Surviving Stevens-Johnson Syndrome

You have a rash across most of your skin that is starting to blister. You feel feverish and your eyes are burning. You remember that you started a new prescription medicine. You could have Stevens-Johnson syndrome.

Stevens-Johnson syndrome is a rare, yet serious condition in which the skin and mucous membranes adversely react to a medication, illness, or infection.

In some cases, the cause cannot be identified. It can be a medical emergency that might require hospitalization. Recovery can take weeks or months depending on the severity.

Sore throat, burning eyes, fever and cough may begin several days before the skin manifestations. Hives, skin pain, facial and/or tongue swelling, sloughing (shedding) of the skin, blisters on the skin and mucous membranes (especially the eyes, nose, and mouth), and a red or purple skin rash that spreads within hours or days may occur.

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.

The illness has several 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, serious condition that can cause shock and organ failure.

·       Eye problems. The rash caused by Stevens-Johnson can lead to swelling in your eyes. In mild cases, this may cause irritation and dry eyes. In severe cases, it can lead to substantial 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 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 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 excluded and the skin reaction stopped, your skin may begin to grow again within several days. In severe cases, full recovery might take several months. When Stevens-Johnson syndrome is diagnosed within 24 to 48 hours of onset, prompt treatment may reduce the span of the illness.

It’s difficult to prevent an initial attack of Stevens-Johnson because you have no idea what will trigger it. However, if you have had Stevens-Johnson already, 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 can be more severe than the first episode, and in many cases, it can be fatal.

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

·       Know what caused your reaction. For example, if your reaction was caused by a medication, be certain to learn the name of that medication and any other closely related medications that may cause similar reactions.

·       Inform your health care providers. Tell your providers about your history of Stevens-Johnson. If your reaction was caused by medication, be sure to tell your providers about it.

·       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 always wear it.