Neuromuscular

FASCIITIS

Immune
Eosinophilic
  Toxic

Fasciitis: Immune


H&E stain

H&E stain
Muscle & Connective tissue pathology
  • Lesions
    • Location: Perimysium & Epimysium (not shown)
  • Perimysium
    • Fragmented & Pale
    • Cellular & Widened in some regions
  • Neighboring muscle fibers: Relatively unaffected.

Acid phosphatase stain

MHC Class I
  Perimysium: Mild staining
  Muscle fibers: Most are normal; Mild increase in some muscle fibers near perimysium
  Capillaries, endomysial: Normal staining

MHC Class I stain

C5b-9 Deposition
  Perimysium: Moderate staining
  Endomysium: Diffuse deposition

MHC Class I stain

Fasciitis: Perimysial Histiocytic Inflammation


H&E stain

Acid phosphatase stain
Inflammation
  • Cells in regions of perimysial damage
    • Large nuclei & Pink cytoplasm
    • Histiocytic (Acid phosphatase & Esterase positive)
  • Occasional regions of undamaged perimysium contain lymphocyte infiltrates (Right).

H&E stain

H&E stain

Fasciitis: MRI

Fasciitis: MRI

Multiple regions of increased T2 signal in leg muscles (L > R)

Fasciitis: Eosinophilic (Shulman syndrome) 1


Fasciitis Perimysium: Fragmentation; Condensation

H&E stain

Fasciitis Perimysium: Palor

H&E stain

Fasciitis Perimysium: Eosinophils; Histiocytes

H&E stain

Fasciitis Perimysium: Histiocytes

Acid phosphatase stain

Fasciitis Perimysium: Alkaline phosphatase positive

Alkaline phosphatase stain

Fasciitis: Toxic


H&E stain
Perimysiun: Damaged structure & cellularity with little involvement of adjacent muscle fibers

VvG stain


Acid phosphatase stain
Histiocytic cells: In perimysial connective tissue

Esterase stain

Esterase stain


H&E stain

H&E stain

H&E stain
Perimysial connective tissue pathology: Fragmented & Hypercontracted

VvG stain

Congo red stain

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References 1. Rheumatology (Oxford) 2023;62:2005-2014, Autoimmun Rev 2014;13:379-82, Rheumatology (Oxford) 2023;62:2005-2014

2/5/2024