Ophthalmologic aspects of amyloidosis

Ophthalmologic signs attributable to amyloid infiltration are virtually unknown in secondary systemic amyloidosis. Amyloid papules and purpura of the skin of the eyelids are common findings in primary systemic amyloidosis (including cases associated with myeloma) and are virtually diagnostic of the disease. Amyloid deposits in the vitreous may be seen in familial primary systemic amyloidosis, but this is inconstant, and they have been observed in nonfamilial cases. Microdeposits of secondary localized amyloid have been found in lipoid proteinosis of the eyelids and in corneas affected with a variety of disorders. Primary localized amyloidosis affects the subconjunctival region but not the skin of the eyelids, and it has also been found in the orbit and in the Gasserian ganglion. The mere recognition of amyloid in an ocular structure can no longer be considered a complete clinicopathologic diagnosis. The possibility of amyloid in extraocular sites, underlying or associated ocular disorders, familial aspects, and myelomatosis must be considered.