Abstract: An 18-year-old girl presented with blisters and erythema on the whole body in one year involving oral mucosal erosion. Physical examination revealed multiple erythemas, superficial erosion, and tense blisters and bullae on the face, chest, back, and extremities, with general symptoms of anemia alopecia, and arthralgia. Antinuclear antibodies (ANA)and autoantibodies to type VII collagen were positive. Biopsy showed slight hyperkeratosis of the epidermis, subepidermal fissure formation, and a large number of inflammatory cell infiltrations in the superficial dermis and around the collagen vessels. Direct immunofluorescence showed linear deposits of IgG at the basement membrane zone, the dermis, and the epidermis, and IIF was negative via the salt-split skin technique. Eventually, bullous systemic lupus erythematosus was diagnosed.

Key words: bullous systemic lupus erythematosus, blisters, bullae, autoimmunity