Angiolymphoid Hyperplasia with Eosinophilia

Spencer Rusin M4, CUMC Deba P Sarma, MD Omaha

Patient Presentation
F 55, presents with a 10-month history of : – Non-ulcerated, painless nodule (0.5 cm) on her lower lip No history of trauma or ear-nose-throat disease.

Differential Diagnosis
Mucocele Lymphocytoma cutis Granuloma faciale Benign and malignant tumors of skin and adnexae Kimura disease Others

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H&E: low power

A well circumscribed dermal nodule composed of central angiomatous vascular proliferation with stromal and peripheral infiltrates of lymphocytes and eosinophils.

Proliferation of small blood vessels, lined by enlarged endothelial cells (epitheliod in appearance) with uniform ovoid nuclei and intracytoplasmic vacuoles.

Prominent eosinophilic and lymphocytic infiltration

Lymphoid aggregate with follicle formation amongst the vascular proliferative cells.

Immunostains

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CD 3 – Peripheral lymphocytes: Positive CD 20 - Peripheral lymphocytes: Positive CD31 – Vascular epitheliod endothelial cells: Positive CK AE1/3 - Negative S-100 - Negative

CD 31 stain highlights the endothelial cells demonstrating a strong angiogenesis component to the nodule.

Diagnosis

Angiolymphoid hyperplasia with eosinophilia (ALHE)

Differential Diagnosis
ALHE

Kimura Disease

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Primarily a localized hyperplasia Infrequent lymphadenopathy • 20% Rare blood eosinophlia Histology: • Vascular proliferation>inflamma tory cells • Epitheliod endothelial cells lining blood vessels • Eosinophils present

Systemic involvement: • Lymphadenopathy • Blood eosinophlia • Nephrotic syndrome as a result of glomerular IgE deposition. Histological presentation of Kimura disease differs from ALHE in two factors. • Vascular proliferation <inflammatory cells • Blood vessels are lined by attenuated endothelial cells, not epitheliod endothelial cells

ALHE

General presentation: – Range from asymptomatic to itchy or painful erythematous nodules, 2-3cm in diameter. – The nodules may pulsate or bleed.

Pre-auricular lesions of ALHE.

ALHE

Hypotheses regarding ALHE’s origin:
– Reactive process to insect bites – Hyperestrogen states – Immunologic mechanisms – Reactive vascular proliferation subsequent to inflammation associated with traumatized blood vessels

One study reported a history of trauma in only 9% of 116 patients with ALHE

ALHE
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Age: 20-50 years, M = F Locations affected by ALHE: • Head and neck:  Specifically the forehead, scalp, and skin around ears. • Trunk and genitalia • Three documented cases of ALHE affecting the lip.

ALHE

Progression of ALHE: – Most common course: ALHE remains stable – Infrequent outcome: ALHE spontaneously regresses Chronic nature of ALHE necessitates treatment. – Recurrence rate ranges from 33-50%

Treatment

Medical: – Isoretinoin – Corticosteroids – interferon alfa-2b Benefits: – Improved cosmetic outcomes Limitations: – Relies on patient compliance – Not a permanent cure

Treatment

Surgical: – Laser therapy  Carbon dioxide laser  Ultralong pulsed dye laser  Nd:YAG laser – Benefits:  Improved cosmetic outcome – Limitations:  Multiple treatments  Adversely affected by the depth of invasion or size of vessels

Treatment

Surgical: – Excisional  Simple surgical excision  Moh’s surgery – Benefits:  Excision of the arterial and venous segments at the base decrease recurrence – Limitations:  Scarring

References

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S. Seregard, “Angiolymphoid hyperplasia with eosinophilia should not be confused with Kimura's disease,” Acta Ophthalmologica Scandinavica, vol. 79, issue 1, pps. 91–93, 2001. S.W. Weiss, J.R. Goldblum, "Enzinger and Weiss's Soft Tissue Tumors, 4th edition," St. Louis: Mosby, 2001. 863-864. G.C. Wells, I.W. Whimster, "Subcutaneous angiolymphoid hyperplasia with eosinophilia, British Journal of Dermatology, vol. 81, pp 1-15, 1969. R.L. Moy, D.B. Luftman, Q.H. Nguyen, J.S. Amenta,"Estrogen receptors and the response to sex hormones in angiolymphoid hyperplasia with eosinophilia," Archives of Dermatology, vol 128, pp. 825-828, 1992. R. Grimwood, J.M. Swinehart, J.L Aeling, "Angiolymphoid hyperplasia with eosinophilia," Archives of Dermatology, vol. 115, pp. 205-207, 1979. P. Von den Driesch, M. Gruschwitz, H. Schell, W. Sterry, “ Distribution of adhesion molecules, IgE, and CD23 in a case of angiolymphoid hyperplasia with eosinophilia,” Journal of the American Academy of Dermatology, vol. 26, issue 5, part 2, pp. 799-804, 1992. T.G. Olsen, E.B. Helwig, "Angiolymphoid hyperplasia with eosinophilia," Journal of American Academy of Dermatology, vol 12, pp. 781-796, 1985. P.G. Henry, J.W. Burnett, “Angiolymphoid hyperplasia with eosinophilia,” Archives of Dermatology, vol. 114, no. 8, pp. 1168-1172, 1978. J.F. Fetsch, S.W. Weiss, “Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia),” Modern Pathology, vol. 4, issue 4, pp. 449-455, 1991. T.G. Olsen, E.B. Helwig, “Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 118 patients,” Journal of the American Academy of Dermatology, vol. 12, issue 5, pp. 781-796, 1985. J. Scurry, G. Dennerstein, J. Brenan, “Angiolymphoid hyperplasia with eosinophilia of the vulva,” Australian and New Zealand Journal of Obstetrics and Gynaecology, vol. 35, issue 3, pp. 347-348, 1995.

References continued

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J.R. Srigley, A.G. Ayala, N.G. Ordonez, A.W. van Nostrand, “Epithelioid hemangioma of the penis. A rare and distinctive vascular lesion,” Archives of Pathology and Laboratory Medicine, vol. 109, pp. 51-54, 1985. J.I. Lopez, S.B. Battaglino, “Angiolymphoid hyperplasia with eosinophilia of the lower lip,” International Journal of Dermatology, vol. 32, issue 5, pp. 361-362, 1993. H. Suzuki, A. Hatamochi, M. Horie, T. Suzuki, S. Yamazaki, “A case of angiolymphoid hyperplasia with eosinophilia (ALHE) of the upper lip,” Journal of Dermatology, vol. 32, no. 12, pp. 991-995, 2005. O.F. Salinas, Y.S. Corredoira, G.A. Rojas, “Angiolymphoid hyperplasia of the lip with eosinophilia. Report of one case,” Revista Medica de Chile, vol. 135, no.5, pp. 636-639, 2007. (in Spanish) A. Satpathy, C. Moss, F. Raafat, R. Slator, “Spontaneous regression of a rare tumour in a child: angiolymphoid hyperplasia with eosinophilia of the hand: case report and review of the literature,” British Journal of Plastic Surgery, vol. 58, issue 6, pps. 865-868, 2005. B.V. Diaz, M.C. Lenoir, A. Ladoux, C. Frelin, M. Demarchez, S. Michel, “Regulation of vascular endothelial growth factor expression in human keratinocytes by retinoids,” Journal of Biological Chemistry, vol. 275, no. 1, pps. 642-650, 2000. F. El Sayed, R. Dhaybi, A. Ammoury, M. Chababi, “Angiolymphoid hyperplasia with eosinophilia: efficacy of isotretinoin?”, Head & Face Medicine, vol. 2, p. 32-36, 2006. T. Kaur, K. Sandhu, S. Gupta, A.J. Kanwar, B. Kumar, “Treatment of angiolymphoid hyperplasia with eosinophilia with the carbon dioxide laser,” Journal of Dermatological Treatment, vol. 15, issue 5, pps. 328-330, 2004. C. Angel, A. Lewis, T. Griffin, E. Levy, A. Benedetto, “Angiolymphoid hyperplasia successfully treated with an ultralong pulsed dye laser,” Dermatologic Surgery, vol. 31, pps. 713-716, 2005. C.J. Miller, M.D. Ioffreda, C.T. Ammirati, “Mohs micrographic surgery for angiolymphoid hyperplasia with eosinophilia,” Dermatological Surgery, vol 30,issue 8, pps. 1169-1173, 2004. T. Rohrer, A.Allan, “ANgiolymphoid hyperplasia with eosinophilia successfully treated with a long-pulsed tunable dye laser,” Dermatologic Surgery, vol. 26, issue 3, pps. 211-214,