Angiolymphoid Hyperplasia with Eosinophilia (Epithelioid Hemangioma)

Angiolymphoid hyperplasia with eosinophilia also known as epithelioid hemangioma is a distinctive vascular lesion which usually occurs during early and mid adult life (20- 40 years).

Clinically, this lesion presents as single or multiple pink to red-brown papules and nodules on the face, scalp and ear.

It is still uncertain whether this lesion is a true neoplasm or a reactive process.
Some cases appear to be associated with trauma, pregnancy, HIV infection, pregnancy and oral contraceptives.

Microscopically, these are well circumscribed lesions usually located in the dermis and subcutis. Angiolymphoid hyperplasia with eosinophilia consists of vascular and inflammatory cellular components. Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 .

The vascular component displays thick and thin walled vessels lined by plump endothelial cells. The epithelioid endothelial cells have rounded nuclei abundant eosinophilic cytoplasm containing occasional vacuoles that represent primitive vascular lumen formation. These endothelial cell protrude into the vessels in a 'tombstone' pattern. Within some thicker vessels there is intravascular proliferations of the endothelial cells.

The inflammatory component consists of lymphocytes scattered eosinophils and mast cells within the stroma. Lymphoid follicle may be present.
In areas the stroma may be myxoid or fibrous in character.

Differential diagnosis: Kimura's disease ; epithelioid hemangioendothelioma; epithelioid angiosarcoma ; injection site granuloma (epithelioid cells are not present).

Kimura's disease is a separate entity which usually affects young Asian men and is commonly associated with lymphadenopathy.

Kimura's disease is a deeper lesion and reactive lymphoid follicles and are present together with a dense infiltration of eosinophils sometimes forming eosinophilic abscesses.
Blood vessels are also increased in number but these are lined by flat endothelial cells.

In Epithelioid hemangioendothelioma the tumour cells are arranged in nests and cords surrounded by hyaline and myxoid stroma. Well formed blood vessels are usualy not present.

IMAGE LINKS: Image1(Iowa) ; Image2 ; Image3

Image4 ; Image5 ; Image6 (ESCOP)

Abstracts:

Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature. Arch Pathol Lab Med. 2005;129(9):1168-71. (Full Text).

Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the lung: a clinicopathologic and immunohistochemical study of two cases. Am J Clin Pathol. 2005;123(5):762-5.

Cutaneous epithelioid angiomatous nodule: a distinct lesion in the morphologic spectrum of epithelioid vascular tumors. Am J Dermatopathol. 2004;26(1):14-21.

Human herpesvirus-8 is not associated with angiolymphoid hyperplasia with eosinophilia.J Cutan Pathol. 2004 Oct;31(9):612-5.

Epithelioid hemangioma of the penis: a clinicopathologic and immunohistochemical analysis of 19 cases, with special reference to exuberant examples often confused with epithelioid hemangioendothelioma and epithelioid angiosarcoma. Am J Surg Pathol. 2004;28(4):523-33.

Angiolymphoid hyperplasia with eosinophilia: evidence for a T-cell lympho-proliferative origin.Hum Pathol. 2002;33(10):1023-9.

Angiolymphoid hyperplasia with eosinophilia: a classic clinical presentation with histologic features of angiosarcoma.Dermatol Surg. 2002 ;28(8):772-5.

Angiolymphoid hyperplasia with eosinophilia with bilateral involvement of the lacrimal glands. Klin Monatsbl Augenheilkd. 2002;219(5):376-9.

Polymerase chain reaction (PCR) for human herpesvirus 8 and heteroduplex PCR for clonality assessment in angiolymphoid hyperplasia with eosinophilia and Kimura's disease. J Cutan Pathol. 2001;28(7):363-7.

Traumatic pseudoaneurysm: a possible early lesion in the spectrum of epithelioid hemangioma/angiolymphoid hyperplasia with eosinophilia. Am J Dermatopathol. 1998;20(2):113-7.

Differential diagnosis of tumorous space-occupying lesions of the parotid gland: angiolymphoid hyperplasia with eosinophilia and Kimura disease. Laryngorhinootologie. 1997;76(2):110-5.

Angiolymphoid hyperplasia with eosinophilia and vascular tumors of the head and neck. Am J Otolaryngol. 1996;17(4):240-5.

Multiple angiolymphoid hyperplasia with eosinophilia of the hand: report of a case and review of the literature.Cutis. 1996;58(5):345-8

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: new observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins. J Cutan Pathol. 1995;22(4):319-26.

Microcirculatory segments identified with monoclonal antibody against alpha-smooth muscle actin: comparison between Kimura's disease and angiolymphoid hyperplasia with eosinophilia. Acta Derm Venereol. 1995;75(1):15-8.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: clinical and histopathologic differences.J Am Acad Dermatol. 1992;27(6 Pt 1):954-8.

Estrogen receptors and the response to sex hormones in angiolymphoid hyperplasia with eosinophilia.Arch Dermatol. 1992;128(6):825-8.

The histiocytoid hemangiomas and other controversies. Pathol Annu.1992;27 Pt 2:51-87.