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Case Report

IMJ. 2016; 8(3): 187-189


PSEUDOXANTHOMA ELASTICUM, GRONBLAD-STRANDBERG SYNDROME REVIEW OF LITERATURE AND CASE REPORT

MARYAM RASHID, AYSHA BABAR, SYED SAIF UR REHAMAN, KAMIL SHUJAAT, SHUJAAT HUSSAIN.

Abstract
Pseudoxanthoma Elasticum is a rare autosomal recessive systemic disease that causes degeneration and fragmentation of elastic fibres with calcification. With the skin manifestations and the simultaneous occurrence of angioid streaks. Pseudoxanthoma Elasticum is also known as Gronblad-strandberg syndrome. It is a multisystem disorder and typically involves skin, eyes and cardiovascular system. Recognition of disease early helps to prevent complications and improves quality of life. We report a case of Pseudoxanthoma Elasticum,to obtain a better understanding of clinical features and early diagnosis of the disease. The case is interesting as patient primarily sought medical consultation due to ocular manifestations of disease.

Key words: Pseudoxanthoma Elasticum, Elastic Fibres, Angiod Streaks.


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