Abstract: Objective: To investigate the clinical, diagnostic and therapeutic characteristics of pediatric bullous systemic lupus erythematosus (pediatric BSLE). Methods: The data of three cases with pediatric BSLE in our hospital from 1998 to 2021 and 15 cases reported in literature were analyzed. Results: Of 18 cases, the average age was 11.22 years old，and the male to female ratio was 16∶2. The bullous was the initial symptom in 10 cases and the bullous preceded lupus nephritis in 12 cases. 12 cases had fever, 9 had oral ulcer and 14 had anemia. The ANA was positive in all patients and 13 patients had simultaneously three or more types of auto-antibodies. The pathological features of skin in 16 cases were the deposition of complement and immunoglobulin in the basement membrane. 10 cases had massive urine protein (≥1.5 g/24 hour). Renal pathological data were obtained in 16 cases, 7 were class V lupus nephritis, 6 were class IV lupus nephritis and 3 were class III lupus nephritis. Glucocorticoid, dapsone and immunosuppressive agent were commonly used therapy. Immunosuppressive agent can reduce the recurrence of disease. Rituximab and Belimumab can be used for refractory patients. Conclusion: Children with bullous systemic lupus erythematosus are characterized by multiple systemic involvement, multiple positive autoantibodies and lupus nephritis. Active immunosuppressive therapy can improve the prognosis, and anti-B cell therapy can be used as an option of therapy.

Key words: bullous lupus, systemic lupus erythematosus, clinical features, pediatric