Bilateral Periorbital Erythema and Swelling as an Initial Presentation of Systemic Lupus Erythematosus: A Rare Case

Abstract

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem involvement due to autoantibody production and immune complex deposition. While classical cutaneous manifestations, such as malar rash, are common, atypical presentations, like periorbital erythema and swelling, are rare and pose diagnostic challenges. Early recognition is crucial to prevent disease progression and complications. Case Presentation: A 16-year-old girl presented with a three-month history of intermittent bilateral periorbital swelling. Clinical examination revealed pallor and localized alopecia with no significant systemic abnormalities. Laboratory investigations showed pancytopenia with normal renal, hepatic, and thyroid functions and unremarkable urinalysis, chest X-ray, and ECG. Autoimmune markers were positive, with a strongly positive ANA titer of 1:1000 and significantly elevated anti-dsDNA antibodies of 380 IU/mL (reference range: 0-200 IU/mL). According to the 2019 EULAR/ACR classification criteria, a diagnosis of SLE was established. The patient was treated with pulse intravenous methylprednisolone (1g daily for three days), followed by oral prednisolone (1 mg/kg/day), in a tapering regimen and hydroxychloroquine at standard doses. She showed marked improvement, with resolution of periorbital swelling, recovery of pancytopenia, and hair regrowth. At two-month follow-up, she remained asymptomatic and continued hydroxychloroquine for maintenance therapy. Conclusion: This case underscores the importance of considering SLE in patients with atypical presentations, like periorbital erythema and pancytopenia. Early diagnosis based on clinical and serological findings, followed by appropriate therapy, can achieve remission and prevent complications. The case highlights the need for heightened clinical suspicion and multidisciplinary management in young patients. © 2025 Bentham Science Publishers.