A histopathologic study of corneal keratopathy in Stevens-Johnson syndrome

Purpose. Evaluation of pathologic changes in cornea buttons and conjunctiva removed from patients with erythema multiforme major. Methods. Histopathologic examination using light microscopy with routine and special stains of 9 native corneas, 2 grafted corneas, and 4 conjunctival specimens removed for penetrating keratoplasty or keratoprostheses. Results. The corneas showed several consistent patterns of injury. The epithelium was either severely denuded (77.8%) or showed marked hyperplasia (33.3%). An inflammatory pannus was commonly (88.9%) seen with stromal neovascularization (88.9%) and chronic inflammatory infiltration (77.8%) of the anterior half of the cornea (100.0%). The stromal was scarred (88.9%) and thinned (77.8%). Endothelial attenuation was frequent (88.9%). The corneal grafts transplanted into Stevens-Johnson patients had changes similar to those in the native cornea including inflammatory pannus and anterior corneal vascularization (100.0%). The conjunctiva biopsies revealed acanthosis and keratinization similar to the corneal specimens (50.0%), a loss of goblet cells (75.0%), chronic stromal infiltration and scarring (75.0%). Conclusions. This study, which represents the only histopathologic series focusing on the corneal changes in erythema multiforme major, indicates that pannus and neovascularization ot the anterior cornea is a common pathway for corneal injury in Stevens-Johnson syndrome and that corneal grafts are subject to the same destructive processes.