Pseudo-Kaposi Sarcoma (Acroangiodermatitis) 

Updated: Jan 08, 2018
Author: Zoltan Trizna, MD, PhD; Chief Editor: Dirk M Elston, MD 

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]

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.

Etiology

Severe, chronic venous stasis and the insufficiency of the muscle pump most commonly result in an elevated capillary pressure. Other factors (eg, an arteriovenous shunt for hemodialysis) may increase venous stasis and lead to the formation of arteriovenous channels. A rare case of acroangiodermatitis associated with a congenital arteriovenous malformation of the leg was also described (Stewart-Bluefarb syndrome).

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.

Prognosis

Individual lesions may persist unchanged for several years. The lesions of acroangiodermatitis can ulcerate and bleed and are at risk of infection. Differentiate developmental arteriovenous malformations of the feet from acroangiodermatitis because the prognosis for the former is more severe. Mortality and morbidity depend on the underlying condition.

Patient Education

Educate patients about skin care and avoiding mechanical trauma that could induce ulceration or bleeding. Recommend that patients reduce their body weight in appropriate cases.

 

Presentation

History

A history of venous stasis, arteriovenous shunt for hemodialysis, or a long-standing arteriovenous malformation is usually present. A limb prosthesis may be present. Patients occasionally experience pruritus and pain.

Physical Examination

Confluent, violaceous or brown-black papules cover large areas of the distal parts of the legs. Ulceration and bleeding are sometimes noted. Bilateral lesions are usually associated with chronic venous insufficiency, whereas unilateral lesions suggest an underlying vascular malformation.[10]  Note the images below.

The physical findings in this patient who is HIV n The physical findings in this patient who is HIV negative remained the same over a 3-year period.
Lesions on the shin of a patient who is HIV negati Lesions on the shin of a patient who is HIV negative.
Classic Kaposi sarcoma on the foot of an elderly p Classic Kaposi sarcoma on the foot of an elderly patient who is HIV negative. Compare this photo to the clinical photos of acroangiodermatitis.

Complications

Complications include ulceration, bleeding, and infection; these conditions may also be related to the underlying problems.

 

DDx

Diagnostic Considerations

Also consider the following:

  • Hemangioproliferative diseases (eg, capillary and cavernous hemangiomas)
  • Hemosiderotic fibrohistiocytic lipomatous lesion [11]
  • Pigmented purpuric lichenoid dermatitis of Blum
  • Purpura annularis telangiectodes (Majocchi purpura)
  • Stewart-Blufarb syndrome [12]
  • Vasculitis

Pseudo-Kaposi sarcoma can be misdiagnosed as AIDS-related Kaposi sarcoma (as a relatively greater prevalence of AIDS-related Kaposi sarcoma exists). Treating acroangiodermatitis as Kaposi sarcoma (eg, with grafting and irradiation) is another diagnostic pitfall. A case report described acroangiodermatitis of the plantar aspect of the foot related to chronic venous insufficiency, misdiagnosed for 2 years.[13]

Differential Diagnoses

 

Workup

Laboratory Studies

Rule out HIV-positive status, especially if Kaposi sarcoma is suspected.

Imaging Studies

Use plethysmography, Doppler ultrasonography, and oscillography to assess the venous flow and to detect underlying vascular malformations. Use arteriography to demonstrate arteriovenous fistulae.

Other Tests

Transcutaneous oxygen pressure can detect hypoxia at the edge of the lesions. Polymerase chain reaction for human herpesvirus 8 can help in differentiating acroangiodermatitis from Kaposi sarcoma; however, this investigational method is not routinely available.

Procedures

Obtain a biopsy sample for dermatopathologic evaluation.

Histologic Findings

In early lesions, a proliferation of capillaries exists deeper in the dermis; the papillary dermis is also affected later on. The neovascularization is accompanied by fibrosis with spindle cells, extravasation of red blood cells, and deposition of hemosiderin. Venules and deeper vertical small veins can also become tortuous and hypertrophic. A few interspersed inflammatory cells and eosinophils may be noted. A mixed perivascular infiltrate is sometimes present. An edematous matrix typically separates the capillary proliferations. Acroangiodermatitis is usually associated with minimal epidermal changes.

Immunohistochemically, the proliferative fibroblastlike spindle cells around the vessels are positive for antifactor XIIIa antibody. Immunostaining for the CD34 antigen demonstrated a strong labeling of endothelial cells of hyperplastic vessels in acroangiodermatitis.[14]

Note the images below.

Acroangiodermatitis on histopathologic examination Acroangiodermatitis on histopathologic examination.
Example of acroangiodermatitis on histopathologic Example of acroangiodermatitis on histopathologic examination.
Higher-power view of acroangiodermatitis on histop Higher-power view of acroangiodermatitis on histopathologic examination.
Classic Kaposi sarcoma on histopathologic examinat Classic Kaposi sarcoma on histopathologic examination.
 

Treatment

Medical Care

Because acroangiodermatitis is rarely reported, most of the treatment reports are of anecdotal nature. Oral erythromycin treatment led to improvement in 2 cases of pseudo-Kaposi sarcoma in patients who had acquired arteriovenous fistula from hemodialysis. Compression therapy led to nearly complete resolution of the lesions in 5 months.[15] Intermittent pneumatic compression therapy was also described.[16] A 3-month course of dapsone (50 mg PO bid) combined with leg elevation and elastic support stockings led to complete regression of the lesions in one patient with acroangiodermatitis.[17]

Surgical Care

Surgical elimination of the shunts is curative in acroangiodermatitis accompanying arteriovenous malformations. Multiple, small fistulae can be destroyed individually or by embolization; however, the latter method can lead to ischemia and necrosis.

Consultations

Consult a phlebologist for the management of the underlying vascular problems. Consult a physiotherapist for management of the underlying circulatory problem. If a limb prosthesis is present, evaluate its mechanical effect on the stump.

Diet

If the patient is obese, losing body weight may improve the circulation of the extremities.

Activity

Activity can be continued as allowed by the condition of the extremities and the underlying condition.

Long-Term Monitoring

Further outpatient care depends on the underlying disease.

 

Medication

Medication Summary

No standard medical therapy exists.

 

Questions & Answers

Overview

What is pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the pathophysiology of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What causes pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the prevalence of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What are the racial predilections of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What are the sexual predilections of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the prevalence of pseudo-Kaposi sarcoma (acroangiodermatitis) by age?

What is the prognosis of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is included in patient education about pseudo-Kaposi sarcoma (acroangiodermatitis)?

Presentation

Which clinical history findings are characteristic of pseudo-Kaposi sarcoma (acroangiodermatitis)?

Which physical findings are characteristic of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What are the possible complications of pseudo-Kaposi sarcoma (acroangiodermatitis)?

DDX

Which conditions are included in the differential diagnoses of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What are the differential diagnoses for Pseudo-Kaposi Sarcoma (Acroangiodermatitis)?

Workup

What is the role of lab studies in the workup of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the role of imaging studies in the workup of pseudo-Kaposi sarcoma (acroangiodermatitis)?

How is hypoxia assessed in the workup of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the role of biopsy in the workup of pseudo-Kaposi sarcoma (acroangiodermatitis)?

Which histologic findings are characteristic of pseudo-Kaposi sarcoma (acroangiodermatitis)?

Treatment

How is pseudo-Kaposi sarcoma (acroangiodermatitis) treated?

What is the role of surgery in the treatment of pseudo-Kaposi sarcoma (acroangiodermatitis)?

Which specialist consultations are beneficial to patients with pseudo-Kaposi sarcoma (acroangiodermatitis)?

Which dietary modifications are used in the treatment of pseudo-Kaposi sarcoma (acroangiodermatitis)?

Which activity modifications are used in the treatment of pseudo-Kaposi sarcoma (acroangiodermatitis)?

What is the long-term monitoring for pseudo-Kaposi sarcoma (acroangiodermatitis)?

Medications

What is the role of medications in the treatment of pseudo-Kaposi sarcoma (acroangiodermatitis)?