title: Childhood macrophagic myofasciitis: A series from the Indian subcontinent.
abstract: Macrophagic myofasciitis (MMF) is a rare disorder, reported mainly in European adults, with occasional childhood cases. We report a series of 6 patients with pediatric MMF from the Indian subcontinent.
  
  
  
  Clinical details, creatine kinase levels, and results of electromyography are described for patients diagnosed with MMF. Fresh-frozen and formalin-fixed muscle biopsies were evaluated by hematoxylin-eosin staining, histochemistry, immunohistochemistry, and electron microscopy.
  
  
  
  Six of 2,218 muscle biopsies were diagnosed as MMF; patient charts were reviewed. The 6 patients were all children; all presented with hypotonia and/or motor delay. Mean age at diagnosis was 16.2 months. There were 4 boys and 2 girls. All had a history of hepatitis B vaccination. Histopathology revealed infiltration by sheets of large periodic acid-Schiff stain-positive histiocytes. Ultrastructural examination demonstrated needle-shaped crystals within histiocytes. One patient had a co-existent neuromuscular disorder, merosin-deficient congenital muscular dystrophy.
  
  
  
  MMF is a rare inflammatory myopathy that should be considered in the differential diagnosis of congenital myopathies in children. Muscle Nerve 56: 71-77, 2017.
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Aanchal
authors.initials: A
authors.lastname: Kakkar
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Madhu
authors.initials: M
authors.lastname: Rajeshwari
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Aasma
authors.initials: A
authors.lastname: Nalwa
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Vaishali
authors.initials: V
authors.lastname: Suri
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Chitra
authors.initials: C
authors.lastname: Sarkar
authors.affiliation: Department Pediatrics (Division of Child Neurology), All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Biswaroop
authors.initials: B
authors.lastname: Chakrabarty
authors.affiliation: Department Pediatrics (Division of Child Neurology), All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sheffali
authors.initials: S
authors.lastname: Gulati
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Mehar C
authors.initials: MC
authors.lastname: Sharma
mesh_terms: D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001706:Biopsy; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005208:Fasciitis / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D005260:Female; D017325:Hepatitis B Vaccines / Q000009:adverse effects; D006801:Humans; D007194:India / Q000453:epidemiology; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D008854:Microscopy, Electron; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009220:Myositis / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D017325:Hepatitis B Vaccines; C067980:CD68 antigen, human; D000097382:CD68 Molecule
keywords: aluminum; congenital myopathy; electron microscopy; hypotonia; mitochondrial myopathy; motor delay