Malignant atrophic papulosis

What is it evil one atrophic papulosis?

Malignant atrophic papulosis is a form of occlusive small boat vasculopathy. the blood vessels Affected include those that supply the skin, gastrointestinal tract, and central nervous system.

Malignant atrophic papulosis is also known as Degos disease.

Who gets malignant atrophic papulosis?

Malignant atrophic papulosis is a very rare disorder. It usually occurs in young white adults, but has been reported in other races and in all ages.

What causes malignant atrophic papulosis?

The underlying cause of occlusion of blood vessels in malignant atrophic papulosis is currently unknown. It is likely due to a genetic mutation of complement or some other clotting factor. Several other theories have been suggested.

What are the clinical characteristics of malignant atrophic papulosis?

Skin

Skin lesions are the characteristic feature of malignant atrophic papulosis. The lesions are usually multiple and occur predominantly on the trunk and arms. They usually begin as small raised red spots 2–5 mm in diameter. After a few days, they enlarge and develop a central white spot that is depressed compared to the red skin surrounding it. They heal leaving depressed porcelain white scars.

Gastrointestinal system

Gastrointestinal complications of malignant atrophic papulosis in 50% patients with systemic disease as a result of lack of blood supply to the lining of the intestine. They can lead to serious complications including drilling of the intestine Common symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing a bowel movement).

Nervous system

Occlusion of blood vessels in the brain in malignant atrophic papulosis in 20% patients can cause strokes, headaches, epilepsy, or nonspecific. neurological symptoms, such as memory loss or altered sensation.

Other organs

Other organs sometimes affected by malignant atrophic papulosis include eyes, kidneys, heart and liver.

How is malignant atrophic papulosis diagnosed?

Malignant atrophic papulosis is diagnosed clinically and by the skin. biopsy Telltale occlusion of small blood vessels.

There are no specific blood tests, and most results are normal. Further investigations including imaging will depend on systemic symptoms and signs.

What is the treatment for malignant atrophic papulosis?

Anti-platelet Medications such as aspirin and dipyridamole can reduce the number of new lesions in some patients with only skin involvement.

There have been reports of benefits from experimental treatments with eculizumab (monoclonal antibody complement C5 binding) and treprostinil (prostacyclin analog term)[1]

What is the prognosis for malignant atrophic papulosis?

Malignant atrophic papulosis is generally considered a serious disease. vascular disease because it leads to the involvement of multiple organs and causes death within 2 to 3 years. But in some cases, the disease is limited to the skin and appears quite benign (harmless).