Authors (including presenting author) :
Chow CH(1), Wong KF(1), Leung SK(1)
Affiliation :
(1) Department of Surgery, Tuen Mun Hospital
Introduction :
Kimura Disease is a rare chronic inflammatory soft tissue disorder of unknown etiology. It commonly presents as painless lymphadenopathy or subcutaneous masses in head and neck region, associated with peripheral blood eosinophilia and elevated IgE level. Pathological examination of surgical specimen is the gold standard for diagnosis. It has high incidence
of renal involvement and associated with nephrotic syndrome. It has a predilection in young Asian male in their second to fourth decade of life. Kimura disease is sometimes difficult to be distinguished from other conditions, including angiolymphoid hyperplasia.
Objectives :
This case series described various clinical presentations of Kimura disease, aiming to help clinicians to have a better understanding of this rare disease and raise clinical suspicion to this diagnosis.
Methodology :
Herein, Kimura disease patients managed in NTWC were recruited via electronic system. Patients’ demographic data, clinical presentation, investigations and clinical outcomes were reviewed.
Result & Outcome :
Four Asian male Kimura disease patients were recruited in this case series, with different onset age ranging from age 13 to 41. Three presented as mass in head and neck region, one of which had orbital involvement; and one presented as groin mass. Lesion ranged from 2 to 12 cm. Three of them were found to have peripheral blood eosinophilia on presentation. (mean ± S.D.
= 4.66 ± 4.07 x 109/L, with reference ranging 0 to 0.5 x 109/L) Different imaging modalities including ultrasound, computed tomography and magnetic resonance imaging were used in these patients as per indicated. All patients included in this study were diagnosed of Kimura Disease pathologically by surgical resection of involved tissue. Histopathological features included lymphoid follicular hyperplasia with germinal center formation, eosinophilic infiltrates with microabscesses, and interfollicular sclerosis. One of them received a course of medical therapy with steroid and antihistamine, where the other three patients received surgical excision only. They were followed up for at least 5 years. All patients had relapse of soft tissue swelling after initial excision, in which two of them had histological diagnosis of recurrent Kimura Disease requiring further surgical excision.
