title: A case report of meningeal Rosai-Dorfman disease associated with IgG4-related disease.
abstract: OBJECTIVE
  Rosai-Dorfman disease is a rare entity that has been described as lymphadenopathy in young patients. Extranodal forms of this disease have been previously observed. The etiology of Rosai-Dorfman disease remains unknown, relationships with the IgG4-related sclerotic disease have been detected. Herein, a rare case of Rosai-Dorfman disease with meningeal involvement and IgG4-related sclerotic disease is reported.
  
  
  METHODS
  A meningeal biopsy in a 35-year-old woman who had a 6-month history of intermittent headache was performed after MRI examination showing diffuse leptomeningeal enhancement without cerebral parenchymal involvement.
  
  
  RESULTS
  A mixed infiltration of lymphocytes, plasma cells, and histiocytes exhibiting emperipolesis was identified. The stroma was fibrous. Immunohistochemical analysis revealed a high number of IgG4-positive plasma cells and a rate of IgG4/IgG-positive plasma cells higher than 50%.
  
  
  CONCLUSIONS
  The pathological results in this patient with meningeal infiltration are suggestive of Rosai-Dorman disease associated with IgG4-related disease. This observation further confirms the link between these two entities.
authors: Arnault
authors: A
authors: Tauziede-Espariat
authors: Marc
authors: M
authors: Polivka
authors: Henri
authors: H
authors: Chabriat
authors: Sahahrazed
authors: S
authors: Bouazza
authors: Damien
authors: D
authors: Sene
authors: Homa
authors: H
authors: Adle-Biassette
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology*; D001706:Biopsy; D003937:Diagnosis, Differential; D057687:Emperipolesis / Q000276:immunology; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008578:Meninges / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G