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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