Degos disease
Encyclopedia
Degos disease is an extremely rare vasculopathy that affects the lining of the medium and small vein
s and arteries, resulting in occlusion
(blockage of the vessel) and tissue infarction
.
The blood vessels affected include those supplying the skin
, gastrointestinal tract
, and central nervous system
. This can result in bowel ischemia (mesenteric ischemia
or ischemic colitis
), chronic skin lesions, ocular lesions, stroke
s, spinal lesions, mononeuritis multiplex, epilepsy
, headache
s or cognitive disorder
s. Pleural
or pericardial effusion
s are also reported.
The outcome of this disease can be fatal with a median survival of 2 to 3 years, although some appear to have a benign form (Degos acanthoma
) which affects only the skin. There are fewer than 50 living patients at present known worldwide, and fewer than 200 reported in the medical literature. Treatment options are limited, consist mainly of Antiplatelet drug
s or anticoagulant
s or immunosuppressant
s, and effect of treatment is limited to case reports.
It has been suggested that this is not a separate disorder, but the final result of several vascular systemic disorders.
The disease is named for Robert Degos who recognised it as a clinical entity in 1942, after it was first described by Kohlmeier in 1941.
Vein
In the circulatory system, veins are blood vessels that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated blood to the heart...
s and arteries, resulting in occlusion
Vascular occlusion
Vascular occlusion is a sudden blockage of a blood vessel, usually with a clot. It differs from thrombosis in that it can be used to describe any form of blockage, not just one formed by a clot. When it occurs in a major vein, it can, in some cases, cause deep vein thrombosis. The condition is...
(blockage of the vessel) and tissue infarction
Infarction
In medicine, infarction refers to tissue death that is caused by a local lack of oxygen due to obstruction of the tissue's blood supply. The resulting lesion is referred to as an infarct.-Causes:...
.
The blood vessels affected include those supplying the skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
, gastrointestinal tract
Gastrointestinal tract
The human gastrointestinal tract refers to the stomach and intestine, and sometimes to all the structures from the mouth to the anus. ....
, and central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...
. This can result in bowel ischemia (mesenteric ischemia
Mesenteric ischemia
Mesenteric ischemia is a medical condition in which inflammation and injury of the small intestine result from inadequate blood supply. Causes of the reduced blood flow can include changes in the systemic circulation or local factors such as constriction of blood vessels or a blood clot...
or ischemic colitis
Ischemic colitis
Ischemic colitis is a medical condition in which inflammation and injury of the large intestine result from inadequate blood supply. Although uncommon in the general population, ischemic colitis occurs with greater frequency in the elderly, and is the most common form of bowel ischemia...
), chronic skin lesions, ocular lesions, stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
s, spinal lesions, mononeuritis multiplex, epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...
, headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
s or cognitive disorder
Cognitive disorder
Most common mental disorders affect cognitive functions, mainly memory processing, perception and problem solving. The most direct cognitive disorders are amnesia, dementia and delirium. Others include anxiety disorders such as phobias, panic disorders, obsessive–compulsive disorder, generalized...
s. Pleural
Pleural effusion
Pleural effusion is excess fluid that accumulates between the two pleural layers, the fluid-filled space that surrounds the lungs. Excessive amounts of such fluid can impair breathing by limiting the expansion of the lungs during ventilation.-Pathophysiology:...
or pericardial effusion
Pericardial effusion
Pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. Because of the limited amount of space in the pericardial cavity, fluid accumulation will lead to an increased intrapericardial pressure and this can negatively affect heart function...
s are also reported.
The outcome of this disease can be fatal with a median survival of 2 to 3 years, although some appear to have a benign form (Degos acanthoma
Acanthoma
An acanthoma is a skin neoplasm composed of squamous or epidermal cells. It is located in the prickle cell layer.Types of acanthoma include pilar sheath acanthoma, a benign follicular tumor usually of the upper lip; clear cell acanthoma, a benign tumor found most frequently on the legs; and Degos...
) which affects only the skin. There are fewer than 50 living patients at present known worldwide, and fewer than 200 reported in the medical literature. Treatment options are limited, consist mainly of Antiplatelet drug
Antiplatelet drug
An antiplatelet drug is a member of a class of pharmaceuticals that decrease platelet aggregation and inhibit thrombus formation...
s or anticoagulant
Anticoagulant
An anticoagulant is a substance that prevents coagulation of blood. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombotic disorders. Some anticoagulants are used in medical equipment, such as test tubes, blood transfusion bags, and renal dialysis...
s or immunosuppressant
Immunosuppressant
An immunosuppressant is any substance that performs immunosuppression of the immune system. They may be either exogenous, as immunosuppressive drugs, or endogenous, as ,e. g., testosterone...
s, and effect of treatment is limited to case reports.
It has been suggested that this is not a separate disorder, but the final result of several vascular systemic disorders.
The disease is named for Robert Degos who recognised it as a clinical entity in 1942, after it was first described by Kohlmeier in 1941.