Lamellar ichthyosis with severe bilateral ectropion and self-healing collodion membrane

Abstract

Congenital ichthyosis is a type of generalized hyperkeratinization of the skin at birth. Three forms of congenital ichthyosis have been defined based on clinical aspects and severity: (1) congenital ichthyosis mitis; (2) congenital ichthyosis tarda; and (3) congenital ichthyosis gravis. Desquamation of the parchment-like hyperkeratinized skin begins shortly after birth and may require several weeks to complete. Skin alterations in the eyelid cause shortening of the anterior lamella, subsequently resulting in ectropion. This affects the upper eyelid more often than the lower eyelid and can lead to complications such as chronic palpebral or bulbar conjunctivitis and keratinization or exposure keratopathy. In this paper, we present two case reports illustrating the course of ichthyosis congenita mitis and ichthyosis congenita tarda.