title: Repair of cicatricial ectropion in a harlequin baby.
abstract: The ichthyoses are a collection of scaling skin diseases or keratinizing skin disorders giving the appearance of "fish skin," of which harlequin ichthyosis is the most severe form. It is characterized by profound thickening of the keratin skin layer, armorlike scales that cover the body, and contraction abnormalities of the eyes, ears, and mouth. We report a case of a 6-week-old boy with harlequin ichthyosis and severe bilateral upper and lower eyelid cicatricial ectropion who underwent surgical repair with full-thickness postauricular skin autografts. To our knowledge, this is the youngest reported case and the only case of harlequin ichthyosis in which postauricular skin grafts were used.
authors.affiliation: St. James University Hospital, Leeds, UK.
authors.forename: Rehna
authors.initials: R
authors.lastname: Khan
authors.forename: Seema
authors.initials: S
authors.lastname: Arora
authors.forename: Nabil
authors.initials: N
authors.lastname: El-Hindy
authors.forename: Bernard Y P
authors.initials: BY
authors.lastname: Chang
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D002921:Cicatrix / Q000209:etiology / Q000601:surgery*; D003241:Consanguinity; D004423:Ear; D004483:Ectropion / Q000209:etiology / Q000601:surgery*; D005865:Gestational Age; D006801:Humans; D017490:Ichthyosis, Lamellar / Q000150:complications*; D007223:Infant; D008297:Male; D016038:Skin Transplantation; D014182:Transplantation, Autologous
chemical_list: C473801:ABCA12 protein, human; D018528:ATP-Binding Cassette Transporters