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

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