Toxic epidermal necrolysis

Background

• Explosive dermatosis with tender erythema, bullae, and subsequent exfolliation
• Most commonly caused by medications
  • Sulfa drugs, penicillins, anticonvulsants, and NSAIDs
  • Other causes: infection, chemicals, malignancy, immunologic factors

Clinical Features

• Malaise, anorexia, myalgias, arthralgias, fever, painful skin, GI symptoms
• Extracutaneous manifestations may persist for 1-2 weeks following skin symptoms
• Exam with warm tender erythema with overlying flaccid bullae, erosions with exfoliation
• Positive Nikolskly's sign (able to rub off superficial layers of skin with pressure)
• Mucosal involvement (oral, conjunctival, respiratory, GU)
• Systemic toxicity
• 25-35% Mortality
  • Death is usually caused by infection, hypovolemia, and electrolyte disorders
• Predictors of poor prognosis include: age, extent of disease, leukopenia, azotemia, and thrombocytopenia

Differential Diagnosis

• Toxic infectious erythemas
• Exfoliative drug eruptions
• Primary blistering disorders
• Stevens-Johnsons syndrome

Erythematous rash

• Positive Nikolsky’s sign
  • Febrile
    • Staphylococcal scalded skin syndrome (children)
    • Toxic epidermal necrolysis/SJS (adults)
  • Afebrile
    • Toxic epidermal necrolysis
• Negative Nikolsky’s sign
  • Febrile
    • Toxic shock syndrome
    • Kawasaki disease
    • Scarlet fever
    • DRESS syndrome
    • Cellulitis
    • Acute generalized exanthematous pustulosis
  • Afebrile
    • Alcohol intoxication
    • Anaphylaxis
    • Cellulitis
    • Dermatitis (multiple types)
    • Drug rash
    • Erysipelas
    • Erythema multiforme
    • Exfoliative erythroderma
    • Scombroid
    • Vancomycin infusion reaction

Evaluation

• History of drug exposure
• Prodrome of malaise and fever
• Positive Nikolsky sign
• Oral, ocular, and/or genital mucositis with painful erosions
• Necrosis and sloughing of the epidermis

• Diagnosis is made my skin biopsy

• SJS vs TEN
  • SJS - skin detachment of <10% of BSA
  • TEN – skin detachment of >30% of BSA

Management

• Monitor cardiopulmonary status closely
• Correct fluid and electrolyte imbalances
• Attend to infectious complications

Disposition

• ICU
• Best cared for in a burn unit
• Immediate derm consult

References

<UpToDate>