The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency.

BACKGROUND: Most patients with chronic mucocutaneous candidiasis (CMC) have a selective defect of cell-mediated immunity against Candida albicans (as demonstrated by cutaneous anergy and decreased lymphoproliferative responses to Candida antigen) and intact antibody responses. Many CMC patients also develop infections with other organisms, suggesting a more extensive immunologic defect.

OBJECTIVES: The aim of this study was to describe a patient with CMC and selective antibody deficiency and identify eight similar previously reported patients.

DATA SOURCES: Relevant articles in the English language derived from searching the MEDLINE database were used.

RESULTS: We describe an 18-year-old male patient who was identified with CMC as an infant and later developed immunoglobulin (Ig)G2, IgG4, and IgA deficiency at age 12 associated with poor antibody responses to vaccine antigens. We have identified eight other previously reported CMC patients with selective antibody deficiencies and bacterial infections. IgG2 deficiency was present in all nine patients, and was associated with IgG4 deficiency in 8 patients and IgA deficiency in 3 patients. Six patients had poor or absent antibody responses to pneumococcal polysaccharide vaccine, and all nine patients developed severe recurrent lung infections.

CONCLUSIONS: We suggest that these cases represent a distinct phenotype of CMC and should be studied for common histocompatibility leukocyte antigen types and molecular defects.