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1/141. Localized soft tissue angiomatosis with subsequent development of angiolymphoid hyperplasia with eosinophilia.

    We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE.
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keywords = auricular, ear
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2/141. recurrence of focal segmental glomerulosclerosis associated with Kimura's disease after kidney transplantation.

    A 13-year-old Brazilian boy with Kimura's disease (eosinophylic lymphoid granuloma) and nephrotic syndrome is reported. Native kidney biopsy showed focal segmental glomerulosclerosis (FSGS). Treatment with prednisolone resulted in partial remission of proteinuria, and he had a progressive loss in renal function, requiring initiation of chronic dialysis, which he underwent for 46 months. After kidney transplantation, the patient developed proteinuria. A renal biopsy showed recurrence of focal segmental glomerulosclerosis, and subsequently he developed renal insufficiency.
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keywords = ear
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3/141. A case of eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma.

    This report describes a patient with eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. A 50-year-old man presented with a complaint of precordial pain. However, the only abnormal finding on examinatioin was eosinophilia (1617 eosinophils/microl). Three years later, the patient developed chronic eczema, and was diagnosed with erythroderma posteczematosa. One year later, a tumor was detected in the right auricule, and a diagnosis of Kimura's disease was made, based on the biopsy findings. The patient developed progressive dyspnea 6 months later and was found to have cardiomegaly and a depressed left ventricular ejection fraction (17%). A diagnosis of eosinophilic myocarditis was made based on the results of a right ventricular endomyocardial biopsy. The eosinophilic myocarditis and erythrodrema were treated with steroids with improvement of both the eosinophilia and left ventricular function.
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keywords = ear
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4/141. Kimura's disease: a case report and literature review.

    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.
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keywords = auricular, ear
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5/141. Kimura's disease of bilateral upper eyelids: a case report.

    We present a rare case of Kimura's disease of bilateral upper eyelids. A 41-year-old Chinese male had multiple masses over retroauricular, submandibular regions and bilateral upper eyelids since 13 years previously. Tumor recurred after each surgical excision, chemotherapy and Co 60 radiotherapy. Eosinophilia and elevation of blood immunoglobulin e (IgE) were noted in this admission. We operated on left upper eyelid to relieve the blocking of vision. A diffuse infiltration with numerous fibrotic bands was noted. Histological section showed a lymphoid hyperplasia, remarkable infiltration of eosinophils and proliferation of capillaries. Oral prednisolone 60 mg was given postoperatively. The patient had a satisfactory appearance and a relief of visual block.
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keywords = auricular, ear
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6/141. angiolymphoid hyperplasia with eosinophilia associated with arteriovenous malformation: a clinicopathological correlation with angiography and serial estimation of serum levels of renin, eosinophil cationic protein and interleukin 5.

    We present a case of angiolymphoid hyperplasia with eosinophilia (ALHE) affecting the auricular area of a 31-year-old man, which clinically mimicked arteriovenous malformation (AVM). The histology and laboratory data distinctively revealed ALHE, while angiography demonstrated typical findings of AVM. Although several reports have hitherto mentioned the relationship between ALHE and AVM, the aetiology of the disease remains unknown. During the 3 years treatment course, we performed angiography several times to assess the efficacy of the treatments and compared the clinical and pathological findings, based on the hypothesis that AVM might be a cause of ALHE. This study showed first, that the clinicopathological findings of ALHE correlated with the extent of AVM shown by angiography, so that AVM could be a primary cause of ALHE. Secondly, systemic corticosteroids and local irradiation therapy produced only a temporary effect on the inflammatory changes of ALHE; therefore, surgical resection is recommended as a curative treatment. Thirdly, the patient's serum levels of renin, eosinophil cationic protein and interleukin 5 corresponded closely with the clinical course of ALHE.
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keywords = auricular, ear
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7/141. eosinophilic granuloma (Kimura's disease) of the orbit: a case report.

    BACKGROUND: eosinophilic granuloma of the soft tissue, Kimura's disease, is a benign slow-growing tumor that is manifested clinically by one or more inflammatory nodules involving mainly the face and scalp, but rarely the eye. CASE REPORT: The patient was a 32-year-old male with swelling of the left lower eyelid, marked peripheral blood eosinophilia and increased serum immunogloblin E. MRI revealed swelling of all rectus muscles of the left eye, but no tumor mass. Corticosteroid treatment reduced the swelling of the eyelid, but it recurred after corticosteroid was discontinued. Eight years later the patient returned with a complaint of increased swelling of the left lower eyelid. An elastic, nontender, soft tumor mass was palpable subcutaneously in the left lower eyelid extending into the orbit. MRI revealed a tumor mass in the left orbital space. The parotid gland was also swollen and palpable. Both tumors were resected surgically, and histopathological study revealed prominent proliferation of lymphoid follicles with germinal centers showing interfollicular infiltration by eosinophils. The pathological findings in the parotid gland were similar. The diagnosis was Kimura's disease. CONCLUSION: This patient is unique in that he had no tumor at the first examination, only swelling of the rectus muscles, and a tumor mass appeared many years later. Unilateral swelling of the rectus muscles may be one of the first signs of Kimura's disease. Not only tumor but also swelling of the rectus muscles limited ocular movement.
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ranking = 0.040221996932629
keywords = ear
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8/141. Kimura's disease of the intraparotid lymph nodes: fine needle aspiration biopsy findings.

    A case of Kimura's disease involving two intraparotid lymph nodes is described in a 16-year-old Caucasian boy presenting with a painless swelling of the right parotid gland. A preoperative diagnosis of Kimura's disease was made on the basis of fine-needle aspiration biopsy (FNAB) and was confirmed by histological evaluation of the surgical specimen. FNAB is a safe and accurate procedure that can be used in the outpatient setting to enable a prompt preliminary diagnosis.
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keywords = ear
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9/141. Kimura disease of the orbit and ocular adnexa.

    Kimura disease (KD) is a distinct clinicopathologic entity that has been the subject of considerable confusion and debate. Although common in Asia, KD rarely occurs in non-Asian patients. Kimura disease shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia (ALHE). Because of this overlap and the rarity of KD in europe and the united states, KD and ALHE have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing KD and ALHE as two separate entities, based on their histologic features. Kimura disease occurs most commonly in the head and neck region and has been described in the orbit, eyelids, and lacrimal gland more frequently than ALHE. Because both diseases can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, they should be considered in the differential diagnosis of orbital lesions occurring in adults. We report two cases of KD involving the orbit and ocular adnexa, and review additional cases reported in the literature. The ophthalmic literature does not clearly reflect the current understanding that KD and ALHE are best considered two separate clinicopathologic entities.
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keywords = ear
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10/141. Epithelioid hemangioma of the temporal artery clinically mimicking temporal arteritis.

    A rare case of epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) arising in the right temporal artery of a 68-year-old Japanese woman was investigated. The patient had been treated with corticosteroids (Predonine 5 mg/day p.o.) for 4 years for idiopathic thrombocytopenic purpura. Headaches in the right temporal region with repeated high fevers first appeared 1 year prior to the biopsy of a nodule at the artery. The clinical diagnosis was temporal arteritis (giant cell arteritis). The specimen consisted of a short segment of the superior arterial branch having a sheet-like proliferation of the epithelioid endothelial cells in the lumen, which was almost occluded, focal rupture of the media, and marked proliferation of the capillaries (neovascularization) radiating out from the media to the adventitia. There were scattered foci of inflammatory cell infiltration composed mainly of small lymphocytes in the adventitia, but there were no histological changes suggestive of giant cell arteritis. The lesion was thus characterized by the proliferation of epithelioid endothelial cells in the lumen of the artery, and neovascularization between the media and adventitia. The histological features were very close to epithelioid hemangioma except for the absence of an eosinophilic reaction. The long-term administration of corticosteroids may have suppressed it. The authors believe this is the first case of epithelioid hemangioma arising from the temporal artery without a history of trauma. It showed some unique features both clinically and histopathologically, which contributed to the study of the yet not clearly classified vascular proliferative lesion, epithelioid hemangioma.
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ranking = 0.050277496165787
keywords = ear
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