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Osler-Weber-Rendu Syndrome in association with port wine spot

Abdul Khalid Awan, Mohammad Mohammad Tariq Baqai, Murtaza Bukhari

Department of Medicine Azad Jammu & Kashmir Medical College Muzaffarabad AJK



Osler-Rendu-Weber syndrome also known as hereditary hemorrhagic telangectasiea (HHT) is an

autosomal dominant vascular disorder. The pathogenesis of this disorder involves dilated postcapillary

venules in the mucus membranes of different organs and large arteriovenous malformations.

Gastrointestinal hemorrhages contribute to iron deficiency anemia in one third of these patients. Port

wine stains belong to the family of arteriovenous disorders. These are due to abnormally dilated

capillaries and produce reddish or pink discoloration of the skin. These are present at birth and

increase in size during the growth period. No association has so far been reported between port wine

stains and hereditary hemorrhagic telangectasiea. We report a case of 41 years old lady presenting with

co- existence of these two lesions.

Key words: Osler-Rendu-Weber syndrome, Port wine stain, GI endoscopy



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