title: [Hypocomplementaemic urticarial vasculitis with bullous lesions and pericardial involvement].
abstract: BACKGROUND
  Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease involving urticarial cutaneous vasculitis, hypocomplementaemia and systemic manifestations. Pericardial involvement occurs in very rare cases. We report a case of HUVS associated with specific pericarditis and bullous lesions.
  
  
  METHODS
  A 63-year-old woman consulted for chronic urticaria that had appeared ten months earlier. Her skin lesions were associated with weight loss of 10 kg, deterioration of respiratory function and abdominal pain. Leukocytoclastic vasculitis was seen in the skin biopsy sample. Hypocomplementaemia and anti C1q antibodies were present and a diagnosis of HUVS was made. During hospitalisation, extensive compressive pericardial effusion was identified, and histological examination of the biopsy revealed specific pericardial lymphocytic vasculitis. During follow-up, four episodes of infectious pneumonitis were noted. Bullous skin lesions were also observed.
  
  
  CONCLUSIONS
  HUVS is a disease caused by an antibody against C1q complement responsible for urticarial lesions and vasculitis antibodies. To our knowledge, there have been only five reports in the literature of pericardial injury associated with HUVS. In our case, histological examination of the pericardium demonstrated lymphocytic vasculitis.
authors.affiliation: Service de dermatologie, centre hospitalier régional d'Orléans, 1, rue Porte-Madeleine BP 2439, 45032 Orléans cedex 1, France; Service d'anatomopathologie, centre hospitalier régional d'Orléans, 14, avenue de l'Hôpital BP 86709, 45067 Orléans cedex 2, France. Electronic address: thibault.kervarrec@etu.univ-tours.fr.
authors.forename: T
authors.initials: T
authors.lastname: Kervarrec
authors.affiliation: Service de dermatologie, centre hospitalier régional d'Orléans, 1, rue Porte-Madeleine BP 2439, 45032 Orléans cedex 1, France.
authors.forename: R
authors.initials: R
authors.lastname: Binois
authors.affiliation: Service d'anatomopathologie, centre hospitalier régional d'Orléans, 14, avenue de l'Hôpital BP 86709, 45067 Orléans cedex 2, France.
authors.forename: C
authors.initials: C
authors.lastname: Bléchet
authors.affiliation: Service de dermatologie, centre hospitalier régional d'Orléans, 1, rue Porte-Madeleine BP 2439, 45032 Orléans cedex 1, France.
authors.forename: É
authors.initials: É
authors.lastname: Estève
mesh_terms: D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications* / Q000276:immunology; D001706:Biopsy; D002196:Capillaries / Q000473:pathology; D002908:Chronic Disease; D015922:Complement C1q / Q000172:deficiency* / Q000276:immunology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008214:Lymphocytes / Q000276:immunology; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology; D010490:Pericardial Effusion / Q000209:etiology*; D010493:Pericarditis / Q000209:etiology*; D010496:Pericardium / Q000473:pathology; D011014:Pneumonia / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology*; D014581:Urticaria / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D015922:Complement C1q; D011241:Prednisone
keywords: Blister; Bulle; Histopathologie; Hypocomplementaemic urticarial vasculitis; McDuffie syndrome; Pathology; Pericarditis; Péricardite; Syndrome de Mac Duffie; Vascularite urticarienne hypocomplémentémique