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Updated: Oct 31 2018

Chronic Mucocutaneous Candidiasis

Images candidiasis.jpg
  • Snapshot
    • A 7-year-old presents to the pediatrician with a “white tongue.” On physical exam, he has notable thrush in his oral mucosa. His past medical history includes chronic diaper rash as a baby. It is otherwise unremarkable. Physical exam shows malformed nails on two fingers, consistent with onychomycosis. Labs are within normal limits. He is started on fluconazole.
  • Introduction
    • Primary immunodeficiency from dysfunction of T-cells
    • Recurrent infections with noninvasive Candida albicans
    • May also be susceptible to other bacterial and viral infections
    • Genetics
      • familial autosomal recessive
      • sporadic autosomal dominant
    • Pathogenesis
      • absent T-cell proliferation
      • autoimmune regulator deficiency (AIRE)
    • Associated conditions
      • autoimmune diseases
        • notably endocrinopathies
  • Presentation
    • Symptoms due to C. albicans infection of the following
      • skin
      • oral mucosa
        • thrush
      • nails
      • diaper area in infants
  • Evaluation
    • All patients should be evaluated for other immunodeficiencies
      • CBC (normal in chronic mucocutaneous candidiasis [CMC])
      • Ig levels (normal in CMC)
    • Absent in vitro response of T-cells to Candida antigen
    • Absent cutaneous reaction to Candida antigen
    • Genetic testing for mutations
  • Differential Diagnosis
    • SCID
    • Immunodeficiency caused by HIV
  • Treatment
    • Chronic therapy with fluconazole
  • Prognosis, Prevention, and Complications
    • Prognosis
      • normal
    • Complications
      • severe lung infections
      • sepsis
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