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Pseudoxanthoma elasticum and nephrocalcinosis: Incidental finding or an infrequent manifestation?

Pseudoxanthoma elasticum (PXE) is an inherited disorder characterized by generalized fragmentation and progressive calcification of elastic tissue. We report two sporadic cases of PXE, both of whom presented with asymptomatic yellowish papules over the flexural sites for cosmetic reasons. Histopatho...

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Detalles Bibliográficos
Autores principales: Gayen, Tirthankar, Das, Anupam, Roy, Sudipta, Biswas, Shamick, Shome, Kaushik, Chowdhury, Satyendra N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4030349/
https://www.ncbi.nlm.nih.gov/pubmed/24860756
http://dx.doi.org/10.4103/2229-5178.131094
Descripción
Sumario:Pseudoxanthoma elasticum (PXE) is an inherited disorder characterized by generalized fragmentation and progressive calcification of elastic tissue. We report two sporadic cases of PXE, both of whom presented with asymptomatic yellowish papules over the flexural sites for cosmetic reasons. Histopathological findings on hematoxylin and eosin and Verhoeff-Van Gieson (VVG) staining were classical of PXE. In addition to this, renal calcification was documented on plain radiography of kidneys, ureters, and bladder (KUB) in both the cases. Paucity of literature describing the association of nephrocalcinosis with PXE prompted the present report.