Chronic mucocutaneous candidiasis in childhood and complications of non-Candida infection: a report of the Pediatric Immunodeficiency Collaborative Study Group

J Pediatr. 1990 Mar;116(3):377-82. doi: 10.1016/s0022-3476(05)82824-0.


We reviewed the clinical course in 43 patients from eight medical centers who were given the diagnosis of chronic mucocutaneous candidiasis, a rare disorder of unknown cause that may occur in childhood. Recurrent or severe infections with organisms other than Candida were seen in 80% of the patients. There were nine cases of septicemia. Seven patients have died; six of these deaths were directly related to non-Candida infectious complications. Endocrine dysfunction, including Addison disease (11 patients) and hypothyroidism (9 patients), was seen in 19 of 43 patients. Immunologic studies failed to reveal a consistent abnormality, although two of five patients with reversed T4/T8 ratios are among those who have died. Ketoconazole was effective in controlling symptoms of candidiasis in most patients. The findings from this study indicate that non-Candida infections cause serious morbidity and may result in death in patients with chronic mucocutaneous candidiasis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Amphotericin B / therapeutic use
  • Autoimmune Diseases / epidemiology
  • Candidiasis / complications*
  • Candidiasis, Chronic Mucocutaneous / complications*
  • Candidiasis, Chronic Mucocutaneous / drug therapy
  • Candidiasis, Chronic Mucocutaneous / immunology
  • Child
  • Child, Preschool
  • Endocrine System Diseases / complications
  • Family
  • Female
  • Flucytosine / therapeutic use
  • Humans
  • Immunoglobulin A / metabolism
  • Immunoglobulin G / metabolism
  • Incidence
  • Infant
  • Infections / complications*
  • Infections / epidemiology
  • Infections / mortality
  • Ketoconazole / adverse effects
  • Ketoconazole / therapeutic use
  • Lung Diseases / complications
  • Male
  • Survival Rate


  • Immunoglobulin A
  • Immunoglobulin G
  • Amphotericin B
  • Flucytosine
  • Ketoconazole