Importance: Anti-type VII collagen autoantibodies are often detectable in patients with bullous systemic lupus erythematosus (BSLE). However, the timing of their appearance preceding the onset of disease is unknown to date.
Observations: We report the case of a 50-year-old woman with a history of SLE who was seen with vesicles and bullae around her lips, trunk, axillae, arms, and thighs. Histologic analysis and immunofluorescence and immunoblot studies confirmed the diagnosis of BSLE. Immunoblotting and enzyme-linked immunosorbent assay studies of the patient's serum obtained 3 months before the onset of BSLE showed the presence of anti-type VII collagen autoantibodies. Levels of anti-type VII collagen IgG increased after bullous lesions appeared. Within 1 month after initiating dapsone therapy and increasing the dosage of prednisone, skin lesions promptly resolved. One year after the onset of BSLE, the anti-type VII collagen IgG decreased below levels observed before the inception of the bullous lesions.
Conclusions and relevance: Anti-type VII collagen autoantibodies can precede the clinical appearance of BSLE. The subsequent increase and decrease in levels of circulating anti-type VII collagen autoantibodies, which mirrored skin disease activity, support a potential role in their initiation of disease.