Pathology of Angiolymphoid Hyperplasia with Eosinophilia (Epithelioid Hemangioma)

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Pathology of Angiolymphoid Hyperplasia with Eosinophilia [Epithelioid Hemangioma]

Dr Sampurna Roy MD 2023

Answer - 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, 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.

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.