title: Retrospective Multicentric Study of 25 Kimura Disease Patients: Emphasis on Therapeutics and Shared Features with Cutaneous IgG4-Related Disease.
abstract: BACKGROUND
  Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce.
  
  
  OBJECTIVE
  Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD).
  
  
  METHODS
  Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD.
  
  
  RESULTS
  Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD.
  
  
  CONCLUSIONS
  Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.
authors.affiliation: Dx00E9;partement de Dermatologie, Hx00F4;pital Saint-Louis, AP-HP, Paris, France.
authors.forename: Diane
authors.initials: D
authors.lastname: Kottler
authors.forename: Stéphane
authors.initials: S
authors.lastname: Barète
authors.forename: Gaelle
authors.initials: G
authors.lastname: Quéreux
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ortonne
authors.forename: Lydia
authors.initials: L
authors.lastname: Deschamps
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
authors.forename: Béatrice
authors.initials: B
authors.lastname: Flageul
authors.forename: Béatrice
authors.initials: B
authors.lastname: Crickx
authors.forename: Anne
authors.initials: A
authors.lastname: Janin
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.forename: Maxime
authors.initials: M
authors.lastname: Battistella
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000276:immunology* / Q000473:pathology / Q000628:therapy*; D016572:Cyclosporine / Q000627:therapeutic use; D062109:Dermatologic Surgical Procedures; D004802:Eosinophilia / Q000209:etiology; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007074:Immunoglobulin G / Q000032:analysis*; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016898:Interferon-alpha / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000737:chemistry; D012008:Recurrence; D012189:Retrospective Studies; D013792:Thalidomide / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016898:Interferon-alpha; D007073:Immunoglobulin E; D013792:Thalidomide; D016572:Cyclosporine