What is Degos Disease?
Degos Disease is n’t a disease that you ’ve believably heard of unless you have studied it or are a frequent viewer of aesculapian dramas . It ’s a very rarified stipulation that almost never occurs- the humanity has seen about 200 case in their records so far . Degos Disease is also recognize as Malignant Atrophic Papulosis . It is describe otherwise after the Dr. who give the disease a clinical realization in 1942 , Dr. Robert Degos . Degos disease is a inherited status that affects the line of descent clot system in the trunk by ruining the blood line clotting agent . Other names by which this disease has been touch to are- Degos Kohlmeier Disease , Papulosis Attophicans Malinga .
What Happens in Degos Disease?
Degos disease causes endovasculitis – which means inflammation of the interior walls of the blood vessels . This , in turn , lead to occlusions in the smooth blood menstruum . Degos disease has been studied to mainly affect arteries that are broadly smaller and not of high-pitched capacity . It is a highly reformist disease that head to trouble such as skin cell death ( infarction ) and of other tissues later on . This happens because when blood catamenia is skip off to a region , the tissue in the area usually die due to lack of nutrition- a prime representative of this beingmyocardial infarction- heart attack .
blockage happens because the cells that line the interior of the blood vessels procreate and shape physical barriers in this status that create the vessel really narrow-minded . Depending on the natural event , Degos disease can either be limited to just the skin and remain a benignant ( not fateful ) condition or a hard disease that can affect most system in the body and most organs- almost sure enough result in the death of the moved patient . Most of the patients affected by Degos disease have significantly reduced life spans and do not survive beyond two to three year following the onset of the symptoms in character of the fateful variant .
What Causes Degos Disease?
While we can by and large point out that the chief cause of Degos disease as a genetic experimental condition , study have shown that there are other case for this disease but what all of them are , is n’t well known yet . However , a few sensible theory that are still being researched upon have been put onwards :
Viral Infection:
The viral infections act by taking over the mastery of the host cellular telephone and for increase their numeral , they modify the desoxyribonucleic acid and make them multiply in large numbers and very quickly .
Auto-immune Conditions:
In autoimmune condition , the body due to some ground assume that it ’s own tissues are either foreign or unhealthy and thus proceed to assail these tissue . When antibodies assail a tissue paper , they ’re produced in larger and larger numbers as they continue to assume every tissue paper is infect .
Genetic Conditions:
genetical conditions generally causes the problems in coagulation – affects the clotting ability of the blood which mean it ’s punishing to stop bleed in such patient .
Genetic factors may also point that the disease could be passed by inheritance but there have n’t been many recorded causa . The factor that ’s inherited is n’t hump . However , it ’s see that only benign forms of Degos Disease are transmitted this path .
Who is at Risk for Degos Disease?
Some studies have shown that Degos Disease strike man more than women , however , the trouble with describe the precise incidence of Degos Disease is that this disease is highly under - diagnosed which could be due to many reasons . Fewer number of cases are fatal than the benign unity and since the rashes are confused with that of other diseases and forms of vasculitis , Degos disease is rarely diagnosed .
From the limited data available it ’s observe that while there are opportunity that multitude of any historic period can be affected , the incidence is more in the untried adults .
Signs and Symptoms of Degos Disease
The effects of Degos Disease depend on the special small and medium - sized artery that have been regard by the condition .
Symptoms of Benign Occurrence of Degos Disease
On the skin- big sized spots know as papule that are either red or different specter of pink appear on the skin . The papules are see to have particular types of brim . In sometimes these smirch heal themselves and leave behind a very characteristic scar- that have a central and very clean atrophic sphere that ’s distinguishable from most other cicatrix .
These lesions appear all over the body except the face , palms of the hand and soles of the foot . Around 40 spot can be key out on average but some cases may present with over a hundred of them . These papules may or may not be itchy and they look like little bumps .
Symptoms of Malignant Occurrence of Degos Disease
It is also known as Systemic Degos . The malignant conditions primarily affect the small intestine and other parts of the GI system . The effects on the Gastrointestinal organization can occur at any prison term follow the skin manifestations- either a few weeks later or year .
However , in very rare cases of Degos Disease , the symptoms of the gastrointestinal system may fall out before the lesion strain on the skin .
People have and observe the following symptoms when the wound is in the small intestine-
The principal function of the little intestine is to absorb all the nutrient that are essential for the physical structure from the digested solid food . Lesions in the small bowel hence lead to something bonk as malabsorption- hence the mortal suffering from Degos Disease experience symptoms such as fatigue , weight unit exit and other identifiable symptoms of malnutrition .
Now in severe display case or with the progression of the Degos disease , the lesions that are present can begin to snap or tear apart . In doing so , the digested food and other contents that are present in the intestine leak out and infix the abdominal dental caries by form pickle in the small intestine ( bang as perforation ) . This is a very fatal and life - threatening situation . The linings ( membranes ) of the abdominal dental caries get fire up during the process and this conduct to a condition known as peritoneal inflammation that requires straightaway intervention .
Next , to the gastrointestinal system , the most commonly affected neighborhood is the Central Nervous System which consists of the Brain and the Spinal Cord . count on the precise locations of these organs that are involve the symptoms may vary but the following are most commonly observed :
Cranial Nerves paralyzes that can cause problems with vision , smell , earreach , taste and even movements . Bells Palsy is an lesson of a cranial face paralysis . There are twelve cranial nerve that emerge like a shot from the brain and supply the major sense organs as well as structures include the diaphragm . Each has a sensory and motor component that when affected do a mixed bag of symptom .
The Heart is another organ that can be affected by Systemic Degos Disease . The wound may come in the natural covering of the heart and induce redness which is know as pericarditis . When this happens , it may be followed by thickening of the pericardium as a healing response which is commonly lasting and head to the formation of scar tissue . The symptom noted by the patient are-
The Eyes may be affected and the following symptoms can be observed-
Lung tissue paper involvement is qualify by inflammation of the coating of the lung tissue- pleuritis ( the membranes are called pleura ) . There might also be accumulated fluid present in the lungs known as pleural effusion .
Differential Diagnosis of Degos Disease
A differential diagnosing gives insight into what another disease it could be that may have one or more of the same symptom and resemble the disease externally to a certain extent . The deviation , however , is that skin lesions are present in the facial neighborhood as well .
Diagnosis of Degos Disease
There is no corroborative laboratory diagnosis of Degos disease . Thereby clinical interrogation along with the riddance of other similar diseases such as the ones mentioned above are the only available methods by which a diagnosis of Degos can be made .
Treatment of Degos Disease
Preventing Degos Disease
Currently given the unknown causal agent of the disease , noesis about the prevention of the disease is n’t yet reach .
