Multiple blue nevi can be seen in the Carney complex, also known as the NAME or LAMB syndrome. This complex of clinical findings includes multiple blue nevi, lentigines, ephelides, myxomas, atrial myxomas, testicular tumors, pituitary tumors, psammomatous melanotic schwannomas, and adrenal tumors. This is a rare syndrome that has been determined to be caused by a genetic defect in the gene PRKAR1A. This is a tumor suppressor gene that encodes a protein kinase A subunit.

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