Overview

Stevens-Johnson Syndrome (SJS) is a rare but serious disorder of the skin and mucous membranes. It is considered a medical emergency that usually results from a severe reaction to medications or infections. SJS is characterized by painful red or purplish rashes that spread and blister, eventually causing the top layer of skin to peel away. It affects not only the skin but also mucous membranes, including the eyes, mouth, and genitals. Early diagnosis and prompt treatment in a hospital setting are critical to prevent life-threatening complications and long-term damage.

Causes

Stevens-Johnson Syndrome is primarily caused by hypersensitivity reactions, most commonly to certain medications or infections. Common triggers include:

• Medications:
  • Antibiotics, particularly sulfonamides like sulfamethoxazole.
  • Anticonvulsants such as carbamazepine, phenytoin, and lamotrigine.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs), especially oxicam derivatives.
  • Allopurinol, used to treat gout.
• Infections:
  • Mycoplasma pneumoniae (a type of bacterial lung infection).
  • Herpes simplex virus (HSV).
  • HIV infection.
• Other causes: In rare cases, SJS may be triggered by vaccines or idiopathic (unknown) reasons.

Symptoms

The symptoms of Stevens-Johnson Syndrome typically begin with flu-like signs, followed by skin and mucous membrane involvement:

• Early symptoms (1-3 days before rash):
  • Fever and chills.
  • Fatigue and malaise.
  • Sore throat and cough.
  • Burning eyes or conjunctivitis.
• Skin symptoms:
  • Painful red or purplish rash that spreads rapidly.
  • Blisters on skin, mouth, eyes, and genitals.
  • Peeling skin that exposes raw areas underneath.
  • Target-like lesions with central blisters.
• Mucous membrane symptoms:
  • Severe mouth ulcers and painful swallowing.
  • Eye irritation, redness, and discharge.
  • Genital discomfort or ulceration.

Diagnosis

Diagnosing SJS involves clinical examination and, in some cases, laboratory and skin biopsy tests:

• Medical history: Reviewing recent medication use, infections, or vaccinations.
• Physical examination: Identifying characteristic rash patterns, mucosal involvement, and skin peeling.
• Skin biopsy: A small skin sample may be taken to confirm the diagnosis by examining tissue under a microscope.
• Blood tests: To check for infection, inflammation markers, and organ function.
• Microbiological testing: Screening for underlying infections like Mycoplasma pneumoniae or herpes simplex virus if suspected.

Treatment

Stevens-Johnson Syndrome requires immediate hospitalization, often in an intensive care unit (ICU) or burn unit, due to the severity of skin damage and risk of complications:

• Discontinuation of the trigger: Immediate stopping of the suspected medication or treating the underlying infection.
• Supportive care:
  • Fluid and electrolyte management to prevent dehydration.
  • Pain management with appropriate medications.
  • Wound care to prevent infection and promote skin healing.
  • Nutritional support, including tube feeding if mouth ulcers are severe.
• Medications:
  • Antibiotics if secondary bacterial infection develops.
  • Corticosteroids or immunoglobulins (IVIG) may be used in selected cases to reduce inflammation, though their use is debated.
  • Eye treatments like lubricating drops or antibiotic ointments to protect the eyes.

Prognosis

The prognosis of Stevens-Johnson Syndrome varies depending on the extent of skin involvement, patient age, overall health, and the speed of diagnosis and treatment. Mild cases involving less than 10% of body surface area have a better outlook, especially with early medical care.

Severe cases may lead to complications such as secondary infections, sepsis, dehydration, vision loss, or long-term scarring of the skin and mucous membranes. The condition can be fatal in some cases, especially in older adults or those with extensive skin involvement. With timely treatment in specialized centers, survival rates have improved, and many patients recover fully, though some may experience lasting complications.

Long-term follow-up, especially with dermatologists and ophthalmologists, is often required to manage chronic symptoms, prevent relapses, and rehabilitate skin and eye health.