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Acroangiodermatitis
Other Resources UpToDate PubMed

Acroangiodermatitis

Contributors: Fandi Xia MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Acroangiodermatitis, or pseudo-Kaposi sarcoma, is a benign angioproliferative disorder most frequently arising in the setting of chronic venous insufficiency. Some consider acroangiodermatitis a clinical variant of stasis dermatitis. Other less commonly reported associations include paralysis, amputation, congenital or acquired arteriovenous malformations, and thrombotic syndromes.

Stewart-Bluefarb syndrome refers to acroangiodermatitis arising in the setting of congenital arteriovenous malformations, typically on the lower extremities. The condition is often diagnosed in teenagers or young adults.

The pathophysiology of acroangiodermatitis remains unclear. Some studies suggest that increased production of vasogenic growth factors, following disruption of normal cutaneous vascular perfusion, may play a role.

Codes

ICD10CM:
I78.9 – Disease of capillaries, unspecified

SNOMEDCT:
769036006 – Acroangiodermatitis of skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • AIDS-associated Kaposi sarcoma
  • Non-AIDS Kaposi sarcoma 
  • Bacillary angiomatosis 
  • Reactive Angioendotheliomatosis
  • Stasis purpura – Pathology shows erythrocyte extravasation without prominent vascular proliferation.
  • Capillaritis / pigmented purpuric dermatoses
  • Telangiectasia – Pathology shows dilated vessels rather than vascular proliferation.
  • Petechiae – Pathology shows extravasated erythrocytes without vascular proliferation; self-limited process.
  • Vasculitis – More acute onset and can resolve without treatment.
  • Ecchymosis (see Traumatic purpura) – Appearance is linked to a history of trauma. 
  • Lichen planus – Pruritic.
  • Livedoid vasculopathy – Painful ulcers healing with stellate white scars. 
  • Lipodermatosclerosis  Indurated, tender, firm plaques.
  • Ischemic ulcer or Stasis ulcer
  • Angiosarcoma of skin
  • Hemangioendothelioma (various types)
  • Hemangioma

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:11/17/2020
Last Updated:11/22/2020
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Acroangiodermatitis
A medical illustration showing key findings of Acroangiodermatitis : Foot or toes, Lower legs
Copyright © 2024 VisualDx®. All rights reserved.