An autopsy case of pseudoxanthoma elasticum: histochemical characteristics View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2007-09-18

AUTHORS

Katsuaki Miki, Takashi Yuri, Nobuhiko Takeda, Kazuya Takehana, Toshiji Iwasaka, Airo Tsubura

ABSTRACT

An autopsy case of pseudoxanthoma elasticum is reported. A Japanese female patient complained of yellow papules on the neck, precordium, and axilla, beginning at 54 years of age. When the patient was 58 years old, in response to her visual disturbance a funduscopic examination was performed, revealing angioid streaks, and skin biopsy identified a characteristic pseudoxanthoma elasticum (PXE) lesion. The patient developed congestive heart failure, and following mitral valve prolapse and regurgitation flow into the left atrium, mitral valve replacement with a prosthetic valve was performed when the patient was 65 years old. Soon afterward, the patient complained of gait disturbance, and she died of congestive heart failure at 68 years of age. Autopsy specimen revealed fragmented, granular, and calcified elastic fibers in the middle to deep dermis and in the thickened subendocardium, and small to medium-sized muscular arteries revealed fragmented, laminated, and calcified elastic lamina; vascular changes were seen in the heart, lung, kidney, gastrointestinal tract, and iliac artery. Disrupted elastic fibers were visualized using the Weigert resorcin fuchsin method and were stained positive by antielastin and antifibronectin antibodies. Calcification was confirmed by von Kossa staining. Affected areas were PAS-positive after diastase digestion, indicating the presence of glycoprotein. Affected areas were colloidal iron-positive, indicating the presence of proteoglycan matrix. More... »

PAGES

172-177

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00795-007-0368-5

DOI

http://dx.doi.org/10.1007/s00795-007-0368-5

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1041423185

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/17874051


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