MICROSCOPIC POLYANGIITIS - A SYSTEMIC VASCULITIS WITH A POSITIVE P-ANCA

A young girl presented with a purpuric rash on lower limbs, fever, eos inophilia, peripheral neuropathy and progressive renal insufficiency. She developed vesicles, purpuric macules and papules on the head, seve ral nodules on the palmar sides of hands and fingers, splinter haemorr hages, and a disfiguring, facial oedemal A renal biopsy specimen discl osed a focal and segmental necrotizing glomerulonephritis with crescen ts. Peripheral ANCA with antimyeloperoxidase specificity [P-ANCA (MPO) ] was positive and cytoplasmic ANCA with PR3 specificity was negative. Treatment with prednisone and cyclophosphamide was started with a goo d clinical response. stabilization of renal insufficiency and disappea rance of P-ANCA (MPO). Our case fulfils the diagnostic criteria for mi croscopic polyangiitis (microscopic polyarteritis, MPA), namely a segm ental necrotizing and crescentic glomerulonephritis associated with ex trarenal vasculitis involving small-sized vessels, without granulomas or asthma, This is a rare disease, which has a poor prognosis in the a bsence of aggressive therapy, and is infrequently reported in dermatol ogical journals.