Eosinophilic Vasculitis
Vasculitic Pathology
Serum testing:
ANCA+ with pANCA- & cANCA-; RNP mildly+

H&E stain
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Vessels
Wall: Damaged; Cellular
Lumen: Lost
Epineurium
Eosinophils (Scattered) & other Cells
Nerve fascicle
Subpoerineurial edema

Congo red stain
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VvG stain
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Vessels
Wall: Damaged; Cellular; No fibrils
Endothelial cells: Large
Lumen: Lost

H&E stain
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Vessels
Wall: Damaged; Cellular; No fibrils
Endothelial cells: Large
Lumen: Lost

VvG stain
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VvG stain
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Vessels: Three types
Within cell focus: No fibrils; Endothelial cells large
Vein: No fibrils
Vascular remnants: Clustered

Congo red stain
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Inflammatory Cell Focus
Contains scattered eosinophils

Gomori trichrome stain
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VvG stain
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Granuloma: Epineurial
All cells in focus stain for acid phosphatase

Acid phosphatase stain
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Acid phosphatase stain
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Vasculopathy: Smaller Epineurial Vessels

VvG stain
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Epineurial Vessels: Small
Surrounded by: Cells; Pale epineurial connective tissue

H&E stain
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Epineurial Vessels: Neovascularization
Multiple small vessels within region of larger vessels

UEA I stain
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Histiocytes in several structures
Epineurial Vessel: Acid phosphatase stained connective tissue inside fibril layer
Perineurium around small nerve fascicle (Arrow): Histiocytic cells
Epineurial connective tissue: Scattered histiocytic cells

Acid phosphatase stain
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H&E stain
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Subperineurial Edema
Pale-stained region under Perineurium

Gomori trichrome stain
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Eosinophils: Within area of subperineurial edema

H&E stain
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Wallerian Degeneration

VvG stain
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Wallerian Degeneration: Early
Myelin sheath
Pale; Vesicular; Irregular (Arrow, Above)
Acid phosphatase stain (Below)
See:
Intramuscular nerve

Acid phosphatase stain
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Wallerian Degeneration: Intramuscular Nerve

VvG stain
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VvG stain
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Wallerian Degeneration: Early
Myelin sheath
Pale; Vesicular; Irregular (Arrow, Above)
Acid phosphatase stain (Below)

Acid phosphatase stain
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Axon Loss
Differential: Intrafasciclar
More loss of large (Green) axons in some regions
NCAM-stained Schwann cells: Empty (Red) without associated axons
Active: Punctate or Irregular neurfilament staining
NCAM(r).jpg)
Neurofilament (Green) + NCAM (Red)
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Axon Loss: Acute
Loss of large axons within pale areas (Myelin)
Punctate or Irregular neurofilament staining

Neurofilament stain
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Axon Loss: Acute
Regions of myelin abnormally express NCAM
NCAM stain
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MBP(r).jpg)
Neurofilament (Green) + MBP (Red)
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Axon Loss: Acute
Regions of myelin (MBP above or P0 below) have no axons
P0(r).jpg)
Neurofilament (Green) + P0 (Red)
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Eosinophilic Vasculitis: Muscle features

H&E stain
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Histiocytic Vasculitis
Vessel location: Perimysium
Perivascular cells: Mostly histiocytes

H&E stain
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Eosinophilic Vasculitis
Fibrinoid Necrosis: Inside fibril layer
Fibril layer: Irregular damaged
Surrounding vessel wall: Contains many histiocytes; Little other structure

VvG stain
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Acid phosphatase stain
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Histiocytic Vasculitis
Vessel location: Perimysium
Perivascular cells: Mostly histiocytes

Acid phosphatase stain
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CD4 stain
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Histiocytic Vasculitis
Vessel location: Perimysium
Perivascular cells: Often stain for CD4 & MxA; Few stain for CD8

MxA stain
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CD8 stain
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Eosinophilic Vasculitis: Myopathic features

H&E stain
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Muscle Fibers
Sizes: Moderately varied
Internal nuclei: Some fibers

VvG stain
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Acid phosphatase stain
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Muscle Fibers: Internal Architecture
Cytoplasmic aggregates
Acid phosphatase positive (Above)
Contain LC3 (Autophagy marker; Below)

LC3 stain
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MHC Class I stain
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Muscle fibers
MHC1 upregulation
Perimysial vessels
Irregular fragments without lumens (Below; Arrow)

MHC Class I stain
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C5b-9 deposition
Location: Connective tissue, Endomysial & Perimysial

C5b-9 stain
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CD163 stain
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Histiocytic markers
CD163 histiocytes: Scattered in endomysium & perimysium (Above)
HAM56: Unusual distribution on endomysium & around surface of muscle fibers (Below)

HAM56 stain
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Eosinophilic Vasculitis
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7/3/2023