Degos disease

Proven content

Degos’ disease – atrophic malignant papularity, systemic inflammatory vascular disease with malignant course, or Köhlmeier-Degos disease, are the most commonly used terms for a very rare disease with unknown causes. The Latin name of the disease is Papulosis atrophicans maligna. Only 130 cases of this disease have been described in the medical literature. It was first described by Michael Köhlmeier and Robert Degos in 1941.

Symptoms of Degos’ disease

The first symptom of Degos’ disease is characteristic skin lesions. They are small, speckled, red moles that appear mainly on the trunk and upper limbs. Skin lesions rarely occur on the hands, soles of the feet, face and scalp. After a few days, the center of the mole collapses. It turns a porcelain-white color, so that older blooms have a white center and a reddish, erythematous coating (like a wedding ring) around it.

Degos’ disease appears to be a disease of blood vessels, a type of vascular thrombosis, because cells in the epithelium of the walls of medium and small veins and the arteries under the skin are inflamed. Simplifying the problem, it can be said that they swell, which restricts blood flow. When this happens, the center of the birthmark dies and the skin above this area becomes dry and white.

The causes of Degos’ disease

The causes of Degos’ disease are as yet unknown. Scientists put forward new hypotheses, but none of them has been sufficiently documented by research. Most often it is talked about hereditary, that is the genetic origin of the diseasebecause the condition has been registered in the family. Other scholars have suggested that it does allergic background. Another, that it is favored by viral or bacterial infections, because paramyxoviruses were isolated in the tested skin samples. Hypotheses concerning blood coagulation disorders also remain unexplained. It can be said that scientists still do not know which comes first. Is the obstruction (obstruction) of blood vessels in the affected areas of the skin due to phlebitis (thrombosis) associated with primary blood clotting disorders (coagulopathy), or is it a consequence of primary pathological changes in the blood vessels (vasculopathy).

The onset of Degos’ disease

According to the observations made so far, the first symptoms of Degos’ disease appear between the ages of 20 and 50, usually spontaneously. We already said in the introduction that the disease is systemic, i.e. it can attack individual systems of our body. Simply put, it’s not just a skin disease.

Skin eruptions appear in most cases before systemic symptoms appear. In each case, it has a different, individual course. It can stop at the stage of birthmarks and remain in this form for many years. The moles are not painful, usually not itchy, and do not require specialist treatment. However, when systemic changes occur, it requires intensive medical supervision because there is a high risk of serious complications.

As we already know, the cause of systemic problems may be ischemia of individual organs, which is a consequence of capillary obstruction. This ischemia often affects the stomach and intestines and, secondly, the central nervous system, mainly the brain. These are dangers that, without medical support, may lead to the patient’s death in a short time, or even a few months.

Degos’ disease – complications

Scientists systematized the complications of Degos’ disease. The most serious ones include:

1. gastritis,
2. pleurisy,
3. myocarditis.

A serious complication of Degos’ disease is ischemia of the small intestine, as it may be perforated and thus lead to peritonitis. It should be added that the symptoms of perforation may not occur at all or appear as nonspecific abdominal pain or digestive problems. Neurological complications also have a bad prognosis. Degos’ disease may develop non-bacterial lymphocytic encephalitis, radiculitis, or osteomyelitis. It also happens that the disease affects the eyes, resulting in double vision or paralysis of the eye muscles.

Treatment of Degos’ disease

So far, no form of treatment has been shown to be quite effective against Degos’ disease. Besides, doctors do not agree which groups of drugs are more or less effective in the fight against the disease.

It is now known that immunosuppressive therapy with e.g. cyclosporin A, azathioprine, cyclophosphamide and steroids does not bring good results. Treatment of Degos’ disease, which is supposed to stop blood clotting and improve circulation, only relieves skin symptoms in some patients and stops the development of generalized symptoms. In the treatment of the disease, acetylsalicylic acid is of particular importance. But many other medications are also used to treat the condition. Many drugs have been tested in the treatment of Degos’ disease. Most turned out to be of little or no effect. One thing is for sure. In the event of gastrointestinal bleeding, intestinal perforation, intestinal infarction, or intracranial bleeding, surgical intervention is necessary, because only in this way can the patient be saved.

It is not known so far whether there is a mild or severe form of the disease. In some people with Degos’ disease, the only symptom of the disease is characteristic skin lesions. Some have only a few, others have thousands. These people lead a normal life, and the birthmarks do not bother them at all. It also happens that Degos’ disease takes an acute course and can sometimes be life-threatening. However, it is not known what contributes to this and not another course of the disease. As we mentioned, only 130 cases of the disease have been described so far, so it is difficult to generalize from it.

Tekst: Anna Jarosz