Pseudoxanthoma elasticum is a rare connective tissue disease which affects the synthesis of the elastic fibres. It is an area of uncertainty for patients affected by this disease requiring surgical revascularization. We present a case report of a 43-year-old gentleman was known to have pseudoxanthoma elasticum syndrome (PXE) and coronary artery disease. He underwent successful off pump LIMA to LAD. Interestingly, this is the first case in UK and second one in the literature managed with an off-pump strategy. The predilection of the histopathological changes in PXE patients, should not prevent the routine use in of the left internal mammary artery as a standard conduit in CABG. Further studies are required to standardize the practice for surgical revascularization in the patients with PXE.
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http://dx.doi.org/10.1177/02676591241288180 | DOI Listing |
J Cosmet Dermatol
December 2024
Department of Biology and Nanomedicine, National and Kapodistrian University of Athens, Athens, Greece.
Background/aim: Pseudoxanthoma elasticum (PXE) is a genetic connective tissue disorder that affects the skin with limited treatment options. A recent technology employing particle-free polycaprolactone (PCL) has shown promising results in treating inner thighs and kness of a 27-year-old female patient. This article provides a case report along with our detailed treatment protocol based on the efficacy of PCL in reversing skin laxity that can be easily incorporated into the therapeutic approaches for patients with PXE.
View Article and Find Full Text PDFDermatol Online J
October 2024
Division of Dermatology, Department of Medicine, School of Medicine, The University of Jordan, Amman, Jordan.
Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare, benign, acquired, gradually-developing chronic elastic tissue disorder that almost exclusively affects post-menopausal women. It is essential to recognize this disease as it mimics the inherited pseudoxanthoma clinically. The pathophysiology behind this disease is multifactorial; it includes intrinsic skin aging, ultraviolet radiation exposure, and genetic components.
View Article and Find Full Text PDFDermatol Online J
August 2024
Department of Dermatology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, New York, New York, USA.
Atherosclerosis
January 2025
Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. Electronic address:
Background And Aims: Pseudoxanthoma elasticum (PXE) patients have more arterial calcification due to lower levels of inorganic pyrophosphate, caused by mutations in the ABCC6 gene, but the relation with vascular complications is poorly understood. Because of the slow progressing nature of arterial disease in PXE patients, there is a need for a valid and reliable intermediate endpoint to be used in future clinical trials. Arterial calcification measured on computed tomography (CT) is a promising candidate, if associated with future cardiovascular events.
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