Abstract: A 29-year-old female presented with hair loss for two years, and edema with rash for more than one month. Blood routine examination is normal. Urine routine test showed that RBC was 6-10/HP; 24 hours urinary protein was 4.29g; quantitative blood albumin was 19.9 g/L. ?Antibody?detection?showed ANA1:10000 (S), anti-dsDNA(+), anti-Sm (+), anti-nRNP (+), IgG 21.8 g/L, C3:0.292 g/L, C4:0.037 g/L. Histopathological examination showed subepidermal blister, neutrophil cell infiltration in dermal papilla, lymphocytic infiltrates in the perivascular of middle level dermis. Direct immunohistochemistry revealed that IgG was positive in the basement membrane zone. The diagnosis of lupus?nephritis and bullous systemic lupus erythematosus was made. After the patient was treated with methylprednisolone, 1mg/kg, once a day combined with cyclophosphamide and hydroxychloroquine for 1 week, the lupus nephritis has improved, but herpes got worse. Then the patient was given methylprednisolone impulse therapy (250mg for 3 days) and minocycline hydrochloride. The lesions were improved.

Key words: bullous systemic lupus erythematosus, lupus nephritis