title: Localized Wegener's granulomatosis.
abstract: BACKGROUND
  Wegener's granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease.
  
  
  OBJECTIVE
  Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG.
  
  
  METHODS
  The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied.
  
  
  RESULTS
  All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti-neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide.
  
  
  CONCLUSIONS
  The limited number of patients is counterbalanced by the rarity of the disease.
  
  
  CONCLUSIONS
  Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.
authors.affiliation: UO Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Dipartimento di Anestesiologia, Terapia Intensiva e Scienze Dermatologiche, Università degli Studi di Milano, Milan, Italy. angelovalerio.marzano@policlinico.mi.it
authors.forename: A V
authors.initials: AV
authors.lastname: Marzano
authors.forename: Y
authors.initials: Y
authors.lastname: Balice
authors.forename: M
authors.initials: M
authors.lastname: Papini
authors.forename: R
authors.initials: R
authors.lastname: Testa
authors.forename: E
authors.initials: E
authors.lastname: Berti
authors.forename: C
authors.initials: C
authors.lastname: Crosti
mesh_terms: D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed