Purpose of Review: Healthy children may develop candidal infections as the result of exposure to antibiotics or corticosteroids, but chronic candidiasis in children after the newborn period is unusual. Chronic mucocutaneous candidiasis (CMC) refers to a group of conditions characterized by recurrent or persistent infections with Candida species, particularly Candida albicans. CMC is a phenotype observed in a spectrum of immunologic disorders, some with endocrinologic and autoimmune features. Recent Findings: CMC can arise secondary to inherited or acquired T cell deficiencies, but in children is largely due to inborn errors impairing the dectin pathway and IL-17 immunity. We review the current understanding of the pathogenesis of chronic mucocutaneous candidiasis and discuss the immunologic pathways by which the immune system handles Candida. We highlight the historical and recent knowledge of CMC in children, emphasizing recent insights into basic science aspects of the dectin pathway, IL-17 signaling, consequences of AIRE gene defects, and clinical aspects of inheritance, and features that distinguish the different syndromes. Summary: The clinical phenotype of CMC has many underlying genetic causes. Genetic testing is required for definitive diagnosis.
- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
- Chronic mucocutaneous candidiasis
- Hyper-IgE syndrome
ASJC Scopus subject areas
- Immunology and Allergy
- Pulmonary and Respiratory Medicine