Ectropion Improvement with Topical Tazarotene in Children with Lamellar Ichthyosis

Lamellar ichthyosis (LI) is a well-described phenotypic subtype of autosomal recessive congenital ichthyosis (ARCI). The condition typically presents at birth with collodion membrane and leads to thick, plate-like scaling of the skin throughout the body, alopecia, and prominent ocular manifestations. Ocular complications include bilateral cicatricial ectropion and lagophthalmos. These ocular complications can lead to chronic exposure keratitis and in some cases corneal ulceration and blindness. No cure for ichthyosis exists. Treatment of ocular complications in LI includes surgical correction, systemic retinoids, and a variety of topical therapies such as emollients, keratolytics, and retinoids.

Five children with LI cared for at our institution were identified and included. Patient age at the start of therapy ranged from 2 weeks to 9 years. Electronic medical records were reviewed and data from pediatric dermatologist and pediatric ophthalmologist visits were obtained. Data were collected before and after treatment of daily or twice-daily 0.05% to 0.1% tazarotene cream applied to the face and eyelids.

All patients had improvement in the degree of ectropion, with complete resolution in two of the five patients. The two patients with lagophthalmos at the time of tazarotene initiation experienced complete resolution. No adverse effects were reported.

Tazarotene cream appears to be effective in the management of ectropion and lagophthalmos in the setting of LI in children, even in the neonatal period.