The Polyendocrine Deficiency Syndromes

IT is currently thought that two distinct disorders are grouped under the general rubric of polyendocrine deficiency syndrome. The first disorder begins at a mean age of about 12 years and is characterized by the association of hypoparathyroidism, primary adrenal insufficiency, and mucocutaneous candidiasis. Alopecia, pernicious anemia, malabsorption, and chronic active hepatitis also occur. This disorder is currently designated as the polyendocrine deficiency syndrome Type I.¹ The inheritance pattern is not always clear, but in some families an autosomal recessive pattern has been observed.² The second disorder begins at an average age of 30 years and is recognized by the.