Abstract

Congenital ichthyoses (CI) are rare genetic skin keratinisation diseases characterised by generalised scaling and a variable degree of erythema and hyperkeratosis. Ocular involvement includes the eyelids, conjunctiva and all layers of the cornea. Ophthalmic input should include regular slit lamp review with the primary aim to prevent a corneal epithelial defect, secondary bacterial infection, scarring or perforation. This review highlights the current literature regarding ophthalmic findings and management of CI.