Roentgen Findings in Pseudoxanthoma Elasticum (Pxe)

Ae, James; Sb, Eaton; Jv, Blazek; Mw, Donner; Rj, Reeves

doi:10.2214/ajr.106.3.642

Cited by 21 publications

(4 citation statements)

References 4 publications

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“…Peripheral vascular disease is well described in PXE, which can be premature in onset, compared with the general population and occur in the absence of other significant risk factors . The hallmark of involvement is atherosclerotic arterial calcifications, possibly secondary to elastic tissue degeneration in the arterial wall.…”

Section: Peripheral Vascular Systemmentioning

confidence: 99%

“…The hallmark of involvement is atherosclerotic arterial calcifications, possibly secondary to elastic tissue degeneration in the arterial wall. The media as well as the intima can be involved; the former resulting in uniform circumferential smooth arterial calcification where as the latter results in irregular asymmetrical calcification and narrowing …”

Section: Peripheral Vascular Systemmentioning

confidence: 99%

“…Dystrophic calcification of the soft tissues is a common association with a variety of connective tissue disorders including, but not limited to PXE . One of the common manifestations in PXE is dermal calcification that can be detected on radiographs or rarely on mammograms where it can mimic micro calcifications . Calcifications also involve other soft tissue structures such as blood vessels, heart valves and visceral organs (Fig.…”

Section: Soft Tissue Calcificationmentioning

confidence: 99%

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Imaging manifestations of acquired elastopathy resembling pseudoxanthoma elasticum in patients with beta thalassaemia major and sickle cell disease

et al. 2015

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Development of an acquired systemic elastopathy resembling pseudoxanthoma elasticum in patients with chronic haemoglobinopathies such as beta thalassaemia major and sickle cell disease is well documented. There is paucity of any comprehensive literature on the radiological manifestations of this entity. This pictorial review aims to describe and illustrate the multi system and multi modality imaging findings of this condition.

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Section: Peripheral Vascular Systemmentioning

confidence: 99%

Section: Peripheral Vascular Systemmentioning

confidence: 99%

Section: Soft Tissue Calcificationmentioning

confidence: 99%

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Imaging manifestations of acquired elastopathy resembling pseudoxanthoma elasticum in patients with beta thalassaemia major and sickle cell disease

et al. 2015

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“…Furthermore, histological alterations have been described in the eyes and brain [ 9 – 11 ]. Using imaging techniques, calcifications have been described juxta-articular, in the soft tissues of the extremities and in the breast [ 6 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

The amount of calcifications in pseudoxanthoma elasticum patients is underestimated in computed tomographic imaging; a post-mortem correlation of histological and computed tomographic findings in two cases

et al. 2018

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ObjectivesPseudoxanthoma elasticum (PXE) is a rare genetic disorder, characterised by elastic fibre degeneration and calcifications in multiple organ systems. Computed tomography (CT) imaging is a potential method to monitor disease progression in PXE patients; however, this method has not been validated. The aim of this study was to correlate histological and computed tomographic findings in PXE patients to investigate the ability of CT scanning to detect these alterations.MethodsPost mortem total body CT scans were obtained from two PXE patients (a 69-year-old male and 77-year-old female). Autopsy was performed, and 38 tissue samples of the first and 45 tissue samples of the second patient were extensively investigated histologically. The findings were compared with the CT scans.ResultsDegenerated and calcified elastic fibres and calcifications were histologically found in the skin, subcutaneous fat, heart, arteries and pleura and around the oesophagus. On CT imaging only the intradermal alterations of the skin and the larger vascular calcifications were detected. The smaller PXE-related abnormalities were not visible on CT.ConclusionsWith CT imaging vascular calcifications and skin alterations can be monitored in PXE patients. However, many of the subtle PXE-related abnormalities found in other organ systems during the autopsy were not visualised by CT scans. Furthermore, we extended the current knowledge on the disease location of PXE with subcutaneous, oesophageal and pleural lesions.Teaching Points • CT can be used to monitor gross vascular calcifications in PXE patients. • Many subtle PXE-related abnormalities are not visualised by CT scans. • PXE-related alterations can also be found in oesophagus, pleura and subcutaneous fat. Electronic supplementary materialThe online version of this article (10.1007/s13244-018-0621-6) contains supplementary material, which is available to authorized users.

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Pseudoxanthoma Elasticum

Rios-Montenegro¹,

Behrens²,

Hoyt³

1972

Arch Neurol

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Pseudoxanthoma elasticum in a 20-year-old man was complicated by right-sided proptosis, conjunctival vascular dilation, and subretinal bleeding from preexisting angioid streaks. Angiography showed multiple anomalies of major systemic arteries, including bilateral carotid rete mirabile and right carotid-cavernous sinus fistula. Electrothrombosis of the right cavernous sinus obliterated the fistula, restored cerebral perfusion by the right carotid system, and promoted prompt resolution of the presenting signs. (26:151-155, 1972)

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Roentgen Findings in Pseudoxanthoma Elasticum (Pxe)

Cited by 21 publications

References 4 publications

Imaging manifestations of acquired elastopathy resembling pseudoxanthoma elasticum in patients with beta thalassaemia major and sickle cell disease

Imaging manifestations of acquired elastopathy resembling pseudoxanthoma elasticum in patients with beta thalassaemia major and sickle cell disease

The amount of calcifications in pseudoxanthoma elasticum patients is underestimated in computed tomographic imaging; a post-mortem correlation of histological and computed tomographic findings in two cases

Pseudoxanthoma Elasticum

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