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1/106. 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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2/106. 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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3/106. 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 = hyperplasia
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4/106. 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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5/106. 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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6/106. Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand.

    A case report of recurrent angiolymphoid hyperplasia with eosinophilia (ALHE) in an otherwise healthy 20-year-old female with manifestation of the disease limited to the left arm and hand is presented together with brief evaluation of the literature as well as the features distinguishing ALHE and Kimura's disease. Immunohistochemical investigations support the hypothesis that ALHE represents a reactive inflammatory lesion rather than a benign vascular neoplasm. A viral cause of ALHE (e.g., HHV8 or Epstein-Barr virus (EBV)) could not be demonstrated. The recurrent nature of the disease is shown by this case, which also demonstrates the need for frequent medical and surgical management.
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keywords = hyperplasia
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7/106. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in the oral mucosa. A case report and review of the literature.

    angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign uncommon entity whose aetiology and pathogenesis is under debate. Clinically, it is characterised by cutaneous papules or nodules. Cases of this entity reported in the oral mucosa are very rare. We describe such a case, discuss the problems of histological differential diagnosis between ALHE and other diseases of the region and review the literature.
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8/106. angiolymphoid hyperplasia with eosinophilia showing characteristics of Kimura's disease.

    angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign condition affecting principally the head and neck region of young females. We describe a 42-year-old female patient of ALHE showing the typical changes of endothelial cells and features similar to Kimura's disease in histologic and immunohistochemical findings.
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keywords = hyperplasia
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9/106. angiolymphoid hyperplasia with eosinophilia: report of a lesion mimicking soft tissue tumor of extremely long duration.

    We present a large sized lesion of the right upper arm in which characteristics of the angiolymphoid hyperplasia with eosinophilia (ALHE) intermingled with those of Kimura's disease (KD). The laboratory findings, the prominent vascular proliferation and the features of endothelial cells were suggestive of ALHE. However, the long duration of the disease, the site of involvement, the abundant lymphoid component forming lymph follicles with germinal centers and the fibrosis are features of KD. In agreement with other reports, our case shows that clinicopathologically there is an overlap between ALHE and KD.
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keywords = hyperplasia
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10/106. angiolymphoid hyperplasia with eosinophilia successfully treated with a long-pulsed tunable dye laser.

    BACKGROUND: To date, both surgical excision and laser treatments for angiolymphoid hyperplasia with eosinophilia (ALHE) have resulted in scarring. OBJECTIVE: Based on the principle of selective photothermolysis, we considered employing the newer long-pulsed tunable dye laser in the treatment of ALHE, as less scarring would be expected and deeper blood vessels could be ablated. methods: After punch biopsy specimens confirmed the diagnosis, the patient was treated on two separate occasions, 2 months apart with a long-pulsed tunable dye laser. RESULTS: The lesions flattened after the initial treatment and resolved after a second treatment. No scarring was detectable and no recurrence was noted in follow-up after 1 year. CONCLUSION: We conclude that the long-pulsed tunable dye laser can be employed successfully to treat superficial lesions of ALHE, particularly in cosmetically sensitive areas.
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keywords = hyperplasia
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