Unmasking Vasculitis: An Atypical Presentation of Systemic Lupus Erythematosus with Fascial Rash

Abstract

Areej M Al Nemer, Nasser m. Alzoabi, Walaa Alshaikh Hasan, Mohammed Dhiya Alshubbar, Abdulmajeed Alharbi, Leena Abdulrahman Almuhaish, Moath Thamer Alkhouzaei and Jury Waleed

Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease with diverse clinical presentations. Rowell syndrome, a rare overlap of SLE and erythema multiforme (EM), presents diagnostic challenges due to its atypical features. It is characterized by EM-like lesions, positive antinuclear antibodies (ANA), and anti-Ro/SSA antibodies.

Case Presentation A 29-year-old Filipino female developed a four-week history of EM-like rashes, followed by fever, lymphadenopathy, vomiting, and epigastric pain. After ruling out infectious and drug-induced causes, immunological testing revealed ANA >1280 (speckled pattern), elevated anti-Smith and anti-SSA antibodies, and low C3. A skin biopsy confirmed leukocytoclastic vasculitis, consistent with cutaneous lupus. The patient met both the EULAR/ACR 2019 criteria for SLE and Zeitouni’s criteria for Rowell syndrome.

Discussion This case highlights the diagnostic challenges of Rowell syndrome, particularly when EM-like lesions precede systemic symptoms. A thorough workup led to early diagnosis and treatment with hydroxychloroquine and corticosteroids, resulting in clinical improvement.

Conclusion Rowell syndrome, though rare, should be considered in patients with persistent EM-like lesions and autoimmune markers. Greater awareness among clinicians can facilitate early recognition, timely management, and improved outcomes for patients with this rare subset of SLE.

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