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Start free with EleplanCushing syndrome due to bilateral macronodular adrenocortical disease
ORPHA:189427Disease
Also called CS due to BMACD · CS due to BMAD · Cushing syndrome due to BMACD · Cushing syndrome due to BMAD · Cushing syndrome due to macronodular adrenal hyperplasia · Cushing syndrome due to primary bilateral macronodular adrenal hyperplasia
What it is
A rare adrenal Cushing syndrome characterized by bilateral benign adrenal macronodules (>1 cm) that potentially produce autonomously variable levels of cortisol excess. Although in most cases are ACTH-independent, non-suppressed ACTH levels have been described.
Key facts
- Prevalence
- <1 / 1 000 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
21- Abdominal obesity
- Abnormal libido
- Abnormal response to corticotropin releasing hormone stimulation test
- Bruising susceptibility
- Depression
- Dorsocervical fat pad
- Emotional lability
- Glucose intolerance
- Hirsutism
- Hyperlipidemia
- Hypertension
- Increased circulating cortisol level
- Increased urinary cortisol level
- Irregular menstruation
- Nephrolithiasis
- Osteoporosis
- Paradoxical increased cortisol secretion on dexamethasone suppression test
- Plethora
- Proximal amyotrophy
- Proximal muscle weakness
- Striae distensae
Sometimes5–29%
8These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
1 modifying gene — variants that can change how the disease behaves, not cause it
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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