Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin detachments with mucocutaneous complications associated with a history of medication use, most commonly anticonvulsants, antibiotics, and non-steroidal anti-inflammatory drugs.
Patients with HIV or active cancer are at an increased risk of SJS/TEN.
SJS/TEN may present with mild symptoms in early disease, but the condition progresses over several days; close monitoring and regular reassessment is essential.
Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management.
Transfer to a burn centre, a specialised wound care centre, or a dermatology intensive care unit is recommended for patients diagnosed with SJS/TEN.
Patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.
Classification is dependent on the percentage of skin involvement: SJS has <10% total body surface area (TBSA) involvement; SJS/TEN overlap has 10% to 30% TBSA involvement; and TEN has >30% TBSA involvement.
History and exam
Key diagnostic factors
- presence of risk factors
- mucosal involvement
- Nikolsky's sign
- patients with active cancer
- anticonvulsant medicines
- recent infection
- recent antibiotic use
- other medicines
- systemic lupus erythematosus
- human leukocyte antigen and genetic predisposition
- smallpox vaccination
- bone marrow transplantation
1st investigations to order
- skin biopsy
- blood cultures
- full blood count
- serum electrolytes
- serum creatinine
- liver function tests
- erythrocyte sedimentation rate
- C-reactive protein
- arterial blood gases and saturation of oxygen
- chest x-ray
- coagulation studies
- skin swab from lesional skin
- Mycoplasma serology
Investigations to consider
- direct immunofluorescence
- Drug rash with eosinophilia and systemic symptoms (DRESS)
- Staphylococcal scalded skin syndrome
- Toxic shock syndrome
- Supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults
- Management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people (0 to 17 years)
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