What is Stevens Johnson Syndrome?

A: Stevens-Johnson syndrome is a rare, serious disorder in which your skin and mucous membranes react severely to a medication or infection.

Stevens Johnson Syndrome (SJS), and TENS (Toxic Epidermal Necrolysis Syndrome ) — another form of SJS — are severe adverse reactions to medication. Adverse drug reactions account for approximately 150,000 deaths per year in the U.S. alone, making drug reactions the fourth leading cause of death in the United States, according to the SJS Foundation.

SJS is one of the most debilitating such reactions recognized. It was first discovered in 1922 by pediatricians A.M. Stevens and F.C. Johnson after diagnosing a child with severe ocular and oral involvement to a drug reaction.

Often, SJS begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of your skin to die and shed. This presents a medical emergency that usually requires hospitalization.

Although SJS afflicts people of all ages, a large amount of its victims are children. More female cases have been reported than male, however it does not discriminate against anyone. The SJS Foundation hears from people around the world who suffer from SJS and TENS

CAUSES

The exact cause of SJS 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 SJS. Drugs commonly associated include:

• Anti-gout medications, such as allopurinol
• Nonsteroidal anti-inflammatory drugs (NSAIDs), often used to treat pain
• Penicillins, which are used to treat infections
• Anticonvulsants, which are used to treat seizures

Infections that can cause SJS include herpes, influenza, HIV, diphtheria, typhoid, hepatitis. In some cases, SJS may be caused by physical stimuli, such as radiation therapy or ultraviolet light.

SYMPTOMS

Signs and symptoms of SJS include facial swelling, tongue swelling, hives, skin pain, a red or purple skin rash that spreads within hours to days, blisters on your skin and mucous membranes – especially in your mouth, nose and eyes – and shedding of your skin. Seek emergency medical care if you develop any of these symptoms.

If you have SJS, several days before the rash develops you may experience fever, sore throat, cough and burning eyes.

PREVENTION

It’s difficult to prevent an initial attack of SJS because you don’t know what will trigger it. However, if you had SJS 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 SJS is usually more severe than the first episode and, in many cases, it can be fatal.

COMPLICATIONS

According to the Mayo Clinic, possible complications of SJS include:

• Secondary skin infection (cellulitis). This acute infection of your skin can lead to life-threatening complications, including meningitis — an infection of the membranes 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.
• 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 on 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.

Doctors often can identify SJS 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

TREATMENT

SJS requires hospitalization, often in an intensive care unit or burn unit.

Stopping medication causes

The first and most important step in treating SJS 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.

Supportive care

Currently there are no standard recommendations for treating SJS. Supportive care you’re likely to receive while hospitalized includes:

• Fluid replacement and nutrition. 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 advanced into your stomach (nasogastric tube).
• Wound care. Cool, wet compresses will help soothe blisters while they heal. 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.
• Eye care. Because of the risk of eye damage, your treatment should include consultation with an eye specialist (ophthalmologist).

Medications

Medications commonly used in the treatment of SJS include:

• Pain medication to reduce discomfort
• Antihistamines to relieve itching
• Antibiotics to control infection, when needed
• Topical steroids to reduce skin inflammation

Experimental medications include:

• Intravenous corticosteroids. For adults, these drugs may lessen the severity of symptoms and shorten recovery time if started within a day or two of when symptoms first appear. For children, they may increase risk of complications.
• Immunoglobulin intravenous (IVIG). This medication contains antibodies that may help your immune system halt the process of Stevens-Johnson syndrome.

Skin grafting

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

If the underlying cause of SJS 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.

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