Kimura Disease: A Rare Eosinophilic Inflammatory Disorder – Pathogenesis, Diagnosis, and Emerging Therapies

Abstract

Kimura disease is a rare, chronic eosinophilic inflammatory disorder, predominantly affecting young Asian males, and typically presents with painless masses in the head and neck region. Its exact etiology remains unclear, but immune dysregulation, including heightened IgE levels and eosinophilic infiltration, is central to its pathogenesis. Although the disease is benign, it can cause significant morbidity through recurrent lesions and potential systemic complications, such as renal involvement and hypercoagulability. Diagnosis relies on clinical presentation, laboratory findings (eosinophilia and elevated serum IgE), imaging studies (such as MRI and ultrasound), and histopathology, with characteristic features including eosinophilic infiltration, follicular hyperplasia, and vascular proliferation. Treatment primarily involves immunosuppressive therapies such as corticosteroids, leflunomide, and cyclosporine, with surgical excision serving as the main approach for localized disease. Recurrence is common, and adjunctive treatments like radiotherapy, IV immunoglobulin, and novel strategies such as 3D printing in radiation delivery offer potential for managing persistent cases. Despite the rarity of Kimura disease, understanding its pathophysiology and treatment options is crucial for improving patient outcomes and minimizing complications.