Primary pigmented nodular adrenocortical disease

rare

A form of bilateral adrenocortical hyperplasia that is often associated with adrenocorticotrophin hormone (ACTH) independent Cushing syndrome and is characterized by small to normal sized adrenal glands containing multiple small cortical pigmented nodules (less than 1 cm in diameter).

Identifiers: MONDO:0015999 Orphanet:189439
Part of: Adrenal gland disorder Hereditary disease
Also known as: pigmented nodular adrenocortical disease · pigmented nodular adrenocortical disease, primary · PPNAD · primary pigmented nodular adrenal dysplasia

Subtypes

All diseases Rare only

Disease	ID	Genes
Isolated primary pigmented nodular adrenocortical disease	MONDO:0958262
Pigmented nodular adrenocortical disease, primary, 1	MONDO:0012509	PRKAR1A
Pigmented nodular adrenocortical disease, primary, 2	MONDO:0012505	PDE11A
Pigmented nodular adrenocortical disease, primary, 3	MONDO:0013616	PDE8B
Pigmented nodular adrenocortical disease, primary, 4	MONDO:0014359	PRKACA

Rare disease

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