Background: We report 7 patients with a distinct and unusual eruption consisting of a neutrophilic dermatosis in conjunction with lupus erythematosus (LE). The significance of these findings and their relevance to LE are discussed. Objective: We aimed to evaluate the significance of eruptive neutrophilic dermatosis in conjunction with LE. Methods: Seven original cases were collected during 10 years at a tertiary referral center, and were reviewed by a single board-certified dermatopathologist. All patient demographics were tabulated and analyzed. Eleven articles reporting 15 similar cases were obtained from a literature review. Results: Of a total of 7 adult patients, 14% (1 of 7) had a history of LE, whereas 86% (6 of 7) exhibited synchronous initial presentation of neutrophilic dermatosis with LE. Of note, 100% (7 of 7) of the patients exhibited cutaneous lesions on sun-exposed sites. Limitations: Only 7 of 400,000 cases showed this phenomenon, giving rise to the idea this may be just by chance. Conclusions: Our data and literature review suggest the existence of a neutrophilic dermatosis distinct from conventional Sweet syndrome that may herald conventional signs and symptoms or represent an initial presentation of cutaneous LE. This neutrophilic dermatosis may share a similar pathogenic mechanism related to ultraviolet exposure. © 2012 by the American Academy of Dermatology, Inc.