You are in: eMedicine Specialties > Dermatology > DISEASES OF THE VESSELS Angiolymphoid Hyperplasia With EosinophiliaArticle Last Updated: Sep 15, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: 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 is a member of the following medical societies: American Academy of Dermatology and Sigma Xi Coauthor(s): Earl Glusac, MD, Professor, Departments of Pathology and Dermatology, Yale University School of Medicine Editors: 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; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey; 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; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System Author and Editor Disclosure Synonyms and related keywords: epithelioid hemangioma, histiocytoid hemangioma, pseudopyogenic granuloma, papular angioplasia, inflammatory angiomatous nodule, inflammatory arteriovenous hemangioma, intravenous atypical proliferation. INTRODUCTIONSection 2 of 9
  BackgroundAngiolymphoid 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, forehead, or scalp. Rare sites of involvement include the hands, 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 a separate entity. While ALHE lesions are superficial, Kimura disease involves deeper tissues such as lymph nodes, salivary glands, and the subcutis. However, a recent report describes ALHE involving the nail bed and underlying bone. PathophysiologyAlthough 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, 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. FrequencyUnited StatesFrequency in the United States is unknown. ALHE is uncommon but not rare. InternationalAlthough 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/MorbidityALHE 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. RaceALHE is seen most commonly in Asians, followed by whites. Although less common, blacks can develop ALHE. SexALHE is slightly more common in females; however, a male predominance has been noted in selected Asian studies. AgeALHE 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. CLINICALSection 3 of 9
HistoryPatients with 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. PhysicalALHE 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. CausesALHE is idiopathic. Whether this condition is a neoplastic or reactive state is uncertain; a reactive cause is favored. DIFFERENTIALSSection 4 of 9
Granuloma Faciale Insect Bites Kimura Disease Lymphocytoma Cutis Pyogenic Granuloma (Lobular Capillary Hemangioma) Sarcoidosis
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| Media file 1: Angiolymphoid hyperplasia with eosinophilia typically exhibits flesh-color to erythematous nodules in the vicinity of the ear. | |
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| Media file 2: Pronounced erythema and nodularity due to angiolymphoid hyperplasia with eosinophilia. | |
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| 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. | |
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Angiolymphoid Hyperplasia With Eosinophilia excerpt
Article Last Updated: Sep 15, 2006
