This is an autosomal recessive polyglandular deficiency state characterised by childhood onset and defective cellular immunity to Candida albicans which is refractory to conventional chemotherapy; however remission is reported with ketoconazole plus transfer factor. There is no HLA linkage.
The autoimmune element comprises:
The associated disorders must be managed conventionally even if the Candida is successfully treated.
Last reviewed 01/2018