ANALYSIS OF THE ACUTE OPHTHALMIC MANIFESTATIONS OF THE ERYTHEMA MULTIFORME STEVENS-JOHNSON SYNDROME TOXIC EPIDERMAL NECROLYSIS DISEASE SPECTRUM

Purpose: To evaluate the epidemiology, possible etiologic factors, com plications encountered, and treatment administered to a group of patie nts with ocular involvement in the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum who were seen at two large tertiary referral centers over a 34-year period. Methods: H ospital records from 1960 to 1994 at the Massachusetts General Hospita l and Shriners Hospital for Crippled Children were reviewed for patien ts with erythema multiforme, Stevens-Johnson syndrome, or toxic epider mal necrolysis. Only patients fulfilling specific clinical diagnostic criteria and those who received a diagnosis by a dermatologist were in cluded in the review. Results: A total of 366 patients with erythema m ultiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis wer e identified. Drugs were the most commonly identified etiologic factor in all three conditions; sulfonamides were the most frequently identi fied agents. Eighty-nine patients (24%) had ocular manifestations at t he time of their acute hospital stay. Ocular involvement was seen in 9 % of patients with erythema multiforme, in 69% with Stevens-Johnson sy ndrome, and in 50% with toxic epidermal necrolysis. The ocular problem s were more severe in patients with both Stevens-Johnson syndrome and toxic epidermal necrolysis. There was no significant difference betwee n the number of patients who were treated with systemic steroids and t hose who were not (P = 0.42). Conclusion: The erythema multiforme/Stev ens-Johnson syndrome/toxic epidermal necrolysis disease spectrum remai ns an important cause of severe visual loss in a significant number of patients. Systemic steroids used during the acute phase of the diseas e appear to have no effect on the development of ocular manifestations . Studies on the acute immunopathogenic mechanisms occurring in these diseases are warranted if more effective therapies are to be found.