Severe chronic mucocutaneous candidiasis. Favourable response to oral therapy with ketoconazole.

Severe, early-onset, chronic mucocutaneous candidiasis was associated with bronchiectasis, oesophageal stricture, short stature and delayed puberty in a male aged 18 years. Topical treatment with antifungal agents, and several courses of intravenously administered transfer factor, amphotericin, and miconazole achieved only minor or transient improvement in the patient's condition. Correction of iron deficiency anaemia did not lead to alleviation of candidiasis. Skin reactivity to Candida antigen was absent and T-lymphocytes, which responded normally to phytohaemagglutinin (PHA), poke-weed mitogen and concanavalin A, had negative macrophage-inhibiting factor (MIF) and blastogenic responses to Candida antigen. Treatment with the orally effective imidazole derivative, ketoconazole, produced improvement within three days and clearing of mucosal lesions within five weeks. The patient then entered puberty spontaneously at the age of 20 years. After 18 months of treatment with ketoconazole, without side effects, the clinical manifestations of mucocutaneous candidiasis have not recurred. Because of the possibility of continuous, long-term administration, ketoconazole represents the best currently available agent for the treatment of chronic mucocutaneous candidiasis.