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:
Image1
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Image2
- 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
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