Familial Cushing's Syndrome Due to Primary Pigmented Nodular Adrenocortical Disease

PRIMARY pigmented nodular adrenocortical disease is a rare adrenal-dependent cause of Cushing's syndrome characterized biochemically by hypercortisolism resistant to dexamethasone suppression, and pathologically by small-to-Normal-Sized adrenal glands, multiple small black cortical nodules containing large cells with eosinophilic cytoplasm and lipofuscin, and internodular cortical atrophy.^{1 2 3 4} The pathogenesis of the disease is unknown; recent data, however, suggest that it may be due to circulating adrenal-stimulating immunoglobulins.^{5 6 7 8 9} Primary pigmented nodular adrenocortical disease occurs sporadically or as part of a familial syndrome called the Carney complex.^{10 11 12 13} The Carney complex is a multisystem tumor syndrome in which the tumors are multicentric in affected organs and…