Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia).
Cushing's syndrome was found in 2 of 4 siblings all of which also had other malformations. After bilateral adrenalectomy no hyperpigmentation was noted. Morphologically the adrenals of both siblings with Cushing's syndrome showed the typical lesions of the so-called microadenomatosis or primary adrenocortical nodular dysplasia with foci of eosinophilic giant cells. This is a new form of Cushing's syndrome due to an inborn error of the adrenals and no hypothalamic-pituitary dysfunction.
D015065:17-Hydroxycorticosteroids / Q000652:urine; D015068:17-Ketosteroids / Q000652:urine; D000273:Adipose Tissue / Q000473:pathology; D000302:Adrenal Cortex / Q000473:pathology*; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D003480:Cushing Syndrome / Q000235:genetics / Q000473:pathology*; D004804:Eosinophils / Q000473:pathology; D006801:Humans; D006965:Hyperplasia; D008297:Male
D015065:17-Hydroxycorticosteroids; D015068:17-Ketosteroids; D000324:Adrenocorticotropic Hormone