Livedo racemosa is characterized by a reticular reddish-purple drawing on the skin. This skin marking can occur idiopathically or as an accompanying symptom of other diseases. A particular variant of livedo racemosa is the so-called Sneddon syndrome, which can lead to strokes, facial deficits, and epileptic seizures.
What is livedo racemosa?
Livedo racemosa is often not a disorder in its own right, but an accompanying condition of other diseases. It appears as a reticular reddish-purple skin marking on the trunk of the body, upper arms, or legs. The skin coloration usually intensifies in cold weather. The causes can be varied. Thus, livedo racemosa can appear idiopathically or in association with other diseases. Overall, the skin markings are based on circulatory disorders caused by inflammatory processes or increased proliferation of endothelial cells within the blood vessels. Therefore, in addition to livedo racemosa, the term vasculitis racemosa is also used. The term livedo reticularis is often used synonymously. However, it must be noted that although livedo racemosa and livedo reticularis show similar skin markings, they are based on different causes. While inflammation and occlusion are causative factors in livedo racemosa, short-term dysregulation within the blood vessels plays a role in livedo reticularis.
Causes
There are multiple causes of livedo racemosa. However, there is always an underlying constriction or even occlusion of smaller and medium-sized blood vessels. The corresponding skin area can no longer be properly supplied with blood and thus with oxygen, resulting in the reddish-purple skin discoloration. The constrictions and occlusions of the blood vessels are triggered by inflammatory or non-inflammatory processes. In the case of inflammation, there is an increased formation of new endothelial cells, which can lead to stenosis. Furthermore, increased viscosity of the blood can also support the formation of microthrombi, which in turn disrupt the blood supply. In idiopathic forms, the cause of the stenosis or inflammation often cannot be determined. This particularly affects young adults and middle-aged women. However, livedo racemosa is often part of a systemic process and thus a concomitant of an underlying disease. This may be infections such as bacterial endocarditis, syphilis or tuberculosis. In many cases, autoimmune diseases play a role such as rheumatoid arthritis, collagenoses, or phospholipid antibody syndrome. Hepatides, pancreatitis or arteriosclerosis may also be accompanied by livedo racemosa. Arterial embolism may also cause livedo racemosa. Similarly, genetic factors may contribute to the development of livedo racemosa.
Symptoms, complaints, and signs
As mentioned earlier, livedo racemosa manifests as a reticular reddish-purple pattern on the skin. It mainly affects the legs, upper arms, or trunk of the body. In idiopathic forms, ulceration of the legs may also occur. Exposure to cold intensifies the discoloration. Unlike livedo reticularis, however, the symptoms do not disappear on warming. A special form of livedo racemosa is the so-called Sneddon syndrome. In Sneddon’s syndrome, small cerebral arteries are narrowed in addition to the skin markings. Here, complications in the form of strokes, facial deficits or epilepsies can occur. In addition to cerebral infarctions, spinal cord infarctions can also occur in rarer cases. Livedo racemosa can announce itself years before its onset through constant attacks of dizziness and headaches. In the extreme form of livedo racemosa, Sneddon’s syndrome, there is a gradual decline in memory and cognitive abilities until dementia develops. The patient is conspicuous by personality changes. In rare cases, neurological symptoms such as seizures, chorea or myelopathies are observed. As a result of the disease, hypertension, valvular heart disease, eye disease, or kidney damage may develop.
Diagnosis and course
When livedo racemosa occurs, the underlying disease must be diagnosed. Furthermore, clarification of the extent to which smaller cerebral arteries are involved is also important.This may be indicated by unexplained strokes or unusual cognitive decline without preceding strokes in younger adults. MRI scans of the brain reveal white matter changes, microbleeds, infarcts, or atrophy. Differentially, various other brain organic and neurological diseases such as cerebral asoconstriction syndrome, vascular dementia, or even migraine must be excluded. Externally, however, the persistent reticular skin markings already suggest that the involvement of the small and middle cerebral arteries is responsible for the neurologic deficits.
Complications
Livedo racemosa always indicates that inflammatory processes are taking place in certain blood vessels. As already known, the reticular bluish-purple skin markings can occur both idiopathically and as an accompanying symptom of serious diseases. Already many of the underlying diseases often lead to severe complications. Livedo racemosa itself, however, 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 severe underlying diseases, livedo racemosa is very often associated with complications of any kind. However, complications are also observed in the idiopathic form of livedo racemosa, which occurs mainly in young women and presents as ulcers on the legs. Usually, livedo racemosa disappears when the underlying disease heals. However, there are also very severe courses with sometimes even fatal complications. One such complication is the so-called Sneddon syndrome. In Sneddon’s syndrome, cerebral arteries are affected. Here, strokes, epileptic seizures, and visual field loss are common. Small hemorrhages and microinfarctions occur in the brain. These can already occur in younger patients. What is noticeable in them is an unusually severe decline in mental performance up to and including dementia within a short period of time. Despite treatment with blood-thinning medications or antiphospholipid antibodies, late damage often remains due to the previous degradation processes in the brain.
When should you see a doctor?
Livedo racemosa must always be treated by a physician. 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, livedo racemosa already causes serious complaints, which are always treated by a doctor. These include, for example, infarctions or disturbances of sensitivity. Furthermore, also facial deficits and dizziness can speak for this disease and have to be treated. If the affected person suffers from eye complaints, the respective diseases must be examined and removed by a doctor. It is not uncommon for the symptoms of livedo racemosa to include kidney problems, and in the worst cases, a kidney transplant or dialysis may be necessary for the patient to survive. Therefore, the treatment itself usually takes place in a hospital and involves a prolonged hospital stay. If the affected person suffers from severe and persistent headaches or dizzy spells, these may likewise be a symptom of livedo racemosa.
Treatment and therapy
In an uncomplicated form of livedo racemosa, the main focus is on treating any underlying disease. However, if during diagnosis there is involvement of cerebral arteries in the form of the so-called Sneddon syndrome, care must be taken to improve microvascular perfusion to prevent late damage to the brain. Therapy with antiphospholipid antibodies has proven effective in apl-positive patients. In apl-negative patients, blood viscosity is probably too high and can be treated by administration of aspirin. ACE inhibitors serve to reduce endothelial cell division, and prostaglandin improves blood flow in microvascular arteries.
Outlook and Prognosis
Because of livedo racemosa, the patient may develop severe symptoms. Most notably, these include epileptic seizures and myocardial infarction. In the worst case, the patient dies due to these symptoms. In livedo racemosa, the affected person suffers from an altered skin color, which is further intensified especially by cold.Similarly, facial deficits may occur, in which the affected person suffers from sensory disturbances or perceptual disturbances and can no longer feel certain regions of the face. Severe headaches and dizziness often occur as well. The patient’s quality of life is greatly reduced by livedo racemosa. Many affected individuals also suffer from eye disorders and seizures. Kidney damage can also occur, which can lead to death if left untreated. The patient is therefore dependent on dialysis. Treatment of livedo racemosa is usually causal and is aimed primarily at the underlying disease. The course of the disease depends to a large extent on the disease itself and how it spreads. Livedo racemosa often leads to a deterioration of mental abilities, resulting in dementia or mental retardation in the patient. Its extent depends greatly on the damage to the brain.
Prevention
A specific recommendation for prophylaxis of livedo racemosa cannot be given because it has no single cause and often occurs in the setting of systemic diseases. However, preventive measures can be taken for many underlying conditions. In general, of course, the risk can 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.
Follow-up
The measures of an aftercare depend in the case of a livedo racemosa usually very much on the exact expression, so that thereby usually no general prediction can be made. However, all skin diseases must first and foremost be examined and treated by a physician to prevent further complications or discomfort. The earlier the disease is detected and treated by a doctor, the better the further course will be. A high standard of hygiene can have a positive effect on the course of recovery. In most cases, treatment is carried out by applying creams or ointments and by taking medication. The affected person should pay attention to regular use and also to the correct dosage in order to permanently alleviate the symptoms. Regular checks by a doctor are very important. In most cases, the disease does not have a negative effect on the life expectancy of the person affected. Contact with other patients can also be useful in this regard.
What you can do yourself
If livedo racemosa is suspected, a physician should first be consulted. In support of this, various self-measures can be taken. First, it is important to promote blood flow to the microvascular arteries. Applications with red vine leaf extract and phyto-extract stimulate the blood flow in the arteries and thus promote the healing of the 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 cold, as this can intensify the discoloration. Warm applications have an analgesic effect and help to loosen the constricted blood vessels. This can be accompanied by a complaints diary in which the type and severity of the symptoms as well as possible previous illnesses and allergies are recorded. This enables the family doctor to quickly diagnose and treat livedo racemosa. However, the most effective remedy is prevention. Prophylactic measures can be taken for a variety of the possible causes of skin signs. In general, the risk can be reduced by a healthy lifestyle. These include exercise, a balanced diet, and avoiding stress, as well as abstaining from stimulants such as alcohol, nicotine, and caffeine.
