title: Adult-Onset Asthma and Periocular Xanthogranulomas Associated with Systemic IgG4-Related Disease.
abstract: OBJECTIVE
  The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).
  
  
  METHODS
  A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD.
  
  
  CONCLUSIONS
  AAPOX can be associated with systemic IgG4-RD.
authors.affiliation: Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.
authors.forename: Christopher K H
authors.initials: CK
authors.lastname: Burris
authors.affiliation: Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.
authors.forename: Maria E
authors.initials: ME
authors.lastname: Rodriguez
authors.affiliation: Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.
authors.forename: Meisha L
authors.initials: ML
authors.lastname: Raven
authors.affiliation: Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.
authors.forename: Cat N
authors.initials: CN
authors.lastname: Burkat
authors.affiliation: Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Albert
keywords: Adult-Onset Asthma; IgG4-RD, orbital; Periocular Xanthogranulomas; maxillary sinus; xanthelasma