Cushing's syndrome due to primary pigmented nodular adrenocortical disease
- André Lacroix, MD
André Lacroix, MD
- Section Editor — Adrenal Disease
- Professor of Medicine
- University of Montreal, Quebec, Canada
Among the causes of Cushing's syndrome are three rare types of nodular adrenocortical diseases that are usually bilateral:
●Adrenocorticotropic hormone (ACTH)-dependent bilateral macronodular hyperplasia secondary to long-term adrenal stimulation in patients with Cushing's disease (pituitary ACTH-secreting tumor) or ectopic ACTH syndrome
●Primary bilateral macronodular adrenal hyperplasia (BMAH)
●ACTH-independent micronodular hyperplasia or dysplasia and its most frequent variant, primary pigmented nodular adrenocortical disease (PPNAD), which may be sporadic or familial (as part of the Carney complex [CNC])
PPNAD and other micronodular adrenocortical disease will be reviewed here. Cushing's disease and BMAH are discussed separately. (See "Causes and pathophysiology of Cushing's syndrome" and "Cushing's syndrome due to primary bilateral macronodular adrenal hyperplasia".)
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- CLINICAL PRESENTATION
- Sporadic or familial
- Cushing's syndrome
- Carney complex (CNC)
- Initial evaluation
- Adrenal imaging
- Sequential low-dose-high-dose dexamethasone suppression test (Liddle's test)
- Pharmacologic therapy
- SUMMARY AND RECOMMENDATIONS