Dental Involvement in a Child with Triple A Syndrome

Background: Allgrove (or triple A) syndrome is a rare autosomal recessive disorder. The triad consists of three most prominent features of the syndrome. First, Alacrimia (absence of tears). Second, achalasia (oesophageal motility disorder). Finally, adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency (Addison's disease). The syndrome is caused by mutations in the AAAS gene on chromosome 12q13. Besides, Consistent oral findings could be noticed such as high arched palate, oral pigmentation, fissured or atrophic tongue and xerostomia. Case Report: We report the case of a 12-year-old girl who presented at the oral department of hospital La Rabta (Tunisia) with specific dental abnormalities. Conclusion: The aim of this report is to highlight dental involvement in Allgrove syndrome that could be of great clinical importance in both diagnosis and dental management.