Pseudoxanthoma elasticum-like (PXL) condition is one of the complications faced by patients with β-thalassemia major (β-TM). Histopathological features include abnormal, mineralized and fragmented elastic fibers in skin, eyes and arterial blood vessels (elastorrhexia). The pathogenesis of PXL lesions in β-TM is not yet completely understood. This study was aimed at analyzing a possible implication of α-Klotho in the clinical manifestation of PXL in patients with β-TM (30 with and 78 without PXL). A significant correlation was observed between Klotho, parathyroid hormone (PTH) and serum calcium (Ca). Our analysis seems to indicate α-Klotho and PTH as factors that can affect the development of PXL.
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http://dx.doi.org/10.1080/03630269.2017.1403927 | DOI Listing |
Dermatol 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 PDFSkinmed
August 2024
Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon;
Biol Pharm Bull
December 2023
Research Laboratories 2, FUJI YAKUHIN Co., Ltd.
Ectopic calcification in the cardiovascular system adversely affects life prognosis. DBA/2 mice experience calcification owing to low expression of Abcc6 as observed in pseudoxanthoma elasticum (PXE) patients; however, little is known about its characteristics as a calcification model. In this study, we explore the suitability of a DBA/2 sub-strain as a PXE-like tissue calcification model, and the effect of a bisphosphonate which prevents calcification of soft tissues in hypercalcemic models was evaluated.
View Article and Find Full Text PDFEur J Dermatol
June 2023
Dermatology, Brest University Hospital, Brest, France.
Actas Dermosifiliogr
October 2023
Servicio de Dermatología del Hospital Universitario de Coimbra, Coimbra, Portugal.
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