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angiolymphoid hyperplasia with eosinophilia

Monday 14 June 2004

synonymes: epithelioid hemangioma, pseudopyogenic granuloma; cutaenous HALE; "Epithelioid hemangioma," "Histiocytoid hemangioma," "Inflammatory angiomatous nodule," "Intravenous atypical vascular proliferation," "Papular angioplasia," "Inflammatory arteriovenous hemangioma," "Pseudopyogenic granuloma"

eM WP

Definition: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a tumor of skin and subcutaneous tissues composed of vessels, a proliferation of a distinctive type of endothelial cell, and a variable component of inflammatory cells.

Images

- Angiolymphoid hyperplasia with eosinophilia (ALHE)

ALHE is a distinct pathologic entity and 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 it is a true neoplasm or a reactive process is at present undecided. It appears to be identical to the lesions known as pseudopyogenic granuloma, atypical pyogenic granuloma, epithelioid hemangioma, histiocytoid hemangioma, intravenous atypical vascular proliferation, and nodular angioblastic hyperplasia with eosinophilia and lymphofolliculosis.

Clinical synopsis

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.

Angiolymphoid hyperplasia with eosinophilia usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck, especially about the ears and on the scalp

Most patients present with lesions in the periauricular region, forehead, or scalp. Rare sites of involvement include the hands, shoulders, breasts, penis, oral mucosa, orbit, and, recently, a report involving the scrotum.

Microscopy

The lesions consist of circumscribed collections of vessels and inflammatory cells.

The vascular component comprises thick- and thin-walled vessels lined by plump endothelial cells. These cells also occur in clumps that appear solid or sometimes contain small lumina. They are ‘epithelioid’ in appearance, with a large nucleus and abundant eosinophilic cytoplasm, and are characteristic of this condition. Prominent cytoplasmic vacuoles are seen in some cells. Normal mitotic figures are sometimes present. Intravascular proliferations of these cells may be seen in the lumina of larger vessels.

There is one report of multinucleated cells, some of which were endothelial sprouts and others fibrohistiocytic cells.

Associated with the vascular and endothelial proliferations is a stromal cellular infiltrate which varies in intensity and consists of lymphocytes (sometimes with lymphoid follicle formation), eosinophils, and mast cells. The stroma may be fibrous or myxoid in character. Sometimes the fibrotic reaction is excessive. In one case it was associated with a florid granulomatous reaction with many multinucleated giant cells, often of Touton type.

Although these epithelioid cells share some enzymes with histiocytes they do not contain lysozyme; they have ultrastructural features of endothelial cells, including Weibel–Palade bodies.

The epithelioid cells stain for CD31, CD34, factor VIII-related antigen, and Ulex europaeus-1 lectin, but not for cytokeratins or epithelial membrane antigen.The vacuoles in their cytoplasm possibly represent primitive vascular lumina.

Angiolymphoid hyperplasia with high endothelial venules

"Angiolymphoid hyperplasia with high endothelial venules" is the term suggested for a cutaneous lesion with a characteristic admixture of lymphoid hyperplasia (without eosinophils) and a vascular proliferation previously called APACHE for "acral pseudolymphomatous angiokeratoma of children". APACHE continues to be the designation attached to this entity. It appears to be a ‘pseudolymphoma’.

Differential diagnosis

- Kimura disease

  • While ALHE shows some similarity to Kimura disease, it is generally regarded as a separate entity.
  • 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.
  • Only one report describes a patient with simultaneous tumors, one consistent with Kimura disease and one consistent with ALHE.
  • Such findings challenge whether or not Kimura disease and ALHE represent a spectrum of the same disease.

- epithelioid hemangioendothelioma
- epithelioid angiosarcoma

  • Exuberant examples may be confused with epithelioid hemangioendothelioma and epithelioid angiosarcoma.

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