title: Recurrent lipoatrophic panniculitis of children.
abstract: BACKGROUND
  Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains.
  
  
  OBJECTIVE
  To depict the profile of children with recurrent idiopathic panniculitis.
  
  
  METHODS
  Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis.
  
  
  RESULTS
  Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed. In two patients, extensive involvement led to loss of the cutaneous fatty tissue. Laboratory abnormalities included increased acute phase reactants, leukocytosis with mild neutrophilia, microcytic anaemia and elevated liver enzymes. Histopathology showed lobar panniculitis without vasculitis and with a mixed infiltrate, composed of neutrophils, mononuclear cells, lymphocytes, macrophages and myeloid cells. Neutrophils and myeloid cells were more prominent in early lesions, whereas macrophages predominated in late stages, leading to lipophagia and lipoatrophy. Immunohistochemistry showed positive staining for myeloperoxidase around the necrotic adipocytes in early stages and CD68/PGM1 macrophages in late stages. Intense STAT1 staining was observed in the inflammatory infiltrate. All patients improved with methotrexate and corticosteroids.
  
  
  CONCLUSIONS
  We present five cases of lobar panniculitis and lipoatrophy in childhood. The clinico-pathologic presentation shares features with other autoinflammatory diseases.
authors: Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain.
authors: A
authors: A
authors: Torrelo
authors: Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain.
authors: L
authors: L
authors: Noguera-Morel
authors: Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain.
authors: A
authors: A
authors: Hernández-Martín
authors: Department of Rheumatology, Hospital del Niño Jesús, Madrid, Spain.
authors: D
authors: D
authors: Clemente
authors: Department of Dermatology, Hospital El Bierzo, León, Spain.
authors: J M
authors: JM
authors: Barja
authors: Department of Microbiology, Hospital Gregorio Marañón, Madrid, Spain.
authors: L
authors: L
authors: Buzón
authors: Department of Pathology, Hospital del Niño Jesús, Madrid, Spain.
authors: D
authors: D
authors: Azorín
authors: Translational Autoinflammatory Disease Section, NIAMS, NIH, Bethesda, USA.
authors: A A
authors: AA
authors: de Jesús
authors: Department of Rheumatology, Hospital del Niño Jesús, Madrid, Spain.
authors: J C
authors: JC
authors: López-Robledillo
authors: Paediatric Histopathology Department, Birmingham Children's Hospital, Birmingham, UK.
authors: I
authors: I
authors: Colmenero
authors: Dermatohistopathologisches Gemeinschaftslabor, Friedrichshafen, Germany.
authors: H
authors: H
authors: Kutzner
authors: Translational Autoinflammatory Disease Section, NIAMS, NIH, Bethesda, USA.
authors: R
authors: R
authors: Goldbach-Mansky
authors: Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain.
authors: L
authors: L
authors: Requena
mesh_terms: D000209:Acute-Phase Proteins / Q000378:metabolism; D017667:Adipocytes / Q000737:chemistry; D000273:Adipose Tissue / Q000737:chemistry* / Q000473:pathology*; D000740:Anemia / Q000209:etiology; D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D001284:Atrophy / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007964:Leukocytosis / Q000097:blood; D008214:Lymphocytes; D008264:Macrophages / Q000737:chemistry; D008297:Male; D009504:Neutrophils; D015434:Panniculitis / Q000097:blood* / Q000150:complications / Q000473:pathology*; D009195:Peroxidase / Q000032:analysis; D012008:Recurrence; D050794:STAT1 Transcription Factor / Q000032:analysis; D000097382:CD68 Molecule
chemical_list: D000209:Acute-Phase Proteins; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D009195:Peroxidase; D050794:STAT1 Transcription Factor; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C494079:STAT1 protein, human