Primary immunodeficiency and chronic mucocutaneous candidiasis: pathophysiological, diagnostic, and therapeutic approaches
Allergol. immunopatol
; 49(1): 118-127, ene.-feb. 2021. ilus, tab, graf
Article
in English
| IBECS
| ID: ibc-199236
Responsible library:
ES1.1
Localization: BNCS
ABSTRACT
Chronic mucocutaneous candidiasis (CMC) is characterized by a chronic or recurrent non-invasive infection, mainly due to Candida albicans, in skin, nails, and mucous membranes, associated in some cases with autoimmune manifestations. The key immune defect is a disruption of the action of cytokine IL-17, whose most common genetic etiology is STAT1 gene gain-of-function (GOF) mutations. The initial appropriate treatment for fungal infections is with azoles. However, the frequent occurrence of drug resistance is the main limitation. Therefore, identification of the underlying inborn error if immunity in CMC may allow to widen therapeutic options aimed at restoring immunological function. Type I and II Janus kinase-inhibitors have been shown to control CMC in cases associated with STAT1 GOF. In this review, we delve into the pathogenesis of CMC and the underlying immune mechanisms. We describe the reported genetic defects in which CMC is the main manifestation. Diagnostic and therapeutic approaches for these patients are also offered
RESUMEN
No disponible
Full text:
Available
Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Candidiasis, Chronic Mucocutaneous
Limits:
Humans
Language:
English
Journal:
Allergol. immunopatol
Year:
2021
Document type:
Article
Institution/Affiliation country:
Hospital Sant Joan de Déu/Spain
/
Hospital Sant Joan de Déu-Hospital Clínic de Barcelona/Spain
/
Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS)/Spain
/
Institut de Recerca Sant Joan de Déu/Spain
/
Sociedad Española de Inmunología Clínica Alergología y Asma Pediátrica (SEICAP)/Spain
/
Universitat de Barcelona/Spain