*Bleeding Disorders Caused by Abnormalities of Vessel Walls

Chapter: Bleeding Disorders Caused by Abnormalities of Vessel Walls
McMaster Section Editor(s): Mark Crowther
Section Editor(s) in Interna Szczeklika: Andrzej Hellmann, Bogdan Ochrem
McMaster Author(s): Mark Crowther
Author(s) in Interna Szczeklika: Krystyna Zawilska
Additional Information

Bleeding disorders caused by abnormalities of the vessel wall develop due to vascular malformation or damage. They are associated with cutaneous or mucosal flat or palpable purpura.

Of note, the commonly used but nonspecific word “purpura” is usually used to describe subcutaneous bleeding, which may be related to blood vessel wall abnormalities (described here) or platelet abnormalities.

1. Inherited disorders of the vessel wall:

1) Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease).

2) Purpura associated with congenital connective tissue diseases: Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta.

2. Acquired bleeding disorders caused by abnormalities of the vessel wall:

1) IgA vasculitis (Henoch-Schönlein purpura) and other forms of vasculitis (eg, serum sickness).

2) Purpura caused by elevated venous pressure: Small, pointlike petechiae on the face and upper trunk, which may be caused by cough, vomiting, or lifting heavy objects, may develop in women after delivery, or may appear on the lower extremities due to venous stasis.

3) Senile purpura.

4) Purpura caused by Cushing syndrome.

5) Ascorbic acid deficiency.

6) Purpura in dysproteinemia (cryoglobulinemia, Waldenström macroglobulinemia) and amyloidosis.

7) So-called simple purpura (mild purpura occurring in young women, which exacerbates during menstruation and is probably related to female sex hormones).

8) Purpura related to trauma or sunburn.

9) Purpura associated with infection: This is usually due to meningococcal, streptococcal, Salmonella spp, or other rare bacterial infections such as Capnocytophaga canimorsus, varicella-zoster virus, influenza virus, measles virus, or malaria. It may also develop in patients with gram-negative sepsis, for instance, Waterhouse-Friderichsen syndrome, pneumococcal sepsis, endocarditis, purpura fulminans (a rapid, severe purpura in the course of infections, which initially manifests as fever, rigors, massive symmetric purpura on the lower extremities and trunk, and progresses to bullous and necrotic lesions that may lead to gangrene or even autoamputation of fingers).

10) Purpura related to thromboembolism: Hemorrhagic and necrotic lesions in patients with disseminated intravascular coagulation, cutaneous necrosis caused by vitamin K antagonists, antiphospholipid syndrome, embolism caused by atherosclerotic plaques.

11) Drug-induced purpura: Purpura located mainly on the skin of the trunk and extremities. It appears after several days to over 2 weeks of administration of certain drugs (eg, allopurinol, cytarabine, atropine, barbiturates, quinidine, phenytoin, isoniazid, methotrexate, morphine, naproxen, nitrofurantoin, penicillins, piroxicam, sulfonamides, iodine) and disappears within several days of their discontinuation.

12) Psychogenic purpura: This develops almost exclusively in women with a tendency to anxiety or personality disorder and depression. Typical manifestations are painful edematous petechiae and erythema on the upper extremities and thighs, which are preceded by pruritus, burning sensation, or pain. The condition is usually mild and follows a pattern of remissions and relapses. No effective treatment is available; psychotherapy may sometimes lead to improvement.

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