What is Stevens Johnson Syndrome?

Stevens Johnson syndrome is a rare but serious disease that affects the skin, mucous membranes, genitals and eyes and should be treated promptly. You can find more information below.

What is Stevens Johnson syndrome?

Stevens Johnson syndrome is a rare, serious disease of the skin and mucous membranes. It is a painful rash that begins with flu-like symptoms and spreads as blisters on the affected skin, following a reaction to medication. Then the upper layer of the affected skin dies, falls off and begins to heal after a few days.

Stevens Johnson syndrome is a medical emergency that usually requires hospitalization. Treatment focuses on eliminating the cause, healing wounds, controlling pain, and minimizing complications as the skin rebounds. It can take weeks to months to heal.

A more serious form of the condition is called toxic epidermal necrolysis (TEN) . This condition includes more than 30% of the skin surface and extensive damage to the mucous membranes.

If this syndrome is caused by a drug, it is necessary to permanently prevent the side effects of this drug and closely related drugs under the supervision of a doctor.

What causes Stevens Johnson syndrome?

Stevens Johnson syndrome is a rare and unpredictable disease. Doctors may not be able to identify the exact cause, but often the condition is triggered by medication, infection, or both. Affected individuals may react while using drugs or up to two weeks after they stop using them.

Medications that can cause Stevens Johnson syndrome include:

  • anti-gout medications such as allopurinol
  • Medicines to treat seizures and mental illness (anticonvulsants and antipsychotics)
  • Antibacterial sulfonamides (including sulfasalazine)
  • Nevirapin (Viramune, Viramune XR)
    • Pain relievers such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others), and naproxen sodium (Aleve)
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Infections that can cause this syndrome include pneumonia and HIV/AIDS .

Who is at risk?

Factors that increase the risk of developing Stevens Johnson syndrome include:

  • HIV infection: People with HIV are about a hundred times more likely to develop this syndrome than normal people.
  • A weakened immune system: The immune system can be affected by organ transplants, HIV/AIDS, and autoimmune diseases .
  • Cancer: People with cancer, especially those with blood cancer, are at increased risk for Stevens Johnson syndrome.
  • History of Stevens Johnson syndrome: If the patient has a drug-related form of this condition, there is a risk of recurrence of the disease if he uses that drug again.
  • Family history of Stevens Johnson syndrome: If a family member has Stevens Johnson syndrome, other family members are also at risk.
  • Genetic factors Having certain genetic variations, especially taking medication for seizures, gout, or mental illness, increases the risk.

What are the symptoms of Stevens Johnson syndrome?

Early signs of Stevens-Johnson syndrome may appear one to three days before a rash develops. Among them are the following:

  • Fire
  • sore mouth
  • Tiredness
  • eye burn

As the syndrome develops, other symptoms include:

  • Unexplained widespread skin pain
  • a red or purplish rash that spreads
  • blisters on the skin
  • Shedding of the skin within days after the blisters form

When should you see a doctor?

Stevens Johnson syndrome requires immediate medical attention. If symptoms of this condition are experienced, seek immediate medical attention. Drug-related reactions may occur during the use of a drug or two weeks after the drug is discontinued.

How is Steven Johnson syndrome diagnosed?

Tests and procedures used to diagnose Stevens Johnson syndrome include:

  • Medical history review and physical exam: Doctors can identify this disease based on medical history, including a review of current and recently discontinued medications, and a physical exam.
  • Skin biopsy: To confirm the diagnosis and rule out other possible causes, the doctor takes a skin sample for laboratory testing (biopsy).
  • Culture: Cultures may be taken from the skin or other sites to confirm or exclude infection.
  • Imaging: Depending on the symptoms, the doctor may order an imaging test such as a chest X-ray to check for pneumonia .
  • Blood tests: These are used to confirm infection or other possible causes.
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How is Stevens Johnson syndrome treated?

Treating Stevens Johnson syndrome will likely require hospitalization in the intensive care unit or burn unit.

Stopping unnecessary medications

The first and most important step in treating this syndrome is to stop taking any medication that may be causing it. Since it is difficult to determine exactly which drug may be causing the problem, the doctor may advise the patient to stop taking all unnecessary drugs.

Supportive care

Supportive care to be received while in hospital includes:

  • Fluid replacement and nutrition: Fluid replacement is an important part of treatment, as skin loss can result in significant fluid loss from the body. Fluids and nutrients can be taken through a tube (nasogastric tube) inserted into the nose and directed to the stomach.
  • Wound care: Cool, wet compresses help blisters heal. The healthcare team may gently remove the dead skin and put petroleum jelly (Vaseline) or a medicated dressing on the affected areas.
  • Eye care: Care from an eye specialist (ophthalmologist) may also be needed.

Medication

Medications used to treat Stevens Johnson syndrome include:

  • pain medication to reduce discomfort
  • medicine (topical steroids) to reduce inflammation of the eyes and mucous membranes
  • Antibiotics to control infection as needed
  • Depending on the severity, other systemic medications, including oral corticosteroids and intravenous immune globulin, although the value of their use is controversial.

Remember, your doctor will decide which medicine to take and how.

If the underlying cause of Stevens Johnson syndrome can be eliminated and the skin reaction stopped, new skin can begin to develop within a few days. In severe cases, full recovery may take several months.

Lifestyle and home remedies

If a person has Steven Johnson syndrome, they should make sure they:

  • Knowing what is causing the reaction: If the condition is caused by a drug, its name and closely related drugs should be learned.
  • Informing healthcare professionals: The patient should tell all healthcare professionals that he or she has such a syndrome. If the reaction was caused by a drug, that drug should also be specifically reported.
  • Wearing a medical information bracelet or necklace: In case of emergency, an information card can be carried to inform the relevant persons.
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Can Stevens Johnson syndrome be prevented?

In general, the following can be done to prevent Stevens Johnson syndrome:

  • Consider genetic testing before taking certain medications: The U.S. Food and Drug Administration recommends screening people of Asian and South Asian descent for a gene variation called HLA-B*1502 before starting treatment.
  • If there is this condition, avoiding the drugs that trigger it: In Stevens-Johnson syndrome, if the doctor said that it was caused by a drug, using this drug and other drugs like it should be avoided. This is key to preventing a relapse, which can often be more severe and fatal than the initial relapse.

Stevens Johnson syndrome complications

Possible complications of Stevens Johnson syndrome include:

  • Dehydration: The areas where the skin is shed lose fluid. Sores in the mouth and throat can make fluid intake difficult and cause dehydration.
  • Blood infection (sepsis): Sepsis occurs when bacteria from an infection enter the bloodstream and spread throughout the body. Sepsis is a rapidly progressive, life-threatening condition that can cause shock and organ failure.
  • Eye problems: The rash caused by this syndrome can cause eye inflammation, dry eyes , and sensitivity to light. In severe cases, it can cause visual impairment and rarely blindness.
  • Pulmonary involvement Steven Johnson syndrome can lead to acute respiratory failure.
  • Permanent skin damage: When the skin regrows after Stevens Johnson syndrome, there may be abnormal swelling, discoloration, and scarring. Persistent skin problems can cause hair loss and nails may not grow normally.

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