Term
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Definition
| Kidney hamartoma, composed of blood vessels, muscle, and mature adipose tissue. Association wilh tuberous sclerosis. |
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Term
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Definition
| Liver angiosarcoma associated with exposure to polyvinyl chloride, arsenic, thorium dioxide. |
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Term
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Definition
| Benign capillary proliferation involving skin and visceral organs in patients. Simulates Kaposi's sarcoma in AIDS. Caused by Bartonella henselae, a gram-negative bacillus. |
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Term
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Definition
Facial lesion in newborns that regresses with age (strawberry)
Adult lesion that does not regresses and frequency increases with age.
(cherry) |
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Term
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Definition
| Most common benign tumor of liver and spleen. May rupture if large. |
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Term
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Definition
| Lymphangioma in the neck associated with Turner's syndrome. |
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Term
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Definition
| Derive from arteriovenous shunts in glomus bodies. Painful red subungual nodule in a digit. |
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Term
| Hereditary telangiectasia |
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Definition
| (AD) Dilated vessels on skin and mucous membranes In mouth and gastrointestinal tract. Chronic iron deficiency anemia. |
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Term
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Definition
| Malignant tumor arising from endothelial cells or primilive mesenchymal cells. Associated wilh human herpesvirus type 8. Raised, red-purple discoloration that progresses from a flat lesion to a plaqueto a nodule that ulcerates. Common sites include skin (most common site), mouth, and gastrointestinal tract. |
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Term
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Definition
| Malignancy of lymphatic vessels. Arises out of long-standing chronic lymphedema (e.g., after modified radicalmastectomy) |
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Term
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Definition
| Vascular, red pedunculaled mass that ulcerates and bleeds easily. Post-traumatic or associated wilh pregnancy (relation lo estrogen); usually regress postpartum. |
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Term
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Definition
| Arteriovenous fistula (disappears when compressed.) Associated with hypcrestrinism (e.g., cirrhosis, pregnancy) |
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Term
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Definition
| Nevus flammeus ("birthmark") on the face in distribulion of ophlhalmic branch of cranial nerve V (trigeminal) Some cases show ipsilateral malformalion of pia maler vessels overlying occipilal and parietal lobes |
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Term
| Von Hippel-Lindau syndrome |
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Definition
| (AD) Cavernous hemangiomas in cerebellum and retina. Increased incidence of pheochromocyloma and bilateral renal cell carcinomas. |
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Term
| Takayasu arteritis ("pulseless disease") |
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Definition
| Granulomatous large vessel vasculitis involving aorlic arch vessels. Young Asian women and children. Absent upper extremity pulse. Discrepancy in blood pressure between arms > 10 mm Hg. Visual defects, stroke. Treatment; corticosteroids. |
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Term
| Giant cell (temporal) arteritis |
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Definition
| Granulomatous large vessel vasculitis involving superficial temporal andophthalmic arteries. Adults > 50 years of age. Temporal headache, jaw claudication (pain when chewing stretches inflamed artery) Blindness on ipsilaleral side. Polymyalgia rheumalica (muscle and joint pain; normal serum creatine kinase) Increased ESRTreatment; corticosteroids. |
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Term
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Definition
| Necrotizing medium-sized vessel vasculitis involving renal, coronary, mesenteric arteries (spares pulmonary arteries). Middle-aged men. Association with HBsAg (30%). Vessels at all stages of acute andchronic infammation Focal vasculitis produces aneurysms (delected with angiography) Organ infarction in kidneys (renalfailure), heart (acute Ml), bowels(bloody diarrhea), skin (ischemiculcer), testicle (testicular pain)Angiography and biopsy of lesions confirm the diagnosis. Treatment; corticosteroids |
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Term
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Definition
| Necrotizing medium-sized vessel vasculitis involving coronary arteries (e.g., thrombosis,aneurysms). Children < 5 years of age. Boys > girls. Cause unknown (probably infectious) Children of Asian descent have highest incidence. Surpassed acute rheumatic heart disease as most common acquired heart disease in children. Fever, erythema and edema ofhands and feel convalescing with desquamated rash; cervicaladenopathy; oral erythema and cracking of the lips Abnormal ECG (e.g., acule Ml) Treatment: inlravenous immunoglobulin; aspirin; corticosteroids contraindicated (danger of vessel rupture). |
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Term
| Thromboangiitis obliterans (Buerger's disease) |
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Definition
| Medium-sized vesse lvasculitis with digital vessel thrombosis and damage to neurovascular compartment. Men 25-50 years of agewho smoke cigarettes. Middle East, Far East, Asia has highest prevalence. Resting pain on the forefoot ischaracteristic, with possible ischemiculcers or gangrene of foot/toes;upper limb ischemia [40% lo 50% of patients) with ulceration and gangrene; Raynaud's phenomenon. Treatment: smoking cessalion essential; intravenous iloprost (prostaglandinanalogue). |
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Term
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Definition
| Medium-sized vessel vasculilis involving digital vessels in fingers and toes; also Up of nose and earsin some cases. Young women. Exaggerated vasomotor response to cold or stress. Paroxysmal digital color changes (white-blue-red sequence) Ulceration and gangrene in chronic cases Treatment: avoid cold temperatures (gloves); calcium channel blockers (e.g., nifedipine). |
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Term
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Definition
| Medium-sized vessel vasculilis involving digital vessels in fingers and toes; also tip of nose and ear sin some cases. Adult men and women Secondary lo olher diseases (e.g., systemic sclerosis. CREST syndrome, SLE). Systemic sclerosis and CREST syndrome (see page 47); digital vasculit iswith vessel fibrosis, dystrophic calcification, ulceration, gangrene Treotmenl: see above. |
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Term
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Definition
| Necrotizing mediumand small-sized vessel vasculitis involving lung (infarctions, renal vessels). Childhood to middle age. Necrotizing granulomas in skin, upper respiratory tract (nasopharynx-saddlenose deformily, chronic sinusitis, collapse of trachea), lower respiratory tract (cavitating nodular lesions) Necrotizing vasculitis in lungs (infarction, hemoptysis), kidneys (crescentic glomerulonephritis) c-ANCA antibodies (>90% of cases) correlate erratically with therapy. Treatment: corticosteroids, cyclophosphamide. 3 Cs: c-ANCA, corticosteroids, cyclophosphamide. |
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Term
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Definition
| Small vessel vasculitis involving skin, lung, brain, Gl tract, and postcapillary venules and glomerular capillaries. Children and adults Precipitated by drugs (e.g.,penicillin), Infections (e.g.,streptococci), Immunedisorders (e.g., SLE). Vessels at same slage of inflammation Palpable purpura, glomerulonephritis p-ANCA antibodies (>8o% of cases). |
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Term
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Definition
| Small vessel vasculitis involving skin, lung, heart vessels. Children and adults. Allergic rhinitis, as thma p-ANCA antibodies (70% of cases), eosinophilia. |
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Term
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Definition
| Small vessel vasculitis involving skin, Cl, renal, joint vessels. Children and young adults Males > females Most common vasculilis In children IgA-anti-lgA immunocomplexes. Often follows a viral URI, group A streptococcal pharyngeal infection pathogens may act as an antigen trigger that causes antibody formation leading lo immunocomplex formalion Palpable purpura of buttocks and lowerextremities Polyarthritis (80%), nephropathy (80%), Gl bleeding Recurrence may occur in one third of cases Mosl have spontaneous recovery in 4 months without therapy. Treotmcrit: corticosteroids mainly used if severe Gl disease or renal disease. |
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Term
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Definition
| Small vessel vasculilis involving skin, Gltract, renal vessels. Different types of cryoglobulinemia (mixed, monoclonal, polyclonal). Adulls Associalion with HCV,type I MPGN, multiple myeloma (monoclonaltype). Cryoglobulins: immunoglobulins that gel at cold temperatures Palpable purpura, acral cyanosis of nose and ears and Raynaud's phenomenon (reverses when in warmroom); glomerulonephritis; arthritis; abdominal pain. |
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Term
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Definition
| Small vessel vasculitis involving skin vessels. Children and adults Involves all microbial pathogens. Rocky Mountain spotted fever: tick transmission of Rickettsia rickettsiae Organisms invade endothelial cells producing vasculitis Petechiae on palms spread to trunk Disseminaled meningococcemia due to Neisseria meningitides Capillary thrombosis produces hemorrhage into skin and confluent ecchymoses. |
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