A 6-year-old girl with chronic mucocutaneous candidiasis (MCC) and an immunologic deficiency syndrome was studied in detail over a period of 2 years. The patient had a severe defect in cell-mediated immunity characterized by failure to mount a 48-hr delayed response to a battery of intradermal skin tests, persistent lymphopenia, and failure to be sensitized with 1-chloro, 2,4-dinitro-benzene (DNCB). Her lymphocytes neither produced migratory inhibitory factor nor consistently responded with an increased 3H-thymidine uptake when placed in tissue culture medium with Candida antigen. There was no evidence of a circulating factor in the patient's plasma capable of inhibiting the incorporation of 3H-thymidine by homologous lymphocytes cultured in the presence of Candida albicans and other antigens. The patient had defective antibody production to some but not all antigens. She failed to produce protective tetanus toxoid antibody titers and remained Schick test positive despite repeated diphtheria, pertussis, and tetanus (DPT) injections. She also had defective antibody responses to repeated paratyphoid-typhoid injections and was found to have a low serum IgA level. This patient possessed autoantibodies against smooth muscle and parietal cells and an inconstant positive antilymphocytotoxic assay. Immunologically unrelated precipitin antibody systems against human and calf thymus were also demonstrated in the patient's serum. Eradication of the Candida infection with intravenous amphotericin B, although resulting in marked clinical improvement, did not alter the immunologic abnormalities.
ASJC Scopus subject areas
- Immunology and Allergy
- Pathology and Forensic Medicine