A Comprehensive Review of Kimura Disease

Purpose: Kimura disease (KD) is a rare, chronic inflammatory disorder that primarily affects the head and neck regions, often mimicking neoplastic conditions. This study aims to provide a comprehensive review of KD, focusing on its clinical presentation, diagnostic challenges, optimal management strategies, and primary histopathologic differential diagnosis. Methods: A systematic review of literature was conducted using PubMed, Scopus, and Google Scholar databases. We analyzed case reports, retrospective studies, and clinical trials published in English. We extracted data on epidemiology, clinical presentation, laboratory findings, histologic features, current understanding of the pathogenesis, treatment, and prognosis. Results: KD predominantly affects young Asian males, presenting with painless subcutaneous masses, peripheral eosinophilia, and elevated serum IgE levels. Histopathology reveals lymphoid follicular hyperplasia with eosinophilic infiltration. Biopsy is required for diagnosis. The pathogenesis of KD is poorly understood, but recent studies have elucidated some potentially important mechanisms of the disease. Treatment options include systemic corticosteroids, surgical excision, radiotherapy, and cytotoxic therapies, with recurrence rates varying among modalities. Conclusion: KD remains a diagnostic challenge due to its overlapping features with a variety of neoplastic and non-neoplastic conditions. While corticosteroids offer temporary relief and can be useful in cases with renal involvement, surgical excision remains the most definitive treatment. Future research should focus on targeted therapies to improve long-term disease control and reduce recurrence.