Kimura’s disease with recurrent bilateral lacrimal gland involvement in a male Japanese child successfully treated with cyclosporine A

Abstract

Background 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. Involvement of the lacrimal glands and limbs is uncommon and seldom reported. Case presentation We 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. Conclusions We report a rare case of KD with a peculiar clinical presentation. The patient responded well to treatment with cyclosporine A.

Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease

“…11,12 This autoimmune mechanism can impacts the development of KD’s clinical manifestations and possibly accounts for the observed efficacy of immunosuppression therapy. Bilateral lacrimal gland enlargement is an infrequent orbital manifestation of pediatric KD, 13 and its association with a lateral ventricular lesion of the brain, to the best of our knowledge, has never been reported. In fact, the only published cases of central nervous system involvement in KD are represented by brain embolis.…”

Kimura Disease of the Periorbita and Earlobe in a 15-Year-Old Male: A Case Report and Review of the Literature

“…Table 1 summarizes the clinical features of patients with periorbital KD. 3–38 The average age of patients was 31.9 years (standard deviation: 17.8 years). A total of 81.1% (30/37) of identified patients were male.…”