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Autoimmune polyglandular syndrome (APS) type 1 and candida onychomycosis


European Journal of Dermatology. Volume 12, Number 3, 283-6, May - June 2002, Cas cliniques

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Author(s) : Bernadette MANZ, Gerhard H. SCHOLZ, Helmut WILLGERODT, Uwe-Frithof HAUSTEIN, Pietro NENOFF

Summary : 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.

Keywords : Candida albicans, fluconazole, hypoparathyroidism, mucocutaneous candidiasis.

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