Orale manifestasjoner ved autoimmunt polyendokrint syndrom type 1

Oral manifestations of autoimmune polyendrocrine syndrome type 1 Autoimmune polyendocrine syndrome type 1 (APS-1) is a rare autoimmune disease characterized by primary adrenal insufficiency (Addison’s disease), chronic mucocutaneous candidiasis and hypoparathyroidism. Disease manifestations vary from individual to individual, and both endocrine and non-endocrine tissues are affected with debute in childhood, but often with delayed diagnosis. Oral manifestations as enamel defects and chronic oral candida infections are among the disease hallmarks. The enamel may be affected to various degrees within the same patient and between disease cases, from minor hypomineralization to serious enamel hypoplasia affecting most of the teeth. This is expected to be underdiagnosed and should be addressed on a larger scale when examining patients with this disease pattern. Chronic oral candidiasis from Candida albicans in oral mucosa and mucosa of the esophagus in APS-1 patients may develop over time to dysplastic changes in the mucosa.Ulceration and atrophic mucosa is furthermore commonly seen. The oral disease hallmarks of APS-1 underline the important awareness by dental health care professionals of this syndrome. This will provide better diagnostics at an earlier stage of the disease and improved treatment by interdisciplinary collaborations between general practitioners, endocrinologists and dental health care providers. Keywords: polyendocrinopathies, autoimmune, developmental defects of enamel, dental enamel hypoplasia