Stevens-Johnson Syndrome: Rare but Serious Skin Condition

What Is Stevens-Johnson Syndrome?

Skin is our body's largest organ, and it serves as a protective barrier against the outside world. When this protective layer becomes compromised, the consequences can be severe. One such condition is Stevens-Johnson Syndrome (SJS).

While SJS is rare, it is a dangerous and potentially life-thretening disorder which affects the skin, mucous membranes, genitals, and eyes. It causes flu-like symptoms along with painful rash that spreads and blisters. If you or someone you know is exhibiting symptoms of SJS, seek medical attention immediately.

Overview

Stevens-Johnson Syndrome falls under the category of dermatologic emergencies and may require hospitalization. It’s often triggered by an adverse reaction to certain medications, infections, or, in some cases, may have an unknown cause. Due to its rapid onset and severity, early diagnosis and prompt medical intervention are critical for better outcomes.

A more advanced form of this condition is referred to as toxic epidermal necrolysis (TEN), characterized by the involvement of over 30% of the skin's surface and substantial damage to the mucous membranes.

Signs and Symptoms

The early symptoms of SJS resemble those of flu and include fever, sore mouth or throat, tiredness, cough, and burning sensation in the eyes. As the condition advances, the symptoms include:

• Unexplained skin pain
• Painful rash:
  A painful red or purplish rash that spreads and blisters
• Blisters
  Blisters on the skin and mucous membranes of the mouth, nose, eyes, and genitals
• Skin shedding
  The top layer of affected skin dies, sheds, and begins to heal after several days
• Facial swelling
• Swollen lips covered in crusty sores
• Respiratory symptoms
  In some cases, SJS can cause cough, difficulty breathing, and/or pneumonia-like symptoms
• Fatigue, weakness, muscle aches, and joint pain

Causes

SJS is often a reaction to certain medications or infections but the exact cause is not known.

Medications that can cause SJS include:

• Anti-gout medications (allopurinol)
• Anti-seizure medications (phenytoin)
• Antipsychotics
• Pain relievers (NSAIDs) such as acetaminophen, ibuprofen
• Antibiotics such as penicillin

Infections that can cause SJS include:

• Herpes virus (Herpes simplex or Herpes zoster)
• Pneumonia
• HIV: In individuals living with HIV, the occurrence of Stevens-Johnson syndrome is approximately 100 times more prevalent compared to the general population.
• Hepatitis A

The risk factors for developing SJS include:

• Weakened immune system: Those with weakened immune systems, whether due to underlying medical conditions or medications that suppress the immune system, may be more susceptible to SJS.
• Family history
• Self-history of the condition
• Ethnicity: Chinese, Southeast Asians, or people of Indian descent are at increased risk

Diagnosis

Detailed medical history is collected and thorough physical examination is carried out. Small samples of the skin or mouth are sent to the laboratory to check for infections and the type of organism causing the infection. Chest X-ray is taken to check for indications of pneumonia. Blood test is done to check for infections or other causes. A small sample of the skin is sent for microscopic examination to confirm SJS and check for other causes such as infections.

Treatment

Treatment starts with avoiding medications that were taken, followed by wound care and medications to relieve pain and cure infections. Various immunomodulatory treatments are available. Fluid replacement is done to replace the fluids lost through the damaged skin.

Complications

SJS)can lead to severe complications, which can be both acute and long-term. Here are some potential complications associated with SJS:

• Secondary Skin Infection (Cellulitis): The extensive skin damage may lead to secondary bacterial infections, which can further complicate the condition and can be life-threatening if they enter the bloodstream (sepsis).
• Sepsis: Sepsis is a potentially life-threatening condition caused by the body's response to an infection, which can spread through the bloodstream and lead to tissue damage, organ failure, and death.
• Eye Problems: SJS can cause severe inflammation of the eyes (conjunctivitis). In more severe cases, it can lead to more significant issues such as scarring, formation of abnormal tissue over the whites of the eyes (symblepharon), and visual impairment or blindness.
• Acute Respiratory Failure: The lungs can be affected, leading to bronchitis, bronchiolitis, and pneumonia.
• Permanent Skin Damage: After recovery, there may be long-term skin problems, including scarring and changes in skin coloration (dyspigmentation).

Prevention

Stevens-Johnson Syndrome (SJS) prevention primarily involves minimizing exposure to known triggers, especially medications associated with a higher risk of this condition. It's important to speak with your healthcare providers, inform them of any prior adverse reactions to medications, and adhere to prescribed dosages and instructions diligently. Regular medication reviews and immediate discontinuation of suspected medications in case of unusual symptoms are crucial. In some cases, consulting specialists or allergists for alternative treatment options with lower Stevens-Johnson Syndrome (SJS) risk may be necessary. Being aware of vaccine-related risks, and genetic predispositions, and promptly reporting any concerning symptoms are important measures in preventing this condition.