This is a systemic amyloidosis disorder with significant corneal disease. The corneal stroma contains linear deposits which are more discrete, more peripheral, more delicate, and more radial than those in lattice type I with which it is sometimes confused. There is also less accumulation of amorphous amyloid material than in type I. The onset is often later as well, and rarely seen in childhood. Corneal sensitivity is reduced. Vision is less affected than in type I lattice dystrophy and patients rarely require keratoplasty, and, if so, later in life.
Amyloid deposits are found in the cornea, sclera, choroid, lacrimal gland, ciliary nerves, and adnexal blood vessels. Ptosis and extraocular muscle dysfunction is not significant.