Stevens-Johnson syndrome is a rare, serious, immune-complex–mediated hypersensitivity complex that typically involves the skin and the mucous membranes. Often, Stevens-Johnson syndrome 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. It presents a medical emergency that usually requires hospitalization.
Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications. Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. If your doctor determines that your case of Stevens-Johnson syndrome was caused by medication, you'll need to permanently avoid the medication and all others related to it.
What causes Stevens-Johnson syndrome?
The exact cause of Stevens-Johnson syndrome can't always be identified. However, Approximately 75% of Stevens-Johnson syndrome cases are caused by medications and 25% by infections and 'other' causes.
Medication causes
Medications are most often the cause of Stevens-Johnson syndrome. Some ARVs used to treat HIV/AIDS have, on rare occasions, caused Stevens-Johnson syndrome. Nevirapine (an NNRTI), Abacavir (an NRTI) and Raltegravir (an Integrase Inhibitor) have all, on occasions, caused Stevens-Johnson syndrome.
Other drugs commonly associated with Stevens-Johnson syndrome include:
Anti-gout medications, such as allopurinol. Nonsteroidal anti-inflammatory drugs (NSAIDs) often used to treat pain. Various forms of penicillin, which are used to treat infections and anticonvulsants, which are used to treat seizures
Infectious causes and existing medical conditions
HIV infection and other viral infections and diseases that decrease your immunity, such as systemic lupus erythematosus — a chronic inflammatory disease — increase your risk of developing Stevens-Johnson syndrome. Herpes (herpes simplex or herpes zoster), Influenza, Diphtheria, Typhoid, Hepatitis, Epstein-Barr virus, enteroviruses, Coxsackievirus, influenza, mumps, lymphogranuloma venereum, rickettsia and variola and other viruses can as well. Various bacteria, fungi and protozoa can also cause it.
Other causes
Occasionally Stevens-Johnson syndrome may be caused by physical stimuli, such as radiation therapy or ultraviolet light. Stevens-Johnson syndrome is a rare and unpredictable reaction. No test is available to help predict who is at risk. Some factors, however, may increase your risk of developing Stevens-Johnson syndrome, including:
Genetics
Carrying a gene called HLA-B12 may make you more susceptible to Stevens-Johnson syndrome.
Facial swelling, Tongue swelling, hives, unexplained widespread 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, shedding (sloughing) of your skin.
If you have Stevens-Johnson syndrome, several days before the rash develops you may experience:
Fevers, sore throat, cough, burning eyes.
What can I do to help myself?
Get to the Hospital. However, if you have time before you go:
Put all the medications you're taking in a plastic bag, including prescription and over-the-counter drugs. This may help your doctor figure out what triggered Stevens-Johnson syndrome.
Ask a family member or friend to come along, if they're available immediately. Someone who knows you well can help inform the medical staff of your medical history and can help take in information about your current illness.
The emergency room doctor is likely to ask you about your other medical conditions and whether you've experienced a flu-like illness recently. If possible, give that some thought on your way to the hospital, and share important information with anyone who is accompanying you to the emergency room.
While you're in the hospital, you'll likely have questions for your doctor. It might help to keep a running list of questions about Stevens-Johnson syndrome, such as:
- What is the likely cause of my condition?
- How do I keep from having this reaction again?
- What restrictions do I need to follow?
- I have other medical conditions. How do I manage them together?
- How long will it take my skin to heal?
- Am I likely to have any permanent damage?
What should I do if I’ve had Stevens-Johnson syndrome?
If you have had it, 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.
It's difficult to prevent an initial attack of Stevens-Johnson syndrome because you don't know what will trigger it. However, if you had Stevens-Johnson syndrome 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.
For other HIV/ARV Side Effects please click these links:
1. Managing Potential Nevirapine Rashes
2. Nevirapine Rash
http://journals.lww.com/aidsonline/Fulltext/2010/03130/Epidemic_Stevens_Johnson_syndrome_in_HIV_patients.22.aspxhttp://emedicine.medscape.com/article/1124127-overview#a1
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