Pseudoexfoliative fibrillopathy in the conjunctiva. A relation to elastic fibers and elastosis.
Pseudoexfoliative (PSX) material in the conjunctiva was found to have a close association with elements of the elastic system in 13 eyes of patients with PSX disease studied ultrastructurally. Pseudoexfoliation aggregates were rare within vascular walls but frequent in proximity to the plasmalemma of stromal fibroblasts. No amyloid was found. A striking feature was the association of PSX fibers with the elastosis seen in many of the specimens. Typical PSX fibers were present in clumps of oxytalan and around small elastic fibers. In five cases with more advanced elastosis, the aggregates of PSX fibers were larger and their usual loose fibrogranular matrix was extensively replaced by a dense elastotic one. Morphology of PSX here varied from typical to thicker, fragmented, and sometimes nondiagnostic fibers. This intermingling of PSX and elastotic materials suggested that PSX fibrillopathy is itself a type of elastosis, possibly resulting from abnormal aggregation of components related to elastic microfibrils.
Streeten
B W
BW
Department of Ophthalmology, State University of New York Health Science Center, Syracuse 13210.
Bookman
L
L
Ritch
R
R
Prince
A M
AM
Dark
A J
AJ
D008841:Actin Cytoskeleton / Q000648:ultrastructure; D000686:Amyloidosis / Q000473:pathology; D001706:Biopsy; D003228:Conjunctiva / Q000473:pathology; D003229:Conjunctival Diseases / Q000473:pathology*; D003285:Contractile Proteins / Q000378:metabolism; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D004547:Elastic Tissue / Q000473:pathology*; D016326:Extracellular Matrix Proteins; D006801:Humans; D008854:Microscopy, Electron; D000072260:RNA Splicing Factors
D003285:Contractile Proteins; D016326:Extracellular Matrix Proteins; D000072260:RNA Splicing Factors; C016598:microfibrillar protein