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Dermatology > DISEASES OF THE VESSELS
Pseudo-Kaposi Sarcoma (Acroangiodermatitis)
Article Last Updated: Feb 1, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Zoltan Trizna, MD, PhD, Private Practice
Zoltan Trizna is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Texas Medical Association
Coauthor(s):
Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School, MD Anderson Cancer Center
Editors: Shyam Verma, MBBS, DVD, FAAD, Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
pseudo-Kaposi's sarcoma, Mali disease, Mail's disease, acroangiodermatitis of Favre-Chaix, acro-angiodermatitis
Background
Since its original description, acroangiodermatitis has been described in amputees (especially in those with poorly fitting suction-type devices), in patients with paralyzed legs, in patients undergoing hemodialysis (from arteriovenous shunts distally), and in association with hepatitis C. It has been documented in chronic venous insufficiency and in vascular malformations (eg, Klippel-Trenaunay syndrome, Stewart-Bluefarb syndrome, Prader-Labhart-Willi syndrome).
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.
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.
Frequency
United States
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.
Mortality/Morbidity
Mortality and morbidity depend on the underlying condition. The lesions of acroangiodermatitis can ulcerate and bleed and are at risk of infection.
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.
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
- 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.
Causes
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.
Sebaceous Adenoma
Other Problems to be Considered
Hemangioproliferative diseases (eg, capillary and cavernous hemangiomas)
Hemosiderotic fibrohistiocytic lipomatous lesion
Pigmented purpuric lichenoid dermatitis of Blum
Purpura annularis telangiectodes (Majocchi purpura)
Vasculitis
Lab 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.
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.
- 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 1 patient with acroangiodermatitis.
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.
No standard medical therapy exists.
Further Inpatient Care
- Further inpatient care depends on the underlying disease.
Further Outpatient Care
- Further outpatient care depends on the underlying disease.
In/Out Patient Meds
- Inpatient and outpatient medications depend on the underlying disease.
Complications
- Complications include ulceration, bleeding, and infection; these conditions may also be related to the underlying problems.
Prognosis
- Individual lesions may persist unchanged for several years.
- Differentiate developmental arteriovenous malformations of the feet from acroangiodermatitis because the prognosis of the former is more severe.
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.
Medical/Legal Pitfalls
- Misdiagnosis of pseudo-Kaposi sarcoma as AIDS-related Kaposi sarcoma (as a relatively greater prevalence of AIDS-related Kaposi sarcoma exists) is a pitfall.
- Treating acroangiodermatitis as Kaposi sarcoma (eg, with grafting and irradiation) is another pitfall.
| Media file 1:
The physical findings in this patient who is HIV negative remained the same over a 3-year period. |
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| Media file 2:
Lesions on the shin of the same patient as in Image 1. |
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| Media file 3:
Acroangiodermatitis on histopathologic examination. |
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| Media file 4:
Example of acroangiodermatitis on histopathologic examination. |
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| Media file 5:
Higher-power view of acroangiodermatitis on histopathologic examination. |
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| Media file 6:
Classic Kaposi sarcoma on the foot of an elderly patient who is HIV negative. Compare this photo to the clinical photos of acroangiodermatitis. |
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| Media file 7:
Classic Kaposi sarcoma on histopathologic examination. |
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Pseudo-Kaposi Sarcoma (Acroangiodermatitis) excerpt Article Last Updated: Feb 1, 2007
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