Soft Tissue Pathology

Path Quiz Case-8   Diagnosis: 

Angiolymphoid Hyperplasiawith Eosinophilia [Epithelioid Hemangioma]

                                                      Dr Sampurna Roy MD

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January 2014 

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Path Quiz Image 1  ; Image2  ;  Image3  ;  Image4

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.


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.

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.



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

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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.

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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.

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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.

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Orbital angiolymphoid hyperplasia with eosinophilia. Presentation as chalazion. Ophthal Plast Reconstr Surg. 1991;7(3):208-21.

Angiolymphoid hyperplasia with eosinophilia of the hand. A case report.J Dermatol Surg Oncol. 1991;17(9):731-4

[Angiolymphoid hyperplasia with eosinophilia--report of a case with reference to the vascular changes and cell infiltrates in this disease].Nippon Hifuka Gakkai Zasshi. 1990;100(9):935-46.

A clinicopathological study of Kimura's disease and epithelioid hemangioma . Yonsei Med J. 1990;31(3):205-11.

Histiocytoid hemangioma: a disease spectrum. Report of a case with simultaneous cutaneous and bone involvement limited to one extremity. J Am Acad Dermatol. 1989;21(2 Pt 2):404-9.

Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: a case report with electron microscopy and immunohistochemistry.Diagn Cytopathol. 1989;5(1):88-94.

Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura's disease in Chinese.Histopathology. 1989;15(6):557-74.

Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia.Am J Surg Pathol. 1988;12(11):843-54.

Epithelioid hemangioma versus Kimura's disease. A comparative clinicopathologic study.Am J Surg Pathol. 1987;11(10):758-66.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. J Cutan Pathol. 1987;14(5):263-71.

Oral angiolymphoid hyperplasia with eosinophilia.Oral Surg Oral Med Oral Pathol. 1986;61(1):73-9.

Renin in angiolymphoid hyperplasia with eosinophilia. Its possible effect on vascular proliferation. Arch Pathol Lab Med. 1986;110(12):1131-5.

Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients.J Am Acad Dermatol. 1985 ;12(5 Pt 1):781-96

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