Introduction. 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 p-ANCA. Case report. 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. Conclusion. 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.
Huge mesenteric fibromatosis presenting with intestinal perforation and acute diffuse peritonitis: a case report
