FFinalistSouthampton · 2027
Dermatology
AKT · Dermatology/Dermatological emergencies

Stevens-Johnson syndrome / TEN

Drug-induced keratinocyte apoptosis (severe cutaneous adverse reaction)

Overview

A spectrum of severe, usually drug-induced, mucocutaneous reactions with keratinocyte apoptosis and skin detachment. SJS (30%), overlap in between. A dermatological emergency with significant mortality — the key is to stop the culprit drug and transfer to a burns/ICU setting.

Recognise

  • Prodrome (fever, malaise) then a painful, dusky/targetoid rash that blisters and SHEDS in sheets (positive Nikolsky sign)
  • Mucosal involvement of ≥2 sites — eyes, mouth, genitals (erosions, haemorrhagic crusting) — is characteristic and distinguishes it from SSSS
  • Onset typically 1–3 weeks after starting the culprit drug

Red flags

  • Any blistering drug rash with mucosal involvement, skin pain, or a positive Nikolsky sign — same-day emergency; ocular involvement risks blindness (ophthalmology)

Differentials & how to tell them apart

Staphylococcal scalded skin syndromechildren, NO mucosal involvement, split is sub-corneal (superficial) and toxin-mediated — not the full-thickness drug reaction of TEN
Erythema multiforme majortypical raised target lesions, usually post-infective (HSV/Mycoplasma), limited mucosal disease, far less skin detachment
Acute generalised exanthematous pustulosis (AGEP)sterile pustules on erythema, drug-induced but not full-thickness detachment
DRESSdrug reaction with eosinophilia, facial oedema, organ involvement — less detachment

Clinical image

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Stevens-Johnson syndrome / TEN — clinical imagesDermNet

Investigations

Clinical + skin biopsy (full-thickness epidermal necrosis). Calculate SCORTEN (prognostic score). Stop ALL non-essential drugs; screen for the culprit (started in the preceding weeks).

Management

Stop the culprit drug immediately + urgent transfer to a burns/intensive-care unit for supportive care

  1. 1STOP the culprit drug immediately. Resuscitate (fluids, electrolytes, analgesia, temperature control) and transfer urgently to a burns unit / ICU; involve dermatology, ophthalmology and ITU.Gate: Mucosal involvement of ≥2 sites plus skin pain and a positive Nikolsky sign separate SJS/TEN from staphylococcal scalded skin syndrome (no mucosal disease, children) — the distinction changes the entire pathway
  2. 2Meticulous wound and eye care; nutritional support; consider ciclosporin or IVIG in specialist centres; treat secondary infection (a leading cause of death).
STOP the culprit drug — the single most important step; common culprits = anticonvulsants (carbamazepine, lamotrigine), allopurinol, sulfonamides, NSAIDs, nevirapine
Supportive care (fluids, analgesia, wound care, warming) — managed like a major burn
Specialist immunomodulation (ciclosporin / IVIG) — considered in specialist centres — evidence debated

Key points

SCORTEN predicts mortality. The skin detaches in sheets like a burn — fluid balance, infection and eye care dominate. Record the culprit drug as a lifelong allergy.

Monitor & prognosis

Fluid balance, SCORTEN, sepsis, ocular and mucosal sequelae.

High mortality (TEN > SJS); long-term ocular and skin scarring in survivors.

Source: NICE CKS; UK dermatology/burns guidance