Practice Essentials
Acroangiodermatitis is a rare disorder that, as the name indicates, involves the blood vessels and skin of the extremities. Two variants exist: The more common variant was described by Mali et al as a consequence of chronic insufficiency of the cutaneous venous network of the foot that presents as violaceous macules and indurated plaques or nodules; it is sometimes termed acroangiodermatitis of Mali.[1] Contributing factors include prosthesis use for amputated limbs (especially poorly fitting suction-type devices)[2, 3] and leg paralysis.[4] The less common variant, sometimes termed Stewart-Bluefarb syndrome, results from arteriovenous (AV) malformations or fistulae (eg, AV shunts placed for hemodialysis) and presents as dusky erythematous papules and plaques.[5, 6]
Individual lesions of acroangiodermatitis may persist unchanged for several years. Alternatively, the lesions can ulcerate and bleed and may become infected. Mortality and morbidity depend on the underlying condition.
Signs and symptoms
A history of venous stasis, AV shunt for hemodialysis, or a long-standing AV malformation is usually present. A limb prosthesis may be present. Patients occasionally report pruritus and pain.
Confluent, violaceous or brown-black papules may 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.[7] Note the images below.
 View Image | The physical findings in this patient who is HIV negative remained the same over a 3-year period. |
View Image | Lesions on the shin of a patient who is HIV negative. |
View Image | Classic Kaposi sarcoma on the foot of an elderly patient who is HIV negative. Compare this photo to the clinical photos of acroangiodermatitis. |
Diagnosis
Rule out HIV infection, especially if Kaposi sarcoma is suspected.
Transcutaneous oxygen pressure can detect hypoxia at the edge of the lesions. Polymerase chain reaction testing for human herpesvirus 8 can help in differentiating acroangiodermatitis from Kaposi sarcoma; however, such testing is not routinely available.
Obtain a biopsy sample for dermatopathologic evaluation.
Adya et al reported that on polarized dermoscopy, an acroangiodermatitis of Mali lesion showed brown dots, globules, and structureless areas, a reddish-pink structureless area, and shiny white lines and globules on a brownish-red background. In addition, there were multiple clods of 4 white dots in rosette formations.[8]
Imaging
Use plethysmography, Doppler ultrasonography, and oscillography to assess the venous flow and to detect underlying vascular malformations. Tognetti et al reported using diagnostic imaging with high-frequency ultrasound, high-resolution dermoscopy, and line-field optical coherence tomography.[9] Use arteriography to demonstrate arteriovenous fistulae.
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.
On immunohistochemistry, the proliferative fibroblastlike spindle cells around the vessels are positive for antibody to factor XIIIa. Immunostaining for the CD34 antigen demonstrated a strong labeling of endothelial cells of hyperplastic vessels in acroangiodermatitis.[10]
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. |
Management
Published accounts of acroangiodermatitis treatment consist largely of case reports, and include the following:
- 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.[11]
- Successful use of intermittent pneumatic compression therapy has been described.[12]
- 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.[13]
- Propranolol, 30 mg daily, increased to 30 mg twice daily after 2 months, successfully treated bilateral acroangiodermatitis on the plantar surfaces in one patient.[14]
Surgical care
In acroangiodermatitis resulting from arteriovenous malformations, surgical elimination of the shunts is curative. Multiple, small fistulae can be destroyed individually or by embolization; however, the latter method can lead to ischemia and necrosis.
Other treatments and consultations:
- Consult a phlebologist for management of 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
- If the patient is overweight or obese, losing weight may improve the circulation of the extremities
- Activity can be continued as allowed by the condition of the extremities and the underlying condition
- Further outpatient care depends on the underlying disease
Patient education
Educate patients about skin care and avoiding mechanical trauma that could induce ulceration or bleeding. Recommend that overweight or obese patients reduce their body weight.
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