We report a case of kimura s disease in a 65yearold woman who presented with generalised itching, abdominal pain, facial puffiness, difficulty in swallowing and loss of appetite. Chronic inflammatory disorder of unknown etiology endemic in asia, affects young men often subcutaneous mass of head and neck including salivary glands, associated with regional lymphadenopathy but normal overlying skin. It is possible that kimura disease represents a florid, subcutaneously deepseated form of the same basic pathogenetic process that gives rise to classic dermal clh. Etiology and pathogenesisit is possible that kimura disease represents a florid, subcutaneously deepseated form of thesame basic pathogenetic process that gives rise to classic dermal clh. Kimura disease is more common in asianmen, whereas ahle is more common in women. Kimura disease is diagnosed by a surgical biopsy and the cause of the disease is unknown. Kimura disease is an inflammatory disease of unknown etiology that frequently. Read kimura s disease of the epiglottis, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. In some patients, kimura s disease is associated with proteinuria. Simultaneous involvement of lymph nodes occurred in 5. Kimura s disease is a benign, but locally injurious disease with a marked predilection for the head and neck.
Pdf kimuras disease is a unique chronic inflammatory angiolymphoid. Systemic manifestations are 29rarely found in alhe. Kimuras disease and angiolymphoid hyperplasia with. Epithelioid haemangioma angiolymphoid hyperplasia with. Kimura s disease kd is a rare, benign disorder associated with chronic inflammatory lesions of unclear etiology. Kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. Kimuras disease kd is also a rare, chronic inflammatory disorder of unknown aetiology. The purpose of this study was to evaluate the ct and mr imaging findings of kd in the head and neck. Aug 22, 2008 kimura disease is a distinct clinicopathological entity of a benign chronic inflammatory disorder of unknown etiology. Kimura disease is a chronic inflammatory disorder that mainly affects the area of the head and neck. Kimuras disease with atypical musculoskeletal presentation. In contrast to that of kimura s disease, the histology of alhe is typified by an. Elevated serum immunoglobulin e levels and peripheral blood eosinophilia are also common. Kimura s disease, a rare entity in the west but endemic in asia, manifests as solitary or multiple subcutaneous nodules, primarily located in the cervical region.
Kimura disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and. She was found to have generalised lymphadenopathy and a fineneedle aspiration cytology initially done revealed reactive lymphadenitis which was inconclusive. The condition primarily involves the subcutaneous tissues, parotid glands, andor lymph nodes. It is similar to angiolymphoid hyperplasia with eosinophilia. It is characterised histologically by dense fibrosis, lymphoid infiltration, vascular proliferation and eosinophilia. The typical clinical presentation is characterized by painless, sometimes disfiguring, subcutaneous nodules, predominantly in the head and neck. Orbital kimuras disease in a white child british journal. It usually presents as a mass lesion, most commonly in the head and neck region. Angiolymphoid hyperplasia with eosinophilia and kimuras disease a clinical and histopathological comparison.
Read kimura s diseasetreatment with steroid, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Final histopathology picture was of kimura s disease. Nonetheless, the diagnosis requires exclusion of infectious etiologies and neoplastic causes with similar histology. Masses generally appear in a persons mid20s and the disease mainly affects asian men. Kimura disease accompanied with nephrotic syndrome in a 45. The typical clinical manifestations include a triad of painless unilateral cervical lymphadenopathy. It is a rare entity in the west, but endemic in asia. Kimura disease kd is a benign, rare form of chronic inflammatory disorder with angiolymphatic proliferation of unknown etiology involving subcutaneous tissues, affecting a population sporadically. Editor, kimura s disease is an uncommon, chronic inflammatory disorder of unknown aetiology which occurs predominantly in orientals and presents with tumourlike swellings mainly in the head and neck region. Slides are filed for future reference or teachingresearch for at least. Kimura disease is more common in asian men, whereas ahle is more common in women. Kimura s disease kd is also a rare, chronic inflammatory disorder of unknown aetiology. Kimuras disease swarnkar m, agrawal a formos j surg. It is endemic in oriental asians, but sporadic and relatively rare in the west, both in whites and blacks alike.
Clinical analysis of kimuras disease in 24 cases from. Kimura s disease kd is a benign chronic inflammatory disorder attributed to an immunemediated hypersensitivity which often presents as a tumorlike swelling in the head and neck region with or without lymphadenopathy, associated with hypereosinophilia and elevated serum immunoglobulin e. Kimura disease kd is a rare benign chronic inflammatory disease that usually involves deep subcutaneous tissue and lymph nodes of the head and neck region with frequent regional. An open biopsy is the chief means by which this disease is diagnosed. Oct 19, 2018 kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. There has been considerable controversy about the relation between kimura s disease and angiolymphoid hyperplasia with eosinophilia alhe. Orbital cases are infrequent and most reported cases are in adults, with only one case in. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in western countries 1. Kimuras disease is a rare lesion that mimics soft tissue tumours. In kimura disease the lesions are deeperseated, with no initial overlying skin lesions. Kimuras disease kd is a rare and benign chronic inflammatory soft tissue disorder of unknown origin, which predominantly inflicts young male adults in asia. Kimura s disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. It was introduced in china by kim and szeto in 1938, but its name was given by kimura in 1948 from japan.
Sep 12, 2006 kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions. Kd is a rare chronic inflammatory disorder of unknown etiology. It is often accompanied by nephrotic syndrome and is a rare, chronic inflammatory disorder of unknown cause 2. Angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease are. Clinical analysis of kimuras disease in 24 cases from china. Chapter 1 introducton histopathology definition it is a branch of pathology which deals with the study of disease in a tissue section. Castleman disease, drug reactions, parasitic infestations, dermatopathic lymphadenopathy. Lymphoid follicles are hyperplastic and contain prominent germinal. Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions. Kimuras disease is a benign rare chronic inflammatory disorder. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. The most common sites were the subcutis of head and neck, and parotid gland. In the present study, 14 cases of kimura s disease were clinicopathologically studied. After the reports are sent to the surgeons either as soft copy or hard copy, the laboratory has to perform the following functions 1.
Pathogenesis, diagnosis, and management of kikuchifujimoto disease darcie deaver, phd, pedro. Postoperatively, the patient was put on oral cetirizine 10 mg daily, and there was no evidence of recurrence until last followup. Clinical and histopathologic differences soo i1 chun, md, and hye goo ji, md seoul, korea background. Angiolymphoid proliferation and eosinophilia are characteristic findings. It involves the subcutaneous tissue, predominantly in the head and neck and frequently associated with regional lymphadenopathy 1. Kimura s disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. Angiolymphoid hyperplasia with eosinophilia dermnet nz. It is endemic in asia, affecting more number of males than females, with a ratio of 3. The histopathology was characteristic and the patient had stable disease on treatment with cetirizine for 20 months.
It is a benign chronic inflammatory condition producing subcutaneous tumour like nodules chiefly in the head and neck region with predilection for periauricular areas. In angiolymphoid hyperplasia with eosinophilia the lesions are smaller and characterised by thickwalled socalled histiocytoid or epithelioid blood vessels. Unusual and rare case of generalised lymphadenopathy. Kimuras disease histopathology image1 histopathology. However, some sporadic cases have been reported in europe and america 1, 2. Igg4related disease is a new disease concept, established this century and characterized by fibrosis and sclerosis of the involved organs, with infiltration of igg4positive plasma cells. It is our intention to present the clinical, laboratory, histology, and immunohistochemical findings of 21 patients with kimura disease, with a special em phasis on. The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia and raised ige levels. Herein, we report a case of an atypical manifestation of kd accompanied with ns in a middleaged man, though the patient was clinically misdiagnosed. Kimura s disease kimura disease and epidemiology most commonly affect young to middleaged adults. Kimura disease is a chronic inflammatory condition characterized as enlarged lymph node or subcutaneous swelling in the head or neck region. Kimura disease is probably distinct from angiolymphoid hyperplasia with eosinophilia. Kikuchifujimoto disease is a rare lymphohistiocytic disorder that affects young women of asian descent more frequently than persons of other ethnic groups. Kimura disease kd is an uncommon chronic inflammatory disorder of unknown etiology, occurs mainly in asian young males, presenting as subcutaneous growing masses, with a predilection for head and neck, with or without satellite lymphadenopathy.
Histology of the lymph node tissue revealed the pres ence of florid follicul ar. Kimuras disease report of a case masquerading as a. Clinical analysis of kimuras disease in 24 cases from china bmc. Kimuras disease kimuras disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population.
This disease is most common in middleaged asian men. Kimura s disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with kimura s disease and nephrotic syndrome have been reported. On histology, in kimura s disease there are occasional eosinophilic microabscesses. It presents as a mass lesion in subcutaneous tissue of the head and neck or the major salivary glands, often associated with regional lymphadenopathy. Herein, we present an atypical manifestation of kimura disease occurring in a caucasian man with steroidresponsive early membranous glomerulonephritis. Apr 27, 2017 kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and neck region. Pdf angiolymphoid hyperplasia with eosinophilia and kimuras. Kimura s disease is a rare disorder that affects the subcu taneous tissue and. Kimura disease is a benign rare chronic inflammatory dis order of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and. Kimura disease genetic and rare diseases information. The first report of kimura disease was from china in 1937, in which kimm and szeto described seven cases of a condition they termed.
Kimura disease genetic and rare diseases information center. Inflammatory disorder characterised by extensive mature eosinophils infiltration with formation of eosinophilic abscesses and presence of many reactive lymphoid follicles. Davis, in diagnostic surgical pathology of the head and neck. Kimura s disease histopathology image1 histopathology soft tissue pathology more information find this pin and more on systemic pathology. Ick yang department of pathology, yonsei university college of medicine, c. This rare disease is endemic in parts of asia japan, china, indonesia, etc. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Kimuras disease of the lacrimal gland mimicking igg4related. Kimura disease is a rare, benign and chronic inflammatory disorder, that primarily affects the lymph nodes and tissue under the skin in the head and neck region of the body. Kimura disease is a chronic inflammatory disease that mainly manifests as a. Kimuras disease is a part of an ill defined spectrum of vascular lesions of the subcutis.
Recurrent auricular inflammation caused by kimuras. Kimura, eosinophilic lymphogranuloma, subcutaneous nodules, local adenopathies, salivary gland hypertrophy, glomerulonephritis. Kimuras disease is a benign, chronic inflammatory soft tissue disorder of unknown origin, occurring predominantly in young adults. The disease occurred at ages ranging from 5 to 75 years. Editor,kimuras disease is an uncommon, chronic inflammatory disorder of unknown aetiology which occurs predominantly in orientals and presents with tumourlike swellings mainly in the head and neck region.
Read epithelioid haemangioma angiolymphoid hyperplasia with eosinophilia and kimuras disease in chinese, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Kimuras disease, eosinophilia, histopathology, treatment background kimuras disease kd is a rare, benign disorder associated with chronic inflammatory lesions of unclear etiology. The key elements for the diagnosis of kimura disease include characteristic clinical features of soft tissue, head and neck mass, and histologic features of follicular hyperplasia with eosinophilia. Kimura s disease is a rare, chronic inflammatory disorder of unknown cause. Kimura s disease histopathology image 2 histopathology soft tissue pathology more information find this pin and more on systemic pathology. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in other races. Above case was diagnosed as kimura disease on histopathology which. From tissue sampling to histopathological evaluation article pdf available in methods in molecular biology clifton, n. Kimuras disease report of a case masquerading as a parotid. Pathogenesis, diagnosis, and management of kikuchi.
Angiolymphoid hyperplasia with eosinophilia alhe is a benign vascular neoplasm mainly affecting middle. It is often accompanied by nephrotic syndrome and is a rare, chronic inflammatory disorder of unknown cause. Kimuras disease kd is a rare, benign disorder associated with chronic inflammatory lesions of unclear etiology. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes. Recurrent auricular inflammation caused by kimuras disease. Angiolymphoid hyperplasia with eosinophilia versus kimura. It most commonly presents in the head and neck region of asian males during their second and fourth decades of life. Kimuras diseasetreatment with steroid, histopathology. An unusual cause of cervical tumor difference is that immunohistochemical stains have shown ig e in the germinal center ofkd butnotalhe1.
The tissue undergoes a series of steps before it reaches the examiners desk to be thoroughly examined microscopically to arrive at a particular diagnosis. Controversy exists about the naming and definition of this condition, in conjunction with angiolymphoid hyperplasia with eosinophilia. Kimuras disease international journal of scientific. Kimura disease is a rare disease with abundant eosinophils. It usually involves the subcutaneous tissue and lymph nodes of the head and neck region. The pathology of kimuras disease is characterized by prominent germinal centers in involved lymph nodes containing cellular, vascular, and fibrous components. Module introduction to histopathology histology and cytology 2 notes once the diagnosis is made, the slides come back to the laboratory. Kimura s disease, eosinophilia, histopathology, treatment background kimura s disease kd is a rare, benign disorder associated with chronic inflammatory lesions of unclear etiology.
Histopathology of kimura s disease demonstrates a marked follicular hyperplasia with follicles surrounded by a large number of eosinophils, lymphocytes, and mast cells. In angiolymphoid hyperplasia with eosinophilia the lesions are smaller and characterised by thick. Angiolymphoid hyperplasia with eosinophilia and kimuras. Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and neck region. Computed tomography ct scan and magnetic resonance imaging mri have been useful in the. Conclusion kimura disease is diagnosed based on histopathological findings of the excised lesion. First described in 1937 by kimm and szeto in china as eosinophilic hyperplastic granuloma, in 1948 kimura reported the disease as unusual granulation and hyperplastic changes of lymphatic tissue. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in western countries. Pdf kimuras disease is a unique chronic inflammatory angiolymphoid proliferative disorder with a poorly understood pathogenesis occasionally.
Kimuras disease of the epiglottis, histopathology 10. Feb 15, 2009 kimuras disease kd is a chronic inflammatory disorder of unknown etiology. Clinically, it presents as solitary or multiple subcutaneous nodules, predominantly in the head and neck region, typically in the preauricular region, forehead and scalp. The diagnosis of kimura disease was made according to its histopathological.
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