Ch. 11 - Inflammatory vascular diseases Flashcards

1
Q

Leukocytoclastic vasculitis

A

Perivascular neutrophilic infiltrate with vessel wall expansion and fibrin deposition. Erythrocyte extravasation.

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2
Q

What are the large-vessel vasculitides?

A

Giant cell arteritis

Takayasu arteritis

Polyarteritis nodosa

Thrombophlebitis

Thromboangiitis obliterans (Buerger’s disease)

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3
Q

Giant cell arteritis (temporal arteritis)

A

Subendothelial (to transmural) granulomatous inflammation involving large muscular artery (usually temporal) in older patient.

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4
Q

Takayasu arteritis

A

Granulomatous vasculitis involving large muscular arteries (usually aorta & branches) in younger patients.

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5
Q

Polyarteritis nodosa

A

Acute or chronic (granulomatous) inflammation of a large artery, especially at branch points. Fat necrosis is common. No small vessel inflammation or glomerulonephritis.

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6
Q

Thrombophlebitis

A

Vasculitis involving veins (no muscularis or internal elastic membrane). Thrombus usually present within vessel.

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7
Q

Thromboangiitis obliterans (Buerger’s disease)

A

Prominent neutrophilic endarteritis with thrombus.

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8
Q

What are the medium-vessel vasculitides?

A

Wegener’s granulomatosis

Churg-strauss syndrome

Microscopic polyangiitis

Rheumatoid vasculitis

Septic vasculitis

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9
Q

Wegener’s granulomatosis

(associations?)

A

Vasculitis (or stellate abscess) involving many vessel sizes with endothelial necrosis. Affects skin, upper respiratory tracts and kidneys.

c-ANCA / anti-proteinase 3 positive.

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10
Q

Churg-strauss syndrome

A

Vasculitis involving many vessel sizes with endothelial necrosis. No giant cells. Affects skin and kidneys.

Associated with asthma, p-ANCA / myeloperoxidase positive.

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11
Q

Microscopic polyangiitis

A

Vasculitis involving many vessel sizes with endothelial necrosis. Need systemic involvement and ANCA positivity to define the syndrome.

p-ANCA / myeloperoxidase positive.

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12
Q

What are the small-vessel vasculitides?

A

Henoch-schonlein purpura

Mixed cryoglobulin dsiease

Drug-induced and idiopathic leukoclastic vasculitis

Mixed connective tissue diseases

Serum sickness

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13
Q

Henoch-schonlein purpura

A

Post-capillary venule inflammation with fibrin and erythrocyte extravasation. IgA deposition in DIF. Affects skin/gut/kidneys of children.

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14
Q

Mixed cryoglobulin disease

A

Vasculitis of small to medium vessels with fibrin and erythrocyte extravasation. Cryoglobulin deposits. Skin & glomeruli involved.

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15
Q

Granuloma faciale

A

Leukocytoclastic vasculitis with eosinophils and later onion-skin fibrosis. “Grenz zone” separates epidermis. Affects facial skin, but no granulomas.

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16
Q

Erythema elevatum diutinum (EED)

A

Leukocytoclastic vasculitis with eosinophils and later onion-skin fibrosis affecting the extensor surfaces.

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17
Q

Sweet’s syndrome (acute febrile neutrophilic dermatosis)

A

Marked papillary dermal edema and neutrophilic infiltrate in a hot tender plaque, usually after URI or with associated MPN.

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18
Q

Urticaria

(early & late)

A

Early: Neutrophils in superficial dermal vessels

Late: Diapedesis of neutrophils, eosinophils, and monocytes. No fibrin or karyorrhexis.

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19
Q

Well’s syndrome (eosinphilic cellulitis)

(cause?)

A

Flame figures (eosinophil degranulation onto collagen)

Usually an exaggerated arthropod reaction.

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20
Q

Differential diagnosis: Perivascular lymphoid infiltrates

A

Pigmenting purpuric eruption (PPE)

Gyrate erythemas

Timid lupus

Polymorphous light eruption

Morbilliform drug eruptions

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21
Q

Pigmenting purpuric eruption

A

Lymphoid peri-capillary infiltrate with erythrocyte extravasation and hemosiderin.

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22
Q

Gyrate erythemas

A

Dense “coat-sleeve” perivascular lymphoid infiltrate that leaves vessels intact.

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23
Q

Timid lupus erythematosus

A

Perivascular lymphoid inflammation with lymphocytes in eccrine coil and dermal mucin.

24
Q

Polymorphous light eruption

A

Perivascular lymphoid inflammation with papillary dermal edema and variable spongiosis.

25
Q

What diseases present with lymphoid vasculitis?

A

Degos disease (malignant atrophic papulosis)

Insect bite

Perniosis

26
Q

Degos disease (malignant atrophic papulosis)

A

Wedge-shaped superficial and deep perivascular lymphoid infiltrate with vascular damage. Eventual avascular necrosis and mucinosis.

(can be a manifestation of lupus)

27
Q

Insect bite

A

Dense wedge-shaped perivascular lymphoid infiltrate with eosinophils, focal vessel damage, and papillary dermal edema.

28
Q

Perniosis

A

Superficial and deep perivascular lymphoid infiltrate with fluffy edema and no fibrin. Affects acral skin.

29
Q

What are the occlusive vascular diseases?

A

Type I cryoglobulinemia

Cholesterol embolization

Livedoid vasculopathy

Stasis change

Various coagulopathies…

30
Q

Type I cryoglobulinemia

(cause?)

A

Occlusion of vessels by pink jelly-like substance and erythrocyte extravasation.

(represents plasma cell dyscrasia)

31
Q

Cholesterol embolization

A

Arteriole containing cholesterol clefts, often after catheterization or anticoagulation.

32
Q

Livedoid vasculopathy

A

Hyalinized vessel walls and thrombi with dermal stasis changes.

33
Q

Stasis change

A

Cannon-ball tufting of vessels in superficial dermis and erythrocyte extravasation (with dermal hemosiderin).

34
Q
A

Giant cell (temporal) arteritis

35
Q
A

Takayasu arteritis

36
Q
A

Polyarteritis nodosa

37
Q
A

Thrombophlebitis obliterans

38
Q
A

Wegener’s granulomatosis

39
Q
A

Churg-strauss syndrome

40
Q
A

Septic vasculitis

41
Q

(vascular IgA deposition on DIF)

A

Henoch-Schonlein purpura

42
Q
A

Granuloma faciale

43
Q
A

Erythema elevatum diutinum

44
Q
A

Urticarial wheal, early

45
Q
A

Sweet’s syndrome

46
Q
A

Urticarial wheal, late

47
Q
A

Well’s syndrome

48
Q
A

Pigmenting purpuric eruption

49
Q
A

Timid lupus erythematosus

50
Q
A

Polymorphous light eruption

51
Q
A

Degos disease

52
Q
A

Insect bite

53
Q
A

Perniosis

54
Q
A

Type I cryoglobulinemia

55
Q
A

Cholesterol embolization

56
Q
A

Livedoid vasculopathy

57
Q
A

Stasis changes