Neuromuscular

Hydroxychloroquine Myopathy

Aggregates
Lipofuscin-like material
Lysosomal accumulations
Ultrastructure
  Autophagic material
  Curvilinear bodies
  Membranous whorls
Vacuoles

Nerve


H&E stain
Muscle fibers
  Vacuoles
    May be Acid phosphatase positive
    May contain: Lipofuscin-like granules; Granular material
  Internal nuclei
  LAMP2 granules

H&E stain


VvG stain

VvG stain

Lipofuscin-like material: In vacuoles & cytoplasm

Congo red stain

Congo red stain

Congo red stain


Esterase stain

Esterase stain

Lysosomal material in muscle fibers: Acid phosphatase & LAMP-2 staining

Acid phosphatase stain

Acid phosphatase stain

Acid phosphatase stain

LAMP-2 stained organelles

LAMP2 stain

Aggregates in muscle fibers

Toluidine blue stain

Curvilinear bodies (Arrow)
  Lysosome-derived cytoplasmic inclusions
  Also occur in ceroid lipofuscinosis

From: J Chen

Curvilinear bodies & Complex lipid structures with globular elements

From: J Chen

Autophagic Degradation Products

From: J Chen

Membranous Whorls

From: J Chen

Neuropathy: Hydroxychloroqine-associated

Toluidine blue stain
Neuropathy
  Large axons
    Loss
    Thinly myelinated
  Regenerating axon clusters
  Endoneurial vessels: Thick walls

Toluidine blue stain

Return to Hydroxychloroquine myopathy
Return to Muscle biopsies

12/27/2018