Primary pigmented adrenocortical dysplasia (PPNAD) in the context of the Carney complex causing Cushing syndrome

Background: Primary pigmented adrenocortical dysplasia (PPNAD) represents a rare disorder of the adrenal glands and frequently occurs in patients with the so-called Carney complex. Carney complex is an autosomal dominant neop lasia syndrome including skin and mucosal lentigines, myxomas, and PPNAD. Case Report: A 37-year-old woman suffered from several episodes of weight g ain/depression and weight loss/mania indicating cyclic hypercortisolism. Fi nally, she developed a full-blown Cushing's syndrome (CS) treated by bilate ral adrenalectomy. She had PPNAD in the setting of Carney complex. Conclusion: PPNAD may lead to different clinical manifestations: 1. sub-cli nical hypercortisolism, 2. intermittent hypercortisolemia, and 3. full-blow n CS. It can be diagnosed with the 6-day Liddle test that typically shows a paradoxical stimulation of cortisol secretion after dexamethasone administ ration. The treatment of choice for PPNAD is bilateral adrenalectomy in ord er to prevent devastating long-term effects of hypercortisolism.