By Pierre Bourdieu
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D. , lacy vacuolation of iris pigment epithelium; see p. 579 in Chap. , in long-standing neural retinal detachment and in proliferating retinal pigment epithelial cells). E. Amyloid may be found in ocular tissues in primary amyloidosis (see p. 227 in Chap. 7 and p. 470 in Chap. 12), such as in primary familial amyloidosis and lattice corneal dystrophy (in which case it is a dystrophic change), or in secondary amyloidosis (see p. 228 in Chap. 7), in which case it is a degenerative change. F. Hyaline degeneration is quite common, consists of acellular, amorphous, eosinophilic material, and may be found in such places as the walls of arteriolosclerotic vessels or in the ciliary processes in elderly people.
3 Neuroﬁbromatosis. A, A plexiform neuroﬁbroma has enlarged the left upper lid; the neuroﬁbroma was removed. B, The gross specimen shows a markedly expanded nerve. A thin slice of the nerve is present at the bottom left. C, In another similar case diffuse proliferation of Schwann cells within the nerve sheath enlarges the nerve (n, thickened abnormal nerves). (A, Courtesy of Dr. ) n n C n n Print Graphic A B Presentation Fig. 4 Neuroﬁbromatosis. A, Iris shows multiple, spiderlike melanocytic nevi, characteristic of neuroﬁbromatosis.
C. Secondary complications such as microcystoid degeneration of the overlying retina (see p. 397 in Chap. 11) and leakage of serous ﬂuid into the subneural retinal space (see p. 402 in Chap. 11) are common. Neuroﬁbromatosis (Figs. 5) I. Neuroﬁbromatosis type 1 (von Recklinghausen’s disease or peripheral neuroﬁbromatosis) A. General information 1. , sphenoid bone dysplasia); a ﬁrst-degree relative who has NF-1. 2. Multiple tumors are found that are derived from Schwann cells of peripheral and cranial nerves and glial cells of the central nervous system.