Ch. 11 - Inflammatory vascular diseases Flashcards
Leukocytoclastic vasculitis
Perivascular neutrophilic infiltrate with vessel wall expansion and fibrin deposition. Erythrocyte extravasation.
What are the large-vessel vasculitides?
Giant cell arteritis
Takayasu arteritis
Polyarteritis nodosa
Thrombophlebitis
Thromboangiitis obliterans (Buerger’s disease)
Giant cell arteritis (temporal arteritis)
Subendothelial (to transmural) granulomatous inflammation involving large muscular artery (usually temporal) in older patient.
Takayasu arteritis
Granulomatous vasculitis involving large muscular arteries (usually aorta & branches) in younger patients.
Polyarteritis nodosa
Acute or chronic (granulomatous) inflammation of a large artery, especially at branch points. Fat necrosis is common. No small vessel inflammation or glomerulonephritis.
Thrombophlebitis
Vasculitis involving veins (no muscularis or internal elastic membrane). Thrombus usually present within vessel.
Thromboangiitis obliterans (Buerger’s disease)
Prominent neutrophilic endarteritis with thrombus.
What are the medium-vessel vasculitides?
Wegener’s granulomatosis
Churg-strauss syndrome
Microscopic polyangiitis
Rheumatoid vasculitis
Septic vasculitis
Wegener’s granulomatosis
(associations?)
Vasculitis (or stellate abscess) involving many vessel sizes with endothelial necrosis. Affects skin, upper respiratory tracts and kidneys.
c-ANCA / anti-proteinase 3 positive.
Churg-strauss syndrome
Vasculitis involving many vessel sizes with endothelial necrosis. No giant cells. Affects skin and kidneys.
Associated with asthma, p-ANCA / myeloperoxidase positive.
Microscopic polyangiitis
Vasculitis involving many vessel sizes with endothelial necrosis. Need systemic involvement and ANCA positivity to define the syndrome.
p-ANCA / myeloperoxidase positive.
What are the small-vessel vasculitides?
Henoch-schonlein purpura
Mixed cryoglobulin dsiease
Drug-induced and idiopathic leukoclastic vasculitis
Mixed connective tissue diseases
Serum sickness
Henoch-schonlein purpura
Post-capillary venule inflammation with fibrin and erythrocyte extravasation. IgA deposition in DIF. Affects skin/gut/kidneys of children.
Mixed cryoglobulin disease
Vasculitis of small to medium vessels with fibrin and erythrocyte extravasation. Cryoglobulin deposits. Skin & glomeruli involved.
Granuloma faciale
Leukocytoclastic vasculitis with eosinophils and later onion-skin fibrosis. “Grenz zone” separates epidermis. Affects facial skin, but no granulomas.
Erythema elevatum diutinum (EED)
Leukocytoclastic vasculitis with eosinophils and later onion-skin fibrosis affecting the extensor surfaces.
Sweet’s syndrome (acute febrile neutrophilic dermatosis)
Marked papillary dermal edema and neutrophilic infiltrate in a hot tender plaque, usually after URI or with associated MPN.
Urticaria
(early & late)
Early: Neutrophils in superficial dermal vessels
Late: Diapedesis of neutrophils, eosinophils, and monocytes. No fibrin or karyorrhexis.
Well’s syndrome (eosinphilic cellulitis)
(cause?)
Flame figures (eosinophil degranulation onto collagen)
Usually an exaggerated arthropod reaction.
Differential diagnosis: Perivascular lymphoid infiltrates
Pigmenting purpuric eruption (PPE)
Gyrate erythemas
Timid lupus
Polymorphous light eruption
Morbilliform drug eruptions
Pigmenting purpuric eruption
Lymphoid peri-capillary infiltrate with erythrocyte extravasation and hemosiderin.
Gyrate erythemas
Dense “coat-sleeve” perivascular lymphoid infiltrate that leaves vessels intact.
Timid lupus erythematosus
Perivascular lymphoid inflammation with lymphocytes in eccrine coil and dermal mucin.
Polymorphous light eruption
Perivascular lymphoid inflammation with papillary dermal edema and variable spongiosis.
What diseases present with lymphoid vasculitis?
Degos disease (malignant atrophic papulosis)
Insect bite
Perniosis
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)
Insect bite
Dense wedge-shaped perivascular lymphoid infiltrate with eosinophils, focal vessel damage, and papillary dermal edema.
Perniosis
Superficial and deep perivascular lymphoid infiltrate with fluffy edema and no fibrin. Affects acral skin.
What are the occlusive vascular diseases?
Type I cryoglobulinemia
Cholesterol embolization
Livedoid vasculopathy
Stasis change
Various coagulopathies…
Type I cryoglobulinemia
(cause?)
Occlusion of vessels by pink jelly-like substance and erythrocyte extravasation.
(represents plasma cell dyscrasia)
Cholesterol embolization
Arteriole containing cholesterol clefts, often after catheterization or anticoagulation.
Livedoid vasculopathy
Hyalinized vessel walls and thrombi with dermal stasis changes.
Stasis change
Cannon-ball tufting of vessels in superficial dermis and erythrocyte extravasation (with dermal hemosiderin).
Giant cell (temporal) arteritis
Takayasu arteritis
Polyarteritis nodosa
Thrombophlebitis obliterans
Wegener’s granulomatosis
Churg-strauss syndrome
Septic vasculitis
(vascular IgA deposition on DIF)
Henoch-Schonlein purpura
Granuloma faciale
Erythema elevatum diutinum
Urticarial wheal, early
Sweet’s syndrome
Urticarial wheal, late
Well’s syndrome
Pigmenting purpuric eruption
Timid lupus erythematosus
Polymorphous light eruption
Degos disease
Insect bite
Perniosis
Type I cryoglobulinemia
Cholesterol embolization
Livedoid vasculopathy
Stasis changes
