Familial chronic muco-cutaneous candidiasis.

Chronic oral candidiasis has been described in association with abnormalities of the endocrine system on many occasions, both in case reports (Sutphin, Albright, and McCune, 1943), and more general reviews (Kunin et al, 1963). Chronic candidiasis has also been recorded in the familial thymic aplasia described by Nezelof (Nezelof et al, 1964), Swiss-type agammaglobulinaemia (Hitzig, 1968) and DiGeorge's syndrome (DiGeorge, 1965). More recently deficiency of the migration inhibitory factor (MIF), where cell mediated immunity appears to be partially defective, has been shown to be a feature of some patients with chronic candidiasis (Valdimarsson et al, 1970). It has also been associated with defective granulocyte function, as in chronic granulomatous disease (Quie et al, 1967) and myeloperoxidase deficiency (Lehrer and Cline, 1969). During the last 3 years a group of patients has been investigated (Wells, 1970) who have chronic oral candidiasis with no other significant clinical abnormality. In some of the kindreds, sibs were found to have the disorder, and there was often a family history of consanguinity. We have suggested that this new group, hitherto undescribed and which we have called familial chronic mucocutaneous candidiasis (FCMC), has a genetically determined abnormality, inherited as an autosomal recessive trait which results in susceptibility to candida infection. It is possible that an inherited biochemical or immunological abnormality results in this picture.