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