Other than the rarity of this disease in the caucasian population, to the best of our knowledge, it is the second case reported in poland so far. Kimuras disease should be considered as a possible diagnosis when a partially or poorly defined subcutaneous mass of high signal intensity on t1 and t2weighted images with homogeneous enhancement, surrounding subcutaneous edema, and internal flow voids is seen in the medial epitrochlear region in an asian person, especially if accompanied by. Kimuras disease is a chronic inflammatory condition of uncertain etiology. Kimuras disease with unusual eosinophilic epithelioid granulomatous reaction. Angiolymphoid hyperplasia with eosinophilia dermnet nz.
Herein, we present an atypical manifestation of kimura disease occurring in a caucasian man with steroidresponsive early membranous glomerulonephritis. This disease is most common in middleaged asian men. 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. Most cases of kimura disease have been reported in asians, and the prevalence among persons of other races is thought to be low.
Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009. It is considered to be due to chronic inflammation of unclear etiology. It mainly affects asian males in their 2nd to 4th decade of life. Kimuras disease is a benign, but locally injurious disease with a marked predilection for the head and neck. Kimura disease typically affects males 80% between 20 and 40 years of age 80% of cases 12, and is most frequently seen in asia.
Kd is a rare chronic inflammatory disorder of unknown etiology. In most of the previously reported cases, kimuras disease typically presents as single or multiple subcutaneous lesions in the head and neck, especially in. Jun 18, 2018 kimuras disease also known as subcutaneous angioblastic lymphoid hyperplasia with peripheral eosinophilia is a rare clinicopathological entity. Kimura s disease is a rare, chronic inflammatory disease characterized by subcutaneous nodules in the head and neck regions associated with regional cervical lymphadenopathy. First described by kimm and szeto in 1937 as eosinophilic hyperplastic lymphogranuloma, it gained prominence as kimuras disease following a report by kimura and coworkers in 1948, which elaborated on an unusual granulation combined with hyperplastic changes in lymphoid tissue. Blood eosinophilia was exclusively observed in kimuras disease. Kimura disease is a chronic inflammatory disorder of unknown. Masses generally appear in a person s mid20s and the disease mainly affects asian men. The first report of kimura disease was from china in 1937, in which kimm and szeto described seven cases of a condition they termed. Kimuras disease kd was firstly described in a chinese literature in 1937,1 and its histological description was produced by kimura et al in 1948 which has given its name 2. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in western countries.
Of uncertain etiology, its tendency to present as a discrete, enlarging mass with associated. Kimuras disease kimuras disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. Elevated serum immunoglobulin e levels and peripheral blood eosinophilia are also common. In some patients, kimuras disease is associated with proteinuria. A 36yearold man presented to the department of surgery. In 1983, enzinger and weiss,29 in their book soft tissue tumor suggested the. Angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease are chronic inflammatory diseases of unknown etiology that present as swellings in skin and lymph nodes of the head and neck. The purpose of this study was to evaluate the ct and mr imaging findings of kd in the head and neck. Kimuras disease is a rare chronic inflammatory disorder. It is similar to angiolymphoid hyperplasia with eosinophilia. Pathology outlines phosphaturic mesenchymal tumor sternoclavicular joint pathology radsource an intro to routine and special staining in histopathology. Kimura disease genetic and rare diseases information center. Usually asian males with elevated serum ige and eosinophilia. Sporadic cases are seen in other geographic regions, however these are uncommon.
Kimuras disease kd was first described in 1937 by kimm, and its histopathology was later characterized by kimura et al in 1948. Eosinophilic abscess, eosinophils infiltrating germinal centers and dermal sclerosis are more common in kimura disease. Kimura disease is not angiolymphoid hyperplasia with eosinophilia. Kimura disease is diagnosed by a surgical biopsy and the cause of the disease is unknown. Kimura disease definition of kimura disease by medical. It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum ige levels.
Weedons skin pathology, 4th ed, churchill livingston, 2016. Refractory hypertension and anemia in endstage renal disease. Masses generally appear in a persons mid20s and the disease mainly affects asian men. It is often accompanied by nephrotic syndrome and is a rare, chronic inflammatory disorder of unknown cause. Kimuras disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. 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. To the best of our knowledge, a report of kimuras disease involving the groin in the english language has not been reported. Herein, we report a case of an atypical manifestation of kd accompanied with ns in a middleaged man, though the patient was clinically. Rare case of eosinophilic pneumonia in patient with kimura. Rare chronic inflammatory disorder of deep subcutaneous tissue, etiology unknown arch pathol lab med 2007. Nephrotic syndrome associated with immune thrombocytopenia. It is benign condition with unknown etiology usually affecting young men of asian race. Kimuras disease is a benign rare chronic inflammatory disorder.
Kimuras disease with atypical musculoskeletal presentation. In about 12% of patient, there may be associated renal disease, usually presenting as proteinuria. A complete clinicalradiological pathology report author. Kimura disease genetic and rare diseases information. Kimuras disease, parotid gland inflammatory diseases, chronic eosinophilic inflammation created date. Immunoperoxidase studies show ige reticular networks in. The vessels of kimura disease generally lack an epithelioid endothelial lining. Li tj, chen xm, wang sz, fan mw, semba i, kitano m. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in other races. Angiolymphoid hyperplasia with eosinophilia and kimuras.
Angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease are chronic. 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. A rare cause af solid mass in the head and neck area and kimuras disease as a diagnostic dilemmabas ve boyun bolgesinde nadir bir kitle nedeni ve tanisal karmasa olarak. It primarily involves the head and neck region, presenting as deep subcutaneous masses and is often accompanied by regional lymphadenopathy and salivary gland involvement. Kimuras disease has a benign clinical course with a triad of features. Kimura s disease kd was first described in 1937 by kimm, and its histopathology was later characterized by kimura et al in 1948. Kimuras disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with kimuras disease and nephrotic syndrome have been reported. Kimura s disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. The subcutaneous masses of kimuras disease are usually found in the head and neck region, sometimes affecting the parotid or minor salivary glands. Kimura s disease presents as subcutaneous painless swelling in head and neck region often mimicking inflammatory or neoplaitic disease. Herein, we report a case of an atypical manifestation of kd accompanied with ns in a middleaged man, though the patient was clinically misdiagnosed. Once thought to be the same disorder, alhe and kimura disease are now.
In most of the previously reported cases, kimuras disease typically presents as single or multiple subcutaneous lesions in the head and neck, especially in the parotid and submandibular regions 36. The case presented in this paper is an exceptional case described in a caucasian female polish. 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. Kimuras disease eosinophilic hyperplastic lymphogranuloma is a rare form of chronic inflammatory. Imaging findings of kimuras disease in the soft tissue of. An open biopsy is the chief means by which this disease is diagnosed. Kimuras disease is a rare, chronic inflammatory disease characterized by subcutaneous nodules in the head and neck regions associated with regional cervical lymphadenopathy.
Kimuras disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. Kimuras disease is a rare chronic inflammatory disorder characterized by the head and neck lymphadenopathy often accompanied by eosinophilia and elevated serum ige. Kimura disease is a rare disease with abundant eosinophils. Angiolymphoid hyperplasia with eosinophilia pathology. Although kimuras disease is not primarily a bcell disorder, it is conceivable that the th2 imbalance may be associated with the production of autoantibodies in some patients. All of our cases showed blood eosinophilia as described. Initial cytology fnac showed angiofollicular hyperplasia suggestive of early lymphoma or castlemans disease. Kimuras disease also known as subcutaneous angioblastic lymphoid hyperplasia with peripheral eosinophilia is a rare clinicopathological entity. The clinical presentation varies from subcutaneous swellings of the head and neck region with regional lymphadenopathy with salivary gland involvement to systemic manifestations like. Kimuras disease, alhe, av malformation avm kimuras disease arvind kohli, gurjit singh introduction kimuras disease is an inflammatory disorder that presents as massive subcutaneous swelling in the periauricular and submandibular region in young asian men. An unusual clinical presentation of kimuras disease. Several studies had described the findings of kimuras disease. Oral surgery, oral medicine, oral pathology and oral radiology. Nonetheless, the diagnosis requires exclusion of infectious etiologies and neoplastic causes with similar histology.
Kimura disease in a 12 year old boy pediatric oncall journal. Kimura disease kd, which is one of the most difficult clinical entities concerning the diagnosis, is a rare, benign, chronic inflammatory disease with unknown etiology. The pathology of kimura s disease is quite different from that of angiolymphoid hyperplasia with eosinophilia epithelioid hemangioma. Kimura disease is an uncommon idiopathic chronic inflammatory disorder that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and neck region. Kimuras disease kd is a rare, benign disorder characterized by subcutaneous masses with regional lymphnode enlargement.
Kimuras disease kd is a rare, chronic allergic inflammatory disorder of unknown etiology1. Angiolymphoid hyperplasia with eosinophilia alhe is a benign vascular neoplasm mainly affecting middle. It is a chronic granulomatous disease of unknown etiology. Kimuras disease is a rare chronic inflammatory disease of unknown etiology, presenting as painless subcutaneous nodules with lymphadenopathy and peripheral eosinophilia, mainly disturbing the head and neck region. Kimura disease accompanied with nephrotic syndrome in a 45. Kimuras disease involving the head and neck region and upper extremities have been documented 38. The pathology of kimuras disease is quite different from that of angiolymphoid hyperplasia with eosinophilia epithelioid hemangioma. Kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. The association of kimuras disease with autoimmune manifestations seems unusual. Angiolymphoid hyperplasia with eosinophilia and kimura disease. It was introduced in china by kim and szeto in 1938, but its name was given by kimura in 1948 from japan.
Angiolymphoid hyperplasia with eosinophilia is also known as epithelioid or histiocytoid haemangioma. Davis, in diagnostic surgical pathology of the head and neck second edition, 2009. Lymphoid nodules with discrete germinal centers can occupy an. Angiolymphoid hyperplasia with eosinophilia is an apparently non malignant, locally proliferating lesion composed of channels of small blood vessels surrounded by lymphocytes and eosinophils these are two types of white blood cell. Oral surg oral med oral pathol oral radiol endod 1996. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Kimura disease can closely resemble angiolymphoid hyperplasia with eosinophilia. Following its recognition as a pathologic entity, numerous reports have been published detailing the pathogenesis, radiologic manifestations, and treatment modalities. Messinadoucet mt, armstrong wb, allison g, pena f, valera kim jk.
The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia and raised ige levels. Kimura disease has been confused with angiolymphoid hyperplasia with. We report here a case of kimuras disease in the groin with mr imaging findings and histopathological correlations. It is a disease of chronic eosinophilic inflammation, probably immunedriven, with angioplasty proliferation. Although the disease can present at any age, most cases. Kimuras disease is a rare chronic inflammatory condition of uncertain etiology which has an affinity for the asian population. This remarkable finding is useful in determining the nature of parotid mass at the preliminary stage of investigation. 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. Imaging findings of kimuras disease occurring in the head and neck region have been reported 38, but imaging findings of kimuras disease involving the upper extremity have not been reported, to our. Kimura s disease should be considered as a possible diagnosis when a partially or poorly defined subcutaneous mass of high signal intensity on t1 and t2weighted images with homogeneous enhancement, surrounding subcutaneous edema, and internal flow voids is seen in the medial epitrochlear region in an asian person, especially if accompanied by. Kimura s disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. Kimuras disease is a chronic benign inflammatory condition endemic to asians.
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