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.
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.
Complications
Complications include ulceration, bleeding, and infection; these conditions may also be related to the underlying problems.
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.
View Image | Acroangiodermatitis on histopathologic examination. |
View Image | Example of acroangiodermatitis on histopathologic examination. |
View Image | Higher-power view of acroangiodermatitis on histopathologic examination. |
View Image | Classic Kaposi sarcoma on histopathologic examination. |
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 Summary
No standard medical therapy exists.
Author
Zoltan Trizna, MD, PhD, Private Practice
Disclosure: Nothing to disclose.
Coauthor(s)
Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, The University of Texas MD Anderson Cancer Center; Distinguished Chernosky Professor and Chair of Dermatology, Professor of Pathology, University of Texas McGovern Medical School at Houston
Disclosure: Book royalties from Elsevier publishers.
Specialty Editors
Michael J Wells, MD, FAAD, Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology
Disclosure: Nothing to disclose.
Jeffrey J Miller, MD, Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center
Disclosure: Nothing to disclose.
Chief Editor
Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Disclosure: Nothing to disclose.
Additional Contributors
Shyam Verma, MBBS, DVD, FAAD, Clinical Associate Professor, Department of Dermatology, University of Virginia School of Medicine; Adjunct Associate Professor, Department of Dermatology, State University of New York at Stonybrook School of Medicine; Adjunct Associate Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Disclosure: Nothing to disclose.
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