Lightning Learning: Stevens-Johnson syndrome (SJS)

Lightning Learning: Stevens-Johnson syndrome (SJS)

STOP!

Stevens-Johnson syndrome (SJS) is a rare type IV hypersensitivity reaction with sheet-like skin loss and mucosal ulceration.

Typically it affects <10% of the total body surface area and (if unrecognised) can become life-threatening. Related conditions SJS & Toxic epidermal necrolysis (TENS) overlap. TENS should be considered if skin loss is 10-30% or >30% respectively.

Triggers?

Most commonly medication (e.g. antibiotics, anticonvulsants, NSAIDs), malignancy (in adults) and infection (in children). 25-50% are idiopathic. NB: there is often a time delay before symptoms present (days → months).

Whilst rare (1-2 per million/year, F>M, x100 risk in HIV) it carries a high mortality (10%). This is due to multisystem complications so contact Intensive Care early.

LOOK

Usually there is a flu-like prodrome. Subsequently expect rapid emergence of mucosal lesions, target skin lesions and blistering, all of which is highly suggestive of SJS (a clinical diagnosis).

Clues for SJS?

  • Skin lesions are accompanied by pain (characteristic of SJS and TEN)

  • Oral mucosal involvement is seen in 90% of SJS

  • Usually two mucosal membranes are involved, most commonly oral mucosa and conjunctiva

  • Mucosal lesions precede skin lesions

Complications can include… respiratory compromise, dehydration, shock, secondary infection, thrombosis, DIC, liver, renal and cardiac failure.

Morbidity with SJS is also high, in particular to blindness. Involve Ophthalmology if eyes are involved.

LEARN

Manage similar to burns: remember fluid resuscitation and analgesia.

The single most important step in ED is to stop the offending drug or treat the offending infection.

Further Reading

Lightning Learning: Visual Acuity in Kids

Lightning Learning: Visual Acuity in Kids