title: Fulminant Wegener's granulomatosis: a case report.
abstract: BACKGROUND
  Granulomatosis Wegener is anti-neutrophil cytoplasmic antibodies (ANCAs)-associated systemic vasculitis of unknown etiology. It is manifested as granulomatous necrotizing inflammation of the upper and lower parts of the respiratory tract, glomerulonephritis and systemic vasculitis involving most frequently the skin and oral mucous membrane. Sera markers of this disease are c-ANCA and pANCA.
  
  
  METHODS
  We presented a female patient aged 52 years with purpuric spots that had appeared on the lower legs ten months before admission to our hospital. The disease ran an aggressive course, and a month before admission hemorrhagic bullae, skin ulcers, hoarseness, dyspnea, generalized arthralgia, fatigue and fever had rapidly developed. Histopathological examination of a skin sample revealed necrotizing vasculitis, so that sera markers concentrations were elevated (c-ANCA, p-ANCA). There was a perforation of the nasal septum found on rhinoscopy. During hospitalization acute abdominal pain occurred, a possible tumor in the small intestine and possible granulomas in the liver were seen by multislice computed tomography (MSCT) examination, with normal findings on the lungs and kidneys. The treatment started with methylprednisolone: 500 mg/d i.v. infusion for consecutive 3 days, then 60 mg/d. On exploratory laparotomy small bowel perforation and diffuse peritonitis were found. Unstable in the postoperative period, the patient died on the day 12 of hospitalization.
  
  
  CONCLUSIONS
  The reported patient was with fulminant Wegener's granulomatosis, dominantly with skin changes and with gastrointestinal manifestation. This case accents the need for rapid systemic clinical evaluation in a severely ill patient with unclear diagnosis.
authors.affiliation: Clinic for Dermatovenerology, Military Medical Academy, Belgrade, Serbia. miroslavdinic@hotmail.com
authors.forename: Miroslav Z
authors.initials: MZ
authors.lastname: Dinić
authors.forename: Lidija Kandolf
authors.initials: LK
authors.lastname: Sekulović
authors.forename: Lidija
authors.initials: L
authors.lastname: Zolotarevski
authors.forename: Rados D
authors.initials: RD
authors.lastname: Zecević
mesh_terms: D000208:Acute Disease; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007155:Immunologic Factors / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007260:Infusion Pumps; D007416:Intestinal Perforation / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D007421:Intestine, Small / Q000473:pathology*; D007813:Laparotomy; D007866:Leg / Q000473:pathology; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000209:etiology; D061270:Nasal Septal Perforation / Q000175:diagnosis / Q000209:etiology; D010538:Peritonitis / Q000150:complications / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D012883:Skin Ulcer / Q000209:etiology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D008775:Methylprednisolone