Stevens-Johnson syndrome (SJS) was initially described by two pediatricians in 2 boys aged 7 and 8 who developed "an extraordinary generalized eruption" with continuous fevers, inflamed buccal mucosa and conjunctivitis. This was published in the American Journal of Diseases of Children in 1922. In 1956, Lyell described in the British Journal of Dermatology a syndrome that he named toxic epidermal necrolysis (TEN). It was characterized by tenderness, erythema and sloughing of the dermis resembling a scald. He believed the necrolysis was related to a toxin from an adverse drug reaction.
The clinical picture is very protean. There is a 2-3 day history of fever, malaise, headache, sore throat and cough. The rash begins as painful, pruritic macules, progressing to skin sloughing with mucosal and visceral involvement. The viscera involved can be the trachea, esophagus, vagina, inner rectal mucosa, eyes or mouth. It appears to be a T-cell-mediated immune reaction, not unlike graft-versus-host disease. The common agents that are indicted are antibiotics and anticonvulsants.
There is some descriptive confusion in differentiating SJS from TEN. There is also a spectrum of severity between the 2 diseases.