Vascular disorders Flashcards
Clinical manifestation of the red blood cell extravasation into mucosa or skin, results from various conditions, including rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders.
Purpura
Refers to visible hemorrhage into mucous membrane or skin, which corresponds to extravasation of RBC around dermal small vessels and chronic hemosiderin deposition.
Purpura
Compromises disorders that result in bruising but are not associated with any specific disease
PRIMARY PURPURA
AKA Purpura Simplex or Devil’s pinches
Occurs as a result of skin fragility
Occur in children as they approach puberty
Simple Purpura
Occurs as result of sudden increase in capillary pressure and usually manifests as petechiae.
Sneezing, coughing, valsalva maneuvers or seizures may cause this problem.
Mechanical Purpura
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.
Senile Purpura
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.
Factitious Purpura
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
Psychogenic 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.
Schamberg’s Purpura
Disorders caused by Vascular Abnormalities
SECONDARY 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.
Henoch–Schonlein Purpura
induced by iodides, quinine, procaine, penicillin, and aspirin
Drug – Induced Purpura
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. Rickettsia, viruses, cocci
Purpura Associated with Infections
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
Henoch–Schonlein Purpura
Ecthyma gangrenosum
May be seen in endocarditis
Septic Emboli to the skin
A unique disorder characterized by sudden onset, fever, prostration, symmetric circumscribed ecchymoses and infarcts of the skin and frequently by gangrene of the extremities.
Purpura Fulminans
Caused by a deficiency in Vitamin C
affects synthesis of collagen
hydroxylation of lysine and proline
Scurvy
Caused by biochemical or hormonal abnormalities
Metabolic Purpura
The major initiating factor appears to diffused vascular injury and intravascular coagulation.
The term purpura fulminans applies to any purpura on rapid onset.
Purpura Fulminans
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.
Cushing’s Syndrome
A disorder of women that presents with recurrent purpura on the lower extremities and resultant hemosiderin staining of the skin similar to Schamberg’s
Waldenstrom’s Purpura
Caused by production of cryoprecipitate serum proteins of protein complexes, results from primary plasma cell dyscrasias or from hepatitis C.
Cryoglobulinemia
Results from hypergammaglobulinemia owing to an increase in plasma viscosity.
Hyperviscosity Syndrome
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.
Amyloidosis
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
Hereditary Hemorrhagic Telangectasia
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.
Angiodysplasia
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.
Ehlers–Danlos 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.
Marfan Syndrome
Autosomal recessive disorder affecting elastic fibers of connective tissue of skin and arteries.
Pseudoxanthoma Elasticum
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.
Osteogenesis Imperfecta
