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

Abstract

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 . . .