Purpose: To characterize changes in fundus autofluorescence in patientswith pseudoxanthoma elasticum (PXE). Fundus autofluorescence intrinsicallyderives from lipofuscin, and the degree of autofluorescence is thought to indicatethe degree of retinal pigment epithelium (RPE) metabolic activity. Methods:Twelve eyes of 6 patients (2 men, 4 women) with PXE were studied with aconfocal scanning laser ophthalmoscope. Patient age ranged from 42 to 62years. The autofluorescence of abnormal retinal areas was compared digitallywith that of neighboring, presumed healthy control areas. When the averagegray level of a fundus region was 2 SDs above or below the average graylevel of a control area, autofluorescence of the fundus region was consideredabnormal. Results: In all 12 eyes, some segments of the angioid streaksshowed decreased fundus autofluorescence, and other segments of the streaksshowed normal autofluorescence. Areas of peripapillary chorioretinal atrophyseen in 2 eyes and of disciform scarring seen in 3 eyes showed decreasedautofluorescence. Solitary or multiple drusen-like spots showed increasedautofluorescence in all 12 eyes. Conclusion: Atrophic and degenerativeRPE regions showed decreased fundus autofluorescence in areas of chorioretinalatrophy and in some segments of the angioid streaks. Some drusen-like spotsshowed increased autofluorescence. The characteristic changes in autofluorescencethat we observed in PXE patients suggest that the content of the drusen-like substancediffers from that of senile drusen and that the drusen-like lesions are similar to thesub-RPE deposits seen in macular dystrophy.