JC EH lin xp ematopathol
Case Study
INTRODUCTIONThe ocular adnexa are supporting tissues for the eye globe, comprising the eyelid, lacrimal gland and sac, extraocular muscles, and orbital interstitial tissue. These sites are frequently affected by lymphoma 1 and inflammatory diseases such as IgG4-related disease.2 Magnetic resonance imaging of ocular adnexal masses cannot differentiate inflammatory diseases from lymphoma. Therefore, excisional biopsy is required to establish the diagnosis and decide the treatment strategy.Kimura disease predominantly occurs in young Asian males and presents as soft subcutaneous granulomas, which are frequently seen in the head and neck. Lymphoid follicular hyperplasia and marked eosinophilic infiltration are the pathological characteristics of Kimura disease, and blood examination commonly reveals eosinophilia and high serum immunoglobulin E (IgE).3-6 Kimura disease rarely manifests as orbital tumors such as lacrimal gland masses. [7][8][9] Pathologically, it remains controversial whether Kimura disease in Asian reports and angiolymphoid hyperplasia with eosinophilia in Western reports are the same entity. 6 In this study, we report a patient with Kimura disease who initially presented with a lacrimal gland mass and then developed nephrotic syndrome one month after the tapering of oral prednisolone. We also reviewed 10 reported Japanese patients with Kimura disease associated with proteinuria.
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CASE REPORTA 42-year-old man noticed upper eyelid swelling on the left side that subsided spontaneously 5 months ago. He again developed left eyelid swelling with left exophthalmos 2 months ago, and was referred to an ophthalmologist. In his past history, he underwent bilateral tonsil extirpation due to otitis media at the age of 5 years. He developed systemic urticaria lasting a week after eating sashimi 3 years ago, and Nephrotic syndrome during the tapering of oral steroids after pathological diagnosis of Kimura disease from a lacrimal gland mass: case report and review of 10 Japanese patients A 42-year-old man with eosinophilia and high serum immunoglobulin E (IgE) developed a lacrimal gland mass on the left side. Excisional biopsy revealed hyperplasia of lymphoid follicles, and infiltration with lymphocytes and eosinophils around lacrimal gland acini, leading to the pathological diagnosis of Kimura disease. IgE-positive cells were mainly found along follicular dendritic cells, and a small number of IgG4-positive cells was present. One month after oral prednisolone was started at 40 mg daily and tapered to 10 mg daily, he developed lower leg edema on both sides and marked proteinuria (10.8 g/day). Renal biopsy showed no glomerular abnormalities, no immunoglobulin deposition, and no tubulointerstitial infiltration with eosinophils, leading to the diagnosis of minimal change nephrotic syndrome. Proteinuria subsided in response to an increased dose of prednisolone to 30 mg daily. Proteinuria relapsed three times in the following 5 years when oral prednisolone was tapered. In conclus...