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Invited Commentary
April 2017

Genetics and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: What Have We Learned?

Author Affiliations
  • 1Uveitis Unit/Retina Division, Will Eye Hospital, Philadelphia, Pennsylvania
JAMA Ophthalmol. 2017;135(4):361-362. doi:10.1001/jamaophthalmol.2017.0136

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of a spectrum of severe cutaneous reactions affecting skin and mucous membranes.1 Affected patients usually develop a prodrome of fever and malaise, followed by the appearance of cutaneous erythema, confluent blisters and/or papules primarily on the trunk, and hemorrhagic erosions of the mouth, eyelids, conjunctiva, and other mucous membranes. The overall incidence of SJS/TEN is approximately 1 to 2 per million person-years,2 but is affected by variables such as genetics, drug use, concurrent autoimmune disease (eg, lupus), cancer, radiotherapy, and certain infections, such as Epstein-Barr virus, Mycoplasma pneumoniae, and human immunodeficiency virus.3 The risk of SJS in patients with HIV infection is as much as 1 per 1000 patient-years.4

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