Stevens Johnson Syndrome (SJS) is a rare and potentially life-threatening skin disorder.
Who Gets SJS?
While the occurrence of SJS is rare and highly unpredictable, there are some risk factors associated with a higher rate of developing the disorder. Any medical condition that compromises the immune system could potentially heighten the risk of developing serious skin disorders, including SJS. Additionally, individuals carrying a gene called HLA-B12 may have an elevated risk, but it is unlikely that an individual would be screened for this gene prior to developing SJS.
What is SJS?
Stevens Johnson Syndrome is a very serious disorder that affects the skin and mucous membranes of the affected individual. Some of the first symptoms to appear are flu-like ailments, such as fever, cough, and a general feeling of being ill. Skin lesions, characterized by red or purple rashes, sores, and blisters, soon follow. The rash will quickly blister, causing the skin to die, dry, and slough off. The mucous membranes of the eyes, nose, and mouth will also be affected in some cases, leading to blisters, swelling, and pain. These symptoms usually occur evenly on both sides of the body.
It is important to remember that SJS can be life threatening. In the event of experiencing any symptoms, immediate medical attention is necessary. Serious complications, including secondary infections, lesioning of the internal organs, irreversible skin damage, sepsis, and fluid loss may occur if treatment for SJS is not delivered in a timely manner.
What Causes SJS?
Stevens Johnson Syndrome is most often caused by an infection or allergic reaction, but the exact cause of SJS is not always apparent.
Allergic reactions are usually caused by hypersensitivity to certain medications. Drugs that are commonly associated with an increased occurrence of SJS include anticonvulsants, nonsteroidal anti-inflammatory drugs, penicillins, sulfonamides, anti-gout drugs, barbiturates, and other medications. Due to the potentially fatal nature of SJS, the drug suspected of causing the disorder is almost always stopped as part of treatment. If SJS is caused by medication, it is important to wear a medical information bracelet containing the name of the medication, and all health care providers should be notified of a patient’s history of SJS.
Some infections, including herpes, HIV, influenza, hepatitis, and other viruses can also cause SJS. Additionally, exposure to harsh stimuli like ultraviolet light and radiation treatment can trigger SJS in a rare number of cases.
How is SJS Treated?
The first step in treating SJS is removing the factor that caused the onset of the disorder. Medication reactions are the most common cause, and it is important to stop taking the medication as directed by a doctor. Additionally, the same medication should be avoided in the future.
When a particular virus, such as herpes simplex, is suspected of causing SJS, the individual may need maintenance treatment with drugs, such as antiviral medications, to prevent relapse.
Pain medications, moist compresses, and topical treatments can help ease a patient’s discomfort during recovery. Fluid replacement is necessary in many cases, especially those in which the mucous membranes have been deeply affected. If secondary infections are a possibility, antibiotics might also be administered to prevent complications.
Stevens Johnson Syndrome has a relatively high fatality rate, but the risk of death is decreased when appropriate treatment is administered quickly.
Resources:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001854/
http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940