Livedovasculopathy is a disease that manifests itself in disorders of blood clotting in small, dermal blood capillaries. Livedovasculopathy causes the affected tissue to die, which is associated with significant pain. In addition, necrosis forms on the skin areas affected by livedovasculopathy. The pathologic changes leave irreversible scars on the skin.
What is livedovasculopathy?
In principle, livedovasculopathy occurs very rarely. Synonymous disease terms include atrophie blanche or livedo-vasculitis. Livedovasculopathy is formed primarily by coagulation disorders in the skin. Livedovasculopathy represents a chronic disease of blood vessels located inside the skin. Tiny capillaries in the upper areas of the skin are affected. Due to livedovasculopathy, occlusions of the blood vessels develop. As a result, the upper skin regions are not supplied with sufficient blood. As a result, the affected skin tissue perishes, which is called skin infarction in medical terminology. Livedovasculopathy results in open skin wounds that are associated with intense pain. The resulting ulcers heal with difficulty. In the majority of cases, livedovasculopathy is diagnosed relatively late. In principle, however, the treatment options available to date have been comparatively successful.
Causes
The mechanism of development of livedovasculopathy has been clarified for the most part; however, not all underlying causes have been investigated. The skin area contains numerous tiny blood vessels that supply the skin with blood, oxygen, and nutrients. Due to currently unknown factors, the tendency of the blood to clot increases in the corresponding areas. This phenomenon is referred to in medicine as increased thrombophilia. The upper skin regions are supplied by only a small number of blood vessels. The occlusion of vessels therefore quickly results in an undersupply of the skin areas, which subsequently die (medical term necrosis). A characteristic feature of livedovasculopathy is that the pathological developments occur mainly in the warm months of the year. Some researchers suspect that a lowered concentration of water in human blood increases the tendency to clotting. This is because blood viscosity is increased at high air temperatures. In addition, livedovasculopathy occurs approximately three times more frequently in female patients than in male patients. The exact causes of this high sex difference are not currently understood.
Symptoms, complaints, and signs
Livedovasculopathy is mainly characterized by a characteristic combination of three leading complaints: First, affected patients show so-called livedo-racemosa signs. These already indicate that the respective person has a particular susceptibility to the development of livedovasculopathy. Another important sign of livedovasculopathy consists of ulcers that manifest themselves in open skin wounds. This is an acute symptom of livedovasculopathy. Eventually, the diseased skin site develops into what is called atrophie blanche, which refers to the scar on the skin. The scar, as a remnant of the acute phase of livedovasculopathy, always remains visible on the skin site.
Diagnosis and course of the disease
A diagnosis of livedovasculopathy is usually based on the clinical signs of the disease. Patients with suspected livedovasculopathy consult their dermatologist. A history is compulsorily taken before clinical examinations. This is followed by an initial visual examination, during which the treating physician examines the affected area in more detail, using special magnifying glasses, for example. Tissue samples, which are analyzed in the laboratory, are also possible. On the basis of the patient’s descriptions of the present complaints as well as the clinical clinical picture, a relatively reliable diagnosis of livedovasculopathy is possible.
Complications
Patients usually suffer from very severe pain as a result of livedovasculopathy. This pain can be stabbing and burning and significantly reduces the patient’s quality of life.The affected person’s entire daily life is also significantly restricted and reduced by livedovasculopathy, so that ordinary activities are usually no longer possible for the affected person. It is not uncommon for this disease to also leave large scars, which are usually irreversible and therefore do not heal. The scars cause the patient to have reduced aesthetics and thus possibly inferiority complexes or reduced self-esteem. Not infrequently, the pain can also occur at night, leading to sleep problems or even depression. Treatment of livedovasculopathy can be done with injections and is not related to any particular complications. In most cases, the symptoms are relatively well limited and treated by these injections, so that the affected person can lead an ordinary life again. However, this does not prevent further outbreaks of this disease, so that the affected person may have to rely on renewed treatment. However, life expectancy is not reduced or limited by this disease.
When should you see a doctor?
When open sores or other signs of illness occur seemingly without cause, expert advice is needed. A dermatologist can determine whether the trigger is livedovasculopathy. Individuals who have a suspicion should seek early advice from their primary care physician, who can make an initial diagnosis and then refer the patient to a specialist if necessary. At the latest when there are subsequent symptoms such as inflammation or scarring of the skin, the condition must be taken to a dermatologist. Women are significantly more likely to suffer from livedovasculopathy. The disease also occurs predominantly in the summer months. If the described symptoms occur and possibly also named risk factors are present, a livedovasculopathy can be assumed. In this case, a medical appointment must be made so that the disease can be quickly diagnosed and treated. If this is done early, permanent damage can usually be avoided. The right doctor is the family doctor or a dermatologist. If the skin changes represent a mental burden, a therapist consultation is recommended.
Treatment and therapy
The options for treatment of livedovasculopathy are comparatively well developed. Basically, the focus of treatment-related measures is to reduce the tendency of the blood to clot. For this purpose, drug therapy is usually used first. The patient is usually given active pharmaceutical ingredients that are also used to prevent thrombosis. Therefore, the physician often prescribes heparins to the persons suffering from livedovasculopathy, which are usually administered in the form of injections. The use of other drugs for the treatment of livedovasculopathy is being investigated in numerous research studies. In one such study, the active ingredient rivaroxaban showed good efficacy. Treatment with this drug requires sensitive coordination between patient and physician regarding dosage as well as duration of administration. The risk of potential bleeding must be thoroughly weighed. The prognosis of livedovasculopathy is comparatively positive. If appropriate treatment measures are taken, good control of livedovasculopathy is possible. In this context, the administration of anticoagulants plays an essential role. As a consequence, pain usually also decreases for the affected patients. However, there is a risk of recurrent livedovasculopathy. For this reason, long-term medical management of affected individuals in a specialized center is advisable.
Outlook and prognosis
If the affected person undergoes medical treatment, he or she has a good chance of alleviating the symptoms. Long-term therapy becomes necessary, since the disorder of blood clotting does not belong to a temporary health impairment. In most cases, therefore, there is no recovery, but freedom from symptoms as soon as medication is taken at regular intervals. If left untreated, livedovasculopathy can lead to numerous complications. In addition to impairments in everyday life, secondary diseases develop. Some of them lead to a life-threatening condition.For an improved assessment of the situation and to avoid health risks, treatment should be initiated at the first irregularities and complaints. Medications help the organism with the process of blood clotting and bring it into a necessary balance. To avoid side effects and the correct dosage of the prescribed drugs, close cooperation with the attending physician is necessary. This improves the general health as well as the quality of life. In some patients there may be periods of freedom from symptoms. During these, no medication needs to be given temporarily. Nevertheless, regular check-ups and medical tests are necessary, as the symptoms can relapse at any time. The more mindful the affected person is with the signals of his organism, the better the health development.
Prevention
Currently, there is little established knowledge regarding effective measures for the prevention of livedovasculopathy. However, as with many other diseases, cessation of tobacco use is recommended for those at risk. This is because nicotine constricts blood vessels and, for this reason, possibly increases the tendency to blood clots.
Aftercare
Aftercare for a blood clotting disorder is provided by means of preventive measures and medical checkups. The type of aftercare also depends on the nature of the blood clotting disorder. In almost every case, it is necessary to have the flow properties of the blood checked regularly and to keep track of the clotting factors. In people with a strong tendency to clotting, follow-up consists primarily of regular checks of the vessels. Since thromboses occur frequently, they must be detected early. Accordingly, prophylactic administration of blood thinners may be useful. In stressful situations involving little exercise, such as long-distance flights, the administration of blood thinners is recommended. In addition, follow-up care also consists of clarifying abnormalities (for example, skin discoloration, blood in the stool or urine) and urgently avoiding injuries. Medications, the administration of hormones or donor factors may be necessary for prophylaxis throughout life. If internal bleeding has led to tissue damage, follow-up care consists of appropriate therapy. For affected muscles or bones, this means using physical therapy. Endurance sports are suitable, but not contact sports. In the case of damage to organs, on the other hand, aftercare must be based on the damage in question.
What you can do yourself
It is not possible to treat livedovasculopathy by self-help measures. Therefore, affected individuals depend on medical treatment to prevent complications and further discomfort. As a rule, livedovasculopathy is treated with the help of drugs that reduce blood clotting. The affected person should ensure that these medications are taken regularly. Likewise, various interactions with other medications must be taken into account, as otherwise there may be a reduced effect. As a rule, no further treatment is required. The affected person should protect his body and thus avoid bleeding and injuries. As a rule, the consumption of nicotine also has a negative effect on the course of the disease and should therefore be discontinued in any case. If the cessation of smoking is not by the sole will, friends or family can also help in this process. In severe cases, withdrawal can also be carried out. Furthermore, contact with other sufferers of livedovasculopathy is often worthwhile, as this can lead to an exchange of information.
