Abstract
A 21-year-old woman, who had a one-year history of pancytopenia with histiocytic necrotizing lymphadenitis and hepatosplenomegaly, presented with a 5 day history of tense bullae, which were localized on the face. These clusters of tense bullae occurred on clinically normal skin, she did not have other skin lesions. A diagnosis of bullous systemic lupus erythematosus (BSLE) was established based on clinical, laboratory, histological, and immunological findings. The bullae showed good responses to dapsone (100mg, daily) and resolved within 10 days with-out scaring. Bullous lesions of SLE may be the first cutaneous manifestation in some patients with SLE and should be considered in the differential diagnosis of the other subepidermal bullous disorders.