Maida, Mark F

Parsa, F. Don

Parsa, Natalie

Wong, David

eVols at University of Hawaii at Manoa

Kimura’s Disease of the EarlobeMark F. Maida MD, David Wong BS, Natalie Parsa BA, and F. Don Parsa MDCase ReportA 25 year-old woman of part-Hawaiian and part-Japanese ancestrypresented with a 10—year history of a recurring mass in her leftearlobe, The patient had both ears pierced ten years ago and withinone year had developed an infection at the site ol the left ear piercing.The earrings were removed, yet within another years time a tenderervthematous mass developed in her left earlobe. The mass wasdiagnosed as a keloid and was injected with steroids six timeswithout benefit, The mass was excised 4 times between 1992 and1998 and recurred after each excision. The patient presented to usin 2002 with a mass in her left earlobe that had been increasing in sizefor the past 4 years,Dermatologic examination revealed a firm and tender 3x4x2 cmlobular, erythematous lesion in the left earlobe Further physicalexam was unremarkable. including the absence of lytnphadenopa—thy. Laboratory examinations were later performed and wereentirely negative, including normal renal function and urine analysis, eosinophil count. ESR, and serum IgE levels, The patient has apast medical history of hypothyroidism treated with levothvroxine,Family history is non—contributory, We treated the patient with 2injections of depo—Medrol 4 weeks apart prior to excision, Duringthe first 12 weeks of follow—up care, the patient received 4 injectionsotdepo—Medrol due to the continual recurrence of the mass, Notingthe lack of improvement and resurgence of the mass following eachpost—excisional injection, the patient was referred to a t’heumatolo—gist and placed on prednisone at 20 mg once a day for 3 weeks withno signs of improvement, Thereafter, the patient was placed oncyclosporine. 100mg three times ada with very rapid response andtotal resolution of the recurring mass on the left earlobe, The patientfailed to fill the prescription for cyclosporine and during the 3 dayslacking medication noted a resurgence of the mass. Upon resumingthe cyclosporine at the aforementioned dosage. the mass resolved.Histopathologic examination of the excised mass revealed reactivelymphoid hyperplasia with scattered follicles showing prominentgerminal centers and foci of vascular proliferation. A careful reviewof the patient’s old chart revealed a pathology report from a previousexcision in 1998 that showed a nodular lesion with dermal and softtissue. i.nfiit.ration of numerous large germinal centers and a poi..v—morphous infiltrate of lymphoid cells, eosinophils, and small bloodvessels located between the. germinal centers, Th..e c.Iinic.ai historyand.pathoiogicai findings of this patientarec.onsist.e.nt with Kimura’sd.isease,Corres•oondence trjF Den Parsa MD1329 Lusitana St #801HonoluLu HI 98813Figure 1 - Firm 4x4x2 cm lobular erythematous lesion in the left Iearlobe._jFigure 2.— Reactive lymphoid hyperpiasia Follicles with prominentgerminal centers and foci of vascular proliferation35Figure 3.— Low power micrograph showing dermal infiltration by alarge germinal center in the middle and part of another germinalcenter in the right lower corner.DiscussionKimuras disease is achronic inflantmatarv skindisease that usuaflyOccUrs in the head and neck) It usuallr presents with a cluster ofsmall, painless, translucent nodules. While subcutaneous tissue andIs mph nodes of the head and neck arc nmt commonly affected. lesstrertueni sites include the oral inucusa. salis air glands, prostate.kidner peripheral ners e. t\ nipanic membrane, and skeletal ninsele:Knyiura’ s disease i’ a rare condition, with fes ci’ than I 2(1 casesreported worldss ide in I 997: There is a predisposition hr Asianmales. 55 ii h a median age 01 on set ut 2( r earsold and a male: femaleratio >d: I .‘ Kimura’s disease is abs avs benign hut ma minnc aneoplasi ic process at presentation.I aboratoir findings associated ss oh Kimnra’s disease includeperipheral eosinophiiia and increased serum IgE. CT and \IR Imaning niar be heipini it lymph node nsolsement is uspected.Renal disease mar he present in I) ofthese patients. characterizedIsv proteinuria.Kimura’s disease was first described in China by Kim and Sietoin I 9g7 as an angiolmphoid prolilei’ative disorder of soft tissueKimura ci al. further described the condition in 1948 asan “unusualgranulation combined with hyperplastic changes in lvmphoid tissue.” i ‘the most prominent histological finding in Kimura’s diseaseis an co. nophilic infiltration of germinal centers which results infolliculolr sis. Lymphoid follicles with active germinal centers arean nvariablc finding. The blood cssels seen in Kimura’s diseaseare thinss aIled. These histopathol cie findings can make thediagnosis ol Knnura’sdisease ss hen in case’ ss here itcan hedffeultto do co elincali\The difCrental daenoss inJudc5 \ mphoma .an. nlcctiou’Iccsscs such as tosopiesmosis or tuberculosis. and kelod t’rmago. iut recently, Ximtna’ disease ha, been found to bc disunniron anvnl\ mhoid hyperplasia ith eoinophils AIHE). - Bothot licsc diseases share a male niednin,iucc. predilection br thenead and neck. tendency to recur, and i\ mphoid and cosino philicnIltrarun. AL HE i mere comm&l\ found it - ptietn:nitcstng a’ tii.ltiplc small dcrmal eruptions. The most nortantmorphologic difference between Kimura’ s disease and ALHE liesit the nature of the blood vessels. ALHE has thickwalled bloodessels lined by plump eosinophilic endothehial cells that marc ntaiit vacuolatedeytoplasm. These cells are not found in Kimura’disease.ConclusionThe etiologr of’ Kimura’ s disease is stl I uncertain. The incommon theory is-a hr persensitis t\ reaction mediated hr I helpem2 cells. Hypotheses n the past included antigenmc stimulation ruman insect bite. parasitic infections ,and neoplasne changes)Kimura’ s disease may rcsols c spontaneously hut has a tendenc’.to recur. Treatments in literature have included surgical excision.s stemic stemoids. and radiothei’ap\ - Our patients his orable reinse t eye I spori ne g is cmi at a ds e of 1(1(1 mg th i-ce i i isics a da.supp nis a recent case meport of siminlar treatment oh Kimura’disease. The duration of treatment is, hos ever, as of yet unknoss n.\\e plan to continue this regimen tbr one year, notwithstanding anrunlas orable side effects.Authors\Ia F Mud %I) ) I\\ an’ S di Pnd F I i i \ I) — I) p rmmnt 5 u s hi \ Bu aS,ItossI I I d itReferenceFigure 4.— A high power view of one of the germinal centers. Apolymorphous infiltrate of lymphoid cell and the characteristic eosinophils and small blood vessels can be seen..5 do36

Kimura's disease of the earlobe.

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Kimura's disease of the earlobe.

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