-Johnson syndrome include :

  • Facial swelling
  • Tongue swelling
  • Hives
  • Skin pain
  • A red or purple skin rash that spreads
  • Blisters on your skin and mucous membranes, especially in your mouth, nose and eyes
  • Shedding (sloughing) of your skin

If you have Stevens-Johnson syndrome, several days before the rash develops, you may experience :

  • Fever
  • Sore throat
  • Cough
  • Burning eyes

Diagnosis :
Doctors often can identify Stevens-Johnson syndrome based on your medical history, a physical exam and the disorder's distinctive signs and symptoms. To confirm the diagnosis, your doctor may take a tissue sample of your skin (biopsy) for examination under a microscope.

Complications :
Possible complications of Stevens-Johnson syndrome include :

  • Secondary skin infection (cellulitis). This acute infection of your skin can lead to life-threatening complications, including meningitis — an infection of the membrane and fluid surrounding your brain and spinal cord — and sepsis.
  • Sepsis. Sepsis occurs when bacteria from a massive infection enter your bloodstream and spread throughout your body. Sepsis is a rapidly progressing, life-threatening condition that can cause shock and organ failure.Bottom of Form
  • Eye problems. The rash caused by Stevens-Johnson syndrome can lead to inflammation in your eyes. In mild cases, this may cause irritation and dry eyes. In severe cases, it can lead to extensive tissue damage and scarring within your eyes that can result in blindness.
  • Damage to internal organs. Stevens-Johnson syndrome can cause lesions of your internal organs, which can result in inflammation of your lungs (pneumonitis), heart (myocarditis), kidney (nephritis) and liver (hepatitis).
  • Permanent skin damage. When your skin grows back following Stevens-Johnson syndrome, it may have abnormal bumps and coloring (pigmentation). Scars may remain on your skin, as well. Lasting skin problems may cause your hair to fall out, and your fingernails and toenails may not grow normally.
Stevens-Johnson syndrome requires hospitalization, often in an intensive care unit or burn unit.

The first step in treating Stevens-Johnson syndrome is to discontinue any medications that may be causing it. Because it's difficult to determine exactly which drug may be causing the problem, your doctor may recommend that you stop taking all nonessential medications.

Medications you may receive while you're hospitalized include :

  • Pain medication to reduce discomfort
  • Antibiotics to control infection, when needed
  • In severe cases, immunoglobulin intravenous (IGIV) to halt the process of Stevens-Johnson syndrome

Because skin loss can result in significant loss of fluid from your body, replacing fluids is an important part of treatment. You may receive fluids and nutrients through a tube placed through your nose and into your stomach (nasogastric tube). Blisters that are still intact are generally left to heal on their own. Your health care team may gently remove any dead skin, and then place a dressing with a topical anesthetic over the affected areas, if needed.

If a large area of your body is affected, skin grafting — removing skin from one area of your body and attaching it to another — may be necessary to help you heal. This treatment is only rarely required.

If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, your skin may begin to grow again within several days. In severe cases, full recovery may take several months. When Stevens-Johnson syndrome is diagnosed within 24 to 48 hours of onset, prompt treatment may shorten the duration of the illness.

Prevention :
It's difficult to prevent an initial attack of Stevens-Johnson syndrome because you don't know what will trigger it. However, if you had Stevens-Johnson syndrome once, and your doctor determined that it was caused by medication, be sure to avoid that medication and others in the same class to prevent another attack. If the herpes virus caused your reaction, you may need to take daily antiviral medications to prevent a recurrence.

A recurrence of Stevens-Johnson syndrome is usually more severe than the first episode and, in many cases, it can be fatal.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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