From the Guidelines
Mucocutaneous candidiasis is caused by autoimmune polyendocrinopathy syndrome type 1 and thymoma, which are autoimmune diseases that lead to immunodeficiency and increased susceptibility to Candida infections 1.
Key Autoimmune Diseases
- Autoimmune polyendocrinopathy syndrome type 1: a rare autoimmune disorder that affects multiple endocrine glands and increases the risk of mucocutaneous candidiasis 1
- Thymoma: a type of tumor that affects the thymus gland and can lead to immunodeficiency and mucocutaneous candidiasis 1
Treatment Considerations
Treatment of mucocutaneous candidiasis typically involves fluconazole as initial therapy, with chronic suppressive antifungal therapy often required to prevent relapses 1. In cases of fluconazole-refractory infections, alternative treatments such as itraconazole solution or posaconazole suspension may be effective 1.
From the Research
Autoimmune Diseases Causing Mucocutaneous Candidiasis
- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) is a rare autosomal recessive disorder that causes chronic mucocutaneous candidiasis (CMC) 2.
- APECED is characterized by a variable combination of endocrine failure, CMC, and dystrophy of the dental enamel and nails 2.
- Autoimmune polyendocrine syndrome type I (APS-I) is also associated with CMC, and patients with APS-I often display autoantibodies against IL-17A, IL-17F, and IL-22 3.
- CMC in APS-I may result from autoimmunity to interleukin (IL)-17 cytokines 3.
- Chronic mucocutaneous candidiasis can be complicated by systemic candidiasis or oral squamous cell carcinoma (SCC) in patients with APECED 4.
Underlying Immune Mechanisms
- The key immune defect in CMC is a disruption of the action of cytokine IL-17, whose most common genetic etiology is STAT1 gene gain-of-function (GOF) mutations 5.
- Inborn errors in IL-17-mediated pathways or Candida spp. sensing molecules are known to cause CMC 6.
- The adaptive and innate immune system components play a significant role in anti-fungal response, which is mediated through IL-17 production by T helper cells 6.