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Rendu-Osler-Weber disease

Telengectasie of the nose in a patient with Rendu Osler

The Rendu-Osler-Weber Syndrome is a rare genetic disease with autosomal dominant leading to Dysplasia of blood which makes them more fragile with bleeding tendency.
It is also called hereditary hemorrhagic telengectasia (hereditary hemorrhagic telangiectasia [HHT]).

Manifesting with skin and mucous membranes and telengectasie with venous malformations atero-.
L ’ epistaxis (bleeding from the nose) important is often the presenting symptom of this disease is real persecution for patients who must frequently be transfused to meet the blood loss.
There is currently no causal therapy and treatments are limited to multidisciplinary management of bleeding and MAV.

Telengectasie oral language and pelvis in a patient with Rendu Osler