Stevens-Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be associated with a preceding history of medication use, most commonly anticonvulsants, antibiotics, and non-steroidal anti-inflammatory drugs. Other associated factors include infections.

The patient may present with Nikolsky's sign, where the epidermal layer easily sloughs off when pressure is applied to the blistered or erythematous area.

Diagnosis is made by clinical presentation and confirmed with skin biopsy.

On diagnosis the offending medicine should be stopped. Management is then supportive.

Patients do best if they are sent to a burn centre for wound care as soon as the diagnosis is suspected or made.

The majority of SJS patients recover (mortality 1% to 5%). SJS can recur either with the same medicine or with another medicine.

TEN has a higher mortality (25% to 30%).

In the long term, patients should ensure they are not re-exposed to the trigger medicine and be careful of self-medicating. They should avoid sunlight during healing and moisturise their skin.

Currently, patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.

Stevens-Johnson syndrome (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 more severe form of erythema multiforme major and a less severe manifestation of toxic epidermal necrolysis (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]Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis: clinical patterns, diagnostic considerations, etiology, and therapeutic management. Semin Cutan Med Surg. 2014 Mar;33(1):10-6. http://www.ncbi.nlm.nih.gov/pubmed/25037254?tool=bestpractice.com [4]Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med. 1994 Nov 10;331(19):1272-85. http://www.ncbi.nlm.nih.gov/pubmed/7794310?tool=bestpractice.com [5]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 [6]Auquier-Dunant A, Mockenhaupt M, Naldi L, et al. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol. 2002 Aug;138(8):1019-24. http://www.ncbi.nlm.nih.gov/pubmed/12164739?tool=bestpractice.com [7]Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol. 1956 Nov;68(11):355-61. http://www.ncbi.nlm.nih.gov/pubmed/13374196?tool=bestpractice.com [8]Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis. Part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013 Aug;69(2):173. http://www.ncbi.nlm.nih.gov/pubmed/23866878?tool=bestpractice.com [9]Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis. Part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013 Aug;69(2):187. http://www.ncbi.nlm.nih.gov/pubmed/23866879?tool=bestpractice.com