• Vol. 35 No. 12, 892–896
  • 15 December 2006

Unravelling the Mystery in a Case of Persistent ACTH-independent Cushing’s Syndrome

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ABSTRACT

Introduction: We present a rare variety of adrenocorticotrophic hormone (ACTH)-independent Cushing’s syndrome known as primary pigmented nodular adrenocortical disease (PPNAD).

Clinical Picture: The patient initially underwent unilateral adrenalectomy for what was thought to be a left adrenal adenoma.

Outcome: Partial resolution of symptoms and demonstrable persistent hypercortisolism after surgery prompted further evaluation with findings leading to the diagnosis of Carney complex. A review of the adrenal histology was consistent with PPNAD.

Conclusion: This entity of PPNAD, which has rarely been reported in Asians, forms part of the Carney complex. The diagnosis may not be simple and straightforward, as illustrated in this patient.


A Malaysian Chinese male patient, CMO, first presented at the age of 22 years with a 2-week history of severe spontaneous low backache with no prior trauma or back injury. A lumbar spine X-ray showing marked osteopenia and compression fracture of the first lumbar vertebra led to findings of osteoporosis confirmed on the dual energy bone densitometry with T scores of –3.93 and –3.70 in the hip and spine respectively.

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