RESUMO
To report the ocular findings in a proven case of Junctional epidermolysis bullosa. A 4-year-old boy known to have epidermolysis bullosa presented with left corneal epithelial defect. After treatment with fusidic acid suspension, lubricants and cycloplegia, he was observed for 3 months. Skin biopsy was submitted for both light and electron microscopy to confirm the diagnosis. Epithelial defect healed after 5 days leaving faint subepithelial opacity at the level of Bowman's layer. Three months later, both palpebral conjunctiva showed marked scarring in addition to the subepithelial haze in the left cornea. Light and electron microscopy of a skin biopsy showed subepidermal cleft involving lamina lucida as a diagnostic feature of Junctional epidermolysis bullosa. Junctional epidermolysis bullosa can be associated with various ocular complications such as superficial corneal scarring. Skin biopsy is needed to confirm the diagnosis