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Lacrimal gland involvement in Kikuchi-Fujimoto disease

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Lacrimal gland involvement in Kikuchi-Fujimoto disease
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  113 Kikuchi-Fujimoto disease Case reports Orbit 0167-6830/98/US$ 12.00 Orbit – 1998   , Vol. 17   , No. 2  , pp. 113-117  © Æolus Press Buren (The Netherlands) 1998  Accepted 1  December 1997 Lacrimal gland involvement in Kikuchi-Fujimoto diseasePamela S. Chavis, M.D.Amna Fallata, 1  M.D.Hailah Al-Hussein, 1  M.D.David Clunie, 2  M.D.Antonio Huaman, 3  M.D. Departments of 1 Ophthalmology, 2 Radiology and 3 Pathology,King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia Correspondence and reprint requests to: Pamela S. Chavis, M.D.746 Wyoming AvenueElizabeth, NJ 07208U.S.A.Tel./Fax: +1-908-289-1153  Acknowledgement: This paper was presented at the HoytSymposium, December 7-8, 1995, inSan Francisco, California. Abstract purpose A 32-year-old Saudi female presented with typical Kikuchi-Fujimoto disease, i.e., fever, cervical lymphadenitis and leukopenia, butthere was also painful upper eyelid swelling with pain on upgaze. met hods A connective tissue disease and lymphoma workup were un-remarkable, as were antibody titers to  Apifia felis  and  Bartonella hense-lae . resul t s Orbital computed tomography showed significant lacrimalgland enlargement. Cervical node biopsy revealed necrotizing lymphad-enitis. concl usion Concomitant lacrimal gland inflammation and cervicallymphadenopathy may be a benign self-limited disease. Key words Lacrimal gland; Kikuchi-Fujimoto disease Case report  A 32-year-old Saudi female presented with a painfulswelling in the right upper eyelid and slight pain on upgaze of oneweek’s duration. Four weeks earlier, however, there had been suddenpainful swelling in the right posterior neck which increased with aconcomitant fever of 40°C and arthralgias despite oral ciproxacillin.Her history was not consistent with tuberculosis, systemic lupus ery-thematosus (SLE), herpes (HSV) or Epstein-Barr virus (EBV) infec-tion, nor upper respiratory infection. There was no history of cat/ kitten lick, bite or scratch. general examinat ion Two firm, tender cervical masses were pal-pable in the posterior neck (2 cm x 1.5 cm and 2 cm x 3 cm);otherwise the general examination was normal.  P.S. Chavis et al. 114ocular examination Visual acuity 20/20 OU; IOP 17 and 15mmHg OD/OS. Mild upper right temporal lid swelling accompaniedminimal proptosis. Extraocular muscle motility was full. Pupils andcolor vision were within normal limitis. Slit lamp biomicroscopy wasnormal. Fundus examination was unremarkable. Goldmann visual fieldswere normal. laboratory data The complete blood count revealed a leukocytecount of 3.8 x 10 9  per liter with 32% polymorphonuclear cells, 10%bands, 50% lymphocytes, 7% monocytes and 1% eosinophils. Serumheterophile, anti-nuclear antibody, anti-DNA antibody, lysozyme, an-giotensin converting enzyme, calcium and phosphorus were normal.The EBV IgM was negative, and IgG was positive; while HSV IgMwas not detectable, types I and II IgG were positive.  Apifia felis  and  Bartonella henselae  indirect fluorescent antibody titers (IFA) werenegative for both IgM and IgG antibodies. Serum protein electro-phoresis showed a polyclonal increase in gamma globulins. Chest X-ray was normal as was computed tomography (CT) of the abdomenand pelvis. However, a CT of the orbit with intravenous contrastdemonstrated enlargement of both lacrimal glands, especially the rightwhich contained a low density 4 mm area (consistent with necrosis,hemorrhage or cyst formation) within a homogeneously enhancinggland (Figure 1). The lacrimal gland biopsy showed a mild lym- Fig. 1 . Coronal CT sections aftercontrast on presentation (left)demonstrate marked enlargement of the right lacrimal gland, with a focalarea of low density superiorly, andless marked enlargement of the leftgland. Two months later (right) bothglands are reduced in size to withinnormal limits, though someasymmetry persists.  115 Kikuchi-Fujimoto disease phocytic infiltrate (Figure 2A). Lymph node biopsy revealed multi-focal areas of necrosis and benign lymphoid proliferation with abun-dant nuclear dust and numerous phagocytosing histiocytes (Figure2B). The perilymphatic tissue and fat were infiltrated by lymphocytes. course The patient was started on oral prednisone 60 mg daily andtapered over 3 weeks with concomitant antacids and Zantac. Theocular pain decreased immediately but the lacrimal gland swellingresolved only slowly over several months (Figure 1). A symptomaticdry eye developed on the right. Discussion A regional lymphadenitis affecting lymph nodes drain-ing skin and/or conjunctival sites of inoculation bears the name of Pari-naud’s oculoglandular syndrome; this has been linked to tuberculosis,tularemia, lymphogranuloma venereum, leptotrichosis and cat scratchdisease. 1-3  The former diagnoses can be easily ruled out by clinicalexamination, screening laboratory tests and clinical course.The clinical criteria of cat scratch disease (CSD) include cat contactwith the presence of a scratch, a positive CSD skin test, regional lym-phadenitis with otherwise normal laboratory studies and characteristichistopathologic changes in the lymph node biopsy. 1,4  The ocular featuresof cat scratch disease include Leber’s stellate neuroretinitis, a multifocalretinitis and branch retinal arteritis; 5,6  none of these features were presentin this patient. However, an atypical presentation of CSD must be con-sidered. About 1% of cases have no direct animal contact but may havehandled an object with which a cat has had contact. 1  Antibody to  Bartonella henselae  can be assayed by a polymerase chain reaction if thediagnosis of CSD needs to be established early in the clinical presenta-tion. 7  In addition to the fact that this patient did not have a history of  Fig. 2 . A. Lacrimal gland section; B.Lymph node section.  P.S. Chavis et al. 116 cat/kitten exposure, her  Apifia felis  and especially  B. henselae  IFA titerswere still negative months after the initial presentation, which is substan-tial evidence that bacillary angiomatosis should not be implicated. 3,4  Herclinical course, as well, was unusual for CSD in that she worseneddespite oral antibiotics and improved on corticosteroids. 4 The differential diagnosis should also include that of orbital pseudo-tumor and lymphadenopathy which could be manifestations of sarcoido-sis, tuberculosis, malignant lymphoma and systemic lupus erythematosus(SLE). When screening laboratory tests are normal, the diagnosis can bemade by nodal and/or orbital biopsy or fine needle aspirate. The histopa-thology in this patient was typical for Kikuchi-Fujimoto disease (KFD)which now can be added to the differential diagnosis of orbital pseudo-tumor. 8 This is the first reported case of ocular involvement in Kikuchi-Fuji-moto disease or subacute histiocytic necrotizing lymphadenitis. 9  Thissyndrome was described independently in 1972 by the two Japanesepathologists Kikuchi and Fujimoto. 10,11  It is typically a benign, self-limited syndrome with a characteristic histopathologic appearance onnodal biopsy. Patchy areas of necrosis are associated with nuclear frag-mentation and a proliferation of crescentic histiocytes and T-cells (Fig-ure 2B). In KFD, the lymph nodes contain plasmacytoid T-cells whichhave medium-sized nuclei with moderately condensed chromatin and aslightly basophilic cytoplasm containing tubuloreticular structures. Plas-ma cells and polymorphonuclear cells are rare. Special stains are nega-tive. 12-14  While biopsy may be necessary to make the diagnosis, KFDmust be suspected for the pathology includes atypical lymphocytes (im-munoblasts or large lymphoid cells) and nuclear debris which can mimiclymphoma to the extent that some reported patients have erroneouslyreceived chemotherapy. 13-15  The KiMIP monoclonal antibody marker forcells of monocyte-macrophage srcin can monitor plasmacytoid cells anddifferentiate inflammation from neoplasm. 16  The pathology of KFD alsoincludes necrotizing and xanthomatous lymph nodes exactly mimickingSLE except for the lack of plasma cells in KFD. 12,14,17 The typical clinical features of KFD include an oriental preponder-ance, onset in the second-fourth decade, posterior cervical lymphaden-opathy (70-98%), fever (61.5%), a rash resembling urticaria, rubella ordrug-induced erythema (25%), leukopenia with a mild granulocytopenia(53.7%) and an increased erythrocyte sedimentation rate (70%). 18-20  This32-year-old, oriental patient had a fever, posterior cervical lymphaden-itis and leukopenia with a relative lymphocytosis. Occasionally reportedinvolvement includes other nodal chains (axillary and supraclavicular),older patients, hepatosplenomegaly, leukocytosis (with circulating atyp-ical lymphocytes), aseptic meningitis and cerebellar ataxia. 18-20 Kikuchi-Fujimoto disease is recognized more by pathologists than byclinicians. 17  The exact incidence is unknown but small numbers of pa-tients have been reported from the Far East, Middle East, and the UnitedStates. It has been diagnosed in 5.7% of abnormal lymph nodes in Japanand 1.4% in Saudi Arabia. 12,20  Six cases of KFD have been recentlyreported in the United States: 2 Afro-Americans, 2 Asians, 1 Indian, and1 Caucasian. 17 The pathology of KFD suggests that the etiology is a hyperimmune  117 Kikuchi-Fujimoto disease reaction. A variety of agents have been implicated, including EBV,toxoplasmosis, Yersinia enterocolitis , HSV 6, parainfluenza virus andlymphogranuloma venereum. 12,16,18,21  Although efforts to isolate a virushave been fruitless, a viral etiology seems likely in keeping with the“tubuloreticular” structures seen in the plasmacytoid cells. 14 The clinical course is spontaneous resolution over 2-4 months withrare reported recurrences (3-4%) and even rarer mortality (due to my-ocarditis). 12,14,17  Corticosteroids have been used for neurologic and nodalinvolvement with rapid resolution of symptoms as in this reported case. 22 References 1Roberge RJ. Cat-scratch disease. SelTopics Infect Dis 1991;9(2):327-34.2Wear DJ, Malaty RH, ZimmermanLE, Hadfield TL, Margileth AM. Catscratch disease bacilli in the con- junctiva of patients with Parinaud’soculoglandular syndrome.Ophthalmology 1985;92:1282-7.3Grossniklaus HE. The cat scratchdisease-bacillary angiomatosispuzzle. Am J Ophthalmol1994;118(3):246-8.4Holley HP Jr. Sucessful treatment of cat scratch disease with cipro-floxacin. JAMA 1991;265:1563-5.5Cohen SM, Davis JL, Gass JDM.Branch retinal arterial occlusions inmultifocal retinitis with optic nerveedema. Arch Ophthalmol 1995;113:1271-6.6Ulrich GC, Waecker NJ, Meister SJ,Peterson TJ, Hooper DG. Cat scratchdisease associated with neuro-retinitis in a 6-year-old girl.Ophthalmology 1992;99(2):246-9.7Dondey JC, Sullivan TJ, RobsonJMB, Gatto J. Application of polymerase chain reaction assay inthe diagnosis of orbital granulomacomplicating atypical oculoglandu-lar cat scratch disease. Ophthalmol-ogy l997;104(7):1174-8.8Tsang WYW, Chan JKC. Fine-needle aspiration cytologicdiagnosis of Kikuchi’slymphadenitis: a report of 27 cases.Am J Clin Pathol 1994;102(4):454-8.9Dorfman RF. Histiocytic necrotizinglymphadenitis of Kikuchi andFujimoto. Arch Pathol Lab Med1987;111:1026-9.10Kikuchi M. Lymphadenitis showingfocal reticulum cell hyperplasiawith nuclear debris andphagocytosis: a clinicopathologicstudy [in Japanese]. Acta HaematolJpn 1972;35:379-80.11Fujimoto Y, Kojima Y, YamaguchiK. Cervical subacute necrotizinglymphadenitis [in Japanese]. InternMed 1972;20:920-7.12Kuo T. Kikuchi’s disease(histiocytic necrotizinglymphadenitis): a clinicopathologicstudy of 79 cases with an analysis of histologic subtypes, immunohistol-ogy, and DNA ploidy. Am J SurgPathol 1995;19(7):798-809.13Kuo T, Shih L-Y. Surgicalpathology of lymph node biopsyspecimens in Taiwan with an updateon adult T-cell leukemia/ lymphoma. In: Hanaoka M, KadinME, Mikata A, editors. LymphoidMalignancy: Immunocytology andCytogenetics. New York: Field andWood, 1990:109-15.14Dylewski J, Berry G, Pham-Dong H.An unusual cause of cervicallymphadenitis: Kikuchi-Fujimotodisease. Rev Infect Dis1991;13(5):823-5.15 Chamulak GA, Byrnes RK,Nathwani BN. Kikuchi-FujimotoDisease mimicking malignantlymphoma. Am J Surg Pathol1990;14(6):514-23.16Hansmann M-L, Kikuchi M, WackerHH, Radzun HJ, Nathwani BN,Hesse K, Parwaresch MR.Immunohistochemical monitoringof plasmacytoid cells in the lymphnode sections of Kikuchi-Fujimotodisease by a new pan-macrophageantibody KiM1P. Hum Pathol1992;23(6):676-80.17Norris AH, Krasinskas AM, SalhanyKE, Gluckman SJ. Kikuchi-Fujimotodisease: a benign cause of fever andlymphadenopathy. Am J Med1996;171:401-5.18Garcia CE, Girdhar-Gopal HV,Dorfman DM. Kikuchi-Fujimotodisease of the neck update. AnnOtol Rhinol Laryngol 1993;102:11-5.19Ikeda S, Ogawa H. Subacutenecrotizing lymphadenitis. J AmAcad Dermatol 1990;22(5):909-12.20Abba AA, Afzal M, Al-Moharab FI,Baez-Giangreco A. Kikuchi disease:a clinicopathological analysis of 13cases from Riyadh Central Hospital.Ann Saudi Med 1995;15(3):288-90.21Hollingsworth HC, Peiper SC, WeissLM, Raffeld M, Jaffe ES. Aninvestigation of the viralpathogenesis of Kikuchi-Fujimotodisease: lack of evidence forEpstein-Barr virus or humanHerpesvirus type 6 as the causativeagents. Arch Pathol Lab Med1994;118:134-40.22Naddaf H, Al-Balla SR, Salmi H,Hafeez MA. Kikuchi diseaseassociated with overlap syndromeresponding to steroids. Ann SaudiMed 1994;14(5):415-6.
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