eMedicine Specialties > Dermatology > Diseases of the Vessels

Angiolymphoid Hyperplasia With Eosinophilia

Sarah K Taylor, MD, Staff Physician, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center
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

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.

Differential Diagnoses

Granuloma Faciale
Insect Bites
Kimura Disease
Lymphocytoma Cutis
Pyogenic Granuloma (Lobular Capillary Hemangioma)
Sarcoidosis

Workup

Laboratory Studies

A CBC count reveals eosinophilia in approximately 20% of patients with angiolymphoid hyperplasia with eosinophilia (ALHE). Given that some patients with ALHE have also been found to have renal disease, urinalysis could be considered.

Procedures

The clinical presentation of papules around the ears may suggest ALHE, but a biopsy is required to establish the diagnosis.

Histologic Findings

Angiolymphoid hyperplasia with eosinophilia (ALHE) shows characteristic histologic features, including a proliferation of small blood vessels, many of which are lined by enlarged endothelial cells with uniform ovoid nuclei and intracytoplasmic vacuoles. These distinctive endothelial cells have been described as having a cobblestone appearance. In addition, a perivascular and interstitial infiltrate composed primarily of lymphocytes and eosinophils (see Media File 3) is present. Eosinophils typically comprise 5-15% of the infiltrate. Rarely, they can account for as much as 50% of the infiltrate. Occasionally, the infiltrate is devoid of eosinophils. Lymphoid aggregates with or without follicle formation are typical.

Treatment

Medical Care

Angiolymphoid hyperplasia with eosinophilia (ALHE) treatment is often challenging. Intralesional corticosteroids and irradiation have been used but are not very effective. Other treatments that have been reported include topical imiquimod, topical tacrolimus, isotretinoin,¹² and interferon alfa-2b.

Surgical Care

Simple surgical excision is sometimes used, but the lesions tend to recur. Mohs micrographic surgery has been attempted in order to address ALHE through better margin control. Excisions that include the arterial and venous segments at the base of the lesion prove most efficacious. The pulsed-dye laser¹³ and carbon dioxide laser have been used with some success. Cryosurgery has also been reported.

Follow-up

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.

Prognosis

Lesions can remit over the course of months or years, but they tend to persist or recur.

Multimedia

Media file 1: Angiolymphoid hyperplasia with eosinophilia typically exhibits flesh-color to erythematous nodules in the vicinity of the ear.

Media file 2: Pronounced erythema and nodularity due to angiolymphoid hyperplasia with eosinophilia.

Media file 3: Histologically, angiolymphoid hyperplasia with eosinophilia is marked by thick-walled blood vessels with protuberant endothelium and a prominent inflammatory infiltrate, which typically includes eosinophils.

References

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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].
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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.

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.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

Further Reading