Livedo racemosa is characterized by a net-like reddish-violet marking on the skin. This skin marking can occur both idiopathically and as an accompanying symptom of other diseases. A special variant of livedo racemosa is the so-called Sneddon syndrome, which can lead to strokes, facial defects and epileptic seizures.
What is a livedo racemosa?
According to Biotionary, livedo racemosa is often not an independent disorder, but a side effect of other diseases. It appears as a web-like reddish-purple skin markings on the trunk, upper arms, or legs. The skin color usually increases when it is cold. The causes can be varied.
Livedo racemosa can appear both idiopathically and accompanied by other diseases. Overall, the skin markings are based on circulatory disorders that are caused by inflammatory processes or increased proliferation of the endothelial cells within the blood vessels. Therefore, in addition to Livedo racemosa, Vasculitis racemosa is also spoken of.
The term livedo reticularis is often used synonymously. However, it must be noted that although Livedo racemosa and Livedo reticularis show similar skin markings, the underlying causes are different. While inflammation and occlusion are the causes of livedo racemosa, short-term dysregulations within the blood vessels play a role in livedo reticularis.
Causes
There are many causes of livedo racemosa. However, there is always a narrowing or even an occlusion of smaller and medium-sized blood vessels. The corresponding skin area can no longer be properly supplied with blood and thus with oxygen, which leads to reddish-purple skin discoloration.
The narrowing and occlusion of the blood vessels are triggered by inflammatory or non-inflammatory processes. Inflammation leads to an increased formation of new endothelial cells, which can lead to a stenosis. Furthermore, increased blood viscosity can support the formation of microthrombi, which in turn disrupt the blood supply.
In the idiopathic forms, the cause of the stenosis or inflammation often cannot be determined. Young adults and middle-aged women are particularly affected. However, livedo racemosa is often part of a systemic process and thus a side effect of an underlying disease. These can be infections such as bacterial endocarditis, syphilis or tuberculosis.
In many cases, autoimmune diseases play a role, such as rheumatoid arthritis, collagen diseases or phospholipid antibody syndrome. Hepatides, pancreatitis or arteriosclerosis can also be accompanied by livedo racemosa. Arterial embolism can also cause livedo racemosa. Likewise, genetic factors can contribute to the formation of a livedo racemosa.
Symptoms, Ailments & Signs
As already mentioned, livedo racemosa manifests itself as a net-like reddish-violet marking on the skin. Legs, upper arms or the trunk are mainly affected. Ulcerations on the legs can also occur in the idiopathic forms. Exposure to cold increases discoloration. In contrast to livedo reticularis, however, the symptoms do not disappear when heated.
A special form of livedo racemosa is the so-called Sneddon syndrome. In Sneddon syndrome, small cerebral arteries are narrowed in addition to the skin markings. This can lead to complications in the form of strokes, facial defects or epilepsy. In rare cases, in addition to cerebral infarctions, spinal cord infarctions can also occur.
A livedo racemosa can be announced years before it breaks out by constant attacks of dizziness and headaches. In the extreme form of livedo racemosa, the Sneddon syndrome, there is a gradual deterioration in memory and cognitive abilities up to dementia.
The patient is characterized by personality changes. In rare cases, neurological symptoms such as seizures, chorea or myelopathies are observed. The disease can result in high blood pressure, heart valve defects, eye diseases or kidney damage.
Diagnosis & History
If livedo racemosa occurs, the underlying disease must be diagnosed. It is also important to clarify to what extent smaller cerebral arteries are involved. Unexplained strokes or unusual cognitive decline without prior strokes in younger adults may indicate this. MRI scans of the brain reveal changes in the white matter, microbleeds, infarcts or atrophies.
Various other organic and neurological diseases such as cerebral asoconstriction syndrome, vascular dementia or migraine must be excluded in the differential diagnosis. Externally, however, the persisting net-shaped skin markings already indicate that the involvement of the small and medium-sized cerebral arteries is responsible for the neurological deficits.
Complications
A livedo racemosa always indicates that inflammatory processes are taking place in certain blood vessels. As already known, the net-like bluish-purple skin markings can occur both idiopathically and as a side effect of serious diseases. Many of the underlying diseases often lead to serious complications.
However, the livedo racemosa itself causes the occlusion of small blood vessels, especially when exposed to cold, and thus impedes the blood supply to the affected areas. Due to the often serious underlying diseases, livedo racemosa is very often associated with complications of all kinds. But complications are also observed in the idiopathic form of livedo racemosa, which mainly occurs in young women, which present themselves as ulcers on the legs.
As a rule, livedo racemosa disappears when the underlying disease has healed. However, there are also very severe courses with sometimes fatal complications. One such complication is the so-called Sneddon syndrome. Cerebral arteries are affected in Sneddon syndrome. This often leads to strokes, epileptic seizures and visual field defects.
Small hemorrhages and microinfarcts occur in the brain. These can occur even in younger patients. What is striking in them is an unusually strong reduction in mental performance, even to the point of dementia within a short period of time. Despite treatment with blood-thinning drugs or antiphospholipid antibodies, long-term damage often remains as a result of the previous degradation processes in the brain.
When should you go to the doctor?
Livedo racemosa must always be treated by a doctor. Without treatment, serious and life-threatening complications and symptoms can occur, which in the worst case can lead to the death of the patient. As a rule, the Livedo racemosa already leads to serious symptoms that are always treated by a doctor. These include, for example, heart attacks or sensory disorders. Furthermore, facial loss and dizzy spells can also indicate this disease and must be treated.
If the person concerned suffers from eye problems, the respective diseases must be examined and removed by a doctor. It is not uncommon for the symptoms of livedo racemosa to also include kidney problems, with the worst case scenario requiring a kidney transplant or dialysis for the patient to survive. The treatment itself therefore usually takes place in a hospital and is associated with a longer hospital stay. If the person concerned suffers from severe and permanent headaches or dizzy spells, these can also be a symptom of livedo racemosa.
Treatment & Therapy
In an uncomplicated form of livedo racemosa, the main focus is on the treatment of the underlying disease. However, if the diagnosis reveals involvement of cerebral arteries in the form of the so-called Sneddon syndrome, care must be taken to improve microvascular blood flow in order to avoid late damage to the brain.
Therapy with antiphospholipid antibodies has proven effective in apl-positive patients. In apl-negative patients, the blood viscosity is probably too high, which can be treated by administering aspirin. ACE inhibitors serve to reduce cell division of endothelial cells and prostaglandin improve blood flow in microvascular arteries.
Outlook & Forecast
Due to livedo racemosa, the patient can develop serious symptoms. These include, above all, epileptic seizures and a heart attack. In the worst case, the patient dies because of these symptoms.
With Livedo racemosa, the affected person suffers from a changed skin colour, which is intensified again above all by the cold. Losses in the face can also occur, in which the person concerned suffers from sensory disturbances or disturbances in perception and can no longer feel certain regions of the face. Severe headaches and dizziness are also common. The patient’s quality of life is greatly reduced by livedo racemosa.
Many sufferers also suffer from eye diseases and seizures. Kidney damage can also occur, which if left untreated can lead to death. The patient is therefore dependent on dialysis.
The treatment of livedo racemosa is usually causal and primarily aims at the underlying disease. The course of the disease depends heavily on the disease itself and how it spreads. Livedo racemosa often leads to a reduction in mental abilities, resulting in dementia or mental retardation in the patient. Its extent depends heavily on the damage to the brain.
Prevention
A concrete recommendation for the prophylaxis of livedo racemosa cannot be given because it has no uniform cause and often occurs in the context of systemic diseases. However, preventative measures can be taken for many underlying conditions. In general, the risk can of course be reduced by a healthy lifestyle, especially since arteriosclerosis can also lead to livedo racemosa. This includes a balanced diet, plenty of exercise, avoiding stress and abstaining from smoking and alcohol.
Aftercare
In the case of livedo racemosa, the follow-up measures usually depend very much on the exact form, so that no general prediction can usually be made. However, all skin diseases must first and foremost be evaluated and treated by a doctor to avoid further complications or discomfort. The earlier this disease is recognized and treated by a doctor, the better the further course.
A high standard of hygiene can have a positive effect on the course of recovery. In most cases, treatment is by using creams or ointments and taking medication.
The affected person should pay attention to regular use and the right dosage in order to permanently relieve the symptoms. Regular check-ups by a doctor are very important. In most cases, the disease does not have a negative impact on the life expectancy of the person affected. Contact with other patients can also be useful.
You can do that yourself
If livedo racemosa is suspected, a doctor should first be consulted. Various self-measures can be taken to support this. The first thing to do is to stimulate blood flow in the microvascular arteries. Applications with red vine leaf extract and phyto extract stimulate blood flow in the arteries and thus promote the healing of skin signs.
If the viscosity of the blood is too high, medication with acetylsalicylic acid can help. In addition, the affected skin area should be protected from the cold, as this can intensify the discoloration. Warm applications have a pain-relieving effect and help to relax the constricted blood vessels. At the same time, a complaints diary can be created in which the type and severity of the symptoms as well as possible previous illnesses and allergies are recorded. This enables the general practitioner to diagnose and treat livedo racemosa quickly.
The most effective remedy, however, is prevention. Prophylactic measures can be taken for a variety of possible causes of skin signs. In general, the risk can be reduced by a healthy lifestyle. This includes exercise, a balanced diet and avoiding stress, but also avoiding stimulants such as alcohol, nicotine and caffeine.
