Kimura's disease masquerading as parotid malignancy

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.

Kimura's disease (KD) is a chronic inflammatory disease, a rare variety. It usually presents as non-tender subcutaneous swelling in head and neck region, predominantly in preauricular and submandibular area and is often associated with cervical lymphadenopathy, marked peripheral eosinophilia and an elevated immunoglobulin E (IgE) level. Renal involvement is the only systemic manifestation.4 Nephrotic syndrome is the most common manifestation of renal disease in KD. Diagnosis through Fine needle aspiration cytology (FNAC) is misleading and can easily be mistaken as a malignant disease. So, diagnosis is therefore only established by histopathological examination. The treatment of KD involves one of three major approaches are surgical excision, irradiation, or steroid therapy. Surgical excision is recommended as the treatment of choice but carries recurrence rates of 33-50%. In our study, we are reporting a case of elderly female who presented with left sided cervical lymphadenopathy for which excision biopsy was done and the histopathology examination shown as kimura lymphadenopathy, a rare case.

Kimura disease (KD) is a rare inflammatory soft tissue disorder of unknown origin most frequent in Asians, the prevalence of which is growing in Western countries. Painless papules and/or nodules with a predilection for the head and the neck region, lymphadenopathies, parotid gland involvement, eosinophilia, and raised IgE levels are parts of its presentation. Renal involvement with various forms of glomerulonephritis, including membranous nephropathy (MN), can occur and is generally associated with a proteinuria that encompasses nephrotic syndrome. Corticosteroids are the mainstay of treatment of KD-associated glomerulonephritis, but steroids withdrawal is often followed by relapsing nephrotic syndrome. Various immunosuppressive agents have been tested to prolong the remission of KD-associated nephrotic syndrome while tapering steroids, but they are only partly effective or associated with significant complications. To the best of our knowledge, we describe here the first case of KD-related membranous glomerulonephritis with a favorable evolution and a sustained remission of 4years under prolonged therapy with mycophenolic acid (MPA). MPA and its active metabolite, mycophenolate mofetil (MMF), treatments as supportive therapies to corticosteroids and ACE inhibitors should be further investigated in KD-related membranous nephropathies. .

Kimura's disease is a rare chronic inflammatory condition of uncertain etiology which has an affinity for the Asian population. It primarily involves the head and neck region, presenting as deep subcutaneous masses and is often accompanied by regional lymphadenopathy and salivary gland involvement. Peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels are characteristic features and the microscopic picture reveals lymphoid proliferation with eosinophilic infiltration. For years, Kimura's disease was believed to be identical to or part of the same disease spectrum as angiolymphoid hyperplasia with eosinophilia (ALHE). Recent reports, however, have confirmed that the two are, in fact, separate entities. We report a case of Kimura's disease in a 22-year-old Indian male who presented with a subcutaneous mass, parotid enlargement and lymphadenopathy. The clinical presentation was suggestive of Kimura's disease and microscopic examination following biopsy of the lesion allowed us to make a definitive diagnosis.

Rare case of Kimura disease of the upper lip: A case report

Kimura disease (KD) is a rare, chronic, inflammatory disorder of unknown etiology characterized by the development of subcutaneous lymphoid masses, usually in the head and neck region; regional lymphadenopathy; peripheral eosinophilia; and elevated levels of serum IgE.KD has been described in China and Japan as "eosinophilic hyperplastic lymphogranuloma" or "atypical granulation associated with hyperplastic abnormalities in the lymphoid tissue".The precise prevalence and incidence and the pathogenesis of KD are unknown.Trauma, infection, an IgE-mediated hypersensitivity reaction, or autoimmune processes have been postulated as possible causes.In this article, we aimed to present a patient who was investigated with elevated serum total IgE and eosinophils and diagnosed as Kimura disease in the light of the current literature.

Kimura’s Disease of the Parotid Region: Report of 2 Cases and Review of the Literature

Kimura's disease, which occurs endemically in the Far East and sporadically in the West, has so far eluded efforts to determine its exact pathogenesis. It presents as solitary or multiple benign swellings of the skin, has a predilection for the periauricular and scalp regions, and often is associated with regional lymphadenopathy. Morphologically, the lesions are characterized by proliferating blood vessels with rich eosinophilic infiltrate. Peripheral blood eosinophilia and raised serum IgE levels are signature features of the condition. The overall prognosis is good. When surgery is not possible, conservative treatment with either corticosteroids or radiation often can produce a favorable response. Complete surgical excision whenever feasible is the preferred treatment despite a high recurrence rate. Based on a recent case of Kimura's disease in a 55-year-old black woman, we discuss the pitfalls in the diagnosis of this chronic inflammatory disorder.

Kimura disease (KD) is a rare chronic inflammatory condition that primarily affects Asian males and typically presents in the head and neck region. We describe an exceptionally rare case of KD involving the lingual tonsil of Waldeyer’s ring in a 39-year-old Japanese man, marking only the second reported instance of lingual involvement and the first specifically affecting the tongue base. The patient presented with a well-circumscribed, 3.5-cm mass extending from the lingual tonsil to the epiglottis. Laboratory findings revealed significant peripheral eosinophilia (13.5%) and elevated serum IgE levels (2,750 IU/mL). Because of the challenging location for conventional biopsy, fine-needle aspiration cytology was performed on associated cervical lymph nodes. Cytological examination identified Warthin-Finkeldey-type multinucleated cells, eosinophilic infiltration, and vascular proliferation, leading to a presumptive KD diagnosis based on cytomorphology. The diagnosis was confirmed through surgical excision and histopathological analysis. This case is noteworthy for two reasons: it documents an extremely rare presentation of KD in the tongue base and underscores the diagnostic value of cytological examination in anatomically difficult locations where surgical biopsy may be unfeasible. The presence of Warthin-Finkeldey-type multinucleated cells in cytological specimens provided a key diagnostic clue, particularly when integrated with clinical and laboratory findings. At six months post-surgery, the patient showed no recurrence. This case highlights the importance of considering KD in the differential diagnosis of head and neck masses, even in atypical locations, and demonstrates the potential utility of cytological examination in diagnosing KD.

Kimura's disease is a rare chronic inflammatory disease of unknown etiology, presenting as painless subcutaneous nodules with lymphadenopathy and peripheral eosinophilia, mainly disturbing the head and neck region. It mainly affects Asian males in their 2(nd) to 4(th) decade of life. One such case of Kimura's disease, which is uncommon in Indian natives, is reported. A male patient presented with an insidious onset of a progressive painless disfiguring swelling over his nose since four years, which was associated with nasal obstruction and post aural swelling with a history of an inconclusive incisional biopsy. Clinical examination showed a bilobed subcutaneous swelling present over the nose and a collapsed nasal valve area on anterior rhinoscopy. FNAC was non-diagnostic and CT scan showed a mildly enhancing mass lesion over the external nose. Complete surgical excision was performed. Diagnosis was confirmed upon postoperative histopathology. During his 2nd week follow up, the patient had a small nasal recurrence, which was treated medically with oral steroids, cetirizine, and pentoxyphylline for 4 weeks. The patient was disease free for 6 months. Kimura's disease, although difficult to diagnosis clinically, should be considered in the differential diagnosis of patients who have a primary lymphadenopathy with eosinophilia with or without subcutaneous nodules. It should be investigated accordingly as the disease has an indolent course and good prognosis.

Kimura disease is a rare chronic inflammatory disorder of unknown etiology. The disease is primarily seen in young Asian males and is characterized by painless subcutaneous swelling. It is often accompanied by renal involvement, predominantly manifesting as nephrotic syndrome. We describe a case of Kimura disease in a 69-year-old Indian male, presenting with left postauricular swelling. Clinical and histopathological characteristics are described along with a brief review of the literature

IntroductionKimura disease (KD), also known as eosinophilic hyperplastic lymphoid granuloma, is a rare benign chronic inflammatory condition, which is featured with the painless progressive mass located in the subcutaneous area of the head and neck region, elevated peripheral blood eosinophils, and raised serum immunoglobulin E (IgE) levels. KD is uncommon in clinical practice, especially with low incidence in children, so it often leads to misdiagnosis or missed diagnosis in pediatric patients.MethodsThe clinical data of 11 pediatric patients with KD in the authors' institution were retrospectively analyzed.ResultsThere were 11 pediatric patients with KD enrolled in total, including 9 male patients and 2 female patients (sex ratio 4.5:1). The median age at diagnosis stood at 14 years (range 5-18 years), the initial symptoms in all patients included painless subcutaneous masses and focal swelling, the duration of symptoms ranged from 1 month to 10 years, and the average duration was 24.45 months. Six patients had single lesions, and 5 had multiple lesions. The highest proportion of lesion regions were parotid gland (n = 5, 31.3%) and retroauricular (n = 5, 31.3%), followed by cervical lymph nodes (n = 4, 25%), and others (n = 2,12.5; elbow n = 1; back n = 1). The eosinophil absolute count elevated in all patients, ranging from 0.71×109/L to 10.35 ×109/L (normal range 0.02-0.52×109/L). IgE levels were increased in all 7 patients who underwent serum immunoglobulin examination (normal range <100 IU/mL). Three patients received oral corticosteroid treatment while 2 relapsed. Three patients received surgical resection combined with oral corticosteroid treatment, and no patient relapsed. The other 3 patients received surgery and radiotherapy, surgery combined with corticosteroid and cyclosporin and corticosteroid combined with leflunomide respectively, and no patient relapsed.ConclusionBase on the study, it is found that Kimura disease is rare and may have the atypical symptoms in pediatric patients, combination therapy is recommended to reduce recurrence, and long-term follow-up should be performed.

Kimura's disease is a rare, chronic inflammatory disease of unknown cause. It is characterized by painless subcutaneous swellings and lymphadenopathy, commonly affecting the head and neck region. This is associated with peripheral blood eosinophilia and raised serum IgE. It has distinct histological features of lymphoid follicles, eosinophilic infiltrate, fibrosis and vascular proliferation. The disease usually has a benign, indolent course. Traditionally, therapeutic options have included surgery, radiotherapy and steroids but response has been less than satisfactory. Recently, cyclosporine has been reported to be effective in the treatment of Kimura's disease. In this article, we present a middle‐aged Chinese female with Kimura's disease for 20 years and her favourable response to cyclosporine.

Kimura disease (KD) is an autoimmune eosinophilic granulomatous disorder with generalized lymphadenopathy. A handful of pediatric patients with renal disease have been described, none of whom have been African-American (AA). We present an AA boy with KD and nephrotic syndrome (NS). Two months after stopping steroids, fever, asthma, eczema, and proteinuria recurred. His NS did not relapse but his platelet count decreased to 51,000/microl (x10(6)/l). On restarting prednisone, his platelet count normalized. A kidney biopsy revealed 23 of 37 glomeruli obsolescent and advanced damage with over 50% of cortical tissue replaced by interstitial fibrosis and chronic inflammation. Glomerular immunofluorescence was largely negative; very intense linear anti-tubular basement membrane (TBM) deposits of IgA, IgG, C3, and C4 were noted. At present, 36 months from onset, serum creatinine is 1.2 mg/dl (106 micromol/l). We present a 4-year-old AA boy with KD, NS, relapsing thrombocytopenia, and renal damage with anti-TBM antibody.

Kimura's disease (KD) is an allergic, inflammatory disorder of unknown cause. The typical presentation is in a young Oriental male with nontender subcutaneous swellings in the head and neck region, lymphadenopathy, peripheral eosinophilia, and elevated serum IgE. Many patients with KD also develop renal involvement. Treatment options range from conservative observation for asymptomatic patients to surgical excision, steroid therapy, and radiotherapy for symptomatic patients. Two cases of KD are presented. These emphasize not only the variability of disease presentation, but also the pitfalls of therapy leading to frequent recurrences. The literature is reviewed, summarizing patient presentation, differential diagnosis, current theories on causation, and therapy. A treatment protocol is also proposed.