Skin nonmelanocytic tumor
Vascular tumors
Angiokeratoma
Author: Joel Tjarks, M.D.
Last author update: 1 May 2016
Last staff update: 2 February 2023 (update in progress)
Copyright: 2001-2023, PathologyOutlines.com, Inc.
PubMed Search: Angiokeratoma skin
Table of Contents
Definition / general | Essential features | Case reports | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Electron microscopy description | Differential diagnosisCite this page: Tjarks J. Angiokeratoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticangiokeratoma.html. Accessed March 26th, 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
- 17 year old boy and 21 year old woman with solitary tumors (J Clin Diagn Res 2015;9:WD01)
- 39 year old man with a family history of Fabry disease (Dermatol Online J 2011;17:5)
- 72 year old man with multiple penile shaft eruptions (An Bras Dermatol 2015;90:150)
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. and Joel Tjarks, M.D.
43 year old man, scrotal lesion
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
