Neuromuscular

Hydroxychloroquine Myopathy

Muscle
  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

Hydroxychloroquine Myopathy: Ultrastructure

Aggregates: Contain Heterogeneous Material
  Membrane-bound vacuoles
  Curvilinear bodies
  Degradation products
  Lipid & Lipofuscin structures
  Membranous whorls
  Mitochondria

From: R Schmidt

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

From: R Schmidt

From: R Schmidt

Complex Lipid Structures

From: R Schmidt

From: R Schmidt

Autophagic Degradation Products

From: J Chen

Membranous Whorls

From: J Chen

From: R Schmidt

Membrane-bound vacuoles
  ? Lysosomal

From: R Schmidt

From: R Schmidt

From: R Schmidt

Hydroxychloroquine Myopathy: Mitochondria

From: R Schmidt

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

12/8/2021