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oa Current Allergy & Clinical Immunology - When you cannot beat back the mould : chronic mucocutaneous candidiasis  - primary immune deficiency

Volume 30 Number 2
  • ISSN : 1609-3607

 

Abstract

Candida spp are normal human commensal fungi that have co-evolved with human beings over millennia. Normal host immunity with a delicate balance of pro- and anti-inflammatory signals maintains a stable host– fungus relationship. T-cells, particularly intact T-helper 17 cell, neutrophils and the type-1 interferon cytokine networks are non-redundant for preventing invasive fungal disease. Self-limiting and/or easily treated mucosal infections to candida occur in immunocompetent hosts, usually associated with altered mucosal-barrier integrity. In contrast, severe, persistent and recurrent mucosal-candida infections – known as ‘chronic mucocutaneous candidiasis’ (CMC) – suggest an underlying immunodeficiency and warrant investigation. To date, more than ten gene defects have been associated with CMC, either alone or in combination with other syndromic features and/or infections. In this review, we present the first South African CMC family with a confirmed gain-of-function (GOF) STAT1 mutation. We discuss host fungal immunity to candida, describing the known gene defects and then outline a clinical approach to the diagnosis, highlighting specific management of issues in the care of patients with CMC.

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/content/journal/10520/EJC-9066393f3
2017-06-30
2021-01-17

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