Angiolymphoid Hyperplasia With Eosinophilia. F 55, Lower Lip

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    Angiolymphoid Hyperplasia withAngiolymphoid Hyperplasia with

    EosinophiliaEosinophilia

    Spencer Rusin M4, CUMCSpencer Rusin M4, CUMC

    Deba P Sarma, MDDeba P Sarma, MDOmahaOmaha

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    Patient PresentationPatient Presentation

    F 55, presents with a 10F 55, presents with a 10--month history of :month history of :

    NonNon--ulcerated, painless nodule (0.5 cm)ulcerated, painless nodule (0.5 cm)

    on her lower lipon her lower lip No history of trauma or earNo history of trauma or ear--nosenose--throatthroat

    disease.disease.

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    Differential DiagnosisDifferential Diagnosis

    MucoceleMucocele

    Lymphocytoma cutisLymphocytoma cutis

    Granuloma facialeGranuloma faciale Benign and malignant tumors of skin andBenign and malignant tumors of skin and

    adnexaeadnexae

    Kimura diseaseKimura disease

    OthersOthers

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    A well circumscribedA well circumscribed

    dermal noduledermal nodule

    composed of centralcomposed of centralangiomatous vascularangiomatous vascular

    proliferation withproliferation with

    stromal and peripheralstromal and peripheral

    infiltrates ofinfiltrates of

    lymphocytes andlymphocytes andeosinophils.eosinophils.

    H&E: low power

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    Proliferation of small blood vessels, lined by enlargedProliferation of small blood vessels, lined by enlargedendothelial cells (epitheliod in appearance) with uniform ovoidendothelial cells (epitheliod in appearance) with uniform ovoid

    nuclei and intracytoplasmic vacuoles.nuclei and intracytoplasmic vacuoles.

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    Prominent eosinophilic and lymphocytic infiltrationProminent eosinophilic and lymphocytic infiltration

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    Lymphoid aggregate with follicle formation amongst theLymphoid aggregate with follicle formation amongst the

    vascular proliferative cells.vascular proliferative cells.

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    ImmunostainsImmunostains

    CD 3CD 3 Peripheral lymphocytes: PositivePeripheral lymphocytes: Positive

    CD 20CD 20 -- Peripheral lymphocytes: PositivePeripheral lymphocytes: Positive

    CD31CD31 Vascular epitheliod endothelial cells:Vascular epitheliod endothelial cells:PositivePositive

    CK AE1/3CK AE1/3 -- NegativeNegative

    SS--100100 -- NegativeNegative

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    CD 31 stain highlights the endothelial cells demonstrating aCD 31 stain highlights the endothelial cells demonstrating a

    strong angiogenesis component to the nodule.strong angiogenesis component to the nodule.

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    DiagnosisDiagnosis

    Angiolymphoid hyperplasia with eosinophiliaAngiolymphoid hyperplasia with eosinophilia

    (ALHE)(ALHE)

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    Differential DiagnosisDifferential Diagnosis

    ALHEALHE

    Primarily a localizedPrimarily a localizedhyperplasiahyperplasia

    InfrequentInfrequentlymphadenopathylymphadenopathy

    20%20%

    Rare blood eosinophliaRare blood eosinophlia

    Histology:Histology:

    VascularVascularproliferation>inflammaproliferation>inflammatory cellstory cells

    Epitheliod endothelialEpitheliod endothelialcells lining bloodcells lining bloodvesselsvessels

    Eosinophils presentEosinophils present

    Kimura DiseaseKimura Disease

    Systemic involvement:Systemic involvement: LymphadenopathyLymphadenopathy Blood eosinophliaBlood eosinophlia Nephrotic syndrome asNephrotic syndrome as

    a result of glomerulara result of glomerularIgE deposition.IgE deposition.

    Histological presentation ofHistological presentation ofKimura disease differs fromKimura disease differs from

    ALHE in two factors.ALHE in two factors. Vascular proliferationVascular proliferation

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    ALHEALHE

    General presentation:General presentation:

    Range from asymptomatic to itchy orRange from asymptomatic to itchy or

    painful erythematous nodules, 2painful erythematous nodules, 2--3cm in3cm indiameter.diameter.

    The nodules may pulsate or bleed.The nodules may pulsate or bleed.

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    PrePre--auricular lesions of ALHE.auricular lesions of ALHE.

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    ALHEALHE Hypotheses regarding ALHEs origin:Hypotheses regarding ALHEs origin:

    Reactive process to insect bitesReactive process to insect bites

    Hyperestrogen statesHyperestrogen states

    Immunologic mechanismsImmunologic mechanisms

    Reactive vascular proliferation subsequent toReactive vascular proliferation subsequent toinflammation associated with traumatized bloodinflammation associated with traumatized blood

    vesselsvessels One study reported a history of trauma in only 9%One study reported a history of trauma in only 9%

    of 116 patients with ALHEof 116 patients with ALHE

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    ALHEALHE

    Age: 20Age: 20--50 years, M = F50 years, M = F

    Locations affected by ALHE:Locations affected by ALHE:

    Head and neck:Head and neck:

    Specifically the forehead, scalp, and skin aroundSpecifically the forehead, scalp, and skin aroundears.ears.

    Trunk and genitaliaTrunk and genitalia

    Three documented cases of ALHE affecting the lip.Three documented cases of ALHE affecting the lip.

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    ALHEALHE

    Progression of ALHE:Progression of ALHE:

    Most common course: ALHE remains stableMost common course: ALHE remains stable

    Infrequent outcome: ALHE spontaneouslyInfrequent outcome: ALHE spontaneouslyregressesregresses

    Chronic nature of ALHE necessitates treatment.Chronic nature of ALHE necessitates treatment.

    Recurrence rate ranges from 33Recurrence rate ranges from 33--50%50%

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    TreatmentTreatment

    Medical:Medical:

    IsoretinoinIsoretinoin

    CorticosteroidsCorticosteroids

    interferon alfainterferon alfa--2b2b

    Benefits:Benefits:

    Improved cosmetic outcomesImproved cosmetic outcomes

    Limitations:Limitations:

    Relies on patient complianceRelies on patient compliance Not a permanent cureNot a permanent cure

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    TreatmentTreatment

    Surgical:Surgical:

    Laser therapyLaser therapy

    Carbon dioxide laserCarbon dioxide laser

    Ultralong pulsed dye laserUltralong pulsed dye laser

    Nd:Nd:YAG laserYAG laser

    Benefits:Benefits:

    Improved cosmetic outcomeImproved cosmetic outcome

    Limitations:Limitations: Multiple treatmentsMultiple treatments

    Adversely affected by the depth of invasion orAdversely affected by the depth of invasion orsize of vesselssize of vessels

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    TreatmentTreatment

    Surgical:Surgical:

    ExcisionalExcisional

    Simple surgical excisionSimple surgical excision

    Mohs surgeryMohs surgery

    Benefits:Benefits:

    Excision of the arterial and venous segments atExcision of the arterial and venous segments atthe base decrease recurrencethe base decrease recurrence

    Limitations:Limitations: ScarringScarring

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    ReferencesReferences

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    S.W. Weiss, J.R. Goldblum, "Enzinger and Weiss's Soft Tissue Tumors, 4th edition," St.S.W. Weiss, J.R. Goldblum, "Enzinger and Weiss's Soft Tissue Tumors, 4th edition," St.Louis: Mosby, 2001.Louis: Mosby, 2001. 863863--864.864.

    G.C. Wells, I.W. Whimster, "Subcutaneous angiolymphoid hyperplasia with eosinophilia,G.C. Wells, I.W. Whimster, "Subcutaneous angiolymphoid hyperplasia with eosinophilia,British Journal of DermatologyBritish Journal of Dermatology, vol. 81, pp 1, vol. 81, pp 1--15, 1969.15, 1969.

    R.L. Moy, D.B. Luftman, Q.H. Nguyen, J.S. Amenta,"Estrogen receptors and the response toR.L. Moy, D.B. Luftman, Q.H. Nguyen, J.S. Amenta,"Estrogen receptors and the response tosex hormones in angiolymphoid hyperplasia with eosinophilia,"sex hormones in angiolymphoid hyperplasia with eosinophilia," Archives of DermatologyArchives of Dermatology,,vol 128, pp. 825vol 128, pp. 825--828, 1992.828, 1992.

    R. Grimwood, J.M. Swinehart, J.L Aeling, "Angiolymphoid hyperplasia with eosinophilia,"R. Grimwood, J.M. Swinehart, J.L Aeling, "Angiolymphoid hyperplasia with eosinophilia,"Archives of DermatologyArchives of Dermatology, vol. 115, pp. 205, vol. 115, pp. 205--207, 1979.207, 1979.

    P. Von den Driesch, M. Gruschwitz, H. Schell, W. Sterry, Distribution of adhesionP. Von den Driesch, M. Gruschwitz, H. Schell, W. Sterry, Distribution of adhesionmolecules, IgE, and CD23 in a case of angiolymphoid hyperplasia with eosinophilia,molecules, IgE, and CD23 in a case of angiolymphoid hyperplasia with eosinophilia,Journal of the American Academy of DermatologyJournal of the American Academy of Dermatology, vol. 26, issue 5, part 2, pp. 799, vol. 26, issue 5, part 2, pp. 799--804,804,1992.1992.

    T.G. Olsen, E.B. Helwig, "Angiolymphoid hyperplasia with eosinophilia,"T.G. Olsen, E.B. Helwig, "Angiolymphoid hyperplasia with eosinophilia," Journal ofJournal ofAmerican Academy of DermatologyAmerican Academy of Dermatology, vol 12, pp. 781, vol 12, pp. 781--796, 1985.796, 1985.

    P.G. Henry, J.W. Burnett, Angiolymphoid hyperplasia with eosinophilia,P.G. Henry, J.W. Burnett, Angiolymphoid hyperplasia with eosinophilia, Archives ofArchives ofDermatologyDermatology, vol. 114, no. 8, pp. 1168, vol. 114, no. 8, pp. 1168--1172, 1978.1172, 1978.

    J.F. Fetsch, S.W. Weiss, Observations concerning the pathogenesis of epithelioidJ.F. Fetsch, S.W. Weiss, Observations concerning the pathogenesis of epithelioidhemangioma (angiolymphoid hyperplasia),hemangioma (angiolymphoid hyperplasia), Modern PathologyModern Pathology, vol. 4, issue 4, pp. 449, vol. 4, issue 4, pp. 449--455,455,1991.1991.

    T.G. Olsen, E.B. Helwig, Angiolymphoid hyperplasia with eosinophilia. A clinicopathologicT.G. Olsen, E.B. Helwig, Angiolymphoid hyperplasia with eosinophilia. A clinicopathologicstudy of 118 patients,study of 118 patients, Journal of the American Academy of DermatologyJournal of the American Academy of Dermatology, vol. 12, issue 5,, vol. 12, issue 5,pp. 781pp. 781--796, 1985.796, 1985.

    J. Scurry, G. Dennerstein, J. Brenan, Angiolymphoid hyperplasia with eosinophilia of theJ. Scurry, G. Dennerstein, J. Brenan, Angiolymphoid hyperplasia with eosinophilia of thevulva,vulva, Australian and New Zealand Journal of Obstetrics and GynaecologyAustralian and New Zealand Journal of Obstetrics and Gynaecology, vol. 35, issue 3,, vol. 35, issue 3,pp. 347pp. 347--348, 1995.348, 1995.

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    References continuedReferences continued

    J.R. Srigley, A.G. Ayala, N.G. Ordonez, A.W. van Nostrand, Epithelioid hemangioma of theJ.R. Srigley, A.G. Ayala, N.G. Ordonez, A.W. van Nostrand, Epithelioid hemangioma of thepenis. A rare and distinctive vascular lesion,penis. A rare and distinctive vascular lesion, Archives of Pathology and LaboratoryArchives of Pathology and LaboratoryMedicineMedicine, vol. 109, pp. 51, vol. 109, pp. 51--54, 1985.54, 1985.

    J.I. Lopez, S.B. Battaglino, Angiolymphoid hyperplasia with eosinophilia of the lower lip,J.I. Lopez, S.B. Battaglino, Angiolymphoid hyperplasia with eosinophilia of the lower lip,International Journal of DermatologyInternational Journal of Dermatology, vol. 32, issue 5, pp. 361, vol. 32, issue 5, pp. 361--362, 1993.362, 1993.

    H. Suzuki, A. Hatamochi, M. Horie, T. Suzuki, S. Yamazaki, A case of angiolymphoidH. Suzuki, A. Hatamochi, M. Horie, T. Suzuki, S. Yamazaki, A case of angiolymphoidhyperplasia with eosinophilia (ALHE) of the upper lip,hyperplasia with eosinophilia (ALHE) of the upper lip, Journal of DermatologyJournal of Dermatology, vol. 32, no., vol. 32, no.12, pp. 99112, pp. 991--995, 2005.995, 2005.

    O.F. Salinas, Y.S. Corredoira, G.A. Rojas, O.F. Salinas, Y.S. Corredoira, G.A. Rojas, Angiolymphoid hyperplasia of the lip withAngiolymphoid hyperplasia of the lip witheosinophilia. Report of one case,eosinophilia. Report of one case, Revista Medica de ChileRevista Medica de Chile, vol. 135, no.5, pp. 636, vol. 135, no.5, pp. 636--639,639,2007. (in Spanish)2007. (in Spanish)

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    B.V. Diaz, M.C. Lenoir, A. Ladoux, C. Frelin, M. Demarchez, S. Michel, Regulation ofB.V. Diaz, M.C. Lenoir, A. Ladoux, C. Frelin, M. Demarchez, S. Michel, Regulation ofvascular endothelial growth factor expression in human keratinocytes by retinoids,vascular endothelial growth factor expression in human keratinocytes by retinoids,Journal of Biological ChemistryJournal of Biological Chemistry, vol. 275, no. 1, pps. 642, vol. 275, no. 1, pps. 642--650, 2000.650, 2000.

    F. El Sayed, R. Dhaybi, A. Ammoury, M. Chababi, Angiolymphoid hyperplasia withF. El Sayed, R. Dhaybi, A. Ammoury, M. Chababi, Angiolymphoid hyperplasia witheosinophilia: efficacy of isotretinoin?,eosinophilia: efficacy of isotretinoin?, Head & Face MedicineHead & Face Medicine, vol. 2, p. 32, vol. 2, p. 32--36, 2006.36, 2006. T. Kaur, K. Sandhu, S. Gupta, A.J. Kanwar, B. Kumar, Treatment of angiolymphoidT. Kaur, K. Sandhu, S. Gupta, A.J. Kanwar, B. Kumar, Treatment of angiolymphoid

    hyperplasia with eosinophilia with the carbon dioxide laser,hyperplasia with eosinophilia with the carbon dioxide laser, Journal of DermatologicalJournal of DermatologicalTreatmentTreatment, vol. 15, issue 5, pps. 328, vol. 15, issue 5, pps. 328--330, 2004.330, 2004.

    C. Angel, A. Lewis, T. Griffin, E. Levy, A. Benedetto, Angiolymphoid hyperplasiaC. Angel, A. Lewis, T. Griffin, E. Levy, A. Benedetto, Angiolymphoid hyperplasiasuccessfully treated with an ultralong pulsed dye laser,successfully treated with an ultralong pulsed dye laser, Dermatologic SurgeryDermatologic Surgery, vol. 31, pps., vol. 31, pps.713713--716, 2005.716, 2005.

    C.J. Miller, M.D. Ioffreda, C.T. Ammirati, Mohs micrographic surgery for angiolymphoidC.J. Miller, M.D. Ioffreda, C.T. Ammirati, Mohs micrographic surgery for angiolymphoidhyperplasia with eosinophilia,hyperplasia with eosinophilia, Dermatological SurgeryDermatological Surgery, vol 30,issue 8, pps. 1169, vol 30,issue 8, pps. 1169--1173,1173,2004.2004.

    T. Rohrer, A.Allan, ANgiolymphoid hyperplasia with eosinophilia successfully treated withT. Rohrer, A.Allan, ANgiolymphoid hyperplasia with eosinophilia successfully treated witha longa long--pulsed tunable dye laser,pulsed tunable dye laser, Dermatologic SurgeryDermatologic Surgery, vol. 26, issue 3, pps. 211, vol. 26, issue 3, pps. 211--214,214,