Stevens-Johnson syndrome

Description

Stevens-Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It is usually a reaction to the medication that begins with flu-like symptoms, followed by a painful rash that spreads and blisters. Then the top layer of the affected skin dies, sheds and begins to heal after several days.

Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization. The treatment focuses on the removal of the cause, the care of wounds, controlling the pain and minimizing complications as your skin regrows. It can take weeks or months to recover.

A more severe form of the condition is called toxic epidermal necrolysis (TEN). Includes more than 30% of the surface of the skin and extensive damage tor mucous membranes.

If your condition was caused by a medication, you'll need to permanently avoid that drug and others like it.

Symptoms

One-to-three days before a rash, which could show the first signs of Stevens-Johnson syndrome, including:

  • Fever
  • A pain in the mouth and throat
  • Fatigue
  • Burning eyes

As the condition develops, other signs and symptoms include:

  • Unexplained widespread pain of the skin
  • A red or purple skin rash that spreads
  • Blisters on the skin and the mucous membranes of the mouth, nose, eyes and genitals
  • The detachment of the skin in a matter of days after blisters form

When to see a doctor

Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical attention if you experience signs and symptoms of this condition. Drug-induced reactions may occur during the use of a drug or up to two weeks after stopping the same.

Causes

Stevens-Johnson syndrome is a rare and unpredictable disease. Your health care provider may not be able to identify its exact cause, but usually the condition is caused by the mediction, infection, or both. You can react to the medication, while you're using it, or up to two weeks after you have stopped using it.

Drugs that can cause Stevens-Johnson syndrome include:

  • Anti-gout medications, such as allopurinol
  • Medications to treat seizures and mental illness (antiepileptics and antipsychotics)
  • Antibacterial sulfonamides (including sulfasalazine)
  • Nevirapine (Viramune, Viramune XR)
  • Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodi -um (Aleve)

Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.

Risk factors

Factors that increase your risk of developing Stevens-Johnson syndrome include:

  • An HIV infection. Among people with HIV, the incidence of Stevens-Johnson syndrome, is approximately 100-fold greater than among the general population.
  • A weakened immune system. The immune system can be affected by an organ transplant, HIV/ Acquired immunodeficiency syndrome (AID'S), and autoimmune diseases.
  • Cancer. People with cancer, especially cancer of the blood, are at greater risk of Stevens-Johnson syndrome.
  • A history of Stevens-Johnson syndrome. If you have had a medication-related form of this condition, you are at risk of a recurrence if the drug is used again.
  • A family history of Stevens-Johnson syndrome. If an immediate blood relative has had Stevens-Johnson syndrome, you may be at greater risk of suffering from it as well.
  • Genetic factors. Have certain genetic variations, puts on at higher risk of Stevens-Johnson syndrome, especially if you are also taking medicines for seizures, gout, or mental illness.

Complications

Stevens-Johnson syndrome complications include:

  • The dehydration. In areas where the skin has been stripped of losing liquid. And sores in the mouth and throat can make eating difficult liquids, resulting in dehydration.
  • Blood infection (sepsis). Sepsis occurs when bacteria from an infection entering the bloodstream and spread throughout the body. Sepsis is a rapidly progressing, life-threatening condition that can cause shock and multiple organ failure.
  • Eye problems. The rash caused by Stevens-Johnson syndrome can lead to inflammation of the eyes, dryness of the eyes and sensitivity to light. In severe cases, can lead to visual impairment and, rarely, blindness.
  • Lung involvement. The condition may lead to an emergency situation in which the lungs cannot get enough oxygen in the blood (acute respiratory failure).
  • Permanent skin damage. When your skin becomes following Stevens-Johnson syndrome, can have unusual lumps and coloring (dyspigmentation). And you may have scars. Lasting skin problems can cause your hair to fall out, and the nails of the hands and feet may not grow as well as they did before.

Prevention

  • Consider genetic testing before taking certain medications. The Food and Drug Administration recommends screening for people of Asian and South Asian descent to gene variation called HLA-B*1502 prior to initiating treatment.
  • If you have had this condition, avoid the medication that triggered it.If you have had Steven's-Johnson syndrome (SJS) and your health care provider told you that you was caused by a medication, avoid that drug and others like it. This is the key to the prevention of the recurrence, which is usually more severe than the first episode and can be fatal. Their immediate blood relatives may also want to avoid this drug because sometimes this condition runs in families.

If you have had this condition, avoid the medication that triggered it. If you have had Stevens-Johnson syndrome (SJS), and his doctor said that it was caused by a medication, avoid that drug and others like it. This is the key to the prevention of the recurrence, which is usually more severe than the first episode and can be fatal.

Their immediate blood relatives may also want to avoid this drug because sometime this condition runs in families.

Stevens-Johnson syndrome

Diagnosis

Tests and procedures used to diagnose Stevens-Johnson syndrome include:

  • A review of your medical history and a physical examination. Health care providers can often be identified Stevens-Johnson syndrome is based on your medical history, including a review of their current and recently stopped medications, and physical examination.
  • Skin biopsy. To confirm the diagnosis and rule out other possible causes, your health care provider removes a saplug of the skin for laboratory testing (biopsy).
  • The culture. To rule out an infection, the doctor will take a sample of skin, tissue or fluid for laboratory testing (culture).
  • The projection image. Depending on your symptoms, your doctor may have you undergo an image as a chest x-ray to check for pneumonia.
  • Blood tests. These are used to confirm the infection or other possible causes.

Treatment

The treatment of Stevens-Johnson syndrome require hospitalization, possibly intensive care unit or burn unit.

Stop drugs

The first and most important step in the treatment of Stevens-Johnson syndrome is to stop taking any medicine that may be causing it. If you are taking more than one medication, it can be difficult to say which drug is causing the problem. So your doctor may ask you to stop taking all medications.

Supportive care

Supportive care is likely that you receive while in the hospital includes:

  • Fluid replacement and nutrition. Because the skin of the loss can result in a majort loss of fluid from your body, to replace the fluids is an important part of the treatment. You may receive fluids and nutrients through a tube inserted in the nose and guide into the stomach (nasogastric tube).
  • Wound care. Cool, wet compresses may help soothe blisters while they heal. Your health care team can gently remove the dead skin and put vaseline (petroleum Jelly) or a medicated dressing to the affected areas.
  • Care of the eyes. You may also need the attention of an eye specialist (ophthalmologist).

Drugs

Drug used in the treatment of Stevens-Johnson syndrome include:

  • Pain medication to reduce discomfort.
  • Medications to reduce the inflammation of the eyes and mucous membranes (topical steroids).
  • Antibiotics to control the infection, when necessary.
  • Other oral or injected (systemic) medications, such as corticosteroids and intravenous immunoglobulin. The studies show that the drug cyclosporine (Neoral, Sandimmune), and etanercept (Enbrel) are useful in the treatment of this disease.

If the underlying cause of the Stevens-Johnson syndrome can be removed, and the reaction of the skin was arrested, again in the skin may start to grow inside of several days. In severe cases, complete recovery may take several months.

Self-care

If you have had Stevens-Johnson syndrome, be sure to:

  • To know what is the cause of your reaction. If hisextradition was caused by a medication, learn his name and the others like him. Avoid them.
  • To inform their health care providers. Tell all of your health care providers that you have a history of Stevens-Johnson syndrome. If the reaction was caused by a medication, tell that one.
  • Wear a medical information bracelet or necklace. Have information about your condition and what caused it to be enrolled in a medical information bracelet or necklace. Always use.

Preparing for your appointment

Stevens-Johnson syndrome is an emergency medical condition. If you have signs and symptoms, call 911 or emergency medical help, or go to an emergency room immediately.

If you have time before you go:

  • Put in a bag of all the medications that I have taken in the last three weeks, including prescription and over-the-counter without a prescription. Take the bag with you, because it can help your doctor to find out what triggered his condition.
  • Ask a family member or a friend to accompany you. You may want to share relevant health information about yourself with your partner, so that this person can help you when you talk with your health care provider.

Questions to your health care provider can do include:

  • Has had a disease similar to the flu recently?
  • What other medical conditions do you have?
  • What medications you have taken in the last three weeks?

While you're in the hospital, it is likely that you will have questions for your doctor. This may help to keep a list of questions that you have, such as:

  • What caused my condition?
  • How can I avoid having this reaction again?
  • What restrictions I need to follow?
  • I have other medical conditions. How can I manage together?
  • How long will it take for my skin to heal?
  • I am likely to have permanent damage?
Symptoms and treatment of Stevens-Johnson syndrome