Abstract
Autoimmune polyglandular syndrome type 1 (APS-1) is a rare condition of autosomal recessive and monogenic inheritance, which is characterized clinically by at least 2 signs of the classic triad: mucocutaneous candidiasis, hypoparathyroidism, and Addison's disease. The aim of this study is to report the systemic and oral manifestations of APS-1 in a 42-year-old woman. Among other systemic characteristics, the patient had hypoparathyroidism, type I diabetes mellitus, and autoimmune hepatitis. Chronic hyperplastic candidiasis (CHC) was the main oral manifestation present, and this was diagnosed clinically and through exfoliative cytology. In addition, microstomy, angular cheilitis, xerostomia, enamel hypoplasia, and microdontia were present. The initial treatment plan included the management of CHC with local and systemic antifungals, which resulted in significant improvement of the lesions. Unfortunately, the patient died from complications of APS-1 and was unable to finalize the proposed treatment plan.
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