Stevens Johnson Syndrome – Symptoms and Causes
Overview
Stevens-Johnson syndrome (SJS) is a rare but serious skin condition that requires immediate medical attention. The disorder affects the skin and mucous membranes.
It typically starts with flu-like symptoms before developing into a painful, blistering rash. In severe cases, the top layer of skin dies and sheds, leaving patients vulnerable to infection.
Recovery from SJS can take weeks or months, depending on the severity. Medical professionals consider it a medical emergency requiring hospitalization.
Toxic epidermal necrolysis (TEN) is a severe condition that affects over 30% of the skin and causes significant harm to mucous membranes.
If medication triggered your SJS, doctors will advise you to permanently avoid that drug and similar medications in the future. This precaution is critical to prevent recurrence of this potentially life-threatening condition.
Treatment involves four key areas:
- Stopping the triggering medication,
- Caring for damaged skin, managing pain, and preventing complications during healing
Patients typically receive care in burn units or intensive care settings where specialists can monitor and treat this complex condition.
Signs and Warning Signals
Early warning signs of Stevens-Johnson syndrome often appear one to three days before the rash develops. These early signs include:
- Fever
- Painful mouth and throat
- Extreme tiredness
- Eye burning or irritation
As the condition gets worse, patients may experience:
- Skin pain with no clear cause
- Red or purple rash that grows larger
- Blisters forming on the skin, mouth, nose, eyes, and genital areas
- Skin peeling off within days after blisters appear
These symptoms need immediate medical attention. The condition can quickly become serious and life-threatening if not treated promptly.
When to See a Doctor
If you notice any signs of Stevens-Johnson syndrome, get emergency medical help right away. Monitor for symptoms when starting new medications, as reactions can occur during use or up to two weeks after stopping.
What Causes Stevens-Johnson Syndrome
Stevens-Johnson syndrome is an uncommon and hard-to-predict condition. Doctors often struggle to pinpoint the exact cause, but it’s typically triggered by medications, infections, or a combination of both.
Medications that may trigger Stevens-Johnson syndrome:
- Allopurinol (used for gout)
- Anticonvulsants and antipsychotics (seizure and mental health medications)
- Antibacterial sulfonamides (including sulfasalazine)
- Nevirapine (HIV medication)
- Common pain relievers like acetaminophen, ibuprofen, and naproxen sodium
Infections linked to Stevens-Johnson syndrome:
- Pneumonia
- HIV
The condition is not contagious, but its unpredictable nature makes it difficult to prevent in people who may be susceptible. Genetic factors might play a role in determining who develops this reaction to certain medications or infections.
Risk Factors
Several factors may increase your chance of developing Stevens-Johnson syndrome:
Health Conditions
- People with HIV have about 100 times higher risk than the general population
- Weakened immune systems from organ transplants, autoimmune diseases, or HIV/AIDS
- Cancer, especially blood cancers
Personal and Family History
- Previous episodes of Stevens-Johnson syndrome make recurrence more likely if the same medication is used again
- Having a blood relative who has experienced the condition
Genetic Factors
- Certain genetic variations increase risk, particularly for people taking medications for:
- Seizures
- Gout
- Mental illness
Having these risk factors doesnโt guarantee someone will develop the condition, but they should be taken into account when prescribing medications that can trigger Stevens-Johnson syndrome.
Complications
Stevens-Johnson syndrome can lead to several serious health issues. When it occurs, patients may face severe complications that require immediate medical attention.
Patients often experience dehydration as fluid is lost through damaged skin areas. Drinking becomes difficult due to painful mouth and throat sores.
Blood infection, or sepsis, represents a life-threatening complication. This happens when bacteria enter the bloodstream, potentially causing shock and organ failure.
Eye problems commonly develop with this condition. These include inflammation, dryness, and sensitivity to light. In severe cases, patients may suffer vision loss or even blindness.
Respiratory complications can be critical. The syndrome may cause acute respiratory failure, where the lungs cannot deliver enough oxygen to the blood.
Long-term skin damage often occurs after recovery. The new skin may have abnormal coloring, bumps, or scarring. Some patients experience hair loss, and nail growth may be affected permanently.
Prevention
Genetic testing is an important step before starting certain medications, especially for people with Asian or South Asian ancestry. The FDA suggests screening for the HLA-B*1502 gene variation as a precautionary measure.
Additional Information
If you’ve previously experienced this condition, it’s crucial to avoid the medication that caused it. Your healthcare provider can identify the triggering drug and similar medications, which you should strictly avoid in the future.
Subsequent episodes are typically more severe than the initial occurrence and can sometimes be life-threatening. Your blood relatives should also consider avoiding the same medication, as this condition may have genetic factors.
Share your medical history with family members so they can inform their healthcare providers about potential risks. Remember to carry medical identification that lists medications you are allergic to, to prevent accidental exposure during emergencies.
