Rare diseases · Sign or symptom
Primary adrenal insufficiency
HP:0008207
What it means
Insufficient production of steroid hormones (primarily cortisol) by the adrenal glands as a result of a primary defect in the glands themselves.
Deficiency of both glucocorticoids and mineralocorticoids.
In everyday care
Most people with this sign do not have a rare disease — it appears in common conditions far more often. These general reference pages cover the same topic:
Rare diseases that can present with this23
Very common80–99%
9- 46,XY difference of sex development-adrenal insufficiency due to CYP11A1 deficiency
- Adrenomyodystrophy
- Autoimmune polyendocrinopathy type 1
- Autoimmune polyendocrinopathy type 2
- Inherited isolated adrenal insufficiency due to partial CYP11A1 deficiency
- Isolated anencephaly/exencephaly
- Neonatal adrenoleukodystrophy
- X-linked cerebral adrenoleukodystrophy
- Xp21deletion syndrome
Common30–79%
3The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 2 diseases where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Addison disease · Addison's disease · Adrenocortical insufficiency · Primary adrenocortical failure
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.