Angioid streaks and elastorrhexis.

Percival, S.P.B.

doi:10.1136/bjo.52.4.297

Cited by 18 publications

(4 citation statements)

References 57 publications

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“…Although not adequately described in all the records, the appearance of this sign preceded by several years the decrease in visual acuity. As reported also by Gronblad and other authors (7,23,32), patients with marked decrease in vision showed, in addition to angioid streaks, macular and peripapillary atrophy and different degrees of retinal-pigment proliferation, known as late manifestations of the disease.…”

Section: Discussionsupporting

confidence: 62%

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Clinical Studies on a Swedish Material of Pseudoxanthoma Elasticum

Ålinder

Boström

1972

Journal of Internal Medicine

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A study of 27 cases of pseudoxanthoma elasticum (PXE) has been made from the point of view of internal medicine. This disease is hereditary and is characterized by typical manifestations of the eyes, skin, mucous membranes and cardiovascular system. Twelve patients died at the time of the investigation, two of malignant diseases, one of gastrointestinal bleeding, and nine of cardiocerebrovascular diseases. All the 15 living patients had typical skin lesions which caused only cosmetic discomfort. In most cases these skin lesions were the first symptom of the disease. Angioid streaks were found in all the patients, nearly all of whom had impairment of vision which had mostly occurred about 20 years later than the skin lesions. The patients with impaired vision also showed different degrees of macular and peripapillary atrophy and retinal‐pigment proliferation. Manifestations of cardiocerebrovascular disease appeared in many of the patients; these symptoms were recognized somewhat later than the eye lesions, but in some cases they had occurred at an early age. Gastrointestinal bleedings occurred in four patients, in one of whom they proved fatal. No characteristic abnormalities were found in the extensive laboratory survey.

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Section: Discussionsupporting

confidence: 62%

“…In about 85 % of the patients with the characteristic skin lesion, the typical angioid streaks are observed in the eyegrounds (l0, 21,32). Ophthalmoscopy has shown them to be red, white or redbrown, straight, or slightly tortuous, bands radiating from the peripapillar zone :nd situated beneath the retinal vessels (8).…”

mentioning

confidence: 99%

Clinical Studies on a Swedish Material of Pseudoxanthoma Elasticum

Ålinder

Boström

1972

Journal of Internal Medicine

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“…19,[22][23][24] Other recognized systemic associations of angioid streaks include Ehlers-Danlos syndrome, Paget disease, hypercalcinosis, and hemoglobinopathies, such as sickle cell disease, which may all have an impact on the connective tissue within BrM. 25,26 Fortunately, similar to a reported pair of identical twins with angioid streaks, 17 our sibling patients did not have any of these aforementioned systemic conditions. For instance, they had neither peau d'orange nor ABCC6 mutations.…”

Section: Discussionmentioning

confidence: 99%

“…As early as 1968, it was suggested that the breakdown of BrM may be a primary pathology in pseudoxanthoma elasticum, whereas in other conditions associated with angioid streaks, BrM breaks may be the result of secondary degeneration. 26,29 Generalized RPE degeneration, calcified BrM, and subretinal macrophage infiltration have been identified in Best disease and in angioid streaks. [30][31][32][33] Thus, within the RPE/BrM complex, RPE degeneration and BrM remodeling are common pathologic processes in both disorders.…”

Section: Discussionmentioning

confidence: 99%