pseudoxanthoma elasticum

pseudoxanthoma elasticum

also called  Grönblad-strandberg Syndrome,  

      inherited disease in which the premature breakdown of exposed skin occurs. It is characterized by eruptions of yellow plaques and thickening and grooving of the skin on the face, neck, and sometimes the armpits, abdomen, and groin. The skin loses its elasticity and hangs loosely from underlying structures. Affected persons show signs of poor circulation in the extremities; leg muscles tire easily, and spontaneous bleeding into the tissues or from any opening is common. The heart muscles have many small calcified deposits. Angioid streaks (streaks resembling blood vessels) of the retina are found in at least 80 percent of the cases. Through the ophthalmoscope the angioid streaks can be seen as brown or brownish black bands that resemble the normal retinal vessels but are generally much wider. These streaks are produced by breaks in the elastin-rich internal membrane of the choroid, which permits the choroidal pigment to be seen. Deterioration of vision may occur because of bleeding or degenerative changes. Pseudoxanthoma elasticum is inherited as an autosomal recessive disorder.

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Universalium. 2010.

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  • PSEUDOXANTHOMA ELASTICUM — (эластическая исевдоксантома), редкое кожное заболевание, описанное подробно Дарье (Darier, 1896). Р. е. может развиться в любом возрасте, одинаково поражает оба пола, иногда имеет семейный характер. Р. е. локализуется на закрытых частях тела, гл …   Большая медицинская энциклопедия

  • Pseudoxanthoma elasticum — (PXE), auch Grönblad Strandberg Syndrom, ist eine autosomal dominat oder rezessiv vererbte Erkrankung[1], bei der die elastischen Fasern des Bindegewebes durch Einlagerung von Mineralsalzen (Calcium) verändert werden. Die Fasern werden brüchig… …   Deutsch Wikipedia

  • Pseudoxanthoma elasticum — Infobox Disease Name = Pseudoxanthoma elasticum Caption = DiseasesDB = 10876 DiseasesDB mult = DiseasesDB2|10881 DiseasesDB2|10885 DiseasesDB2|10894 ICD10 = ICD10|Q|82|8|q|80 (ILDS Q82.81) ICD9 = ICD9|757.39 ICDO = OMIM = 264800 MedlinePlus =… …   Wikipedia

  • Pseudoxanthoma elasticum — (Abbreviated PXE). A genetic disorder of elastic fiber degeneration with tiny areas of calcification in the skin, back of the eyes (retinae), and blood vessels. PXE is inherited from the parents, either as an autosomal recessive or as an… …   Medical dictionary

  • Pseudoxanthoma elasticum — Grönblad Strandberg Syndrom …   Universal-Lexikon

  • Pseudoxanthoma elasticum — Pseu|do|xan|tho̱ma elạsticum [↑pseudo... u. ↑Xanthom; ↑elastisch] s; : Auftreten xanthomartiger Knötchen im elastischen Gewebe, das zuerst verhärtet und dann zerfällt …   Das Wörterbuch medizinischer Fachausdrücke

  • pseudoxanthoma elasticum — a hereditary disease in which elastic fibres (see elastic tissue) become calcified. The skin becomes lax and yellowish nodules develop in affected areas; this is accompanied by degenerative changes in the blood vessels and angioid streaks in the… …   The new mediacal dictionary

  • elasticum — see PSEUDOXANTHOMA ELASTICUM …   Medical dictionary

  • PXE — • pseudoxanthoma elasticum …   Dictionary of medical acronyms & abbreviations

  • псевдоксантома эластическая — (pseudoxanthoma elasticum; псевдо + ксантома; син.: Гренблад Страндберга синдром, Турена системный эласторексис, эластома кожи, эласторексис генерализованный) наследственная болезнь, характеризующаяся нарушением образования эластических волокон с …   Большой медицинский словарь

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