ANCA associated vasculitis allergica cutis (VAC) and mild proliferative necrotizing glomerulonephritis

We report here the case of a 69-year-old male with vasculitis allergica cutis (VAC) accompanied by a nephrotic syndrome associated with serum perinuclear-staining anti-neutrophil cytoplasmic antibody (P-ANCA). Renal biopsy specimens showed mild proliferative glomerulonephritis with crescentic and necrotizing lesion in the glomeruli, while lesional skin biopsy specimens showed leukocytoclastic vasculitis in the deep dermis. Pauci-immune deposits were evident (deposits of immunoglobulins and complements were absent) by immunofluorescence microscopy in both tissues. The appearance of weakly positive P-ANCA associated with skin lesions and renal dysfunction occurred twice in this patient. Following corticosteroid administration, renal dysfunction was remarkably diminished and proteinuria decreased. P-ANCA titer were decreased, reaching the normal level. ANCA associated glomerulonephritis complicated by VAC has not been reported previously. In the present case, the vasculitis cutis and glomerulonephritis is assumed to be associated with the appearance of weakly positive P-ANCA.