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50 Cards in this Set
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Spider telangiectasia
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Dilated small vessel surrounded by radiating fine channels
Associated with hyperestrinism (seen in liver dz and pregnancy) |
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Another name for hereditary hemorrhagic telangiectasia
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Osler-Weber-Rendu syndrome
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What is hereditary hemorrhagic telangiectasia?
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Autosomal dominant condition
localized dilation and convolution of venules and capillaries of skin and mucous membranes Complicated by epistaxis adn GI bleeding |
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What is hemangioma?
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Malformation of larger BV filled with blood
Most common tumor of infancy --> Port wine stain birthmarks |
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Capillary hemangioma
Location |
tangle of closely packed capillary channels seen in skin, subQ tissue, lips, liver, spleen, and kidney
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Cavernous hemangioma
Location Who Component of what other dz Histology |
Large cavernous vascular spaces in the spleen, liver, pancreas, and brain
Can occur in von Hippel Lindau dz Often seen inchildren Microscopically: Huge dilated spaces lined with stroma |
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Mode of transmission of von Hippel-Lindau disease
What else is this dz associated with? Features of this dz |
Autosomal dominant
Renal cell carcinoma Component of cavernous hemangioma Hemangioblastoma of cerebellum , brain stem and retina Adenomas and cysts are also common in liver, kidney, and pancreas |
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Glomangioma
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Small purplish painful subungual nodule in finger or to e
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Cystic hygroma
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cavernnous lymphangioma that occurs in neck or axilla
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Lymphangioma
Location Gross appearance Microscopic appearance |
In head, neck, axilllary subQ tissue
Grossly: Cutaneous nodules/pedunculated lesions/well demarcated compressible pink grey masses Microscopically: Networks of EC lined spaces. Contain no RBCs |
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Hemangioendothelioma
Behavior and histology |
Behavior is between malignant and benign
Ascular channels with masses and sheets of pleiomorphic spindle shaped large plump cells of endothelial origin |
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Hemangiopericytoma
Behavior and location |
Behavior is between malignant and benign
Arise in lower extremities adn retroperitoneum |
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Hemangiosarcoma
Behavior, histology, location |
Most malignant
Very rare small, well-demarcated nodules --> large fleshy gray-white soft tissue Occurs in skin, musculoskeletal system, breast and/or liver |
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What are risk factors for hemangiosarcoma
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Arsenic
Thorium dioxide (thorostat) PVC (associated with angiosarcoma of the liver) Can also occur in setting of chronic lymphedema (ex: 10 yrs after radical mastectomy for breast CA. Tumor arises from dilated lymphatic vessels) Also caused by chronic indwelling of foreign objects |
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4 types of KS and who gets it
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Classic KS- Ashkenazi Jews/Mediterraneans
Endemic (African) KS- young African men and children; 10% of all cancers in Africa; restricted to lymph nodes Epidemic KS- AIDS, esp homosexual males; caused by KS herpesvirus. Lesions in skin, mucous membranes, LN, GI, viscera Transplant KS- transplant pts (who are immunosuppressed). Cutaneous and visceral involvement. Stops if immunosuppressants are stopped |
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Characteristics of polyarteritis nodosa
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Necrotizing immune complex inflammation of small and medium sized arteries
Arterial media and internal elastic lamella are destroyed --> aneurysmal nodules |
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What is associated with polyarteritis nodosa?
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HBV
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Clincal manifestations of polyarteritis nodosa?
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Fever
Weight-loss Malaise Abdominal pain HA Myalgia HTN ARthritis Ischemic heart dz |
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Where can polyarteritis nodosa occur
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kidneys
coronary arteries musculoskeleta system GI tract CNS, PNS Eye Skin |
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What is elevated in polyarteritis nodosa
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P-ANCA
(serum AB to neutrophilic myeloperoxidase) |
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Most common cause of death from polyarteritis nodosa
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renal lesions and HTN
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Churg-Strauss syndrome
Location Clinical manifestations |
Necrotizing vascuitis (considered a variant on polyarteritis nodosa)
Involvement of pulmonary vasculature Marked peripheral eosinophilia Clnical manifestations of asthma |
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What is elevated in Churg-Strauss syndrome
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P-ANCA
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Microscopic polyangitis
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Distinct from PAN by involvement of small BV (PAN is small and medium sized BV)
Lesions all at same histological stage Acute inciting event --> immune complexes in previously sensitized host Lesions can be only in skin, or can combine lung, brain, heart, and kidney |
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Histology of mcroscopic polyangitis
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fibrinoid necrosis
Affected BV may show only fragmented neutrophilic nuclei around and within small BV walls |
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Histology of PAN?
Acute lesions Healing lesions Healed lesions |
Acute lesions are well-demarcated, induce thrombosis --> distal ischemic injury
Healing lesions show fibroblast proliferation superimposed on ongoing fibrinoid necrosis Healed lesions show marked fibrotic thickening of arteries with elastic lamina fragmentation and sometimes aneurysmal dilation |
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Hypersensitivity vasculitis
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Immune complex mediated vasculitides with acute inflammation of small BV
Lesions are all the same age |
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Clinical features of hypersensitivity vasculitis
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palpable purpura when skin is involved
can also involve glomeruli or GI tract |
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Causes of hypersensitivity vasculitis
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drugs
food infectious organisms complication of systemic illness (CT d/o or malignancy) |
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Dinstinctive syndromes seen in hyperesensitivity vasculitis
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Henoch-Schonlein purpura
Serum sickness |
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Henoch-Schonlein purpura
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Common in young kids
hemorrhagic urticaria of extensor surfaces of arms, legs, buttocks Fever, arthraliga, GI and renal involvement are all common CAn sometimes be post-streptococcal Associated with antecedent upper respiratory infections |
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Serum sickness
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Deposition of Ag-AB complexes in heart, joints, and kidneys
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Wegener granulomatosis
Etiology Characteristics Affected organs |
Unknown
necrotizing granulomatous vasculitis of small and medium sized vessels Respiratory tract, kidneys |
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Clinical features of Wegener granulomatosis
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Respiratory tract signs and symptoms
Paranasal sinuses and lungs are especially affected Necrotizing glomerulonephritis |
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histology of WEgener granulomatosis
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Fibrinoid necrosis of small arteries and veins
Granuloma formation with giant cells prominent |
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Marker for Wegener granulomatosis
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C-ANCA
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Giant Cell arteritides
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Seen in medium to large sized arteries
Characterized by granuloma formation with giant cells and infiltrates of mononuclear cells, neutrophils, eosinophils |
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2 types of giant cell arteriritis
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Temporal arteritis
Takayasu arteritis |
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Temporal arteritis
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most frequently occurring vasculitis
Systemic Common in elderly Most commonly affects branches of carotid artery (esp temporal artery) |
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Clinical manifestations of temporal arteritis
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malaise
fatigue HA Jaw claudication Tenderness Absent pulse Palpable nodules along course of involved artery visual impairment polymyalgia rheumatica Elevated ESR |
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Polymyalgia rheumatica
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Component of temporal arteritis
proximal muscle pain Periarticular pain morning stiffness |
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Takayasu arteritis
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inflammation and stenosis of medium and large sized arteries
Aortic arch and branches are frequently involved |
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Clinical features of Takayasu arteritis
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Absent pulses in carotid, radial, or ulnar arteries
Fever, night sweats, malaise, myalgia, arthritis Eye problems Painful skin nodules |
skin?
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Another name for Kawasaki Dz
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Mucocutaneous lymph node syndrome
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What is Kawasaki Dz?
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Acute, self limited ilness of infants and children
Acute necrotizing vasculitis of small and medium sized BV |
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Clinical features of Kawasaki dz
What can it lead to? |
Fever
emorrhagic edema of conjunctivae, lips, and oral mucosa Cervical lymphadenopathy Coronary artery vasculitis with aneurysm formation |
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Another name for Buerger dz
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Thromboangiitis obliterans
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What is Buerger dz?
Population Risk factor |
Acute inflammation involving small and medium sized arteries of extremities
Extends to adjacent veins and nerves Jewish population, young men Cigarette smoking |
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Clinical features of Buerger dz?
Histologic features of Buerger dz? |
Painful ischemia --> gangrene
Neutrophilic abscesses Mural thrombi with giant cell formation Late lesions show organiziation and recanalization |
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Lymphomatoid granulomatosis
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rare grnulomatous vasculitis
Infiltration by atypical lymphocytoid and plasmacytoid cells Chronic inflammatory condition --> lymphoproiliferative neoplasm (T-cell non-hodgkin's lymphoma) |
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