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Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management.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.
SJS is a severe skin detachment with mucocutaneous complications. It is an immune reaction to foreign antigens. SJS is a less severe manifestation of TEN.
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
Areta Kowal-Vern, MD
Adjunct Research Faculty
Arizona Burn Center
Valleywise Health Medical Center
AK-V performs occasional manuscript reviews on various topics for Research Square, Inc. and the Journal of Burn Care and Research in the US for which she receives a nominal monetary fee. AK-V is an author of a reference cited in this topic.
Barbara A. Latenser, MD
Director Burn Unit
Department of Surgery
University of Iowa Hospitals
BAL declares that she has no competing interests.
Tina Palmieri, MD
Assistant Chief of Burns
TP declares that she has no competing interests.
Venkat Gudi, MB BS
Department of Dermatology
West Suffolk Hospital
Bury St. Edmunds
VG declares that he has no competing interests.
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