Vascular- Disorders PDF

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Summary

DISORDERS OF PRIMARY HEMOSTASIS Vascular Disorders (Primary and Secondary Purpura) Purpura  Clinical manifestation of the red blood cell extravasation into mucosa or skin, results from various conditions, including rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders.  Ref...

Description

DISORDERS OF PRIMARY HEMOSTASIS Vascular Disorders (Primary and Secondary Purpura) Purpura  Clinical manifestation of the red blood cell extravasation into mucosa or skin, results from various conditions, including rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders.  Refers to visible hemorrhage into mucous membrane or skin, which corresponds to extravasation of RBC around dermal small vessels and chronic hemosiderin deposition.

Vascular Purpura

PRIMARY PURPURA Compromises disorders that result in bruising but are not associated with any specific disease

1. Simple Purpura   

AKA Purpura Simplex or Devil’s pinches Occurs as a result of skin fragility Occur in children as they approach puberty

2. Mechanical Purpura 

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Occurs as result of sudden increase in capillary pressure and usually manifests as petechiae. Sneezing, coughing, valsalva maneuvers or seizures may cause this problem.

3. Senile Purpura VASCULAR PURPURA

Primary Purpura

Simple Purpura Mechanical Purpura Senile Purpura Factitious Purpura Schamberg’s Purpura

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Secondary Purpura Infectious Purpura Allergic Purpura

Metabolic Purpura Psychogenic Purpura Purpura Second to Dysproteinemia

Vascular and Connective Tissue Disorder

Waterhouse–Friderichsen Syndrome Purpura Fulminans Septic Emboli Henoch–Schonlein Purpura Drug Sensitivity Scurvy Cushing’s Syndrome Diabetes Mellitus Protein C Deficiency Gardener–Diamond Syndrome DNA Hypersensitivity Waldenstrom’s Purpura Cryoglobulinemia Amyloidosis Hyperviscosity Syndrome Hereditary Hemorrhagic Telangectasia Angiodysplasia Giant Hemangioma (Kasabach–Merrit Syndrome) Ehlers–Danlos Syndrome Marfan Syndrome Pseudoxanthoma Elasticum Osteogenesis Imperfecta

AKA Purpura Sinelis Seen in older individuals, or in individuals undergoing corticosteroid therapy Purpuric lesions on the hand and arms Easy bruising in older people because their skin is more fragile.

4. Factitious Purpura 

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

5. Psychogenic Purpura  

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AKA Gardner – Diamond Syndrome Seen in individuals with emotional problems, often after severe trauma or extensive surgery, which may be hypertensive to RBC membrane components or DNA hypersensitivity. Painful Bruising Syndrome

6. Schamberg’s Purpura  

Also referred to as Progressive Pigmentary Purpura Characterize by the development of cayenne pepper petechiae (hallmark) on a background of hyperpigmented brown or orange oval patches often seen in tibial regions bilaterally as a chronic eruptions.

 The major initiating factor appears to diffused vascular injury and intravascular coagulation.  The term purpura fulminans applies to any purpura on rapid onset.

SECONDARY PURPURA Disorders caused by Vascular Abnormalities Autoimmune Vascular Purpura 

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The Allergic Purpura  This is a syndrome characterized by a relatively distinctive purpuric eruption in association with various constitutional and localized symptoms. The disorder is the result of an autoimmune process or allergic vasculitis.  E.g. Henoch–Schonlein Purpura  Result of allergic vasculitis which involves the skin, GIT, kidneys, heart and CNS.  It is considered as an immune complex disease 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.  Classic triad: Arthritis, Abdominal pain, Purpura  Diagnosis: Increased BUN and creatinine; IgA , CRP

Metabolic Purpura Caused by biochemical or hormonal abnormalities 

Scurvy  Caused by a deficiency in Vitamin C

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Cushing’s Syndrome  Caused by corticosteroid excess results in purpura  Other cutaneous stigmata includes a “moon face”, pigmented abdominal striae, a “buffalo hump” on the lower neck and upper back.

Purpura Secondary to Dysproteinemia 

Waldenstrom’s Purpura  A disorder of women that presents with recurrent purpura on the lower extremities and resultant hemosiderin staining of the skin similar to Schamberg’s

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Cryoglobulinemia  Caused by production of cryoprecipitate serum proteins of protein complexes, results from primary plasma cell dyscrasias or from hepatitis C.

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Hyperviscosity Syndrome  Results from hypergammaglobulinemia owing to an increase in plasma viscosity.

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Amyloidosis  Bleeding is caused by deposition of amyloid protein around small blood vessels, resulting in vessel fragility  Low factor X levels from binding of Factor X to amyloid fibrils, hyperfibrinolysis related to excessive urokinase activity and platelet function alterations may enhance bleeding tendencies.

Drug – Induced Purpura  This is a Purpura induced by iodides, quinine, procaine, penicillin, and aspirin

Purpura Associated with Infections A wide variety of infections may produce Purpura by means of vascular damage which results from direct endothelial injury by the infectious agents. E.g. Ricketsia, viruses, cocci 

Septic Emboli to the skin  Ecthyma gangrenosum  May be seen in endocarditis

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Purpura Fulminans  A unique disorder characterized by sudden onset, fever, prostration, symmetric circumscribed ecchymoses and infarcts of the skin and frequently by gangrene of the extremities.

Disorders Caused by Structural Malformations of Vessels and Perivascular Tissues

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Hereditary Hemorrhagic Telangectasia  AKA Osler – Weber – Rendu Syndrome  Vascular malformation involves vessels throughout the body, which is dilated, tortuous and disorganized.  It is characterized by the presence of widespread telangectasia lesions of the skin and mucus membrane.  Lesions develop on the tongue, lips, palate, face, and hands.  IDA is usual

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Angiodysplasia  Disorder involving the blood vessels of GIT  Cutaneous lesion of hereditary hemorrhagic telangectasia is not present.  Has been associated with von Willebrand’s disease.  Hemicolectomy has been used to treat the GIT bleeding.

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Hereditary Disorders of Connective Tissue

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Ehlers–Danlos Syndrome  AKA Cutis Hyperelastica  Autosomal dominant disorder characterized by hyper distensible joints and fragile skin  Bleeding is due to abnormalities of collagen in blood vessel walls leading to vascular fragility.

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Marfan Syndrome  Autosomal dominant genetic disorder due to mutation of the gene for fibrillin resulting in abnormalities of connective tissues and risk for bleeding and bruising.

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Pseudoxanthoma Elasticum  Autosomal recessive disorder affecting elastic fibers of connective tissue of skin and arteries.

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Osteogenesis Imperfecta

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AKA Lobstein Syndrome / Brittle Bone Disease Rare autosomal dominant disorder caused by mutation of genes which codes for peptides of Type 1 collagen. Individuals may demonstrate easy bruising, epistaxis, hemoptysis, and intracranial bleeding....