|  | Conditions & Treatments | Neurological Disorders

Steven-Johnson Syndrome (SJS): Causes, Symptoms & Treatment

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a single disease spectrum of severe, immune-mediated cutaneous adverse reactions (SCARs), distinguished by body surface area (BSA) detachment: SJS <10%, SJS/TEN overlap 10–30%, TEN >30%. Mortality: SJS ~5%, TEN ~30%.

Pathophysiology

  • Drug-specific T-cell response (CD8+ cytotoxic) → granulysin, perforin, Fas-FasL → massive keratinocyte apoptosis.
  • Genetic risk: HLA-B*15:02 (carbamazepine, oxcarbazepine, phenytoin in Asians); HLA-B*58:01 (allopurinol in Han Chinese, Thai, Koreans); HLA-A*31:01 (carbamazepine in Europeans/Japanese).

Common Culprit Drugs

  • Anticonvulsants: Carbamazepine, lamotrigine, phenytoin, phenobarbital, valproate.
  • Antibiotics: Sulfonamides (TMP-SMX), penicillins, cephalosporins, quinolones.
  • NSAIDs: Oxicams (piroxicam, meloxicam), others.
  • Allopurinol (gout) — high risk in renal impairment, HLA-B*58:01+.
  • Antiretrovirals: Nevirapine, abacavir (HLA-B*57:01).

Onset typically 4–28 days after drug initiation (longer for anticonvulsants).

Clinical Features

  • Prodrome (1–3 days): Fever, malaise, sore throat, cough, myalgia.
  • Mucosal involvement (≥2 sites): Oral (erosions, hemorrhage), ocular (conjunctivitis, pseudomembranes), genital, anal.
  • Skin: Targetoid macules → flaccid blisters → sheet-like epidermal detachment (Nikolsky sign +). Face, trunk, proximal extremities.
  • Systemic: Fever, hypotension, sepsis, multi-organ failure (hepatic, renal, pulmonary).

Diagnosis

  • Clinical (mucosal + cutaneous detachment).
  • Skin biopsy: Full-thickness epidermal necrosis, sparse dermal infiltrate, satellite cell necrosis.
  • SCORTEN (TEN severity score): Age >40, HR >120, BSA >10%, bicarbonate <20, BUN >28, glucose >252, malignancy → predicts mortality.

Emergency Management

  1. STOP the culprit drug immediately — single most critical step.
  2. Refer to burn unit / ICU — fluid/electrolyte management, wound care, nutritional support, infection surveillance.
  3. Supportive care: Ophthalmology consult (daily) — prevent synechiae, blindness. Pain control. VTE prophylaxis. Stress ulcer prophylaxis.
  4. Immunomodulatory therapy (controversial, no RCT proof):
    • IVIG 1–3 g/kg (early, within 48–72 hr).
    • Cyclosporine 3–5 mg/kg/d.
    • Etanercept (TNF-α inhibitor) — emerging evidence.
    • Avoid systemic steroids (infection risk, delayed healing) — except possibly early, short-course in SJS.

Long-Term Sequelae

  • Ocular: Dry eye, symblepharon, corneal scarring, blindness.
  • Mucosal: Stenosis (esophageal, urethral, vaginal).
  • Skin: Dyspigmentation, scarring, alopecia.
  • Psychological: PTSD, depression.

SJS/TEN is a dermatologic emergency. Early drug cessation and specialized supportive care determine survival.

Neurological Disorders - Related Articles