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Vascular bleeding disorders

  • Lesion

When the small vessels themselves are weak, the skin and mucosa bleed even though platelets and clotting tests are normal.

Updated

What it is

Vascular bleeding disorders are common but rarely severe. They cause petechiae and purpura of skin and mucous membranes with normal platelet counts and clotting tests. Mechanisms include infections that damage endothelium (meningococcaemia, rickettsiae, infective endocarditis), immune-complex vasculitis caused by drugs or IgA, defective collagen (scurvy, Ehlers–Danlos syndrome), loss of supporting tissue (old age, glucocorticoid excess), malformed vessels (hereditary haemorrhagic telangiectasia) and amyloid infiltration of vessel walls.

Structure

  • Telangiectasias: dilated, thin-walled capillaries and venules lined by a single endothelial layer.
  • Leukocytoclastic vasculitis: fibrinoid necrosis of venules, neutrophils and nuclear dust, red cell extravasation; IgA deposits in IgA vasculitis.
  • Scurvy: poorly formed collagen around vessels and hair follicles with haemorrhage.

Step by step

  1. Endothelium is damaged (infection, immune complexes) or the vessel support is weak (collagen, amyloid, atrophy).
  2. Small vessels rupture with ordinary pressures or minor trauma.
  3. Red cells leak into skin and mucosa: petechiae, purpura, ecchymoses.
  4. Platelet and coagulation function are intact, so bleeding is usually limited.
  5. Malformed vessels (HHT) bleed repeatedly from exposed surfaces, causing iron deficiency.

Causes of vascular purpura

CauseMechanismKey features
ScurvyDefective collagen hydroxylationPerifollicular haemorrhage, gum bleeding
Ehlers–Danlos syndromeInherited collagen defectsEasy bruising, hyperelastic skin, joint laxity
Cushing syndrome and old ageLoss of perivascular supportSenile purpura on forearms, thin skin
IgA vasculitisIgA immune-complex vasculitisPalpable purpura, abdominal pain, arthritis, nephritis
Hereditary haemorrhagic telangiectasiaMalformed thin vessels and arteriovenous malformationsEpistaxis, GI bleeding, lung and brain AVMs
AmyloidosisAmyloid in vessel wallsPeriorbital purpura, macroglossia

Clinical notes

  • Fever with spreading petechiae suggests meningococcaemia, an emergency.
  • Drug-induced hypersensitivity vasculitis is a common cause of purpura without thrombocytopenia.

Test yourself

Think of the answer, then tap the question to see it.

Which blood tests are abnormal in vascular bleeding disorders?

Usually none: platelet count, PT and PTT are normal.

Which genes are involved in hereditary haemorrhagic telangiectasia?

Genes of the TGF-β signalling pathway, such as endoglin (ENG) and ACVRL1.

Why does scurvy cause bleeding?

Vitamin C is needed to hydroxylate collagen; weak collagen fails to support small vessels.

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: Hereditary hemorrhagic telangiectasia: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Bleeding Disorders | MedlinePlus (MedlinePlus, US National Library of Medicine, 2026).

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Representative model, for educational purposes only.