Stevens Johnson Syndrome : Symptoms and Treatment

What is Stevens-Johnson Syndrome (SJS)?

Stevens-Johnson Syndrome (SJS) is a rare but serious disorder that affects the skin and mucous membranes. It is often triggered by a reaction to medications or infections. SJS is a medical emergency, as it can lead to severe skin detachment, blistering, and complications that can be life-threatening. If untreated, the condition may progress to toxic epidermal necrolysis (TEN), a more severe form of the disease, characterized by widespread skin detachment and systemic complications.

Symptoms of Stevens-Johnson Syndrome

SJS typically starts with non-specific symptoms, followed by more severe manifestations.

Early Symptoms (Prodromal Phase):

Fever

Fatigue or malaise

Sore throat

Cough

Red or watery eyes

Later Symptoms (After 1–3 Days):

Skin Rash: Begins as red or purplish spots, usually painful, and spreads quickly.

Blisters: On the skin and mucous membranes (e.g., mouth, eyes, genitals).

Skin Peeling: Large areas of skin may slough off, leaving raw, exposed skin.

Mucosal Involvement: Painful sores in the mouth, throat, nose, and genitals.

Eye Problems: Redness, swelling, and sensitivity to light; in severe cases, blindness.

Causes of Stevens-Johnson Syndrome

The exact cause of SJS is not always clear, but it is often associated with:

Medications

Antibiotics: Sulfonamides (e.g., sulfamethoxazole), penicillins, and fluoroquinolones.

Anti-seizure drugs: Phenytoin, carbamazepine, lamotrigine.

NSAIDs: Ibuprofen, naproxen, and others.

Allopurinol: Used for gout treatment.

Infections

Viral: Herpes simplex virus, HIV, hepatitis, and influenza.

Bacterial: Mycoplasma pneumoniae.

Fungal: Histoplasmosis.

Other Factors

Genetic predisposition: Certain genetic markers (e.g., HLA-B*1502) increase the risk, especially in certain ethnic groups.

Vaccinations or physical trauma (rarely).

Who is at Risk of SJS?

Some people have a higher risk of developing Stevens-Johnson Syndrome:

Genetic predisposition: People with certain HLA gene types (e.g., HLA-B*1502 in Asian populations).

Age: While it can affect anyone, older adults are at higher risk.

Weakened immune system: HIV patients, cancer patients, or those on immunosuppressive drugs.

History of SJS: Recurrence risk is higher if exposed to the same drug or trigger.

Complications of Stevens-Johnson Syndrome

If not treated promptly, SJS can lead to severe complications, including:

Infections: Bacterial infections from skin lesions, which may spread to the bloodstream (sepsis).

Organ Damage: Liver, kidneys, and lungs can be affected.

Dehydration and Electrolyte Imbalance: Due to loss of fluids from damaged skin.

Scarring and Disfigurement: Permanent scars on the skin and mucous membranes.

Vision Problems: Severe cases may lead to blindness.

Diagnosis of Stevens-Johnson Syndrome

Medical History and Physical Exam

A detailed history of recent medications or infections is taken, along with a close examination of the rash and mucosal involvement.

Skin Biopsy

A small sample of affected skin is analyzed under a microscope to confirm the diagnosis.

Blood Tests

These may help identify the cause (e.g., infection or drug reaction) and monitor organ function.

Treatment of Stevens-Johnson Syndrome

SJS is treated in a hospital setting, often in an intensive care unit (ICU) or burn unit.

Immediate Steps

Stop the Trigger: Discontinue the suspected medication or treat the underlying infection.

Supportive Care: Includes IV fluids, pain management, and wound care.

Medications

Corticosteroids: Reduce inflammation (controversial in severe cases).

Immune-Modulating Therapies: Intravenous immunoglobulin (IVIG) or cyclosporine.

Antibiotics/Antifungals: If secondary infections develop.

Nutritional Support

Feeding tubes may be required in severe cases where oral intake is difficult due to mucosal involvement.

Prevention of Stevens-Johnson Syndrome

Avoid Known Triggers

Inform healthcare providers about any history of SJS or adverse drug reactions.

Genetic testing may help identify susceptibility (e.g., HLA-B*1502 screening for carbamazepine users in Asia).

Monitor Medication

Take medications as prescribed and avoid self-medication.

Report unusual symptoms, especially during the first few weeks of a new medication.

Stevens-Johnson Syndrome vs. Toxic Epidermal Necrolysis (TEN)

SJS and TEN are part of the same spectrum but differ in severity:

SJS: Affects <10% of the body surface area.

TEN: Affects >30% of the body surface area.

Overlap Syndrome: 10–30% of the body surface area involved.

FAQs About Stevens-Johnson Syndrome

1. Is Stevens-Johnson Syndrome fatal?
SJS can be life-threatening if untreated, with a mortality rate of 5–10%. Prompt medical care reduces risks.

2. Can SJS recur?
Yes, if exposed to the same trigger. Avoiding triggers is crucial.

3. How long does recovery take?
Recovery may take weeks to months, depending on severity and complications.

4. Are there long-term effects of SJS?
Long-term issues may include scarring, chronic pain, vision loss, or psychological distress.

Conclusion

Stevens-Johnson Syndrome is a rare yet severe condition that requires prompt diagnosis and medical care. Identifying the trigger and providing supportive treatment are key to improving outcomes. If you or someone you know develops unusual skin symptoms after taking medication or an infection, seek medical attention immediately.