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derm.sjs-ten.core.v1PRODUCTION
derm.sjs-ten.core.v1

Stevens-Johnson syndrome / toxic epidermal necrolysis (SJS/TEN)

dermatologyacuteadultpediatric
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Position on the SJS↔TEN continuum by %BSA epidermal detachment (SJS <10%, overlap 10–30%, TEN >30%); SJS/TEN is one disease, EM-major/SSSS/autoimmune-bullous are distinct (Bastuji-Garin consensus classification, Arch Dermatol 1993; PMID 8420497)

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spectrum position framed; scope is drug-induced SCAR continuum

Patient inputs (18)

Lesional skin biopsy — full-thickness epidermal necrosis + DIF-negative confirms SJS/TEN and excludes SSSS (intraepidermal) and autoimmune bullous (DIF+) (Bastuji-Garin; PMID 8420497; Creamer; PMID 27317286)

ALDEN causality scoring — identify single most-probable culprit by latency 4–28 d (Sassolas ALDEN, Clin Pharmacol Ther 2010; PMID 20375998)

Pyrexia from extensive skin loss vs sepsis; thermoregulation in denuded skin (Creamer UK guideline; PMID 27317286)

≥2 mucosal sites is a SJS/TEN-defining feature and differentiates from SSSS (mucosa spared) (Bastuji-Garin classification; PMID 8420497)

Epidermal-detachment %BSA defines spectrum tier (SJS <10 / overlap 10–30 / TEN >30) and is a SCORTEN variable (Bastuji-Garin; SCORTEN, J Invest Dermatol 2000; PMID 10951229)

BUN >10 mmol/L is a SCORTEN variable (SCORTEN, J Invest Dermatol 2000; PMID 10951229)

Serum bicarbonate <20 mmol/L is a SCORTEN variable (SCORTEN; PMID 10951229)

Glucose >14 mmol/L is a SCORTEN variable (SCORTEN; PMID 10951229)

Baseline + serial CBC for infection surveillance (NO prophylactic antibiotics — culture-directed only) (Creamer UK guideline; PMID 27317286)

Acute ocular-surface involvement mandates EMERGENT ophthalmology (amniotic-membrane window <7 d) (Creamer UK guideline; PMID 27317286)

Hypotension → distributive/hypovolemic shock from denuded skin → sepsis pathway (Creamer UK guideline; PMID 27317286)

HR >120 is a SCORTEN variable; tachycardia signals sepsis/hypovolemia from skin loss (SCORTEN, J Invest Dermatol 2000; PMID 10951229)

Renal function gates cyclosporine dosing/contraindication and informs ABCD-10 (dialysis) (González-Herrada, J Invest Dermatol 2017; PMID 28634032; Noe ABCD-10, JAMA Dermatol 2019; PMID 30840032)

Transaminitis flags DRESS overlap (eosinophilia + organ involvement) vs pure SJS/TEN (Mockenhaupt EuroSCAR; PMID 17805350)

HIV raises SJS/TEN incidence ~100-fold (esp. sulfonamides, nevirapine) (Mittmann, Br J Dermatol 2012; PMID 22145749)

Prior SCAR or culprit-class exposure → never re-expose; informs ALDEN (Sassolas ALDEN; PMID 20375998)

HLA-B*15:02 (carbamazepine/aromatic AEDs) and HLA-B*58:01 (allopurinol) are strong genetic risk markers (Chung Nature 2004 PMID 15057820; Hung PNAS 2005 PMID 15743917; CPIC PMID 29392710)

Pregnancy alters immunomodulator choice (cyclosporine acceptable; avoid etanercept unless benefit outweighs) (Creamer UK guideline; PMID 27317286)

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Severity triggers (9)

9 need judgement
  • informationallife_threateningsheet_epidermal_detachment_or_positive_nikolsky
    Sheet-like flaccid bullae / positive Nikolsky / >10% BSA epidermal detachment (Bastuji-Garin; PMID 8420497)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningairway_mucosal_sloughing
    Airway mucosal sloughing, hoarseness, stridor, or respiratory distress (Creamer UK guideline; PMID 27317286)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningscorten_ge_3_high_mortality
    SCORTEN ≥3 (or ABCD-10 ≥3) at admission or day-3 — predicted mortality ≥35% (SCORTEN, J Invest Dermatol 2000; PMID 10951229; Guégan day-3; PMID 16374461)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsepsis_from_denuded_skin
    qSOFA ≥2 / NEWS2 high / hypotension on adequate fluids with extensive skin loss (Creamer UK guideline; PMID 27317286)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningrapidly_progressive_detachment
    Rapid expansion of detachment over hours (e.g., SJS → TEN within 24–48 h) (Bastuji-Garin; PMID 8420497)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereacute_ocular_surface_involvement
    Acute conjunctival injection, pseudomembrane, epithelial defect, or limbal involvement (Creamer UK guideline; PMID 27317286)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehigh_risk_culprit_or_pharmacogenomic_match
    Allopurinol / aromatic anticonvulsant / sulfonamide / nevirapine / oxicam NSAID culprit, OR HLA-B*15:02 (CBZ) / HLA-B*58:01 (allopurinol) positive (Mockenhaupt EuroSCAR; PMID 17805350; Chung Nature 2004; PMID 15057820; Hung PNAS 2005; PMID 15743917)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehiv_host_with_scar
    HIV-positive host presenting with SCAR (esp. on sulfonamide / nevirapine) (Mittmann, Br J Dermatol 2012; PMID 22145749)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepediatric_blistering_rash_vs_ssss_or_mirm
    Child with blistering rash — must distinguish SJS/TEN from SSSS (mucosa spared, superficial split) and MIRM (mucosa-predominant, post-Mycoplasma) before immunomodulation (Canavan MIRM, J Am Acad Dermatol 2015; PMID 25592340)
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

SJS/TEN management — culprit withdrawal + supportive cornerstone ± contested immunomodulation
axis: sjsten_managementstep 1 - Withdraw culprit + supportive critical-care cornerstone
Selected step "Withdraw culprit + supportive critical-care cornerstone" — ALL patients, day 0 (universal backbone — the single highest-impact intervention)
  • discontinue probable culprit + all non-essential drugs
    first line
    culprit_withdrawal
    n/a
    Garcia-Doval Arch Dermatol 2000 (PMID 10724193) — early withdrawal lowers mortality; delayed withdrawal of long-half-life culprits raises death OR ~1.9/day; ALDEN-highest culprit (Sassolas; PMID 20375998)
  • isotonic crystalloid resuscitation (titrate to urine output; ~⅔ of equivalent-burn formula)
    first line
    supportive_fluid
    IV
    Creamer UK guideline (PMID 27317286) — TEN insensible loss is lower than thermal burn; titrate to UOP 0.5–1 mL/kg/h, avoid over-resuscitation
  • non-adherent wound care + temperature/airway/ocular protection + analgesia + nutrition (NO prophylactic systemic antibiotics)
    first line
    supportive_bundle
    topical/supportive
    Creamer UK guideline (PMID 27317286) — supportive care is the only universally agreed intervention; infection surveillance not prophylaxis; early ophthalmology

ed playbook — drug actions (4)

  1. 1. STOP probable culprit + all non-essential drugs
    n/a • n/a • immediate
    trigger: SCAR suspected — do NOT wait for biopsy (Garcia-Doval; PMID 10724193)
    Earliest possible withdrawal is the single highest-impact intervention; delay raises mortality
  2. 2. isotonic crystalloid resuscitation
    titrate to UOP 0.5–1 mL/kg/h (~⅔ equivalent-burn formula) • IV • continuous
    trigger: Extensive detachment / hypovolemia (Creamer UK guideline; PMID 27317286)
    TEN insensible loss lower than thermal burn — avoid over-resuscitation
  3. 3. analgesia (opioid titration) + non-adherent dressings
    titrate to pain • IV/topical • continuous
    trigger: Painful denuded skin (Creamer UK guideline; PMID 27317286)
    Wound-care + analgesia are supportive cornerstones; NO prophylactic systemic antibiotics
  4. 4. lubricating/preservative-free ocular care + EMERGENT ophthalmology
    frequent • topical • q1-2h
    trigger: Any ocular involvement (Creamer UK guideline; PMID 27317286)
    Amniotic-membrane window <7 d for best long-term visual outcome

Auto-drafted A&P note

ed

Subjective

- Possible entry pathways: Painful dusky macules / atypical targets → flaccid bullae + ≥2 mucosal sites (Bastuji-Garin classification, Arch Dermatol 1993; PMID 8420497); New high-risk drug (allopurinol, aromatic anticonvulsant, sulfonamide, nevirapine, oxicam NSAID) started 4–28 d prior (Sassolas ALDEN, Clin Pharmacol Ther 2010; PMID 20375998; Mockenhaupt EuroSCAR, PMID 17805350); Prodromal fever / odynophagia / photophobia 1–3 d before rash + oral/ocular/genital erosions (Creamer UK guideline, Br J Dermatol 2016; PMID 27317286).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Stevens-Johnson syndrome / toxic epidermal necrolysis (SJS/TEN)** (derm.sjs-ten.core.v1).
Phenotype framing: SJS/TEN vs DRESS vs AGEP vs erythema multiforme major vs SSSS vs autoimmune bullous (pemphigus/bullous pemphigoid/paraneoplastic) vs acute GVHD vs generalized bullous fixed drug eruption vs MIRM (pediatric) (Mockenhaupt EuroSCAR; PMID 17805350; Canavan MIRM, J Am Acad Dermatol 2015; PMID 25592340)
Scope: Position on the SJS↔TEN continuum by %BSA epidermal detachment (SJS <10%, overlap 10–30%, TEN >30%); SJS/TEN is one disease, EM-major/SSSS/autoimmune-bullous are distinct (Bastuji-Garin consensus classification, Arch Dermatol 1993; PMID 8420497)

No severity triggers fired against current inputs.

Plan

Regimen axis: **SJS/TEN management — culprit withdrawal + supportive cornerstone ± contested immunomodulation** — step "Withdraw culprit + supportive critical-care cornerstone".
1. discontinue probable culprit + all non-essential drugs n/a (culprit_withdrawal, first line) — Garcia-Doval Arch Dermatol 2000 (PMID 10724193) — early withdrawal lowers mortality; delayed withdrawal of long-half-life culprits raises death OR ~1.9/day; ALDEN-highest culprit (Sassolas; PMID 20375998)
2. isotonic crystalloid resuscitation (titrate to urine output; ~⅔ of equivalent-burn formula) IV (supportive_fluid, first line) — Creamer UK guideline (PMID 27317286) — TEN insensible loss is lower than thermal burn; titrate to UOP 0.5–1 mL/kg/h, avoid over-resuscitation
3. non-adherent wound care + temperature/airway/ocular protection + analgesia + nutrition (NO prophylactic systemic antibiotics) topical/supportive (supportive_bundle, first line) — Creamer UK guideline (PMID 27317286) — supportive care is the only universally agreed intervention; infection surveillance not prophylaxis; early ophthalmology

Setting playbook (ed) — Recognize SCAR, score SCORTEN/ABCD-10, STOP the probable culprit immediately, initiate supportive resuscitation, and place biopsy + burn-unit/ophthalmology referrals (Creamer UK guideline; PMID 27317286; Garcia-Doval; PMID 10724193)
4. STOP probable culprit + all non-essential drugs n/a n/a immediate — SCAR suspected — do NOT wait for biopsy (Garcia-Doval; PMID 10724193) (Earliest possible withdrawal is the single highest-impact intervention; delay raises mortality)
5. isotonic crystalloid resuscitation titrate to UOP 0.5–1 mL/kg/h (~⅔ equivalent-burn formula) IV continuous — Extensive detachment / hypovolemia (Creamer UK guideline; PMID 27317286) (TEN insensible loss lower than thermal burn — avoid over-resuscitation)
6. analgesia (opioid titration) + non-adherent dressings titrate to pain IV/topical continuous — Painful denuded skin (Creamer UK guideline; PMID 27317286) (Wound-care + analgesia are supportive cornerstones; NO prophylactic systemic antibiotics)
7. lubricating/preservative-free ocular care + EMERGENT ophthalmology frequent topical q1-2h — Any ocular involvement (Creamer UK guideline; PMID 27317286) (Amniotic-membrane window <7 d for best long-term visual outcome)

Non-pharmacologic actions:
- Lesional + perilesional skin biopsy (frozen H&E for rapid full-thickness-necrosis read + DIF) (Creamer UK guideline; PMID 27317286)
- Burn-unit / ICU referral if >10% BSA or rapid progression (Palmieri; PMID 11882797)
- Multidisciplinary alert: dermatology, ophthalmology, urology/gynecology, ICU/burns (Creamer UK guideline; PMID 27317286)

AVOID / contraindication checks:
- Cyclosporine avoid if severe renal impairment or uncontrolled htn (González Herrada; PMID 28634032)
- Cyclosporine avoid if active serious infection (Creamer UK guideline; PMID 27317286)
- Etanercept avoid if active infection or latent tb (Wang RCT; PMID 29400697)
- Corticosteroid short pulse only prolonged course harmful (Zimmermann meta analysis; PMID 28329382)
- Never re expose culprit or cross reacting class (Sassolas ALDEN; PMID 20375998; CPIC HLA B; PMID 29392710)
- No prophylactic systemic antibiotics culture directed only (Creamer UK guideline; PMID 27317286)
- Pregnancy cyclosporine acceptable avoid etanercept unless benefit outweighs (Creamer UK guideline; PMID 27317286)
- Hla b 1502 screen before carbamazepine aromatic aeds hla b 5801 before allopurinol (Chung Nature 2004 PMID 15057820; Hung PNAS 2005 PMID 15743917; CPIC PMID 29392710)

Monitoring

Regimen monitoring:
- daily %BSA and re-epithelialization (Creamer UK guideline; PMID 27317286)
- fluid electrolyte glucose q6-12h (Creamer UK guideline; PMID 27317286)
- SCORTEN day-1 and day-3 recompute (SCORTEN PMID 10951229; Guégan PMID 16374461)
- cyclosporine trough + renal function during therapy (González-Herrada; PMID 28634032)
- infection surveillance blood and skin cultures (NOT prophylactic) (Creamer UK guideline; PMID 27317286)
- ophthalmology review q24h while acute (Creamer UK guideline; PMID 27317286)

Setting (ed) monitoring:
- Vitals + UOP q1h (Creamer UK guideline; PMID 27317286)
- Reassess %BSA + new mucosal involvement q4-6h (Bastuji-Garin; PMID 8420497)

Follow-up plan: Ocular (symblepharon, dry eye, vision loss), cutaneous (dyspigmentation, scarring), oral, genital (synechiae), pulmonary (bronchiolitis obliterans) sequelae screen; psychological support; HLA pharmacogenomic counseling + lifelong culprit-class + cross-reactor avoidance card (Creamer UK guideline; PMID 27317286; CPIC HLA-B guideline, Clin Pharmacol Ther 2018; PMID 29392710)
- Close-out criterion: sequelae plan + pharmacogenomic avoidance card finalized

Monitoring phase: Daily %BSA + re-epithelialization, fluid/electrolyte/glucose, infection surveillance (culture-directed, NOT prophylactic), day-3 SCORTEN recompute, ophthalmology review q24h while acute (Creamer UK guideline; PMID 27317286; Guégan; PMID 16374461)

Disposition

Current setting: ed — Recognize SCAR, score SCORTEN/ABCD-10, STOP the probable culprit immediately, initiate supportive resuscitation, and place biopsy + burn-unit/ophthalmology referrals (Creamer UK guideline; PMID 27317286; Garcia-Doval; PMID 10724193)

Disposition criteria:
- Admit ALL — SJS/TEN is never managed outpatient (Creamer UK guideline; PMID 27317286)
- Burn unit / ICU if >10% BSA, SCORTEN ≥2, or rapid progression (Palmieri PMID 11882797; SCORTEN PMID 10951229)
- Dermatology ward (with critical-care input) only if limited SJS, SCORTEN 0–1, stable (Creamer UK guideline; PMID 27317286)

Escalation triggers (move to higher acuity):
- Detachment >10% BSA or rapid progression → burn unit / ICU (Palmieri; PMID 11882797)
- Airway mucosal sloughing / stridor → early bronchoscopy + airway protection (Creamer UK guideline; PMID 27317286)
- Sepsis physiology → protocol.septic_shock / id.sepsis.core.v1 (Creamer UK guideline; PMID 27317286)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Sheet-like flaccid bullae / positive Nikolsky / >10% BSA epidermal detachment (Bastuji-Garin; PMID 8420497)
- [LIFE_THREATENING] Airway mucosal sloughing, hoarseness, stridor, or respiratory distress (Creamer UK guideline; PMID 27317286)
- [LIFE_THREATENING] SCORTEN ≥3 (or ABCD-10 ≥3) at admission or day-3 — predicted mortality ≥35% (SCORTEN, J Invest Dermatol 2000; PMID 10951229; Guégan day-3; PMID 16374461)

Citations

- Creamer UK BAD/BBA SJS/TEN management guideline (Br J Dermatol 2016) + Bastuji-Garin consensus classification (Arch Dermatol 1993) + SCORTEN / day-3 SCORTEN / ABCD-10 prognostication + Sassolas ALDEN drug causality + Mockenhaupt EuroSCAR culprit registry + Garcia-Doval early-withdrawal mortality + Zimmermann/González-Herrada/Wang/Barron contested-immunomodulation comparative-mortality literature + CPIC HLA-B pharmacogenomics [PMID:27317286](https://pubmed.ncbi.nlm.nih.gov/27317286/)
- Cited evidence (PMID 8420497) [PMID:8420497](https://pubmed.ncbi.nlm.nih.gov/8420497/)
- Cited evidence (PMID 10951229) [PMID:10951229](https://pubmed.ncbi.nlm.nih.gov/10951229/)
- Cited evidence (PMID 16374461) [PMID:16374461](https://pubmed.ncbi.nlm.nih.gov/16374461/)
- Cited evidence (PMID 30840032) [PMID:30840032](https://pubmed.ncbi.nlm.nih.gov/30840032/)

Last reconciled with current guidelines: 2026-05-22.
References
  • Creamer UK BAD/BBA SJS/TEN management guideline (Br J Dermatol 2016) + Bastuji-Garin consensus classification (Arch Dermatol 1993) + SCORTEN / day-3 SCORTEN / ABCD-10 prognostication + Sassolas ALDEN drug causality + Mockenhaupt EuroSCAR culprit registry + Garcia-Doval early-withdrawal mortality + Zimmermann/González-Herrada/Wang/Barron contested-immunomodulation comparative-mortality literature + CPIC HLA-B pharmacogenomics — PMID:27317286
  • Cited evidence (PMID 8420497) — PMID:8420497
  • Cited evidence (PMID 10951229) — PMID:10951229
  • Cited evidence (PMID 16374461) — PMID:16374461
  • Cited evidence (PMID 30840032) — PMID:30840032