Table of Contents
- What is Stevens-Johnson Syndrome?
- How SJS Differs from Other Skin Rashes
- Early Warning Signs to Watch For
- Progression of Skin and Mucosal Symptoms
- Drugs That Most Commonly Cause SJS
- Infection-Related SJS Triggers
- Who is Most at Risk?
- How SJS is Diagnosed
- Differentiating SJS from TEN
- Emergency Room Protocols
- Hospital Care and Burn Unit Management
- Supportive Treatment Options
- Long-Term Complications and Scarring
- Psychological Impact and Recovery
- Prevention and Medication Alerts
- Frequently Asked Questions
What is Stevens-Johnson Syndrome?
Stevens-Johnson Syndrome is a severe skin reaction, most often caused by medications or infections. It affects the skin and mucous membranes, including the eyes, mouth, nose, and genitals. In SJS, the body's immune system reacts violently, causing the top layer of skin to die and shed.
It is considered a medical emergency due to the risk of infection, dehydration, and systemic shock. Quick identification and withdrawal of the trigger are vital for survival and recovery.
How SJS Differs from Other Skin Rashes
Unlike common allergic rashes or hives, SJS causes painful blisters and erosions. The rash typically starts as red or purplish spots that evolve into blisters and then peel away in sheets.
SJS affects both the skin and mucous membranes and is often confused with more benign skin conditions in its early phase, making prompt and accurate diagnosis critical.
Early Warning Signs to Watch For
The initial symptoms of SJS often mimic a viral illness, including fever, sore throat, fatigue, and burning eyes. These symptoms precede the skin reaction by a few days.
Other signs include mouth ulcers, red eyes, and sensitivity to light. Once the rash appears, it spreads rapidly and blisters, making early intervention key to limiting severity.
Progression of Skin and Mucosal Symptoms
The rash typically spreads over hours to days, developing into blisters that burst and peel. The affected skin is often painful to the touch and prone to infection.
Ulcers in the mouth, throat, and genital area cause difficulty in eating, urinating, or swallowing. Eye involvement can lead to conjunctivitis or, in severe cases, blindness.
Drugs That Most Commonly Cause SJS
Several medications are known to trigger SJS, including:
- Antibiotics (e.g., sulfonamides, penicillins)
- Anticonvulsants (e.g., carbamazepine, lamotrigine, phenytoin)
- NSAIDs (e.g., ibuprofen, naproxen)
- Allopurinol (used to treat gout)
Symptoms may develop within 1–3 weeks after starting the medication. Immediate discontinuation is essential upon the first signs of a reaction.
Infection-Related SJS Triggers
While medications are the most common cause, infections—especially in children—can also trigger SJS. These include:
- Herpes simplex virus
- Mycoplasma pneumoniae
- Epstein-Barr virus
Infection-related SJS typically has a more gradual onset and may respond differently to treatment.
Who is Most at Risk?
Although SJS can affect anyone, certain people are at higher risk, including:
- Individuals with a personal or family history of drug allergies
- Those with autoimmune diseases
- HIV-positive individuals (20–100x higher risk)
- Children and young adults taking high-risk medications
Genetic testing for the HLA-B*1502 gene is recommended for people of Asian descent before taking certain anticonvulsants due to increased susceptibility.
How SJS is Diagnosed
Diagnosis is primarily clinical, based on symptoms and recent medication or infection history. Skin biopsy is used to confirm the diagnosis and rule out similar conditions.
Blood tests, cultures, and imaging may be ordered to assess organ function and detect complications like sepsis or pneumonia.
Differentiating SJS from TEN
SJS and Toxic Epidermal Necrolysis (TEN) are part of a spectrum. The difference lies in the amount of skin affected:
- SJS: Less than 10% of body surface area
- TEN: More than 30%
- SJS/TEN overlap: Between 10% and 30%
TEN is more severe, with a higher risk of complications and mortality. Treatment strategies are similar but may vary in intensity.
Emergency Room Protocols
Upon arrival, immediate discontinuation of suspected drugs is the top priority. Vital signs, fluid status, and airway integrity are closely monitored.
Patients are typically admitted to an ICU or burn unit. Early transfer to specialized centers improves outcomes and reduces long-term damage.
Hospital Care and Burn Unit Management
Patients with SJS are treated similarly to burn victims. Care includes:
- Wound care and infection prevention
- Fluid and electrolyte management
- Pain control and temperature regulation
- Nutrition support, often via feeding tube
Multidisciplinary teams involving dermatologists, intensivists, and ophthalmologists coordinate treatment.
Supportive Treatment Options
There is no cure for SJS, but supportive therapies significantly impact recovery. These may include:
- IV immunoglobulin (IVIG)
- Systemic corticosteroids (controversial and case-dependent)
- Plasmapheresis or biologics in severe cases
Topical treatments help protect skin and mucosal surfaces during healing.
Long-Term Complications and Scarring
Survivors may face lasting effects, including:
- Scarring and pigmentation changes
- Ocular damage (dry eye, corneal scarring)
- Respiratory issues from airway involvement
- Chronic pain or PTSD
Regular follow-ups are essential to monitor healing and prevent relapse or complications.
Psychological Impact and Recovery
The trauma of SJS can leave deep emotional scars. Survivors often struggle with anxiety, depression, or PTSD. Supportive counseling and peer groups are critical to recovery.
Involving mental health professionals early can aid in emotional healing, coping strategies, and rebuilding confidence after hospitalization.
Prevention and Medication Alerts
Genetic screening and detailed medication histories are key preventive tools. Patients with known drug allergies should wear medical alert bracelets and inform all healthcare providers.
Pharmacogenetic testing for certain populations (e.g., HLA-B*1502 for carbamazepine) can prevent high-risk prescriptions. Electronic health records and alerts further enhance safety.
Frequently Asked Questions
1. Can SJS be prevented?
Not entirely, but knowing your medication sensitivities and undergoing genetic testing if you're at risk can reduce your chances significantly.
2. What is the survival rate for SJS?
With timely treatment, the survival rate is high—especially for SJS (not TEN). Overall prognosis improves with rapid diagnosis and supportive care.
3. How long is recovery from SJS?
Hospital stays may last several weeks. Full recovery, including skin healing and emotional adjustment, may take months. Long-term monitoring is often required.
4. Are children affected by SJS?
Yes. SJS in children is more likely to be infection-related than drug-induced. Outcomes are generally good with appropriate treatment.
5. Can SJS come back?
Yes, if the person is re-exposed to the triggering drug. Once you've had SJS, you should avoid the offending medication permanently.
Act Fast—Because Every Minute Counts
Stevens-Johnson Syndrome is a race against time. Recognizing the early symptoms and acting quickly can save lives and prevent irreversible damage. If you or someone you know experiences a sudden rash along with flu-like symptoms after starting a new medication, don’t wait—go to the emergency room immediately. Your vigilance could mean the difference between crisis and recovery.