Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of the same pathology, where a disproportional immune response causes epidermal necrosis, resulting in blistering and shedding of the top layer of skin. Generally, SJS affects less that 10% of body surface area whereas TEN affects more than 10% of body surface area.
Certain HLA genetic types are at higher risk of SJS and TEN.
Causes
Medications
- Anti-epileptics
- Antibiotics
- Allopurinol
- NSAIDs
Infections
- Herpes simplex
- Mycoplasma pneumonia
- Cytomegalovirus
- HIV
Presentation
The condition has a spectrum of severity. Some cases are mild whilst others are very severe and can potentially be fatal.
Patients usually start with non-specific symptoms of fever, cough, sore throat, sore mouth, sore eyes and itchy skin. They then develop a purple or red rash that spreads across the skin and starts to blister.
A few days after the blistering starts, the skin starts to break away and shed leaving the raw tissue underneath. Pain, erythema, blistering and shedding can also happen to the lips and mucous membranes. Eyes can become inflamed and ulcerated. It can also affect the urinary tract, lungs and internal organs.
Management
SJS and TEN are medical emergencies and patients should be admitted to a suitable dermatology or burns unit for treatment. Good supportive care is essential, including nutritional care, antiseptics, analgesia and ophthalmology input. Treatment options include steroids, immunoglobulins and immunosuppressant medications guided by a specialist.
Complications
- Secondary infection: The breaks in the skin can lead to secondary bacterial infection, cellulitis and sepsis.
- Permanent skin damage: Skin involvement can lead to scarring and damage to skin, hair, nails, lungs and genitals.
- Visual complications: Depending on the severity, eye involvement can range from sore eyes to severe scarring and blindness.
Last updated January 2020