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1/65. 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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2/65. 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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3/65. Kimura's disease: a case report.

    Kimura's disease is a rare, idiopathic condition that usually affects young men of Asian descent. The decrease is characterized by swelling and lesions in the head and neck region, with involvement of the subcutaneous soft tissue, major salivary glands, and lymph nodes. patients almost always have eosinophilia and elevated serum immunoglobulin e levels. The diagnosis is established by biopsy. Kimura's disease is usually self-limiting. Its etiology is unknown but is thought to be a manifestation of an aberrant allergic response. In this paper, we describe the case of a 30-year-old patient who was diagnosed with Kimura's disease at our institution.
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4/65. nephrotic syndrome associated with Kimura disease.

    Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.
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5/65. Uptake of 111In-pentetreotide and 99mTc-labeled autologous granulocytes in Kimura's disease.

    Uptake of 111In-pentetreotide (OctreoScan) and 99mTc-labeled autologous granulocytes by the lesions of a 37-year-old female from thailand with Kimura's disease is described. This is a benign chronic inflammatory condition that is endemic in Asians. It is characterized by adenopathy and subcutaneous nodules mostly affecting the head and neck area or the salivary glands. Although these examinations have previously not been described in Kimura's disease, uptake of the radiopharmaceuticals in the lesions can be expected from their histological appearance. With increasing medical, social and economic interactions with Asia, it is important to recognize this cause of adenopathy, including its appearance at various nuclear medicine examinations.
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6/65. Kimura's disease in a teenage male: report of one case.

    A 14-year-old boy gradually developed several subcutaneous masses over bilateral peri-auricular regions, neck, and both upper arms near the elbows over the past 2 years. The recent development of eyelid puffiness elicited his seeking medical attention. hypereosinophilic syndrome was first considered due to the markedly elevated blood eosinophil count without other evidence of allergy or parasite infestation, but the absence of organ involvement made this diagnosis questionable. The histopathological investigation of the subcutaneous mass in the upper arm showed lymph node-like tissue with markedly reactive follicular hyperplasia and eosinophilic abscesses associated with granulomatous inflammation, thus leading to the final diagnosis of Kimura's disease. Oral prednisolone was prescribed successfully. This illness occurs mainly in young oriental males. We report this case to introduce it to pediatric clinicians as an important differential diagnostic entity in eosinophilia-associated diseases and reactive lymphadenopathy.
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7/65. Kimura's disease presenting as subcutaneous facial plaque in an African American.

    Kimura's disease is a benign, uncommon, chronic inflammatory condition that usually presents with painless subcutaneous nodules or plaques in the head and neck region. Although the disease is predominantly found in Asian populations, there are occasional cases reported among Caucasians and rare occurrences in African populations. The etiology and pathogenesis of the disease are unknown and the clinical presentation can mimic several benign and malignant disease states. The accurate diagnosis of Kimura's disease is based on clinical and histopathological findings. There is no evidence of malignant transformation and occasional spontaneous resolution occurs. Various treatment modalities have been suggested in the management of this condition. Oral corticosteroids have been the mainstay of therapy, even though steroid withdrawal can result in lesion recurrence.
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8/65. Kimura's disease: a report of three cases with a brief review of literature.

    Kimura's disease (KD) is an uncommon chronic inflammatory condition of unknown aetiology involving subcutaneous tissue, presenting as a tumor like lesion with a predilection for the head and neck region. Clinically it is often confused with parotid tumor with lymph node metastasis. It is difficult to diagnose before tissue biopsy. Fine needle aspiration cytology has only limited value. Unless the pathologists are aware of this entity, it might be misdiagnosed. Surgery, radiotherapy and steroid therapy have been tried but none is proved best and recurrence is common. Three cases of KD seen in our hospital and the problems encountered in them are presented.
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9/65. Kimura's disease in a chronic hemodialysis patient.

    Eosinophilia is not an uncommon finding in chronic dialysis patients. It is usually benign in nature although definite pathogenesis is unknown. We have encountered a young uremic Chinese adult who developed Kimura's disease after being on maintenance hemodialysis for about 3 years. Asymptomatic eosinophilia had been noted for 1 year and 8 months prior to the development of progressively enlarged neck masses, which leads to the diagnosis of Kimura's disease. In contrast to most cases, eosinophilia was first noticed before the neck masses appeared. There is often a close correlation between Kimura's disease and glomerular disease, where renal involvement is considered as a systemic manifestation. However, we do not have strong evidence to support this relationship between terminal renal failure and Kimura's disease in this patient. To our knowledge, our patient is the first reported case of Kimura's disease occurring in chronic hemodialysis patients. Eosinophilia persisted for nearly 2 years before the neck mass developed and recurred after the excision. Besides, our patient also demonstrated a chronic and recurrent course typical of Kimura's disease.
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10/65. angiolymphoid hyperplasia with eosinophilia of the tongue: report of a case and review of the literature.

    angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon disorder manifested as solitary or multiple nodules in dermis and subcutaneous tissue of head and neck region. ALHE affecting tongue is very rare. We report a 56-year-old man with ALHE of the tongue and review the previously reported cases of ALHE of the tongue.
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