04/09/2021
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), or Lyell syndrome, are rare, delayed-type hypersensitivity reactions associated with high levels of morbidity and mortality.
The clinical hallmark of SJS and TEN is epidermal and mucosal detachment and necrosis.
The 2 entities are defined by the body surface area (BSA) of detachable or detached skin:
- < 10% of the BSA affected in SJS,
- 10% to 30% in overlap syndrome, and
- > 30% in TEN.
Current clinical practices, including the use of systemic agents, such as cyclosporine, systemic glucocorticoids, intravenous immunoglobulins (IVIGs), and antitumor necrosis factor (TNF) agents, are based only on retrospective case reports of series and empirical experience.
The association of these agents with the outcome, however, has not been proven in prospective trials.
The distribution of General Culprit Drugs & Treatments is illustrated in the figures.
References:
1. Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O. Severe cutaneous adverse reactions to drugs.Lancet. 2017;390(10106):1996-2011. doi:10.1016/S0140-6736(16)30378-6
2. Kuijper EC, French LE, Tensen CP, Vermeer MH,Bouwes Bavinck JN. Clinical and pathogenic aspects of the severe cutaneous adverse reaction epidermal necrolysis (EN).J Eur Acad DermatolVenereol. 2020;34(9):1957-1971. doi:10.1111/jdv.16339
3. Hsu DY, Brieva J, Silverberg NB, Silverberg JI.Morbidity and mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis in United States adults.J Invest Dermatol. 2016;136(7):1387-1397. doi:10.1016/j.jid.2016.03.023
4. Chaby G, Maldini C, Haddad C, et al. Incidence of and mortality from epidermal necrolysis(Stevens-Johnson syndrome/toxic epidermal necrolysis) in France during 2003-2016:a four-source capture-recapture estimate.Br J Dermatol. 2020;182(3):618-624. doi:10.1111/bjd.18424
5. JAMA Dermatol. doi:10.1001/JAMA Dermatol.2021.3154Published online August 25, 2021.
