Chronic mucocutaneous candidiasis is persistent or recurrent candidal infection due to T-cell defects.
Inheritance is autosomal dominant or recessive. Patients have cutaneous anergy to Candida, absent proliferative responses to Candida antigen (but normal proliferative responses to mitogens), and intact antibody response to Candida and other antigens. Candidiasis recurs or persists, usually beginning during infancy but sometimes during early adulthood. Life span is not affected.
Patients with the recessive form (autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy) develop endocrine disorders (eg, hypoparathyroidism, adrenal insufficiency, hypogonadism, thyroid disease, diabetes), and hepatitis.
Thrush is common, as are infections of the scalp, skin, nails, and GI and vaginal mucosa. Severity varies. Nails may be thickened, cracked, and discolored, with edema and erythema of the surrounding periungual tissue, resembling clubbing. Skin lesions are crusted, pustular, erythematous, and hyperkeratotic. Scalp lesions may result in scarring alopecia. Infants often present with refractory thrush, candidal diaper dermatitis, or both.
Diagnosis is based on the presence of recurrent candidal skin or mucosal lesions when no other known causes of candidal infection (eg, diabetes, antibiotic use) are present. Candidal lesions are confirmed by other tests (eg, by potassium hydroxide wet mount of scrapings).
Usually, the infections can be controlled with a topical antifungal. However, long-term treatment with a systemic antifungal drug (eg, amphotericin B, fluconazole, ketoconazole) may be needed. Topical antifungals are usually ineffective. Sometimes an immunomodulator (eg, transfer factor) is also used.
Last full review/revision September 2008 by Rebecca H. Buckley, MD