Background Systemic lupus erythematosus (SLE) is a heterogeneous chronic rheumatic autoimmune disease with a range of clinical manifestations, characterized by impaired activation of cellular and humoral links of immunity, uncontrolled hyperproduction of autoantibodies of a wide spectrum to nuclear antigens and the formation of immune complexes that cause immuno-inflammatory damage to tissues and organs. Vasculitis in connective tissue disease (CTD) is quite rare, it is reported in approximately 10% of patients with CTD, SLE shows the highest association rate.

Purpose To report a case of a patient with SLE with ulcerative necrotic vasculitis of the upper and lower extremities with the formation of dry necrosis in combination with lupus nephritis (LN).

Methods A 26-year-old female patient with SLE for 1 year was diagnosed based on ulcerative necrotic vasculitis of the upper and lower extremities with the formation of dry necrosis, Class V LN (proteinuria, erythrocyturia, cylindruria), adhesive pericarditis, immunological disorders (highly positive antibodies to Sm+, anti-Ro/SS-A+), ANF hep-2 positivity.

Results An anamnesis of the disease, physical, laboratory and instrumental examination and treatment tactics are presented in table 1.

Conclusions Necrotizing vasculitis is always secondary to SLE and it is necessary to exclude antiphospholipid syndrome. Vessels of any size may be involved, but mainly medium vessels vasculitis in SLE is reported. The most common clinical manifestations are ischemic lesion, ulcers, gangrene etc. It may be an early warning sign for systemic involvement. The combination of glucocorticoids, hydroxychloroquine, cytostatic (azathioprine), anti-B-cell therapy (rituximab) is highly effective. No recurrence of vasculitis was detected within 7 months after correction of therapy.