Kimura’s disease (KD), also known as eosinophilic lymphogranuloma, was first discovered by the Japanese scholar Kimura and received its name. This disease is rare, and we report one case and review the literature. Case summary: The patient, a 57-year-old male, came to our hospital with a primary cause of bilateral upper eyelid swelling with lymph node enlargement for 3 years. The patient presented 3 years ago with no cause for bilateral upper eyelid swelling and discomfort, without redness, swelling, or pain. A few months later, multiple submandibular lymph nodes were found to be swollen, slightly hard in texture, without adhesions, up to 2.5 cm × 0.8 cm, with mild itching sensation, no tenderness, no fever, cough, arthralgias, etc. There was no history of drug and food allergy, asthma, tropical diseases or occupational diseases, and the eosinophils were significantly increased in local hospital laboratory tests (the ratio was up to 48%). The application of prednisone at a maximum of 40 mg/d caused the swelling of both upper eyelids to subside, leaving a mild swelling on the right side, and the eosinophils could be reduced to normal, but once the hormone was reduced to less than 10 mg/d or discontinued, the eosinophils increased again. Laboratory tests: blood before hormone use: WBC 11.84×109/L, eosinophils 35.5%, count 4.2×109/L; blood after hormone use: WBC 17.41×109/L, L0.432%, eosinophils normal. Immunological IgG 6.68g/L (normal 6.94-16.18), IgE 1900IU/ml (normal 0-100), IgM 0.42g/L (normal 0.6-2.63), urine routine, IgA, complement C3 and C4, ESR, various autoantibodies, allergens and parasite examination were negative or normal. In the two bone marrow aspirations before the use of hormones, the rest were normal except for an increased eosinophil ratio of 28.5%-30%, and the bone marrow picture was normal after the use of hormones. Ultrasound of the heart: left atrium was enlarged, tricuspid regurgitation was mild, pulmonary artery pressure was mildly increased (pulmonary artery systolic pressure = 40 mmHg), and left atrial pressure was slightly high (13 mmHg). Lymph node ultrasound: there were multiple lymph node-like echogenicity in both necks, with a maximum of 2.5 cm0.8 cm on the right side and 1.00.5 cm on the left side, with clear borders, some medullary structures were unclear, and there was no abnormal blood flow signal. Pathology of cervical lymph nodes: marked hyperplasia of lymph node germinal centers, progressive transformation of some germinal centers, extensive infiltration of mature eosinophils, hyperplasia of paracortical areas, visible hyperplastic small vessels with vitreous changes, varying degrees of sclerosis of sinus and paracortical areas. The diagnosis of Kimura disease was confirmed. After treatment with prednisone 30 mg/d, the patient was advised to undergo further radiotherapy to reduce recurrence. Discussion Kimura disease is a chronic progressive immune disease of unknown cause involving superficial lymph nodes and soft tissues of the head and neck. It occurs mainly in Japan, China, and Southeast Asia, and predominantly affects young and middle-aged males, with only 300 cases reported to date due to its low incidence. The ratio of men to women is about 4-7:1, and some literature reports that the ratio of men to women can be as high as 6-10:1. The disease can develop at any age, with the highest incidence at 20-50 years old, accounting for about 70%. The etiology and pathogenesis of the disease are still unclear and may be related to immune dysfunction. Pathological studies have revealed mast cell hyperplasia and IgA, IgM and complement C3 deposition around the hyperplastic small vessels, suggesting that the disease is an immune-mediated inflammatory reactive disease. It has been hypothesized that the disease may be associated with certain viral infections in which toxins alter the immunoregulatory role of T lymphocytes or induce IgE-mediated type I metaplasia, leading to the release of lymphokines, resulting in characteristic lymph node changes and associated renal damage, and thus Th2 cell immune dysregulation may be its etiology. The disease has a slow onset and long course, with involvement of the soft tissues of the head and neck, parotid and submandibular glands and superficial lymph nodes of the head and neck being the most common. The most common clinical manifestations are multiple soft tissue masses, mainly painless masses, mostly without self-consciousness, often located in the maxillofacial region, with unclear borders, adhesions to the skin, poor mobility, and a diameter of 1 to 10 cm. The texture of the masses resembles soft rubber in the early stages and gradually becomes hard and tough. Lymph node enlargement is also common, mostly in the subchin, submandibular and cervical areas. 40% to 100% of cases have pruritus and hyperpigmentation, mostly in the skin at the lump. In some cases, the lesions invade only the lymph nodes without subcutaneous masses. With the development of the disease, some patients can be combined with renal injury after several months, mainly manifested as nephrotic syndrome, and membranous lesions are the most common, and 12%-16% can be accompanied by proteinuria. In the present case, the main manifestation was bilateral cervical lymph node enlargement at common sites, but bilateral upper eyelid swelling has not been reported. Laboratory tests for this disease are characterized by a marked increase in the proportion and count of eosinophils in the peripheral blood picture, with the proportion mostly ranging from 10% to 20%, up to 69%, and elevated serum IgE. Eosinophils are significantly elevated in the bone marrow image of bone marrow aspiration, mainly in the late juvenile and mature stages. Imaging is non-specific and not easily distinguished from malignant tumors, lymphomas and hemangiomas, but there is generally no bone destruction, unlike malignant tumors. Pathologically, the mass has no perithelium and no obvious boundary with the surrounding tissues. Microscopically, we can see massive capillary hyperplasia, swelling and obvious proliferation of vascular endothelial cells, resulting in wall thickening and even lumen obstruction. There was a large number of lymphocytes and eosinophils infiltrating the vascular endothelial proliferation area, and lymphoid follicles were formed, and eosinophils were densely packed to form limited “eosinophilic small abscess” foci. The lymphatic follicles in the affected lymph nodes are actively proliferating, the growth centers are enlarged, and eosinophils are infiltrating the cortex, medulla, and subepithelium. The disease should be distinguished from angiolymphoid hyperplasiawitheosinophilia (ALHE). ALHE occurs in Western women, and the vascular lesions are characterized by proliferation and differentiation of angioblasts, formation of new infantile blood vessels, and large epithelial-like changes in the endothelium, often in the form of tombstone-like protrusions into the lumen. In contrast, Kimura disease has no such changes. There are recent case reports of the coexistence of these two diseases. The disease is a benign lesion with a good prognosis, but is more likely to recur. The disease is sensitive to radiotherapy, and it is the treatment of choice at home and abroad, with an efficiency of more than 90%. The total recommended dose in China is 26-30 Gy for 2-3 weeks, with conventional split dose, and the irradiation field includes the lesion and swollen area, and recurrence is rare if the dose is >26 Gy. In clinical practice, single local tumor is usually resected surgically first, because the tumor boundary is unclear, the surgery is not easy to completely resect, and it is very easy to recur after resection, the recurrence rate can be as high as 40%, so it is appropriate to perform frozen section examination during surgery to ensure sufficient resection range and reduce recurrence. Clinical experience shows that the cure rate can be significantly improved by adding small doses of radiotherapy after surgery or combined with glucocorticoid therapy after surgery. Oral prednisone 30-60 mg/d can significantly reduce the size of the mass or make it disappear, but it is easy to recur after stopping the drug and should not be used as the only treatment, and the patient in this case recurred during the hormone reduction. If there is renal damage, corticosteroids and immunosuppressants can be used, but relapse is also common after stopping the medication. For patients with recurrence or ineffectiveness after surgery or drug treatment, radiation therapy is equally effective, so if there are no contraindications and conditions allow, radiation therapy should be preferred. Single mass can be considered surgical excision, postoperative supplemented with low-dose radiotherapy and or glucocorticoid therapy; and for multiple patients can be surgery combined with radiotherapy, take the size of the cut, first put after the cut of the combined treatment to reduce recurrence.