We describe a patient with systemic lupus erythematosus (SLE) with demonstrated direct immunofluorescent staining of epidermal nuclei in a rim pattern, the second case of in vivo antinuclear antibody (ANA) producing a rim pattern in normal epidermis. A review of the literature showed that 258 cases have been reported of in vivo ANA deposition in epidermal nuclei from nonlesional skin. This phenomenon, which is most common in patients with mixed connective tissue disease, SLE, and scleroderma, correlates frequently with antibodies to extractable nuclear antigens. In vivo ANA have also been described in renal and pulmonary tissue from patients with SLE. In most cases, the pathogenic significance of the phenomenon is unknown.