Enamel hypoplasia secondary to candidiasis endocrinopathy syndrome: case report

The clinical features of a 7-year-old girl with enamel hypoplasia secondary to autoimmune hypoparathyroidism and chronic mucocutaneous candidiasis are detailed. The combination of features are typical of a rare, probably genetically determined immunodeficiency termed candidiasis endocrinopathy syndrome (CES). Affected individuals have chronic mucocutaneous candidiasis and a spectrum of autoimmune endocrinopathies, including hypoparathyroidism, adrenocortical hypofunction, and diabetes mellitus. Treatment includes long-term management of the candidal infection and correction of any associated endocrinopathy.