Persistent infection with Candida usually occurs as a result of a defect in cell-mediated immunity or may be associated with iron deficiency. Hyperplastic mucocutaneous lesions, localized granulomas, and adherent white plaques on affected mucous membranes are the prominent lesions that identify chronic mucocutaneous candidiasis (CMC) (Figure 5-24).
In many cases, persistent and significant predisposing factors can be identified. Two categories of CMC have been described: (1) syndrome-associated CMC and (2) localized and diffuse CMC. Syndrome-associated CMC is further categorized as either familial or chronic. The familial form, candidiasis endocrinopathy syndrome (CES), is a rare autosomal recessive disorder characterized by an onset of CMC during infancy or early childhood, associated with the appearance of hypoparathyroidism, hypoadrenocorticism, and other endocrine anomalies.Patients develop persistent oral candidiasis and hyperplastic infections of the nail folds at an early age. Some patients also have low serum iron and iron-binding capacity. The other syndrome-associated form is chronic candidiasis associated with thymoma, which appears with other autoimmune abnormalities such as myasthenia gravis, polymyositis, bullous lichen planus,and hypogammaglobulinemia. Localized CMC is a variant associated with chronic oral
candidiasis and lesions of the skin and nails. Lesions usually begin within the first two decades of life. The diffuse variant is characterized by randomly occurring cases of severe mucocutaneous candidiasis with widespread skin involvement and development of Candida granulomas. It is often associated with other opportunistic fungal and bacterial infections. A majority of patients are iron deficient.
Both oral and cutaneous lesions of CMC can be controlled by the continuous use of systemic antifungal drugs; once treatment is discontinued, however, the lesions rapidly reappear.