eMedicine Specialties > Dermatology > Diseases of the Vessels

Angiolymphoid Hyperplasia With Eosinophilia

Author: Sarah K Taylor, MD, Staff Physician, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center
Coauthor(s): Jon H Meyerle, MD, Assistant Professor, Department of Dermatology, Johns Hopkins University School of Medicine; Consulting Staff, Laboratory Director, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center; Earl J Glusac, MD, Professor, Departments of Pathology and Dermatology, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Dec 11, 2008

Introduction

Background

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic condition that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. Most patients present with lesions in the periauricular region,1 forehead, or scalp. Rare sites of involvement include the hands,2 shoulders, breasts, penis, oral mucosa, and orbit.

A distinct pathologic entity, ALHE is marked by a proliferation of blood vessels with distinctive large endothelial cells. These blood vessels are accompanied by a characteristic inflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistent and is difficult to eradicate. Whether ALHE represents a benign neoplasm or an unusual reaction to varied stimuli, including trauma, remains unclear.
 
While ALHE shows some similarity to Kimura disease, it is generally regarded as a separate entity.3,4 While ALHE lesions are superficial, Kimura disease involves deeper tissues such as lymph nodes, salivary glands, and the subcutis. However, a 2006 report describes ALHE involving the nail bed and underlying bone.5 Further, no reports describe a patient with simultaneous tumors, one consistent with Kimura disease and one consistent with ALHE.6 Such findings challenge whether or not Kimura disease and ALHE represent a spectrum of the same disease.

Pathophysiology

Although ALHE may be a benign tumor, numerous factors suggest that it is an unusual reactive process. Approximately half the patients have multifocal lesions that are grouped anatomically. ALHE has occurred following various forms of trauma or infection. Histologically, most cases of ALHE show damaged and/or tortuous arteries and veins at the base of the lesion, suggesting that arteriovenous shunting may play a role in the pathogenesis. Hyperestrogenemia (eg, in pregnancy,7 with oral contraceptive use) may foster lesion growth. Additionally, the distinctive inflammatory infiltrate in ALHE appears to be an intrinsic (not secondary) component of the lesion. Approximately 20% of patients have blood eosinophilia. However, immunoglobulin E levels are not elevated.8

Additionally, 3 reported cases describe follicular mucinosis and ALHE occurring in the same biopsy specimen.9 Interestingly, one report of monoclonality in a patient with ALHE who subsequently progressed to mycosis fungoides raises the question of whether or not ALHE could be an early form of T-cell lymphoma.10 It should be stressed that most cases are entirely benign.

Frequency

United States

Frequency in the United States is unknown. ALHE is uncommon but not rare.

International

Although frequency is unknown, cases have been reported worldwide. ALHE is uncommon but not rare; it may be more common in Japan than in other countries.

Mortality/Morbidity

ALHE can persist for years, but serious complications (eg, malignant transformation) do not occur. A few cases of nephropathy have been reported in patients with ALHE; however, the association is not strong. This is in contrast to the related entity, Kimura disease, for which the association with nephrotic syndrome is strong. Of note, although, coexistence of Kimura disease and ALHE in the same patient, along with minimal-change glomerulopathy, has been reported.11

Race

ALHE is seen most commonly in Asians, followed by whites. Although less common, blacks can develop ALHE.

Sex

ALHE is slightly more common in females; however, a male predominance has been noted in selected Asian studies.

Age

ALHE presents most commonly in patients aged 20-50 years, with mean onset of 30-33 years. This condition is rare in elderly patients and in the non-Asian pediatric population.

Clinical

History

Patients with angiolymphoid hyperplasia with eosinophilia (ALHE) typically present with an expanding nodule or group of nodules, usually in the vicinity of the ear (see Media File 1). The lesion(s) may be associated with pain or pruritus. Uncommon symptoms include pulsation and spontaneous bleeding.

Physical

Angiolymphoid hyperplasia with eosinophilia (ALHE) typically appears as dome-shaped, smooth-surfaced papules or nodules (see Media File 2). 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 lesions range from erythematous to brown and 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.

Causes

Angiolymphoid hyperplasia with eosinophilia (ALHE) is idiopathic. Whether this condition is a neoplastic or reactive state is uncertain; a reactive cause is favored.

More on Angiolymphoid Hyperplasia With Eosinophilia

Overview: Angiolymphoid Hyperplasia With Eosinophilia
Differential Diagnoses & Workup: Angiolymphoid Hyperplasia With Eosinophilia
Treatment & Medication: Angiolymphoid Hyperplasia With Eosinophilia
Follow-up: Angiolymphoid Hyperplasia With Eosinophilia
Multimedia: Angiolymphoid Hyperplasia With Eosinophilia
References
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References

  1. Hamilton TK, Baughman RD, Perry AE. Persistent pruritic plaque of the ear. Arch Dermatol. Apr 1999;135(4):464-5, 467-8. [Medline].

  2. 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. Oct 1999;26(9):436-40. [Medline].

  3. 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. Dec 1989;15(6):557-74. [Medline].

  4. Googe PB, Harris NL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. J Cutan Pathol. Oct 1987;14(5):263-71. [Medline].

  5. Tsuboi H, Masuzawa M, Katsuoka K. Angiolymphoid hyperplasia with eosinophilia affecting the nail bed and underlying bone. J Dermatol. Jun 2006;33(6):399-402. [Medline].

  6. 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. Jul-Aug 2008;24(4):310-1. [Medline].

  7. 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. Sep 2005;129(9):1168-71. [Medline].

  8. Angiolymphoid Hyperplasia with Eosinophilia and Kimura Disease. In: Wolff, Goldsmith, Katz, Gilchrest, Paller, Leffell, eds. Fitzpatrick's Dermatology in General Medicine. Vol 1. 7th ed. New York, NY: McGraw-Hill Medical; 2008:313-14.

  9. Joshi R. Angiolymphoid hyperplasia with follicular mucinosis. Indian J Dermatol Venereol Leprol. Sep-Oct 2007;73(5):346-7. [Medline].

  10. 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. May 29 2008;3:22. [Medline].

  11. 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. Aug 18 2008;40(4):405-7. [Medline].

  12. Carlesimo M, Mari E, Tammaro A, Persechino S, Camplone G. Angiolymphoid hyperplasia with eosinophilia treated with isotretinoin. Eur J Dermatol. Nov-Dec 2007;17(6):554-5. [Medline].

  13. Abrahamson TG, Davis DA. Angiolymphoid hyperplasia with eosinophilia responsive to pulsed dye laser. J Am Acad Dermatol. Aug 2003;49(2 Suppl Case Reports):S195-6. [Medline].

  14. Fetsch JF, Weiss SW. Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia). Mod Pathol. Jul 1991;4(4):449-55. [Medline].

  15. Mehregan AH, Shapiro L. Angiolymphoid hyperplasia with eosinophilia. Arch Dermatol. Jan 1971;103(1):50-7. [Medline].

  16. Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients. J Am Acad Dermatol. May 1985;12(5 Pt 1):781-96. [Medline].

  17. Tsang WY, Chan JK. The family of epithelioid vascular tumors. Histol Histopathol. Jan 1993;8(1):187-212. [Medline].

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Further Reading

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Keywords

angiolymphoid hyperplasia with eosinophilia, ALHE, epithelioid hemangioma, histiocytoid hemangioma, pseudopyogenic granuloma, papular angioplasia, inflammatory angiomatous nodule, inflammatory arteriovenous hemangioma, intravenous atypical proliferation.

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Contributor Information and Disclosures

Author

Sarah K Taylor, MD, Staff Physician, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Jon H Meyerle, MD, Assistant Professor, Department of Dermatology, Johns Hopkins University School of Medicine; Consulting Staff, Laboratory Director, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center
Jon H Meyerle, MD is a member of the following medical societies: American Academy of Dermatology and Sigma Xi
Disclosure: Nothing to disclose.

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.

Medical Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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