Chronic mucocutaneous candidiasis
What is chronic mucocutaneous candidiasis?
Chronic mucocutaneous candidiasis is a rare condition occurring in childhood in which candida infection is persistent and widespread. 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.
- Genetic predisposition with autosomal dominant inheritance (i.e. an abnormal gene from one parent) or autosomal recessive inheritance (an abnormal gene is inherited from each parent).
- Endocrine conditions: hypoparathyroidism, hypothyroidism, hypoadrenalism, diabetes mellitus (autoimmune polyendocrinopathy type 1, or APECED syndrome in which there is mutation of the AIRE gene).
- Immune defects include malfunctioning T-lymphocytes, low levels of immunoglobulin.
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, myositis, aplastic anaemia, neutropaenia and hypogammaglobulinaemia.
Clinical features of chronic mucocutaneous candidiasis
Chronic mucocutaneous candidiasis usually presents before the age of 3 years with one or more of the following:
- Chronic oral candidiasis (thrush).
- Paronychia and candidal onychomycosis (nail infection).
- Widespread candidiasis of the skin, especially scalp, trunk, hands and feet.
- Granulomas (scaly crusted lumps in the mouth, on the skin and/or nails).
Affected patients are also susceptible to infection with dermatophyte fungi (tinea) and human papilloma virus (warts).
How is chronic mucocutaneous candidiasis diagnosed?
The diagnosis of chronic mucocutaneous candidiasis, is made on clinical grounds. Microscopy and culture of skin swabs and scrapings confirm the presence of organisms.
How is chronic mucocutaneous candidiasis treated?
The underlying defect is not as yet treatable. Treatment depends on antifungal agents orally for repeated or prolonged courses often in higher doses than is normally necessary for candida infections.
Drug resistance may occur.
