When Degos disease is a very infrequent disease that affects the arterioles. To date, only about 150 cases of Degos syndrome are known, with a potential unreported number to be considered. In Degos syndrome, tiny damage to blood vessels occurs.
What is Degos Syndrome?
Degos syndrome is synonymous by some doctors as papulosis atrophicans or atrophic papulosquamous dermatitis. Degos syndrome has a very low prevalence in the population. As part of Degos syndrome, there is multiple damage to tiny blood vessels.
The lesions arise primarily from the fact that the blood vessels expand and are subject to destructive processes. As soon as the Degos syndrome also affects the brain and the blood vessels of the internal organs, the disease poses a threat to the lives of those affected. Degos syndrome was described for the first time in 1940 by the Austrian doctor Köhlmeier.
The pathologist assigned Degos syndrome to thrombangiitis obliterans and assumed that it was a special form of this disease. A short time later, the French dermatologist Degos found that the disease developed independently of the thrombangiitis obliterans. Sometimes there is a risk of confusion between Degos syndrome and Erythrokeratoderma en cocardes Degos. It is a skin disease that is rare and inherited. Currently only between 100 and 150 cases of Degos syndrome are known.
Causes
So far, there is hardly any reliable knowledge about the causes of the development of Degos syndrome in sick people. Further research into the disease is being sought, but in view of the low number of cases of the disease, this is currently only inadequately implemented. Degos syndrome may have a genetic component that promotes the development of the disease.
External factors are also potential triggers for Degos syndrome. There may also be a genetic disposition for Degos syndrome, which, however, only breaks out if there are corresponding environmental factors.
Symptoms, ailments & signs
Degos syndrome manifests itself through various typical signs. In the beginning, Degos syndrome manifests itself as tiny spots on the skin that are reddish in color. The diameter of this reddening is usually no more than one centimeter.
A few days after the red spots form, papules appear. Damage to the skin develops through these papules. At the edge of the papules there are blood vessels that have pathological enlargements. Over time, these lesions spread all over the skin in some patients.
However, they hardly show up in the area of the face, the soles of the feet and the inner surfaces of the hands. Degos syndrome is divided into a benign and a malignant type. The benign form of Degos syndrome occurs when only the skin is affected by the damage.
The malignant form of Degos syndrome occurs when the lesions also spread to the brain and internal organs. In this case, the sick people suffer from an increased risk of thrombosis, stroke and infarction of organs. For these reasons, numerous patients with malignant Degos syndrome die.
In the malignant type of Degos syndrome, the urinary bladder, kidneys, pancreas and pleura are affected by damage to the blood vessels. So far it is not known whether the benign form of Degos syndrome is merely an early stage of the disease which, depending on age, leads to death sooner or later when it first manifests.
In the malignant type, patients often die after a few months or years. It is particularly common for people to suffer gastrointestinal breakthroughs or brain insults, resulting in death.
Diagnosis
The diagnosis of Degos syndrome is usually made by a dermatologist, as the signs of the disease are mainly noticeable on the skin. An infestation of the internal organs, however, can hardly be determined. The typical symptoms of Degos syndrome, such as the skin lesions without itching or pain, indicate the disease.
After an initial consultation with the patient, the doctor examines the affected areas on the skin with the help of magnifying glasses. As a rule, the specialist also takes swabs of the diseased areas, which are analyzed in the laboratory. For example, there are indications of the presence of certain cell types that contribute to the diagnosis of Degos syndrome.
Complications
In most cases, Degos syndrome leads to damage and malformations in the blood vessels. This damage can have various negative effects on the body. First and foremost, patients suffer from severe red spots on the skin.
The spots can be distributed in different places and are not particularly large. Due to the inconspicuous symptom, diagnosis is made late in many cases. After the stain, papules usually form, which can damage the skin. Often the patients also suffer from itching in the affected areas.
In many cases, these complaints lead to a reduced self-esteem, as the patient feels uncomfortable with the symptoms and is ashamed of them. In the worst case, Degos syndrome can also develop in the brain and lead to a stroke. The patient can also die if there is no immediate treatment.
Targeted treatment is not possible. However, the symptoms can be limited so that life-threatening conditions do not arise. Life expectancy is not reduced if the patient takes the prescribed medication. However, it is not possible to cure the syndrome in the long term, so the sufferer has to rely on it for his entire life.
When should you go to the doctor?
If tiny, reddish-discolored patches of skin that develop into nodules are noticed, a doctor should be asked for advice. It is true that these noticeable skin changes are not necessarily due to Degos syndrome, but it is a serious disease that needs to be clarified. If this does not happen, the symptoms usually worsen quickly. In the case of Degos syndrome, the lesions spread over the entire skin. Anyone who notices such a spread should consult a dermatologist immediately.
This is especially true when the face, soles of the feet and inner surfaces of the hands do not show any skin spots – a typical indication of Degos syndrome. If the skin disease is left untreated, further symptoms can arise. In the benign form, the lesions continue to widen and ultimately make a doctor’s visit essential. In the malignant form, the spots sometimes also spread to the brain and internal organs. This can lead to organ infarctions, thromboses or strokes, which require immediate emergency medical treatment.
Treatment & Therapy
The treatment options for Degos syndrome are so far only relatively limited. Because the causes of Degos syndrome are not known, so that causal therapeutic approaches are fundamentally impossible. Various attempts have used immunosuppressive drugs in patients with Degos syndrome, but they have not been successful. The best results were seen with the administration of acetylsalicylic acid.
Medical agents with an anticoagulant effect have also already been tried, but have hardly proven themselves. Acetylsalicylic acid is therefore currently prescribed in most cases because it reduces the aggregation of platelets.
Outlook & forecast
Since Degos syndrome is a very rare disease, no general prognosis can be given. However, there is no self-healing, and the syndrome can only be treated symptomatically. A causal cure is not possible.
If there is no treatment, the patients suffer from reddened skin or from spots on the skin. Papules or pustules can also form on the skin, which can significantly reduce the aesthetics.
Degos syndrome also increases the likelihood of a stroke or thrombosis, so that those affected depend on regular examinations. If left untreated, life expectancy can be reduced. The syndrome can also attack the kidneys or other internal organs, causing tumors to develop in these organs.
Treatment of the syndrome is done by taking medication that can relieve the discomfort. Regular examinations of the internal organs are necessary in order to detect tumors at an early stage. As a rule, those affected with Degos syndrome are dependent on lifelong therapy, as the disease cannot be completely cured.
Prevention
The Degos syndrome has so far hardly been researched through scientific studies, in particular the exact causes are not yet known. Therefore, targeted prevention of Degos syndrome is currently not possible. So far, following a successful diagnosis, there have only been symptomatic therapeutic approaches, which, however, have achieved little success.
Aftercare
Since Degos syndrome is a hereditary disease, it cannot be completely cured. As a rule, therefore, there are no special follow-up measures available to the person affected, so that the patient is primarily dependent on rapid and, above all, early detection of the disease. Independent healing cannot occur in Degos syndrome.
The focus of this disease is therefore on early detection so that further complications can be avoided. The symptoms of this syndrome can be relieved by various medications. The person affected should always ensure that they are taken regularly and that the dosage is correct, so that the symptoms can be properly alleviated.
In many cases, a varied diet can reduce the symptoms and improve the patient’s quality of life. Furthermore, with Degos syndrome, regular examinations by a general practitioner are very useful. Above all, blood samples should be taken regularly.
The syndrome significantly increases the likelihood of a stroke, so that the patient should generally pay attention to a healthy lifestyle and a balanced diet. Whether the disease will lead to a reduced life expectancy cannot generally be predicted.
You can do that yourself
Degos syndrome is a serious disease, the cause of which has not yet been treated. The self-help measures focus on taking the medication according to the doctor’s instructions and informing him of any side effects and interactions so that optimal medication can be achieved.
The doctor will also recommend the patient a healthy lifestyle with a balanced diet and exercise. The disease cannot be cured by this, but it progresses much more slowly when the immune system is strengthened. Degos syndrome usually also represents an emotional burden for those affected. The noticeable skin changes can cause inferiority complexes or even depression, which must be treated at an early stage.
The sick person should turn to a therapist if they have mental problems. If desired, the doctor can also establish contact with other affected persons. Above all, going to a self-help group helps to accept the illness and its manifestations and to lead a full life despite the symptoms.
In the case of a malignant expression, the sick person needs support in everyday life. This is where relatives and friends are asked, who have to watch out for any symptoms and, if in doubt, should call in the emergency services.
