Stevens-Johnson syndrome and toxic epidermal necrolysis

Overview & Definition

Picture a seven-year-old who started lamotrigine six weeks ago for new-onset epilepsy and now arrives with fever, painful red eyes, blistering lips and tender purple target lesions spreading across the trunk, the skin wrinkling and shearing where you touch it. This is Stevens-Johnson syndrome in its classic drug-induced form — one of the true dermatological emergencies of childhood, where the speed of recognition, the withdrawal of the offending drug and the quality of supportive care decide whether the child survives and whether they keep their sight. The clinician's task is to recognise the reaction, grade its severity, remove the cause, and deliver burns-grade supportive care while an honest eye is kept on the ocular surface. [6] [2]

Stevens-Johnson syndrome and toxic epidermal necrolysis are a single disease spectrum of severe, usually drug-induced, mucocutaneous reaction characterised by full-thickness necrosis and detachment of the epidermis. They are uncommon — an incidence of the order of one to two per million people per year in children — but they carry mortality up to around 30 percent for TEN in mixed cohorts, and they leave a heavy burden of long-term sequelae in survivors, above all in the eye. [6] [7]

The clinician's task has three layers. The first is recognition — distinguishing SJS and TEN from their mimics, above all staphylococcal scalded skin syndrome where the mucosae are spared, and from Mycoplasma-induced rash and mucositis where the skin is relatively spared. The second is grading and resuscitation — calculating SCORTEN, stopping the culprit drug, and escalating the child to a burn-capable or intensive-care setting. The third is the long view — protecting the ocular surface from the first hours, anticipating the metabolic and infectious stresses of extensive skin loss, and counselling the family on lifelong drug avoidance and HLA pharmacogenetic screening. [1] [10]