What is Malignant Atrophic Papulosis or Degos disease?
Malignant Atrophic Papulosis which is also known by the name of Degos Disease is an extremely rare pathological condition characterized by blockage of medium and small sized arteries, thus reducing the blood flow to the organs that are supplied blood and nutrients by those arteries.
The primary characteristic feature of Malignant Atrophic Papulosis or Degos Disease is the presence of skin lesions, which do not go away and may persist for years together. In some cases, the symptoms of Malignant Atrophic Papulosis or Degos Disease remains limited to the skin whereas in some other cases other organs of the body may also be affected. Apart from the skin lesions, lesions formed due to Malignant Atrophic Papulosis is mostly found in the gastrointestinal tract, which may cause severe abdominal pain, persistent diarrhea and unintentional weight loss.
If prompt treatment is not given then it may lead to potentially life threatening complications like perforated bowel and peritonitis. What exactly causes Malignant Atrophic Papulosis or Degos Disease is not yet known but certain viral infections or an abnormality in the ability of the body to clot blood are believed to be some of the reasons for the development of it.
What Causes Malignant Atrophic Papulosis or Degos Disease?
As stated above, the exact cause for Malignant Atrophic Papulosis or Degos Disease is not yet known. In some cases, there is abnormal multiplication of the lining of the small and medium sized arteries, which result in blockages and reduced blood supply to the organs to which they cater to. Why this multiplication occurs is something which is not yet known, but there are many theories postulated as to be the probable cause of it.
There are mainly three theories which researchers believe may be related to the development of it. These are viral infection, abnormality in the body’s ability to clot blood, and autoimmune disorders where the body’s defense mechanism starts attacking the healthy tissue.
There are some cases where Malignant Atrophic Papulosis or Degos Disease have been found to run in families, but these cases are few and far between, although a genetic link has not been ruled out in the development of it.
What Are The Symptoms of Malignant Atrophic Papulosis or Degos Disease?
In majority of the cases of Malignant Atrophic Papulosis or Degos Disease, the presenting symptom is the presence of skin lesions which appear as a rash. These lesions or rash may be of varying shape normally on the trunk and the upper and lower extremities. Initially, there will be only a few lesions but as the disease progresses the number of lesions multiply abnormally and may even cross 100. Normally, the palms of the hand, the face, and the soles of the feet are not affected by the lesions caused by Malignant Atrophic Papulosis or Degos Disease.
There may be at times itching associated with these lesions. During the initial phase of the condition, the lesions may be pink or red in color but gradually the lesions become reddish in the borders and white at the center. In some cases, apart from the skin other organ systems can also get affected, which can lead to serious complications. The organ involved and the severity of the symptoms are significantly variable.
Gastrointestinal tract is the area that is most affected other than the skin by Malignant Atrophic Papulosis or Degos Disease. The involvement of the gastrointestinal system may start from a few weeks to a few years after the development of the initial lesions from it.
The lesions formed in the gastrointestinal system as a result of Malignant Atrophic Papulosis or Degos Disease may cause an individual to have severe abdominal pain, cramping, nausea, vomiting, diarrhea, bloating, constipation, bloody stools, or hemoptysis. Unintentional weight loss, fatigue, and weakness can also be felt by the affected individual.
As the condition advances and there is more involvement of the GI tract there may be perforation resulting in the contents of the intestine leaking into the abdominal cavity causing a condition called peritonitis.
In some cases, there may be central nervous system involvement as well in individuals with Malignant Atrophic Papulosis or Degos Disease. In such cases, the individual may experience headaches, dizziness, seizures, cranial nerve palsy, hemiparesis, strokes, or cerebral infarction. The individual may also complain of memory loss as a result of CNS involvement in it.
Malignant Atrophic Papulosis or Degos Disease may also involve the heart, kidneys, eyes, and lungs with varying degree of symptoms including diplopia, ptosis, cataracts, and shortness of breath with activity, chest pain, pericarditis, pleuritis, and pleural effusion.
How Is Malignant Atrophic Papulosis Diagnosed?
A thorough clinical evaluation is required for the diagnosis of Malignant Atrophic Papulosis or Degos Disease. The characteristic lesions found on the surface of the skin may point towards a diagnosis of it.
If the patient has a direct family member with a prior history of Malignant Atrophic Papulosis, then that may also point towards a diagnosis of it.
A microscopic analysis of the skin lesions may reveal the typical characteristics seen in this condition and confirm the diagnosis of it. Since this condition is extremely rare and most standard lab tests reveal normal results, there is not one specific test designed for diagnosing it.
How Is Malignant Atrophic Papulosis Treated?
There is no specific treatment so far to treat Malignant Atrophic Papulosis or Degos Disease. The treatment is basically symptomatic and supportive. In cases where the GI tract is involved, then periodic monitoring may be recommended to look for any signs of perforation or other complications which may be potentially serious. If such a complication is identified then surgical procedure may be required to correct such conditions. Other than this, only periodic monitoring is what is done in majority of the cases of Malignant Atrophic Papulosis or Degos Disease.