Kimura's disease as a mimicker of cavernous hemangioma: A case report and literature review.

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 Diagnosed in the Department of Orthopedic Surgery Treated With Wide Excision: Report of Two Cases.

BACKGROUND/AIM: Kimura's disease is a rare chronic inflammatory disorder that commonly affects the head and neck regions, occurring predominantly in Asian men. Elevated eosinophil count and IgE levels in the peripheral blood examination are suggestive of this disease. In this study we report two cases of Kimura's disease, treated with wide excision. CASE REPORT: The first case was a 58-year-old man presented with asymptomatic left neck mass. The second case was a 69-year-old man with swelling of the right upper arm, which was suggestive of soft tissue mass. Needle biopsy results were suggestive of Kimura's disease in both cases. Elevated WBCs at 8,380/µl (neutrophils: 45%, eosinophils: 33%) for the first case and 5,370/µl (neutrophils: 61.8%, eosinophils: 3.5%) for the second one, and serum IgE at 14.988 IU/ml for the first case and 1,315 IU/ml for the second one were observ. For definitive treatment and diagnosis, wide excisions were performed. Final histopathological results revealed Kimura's disease. Surgical margins were negative even though an ill-demarcated lesion for the first case and high infiltration to the muscle for second case were confirmed. CONCLUSION: Wide excision was performed in both cases of Kimura's disease and no recurrence was observed until the final follow-up. Wide excision with negative surgical margin should be recommended for the treatment of Kimura's disease.

Angiolymphoid Hyperplasia of the External Auditory Canal; Reconstruction of a Complex Defect.

Angiolymphoid hyperplasia (AH) was first described by Wells and Whimster in 1969 as a benign vasoproliferative pathology with a varied infiltrate of eosinophils, lymphocytes, and plasmatic cells. Clinical presentation has been described in the literature as small red-bluish nodules, less than 3 cm in diameter that can bleed in 25% of the cases and be pruritic and painful in 37% and 20% of the cases, respectively. Particularly, AH can appear in the ear; nevertheless, other regions have been affected, including the scalp, lips, tongue, orbit, muscle, and bone. Most of these cases have occurred in adults with an unknown etiology; however, an inflammatory process has been associated in approximately 20% with eosinophilia. No malignancy has been reported.

Enfermedad de Kimura en paciente no asiática / Kimura’s disease in a non-asian patient

La enfermedad de Kimura es un trastorno inflamatorio benigno poco frecuente, de etiología desconocida y que afecta principalmente a sujetos de origen asiático. Clínicamente se manifiesta como masas subcutáneas, indoloras, sobre todo en la zona de cabeza y el cuello. Suele acompañarse de linfadenopatías, y aumento de los niveles de eosinófilos e IgE en sangre periférica. Se presenta un caso de enfermedad de Kimura, diagnosticado en una mujer caucásica tras la exéresis-biopsia de una masa mandibular de gran tamaño. Describir esta patología ayudará a incluir la misma en el diagnóstico diferencial de masas, sobre todo a nivel cervico-facial, favoreciendo así su adecuado manejo diagnóstico-terapéutico. (AU)

Kimura’s disease is a rare benign inflammatory disorder of unknown etiology that mainly affects individuals of Asian origin. Clinically it manifests as subcutaneous, painless masses, especially in the head and neck area. It is usually accompanied by lymphadenopathies and an increase in eosinophil and IgE levels in peripheral blood. A case of Kimura’s disease is presented, diagnosed in a Caucasian woman after the excision-biopsy of a large mandibular mass. Describing this pathology will help to include it in the differential diagnosis of masses, especially at the cervico-facial area, thus favoring its adequate diagnostic-therapeutic management. (AU)

[Kimura's disease. (Clinical case study)]. / Bolezn' Kimury. (Sobstvennoe nablyudenie).

The author presents his own observation of a patient with Kimura's disease (CD), which is a rather rare chronic inflammatory disease of soft tissues. The results of clinical examination and surgical treatment of this disease are presented. It is emphasized that for the diagnosis of CD, first of all, an excision biopsy of the lesion is necessary, as well as the presence of eosinophilia in the patient, an increased level of immunoglobulin E (IgE) in peripheral blood, an increase in regional lymph nodes.

Kimura's disease of the left arm.

Kimura's disease is a rare, benign entity that causes subcutaneous angioblastic lymphoid hyperplasia with eosinophilia. It usually presents with subcutaneous lymphoid swellings with regional lymphadenopathy and salivary gland masses. Kimura's disease is frequently associated with renal involvement, which includes proteinuria and nephrotic syndrome as the most common presentations. This report presents a case of a man in his early 20s with a swelling in the medial aspect of the distal left arm with two previous episodes of nephrotic syndrome. Multiple enlarged axillary lymph nodes and epitrochlear nodes were noted. Ultrasonography revealed a mixed echogenic mass with enlarged lymph nodes. MRI showed a heterogeneous hyperintense lesion. Image-guided fine-needle aspiration showed multiple lymphocytes and eosinophils suggestive of Kimura's disease. The swelling was surgically excised under general anaesthesia. Histopathology of the excised mass confirmed the diagnosis of Kimura's disease.

[Kimura's disease mimicking primary neoplasm of the parotid gland: A case report]. / Enfermedad de Kimura simulando neoplasia primaria de glándula parótida: Reporte de caso.

Background: Kimura's disease is an infrequent inflammatory disorder, of unknown etiology, with few reports outside of Asia. It presents as a nodule or tumor predominantly in the postauricular region, neck and parotid gland. It is histologically characterized by follicular hyperplasia with wellformed mantle zones, preservation of nodal architecture, prominent eosinophilic infiltrate in the germinal centers and interfollicular areas; and associated with elevated levels of IgE and peripheral eosinophilia. Clinical case: We present a case of a 23-year-old man from Mexico, he presented with a recurrent tumor in the right parotid gland, previously treated with surgical resection. Imaging studies were performed and a primary neoplasm of the salivary gland was suspected, he was treated with surgical resection. The histological diagnosis was Kimura's disease. Conclusions: Communication and divulgation of this rare inflammatory disorder expans the knowledge for the differential diagnosis of tumors of the head and neck, and salivary glands, mainly in men with peripheral eosinophilia and elevated IgE; it can sometimes simulate malignant neoplasms, leads to inadequate diagnostic and therapeutic approaches.

Introducción: la enfermedad de Kimura es un desorden inflamatorio poco frecuente, de etiología desconocida y raramente reportado fuera del continente asiático. Se presenta como un nódulo o tumor predominantemente en la región retroauricular, cervical o glándula parótida. Se caracteriza histológicamente por hiperplasia folicular con zonas del manto bien formadas, preservación de la arquitectura ganglionar, infiltrado eosinofílico prominente en los centros germinales y áreas interfoliculares; generalmente asociada a niveles elevados de IgE y eosinofilia periférica. Caso clínico: presentamos el caso de un hombre de 23 años, de origen mexicano que se presentó con un tumor recidivante a dos años de resección quirúrgica previa en glándula parótida derecha, se realizaron estudios de imagen y se sospechó de neoplasia primaria de glándula salival, fue tratado con resección quirúrgica. El diagnóstico histológico fue de enfermedad de Kimura. Conclusiones: la comunicación y difusión de este raro desorden inflamatorio amplía la base del conocimiento para el diagnóstico diferencial de tumores de cabeza y cuello, y glándulas salivales, predominantemente en hombres con eosinofilia periférica y elevación de IgE; que en ocasiones puede simular neoplasias malignas, llevando a abordajes diagnósticos y terapéuticos inadecuados.

Kimura's disease successively affecting multiple body parts: a case-based literature review.

BACKGROUND: Kimura's disease is a rare, benign, chronic inflammatory disease that presents as painless, solid masses mainly affecting the deep subcutaneous areas of the head and neck, especially the salivary glands, parotid glands and nearby lymph nodes. It is characterized by elevated peripheral blood eosinophil and Immunoglobulin E (IgE) levels. CASE PRESENTATION: A 31-year-old Asian male presented with an orbital space-occupying lesion lasting for 1.5 years. Ten years prior, surgical excision of bilateral fossa cubitalis and groin masses was performed, and the pathological examination showed "lymphoproliferative disease". One year later, masses reappeared near the surgical sites; they grew slowly and shrank after glucocorticoid treatment. At this point, admission examinations showed in the peripheral blood an eosinophil proportion of 13.4%, a total IgE level of 26,900.00 IU/mL, prurigo present on the whole body, and multiple palpable masses near the bilateral fossa cubitalis and groin. The left eyeball was exophthalmic. The left elbow mass was excised, and the pathological examination confirmed Kimura's disease. Oral glucocorticoid therapy is taken and tapering regularly. The eosinophil count returned to normal, the IgE level gradually decreased, the orbital space-occupying lesion and elbow and groin masses shrank significantly, and the whole-body skin prurigo disappeared. Currently, the patient has been in a stable condition for eighteen months. CONCLUSION: Our case provides a novel insight that Kimura's disease should be involved in the differential diagnosis of inflammatory lesion mass of orbit and also supports systemic regular glucocorticoid as a valuable therapy of such condition, but close follow-up and long-term observation are crucial.

Treatment algorithm for Kimura's disease: A systematic review and meta-analysis of treatment modalities and prognostic predictors.

BACKGROUND: Kimura's disease is a rare, chronic inflammatory condition that usually manifests as highly recurrent head and neck tumors. OBJECTIVE: Systematic review of recurrence predictors following surgical excision. MATERIALS AND METHODS: The pathologically confirmed cases at the tertiary medical center were reviewed. PubMed, Medline, the Cochrane Library, Web of Science, Airiti Library containing grey literature were searched through August 31st, 2019. RESULTS: A total of 31 articles were included for meta-analysis which revealed that surgical excision resulted in a lower recurrence rate (pooled odds ratio [POR] = 3.15, 95% confidence interval [CI] = 1.12-8.82; p = 0.03) than conservative measures. Surgery was an effective single treatment modality for patients with tumors smaller than 3 cm (POR = 2.89, 95% CI: 1.20-6.95; p = 0.02), symptom duration shorter than 5 years (POR = 3.11, 95% CI, 1.03-9.38; p = 0.04), peripheral blood eosinophilia less than 20% (POR = 4.49, 95% CI: 1.46-13.84; p = 0.009) or serum IgE level less than 10000 IU/ml (POR = 8.30, 95% CI: 1.05-65.34; p = 0.04). CONCLUSIONS: Directing patients with Kimura's disease through the treatment algorithm will reduce the recurrence rate. Combination adjuvant therapy with surgery is recommended for the following conditions -- a tumor greater than or equal to 3 cm in size, symptom duration longer than or equal to 5 years, peripheral blood eosinophilia greater than or equal to 20%, or serum IgE greater than or equal to 10000 IU/ml to achieve the optimal therapeutic outcome. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42020173258 (http://www.crd.york.ac.uk/PROSPERO).

Epithelioid hemangioma of liver-The first reported case.

Epithelioid hemangioma, otherwise known as angiolymphoid hyperplasia with eosinophilia, is a rare benign vasoproliferative disease with an unknown etiology. We report the case of a 42-year-old man with routine bloods test showing mildly elevated serum bilirubin level. CT scan revealed a lesion in the left liver lobe not typical of FNH. PET/CT scan and a dynamic liver MRI were consistent with a malignant mass. Surgical resection was performed. Histopathology of the 45 mm mass reported well-defined, slightly lobular proliferations of capillary-sized vessels around several central muscular vessels. Although the endothelial cells revealed a "hobnail" appearance, none of them showed pleomorphism or mitotic activity. Endothelial cells showed reactivity for the endothelial markers (CD34, CD31) and smooth muscle was detected in the blood vessel walls via immunohistochemistry. Despite its benign nature, epithelioid hemangioma of the liver can lead to a diagnostic and therapeutic dilemma due to the malignant looking features on imaging modalities.

Recurrent angiolymphoid hyperplasia with eosinophilia during several pregnancies.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign vascular tumor. Although its etiology is not clearly known, infection, hormonal factors, and trauma are the suspected reasons. There are few cases considered to be related to pregnancy described in the literature. Granulomas are rarely seen in the histopathology and only four patients with granulomas have been described. Herein, we will present a 28-year-old woman who has been pregnant three times; in every pregnancy, she developed pink nodular lesions on the back of her ear. In the first two pregnancies, the lesions had appeared during the pregnancy and regressed completely in the postpartum period. In her third pregnancy, the condition emerged again and lasted 1.5 years after birth of her child. Histopathology exhibited ALHE with granulomas. Although the relationship between ALHE and pregnancy is already known, there are few cutaneous ALHE cases associated with pregnancy reported in the literature. This association is further supported by this case having recurrent ALHE during each pregnancy period. Also, the presence of naked granuloma in histopathology, unlike most of the cases, contributes to the current histopathological data.

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.

Clinical analysis of Kimura's disease in 24 cases from China.

BACKGROUND: We reviewed details of Chinese Kimura's disease (KD) cases. A full clinical analysis was subsequently performed to improve the accuracy of clinical diagnosis and treatment of KD. METHODS: A total of 24 patients with pathologically confirmed KD treated between March 2008 and March 2018 were reviewed retrospectively for clinical and histopathological analysis. RESULTS: In the 24 KD cases, 20 were male and 4 were female with the age of onset ranging from 5 to 65 years. Lesion diameter ranged from 0.6 cm to 7 cm with unilateral involvement being more popular (79%). Imaging examination had a high detection rate for KD involving the parotid gland and subcutaneous but had low specificity. Microscopic analysis indicated that KD mainly involved subcutaneous soft tissue and lymph nodes. The prominent feature of lymphoid tissue was germinal center hyperplasia surrounded by several lobules associated with hyperplastic vascular structures. Out of the 24 patients, 11 experienced recurrence of disease after treatment (surgical resection: 46.2%, surgical resection followed by oral corticosteroids: 71.4% and surgical resection combined with radiotherapy: 0%). CONCLUSIONS: Our analysis revealed clinical, imaging, and histological characteristics of KD. A better understanding of the disease will help clinicians reduce misdiagnosis and improve the diagnostic rate upon patient first clinical visit.

Kimura's disease involving bilateral lacrimal glands and extraocular muscles along with ipsilateral face: A unique case report.

A 23 year female presented with bilateral recurrent swelling of eyelids along with ptosis and proptosis for last 3 years. She also had swellings over the right cheek, parotid gland, and retro auricular area along with regional lymphadenopathy. Systemic laboratory workup revealed raised serum IgE and a high peripheral eosinophil count. Computed tomography and magnetic resonance imaging showed bilateral enlargement of extraocular muscles, lacrimal glands, and ipsilateral parotid gland. Excision biopsy of the retro-auricular lymph node was suggestive of Kimura's disease (KD). The patient responded well to systemic corticosteroid. KD rarely affects orbit, but it should be included in the differential diagnosis of inflammatory diseases of the orbit. To our knowledge, this is the first reported case of KD from India involving the orbit, lacrimal gland, extraocular muscles, parotid gland and buccal area.

Extramedullary Hematopoiesis: An Unusual Finding in Angiolymphoid Hyperplasia With Eosinophilia.

Extramedullary hematopoiesis is the process of blood cell synthesis occurring outside the medulla of the bone marrow. During fetal development, extramedullary hematopoiesis is considered physiological; however, it is considered pathologic when occurring outside the neonatal period. Angiolymphoid hyperplasia with eosinophilia (ALHE) describes a lesion characterized histologically by plump endothelial cells associated with a mixed inflammatory infiltrate of lymphocytes, plasma cells, mast cells, and eosinophils. This report describes an adolescent boy with an isolated preauricular facial lesion. After excision, histopathology confirmed a diagnosis of ALHE with the additional finding of trilineage extramedullary hematopoiesis. To the authors' knowledge, this is the first reported case of extramedullary hematopoiesis in ALHE.

Angiolymphoid Hyperplasia With Eosinophilia: New Concept to Lower Recurrence.

Angiolymphoid Hyperplasia with Eosinophilia (AHLE) is a rare benign vascular disorder of unknown etiology. While World Health Organization recognizes it as a benign neoplasm, its progressive nature and high recurrence rate are consistent with being a locally malignant tumor. It may present as solitary or multiple nodules with more predilection to head and neck region, especially auricular area. Despite variety of available treatment options, high recurrence rate remains a compelling issue. The spectrum of these treatment modalities includes topical or systemic steroids use, intralesional injection of certain materials such as Bleomycin, Vinblastine or interferon, or even surgical excision.In our study, the authors present a case of middle-aged female with recurrent AHLE in left concha and preauricular region. Excision of the lesion with 4 mm of the normal surrounding skin, preserving auricular cartilage, was done while the resultant defect was covered by grafted Temporoparietal Fascia Flap. Our 1-year follow-up period showed aesthetically accepted outcome with no recurrence.The authors hypothesize that treating AHLE as a locally malignant tumor by excising the lesion with 4 mm safety margin decreases recurrence rates. Moreover, reconstruction by grafted temporoparietal fascia flap achieves a reasonable aesthetic outcome with minimal donor site morbidity.

Combination of electrocoagulation and photodynamic therapy for angiolymphoid hyperplasia with eosinophilia in the external ear.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an inflamed vascular tumefaction of uncertain pathogenesis and is characterized by higher recurrence rates after surgical excision or other approaches. In the present study, we performed a new approach by combining electrocoagulation with photodynamic therapy (PDT) for the treatment of two ALHE patients. They had multiple treatments in the past and had poor therapeutic effects with relapse each time. After informed consent was obtained, electrocoagulation was used to remove the superficial lesions of ALHE, and the first session of PDT was immediately applied to the lesion. A total of three sessions of PDT were applied to each patient with an interval of 7-10 days. Through more than 12 months of follow-up, the two patients showed complete regression, and no recurrence was observed. Meanwhile, patients were very satisfied with the cost and cosmetic outcomes of the combination treatment. Based on our results, we strongly recommend the combination of electrocoagulation with PDT as a favourable treatment for ALHE, especially in the external ear and other areas that are inconvenient for routine surgery.

A fleshy papule on the eyelid.

Angiolymphoid hyperplasia with eosinophilia is an uncommon tumor affecting the head and neck region. It usually presents as solitary or multiple erythematous or brownish papules and nodules. It is considered a reactive angioproliferative disorder by some, whereas others believe it to be a neoplastic growth. Involvement of the eyelid is a rare occurrence. We report an instance of angiolymphoid hyperplasia with eosinophilia involving the eyelid in a 19-year-old woman with review of literature.