Stevens-Johnson syndrome and toxic epidermal necrolysis

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Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management.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.[1]Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia. Am J Dis Child. 1922 Dec;24(6):526-33. It is an immune reaction to foreign antigens. SJS is a less severe manifestation of TEN.[2]Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermatol. 2015 Dec;16(6):475-93. http://www.ncbi.nlm.nih.gov/pubmed/26481651?tool=bestpractice.com

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.[3]Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993 Jan;129(1):92-6. http://www.ncbi.nlm.nih.gov/pubmed/8420497?tool=bestpractice.com