Chronic mucocutaneous candidiasis

Author: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2003. Updated by Dr Jannet Gomez, August 2017.

What is chronic mucocutaneous candidiasis?

Chronic mucocutaneous candidiasis is a rare genetic condition occurring in childhood that affects skin, nails and mucosal sites. It is characterised by persistent and widespread candida infection. It is also called chronic mucocutaneous candidosis.

What causes chronic mucocutaneous candidiasis?

Chronic mucocutaneous candidiasis is due to primary immune deficiency. It is associated with various syndromes and laboratory abnormalities.

Rarely, chronic mucocutaneous candidiasis develops in adult life. This is often as a result of a thymoma (tumour of the thymus gland) and is associated with internal diseases such as myasthenia gravis or myositis, or bone marrow defects such as aplastic anaemia, neutropenia and hypogammaglobulinaemia.

Human immunodeficiency virus (HIV) infection and hyper-IgE syndrome may also present with chronic mucocutaneous candidiasis.

Clinical features of chronic mucocutaneous candidiasis

Chronic mucocutaneous candidiasis usually presents before the age of 3 years, with one or more of the following:

Affected patients are also susceptible to infection with dermatophyte fungi (tinea) and human papilloma virus (warts).

Complications from chronic mucocutaneus candidiasis

Chronic mucocutaneous candidiasis may result in premature death due to disseminated candida infection, sepsis, pneumonia, or mycotic aneurysms.

Patients have an increased risk of skin cancer, and ear, nose, and oesophageal cancers.

How is chronic mucocutaneous candidiasis diagnosed?

The diagnosis of chronic mucocutaneous candidiasis is made on clinical grounds.

Microscopy and culture of skin swabs and skin scrapings confirm the presence of organisms.

Genetic testing may reveal a specific mutation.

How is chronic mucocutaneous candidiasis treated?

The underlying defect is not as yet treatable.

General measures

To reduce the incidence and severity of candida infection:

Antifungal treatment

Treatment depends on repeated or prolonged courses of oral antifungal medications, often in higher doses than is normally necessary for candida infections.  Oral antifungal medications used for chronic mucocutaneous candidiasis include:

Drug resistance may occur.

Immunological therapy

Options to improve immune status include:

Evaluation and treatment of associated  endocrinopathies should be undertaken in consultation with an endocrinologist.

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