CHRONIC MUCOCUTANEOUS
CANDIDOSIS
Hay
R.J.,
St John’s Institute of Dermatology ( KCL ), St Thomas’ Hospital,
London, UK
Chronic mucocutaneous candidosis ( CMC ) is a chronic condition presenting, usually, in
early childhood with persistent superficial ( mouth, skin and nail )
infections caused by Candida species, and often later complicated by
other infections including dermatophytosis and human papilloma ( HPV )
virus infections and seborrhoeic dermatitis. There are now know to be at
least six different variants of CMC presenting as recessive or dominant
conditions with or without endocrinopathy ( recessive – hypoparathyroidism,
hypoadrenalism, dominant – hypothyroidism ) and sporadically occurring or
adult onset associated with thymoma types. Immunological deficiencies
identified range from defective phagocytic killing of Candida to an
enhanced TH2 switch process. These do not necessarily reflect the
heterogeneous nature of CMC. Clinical progress is variable and many
children improve in their late teens. Systemic involvement apart from
bronchiectasis is rare, but can occur eg cryptococcal meningitis,
tuberculous peritonitis. Of 26 children studied in the Institute of
Dermatology, London complications appearing late in the disease include:
bronchiectasis ( 4) oesophageal stenosis ( 4 ), hyperkeratotic
dermatophytosis ( 4 ), squamous carcinoma of the oral mucosa ( 3),
refractory warts ( 5). Long term management involves several specific goals
which include the avoidance of antifungal resistance ( occurring in 5/26
patients ), genetic counselling, and early identification of complications.
Endocrine disease may develop years after the first appearance of oral
candidosis and children should be screened at regular intervals.
Immunological therapy has played only a small role in the management of
these patients.