Chronic Mucocutaneous Candidiasis in Korea / 대한의진균학회지

ABSTRACT

Chronic mucocutaneous candidiasis (CMCC) is a general term used to denote a complex group of disorders characterized by a recurrent and persistent infection of the skin, mucous membranes and nails with organism of the genus candida, most frequently Candida albicans. It is often associated with an endocrinopathy and cell mediated immunopathy. CMCC is not a single disease entity, but rather a final common pathway for multiple predisposing abnormalities of the immune system that ranges from severe, life-threatening immunodeficiency syndromes to subtle deficiencies, especially of cell mediated immunity. Conditions that have been associated with CMCC include; candida esophagitis or laryngitis, endocrinopathies (usually hypoparathyroidism, hypadrenalism, hypothyroidism), circulating autoimmune antibodies, diabetes mellitus, vitiligo with antibodies to melanocytes, iron deficiency, chronic active hepatitis, pernicious anemia, malabsorption, alopecia totalis, dental enamel dysplasia, keratoconjunctivitis, pulmonary fibrosis, KED syndrome (keratitis, ichthyosis, and deafness), and recurrent pyogenic, viral or other fungal infections. When CMCC first appaears in adulthood, it is often associated with a thymoma. There is virtually no propensity for disseminated, visceral candidiasis. A suitable clinical classification of the major subtypes of CMCC was described by Lehner and Wells et al into six groups. Kirkpatrick et al found the mean age of onset of CMCC to be 3 years and both boys and girls are affected equally. We review CMCC and 5 reported CMCC cases in the dermatologic literatures in Korea.