Paeds · paediatric-dermatology
Stevens-Johnson syndrome and toxic epidermal necrolysis
Also known as Stevens-Johnson syndrome · SJS · Toxic epidermal necrolysis · TEN · Lyell syndrome · SJS-TEN overlap · Mycoplasma-induced rash and mucositis
Fellowship topic on Stevens-Johnson syndrome (SJS), SJS-TEN overlap and toxic epidermal necrolysis (TEN) in children: a severe, usually drug-induced, T-cell-mediated mucocutaneous reaction causing full-thickness epidermal necrosis and detachment, classified by the percentage of body-surface-area detached (under 10, 10 to 30, over 30); the allopurinol, anticonvulsant, sulfonamide and nevirapine culprits and the HLA-B pharmacogenetic associations including HLA-B*15:02 for carbamazepine; the granulysin, Fas-FasL and perforin-granzyme effector mechanisms; the Mycoplasma-induced rash and mucositis syndrome distinct from drug-induced SJS; the differential from staphylococcal scalded skin syndrome where mucosae are spared; SCORTEN severity scoring with its seven risk factors and mortality bands; and management centred on immediate withdrawal of the culprit drug, burn-unit or PICU supportive care, meticulous eye care, and the lack of proven survival benefit for IVIG, cyclosporine and corticosteroids.
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Target exams
Red flags
- A child with a new drug in the preceding one to eight weeks who develops target lesions, blistering, skin tenderness, sheet-like peeling and mucositis of the eyes, mouth or urogenital tract — suspect SJS or TEN and stop the drug immediately
- Epidermal detachment on more than 10 percent of body surface area, or a positive Nikolsky sign with skin shearing on light pressure — SJS-TEN overlap or TEN; admit to a burn unit or PICU
- Acute ocular involvement — pain, photophobia, conjunctival blistering or corneal erosion — the commonest and most disabling long-term complication; involve ophthalmology within hours
- Tachycardia over 120, extensive detachment, rising urea or low bicarbonate on day one — a high SCORTEN portending mortality of 35 to 90 percent; escalate to intensive care
- Respiratory symptoms with prominent mucositis and sparse skin lesions in a school-age child or adolescent — consider Mycoplasma-induced rash and mucositis, which mimics SJS but is usually drug-independent and carries a better prognosis
Life stages
Care settings
Clinical exam formats
Board mappings
- General Paediatrics and Dermatology: recognition of SJS, SJS-TEN overlap and TEN as a drug-induced mucocutaneous emergency, mucosal involvement, and the need to stop the culprit drug and escalate
- Immunology and Allergy: severe delayed drug hypersensitivity reactions and HLA pharmacogenetic screening
- Renewed curriculum — Dermatology and acute care: SCORTEN severity scoring and burn-unit or PICU supportive care
- Pharmacogenomics: HLA-B*15:02 and carbamazepine, HLA-B*58:01 and allopurinol, pre-prescription screening
- General Paediatrics: recognition and immediate management of the blistering, mucositis-positive child and the principle of stopping the culprit drug
- Critical care and retrieval: triage, SCORTEN, transfer to a burn-capable centre
- Long Case / Structured discussion: a child recovering from SJS-TEN — cause, acute management, ocular and skin sequelae, drug avoidance and family counselling
- Communication station: explaining drug avoidance, the role of immunomodulation uncertainty, and genetic screening to a family
- Level 2 / 3 — Dermatology and Immunology: severe cutaneous adverse drug reactions, SJS/TEN spectrum and mucocutaneous emergency management
- Critical care and safe prescribing: high-risk medicines, HLA screening and medication safety
- Foundation of Practice (FOP): clinical recognition of SJS and TEN, common culprit drugs and the mucosal sign
- Applied Knowledge in Practice (AKP): SCORTEN risk factors, supportive care priorities and the evidence on immunomodulation
- History-taking and management: the drug-exposed child with target lesions and mucositis
- Communication: explaining drug avoidance, ocular follow-up and family screening
- General Pediatrics Content Outline — SJS and TEN: recognition, culprit drugs, supportive care and complications
- Allergy and Immunology: severe delayed drug hypersensitivity and HLA pharmacogenetics
- Dermatology and Critical Care: the blistering mucocutaneous emergency and burn-unit management
- Patient Care: recognition, withdrawal of the culprit drug, severity scoring and burn-unit supportive care of SJS and TEN
- Systems-Based Practice and Patient Safety: medication safety, HLA screening and care coordination across dermatology, ophthalmology and critical care
- Medical Knowledge: T-cell-mediated pathophysiology, high-risk drugs, SCORTEN and the ocular complication
- Medical Expert: SJS and TEN diagnosis, severity scoring, supportive care and sequelae
- Collaborator and Health Advocate: coordinated care across burn units, ophthalmology and pharmacogenetic services
- Communicator: explaining drug avoidance, genetic screening and long-term follow-up to families
Two blistering emergencies — SJS/TEN versus staphylococcal scalded skin syndrome
SJS / TEN (drug-induced)
Staphylococcal scalded skin syndrome
SCORTEN is calculated within the first 24 hours (re-check at day 3). Give one point for each of seven independent risk factors: age over 40 years, active malignancy, heart rate over 120 beats per minute, initial epidermal detachment over 10 percent of body surface area, serum urea over 10 mmol per litre, serum bicarbonate under 20 mmol per litre, and serum glucose over 14 mmol per litre. The mortality ladder rises steeply: a score of 0 to 1 means about 3 percent mortality, 2 means 12 percent, 3 means 36 percent, 4 means 58 percent, and 5 or more means 90 percent. In a child, the age and malignancy points are usually absent, so a climbing score reflects tachycardia, detachment extent and renal or metabolic derangement — the markers that demand the burn unit. [1] [8]
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]
References12ShowHide
- [1]Bastuji-Garin S; Fouchard N; Bertocchi M; Roujeau JC; et al SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol, 2000.PMID 10951229
- [2]Levi N; Bastuji-Garin S; Mockenhaupt M; Roujeau JC; et al Medications as risk factors of Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a pooled analysis. Pediatrics, 2009.PMID 19153164
- [3]Lonjou C; Borot N; Sekula P; Ledger N; et al A European study of HLA-B in Stevens-Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs. Pharmacogenet Genomics, 2008.PMID 18192896
- [4]Chung WH; Hung SI; Hong HS; Hsih MS; et al Medical genetics: a marker for Stevens-Johnson syndrome. Nature, 2004.PMID 15057820
- [5]Canavan TN; Mathes EF; Frieden I; Shinkai K Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review. J Am Acad Dermatol, 2015.PMID 25592340
- [6]Halevy S; Ghislain PD; Mockenhaupt M; Fagot JP; et al Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol, 2008.PMID 17919772
- [7]Iriarte C; Karim SA; Nassim JS; Grenier PO; et al Infantile Stevens Johnson syndrome and toxic epidermal necrolysis: A systematic review of clinical features and outcomes in children ages 12 months and under. Pediatr Dermatol, 2022.PMID 35676891
- [8]Sekula P; Liss Y; Davidovici B; Dunant A; et al Evaluation of SCORTEN on a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis included in the RegiSCAR study. J Burn Care Res, 2011.PMID 21228709
- [9]Zimmermann S; Sekula P; Venhoff M; Motschall E; et al Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol, 2017.PMID 28329382
- [10]AlFada M; Alotaibi H; Alsharif S; Alani AH; et al Systematic review, methodological appraisal, and recommendation mapping of clinical practice guidelines for managing patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatolog Treat, 2025.PMID 40010698
- [11]Novack DE; Braskett M; Worswick SD; Adler BL Drug patch testing in Stevens-Johnson syndrome and toxic epidermal necrolysis: A systematic review. Ann Allergy Asthma Immunol, 2023.PMID 36649833
- [12]Chung WH; Hung SI Recent advances in the genetics and immunology of Stevens-Johnson syndrome and toxic epidermal necrosis. J Dermatol Sci, 2012.PMID 22541332