Stevens-Johnson Syndrome

Stevens-Johnson Syndrome (SJS) is a very rare skin condition that occurs in less than one person per million of population. Older people and/or people infected with the HIV virus seem to be at a higher risk of contracting the skin disorder. The disease is commonly thought to be a less severe form of Toxic Epidermal Necrolysis (TEN). Due to the serious nature of both of these diseases, patients who are diagnosed with these disorders are usually hospitalized.

Symptoms

The most common symptom is a patchy rash that results in the blistering of the mucous surfaces, including the mouth, throat, eyes, anus and genitals. Several days of flu-like symptoms (i.e. fever, headache, cough, body aches) may precede the eruption. The rash can spread over the face and trunk – and on to other parts of the body. Blisters usually form in the centers of these patchy rashes. The skin around the blistering areas is loose and easy to rub off.

With TEN, larger portions of the skin are affected with associated skin shedding and peeling. These areas become quite painful. This stage of the disease can last from two days to two weeks.

In some cases, digestive and respiratory membranes can become inflamed resulting in diarrhea and breathing difficulty. Eyes can swell shut and corneas can become scarred. Sometimes urination can be painful. In other cases, hair and nails can fall off.

Hospitalization is usually required so that the threat of secondary infections can be properly treated.

Causes

The most common cause of Stevens-Johnson Syndrome is a severe allergic reaction to specific types or classes of medications including:

• NSAIDs (non-steroidal anti-inflammatory drugs)
• Sulfonamides and penicillin (antibiotics)
• Barbiturates (sedatives)
• Lamotrigine, phenytoin or carbamazepine (anticonvulsants)

Additional causes can include viral infections (i.e. herpes simplex, mumps, Epstein-Barr, etc.), carcinomas/lymphomas and presence of the gene marker HLA-B12. In up to one-half of the cases, a specific cause is not identified.

Treatment

Treatment usually starts with hospitalization, followed by a discontinuance of all non-essential medications. If infection is suspected, antibiotics are usually prescribed. Intravenous immunoglobulin (IVIG) has been found to be effective in reducing the length of the reaction and improving symptoms for patients who have severe SJS or TEN. Topical pain anesthetics and antiseptics may also be used. An eye doctor is also usually involved to help minimize any long-term problems associated with scar tissue that can form inside the eyelids, as well as other ocular issues.

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