title: Harlequin ichthyosis: a case report.
abstract: Harlequin Ichthyosis (HI) is a very rare subtype of a group of hereditary disorders that have in common the accumulation and shedding of significant amounts of hyperkeratotic epidermis. HI has a very specific clinical presentation that includes eclabium, fish mouth appearance, mouth breathing and xerostomia. The addition of a very high caloric demand to the clinical features place these affected individuals at extremely high risk for developing rampant dental decay. This case report discusses a two-year-old boy with HI that presents with severe early childhood caries. The specific accommodations that were made to accomplish required dental work under general anesthesia are presented. A case is made for the inclusion of dentists in the management of these cases once a diagnosis is made.
authors.affiliation: Department of Pediatric Dentistry, College of Dentistry, University of Illinois at Chicago, Chicago, Ill., USA. rodney@uic.edu.
authors.forename: Rodney Joseph
authors.initials: RJ
authors.lastname: Vergotine
authors.affiliation: Department of Pediatric Dentistry, College of Dentistry, University of Illinois at Chicago, Chicago, Ill., USA.
authors.forename: Maribel Reyes
authors.initials: MR
authors.lastname: de Lobos
authors.affiliation: Department of Pediatric Dentistry, College of Dentistry, University of Illinois at Chicago, Chicago, Ill., USA.
authors.forename: Marilia
authors.initials: M
authors.lastname: Montero-Fayad
mesh_terms: D000766:Anesthesia, Dental; D000768:Anesthesia, General; D002675:Child, Preschool; D003442:Crowns; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D017490:Ichthyosis, Lamellar / Q000175:diagnosis*; D008046:Lip / Q000002:abnormalities; D008297:Male; D009056:Mouth Abnormalities / Q000175:diagnosis; D014081:Tooth Extraction / Q000379:methods