Publication details

Sight-threatening Complication of Cicatricial Ectropion in a Patient with Lamellar Ichthyosis - Case Report



Year of publication 2020
Type Article in Periodical
Magazine / Source Acta Dermatovenerologica Croatica
MU Faculty or unit

Faculty of Medicine

Keywords ichthyosis; corneal perforation; ectropion; penetrating keratoplasty
Description We report a case of lamellar ichthyosis and sight-threatening complications of cicatricial ectropion in an adult male patient which was sur- gically managed with tectonic penetrating keratoplasty. We present a case of autosomal-recessive lamellar ichthyosis in a 47-year-old man who was re- ferred to our outpatient eye clinic for treatment of primary keratouveitis of the right eye with keratolysis and exudation in the anterior chamber. A di- agnosis of cicatricial ectropion with serious lagophthalmos was established on examination. The patient underwent tectonic penetrating keratoplasty, cataract extraction, and intra-ocular lens placement with no perioperative complications. The patient was subsequently treated with oral fluconazole 200 mg once daily for 12 days due to a positive fungal culture for Candida albicans and systemic oral acyclovir 250 mg 3 times per day for 12 days as pro- phylaxis for a labial herpetic infection. Post-operative complications included corneal rejection and nonhealing neurotropic epithelial defect of the graft. Long-term treatment with topical cyclosporine (Ikervis®) and dexametha- sone led to resolution of the corneal rejection. Lubrication with artificial tears containing hyaluronic acid, perfluorohexyl octane (Evotears®), and vitamin A ointment led to symptomatic relief of dry eye disease. The patient was re- ferred to a dermatologist and was started on systemic retinoid acitretin at a dose of 0.5 mg/kg per day. Ten months after surgery, the patient’s visual acuity was 0.1 based on the Snellen chart and the corneal graft was stable. Infection in the cornea can rapidly progress to corneal melting in patients with severe cicatricial ectropion. A good patient outcome depends on the interdisciplinary approach to patient management by the ophthalmologist, dermatologist, and plastic surgeon.

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