Cicatricial Ectropion Secondary to Lamellar Ichthyosis
A patient with history of lamellar ichthyosis, a congenital autosomal recessive skin disease, presented to Bascom Palmer Eye Institute with repeated complications of severe bilateral exposure keratopathy due to cicatricial ectropion, which led to a corneal perforation of the right eye. Lamellar ichthyosis presents at birth, and is characterized by the presence of large, dark, plate-like scales covering most of the body. Taut and thickened skin leads to cicatricial changes to the eyelids. Due to lack of available skin for skin grafting in ectropion repair in this case, decision was made to pursue mucous membrane grafting to the external right upper eyelid. Scar bands were released through dissection of the right upper eyelid until it was allowed to mobilize inferiorly and ectropion was corrected. Buccal mucosa from the upper and lower lip was harvested and sutured to the right upper eyelid. At six months post surgery, the mucous membrane graft is healthy. Lagophthalmos is significantly reduced, and the cornea is stable. A similar mucous membrane grafting procedure is planned for the left upper eyelid. Existing literature supports the effectiveness of mucous membrane grafts in cases where healthy skin is unavailable for grafting. This approach should be considered in severe cicatricial ectropion associated with diffuse skin disorders.
Presentation Date: 08/14/2025
Issue Date: 09/19/2025
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