A Comprehensive Review of Kimura Disease

Abstract

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.

Keywords: Eosinophilia; Head and neck pathology; Hematopathology; Kimura disease.

Fig. 1

41-year-old male (Marshal Islander/Asian/Pacific Islander) presented with a large, recurrent parotid mass eight years after initial surgical excision (a). The patient’s serum IgE level was 5783 IU/ml (normal range 6-495 IU). Magnetic resonance imaging revealed a large T1 isointense mass in the left parotid region (b). The mass is hyperintense on the T2-weighted image (c). An additional lesional focus is present in the postauricular region. The mass on punch biopsy (d) showed a deeply seated lesion composed of a mixed inflammatory infiltrate with prominent eosinophilia and indistinct margins. Eosinophilic vasculitis (e) and subcutaneous secondary follicles with surrounding eosinophils (f) were present

Fig. 2

Histopathologic features of Kimura disease in a lymph node. (a) Low power showing follicle lysis. (b) High power showing an eosinophilic abscess. (c) High power showing eosinophilic vasculitis. (d) High power showing an eosinophilic granuloma. (e) High power showing a Warthin-Finkeldy polykaryocyte. (f) IgE reticular staining in a germinal center

Fig. 3

Differential diagnosis of epithelioid hemangioma. (a) Low power showing a subcutaneous nodule. (b) Low power of vessels showing plump endothelial cells (epithelioid change). (c) High power of mixed inflammation with eosinophils and vascular hyperplasia. (d) High power of prominent plump endothelial cells. (e) ERG expression in the endothelial cells

Fig. 4

Differential diagnosis of nodal T follicular helper cell lymphoma. (a) Low power image of nodal T follicular helper cell lymphoma, NOS. (b) High power showing characteristic features of neoplastic T-cells with background eosinophils. (c) PD1 expression in the neoplastic T-cells

Fig. 5

Differential diagnosis of classic Hodgkin lymphoma. (a) Low power showing effacement of lymph node architecture. (b) Intermediate power showing Hodgkin-Reed-Sternberg cells and numerous eosinophils

Fig. 6

Differential diagnosis of Langerhans histiocytosis. (a) Low power showing effacement of lymph node architecture. (b) High power showing Langerhans cells with characteristic nuclear grooves and eosinophils