Kimura's Disease

Kimura's disease: A clinicopathologic study of 54 chinese patients

Much controversy has existed with regard to the relationship between Kimura's disease and angiolymphoid hyperplasia with eosinophilia. They were initially thought to represent the same disease spectrum, but it has now been widely accepted that they are two separate disease entities. To our knowledge, there have been no reports to date describing a case of both Kimura's disease and angiolymphoid hyperplasia with eosinophilia coexisting in the same patient. We describe a patient presenting initially with a right postauricular subcutaneous swelling and subsequently developing multiple erythematous facial papules and nodules. The medical literature is reviewed. Clinical features of the right postauricular subcutaneous swelling and multiple erythematous facial papules/nodules suggest Kimura's disease in the former and angiolymphoid hyperplasia with eosinophilia in the latter. Histopathological examinations of these lesions helped to confirm the diagnosis of Kimura's disease and angiolymphoid hyperplasia with eosinophilia, respectively. Kimura's disease and angiolymphoid hyperplasia with eosinophilia can coexist in the same patient. Coexistence of the two types of lesions in one patient may also be considered evidence that Kimura's disease and angiolymphoid hyperplasia with eosinophilia form a spectrum in one disease.

Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura disease were previously considered the same entities and are now considered a distinct disorder clinically and histologically. ALHE is a benign vasoproliferative disorder with unclear etiology. The clinical presentation of ALHE includes the involvement of skin and vascular structures sparing lymph nodes. It predominantly involves the head and neck region, extremities, and rarely orbit, oral mucosa, bones, and colon. On the other hand, Kimura disease is a rare benign chronic inflammatory disorder of unknown etiology that predominantly involves subcutaneous lymphoid masses and regional lymph nodes of the head and neck region. Both disorders are classified under hypereosinophilia (HE); however, Kimura disease is more associated with peripheral eosinophilia. It is tough to differentiate both the disorders clinically from each other and also from other HE syndromes including eosinophilic granulomatosis with polyangiitis and systemic HE syndromes. However, tissue diagnosis is the key to differentiation. Here, we describe a female at her 50s without any prior comorbidities, presented to our OPD with atypical multiple symmetrical soft tissue swellings which were of diagnostic dilemmas. She showed features of both ALHE and Kimura disease in investigations. As there is no specific recommendation for treatment, she was started with oral glucocorticoid and weekly methotrexate showing a good response in follow-up visit.

The clinicopathologic features of nine patients with Kimura's disease and 15 patients with angiolymphoid hyperplasia with eosinophilia (ALHE) were studied and compared in order to clarify the confusion between these two entities. The common features shared by both conditions included male predominance, predilection for the head and neck regions, tendency to recur, and vascular nature of the lesion with lymphoid and eosinophilic infiltrates. However, Kimura's disease was usually seen in younger individuals for a longer duration and occurred as a deeply seated, large soft-tissue mass, without significant change of the overlying skin initially. In addition, it was often accompanied by peripheral blood eosinophilia and elevated serum IgE. In contrast, ALHE lesions were multiple small dermal papular or nodular eruptions observed in older patients and present for a shorter duration; they were less frequently accompanied by peripheral blood eosinophilia. The main histopathological difference was the presence of "histiocytoid" or "epithelioid" blood vessels in ALHE but not in Kimura's disease. Kimura's disease was further characterized by eosinophilic folliculolysis; IgE deposits in the germinal centers; and frequent involvement of regional lymph nodes, salivary glands, and skeletal muscles. The eosinophilic infiltration, especially the formation of eosinophilic microabscesses, along with increased number of small blood vessels, perinodal eosinophilic infiltration, and eosinophilic folliculolysis characterized the nodal involvement by Kimura's disease. Our study indicates that Kimura's disease and ALHE are two distinct clinicopathologic entities. We place particular emphasis on the involvement of regional lymph nodes in Kimura's disease. In addition, we observed Charcot-Leyden crystals and polykaryocytes in both conditions. One of the patients with Kimura's disease also had an associated nephrotic syndrome.

The analysis of published clinical observations on the diagnosis and treatment of patients with Kimura's disease, which is a rather rare chronic inflammatory disease of human soft tissues of unknown etiology, is presented. The onset of Kimura's illness is hardly noticeable. The disease proceeds sluggishly, with a varied clinical picture, most often with the formation of single or multiple subcutaneous nodules or tubercles and swelling in the head and neck on one or both sides. Other localizations of lesions are also described: limbs, orbit, eyelids, palate, pharynx, armpit, chest, inguinal region. The affected areas gradually increase in size over many months or years. Other clinical manifestations of Kimura's disease are presented in the form of edema of the extraocular muscle, juvenile temporal arteritis, sleep apnea in cases of damage to the larynx, and life-threatening coronary artery spasm in children. There are reports of other rare systemic clinical signs of Kimura's disease in the form of pruritus, eczema and rash. The disease is often associated with regional lymphadenopathy and/or salivary gland involvement, eosinophilia, and an increase in serum immunoglobulin E from 800 to 35,000 IU/mL. The disease may be accompanied by kidney damage in the form of glomerulonephritis and nephrotic syndrome. Most reported cases of Kimura's disease occur in young Asian men in their 20s and 30s, sporadically the disease is diagnosed in Europeans, North Americans, Caucasians, and Africans. Diagnosis of Kimura's disease is often clinically difficult. It is primarily based on excisional biopsy of the lesion or regional lymph nodes, and the histopathological findings are consistent regardless of the location of the lesion. Morphologically, Kimura's disease is characterized by the presence in biopsy specimens of such histological features as reactive follicular hyperplasia with prominent follicles surrounded by fibrous tissue. Interfollicular infiltrates are rich in plasma cells and eosinophils, which form diffuse eosinophilic microabscesses associated with granulomatous inflammation. The vessels are numerous, with an increase in postcapillary venules. At the same time, the blood vessels remain thin-walled, with cuboidal endothelial cells. Treatment issues are highlighted, and differences between Kimura's disease and angiolymphoid hyperplasia with eosinophilia are presented.

Selection de resumes interessant les acquisitions nouvelles en pathologie tumorale des tissus mous au cours des annees 88 et 89: description de 2 nouvelles entites. La tumeur fibrohistiocytaire plexiforme et le myofibroblastome palissadique, distinction entre haemangiome epithelioide et maladie de KIMURA et interet de l'anticorps anti EMA comme outil diagnostique courant dans le cadre des tumeurs des tissus mous

Kimura's disease is a rare chronic inflammatory condition of uncertain etiology which has an affinity for the Asian population. It primarily involves the head and neck region, presenting as deep subcutaneous masses and is often accompanied by regional lymphadenopathy and salivary gland involvement. Peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels are characteristic features and the microscopic picture reveals lymphoid proliferation with eosinophilic infiltration. For years, Kimura's disease was believed to be identical to or part of the same disease spectrum as angiolymphoid hyperplasia with eosinophilia (ALHE). Recent reports, however, have confirmed that the two are, in fact, separate entities. We report a case of Kimura's disease in a 22-year-old Indian male who presented with a subcutaneous mass, parotid enlargement and lymphadenopathy. The clinical presentation was suggestive of Kimura's disease and microscopic examination following biopsy of the lesion allowed us to make a definitive diagnosis.

Although Kimura's disease has often been considered to be identical to angiolymphoid hyperplasia with eosinophilia (epithelioid haemangioma), recent studies suggest that they are different clinicopathological entities. In this study, we have made a detailed morphological comparison of 10 cases of epithelioid haemangioma and 40 cases of Kimura's disease occurring in the Chinese population. The epithelioid haemangiomas occurred in the subcutaneous tissue, skin and maxillary antrum, whereas Kimura's disease affected the subcutaneous tissue, major salivary glands and lymph nodes. Distinctive features of epithelioid haemangiomas were exuberant proliferation of vessels lined by cuboidal to hobnail endothelial cells with irregular nuclei and cytoplasmic vacuoles, fibromyxoid matrix, involvement of muscular coat of blood vessels and zonation of inflammatory infiltrate towards the peripheral portion of the lesion. Distinctive features of Kimura's disease were florid lymphoid infiltrate with prominent lymphoid follicles, vascularization of germinal centres, germinal centre necrosis, marked eosinophilia with or without eosinophil abscess formation, proliferation of high endothelial venules, and sclerosis. The histological features suggest that epithelioid haemangioma is a proliferation of atypical endothelial cells, possibly neoplastic, that is associated with a variable inflammatory infiltrate, whereas Kimura's disease is primarily an inflammatory condition in which high endothelial venules are usually found.

Kimura Disease of the Orbit and Ocular Adnexa

Microcirculatory systems, which can be divided into several segments, have crucial physiological functions. We investigated whether monoclonal antibody against alpha-smooth muscle actin (alpha SMA) was useful for the identification of microcirculatory segments, according to the structure of their periendothelial cells, in two pathological cutaneous conditions. We examined skin specimens from patients with Kimura's disease and with angiolymphoid hyperplasia with eosinophilia, since little information is available on microvascular segments in these conditions. Immunostaining for alpha SMA revealed the morphological characteristics of the periendothelial cells clearly enough to identify five segments in the proliferative microvessels of Kimura's disease and angiolymphoid hyperplasia with eosinophilia. In Kimura's disease, postcapillary venules were predominant, while each vascular segment in angiolymphoid hyperplasia with eosinophilia was uniform. Vessels without periendothelial cells were detected to a greater extent in angiolymphoid hyperplasia with eosinophilia than in Kimura's disease. The antibody against alpha SMA appeared to be useful in the observation of periendothelial cells for the identification of vascular segments in pathological cutaneous conditions.

Kimura's disease: imaging patterns on computed tomography

Background: Kimura disease is a rare form of chronic inflammatory disorder with unknown etiology. It occurred at subcutaneous tissues, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and salivary gland in the East. Aim and Objectives: The purpose of this paper is to present clinical picture of our cases with literatures review. Materials and Methods: We reported and analyzed 16 cases of Kimura disease from 1996 to 2005 in our hospital, including 14 males and 2 females. Besides, a review of publications in English or Chinese (MEDLINE 1966-2006) as Kimura ('s') disease was performed and compared to our series. Results: The disease presented as either single or multiple lesions over head and neck region, and mainly involved lymph nodes, subcutaneous tissues, and major salivary glands in isolation or in combination. Most cases had peripheral blood eosinophilia but no renal involvement was found. All patients have received at least tumor excision once and oral steroid were used in 5 patients. Follow-up data showed recurrence on 5 patients. Conclusion: The clinical presentation and histopathology of our cases is in accordance with other Kimura cases reported in the literatures and the overall prognosis of Kimura disease is good except high recurrent rate. It remains a therapeutic enigma until its pathogenesis is resolved. In our opinion, complete surgical excision whenever feasible is the preferred treatment despite a not low recurrence rate, especially when the lesions are disfiguring. In the case of head and neck diffuse infiltrating or repeated recurrence, conservative surgical excision is also the first choice.

Kimura's disease is a rare chronic inflammatory disorder with an unknown etiology which presents with the involvements of salivary glands and subdermal tissue in the head or neck region, in particular. Besides the most common manifestations of the disease; as the involvements of preauricular region, submandibular region, cervical lymph nodes and major salivary glands, nephrotic syndrome, as an additional systemic pathology, may be also seen due to glomerulonephritis. The differential diagnosis is made with the identification of characteristic histological features as follicular hyperplasia, eosinophil infiltration, and proliferation of the postcapillary venules. Although Kimura's disease may occasionally resemble angiolymphoid hyperplasia with eosinophilia, both have different clinical and histological features. In this case report, we discuss the clinical, histopathological features and treatment of a 57-year-old male patient admitted with a slowly progressing mass in his right buccal region for almost two years, which was approximately 3x3 cm in diameter and soft to palpation, and was excised completely by an intraoral approach and reported histopathologically as Kimura's disease in the light of literature.

Kimura's disease is a chronic inflammatory condition producing subcutaneous tumor-like nodules, mainly in the head and neck region. Most patients have involvement of regional lymph nodes and major salivary glands. The authors present two cases of Kimura's disease with parotid gland and postauricular lymph node involvement. With Tl-201 SPECT, elevated uptake was noted on early and delayed images. Kimura's disease should be included in the differential diagnosis when increased Tl-201 uptake in head and neck mass lesions is noted.

Rare case of Kimura disease of the upper lip: A case report