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Wang Yu, Yuan Liyan, Ji Suyun, Xie Jie, Xue Ruzeng, Gu Mei, Chen Yongfeng, Yang Bin. Department of Dermatology, Guangdong Provincial Dermatology Hospital, Guangdong Provincial Center for Skin Disease and STI Control, Guangzhou 510091, China Corresponding author: Yang Bin, Email: yangbin101@hotmail.com ��Abstract�� Objective To investigate the clinical and histopathological features of bullous systemic lupus erythematosus ��BSLE��, and to improve understanding of BSLE. Methods Lesions from 5 cases of BSLE were subjected to histopathological examination, and direct immunofluorescence ��DIF�� assay with mouse antibodies against human immunoglobulins ��IgG, IgM, IgA�� and complement component C3. The clinical and histopathological features, treatment and prognosis of BSLE were retrospectively analyzed. Results Skin lesions mostly occurred in sun-exposed areas such as the face and upper extremities, and included blisters or bullae arising on normal-appearing skin. Typical histopathological changes in these patients were subepidermal blisters, and infiltration of plenty of neutrophils within blisters and dermal papillae. Immunopathological study showed band-like deposition of immunoglobulins and/or complement along the basement membrane zone. Patients with BSLE responded well to dapsone, and glucocorticoids combined with immunosuppressive agents were also effective for the treatment of BSLE. Conclusions BSLE may be related to lupus nephritis activity. Glucocorticoids combined with dapsone or with immunosuppressive agents are effective for the treatment of BSLE.