Amyloidosis of the cornea.

The observed incidence of ocular amyloid is to some extent a function of the diligence with which it is sought, recent reports having shown that it is by no means so rare as was once believed. In the last decade there have been several reports of conjunctival amyloidosis, associated in most instances with trachomatous pannus formation (Mathur and Mathur, 1959; Madangopal, I962; Richlin and Kuwabara, I962; Norn, I964; Halasa, I965; Stansbury, I965; and Smith and Zimmerman, I966). Vitreous opacities, perivascular deposition, and ocular nerve involvement are prominent in one category of systemic familial amyloidosis (Falls, Jackson, Carey, Rukavina, and Block, 1955; Kaufman, 1958; Kaufman and Thomas, 1959; and Paton and Duke, I966). Involvement of the limbus in predominantly conjunctival lesions has been reported by Coats (I915) and Renard, Dhermy, and Nguyen Van Ba (I965), but descriptions of amyloidosis in the cornea proper have until recently been exceptionally rare. The first case appears to have been that of Lewkojewa (I930) in which amyloid was deposited immediately beneath the epithelium, in the absence of any obvious predisposing cause, in an 8-year-old boy. No further cases were reported until Stafford and Fine ( I 966) described an I I-year-old girl with similar corneal deposits in an eye which showed several long-term complications of retrolental fibroplasia. Shortly afterwards McPherson, Kiffney, and Freed (i 966), as a result of a retrospective study of 200 eyes, reported six more cases, all of which occurred in conjunction with chronic ocular disease of various types, and in one of which there was evidence of stromal deposition. More recently Collyer (I968) has described an example of corneal amyloidosis in an eye which had sustained a penetrating limbal wound some 4I years earlier. Another example of stromal amyloidosis, linked in this instance with hypergammaglobulinaemia, was presented by Konig and Pur (i 966). In addition it has been shown by Seitelberger and Nemetz (I 96 I) and Klintworth (I967) that the genetically determined lattice dystrophy of the corneal stroma is almost certainly yet a further type of familial amyloidosis. Two instances of corneal disease due to subepithelial amyloid deposition have recently been seen at the Institute of Ophthalmology and a retrospective study of eleven cases originally diagnosed as nodular corneal dystrophy (including Salzmann's dystrophy) has revealed a further three. An analysis of these five cases is the purpose of this report.