[Erythema exsudativum multiforme major]

Erythema exudativum multiforme major (EEMM), also know as Stevens-Johnson syndrome, may cause severe conjunctival and corneal alterations. The etiology remains unknown. The aim of are study was to evaluate the clinical course, therapy and prognosis of ocular involvement.

In a retrospective study, we evaluated ten patients with EEMM who were treated between 1986 and 1994 at the University Eye Clinic Tübingen.

The age of the four female and six male patients varied between 5 and 70 years. Drugs as a possible precipitating factor were found in all cases (acetylsalicylicacid, sulfonamide, erythromycin, cotrimoxazole, Valproinicacid, paracetamol). Eight patients experienced an infection at the same time. Clinically, we were able to differentiate two groups regarding severity of the disease. Seven patients were characterized by ocular alterations that only involved the conjunctiva, leading to conjunctival scars. In those cases local treatment with antibiotics and corticosteroids was effective. In the second group severe ocular damage occurred, with symblephara, keratinization and consecutive perforating keratoplasty. Secondary infections became evident in a total of six cases from the two groups.

Drugs as a possible precipitating factor could be identified in all cases. In eight of ten patients previous infection was known. This agrees with literatures reports of viral infections (e.g. herpes simplex) as cofactors causing EEMM, especially for the aggressive form, remains unresolved. The danger of possible superinfection should always be taken into consideration.