Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.

Kimura disease is a rare chronic inflammatory disorder mostly reported in males in second to third decades from south-east Asia. Head and neck is the most commonly involved region. The usual presentation is painless facial swelling with salivary gland involvement and regional lymphadenopathy. The diagnosis can be suspected on imaging but needs to be proven on histopathology. We describe three histopathology proven cases of Kimura disease and emphasize the specific clinic-radiological findings which can help in early identification of the condition and its differentiation from other diseases with similar presentation. Gray scale Ultrasound, contrast-enhanced ultrasound, and contrast-enhanced Magnetic Resonance Imaging (CE-MRI) were done in all patients and Computed Tomography (CT) was done in one. The salient imaging features were ill-defined solid mass in the subcutaneous location of the facial region which was heteroechoic on Gray scale Ultrasound, heterogeneously hyperintense on T2W MRI and without much diffusion restriction. There was involvement of salivary glands with ipsilateral cervical adenopathy. The lesion showed avid contrast enhancement on contrast-enhanced ultrasound and CE-MRI. We also present a systematic literature review of clinico-radiological features in Kimura disease in the head and neck. The articles with histopathologically proven cases and satisfactory description of imaging findings were included and we have compared the findings in our cases with published literature. In addition, we have included examples of radiological mimics of this disease. Certain imaging findings are highly suggestive and can aid in early diagnosis of Kimura disease which helps in early initiation of treatment with better patient prognosis.

Kimura Disease: A Rare Cause of Head and Neck Tumour in Children- A Case Report.

BACKGROUND: Kimura disease is a rare inflammatory condition classically manifested as painless subcutaneous nodules in the head and neck region and associated with regional cervical lymphadenopathy and salivary gland involvement. OBJECTIVE: The purpose of this report is to illustrate the diagnostic difficulty due to its rarity and non-specific clinical presentation. CASE PRESENTATION: We present a case of Kimura disease with bilateral eyelid swelling, parotid involvement, and cervical lymphadenopathy in a young boy. Computed Tomography (CT) showed ill- defined enhancing masses in both upper eyelids with lacrimal gland infiltration, multiple nodular lesions in both parotid glands, and bilateral enlargement of cervical nodes. After several multidisciplinary discussions and trials of medications, Kimura's disease was finally confirmed by histology examination. CONCLUSION: In conclusion, a rare disease such as Kimura must be considered earlier if the patient is not responding to the treatment.

Comparison between Kimura's disease and angiolymphoid hyperplasia with eosinophilia: case reports and literature review.

Kimura's disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura's disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.

Kimura disease of the breast: Case report and literature review of current management.

We report a rare case of Kimura disease in a 50-year old female patient who attended our tertiary level Breast Surgery Clinic.

Kimura disease with nephrotic syndrome in a child- A rare association.

Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.

Kimura's Disease.

Kimura's disease (KD) is a benign, rare and chronic inflammatory disorder of unknown etiology and it affects subcutaneous tissues, lymph nodes, and salivary glands. The clinical manifestation of cases reported is mainly small neoplasms or enlarged lymph nodes, while cases for large masses are rarely reported. This case reveals a female patient of KD with large masses in the head and neck region. The scope of the tumor is huge, which seriously affects the facial shape and psychology of the patient, thereby affecting the daily life of the patient. Although the patient had surgically removed the same site tumor 8 years ago, it was not possible to determine whether it was a recurrent case because the postoperative pathological diagnosis of first treatment was not confirmed. Under the general anesthesia, the right head and neck masses were removed. During the operation, the facial nerve branches were wrapped by the masses. After consulting the family members, it was agreed that the facial nerve should not be preserved for maximum extent removal of the tumor. Although the patient has facial paralysis symptoms after surgery, the facial shape and patient's psychology are greatly improved, and the patient is satisfied with the treatment effect. Surgical treatment is one of the effective ways to treat huge tumors in the head and neck of KD. This study has certain guiding significance for clinicians to treat huge tumors of head and neck in KD.

Rare lesion, unusual location, uncommon presentation: a case of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferation of unknown pathogenesis that may be related to trauma. Although it affects mainly the head and neck, the zygomatic area is rarely involved. We report a case that affected the zygomatic region of a 46-year-old black man. The lesion had been present for about a year and the patient reported that it appeared after a facial injury during a soccer match. Clinical and tomographic investigations suggested a benign tumour, and the lesion was excised through an intraoral approach. Histopathological examination showed the unexpected diagnosis of ALHE. This tumour was interesting because of its rarity, and also because of its unusual site within the head and neck region. The diagnosis of ALHE is hardly ever considered in the differential diagnosis of zygomatic nodules.

An unexpected asymptomatic epiglottal site of Kimura disease.

INTRODUCTION: Kimura disease (KD) is a chronic lymphoproliferative disorder of unknown etiology that affects the skin and lymph nodes, mostly observed in males of Asian descent. The natural history of asymptomatic epiglottal KD remains unknown. This rare site of KD is often only diagnosed when tumor growth starts to obstruct the upper airways. OBSERVATION: A 34-year-old North African male presented with fatigue and multiple, slowly progressive, fluctuating skin nodules in the right mandibular and retroauricular regions. Computed tomography of the head and neck revealed a large soft tissue tumor close to the right mandibular body and unexpected thickening of the epiglottis. Transnasal laryngoscopy confirmed the CT findings and showed thickening of the epiglottis. The diagnosis of KD was based on histological examination of biopsy specimens taken from the right mandibular tumor, a cervical lymph node, and the epiglottis. DISCUSSION: Most cases of KD with epiglottal involvement present with dysphonia and dysphagia. No consensus guidelines are available concerning the complementary investigations that should be performed. This case report raises the question of whether patients with suspected KD should be systematically screened for lesions in unusual and potentially dangerous anatomic sites.

Hypervascular Pseudonodular Plaque-Like Ultrasound Morphology in Angiolymphoid Hyperplasia. / Morfología ecográfica en placa pseudonodular hipervascular en hiperplasia angiolinfoide.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by solitary or multiple angiomatous lesions. It is most common in young or middle-aged women, and the lesions typically affect the head and neck, showing a particular predilection for the periauricular region. The differential diagnosis in patients with ALHE is broad and includes both benign and malignant conditions. We report on a series of cases of periauricular ALHE in which ultrasound imaging revealed an hypervascular, pseudonodular and plaque-like morphology with clinical and histologic correlations. It also evidenced vascular communication between lesions that appeared to be separate on clinical examination. Familiarity with such ultrasound presentations could help to improve diagnostic accuracy and facilitate disease monitoring in patients with ALHE.

Angiolymphoid Hyperplasia With Eosinophilia of the Lung / Hiperplasia angiolinfoide con eosinofilia del pulmón

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Kimura's Disease Presenting As Scrotal Mass: A Difficult Diagnosis.

Herein we report a case of Kimura's disease with unusual manifestations. A 46-year- old Chinese man presented with mass in scrotum which gradually increased in size for approximately 7 years. A computerized tomographic scan of abdomen revealed a soft-tissue density shadow in scrotum and enlargement of lymph nodes in groin and retroperitoneum. The scrotal mass was excised and the biopsy specimen revealed angiolymphoid hyperplasia with infiltration of eosinophils. In conclusion, clinical doctors should pay attention that the patient with eosinophilia and scrotal mass could be indicative for Kimura's disease.

Angiolymphoid Hyperplasia With Eosinophilia Within a Vascular Malformation: Case Report and Review of the Literature.

OBJECTIVES: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign microvascular proliferation tumor. The etiology of ALHE is unknown, though some hypothesize it occurs in reaction to focal trauma. This study presents a case of AHLE within a vascular malformation, its treatment, and a review of the literature. METHODS: A retrospective case report was performed with a 4-year follow-up. Medical records including clinic notes, radiography, operative reports, pathology, and long-term follow-up were reviewed. RESULTS: Radiologic imaging revealed a vascular malformation with highly tortuous, corkscrew-shaped arterial vessels. Histology revealed midsized vessels with plump, epithelialized endothelium and focal areas of lymphocytic infiltrate punctuated with eosinophils, consistent with ALHE. CONCLUSION: This case demonstrates a unique presentation of ALHE in association with vascular malformation. Initial physical exam lacked the classic dermatologic presentation of clusters of red-to-brown nodules; however, the corkscrew vessels lined by epithelized endothelial cells and eosinophilic infiltrate were pathognomonic for ALHE.

An Unusual Clinical Presentation of Kimura Disease Manifesting with a Typical Cephalocervical Lesion and an Atypical Subcutaneous Hip Mass Lesion.

Kimura's disease (KD) is a rare lymphoproliferative inflammatory disorder, typically presenting as firm, painless or pruritic single or multiple subcutaneous lesions in the head and neck, especially in the parotid and submandibular regions. We herein report a case of a 39-year-old Japanese man presenting with a typical cephalocervical KD lesion around the salivary glands with a rare association with a distant subcutaneous mass at the hip. We also emphasize the radiologically and clinically important features in the differential diagnosis and management. To the best of our knowledge, this case is the first report of KD manifesting with a typical cephalocervical lesion and an atypical subcutaneous hip mass lesion.

Kimura disease: No age or ethnicity limit.

Kimura disease is a chronic inflammatory disease that mainly manifests as a lump in the cervical region. Although the underlying pathophysiology is not clear yet, the diagnosis can be established based on specific histopathological characteristics. The first case of this disease was described in China, as well as the majority of subsequent cases that were also described in the Far East countries made Kimura disease traditionally a disease of adult patients of Asian descent. This report describes the occurrence of Kimura disease in pediatric non-Asian patient with a similar clinicopathologic presentation.

Co-existence of lip and epiglottis Kimura's disease.

Kimura's disease is a rare, chronic inflammatory condition of unknown etiology; with a predilection in the head and neck region. However, the involvement of the lip and epiglottis is extremely rare, and poses a diagnostic challenge. Here, we report a case of a 32-year-old Saudi male presenting with lip mass and epiglottic swelling without any history of hoarse voice or airway compromise. Serology showed elevated immunoglobulin E levels, and histopathological examination of biopsied lesions revealed well-developed lymphoid follicles with eosinophilic infiltration confirming Kimura's disease. At the time of last follow-up, his condition was satisfactory without any signs of recurrence.

Kimura's disease associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease: a case report and review of the literature.

A 33-year-old Chinese man with 9-year history of Kimura's disease (KD) was admitted with a 1-month history of recurrent claudication. He did not have any clinical discomfort and had not taken any preventive medication in the past. He accepted percutaneous transluminal angioplasty and the pathologic diagnosis was reportedly consistent with necrotizing eosinophilic vasculitis. This is the rare reported case of KD associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease and the difficulties encountered in establishing an accurate diagnosis with unusual presentations. This case also highlights the possibility of recurrent complications without aggressive medical treatment in such unusual eosinophilic disorders.

Ultrasonographic findings of Kimura's disease presenting in the upper extremities.

OBJECTIVE: To describe ultrasound findings of Kimura's disease arising in the upper extremities. MATERIALS AND METHODS: Five patients with Kimura's disease confirmed by surgical resection were retrospectively reviewed by two musculoskeletal radiologists and a pathologist. RESULTS: All six lesions involved the epitrochlear area and appeared as partially (n = 5) or poorly (n = 1) marginated subcutaneous masses with the presence of curvilinear hyperechoic bands intermingled within the hypoechoic components by US. Moderate (n = 4) to severe (n = 2) vascular signals were observed in some proportion of the hyperechoic bands by color Doppler US. The associated findings were the increased echogenicity of surrounding subcutaneous fat (n = 6) and adjacent lymphadenopathy (n = 4). Microscopic examination showed proliferation of lymphoid follicles with prominent germinal centers and intervening fibrosis. CONCLUSIONS: In this study, Kimura's disease arising in the upper extremities showed a partially defined hypoechoic subcutaneous mass with internal hyperechoic bands and moderate-to-severe vascularities, increased echogenicity of the surrounding subcutaneous fat and adjacent lymphadenopathy on US. Thus, when these US features are observed in the typical epitrochlear region of an Asian individual, especially if accompanied by peripheral eosinophilia, Kimura's disease should be considered as a possible diagnosis.