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Front Immunol. 2018 Nov 19;9:2570. doi: 10.3389/fimmu.2018.02570. eCollection 2018.

Chronic Mucocutaneous Candidiasis in Autoimmune Polyendocrine Syndrome Type 1.

Author information

1
Department of Endocrinology and Metabolism, CHU Lille, Lille, France.
2
Department Parasitology-Mycology, CHU, Lille, France.
3
Inserm, U995-LIRIC, Fungal Associated Invasive & Inflammatory Diseases, Lille, France.
4
Department of Endocrinology, Polyclinique Aguilera, Biarritz, France.
5
Inserm, Center de Recherche des Cordeliers, Sorbonne Université, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
6
UMR 1190, Translational Research in Diabetes Inserm, Lille, France.
7
European Genomic Institute for Diabetes, Univ Lille, Lille, France.

Abstract

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is an autosomal recessive disease caused by mutations in the autoimmune regulator (AIRE) gene, characterized by the clinical triad of chronic mucocutaneous candidiasis (CMC), hypoparathyroidism, and adrenal insufficiency. CMC can be complicated by systemic candidiasis or oral squamous cell carcinoma (SCC), and may lead to death. The role of chronic Candida infection in the etiopathogenesis of oral SCC is unclear. Long-term use of fluconazole has led to the emergence of Candida albicans strains with decreased susceptibility to azoles. CMC is associated with an impaired Th17 cell response; however, it remains unclear whether decreased serum IL-17 and IL-22 levels are related to a defect in cytokine production or to neutralizing autoantibodies resulting from mutations in the AIRE gene.

KEYWORDS:

IL-17; IL-22; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED); autoimmune regulator (AIRE) gene; chronic mucocutaneous candidiasis (CMC)

PMID:
30510552
PMCID:
PMC6254185
DOI:
10.3389/fimmu.2018.02570
[Indexed for MEDLINE]
Free PMC Article

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