Skin nonmelanocytic tumor
Vascular tumors
Angiokeratoma


Topic Completed: 1 May 2016

Minor changes: 15 January 2021

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PubMed Search: Angiokeratoma skin

Joel Tjarks, M.D.
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Cite this page: Tjarks J. Angiokeratoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiokeratoma.html. Accessed May 8th, 2021.
Definition / general
  • Benign vascular lesion characterized by superficial vascular ectasia and overlying epidermal hyperplasia (acanthosis or hyperkeratosis)
  • Lesions may be solitary or multiple / diffuse

    Five types with similar histology:
    • Angiokeratoma of Mibelli: seen in children and adolescents on dorsum of toes and fingers
    • Angiokeratoma of Fordyce: scrotal skin of elderly
    • Angiokeratoma corporis diffusum: clustered papules in a bathing suit distribution; associated with Anderson-Fabry disease (X-linked recessive lysosomal storage disease)
    • Angiokeratoma circumscriptum: least common type, usually congenital, associated with nevus flammeus, cavernous hemangioma
    • Idiopathic solitary or multiple angiokeratomas
Essential features
  • Benign vascular lesion
  • Characterized by superficial vascular ectasia and overlying epidermal hyperplasia
  • May occur in a variety of clinical settings
  • Associated with Anderson-Fabry disease (X-linked recessive lysosomal storage disease)
Case reports
Gross description
  • Small red to brown / black papule or nodule with verrucous surface, can be clustered
Gross images

Images hosted on other servers:

Various images

Microscopic (histologic) description
  • Vascular ectasia of the papillary dermis which may appear to extend into the epidermis
  • Overlying epidermal hyperplasia characterized by acanthosis, elongation of the rete and hyperkeratosis, with the epidermis encircling the dilated vascular spaces
  • Often thrombosis within the vascular ectasia

Microscopic (histologic) images

Contributed by Sabrina C. Sopha, M.D.

43 year old man, scrotal lesion



Contributed by Joel Tjarks, M.D.

Various images



Contributed by Angel Fernandez-Flores, M.D., Ph.D.

Various images

Electron microscopy description
  • In patients with Anderson-Fabry disease, see lipid bodies and lamellar inclusions in endothelial cells, pericytes and smooth muscle cells in angiokeratomas
Differential diagnosis
  • Hemangioma (verrucous, lobular capillary, etc.)
  • Lymphangioma
  • Venous lake
  • Clinical differential diagnosis may include pigmented / melanocytic lesions due to thrombosis
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