We report a case of a 17-year-old Japanese boy with vesiculobullous systemic lupus erythematosus. He had a facial “butterfly” erythema and blistering eruption develop. Antinuclear, RNP, and Sm antibodies were positive. The American Rheumatism Association criteria for a diagnosis of systemic lupus erythematosus were met. A skin biopsy specimen showed papillary microabscesses of neutrophils and subepidermal blisters and deposits of IgG, IgA, IgM, and C3 by direct immunofluorescence. Indirect immunofluorescence on salt-split skin showed linear IgG, IgA, and C3 deposits on the dermal side. IgG and IgA autoantibodies in his serum reacted with the 290-kd proteins of type VII collagen from human dermal extracts and the recombinant fusion proteins from the noncollagenous domain of type VII collagen by Western immunoblot. To our knowledge, this is the first report of vesiculobullous systemic lupus erythematosus with circulating IgG and IgA autoantibodies to type VII collagen. (J Am Acad Dermatol 2002;47:S283-6.)