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Pseudoxanthoma Elasticum:Report of a Rare Granulomatous Variant
Jennifer C. Lee , Chih-Hsun Yang , Su-Chin Shen , Hong-Shang Hong
Dermatol Sinica 22 : 138-141, 2004

Pseudoxanthoma elasticum(PXE) is an inherited disorder of the connective tissue characterized by degeneration and calcification of elastic fibers in the skin and arterial walls of ocular, cardiovascular, and gastrointestinal systems. This granulomatous variant has seldom been investigated. This article describes a middle-aged male exhibiting typical PXE cobblestone-like changes on his neck skin. A skin biopsy specimen revealed, in addition to the pathognomonic finding of basophilic clumped elastic fibers in the reticular dermis, aggregation of epitheloid cells forming well-demarcated granulomas and multinucleated giant cells engulfing the calcified, degenerative elastic fibers.

   
   
 
 
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