Notably, it is a referred, highly indexed, online international. The diagnosis of bd requires the elimination of many other diseases because of the absence of specific diagnostic criteria. Kimura disease is diagnosed by a surgical biopsy and the cause of the disease is unknown. Declaration of patient consent the authors certify that they have obtained all appropriate patient consent forms. Department of nephrology, auckland city hospital, auckland, new zealand. An open biopsy is the chief means by which this disease is diagnosed. Hiroshi et al had described blood eosinophilia at mean count of 35. We report a case of nephrotic syndrome associated with kimura s disease. Successful treatment of kimuras disease with a 595nm. Kimuras disease in an old case of leprosy hazarika d.
Kimuras disease kd is an uncommon, chronic inflammatory disease characterized by tumorlike lesions in the soft tissue and lymph nodes and increased peripheral. May 2001 september 2003 february 2005 scientific editor. Here we describe a patient with kimuras disease involving the orbits. It was introduced in china by kim and szeto in 1938, but its name was. Because this condition rarely causes health problems, people with kimura disease have a good outcome.
Diagnostic surgical pathology of the head and neck. The typical clinical presentation is characterized by painless, sometimes disfiguring, subcutaneous nodules, predominantly in the head and neck. Interleukin5 stimulates the differentiation and activation of eosinophils and interleukin4 and interleukin initiate and enhance ige synthesis. Kimuras disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. Kimura disease in a maori man with nephrotic syndrome. Pdf kimuras disease is a rare chronic inflammatory condition of uncertain etiology. Aug 04, 2016 kimura s disease is a rare disorder and its occurrence in a treated case of leprosy posed a diagnostic challenge, though the association was probably coincidental. 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. Kd is a rare chronic inflammatory disorder of unknown etiology. Kimura disease is a rare benign inflammatory disease that characteristically manifests as enlargement of cervical lymph nodes and salivary glands. Kimura disease genetic and rare diseases information. Letter to the editor open access kimura disease accompanied.
Rare chronic inflammatory disorder involving deep subcutaneous tissue of head and neck, often with lymphadenopathy and salivary gland involvement. Buergers diseaselike vasculitis associated with kimuras. The first report of kimura disease was from china in 1937, in which kimm and szeto 1 described seven cases of a condition they termed eosinophilic hyperplastic. Letter to the editor open access kimura disease accompanied with nephrotic syndrome in a 45yearold male yu gong1, junying gu1, sony labh2 and yuling shi1 abstract kimura. Lesions are usually located on the head and neck area kung et. It is a rare entity in the west, but endemic in asia. Kimuras disease kd, a chronic inflammatory disease of uncertain etiology, manifests as a painless subcutaneous swelling in the head and. It is often accompanied by nephrotic syndrome and is a rare, chronic inflammatory disorder of unknown cause 2. Herein, we present an atypical manifestation of kimura disease occurring in a caucasian man with steroidresponsive early membranous glomerulonephritis. Kimura disease kd is a rare chronic inflammatory disorder of unknown etiology. Surgical removal is the preferred option, but the treatment remains controversial. Kimuras disease kd is a rare and benign chronic inflammatory soft tissue disorder of unknown origin, which predominantly inflicts head and neck of young male adults in. The disease is described as reactive and data on clonality is absent.
Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes. This remarkable finding is useful in determining the nature of parotid mass at the preliminary stage of investigation. 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. Reasons such as an allergic reaction, tetanus toxoid vaccination, or an alteration of immune regulation are suspected. It usually presents as subcutaneous mass in the head and neck region and is frequently associated with regional lymphadenopathy or salivary gland involvement 1, 2. Lowdose cyclosporine for rapid remission and maintenance in. Rare chronic inflammatory disorder of deep subcutaneous tissue, etiology unknown arch pathol lab med 2007.
Kimuras disease is partly a thelper cell 2 th2mediated disorder with overexpression of the th2 cytokines, interleukin4 il4, interleukin5 il5, and interleukin il. Herein, we report a case of an atypical manifestation of kd accompanied with ns in a middleaged man, though the patient was clinically misdiagnosed. Characteristic features of the disease include painless subcutaneous mass in the head and neck region, elevated serum eosinophilia. Nephrotic syndrome associated with kimuras disease. Kimuras disease, a rare entity in the west but endemic in asia, manifests as solitary or multiple. Successful treatment of kimuras disease with leflunomide. Kimura disease kd is an uncommon chronic inflammatory disorder of unknown etiology, occurs mainly in asian young males, presenting as subcutaneous growing masses. Kimura disease genetic and rare diseases information center. Kimura s disease is a benign rare chronic inflammatory disorder. Kimura s disease is also distinguished from castlemans disease by the infiltration of eosinophils in the former and the proliferation of plasma cells in the latter 3. Successful treatment of kimuras disease with leflunomide and. Lowdose cyclosporine for rapid remission and maintenance. Kimura disease kd is an uncommon idiopathic inflammatory disease khoo and chan, cutis 701.
It was introduced in china by kim and szeto in 1938, but its name was given by kimura in 1948 from japan. The condition primarily involves the subcutaneous tissues, parotid glands, andor lymph nodes. Kimura disease is a chronic inflammatory disorder that mainly affects the area of the head and neck. The course of kimura disease is chronic, with lesions frequently persisting or recurring despite treatment. Kimuras disease is a benign, but locally injurious disease with a. Kimura s disease consists of lesions that appear as single or multiple redbrown papules or as subcutaneous nodules with a predilection for the head and neck region.
Pdf kimura disease kd is a chronic inflammatory disorder with. The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia and raised ige levels. Orbital cases are infrequent and most reported cases are in adults, with only one case in. Electrodiagnosis in diseases of nerve and muscle jun kimura. Author information 1 department of orthopaedics, all india institute of medical sciences, new delhi 110029, india.
Aug 15, 2014 kimuras disease kd is an uncommon, chronic inflammatory disease characterized by tumorlike lesions in the soft tissue and lymph nodes, elevated peripheral blood eosinophil count and serum immunoglobulin e ige. For language access assistance, contact the ncats public information officer. Although it principally affects the skin and soft tissues, there is a high prevalence of associated renal disease. Kimuras disease is a rare disorder and its occurrence in a treated case of leprosy posed a diagnostic challenge, though the association was probably coincidental. The treatment of kimura s disease includes surgery, oral corticosteroids and radiation therapy. It is commonly seen in asia, where it affects males more than females, but it is rare in western countries. Kimura disease in a 12 year old boy pediatric oncall journal. Kimuras disease in an old case of leprosy hazarika d, jahan. Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph. Kimura disease typically affects males 80% between 20 and 40 years of age 80% of cases 12, and is most frequently seen in asia. Electrodiagnosis in diseases of nerve and muscle principles and practice. Increased risk of psychiatric disorders in adult patients with vitiligo.
Blood eosinophilia was exclusively observed in kimura s. All of our cases showed blood eosinophilia as described. 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. Kimura disease is a relatively harmless health condition that results in the afflicted person developing lumps on the head or neck. Masses generally appear in a persons mid20s and the disease mainly affects asian men. Characteristic features of the disease include painless subcutaneous mass in the head and neck region, elevated serum. Kimuras disease kd was first described by kimm and szeto in 1937 and the definitive histological description of the disease was subsequently reported by kimura in 1948. Kimuras disease is a rare, benign, immunemediated inflammatory disorder of unknown aetiology. Treatment includes surgery, medication, or a combination of the two. Here, we report a 25yearold male patient with asthma, reynaud phenomenon, eosinophilic panniculitis, bilateral inguinal lymphadenopathy and peripheral blood eosinophilia. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. 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. The first report of kimura disease was from china in 1937, in which kimm and szeto described seven cases of a condition they termed. If you have problems viewing pdf files, download the latest version of adobe reader.
The clinical presentation was suggestive of kimuras disease and. Kimura s disease is a rare, benign chronic inflammatory condition which commonly occurs among the oriental population. Clinical analysis of kimuras disease in 24 cases from china bmc. Kimuras disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with kimuras disease and nephrotic syndrome have been reported. This chronic inflammatory soft tissue disease is characterized by lymphoid and angiomatous tissue proliferation with lymphadenopathy, eosinophilia, and elevated serum ige levels khoo and chan, cutis 701. Kimuras disease is a rare, benign, slow growing chronic inflammatory swelling with a predilection for the head and neck region and almost always with peripheral blood. Kimuras disease is a chronic inflammatory disorder of unknown etiology. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare. Letter to the editor open access kimura disease accompanied with nephrotic syndrome in a 45yearold male yu gong1, junying gu1, sony labh2 and yuling shi1 abstract kimura disease kd is an uncommon chronic inflammatory disorder of unknown etiology, occurs mainly in asian. Several studies had described the findings of kimuras disease. Orbital kimuras disease in a white child british journal. 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. A rare case of kimuras disease was seen in a 42yearold female patient who presented with swelling in left pre auric ular region. Clinically, kd is always accompanied by enlarged regional lymph nodes, eosinophilia and markedly elevated serum ige levels 2,5.
Kimura s disease is partly a thelper cell 2 th2mediated disorder with overexpression of the th2 cytokines, interleukin4 il4, interleukin5 il5, and interleukin il. 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. The cause of kimura disease is unknown, and research is hindered by its rarity. Males are affected by kimura disease more commonly than females, with a 3. It is often accompanied by nephrotic syndrome and is a rare, chronic inflammatory disorder of unknown cause. Kimura disease is usually seen in young adults during the third decade of life, with the median age being 2832 years. Sporadic cases are seen in other geographic regions, however these are uncommon. Kimura disease accompanied with nephrotic syndrome in a 45.
Kimuras disease kd is a rare, chronic inflammatory disorder that occurs predominantly in asian males and is characterized by recurrent painless swellings involving the. Electrodiagnosis in diseases of nerve and muscle jun. 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. It typically presents as solitary or multiple subcutaneous nodules, that slowly. This chronic inflammatory soft tissue disease is characterized. Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in other races. However, the masses tend to develop again after treatment. Kimuras disease consists of lesions that appear as single or multiple redbrown papules or as subcutaneous nodules with a predilection for the head and neck region. Kimura disease is a rare, benign, chronic inflammatory disorder. Several studies had described the findings of kimuras. Doctor claire larroche1, professor olivier bletry date of creation. Kimura s disease is a rare, benign, slow growing chronic inflammatory swelling with a predilection for the head and neck region and almost always with peripheral blood eosinophilia and elevated serum ige levels.
Kimura disease kd is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of asian race but is rare in western countries. Blood eosinophilia was exclusively observed in kimuras disease. Kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. Editor,kimuras disease is a chronic inflammatory disorder of unknown aetiology. Kimura disease is an inflammatory disease of unknown etiology that frequently involves the subcutaneous nodules in the head and neck, salivary glands, and regional lymph. It is a disease of chronic eosinophilic inflammation, probably immunedriven, with angioplasty proliferation.
The purpose of this study was to evaluate the ct and mr imaging findings. Pdf kimuras disease an unusual presentation involving. 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. Kimuras disease of the lacrimal gland mimicking igg4. Additionally, recurrence after treatment is well described.
Buergers disease bd is a nonatherosclerotic inflammatory disorder of unknown etiology that affects small and mediumsized vessels of the extremities. Kimuras disease is a benign rare chronic inflammatory disorder. Kimuras disease with eosinophilic panniculitis treated. Kimuras disease as a solitary intrapulmonary lymphadenopathy. Kimura disease is an inflammatory disease of unknown etiology that frequently involves the subcutaneous nodules in the head and neck, salivary glands, and regional lymph nodes. May 27, 2008 kimura disease kd is an uncommon idiopathic inflammatory disease khoo and chan, cutis 701. Kimura disease can lead to disfigurement secondary to the growth of untreated lesions, particularly given the predilection for the head and neck. Editor,kimuras disease is an uncommon, chronic inflammatory disorder of unknown aetiology which occurs predominantly in orientals and presents with tumourlike.
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