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Degos Disease: Facts, Causes, Symptoms and Treatment

  • Date: 2010/02/13 (Rev: 2016/10/04) Thomas C. Weiss - Disabled World
  • Synopsis : Degos disease is a very rare form of illness that was first described by Kohlmeier in the year 1941 and documented as a distinct form of illness.

Main Document

Degos disease is a very rare form of illness that was first described by Kohlmeier in the year 1941 and documented as a distinct form of illness by Degos the same year.

Defining Degos Disease:

Degos disease appears to involve a disease that affects a person's blood vessels. The cells in the linings of the walls of the person's medium and small veins and arteries underneath their skin swell when they become inflamed, causing their blood flow to become restricted. In places where this happens spots or, 'lesions,' appear on their skin. The spots are small, red and slightly raised. As these spots or lesions develop, the center becomes white and dry and may itch.

Degos disease may affect blood vessels in additional parts of the person's body. The person's stomach, eyes, and central nervous system may become involved.

Additional names for Degos disease include:

  • Degos syndrome
  • Kahlmeier disease
  • Degos - Kahlmeier disease
  • Kahlmeier - Degos disease
  • Thromboangiitis obliterans
  • Malignant atrophic papulosis
  • Erythrokeratoderma en cocardes

Degos disease is a very rare form of illness that was first described by Kohlmeier in the year 1941 and documented as a distinct form of illness by Degos the same year. Since that time, approximately one-hundred and thirty people have been diagnosed and documented through scientific literature. The symptoms of Degos disease usually begin to appear between the ages of twenty and fifty in persons who are affected. The illness commonly begins sporadically, although on extremely rare occasions the disease has affected more than one member of the same family.

Causes of Degos Disease:

The cause of Degos disease is currently unknown. Among the ideas proposed as causes are genetic factors, auto-immunological process, allergic vasculitis, an infectious agent, and coagulopathiens. None of these things have yet been proven. The suggestion of a genetic predisposition as a cause of the lesions affecting more than one member of the same family may have some viability. Within the families that have been affected by Degos disease it has been suggested that members of the first degree may have an autosomal dominant factor present.

There has also been some degree of evidence through electron microscopy of an infectious cause for Degos disease. Tests of DNA from skin samples of people who have the disease still leave questions unanswered. The main question is whether the obliteration of the person's blood vessels in the skin areas that are affected are the result of a thrombosis in the context of a primary coagulopathy, or whether it has happened due to a primary vasculopathy through the release of tissue-active factors within the range of endothellium defects. At this time, there have been no laboratory findings that are conclusive in regards to the lesions. There are; however, a large number of reports of people with defects in their blood clotting system.

People with the lesions may present anticardiolipin antibodies, as well as Lupus anticoagulans antibodies, which accompany an increased thrombosis tendency or susceptibility. The presence of a lowered fibrinolyse may be a cause of capillary thrombosis in the lesions people with Degos disease experience. Scientists have observed a complete loss of fibrinolytic activity at the center of skin lesions, as well as a reduction of fibrinolytic activity in the person's peripheral blood in people with the disease. Interestingly, some people with Degos disease had an increased level of figrinogen. Reduced levels of plasminogen, as well as an increase in plasminogen-activator inhibitors, has been documented as well in relation to the lesions associated with Degos disease.

Symptoms of Degos Disease:

People with Degos disease experience the characteristic skin lesions, in most cases, before the more systemic effects of the disease. The illness may remain limited to the person's skin for a number of years, although if a systemic development does happen, there is a need for close-knit medical control of the disease . Serious problems, ones that can affect the person's organs, can occur. The person may experience oxygen deficiency in their tissues that is caused by blockages of their capillaries, where all of their organs may be affected.

The most serious aspects of Degos disease involve a progression involving the person's intestine and stomach, as well as their nervous system and brain. People with Degos disease, despite only experiencing the skin lesions associated with the disease for many years, can still develop more systemic aspects of the illness. The systemic manifestation of Degos disease can, in many cases, lead towards very grave complications in only a few months time.

The most frequently serious complications of Degos disease involve inflammation of the person's abdominal linings or, 'Peritonitis,' inflammation of their lung membrane or, 'Pleurisy,' as well as heart inflammation or, 'Pericarditis.' Complications of the person's intestine usually involve their small intestine, with the peritonitis following an intestine tear. The person may or may not experience any symptoms prior to the intestinal tear.

There have also been some neurological complications reported in association with Degos disease. The complications involve consequences of a degeneration of the person's cerebral blood vessels or, 'Cerebral vasculopathy.' Other neurological complications have involved non-bacterial inflammation of the brain or, 'Asceptic Meningitis,' brain inflammation or, 'Enzephalitis,' nerve inflammation or, 'Radiculoneuritis,' and backbone marrow inflammation or, 'Myelitis.' Complications involving the person's eyes are rare, but can involve the person's eyelids, the outer surface of the person's eye, their retina and choroidea, and neurological issues such as double vision or paralysis of their eye muscles.

Diagnosing Degos Disease:

The skin lesions associated with Degos disease commonly present themselves as wedge-shaped, a characteristic feature of the tissue that may not always be present. As the disease progresses, so do the lesions. These characteristic skin changes associated with the lesions permit a physician to make a conclusive diagnosis. The lesions begin as small red moles around the person's trunk and upper extremities. After a few days, the center of the lesions fall in. Older lesions develop a porcelain white center with a red outer ring.

Treatment of Degos Disease:

Medical science has not yet created a proven, effective treatment for Degos disease. Attempts at using immunosuppressive therapies have not proven successful. Treatments with anti-coagulants and the promotion of blood circulation have, in some cases, produced a decrease in the person's skin symptoms and/or a delay in the progression of their illness. Doctors are not in agreement on which medications should be used in the treatment of Degos disease. Many of the people with the disease are taking blood thinners such as Trental or aspirin. Others have been prescribed immunosuppressants such as Ciclosporin.

The fact that the majority of people with Degos disease experience skin lesions as a first symptom of the disease finds them visiting a dermatologist, at which time a diagnosis is made. The skin lesions themselves are not painful and many times do not itch; they commonly do not require treatment. People with Degos disease who experience gastrointestinal or neurological symptoms need to see a doctor in order to detect systemic disease.

The lack of a known and successful medical therapy for Degos disease finds people with the illness attempting various medications to help. Antiplatelet medications such as dipyridamole or aspirin might reduce the number of new lesions in some people who experience only skin involvement. Some people believe that intravenous immunoglobulin could have a place in the treatment of the disease. One of the forms of treatments that has been pursued without success is the use of topical corticosteroids.

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