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Drug reactionEmergencyICD-10 L51.1 / L51.2

Stevens-Johnson syndrome / TEN

SJS / TEN ยท Lyell syndrome (TEN) ยท epidermal necrolysis spectrum

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe immune-mediated mucocutaneous reactions โ€” most commonly drug-induced โ€” with full-thickness epidermal necrosis and high mortality (5-50%). They differ in body-surface-area (BSA) of detachment: SJS <10%, SJS/TEN overlap 10-30%, TEN >30%. Immune-checkpoint inhibitors are an increasingly recognised cause. Management is a dermatological emergency requiring critical care, multidisciplinary input and use of the SCORTEN prognostic score and ALDEN drug-causality algorithm.

CurrentLast reviewed 16 May 2026
Clinical image of Stevens-Johnson syndrome / TEN
Stevens-Johnson syndrome / TEN. Image sourced from DermNet New Zealand. Used under CC BY-NC-ND 4.0. No endorsement implied.

Aetiology

  • Drugs account for ~80%; onset typically 4-28 days post-exposure.
  • Highest-risk drugs:
    • Allopurinol (HLA-B*58:01 โ€” esp. Asian populations).
    • Anticonvulsants โ€” carbamazepine (HLA-B*15:02), phenytoin, lamotrigine, oxcarbazepine.
    • Sulfonamides โ€” sulfamethoxazole, sulfasalazine.
    • NSAIDs โ€” especially oxicams.
    • Paracetamol โ€” rare reported trigger.
    • Antiretrovirals โ€” especially nevirapine.
    • Antibiotics โ€” penicillins, cephalosporins, vancomycin, fluoroquinolones.
    • Immune-checkpoint inhibitors โ€” nivolumab, pembrolizumab, ipilimumab; later onset (weeks-months).
  • Infections โ€” particularly Mycoplasma pneumoniae (more commonly mucocutaneous SJS-like reaction termed MIRM โ€” Mycoplasma-induced rash and mucositis), HSV.
  • Idiopathic in some.

Clinical features

  • Prodrome 1-3 days: fever, malaise, sore throat, eye irritation.
  • Skin: tender atypical targetoid macules โ†’ flaccid bullae โ†’ sheets of epidermal detachment; positive Nikolsky and Asboe-Hansen signs.
  • Mucosal involvement (โ‰ฅ2 sites in โ‰ฅ90%): oral, ocular, genital, anal, respiratory (bronchial casts in TEN).
  • Sites: face / upper trunk first; progression centrifugal.
  • Detachment extent:
    • SJS: <10% BSA.
    • SJS/TEN overlap: 10-30% BSA.
    • TEN: >30% BSA.
  • Complications: fluid loss, hypothermia, sepsis (S. aureus, Pseudomonas), pneumonia, AKI, GI bleeding, multi-organ failure.

SCORTEN and ALDEN

SCORTEN โ€” calculated within 24 h and at day 3; one point each:

  • Age >40 years
  • Heart rate >120 bpm
  • Underlying malignancy
  • BSA detached >10%
  • Serum urea >10 mmol/L
  • Bicarbonate <20 mmol/L
  • Glucose >14 mmol/L

Mortality: 0-1 = 3%; 2 = 12%; 3 = 35%; 4 = 58%; โ‰ฅ5 = 90%.

ALDEN score: algorithmic drug-causality assessment based on temporal relationship, drug notoriety, alternative cause, response to dechallenge, previous reaction. Use to identify the most probable single drug culprit.

Investigations

  • Detailed drug history โ€” every drug taken in preceding 8 weeks.
  • Skin biopsy: full-thickness epidermal necrosis with minimal dermal infiltrate; rules out DDx (AGEP, GVHD, paraneoplastic pemphigus, autoimmune blistering, SSSS).
  • Bloods: FBC, U&E, LFT, glucose, lactate, ABG / bicarbonate, CRP, coagulation, blood cultures.
  • HIV testing.
  • HLA typing in selected high-risk drug rechallenge dilemmas (after recovery).
  • Mycoplasma serology / PCR in young patients with mucositis.

Differential diagnosis

  • Erythema multiforme major โ€” true targets; HSV-driven; mucosal involvement; <10% BSA and less severe.
  • Staphylococcal scalded-skin syndrome (SSSS) โ€” paediatric; intraepidermal split; toxin-mediated.
  • Generalised bullous fixed drug eruption โ€” well-demarcated; recurs at same sites; less mucosal.
  • AGEP โ€” pustular; rapid onset; less detachment.
  • Paraneoplastic pemphigus โ€” chronic course; lichenoid component.
  • Acute GVHD โ€” post-HSCT context.
  • Linear IgA disease, autoimmune bullous diseases โ€” direct immunofluorescence.

Management

  • Immediate:
    • Stop all non-essential drugs; identify and discontinue likely culprit (ALDEN).
    • Transfer to specialist burns or critical care unit with multidisciplinary team (dermatology, ophthalmology, ENT, urology, gynaecology, plastics).
    • Supportive care: fluid resuscitation, electrolyte balance, warming, nutrition (NG / parenteral), analgesia, infection prevention.
  • Disease-modifying therapy:
    • Ciclosporin 3-5 mg/kg/day for 10-14 days โ€” emerging UK first-line; reduces mortality.
    • Etanercept 50 mg SC ร— 1-2 doses โ€” RCT (Wang 2018) showed mortality benefit.
    • IVIG (2-3 g/kg total) โ€” mixed evidence; consider in combination.
    • Corticosteroids โ€” controversial; high-dose short-course may be beneficial early; avoid prolonged use.
  • Ophthalmology: daily review; amniotic membrane transplant for severe ocular involvement.
  • Skin care: minimise debridement (leave detached epidermis as biological dressing); silicone-based non-adherent dressings; petrolatum-impregnated gauze.
  • Long-term:
    • Drug allergy MedicAlert; document on EPMA and care record.
    • Avoid first-degree-relative testing for HLA risk unless considering same drug class.
    • Follow-up for ocular sequelae, post-inflammatory pigment change, scarring, psychological impact, BOOP.

References

  1. Bastuji-Garin S et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000;115:149-153.
  2. Sassolas B et al. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson syndrome and toxic epidermal necrolysis. Clin Pharmacol Ther. 2010;88:60-68.
  3. Wang CW et al. Randomized, controlled trial of TNF-ฮฑ antagonist in CTL-mediated severe cutaneous adverse reactions. J Clin Invest. 2018;128:985-996.
  4. British Association of Dermatologists. UK guidelines for the management of Stevens-Johnson syndrome / toxic epidermal necrolysis in adults 2016. Br J Dermatol. 2016;174:1194-1227.
  5. Schneider JA, Cohen PR. Stevens-Johnson syndrome and toxic epidermal necrolysis: a concise review. Adv Ther. 2017;34:1235-1244.

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