Chronic mucocutaneous moniliasis with impaired delayed hypersensitivity

Abstract

Three patients, including two brothers, with chronic mucocutaneous moniliasis and endocrinopathy were evaluated from an immunological viewpoint. Each patient had defective delayed hypersensitivity to Candida albicans as manifested by a negative skin test and absent lymphocyte response after in vitro exposure to the antigen. Cutaneous responses to other antigens were intact, and there were no demonstrable abnormalities in humoral immunity. In one case, the in vitro lymphocyte response was restored following an injection of a leucocyte extract from a skin test positive subject, although the cutaneous lesions remained unchanged.

It is possible that cells of both the endocrine and lymphoid systems share a defect in synthesis and release of their respective humoral products. In the case of lymphoid cells, the defect would be limited to populations of cells participating in delayed hypersensitivity, and impair production of mediators such as macrophage migration inhibiting factor, lymphotoxin, etc., while sparing the processes of antibody synthesis. Alternatively, the selective failure in delayed hypersensitivity may result from defective lymphocyte differentiation or from loss of immune responsiveness through densensitization by chronic exposure to the fungal antigens.