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
What diseases present with lymphoid vasculitis?
Degos disease (malignant atrophic papulosis) Insect bite Perniosis
26
Degos disease (malignant atrophic papulosis)
Wedge-shaped superficial and deep perivascular lymphoid infiltrate with vascular damage. Eventual avascular necrosis and mucinosis. (can be a manifestation of lupus)
27
Insect bite
Dense wedge-shaped perivascular lymphoid infiltrate with eosinophils, focal vessel damage, and papillary dermal edema.
28
Perniosis
Superficial and deep perivascular lymphoid infiltrate with fluffy edema and no fibrin. Affects acral skin.
29
What are the occlusive vascular diseases?
Type I cryoglobulinemia Cholesterol embolization Livedoid vasculopathy Stasis change Various coagulopathies...
30
Type I cryoglobulinemia | (cause?)
Occlusion of vessels by pink jelly-like substance and erythrocyte extravasation. (represents plasma cell dyscrasia)
31
Cholesterol embolization
Arteriole containing cholesterol clefts, often after catheterization or anticoagulation.
32
Livedoid vasculopathy
Hyalinized vessel walls and thrombi with dermal stasis changes.
33
Stasis change
Cannon-ball tufting of vessels in superficial dermis and erythrocyte extravasation (with dermal hemosiderin).
34
Giant cell (temporal) arteritis
35
Takayasu arteritis
36
Polyarteritis nodosa
37
Thrombophlebitis obliterans
38
Wegener's granulomatosis
39
Churg-strauss syndrome
40
Septic vasculitis
41
(vascular IgA deposition on DIF)
Henoch-Schonlein purpura
42
Granuloma faciale
43
Erythema elevatum diutinum
44
Urticarial wheal, early
45
Sweet's syndrome
46
Urticarial wheal, late
47
Well's syndrome
48
Pigmenting purpuric eruption
49
Timid lupus erythematosus
50
Polymorphous light eruption
51
Degos disease
52
Insect bite
53
Perniosis
54
Type I cryoglobulinemia
55
Cholesterol embolization
56
Livedoid vasculopathy
57
Stasis changes