Ocular manifestations, complications and management of congenital ichthyoses: a new look

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.

Management of congenital ichthyoses: European guidelines of care, part two

“…The primary aim of ophthalmic management is to maintain normal visual development and protect the ocular surface integrity while minimizing the risk of corneal epithelial defects …”

Ocular manifestations of dermatological diseases part II: genodermatoses

“…Patients with both KID and IFAP syndrome should be followed and closely monitored by ophthalmology to prevent further progression of ocular diseases 3,5 …”

“…Non-syndromic are more common and more commonly associated with ocular findings. 3 Non-syndromic Ichthyosis vulgaris. The most common type and is inherited in an autosomal dominant pattern.…”

“…Ocular findings are not common in this type; the most noticeable finding is nonerythematous eyelid scales and Salzmann nodule. 3 Lamellar ichthyosis. It is an autosomal recessive disorder, manifested by collodion baby in early life and characteristic large plate-like scales.…”

“…4 Patients with both KID and IFAP syndrome should be followed and closely monitored by ophthalmology to prevent further progression of ocular diseases. 3,5 Conradi-H € unermann-Happle syndrome. An x-linked, dominant disorder, most commonly seen in girls and fatal antenatally in boys.…”