Skin nonmelanocytic tumor
Vascular tumors

Topic Completed: 1 May 2016

Minor changes: 14 October 2020

Copyright: 2001-2020 ,, Inc.

PubMed Search: Angiokeratoma skin

Joel Tjarks, M.D.
Page views in 2019: 11,085
Page views in 2020 to date: 7,593
Cite this page: Tjarks J. Angiokeratoma. website. Accessed October 21st, 2020.
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

Images hosted on PathOut server:

Images contributed by Dr. Sabrina C. Sopha:

43 year old man, scrotal lesion

Images contributed by Dr. Joel Tjarks, M.D., University of South Dakota (USA):

Various images

Images contributed by Angel Fernandez-Flores, M.D., Ph.D., Hospital El Bierzo and Clinica Ponferrada (Spain):

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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