Primary adrenal deficiency

Hyperpigmentation of the buccal mucosaPrimary adrenal deficiencyPrimary adrenal deficiency
  • History / PE:
    Hyperpigmentation (increased ACTH)
    Fatigue
    Orthostatic hypotension
    Weight loss
    GI complaints
  • Diagnosis:
    Markedly elevated ACTH
    Eosinophilia
    Hyponatremia , hyperkalemia
    Metabolic acidosis
    Synthetic ACTH stimulation test (confirmatory)
  • Complications:
    Addisonian crisis
  • Notes:
    Random plasma cortisol > 20 micrograms/dL excludes diagnosis
    Only see hyperpigmentation in primary adrenal insufficiency, secondary to increased levels of ACTH
  • Associated With:
    Addison's disease (autoimmune primary adrenocortical deficiency)
    Autoimmune adrenalitis (MCC in developing countries)
    Cessation of exogenous steroid use
    Tuberculosis (see adrenal calcifications)
    CMV
    Waterhouse-Friedrichsen syndrome
    Fungal infections
  • Differential Diagnosis:
    Peutz-Jeghers syndrome



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August 4th 2010