Chronic mucocutaneous candidiasis

Chronic mucocutaneous candidiasis is a rare condition occurring in childhood in which candida infection is persistent and widespread.

It may be associated with:

• 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 e.g. hypoparathyroidism, hypothyroidism, hypoadrenalism, diabetes mellitus.
• Immune defects i.e. 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

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).

Diagnosis

The diagnosis of chronic mucocutaneous candidiasis, is made on clinical grounds. Microscopy and culture of skin swabs and scrapings confirm the presence of organisms.

Management

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.

• Fluconazole
• Itraconazole
• Ketoconazole

Drug resistance may occur.