About degos disease

What is degos disease?

Degos disease is a very rare disease in which the arteries, which are small and medium-sized, get blocked restricting the proper flow of blood to the affected areas. Also, you may experience skin lesions, and these skin lesions can last from weeks to years depending on the amount of damage. Most people develop lesions on the skin only, but in some severe cases, damage may be caused to the intestines as well. Apart from lesions on the intestines, other organs might be affected as well. If you develop systemic Degos disease i.e. severe Degos disease, the exact cause of which is unknown, it might be life-threatening as well because of complications like perforated bowels and inflammation of the abdominal cavity. If you want to know the statistics of the disease, then you should know that the exact statistics are not possible because in most cases the disease will go undetected, and the statistics pertain to only the serious form of the disease, i.e. the systemic Degos disease only. If you develop skin lesions, you should consult your doctor immediately.The disease was first identified in 1941, and only 200 cases of the disease have been reported in the medical literature. Most researchers believe that the disease goes undetected in most cases, and the actual number of cases will be significantly higher than the reported cases. Although the disease can affect people of all ages, it is found to be more common in young adults. Also, males have been found to be more susceptible to the disease. 

What are the symptoms for degos disease?

Degos disease is a very rare disease which causes lesions on the skin. Most cases go undetected, and it is only the systemic Degos disease which causes serious symptoms which might be life-threatening as well. The exact symptoms are given below:- ‚Äč

1) The most common initial symptoms are Skin lesions or rashes. You may develop bumps of different shapes and sizes. Initially, you have very few lesions, but with time it will reach around 40, or in some cases even hundreds. You may develop lesions on the trunk or upper arms or upper thighs. 

2) In some cases, the blood vessels of other areas may get involved, which may cause you to develop complications in other areas as well. The organs affected, and the severity may vary from case to case. The most common area affected is the gastrointestinal tract. You may develop lesions in the small intestines as well which may cause you to develop abdominal pain, cramps, etc. The inflammation of the abdominal cavity may be life-threatening as well in very rare cases.

3) In some cases of systemic Degos disease, your nervous system might be affected as well leading to headaches, dizziness, seizures, etc. You may also suffer from memory loss or altered sensations in very rare cases. 

What are the causes for degos disease?

Degos disease goes undetected in most cases, and there are only 200 cases of the disease in medical records. Thus, much research has not been carried out on the disease, and the exact causes of the disease are unknown. The disease causes the cells lining the arteries to multiply, resulting in the blocking of arteries. The blood flow gets restricted because of the blocking of arteries, which may result in skin lesions and other complications of the disease. As the exact cause is not well researched, there are three main theories as to how the disease is caused:-

1) The primary theory is that it is caused by a viral infection and is transmissible. 

2) One more theory is that the blood clotting ability of the body might be different from case to case, and this may cause the disease. 

3) The third theory is that the autoimmune system of the body might attack healthy tissues causing the problem. Also, some cases of the Degos disease have been linked to genetics and can be hereditary. Usually, the genetic form of the Degos disease is seen to affect the skin only and is not very severe.

What are the treatments for degos disease?

As Degos disease is a very rare disease, there is no standard treatment procedure for the disease. The doctors usually have the treatment for particular symptoms that may occur. If you have been diagnosed with the Degos disease, you should get a regular examination of the gastrointestinal tract to make sure that there is no inflammation. Some gastrointestinal bleeding may require surgery.In some cases, drugs that stop the activity of platelets have been used to treat the disease. These drugs are referred to as anti-platelet or anti-coagulation drugs. If you do not have systemic Degos disease, you can use these drugs to treat lesions in the skin and the eye. Drugs which are used to suppress the immune system of the body have been used, but in the research, they were found to be not successful. In a particular case in a medical journal, nicotine patches helped in reducing lesions. Some researchers suggest an intravenous injection of immunoglobulin as a treatment. As of now, more research is necessary to streamline the treatment process for the disease. With more data in the future, the treatment is set to be standardized. If you want to read the latest research on the treatment, you can read the various medical journals to understand the effectiveness of the various treatment procedures. 

What are the risk factors for degos disease?

The Degos disease is a very rare disease in which the arteries are blocked causing skin lesions. There are only 200 cases in the medical records. Also, most cases of the disease go undetected, and it is only the severe disease, i.e. systemic Degos disease, which is detected in most cases. The 3 main causes of the disease are:-

1) Viral infection

2) Autoimmune disorders

3) Blood clotting issues in the body

Also, the disease is found to be genetic and can be inherited. Thus, if you have a family history of the disease, you are at risk of the disease. The disease is very rare and the exact risk factors are unknown. If you have the symptoms, you should contact a doctor immediately.

Is there a cure/medications for degos disease?

Degos disease is an extremely rare disease. It is also called Köhlmeier-Degos disease. It is a fatal disease and is life-threatening. In this disease, the small and medium-sized arteries in the body get blocked, resulting in restricted blood flow to affected parts. It is a blood vessel disorder, and its signs and symptoms vary from one individual to another. There is no effective treatment for Degos disease as of now, and the treatment is often targeted towards the particular symptoms in each patient. There is very little knowledge on how this disease actually works. Following are the treatment that have been tried and tested to treat this disease:

There have been attempts to treat it using antithrombotic agents. These were found to be effective in a few individuals.

Immunosuppressive agents like corticosteroids have been tested, but it was found that they worsened the disease. Thus, this treatment plan was unsuccessful.

Fibrinolytic agents, anticoagulants, and prostaglandin E1 have been tried as treatments, but they failed to produce the desired results.Doctors are uncertain regarding which medicine should be used for the treatment.

Intravenous immunoglobulin treatment is another hit and trial method that might help in the treatment of this disease.Infliximab was found to be effective in some cases.

Experimental treatment using eculizumab has benefitted some individuals. But this is very expensive and needs to be infused after every fourteen days.

According to reports, the use of Treprostinil helped in clearing the gastrointestinal tract and CNS. In some patients, Degos disease affects the gastrointestinal tract. Thus, regular monitoring can help detect abnormalities in the intestines. Sometimes surgical procedures may be necessary.

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