Stevens-Johnson syndrome and toxic epidermal necrolysis

Last reviewed: March 2020

Last updated: September 2019

Summary

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be associated with a preceding history of medication use, most commonly anticonvulsants, antibiotics, and non-steroidal anti-inflammatory drugs. Other associated factors include infections.

The patient may present with Nikolsky's sign, where the epidermal layer easily sloughs off when pressure is applied to the blistered or erythematous area.

Diagnosis is made by clinical presentation and confirmed with skin biopsy.

On diagnosis the offending medicine should be stopped. Management is then supportive.

Patients do best if they are sent to a burn centre for wound care as soon as the diagnosis is suspected or made.

The majority of SJS patients recover (mortality 1% to 5%). SJS can recur either with the same medicine or with another medicine.

TEN has a higher mortality (25% to 30%).

In the long term, patients should ensure they are not re-exposed to the trigger medicine and be careful of self-medicating. They should avoid sunlight during healing and moisturise their skin.

Currently, patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.

Definition

Stevens-Johnson syndrome (SJS) is a severe skin detachment with mucocutaneous complications.[1] It is an immune reaction to foreign antigens. SJS is a more severe form of erythema multiforme major and a less severe manifestation of toxic epidermal necrolysis (TEN).[2] Classification is dependent on the percentage of skin involvement: SJS has <10% total body surface area (TBSA) involvement; SJS/TEN overlap has 10% to 30% TBSA involvement; and TEN has >30% TBSA involvement.[3][4][5][6][7][8][9]