Kimura’s Disease With Recurrent Bilateral Lacrimal Gland Involvement in a Male Japanese Child Successfully Treated With Cyclosporine A

Abstract

BackgroundKimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, typically localized to the neck and head. Involvement of the lacrimal glands and limbs is uncommon and seldom reported. Case presentationWe report a case of a 4-year-old Japanese boy presenting with bilateral upper eyelid swelling with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological, and laboratory findings, the patient was diagnosed with KD. An itchy subcutaneous mass on the left arm developed at the age of 14 years. Treatment with steroids was effective, but the masses relapsed within a few months as the steroids were tapered after the patient developed side effects. Treatment with cyclosporine A was initiated, which led to the improvement of clinical features and serial levels of cytokines. ConclusionsWe report a rare case of KD with a peculiar clinical presentation. The patient responded well to treatment with CsA.