Biomaterial Database

Reticular-like pigmentary patterns in pseudoxanthoma elasticum. 1988

H R McDonald, and H Schatz, and T M Aaberg

Department of Ophthalmology, University of California, San Francisco.

Of 14 consecutive patients with pseudoxanthoma elasticum (PXE), 9 (64%) had a spectrum of peculiar, sometimes reticular, pigmentary changes in the retina. Ten of 18 (56%) eyes had a random scattering of small, round pigment dots throughout the macula and around the optic nerve, sometimes extending to the equator. In four of 18 (22%) eyes, the pigment clumps appeared in lines, resembling a string of pearls. These strings occasionally radiated from the macula in a spoke-like fashion. In 4 of the 18 (22%) eyes, the pigment clumping occurred in a fishnet, or truly reticular, pattern. Of the 18 eyes in these 9 patients, 12 (67%) had subretinal neovascularization. Of the ten eyes in the five patients who did not show this pigmentary change, two (20%) had subretinal neovascularization.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010857	Pigment Epithelium of Eye	The layer of pigment-containing epithelial cells in the RETINA; the CILIARY BODY; and the IRIS in the eye.	Eye Pigment Epithelium
D011561	Pseudoxanthoma Elasticum	An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE.	Gronblad-Strandberg Syndrome,Groenblad-Strandberg Syndrome,Pseudoxanthoma Elasticum, Autosomal Dominant,Pseudoxanthoma Elasticum, Forme Fruste,Pseudoxanthoma Elasticum, Incomplete,Elasticum, Incomplete Pseudoxanthoma,Elasticums, Incomplete Pseudoxanthoma,Gronblad Strandberg Syndrome,Incomplete Pseudoxanthoma Elasticum,Incomplete Pseudoxanthoma Elasticums,Pseudoxanthoma Elasticums, Incomplete,Syndrome, Gronblad-Strandberg
D012164	Retinal Diseases	Diseases involving the RETINA.	Disease, Retinal,Diseases, Retinal,Retinal Disease
D005260	Female		Females
D005654	Fundus Oculi	The concave interior of the eye, consisting of the retina, the choroid, the sclera, the optic disk, and blood vessels, seen by means of the ophthalmoscope. (Cline et al., Dictionary of Visual Science, 4th ed)	Fundus of the Eye,Ocular Fundus,Fundus, Ocular
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly