Fluorescein Study of Angioid Streaks with Pseudoxanthoma Elasticum.

Choi, Dong Kwang

Case Report

Journal of the Korean Ophthalmological Society 1979;20(3):433-442.
Published online March 1, 1979.

Fluorescein Study of Angioid Streaks with Pseudoxanthoma Elasticum.

Dong Kwang Choi

Don Wha Moon Kong's Eye Clinic, Seoul, Korea.

Pseudoxanthoma Elasticum 과 동반한 Angioid Streaks 의 형광 안저소견

최동광 ( Dong Kwang Choi )

Abstract

Angioid streaks appear ophthalmoscopically as a network of red or dark brown streaks emanating from the optic disc. Angioid streaks may occur alone or in association with various. systemic diseases including pseudoxanthoma elastic urn CGroenblad-Strandberg syndrome), Senile elastosis of the skin, sickle cell cell anemia, some hypertensive cardiovascular disorders, lead poisoning, thrombocytopenic purpura, and familial hyper phosphatemia. PXE is complicated by the formation of angioid streaks in the fundi of approximately 85% of patients and this form of the disease is known as the Groenblad-Strandberg syndrome. PXE is transmitted as an autosomal dominant or recessive trait, the disease usually appears by age 30, although it may appear in childhood or in old age. Usually the incidence in woman is twice that in man. It is characterized by loss of central vision in 70% of patients and peaud' orange skin lesions. located on the neck, abdomen, axilla, perineum, thighs and groin areas. Author presented a study of two cases of PXE, which fluorescein pattern showed hyperfluorescence of angioid streaks, choroidal neovascularization and hemorrhagic serous detachment of the sensory retina involving the fovea. Recently sealing of the choroidal neovascularization by argon laser may be effective in preventing the visual loss. But unfortunately this case was not performed photocoagulation due to danger of central vision loss.