Kimura's Disease - An Unusual Presentation.

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.

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'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.

Kimura disease is a rare entity typically involving painless subcutaneous nodules of the head and neck that display characteristic pathologic findings and peripheral blood eosinophilia.1 Historically, this disease primarily affects young males of East Asian descent, with a peak incidence in the second and third decade of life.2 To create awareness that this condition should be considered when subcutaneous nodules appear in the head and neck region in persons not of Asian descent, we report an unusual case of Kimura disease in an African American male in his 60s.

Kimura's disease (KD) is a benign chronic inflammatory disorder attributed to an immune-mediated hypersensitivity which often presents as a tumor-like swelling in the head and neck region with or without lymphadenopathy, associated with hypereosinophilia and elevated serum immunoglobulin E. Most cases have been described predominantly in Chinese and Japanese people. KD has been confused with angiolymphoid hyperplasia with eosinophilia, from which it probably should be distinguished as a separate entity. The course is usually benign except for the potential cosmetic disfigurement. The diagnosis may be suggested by a fine-needle aspirate but is established by a biopsy. A case of KD in a 42-year-old male with recurrent left submandibular swelling is presented together with a brief 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 (KD) is an immune-mediated disorder which mainly affects Asian men. It appears as head and neck subcutaneous masses, with inflammatory infiltrate and elevated serum immunoglobulin E levels. The clinical presentation of KD resembles that of various diseases. Here, we report the case of a 30-year-old Filipino man with KD mimicking cavernous hemangioma who was treated by surgery. Careful survey for possible KD cases is crucial. Misdiagnoses are prone to futile interventions and unwanted effects. Surgery with adjuvant therapy is superior to other forms of KD treatment.

Kimura’s disease is a chronic infl ammatory disease producing subcutaneous tumor-like nodules chiefl y in the head and neck region. It is characterized histologically by lymphoid follicles, intense aggregations of eosinophils, vascular proliferation and fi brosis combined with peripheral blood eosinophilia. We report a 56-year-old man who presented with dysarthria and right hemiparesis. On physical examination, a non-ender mass 5 × 6 cm was identifi ed in the right submandibular area. He was diagnosed with Kimura’s disease by biopsy. One month after admission, he complained of paresthesia in the left lower extremity. CT angiography revealed thromobosis in the left femoral vein and bilateral pulmonary embolism. To our knowledge, the concomitant occurrence of acute cerebral infarction, deep vein thrombosis and pulmonary embolism in patients with Kimura’s disease has never been reported. Eosinophil-derived cytotoxic proteins are presumed to be fundamental to the pathogenesis of these disorders in Kimura’s disease. (Korean J Stroke 2011;13:85-88)

Kimura's disease is a rare chronic inflammatory disease of unknown etiology, which was initially described in 19371 but popularized in 1947 by Kimura.2 Only about 200 cases have been reported in the literature hence mis-diagnosis and non-recognition are common. It generally affects the skin, soft tissues and lymphatics of the head and neck region and, rarely, it may involve other regions like retro peritoneum, groin, and trunks and limbs.3–6 The lymph node groups, especially the axillary, epitrochlear, popliteal and inguinal, may be more commonly involved. Presentation in the form of subcutaneous swellings, lymphadenopathy and recurrences after treatment being common make confusion with malignancy likely as in the presented case. All forms of treatment have been tried – including surgery, radiotherapy, immunosuppressive therapy, etc. However, there is no consensus on the correct modality in view of the limited number of reported cases and the treatment remains individualized. There is a very high incidence of recurrence following surgery or other treatment modalities.6–8 The disease more commonly affects Asian men in their second and third decades. The usual clinical features include multiple, painless, subcutaneous swellings and generalized lymphadenopathy accompanied by peripheral eosinophilia and raised serum immunoglobulins, especially IgE. Unlike most allergic disorders, the overlying skin is usually healthy. The only known systemic manifestation is renal involvement in the form of glomerulonephritis and, rarely, nephrotic syndrome. It is thought to be more common in Asia – especially in Japan and China – however, sporadic cases from the West have also been reported.3 In the presented case, on account of history of recurrence and the hard consistency of the gland associated with regional lymphadenopathy there was reason to suspect malignancy. Fine needle aspiration cytology (FNAC) was inconclusive on two occasions and the patient was treated as a suspected case of parotid malignancy. Kimura's disease was thus a histo-pathological surprise.

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.

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 is a very rare disorder and causes swellings and lymphadenopathy, especially in neck and axillary region. The aetiology is still unknown but allergic reactions or an alteration of immune system should be taken into consideration. Here, we report a 45-year-old Turkish male patient who presented to the Uludag University Medical Faculty Emergency Medicine Department with complaint of enlarged lymph nodes in the neck region and pruritus. His leukocyte count and eosinophil rate were found to be elevated in the emergency laboratory results. During his follow up the nodes were removed and histopathologically evaluated as Kimura's disease. In conclusion, one can say that the diagnosis of Kimura's disease can be very difficult and histopathological features play an important role. However, this diagnosis should not be underestimated in young male patients with lymph nodes in cervical area especially in the emergency department.

Kimura disease (KD) is a chronic inflammatory disease characterized by slowly growing subcutaneous nodules in the face and the neck region. Although a concomitant hypercoagulable state can accompany KD, massive thromboses in patients with KD have rarely been reported. Here, we report a case of KD complicated with bowel infarction and multiple arterial thromboses in the upper and lower extremities. The patient underwent bowel resection and was successfully treated with corticosteroid and anticoagulation.