Pseudo-Kaposi Sarcoma (Acroangiodermatitis)

Updated: Jan 08, 2016
  • Author: Zoltan Trizna, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Since its original description, acroangiodermatitis has been described in amputees (especially in those with poorly fitting suction-type devices), [1, 2] in patients with paralyzed legs, [3] in patients undergoing hemodialysis (from arteriovenous shunts distally), [4] and in association with hepatitis C. It has been documented in chronic venous insufficiency, in vascular malformations [5] (eg, Klippel-Trenaunay syndrome, [6] Stewart-Bluefarb syndrome, [7] Prader-Labhart-Willi syndrome), protein-C deficiency, [8] and due to symmetrical arteriovenous fistulae. [9]

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Pathophysiology

Acroangiodermatitis is a hyperplasia of preexisting vasculature, as opposed to Kaposi sarcoma, in which the vascular proliferation is independent of the existing vessels. It is usually seen as a complication of severe chronic venous stasis (hypostasis and elevated venous pressure) of the lower legs and the feet. Conversely, though less common, congenital or acquired arteriovenous anomalies can result in high venous pressure. Acroangiodermatitis can occur in amputees of the lower extremity.

Severe chronic venous stasis and the insufficiency of the calf muscle pump result in an elevated capillary pressure. Plethysmographic studies demonstrate the insufficiency of both the muscular pump of the calf and the venous pump of the foot in acroangiodermatitis.

The lack of the muscle pump and the disturbed innervation of vessels both may be of pathogenetic importance in paralyzed extremities. Others suggest that paralysis could generate the cutaneous lesions by increasing venous stasis and enhancing arteriovenous channels. In Klippel-Trenaunay syndrome, a high perfusion rate and a high oxygen saturation may be involved in the development of the lesions.

Acroangiodermatitis can occur in cases of acquired iatrogenic arteriovenous fistula from hemodialysis. Some cases have been reported that may resolve after thrombosis or surgical elimination of the shunt.

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Epidemiology

Frequency

Fewer than 100 cases have been reported. It is probably uncommon but not rare. A tendency to not report additional cases that do not provide any new information to the literature is likely.

Race

No exact data are available.

Sex

The condition is more frequent in males than in females.

Age

Most cases have been described in adults.

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