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
• Kawasaki's disease
• Stevens-Johnsons syndrome

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 percent of BSA
  • TEN – skin detachment of >30 percent 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

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