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Oral health in Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
E McGovern1, P Fleming, C Costigan
1Dental Dept., Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. eleanor.mcgovern@dental.tcd.ie
Summary
Patients with Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED) exhibit poor oral health, including higher rates of enamel defects and dental issues. These enamel defects correlate with the timing of systemic disease during tooth development.
Area of Science:
- Oral Health
- Genetics
- Immunology
Background:
- Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED) is a rare genetic disorder.
- It is characterized by immune-mediated endocrine destruction, candidiasis, and ectodermal issues like enamel hypoplasia.
Purpose of the Study:
- To investigate oral health and enamel defects in APECED patients.
- To correlate enamel defect timing with disease progression.
Main Methods:
- 16 APECED patients were compared to age/gender-matched healthy controls.
- Oral health assessed for periodontal disease, caries, erosion, and enamel defects.
- Enamel defect timing linked to medical history and diagnosis.
Main Results:
- APECED patients had poorer oral health, with increased periodontal disease, caries, and erosion.
- Significantly higher prevalence of enamel defects (pits, grooves, missing enamel) in APECED patients.
- Enamel defects showed chronological patterns, associated with hypoparathyroidism, gastrointestinal dysfunction, and candidiasis history.
Conclusions:
- APECED patients present with compromised oral health and a higher incidence of enamel defects.
- Enamel defects in APECED patients occur chronologically and are linked to systemic disease events during tooth development.
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