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Cushing's syndrome due to primary pigmented nodular adrenocortical disease

André Lacroix, MD
Section Editor
Lynnette K Nieman, MD
Deputy Editor
Kathryn A Martin, MD


Among the causes of Cushing's syndrome are three rare types of nodular adrenocortical diseases that are usually bilateral:

Corticotropin (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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Literature review current through: Nov 2017. | This topic last updated: Mar 16, 2016.
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