Neuromuscular

IPEX: Muscle pathology


Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) syndrome

  Forkhead Box P3 (FOXP3) ; Chromosome Xp11.23: Recessive

Muscle Pathology



Congo red stain
Perivascular cells: Around small perimysial vessels

VvG stain

H&E stain
Perivascular cells: Around small perimysial vessels

H&E stain

Perivascular cells: Some are histiocytic
Perimysial cells cells: Large; Histiocytic

Acid Phosphatase stain

Perimysial arteries & Veins
  Reduced staining for fibrils in vessel walls
  No inflammatory cells

VvG stain


VvG stain
Perimysium
  Structure: Mildly irregular
  Cells: Histiocytic; Some are perivascular

Acid Phosphatase stain


NADH stain
Muscle fibers
  Internal architecture: Irregular or coarse

NADH stain


Congo red stain
Muscle fibers
  Nuclei: Large
  Cytoplasm: Basophilic

Gomori trichrome stain

Muscle fibers
  Type 2C fibers: Scattered

ATPse pH 4.3 stain

Muscle fibers
  No fiber type grouping

ATPse pH 9.4 stain

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5/26/2016