APS-I/APECED: the clinical disease and therapy

摘要: The clinical picture and course of APS-I or APD-I/APECED is widely variable: the list possible disease components includes some 30 disorders. initial manifestation may not include any known characteristic components, namely, mucocutaneous candidiasis, hypoparathyroidism, adrenocortical insufficiency. Although mutation detection available, it does help to exclude this disease. Diagnostic strategy needs be based on knowledge picture, including features ectodermal dystrophy.