Chronic mucocutaneous candidiasis

Key words: Chronic mucocutaneous candidiasis, Oral thrush, Onychomycosis, Interleukin-17, Fluconazole

The mucocutaneous candidiasis (CMC) is a chronic condition characterized by persistent or recurrent Candida infections ranging from superficial infections such as oral thrush, candidal onychomycosis and genital mucosa infections to systemic potentially life-threatening infections. Although oral thrush is a common finding in normal infants, relapsing Candida infections can constitute an alerting sign of primary immunodeficiencies. In severe combined immunodeficiencies, chronic granulomatous disease, common variable immunodeficiency, or hyper IgE syndromes (e.g., Job syndrome, DOCK8 syndrome) fungal infections are usually associated with viral or bacterial infections. In other cases, CMC is associated to autoimmune polyendocrinopathy and ectodermal dystrophy (an autosomal recessive condition known as APECED). Finally, CMC patients may present selected susceptibility to Candida without evidence of other accompanying manifestations. Heterozygous mutations of STAT1 constitute the leading cause of isolated CMC, but CARD9 and CLEC7A mutations have also been observed in patients with autosomal recessive CMC. Treatment of CMC patients is based on daily prophylaxis with fluconazole or itraconazole, but resistance to antifungal drugs has also been observed.