Primary pigmented micronodular disease of the adrenals

J Endocrinol Invest. 1990 Feb;13(2):171-5. doi: 10.1007/BF03349532.

Abstract

Primary pigmented micronodular disease is a peculiar form of ACTH-independent Cushing's syndrome characterized by the familial occurrence, the frequent association with malformations and the pathological adrenocortical picture consisting in micronodules with cellular deposition of lipofuscinic pigment. We describe here a case occurring in a 14-year-old girl.

Publication types

  • Case Reports

MeSH terms

  • Adipose Tissue / pathology
  • Adolescent
  • Adrenal Cortex / pathology*
  • Adrenal Medulla / pathology
  • Adrenocorticotropic Hormone / blood
  • Cushing Syndrome / metabolism
  • Cushing Syndrome / pathology*
  • Dexamethasone
  • Female
  • Humans
  • Hydrocortisone / blood
  • Hydroxysteroids / urine
  • Lipofuscin / analysis*
  • Lymphocytes / pathology
  • Metyrapone
  • Pigments, Biological / analysis*

Substances

  • Hydroxysteroids
  • Lipofuscin
  • Pigments, Biological
  • Dexamethasone
  • Adrenocorticotropic Hormone
  • Hydrocortisone
  • Metyrapone