Kimura disease is a rare benign chronic inflammatory disorder of the soft tissue with predominant occurrences in young Asian male adults. However, there is limited information of Kimura disease in Southeast Asia. Hence, the clinical characteristics and outcomes of Kimura disease in Malaysia was investigated. Published Kimura disease cases from Malaysia were comprehensively searched in PubMed and Google Scholar up to December 2020 using the keywords “Kimura Disease” and “Malaysia”. Twenty-three papers were identified for review and case series of seven Kimura disease patients from a hospital in Malaysia were descriptively analyzed. A total of 60 cases were obtained from both sources. Eighty-seven percent were men with a male:female ratio of 6.5:1 and majority of the cases were Malays (77.1%). Median age of onset was 22.0 (IQR 12.5-31.5) years, while the median duration before diagnosis of Kimura disease was 2.0 (IQR 0.8-5.0) years. Head and neck region (95%) were the most frequently involved anatomical site. Peripheral eosinophilia was detected in 88.9% of the cases at presentation and renal involvement was observed in four (22.2%) patients. Surgery (57.5%) was the commonest first choice of treatment, followed by a combination of surgery and steroid (29.8%), steroid alone (10.6%), and a combination of steroid and immunosuppressive agent (2.1%). Local recurrences were observed in 28.6% of the cases. In conclusion, a much younger age of disease onset was found among Malaysians. A high recurrence rate of one in every four patients was observed, indicating the need for further evaluations of treatment strategies.
Kimura Disease

ABSTRACT Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.
Kimura Disease ; Exophthalmos