Kimura Disease as a Rare Cause of Proptosis: A Case Report

Evelyn Li Min Tai,V Sha Kri Eh Dam,Fatihatul Munirah Amiruddin,Khairil Amir Sayuti,Irfan Mohamad,Faezahtul Arbaeyah Hussain,Adil Hussein

doi:10.21315/aos2021.16.2.15

Evelyn Li Min Tai, V Sha Kri Eh Dam + Show 5 more

Open Access

https://doi.org/10.21315/aos2021.16.2.15

Copy DOI

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.

Highlights

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause that mainly affects the head and neck region (Zhang & Jiao, 2019)
KD presents with painless subcutaneous swelling in the head and neck region and predominantly with lymph node involvement (Fouda et al, 2011)
Proptosis is described as abnormal protrusion of the eyeball that has varied aetiology, including neoplastic, vascular, traumatic, (a) metabolic, infective and inflammatory origins (George & Nanu, 2016)

Summary

INTRODUCTION

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause that mainly affects the head and neck region (Zhang & Jiao, 2019) It was first described in 1937 but became widely recognised as Kimura disease in 1948 after the definitive histologic description was published (Kim & Szeto, 1937; Kimura et al, 1948; Li et al, 1996). There was no history of prolonged cough, fever, loss of appetite or weight loss, and the patient denied tuberculosis contact; he was a chronic smoker He had experienced painless left post-auricular swelling for one year and right pre-auricular swelling for two weeks. Extraocular movements were full and Hertel exophthalmometry was equal (17 mm) bilaterally His vision remained 6/6 in both eyes, with normal optic nerve function

DISCUSSION

CONCLUSION