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Chronic mucocutaneous candidiasis is a hereditary immunodeficiency disorder due to malfunction of T cells (lymphocytes).
Because T cells malfunction, the body is less able to fight fungal infections, including infection with Candida (candidiasis—see Fungal Infections: Candidiasis), a yeast. The ability to fight other infections is not reduced.
Candidal infections develop and persist, usually beginning during infancy but sometimes during early adulthood. The fungus may cause mouth infections (thrush) and infections of the scalp, skin, and nails. Membranes lining the mouth, eyelids, digestive tract, and vagina may also be infected. In infants, the first symptoms are often thrush that is difficult to treat, diaper rash, or both. Severity varies. The disorder may affect one nail or cause a disfiguring rash that covers the face and scalp. The rash is crusted and thick and may ooze. On the scalp, the rash may cause hair to fall out. Many people also have endocrine disorders, such as underactive parathyroid glands (hypoparathyroidism) and underactive adrenal glands (Addison's disease), as well as hepatitis and autoimmune disorders, such as Graves' disease.
Doctors suspect this infection when people have the characteristic rash. The diagnosis can be confirmed by examining a sample from the infected area under a microscope and identifying the yeast.
Usually, the infections can be controlled with an antifungal drug applied to the skin. If infections persist, they can be effectively treated with fluconazole or another similar antifungal drug taken by mouth. Drugs may have to be taken for a long time. Usually, this disorder is chronic but does not affect life span.
Last full review/revision September 2008 by Rebecca H. Buckley, MD
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