Abstract
BackgroundKimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, that are 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. However, as the steroids were tapered after the patient developed side effects, the masses relapsed within a few months. Treatment with cyclosporine A was then initiated, which led to an 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 cyclosporine A.
Highlights
Kimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology
We report a rare case of KD with a peculiar clinical presentation
We describe a unique case of KD in a 4-year-old Japanese boy
Summary
Kimura’s disease (KD) is a rare chronic inflammatory disease of unknown etiology. Clinically, KD is characterized by nodular subcutaneous masses, that are typically localized to the neck and head. To the best of our knowledge, this is the youngest case of KD with bilateral lacrimal gland and arm involvement reported in the English literature. Case presentation A 4-year-old Japanese boy presented with bilateral upper eyelid swelling and discomfort.
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