Vascular Purpura

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Non-thrombocytopenic purpura
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Disorders simulating purpura

Congenital Acquired
1- Hereditary hemorrhagic telangectasia
2- Kasabach Merritt syndrome
3- Ehlar Danlos syndrome
4- Fabry syndrome
5- Hereditary hemangioma

Primary Secondary
1- Ease of Bruising 1- Infection
2- Senile purpura 2- Allergic or immunological:
3- Mechanical purpura - Henoch schenolin purpura: bleeding
4- Factitial (artificial) purpura - Behcet’s disease: Vasculitis & thrombosis
- SLE
- PAN
- Drugs as Sulpha.
3- Metabolic:
- Scurvy
- Steroid purpura
- Uremia
- Hypercalcemia
4- Psycogenic: Autoerythrocytic sensitization
5- Miscelleneous:
- Dysproteinemia
- Embolic
- Orthostatic
- External
 Hereditary Hemorrhagic Telangectasia
 Pathogenesis: AD mutation in genes encoding for proteins of the TGF-β signaling
→ arterio-venous malformation.
 Clinical picture (Diagnostic criteria):
- Epistaxis
- Muco-cautaneous telangectasia (0.5-3 mm spots that blanche on pressure)
- Visceral lesions
- Affection of at least 3 of the first degree relatives

Kasabach Merritt syndrome

 Pathogenesis: Abnormal endothelium → PLT adhesion and activation +
consumption of coagulation factors → systemic haemostatic defect
 Clinical picture: Kaposiform hemangioendothelioma - bleeding – microangiopathic
HA

Ehlar Danlos syndrome

 Pathogenesis: AD disorder of connective tissue
 Clinical picture: Hyperelastic skin – joint laxity – fragile blood vessels poor wound
healing

Fabry syndrome
 Pathogenesis: AD mutation of α galactosylase enzyme → defect in the glycolytic
pathway → lipid deposition on the blood vessel wall → rupture
 Clinical picture: Petechiae – hyperkeratosis – renal failure

Ease of Brusing (Purpura Simplex)

 Recurrent petechiae and itching in the lower limb of old females with normal coagulation
tests.

Senile Purpura
 Senility → weak collagen and muscles + ↓ elastin → lax skin + Purpuric rash on
mucous membranes and extensor surfaces of limbs (↑ with constipation).

Mechanical Purpura
 Chronic cough or Constipation
 Thrombosis or pressure from outside→ ↑ intra-luminal pressure of any vein

Factitial Purpura
 Purpuritic rash in accessible sites of children and hestrical patients

Infection (Vasculitis)
 Severe viral or bacterial infection as Rickettsia – meningitis – Dengue fever →
Toxins – DIC - Immunological

Henoch Schenolin Purpura

 Allergy (drug or food) → → vascular wall → IgA deposition → complement
activation → capiilaritis and arteriolitis of:
- Skin (buttocks – extensor surfaces): itching – pain – purpuritic eruption
- Kidney (glomerulonephritis): hematuria – oliguria – proteinuria – hypertension
- Joints: arthritis
- GIT (edema and enteritis): Melena – abdominal pain – itussusception

Scurvy
 ↓Vit C → abnormal hydroxyproline and collagen → fragile vessels →
perifollicular bleeding and eccymosis

Steroid purpura
 Steroids → ↑collagen catabolism →weak vessel wall

Hypercalcemia
 Ca ppt on the vessel wall → rigidity → fragility.

Autoerythrocytic sensitization (Diamond Gardner syndrome)

 Adult female + psychological problems + preceeding operation:
- Operation → hemolysis → sensitization to Hb → immune complex deposition
on blood vessel wall
- DNA sensitization against leukocyte nucleus
Dysproteinemia
1- Hypergammaglobulinemia: PPT of abnormal proteins on blood vessel wall
2- Cryoglobulinemia
3- Amyloidosis: amyloid deposition on blood vessel wall
4- Hyperviscosity as in polycythemia
Embolic Purpura
 Thrombosis – DIC – Fat embolism – Cholesterol embolism → blood vessel
obstruction → ↓ blood supply to the vessel wall
Orthostatic Purpura
 Lower limbs in varicose viens
External Purpura
 Areas exposed to persistent pressure