Histopathology of Pseudoxanthoma Elasticum and Related Disorders : Histological Hallmarks and Diagnostic Clues
Joint Authors
Hosen, Mohammad J.
Vanakker, Olivier M.
De Paepe, Anne
Lamoen, Anouck
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-15, 15 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-08-30
Country of Publication
Egypt
No. of Pages
15
Main Subjects
Natural & Life Sciences (Multidisciplinary)
Diseases
Abstract EN
Among ectopic mineralization disorders, pseudoxanthoma elasticum (PXE)—a rare genodermatosis associated with ocular and cardiovascular manifestations—is considered a paradigm disease.
The symptoms of PXE are the result of mineralization and fragmentation of elastic fibers, the exact pathophysiology of which is incompletely understood.
Though molecular analysis of the causal gene, ABCC6, has a high mutation uptake, a skin biopsy has until now been considered the golden standard to confirm the clinical diagnosis.
Although the histological hallmarks of PXE are rather specific, several other diseases—particularly those affecting the skin—can present with clinical and/or histological characteristics identical to or highly resemblant of PXE.
In this paper, we will summarize the histopathological features of PXE together with those of disorders that are most frequently considered in the differential diagnosis of PXE.
American Psychological Association (APA)
Hosen, Mohammad J.& Lamoen, Anouck& De Paepe, Anne& Vanakker, Olivier M.. 2012. Histopathology of Pseudoxanthoma Elasticum and Related Disorders : Histological Hallmarks and Diagnostic Clues. Scientifica،Vol. 2012, no. 2012, pp.1-15.
https://search.emarefa.net/detail/BIM-483991
Modern Language Association (MLA)
Hosen, Mohammad J.…[et al.]. Histopathology of Pseudoxanthoma Elasticum and Related Disorders : Histological Hallmarks and Diagnostic Clues. Scientifica No. 2012 (2012), pp.1-15.
https://search.emarefa.net/detail/BIM-483991
American Medical Association (AMA)
Hosen, Mohammad J.& Lamoen, Anouck& De Paepe, Anne& Vanakker, Olivier M.. Histopathology of Pseudoxanthoma Elasticum and Related Disorders : Histological Hallmarks and Diagnostic Clues. Scientifica. 2012. Vol. 2012, no. 2012, pp.1-15.
https://search.emarefa.net/detail/BIM-483991
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-483991