title: [An atypical form of Kimura's disease].
abstract: BACKGROUND
  Kimura's disease is a rare benign chronic inflammatory disease of unknown aetiology.
  
  
  METHODS
  An 18-year-old atopic Brazilian patient consulted for recurrent facial eczema that improved slightly under topical corticosteroids. He presented a large infiltrated and eczematous subcutaneous plaque on the right supraorbital area, together with eyelid oedema. This was associated with complete right eyebrow alopecia, oedema of the upper lip and infiltrated facial papules. The tests showed hypereosinophilia (4000/mm(3)), a high IgE level (3786 kIU/L) and proteinuria (0.3g/24h). Histological examination revealed a lymphocytic eosinophil-rich inflammatory infiltrate in the superficial and deep dermis, with some lymphoid follicles in depth and proliferation of post-capillary venules. All of these elements led to the diagnosis of Kimura's disease.
  
  
  CONCLUSIONS
  We report an unusual clinical form of Kimura's disease in a Brazilian patient. This disease has been classically described in young Asian men. In our case, a particularly large infiltrated and oedematous subcutaneous plaque was noted. In fact, the more common forms appear as subcutaneous nodules on the head and neck, which may be associated with locoregional adenopathy, involvement of the salivary glands, hypereosinophilia and raised serum IgE. In our case, the skin lesions, characterized by a large infiltrated and oedematous subcutaneous plaque, were unusual and could evoke IgG4-related disease.
  
  
  CONCLUSIONS
  We report a case of Kimura's disease with an atypical presentation. The diagnosis was established by comparing certain histopathological features, and the possibility of IgG4-related disease was discussed.
authors.affiliation: Service de dermatologie, faculté de médecine, université de Caen Basse-Normandie, CHU de Caen, avenue Côte-de-Nacre, 14000 Caen, France. Electronic address: mariannechopinaud@hotmail.com.
authors.forename: M
authors.initials: M
authors.lastname: Chopinaud
authors.forename: C
authors.initials: C
authors.lastname: Morice
authors.forename: F
authors.initials: F
authors.lastname: Comoz
authors.forename: A
authors.initials: A
authors.lastname: Petit
authors.forename: M
authors.initials: M
authors.lastname: Battistella
authors.forename: A
authors.initials: A
authors.lastname: Stefan
authors.forename: L
authors.initials: L
authors.lastname: Verneuil
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D001938:Brazil / Q000208:ethnology; D016572:Cyclosporine / Q000627:therapeutic use; D020405:Dermis / Q000473:pathology; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011507:Proteinuria / Q000209:etiology; D016176:T-Lymphocyte Subsets / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D007073:Immunoglobulin E; D016572:Cyclosporine
keywords: Adenopathy; Adénopathies; Hyper-IgG4; Hypereosinophilia; Hyperéosinophilie; IgG4-related disease; Kimura's disease; Maladie de Kimura; Nodules sous-cutanés; Subcutaneous nodules