Angioid Streaks and Pseudoxanthoma Elasticum

SCHOLZ (1941), reviewing the literature on angioid streaks and pseudoxanthoma elasticum, collected a total of 188 cases, only seventeen of which were reported from Great Britain.A further case is described in this article.Angioid streaks were first described by Doyne (1889) and then by Plange (1891); Knapp (1892) gave them their present name.The fundus appear- ance in this disease was described by Verhoeff (1928) as follows:A more or less broken peripapillary ring of pigment from which pigmented streaks extend out into the fundus.The appearance of the streaks varies from dark red to brown or even black.The edges of the streaks are serrated and along their course there may be reddish dots.They lie beneath the retinal vessels and above the choroidal ones.Usually there is a complete or incomplete greyish zone about the papilla.The entire fundus may have a mottled appearance or the degenerative changes may appear only in the periphery.Both eyes are involved.However, since Verhoeff published this account, two cases of monocular angioid streaks have been described: one by Gronblad (1932) and one by Isayama (1940).The most common presenting symptoms of patients with angioid streaks is diminution of vision.This is due to associated macular abnormalities which were present in 140 out of the 188 cases collected by Scholz (1941).These abnormalities may be haemorrhage, scarring, abnormal pigmentation, hole at the macula, exudate, or a specific type of retinitis such as disciform degeneration or retinitis circinata.Flame-shaped and blot-haemorrhage may occur in any part of the fundus.A peculiar type of haemorrhage caused by trauma described by Marchesani and Wirz (1931) comprises large tear-drop-like haemorrhages in the layer between the retina and choroid, rather similar to pre-retinal haemorrhages but at a deeper level.The diagnosis is revealed by examining the untraumatized eye.These haemor- rhages are absorbed completely, but the authors suggest that they leave behind them white streaks in the retina similar to those which remain after a choroidal detachment.Other abnormalities of the retina may also be seen, such as pigment and hyaline bodies, especially drusen of the optic disc.Pseudoxanthoma elasticum was first described by Balzer (1884), whose