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Dermatology (Skin Conditions)

Stevens-Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN)

Severe, life-threatening mucocutaneous hypersensitivity reactions, usually triggered by medications, characterized by extensive necrosis and detachment of the epidermis. SJS involves <10% body surface area (BSA) detachment, SJS/TEN overlap 10-30%, TEN >30%.

Also known as: SJS, TEN, Lyell's Syndrome

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Referral and warning signs

When to refer

IMMEDIATE URGENT REFERRAL/TRANSFER to Hospital (H/RR) with capacity for intensive care / burns management / specialist dermatology input for ALL suspected cases of SJS/TEN.

Red flags

  • Rapidly progressing widespread rash with blistering/detachment.
  • Positive Nikolsky's sign.
  • Severe mucosal involvement (eyes, mouth, genitals).
  • Fever, systemic toxicity.
  • Significant BSA involvement (>10%).

Signs and symptoms

  • Prodrome: Fever, malaise, headache, cough, sore throat, conjunctivitis (1-14 days before rash).
  • Skin Eruption:
  • - Starts as diffuse erythema (redness) or macular rash (may be target-like initially).
  • - Rapid progression to blistering and full-thickness epidermal detachment ('Nikolsky's sign' positive - slight rubbing causes skin to separate).
  • - Widespread painful, denuded areas resembling burns.
  • Mucosal Involvement (Almost always present, often severe):
  • - Ocular: Severe conjunctivitis, pseudomembranes, corneal erosions/ulcers, symblepharon (adhesions).
  • - Oral/Pharyngeal: Painful erosions, crusting on lips, stomatitis, difficulty eating/drinking.
  • - Genital/Anal: Erosions, pain.
  • Systemic Symptoms: High fever, tachycardia, potential for sepsis, dehydration, electrolyte imbalance, organ involvement (liver, kidney, lung).

Risk factors

  • Medications (Most common cause):
  • - High Risk: Allopurinol, Lamotrigine, Carbamazepine, Phenobarbital, Phenytoin, Sulfonamides (esp. Cotrimoxazole), Nevirapine, NSAIDs (oxicam type).
  • - Onset typically 1-3 weeks after starting new drug (can be longer).
  • Infections (Less common trigger, esp. in children): Mycoplasma pneumoniae, Herpes Simplex Virus (HSV).
  • Genetic predisposition (certain HLA types associated with specific drug reactions).
  • HIV infection (higher risk, esp. with sulfonamides/nevirapine).
  • Malignancy.
  • Systemic Lupus Erythematosus (SLE).

Diagnosis

Clinical assessment

Diagnosis based on characteristic rapidly progressive mucocutaneous eruption with epidermal detachment, often with history of recent new medication exposure. Assess BSA involvement to differentiate SJS/TEN.

Differential diagnosis

  • Staphylococcal Scalded Skin Syndrome (SSSS) (usually young children, superficial split, less mucosal involvement, no drug cause).
  • Erythema Multiforme Major (target lesions prominent, often infection-triggered, less detachment).
  • Pemphigus / Pemphigoid (autoimmune blistering, usually no necrosis/detachment).
  • Severe drug eruptions (AGEP, DRESS) - different morphology.
  • Thermal burns.
  • Graft-versus-Host Disease (GVHD).

Investigations

  • Skin Biopsy (Urgent): Shows full-thickness epidermal necrosis and dermo-epidermal separation. Helps differentiate from SSSS/autoimmune blistering. (Specialist).
  • Identify and STOP Suspected Causative Drug(s) IMMEDIATELY.
  • Blood tests: FBC (anaemia, neutropenia), Urea/Electrolytes/Creatinine (assess hydration/renal function), LFTs, Blood cultures (if sepsis suspected), Glucose.
  • Chest X-ray (if respiratory involvement).
  • Severity Scoring (e.g., SCORTEN) to predict prognosis.

Diagnostic criteria

Clinical presentation + History of drug exposure +/- Skin biopsy confirmation.

Management

Non-pharmacological management

  • IMMEDIATE CESSATION of suspected causative drug(s).
  • Refer URGENTLY to specialized unit (Burns Unit / ICU / Dermatology High Dependency). Management is supportive and multidisciplinary.
  • Skin Care: Gentle handling, minimize trauma. Non-adherent dressings (paraffin gauze, silicone) or biological dressings. Keep warm (prevent hypothermia).
  • Fluid & Electrolyte Management: Aggressive IV fluid resuscitation (similar to burns), monitor urine output, replace electrolytes.
  • Nutritional Support: Often requires NGT or parenteral nutrition due to painful oral/oesophageal involvement.
  • Eye Care: URGENT Ophthalmology input. Frequent lubrication (preservative-free artificial tears), lysis of adhesions, topical antibiotics/steroids as directed by ophthalmologist.
  • Mouth Care: Gentle cleansing, antiseptic/analgesic mouthwashes.
  • Pain Management: Opioids often required.
  • Infection Control: Barrier nursing, monitor for sepsis.
  • Temperature Regulation.

Pharmacological treatment

  • No proven specific disease-modifying therapy.
  • Systemic Corticosteroids: CONTROVERSIAL. Some use early high-dose, others avoid due to infection risk. No clear evidence of benefit, may increase mortality.
  • Intravenous Immunoglobulin (IVIG): CONTROVERSIAL. May halt progression if given early (specialist decision).
  • Ciclosporin: Emerging evidence, specialist use.
  • Antibiotics: Prophylactic use controversial. Treat documented infections based on cultures.
  • Anticoagulation: Prophylaxis for DVT (e.g., LMWH) due to immobility.

Monitoring

Close monitoring of vital signs, fluid balance, electrolytes, renal/liver function, BSA detachment, signs of sepsis, eye involvement, nutritional status in specialized unit.

Complications

  • Sepsis (major cause of death).
  • Dehydration / Electrolyte imbalance / Acute Kidney Injury.
  • Respiratory failure (mucosal sloughing, ARDS).
  • Gastrointestinal involvement (diarrhoea, bleeding).
  • Severe ocular complications (corneal scarring/perforation, dry eye, symblepharon, blindness).
  • Skin scarring, pigmentary changes, nail loss.
  • Strictures (oesophageal, vaginal, urethral).
  • Long-term psychological sequelae.
  • High mortality rate (SJS ~10%, TEN >30%).

Prevention

  • Avoid re-exposure to causative drug(s) - LIFELONG avoidance.
  • Careful drug history taking.
  • Awareness of high-risk drugs.
  • Genetic screening for certain HLA types before starting specific drugs (e.g., HLA-B*1502 before Carbamazepine in some Asian populations) - not widely available.
  • Patient education on early signs of severe reactions.

Patient education

  • (After recovery):
  • Clear identification of the causative drug(s) to be avoided lifelong.
  • Importance of informing ALL future healthcare providers about the reaction.
  • Wearing medical alert identification.
  • Information about potential long-term complications (skin, eyes).
  • Need for long-term ophthalmology follow-up if eyes involved.
  • Psychological support resources.

Uganda-specific notes

SJS/TEN seen, often related to Cotrimoxazole, Nevirapine, Anticonvulsants, Allopurinol. Management capacity in specialized units (burns/ICU) is extremely limited. Focus on early recognition, stopping culprit drug, supportive care (fluids, nutrition, basic wound care), and referral if possible. High mortality.

References

  • Uganda Clinical Guidelines (UCG) 2023

    Chapter 22, Section 22.7.1

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