Description, Causes and Risk Factors:
Alternative Names: Degos syndrome, malignant atrophic papulosis, Kohlmeier-Degos syndrome.
It is a rare, often fatal, multisystem disorder in which pathognomonic skin lesions are frequently associated with infarctive lesions of other viscera, particularly the gastrointestinal tract. Some patients have only cutaneous lesions and a relatively benign course.
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 coagulopathies. 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 endothelium 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 anticoagulants 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 fibrinogen, 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.
Degos disease is thought to be more common in middle-aged people. It is rare in children. Multiorgan involvement appears to be more common in men, while the mild (more benign) form may be more common in females.
Typical cutaneous lesions are 0.3 to 1.0 papules. They have an atrophic, porcelain-white center and an erythematous, telangiectatic border. These lesions are usually asymptomatic, and appear in crops. Many organs may be affected, gastrointestinal and the central nervous system is involved by thrombosis and infarctions.
The lesions occur predominantly on the trunk and upper limbs. The palms, soles and face are usually spared and the scalp is not affected. Similar lesions in the gut give rise initially to abdominal cramps, vomiting and enteritis. These symptoms usually manifest themselves from 3 weeks to 3 years after the appearance of skin papules, but occasionally precede these. Sooner or later they are replaced by an acute, terminal intestinal crisis due to Hematemesis or perforation leading to peritonitis as seen in our case. Mucous membrane involvement is very rare and when it is seen it is usually ocular, in the form of avascular patches over the bulbar conjunctiva surrounded by collateral vessels as evident in our case. Sclera, episclera, retina, choroid and optic nerve may be involved very rarely. When the nervous system is involved the cerebral infarctions manifest as headache, transient neurological defects, multi-infarct dementia and epilepsy. The pericardium, myocardium, pleura, lungs, liver, pancreas and genitourinary tract also may be involved.
Skin biopsy is helpful for diagnosis. Histological studies show a wedge-shaped area of cutaneous ischemia, arising from epidermis and reaching hypodermis, with sclerosis and necrosis. Thrombotic vessels can be seen, without vasculitis, but with a thickened wall and sometimes fibrinoid necrosis. Prognosis is poor; intestinal perforations and peritonitis are the leading causes of death.
The characteristic features of dagos' disease can rarely be confused. A similar cutaneous - intestinal syndrome (having combination of macular, blistering and crusting lesions of the skin with oropharyngeal ulceration and death from perforation of many intestinal ulcers) does not show the characteristic histology. Identical lesions have been described in cases of rheumatoid arthritis, lupus erythematosus and scleroderma and one Crohn's 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 anticoagulants 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.
Aspirin, dipyridamole, prostaglandin E1 and fibrinolytic therapy have also been advocated.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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