Acroangiodermatitis is a benign vascular hyperplasia with a predominant lesion of the lower extremities, which in clinical and histological manifestations resembles Kaposi’s sarcoma. It is characterized by the appearance of spots and papules on the skin of the feet and shins, which gradually turn into plaques of red-purple or bluish color. The diagnosis is established on the basis of the clinical picture, histological examination data and angiography. Treatment approaches are determined by the form of the disease. Medical treatment is aimed at normalizing the tone of the veins, blood circulation in the tissues. The presence of arteriovenous fistulas is an indication for surgery.
ICD 10
I78.9 L30.8
General information
The term “Acroangiodermatitis” (pseudosarcoma telangiectatic, Kaposi’s pseudoangiosarcomatosis) in clinical dermatology refers to two diseases with their own pathogenesis, clinical course features and different treatment approaches: Mali acroangiodermatitis and Stewart-Blufarb syndrome. The first develops mainly in people 50-60 years old, the second is mostly diagnosed in adolescents and young people. Both forms of telangiectatic pseudosarcoma are among the rare dermatological diseases, mainly found in male patients.
Causes
The basis of the appearance of kaposh-like elements is chronic venous insufficiency. Increased venous pressure, stagnation in tissues cause the development of local metabolic disorders, vascular proliferation processes. The main reasons for the formation of characteristic skin manifestations are:
- Inferiority of veins. Insufficiency of venous valves, loss of elasticity of vascular walls leads to deformation of veins, their overstretching and overflow with blood. A vicious circle is formed: the wider the lumen of the vessel, the worse the venous valves cope with their function. Violation of the outflow of venous blood from the lower extremities causes progressive trophic disorders.
- Arteriovenous anomalies. Arteriovenous shunts located in the tissues of the lower extremities play a key role in the genesis of acroangiodermatitis. The pressure in the arteries is much higher than in the veins, in this regard, in the area of the anastomosis, arterial blood is discharged into the vein, bypassing the capillary bed. Arterial vessels below the shunt area receive less blood. This leads to chronic tissue ischemia, which can progress as the diameter of the fistula increases.
Risk factors include any external influences and internal changes that can provoke the development or aggravate the course of existing venous insufficiency: smoking, pathology of the endocrine glands, insufficient physical activity. The appearance of arteriovenous anastomoses can be the result of injuries.
Pathogenesis
Clinical manifestations of the disease (spots, papules and plaques) are secondary to the violation of blood outflow through the veins of tissue changes. Under conditions of hypoxia, capillary proliferation and progressive perivascular sclerosis are observed in the affected area. The walls of venules and deeper vertical veins are hypertrophied. Vessels acquire tortuosity. The capillaries of the skin twist in spirals, double in places, acquire an angiomatous appearance.
The iron-containing pigment hemosiderin is actively deposited around the dilated vessels, and extravasates of erythrocytes are detected. These deposits largely determine the characteristic color of spots and plaques on the skin. Congestive dermatitis with pseudosarcoma of the Mali type covers the upper part of the dermis, and with Stewart-Bluefarb syndrome, the dermis covers the entire depth.
Symptoms
Angiosarcoma of Mali
Kaposh-like elements on the skin have the appearance of plaques, the color of which changes over time from light pink to bluish-burgundy. The appearance of plaques may be preceded by the development of dermatitis, in which the skin of the shins and the back of the feet acquires an ochre-yellow hue. The lesion is usually symmetrical. Long-existing spots are not prone to ulceration, do not itch, do not hurt. Plaques are soft to the touch, their palpation does not cause unpleasant sensations in the patient.
Stewart-Bluefarb syndrome
In patients with this form of acroangiodermatitis, characteristic spots may appear against the background of clinical manifestations of a functioning arteriovenous anastomosis. The lesion is predominantly unilateral. The veins above the anastomosis area expand significantly and become noticeable under the skin, often pulsating noticeably. The skin is hot due to the active influx of arterial blood, compensatory expansion of capillaries. Spots and plaques of red-blue color gradually acquire a brownish hue. With the progression of the disease, the surface of the elements ulcerates.
Complications
A feature of the functioning of the arteriovenous shunt is the gradual and steady expansion of its lumen. Progressive circulatory disorder in the absence of the effect of conservative therapy and sparing surgical interventions leads to the appearance of extensive non-healing ulcers, the formation of tissue necrosis sites. In severe cases, progressive ischemia is an indication for amputation of a limb.
Diagnostics
The appearance of local dermatological symptoms, regardless of the patient’s age, requires a comprehensive examination. Excluded diagnoses include Kaposi’s sarcoma, Masson’s pseudoangiosarcoma, and spindle cell hemangioma. According to the test results, it is possible to determine the nature of pathological changes, their prevalence and degree of development. If necessary, a vascular surgeon may be involved in the diagnosis, in addition to a dermatologist. A comprehensive examination of a patient with suspected acroangiodermatitis includes:
- General inspection. A functioning vascular fistula can be detected by a noticeable pulsation of dilated veins. It also gives out a characteristic noise that can be heard with a stethoscope. The frequency of noise occurrence corresponds to the heart rate. Local hyperthermia and hyperhidrosis indirectly indicate the presence of arteriovenous anastomosis.
- Histological examination. A fragment of the skin of the affected area is taken for analysis. According to the existing features of the structure of tissue structures, a specialist can distinguish between diseases from the group of benign vascular proliferation and true sarcoma, and make the correct diagnosis. Pseudosarcoma is not characterized by the presence of vessels with a stellate lumen, C034 antigens on the surface of endothelial and perivascular cells.
- Angiography of the lower extremities. It involves the use of a radiopaque substance that is injected into the lumen of the arteries of the affected lower leg. On X-rays of young patients, you can see the junctions of arteries and veins. The study is prescribed for the differential diagnosis of benign vascular hyperplasia with Kaposi’s sarcoma, determining the location and size of shunts.
- Ultrasound examination of blood vessels. Ultrasound of the vessels of the lower extremities with a doppler is used to determine vascular anastomoses. According to ultrasound data, an angiosurgeon can make a conclusion about the expediency and scope of surgical treatment. Removal of areas of dilated veins without taking into account the existing vascular anastomoses can lead to a rapid relapse of the disease.
Treatment
Conservative therapy
Approaches to the therapeutic treatment of both types of pathology are largely similar and involve the elimination of hemodynamic disorders in the vessels of the lower extremities. Treatment regimens are selected taking into account the type and stage of the disease, the presence of concomitant health problems. The patient is recommended to give up smoking, normalize weight, increase physical activity. It is necessary to carry out a whole range of activities, which includes:
- Correction of CVI. Treatment of chronic venous insufficiency involves the appointment of drugs of multidirectional action. Bioflavonoid-based phleboprotectors increase the tone of the vascular wall. Disaggregants reduce blood viscosity, prevent the formation of blood clots. Angioprotectors have a complex effect on blood vessels, improve microcirculation, activate metabolic processes in tissues.
- Compression therapy. A good effect in the long term is the use of an elastic bandage or special compression underwear (stockings). The degree of compression is determined for each of the patients individually. Daily bandaging of the shins can significantly slow down the deformation of the veins, and in the presence of a joint, reduce the discharge of arterial blood into the venous channel.
- Local therapy. A pronounced venotosinating effect allows you to get regular use of creams and gels with antiplatelet agents and flavonoids in the composition. The healing of erosions and ulcers that have arisen against the background of trophic disorders involves the local use of broad-spectrum antibacterial drugs, wound healing agents.
- Physical therapy. In the complex therapy of trophic disorders in acroangiodermatitis, a medical laser, enzyme electrophoresis, and long-wavelength UFO are used to cleanse the bottom of ulcers from purulent-necrotic plaque, stimulate reparative processes. At the healing stage, it is advisable to carry out darsonvalization, irradiation with infrared rays, ultratonotherapy.
Surgical treatment
Indications for surgery are advanced cases of acroangiodermatitis, ineffectiveness of conservative therapy, the presence of a vascular shunt. The scope of surgical treatment is determined individually. The main operational methods for the elimination of trophic disorders include:
- Elimination of arteriovenous fistula. During the operation, the communication sections between the arteries and veins are disconnected. The elimination of arterial theft syndrome allows you to quickly normalize the blood supply to ischemic areas, reduce the pressure in the veins, normalize the outflow of blood through them. Special instruments used in endovascular surgery allow for intervention with minimal tissue damage.
- Skin plastic surgery. The method of skin transplantation allows to restore the integrity of the skin in the locations of long-term non-healing ulcerative defects in pseudosarcoma. Autodermoplasty can be performed using local tissues. If they are not enough, a skin flap with the help of a dermatome can be obtained from the femoral part of the leg or abdomen. Before the transplant, it is mandatory to clean the bottom of the ulcer from necrotic tissues.
- Amputation of the affected limb. Radical surgery is indicated in cases where it is not possible to normalize blood circulation in the affected limb. The level of stump formation is determined by the doctor taking into account the clinical picture and the results of the examination. The operation is carried out as planned, after a period of intensive preparation and comprehensive examination.
Prognosis and prevention
At the early stages of pathology development, the elimination of causal factors contributes to the rapid regression of clinical manifestations of acroangiodermatitis. In young people, recovery occurs when the vascular anastomosis is excised. Complex treatment of venous insufficiency with subsequent supportive courses of medication and physiotherapy allows patients with Mali angiodermatitis to achieve remission.
In the absence of treatment, the decrease in hemodynamics and trophic disorders steadily progress. As a prevention of pseudosarcoma, doctors recommend giving up harmful habits, reviewing the diet, adjusting body weight. Normalize blood circulation in the lower extremities by walking, cycling, jogging.
