Stevens-Johnson Syndrome: Causes, Symptoms, and Treatments - Dermatology

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Stevens-Johnson Syndrome (SJS) is a severe and potentially life-threatening condition characterized by the detachment of the epidermis from the dermis, leading to widespread skin and mucosal lesions. It is often considered a medical emergency and requires immediate attention.

Causes
The exact cause of Stevens-Johnson Syndrome is not always clear, but it is frequently associated with certain medications, infections, and sometimes, it can occur without a known trigger. Common culprits include:
1. Medications: The most common triggers are medications, particularly anticonvulsants (like phenytoin), antibiotics (such as sulfonamides), and non-steroidal anti-inflammatory drugs (NSAIDs).
2. Infections: Viral infections, such as herpes simplex virus, Mycoplasma pneumoniae, and cytomegalovirus, can also precipitate SJS.

3. Idiopathic: In some cases, no identifiable cause can be found, which is referred to as idiopathic SJS.


Symptoms
The symptoms of Stevens-Johnson Syndrome can vary but typically include:
- Skin Rash: The hallmark of SJS is a painful, red, or purplish rash that spreads and blisters. The rash often resembles a burn and can cover large areas of the body.

- Mucosal Involvement: At least two mucosal surfaces are usually affected, including the eyes, mouth, and genital areas. This can lead to severe complications, such as conjunctivitis or oral ulcers.

- Flu-like Symptoms: Patients may initially experience flu-like symptoms, including fever, sore throat, and fatigue, before the rash appears.

- Systemic Symptoms: In severe cases, patients may experience systemic symptoms such as difficulty breathing, rapid heartbeat, and low blood pressure.


Treatment
The treatment of Stevens-Johnson Syndrome primarily focuses on supportive care and addressing the underlying cause. Key treatment strategies include:
1. Immediate Discontinuation of Offending Agents: If a medication is identified as the trigger, it must be stopped immediately to prevent further progression of the syndrome.

2. Supportive Care: Patients often require hospitalization, particularly in a burn unit or intensive care setting, where they can receive specialized care. Supportive measures may include:
- Fluid Replacement: To prevent dehydration, especially if there is extensive skin loss.

- Pain Management: Analgesics are used to manage severe pain associated with skin lesions.

- Wound Care: Similar to burn care, the management of skin lesions is crucial to prevent infection and promote healing.

3. Medications: In some cases, corticosteroids may be administered to reduce inflammation, although their use is controversial and should be carefully considered. Other immunosuppressive agents may also be used in severe cases.

4. Management of Complications: Close monitoring for complications such as secondary infections, respiratory distress, and ocular complications is essential. Referral to specialists, such as ophthalmologists for eye involvement, may be necessary.


Conclusion
Stevens-Johnson Syndrome is a serious condition that requires prompt recognition and treatment. Understanding its causes, symptoms, and treatment options is crucial for healthcare providers to manage this potentially life-threatening syndrome effectively. Early intervention can significantly improve outcomes and reduce the risk of long-term complications. If you suspect SJS in yourself or someone else, seek immediate medical attention.