Primary pigmented nodular adrenal disease presenting as hypertensive crisis

We present a case of a young girl who presented with hypertensive crisis and recent onset weight gain with hirsutism. On evaluation for Cushing syndrome (CS), her cortisol concentration was high, showed a paradoxical cortisol rise on dexamethasone suppression and the adrenocorticotropic hormone (ACTH) was low. Adrenal imaging showed normal adrenal morphology. Genetic diagnosis of primary pigmented nodular adrenal disease (PPNAD) was made. She was operated for bilateral adrenalectomy and histopathology confirmed the diagnosis of PPNAD. Our case highlights the rare aetiology of PPNAD as a cause of CS resulting in a hypertensive crisis. To the best of our knowledge, this is the youngest case of ACTH independent CS presenting as hypertensive encephalopathy.