Systemic vasculitis is a heterogeneous group of diseases characterized by infiltrates consisting of neutrophils infiltrated the walls of blood vessels of various caliber. The most common clinical manifestation is renal involvement, nervous system, lungs and skin. These disorders may be manifested by changes in the image of urine in the form of a nephritic syndrome, deterioration of renal function, haemoptysis and ulceration of the skin. Characteristic for systemic vasculitis is the presence of neutrophil cytoplasmic antibodies (ANCA), which binds to the specific antigens generate immune complexes, which then activates a cascade of inflammatory reactions and lead to damage of vascular walls. The treatment includes steroids and cyclophosphamide and therapeutic plasma exchange treatments, and after reaching remission therapy is continued by low-dose steroids and azathioprine. A case of a man with systemic vasculitis is presented, in whom symptoms were present in the form of nephritic syndrome and RPGN, and has developed sores on the skin of abdomen and lower limbs. After the use of prednisone at a dose of 1 mg/kg and pulses of cyclophosphamide, remission has been achieved, patient restored normal kidney function, and the massive skin lesions have healed.