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Primary pigmented nodular adrenal disease presenting as hypertensive crisis
  1. Chirantap Markand Oza1,
  2. Sajili Mehta2,3,
  3. Vaman Khadilkar1,4 and
  4. Anuradha Khadilkar1,4
  1. 1Growth and Endocrine Unit, Hirabai Cowasji Jehangir Medical Research Institute, Pune, Maharashtra, India
  2. 2Pediatric Endocrinology, Surya Mother and Child Care Super Speciality Hospital, Pune, Maharashtra, India
  3. 3Pediatric Endocrinology, MIMER, Pune, Maharashtra, India
  4. 4Interdisciplinary School of Health Sciences, Savitribai Phule Pune University, Pune, Maharashtra, India
  1. Correspondence to Dr Anuradha Khadilkar; anuradhavkhadilkar{at}


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.

  • Adrenal disorders
  • Hypertension

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  • Contributors CMO, SM, VK and AK played a role in the clinical management, planning, execution, analysis, writing of the manuscript and that they all agree and accept responsibility for the contents of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.