Combined medical and surgical management for cicatricial ectropion in lamellar ichthyosis: A report of three cases

Abstract

Ichthyosis is a rare inherited skin disorder characterized by abnormal keratinization of the epidermis. Cicatricial ectropion is the most common ophthalmic feature of congenital ichthyosis. Progressive subepithelial cicatrization and abnormal cornification of eyelid skin cause progressive ectropion in both eyelids, leading to lagophthalmos and corneal exposure. Surgical correction of cicatricial ectropion in these cases is challenging with unsatisfactory results. Proper processing of the donor and recipient site with lubricants and topical retinoids before surgery makes grafting easier and its survival better. We present three cases of lamellar ichthyosis with cicatricial ectropion managed with combined preoperative topical therapy followed by surgery. All patients had extremely good surgical outcomes, with none of them requiring repeat surgery.

Keywords: Cicatricial ectropion; lamellar ichthyosis; skin grafting.

Figure 1

(a) Preoperative external photo of the infant showing cicatricial ectropion of both upper lid. Scales all over the face can be seen. (b) Postoperative photograph showing good graft uptake with correction of ectropion. (c) Postoperative photograph showing good anterior lamella of both the upper lids at 3 years of follow-up

Figure 2

(a) Preoperative external photograph of the young boy with cicatricial ectropion of both the lower lids. (b) Postoperative photograph of the patient showing correction of lower lid ectropion. Notice the improvement in the condition of upper lids, forehead, and nose because of medical therapy

Figure 3

(a) Preoperative external photograph of an adult lady with ichthyosis cicatricial ectropion of all four lids. (b) Postoperative photograph showing good graft uptake in all the four lids and good correction of ectropion