Author: Dr Darshan Singh MBChB, Registrar, Department of Dermatology, Waikato Hospital, Hamilton, New Zealand, 2001. Updated by A/Prof Amanda Oakley, January 2016.
Malignant atrophic papulosis is a form of occlusive small vessel vasculopathy. The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system.
Malignant atrophic papulosis is also known as Degos disease.
Malignant atrophic papulosis is a very rare disorder. It generally occurs in young Caucasian adults but has been reported in other races and at all ages.
The underlying cause of the occlusion of the blood vessels in malignant atrophic papulosis is at present unknown. It is likely due to a genetic mutation of complement or some other clotting factor. Several other theories have been suggested.
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 2–5 mm 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 in 50% of patients with systemic disease result from lack of blood supply to the lining of the gut. They may lead to serious complications including 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).
Occlusion of blood vessels in the brain in malignant atrophic papulosis in 20% of patients can lead to strokes, headaches, epilepsy or non-specific neurological symptoms, such as memory loss or altered sensation.
Other organs sometimes affected by malignant atrophic papulosis include eyes, kidneys, heart and liver.
Malignant atrophic papulosis is diagnosed clinically and by skin biopsy revealing 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.
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 generally regarded as a serious vascular disease, because it leads to involvement of multiple organs and results in death within 2–3 years. But in some cases, disease is limited to the skin and appears fairly benign (harmless).
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