Eyelid abnormalities in lamellar ichthyoses

PURPOSE: To describe eyelid abnormalities in lamellar ichthyoses (LI). DESIGN: Retrospective observational case series. PARTICIPANTS: Eight patients with classic LI and two patients with congenital ichthyosiform erythroderma. METHODS: Results of eyelid and corneal examinations of 10 patients with LI were reviewed and analyzed with emphasis on the relationship between eyelid ectropion and corneal damage. RESULTS: All patients presented with cicatricial lagophthalmos. Of the eight patients with classic LI, five had ectropion of the four eyelids, one had only lower ectropion, and two had no degree of ectropion. Two patients with congenital ichthyosiform erythroderma had distinct eyelid abnormalities, including madarosis and eyelash retraction. Loss of vision caused by corneal damage was found in three patients with classic LI. Of these three patients, two did not have upper eyelid ectropion. CONCLUSIONS: Severe corneal damage can occur in LI even if there is no upper or lower eyelid ectropion.