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Chronic Mucocutaneous Candidiasis

By

James Fernandez

, MD, PhD, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University

Last full review/revision Apr 2021| Content last modified Apr 2021
Click here for the Professional Version
Topic Resources

Chronic mucocutaneous candidiasis, a hereditary immunodeficiency disorder, is persistent or recurring infection with Candida (a fungus) due to malfunction of T cells (lymphocytes).

  • Chronic mucocutaneous candidiasis causes frequent or chronic fungal infections of the mouth, scalp, skin, and nails.

  • To diagnose the disorder, doctors examine a sample from the infected area under a microscope and do blood tests to check for the mutations that cause the immunodeficiency.

  • Antifungal drugs can usually control the infection, but they must be taken for a long time.

Chronic mucocutaneous candidiasis is due to a mutation in specific genes. Depending on which gene has the mutation, one or two mutations (one from each parent) may be needed to cause the disorder.

Symptoms

In people with chronic mucocutaneous candidiasis, candidal infections develop and recur or persist, usually beginning during infancy but sometimes during early adulthood.

In infants, the first symptoms are often thrush that is difficult to treat, diaper rash, or both. Severity varies.

Chronic mucocutaneous candidiasis may cause one or more nails to thicken, crack, and become discolored. A disfiguring rash may cover the face and scalp. The rash is crusted and thick and may ooze. On the scalp, the rash may cause hair to fall out.

Candidal Infections

Usually, this disorder is chronic, but it does not affect life span.

Many people also have the following:

Diagnosis

  • Examination of a sample from the infected area under a microscope

  • Sometimes genetic testing

Doctors suspect Candida infection when people frequently have mouth, scalp, skin, and nail infections with the characteristic skin changes. Examining a sample from the infected area under a microscope and identifying the yeast can confirm that a Candida infection is the cause.

Because people without an immunodeficiency disorder occasionally develop Candida infection, doctors then check for common risk factors for Candida infection, such as diabetes or recent use of antibiotics. If people with frequent Candida infections have no risk factors for Candida infections, the diagnosis is likely to be chronic mucocutaneous candidiasis.

Blood tests to check for a specific genetic mutation can confirm the diagnosis.

Treatment

  • Antifungal drugs

  • Sometimes immune globulin

Usually, the infections of chronic mucocutaneous candidiasis 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.

Immune globulin (antibodies obtained from the blood of people with a normal immune system) is sometimes given. It may be injected into a vein (intravenously) once a month or under the skin (subcutaneously) once a week or once a month.

Endocrine and autoimmune disorders are treated as needed. Stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more has been used in a few people with specific genetic mutations; however, transplantation is not often used in people with chronic mucocutaneous candidiasis.

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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