Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome).

نویسنده

  • D Viljoen
چکیده

Pseudoxanthoma elasticum (PXE) is a rare disorder of elastin which primarily involves the skin, cardiovascular system, and eyes, but also manifests in the central nervous system, skeleton, and gastrointestinal tract. Rigall first described the cutaneous lesions of PXE in 1881 and thought the disorder was akin to the xanthomatoses. In 1896, Darier2 showed the typical histological changes in elastin fibres, thus proving that PXE was a separate condition. Gronblad and Strandberg subsequently recognised the association between ophthalmological and dermatological features in PXE and their names are sometimes eponymously applied to the disorder.3

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عنوان ژورنال:
  • Journal of medical genetics

دوره 25 7  شماره 

صفحات  -

تاریخ انتشار 1988