Objective: To study the prevalence, type, and features of pattern dystrophy in patients with pseudoxanthoma elasticum (PXE). Methods: Asearch of the photograp hic records at theVanderbilt Eye Institute using the “angioid streaks and pseudoxanthoma elasticum”yielded 28 names. Of the 23 subjects meeting the p atient selection criteria, 22 were confirmed to have a positive diagnosis for PX E after reviewing themedical history information. The diagnosis was confirmed by the constellation of fundus findings in all 22 subjects, by a clinical examinat ion of the skin in 9, and by a skin biopsy specimen in 1. Results: Pattern dystr ophy was present in 16 patients (27 eyes) of those with PXE. Fourteen patients ( 23 eyes) had fundus pulverulentus, 3 patients (5 eyes) had butterfly-shaped dys trophy,and 1 patient (2 eyes) each had fundus flavimaculatus and reticular dystr ophy. One eye of one patient developed solitary vitelliform pattern dystrophy du ring follow-up. Two patients showed progression from one pattern into another d uring follow-up. Another patient, who at first showed no evidence of pattern dy strophy in either eye, developed fundus pulverulentus in both eyes 5 years later . One patient had simultaneous evidence of 2 types: butterfly and fundus flavima culatus pattern in each eye. Angioid streaks were seen in each eye of all patien ts. Peau d’orange was noted in 18 patients, optic nerve drusen in 5, and retina l crystalline bodies in 9. Choroidal neovascular membrane was present in 15 pati ents. Conclusions: All 5 varieties of pattern dystrophy, 2 ofwhichwere not previ ously associatedwith PXE, were seen in patients with PXE. Fluorescein angiogram was useful in delineating the type and extent of pattern dystrophy. Objective: To study the prevalence, type, and features of pattern dystrophy in patients with pseudoxanthoma elasticum (PXE). Methods: Asearch of the photograp hic records at the Vanderbilt Eye Institute using the “angioid streaks and pseudoxanthoma elasticum” yielded 28 names. Of the 23 subjects meeting the pientient selection criteria, 22 were confirmed to have a positive diagnosis for PX E after reviewing the medical history information. The diagnosis was confirmed by the constellation of fundus findings in all 22 subjects, by a clinical examinat ion of the skin in 9, and by a skin biopsy specimen in 1. Fourteen patients (23 eyes) had fundus pulverulentus, 3 patients (5 eyes) had butterfly-shaped dys trophy, and 1 patient (2 eyes) each had fundus flavimaculatus and reticular dystr ophy. One eye of one patient developed solitary vitelliform pattern dystrophy du ring follow-up. Two patients showed progr e patient from one pattern into another d uring follow-up. Another patient, who at first showed no evidence of pattern dy strophy in either eye, developed fundus pulverulentus in both eyes 5 years later. One patient had simultaneous evidence of 2 types: butterfly and The fundus flavima culatus pattern in each eye. Angioid streaks were seen in each eye of all patien ts. Peau d’orange was noted in 18 patients, optic nerve drusen in 5, and retina l crystalline bodies in 9. Choroidal neovascular membrane was present in 15 pati ents. Conclusions: All 5 varieties of pattern dystrophy, 2 of whwewere not previ ously associated with PXE, were seen in patients with PXE. Fluorescein angiogram was useful in delineating the type and extent of pattern dystrophy.