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: Rehna Khan, Seema Arora, Nabil El-Hindy, Bernard Y P Chang
• Journal: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus (J AAPOS) Vol. 13 Issue 4 Pg. 415-6 (Aug 2009) ISSN: 1528-3933 [Electronic] United States
• PMID: 19683196 (Publication Type: Case Reports, Journal Article)

Chemical References

• ABCA12 protein, human
• ATP-Binding Cassette Transporters

Topics

• ATP-Binding Cassette Transporters (genetics)
• Cicatrix (etiology, surgery)
• Consanguinity
• Ear
• Ectropion (etiology, surgery)
• Gestational Age
• Humans
• Ichthyosis, Lamellar (complications)
• Infant
• Male
• Skin Transplantation
• Transplantation, Autologous