증례 : 가족적으로 발생한 쿠싱증후군 - 색소침착성 다결절성 부신피질 이형성증 3 예 -

Pigmented multinodular adrenocortical dysplasia is a rare cause of Cushing's syndrome in infants, children and young adults. The disease is characterized by an ACTH independent adrenocortical hyperfunction without autonomously functioning adrenocortical neoplasm. Clinical symptoms are features of Cushing's syndrome and spotty facial pigmentation (lentigines and blue nevi). The bio- chemical findings include: 1) no suppression of urinary corticosteroid excretion after administration of dexamethasone. 2) no response to metyrapone or ACTH administraion; and 3) undetectable, low, or normal plasma levels of ACTH. Adrenal glands are normal or slightly enlarged and may or may not have discernible nodules on computed tomography scans. Bilateral total adrenalectomy cures the Cushing's syndrome. The familial occurrence, especially when to be found in combination with extra adrenal disorders, was considered indicative of 'Carney's complex'. Recently we experienced 3 cases of Cushing's syndrome due to pigmented multinodular adrenocortical dysplasia in one family, so we report the cases with a review of literatures.