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Occasionally, abnormalities of the vasculature and integument may lead to bleeding despite normal hemostasis; congenital or acquired disorders may be causative. Congenital abnormalities include Ehlers-Danlos syndrome, osteogenesis imperfecta, hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) (see Chapter 40), and Marfan syndrome. Acquired disorders include integument thinning due to prolonged corticosteroid administration or normal aging, amyloidosis, vasculitis, and scurvy (acquired defects). The bleeding time often is prolonged. The bleeding time reflects the integrity of the vasculature (which is abnormal in collagen synthesis disorders) in addition to activity of platelets and coagulation factors. If possible, treatment of the underlying condition should be pursued, but if this is not possible or feasible (ie, congenital syndromes), globally hemostatic agents such as antifibrinolytic agents or DDAVP can be considered for treatment of bleeding.

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