← Back to dossier
Patient handout

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

PRODUCTION

1. Your condition

This handout is for stevens-johnson syndrome / toxic epidermal necrolysis (sjs/ten). Your care team identified this based on: painful dusky macules / atypical targets → flaccid bullae + ≥2 mucosal sites (bastuji-garin classification, arch dermatol 1993; pmid 8420497).

Other reasons your team may use this plan: 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); positive nikolsky sign + sheet-like epidermal detachment (bastuji-garin classification; pmid 8420497).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
discontinue probable culprit + all non-essential drugsn/aGarcia-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)IVCreamer 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)topical/supportiveCreamer UK guideline (PMID 27317286) — supportive care is the only universally agreed intervention; infection surveillance not prophylaxis; early ophthalmology

Plan: SJS/TEN management — culprit withdrawal + supportive cornerstone ± contested immunomodulation

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • 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)
  • Acute conjunctival injection, pseudomembrane, epithelial defect, or limbal involvement (Creamer UK guideline; PMID 27317286)
  • 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)(life-threatening)
  • qSOFA ≥2 / NEWS2 high / hypotension on adequate fluids with extensive skin loss (Creamer UK guideline; PMID 27317286)(life-threatening)
  • Rapid expansion of detachment over hours (e.g., SJS → TEN within 24–48 h) (Bastuji-Garin; PMID 8420497)(life-threatening)
  • 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)
  • HIV-positive host presenting with SCAR (esp. on sulfonamide / nevirapine) (Mittmann, Br J Dermatol 2012; PMID 22145749)
  • 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)

5. Follow-up

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)

6. Sources

Guideline: 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

  1. pubmed.ncbi.nlm.nih.gov/27317286
  2. pubmed.ncbi.nlm.nih.gov/8420497
  3. pubmed.ncbi.nlm.nih.gov/10951229