Degos: Rare disease

Degos disease is a dangerous disease that affects many different organs in the body. The exact cause of the disease is still unknown, but it is a rapidly progressive disease that can cause death.

Degos disease (also known as Degos Kohlmeier's disease, malignant papules) is a disease that affects the structure of blood vessels (mainly small arteries).
The main pathogenic mechanism is the process of inflammation of the inner membrane of blood vessels, causing these structures to proliferate and grow uncontrollably, making the lumen narrow. As a result, the blood vessels will be blocked, causing lack of blood supply to the tissues downstream of the blood vessels, symptoms of the disease only manifest when these tissues die.
Degos disease can be limited to a skin disease or a systemic disease of the whole body, affecting many organs such as the digestive system, nervous system, heart, eyes... and causing rapid death . Most patients with Degos disease die about 2 to 3 years after the onset of symptoms (in the case of systemic disease).

Currently, the main cause of Degos disease is still not exactly determined. Many theories suggest that the main cause is genetic in addition to a number of risk factors as follows:
2.1. Infection with viruses When the body is infected with a virus, the body's cells are disrupted in the process of development, the DNA structures are affected and the DNA replication process is not controlled, leading to an increase in the number of unusual way.
2.2. Autoimmune Condition Autoimmune is a condition in which the body's immune system for some reason perceives the body's tissues as foreign and attacks itself. Antibodies are produced massively and as a result all the surrounding tissues of the organ are affected as well.

3.1. Degos disease in the skin disease The skin lesions of Degos disease are circular patches or papules 1-5mm in size, red or pink in color. Outside of these lesions, there is a very special border around the skin, with a prominent pink color on the surface of the skin. The centers of these papules are concave due to skin atrophy and are porcelain white. This skin disease can appear all over the body, except for the face, palms, and soles of the feet. The amount varies but is usually very large, spreading throughout the body. Also, the papules or patches on the skin of Degos disease are not itchy and look like small lumps.

3.2. Effects of Degos Disease on Other Organs Degos disease is considered a systemic disease, affecting many different organs. In particular, Degos disease mainly affects the digestive system such as the small intestine and various organs.
Gastrointestinal manifestations: Symptoms of Degos disease in the digestive system often follow the skin manifestations with varying duration, sometimes a few weeks or up to several years. However, in some special cases, the digestive system can be affected before the damage to the skin. Signs of Degos disease include:
Abdominal pain of varying degrees. Diarrhea, frequent loose stools. In contrast to diarrhea, the patient may still be constipated. Presence of blood in the stool. Nausea, vomiting and sometimes vomiting blood. As a result, the small intestine is malabsorption of nutrients, so people with Degos disease often feel tired, lose weight or malnourished. The main mechanism of Degos disease on the digestive system is still the blockage of blood vessels supplying the small intestine, so that the small intestine tissues are not supplied with nutrients, they will necrosis, gradually die and can be torn or perforated. Therefore, internal substances such as food and digestive juices leak out of the abdominal wall, causing peritonitis and the patient can die if not treated surgically in time. Central nervous system manifestations: Besides the digestive system, the brain and spinal cord are the next organs of the nervous system affected by Degos disease. Depending on the location of the injury, the patient has different symptoms such as:
Headache Convulsions Paralysis of the cranial nerves such as facial paralysis, paralysis of the taste and smell nerves... Weakness in half human or quadriplegic in the setting of stroke due to cerebral vascular occlusion. Perceptual disturbances such as lethargy, lethargy and even coma. Lost memory.

Manifestations of Degos disease in the heart: The heart is an organ that can be affected by Degos disease. Possible sites of injury are the pericardium (pericarditis) and permanent scar tissue formation. Other common symptoms of cardiovascular disease include:
Fatigue, lethargy. Shortness of breath. Severe chest pain. Manifestations of Degos disease in the eyes: The eyes can be affected with the following symptoms:
Diplopia – diplopia is when the patient looks at an object in two. Cataract . Eyelid droop. Papilledema due to optic neuritis.

Because the cause of Degos disease has not been determined yet, the main treatment is symptomatic treatment depending on the affected organ, supporting the patient's condition.
Cases of peritonitis due to small bowel perforation are treated mainly by surgical intervention, in addition to accompanying treatments such as antibiotics, anti-inflammatory, nutrition and physical enhancement
In addition, due to the main mechanism Degos disease is a blood vessel blockage, so some drugs with anticoagulant effect, preventing the formation of blood clots are used to treat such as aspirin, clopidogrel.
Periodic health check-ups help to detect diseases early, so that there are treatment plans for optimal results. Currently, Vinmec International General Hospital has general health checkup packages suitable for each age, gender and individual needs of customers with a reasonable price policy.
The patient's examination results will be returned to the home. After receiving the results of the general health examination, if you detect diseases that require intensive examination and treatment, you can use services from other specialties at the Hospital with quality treatment and services. outstanding customer service.