Stevens-Johnson syndrome and toxic epidermal necrolysis

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Stevens-Johnson syndrome and toxic epidermal necrolysis

• Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. 
  
• In SJS, there is typically between 1 to 10% of skin detachment.
  
• When skin detachment ranges between 10 to 30%, it is considered an overlap between SJS and TEN.
  
• TEN is characterized by more than 30% skin detachment.
  
• Drugs, especially sulfa drugs, antiseizure drugs, and antibiotics, are the most common causes. 
  

• SJS and TEN typically begin with a prodrome of fever, malaise, and headache. A rash then develops, most commonly on the face, upper torso, palms, and soles. The rash initially resembles target lesions, but it soon coalesces into large blisters. Nikolsky's sign, which refers to the easy detachment of the epidermis upon slight pressure, is a distinctive feature. The mucous membranes of the eyes, mouth, genitals, respiratory tract, gastrointestinal tract, and kidneys are frequently affected, leading to potential complications such as pneumonia or kidney failure.
• Diagnosis of SJS and TEN is primarily clinical, but a skin biopsy confirms the diagnosis, showing the detachment of the epidermis and apoptosis of keratinocytes. Differential diagnoses include viral exanthems, drug rashes, toxic shock syndrome, staphylococcal scalded skin syndrome, and erythema multiforme.
• While SJS affects a smaller area of the skin, TEN involves a more extensive loss of skin, resembling a severe burn and posing a higher risk of mortality. Treatment for both SJS and TEN involves hospitalization, discontinuation of suspected drugs, and intensive supportive care in specialized units like burn centers or intensive care units

• Treatment is supportive care; cyclosporine, plasmapheresis or IV immune globulin, early corticosteroid therapy, and tumor necrosis factor-alpha inhibitors have been used. Mortality can be as high as 7.5% in children and 20 to 25% in adults but tends to be lower with early treatment.