Angioid streaks and elastorrhexis.

ANGIOID streaks are thought to be due to breaks in the elastic lamina of Bruch's membrane. Ophthalmoscopically they present a jagged peripapillary ring which may be incomplete and from which radiate irregular spokes of width similar to retinal vessels. With time, they may progress, or become less distinct. Their colour varies from reddish-brown to dark brown according to the density of the overlying retinal pigment. But they may be pale grey or even in parts white, owing to proliferation of scar tissue. Elastorrhexis is a systemic disease which commonly presents with a skin disorder known as pseudoxanthoma elasticum (PXE). The sites of predilection for PXE lesions are the neck, axillae, perineum, antecubital and popliteal fossae, abdomen, and oral mucosa. The skin is easily stretched and may hang in folds. Yellowish waxy papules varying in size from a pinpoint to a pea may give rise to a "plucked chicken" appearance. PXE and angioid streaks have been reported in all races and in all decades of life although the peak age lies between 25 and 50. They are frequently associated as the GronbladStrandberg syndrome. This is often complicated by systemic vascular disease and as a systemic condition it is termed elastorrhexis. Other ocular manifestations include a mottled or peau d'orange appearance at the posterior pole of the fundus, drusen, and a disciform maculopathy. Choroidal and retinal haemorrhages often precede the maculopathy, and together with it are the most important cause of ocular symptoms. Solar (or actinic) elastosis (SE) is an elastic disorder of skin not unlike PXE, but as it may occur only in areas of skin exposed to the sun any relationship with other diseases has generally been regarded as purely incidental. However, it is stated that senile elastosis (a synonym of solar elastosis) may be associated with angioid streaks (Duke-Elder, 1966). It is the object of this paper to provide a short review of elastorrhexis, to present several cases of both SE and elastorrhexis manifesting in the same family, to assess the relevance of SE, and to attempt a classification of angioid streaks.