Severe Bilateral Ectropion in Lamellar Ichthyosis: A Case Report.

ABSTRACT

BACKGROUND Lamellar ichthyosis is a rare type of hereditary ichthyosis disease that is responsible for generalized dry skin and severe scaling. Congenital ectropion and eclabium are often associated with this condition. The ectropion can cause many ophthalmologic complications, mostly due to corneal exposure caused by the lack of eyelid cleft closure. The corneal exposure can cause ulceration and even corneal perforation leading to phthisis bulbi. CASE REPORT We report the case of a North African newborn baby diagnosed with congenital lamellar ichthyosis responsible for severe skin scaling, eclabium, and bilateral ectropion of both the upper and lower eyelids. The therapeutic care of the baby was ensured by a collaboration between ophthalmologist and dermatologist. The newborn was treated by oral acitretin and skin emollients for his dermatological conditions and on the ophthalmological level he was put on lubricating and moisturizing eye drops and gel, associated with daily repeated eyelid massage. The evolution after 6 months of treatment showed excellent results on both the dermatological and the ophthalmological level. CONCLUSIONS At the stage of cicatricial ectropion in lamellar ichthyosis, the management is mainly based on a chirurgical approach. However, if the ectropion is managed early enough, the treatment could be medical, which is much easier, produces very good results, and prevents the progression toward eyelid fibrosis and its associated complications that can make the management of the ectropion much harder.