Pseudoexfoliative fibrillopathy in visceral organs of a patient with pseudoexfoliation syndrome
B. W. Streeten, Z. Y. Li, R. N. Wallace, R. C. Eagle Jr and A. A. Keshgegian
Department of Ophthalmology, State University of New York Health Science Center, Syracuse 13210.
Evidence is increasing that pseudoexfoliative material develops in
widespread areas of skin and parabulbar tissues as well as intraocularly.
To determine whether this process is even more diffusely distributed,
ultrastructural examination was performed on visceral and ocular tissues of
a patient with long-standing glaucoma found to have bilateral ocular
pseudoexfoliation at autopsy. Aggregates consistent with pseudoexfoliative
material were present in the lung, heart, liver, and gallbladder, in
addition to the classic intraocular sites. The aggregates were in the
fibrovascular septa and stroma of these organs, most frequently adjacent to
elastic and oxytalan fibers. They stained positively for elastin and human
amyloid P protein, like the ocular sites, in preliminary immunologic
testing. Rare atypical aggregates were seen in one of the four control
patients. These findings suggest that pseudoexfoliation is a systemic
process involving abnormal matrix synthesis, particularly as related to
elastic tissue components.
