Degos syndrome is a very rarely occurring disease that affects the arterioles. To date, only about 150 cases of Degos syndrome have been reported, with a potential number of unreported cases to consider. Degos syndrome results in minute damage to blood vessels.
What is Degos syndrome?
Degos syndrome is also known synonymously by some medical professionals as papulosis atrophicans or atrophic papulosquamous dermatitis. Degos syndrome occurs with very low prevalence in the general population. In Degos syndrome, there is multiple damage to tiny blood-bearing vessels. The lesions occur primarily because the blood vessels dilate and undergo destructive processes. Once Degos syndrome also affects the brain and blood vessels of internal organs, the disease poses a threat to the lives of affected individuals. Degos syndrome was first described in 1940 by the Austrian physician Köhlmeier. The pathologist assigned Degos syndrome to thrombangiitis obliterans and assumed that it was a special manifestation of this disease. A short time later, the French dermatologist Degos stated that the disease develops independently of thrombangiitis obliterans. Sometimes there is a risk of confusion between Degos syndrome and erythrokeratoderma en cocardes Degos. This is a disease of the skin that is rare and inherited. Currently, only between 100 and 150 cases of Degos syndrome are known.
Causes
To date, there is little established knowledge regarding the causes for the development of Degos syndrome in affected individuals. Further research into the disease is being sought, but in view of the small number of cases of the disease, it is currently poorly implemented. It is possible that Degos syndrome has a genetic component that promotes the development of the disease. In addition, external factors may be potential triggers of Degos syndrome. It is also possible that there is a genetic predisposition to Degos syndrome, but that it only develops in the presence of appropriate environmental factors.
Symptoms, complaints, and signs
Degos syndrome is manifested by several typical signs. Initially, Degos syndrome manifests itself by tiny patches on the skin that have a reddish tinge. The diameter of these rednesses is usually not more than one centimeter. A few days after the formation of the red spots, papules appear. Damage to the skin develops as a result of these papules. At the edge of the papules there are blood vessels that have pathological dilatations. Over time, these lesions spread over the entire skin in some patients. However, they almost do not show up on the face, soles of the feet, and inner surfaces of the hands. Degos syndrome is divided into benign and malignant types. The benign form of Degos syndrome is present when only the skin is affected by the damage. The malignant form of Degos syndrome exists when the lesions also spread to the brain and internal organs. In this case, the affected individuals suffer from an increased risk of thrombosis, stroke and infarction of organs. For these reasons, many patients with malignant Degos syndrome die. In the malignant type of Degos syndrome, blood vessel damage affects the urinary bladder, kidneys, pancreas, and pleura, among other organs. To date, it is not known whether the benign type of Degos syndrome is merely an early stage of the disease that leads to death sooner or later, depending on the age of initial manifestation. In the malignant type, patients often die after several months or years. Particularly frequently, individuals suffer breakthroughs of the gastrointestinal tract or insults of the brain, as a result of which they die.
Diagnosis
Diagnosis of Degos syndrome is usually made by a dermatologist because the signs of the disease are most noticeable on the skin. In contrast, an affection of the internal organs is hardly detectable. The typical symptoms of Degos syndrome, such as skin lesions without itching or pain, indicate the disease. After an initial patient interview, the physician examines the affected areas on the skin with the aid of magnifying glasses.As a rule, the specialist also takes swabs of the diseased areas, which are analyzed in the laboratory. This reveals, for example, evidence of the presence of certain cell types that contribute to the diagnosis of Degos syndrome.
Complications
In most cases, Degos syndrome results in damage and malformations to the blood vessels. These damages can have different 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, in many cases it is diagnosed at a late stage. After the spotting, there is usually the formation of papules, which can damage the skin. Often, patients also suffer from itching in the affected areas. Due to these complaints, in many cases there is a decreased self-esteem, as the patient feels uncomfortable with the symptoms and ashamed of them. In the worst case, Degos syndrome can also develop in the brain, leading to a stroke. In this case, the patient may also die if no immediate treatment occurs. A specific treatment is not possible. However, the symptoms can be limited so that life-threatening conditions do not occur. 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 affected person depends on them for his or her entire life.
When should one go to the doctor?
When tiny, reddish discolored patches of skin are noticed, from which nodules form, a doctor should be consulted for advice. Although these noticeable skin changes are not necessarily due to Degos syndrome, they are due to a serious condition that needs to be clarified. If this is not done, the symptoms usually worsen rapidly. 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 if the face, soles of the feet and inner surfaces of the hands do not show any skin patches – a typical indication of Degos syndrome. If the skin disease remains untreated, further complaints may develop. In the benign form, the lesions continue to increase and eventually make a visit to the doctor indispensable. In the malignant form, the spots sometimes spread to the brain and internal organs. As a result, organ infarcts, thromboses, or strokes may occur, requiring immediate emergency medical treatment.
Treatment and therapy
Treatment options regarding Degos syndrome have been relatively limited. This is because the causes of Degos syndrome are unknown, making causative therapeutic approaches fundamentally impossible. Various attempts used immunosuppressive drugs in the patients with Degos syndrome, but they were not successful. The best success was seen with the administration of acetylsalicylic acid. Medical agents with an anticoagulant effect have also been tried, but have shown little success. Acetylsalicylic acid is therefore currently prescribed in most cases because it reduces platelet aggregation.
Prospect and prognosis
Because Degos syndrome is a very rare disorder, no general prognosis can be given in this regard. However, self-healing does not occur, and the syndrome can only be treated symptomatically. A causal cure is not possible. If there is no treatment, the patients suffer from a reddened skin or also spots on the skin. Papules or pustules can also form on the skin, which can significantly limit the aesthetics. Similarly, Degos syndrome increases the likelihood of stroke or thrombosis, making sufferers dependent on regular checkups. If left untreated, it can therefore also lead to a reduced life expectancy. The syndrome can also affect the kidneys or other internal organs, leading to the development of tumors in these organs. The syndrome is treated by taking medications that can alleviate the symptoms. 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
Degos syndrome has hardly been researched by scientific studies, especially the exact causes are not yet known. Therefore, targeted prevention of Degos syndrome is also currently not possible. To date, only symptomatic therapeutic approaches exist after a successful diagnosis, but these have had only limited success.
Follow-up
Because Degos syndrome is a hereditary disease, there is no complete cure. Therefore, as a rule, no special measures of aftercare are available to the affected person, so that the patient is primarily dependent on rapid and, above all, early detection of the disease. An independent cure cannot occur with Degos syndrome. Therefore, the main focus of this disease is early detection so that further complications can be avoided. The symptoms of this syndrome can be alleviated by various medications. The affected person should always ensure that the medication is taken regularly and in the correct dosage so that the symptoms can be properly alleviated. In many cases, a varied diet can also limit the symptoms and increase the patient’s quality of life. Furthermore, regular examinations by a general practitioner are also very useful in Degos syndrome. 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 also to a balanced diet. Whether there is a reduced life expectancy due to the disease cannot be predicted in general.
Here’s what you can do yourself
Degos syndrome represents a serious condition that, to date, has no causal treatment. Self-help measures focus on taking medications as prescribed by the physician and informing the physician of any side effects or interactions so that optimal medication can be achieved. The physician will also recommend a healthy lifestyle with a balanced diet and exercise to the patient. Although this will not cure the disease, it will progress much more slowly if the immune system is strengthened. Degos syndrome is usually also a psychological burden for the affected person. The conspicuous skin changes can cause inferiority complexes or even depression, which must be treated at an early stage. The sufferer should consult a therapist if he or she is experiencing psychological problems. If desired, the medical professional can also establish contact with other sufferers. Above all, going to a self-help group helps to accept the disease and its manifestations and to lead a full life despite the complaints. In the case of a malignant manifestation, the sufferer needs support in everyday life. This is where the relatives and friends are needed, who must pay attention to any symptoms and, in case of doubt, should call in the emergency services.
