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Author: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2003. Updated by Dr Jannet Gomez, August 2017.
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.
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.
Chronic mucocutaneous candidiasis usually presents before the age of 3 years, with one or more of the following:
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.
The diagnosis of chronic mucocutaneous candidiasis is made on clinical grounds.
Genetic testing may reveal a specific mutation.
The underlying defect is not as yet treatable.
To reduce the incidence and severity of candida infection:
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.
Options to improve immune status include:
Evaluation and treatment of associated endocrinopathies should be undertaken in consultation with an endocrinologist.
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