Stevens Johnson Syndrome a Rare Skin Disorder

posted on:
January 30, 2008

author:
Staff

Stevens-Johnson syndrome is a rare, serious disorder of the skin and mucous membranes. It 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. 

My interest in writing this article is my own experience with Stevens Johnson syndrome. It is something I will live with forever.

That is not a fancy bracelet you see me wear on my right wrist. Look closer next time you see me. It is a medical bracelet. So many people have asked me what Steven Johnson syndrome is and why I was gone last year for five months. I was recovering from a near death experience of Stevens Johnson syndrome.

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

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

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

* Nonsteroidal anti-inflammatory drugs (NSAIDs)

* Sulfonamides and penicillin, which are used to treat infections

* Anticonvulsants, which are used to fight seizures

Doctors can recognize Stevens-Johnson syndrome from your medical history. As well as a physical exam and the disorder’s distinctive signs and symptoms a doctor will also be able to pin point this syndrome. To substantiate 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 can lead to life-threatening complications, including meningitis, an infection of the membrane and fluid surrounding your brain and spinal cord, and sepsis.

* Sepsis. This 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.

* Eye problems. The rash caused by Stevens-Johnson 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.

Treatment

Stevens-Johnson syndrome requires hospitalization, often in an intensive care unit or burn unit.

The first step in treating Stevens-Johnson 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 because you don’t know what will trigger it. However, if you had Stevens-Johnson once, and your doctor determined that it was caused by medication, be sure to avoid that medication and others in the same class. 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.

Self-care

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

* Know what caused your reaction. If your case of Stevens-Johnson syndrome was caused by a medication, learn the name of that medication and any other closely related medications that may cause the same reaction.

* Inform your health care providers. Tell all your health care providers that you have a history of Stevens-Johnson syndrome. If the reaction was caused by medication, provide your caregivers with the name of that medication.

* Wear a medical information bracelet or necklace. Have information about your condition and what caused it inscribed on a medical information bracelet or necklace, and wear it at all times.

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