Ectropion surgery might not be a long‐term solution for harlequin ichthyosis

Abstract

Harlequin ichthyosis is the most extreme form of all autosomal recessive congenital ichthyosis caused by biallelic loss of function mutations in an ABC transporter gene/ABCA12. 1,2 Respiratory insufficiency and infection occasionally do not permit the affected neonates to survive. However, the introduction of systemic retinoids and improvement of neonatal care has resulted in a prolonged survival rate. The survivor will develop exfoliative erythroderma, palmoplantar keratoderma, persistent ectropion, eclabion, and joint contractures.The tautness of eyelid skin and transepidermal dehydration lead to severe and cicatricial ectropion, causing further exposure keratopathy and eventually, corneal ulceration. 3 Although corneal damage might

Management of Harlequin Ichthyosis: A Brief Review of the Recent Literature

“…Another surgical case of HI reports that the uptake of neotigason prior to and after the surgery (20 days prior), followed by topical administration of acitretin (or tazarotene), did not manage to prevent or delay the reoccurrence of ectropion. Further studies are needed on this matter [ 57 ].…”