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Harlequin ichthyosis: a case report
Rodney Joseph Vergotine1, Maribel Reyes de Lobos2, Marilia Montero-Fayad2
1Department of Pediatric Dentistry, College of Dentistry, University of Illinois at Chicago, Chicago, Ill., USA. rodney@uic.edu.
Pediatric Dentistry
|February 21, 2014
Summary
Harlequin Ichthyosis (HI), a rare genetic disorder, causes severe skin issues and high caloric needs, increasing dental decay risk. Early dental intervention and specialized care are crucial for managing caries in affected children.
Area of Science:
- Dermatology
- Genetics
- Pediatric Dentistry
Background:
- Harlequin Ichthyosis (HI) is a rare, severe genetic disorder characterized by abnormal skin development.
- Patients exhibit unique facial features like eclabium and xerostomia, alongside high metabolic demands.
Observation:
- This case report details a 2-year-old boy diagnosed with Harlequin Ichthyosis.
- The child presented with severe early childhood caries, a common complication due to xerostomia and high caloric intake.
Findings:
- Dental management required significant accommodations, including dental work performed under general anesthesia.
- The case highlights the extreme risk of rampant dental decay in individuals with HI.
Implications:
- Early and integrated dental care is essential for patients diagnosed with Harlequin Ichthyosis.
- Inclusion of dental professionals in multidisciplinary care teams is recommended for optimal patient outcomes.
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