Steven-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) (3)

Question 1

What are SJS and TEN?

How do both conditions differ?

What are its complications?

Answer 1

➊ It’s 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

➋ * SJS affects < 10% of the body surface area
* TEN affects > 10% of the body surface area

➌ * Secondary infection - The breaks in the skin can lead to secondary bacterial infection, e.g. cellulitis, 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.

Question 2

What are its causes?

How does it present?

How is it definitively diagnosed?

Answer 2

➊ * Medications - Anti-epileptics, Abx, Allopurinol, NSAIDs
* Infections - HSV, Mycoplasma pneumonia, CMV, HIV

➋ * Patients usually start with non-specific symptoms of fever, cough, sore throat, sore mouth, sore eyes and itchy skin. This then develops into a purple/red rash that spreads and starts to blister.
* A few days after the blistering, the skin sheds, leaving raw tissue exposed

➌ Skin biopsy - Shows a layer of subepidermal skin blisteringand dead, thickened epithelial tissue

Question 3

How is it managed?

Answer 3

• These are medical emergencies and pts should be admitted to a suitable dermatology or burns unit for treatment
• Good supportive care is essential, including nutritional care, abx, analgesia and ophthalmology input
• Treatment options include steroids, immunoglobulins, and immunosuppressants