Ocular pigmented spots and eyelid myxomas.

Ophthalmic abnormalities are part of a recently recognized syndrome that includes myxomas, spotty pigmentation, and endocrine overactivity. The complex is familial with mendelian dominant mode of inheritance. The 63 patients (27 males and 36 females) we have identified with this complex had the following ophthalmic abnormalities: facial and eyelid lentigines in 44 patients (70%); pigmented lesions on the caruncle or conjunctival semilunar fold in 17 patients (27%); and eyelid myxomas in ten patients (16%). Because these findings usually preceded the most serious component of the syndrome, cardiac myxoma, early recognition of their significance is important.