Bilateral Congenital Entropion with Cutis Laxa. Issue 3 (11th February 2014)

• Record Type: Journal Article
• Authors: Al‐Faky, Yasser H.; Salih, Mustafa A.; Mubarak, Mohammed; Al‐Rikabi, Ammar C.
• Is Part Of: Pediatric dermatology. Volume 31:Issue 3(2014)
• Page Start: e82
• Page End: e84
• Publication Date: 2014-02-11
• Subjects: Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/pde.12255
• Languages: English
• ISSNs: 0736-8046

Abstract

<abstract abstract-type="main" id="pde12255-abs-0001"> <title>Abstract</title> <p>Cutis laxa is a rare connective tissue disorder characterized by redundant and pendulous skin due to a defect in the elastic fiber network. Two cases of entropion associated with cutis laxa have been reported, although entropion was due to elongation of the anterior lamella or horizontal lid laxity. Thorough systemic and ophthalmic evaluations were performed, as well as chart review for the perinatal period. Surgical correction of entropion through posterior tarsotomy was done. An infant boy with dysmorphic features and furrowing of the skin of the entire body without hyperelasticity, which is typical for cutis laxa, presented with bilateral congenital entropion. We report here for the first time a different etiology of congenital entropion with cutis laxa: the eyelashes were abnormally directed due to the unusual location of their roots, which were embedded within the tarsus. Moreover, this is the only case of cutis laxa with congenital entropion involving both upper and lower eyelids. Congenital entropion can be associated with cutis laxa. Although elongation of the anterior lamella and horizontal lid laxity predispose to such an entropion, abnormal location of the roots of the eyelashes might be encountered and marginal eyelid rotation surgery is indicated.</p> </abstract>