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Angiokeratoma circumscriptum in Adult
Other Resources UpToDate PubMed

Angiokeratoma circumscriptum in Adult

Contributors: Rajini Murthy MD, Susan Burgin MD
Other Resources UpToDate PubMed


Angiokeratoma circumscriptum is a benign, relatively uncommon form of cutaneous vascular ectasia. It presents as one or more dark red, maroon, or blue-black papule, plaque, or nodule that may have a keratotic or verrucous surface. It is most often found unilaterally on a lower limb.

Angiokeratoma circumscriptum has been associated with Klippel-Trenaunay-Weber syndrome (osteohypertrophy of a limb), Cobb syndrome, port-wine stain, cavernous hemangiomas, and arteriovenous fistulas. The condition is present at birth or develops by early childhood, with lesions often increasing in size during adolescence. Female patients are affected more commonly than male patients.


D23.9 – Other benign neoplasm of skin, unspecified

21848000 – Angiokeratoma circumscriptum

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Differential Diagnosis & Pitfalls

  • Hemangioma
  • Lobular capillary hemangioma (pyogenic granuloma)
  • Cherry hemangioma
  • Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
  • Blue rubber bleb nevus syndrome
  • Lymphangioma circumscriptum
  • Common acquired nevus
  • Spitz nevus
  • Kaposi sarcoma (Non-AIDS Kaposi sarcoma, AIDS-associated Kaposi sarcoma)
  • Melanoma

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Last Reviewed:10/04/2021
Last Updated:10/04/2021
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Angiokeratoma circumscriptum in Adult
A medical illustration showing key findings of Angiokeratoma circumscriptum : Ankle, Arm, Lower leg, Present at birth, Scaly plaque, Thigh, Unilateral distribution
Copyright © 2024 VisualDx®. All rights reserved.