Fukuyama CMD

Fukuyama Congenital Muscular Dystrophy

Childhood Pathology

H&E stain
From: I Nishino

H&E stain
From: I Nishino

Muscle fibers
Sizes: Varied
Small fibers: Scattered
Basophilic fibers: Scattered
Internal nuclei: Common
Endomysial connective tissue: Increased
Perimysium: May be replaced by fat

Gomori trichrome stain

Muscle fiber size: Varied
Endomysial connective tissue: Increased

Acid phosphatase stain

Positive staining cells associated with necrotic fiber

NADH stain

Scattered small immature muscle fibers with
coarse internal architecture

ATPase pH 11 stain

Adult Pathology

H&E stain

Muscle fibers
Sizes: Varied
Small & Hypertrophic fibers: Scattered
Basophilic fibers: Scattered
Internal nuclei: Common
Necrotic fibers: Few; Scattered
Partially fused (Split) fibers
Endomysial connective tissue: Increased
Perimysium: May be replaced by fat

H&E stain

H&E stain

Muscle fibers
Small & Hypertrophic fibers: Scattered
Internal nuclei: Common
Necrotic & Regenerating fibers: Few; Scattered
Partially fused (Split) fibers
Endomysial connective tissue: Increased

H&E stain

VvG stain

Muscle fibers
Partially fused (Split) fibers
Non-fused small fibers closely neighboring other fibers

Gomori trichrome stain

Muscle fibers
Partially fused (Split) fibers
Non-fused small fibers closely neighboring other fibers
Endomysial connective tissue: Increased

NADH stain

Muscle fibers
Pale-stained fibers: Linear pattern; Pale centers

ATPase pH 4.3 stain

Muscle fiber types: Varied patterns in different fascicles
Type 1 fiber predominance
Type 1 fibers smaller than type 2

ATPase pH 4.3 stain

ATPase pH 4.3 stain

Acid phosphatase stain

Muscle fibers
Histiocytic cells replace necrotic fibers

Alkaline phosphatase stain

Endomysial Capillaries
May be mis-oriented: Longitudinal orientation
Mildly increased numbers stained

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3/21/2023