Clinical effects of dupilumab: A novel treatment for Kimura disease.

BACKGROUND: Kimura disease (KD) is a rare chronic inflammatory disorder involving the Th2 pathway. Although medical treatment with steroids or other immunosuppressants is available, they may cause developmental issues in the pediatric population. Surgical intervention has also been suggested; however, it is associated with high recurrence rates. CASE PRESENTATION: A 14-year-old boy presented with left retroauricular lymph node enlargement at the age of 5 years. At the age of 7 years, he was diagnosed with nephrotic syndrome which subsided after steroid treatment for approximately 6 years. The retroauricular lymph node was surgically excised, and KD was confirmed. However, recurrent enlargement of the left retroauricular and neck lymph nodes occurred after 2 years. Persistently high IgE levels and fluctuating eosinophil counts were observed following steroid treatment. Dupilumab was prescribed because of the difficulty in tapering the steroid dosage. A loading dose of 600 mg was administered, followed by a maintenance dose of 300 mg every 2 weeks. The IgE level decreased after 3 months, and a low eosinophil count was maintained after steroid discontinuation. Follow-up computed tomography revealed a decrease in the size of the lymph nodes with no side effects such as conjunctivitis. CONCLUSION: Traditional treatments have raised developmental concerns in the pediatric population and are associated with high recurrence rates. Dupilumab targets the Th2 pathway and provides effective results, with few adverse effects. Dupilumab may be a therapeutic option for KD and other diseases involving the Th2 pathway.

A case of minimal change nephrotic syndrome complicated by Kimura disease treated with rituximab.

Kimura disease (eosinophilic granuloma of the soft tissue) is a benign granulomatous disease complicated by nephrotic syndrome. Herein, we report a case of recurrent minimal change nephrotic syndrome (MCNS) complicated by Kimura disease that was successfully treated with rituximab. A 57-year-old man presented to our hospital with relapsed nephrotic syndrome with worsening swelling of the right anterior ear and elevated serum IgE. MCNS was diagnosed on renal biopsy. Treatment with 50 mg of prednisolone rapidly placed the patient in remission. Therefore, RTX 375 mg/m2 was added to the treatment regimen, and steroid therapy was tapered. Early steroid tapering was successful, and the patient is currently in remission. In this case, the nephrotic syndrome flare-up was accompanied by worsening Kimura disease. Rituximab reduced the worsening of symptoms related to Kimura disease, including head and neck lymphadenopathy and elevated IgE levels. Kimura disease and MCNS may share a common IgE-mediated type I allergic condition. Rituximab effectively treats these conditions. In addition, rituximab suppresses Kimura disease activity in patients with MCNS, enables early tapering of steroids, and reduces the total dose of steroids.

Concurrent Warthin tumor and Kimura disease: a case report.

BACKGROUND: Warthin tumor (WT) is a common benign salivary tumor of the parotid gland. Clinically, it occurs in men in their fifth to seventh decades who typically smoke cigarettes. WTs have been reported with different head and neck neoplasms and other salivary gland tumors within the same or another salivary gland. Kimura disease (KD) is a rare chronic inflammatory disease with unknown etiology affecting young to middle-aged Asian men. KD presents as an asymptomatic nodule in the head and neck area, with regional lymphadenopathy and salivary gland involvement. CASE PRESENTATION: A 64-year-old Arabic man presented with a 10-year history of an asymptomatic swelling of the left face. Computed tomography showed a well-defined, multicystic mass with heterogeneous enhancement. The resected mass was composed of two distinct components. There was a well-demarcated proliferation of papillary and cystic oncocytic epithelium with lymphoid stroma, consistent with WT. Some areas exhibited sclerotic fibrosis, with multiple lymphoid follicles showing folliculolysis, follicular hyperplasia, and eosinophilic infiltrate. The patient's immunoglobulin E level serum was elevated, confirming a coexisting KD. The patient underwent a left superficial parotidectomy, with no recurrence at a 30-month follow-up. CONCLUSION: This report describes the first concurrent case of WT and KD in the parotid gland.

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

PURPOSE: The authors describe a case of Kimura disease (KD) affecting the lacrimal gland, the ipsilateral periorbita, and the contralateral earlobe. The authors also review the literature for characteristics and outcomes of KD affecting the periorbita or earlobe. METHODS: The authors review retrospectively a single chart and abstract data from the English language literature. RESULTS: A 15-year-old Samoan male presented with a left upper eyelid mass and later the contralateral earlobe and ipsilateral periorbita. Surgical resection was partially effective and normal vision and eye motility were maintained. Histopathology was consistent with KD. Thirty-seven cases of periorbital KD were identified in the literature. The average age of patients with periorbital KD was 31.9 years (standard deviation: 17.8 years), with 81.1% (30/37) of patients being male and 69.4% (25/36) Asian. Over half of periorbital KD patients had lacrimal gland involvement (51.3%; 19/37). Fifteen patients were initially treated with surgery; 3 (20%) had recurrence of the disease. Another 15 patients were initially treated with corticosteroids; 12 (80%) had recurrence of the disease. Ophthalmologic data, when reported, demonstrated that, most patients had no visual (77.8%; 14/18 patients) or motility disturbances (82.4%; 14/17 patients) but most had proptosis 71.4% (10/14). Only 4 cases of earlobe involvement in KD were identified, all in women. CONCLUSIONS: In the literature, orbital KD was rare but often affected the lacrimal gland and caused proptosis, commonly in young adult Asian males. Vision and extraocular motility were usually normal. Earlobe involvement was very rare. This unique case of KD that affected the orbit and contralateral earlobe corroborates the prior literature that KD is prone to recurrence but may not adversely affect vision or extraocular motility.

A Rare Kimura's Disease in the Oral Cavity with Severe Sleep Apnea: Case Report and Literature Review.

Kimura's disease (KD) is a rare chronic inflammatory disorder that commonly occurs in Asian males. It mainly presents as painless subcutaneous masses or lymphadenopathy in the head and neck region. The incidence of KD in the oral cavity is quite rare. We reported a rare case of a 53-year-old male who had KD in his soft palate, hard palate and bilateral tonsils associated with severe sleep apnea. This patient underwent radiotherapy and exhibited a good response to the treatment. Throughout the 12-month follow-up period, the patient's condition remained satisfactory. Of the other 14 reviewed cases of KD in the oral cavity, the lesions can occur in the buccal mucosa, hard and soft palate, and mouth floor with specific clinical features. We further summarized their manifestations and treatments in order to guide the future identification and management of KD with lesions in the oral cavity.

Kimura's disease of the left arm.

Kimura's disease is a rare, benign entity that causes subcutaneous angioblastic lymphoid hyperplasia with eosinophilia. It usually presents with subcutaneous lymphoid swellings with regional lymphadenopathy and salivary gland masses. Kimura's disease is frequently associated with renal involvement, which includes proteinuria and nephrotic syndrome as the most common presentations. This report presents a case of a man in his early 20s with a swelling in the medial aspect of the distal left arm with two previous episodes of nephrotic syndrome. Multiple enlarged axillary lymph nodes and epitrochlear nodes were noted. Ultrasonography revealed a mixed echogenic mass with enlarged lymph nodes. MRI showed a heterogeneous hyperintense lesion. Image-guided fine-needle aspiration showed multiple lymphocytes and eosinophils suggestive of Kimura's disease. The swelling was surgically excised under general anaesthesia. Histopathology of the excised mass confirmed the diagnosis of Kimura's disease.

Eosinophilic interstitial nephritis and cardiac insufficiency in Kimura's disease: a case report.

BACKGROUND: Kimura's disease (KD) is a rare chronic inflammatory disease and the etiology remains uncharacterized. The typical manifestations are painless lymph node or subcutaneous masses. There is currently no report of prominent renal interstitial injury and cardiac insufficiency in KD. CASE PRESENTATION: A 45-year-old man was referred to our hospital with dark urine, subcutaneous masses in forehead and right retroauricular, multiple lymphadenopathy and unexplained cardiac insufficiency. Renal biopsy demonstrated eosinophilic interstitial nephritis. Laboratory tests revealed eosinophilia and a high level of serum IgE. A biopsy of cervical lymph node was performed and KD was diagnosed. Treatment with oral prednisone resulted in a decrease of eosinophil, serum IgE, improvement of cardiac function, and regression of the subcutaneous mass. CONCLUSIONS: We describe an extremely rare KD case presenting with eosinophilic interstitial nephritis, cardiac insufficiency and significant response to prednisone. The clinicians should improve the disease awareness and find optimal treatment.

Eosinophilic chronic rhinosinusitis and concurrent Kimura's disease treated with mepolizumab.

Kimura's disease is a rare, benign, chronic inflammatory disorder characterised by its eosinophilic infiltrate. Patients often present with one or more progressively enlarging subcutaneous lymph nodes in the head and neck region or enlarging salivary glands. We describe the case of a 26-year-old man presenting with severe peripheral eosinophilia and upper airway inflammatory symptoms, who later developed cervical lymphadenopathy and formally diagnosed with Kimura's disease. Based on our English-language MEDLINE literature search, to our knowledge this is the first case report describing treatment of Kimura's disease with mepolizumab.

Is Kimura's disease associated with juvenile temporal arteritis? A case report and literature review of all juvenile temporal arteritis cases.

Both juvenile temporal arteritis (JTA) and Kimura's disease are eosinophilic inflammatory conditions but exhibit different clinical manifestations. Here, we describe a case involving a 40-year-old man who developed JTA secondary to Kimura's disease. Approximately 3 years before admission, masses appeared on both posterior auricles. A biopsy of the right posterior auricle mass led to a diagnosis of Kimura's disease. Approximately 4 months before admission, both masses increased in size, and almost simultaneously, the left temporal artery became distended. Histopathology of a biopsy of the left temporal artery revealed inflammatory findings with marked eosinophil infiltration and significant intimal hyperplasia with stenosis of the vascular lumen, indicating JTA. An analysis of the 48 reported cases of JTA, identified in a literature review, and the present case, revealed that Kimura's disease was detected in 6 cases, all of which involved Asians. In conclusion, this case and the literature review suggest that JTA can be accompanied by another eosinophilic inflammation-based disorder, Kimura's disease, particularly in Asians. This newly highlighted relationship between JTA and Kimura's disease could lead to a better understanding of JTA, which is an extremely rare disease.

Oropharyngeal Kimura's disease: a diagnostic dilemma and therapeutic challenge.

Kimura's disease is a chronic inflammatory disorder involving subcutaneous tissue and locoregional lymph nodes of head and neck region, characterised by angiolymphoid proliferation. It is typically associated with peripheral eosinophilia, increased serum IgE and lymphadenopathy. Diagnosis is usually based on histopathological examination. Though various treatment modalities have been put forward, surgical management remains the first line of treatment, followed by systemic steroids application. This case report discusses the diagnostic dilemma and therapeutic management of a rare clinical presentation and management of oropharyngeal Kimura's disease in a young immunocompetent woman.

Dyspnea and Cough: A Rare Report of the Kimura Disease.

BACKGROUND: Known as a rare disease, Kimura disease is a chronic, allergic and inflammatory process. It may overlap other allergic conditions, as well. CASE PRESENTATION: This study is going to present a 36-year-old woman, with cough, dyspnea and bone pain. Other differential diagnoses were excluded during the investigations. The definite diagnosis was made by excisional biopsy and pathological investigations. CONCLUSION: This was a rare medical condition with remarkable diagnostic challenge.