Degos Disease: Facts, Causes, Symptoms, Treatment

Topic: Types of Disability
Author: Thomas C. Weiss
Published: 2010/02/13 - Updated: 2023/01/28
Contents: Summary - Definition - Introduction - Main Item - Related Topics

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. The symptoms of Degos disease usually begin to appear between the ages of twenty and fifty in persons affected. The illness commonly begins sporadically, although, on infrequent occasions, the disease has affected more than one member of the same family. The skin lesions associated with Degos disease commonly present as wedge-shaped, a characteristic tissue feature that may not always be present.

Introduction

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

The U.S. Social Security Administration (SSA) has included Degos Disease as a Compassionate Allowance to expedite a disability claim.

Main Item

Degos disease is a very rare form of illness that was first described by Kohlmeier in 1941 and documented as a distinct form of illness by Degos the same year. Since then, 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 affected. The illness commonly begins sporadically, although, on infrequent occasions, the disease has affected more than one member of the same family.

Causes

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 affected by Degos disease, it has been suggested that members of the first degree may have an autosomal dominant factor.

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 with the disease still leave questions unanswered. The main question is whether the obliteration of the person's blood vessels in the affected skin areas results from 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 regarding 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 and Lupus anticoagulans antibodies, which accompany an increased thrombosis tendency or susceptibility. The presence of a lowered fibrinolysis 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 and 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 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 several years, although if a systemic development does happen, there is a need for tight 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 caused by blockages of their capillaries, where all 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 to grave complications in only a few months.

The most frequent 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 before the intestinal tear.

Some neurological complications have also been reported associated with Degos disease. The complications involve consequences of degeneration of the person's cerebral blood vessels or 'Cerebral vasculopathy.' Other neurological complications have involved non-bacterial inflammation of the brain, 'Asceptic Meningitis,' brain inflammation or 'Enzephalitis,' nerve inflammation or 'Radiculoneuritis,' and backbone marrow inflammation or 'Myelitis.' Complications involving the person's eyes are rare. Still, they 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

The skin lesions associated with Degos disease commonly present as wedge-shaped, a characteristic tissue feature 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 falls 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 a delay in the progression of their illness. Doctors disagree on which medications should be used to treat Degos disease. Many people with the disease take blood thinners such as Trental or aspirin. Others have been prescribed immunosuppressants such as Ciclosporin.

The fact that most people with Degos disease experience skin lesions as a first symptom of the disease finds them visiting a dermatologist when a diagnosis is made. The skin lesions are not painful and often do not itch; they commonly do not require treatment. People with Degos disease who experience gastrointestinal or neurological symptoms need to see a doctor 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 treatment that has been pursued without success is the use of topical corticosteroids.

Author Credentials:

Thomas C. Weiss is a researcher and editor for Disabled World. Thomas attended college and university courses earning a Masters, Bachelors and two Associate degrees, as well as pursing Disability Studies. As a Nursing Assistant Thomas has assisted people from a variety of racial, religious, gender, class, and age groups by providing care for people with all forms of disabilities from Multiple Sclerosis to Parkinson's; para and quadriplegia to Spina Bifida. Explore for comprehensive insights into his background, expertise, and accomplishments.

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Cite This Page (APA): Weiss, T. C. (2010, February 13 - Last revised: 2023, January 28). Degos Disease: Facts, Causes, Symptoms, Treatment. Disabled World. Retrieved October 13, 2024 from www.disabled-world.com/disability/types/degos-disease.php

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