[Use of topical cutaneous N-acetylcysteine in the treatment of major bilateral ectropion in an infant with lamellar ichthyosis]

Abstract

Purpose: To report for the first time bilateral ectropion treatment in an infant with severe lamellar ichthyosis associating N-acetylcysteine applied directly to the skin and oral acitretin.

Methods: An 8-week-old male child with major bilateral ectropion due to lamellar ichthyosis was given treatment associating oral acitretin (Soriatane) and topical N-acetylcysteine. Though the precorneal tear film quality could be maintained, after 1 month of initial treatment with acitretin only, bilateral upper eyelid ectropion remained threatening for the child's cornea. The adjunction of topical N-acetylcysteine enabled a complete regression of ectropion. No complementary surgery was needed and the eyelids remained well positioned.

Conclusion: Topical N-acetylcysteine has been proved to have an antiproliferative effect on keratinocytes in vitro and in vivo. It may be useful in the treatment of major forms of ectropion in children with lamellar ichthyosis. Its association with conventional acitretin treatment may prevent unnecessary surgery.