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.

journal_name

Pediatr Dent

journal_title

Pediatric dentistry

authors

Vergotine RJ,de Lobos MR,Montero-Fayad M

subject

Has Abstract

pub_date

2013-01-01 00:00:00

pages

497-9

issue

7

eissn

0164-1263

issn

1942-5473

journal_volume

35

pub_type

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