What is Malignant Atrophic Papulosis or Degos disease?
Malignant Atrophic Papulosis which is also know by the name of Degos Disease is an extremely rarified pathological stipulation characterized by blockage of medium and small sized arteries , thus lose weight the blood current to the organ that are add blood and food by those artery .
The elementary characteristic feature of Malignant Atrophic Papulosis or Degos Disease is the comportment of skin lesions , which do not go by and may prevail for years together . In some cases , the symptom of Malignant Atrophic Papulosis or Degos Disease remain circumscribed to the cutis whereas in some other cases other harmonium of the consistence may also be affected . aside from the skin lesions , lesions formed due to Malignant Atrophic Papulosis is mostly found in the gastrointestinal tract , which may cause severeabdominal pain , persistentdiarrheaand unwilled weight going .
If prompt discourse is not given then it may go to potentially living jeopardise tortuousness like perforated bowel and peritonitis . What exactly induce Malignant Atrophic Papulosis or Degos Disease is not yet have intercourse but certain viral infections or an irregularity in the ability of the trunk to clabber blood are believed to be some of the reasons for the development of it .
What Causes Malignant Atrophic Papulosis or Degos Disease?
As stated above , the accurate cause for Malignant Atrophic Papulosis or Degos Disease is not yet known . In some cases , there is unnatural propagation of the lining of the small and intermediate sized arterial blood vessel , which result in blockage and thin blood supply to the organs to which they cater to . Why this times take place is something which is not yet known , but there are many theories postulated as to be the probable cause of it .
There are mainly three hypothesis which researchers trust may be interrelate to the growth of it . These are viral infection , abnormality in the physical structure ’s ability to clot blood , and autoimmune disorders where the dead body ’s defense mechanics starts attacking the goodish tissue .
There are some cases where Malignant Atrophic Papulosis or Degos Disease have been found to run in syndicate , but these fount are few and far between , although a genetic liaison has not been ruled out in the development of it .
What Are The Symptoms of Malignant Atrophic Papulosis or Degos Disease?
In majority of the case of Malignant Atrophic Papulosis or Degos Disease , the presenting symptom is the comportment of skin lesions which seem as a roseola . These lesions or rash may be of varying shape normally on the trunk and the upper and low extremities . ab initio , there will be only a few lesions but as the disease come on the telephone number of lesions multiply abnormally and may even cross 100 . Normally , the palms of the hand , the face , and the soles of the feet are not affected by the lesions get by Malignant Atrophic Papulosis or Degos Disease .
There may be at times itching associated with these lesion . During the initial phase of the condition , the lesions may be pinkish or carmine in colour but bit by bit the lesion become reddish in the delimitation and white-hot at the substance . In some cases , apart from the peel other harmonium system can also get feign , which can lead to serious complicatedness . The harmonium involved and the severity of the symptoms are significantly variable .
Gastrointestinal tract is the area that is most affected other than the peel by Malignant Atrophic Papulosis or Degos Disease . The liaison of the gastrointestinal system may take up from a few weeks to a few years after the development of the initial lesions from it .
The lesions formed in the GI scheme as a result of Malignant Atrophic Papulosis or Degos Disease may cause an soul to have severe abdominal nuisance , cramping , sickness , puking , diarrhea , bloat , irregularity , blooming feces , or haemoptysis . unplanned weight loss , weariness , and helplessness can also be felt by the affected individual .
As the condition advances and there is more involvement of the GI piece of ground there may be perforation resulting in the content of the intestine leaking into the abdominal cavity causing a condition called peritonitis .
In some instance , there may be central nervous system amour as well in mortal with Malignant Atrophic Papulosis or Degos Disease . In such cases , the individual may experienceheadaches , dizziness , seizure , cranial nerve palsy , hemiparesis , throw , or cerebral infarct . The individual may also plain ofmemory lossas a resolution of CNS involvement in it .
Malignant Atrophic Papulosis or Degos Disease may also involve the substance , kidney , middle , and lungs with varying degree of symptoms includingdiplopia , ptosis , cataracts , andshortness of breathwith activity , bureau pain , pericarditis , pleuritis , and pleural effusion .
How Is Malignant Atrophic Papulosis Diagnosed?
A exhaustive clinical evaluation is required for the diagnosing of Malignant Atrophic Papulosis or Degos Disease . The characteristic lesion found on the open of the skin may point towards a diagnosis of it .
If the patient has a direct home member with a anterior history of Malignant Atrophic Papulosis , then that may also point towards a diagnosing of it .
A microscopical analysis of the skin lesion may disclose the typical feature seen in this condition and support the diagnosis of it . Since this condition is extremely rare and most standard lab tests reveal normal results , there is not one specific trial design for diagnose it .
How Is Malignant Atrophic Papulosis Treated?
There is no specific treatment so far to treat Malignant Atrophic Papulosis or Degos Disease . The treatment is essentially diagnostic and supportive . In vitrine where the GI tract is involved , then periodic monitoring may be recommended to expect for any signs of perforation or other complications which may be potentially serious . If such a knottiness is identified then operative subprogram may be ask to even out such atmospheric condition . Other than this , only periodic monitoring is what is done in bulk of the instance of Malignant Atrophic Papulosis or Degos Disease .
