Autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy

Patients with APECED have autoimmune polyendocrinopathies (hypoparathyroidism, adrenocortical failure, IDDM, gonadal failure, hypothyroidism, pernicious anemia, and hepatitis), chronic mucocutaneous candidiasis, ectodermal dystrophies (vitiligo, alopecia, keratopathy, dystrophy of dental enamel, nails and tympanic membranes). Chronic candidiasis is classically the first clinical sign, occurring before the age of 5. It is followed by hypoparathyroidism and the Addison disease. In addition, a high proportion of patients develop squamous cell carcinoma of the oral mucosa.

Therapeutic options

  • Candidiasis needs an antimycotic treatment. Hypoparathyroidism can be influenced by calcium and vitamin D therapy. In case of adrenal failure hydrocortisone replacement is needed. Vitamin and mineral replacement can be useful to compliment hormonal replacement.
  • Poliglandular autoimmune syndrome, type I, eMedicine

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