Annular leukocytoclastic vasculitis in a patient with PR3-antineutrophil cytoplasmic antibody-positive ulcerative coliti
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Annular leukocytoclastic vasculitis in a patient with PR3-antineutrophil cytoplasmic antibody-positive ulcerative colitis Annular leukocytoclastic vasculitis (ALV), which was firstly described by Degos and Guiliane [1], is a rare clinical variant of leukocytoclastic vasculitis. It can be accompanied by infections, drugs, and several systemic diseases such as ulcerative colitis (UC) [2-4]. Here, we report a patient with proteinase 3 cytoplasmic-anti-neutrophil cytoplasmic antibody (PR3-ANCA)-positive UC who developed ALV accompanied by pauci-immune glomerulonephritis and haemorrhages of the eyes and palate mucosa. A 28-year-old man with a 10-year history of biopsy-proven UC was referred to our hospital with an acute purpuric rash. He felt general discomfort and abdominal pain. On examination, we observed well-defined annular purpuric lesions on the trunk and proximal extremities. Moreover, there were haemorrhagic lesions in the eyes and palate mucosa (figure 1). There was no evidence of fever or oedema. Routine histology of two skin biopsies revealed small-vessel vasculitis (leukocytoclastic type) with endothelial swelling, extravasation of erythrocytes, and fibrinoid degeneration of the vessel wall, affecting the entire dermis. The cell
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infiltrates mainly consisted of neutrophil granulocytes. Direct immunofluorescence showed strong C3, C4, and fibrinogen deposits around the small vessels of the upper dermis. However, no specific immunoglobulin deposits were detected. Colonoscopy and biopsy confirmed diagnosis of active UC. Ophthalmologic split-lamp investigation revealed bilateral episcleritis. Significant laboratory results were as follows: calprotectin at 709.6 mg/kg (
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