About malignant atrophic papulosis

What is malignant atrophic papulosis?

Degos disease is an extremely rare disorder in which small and medium sized arteries become blocked (occlusive arteriopathy), restricting the flow of blood to affected areas. Degos disease usually causes characteristic skin lesions that may last for a period of time ranging from weeks to years. In some individuals, Degos disease will be limited to the skin (benign cutaneous Degos disease); other individuals will also develop symptoms affecting other organ systems (systemic Degos disease). Systemic Degos disease is most frequently characterized by lesions in the small intestine, but other organs are also affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. The systemic form of Degos disease can cause life-threatening complications such as perforated bowels leading to inflammation of the abdominal cavity (peritonitis). The exact cause of Degos disease is unknown.

Many researchers caution that statistics concerning Degos disease are inaccurate because many individuals go undiagnosed and most medical reports disproportionately discuss the more serious systemic form. It is important to note that some individuals only develop skin lesions (which are not associated with life-threatening complications) and do not go on to develop systemic Degos disease. Affected individuals should talk to their physicians and medical team about their specific case and associated symptoms.

What are the symptoms for malignant atrophic papulosis?

Diarrhea symptom was found in the malignant atrophic papulosis condition

It is characterized by blockages of small to medium sized blood vessels.  This slows or stops the flow of blood through the affected vessels. Severity of symptoms depends on the extent and location of the affected blood vessels. Some individuals with Degos disease have isolated skin involvement and develop porcelain-white macules on their skin. Other individuals have more wide spread disease. 

What are the causes for malignant atrophic papulosis?

The cause of this condition is currently unknown.

What are the treatments for malignant atrophic papulosis?

Malignant atrophic papulosis is an uncommon vasculopathy that affects the skin, gastrointestinal tract, and nervous system. The systemic variant has a rapid clinical course and a high death rate.
Treatment

  • There is no effective treatment for atrophic papulosis. Anticoagulants and blood perfusion drugs such as acetylsalicylic acid, pentoxifylline, dipyridamole, ticlopidine, and heparin have achieved partial regression of skin lesions in isolated cases.
  • As a result, they are a viable first-line therapy option in newly diagnosed patients.
  • Eculizumab, which reduces C5b-9 membrane attack complex deposition, alleviated initial cutaneous and intestinal lesions but did not prevent systemic illness development or progression.
  • A case of eculizumab-resistant malignant atrophic papulosis with intestinal and brain symptoms was successfully treated with subcutaneous treprostinil.
  • Fibrinolytic and immunosuppressive therapies, such as cyclosporine A, azathioprine, cyclophosphamide, or corticosteroids, have proved ineffective.
  • Because of the disease's severe and potentially fatal version, annual follow-up is required.
  • A few even propose quarterly follow-up for the initial seven years of sickness and then annually after that.
  • To measure long-term prognosis, skin inspection, brain magnetic resonance tomography, gastroscopy, colonoscopy, chest x-ray, and abdominal ultrasound should be performed.


Symptoms
Skin lesions,Abdominal pain,Gastrointestinal bleeding,Strokes,Headaches,Memory loss or altered sensation
Conditions
Perforation of the bowel,Epilepsy
Drugs
Acetylsalicylic acid,Pentoxifylline,Dipyridamole,Ticlopidine,Heparin

What are the risk factors for malignant atrophic papulosis?

Malignant atrophic papulosis is an uncommon vasculopathy that affects the skin, gastrointestinal tract, and nervous system. The systemic variant has a rapid clinical course and a high death rate. Occlusive small vessel vasculopathy is a kind of malignant atrophic papulosis. Blood arteries supplying the epidermis, gastrointestinal tract, and central nervous system are among those impacted. Degos disease is another name for malignant atrophic papulosis.

  • Malignant atrophic papulosis is an extremely rare condition.
  • However, it is found to be most common in young Caucasian individuals, but it has been documented in people of many ethnicities and ages.
  • In the medical literature, around 200 cases of Degos illness have been described. The disorder's exact prevalence is unknown.
  • Many experts believe that Degos illness is underdiagnosed, making determining its true prevalence in the general population challenging.
  • Degos disease can afflict people of any age, but it is more frequent in teenagers and young adults.
  • Males have been impacted more frequently than females in the recorded cases.
  • However, one study discovered that women were more likely than males to have the benign cutaneous variant of Degos disease.


Symptoms
Skin lesions,Abdominal pain,Gastrointestinal bleeding,Strokes,Headaches,Memory loss or altered sensation
Conditions
Perforation of the bowel,Epilepsy
Drugs
Acetylsalicylic acid,Pentoxifylline,Dipyridamole,Ticlopidine,Heparin

Is there a cure/medications for malignant atrophic papulosis?

No specific therapy has been identified for Malignant atrophic papulosis or Degos disease.

  • Treatment is directed toward the specific symptoms that are apparent in each individual.
  • In diagnosed cases of Degos disease, regular examination of the gastrointestinal tract may detect intestinal perforation before symptoms of acute complications appear.
  • Some associated complications, such as gastrointestinal bleeding or intestinal perforation, may require surgical intervention.
  • In some patients with only skin involvement, antiplatelet medications such as aspirin and dipyridamole may minimize the number of new lesions.
  • Experiment therapies with eculizumab (monoclonal antibody binding C5 complement) and treprostinil have shown promise (prostacyclin analog).


Symptoms
Skin lesions,Abdominal pain,Gastrointestinal bleeding,Strokes,Headaches,Memory loss or altered sensation
Conditions
Perforation of the bowel,Epilepsy
Drugs
Acetylsalicylic acid,Pentoxifylline,Dipyridamole,Ticlopidine,Heparin

Video related to malignant atrophic papulosis