Ullrich Congenital Muscular Dystrophy

Muscle histology

H&E stain	H&E stain
Chronic inactive myopathy Muscle fiber size: Varied Endomysial connective tissue: Increased Internal nuclei Few immature muscle fibers (ATPase pH 4.3)

H&E stain

H&E stain

Gomori trichrome stain

ATPase pH 4.3 stain

Connective tissue molecules: Collagen VI & IV

Collagen IV

Collagen VI

Collagen VI + Collagen IV

Collagen Locations: Normal Collagen IV (Top left) Muscle basal lamina Vessels Collagen VI (Top right) Endomysial connective tissue Vessels Perimysium Present in most regions with Collagen IV (Left}

Collagen IV

Collagen VI

Collagen VI + Collagen IV

Collagen Locations: Ullrich Collagen IV (Top left) Muscle basal lamina Vessels Collagen VI (Top right) Endomysial connective tissue: Abnormal granular pattern Muscle basal lamina: Reduced amount Often does not co-localize with Collagen IV (Left}