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Rev. argent. dermatol ; 87(1): 6-14, ene.-mar. 2006. ilus, tab
Article in Spanish | LILACS | ID: lil-634305

ABSTRACT

Enfermedad hereditaria rara, el pseudoxantoma elástico es un trastorno genético del tejido conectivo, que se caracteriza por fragmentación de las fibras elásticas y posterior calcificación de éstas afectando dermis, vasos sanguíneos y la membrana de Bruch de retina. El patrón de herencia es muy variable, lo que hace posible que esta enfermedad pueda estar subdiagnosticada. La escasa incidencia de esta patología justifica la presentación de dos casos que tuvieron solamente manifestaciones cutáneas.


Hereditary disease does not frequent pseudoxanthoma elastic; is a genetic upheaval of the conective weave, that characterizes by fragmentation of elastic fibers and later calcification of these, affecting dermis, blood vessels and membrane of Bruch of retina. The inheritance pattern is very variable which causes that disease; can be subdiagnosed. The litlle incidence of this pathology, causes that in our professional experience we have been able to diagnose two cases in which the manifestations were cutaneous.


Subject(s)
Humans , Female , Adult , Middle Aged , Pseudoxanthoma Elasticum/diagnosis , Pseudoxanthoma Elasticum/classification , Pseudoxanthoma Elasticum/therapy , Signs and Symptoms
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