title: Autoimmune polyglandular syndrome (APS) type 1 and candida onychomycosis.
abstract: A 21-year-old female presented at age 2 years with a chronic mucocutaneous candidiasis and at age 3 alopecia totalis. Later, chronic hypoparathyroidism and autoimmune adrenal insufficiency appeared. In addition, malabsorption syndrome and signs of pernicious anaemia occurred. The onychomycosis totally improved under systemic treatment with fluconazole (Diflucan), endocrine and organ failure with replacement therapy. The autoimmune polyglandular syndrome (APS 1) is a rare autosomal recessive inherited disease. Chronic mucocutaneous candidiasis (CMC) generally presents very early in life and is the most frequent of the three main diseases of APS type 1 (chronic hypoparathyroidism, autoimmune Addison's disease). It can be considered as a precocious marker of APS type 1. Consequently, all patients affected by isolated CMC, especially children, should be evaluated and carefully followed up by immunological, biochemical, and clinical tests to recognize signs and symptoms of imminent or ongoing endocrine glandular failure.
authors.affiliation: Department of Dermatology, University of Leipzig, Stephanstrasse 11, Germany. BernMa@gmx.de
authors.forename: Bernadette
authors.initials: B
authors.lastname: Manz
authors.forename: Gerhard H
authors.initials: GH
authors.lastname: Scholz
authors.forename: Helmut
authors.initials: H
authors.lastname: Willgerodt
authors.forename: Uwe-Frithof
authors.initials: UF
authors.lastname: Haustein
authors.forename: Pietro
authors.initials: P
authors.lastname: Nenoff
mesh_terms: D000328:Adult; D000935:Antifungal Agents / Q000627:therapeutic use; D002177:Candidiasis / Q000150:complications*; D002178:Candidiasis, Chronic Mucocutaneous / Q000150:complications; D005260:Female; D015725:Fluconazole / Q000627:therapeutic use; D006229:Hand Dermatoses / Q000150:complications / Q000188:drug therapy; D006801:Humans; D014009:Onychomycosis / Q000150:complications* / Q000188:drug therapy; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications*
chemical_list: D000935:Antifungal Agents; D015725:Fluconazole