Skin nonmelanocytic tumor

Vascular tumors


Last author update: 1 May 2016
Last staff update: 17 July 2023 (update in progress)

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PubMed Search: Angiokeratoma skin

Joel Tjarks, M.D.
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Cite this page: Tjarks J. Angiokeratoma. website. Accessed November 29th, 2023.
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, Joel Tjarks, M.D., Angel Fernandez-Flores, M.D., Ph.D. and @RaulSGonzalezMD on Twitter

43 year old man, scrotal lesion


Angiokeratoma, vulva

Various images

Angiokeratoma Angiokeratoma


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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