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Kimura Disease Workup Author: Evan W Piette, MD; Chief Editor: William D James, MD more... Updated: Mar 20, 2014 Laboratory Studies Nearly all patients with Kimura disease demonstrate peripheral eosinophilia and elevated levels of serum IgE. In one series, the number of eosinophils was closely correlated to the sizes of the neck masses. [19] Blood urea nitrogen, creatinine, and urinary protein levels should be obtained to exclude concomitant renal dysfunction (especially nephrotic syndrome). Serum eosinophil cationic protein levels parallel the course of the disease. [20] Imaging Studies The appearance of Kimura disease on imaging modalities, including CT scanning and MRI, is variable and is thought to be due, at least in part, to the variable degrees of vascular proliferation and fibrosis within individual lesions. One of the largest case series to date notes the characteristic findings to be multiple illdefined, enhancing lesions around the parotid gland, with associated lymphadenopathy. [15] Procedures Incisional biopsy is recommended to obtain the diagnosis of Kimura disease. Histologic Findings Lymphoid nodules with discrete germinal centers can occupy an area extending from the reticular dermis to the fascia and muscle. A marked eosinophilic infiltrate and eosinophilic abscesses are present. Centrally, thickwalled vessels are present with hobnail endothelial cells. Immunohistochemical evaluation of the lymphoid nodules demonstrates a polymorphous infiltrate without clonality. [7, 15] Reports have also demonstrated the presence of plasmacytoid dendritic cells in a lesion of Kimura disease. [21] Contributor Information and Disclosures Author Evan W Piette, MD Resident Physician, Department of Dermatology, Hospital of the University of Pennsylvania Evan W Piette, MD is a member of the following medical societies: American Academy of Dermatology Disclosure: Nothing to disclose. Coauthor(s) Alaina J James, MD, PhD Assistant Professor, Department of Dermatology, The Hospital of the University of Pennsylvania Alaina J James, MD, PhD is a member of the following medical societies: American Academy of Dermatology, National Medical Association, and Women's Dermatologic Society Disclosure: Nothing to disclose. Specialty Editor Board Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc Disclosure: Nothing to disclose. Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association Disclosure: Nothing to disclose. Daniel S Loo, MD Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology Disclosure: Nothing to disclose. Joel M Gelfand, MD, MSCE Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

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  • 4/28/2015 KimuraDiseaseWorkup

    http://emedicine.medscape.com/article/1098777workup#showall 1/3

    KimuraDiseaseWorkupAuthor:EvanWPiette,MDChiefEditor:WilliamDJames,MDmore...

    Updated:Mar20,2014

    LaboratoryStudiesNearlyallpatientswithKimuradiseasedemonstrateperipheraleosinophiliaandelevatedlevelsofserumIgE.Inoneseries,thenumberofeosinophilswascloselycorrelatedtothesizesoftheneckmasses.[19]

    Bloodureanitrogen,creatinine,andurinaryproteinlevelsshouldbeobtainedtoexcludeconcomitantrenaldysfunction(especiallynephroticsyndrome).

    Serumeosinophilcationicproteinlevelsparallelthecourseofthedisease.[20]

    ImagingStudiesTheappearanceofKimuradiseaseonimagingmodalities,includingCTscanningandMRI,isvariableandisthoughttobedue,atleastinpart,tothevariabledegreesofvascularproliferationandfibrosiswithinindividuallesions.Oneofthelargestcaseseriestodatenotesthecharacteristicfindingstobemultipleilldefined,enhancinglesionsaroundtheparotidgland,withassociatedlymphadenopathy.[15]

    ProceduresIncisionalbiopsyisrecommendedtoobtainthediagnosisofKimuradisease.

    HistologicFindingsLymphoidnoduleswithdiscretegerminalcenterscanoccupyanareaextendingfromthereticulardermistothefasciaandmuscle.Amarkedeosinophilicinfiltrateandeosinophilicabscessesarepresent.Centrally,thickwalledvesselsarepresentwithhobnailendothelialcells.Immunohistochemicalevaluationofthelymphoidnodulesdemonstratesapolymorphousinfiltratewithoutclonality.[7,15]ReportshavealsodemonstratedthepresenceofplasmacytoiddendriticcellsinalesionofKimuradisease.[21]

    ContributorInformationandDisclosuresAuthorEvanWPiette,MDResidentPhysician,DepartmentofDermatology,HospitaloftheUniversityofPennsylvania

    EvanWPiette,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatology

    Disclosure:Nothingtodisclose.

    Coauthor(s)AlainaJJames,MD,PhDAssistantProfessor,DepartmentofDermatology,TheHospitaloftheUniversityofPennsylvania

    AlainaJJames,MD,PhDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatology,NationalMedicalAssociation,andWomen'sDermatologicSociety

    Disclosure:Nothingtodisclose.

    SpecialtyEditorBoardTakejiNishikawa,MDEmeritusProfessor,DepartmentofDermatology,KeioUniversitySchoolofMedicineDirector,SamonchoDermatologyClinicManagingDirector,TheWaksmanFoundationofJapanInc

    Disclosure:Nothingtodisclose.

    MichaelJWells,MDAssociateProfessor,DepartmentofDermatology,TexasTechUniversityHealthSciencesCenter,PaulLFosterSchoolofMedicine

    MichaelJWells,MDisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanAcademyofDermatology,AmericanMedicalAssociation,andTexasMedicalAssociation

    Disclosure:Nothingtodisclose.

    DanielSLoo,MDAssociateProfessorofDermatology,ResidencyProgramDirector,DepartmentofDermatology,TuftsMedicalCenter

    DanielSLoo,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatologyandAssociationofProfessorsofDermatology

    Disclosure:Nothingtodisclose.

    JoelMGelfand,MD,MSCEMedicalDirector,ClinicalStudiesUnit,AssistantProfessor,DepartmentofDermatology,AssociateScholar,CenterforClinicalEpidemiologyandBiostatistics,UniversityofPennsylvania

    javascript:showrefcontent('refrenceslayer');javascript:showrefcontent('refrenceslayer');javascript:showrefcontent('refrenceslayer');http://www.aad.org/http://www.alphaomegaalpha.org/http://www.ama-assn.org/http://www.womensderm.org/http://www.dermatologyprofessors.org/javascript:showrefcontent('refrenceslayer');http://www.aad.org/http://googleads.g.doubleclick.net/aclk?sa=L&ai=B73kQbX8_Vd74GIW7vASh84HgA6LsuP4FAAAAEAEguvzWHjgAWOqFk_S-AWDpgoCA5A2yARZlbWVkaWNpbmUubWVkc2NhcGUuY29tugEJZ2ZwX2ltYWdlyAEC2gE0aHR0cDovL2VtZWRpY2luZS5tZWRzY2FwZS5jb20vYXJ0aWNsZS8xMDk4Nzc3LXdvcmt1cMACAuACAOoCIy80MzEyNDM0L3Byb2Zwcm9tby9tZWRzY3ByZWZkZXNrdG9w-AL-0R6QA_QImAPgA6gDAcgDmQTQBJBO4AQBkAYBoAYU2AcB&num=0&cid=5GgfCPLqvV3eMbtfTss-W3vd&sig=AOD64_1m_QAt4w0uuXYbq5bizu9fI71jZw&client=ca-pub-3100878564080714&adurl=http://www.medscape.com/public/infosite%3Fsrc%3Dad_ldr_gihttp://www.texmed.org/javascript:showrefcontent('refrenceslayer');http://adclick.g.doubleclick.net/aclk%253Fsa%253DL%2526ai%253DBBDxdbX8_VeP4GIW7vASh84HgA4LjzY8HAAAAEAEguvzWHjgAWPr9yIKJAmDpgoCA5A2yARZlbWVkaWNpbmUubWVkc2NhcGUuY29tugEJZ2ZwX2ltYWdlyAEJ2gE0aHR0cDovL2VtZWRpY2luZS5tZWRzY2FwZS5jb20vYXJ0aWNsZS8xMDk4Nzc3LXdvcmt1cMACAuACAOoCIy80MzEyNDM0L3Byb2Zwcm9tby9tZWRzY3ByZWZkZXNrdG9w-AL-0R6QA_QImAPgA6gDAdAEkE7gBAGQBgGgBh_YBwA%2526num%253D0%2526cid%253D5Gi3n9yNnnkeC294ad0R80nh%2526sig%253DAOD64_19NUVptwNYCYVhk6o8lpeaYfs3Ag%2526client%253Dca-pub-3100878564080714%2526adurl%253Dhttp://www.medscape.org/public/cme-latest/pcp?src=cme_latestcollection_pcp_adhttp://www.nmanet.org/javascript:showrefcontent('authordisclosures');http://www.aad.org/http://googleads.g.doubleclick.net/aclk?sa=L&ai=BDdzTbX8_VeH4GIW7vASh84HgA6LsuP4FAAAAEAEguvzWHjgAWOLrm_S-AWDpgoCA5A2yARZlbWVkaWNpbmUubWVkc2NhcGUuY29tugEJZ2ZwX2ltYWdlyAEC2gE0aHR0cDovL2VtZWRpY2luZS5tZWRzY2FwZS5jb20vYXJ0aWNsZS8xMDk4Nzc3LXdvcmt1cMACAuACAOoCIy80MzEyNDM0L3Byb2Zwcm9tby9tZWRzY3ByZWZkZXNrdG9w-AL-0R6QA_QImAPgA6gDAcgDmQTQBJBO4AQBkAYBoAYU2AcB&num=0&cid=5GhiVFadle3ZKZ1e-5o6qJU9&sig=AOD64_3300ZvZBSFjl-HAX4_PKjlqgEpXA&client=ca-pub-3100878564080714&adurl=http://www.medscape.com/public/infosite%3Fsrc%3Dad_sq_gihttp://googleads.g.doubleclick.net/aclk?sa=L&ai=Bi_dZbX8_VeD4GIW7vASh84HgA6LsuP4FAAAAEAEguvzWHjgAWPKAm_S-AWDpgoCA5A2yARZlbWVkaWNpbmUubWVkc2NhcGUuY29tugEJZ2ZwX2ltYWdlyAEC2gE0aHR0cDovL2VtZWRpY2luZS5tZWRzY2FwZS5jb20vYXJ0aWNsZS8xMDk4Nzc3LXdvcmt1cMACAuACAOoCIy80MzEyNDM0L3Byb2Zwcm9tby9tZWRzY3ByZWZkZXNrdG9w-AL-0R6QA_QImAPgA6gDAcgDmQTQBJBO4AQBkAYBoAYU2AcB&num=0&cid=5GiDqvo9_z_oWtNHa82ORIif&sig=AOD64_1EG3dOekOSauieRXTVIS9pOrN89g&client=ca-pub-3100878564080714&adurl=http://www.medscape.com/invitation/viewTracker.do%3Fsrc%3Dad_sq_gmit&nm=1http://www.aad.org/http://www.sidnet.org/

  • 4/28/2015 KimuraDiseaseWorkup

    http://emedicine.medscape.com/article/1098777workup#showall 2/3

    JoelMGelfand,MD,MSCEisamemberofthefollowingmedicalsocieties:SocietyforInvestigativeDermatology

    Disclosure:AMGENConsultingfeeConsultingAMGENGrant/researchfundsInvestigatorGenentechGrant/researchfundsinvestigatorCentocorConsultingfeeConsultingAbbottGrant/researchfundsinvestigatorAbbottConsultingfeeConsultingNovartisinvestigatorPfizerGrant/researchfundsinvestigatorCelgeneConsultingfeeDMCChairNIAMSandNHLBIGrant/researchfundsinvestigator

    ChiefEditorWilliamDJames,MDPaulRGrossProfessorofDermatology,ViceChairman,ResidencyProgramDirector,DepartmentofDermatology,UniversityofPennsylvaniaSchoolofMedicine

    WilliamDJames,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatologyandSocietyforInvestigativeDermatology

    Disclosure:Nothingtodisclose.

    AdditionalContributorsSenaJLee,MD,PhDResidentPhysician,DepartmentofDermatology,HospitalofUniversityofPennsylvania

    Disclosure:Nothingtodisclose.

    PatriciaMercado,MDAssociateProfessorofDermatology,UniversityofAlabamaatBirminghamSchoolofMedicine

    Disclosure:Nothingtodisclose.

    LauraMTamburin,MDAffiliatedDermatology,PC

    Disclosure:Nothingtodisclose.

    KarolynAWanat,MDResidentPhysician,DepartmentofDermatology,UniversityofPennsylvaniaSchoolofMedicine

    KarolynAWanat,MDisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanAcademyofDermatology,AmericanMedicalAssociation,andAmericanMedicalWomen'sAssociation

    Disclosure:Nothingtodisclose.

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  • 4/28/2015 KimuraDiseaseWorkup

    http://emedicine.medscape.com/article/1098777workup#showall 3/3

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