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Pseudoxanthoma elasticum - a rare case report

Vijayalakshmi S .

Abstract


Pseudoxanthoma elasticum is an autosomally inherited disorder with incidence ranging from 1 in 70,000 to 1 in 100,000 live births. It is characterized by progressive calcification and fragmentation of elastic fibers in the skin, the retina, and the cardiovascular system. The diagnostic criteria are the typical histopathological appearance in the skin biopsy and the presence of angioid streaks in the retina. We report a case of 25 year old female with history of asymptomatic skin lesions over both sides of the neck since 13 years. Punch biopsy of skin sent for histopathological examination and special stain which revealed the diagnosis of pseudoxanthoma elasticum. Fundus examination of eye showed evidence of Angioid streaks.

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References


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