Kimura disease: A rare presentation in the rheumatology clinic

Abstract

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling in head and neck region, accompanied by regional lymphadenopathy and frequent salivary gland enlargement. Blood and tissue eosinophilia, and elevated immunoglobulin E (IgE) levels, are common associations. Characteristic histopathological findings of biopsy specimens obtained from the subcutaneous swellings or lymph nodes include eosinophilic infiltrates, follicular hyperplasia, and proliferation of postcapillary venules. The course is usually waxing and waning, albeit benign. Early diagnosis may spare the patient from unnecessary invasive procedures. We herein, describe a case of KD in an 18 years old male, who presented with subcutaneous swelling in the both cheek (right more than left) in 2014, underwent multiple surgical interventions for the same, before being reassessed and finally diagnosed as KD in 2020, based upon peripheral blood eosinophilia, raised serum IgE levels and histopathological findings. He subsequently made a good recovery on oral steroids.

Keywords

• angiolymphoid hyperplasia with eosinophilia
• eosinophilia
• immunoglobulin e
• kimura disease
• subcutaneous cheek swelling