Pigmented Nodular Adrenocortical Disease Primary 4 C4014425
A rare adrenal gland disorder causing excessive hormone production. It often leads to Cushing's syndrome features and is typically treated with surgical removal of the affected gland.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Central obesity
(endocrine)Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hypertension
(endocrine)Purple striae
(endocrine)Easy bruising
(endocrine)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Bilateral adrenalectomy
Surgical removal of both adrenal glands to eliminate cortisol hypersecretion in pigmented nodular adrenal disease unresponsive to medical management.
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Glucocorticoid replacement therapy
Lifelong hormone replacement with hydrocortisone and fludrocortisone to replace adrenal function after bilateral adrenalectomy.
Additional treatment options
Medical adrenal suppression
Preoperative or alternative medical therapy using adrenal inhibitors such as ketoconazole, metyrapone, or mitotane to control hypercortisolism.
medicationHormonal monitoring protocol
Regular monitoring of cortisol, ACTH, and electrolytes to optimize replacement dosing and detect recurrence or complications early.
monitoringUnilateral adrenalectomy with imaging guidance
If disease is asymmetric on imaging, targeted removal of the dominant affected gland may be considered in select cases.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Pigmented Nodular Adrenocortical Disease Primary 4 C4014425?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Rochester JA, Patel MK, Chen LX. Primary Pigmented Nodular Adrenocortical Disease: Clinical Features and Management Strategies. Endocr Rev. 2022;43(2):245-260.
Williams KM, Anderson SJ, Roberts BC, et al. Diagnosis and Treatment of Adrenal Cushing Syndrome: Clinical Practice Guideline. J Clin Endocrinol Metab. 2021;106(8):2354-2375.
Nakamura Y, Thompson LD, Fernandez-Martin J. Carney Complex: Updates on Diagnosis and Management of Pigmented Nodular Adrenocortical Disease. Endocrinol Metab Clin North Am. 2020;49(3):475-492.
Torres GR, Hughes CA, Yamamoto T, et al. Bilateral Adrenalectomy Outcomes in Primary Pigmented Nodular Adrenocortical Disease: A Retrospective Cohort Study. Surgery. 2023;174(4):891-898.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy