Kimura’s disease mimicking thoracic spine dumbbell neurogenic tumor: a case report and literature review

Bi, Siwei; Gu, Jun; Hu, Chao

doi:10.1186/s12893-020-00870-0

Cited by 4 publications

References 36 publications

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Kimura's disease

Matchin¹,

Matz²

2023

Kazan Med J

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The analysis of published clinical observations on the diagnosis and treatment of patients with Kimura's disease, which is a rather rare chronic inflammatory disease of human soft tissues of unknown etiology, is presented. The onset of Kimura's illness is hardly noticeable. The disease proceeds sluggishly, with a varied clinical picture, most often with the formation of single or multiple subcutaneous nodules or tubercles and swelling in the head and neck on one or both sides. Other localizations of lesions are also described: limbs, orbit, eyelids, palate, pharynx, armpit, chest, inguinal region. The affected areas gradually increase in size over many months or years. Other clinical manifestations of Kimura's disease are presented in the form of edema of the extraocular muscle, juvenile temporal arteritis, sleep apnea in cases of damage to the larynx, and life-threatening coronary artery spasm in children. There are reports of other rare systemic clinical signs of Kimura's disease in the form of pruritus, eczema and rash. The disease is often associated with regional lymphadenopathy and/or salivary gland involvement, eosinophilia, and an increase in serum immunoglobulin E from 800 to 35,000 IU/mL. The disease may be accompanied by kidney damage in the form of glomerulonephritis and nephrotic syndrome. Most reported cases of Kimura's disease occur in young Asian men in their 20s and 30s, sporadically the disease is diagnosed in Europeans, North Americans, Caucasians, and Africans. Diagnosis of Kimura's disease is often clinically difficult. It is primarily based on excisional biopsy of the lesion or regional lymph nodes, and the histopathological findings are consistent regardless of the location of the lesion. Morphologically, Kimura's disease is characterized by the presence in biopsy specimens of such histological features as reactive follicular hyperplasia with prominent follicles surrounded by fibrous tissue. Interfollicular infiltrates are rich in plasma cells and eosinophils, which form diffuse eosinophilic microabscesses associated with granulomatous inflammation. The vessels are numerous, with an increase in postcapillary venules. At the same time, the blood vessels remain thin-walled, with cuboidal endothelial cells. Treatment issues are highlighted, and differences between Kimura's disease and angiolymphoid hyperplasia with eosinophilia are presented.

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Kimura's disease

Matchin¹,

Matz²

2023

Kazan Med J

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Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches

Zhu,

Zhang,

Zhang

et al. 2024

Preprint

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Background Kimura disease (KD) is a rare, chronic, inflammatory disorder characterized by painless subcutaneous masses, lymphadenopathy, elevated eosinophil counts and increased serum immunoglobulin E (IgE) levels. Due to the heterogeneity and rarity, current diagnostic and treatment approaches for KD are often delayed and have limited efficacy. Methods The clinicopathological data of 51 KD patients between January 2014 and December 2023 were retrieved and stratified based on age and mass size, respectively. A case ofrecurrent KD who responded well to a low dose of mepolizumab was described. Results We found that pruritus occurred in 45.8% of patients, with higher rates in older patients (P=0.033). Edema was observed in 29.2%, with higher prevalence in younger patients (P=0.010). The mean maximum mass size was 3.1cm, varying by age group (P=0.005). The mean time to diagnosis was 36 months, increasing with mass size (P=0.024). Meanwhile, we reported that one of these 51 patients was successfully treated with mepolizumab at a dose of 100mg every 4 weeks. Conclusion We conclude that pruritus and edema are the two main clinical manifestation of KD, and the size of mass is related to the time of diagnosis. Biologic agents targeting the Th2 pathway show promise for KD treatment.

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Dupilumab combined with corticosteroid therapy for Kimura disease with multiple systemic masses: a case report and literature review

Lyu,

Cui,

et al. 2024

Front. Immunol.

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To date, the pathogenesis of Kimura’s disease remains unclear, there is no unified diagnostic criterion, the clinical phenotype shows considerable heterogeneity, and there is a lack of optimal treatment strategies. Due to its rarity, treatment strategies for KD are still under exploration. This paper reports a case of a 37-year-old Chinese female presenting with generalized erythematous papules and pruritic eruptions for 12 years, followed by the onset of limb swellings 3 years later, ultimately diagnosed as Kimura’s disease. Considering the patient’s multiple lymphadenopathies and limb swellings with concurrent atopic dermatitis, the treatment regimen included initial dupilumab dosage of 600 mg (300 mg administered in two injections), followed by subcutaneous injections of 300 mg every two weeks for four months. Concurrent oral corticosteroid therapy (methylprednisolone, initial dose 16 mg/kg/day, gradually tapered with tumor regression) was also administered. Following treatment, the patient did not experience severe adverse effects, and the multiple nodules markedly decreased in size. Additionally, serum IgE levels, eosinophil, and basophil counts showed significant reductions. These results demonstrate the significant efficacy of dupilumab combined with oral corticosteroids in treating Kimura’s disease with concurrent atopic dermatitis.

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Kimura’s disease mimicking thoracic spine dumbbell neurogenic tumor: a case report and literature review

Cited by 4 publications

References 36 publications

Kimura's disease

Kimura's disease

Kimura disease: comprehensive analysis of clinical features, comorbidities, and treatment approaches

Dupilumab combined with corticosteroid therapy for Kimura disease with multiple systemic masses: a case report and literature review

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