Malignant atrophic papulosis
Malignant atrophic papulosis, also known as Degos disease, is a rare disorder. It generally occurs in young Caucasian adults.
Malignant atrophic papulosis affects the lining of the small blood vessels resulting in occlusion (blockage). The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system.
What is the cause of malignant atrophic papulosis?
The underlying cause of the occlusion of the blood vessels in malignant atrophic papulosis is unknown. Three possible mechanisms are:
- Viral infection
- Disturbance in immunity
- Abnormality in the clotting system of blood
What are the clinical features of malignant atrophic papulosis?
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 start as small red raised spots of 2-5mm in diameter. After a few days they enlarge and develop a central white spot that is depressed in comparison to the red skin around it. They heal leaving depressed porcelain-white scars.
Gastrointestinal complications of malignant atrophic papulosis result from lack of blood supply to the lining of the gut and may lead to serious complications like perforation of the bowel. The usual symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing blood with the bowel motion).
Involvement of the nervous system in malignant atrophic papulosis can lead to strokes, headaches, epilepsy or non-specific neurological symptoms like memory loss or altered sensation.
Other organs can also be involved such as eyes, kidneys, heart and liver.
What is the prognosis for malignant atrophic papulosis?
Malignant atrophic papulosis is generally regarded as serious because it leads to involvement of multiple organs resulting in death within 2-3 years. But in some cases it remains limited to skin involvement and appears benign (harmless).
What is the treatment of malignant atrophic papulosis?
Anti-platelet drugs like aspirin and dipyridamole may reduce the number of new lesions in some patients with only skin involvement.
There have been reports of benefit from experimental treatments using eculizumab (monoclonal antibody binding C5 complement) and treprostinil (prostacyclin analogue).
- Malignant Atrophic Papulosis Is Challenging to Diagnose, Treat – The Rhematologist, July 2014