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42 Cards in this Set

  • Front
  • Back
Giant Cell (TEMPORAL) arteritis: what is it?

What does it look like?
most common form of systemic vasculitis; affects large and small arteries, primarily those in the head

nodular thickening, lumen reduction, granulomatous inflammation(w/ giant cells), and fragmentation of the internal elastic lamina
What causes Giant Cell (TEMPORAL) arteritis?

How does it present?
cause is unknown but immunogenic mech is suspected

PW facial pain
tenderness of temporal artery
visual impairments
Polymagia rheumatica

Rare b4 age 50
Takayasu Arteritis: what is it?

What does it look like?
also known and the pulseless disease; characterized by inflammation and stenosis of medium to large arteries (aortic arch and its branches= AORTIC ARCH SYNDROME)

Granulomatous vasculitis and fibrous thickening w/ narrowing or virtual obliteration of origins
What causes Takayasu Arteritis?

How does it present?
cause is unknown but immunogenic mech is suspected

absent pulse
nonspecefic findings such as fever, night sweats, malaise, arthritis, eye problems and skin nodules
What is Polyateritis nodosa?

What does it look like?
a segmental transmural necrotizing arteritis characterized by necrotizing immune complex formation of small and medium sized arteries; also fibrinoid necrosis in any organ

-destruction of arterial media and internal elastic lammella resulting in aneurysmal nodules
-necrotic lesions in the kidney, coronary arteries, musculoskeletal system, GI tract, aand CNS
What causes Polyarteritis nodosa?
- Imparied perfusion leads to ulcers, infarcts, ischemic atrophy of hemorrhage
- associated w/ hepatitus B in 30% of patients
What are the clinical manifestations of Polyarteritis nodosa?
manifested by fever, wieght loss, malaise, abdominal pain, headache, mylagia, and HT

elevated serum Abs to neutrophilic myeloperoxidase

symptoms remmitant and episodic

fatal if untreated, steroids give remission in 90% of cases

typically affects young adults
What is Kawasaki Disease?

What causes it?
leading cause of acquired heart disease in North America and Japan; a PAN like vasculitis often involving the coronary arteries in young children and infants

cause is unknown but there is evidence for immune reaction
How does Kawasaki Disease present?
associated w/ mucocutaneous syndrome manifested by:
conjunctival and oral erythema and erosion
edema of hands and feet
erythma of the palms and soles
skin rash
cervical lymph node enlargement

20% develop cardiac sequelae(aneurysm)

acute fatalities in 1%
Microscopic polyangitis
(Microscopic polyarteritis, Hypersensitivity/ Leukocytoclastic Vasculitis)

What is it?
group of immune complex mediated vasculitides (necrtotizing vasculitis) characterized by acute inflammation of small bvs(arterioles, venules, capillaries)
What causes Microscopic polyangitis?
usually and immune reaction to drugs, microorganisms, heterologous proteins or tumor antigens
What are clinical manifestations of Microscopic polyangitis?
presents with hemoptysis, arthhralgia, abdominal pain, hematuria, proteinuria, hemorrhage, muscle pain and weakness

palpable purpura when skin is involved but can involve any site inc. glomeruli or GI tract

70% have pANCAs

most respond well to removal of offending agent
Wegener granulomatosis: what is it?

What does it look like?
acute necrotizing vasculitis of small to medium sized vessels of the U/L respiraory tract and/or kidneys

fibrinoid necrosis of small arteries and veins, infiltration of neutrophils w/ subsequent mononuclear cell infiltration, granuloma formation w/ giant cells
What causes Wegener granulomatosis?

What are clinical features (b/s presentaion)?
may represent some form of hypersensitivity to to inhaled infectious or environmental agents

affects males more than females w/ peak incidence in fifth decade

cytoplasmic ANCAs present in serum
How does Wegener granulomatosis present?
persistant pneumonitit with nodular and cavitary infiltrates
chronic sinunitis
mucosal ulcerations of nasopharynx
renal disease
skin rashes
muscle pains
articular involvement

80% die w/in year if untreated
What is Thromboangitis obliterans(Burger disease)?
an acute inflammation involving small to medium sized arteries of extremeties that can extend to adjacent veins & nerves
What are clinical manifestations of Thromboangitis obliterans(Burger disease)?
mosty common in men

usually occurs before age 35 in cigarette smokers due to hypersensitivity to tobacco extracts

results in painful ischemic disease w/ later complications inc. ulceration of toes, feet, and fingers; occasionally gangrene
Raynaud Phenomenon: what is it and what causes it?
parysomal pallor or cyanosis of the digits due to cold-induced vasconstriction of distal digital bvs
What are clinical features of Raynaud Phenomenon?
median age of onset 14, patients usually present at age >30

fingers usually change colors, red, white, blue

severe episodes, skin lesions

CT disorders should be looked at for underlying cause
Varicose Veins: what are they and what causes them?
abnormally dilated, tortuous veins in mainly the superficial veins of legs; also present in esophagus and anus

caused by variations in wall thickness, phlebosclerosis, increased venous pressure
What is clinical course of Varicose Veins?
most commonc in obese women over age 50

presents with edema, pain, thrombosis
stasis dermatitis, ulceration, poor wound healing, infections, and embolisms (rare)
Thrombophlebitis and Phlebothromosis...
arises most often in the deep veins of the lower extremeties
What factors predispose to Thrombophlebitis and Phlebothrombosis?
cardiac failure, neoplasi, pregnancy, obesity, postoperative state, prolonged bed rest or immobilization
What are clinical manifestations of Thrombophlebitis and Phlebothromosis?
migratory thrombophlebitis

leg thrombi usually don't produce symptoms --> Homan sign

may give rise to pulmonary embolism

*plegmasia alba dolens: ileofemoral venous thrombosis in pre and post partum women
What is Lymphangitis?
occurs when bacteria infections spread through lymphatics

they cause painful subcutaneous red streaks, and lymph node enlargement

may lead to septicemia
What is Lymphedema?
an abnormal accumulation of interstitial fluid

What are General Hemangiomas?

Are they malignant?
localized, superficial lesions most often of the head or neck

may be internal, 1/3 are on the liver

rarely malignant
Capillary Hamangioma: what is it?
the largest single type of vascular tumor that consists of tangles of closesly packed cappilary like channels that may occur in the skin, subq tissue, lips, liver, spleen, or kidneys

juvenile(strawberry) hemangioma: in 1/200 newborns
Describe Cavernous Hemangiomas?
consist of larger cavernous vascular spaces in the skin and mucosal surfaces and internal organs such as liver pancreas and spleen

can occur in von Hippel-Lindau disease
What is pyogenic granuloma?

exophytic, polypoid red nodule on the skin, gingiva or oral mucosa, bleed easily and often ulcerate

resembles exuberant granulation tissue

can recur following removal

*Granuloma Gravidarum - occurs in 1% of pregnacies
Lymphanigoma Circumscription?
capillary lymphangioma

occurs in head an neck region and axilla

histologically similar to hemangiomas w/o the blood
Cystic hygroma
cavernous hygroma

occurs in neck and axilla of children

can be large up to 15 cms
Glomus Tumor

most common in the distal portions of digits, under fingernails

painful slightly elveated red-blue nodules

glomus cells proliferate forming nests, aggregates and masses
Spider telangiectasia
small dilated vessel surrounded by radiating fine channels on face, neck and upper chest

associated with hyperestrinism

seen in chronic liver disease and pregnacy
Hereditary hemorrhagic telangiectasia
autosomal dominant condition characterized by local dilationand convolution of venules and capillaries of the skin and mucous membranes

Ossle-Weber-Rendu disease
What causes port wine stains and birthmarks?
Nevus Flemmeus
What is Baccillary Angiomatosis?

What causes it?
oppurtunistic infection involving skin, bones, brain; manifests as vascular proliferations resembles tumors

caused by infection with gram negative bacilli of the Bartonella family
What is Kaposi Sarcoma?

What are the 4 types?
low grade malignant vascular tumor that occurs in several forms

Chronic (classic or European)
Transplant associated
AIDS associated
What does Kaposi sarcoma look like histologically?
dense proliferation of spindle cells, plump endothelial cells and extravasated RBCs

atypia and mitoses are common
What is the pathogenesis is associated with Kaposi sarcoma?
majority of the lesions are infected with KS associated herpes virus (human herpes virus 8)

Immunosuppresion is an important cofactor
What is hemangioendothelioma?
wide spectrum of vascular neoplasms intermediate in behavior between a benign and malignant tumor

epitheliod occur in medium to large sized veins
What are the malignant vascular tumors?
angiosarcoma - rare malignant tumors occuring in the skin soft tissue, breast and liver

Hemangiopericytoma - neoplasm with fleshy spongy consistency
derived from pericytes, slowly growing masses in the pelvic retroperitoneumor lower ext of middle aged women