Johnson-Stevens Syndrome

Stevens-Johnson Syndrome (SJS): Symptoms, Causes, Types, Diagnosis, and Treatments

Stevens-Johnson Syndrome (SJS) is a rare but life-threatening medical condition that affects the skin and mucous membranes. Characterized by the peeling of skin and severe blisters, SJS requires immediate medical attention. While it often starts with flu-like symptoms, it can rapidly progress into widespread skin damage and systemic complications. Understanding this complex condition is crucial for early detection, proper treatment, and prevention of long-term consequences.

This comprehensive guide explores Stevens-Johnson Syndrome, covering its symptoms, causes, types, diagnosis, and treatment options in detail. Whether you’re a healthcare enthusiast, caregiver, or simply someone looking to stay informed, this article provides everything you need to know.

---

What is Stevens-Johnson Syndrome?

Stevens-Johnson Syndrome is a rare, serious disorder of the skin and mucous membranes, most often triggered by a reaction to a medication or an infection. It is considered a medical emergency and often requires hospitalization.

SJS falls under a broader category of severe cutaneous adverse reactions (SCARs) and is often considered a milder variant of a more extensive condition known as Toxic Epidermal Necrolysis (TEN). Both disorders involve extensive cell death that causes the epidermis to detach from the dermis, leading to open wounds, pain, and increased risk of infection.

---

Prevalence and Risk Factors

SJS is extremely rare, affecting about 1 to 2 people per million per year. Despite its rarity, its severity makes it a critical concern in clinical settings.

Risk factors include:

• Age: More common in adults, but children can be affected.
• Genetics: Certain HLA gene variants increase susceptibility.
• Immunosuppression: HIV/AIDS patients are at a higher risk.
• Medications: Specific drugs are more likely to trigger SJS.
• Previous history: People who have had SJS once are at high risk of recurrence if re-exposed to the same trigger.

---

Symptoms of Stevens-Johnson Syndrome

The symptoms of SJS can develop quickly—typically within 1 to 3 weeks of exposure to the trigger.

Early Symptoms (Prodromal Phase)

• Fever (often >39°C/102°F)
• Sore throat
• Cough
• Red or itchy eyes (conjunctivitis)
• Malaise or fatigue
• Burning sensation on the skin

These symptoms are often mistaken for the flu, which delays diagnosis and treatment.

Advanced Symptoms (Acute Phase)

As the condition progresses:

• Painful red or purplish rash
• Blisters on skin and mucous membranes (mouth, eyes, genitals)
• Skin shedding or sloughing (epidermal detachment)
• Difficulty swallowing
• Photophobia (sensitivity to light)
• Urinary retention or painful urination
• Crusting of lips and oral ulcers

Complications include:

• Sepsis
• Pneumonia
• Permanent skin damage
• Eye complications (scarring, vision loss)
• Organ failure (rare but possible)

---

Causes of Stevens-Johnson Syndrome

1. Medications (Most Common Cause)

Approximately 80% of SJS cases are triggered by drugs. Common culprits include:

• Antibiotics: Sulfonamides (e.g., sulfamethoxazole), penicillins
• Anticonvulsants: Phenytoin, carbamazepine, lamotrigine
• NSAIDs: Ibuprofen, naproxen (especially in children)
• Allopurinol: Used to treat gout
• Antipsychotics and antidepressants

2. Infections

• Herpes Simplex Virus (HSV)
• Mycoplasma pneumoniae (especially in children)
• HIV
• Hepatitis
• Epstein-Barr Virus (EBV)

3. Vaccinations (Rare)

Some vaccinations may rarely trigger SJS, especially in genetically predisposed individuals.

4. Idiopathic (Unknown Causes)

In 25-30% of cases, no identifiable cause is found, which makes prevention difficult.

---

Types and Classification of SJS

SJS is often categorized based on the percentage of body surface area (BSA) affected.

1. Stevens-Johnson Syndrome (SJS)

• <10% of BSA is affected.
• Milder form compared to TEN but still serious.
• Blisters usually affect the trunk, face, and mucous membranes.

2. Toxic Epidermal Necrolysis (TEN)

• 30% of BSA is affected.
• High mortality rate (30-40%)
• Greater risk of organ failure and sepsis.

3. SJS/TEN Overlap

• 10-30% of BSA involvement.
• Shares features of both SJS and TEN.
• Intermediate prognosis.

---

Diagnosis of Stevens-Johnson Syndrome

Early and accurate diagnosis is crucial to reduce complications and fatality.

1. Clinical Examination

• Identification of skin lesions and mucous involvement.
• History of drug intake or infection 1-3 weeks prior.

2. Skin Biopsy

A skin biopsy is the gold standard for confirming diagnosis.

• Shows necrotic keratinocytes and subepidermal blistering.
• Helps rule out other bullous skin diseases.

3. Blood Tests

• Complete Blood Count (CBC): May show leukopenia or anemia.
• Liver and kidney function tests: To monitor systemic involvement.
• Electrolyte imbalance: Due to fluid loss from skin lesions.

4. Imaging

Chest X-rays may be performed to check for pneumonia or pulmonary complications.

5. SCORTEN

A scoring system used to predict prognosis and mortality. Parameters include:

• Age >40 years
• Heart rate >120 bpm
• Cancer or hematologic malignancy
• BSA >10%
• Serum urea >10 mmol/L
• Serum glucose >14 mmol/L
• Serum bicarbonate <20 mmol/L

Higher SCORTEN = worse prognosis.

---

Treatment of Stevens-Johnson Syndrome

There is no specific cure for SJS. Management is primarily supportive and symptomatic, and early discontinuation of the offending drug is essential.

1. Hospitalization (ICU or Burn Unit)

Due to the extensive skin damage, patients are usually treated in intensive care or burn centers.

2. Supportive Care

• Fluid and electrolyte balance
• Nutritional support: Often through a nasogastric tube
• Temperature regulation: Hypothermia is a concern due to skin loss
• Pain management

3. Wound Care

• Non-adhesive dressings
• Debridement if necessary
• Topical antibiotics to prevent secondary infections

4. Medications

a. Corticosteroids

• Used in early stages to reduce inflammation
• Controversial—may increase infection risk

b. Intravenous Immunoglobulins (IVIG)

• Helps block immune responses
• Shown to reduce mortality, especially in TEN

c. Immunosuppressants

• Cyclosporine: Has shown promise in reducing skin detachment
• TNF-alpha inhibitors (e.g., etanercept): Used in some cases

d. Antibiotics

• Used only when there is a confirmed secondary infection

e. Ophthalmologic Treatments

• Lubricants, steroids, or antibiotics to prevent eye damage
• Severe cases may need amniotic membrane transplantation

5. Long-Term Management

Patients may face several chronic complications:

• Skin discoloration
• Eye damage (dry eye, scarring, blindness)
• Respiratory problems
• Genital scarring

Regular follow-ups with dermatologists, ophthalmologists, urologists, and psychologists may be needed.

---

Prevention of Stevens-Johnson Syndrome

While not all cases are preventable, some precautions can reduce the risk.

1. Genetic Testing

• Individuals with HLA-B*1502 gene (common in Asians) should avoid carbamazepine.
• Genetic screening before prescribing high-risk drugs is becoming standard in some countries.

2. Drug Avoidance

• Once a drug is identified as the cause, it should be permanently avoided.
• Medical alerts (bracelets or wallet cards) can help in emergencies.

3. Regular Monitoring

• Monitor for early symptoms when starting new medications.
• Consult a healthcare provider immediately if a rash develops.

---

Prognosis and Recovery

Recovery from SJS depends on:

• Age
• Severity (BSA affected)
• Prompt discontinuation of the trigger
• Overall health

Survival Rates

• SJS: ~90% survival with prompt treatment
• TEN: Mortality rates up to 40%

Recovery Time

• Acute phase lasts 2-4 weeks
• Full skin healing may take months
• Eye or organ complications may persist longer

Quality of Life

Many survivors experience:

• Emotional trauma
• Chronic pain
• Visual impairment
• Scarring

Rehabilitation and counseling are important parts of recovery.

---

Living with a History of SJS

Having had SJS once places an individual at high risk of recurrence. Patients must:

• Maintain a detailed list of allergies
• Wear medical ID tags
• Communicate their medical history to all healthcare providers
• Undergo genetic testing where available

Support groups and online communities can offer valuable emotional and psychological assistance.

---

Conclusion

Stevens-Johnson Syndrome is a devastating but rare condition that requires immediate medical intervention. With increasing awareness, genetic testing, and careful drug use, the incidence and severity of SJS can be significantly reduced.

Early recognition of symptoms, discontinuation of the causative agent, and supportive care are the cornerstones of effective management. For survivors, long-term monitoring and rehabilitation are essential to restore quality of life.

If you or someone you know is starting a new medication or has a history of drug allergies, stay alert. Timely action can mean the difference between a manageable condition and a life-threatening crisis.

Frequently Asked Questions (FAQs) About Stevens-Johnson Syndrome

For more details keep visiting our Website & Facebook Page.