Overview of Vascular Bleeding Disorders

Bleeding may result from abnormalities in

Vascular bleeding disorders result from defects in blood vessels, typically causing cutaneous or mucosal lesions called petechiae, purpura, and ecchymoses depending on their size but, except for hereditary hemorrhagic telangiectasia Hereditary Hemorrhagic Telangiectasia Hereditary hemorrhagic telangiectasia is a hereditary disorder of vascular malformation transmitted as an autosomal dominant trait affecting men and women. (See also Overview of Vascular Bleeding... read more , seldom leading to serious blood loss. Small lesions (< 2 mm) are termed petechiae, and larger lesions are termed purpura if they are > 2 mm and <10 mm or ecchymoses if > 10 mm.

Bleeding may result from deficiencies of vascular and perivascular collagen in Ehlers-Danlos syndrome Ehlers-Danlos Syndromes Ehlers-Danlos syndromes are hereditary collagen disorders characterized by joint hypermobility, dermal hyperelasticity, and widespread tissue fragility. Diagnosis is clinical. Treatment is supportive... read more and in other rare hereditary connective tissue disorders (eg, pseudoxanthoma elasticum Pseudoxanthoma Elasticum Pseudoxanthoma elasticum is a rare genetic disorder characterized by calcification of the elastic fibers of the skin, retina, and cardiovascular system. Diagnosis is clinical. There is no specific... read more , osteogenesis imperfecta Osteogenesis Imperfecta Osteogenesis imperfecta is a hereditary collagen disorder causing diffuse abnormal fragility of bone and is sometimes accompanied by sensorineural hearing loss, blue sclerae, dentinogenesis... read more , Marfan syndrome Marfan Syndrome Marfan syndrome consists of connective tissue anomalies resulting in ocular, skeletal, and cardiovascular abnormalities (eg, dilation of ascending aorta, which can lead to aortic dissection)... read more ).

Hemorrhage may be a prominent feature of scurvy Vitamin C Deficiency In countries with low rates of food insecurity, vitamin C deficiency can occur as part of general undernutrition, but severe deficiency (causing scurvy) is uncommon. Symptoms include fatigue... read more or of immunoglobulin A–associated vasculitis Immunoglobulin A–Associated Vasculitis (IgAV) Immunoglobulin A–associated vasculitis (formerly called Henoch-Schönlein purpura) is vasculitis that affects primarily small vessels. It occurs most often in children. Common manifestations... read more , a hypersensitivity vasculitis common during childhood.

In vascular bleeding disorders, results of tests of hemostasis Laboratory Tests of Hemostasis by Phase are usually normal. For most disorders, diagnosis is clinical; specific tests are available for some (eg, immunoglobulins in specific dysproteinemias).

Treatment focuses on controlling the bleeding when possible and providing supportive care. Some patients require blood transfusions. Many patients require iron therapy to replace iron lost due to repeated mucosal bleeding (see treatment of Iron Deficiency Anemia Treatment Iron deficiency is the most common cause of anemia and usually results from blood loss; malabsorption, such as with celiac disease, is a much less common cause. Symptoms are usually nonspecific... read more ).