History

Patients with angiolymphoid hyperplasia with eosinophilia (ALHE) typically present with an expanding flesh-color to erythematous nodule or group of nodules, usually in the vicinity of the ear (see the image below). The lesion(s) may be associated with pain or pruritus. Uncommon symptoms include pulsation and spontaneous bleeding.

Angiolymphoid hyperplasia with eosinophilia typically exhibits flesh-color to erythematous nodules in the vicinity of the ear.

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Physical Examination

Angiolymphoid hyperplasia with eosinophilia (ALHE) typically appears as dome-shaped, smooth-surfaced papules or nodules (see the image below). Approximately 85% of lesions occur in the skin of the head and neck; most of them are on or near the ear or on the forehead or scalp. The extremities are the next most common site. Involvement at other sites is rare. However, case reports have described ALHE affecting the penis^[19]and the conjunctiva.^[20]

Pronounced erythema and nodularity due to angiolymphoid hyperplasia with eosinophilia.

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The lesions range from erythematous to brown, and they may be eroded or crusted. Approximately 80% of patients present with isolated lesions, while the remaining patients usually demonstrate grouped papules or nodules in a single region. Rarely, the lesions may be pulsatile. Most lesions are 0.5-2 cm in diameter, with a range of 0.2-8 cm. Larger nodules tend to be deeply centered within the subcutis.

Complications

Conductive hearing loss resulting from obstruction of the auditory canal can occur in severe cases of angiolymphoid hyperplasia with eosinophilia (ALHE). Diplopia and proptosis was noted in one patient with orbital involvement.

Differential Diagnoses

Hamilton TK, Baughman RD, Perry AE. Persistent pruritic plaque of the ear. Arch Dermatol. 1999 Apr. 135(4):464-5, 467-8. [QxMD MEDLINE Link].
Arnold M, Geilen CC, Coupland SE, Krengel S, Dippel E, Spröder J, et al. Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand. J Cutan Pathol. 1999 Oct. 26(9):436-40. [QxMD MEDLINE Link].
Chen JF, Gao HW, Wu BY, Tsai WC, Chiang CP. Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: a rare case report with molecular evidence of T-cell clonality. J Dermatol. 2010 Apr. 37(4):355-9. [QxMD MEDLINE Link].
Chan JK, Hui PK, Ng CS, Yuen NW, Kung IT, Gwi E. Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura's disease in Chinese. Histopathology. 1989 Dec. 15(6):557-74. [QxMD MEDLINE Link].
Googe PB, Harris NL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. J Cutan Pathol. 1987 Oct. 14(5):263-71. [QxMD MEDLINE Link].
Tsuboi H, Masuzawa M, Katsuoka K. Angiolymphoid hyperplasia with eosinophilia affecting the nail bed and underlying bone. J Dermatol. 2006 Jun. 33(6):399-402. [QxMD MEDLINE Link].
Esmaili DD, Chang EL, O'Hearn TM, Smith RE, Rao NA. Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia. Ophthal Plast Reconstr Surg. 2008 Jul-Aug. 24(4):310-1. [QxMD MEDLINE Link].
Zarrin-Khameh N, Spoden JE, Tran RM. Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature. Arch Pathol Lab Med. 2005 Sep. 129(9):1168-71. [QxMD MEDLINE Link].
Marcum CB, Zager JS, Bélongie IP, Messina JL, Fenske NA. Profound proliferating angiolymphoid hyperplasia with eosinophilia of pregnancy mimicking angiosarcoma. Cutis. 2011 Sep. 88(3):122-8. [QxMD MEDLINE Link].
Angiolymphoid Hyperplasia with Eosinophilia and Kimura Disease. Wolff, Goldsmith, Katz, Gilchrest, Paller, Leffell, eds. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Medical; 2008. Vol 1: 313-14.
Joshi R. Angiolymphoid hyperplasia with follicular mucinosis. Indian J Dermatol Venereol Leprol. 2007 Sep-Oct. 73(5):346-7. [QxMD MEDLINE Link].
Kempf W, Haeffner AC, Zepter K, et al. Angiolymphoid hyperplasia with eosinophilia: evidence for a T-cell lymphoproliferative origin. Hum Pathol. 2002 Oct. 33(10):1023-9. [QxMD MEDLINE Link].
Andreae J, Galle C, Magdorf K, et al. Severe atherosclerosis of the aorta and development of peripheral T-cell lymphoma in an adolescent with angiolymphoid hyperplasia with eosinophilia. Br J Dermatol. 2005 May. 152(5):1033-8. [QxMD MEDLINE Link].
Gonzalez-Cuyar LF, Tavora F, Zhao XF, Wang G, Auerbach A, Aguilera N, et al. Angiolymphoid hyperplasia with eosinophilia developing in a patient with history of peripheral T-cell lymphoma: evidence for multicentric T-cell lymphoproliferative process. Diagn Pathol. 2008 May 29. 3:22. [QxMD MEDLINE Link].
Jeon EK, Cho AY, Kim MY, Lee Y, Seo YJ, Park JK, et al. Angiolymphoid hyperplasia with eosinophilia that was possibly induced by vaccination in a child. Ann Dermatol. 2009 Feb. 21(1):71-4. [QxMD MEDLINE Link]. [Full Text].
Korekawa A, Kaneko T, Hagiwara C, et al. Angiolymphoid hyperplasia with eosinophilia in infancy. J Dermatol. 2012 May 17. [QxMD MEDLINE Link].
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Wang YH, Yin HF. [One patient with Kimura's disease and angiolymphoid hyperplasia with eosinophilia also suffers from kidney injury]. Beijing Da Xue Xue Bao. 2008 Aug 18. 40(4):405-7. [QxMD MEDLINE Link].
Dewan P, Francis ND, Lear JT, Bunker CB. Angiolymphoid hyperplasia with eosinophilia affecting the penis. Br J Dermatol. Sept 2008. 159(3):755-7. [QxMD MEDLINE Link].
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Hamaguchi Y, Fujimoto M, Matsushita Y, Kitamura-Sawada S, Kawano M, Takehara K. IgG4-related skin disease, a mimic of angiolymphoid hyperplasia with eosinophilia. Dermatology. 2011. 223(4):301-5. [QxMD MEDLINE Link].
Llamas-Velasco M, Kempf W, Cota C, Fernández-Figueras MT, Lee J, Ferrara G, et al. Multiple Eruptive Epithelioid Hemangiomas: A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B. Am J Surg Pathol. 2019 Jan. 43 (1):26-34. [QxMD MEDLINE Link].
Ortins-Pina A, Llamas-Velasco M, Turpin S, Soares-de-Almeida L, Filipe P, Kutzner H. FOSB immunoreactivity in endothelia of epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia). J Cutan Pathol. 2018 Jun. 45 (6):395-402. [QxMD MEDLINE Link].
Lembo S, Balato A, Cirillo T, Balato N. A Long-Term Follow-Up of Angiolymphoid Hyperplasia with Eosinophilia Treated by Corticosteroids: When a Traditional Therapy is Still Up-to-Date. Case Rep Dermatol. 2011 Mar 5. 3(1):64-7. [QxMD MEDLINE Link]. [Full Text].
Horst C, Kapur N. Propranolol: a novel treatment for angiolymphoid hyperplasia with eosinophilia. Clin Exp Dermatol. 2014 Oct. 39(7):810-2. [QxMD MEDLINE Link].
Chacon AH, Mercer J. Successful management of angiolymphoid hyperplasia with eosinophilia in a split-face trial of topical tacrolimus and timolol solution. G Ital Dermatol Venereol. 2014 Jul 11. [QxMD MEDLINE Link].
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Harada S, Nomura T, Miyauchi T, Shinkuma S, Fujita Y, Arita K, et al. Complete remission of angiolymphoid hyperplasia with eosinophilia using topical tacrolimus. Eur J Dermatol. 2017 Apr 1. 27 (2):194-196. [QxMD MEDLINE Link].
Braun-Falco M, Fischer S, Plötz SG, Ring J. Angiolymphoid hyperplasia with eosinophilia treated with anti-interleukin-5 antibody (mepolizumab). Br J Dermatol. 2004 Nov. 151(5):1103-4. [QxMD MEDLINE Link].
Carlesimo M, Mari E, Tammaro A, Persechino S, Camplone G. Angiolymphoid hyperplasia with eosinophilia treated with isotretinoin. Eur J Dermatol. 2007 Nov-Dec. 17(6):554-5. [QxMD MEDLINE Link].
Abrahamson TG, Davis DA. Angiolymphoid hyperplasia with eosinophilia responsive to pulsed dye laser. J Am Acad Dermatol. 2003 Aug. 49(2 Suppl Case Reports):S195-6. [QxMD MEDLINE Link].
Sotiriou E, Apalla Z, Patsatsi A, Panagiotidou DD, Ioannides D. Angiolymphoid hyperplasia with eosinophilia: good response to photodynamic therapy. Clin Exp Dermatol. 2009 Dec. 34(8):e629-31. [QxMD MEDLINE Link].
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Media Gallery

Angiolymphoid hyperplasia with eosinophilia typically exhibits flesh-color to erythematous nodules in the vicinity of the ear.
Pronounced erythema and nodularity due to angiolymphoid hyperplasia with eosinophilia.
Histologically, angiolymphoid hyperplasia with eosinophilia is marked by thick-walled blood vessels with protuberant endothelium and a prominent inflammatory infiltrate, which typically includes eosinophils.

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Author

Gauri Panse, MBBS, MD Fellow in Dermatopathology, Department of Dermatology, Yale University School of Medicine

Gauri Panse, MBBS, MD is a member of the following medical societies: American Society for Clinical Pathology, American Society of Dermatopathology, College of American Pathologists, International Society of Dermatopathology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Coauthor(s)

Earl J Glusac, MD Professor, Departments of Pathology and Dermatology, Yale University School of Medicine

Earl J Glusac, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Jon H Meyerle, MD Assistant Professor, Department Dermatology, Uniformed Services University of the Health Sciences; Assistant Professor, Department of Dermatology, Johns Hopkins University School of Medicine; Chief, Immunodermatology, Walter Reed National Military Medical Center

Jon H Meyerle, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Sarah K Taylor, MD Staff Physician, Eisenhower Army Medical Center Dermatology, Ft Gordon

Disclosure: Nothing to disclose.

Acknowledgements

Ashley R Mason, MD Dermatopathology Fellow, Department of Dermatology, Yale University School of Medicine

Ashley R Mason, MD is a member of the following medical societies: American Academy of Dermatology and International Society of Dermatopathology

Disclosure: Nothing to disclose.