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 Vascular Tumours

Pathology of Angiokeratoma

Dr Sampurna Roy MD              


Dermatopathology Quiz Case 87

Diagnosis: Angiokeratoma





Angiokeratoma is a vascular lesion characterized by dilatation of superficial dermal vessels together with epidermal changes.



Clinical variants:

1. Mibelli type:

The "Mibelli-type" occurs on the acral sites, mainly digits, of young people affected by repeated attacks of chilblain, which result in a deleterious effects on vessel walls. 

2. Fordyce type:

The "Fordyce-type" occurs on the scrotal skin of young and adults as a secondary effect to an increased blood pressure in scrotal veins.

An equivalent form affecting adult females and occurring analogously on the skin of the vulva has been recorded.

3. Solitary and multiple types: 

The "solitary and multiple papular types" of young individuals affect the lower extremities and is considered a consequence of a congenital deficiency of elastic tissue in regional veins.

4. Angiokeratoma circumscriptum: 

This is a nevoid hamartomatous lesion arising early in life during infancy or childhood, sometimes in association with other congenital malformation of other sites.

5. Angiokeratoma corporis diffusum:

It is a clinical variant of angiokeratoma that is typically associated with an enzyme deficiency in the metabolism of glycoprotein, most notably Fabry disease, resulting in many other systemic manifestations.


Microscopic features:

- Dilatation of papillary dermal vessels forming cavernous spaces.

- Irregular acanthosis and elongation of rete ridges.

- Elongated rete ridges may surround the vessels.

- Thrombosis of some of the vessels

- Collarette at the margin may be  present.

- In Anderson Fabry disease, frozen section of the lesion reveals PAS positive and Sudan black positive granules in the endothelial cells, pericytes, arrectores pilorum and sweat glands.


Differential diagnosis:

Verrucous hemangioma- Dilated vessels are present in the deeper levels 


Further reading:

Solitary angiokeratoma of the tongue.

Angiokeratoma corporis diffusum (Fabry disease).

Fabry disease: angiokeratoma, biomarker, and the effect of enzyme replacement therapy on kidney function.

Fucosidosis with angiokeratoma. Immunohistochemical & electron microscopic study of a new case and literature review.

Vascular tumors of the skin: a selective review.

Angiokeratoma of vulva with coexisting human papilloma virus infection: a case report 

Angiokeratoma of the scrotum: a case of scrotal bleeding

Angiokeratoma circumscriptum arranged in a systematized band-like pattern suggesting mosaicism

Angiokeratoma of tongue: a series of 14 cases.




Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

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