Skin - Nonmelanocytic tumors
Vascular tumors
Angiokeratoma

Authors: Joel Tjarks, M.D. (see Authors page)

Revised: 18 May 2016, last major update May 2016

Copyright: (c) 2001-2016, PathologyOutlines.com, Inc.

PubMed Search: Angiokeratoma skin
Cite this page: Angiokeratoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiokeratoma.html. Accessed December 8th, 2016.
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

Micro 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

Micro Images

Images hosted on PathOut server:

Contributed by Dr. Joel Tjarks, M.D., University of South Dakota (USA)


Contributed by Angel Fernandez-Flores, M.D., PhD, Hospital El Bierzo and Clinica Ponferrada (Spain)



Images hosted on other servers:

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