Kimura Disease: A Detailed Analysis of Clinical and Radiological Manifestations in a Retrospective Case Series

Abstract

Background: Kimura disease (KD) is a rare chronic inflammatory disease that affects mainly young Asian men and is characterized by painless subcutaneous masses, lymphadenopathy, and elevated serum IgE levels. Despite its benign nature, KD poses a diagnostic and therapeutic challenge due to its rarity and clinical variability.

Objective: This study aimed to provide a comprehensive analysis of the clinical and radiological features of KD in a retrospective case series, to assess treatment outcomes, and to discuss the implications for diagnosis and management.

Methods: We retrospectively analyzed four histologically confirmed cases of KD admitted to Zhejiang Provincial People's Hospital from January 2018 to October 2023. Clinical and radiological data were retrospectively analyzed, and imaging findings were analyzed by two neuroradiologists to determine lesion characteristics and contrast enhancement patterns.

Results: Our findings showed that the patients were predominantly male, with a mean age of 43 years and an age range of 13-71 years. All patients presented with painless subcutaneous masses and three of them had peripheral blood eosinophilia and elevated serum IgE levels. Radiographically, the lesions were predominantly ill-defined with heterogeneous enhancement, accompanied by subcutaneous fat atrophy. Complete surgical excision and oral corticosteroids were effective treatments, and no recurrence was noted during follow-up.

Conclusion: KD should be considered in the differential diagnosis of painless subcutaneous masses in the head and neck region, especially in the presence of eosinophilia and elevated IgE levels. Our findings contribute to the understanding of KD's clinical and radiological spectrum and highlight the need for long-term follow-up due to the risk of recurrence.

Keywords: CT; Kimura disease; MRI; computed tomography; eosinophilia; magnetic resonance imaging.

Figure 1

Kimura’s disease in a 13-year-old man with a slowly growing mass for 36 months. (a and b) Axial contrast-enhanced computed tomography (CT) shows multiple ill-defined masses located in the bilateral parotid region and submandibular gland; lesions show a heterogeneous moderate enhancement and diffuse atrophy of subcutaneous fat (arrow). (c–e) The mass is hypo-signal on T1-weighted images, and hyper-signal on T2-weighted and T2-weighted fat suppression (T2-FS) images (arrow). (f) Sagittal T2-FS showing multiple swollen lymph nodes in the neck (circle).

Figure 2

Kimura’s disease in a 42-year-old man with two painless masses for 1 month. (a and b) Axial contrast-enhanced computed tomography (CT) shows two well-defined masses located in the bilateral retroauricular region; lesions show a homogeneous moderate enhancement (arrow). (c) Photomicrograph shows hyperplasia of lymphoid follicular tissue in lymph nodes with germinal center formation, intense eosinophilic infiltration, and eosinophil micro-abscess.

Figure 3

Kimura’s disease in a 46-year-old man with a single painless mass in the left cheek for 10 months. (a–d) The mass is hyper-signal on T2-weighted and T2-weighted fat suppression (T2-FS) images with strips of small low-signal vascular shadows within it; lesions show a homogeneous significant enhancement (arrow).

Figure 4

Kimura’s disease in a 71-year-old man with two painless masses for 180 months. (a and b) Axial contrast-enhanced computed tomography (CT) shows two ill-defined masses located in the left preauricular and retroauricular region with atrophy of the subcutaneous fat; lesions show a homogeneous slight enhancement (arrow). (c) Photomicrograph shows multifocal infiltration of lymphocytes and eosinophils in the dermis.