Disorders Of Primary Hemostasis Vascular Disorders (Primary And Secondary Purpura)

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Clinical manifestation of red blood cell extravasation into mucosa or skin, results from various condition.

Refers to visible hemorrhage into mucus memebrane or skin, which corresponds to extravasation of red blood cell.

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Purpura

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Unknown cause.

Compromises disorders that result in bruising but are not associated to any specific disease.

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Primary Purpura

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It occurs as a result of skin fragility.

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Simple purpura

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Simple Purpura is also known as?

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"Devil's pinches"

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Occurs as a result of sudden increase in capillary pressure and usually manifests as petechiae (sneezing, coughing, valsalva maneuvers or seizure may cause this problem).

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Mechanical purpura

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Seen in older individuals or in individuals undergoing corticosteroid therapy, purpuric lesions occur on the hand and arms.

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Senile purpura

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It is caused by self-induced trauma and usually is found on areas of the body that are easily accessible (pinching, suction or a blow to the skin).

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Factitious purpura

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It is seen in individuals with emotional problems, often after severe trauma or extensive surgery, which may be hypersensitive to RBC membrane conponents or DNA hypersensitivity.

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Psychogenic purpura

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It is characterized by the development of cayenne pepper petechiae on a background of hyperpigmented brown or orange oval patches seen in tibial regions bilaterally as a chorinc eruptions.

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Schamberg's purpura

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Schamberg's purpura is also known as?

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Progressive pigmentary purpura

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Disorders caused by vascular abnormalities.

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Secondary Purpura

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This is a syndrome characterized by a relatively distinctive purpura eruption.

The disorders is the result of an autoimmune process or allergic vasculitis.

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Allergic purpura

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This is a purpura induced by iodides, quinine, procaine, penicillin and aspirin.

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Drug induced purpura

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Result of allergic vasculitis which involves the skin, GIT, kidneys, heart and CNS.

Immune complex dse. and is characterized by involvement of capillaries with diffuse infiltration of neutrophils, lymphocytes and macrophages.

It is commonly seen in children, renal dysfunction is common and typically reversible in children.

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Henoch-Schonlein purpura

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A wide variety of infection may produce purpura by means of vascular damage which result from direct endothelial injury by the infectious agent eg. Ricketssia, Viruses, Cocci etc.

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Infectious purpura

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to the skin (Ecthyma gangrenosum) may be seen in endocarditis.

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Septic emboli

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A unique disorder characterized by sudden rapid onset fever, postration, symmetric circumscribed ecchymoses and infarcts of the skin and frequently by gangrene of extremities.

Applies to any purpura of rapid onset.

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Purpura fulminans

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Caused by biochemical or hormonal abnormalities.

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Metabolic Purpura

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It is caused by a deficiency in Vit. C

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Scurvy

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Caused by corticosteroid excess results in purpura; other cutaneous stigmata includes a "moon face", pigmented abdominal striae, "buffalo hump" on the lower neck and upper back.

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Cushing's syndrome

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Is a disorder of women that represents recurrent purpura on the lower extremities and resultant hemosiderin staining of the skin similar to schamberg's.

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Waldenstrom's purpura

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Is caused by production of cryoprecipitable serum proteins or protein complexes (result from primary plasma cell dyscrasias or from hepatitis C)

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Cryoglobulinemia

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Results from hypergammaglobulinemia owing to an increase in plasma viscosity.

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Hyperviscosity syndrome

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Bleeding is caused by deposition of amyloid protein around small blood vessels, resulting in vessel fragility.

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Amyloidosis

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Vascular malformation involves vessels throughout the body, which are dilated, tortous and disorganized. It is characterized by the presence of widespread telangiectatic lesions of the skin and mucous membrane.

Lesions develop on the tongue, lips, palate, face and hands.

IDA IS USUAL.

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Hereditary hemorrhagic telangiectasia

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Hereditary hemorrhagic telangiectasia is also known as?

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Osler-Weber-Rendu Syndrome

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Disorder involving the blood vessels of the GIT.

Has been associated with Von Willerbrand's disease.

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Angiodysplasia

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It has been used to treat the GIT bleeding.

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Hernicolectomy

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Is an autosomal-dominant disorder characterized by hyperdistensible joints and fragile skin; bleeding is due to abnormalities of collagen in blood vessel walls leading to vascular fragility.

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Ehler-danlos syndrome

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Is an autosomal-dominant genetic disorder due to mutation of the gene for fibrillin resulting in abnormalities of C.T and risk for bleeding and bruising.

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Marfan syndrome

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Is an autosomal-recessive disorder affecting elastic fibers of connective tissue of skin and arteries.

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Pseudoxanthochroma elasticum

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Is a rare autosomal-dominant disorder caused by mutation of genes which code for peptides of Type 1 Collagen; individuals may demonstrate easy bruising, epistaxis, hemoptysis and intracranial bleeding.

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Osteogenesis imperfecta