Stevens-Johnson Syndrome (SJS): Causes and Treatment

Stevens-Johnson Syndrome (SJS) is a rare but severe hypersensitivity reaction that affects the skin and mucous membranes. Medications or infections usually trigger it, leading to life-threatening complications. Early diagnosis and immediate treatment significantly improve patient outcomes.

Causes of Stevens-Johnson Syndrome

Several factors can trigger SJS, including:

• Medications (most common cause)
• Infections (e.g., Mycoplasma pneumoniae, herpes simplex virus)
• Unknown factors (idiopathic cases)
• Genetic predisposition (certain HLA genes increase susceptibility)

Drugs That Cause Stevens-Johnson Syndrome

Several medications increase the risk of SJS, including:

1. Antibiotics

• Sulfonamides (e.g., sulfamethoxazole)
• Penicillins
• Cephalosporins

2. Anticonvulsants

• Phenytoin
• Carbamazepine
• Lamotrigine
• Phenobarbital

3. Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

• Ibuprofen
• Naproxen
• Oxicam derivatives (e.g., piroxicam)

4. Other Drugs

• Allopurinol (for gout treatment)
• Nevirapine (used in HIV treatment)

Patients who develop SJS should permanently avoid the triggering drug.

For more details on drug safety, visit FDA Drug Safety.

Pathogenesis of Stevens-Johnson Syndrome

SJS results from an abnormal immune response that causes widespread keratinocyte apoptosis (cell death). The process involves:

1. Drug metabolism and immune response: Certain drugs bind to immune cells, triggering a severe immune reaction.
2. Cytotoxic T-cell activation: The immune system mistakenly attacks skin and mucosal cells.
3. Apoptosis of keratinocytes: Destroyed skin cells lead to blistering, necrosis, and detachment.

Signs and Symptoms of Stevens-Johnson Syndrome

SJS begins with flu-like symptoms, followed by painful skin and mucosal involvement:

• Early Symptoms:
  • Fever
  • Sore throat
  • Fatigue
  • Eye irritation
• Progressive Symptoms:
  • Painful skin rash (red or purple patches)
  • Skin blisters and peeling
  • Painful ulcers in the mouth, throat, and genital region
  • Eye complications (conjunctivitis, corneal damage)

When the condition affects less than 10% of body surface area (BSA), it qualifies as SJS. If it involves more than 30% BSA, it is classified as Toxic Epidermal Necrolysis (TEN).

Is Stevens-Johnson Syndrome a Case of Medical Negligence?

SJS does not always result from negligence, as it often occurs unpredictably. However, negligence may be a factor if:

• A known drug allergy was ignored.
• A doctor prescribed a high-risk medication despite contraindications.
• The condition was misdiagnosed, or treatment was delayed.

If negligence seems likely, legal consultation may be necessary. Read more on Medical Negligence in Dermatology.

Treatment and Management of Stevens-Johnson Syndrome

SJS requires urgent hospitalization, often in an ICU or burn unit. Treatment includes:

1. Stopping the Offending Drug

• Discontinuing the suspected medication immediately is crucial.

2. Supportive Care

• Fluids and Electrolyte Management: Prevents dehydration and organ failure.
• Pain Management: Uses non-opioid or opioid analgesics as needed.
• Wound Care: Involves burn-like treatment with non-adhesive dressings.

3. Medications for SJS

• Corticosteroids: May help in early stages but remain controversial.
• IV Immunoglobulin (IVIG): Helps neutralize harmful immune responses.
• Cyclosporine: Reduces immune activity and severity.
• Antibiotics: Only necessary if secondary infections develop.

4. Eye Care

• Ophthalmologic consultation helps prevent long-term vision issues.

Prevention of Stevens-Johnson Syndrome

To lower the risk of SJS:

• Avoid known trigger medications.
• Undergo genetic screening if at high risk (e.g., HLA-B*1502 testing before taking carbamazepine in Asian populations).
• Recognize early drug reactions and stop medication promptly.

FAQs

1. What is the survival rate of Stevens-Johnson Syndrome?

Survival depends on severity. The mortality rate for SJS ranges from 5-10%, while Toxic Epidermal Necrolysis (TEN) has a mortality rate of 30-50%.

2. How long does recovery take?

Recovery often takes weeks to months, and long-term complications may occur.

3. Can SJS be cured?

There is no cure, but early treatment improves survival and reduces complications.

4. Who faces a higher risk of SJS?

Individuals with autoimmune diseases, previous drug reactions, or genetic predispositions have an elevated risk.

5. Can vaccines cause SJS?

SJS following vaccination is rare, but isolated cases have been reported.

Stevens-Johnson Syndrome is a life-threatening condition that demands immediate medical attention. Awareness, early diagnosis, and timely intervention improve outcomes for affected individuals.